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[Congenital lipoid hyperplasia of the adrenal gland and male pseudohermaphroditism: clinical aspects of two siblings].

Two siblings of consanguinous parents, one male and one female, presented with symptoms of adrenal insufficiency related to respiratory infection at the age of two and three months, respectively. Besides a reduction of the synthesis of gluco- and mineralocorticoids, the sexual hormones were found to be reduced as well. Therefore, the boy showed a female sexual phenotype (male pseudohermaphroditism). Additionally, minor malformations including epicanthal folds, anti-mongoloid palpebral fissures, low-set ears were noticed, which have not been reported in children with the suspected diagnosis previously. The female sibling had typical Addison's crisis twice during the following years. Endocrinological tests yielded evidence for Cholesterol-20,22-desmolase deficiency.

Adrenal Cortex Function Tests↗

[The computer tomographic differential diagnosis of benign and malignant lipoid masses in the mediastinum and their spread (author's transl)].

Details of the tissues in the mediastinum can nowadays be demonstrated by computer tomography (CT) pictorially and quantitative measurements of their density coefficient carried out. The diagnosis of a fat-containing tumour or of benign lipomatosis of the mediastinum and their differentiation from malignant lesions is important for treatment and prognosis. In the past this could only be done by surgery. CT permits this differentiation pre-operatively and by a non-invasive method. The extent of malignant tumours and their relationship to other mediastinal structures and their spread beyond the mediastinum can be determined.

Adolescent↗