An Autopsy Case of Congenital Lipoid Hyperplasia of the Adrenal Cortex

Tsutsui, Yoshihiro; Hirabayashi, Norio; Ito, Gen

doi:10.1111/j.1440-1827.1970.tb02748.x

Cited by 6 publications

(4 citation statements)

References 12 publications

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“…Light and electron microscopic studies have consistently shown that the adrenocortical cells are hyper plastic and filled with lipids and cholesterol [6][7][8], The testicular histopathology, however, has been poorly de fined. Although hyperplasia and lipid accumulation of Leydig cells have been described in a 14-month-old male patient with lipoid CAH [9], this appears to be the sole report clearly describing histological findings of Leydig cells.…”

Section: Introductionmentioning

confidence: 99%

Testicular Histopathology in Congenital Lipoid Adrenal Hyperplasia: A Light and Electron Microscopic Study

Aya

Ogata²,

Sakaguchi³

et al. 1997

Horm Res

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This paper describes the testicular histopathology in 2 male patients with congenital lipoid adrenal hyperplasia who underwent orchiectomy at age 7 (case 1) and 12 (case 2) years, respectively. In both cases, seminiferous tubules were normal in diameter (mean: case 1 88.7 μm; case 2 115.9 μm) and consisted of an age-appropriate number of type A spermatogonia (mean: case 1 1.7/tubule; case 2 4.0/tubule) and an increased number of Sertoli cells (mean: case 1 34.7/tubule; case 2 27.9/tubule). The interstitial space was abundant and contained normal to increased numbers of Leydig cells (mean: case 1 4.0/ tubule; case 2 6.1/tubule) that were filled with lipid droplets. We suspect that lipid accumulation in Leydig cells is consistent with defective conversion of cholesterol into pregnenolone and thus constitutes the characteristic feature of this disease, while the remaining findings appear to be common to disorders associated with hypergonadotropic hypogonadism.

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Section: Introductionmentioning

confidence: 99%

Testicular Histopathology in Congenital Lipoid Adrenal Hyperplasia: A Light and Electron Microscopic Study

Aya

Ogata²,

Sakaguchi³

et al. 1997

Horm Res

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“…In young patients, seminiferous tubules usually show both Sertoli and germ cells, although Sertoli-only tubules may also be seen [20,21]. In some patients, Leydig cells show lipid accumulation [20,[22][23][24], while others report an absence of lipids [4,19,[25][26][27]. Around puberty, most patients seem to show normal testicular morphology for their age [23,24], although some show lipid accumulation in the Sertoli cells [28].…”

Section: Lipoid Congenital Adrenal Hyperplasiamentioning

confidence: 99%

Perspectives in Pediatric Pathology, Chapter 6. Male Undermasculinization

et al. 2015

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Normal male development requires three conditions: (1) adequate differentiation of the fetal testis; (2) synthesis and secretion of testicular hormones; and (3) effective action of these hormones on target organs. This requires the combined action of the inhibitory anti-müllerian hormone (AMH, secreted by Sertoli cells) to block the development of the uterus and fallopian tubes from the müllerian duct, together with the trophic stimulus of testosterone (a Leydig cell product), which leads to virilization of the wolffian ducts. Additionally, the development of external genitalia depends on the conversion of testosterone to dihydrotestosterone by the enzyme 5-α-reductase. Failure of any of these mechanisms leads to deficient virilization or the so-called "male pseudohermaphroditism" syndromes.

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“…Additional patients with this biosynthetic defect have since been described (Prader and Siebenmann 1957;Dhom 1958;Nouri 1961;Sasano et al 1963;Bonnette et al 1964;O'Doherty 1964;Ohnishi 1964;Camacho et al 1968;Moragas and Ballabriga 1969;Tsutsui et al 1970;Iwamasa et al 1973;Kirkland et al 1973;Tanae et al 1976;Koizumi et al 1977;Hierro et al 1979). A disproportionate number of these patients were Japanese.…”

Section: E Cholesterol Desmolase Deficiencymentioning

confidence: 99%

Congenital Adrenal Hyperplasia

New

Levine

1984

Monographs on Endocrinology

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This work is subject to copyright. All rights are reserved, whether the whole or part of the material is concerned, specifically those of translation, reprinting, fe-use of illustrations, broadcasting, reproduction by photocopying machine or similar means, and storage in data banks. Under §54 of the German Copyright Law where copies are made for other than private use. a fee is payable to 'Verwertungsgesellschaft Wort'. Munich. 1[. Springer-Verlag Berlin. Heidelberg 1984 Softcover reprint of the hardcover 1 st edition 1984The use of registered names, trademarks, etc. in this publication does not imply. even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use.Product Liability: The publisher can give no guarantee for information about drug dosage and application thereof contained in this book. In every individual case the respective user must check its accuracy by consulting other pharmaceutical literature.

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An Autopsy Case of Congenital Lipoid Hyperplasia of the Adrenal Cortex

Cited by 6 publications

References 12 publications

Testicular Histopathology in Congenital Lipoid Adrenal Hyperplasia: A Light and Electron Microscopic Study

Testicular Histopathology in Congenital Lipoid Adrenal Hyperplasia: A Light and Electron Microscopic Study

Perspectives in Pediatric Pathology, Chapter 6. Male Undermasculinization

Congenital Adrenal Hyperplasia

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