[Endocrine manifestations of hydrocephalus].
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Biomedical subjects
Publications and source records attributed to B Liberman.
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We describe two patients with infantile nephropathic cystinosis who presented nephrogenic diabetes insipidus in addition to Fanconi syndrome. After the diagnosis was confirmed by slit-lamp examination that showed crystallization of the cornea and by the presence of cystine crystals in the bone marrow, the patients underwent conservative and supportive treatment including correction of acidosis, replacement of fluid losses and protection from bone demineralization with replacement of phosphorus, calcium and vitamin D. Patient 1 deceased after an episode of bronchopneumonia complicated by profound acidosis. Patient 2 was started on cysteamine which effectively reduce cystine in body tissues and prevents or slows progression of end-organ damage.
Pregnancy occurring in patient with Sheehan's syndrome is seldom described. It depends on the preservation of LH and FSH secretion after the pituitary apoplexy event. PURPOSE--To report a patient with Sheehan's syndrome who became pregnant twice after the pituitary apoplexy episode and to discuss the maintenance of gonadotrophic function. METHOD--Clinical aspects are described and the pituitary reserve evaluation was performed as well as a computerized tomography and a magnetic resonance imaging of the brain. RESULTS--Gonadotrophic and thyrotrophic function were preserved and the neuroradiologic evaluation disclosed an empty sella turcica preservation of the posterior lobe of the hypophysis. CONCLUSION--A patient with Sheehan's syndrome is reported in whom the LH and FSH pituitary secretion was preserved allowing normal pregnancy twice after the pituitary apoplexy.
We compared 1.5 T magnetic resonance (MR) image findings in 193 patients with congenital pituitary insufficiency. One hundred and thirty nine of the MR studies were obtained in patients who had isolated growth hormone deficiency (GHD). Other fifty-four patients had multiple pituitary hormone deficiency (MPHD). On MR images, normal anterior and posterior lobes of the pituitary gland can be clearly differentiated because the posterior lobe has a characteristic high intensity on T1-weighted images. In fifty-four patients, the high-intensity of the posterior lobe was not seen, but a similar high signal intensity was observed at the proximal stump in fifty-one patients. This high-intensity area is the newly formed ectopic posterior lobe, which also secrets anti-diuretic hormone just as the posterior lobe would. MR imaging can demonstrate the transection of the pituitary stalk and the formation of the ectopic lobe, revealing to be a usefull diagnostic tool in the definition of the type of alteration in growth defects of endocrine origin.