adreno leukodystrophy

The adreno-genital syndrome.

Anaesthesia in Adreno-cortical Hypereunction

PATIENTS WITH adreno-cortmal hyperfunctaon-are not frequently presented to anaesthetists. The proper anaesthetac management for adrenalectomy m these cases reqmres a basic understanchng of the physlopathology of the synch'omes caused by hyperfunetlon of the adrenal cortex. The adrenal cortex secretes three types of sterold hormones, file glucocorticolds, and the mmeralo-corticolds and hormones ...

[Globoid cell leukodystrophy].

A 29 year old male with onset of globoid cell leukodystrophy at age 14 is described. This is the first case of enzymatically confirmed globoid cell leukodystrophy with onset of symptoms after the age of ten. This patient is unique because of the late onset and slow progression and extends the clinical spectrum of globoid cell leukodystrophy. Globoid cell leukodystrophy is caused by the deficien...

The Effect of Potassium Deficiency upon Adreno- Cortical Secretion

The potassium-deficient rat exhibits several features in common with the untreated adrenalectomized rat, among which are subnormal growth. lowered blood pressure (1-3), diminished blood pressure responsiveness to pressor substances (4), and decreased tolerance to trauma. It therefore was considered important to determine whether the depressor response induced by potassium depletion might in fac...

Adreno-cortical tumors: clinico-pathological profile of five cases.

In children, upto two-thirds of all adreno-cortical neoplasms present with virilization with or without hypercortisolism(l). These tumors may however remain non-functioning or may present with varying symptom complexes including feminization and hyperaldosteronism(2). The clinical and biochemical features of five children with adreno-cortical neoplasms presenting over a period of five years (19...

MR of childhood metachromatic leukodystrophy.

PURPOSE To investigate the MR findings of childhood metachromatic leukodystrophy (MLD). METHODS Nine MR imaging studies in seven children (five girls and two boys, 10 to 32 months old) with MLD were evaluated retrospectively for the extent and progression of white matter abnormalities and the presence of contrast enhancement. RESULTS All seven cases showed symmetric, confluent high signal i...