PubMed HealthSearch

Biomedical subjects

Y Rotem

Publications and source records attributed to Y Rotem.

At least 37 records · Page 2Linked to original sources

Familial cholestatic cirrhosis associated with Kayser-Fleischer rings.

A brother and sister who suffered from pruritus since infancy developed hepatic cirrhosis early in life. Although this clinical picture has never been seen in Wilson's disease, Kayser-Fleischer rings in the boy made further studies necessary. Oral radiocopper loading tests administered to both children and to their parents served to exclude Wilson's disease conclusively. Determinations of the concentrations and patterns of bile acids in the serum indicated that the abnormalities observed in these children are not related to errors in bile acid synthesis. Although a defect in bile acid transport is present, it appears to have occurred as a consequence of the liver disease.

Child

Quantitation of renal antigen excretion in the urine of normal children and of children with various renal diseases. I. Quantitation of renal antigens in random urine samples.

This study reports a serologic method for the measurement of kidney-derived antigens in the urine of healthy children and of children with renal diseases. Two hundred twenty patients were studied. Four groups were recognized: group A, patients with no evidence of renal disease; group B, patients with past history of active urinary tract infection; group C, patients with active urinary tract infection; group D, patients with other renal diseases. Urinary renal antigen concentration was tested by the complement fixation method, in which titers of antigens in the urine were compared with a standard human renal antigen extract. The distribution of renal antigen concentrations in group C differed significantly (P(X2Y less than 0.001) from the other three groups. About 85% of patients in groups A, B, and D had levels below 0.6 mg/ml, whereas in group C only 53% of patients had similar concentrations. After factoring the results by the urinary concentration of creatinine, 85% of patients in group C had antigen levels above 0.6 mg/ml as opposed to 24%, 44%, and 27% in groups A, B, and D, respectively. The results of the study are consistent with the assumption that the rate of discharge of renal antigenic material in the urine is accelerated in certain renal diseases.

Antigens

Salt conservation in familial dysautonomia (Riley-Day syndrome).

In some patients with familial dysautonimia, plasma renin activity shows a paradoxical response to postural stimuli, i.e., levels of plasma renin activity are high when the patient is in the supine position and fall significantly during subsequent ambulation. Furthermore, there is no coordinated release of plasma renin activity and aldosterone. The aim of the present study was to determine whether these findings are accompanied by a disturbance of salt conservation. Six patients were studied in a summer camp while on normal and low-salt diets. Plasma and urinary aldosterone levels rose sharply and appropriately when four of the patients were placed on a low-sodium diet. In these subjects, urinary sodium output fell sharply although three of them failed to attain sodium equilibrium by the third day of the low-sodium regimen. Elevation of early morning plasma renin activity appeared to correlate with an inversion in the normal day-night rhythm in urinary volume.

Adolescent

The association of HL-A-B8 and childhood celiac disease in an Israeli population.

The frequency of histocompatibility antigens (HL-A) was studied in 33 children with celiac disease. HL-A-B8 phenotype was detected in 27.3% of the celiac patients and 7.5% of 395 carefully matched control subjects (X2 for heterogeneity=14.23; P less than 10(-4)). HL-A typing, especially in children, is advocated both as a diagnostic and a prognostic measure. The importance of ethnically matched control subjects is discussed together with a proposal for the construction of such control groups.

Adolescent

Lymphangiomatosis with splenic involvement.

Severe splenomegaly and anemia developed in a 5-year-old girl with diffuse lymphangiomatosis of the upper part of the body. Radioisotope scanning and celiac angiography demonstrated lymphangiomatosis of the spleen, a rare but diagnosable condition. Intractable infection in areas of ulcerated skin led to her death from overwhelming sepsis.

Anemia

Congenital malformations in four siblings of a mother taking anticonvulsant drugs.

Four siblings have various congenital malformations attributable to the teratogenic effect of anticonvulsant drugs. Their mother has 23-year history of continuous medication for seizures. Since the malformations noted in her four offspring are more extensive and severe in each subsequent child, the question arises as to the possible cumulative effect of antiepileptic drugs in producing congenital malformations. The observations in this family strongly support the need to carefully evaluate all offspring of mothers receiving anticonvulsant drugs.

Abnormalities, Drug-Induced

Reconstruction of upper cervical esophagus.

We describe a surgical technique for reconstruction of the upper cervical esophagus after a segment has been excised. The technique involves the downward rotation of a full thickness pharyngeal flap to close the defect. The flap is obtained from the posterior wall of the hypopharynx and oropharynx. We describe a case in which this technique was used.

Child

A case of maternal hyperparathyroidism presenting as neonatal tetany.

A three-week-old infant presented with tetany and with serum calcium and phosphate levels compatible with the diagnosis of hypoparathyroidism. Investigation of the asymptomatic mother revealed serum calcium and phosphate levels and urinary phosphate clearance values diagnostic of hyperparathyroidism. The maternal hyperparathyroidism was treated by removal of a parathyroid adenoma.

Adenoma