Primary empty sella, galactorrhea, hyperprolactinemia and renal tubular acidosis

Primary empty sella, galactorrhea, hyperprolactinemia and renal tubular acidosis

Hyperproiactinemia Gaiactorrhea Human growth hormone intracellular cations Electrolyte disturbances Chondrocaicinosis Spironoiactone A young man. i...

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Hyperproiactinemia Gaiactorrhea Human growth hormone

intracellular cations

Electrolyte disturbances Chondrocaicinosis Spironoiactone

A young man. investigated because of tetanic convulsions and arthritic pains, was shown to have hypomagnesemia, hypermagnesuria, hypokalemia. hypercalciuria, progressive nephrocalcinosis and chondrocalcinosis. Renal function was normal except for the abnormal excretion of electrolytes. Renal sodium conservation was normal. Light and electron microscopic studies of renal biopsy specimens disclosed several abnormal tubules. lmmunofluorescent staining showed deposits of immunoglobulins in the glomeruli and tubules. Magnesium therapy was started and the progression of nephrocalcinosis was halted. Results of the morphologic and electrolyte balance studies are discussed. The patient was found to exhibit several features which have not been described before in connection with hypomagnesemia of unknown origin.

Runeberg L, Collan Y, Jokinen EJ, Lahdevirta J, Aro A: Hypomagnesemia due to renal disease of unknown etiology. Am J Med 59: 873-881. 1975.

Magnesium loss

Renal disease

Discussed here is a 41 year old woman with galactorrhea associated with the empty sella syndrome and mild renal tubular acidosis. Basal serum protactin (PRL) levels were normal, but a 24 hour serum PRL secretory profile demonstrated an increased mean PRL concentration. Serum PRL was appropriately suppressed by the administration of Ldopa; however, chlorpromazine stimulation resulted in a blunted serum PRL response. Pituitary luteinizing hormone, follicle stimulating hormone, ACTH and thyroid stimulating hormone were normal. Thus, galactorrhea associated with an enlarged sella does not establish the diagnosis of a pituitary tumor, and pneumoencephalography must be performed to exclude the empty sella syndrome.

Bar RS, Mazzaferri EL, Malarkey WB: Primary empty sella, gahctorrhea, hyperprolactinemis and renal tubular acidosis. Am J Med 59: 863-866, 1975.

Renal tubular acidosis

Empty seiia syndrome immune complex disease

Giomeruionephritis Ultrastructure

Cryoprecipitates

Steatorrhea

Chronic diarrhea

IgA deficiency ACTH secretion

A 59 year old woman with insulin-dependent diabetes mellitus and chronic diarrhea was found to have mild steatorrhea. selective plasma IgA deficiency and adrenal insufficiency. Significant adrenal secretion of corticosteroids resulted only after prolonged stimulation with large doses of exogenous ACTH. Plasma ACTH levels were not elevated during clinical adrenal insufficiency or after metyrapone administration but did respond normally to vasopressin and insulin-induced hypoglycemia. These studies were interpreted as showing both primary adrenal insufficiency and impaired pituitary reserve for ACTH secretion in response to the feedback stimulus. No deficiency was found in secretion of other pituitary tropic hormones. Jejunal biopsy showed a lack of IgA-containing plasma cells. With cortisone replacement. diarrhea subsided and a malabsorption pattern on a film of the small bowel was no longer seen. IgA deficiency has been noted frequently with steatorrhea but rarely with diabetes and only once previously with adrenal insufficiency.

Hagen GA, Bolman RM Ill, Frank JP: Atypical adrenal insufficiency with failure of the pituitary feedback receptor. A case with associated diabetes mellitus and selective IgA deficiency with steatorrhea. Am J Med 59: 882-888, 1975.

Adrenal insufficiency

Diabetes meiiitus

Comparative studies of renal biopsy specimens and in vitro cryoprecipitate were carried out in a patient with mixed immunoglobulin G (IgG)-immunoglobulinM (IgM) cryoglobulinemia associated with glomerulonephritis. The IgM isolated from the cryoprecipitate was an antibody with anti-IgG activity. Proliferative endocapillary glomerulonephritis was found in the kidney, with large amorphous deposits in the capillary walls. On immunofluorescent examination, these deposits contained IgG and C3. Ultrastructural studies of both cryoprecipitate and glomerular deposits revealed unusual structures designated as “cylindrical or annular bodies.” The morphologic characteristics of these bodies were exactly the same in the kidney and in the cryoprecipitate. These findings suggest an identity between the glomerular deposits and the circulating cryoglobulin, supporting the hypothesis that the glomerulonephritis reported here is an immune-complex disease.

Cordonnier D. Martin H, Groslambert P, Micouin C, Chenais F, Stoebner P: Mixed IgG IgM cryoglobulinemia with glomerulonephritis. Immunochemical, fluorescent and ultrastructural study of kidney and in vitro cryoprecipitate. Am J Med 59: 667-872, 1975.

Giomeruiar deposits

Cryogiobuilnemia