Sir, this patient has bilateral enlarged kidneys. There are bilateral masses in the flanks which are bimanually palpable and ballotable with a nodular surface. Of note, I am able to get above both masses. Percussion note was resonant over both kidneys and they move inferiorly with respiration. They are not tender in nature and there was no renal bruit.
There is no associated hepatomegaly and the liver span is 12 cm at the right mid-clavicualar line. The spleen is not enlarged. There is no ascites detected clinically and the bladder is not palpable or percussible.
The patient does not have a sallow appearance and not cachexic looking. There are no pruritic scratch marks or bruising. There is also no leukonychia or Terry‟s nails. There is no conjunctival pallor to suggest anaemia and no features of polycythemia such as a plethoric facies or conjunctival suffusion. Patient is not in fluid overload as there is no pedal oedema, he is able to lie flat and is not oxygen dependent. There is no Kussmaul‟s breathing pattern and also no flapping tremor or uremic fetor.
He does not have any acromegalic features, no DM dermopathy and no adenoma sebaceum to suggest tuberous sclerosis.
There is no evidence of renal replacement therapy such as AVF, TK cathether or a transplanted kidney.
I would like to complete my examination by
checking the patient‟s temperature chart for fever, blood pressure for hypertension
fundoscopy for hypertensive changes
urine disptick for hematuria, proteinura and pyuria Cardiovascular examination for signs of MVP or AR
Neurological examination for a third nerve palsy secondary to berry aneurysm or any evidence of a stroke FHx of aneurysm or SAH (5% risk overall but 20% if positive FHx)
In summary, this middle age gentleman has got bilateral enlarged kidneys with no complications of chronic renal failure detected clinically. There is also no evidence that the patient is undergoing renal replacement therapy. The most underlying etiology is Adult Polycystic kidney disease.
Questions
What are the causes of bilateral enlarged kidneys?
APCK Commoner
o Acromegaly (hepatosplenomegaly) o Early diabetic nephropathy o Bilateral hydronephrosis Rare
o Tuberous sclerosis o Amyloidosis
o Von-Hippel Lindau disease Autosomal dominant
Multiple angiomata in the retina, CNS Cysts in liver, kidneys pancreas RCC, phaeochromocytoma
What are the conditions that can result in bilateral renal cysts?
o Polycystic kidneys
Dominant and recessive Simple cyst
Von Hippel Lindau Tuberous sclerosis
66 What are the complications of APCK? (Renal and Extra-renal Cx)
Fever
o UTI, pyelonephritis, pyocyst Hypertension (75%)
o Activation of RAA from intra-renal ischaemia from architectural distortion o Malignant hypertension
Renal artery stenosis from compression Renin producing cyst
Pain
Haemorrhage into cyst Upper tract obstruction
Massively enlarged cyst Clot
Stone Anaemia
o CRF
o Persistent gross hematuria Polycythaemia
o Increased erythropoietin production Malnutrition
o CRF
o Bilateral renal enlargement with early satiety Acute renal failure
o Malignant hypertension o UTI
o Nephrolithiasis (Uric acid) Chronic renal failure
Renal cell carcinoma (rare) Extra-renal
o Abdominal – cysts in liver, spleen, pancreas, ovaries; colonic diverticular disease o Cardiac – MVP(25%), AR, TR
o Intracranial aneurysm (III nerve palsy), SAH (3%) What are the complications of CRF?
Fluid
Electrolytes – Hyperkalaemia Acid-base – Metabolic acidosis Uremia and its complications Hypertension
Anaemia (NCNC)
Secondary and tertiary hyperparathyroidism Renal bone disease
Why are patients with CRF sallow?
Impaired execretion of urinary pigments combined with anaemia
What are the types of signs in the nails that you can detect in patients with CRF?
Hypoalbuminaemia o Leukonychia
o Muehrcke‟s nails (paired white transverse line near the distal end of nails) Renal failure
o Terry‟s nails (distal brown arc 1mm or >)
o Mee‟s line (single white line; also in arsenic poisoning) o Beau‟s line (non-pigmented indented band = catabolic state) What are the causes of anaemia in patients with CRF?
Erythropoeitin deficiency Anaemia of chronic disease
Fe deficiency anaemia – blood loss, nutrition Folate deficiency – nutrition
What is Adults Polycystic Kidney disease?
Multisystemic, progressive disease, 1 in 400 to 1 in 1000 people
Characterised by cysts formation and enlargement in the kidneys and other organs Autosomal dominant with almost 100% penetrance
Focal cystic dilatation of the renal tubules 2 predominant type
o 85% - APCKD 1 on Ch 16 o 15% - APCKD 2 on Ch 4
o 3rd type of which loci is not fully known Presents clinically in the 3rd or 4th decades with
o Hematuria, hypertension, recurrent UTI, pain and uremia o Stroke
By age 60 years, 50% will require RRT
Poorer Px – males, PCK 1 and early onset of clinical features Mortality
o ESRF (1/3)
o Stroke and other hypertensive Cx (1/3/) o Others
How do you investigate?
Blood Tests o FBC o Biochemical
o CRF – Ca, PO4, iPTH, Uric acid, urinalysis USS (useful >20 years old); Ravine‟s criteria
o At risk patients, 20-30yrs: 2 cysts in 1 kidney or 1 cyst in each kidney o At risk patients, 30-60 yrs: 2 cysts in each kidney
o At risk patients, >60 yrs: 4 cysts in each kidney Other imaging(CT and MRI)
MRA for patients with high risk of an aneurysm, Ba enema and Echocardiogram Genetic testing
o For young people with no cysts on USS who are potential organ donors How would you manage?
Education and counselling, regular follow up, screening of first degree relatives
Avoidance of medications that can precipitate renal impairment such as NSAIDs or tetracycline antibiotics Medical treatment
o Hypertension with ACE inhibitors or ATII RA
o UTI, cysts infection – usually GN bacteria therefore use Bactrim or fluroquinolones with good renal tissue penetration o Pain treatment
o Renal failure – medical treatment and RRT for those with ESRF o Antibiotic prophylaxis
Surgical treatment o Pyocyst – drainage o Cystectomy o Nephrectomy o Alcohol sclerosant o RRT
o Aneurysm clipping, MVP with MR
68 30. Transplanted Kidney
Presentation
Sir, this patient has a transplanted kidney in the right iliac fossa associated with bilateral enlarged kidneys with a functioning AVF with features of cyclosporine and chronic steroid use.
There is presence of a rounded palpable mass in the right iliac fossa with an overlying scar. It is non-tender. In addition there are bilateral masses in the flanks which are bimanually palpable and ballotable with a nodular surface. I am able to get above these masses and they are not tender. They move inferiorly with respiration and percussion note is resonant over them. There is no associated ascites and no renal bruit.
The liver and spleen are both not enlarged.
The patient does have features of renal impairment with a sallow appearance and is thin looking. He does not have any bruises or pruritic scratch marks and no leukonychia or Terry‟s nails were detected. There is also no conjuctival pallor to suggest anaemia and there are also no features of polycythemia such as a plethoric facies or conjunctival effusion. He is also not in fluid overload with no pedal edema and is able to lie flat and is not oxygen dependent. There is no Kussmaul‟s breathing with no uremic fetor or flapping tremor of the hands.
There is presence of an arterio-venous fistula in the right upper limb. It is functioning with a good thrill. There are no recent needle puncture marks and no aneurysm was noted.
There is presence of diabetic dermopathy noted on the lower limbs.
There is no evidence of transplant related hepatitis B or C with no jaundice or stigmata of chronic liver disease.
Patient has hypertrichosis and gum hypertrophy which are complications of cyclosporine usage. Moreover, he has a Cushingnoid habitus with steroid purpura and thin skin, suggesting chronic steroid usage.
I would like to complete my examination:
Temperature chart for fever BP for hypertension
Fundoscopy for hypertensive changes
Urine analysis for hematuria, proteinura or pyuria CVM – MVP or AR
Neurological – III nerve palsy or PHx of stroke
In summary, this middle age gentleman has a transplanted kidney for underlying Adult Polycystic kidney disease with previous dialysis. The graft is functioning well as he is not uremic and is well with features of cyclosporin and steroid use.
Questions
What are the differential diagnoses for a right iliac fossa mass?
Transplanted kidneys
Carcinoma of the caecum (hard mass, LNs) Abscess – appendicular, ileocecal
Crohn‟s disease (mouth ulcers, PR for fistulas) Ovarian tumors (in females)
Others
o Amoebiasis, TB lymphadenitis, actinomycosis o Carcinoid
o Ectopic kidney
What are your differential diagnoses for a left iliac fossa mass?
Transplanted kidney
Colonic carcinoma (hard mass, hepatomegaly LNs) Diverticular abscess
Fecal mass Ovarian tumors Others – lymphadenitis
What are the common kidney diseases leading to transplant?
DM
Hypertension GN
How does renal transplant compare with dialysis?
Higher patient survival rates
Better quality of life with lower hospitalisation rates
What are the causes of transplant loss?
Patient death Allograft failure o Immunological
Acute rejection
Single most important event determining graft survival
Can result in rapid loss of graft or progression to chronic rejection or chronic allograft nephropathy Treated with pulse steroid or anti-lymphocyte antibody therapy
Chronic rejection o Non-immunological
Renovascular thrombosis Ischaemia reperfusion injury
Nephrotoxicity from calcineurin inhibitors CMV, polyoma virus
DM, hypertension, hyperlipdaemia o Others
Recurrence of primary disease (GN and DM) Chronic allograft nephropathy
What is delayed graft function?
Defined as requirement of dialysis in the first week post transplant o Immunological – acute rejection
o Non-immunological – ischaemia reperfusion injury, donor hypertension What are the strategies one can use to reduce graft loss?
Immunological
o Live donor better than cadaveric o HLA matched at A, B and DR loci o Absence of pre-sensitisation
Previous transplant Pregnancies Transfusions Idiopathic
o Immunosuppresive therapy to reduce acute rejection Traditionally use of steroid and cyclosporin Others
Calcineurin inhibitors eg Cyclosporin and tacrolimus Mycophenolate mofetil
Sirolimus Non-immunological
o Pre-transplant
Donor factors – old age, CVA, hypertension
Recepient factors – older, male, obese, diabetic, hypertension o Technical factors
increase cold ischemia time – LD transplant, renoprotective preservative solutions
hyperfiltration from inadequate nephron dose – match size and better if male to female; use of ACE inhibitors o Post-transplant
Calcineurin inhibitors induced nephrotoxicity Monitor levels
Use others such as sirolimus or MMF CMV infections and polyoma virus
Prophylaxis with ganciclovir for CMV No Rx for polyoma virus
Treat BP (<130/80) and hyperlipidaemia and DM What are the complications of cyclosporine?
Hirsutism/hypertrichosis Hypertrophy of the gums Hypertension
Hyperkalaemia, hyperuricaemia, hypercholesterolaemia, hypomagnesemia Hepatotoxicity
Hemolytic uremic syndrome Hiccuping (gastroparesis) Hole-in-bones (osteoporosis) Nephrotoxicity
Neoplasia (lymphoproliferative)
Neurological (tremors, headaches, seizures and strokes)
70 What are the complications of chronic steroid use?
Skin – thin skin, telangiectasia, steroid purpura Cushingoid habitus
Osteoporosis, AVN femoral head Peptic ulcer disease
Hypertension Diabetes mellitus Cataracts Steroid psychosis How do you manage?
Education and counselling, regular follow up, compliant Treat underlying cause
Require preparation prior to transplant
Post transplant management to reduce graft loss (See above)
NEURO!
31. Approach to Examination of the Face