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PSEUDOHERMAPHRODITISM

WITH

MULTIPLE

CONGENITAL

ANOMALIES

Report

of a Case

By Edgar J. Schoen, M.D.,* Alexander L. King, M.D., A. LaMont

Baritell, M.D., and William F. Knigge, M.D.

CLINICAL

NOTES

H

ERMAPHIiODITISM, as reviewed in the

excellent monograph by Hugh

Young,’ was known to the ancient Hebrews

audI Greeks. In spite of the early

recogni-tion of this syndrome and the many inter-esting case reports in the literature

(Neu-gebauer in 1908 noted over 1200

indivi-dual cases of hermaphroditism), the etiol-ogy tfldi classification of the various types

of hermaphroditism have never been

corn-pletely agreedi upon.

Within recent years the role of congenital

adirenal hyperpbasia in the causation of the

syndrome of female

pseudohermaphrodit-islil has been well established,’’ 15 but the factors involved in the etiology of male pseudlohermaphnoditism and true

hermaph-roditisrn are still in doubt. The

impor-tance of both zygotic and hormonal factors

in sex differentiation has been demon-stratedi IJy a wealth of experimental data.31’

Accompanying the many theories of sex

differentiation have been a number of

proposed classifications of

hermaphrodit-The simplest andi most popular

classification today is the division of her-maphrodites according to gonadal sex. Thus, a true hermaphrodite has germinal cells of 1)0th sexes. Pseudohermaphrodites have the

gonads of only one sex but their external

genitalia are such as to cause doubt as to

true sex; male pseudiohermaphrodites hav-ing testes andi female

pseudohermaphro-dites ovaries. Although exploratory

laparot-omy is still necessary for final diagnosis of

From the l)epartments of Pediatrics, Surgery

and Urology, Kaiser Foundation Hospital,

Oak-Iind, California.

OADDRESS: 280 MacArthur Boulevard West,

Oakland 11, California.

male pseudohermaphroditism and true

hermaphroditism, the elevated urinary

17-ketosteroid excretion in combination with

typical genital anomalies usually obviates the need for operation in female

pseudo-hermaphroditism. Recently methods have been described for determining

chro-mosomal sex by skin biopsy2#{176} and blood

2 1 These chromosomal studies may prove to be of considerable aid in the

diag-nosis of hermaphroditism butthey have not yet eliminated the need for exploratory

laparotomy.

In contrast to hermaphroditisrn the

syn-drome of ovarian agenesis has been recog-nized only comparatively recentby.”’

Al-though abnormalities of the genital system (female sexual infantilism) in combination with other congenital anomalies make tip the syndrome, in the cases previously

tie-scribed there has been no doubt as to sex. “Turner’s syndrome” has been described in

both males and females, but none have been pseudohermaphrodites.

The present report describes a case con-sidered to be male pseudohermaphroditism

in combination with other congenital

anomalies. Most of the congenital anomalies present in this patient have previously been

described in the syndrome of ovarian agen-esis but the authors are unaware of any report of a similar case.

History

REPORT

OF

A CASE

M.G., an 1 1%-year-old white “female,” was

admitted on August 23, 1954, and discharged on September 18, 1954. She was referred for

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364 SCUOEN - PSEUDOHERMAPHRODITISM

approximately 7 pounds at birth. There was no neonatal distress. At birth it was noted that she

Ila(1 l’cUIlitr genitalia and an abnormally

shaped head. She was considered to be a

fe-male

and

was reare(l as a girl. Growth and

(lcvvlopnleil t were grossly ttormal. The patient has never had convulsions, but there is a

his-tory of occasional frontal headaches. She wears glasses to correct au eve niuscle weakness.

She has been tinted! to be almost completely (leaf in the right ear.

i’here is no family history of sexual

anoma-lies 01 other coulg(uuital ailoinalies.

Fin. I. An I lh-vear-old male i)sct1ohertii:ip1iro-(lite. Note the stock bod l jId, short phallus, (111(1 lnoruuially Slhtj)cd head.

Physical Findings

The child is a vell-deveIoped and well-nourished, blonde, white “female.” The body build is stocky. neither typicaII’ masculine nor feminine (Fig. 1

)

with accentuation of the

bum-bar curve. Temperature, normal; pulse, 84;

res-pir1ti1i, 20/mm. ; 1)bood pressure, 1 16 ‘80; height, 54 inches (15th percetitile), and

weight, 80 Potindls (50th percettile). The head is abnormally slia1wcl, there being a decreased anteroposterior diameter and flat-tening of the supraorbital ridges. giving her eyes an excessive irnii11euice. No hirsutism or abnormal pigmentation was seen

on the skin. The eyes showed an alternating

esotropia ; ophthalmoscopic examination was normal. A complete percepti’e deafness was

presetit in the right ear. The neck appeared

short Iflit there was uio ‘ebbing. iliere was no evidlence of breast development. Examination

of the genitalia (Figs. 2 anl :3) showed a

plial-Ins, 1 .5 cm. iii leiigth, which had the

(IP1)ear-aitce of a clitoris. The urethra was 1)asterior to the plitlItus. There was no scrotum or labia,

auirl ho vaginal opening or dlifliple in the

median ra1)he between the urethra and the auiiis. No l)erineal or inguinal masses were

pal-1)7tbk. On rectal examination no I)r5ttte,

cervix, or adnexae could1 be felt. Citbitus valgus

vas not present.

Laboratory Findings

Urine an(l complete l)bOOd count were nor-nial. Boentgenograms: The chest and pelvis

were normal. PeIograms were normal. The dorsal ali(l lumbar spine showed a sl)iIi bifida

occtilta at the level of L-5 and S-I

.

The wrist

showed a bone age of 7 years 10 months (Todd

Standards) as compared vith a chronological

age of 1 1% years. The skull (Fig. 4) shoWedb a

congenital abnormality of shape with the so-called ‘steeple frontal area. The calyarium had prominent convolutional markings. The

sella turcica was increased in size and depth,

without actual destruction, but with some

thinning of the dorsum sellae.

Audiometry revealed almost cimplete per-ceptive deafness on the right, most marked in the higher tones. There was also diminished

perception of higher tones on the left. An in-sulin tolerance test (0. 1 unit kg. intraveili)usly)

was normal. Serum protein-bound iodine was

(3)

excre-CLINICAL NOTES 365

demonstrating the small phallus, pigmeation on

either side of the median raphe, and absence of scrottini.

or structures grossly resembling

could be felt.

corpora cavernoa

tion#{176}on 3 occasions showed values of 3.2, 2.1

and 1.9 mg./24 hr. (normal 2-8 mg./24 hr. for

this age). The 17-hydroxycorticosteroids#{176} on 2

occasions were 4.9 and 4.1 mg./24 hr. (normal

4 to 13 mg.24 hr.). Fifteen units of a standard

titered 1)atch of ACTH (supplied by Dr. P. Forsham) caused the 1 7-hdroxycorticosteroid

excretion to rise to 12.4 mg./24 hr., a threefold

rise, which is considered to be a normal

adlrenocortical response.23 24 A gonadotropic

hormonef assay showed values of less than 5

mouse units; an estrogenf assay showed values of less than 2 g. 24 hr. (estrone equivalents). (Both are within normal limits for either boys

O Determinations of urinary 17-ketosteroids and

17-hydroxycorticosteroids were performed at the

University of California Metabolic Laboratory

through tile courtesy of Dr. Peter Forshani using a

modification of the Callow-Callow-Emmen

reac-tion for the l7-ketosteroids and a niodified

Porter-Silber technique for the 17-hydroxycorticosteroids.”

I Estrogen and gonadotropic hormone determina-tions were performed at the University of

Cali-fornia Gynecology Endocrine Laboratory.

or girls of this age group.)

Urethroscopic examination revealed that the

urethra had the appearance of a male urethra

with a structure like a verumontanum in the

region of the apex of the prostatic urethra.

No sinuses or openings into the urethra could be seen and the bladder and ureteral orifices appeared normal.

Using the technique described by Moore

et al.,2 a skin biopsy was taken for determina-tion of chromosomab sex. This was reviewed by Dr. Jackson Crane (University of California Department of Pathology) who found that 5 per cent of the cells had “chromatin bodies,” a count considered characteristic of male chromosomal sex.2#{176}In addition a blood smear examination was carried out at this hospital (Dr. N. L. Morgenstern) according to the tech-nique described by Davidson and Smith2’ and only 1 questionable chromatin structure was seen in more than 300 polymorphonuclear Ieu-kocytes, a count also considered indicative of male chromosomal sex.

(4)

in-SCHOEN - PSEUDOHERMAPHRODITISM

Fin. 4. Roentgenograni of the skull, showing the oxycephaly, with “steeple” frontal

area, hammered silver” appearance and ballooning of the pituitary fossa.

telligence intid’1it vas estimated to be

be-tween 85 ail(i 95. The psychiatrist felt at fiist

that the 1)atwutts 1)sVchOseXUal orientation vas

(h(.’fihiitel\ female. She overcompensateci for bier feelings of confusion al)Out her genitalia by

cx-1’”g ignorance of any genital

abnormali-ties. In later interviews she expressed a great deal of ambivalence regarding her sexual status.

Course

After a joint conference with the several

speciilties involved, exploratory laparotomy was performed. On entering the abdbominal caVity the surgeon saw ilO evidence of a uterus, ovi(iucts or other female internal genitalia. On either side there was a small oval gonad, each

about I .5 cm. in diameter. The one on the left

vas intra-abdoininal, being about :3 cm. al)ove the internal inguinal ring; the one on the right

was fotmd to be lying within the inguinab canal. Grossly, they had the appearance of

small testes, each with a vas deferens. The

substance of the gonad itself on each side was

soft and fatty. Biopsies were taken from both

these organs and the abdomen was closed.

It was felt that adequate biopsy specimens were taken, especially on the right where it

was thought that about a quarter of the gonad was removedl.

The pathology report (Dr. N. L. Niorgen-stern) noted that the specimen from the left

revealedi fat andi fibrous tissue containing a few

veins. There were several small duct-like struc-tures hued by a single layer of tall columnar cells surrounded by a circumferentially ar-ranged smooth muscle coat. This resembled an

epoophoron (Fig. 5). The specimen from the

right (Fig. 6) revealed a loose collagenous matrix inwhich were tul)Ules lined by cuboidal cells with pale-staining cytoplasm. In a few

(5)

ar-CLINICAL NOTES 367

FIG. 5 (Upper). Photoniicrograph of specimen from biopsy on the left, which seems to resemble an

epoophoron (see description in text).

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368 SCHOEN - PSEUDOHERMAPHRODITISM

rangedi smooth muscle coat slightly thinner than that seen around the structure on the left. The specimen on the right was considered to be an immature epididvmis. The pathologist noted that although there was no tissue present resembling male gonad, the specimens from both the right and tile left represented wobffian dierivatives.

M an agemen t

DISCUSSION

The problem presented is that of an

11%-year-old child, reared as a girl, who shows: 1) hypoplastic genitalia (sex designation

un-certain); 2) complete absence of female in-ternal genitalia on exploratory baparotomy; 3) skin biopsy indicative of “male”

chromo-souiial pattern; 4) gonadls showing the pres-ence of wolffian derivatives, and grossly

resembling testes, and 5) other congenital anomalies.

It is of interest that the majority of cases

of male pseudohermaphroditism reported were reared as females and actually

femi-nized at puberty. It has been known for some

tune that normal adult unabes excrete estro-gen, and there is evidence that the site of

this estrogen formation is in the testis.27

However, it has been considered that in

feminized male pseudohermaphrodites

es-trogen plays an increased role, either due to primary estrogen difference or to a varied

end organ response.2S34

Recently Schneidler et #{252}l.,2)Morris,36 and

Botella-Llusia7 have called attention to

feminization in male

pseudohermaphro-dites. Schneider et al.5 reported 6 cases in

1 family. Morris3 collected 80 cases from the literature, added 2 of his own and

re-ferred to this syndrome as “testicular fem-inization.” Some of the characteristics of

the syndrome as described by both Schneider and Morris were: 1) female body contour; 2) normal female breasts; 3) ab-sent or sparse axillary and pubic hair; 4)

female external genitalia-often underde-veloped labia, small clitoris audi a short blind vagina with no cervix, and 5) gonads which are usually intra-abdominal testes.

There is, in most instances, hyperplasia of

the Leydig cells and absence of

spermato-genesis in the tubules.

This patient did not have the female ex-ternal genitalia reported in these previous

cases except for the small phallus, and since she has not undergone puberty, the female

body contour and breasts remain in

ques-tion.

The robe of psychosexual factors in up-bringing has been increasingly emphasized during the past few years.25’ 26, 30, 38-40

El-lis38 reviewed 84 cases of hermaphroditism from the literature and noted that in the majority of cases the libido and sex robe

corresponded to the sex in which the pa-tient was reared.

Thus, the nature of the external geni-tahia, the psychosexual orientation of this

patient, and the possibility that she might feminize at puberty have bed the authors to feel that she should be continued as a fe-male. If at puberty she masculinizes or remains eunuchoid the present plan is to

administer estrogen with or without surgi-cal removal of the gonads. The ultimate decision will depend upon the

circum-stances at the time of puberty.

Possible Relation to Turner’s Syndrome

When one is confronted with a case in

which genital abnormalities are accom-panied by multiple congenital anomalies of other systems the syndrome of ovarian agenesis, or Turner’s syndrome, comes to mind. The classical triad originally de-scribed by Turner in 193841 consisted of

sexual infantilism, congenital webbed neck and cubitus vabgus. Since that time other

anomalies have been noted to be associated with the syndrome,47 among them being: congenital deafness; ocular abnormalities such as strabismus, coboboma, cataracts, ptosis, and mild exophthabmos; vascular anomalies such as coarctation of the aorta;

bony anomalies such as spina bifida,

re-tarded bone age, genu valgus, osteoporosis, and fusion of cervical vertebrae, and mental retardation. The present case showed

(7)

CLINICAL NOTES 369

retarded bone age, short neck (but no

web-bing or fusion of cervical vertebrae), some

shortness of stature and oxycephaly in ad-dition to her genital anomalies.

In 1942, Abbright et al.42 and Varney et al.’ emphasized the importance of elevated

gonadotropins and lack of ovarian function in ovarian agenesis. Although this patient

did not have any gonadotropins demon-strable in her urine this finding is of limited significance because she has not shown evi-dence of puberty as yet. In addition the

cases of Hertz et al.,44 Martin and

Cour-voisier,46 and Dorif et al.5 failed to show elevated gonadotropin excretion in adults.

Since the initial description of the

syn-drome of ovarian agenesis about 100 cases in females have been brought to attention. In 1943 Fbavell9 reported the first case of

what was considered to be “Turner’s syn-drome” in a male. Up to the present time 8 other cases have been reported in males,

the most recent by Prunty et al.8 However,

in the cases of the males in whom biopsy

of the testes were reported, all have shown

gonadal elements, although there is often

hypoplasia, usually of the interstitial cells. No case in the male with complete absence

of germ cells, analogous to ovarian agenesis

in the female, is knowi to have been re-ported. Neither is there a known case of “Turner’s syndrome” in a

pseudohermaph-rodite. The cases of male pseudoher-maphroditism reported in the literature

have been free of any group of congenital

anomalies other than those of the genitalia.

The demonstration of a case in a male

similar to ovarian agenesis in the female has significance regarding the mechanism

of sex differentiation which overshadows its

interest as a simple medical rarity. In this respect, Wilkins and Fleischmann,6

report-ing on 3 cases of ovarian agenesis with com-plete back of ovarian development, stated that “for conclusive proof that sex

differ-entiation is determined solely by zygotic factors it would be necessary to find male

individuals analogous to our patients and

lacking entirely in gonadal development.”

Whether or not the present patient is such

an individual cannot be definitely stated at this time. Final proof would depend upon

the demonstration of total absence of gona-dal tissue in serial sections of both gonads.

SUMMARY

The present paper reports a case of

prob-able male pseudohermaphroditism in combination with multiple congenital anomalies. The management of the case and the possible relationship to “Turner’s syndrome” are discussed.

ACKNOWLEDGMENTS

The authors express their appreciation to Dr. Janet MacArthur and Dr. Nathan B. Talbot, Massachusetts General Hospital, Boston, Massachusetts, for suggestions in the preparation of this paper.

REFERENCES

1. Young, H. H. : Genital Abnormalities, Hermaphroditism and Rebated Adrenal Disorders. Baltimore, Williams &

Wilk-ins, 1937.

2. von Neugebauer, F. L. : Hermaphroditis-mus beim Menschen. Leipzig, W. Klink-hardt, 1908.

3. Moore, C. R. : Embryonic Sex Hormones

and Sex Differentiation. Springfield, Thomas, 1947.

4. Goldschmidt, R. : Die Sexueblen Zwischen-stufen. Berlin, Springer, 1931.

5. Jost, A. : Recherches sur be contr#{244}le

hor-monal de 1’ organogen#{232}se sexuelle du lapin et remarques sur certaines

malfor-mations de 1’ appareil genital humain.

Gyn#{233}c.et Obst., 49:44, 1950.

6. Wilkins, L., and Fleischmann, W.: Ovarian

agenesis: Pathology, associated clinical symptoms and the bearing on the

theories of sex differentiation.

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Gun.

Endocrinol., 4:357, 1944.

7. Wilkins, L., Grumbach, M. M., and Van

Wyk,

J. J.:

Chromosomal sex in ovarian

agenesis. Letters to the Editor.

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Clin.

Endocrinob, 14:1270, 1954.

8. Lilbie, F. R.: The Free-martin; a study of the action of sex hormones in the

foetal life of cattle.

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Exper. Zo#{246}l.,23:

371, 1917.

(8)

370 SCHOEN - PSEUDOHERMAPHRODITISM

and hormonal content of fetal horse gonads. Anat. Rec., 56:275, 1933. 10. Greene, R. R. : Hormonal factors in sex

inversion; the effects of sex hormones on

embryonic sexual structures of the rat. Biol. Symposia, 9:105, 1942.

11. Turner, C. D.: Modification of sexual

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Morphob., 65:353, 1939.

12. Humphrey, R. R. : Sex inversion in Am-phibia. Biol. Symposia, 9:81, 1942.

13. Schiller, W. : Congenital and acquired sex changes. Internat. Clinics, 3:86, 1940. 14. Talbot, N. B., Butler, A. NI., and Berman,

R. A. : Adrenal cortical hyperplasia with

virilism; diagnosis, course, and

treat-ment.

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Glin. Investigation, 21:559, 1942.

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of Endocrine Disorders in Childhood and Adolescence. Springfield, Thomas, 1950.

16. Howard, F. S.: The surgery of intersexuabs.

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UroI., 65:636, 1951.

17. Moszkowicz, L. : Die Entstehung des

Hermaphroditismus. Wien.

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Wchn-schr., 49:545, 1936.

18. Creevy, C. D. : Pseudbohermaphroditism, a report of five cases. Internat. S. Digest,

16:195, 1933.

19. McGahey,

J.

F. : A new conception of her-maphroditism. Surg., Gynec. & Obst.,

67:646, 1938.

20. Moore, K. L., Graham, M. A., and Barr, M. L. : The detection of chromosomal

sex in hermaphrodites from a skin

hi-opsy. Surg., Gvnec., & Obst., 96:641, 195:3.

21. Davidson, W. NI., and Smith, D. R.: A

morphologic sex difference in

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22. Callow, N. H., Callow, R. K., Emmens, C. W. and Stroud, S. W.: Methods of extracting compounds rebated to the steroid hormones from human urine.

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23. Liddle, G. W., Island, D., Rinfret, A. P., and Forsham, P. H.: Factors enhancing response of human adrenal to ACTH: Is there an adrenal growth factor?

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Clin. Endocrinol. & Metab., 14:839, 1954. 24. Forsham, P. H.: Personal commuiiication. 25. Hamblen, E. C., Garter, F. B., Wortham,

j. T., and Zanartu,

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26. Greenhill,

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P., and Schmitz, H. E.:

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Effects of chorionic gonadotropin in

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28. Witschi, E., and Mengert, W. F. :

En-docrine studies on human hermaphro-dites and their bearing on the inter-pretation of homosexuality.

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29. Novak, E. : Sex determination, sex differ-entiation and intersexuality, with report of unusual case. J.A.M.A., 105:413, 1935.

30. Bettinger, N. F. : Hermaphroditism. Surg.,

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31. Goldberg, M. B., and Maxwell, A. F.: Male pseudohermaphroditism proved

by surgical exploration and microscopic examination; a case report with specula-tions concerning pathogenesis.

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32. Nelson, W. 0. : Estrogen secretion by the

testes. Federation Proc., 10:97, 1951.

33. Mischell, D. R. : Familial intersexuality; a report of three unusual cases. Am.

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34. Beatty, D. C., Champ, C.

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and Swyer, C. I. NI. : A case of male pseudohermaph-roditism. Brit. M.

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I:1369, 1953. 35. Schneider, R. W., Van Ommen, R. A., and

Hoerr, S. 0. : Hereditary occurrence of testes and absence of sexual hair in amenorrheic women : type of pseudo-hermaphroditism.

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36. Morris,

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M. : The syndrome of testicular

feminization in male pseudohermaphro-dites. Am.

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Obst., 65:1192, 1953. 37. Botebla-Llusia,

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and Nogales, F.: Uber

das Syndrom des Pseudohermaphroditis-mus masculinus mit totaber

Feminisi-erung. Arch. Gynak. (Munich), 182:675,

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38. Ellis, A.: Sexual psychology of human

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39. Finesinger,

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V., and Sulkow-itch, H. W.: Clinical, psychiatric and psychoanalytic study of case of male pseudohermaphroditism. Am.

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E.: A

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CLINICAL NOTES 371

The importance of psychiatry in the

sur-gery of th:s condition. S. Glin. North America, 27:1218, 1947.

41. Turner, H. H.: A syndrome of infantilism, congenital webbed neck and cubitus valgus. Endocrinology, 23:566, 1938. 42. Albright, F., Smith, P. H., and Fraser, R.:

Syndrome characterized by primary

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43. Varnev, R. F., Kenyon, A. T., and Koch,

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45. Dorif, C. B., Appebman, D. H., and Live-son, A.: Report of a case showing con-genital defects, short stature, retarded sexual development and no urinary gonadotropins.

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46. Martin, E., and Courvoisier, B.: Nanisme

et glande endocrine. Lo stetoscopio, 12: 835, 1951.

47. Talbot, N. B., Sobel, E. H., McArthur,

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D.: Functional

Endocrinology from Birth through Ado-lescence. Commonwealth Fund, Har-vard, 1952.

48. Prunty, F. T. C., .McSwiney, R. R., and

Clayton, B. E.: Primary gonadal in-sufficiency in a girl and a boy: metabolic effects of estrogen and testosterone.

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Glin. Endocrinol., 13:1480, 1953.

49. Flavell, G.: Webbing of neck with Turner’s syndrome in the male. Brit.

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Surg.,

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1955;16;363

Pediatrics

Edgar J. Schoen, Alexander L. King, A. LaMont Baritell and William F. Knigge

Report of a Case

PSEUDOHERMAPHRODITISM WITH MULTIPLE CONGENITAL ANOMALIES:

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1955;16;363

Pediatrics

Edgar J. Schoen, Alexander L. King, A. LaMont Baritell and William F. Knigge

Report of a Case

PSEUDOHERMAPHRODITISM WITH MULTIPLE CONGENITAL ANOMALIES:

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The natural radioactivity in the environment is the main source of radiation exposure for human body. Natural radionuclide in soil contributes a significant amount of

In this work, a simple bit loading algorithm (SBL) is proposed for a long-term evolution- advanced (LTE-A) vehicular channel to minimize the total energy required to

Based on proximate analysis, biscuits with the addition of nilem protein concentrate have a water content of 3.72%, ash content of 1%, protein content of 22.34% and fat content