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Biomedical subjects

L Hornstein

Publications and source records attributed to L Hornstein.

At least 19 recordsLinked to original sources
PubMed1995

The diagnostic reliability of anti-endomysial antibody in celiac disease: the north Israel experience.

Assessment of anti-endomysial antibody (EMA) for the diagnosis and follow-up of celiac disease (CD) is widely accepted, but its applicability has never been investigated in Israel. We therefore investigated EMA as a diagnostic tool in a pediatric population, residing in the northern part of Israel, with suspected celiac disease. EMA was assessed by an indirect immunofluorescence assay using monkey esophageal sections as a substrate in 22 active CD, 17 non-active CD, and 22 non-CD children. EMA titers were correlated with small bowel mucosal pathology. Sensitivity and specificity of EMA were 100%. EMA levels are diet dependent, increasing on a gluten-containing diet and decreasing with gluten withdrawal after 3-12 months. EMA is a reliable marker for the diagnosis of CD, and reflects gluten intake changes and patients' dietary compliance. In our laboratory, EMA is suitable for the diagnosis and follow-up of CD.

Atrophy↗
PubMed1993

Cardiovascular abnormalities in children with fragile X syndrome.

It has been suggested by several authors that the prevalence of mitral valve prolapse and aortic root dilation is increased in individuals with the fragile X syndrome. The prevalence of these abnormalities in young children with fragile X has not been established. Sixteen boys and 1 girl 13 years or younger were studied. Only 1 child (6%) had the typical examination findings of mitral valve prolapse, although his echocardiogram was normal. From this study it appears that cardiac abnormalities in fragile X syndrome develop in late childhood and adolescence. It seems important to follow these children carefully so that these cardiac abnormalities can be documented when they do develop.

Adolescent↗
PubMed1991

Radio-ulnar synostosis in Williams syndrome. A frequently associated anomaly.

Limitation of supination and pronation of the forearm has been occasionally described in individuals with Williams syndrome. It was found in 26% of 23 subjects examined, and was associated with a spectrum of severity of radio-ulnar synostosis on radiography. The occurrence of this functional motor impairment in a substantial subset of persons with Williams syndrome is relevant, because its evaluation is important in habilitation planning.

Abnormalities, Multiple↗
PubMed1991

Hepatitis B virus infection in Ethiopian immigrants to Israel.

Two groups of immigrants from Ethiopia, one of 86 and the other of 165 individuals, aged 0-40, were examined for hepatitis B virus (HBV) infection in 1987-88, 3-7 years after their arrival in Israel. The results were compared with those obtained in the same age-group among Ethiopians who immigrated to Israel in 1980-82. The immigrants were found to be in good physical condition, their liver function tests were normal and no clinical evidence of chronic liver disease was found. Of the 22 children aged 0-4, 16 had anti-HBs as a result of vaccination at birth against HBV and they were excluded from the comparative study. In the age-groups 5-40 there was no significant change in the percentage of individuals positive for HBsAg, anti-HBs or anti-HBc only, compared with the group examined in 1980-82. There were two significant findings in this study: a) In 1987-88 [corrected], 8-9% of HBsAg-positive individuals had HBeAg and 64-81% had anti-HBe, while in 1980-82, 36% of those positive for HBsAg had HBeAg and only 25% had anti-HBe. b) At the time of arrival recent infection by HBV was indicated by the presence of IgM anti-HBc in 57% of those positive for HBsAg and 21% in whom anti-HBc was the sole serological HBV marker. In 1987-88 no IgM anti-HBc was found in HBsAg-positive persons or in those with anti-HBc only. These results indicate that most HBV infections in this population had occurred before their arrival in Israel. There is a profound change in the epidemiology of HBV infection in this Ethiopian population following immigration, which is probably due to environmental changes as well as to vaccination against HBV of all young children aged less than or equal to 3 years.

Adolescent↗
PubMed1990

Excretion of urinary protein induced by extracorporeal piezoelectric lithotripsy.

An investigation was carried out into renal injury caused by extracorporeal piezoelectric lithotripsy (EPL) using an EDAP lithotriptor. Four urinary proteins, with a molecular weight range of 160000-14500, immunoglobulin G (IgG), N-acetyl-beta-glucosaminidase (NAG), albumin and lysozyme, were monitored in 27 patients 1 day before and 1, 7, 30, 90 and 180 days after unilateral EPL treatment. All patients had non-obstructive renal stones, previously untreated. Apart from 5 patients with stablised hypertension and 6 with persistent urinary infections due to the infected stones, all patients appeared healthy, as confirmed by clinical, haematological and biochemical investigations. Only albumin levels increased significantly 1 day after treatment; statistically nonsignificant increases and decreases were recorded in the levels of NAG and lysozome respectively. IgG was beyond the limit of detection (less than 0.5 mg%) in all patients. The albumin level returned to normal 7 days after treatment. The EPL-induced increase in albumin was recorded in 88% of patients, compared with increased levels of NAG in 46% and lysozyme in 64%, mainly in those with infected stones. These findings indicated a transient glomerular injury after EPL treatment.

Acetylglucosaminidase↗
PubMed1990

Persistent Schistosoma mansoni infection in Yemeni immigrants to Israel.

Following sporadic reports on persistent Schistosoma mansoni (S.m) infections in Israelis of Yemeni origin, we systematically surveyed a group of 129 individuals who immigrated to Israel 38 years ago. Physical examination was uneventful in all members of the study group. A single stool examination revealed S.m eggs in 15 of 129 subjects (12%). Specific anti-S.m. IgE was detected in the sera of 48 individuals (37%). Among the 15 egg-positives, 14 had specific IgE (sensitivity 93.3%), but only 80 of the 114 egg-negatives were also negative for specific IgE (specificity 70%). This specific IgE positivity among egg-negatives in the study group (34/114) and its complete absence from two control groups, one of them comprising Yemenites born in Israel, suggest either an S.m infection with low egg output stemming from a low worm burden or low fecundity of senescent worms, or occult infections. Since the S.m infections could not have been contracted in Israel, our findings point to inordinately persistent infections in the members of our study group, and incidentally also to the diagnostic usefulness of specific IgE testing in such cases.

Adult↗
PubMed1990

Evidence for the existence of circulating monoclonal B-lymphocytes in multiple myeloma patients.

Multiple myeloma is characterized by the proliferation of a single clone of plasma cells producing a homogeneous immunoglobulin fraction. In this disease, plasma cells home essentially in the bone marrow. However, controversy exists whether peripheral blood B-lymphocytes in patients with multiple myeloma (MM) are part of the malignant clone. We investigated clonal immunoglobulin gene rearrangement (IgGR) in T-cell-depleted peripheral blood mononuclear cells as well as in bone marrow of these patients. Seven out of 17 MM patients demonstrated an identical IgGR in bone marrow and peripheral mononuclear cells, these patients were in an active stage of the disease. In nine patients in plateau phase, clonal IgGR could not be detected in peripheral blood. Peripheral mononuclear cells from ten patients with monoclonal gammopathies of undetermined significance (MGUS) were also examined and no IgGR was detected. The existence of monoclonal B-lymphocytes in the circulation of patients with MM suggests a mechanism whereby the malignant clone homes in the bone marrow through peripheral blood. These findings may also be used for the evaluation of patients with active myeloma and the determination of plateau phase.

B-Lymphocytes↗
PubMed1989

Stroke in an infant prior to the development of manifestations of neurofibromatosis.

Although the association of strokes and von Recklinghausen neurofibromatosis (NF-1) in young children is uncommon, it is obviously an important complication of this disorder. The few cases that have been described were reported primarily in the radiological literature. Moreover, most of the children reported were already known to have NF-1 or they had a positive family history for it. We report an infant who, at 7 weeks of age, suffered a stroke with resulting hemiparesis, prior to the development of other manifestations of NF-1. Pediatricians and neurologists need to be aware of this association and of the need for careful follow-up of infants with strokes, with particular attention to signs of neurofibromatosis.

Cerebrovascular Disorders↗
PubMed1989

Fragile X syndrome: recognition in young children.

In recent years, a number of articles have appeared in the literature concerning the fragile X syndrome; however, in few cases was the diagnosis of the syndrome in young children discussed. A review of 20 children younger than 7 1/2 years of age who had the fragile X syndrome seen at the Cincinnati Center of Developmental Disorders was undertaken in an attempt to establish guidelines that would aid the practicing physician in determining which children should have a chromosomal analysis. All children were developmentally delayed; 95% had speech delays. Short attention span with hyperactivity, temper tantrums, mouthing of objects persisting at an age beyond when it would be expected, autistic behaviors, and poor gross motor coordination were seen in 50% or more of the children. Mental retardation was present in the family history of 65%, and 90% had a family history of at least one of the following: mental retardation, learning disabilities, or hyperactivity. The most common physical findings were long and/or wide and/or protruding ears, prominent jaw and/or long face, high arched palate, and a flattened nasal bridge. The fragile X syndrome can be recognized by noting key aspects of the behavioral and family histories as well as the physical findings.

Age Factors↗
PubMed1988

Bone marrow biopsy in multiple myeloma: a clinical pathological study.

Forty-two bone marrow aspirates and biopsies during follow-up examinations from patients with multiple myeloma were reviewed to determine whether the results correlate with the clinical state of the patient at the time of examination. The percentage of plasma cells on biopsy and aspiration, cytological immaturity, patterns of plasma cell infiltration, and the presence or absence of multiple lymphoid nodules and marked fibrosis were cross-tabulated with clinical parameters (hemoglobin levels, osteolytic lesions, and renal function). Hemoglobin levels less than 10 g/dl were more frequent in those with greater than 70% plasma cells on either aspiration or biopsy (P less than 0.05). A nodular histological pattern on biopsy, however, had a higher correlation with hemoglobin levels less than 10 g/dl, and serum creatinine levels greater than 2 mg/dl, than did plasma cell number. The presence of lymphoid nodules correlated with less lytic bone lesions. The degree of fibrosis and plasma cell immaturity did not correlate with any of the clinical parameters. Our findings suggest that reports on bone biopsies should include in addition to the number of plasma cells, the pattern of plasma cell infiltration and the presence or absence of multiple lymphoid nodules.

Biopsy↗
PubMed1987

Linear nevi, hemihypertrophy, connective tissue hamartomas, and unusual neoplasms in children.

The association of cutaneous nevi, hemihypertrophy, and connective tissue overgrowth has been described in many patients. We report long-term follow-up in two boys with such association who in addition developed testicular neoplasms. One patient also had progressive spinal cord compression as a result of stenosis of the spinal canal, and required amputation of a severely deformed limb. The importance of careful, long-term management in such patients is emphasized.

Adenocarcinoma↗
PubMed1987

Uncombable-hair syndrome.

Four children had short, unmanageable, pale blond hair. They had no associated abnormalities and no family histories of abnormal hair. Light microscopy of the hair was normal in three patients, with pili torti present in the fourth. Electron microscopy of hairs from all four children revealed longitudinal grooves in the hair shaft, diagnostic of uncombable-hair syndrome.

Child↗
PubMed1986

Bisalbuminaemia in pancreatic ascites.

A patient with pancreatic ascites is described. Electrophoresis of the patient's serum demonstrated bisalbuminaemia, which disappeared after resolution of the ascites.

Albumins↗
PubMed1985

Metacarpophalangeal pattern profile analysis in Sotos syndrome.

The metacarpophalangeal pattern profile (MCPP) was analyzed on 16 Sotos syndrome patients. A mean Sotos syndrome profile was produced. Correlation studies confirm clinical homogeneity of Sotos syndrome individuals. Discriminant analysis of Sotos syndrome patients and normal individuals produces a function of two MCPP variables and age, which may provide a useful tool for diagnosis.

Adolescent↗
PubMed1985

Monocyte suppressor function in burns: T cell-monocyte interaction in mediating suppression.

Reduced in vitro T cell mitogen-induced transformation, low proportion of T cells and increased proportion of non-T cells were found in blood mononuclear cells of patients with severe burns 3-12 days after the injury. High spontaneous proliferation of non-T cells was observed and could be related mainly to the B cell fraction. Monocytes mediated suppression of mitogen-stimulated T cell proliferation. We further studied the role of monocytes in the enhanced suppressor activity of Con A-activated T cells and found that in this assay system, the patient's T cells mediated suppression in collaboration with monocytes. In vitro, increased suppressor function was probably the result of in vivo stimulation of inhibitory activity ascribed to both monocytes and T cells of patients. Addition of indomethacin to cell cultures markedly reduced suppression of lymphocyte proliferation. Less significant reduction was noted when the patient's T cells were activated in vitro by Con A. Adjuvant treatment of burn patients with indomethacin may play a role in alleviating suppression of immune response in these patients.

Adolescent↗