Biomedical subjects
Publications and source records attributed to D Katznelson.
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Eighty-one children undergoing tonsillectomies and adenoidectomies (T and A) were compared with a control group of 88 hospital patients. A significantly higher incidence of prior T and A was found to have been performed on the siblings and parents of the T and A group than the controls. Environmental, genetic or attitudinal factors could be responsible for this familial clustering.
Uric acid metabolism was evaluated in 16 Israeli cystic fibrosis patients, all of whom were taking pancreatic enzyme supplements. The findings were compared with those in a recent study of uric acid metabolism in 65 healthy Israeli children. Hyperuricemia of 4.9 +/- 0.2 (SE) mg/dl was found in the cystic fibrosis patients, compared with a normal level of 3.1 +/- 0.2 mg/dl ( P < 0.05). In five patients, 24-h urine collections were assayed and all showed hyperuricosuria. Thirteen patients had high urinary uric acid/creatinine ratios. Hyperuricosuria increased with the age of the patient and when the administered dosage of pancreatic enzyme exceeded 10,000 lipase units/kg body weight. Despite normal levels of serum creatinine and urea, and normal creatinine clearance in the cystic fibrosis patients, risk of future renal damage seems obvious and preventive measures should be considered.
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Fat absorption was studied in 10 patients recovering from an episode of acute infectious gastroenteritis who failed to gain weight despite adequate caloric intake. Three patients restudied after clinical improvement and three other infants with failure to thrive, unrelated to gastrointestinal problems, served as control subjects. Fat balance studies during the ingestion of a formula containing long-chain fatty acids demonstrated significant degrees of steatorrhea in patients (mean CFA 70.6 +/- 10.7 compared to 90.3 +/- 2.4 in control subjects). The administration of a test meal demonstrated a marked deficiency of duodenal bile acid concentration and of fat incorporation into the micellar phase in patients. Fecal bile acid excretion was significantly increased in patients (mean 33.9 +/- 11.6 microM/kg/day) as compared to control subjects (mean 13.5 +/- 3.1 microM/kg/day). Bacterial overgrowth and abnormalities of the small intestinal mucosa were not constant. Ileal dysfunction and associated bile acid loss are possible causes of disturbed fat assimilation following acute intestinal infection in children.
Oral triglyceride (TG) loading tests were performed in four groups of children: normal controls, patients with chronic nonspecific diarrhoea (CND), biopsy-proved mucosal pathology (MP), and disturbed intraluminal fat malabsorption (IFM). The rise of plasma TG levels greater than or equal to 55 mg/100 ml (greater than or equal to 0.6 mmol/l) can discriminate between patients with gastrointestinal disease and normal controls or patients with functional disturbances. The postmeal plasma TG rise correlates well with the coefficient of fat absorption (CFA) in normal subjects, patients with CND, and patients with IFM, but not in patients with MP who showed a disproportionally low plasma TG rise compared with their CFA. This test can serve as a useful tool in diagnosis and clinical evaluation for children with gastrointestinal disorders.
Identical male twins suffering from congenital hypoaldosteronism due to a rare adrenal enzyme deficiency between corticosterone and aldosterone were followed-upfrom birth till their present age of 13 years. The symptoms of salt loss disappeared and normal growth rate resumed following treatment with DOCA and salt supplementation. Discontinuation of mineralocorticoid administration at the age of 7 years resulted during a 5-year period in a marked decline in their growth rate. Labororatory data revealed a persistent, albeit less pronounced, metabolic impairment. Mineralocorticoid administration was resumed and the twins entered normal puberty and increased their growth rate, emphasizing their need for continued mineralocorticoid administration to maintain adequate growth rate and development.
A study of acute gastroenteritis in children was carried out with the aim of establishing the prevalence of human reoviruslike agent (HRLA) and its relation to other enteric pathogens in Israel. The stools of 384 children with acute diarrhea referred to a pediatric emergency service were screened for HRLA by counterimmunoelectroosmorphoresis (CIEOP) and for pathogenic bacteria. Evidence of HRLA infection was found in 65 patients (17%). The highest infection rate prevailed during the cool season (25%), with a peak prevalence (41%) in November, when both the temperature and humidity were low. A very high proportion of HRLA was found in children younger than 36 months and no HRLA infection was observed in those older than nine years. The highest prevalence occurred in infants younger than six months, a situation rarely encountered in other countries. The main clinical features of HRLA infection were fever, vomiting, dehydration, signs of upper respiratory infection and carbohydrate intolerance. Bacterial pathogens accounted for 45% of enteric infections. Shigella species predominated (28%) during the summer season, especially in older children. In 38% of the study group, no etiologic agent could be detected. None of the 50 control subjects showed evidence of viral or bacterial pathogens in stools.
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Three cystic fibrosis infants with severe respiratory distress had increased intracranial pressure (with bulging fontanels) which cleared parri passu with improvement in the chest condition. It is proposed that the intracranial hypertension is a result of raised venous pressure, itself secondary to the intrathoracic obstruction to venous return, consequent on the bronchial obstructive disease.
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A total of 140 cases of cystic fibrosis in 110 families was identified in Israel during the years 1946--75, and there was reason to suspect inadequate ascertainment. Cystic fibrosis is frequent among the Ashkenazic and Arab populations with an incidence approximating that for Caucasians of European ancestry (1:1,800 to 1:4,000 live births). It is about half as frequent in the Sephardim, rare in the Iraqi or Yemenite Jews and perhaps absent in Iranian Jews. The clinical picture of cystic fibrosis in Israel in all the ethnic groups was found to be the same as elsewhere in the world. In-depth.interviews of 50 families were conducted to assess their understanding of the genetics of the disease and their attitude toward family planning.
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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.