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Textbooks: Nelson Essentials of Pediatrics, Pediatric Secrets, First Aid for Pediatric Clerkship

--- Common Problems in Pediatrics

Preventative: Immunizations, Normal Growth and Development Cough: URI, Asthma, Pneumonia, Bronchiolitis, Allergic Rhinitis

Fever: Common Viral Illnesses, UTI, Occult Bacteremia, Meningitis, Febrile Seizures Sore Throat: Group A Beta-Hemolytic Streptococcal Pharyngitis, Mononucleosis Ear Pain: Otitis Media, Otitis Externa

Abdominal Pain: Gastroenteritis, UTI, PID, Functional Abdominal Pain

Dermatitis: Atopic Dermatitis, Viral Exanthems, Impetigo, Monilial and Tinea Infections, Scabies Heart Murmurs: Innocent Murmurs, Septal Defects

Developmental: Developmental Delay, Failure to Thrive Hematology: Sickle Cell Disease, Thalassemias

Nephrology: UTI, Nephrotic/Nephritic Syndromes, Proteinuria

Chronic: Allergies, Asthma, Cerebral Palsy, Cystic Fibrosis, Diabetes Mellitus, Seizure Disorders

--- Procedures: NEJM Videos In Clinical Medicine: http://www.nejm.org/multimedia/videosinclinicalmedicine --- How To Succeed – First Aid For The Pediatrics Clerkship (Stead, Stead, & Kaufman)

Be On Time: Most wards teams begin rounding around 8am. Give yourself at least 15 minutes per patient for pre-rounding to learn about events that occurred overnight or lab/imaging results.

Dress In A Professional Manner: Regardless of what the attending wears. A short white coat should be worn over your professional dress clothes unless it is discouraged.

Act In A Pleasant Manner: The medical rotation is often difficult, stressful, and tiring. Smooth out your experience by being nice to be around. Smile a lot and learn everyone’s name. Don’t be afraid to ask how your resident’s weekend was. If you do not understand or disagree with a treatment plan or diagnosis, do not “challenge.” Instead, say “I’m sorry, I don’t quite understand, could you please explain...” Show kindness and compassion toward your patients. Never participate in callous talk about patients.

Take Responsibility: Know everything there is to know about your patients: their history, test results, details about their medical problem, and prognosis. Keep your intern or resident informed of new developments that they might not be aware of, and ask them for any updates you might not be aware of. Assist the team in developing a plan; speak to radiology, consultants, and family. Never give bad news to patients or family members without the assistance of your supervising resident or attending.

Respect Patient’s Rights:

1) All patients have the right to have their personal medical information kept private. This means do not discuss the patient’s information with family members without that patient’s consent, and do not discuss any patient in

hallways, elevators, or cafeterias.

2) All patients have the right to refuse treatment. This means they can refuse treatment by a specific individual (you, the medical student) or of a specific type (no nasogastric tube). Patients can even refuse life-saving treatment. The only exceptions to this rule are if the patient is deemed to not have the capacity to make decisions or understand situations, in which case a health care proxy should be sought, or if the patient is suicidal or homicidal.

3) All patients should be informed of the right to seek advanced directives on admission. Often, this is done by the admissions staff, in a booklet. If your patient is chronically ill or has a life-threatening illness, address the subject of advanced directives with the assistance of your attending.

More Tips: Volunteer, be a team player, be honest, and keep patient information handy.

Present In An Organized Manner: “This is a [age] year old [gender] with a history of [major/pertinent history such as asthma, prematurity, etc. or otherwise healthy] who presented on [date] with [major symptoms, such as cough, fever, and chills], and was found to have [working diagnosis]. [Tests done] showed [results]. Yesterday/ overnight the patient [state important changes, new plan, new tests, new medications]. This morning the patient feels [state the patient’s words], and the physical exam is significant for [state major findings]. Plan is [state plan].”

On Outpatient: The ambulatory part of the pediatrics rotation consists of mainly two parts: focused histories and physicals for acute problems and well-child visits. Usually, you will see the patient first, to take the history and do the physical exam. It is important to strike a balance between obtaining a thorough exam and not upsetting the child so much that the attending won’t be able to recheck any pertinent parts of it. For acute cases, present the patient distinctly, including an appropriate differential diagnosis and plan. In this section, be sure to include possible etiologies, such as specific bacteria, as well as a specific treatment (e.g., a particular antibiotic, dose, and course of

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treatment). For presentation of well-child visits, cover all the bases, but focus on the patients’ concerns and your findings. There are specific issues to discuss depending on the age of the child. Past history and development is important, but so is anticipatory guidance–prevention and expectations for what is to come. The goal is to be both efficient and thorough.

--- Top 100 Secrets – Pediatric Secrets (4th, Polin & Ditmar)

1) Methods to increase compliance by adolescents with medical regimens include the following: simplifying the regimen, making the patient responsible, discussing potential side effects, using praise liberally, and educating the patient.

2) A pelvic examination is not required before prescribing oral contraceptives for teenagers without risk factors. Appropriate screening for sexually transmitted diseases and possible cervical dysplasia can be scheduled, but delaying oral contraception unnecessarily increases the risk of pregnancy.

3) Emergency contraception should be discussed with all sexually active adolescents; 90% of teenage pregnancies are unintended.

4) Teenagers with attention deficit hyperactivity disorder (ADHD) and conduct disorders are at high risk for substance abuse disorders. Substance abuse is often associated with comorbid psychiatric disorders.

5) Calluses over the metacarpophalangeal joints of the index and/or middle fingers (Russell sign) may indicate repetitive trauma from self-induced attempts at vomiting in patients with eating disorders.

6) Appreciating that ADHD is a chronic condition (like asthma or diabetes) is useful for management strategies, follow up, and ongoing patient/parental education and involvement.

7) Although colic is common and resolves spontaneously by 3 months, do not underestimate the physical and psychological impact of the condition on a family.

8) Bilingual children develop speech milestones normally; two-language households should not be presumed as a cause of speech delay.

9) Most amblyopia is unilateral; vision testing solely with both eyes open is inadequate. 10) Congenitally missing or misshapen teeth can be markers for hereditary syndromes.

11) Syncope in a deaf child should lead one to suspect prolongation of the QT wave on the electrocardiogram. 12) Bounding pulses in an infant with congestive heart failure should cause one to consider a large patient ductus arteriosus.

13) If a bruit is heard over the anterior fontanel in a newborn with congestive heart failure, suspect a systemic arteriovenous fistula.

14) The chief complaint in a child with congestive heart failure may be nonspecific abdominal pain. 15) Diastolic murmurs are never innocent and deserve further cardiac evaluation.

16) Patients with atypical Kawasaki disease (documented by coronary artery abnormalities despite not fulfilling classic criteria) are usually younger (<1 year old) and most commonly lack cervical adenopathy and extremity changes.

17) Neonates with midline lumbosacral lesions (e.g., sacral pits, hypertrichosis, lipomas) should have screening imaging of the spine performed to search for occult spinal dysraphism.

18) Hemangiomas in the "beard distribution" may be associated with internal airway hemangiomas. 19) Infantile acne necessitates an endocrine workup to rule out precocious puberty.

20) If a child develops psoriasis for the first time or has a flare of existing disease, look for streptococcal pharyngitis.

21) Look for associated autoimmune thyroiditis in children who present with a family history of thyroid disease and extensive alopecia areata or vitiligo.

22) Most cardiac arrests in children are secondary to respiratory arrest. Therefore, early recognition of respiratory distress and failure in children is crucial.

23) Because children are much more elastic than adults, beware of internal injuries after trauma; these can occur without obvious skeletal injuries.

24) Because children get colder faster than adults as the result of a higher ratio of body surface area to body mass, be sure that hypothermia is not compounding hemodynamic instability in a pediatric trauma patient in shock.

25) Hypotension and excessive fluid restriction should be avoided at all costs in the child in shock with severe head injury because such a patient is highly sensitive to secondary brain injury from hypotension.

26) The most common finding upon the examination of a child's genitalia after suspected sexual abuse is a normal examination.

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27) Because the size of a normal hymenal opening in a prepubertal child can vary significantly, the quality and smoothness of the contours of the hymenal opening, including tears and scarring, are more sensitive indicators of sexual abuse.

28) Palpation for an enlarged or nodular thyroid is one of the most overlooked parts of the pediatric physical examination in all age groups.

29) Because 20-40% of solitary thyroid nodules in adolescents are malignant, an expedited evaluation is needed if a nodule is discovered.

30) Unless a blood sugar level is checked, the diagnosis of new-onset diabetic ketoacidosis can be delayed because abdominal pain can mimic appendicitis, and hyperventilation can mimic pneumonia.

31) Beware of syndrome of inappropriate antidiuretic hormone secretion and possible cerebral edema if a normal or low sodium level begins to fall with fluid replenishment during the treatment of diabetic ketoacidosis.

32) Acanthosis nigricans is found in 90% of youth diagnosed with type 2 diabetes.

33) Growth hormone deficiency present during the first year of life is associated with hypoglycemia; after the age of 5 years, it is associated with short stature.

34) Fecal soiling is associated with severe functional constipation.

35) More than 40% of infants regurgitate effortlessly more than once a day.

36) Nasogastric lavage is a simple method for differentiating upper gastrointestinal bleeding from lower gastrointestinal bleeding.

37) Conjugated hyperbilirubinemia in any child is abnormal and deserves further investigation.

38) Potential long-term complications of pediatric inflammatory bowel disease include chronic growth failure, abscesses, fistulas, nephrolithiasis, and toxic megacolon.

39) Bilious emesis in a newborn represents a sign of potential obstruction and is a true gastrointestinal emergency. 40) In patients with Down syndrome and behavioral problems, do not overlook hearing loss (both sensorineural and conductive); it occurs in up to two thirds of patients with this condition, and it can be a possible contributor to those types of problems.

41) Fluorescence in situ hybridization (FISH) is indicated for the rapid diagnosis of trisomies 13 and 18 and multiple syndromes in children with moderate to severe mental retardation and apparently normal chromosomes

(subtelomeric FISH probes).

42) Three or more minor malformations should raise concern about the presence of a major malformation. 43) The diagnosis of fetal alcohol syndrome is problematic in infants because facial growth and development can modify previously diagnostic features over a 4- to 6-year period.

44) Diabetes mellitus is the most common teratogenic state; insulin-dependent diabetic mothers have infants with an eight-fold increase in structural anomalies.

45) An infant with nonsyndromic sensorineural hearing loss should be tested for mutations in the connexin 26 gene. Mutations in that gene contribute to at least about 50% of autosomal recessive hearing loss and about 10-20% of all prelingual hearing loss.

46) In children <12 years old, the lower limit of normal for the mean corpuscular volume (MCV) can be estimated as 70 + (the child's age in years)/mm3. For a patient that is more than 12 years old, the lower limit for a normal MCV is 82/mm3.

47) In the setting of microcytosis, an elevated red blood cell distribution width index suggests a diagnosis of iron deficiency rather than thalassemia.

48) After iron supplementation for iron-deficiency anemia, the reticulocyte count should double in 1-2 weeks, and hemoglobin should increase by 1 gm/dL in 2-4 weeks. The most common reason for persistence of iron deficiency anemia is poor compliance with supplementation.

49) Children with elevated lead levels are at increased risk for iron deficiency anemia because lead competitively inhibits the absorption of iron.

50) Chronic transfusion therapy to reduce sickle hemoglobin levels to 30-40% of the total lowers the likelihood of stroke.

51) Because 30% of patients with hemophilia have no family history of the disorder, clinical suspicion is important in the presence of excessive and frequent ecchymoses.

52) Marked neutropenia (<500/mm3 absolute neutrophil count) in a previously healthy child often heralds the onset of overwhelming sepsis.

53) The determination of immunoglobulin G subclass concentrations is meaningless in children who are less than 4 years old.

54) Neutrophil deficiency should be considered in a newborn with a delayed separation of the umbilical cord (>3 weeks).

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55) Clinical features of autoimmunity do not exclude the diagnosis of a primary immunodeficiency.

56) A male child with a liver abscess should be considered to have chronic granulomatous disease until it is proven otherwise.

57) The most common congenital infection is cytomegalovirus, which in some large screening studies occurs in up to 1.3% of newborns, although most of these infants remain asymptomatic.

58) Up to 25% of infants <28 days old with bacterial sepsis and positive blood cultures will have culture-confirmed meningitis.

59) Erythematous papules with a pale center ("doughnut lesions") located on the hard and soft palates are pathognomonic for streptococcal pharyngitis.

60) The red man syndrome, which is a complication of vancomycin administration, can usually be avoided by slowing the rate of drug infusion or by premedicating with diphenhydramine.

61) A petechial-purpuric rash in a glove-and-stocking distribution should raise the possibility of infection with parvovirus B19.

62) Perinatal asphyxia accounts for less than 15% of cases of cerebral palsy.

63) Because primary and secondary apnea are indistinguishable in newborns, the initial clinical response should be identical in the delivery room.

64) Hyperbilirubinemia is generally not an indication for the cessation of breast-feeding but rather for increasing its frequency.

65) Sepsis is in the differential diagnosis of virtually every neonatal sign and symptom. 66) Breast feeding lowers the risks of necrotizing enterocolitis and nosocomial sepsis.

67) Ten percent of febrile infants with documented urinary tract infections have normal urinalyses; this emphasizes the importance of obtaining a urine culture if clinical risk factors are present.

68) Vigorous correction of constipation has been shown to diminish both enuresis and the frequency of urinary tract infections.

69) Chromosomal and endocrinologic evaluation should be done if testes are bilaterally undescended and nonpalpable or one or two testicles are undescended with hypospadias present.

70) In patients with acute renal failure, the measurement of urinary indices (urine sodium concentration, fractional excretion of sodium, urine specific gravity, and osmolality) should be done before initiating any therapy to help distinguish between prerenal, renal, and postrenal etiologies.

71) The two most productive facets of patient evaluation to explain renal disease as a possible cause of symptoms are as follows: (1) the measurement of blood pressure and (2) the examination of the first morning void after the bladder is emptied of urine stored overnight (when a specimen is most likely to be concentrated).

72) The most common cause of persistent seizures is an inadequate serum antiepileptic level.

73) Antiepileptic drugs in tablet and capsule form produce less variation in blood concentrations than liquid preparations, particularly suspensions, do.

74) Resist polypharmacy: three or more medications have not been shown to improve seizure control as compared with one or two drugs, and side effects and compliance become much more problematic.

75) The diagnosis of cerebral palsy is rarely made at <1 year old because neurologic findings in infancy are subject to significant change.

76) Migraine headaches are usually bilateral in children but unilateral (75%) in adults.

77) Seizures with fever in patients older than 6 years of age should not be considered febrile seizures.

78) Children with fever and neutropenia must continue to receive broad-spectrum antibiotics until definitive signs of marrow recovery are documented, typically with the presence of a peripheral monocytosis and an absolute

neutrophil count >200/mm3 and rising.

79) Empiric antifungal agents are administered to children with neutropenia who remain febrile or develop new fever within 3 to 7 days of starting broad-spectrum antibiotics because the risk of invasive fungal infection increases with the duration and depth of neutropenia.

80) After age and white blood cell count, early response to therapy is the most important prognostic feature for children with acute lymphoblastic leukemia.

81) Leukemias and lymphomas that have a high proliferation and cell turnover rate (e.g., Burkitt's lymphoma, T-cell lymphoblastic leukemia) place patients at the highest risk of complications from tumor lysis syndrome.

82) Eighty percent or more of patients who present with acute lymphoblastic leukemia have a normochromic, normocytic anemia with reticulocytopenia.

83) Because it changes more quickly as inflammation changes, C-reactive protein is better than sedimentation rate for monitoring the response to therapy in patients with osteomyelitis.

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84) Pseudoparalysis (with decreased arm or leg movement) with no systemic illness may be a presenting sign in an infant with osteomyelitis.

85) Back pain is atypical for scoliosis and may point to another diagnosis.

86) Consider magnetic resonance imaging for patients with scoliosis and the less common left-sided thoracic curves because 5-7% of these patients can have intraspinal abnormalities (e.g., hydromelia).

87) A plain x-ray is unreliable in the diagnosis of developmental dysplasia of the hip in infants less than 6 months of age because ossification of the femoral head is incomplete.

88) Older children with unexplained unilateral deformities (e.g., pes cavus) of an extremity should have screening magnetic resonance imaging to evaluate for intraspinal disease.

89) Asthma rarely causes clubbing in children. Consider other diseases, particularly cystic fibrosis.

90) Most children with recurrent pneumonia or persistent right middle lobe atelectasis have asthma. But … all that wheezes is not asthma.

91) Home peak flow monitoring is most helpful in those asthmatic patients with very labile disease or poor symptom recognition.

92) A normal respiratory rate strongly argues against a bacterial pneumonia.

93) Upper lobe pneumonias with radiation of pain to the neck can cause meningismus and mimic appendicitis; lower lobe pneumonias can present with abdominal pain.

94) Nasal polyps or rectal prolapse in children suggests cystic fibrosis.

95) The three most common causes of anaphylaxis in pediatric hospitals and emergency departments are latex, food, and drugs. Suspected allergies to shellfish, peanuts, and nuts warrant a prescription for an epinephrine pen because of the increased risk of future anaphylaxis.

96) Up to 10% of normal, healthy children may have low-level (1:10) positive-antinuclear antibody (ANA) testing that will remain positive. Without clinical or laboratory features of disease, it is of no significance.

97) The daily spiking fevers of systemic juvenile rheumatoid arthritis can precede the development of arthritis by weeks to months.

98) Antistreptolysin O antibodies are positive in only 80% of patients with acute rheumatic fever. Test for anti-DNase B antibodies to increase the likelihood to more than 95% when diagnosing a recent group A beta-hemolytic infection.

99) Because up to 10% of patients can have asymptomatic Borrelia burgdorferi infection and because both

immunoglobulin M and immunoglobulin G antibodies to B. burgdorferi can persist for 10-20 years, the diagnosis of Lyme disease in older children and adolescents can be tricky in patients with atypical clinical presentations. 100) Abdominal pain (mimicking an acute abdomen) and arthritis can frequently precede the rash in Henoch-Schönlein purpura disease and thus confuse the diagnosis.

--- Kaplan Videos (2001) – Neonatology with Dr. Eduardo Pino, MD

---Newborn Skin

* Newborn infant at birth is noted to have acrocyanosis, a heart rate of 140, grimaces to stimulation, and is active with a lusty cry. What is her APGAR score?

* APGAR score 0: no heart rate, no respirations, no muscle tone, no reflex irritability (e.g. bulb suction), blue body. * APGAR score 1: HR < 100, weak cry, some extremity flexion, some reflex motion, pink body blue extremities. * APGAR score 2: HR > 100, vigorous cry, arms/legs flexed, reflex cry/withdrawal, pink all over.

* Virginia Apgar, an anesthesiologist, developed the APGAR score. Useful tool for immediate assessment of the child. It is not used for later in life, only at birth. Useful for determining progression of resuscitation.

* APGAR is for appearance, pulse, grimace, activity, and respirations.

* Score 0 to 3 at one minute requires resuscitation. Do not wait for one minute though. 4 can be poor also. * Poor score at 5 minutes does not predict subsequent cerebral palsy. Poor score at 20 minutes predicts higher morbidity and mortality.

* Score 8 to 10 is good. Scores 5 to 7 are fair. Newborn is any child under the age of 28 days.

* Newborn infants has a blue-gray pigmented lesion on the sacral area. It is clearly demarcated and does not fade into the surrounding skin. What is the most likely diagnosis? Answer is Mongolian spot. They are more commonly seen in dark-skinned races. Up to 5% of Caucasian newborns will have it.

* Differential diagnosis includes child abuse, so document Mongolian spots to prevent subsequent issues. Bruises will fade into surrounding skin; in that case consider child abuse. Mongolian spots can occur on any part of the body, typically seen on the buttocks or sacral area. They fade in months to a year.

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* Erythema toxicum is very common in newborns and should be differentiated from staphylococcal scalded skin syndrome (SSSS). Erythema toxicum does not appear in the first day of life, usually shows in 2-3 days. It is characterized by erythematous macules that can become blisters or pustules. It is migratory, such that the next day there may be clearing in some spots and new erythema in other spots.

* Pustules of erythema toxicum neonatorum (red rash of the newborn), if scraped, will contain many eosinophils. * Staphylococcal scalded skin may be present in the first day of life, may present with pustules, skin will start to peel off. If you scrap it, it will form a blister like a burn (Nikolsky sign). Pustules will be full of neutrophils. * SSS babies will be ill, toxic in appearance, can have sepsis.

* Treat erythema toxicum with reassurance of the parents.

* Sebaceous gland hyperplasia looks like lots of little whiteheads, in areas that are more oily like nose. Treatment is to leave them alone.

* Milia comes in fine-white (miliaria crystallina) and red (miliaria rubra). Benign.

* Ebstein pearls are commonly seen in the mouth (mucous membranes) along the midline, just a collection of stratified epithelium and tends to go away. Do not confuse with torus palatinus, an actual deformity of the hard palate where it curves down. That is permanent and can be covered by mucous membrane, cause no issues. * Cutis marmorata looks like cobblestone blood-vessels. Can occur when the baby is cold. It is secondary to

vasomotor instability. As they get older, it gets better. Cutis marmorata telangiectasia congenita does not go away as the child gets older. Cutis marmorata sometimes seen in Down syndrome.

* Neonatal acne (acne neonatorum) is a heightened receptor response to circulating estrogens. Usually shows around a week to two weeks. Goes away, no need to treat with acne medications.

* Newborn has a flat salmon-colored lesion on the glabella, which becomes darker red when he cries. What is the best course of management? This is a salmon patch, also known as nevus flammeus. Best course of management is reassurance of parents. This goes away as the child gets older. Nevus simplex is also known as “stork bite.” * Salmon patch seen over the eyelids, bridge of the nose, and back of the neck. Also known as a “stork bite” (on the neck) or “angel kisses” (on the forehead/nose).

* Nevus flammeus divided into salmon patch (goes away) and port-wine stain (does not go away, hemangioma, associated with Sturge-Weber syndrome. 50% of babies are born with salmon patch. Facial lesions tend to go away, neck lesions may stay but are covered with hair.

* Capillary hemangioma, also known as strawberry hemangioma usually starts as flat macular lesion. May get larger during first few months of age, raised lesion. After first year of life, they tend to regress slowly. In general, there is no need to go cut them out as this results in bleeding since they are a collection of blood vessels. No need to do laser treatment either. Only do treatment if the lesion is over a vital structure.

* Baby presents with capillary hemangioma on the skin and stridor. Think about subglotic hemangioma. * As capillary hemangiomas get better, they start to turn bluish and get boggy like an abscess.

* Nevus sebaceous (nevus of Jadassohn) is present at birth. It is salmon fleshy colored and there will be no hair growth coming through the lesion. They are only seen on the scalp. Treatment is to follow until adolescent age and then remove. There is some minor risk of malignancy, so remove after child is no longer growing.

* Café-au-lait are light-brown in color (coffee with milk) and also known as “giraffe spots.” They may or may not be associated with underlying diseases (neurofibromatosis, McCune Albright syndrome, Von Hipple Lindau). * Harlequin baby will have redder skin closer to the ground/gravity. This is due to vasomotor instability.

---Birth Trauma

* Cephalhematoma is a collection of fluid underneath the bone. Must be differentiated from caput hematoma. A cephalhematoma is a subperiosteal bleed, since it is beneath the bone it is limited by the bone, thus it will not cross the suture lines. A caput is a scalp swelling so it does cross suture lines.

* Cephalhematomas tend to get worse over a few days and can take weeks to months to resolve. As the resolve, you can feel the volcano rim or crater, which is the edge of the cephalhematoma. Even if they are bilateral, they will not cross the suture line so you should be able to feel a groove in between the hematomas.

* Caput hematomas starts to get better as soon as the baby is delivered.

* Differential diagnosis of a cephalhematoma includes a depressed skull fracture.

* Subcutaneous fat necrosis is a type of birth injury. Associated with birth trauma or forceps use. Will be firm rubbery nodules, can be seen anywhere like cheeks, buttocks, back, extremities.

* Brachial palsies occur with stretching of the brachial plexus, such as forceps or arm pulling.

* Erb-Duchenne palsy involves C5-C6 and arm will be internally rotated, wrist flexed, “waiter’s tip” or “secret smoker.” Ipsilateral hemi-diaphragmatic paralysis means C4 is also affected.

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* Klumpke palsy involves C8-T1 and hand will have fingers flexed, “claw hand.” If sympathetic fibers of T1 are affected the child may have a Horner syndrome. Horner is anhidrosis, ptosis, myosis. Horner is a guy whose forehead is dry, can’t see the sky, and has a small eye.

* Facial palsy will have no ipsilateral movement with crying. These palsies tend to be fairly mild and resolve. * Clavicle is the most commonly fractures bone during delivery. Babies tend to be large for gestational age, such as when the mother has gestational diabetes. Shoulder dystocia during delivery predisposes to fracture. Baby may not move their arm well or has an asymmetric Moro response or mother notices a lump (callous already forming). For the most part, these fix themselves, no need to splint in a figure-of-eight. May feel crepitus on affected side. * Subconjunctival hemorrhages are temporary. You see blood in the eye, due to birth pressure.

---Congenital Anomalies

* Coloboma is a defect in the iris, may look like a keyhole, also known as “latch key eye”. Can have a coloboma of the eyelid, choroid, or retina.

* Aniridia is lack of iris, usually bilateral. Baby will not have a colored part of the eye. Aniridia plus hemi-hypertrophy is associated with Wilms tumor.

* Congenital cataract will have no red-reflex on the affected side. Eye appears cloudy, there is an opacity in the lens. Baby may be born with cataract or be developing it due to something like galactosemia.

* White reflex implies retinoblastoma until proven otherwise. Red reflex and be red or orange or tan.

* Pre-auricular skin tags are generally a normal variant. There is a slightly higher association with cleft lip/palate. * Pre-auricular dimple or pit is a normal variant. There is a slightly higher association with hearing abnormalities. * Microtia is grossly malformed misshapen ears. Much higher association with renal abnormalities (e.g. Potter). * Macroglosia is huge tongue, can obstruct the airway and cause feeding difficulties. Can be seen in Down syndrome, Beckwith-Wiedemann Syndrome, or a normal variant.

* Ankyloglossia also known as “tongue tie” at the bottom of the tongue. Generally no need to intervene. Do not snip the attachment as there is an artery that runs through this area. If baby can get tongue to edge of gums (most can) they will be able to nurse well and speak well.

* Branchial cleft cysts are generally unilateral and can become infected, drain, require antibiotics, and sometimes need to be closed or removed.

* Congenital torticollis also known as “wry neck” is balling-up of sternocleidomastoid. Child will keep its head to the side. You may be able to feel a knot on that side of the neck. Treat with passive range of motion, moving head to opposite side. Some torticollis patients may have a hemi-vertebra in the neck associated with certain syndromes. * Breast hypertrophy is due to heightened response of receptors to circulating hormones. It goes away as the baby gets older. There may even be discharge from the breast.

* Supernumerary nipples (polythelia) will be anywhere along the mammary “milk” lines. There is an association with renal and cardiovascular anomalies. Most people with polythelia do not have problems though.

* Poland syndrome is absence of the pectoralis muscle with amastia on that side, can have rib deformities, webbed fingers, and radial nerve aplasia.

* Pectus excavatum also known as “funnel chest” and pectus carinatum also known as “pigeons chest”. These are normal variants and should be left alone. Very rarely do they cause cardiac problems, such as cor pulmonale from pectus excavatum. Most common reason for having them fixed is cosmetic.

* Polycystic kidney is the most commonly palpated mass in the abdomen of a neonate. Next most common is likely a large bladder, maybe secondary to posterior urethral valves.

* Umbilical hernias are commonly seen. This is incomplete closure of the fascia umbilical ring. Old school myth was to put a large coin on it and tape it down. Treatment is to leave it alone. After about a year, they will have closed either way (coin taping or leaving it alone). Nearly all close by age 5.

* Omphalocele is due to embryologic development. Intestines come out in-utero, do 270-degree flip, and come back in. In omphalocele, the intestines did not come back in. There is a sac “-cele” with this deformity. This is a midline defect and is a surgical emergency. Generally, this is diagnosed on ultrasound so the surgical team is ready at birth. * Gastroschisis does not have a sac. This is usually to the right of the umbilicus. Since it is not contained, there is a much higher risk for volvulus and malrotation. More likely to have ischemia. Surgical emergency.

* Imperforate anus can occur. Never take a newborn’s temperature with a rectal thermometer as this could perforate the anus. Symptoms include no defecation within the first 24 hours of life.

* Epispadias is urethral meatus opening on the dorsum of the penis. Hypospadias is on the ventral side. Right below the tip of the glans is a first-degree, along the shaft is a second-degree, and at the base of the shaft is third-degree hypospadias. Usually there is a hooded prepuce seen in hypospadias or isolated chordee. Hypospadias is a contraindication for circumcision as all the tissue will be needed for the repair.

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* Retractile testicle means there is an active cremasteric reflex, where testicle slides up with touching or cold temperature. Undescended testicle means it is not palpable or is palpable in the inguinal canal but cannot be brought down. If it is undescended at one year of age, it has to be surgically brought down as there is a higher risk of malignancy and will become atrophic.

* Ebstein pearls can also occur on the penis; anywhere with mucous membranes.

* Hydrocele suspected with enlarged scrotum. Can be transilluminated. Hernias reduce, hydroceles do not. * Syndactyly is when fingers do not separate in development. X-ray to determine if 1 or 2 fingers. If 2 fingers, surgery may be considered.

* Polydactyly is extra fingers. If there is a well developed bone with vascular supply, consider leaving finger. If no bone present in extra finger or just a stalk, tie-off with a suture and it will auto-amputate.

* Amniotic band occurs when there is a little tear in the amnion in-utero and the baby gets it’s finger through. When the amnion heals up, it compresses and creates a band. Could cause phocomelia where entire arm is deformed. ---Neonatal Screening Tests

* One-month old fair-haired fair-skinned baby presents with projectile vomiting of 4-days duration. Physical exam reveals a baby with eczema and a musty (mousy) odor. Which screening test would most likely be abnormal? This baby presents similar to pyloric stenosis. This is phenylketonuria (PKU).

* Major three for screening are PKU, galactosemia, hypothyroidism. Some places will test for maple syrup urine disease or sickle cell anemia.

* PKU is a defect in the hydroxylation of phenylalanine to tyrosine. It is autosomal recessive. What are the chances that this family will have another affected child? Answer is 1 in 4. Occurs in about 1:10,000 live births.

* In PKU, babies are normal at birth. Mental retardation is the most common manifestation. Commonly seen in fair-haired, fair-skinned, blue-eyed population. Rash looking like atopic dermatitis or eczema may be present.

* Screening test for PKU is best done at 48-72 hours after starting to take in protein.

* If positive PKU screening test, then do blood levels of phenylalanine (high) and tyrosine (normal levels). * Treatment for PKU is dietary, low phenylalanine formula and then low phenylalanine diet. Avoid things like aspartame (sugar substitute), which is common in diet drinks.

* Complications include mental retardation, microcephaly, and congenital heart disease.

* Galactosemia is a defect in galactose-1-phosphate-uridyltransferase. Patients are unable to metabolize galactose and the levels will accumulate in the kidney, liver, and brain. It is autosomal recessive.

* Duarte variant of galactosemia is asymptomatic, no clinical significance.

* Babies will have a variety of symptoms including vomiting, jaundice, hypoglycemia, seizures, cataracts (due to galactitol in lens), enlarged liver or spleen, gain weight poorly, high risk of E. coli sepsis.

* Treatment is to eliminate galactose from the diet. One of the few contraindications to breast feeding, should use soy formula. Although they are treated, babies tend not to do as well, may have developmental delay or speech and learning problems.

---Maternal Diabetes

* You are called to see a 9.5lb newborn infant who is jittery. Physical exam reveals a large plethoric (red, ruddy) baby who is tremulous. A murmur is heard. Blood sugar is low. This is a baby born to a mother with diabetes or who developed gestational diabetes.

* Babies tend to be macrosomic (big baby). Babies tend to develop hypoglycemia because they have high circulating levels of sugar in-utero so the baby overproduces insulin. Once born, the increased insulin and lack of maternal sugar leads to hypoglycemia. Insulin works as a growth hormone, so not only is the baby getting lots of calories it is hormonally enhanced in size.

* Since they are large for gestational age, they can get birth trauma such as broken clavicle or shoulder dystocia. * Along with hypoglycemia, they can get hypocalcemia and hypomagnesemia. Sometimes they will have respiratory distress syndrome because insulin can block surfactant production.

* Infants of diabetic mothers are at higher risk for hypertrophic cardiomyopathy. In general, they will get better over time, by about 6 months of age.

* Infants can get hyperbilirubinemia and polycythemia. At higher risk for other congenital anomalies like ventricular septal defects, atrial septal defects, and transposition of the great arteries. At higher risk for lumbosacral agenesis and specifically small or lazy left colon, which can appear like Hirschsprung or meconium ileus.

* Treatment is to control mother’s blood sugar while fetus is in-utero. After birth monitor the babies and treat hypoglycemia until they adjust their insulin levels.

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--- Size For Gestational Age

* Most scales to determine if child is appropriate for age include a physical scale and neuromaturation scale. * Ballard scale (Dubowitz is another), looks at muscular maturity, posture, skin, lanugo, plantar creases, breast development, ear stiffness, genitalia, etc.. Repeat exam 24 hours later, helps compensate for things like depressant medications taken by mother. Premature babies will have floppy ears because stiff cartilage has not developed. * Small for gestational age does not matter if baby is pre-term, term, or post-term. Only says if the size is appropriate for that particular gestational age.

* Shiny skin in a baby implies little subcutaneous tissue, so there was not enough time to develop the tissue. * Labia majora being wide and not covering labia minora is also a sign of prematurity.

* As male gets to term, scrotum gets darker, more wrinkles, and testes descend into scrotum. * Lots of lanugo hair implies pre-term.

* Flexion a both hips and knees is a good sign. Arm flexion as well. Lusty cry is good.

* Most babies start to peel at two-weeks of age. No need to put lotion on it, leave skin alone. A post-term baby may have peeling at birth and longer nails.

* Term infant weights 4lbs at birth. Physical exam reveals a small infant with a disproportionally large head. The mother has a history of smoking during pregnancy (risk of prematurity, IUGR). This baby is small for gestational age, intrauterine growth restriction (IUGR). Small for gestational age is below the third percentile for that particular gestational age.

* Symmetric means the baby is the same proportions. Asymmetric is when the head is bigger than the body, these babies have a better prognosis. It implies that the brain has been spared.

* IUGR is associated with any factors that decrease oxygenation to fetus including chromosomes, TORCH infections, congenital anomalies, irradiation, insulin deficiency. Placental factors include small placenta, infracted placenta, partial abruption, twin to twin transfusion. Maternal factors include toxemia of pregnancy, hypertension, malnutrition, tobacco use, narcotic use, alcohol use.

* Ballard scoring helps to determine gestational age, then weight and plot out baby.

* IUGR babies are at higher risk of cold stress (not enough fat) and hypoglycemia (no glycogen stores). * Small for gestational age (SGA) babies at risk for polycythemia because they have more hypoxia and produce more hemoglobin and cells.

--- Neonatal Drug Withdrawal

* A two day old infant is noticed to have course jitters and is very irritable, with a high-pitched cry. A low grade fever is reported as well as diarrhea. Maternal history is positive for heroin use.

* Moms will say “as soon as I heard I was pregnant I quit doing drugs.” Do not believe them, drug test. * Most common elicit drugs that a baby goes through withdrawal from are narcotics and cocaine. * You can urine drug screen the baby as well.

* Heroin has a shorter half-life than methadone so the babies will withdraw sooner. Heroin a couple of days, methadone a couple of weeks.

* Hyperactivity, irritability, fever, diarrhea (classic), fussy baby, inconsolable, always sucking, think withdrawal. * Phenobarbital takes a couple of weeks for withdrawal symptoms.

* Treatment is put child in long acing narcotics (e.g. methadone) and slowly wean baby. Minimize stimulus, swaddle baby, wrap up baby they like to be held tightly and closely.

* Complications of neonatal drug withdrawal is low birth weights, higher risk for anomalies, higher risk for sudden infant death syndrome, and higher risk for mother’s complications of drug use (e.g. hepatitis, HIV).

--- Respiratory Diseases

* Shortly after birth, a 33 week gestation infant develops tachypnea, nasal flaring, grunting, and requires intubation. Chest radiograph shows a hazy ground-glass appearance of the lungs. Suspect respiratory distress syndrome (RDS). This is secondary to surfactant deficiency, seen almost exclusively in preterm babies.

* Surfactant decreases surface tension, preventing alveoli from collapsing. So lack of surfactant causes alveoli to collapse leading to atelectasis. The atelectasis leads to the ground-glass haziness.

* Blood gases will be poor due to ventilation-perfusion mismatch caused by the atelectasis.

* Valsalva maneuver increases pressure in the chest and helps to keep alveoli open. When child needs to let out the air to breath in, they will grunt.

* Usually it takes about three days for child to get better. One of the first signs of improvement is dieresis. No real diagnostic test for RDS, more of a clinical diagnosis with help from the x-ray. Aside from the poorly demarcated

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heart border and haziness, there may be air bronchograms.

* Treatment of RDS starts with prevention, getting baby to term and giving mother steroids to help mature the babies lungs. Can treat newborn with surfactant, ventilate if needed, give fluids if needed, antibiotics if needed. * Complications include pneumothorax, plugging, intraventricular hemorrhages, sepsis, pulmonary interstitial emphysema, chronic lung disease (bronchopulmonary dysplasia).

* Transient tachypnea of the newborn (TTN), similar to RDS symptoms but with a term baby. Associated with a rapid second stage of labor or Cesarean section as baby does not get the birth squeeze that helps production of surfactant. Baby will have tachypnea, may require a little oxygen, fairly clear x-ray, tends to go away in a few days. * Meconium aspiration syndrome has symptoms of respiratory distress but with history of meconium-stained amniotic fluid. Seen in term babies. Rupture membranes with meconium is a clue, meconium under the baby’s fingernails or around the umbilicus is another clue. CXR is typical of aspiration pneumonia. Meconium is like thick pudding, supposedly sterile but can cause pneumonitis and air-trapping if it get into the lungs.

* Air-trapping in the lungs leads to hypoxia. This causes vasodilation everywhere in the body except the lungs. In the lungs, hypoxia causes vasoconstriction, leading to shunting. In newborn, the shunting goes through a patent ductus arteriosus or foramen ovale. This causes more deoxygenated blood to go out into the body, causing more hypoxia, further vasoconstricting the pulmonary vessels, which can cause persistent fetal circulation or primary pulmonary hypertension of the newborn.

* Treatment for meconium aspiration is to prevent it with good suctioning of the oropharynx once the head is delivered. New recommendations is not to intubate babies and suction them unless they are having severe respiratory problems with cyanosis. Prevention of hypoxia includes good ventilator management, pulmonary vasodilators such as nitric oxide, and sometimes extracorporeal membrane oxygenation (ECMO).

* Diaphragmatic hernia and choanal atresia are uncommon causes of respiratory distress.

* With respiratory symptoms, always think about the heart and worry about metabolic acidosis, hypoglycemia, hypothermia. Others include hemorrhage, edema, drugs, twin-twin transfusion, and hyperviscosity.

* Diaphragmatic hernia symptoms are child with respiratory distress, scaphoid abdomen (flat or sunken), bowel sounds in the chest, x-ray showing gastric air bubble or bowel above the diaphragm.

* Treatment for diaphragmatic hernia in infant is surgical. Once surgery is completed, problem is that the affected side (almost always left) is not very well developed, pulmonary hypoplasia. Shift of thoracic contents to the contralateral side (right) leads to poor development of that lung as well.

--- Jaundice In The Newborn

* Indirect bilirubin is 11.2, direct is 0.4, physical exam is unremarkable except visible jaundice.

* Neonatal jaundice occurs when indirect bilirubin is deposited in the skin. Hyperbilirubinemia is physiologic or pathologic. Bilirubin is described as conjugated (direct) or unconjugated (indirect).

* Physiologic jaundice is high unconjugated.

* Major source of bilirubin is hemoglobin, also myoglobin and cytochome-oxidase system.

* Infants who are hypoxic in-utero will make more hemoglobin, so when born they may have hemoglobins if 18 or 19, higher circulating red blood cell volumes, shorter lasting red cells (60-90 days). As these RBCs die off there is a load of hemoglobin released, immature liver cannot handle this so it gets stored in the skin.

* Physiologic jaundice is jaundice that does not present in the first day of life, does not go high (max 12.9-15), peaks around the 3-5 day mark, goes away about 7-10 days. Preterm babies are given 10-14 days to resolve physiologic jaundice. No treatment needed

* Breast milk jaundice usually presents by one-week of age, non-esterified fatty acids displace the bilirubin from the albumin molecule leading to yellow baby, no treatment needed.

* Babies with conjugated hyperbilirubinemia and clay-colored acholic stools, suspect biliary atresia.

* Work-up jaundice by getting bilirubin level with conjugated and unconjugated, hemoglobin, and blood type of both baby and mother.

* Phototherapy is good for unconjugated hyperbilirubinemia, photoisomerizes it to a form that is easier to metabolize. Do not put a baby with direct (conjugated) hyperbilirubinemia under light therapy or they will get bronze baby syndrome.

* Exchange transfusions can be used if child is still hemolyzing after light therapy, usually for Rh incompatibility. * ABO incompatibility is more common than Rh, but it is milder. Rh incompatibility is preventable by giving mother Rh immune globulin (RhoGAM).

* Genetic disorders causing jaundice include spherocytosis, G6PD, pyruvate kinase defect, hemoglobinopathies (thalassemias), galactosemia.

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* Jaundice can come from extravascular blood such as petechiae, hematoma, pulmonary or cerebral hemorrhages, and swallowed blood. Example would be facial bruising during a difficult delivery, like face to pubis presentation. * Jaundice can come from polycythema such as maternal-fetal transfusion, twin-to-twin-transfusion, or placental transfusion (cord stripping toward baby).

* Mechanical obstructions can cause jaundice, such as atresia, stenosis, Hirschsprung, meconium ileus, meconium plug syndrome.

* Under-secretion jaundice with Gilbert syndrome, Crigler-Najjar syndrome.

* Other jaundice causes are TORCH infections, hepatitis, prematurity, infants of diabetic mothers. * In twin-to-twin-transfusion, larger baby has higher risk for jaundice and higher risk for problems. * Phototherapy can cause some diarrhea, but that’s alright cause they’re removing the bilirubin.

* Double volumes transfusions help to remove circulating antibodies, remove bilirubin, and increase hemoglobin. * Erythroblastosis fetalis (hydrops fetalis) occurs with hemolysis in-utero, low hemoglobin high output failure, causing anasarca and death.

--- Neonatal Sepsis

* A 3week old infant presents with irritability, poor feeding, temperature of 102F, and grunting. Physical exam reveals a bulging fontanelle and delayed capillary reflex.

* Sepsis is a systemic response to infection. Divided in newborns into early onset and late onset. Early onset is within the first 7 days of life. Late onset is 8-28days of life.

* Neonatal sepsis risk factors include maternal infection, prematurity, prolonged rupture of membranes.

* Most common bacterial cause of neonatal sepsis is group B strep (GBS). E. coli and listeria are distant second and third. Initial choice of antibiotics covers all three.

* Viral causes of neonatal sepsis include herpes simplex and enteroviruses.

* Signs and symptoms are non-specific, grunting, fussiness, poor feeding, tachypnea, respiratory distress, apnea. * Newborns do not always have fever. Sometimes they will present with hypothermia or temperature instability. * Neonates with fontanels usually do not present with nuchal rigidity as the pressure can go to the fontanelle, causing it to be fuller/bulging.

* Work-up includes CBC, lumbar puncture to rule out meningitis, blood culture, urine culture, skin lesion culture, chest x-ray to rule out pneumonia.

* Treatment is antibiotics (ampicillin + 3rd generation cephalosporin), fluid management, attention to details. * Antibiotic choice could also be ampicillin + aminoglycoside. Worry with aminoglycoside about nephrotoxicity and ototoxicity. Follow aminoglycoside levels and do hearing screens later on.

* Penicillin or ampicillin given to mother at least 4 hours prior to delivery will reduce the risk of neonatal sepsis from group B streptococcus.

* Differential includes respiratory diseases, metabolic diseases, intracranial hemorrhage, TORCH infections. --- Transplacental Infections

* TORCH: Toxoplasmosis, Other (syphilis, varicella), Rubella, Cytomegalovirus, Herpes simplex virus. * Majority of TORCH infections occur when mother is in the first or second trimester.

* Toxoplasmosis is transmitted by ingesting undercooked or raw infected meat. It is also found in cat feces, infection transmitted from handling used kitty litter.

* Toxoplasmosis can be mild in the adult, but the problem is when the baby catches it via the mother.

* Signs include intracranial calcifications, 40% infection rate if during 1st or 2nd trimester, IUGR, microcephaly, spasticity, seizures, blindness, retinitis, hepatosplenomegaly (HSM).

* Diagnosis is via isolation from placenta or cord blood, TORCH IgM titers, IgM ELISA. Toxoplasmosis immunosorbent agglutination assay can be used.

* Treatment of toxo during pregnancy is spiramycin or pyrimethamine + sulfonamide. Postnatal (baby) treatment is pyrimethamine + sulfadiazine + leucovorin for six months. Leucovorin decreased the problems with bone marrow suppression seen in chronic sulfa drug use. Steroids for chorioretinitis.

* Cytomegalovirus (CMV) is the most common congenital, 40% transmission to baby. * Symptoms include SGA, petechiae, HSM, thrombocytopenia, direct hyperbiliribunemia. * Culture urine, blood, CSF, throat, placenta. Look for periventricular calcifications (in brain).

* CMV causes chorioretinitis as well. Calcification around periventricular area. “V” in CMV and ventricular. * No treatment for CMV, usually babies do not do well, get mental retardation, microcephaly, liver problems. * Varicella (chickenpox) is divided into neonatal (delivered within a week before or after maternal disease onset) and congenital.

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* Neonatal treated with varicella zoster immune globulin (VZIg), if the mother develops the disease 5 days before to 2 days after delivery. Give acyclovir as well. This is more serious because the mother has not developed antibodies. * Congenital varicella associated with limb-hypoplasia, scaring, microcephaly, chorioretinitis. Give acyclovir. * Rubella has an 80% transfer rate if mother is infected in first trimester.

* Rubella clinically causes mental retardation, microcephaly, heart defects (PDA, pulmonary stenosis), cataracts, HSM, thrombocytopenia, deftness, “blueberry muffin” baby.

* Blueberry muffin due to purpuric lesions from thrombocytopenia, add jaundice and it looks like the muffin. * Diagnose via IgM titers for rubella. Prevention is to immunize mother prior to pregnancy. Anyone born after 1957 should receive a second MMR vaccination.

* Herpes simplex is acquired during passage through the birth canal. C-section not 100% for preventing HSV. * Primary disease is when the mother gets the disease for the first time, so no antibodies, thus lots of transmission to the baby. With recurrent genital herpes there is low transmission rates.

* Delivery can be vaginal if culture is negative and no lesions seen. Do C-section if any lesions or PROM. * Local HSV is present at 5-14 days on skin, eyes, mouth (SEM). Disseminated HSV presents at 5-7 days with pneumonia, shock, hepatitis. CNS HSV presents at 3-4 weeks with lethargy, seizures, instability.

* If the CSF tap comes back “clean” but HSV symptoms, the tap is not clean. Think HSV.

* SEM (local) HSV has about a 95% chance of normal development. Disseminated is about 60%. CNS is about 35%, with only 5% if seizures present. Mortality is high with disseminated especially with pneumonitis. * Treatment of HSV is with acyclovir.

* Syphilis is divided into early (first 2 years of life) or late manifestations.

* Symptoms of early syphilis are fever, anemia, FTT, maculopapular rash, snuffles (runny nose), HSM.

* If you’re suspecting syphilis and baby has glistening snuffles below nose, that’s not boogers, that’s treponems, you better put on a glove. That’ll be hard to explain, how you got syphilis from a baby.

* Late stage manifestations include periostitis, saber (shaped) shins, Hutchinson (notched) teeth, saddle nose, rhagades (linear scars at angle of mouth), Clutton joints (symmetric joint swelling), Jarisch-Herxheimer reaction (fever and rash with penicillin, systemic reaction to treponems being killed).

* Diagnosis with RPR and FTA-ABS. Dark field microscopy can be done too.

--- Gastrointestinal

* A newborn is noted to have choking and gagging with its first feed, then develops respiratory distress. Chest x-ray shows an aspiration pneumonia and a feeding tube coiled in the esophagus. This is a tracheoesophageal fistula (TEF). There are various kinds, most common is esophagus into a blind pouch and a fistula into the trachea distally. * TEF associated with prenatal history of polyhydramnios.

* Diagnosis is clinical. Treatment is surgical repair.

* Look for other abnormalities, such as congenital heart disease such as a PDA, vascular rings, coarctation. Look for vertebral anomalies, anal-rectal anomalies, renal issues. Mainly look at the heart.

* Double-bubble sign seen in duodenal atresia. History will be bilious vomiting. Higher association with Down syndrome.

* Hirschsprung disease (also called congenital aganglionic megacolon) should be suspected in any newborn that has not passed stool in the first 24-48 hours. Also consider imperforate anus, meconium plug, and meconium ileus. On barium enema, the enlarged area is normal. The thing area is where the problem is, stool backs up proximally. * Gold standard (best test) for diagnosis for Hirschsprung disease is colon biopsy looking for aganglionic area. * Necrotizing enterocolitis (NEC) is the most common medical and surgical GI emergency of the newborn. Look for a history of a preterm infant and perinatal asphyxia (low APGAR scores). As baby gets fed, they get bloody stools or starts to get distended, lethargic.

* Most appropriate test is an abdominal film, looking for pneumatosis intestinalis. Pneumatosis intestinalis is air inside the bowel wall (not just in the bowel lumen). Air inside the bowel wall implies NEC.

* Treatment for NEC is bowel rest, antibiotics, and sometimes surgery to remove parts of bowel if it gets necrotic. --- Neonatal Seizures

* In the newborn intensive care unit (NICU), a newborn is noted to have sucking movements, tongue thrusting, and brief apneic spells. The blood counts and chemistries are normal.

* Neonatal seizures do not generally presents with tonic/clonic seizures like they do in older adults.

* Causes of neonatal seizures include hypoxic ischemic encephalopathy (most common). Seizures typically present at 12-24 hours after birth.

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prematurity as cause of intraventricular hemorrhage.

* With intraventricular hemorrhage, may see bulging fontanelle or bloody spinal that that does not clot. Clotting would imply a traumatic tap, not clotting would imply intraventricular hemorrhage.

* Ultrasound or CT scan of head for diagnosis.

* In seizures, always check metabolic causes. Always check a blood sugar. Hypoglycemia can cause seizures and is easily correctable. Hypocalcemia can also cause seizures.

* Focal seizures present with rhythmic twitching, usually of the face and extremities. Multifocal clonic involves many muscle groups. Tonic (stiff) and clonic (jerking).

* Subtle seizures present with tongue thrusting and apneic spells. This is more common in newborns than say a tonic-clonic seizure.

* Diagnostic tests for seizures include blood sugar, electrolytes, EEG, CT or ultrasound of head, lumbar puncture. --- Teratogens

* Any female of childbearing age who is going to go on isotretinoin for their acne must get a pregnancy test and to be told not to get pregnant. Can cause facial and ear anomalies, congenital heart disease.

* Phenytoin can cause characteristic facies, hypoplastic nails, “Cupid’s bow” mouth. * Thalidomide causes limb abnormalities.

* Tetracycline causes enamel hyperplasia and teeth discoloration.

--- Newborn Resuscitation

* You are called to attend the delivery of a 22yo G1P0. She has been followed in the prenatal clinic and all cultures are negative. The patient is term and meconium is visualized.

* Newborn’s past medical history is the mom’s history. Ask about medications the mother is taking. Ask how many babies are being born.

* Resuscitation in the neonate includes twin gestation, prematurity, and meconium.

* In children who need resuscitation, it is usually caused by accidents or illnesses particularly respiratory disease. The majority of codes in children are respiratory in nature, ensure airway management.

* 1996 JAMA article Cardiopulmonary Resuscitation on Television - Miracles and Misinformation portrays successful resuscitation in the majority of cases. This type of information can lead the general public to believe resuscitation is far more successful than it really is (majority die if resuscitation efforts are needed).

* Hypotension is a bad sign. This implies compensatory mechanisms (e.g. catecholamines) have been used up. * In children, the younger you are the faster your heart rate and respiratory rate.

* Treatment is ABC: airway, breathing, circulation. Temperature is important also. * Airway: is the airway patent? Do I need to intubate?

* Breathing: are the retractions, grunting, nasal flaring?

* Circulation: heart rate? Blood pressure? Skin color? Urine output? Capillary refill?

* Newborn intubation blade is 0 or 1 (Miller). Endotracheal tube (ETT) about size 3. ETT = (age + 16) / 4. In children under 8-years of age, it is recommended that an uncuffed ETT be used.

* Do not do Heimlich maneuver on children under age 1, do back slaps. No blind finger sweeps on anybody. --- Kaplan Videos (2001) – Growth & Development with Dr. Eduardo Pino, MD

---Breastfeeding

* A nursing mother asks if her 3 month old baby requires any vitamin supplementation. Answer is no. What about fluoride, vitamin D, iron? Not yet.

* Age 0-12 months is 100kcal/kg/day, 2.5-3gm/kg/day protein. * Age 1-7 years is 75-90kcal/kg/day, 1.5-2.5gm/kg/day protein. * Age 7-12 years is 60-75kcal/kg/day, 1.5-2.5gm/kg/day protein. * Age 12+ years is 30-60kcal/kg/day, 1-1.5gm/kg/day protein.

* “Breast is best,” human breast milk is the best a baby can have. Recommended that it be used exclusively for up to six months. Breast milk plus baby foods up to a year of age. After 1 year, tend to go off breast milk to whole milk. * Advantages of breast milk include convenience (pre-mixed, right temperature), no sterilization required, maternal-infant bonding, less frequent hospitalizations, possible increase in IQ, optimal absorption of nutrients/vitamins/trace elements, possible protection from allergen exposure, much less obesity risk.

* Breast milk has IgA, lactoglobulins, maternal macrophages. Less risk of URIs and otitis media.

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as mother is losing an additional 500 calories a day by nursing. Uterus regresses to normal size faster (oxytocin). * Part of newborn care is administering vitamin K (1mg IM) to prevent hemorrhagic disease of the newborn. Vitamin K is made in the gut with the help of bacteria, which newborns do no have.

* Vitamin K is present is breast milk. Fluoride is recommended after 6 months of age. Fluoride is also recommended if you are using ready-to-feed formula. If you are mixing formula with water, you have to know what the local water supply contains (fluoride or not). Vitamin D to baby only if mother does not have adequate vitamin D intake, example would be cultures that are fully clothed and do not get enough sun exposure for vitamin D conversion. Iron fortified foods given at 4-6 months of age.

* Few contraindications to breast feeding, such as active TB, syphilis, HIV, varicella, galactosemia, herpes if active lesions on the breast.

* Drug affecting breastfeeding include atropine, anticoagulants (heparin safe), antithyroid drugs, antimetabolites, cathartics (except senna), dihydrotachysterol, iodides, narcotics, radioactive preparations, bromides, ergot, tetracyclines, metronidazole, antineoplastics, alkaloids, chloramphenicol, cyclosporin, nicotine, alcohol, steroids, diuretics, oral contraceptives, nalidixic acid, sulfonamides, lithium, reserpine, diphenylhydantoin, barbiturates. * Absolute drug contraindications to breastfeeding include antineoplastics (chemo), alkaloids, chloramphenicol, cyclosporin, nicotine, alcohol, lithium, radiopharmaceuticals (iodine), atropine.

* Relative drug contraindications to breastfeeding include seizure medications, neuroleptics, sedatives, tranquilizers, metronidazole, tetracycline, sulfa, steroids.

* Mastitis is not a contraindication to nursing. Breastfeeding during mastitis helps mother recover quicker. Mother can take antibiotic and still nurse.

--- Formula Feeding

* Formula feeding is used as a substitute or as a supplement. A majority of formulas are cow’s milk based and are adjusted to be as close to breast milk as possible.

* Common formula is 20 calories per ounce.

* Human milk is 1.2g/dL protein (7% calories), 4g/dL fat (54% calories), 6.8g/dL carbohydrates (40% calories). * Soy formula is 1.5g/dL protein (9% calories), 3.8g/dL fat (50% calories), 6.9g/dL carbohydrates (41% calories). * Whole milk is 3.3g/dL protein (20% calories), 3.7g/dL fat (50% calories), 4.9g/dL carbohydrates (30% calories). * Whole cow’s milk is good for baby cows or anyone over 1 year of age. Higher risk for allergies, GI blood loss, and anemia if started early. Iron is not absorbed well and they get GI blood loss.

* 13mo comes in with a hemoglobin of 6. Mother has been using cow’s milk since 4mo. That’s the cause. * Whole cow’s milk has a high solute load on the kidney. 87mEq/mL in human, 227mEq/mL in cow’s. * Vitamin D is recommended if the formula does not have it, but just get out in the sun and you’ll be fine. * Goat’s milk must be supplemented with folic acid.

* If poor iron intake, foods contain iron and are started at 6 weeks.

* Solids should be introduced at about 4-6 months of age. Baby can hold its head up, suck-swallow mechanism is better, lower risk of allergies. Start a new solid one at a time, one per week to determine if they like it and allergies. * A mother states that her infant is having episodes of inconsolable crying every night for the past 5 nights. He draws his legs up and his abdomen becomes rigid. The episodes resolve as quickly as they come on and the rest of the day he acts normally. No diagnostic tests for colic.

* Usually colic starts about 3 weeks of age and lasts to 3 months of age, no one really understands it, cramping thing that happens, babies cry then swallow air and get more cramping, pass gas, draw their legs up.

* Generally, rhythmic motions help for colic. Patting the baby, bouncing the baby, car rides. Sometimes you can use anti-gas medications like simethicone. Typically occurs at the same time of night.

* Differential for colic includes intussusception, hernias, strangulated hair (e.g. mother’s hair around toe).

* A 3yo boy is seen for chronic illness. He appears edematous and apathetic with thin hair. Generalized dermatitis is noted. Sparse hair and decreased muscle tone is noted. This is kwashiorkor, protein-calorie malnutrition.

* Vitamin deficiencies are often associated with kwashiorkor. Usually it occurs after they wean from the breast because they aren’t getting the nutrition they need, seen in poor societies.

* Edema is due to protein loss, thus loss of oncotic pressure in abdomen. Decreased serum albumin, decreased blood glucose, decreased essential amino acids. Mortality can reach 30-40%.

* Treatment of kwashiorkor is slow feeding, replacing quickly can make things worse, plus give vitamins. * Vitamin A deficiency causes night blindness. Thiamin deficiency leads to beri beri. Niacin deficiency leads to pellagra.

* No honey is recommended in the first year of life due to risk of infantile botulism. * Botulism presents as a descending paralysis (versus Guillain-Barre which is ascending).

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* Infantile botulism is due to the spores themselves. In adults, botulism is due to the toxin.

--- Growth, Disorders of Height, & Disorders of Weight

* A father is concerned that their 13yo son is short. The child has been very healthy, is below the 5th percentile for height and has been his whole life. Physical exam is normal. Father is 6’3”, mother is 5’10”. Father was a “late bloomer” meaning growth spurt was later than usual.

* Growth is an increase in size, form, and biologic maturation.

* Growth chart shows length until they are old enough to stand, then it is height. * 2 standard deviations above 50th percentile is 97th percentile (high normal). * 2 standard deviations below 50th percentile is 3rd percentile (low normal).

* A child that is short most of their early years than ends up in the normal range is constitutional growth delay. * Child that is just below the minimal for normal height but is following the curve. Father is 5’6”, mother 5’1”. The tallest person in the family is 5’7”. This is familial short stature.

* A child that is 50th percentile for several years than drops to below 3rd percentile is worrisome. This could be a pituitary tumor, craniopharyngioma, Turner syndrome, or other causes of falling off the growth curve.

* Pathologic short stature and constitutional short stature both start with child in normal range for height.

Constitutional will eventually reach adult height, pathologic will fall off. Past family member history is important. * Physical exam may be helpful if a syndrome is involved. Any female with short stature should be evaluated for Turner syndrome (e.g. karyotype).

* Labs could include CBC, urinalysis (chronic renal disease), LFTs, TFTs (hypothyroid), growth hormone. * Wrist films can tell you bone age. If patient is 12yo and short, bone age is 9yo, you know there is room for the patient to grow still.

* If you suspect pituitary tumor, craniopharyngioma, do a skull film looking for an enlarged sella turcica. * Treatment is to correct the underlying disease. Growth hormone will help some patients.

* Differential includes hypopituitarism, deprivational dwarfism, Turner, hypothyroidism, chronic disease. * Majority of patients who are tall are normal. Doubtful that any NBA basketball player has Marfan syndrome. * Causes of increased height include obesity, growth hormone excess (gigantism, acromegaly), androgen excess (tall as children, short as adults), hypothyroidism, homocystinuria, cerebral gigantism (Sotos syndrome), Beckwith-Wiedemann syndrome, Weaver-Smith syndrome, Klinefelter syndrome.

* Disorders of weight include failure to thrive and obesity.

* A baby weighs 16lbs at 1 year of age. His birth weight was 8lbs. Parents state that the baby feeds well. Physical exam reveals a baby with little subcutaneous fat, long dirty fingernails, impetigo, and a flat occiput.

* Failure to thrive (FTT) means you are not gaining weight appropriately or losing your rate of weight gain. * FTT causes include malnutrition, malabsorption (infection, celiac disease, chronic diarrhea), allergies, immune deficiencies, chronic diseases.

* Baby should double their birth weight in about 4-5 months. By 1yo, they should have tripled their birth weight. * Impetigo, flat occiput, and long dirty fingernails implies the baby is not being taken care of. Baby is not being cleaned or cared for, occiput is flat because baby is just laying their on its back all day long.

* Hospitalization may be necessary to document how many calories they are getting, to teach the parents to do it appropriately, to get the family the resources they need to feed the baby.

* In children who do not gain weight after these measures, consider a swear chloride test for cystic fibrosis (CF). * Obesity is a generalized over-accumulation of fat, generally due to overeating without exercise. Mother may give the baby a bottle every time it cries, so it gets use to more calories. Grandma may say “a fat baby is a healthy baby.” * Treatment is diet and exercise.

* There are some syndromes associated with being overweight and obese, but they are rare. Exception to the rule. * Risk factors are parental obesity, family inactivity (T.V., video games), feeding in response to any crying, too much fruit juice in first year of life, and some syndromes.

* Children present not only fat, but possibly with increased height. Boys may have increased fat tissue in the mammary region (looks like gynecomastia), abdominal striae, pubic fat pad in boys makes it look like a micropenis. * Body mass index (BMI) curve over 95% or over 30.

* Complications include risk of adult obesity (with hypertension, hyperglycemia, stroke, MI), cardiovascular problems, slipped capital epiphysis, sleep apnea.

* Differential includes endocrine and genetic causes, rarely.

--- Fluids & Electrolytes

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