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Biomedical subjects

E Lahat

Publications and source records attributed to E Lahat.

At least 73 records · Page 4Linked to original sources
PubMed1993

Regulation of synthesis of complement proteins in HEp2 cells.

We investigated the synthesis of complement proteins in HEp2 cells and its regulation. Using metabolic labeling with [35S]methionine, immunoprecipitation, and SDS-PAGE, we demonstrated that HEp2 cells synthesized C1r, C1s, C1 inhibitor, C3, C4, factor B, and factor H. The constitutive synthesis of C1r and C1s were the highest among these proteins, whereas the synthesis of C4 was observed only when the cells were stimulated with IFN-gamma. C1r and C1s were secreted readily, whereas the rate of the secretion of C4 and C1 inhibitor was much slower. Activated forms of C1r, C1s, and complexes of C1 inhibitor--C1s were found. IL-1 and TNF induced 6- and 7-fold increases in the synthesis of C3 and factor B. IFN-gamma induced increases in the synthesis of all seven proteins, the most pronounced effects being on the synthesis of C4 and C1 inhibitor--15- and 44-fold, respectively.

Complement System Proteins↗
PubMed1993

Antipyretic effectiveness of acetaminophen in febrile seizures: ongoing prophylaxis versus sporadic usage.

A controlled clinical study compared the antipyretic effectiveness of acetaminophen administered at regular 4h intervals (group 1, n = 53) versus sporadic usage contingent upon a body temperature above 37.9 degrees C (group 2, n = 51) in 104 children presenting with simple febrile convulsions. The incidence of febrile episodes or temperature values were similar in spite of significantly larger amounts of acetaminophen administered to patients in group 1. Four and 4 children in groups 1 and 2, respectively, had a second episode of febrile seizures, in all of them within the first 24h of admission. We conclude that the prophylactic administration of acetaminophen in children with febrile seizures is not effective in the prevention of fever, the reduction of its degree, or in preventing the early recurrence of febrile seizures.

Acetaminophen↗
PubMed1993

Chorea as a manifestation of rheumatic fever--a 30-year survey (1960-1990).

Sydenham chorea, a major manifestation of acute rheumatic fever, has been the most common form of acquired chorea during childhood. Despite the recent dramatic decline in both incidence and severity of rheumatic fever in our area, the frequency of carditis was unchanged. This study investigated retrospectively the incidence of chorea in the last three decades (1960-1990) in our area. During the 30 years of the survey, 28 patients with Sydenham chorea were treated in our centre of whom 10 were seen between 1960-1970, 17 between 1970-1980, and only one patient between 1980-1990.

Acute Disease↗
PubMed1993

Congenital contractural arachnodactyly in two double second cousins: possible homozygosity.

A Bedouin family with two girls affected by severe congenital contractural arachnodactyly (CCA) is described. The girls were double second cousins. One of the girls also had ambiguous genitalia, an anomaly not generally associated with this disorder. The two children were both the product of first-cousin Bedouin parents from the same family. It is possible that both sets of parents were heterozygous for CCA; thus the infants may have been homozygous for CCA, which is usually an autosomal dominant condition. No instance of homozygous CCA has previously been reported. This family suggests genetic heterogeneity in CCA and that, in some rare families, the mode of inheritance may be autosomal recessive.

Consanguinity↗
PubMed1992

Inhibition of human red blood cell glutathione reductase by valproic acid.

Glutathione reductase (GR) one of the enzymes of the glutathione redox cycle, plays a salient role in maintaining appropriate cellular levels of reduced glutathione. The enzyme in human red blood cells is inhibited in vitro by the anticonvulsant drug valproic acid (VPA). The inhibition is dose-dependent, reversible, uncompetitive and does not depend on the redox state of the enzyme. VPA also inhibits red blood cell GR activity in children being treated with the drug. The level of serum VPA correlates significantly with the suppression of GR activity.

Adolescent↗
PubMed1992

Severe periodic febrile myalgia in infancy due to carnitine palmitoyltransferase deficiency.

A 7 1/2-yr-old girl suffered, since early infancy, severe recurrent myalgia during periodic attacks of fever, vomiting and pharyngitis. Neither myoglobinuria nor exercise-induced muscle pain was present. She was found to have carnitine palmitoyltransferase deficiency (CPTD) in leukocytes, fibroblasts and muscle. This case exemplifies the importance of looking for an associated metabolic etiology of recurrent febrile myalgia even in the absence of myoglobinuria.

Carnitine O-Palmitoyltransferase↗
PubMed1992

Brainstem auditory evoked potentials in familial dysautonomia.

The brainstem function of eight children with familial dysautonomia was investigated by brainstem auditory evoked potentials and compared with that of age-matched healthy control children. All median latencies of waves III and V in the study group were significantly higher than those of the control children. Brain transmission times of waves I-III and I-V were significantly prolonged in the study group compared with the control group. These results provide additional objective support for the assumption that the brainstem function of children with familial dysautonomia is affected by this disease.

Adolescent↗
PubMed1991

Autosomal recessive lethal infantile cytochrome C oxidase deficiency.

Three bedouin children with mitochondrial myopathy due to cytochrome c oxidase deficiency presented with progressive muscle weakness, failure to thrive, proximal renal tubular acidosis, and lactic acidemia leading to death. Two died by age 5 months and one by age 16 months. Cytochrome c oxidase was markedly reduced in skeletal muscle extracts of all three. Three other children of the same family with most probably the same metabolic aberration are also described. We suggest an autosomal recessive inheritance for this lethal mitochondrial myopathy.

Acidosis, Renal Tubular↗
PubMed1991

Intraosseous neurilemmoma of skull--single case report.

Intraosseous neurilemmoma is a rare benign tumour of the bone with characteristic radiological and histological features. The most common places of this tumor are mandible, sacrum and vertebral bodies. A case of a not previously reported location of this tumor in the vault of the skull is presented.

Biopsy↗
PubMed1990

Recurrent seizures in children with Shigella-associated convulsions.

Fifty-five children with Shigella-associated convulsions were followed prospectively to investigate their risk of subsequent febrile or nonfebrile seizures. The duration of the follow-up period was between 6.9 and 14.1 years (9.7 +/- 3.1 years). No case of nonfebrile seizures and only 2 cases (3%) of subsequent febrile seizures were observed during this period. We conclude that although febrile and Shigella-associated convulsions share many clinical features, the natural history of these two conditions seems to be distinctly different. Shigella-related convulsions are not associated with an increased incidence of subsequent febrile or nonfebrile convulsions.

Child↗
PubMed1989

Pediatric brain stem tumor presenting as a pure motor hemiparesis.

The clinical syndrome of pure motor hemiparesis occurs with lacunar lesions in the internal capsule or in the basis pontis in hypertensive adults. It has been described in three patients with brain stem tumors of which two were children. A 3 1/2 years old boy with a brain stem tumor which presented as pure motor hemiparesis is described.

Brain Neoplasms↗