Hypothalamic hypopituitarism following Shigella encephalopathy.
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Biomedical subjects
Publications and source records attributed to B Z Garty.
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UNLABELLED: Herpes simplex virus (HSV) is an uncommon cause of acute laryngitis in immunocompetent patients since it mostly occurs in immunocompromised subjects. We present two previously healthy children with prolonged gingivostomatitis and stridor (lasting 3 and 4 weeks) in whom HSV-1 was isolated from subglottal ulcers. CONCLUSION: HSV should be considered a possible pathogen in cases of prolonged or atypical croup not only in immunocompromised or elderly patients but also in otherwise healthy children.
BACKGROUND: The worldwide increase in the incidence, prevalence, and severity of asthma may suggest that environmental factors play a role in these epidemiologic changes. OBJECTIVE: To examine the correlations between air pollutants, weather conditions, airborne allergens, and the incidence of emergency room (ER) visits of children with acute asthma attacks. DESIGN: One-year prospective study. Data of daily concentration of air pollutants, weather conditions, and selective airborne allergens were collected and compared with the number of ER visits of asthmatic children. SUBJECTS: 1076 asthmatic children (aged 1 to 18 years) who presented at the Pediatric ER between January 1 and December 31, 1993. RESULTS: Correlations between fluctuations in ER visits of asthmatic children and various environmental parameters were more relevant for weekly than for daily values. Emergency room visits correlated positively with concentrations of NOx, SO2 and with high barometric pressure; and negatively with O3 concentration and minimal and maximal temperature. There were no significant correlations with concentrations of particulates, humidity, or airborne pollen and spores. An exceptionally high incidence of ER visits of asthmatic children was observed during September. This peak coincided with the beginning of the school year and the Jewish holidays. The correlations between ER visits and the environmental factors increased significantly when the September peak was excluded, revealing that 61% of the variance in ER visits was explained by NOx, SO2, and 03 concentrations, 46% by weather parameters, 66% by NOx, SO2 and barometric pressure, and 69% by the combination of air pollutants and weather parameters. CONCLUSION: The major factors found to be associated with ER visits of asthmatic children were high NOx, high SO2, and high barometric pressure. Negative correlation was found between ER visits of asthmatic children and ozone concentrations. The particularly high number of ER visits at the beginning of the school year and the Jewish holidays was probably associated with an increase in the number of viral infections and/or emotional stress.
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A 12-year-old girl with recent history of pseudotumor cerebri developed severe postural headache due to intracranial hypotension after lumbar puncture. Meningeal enhancement and thickening were demonstrated on magnetic resonance imaging. Repeated imaging 3 months later disclosed no abnormalities. This is the first report of a child with evident meningeal enhancement on magnetic resonance imaging related to lumbar puncture.
OBJECTIVE: To evaluate whether the difference in aggregation of cerebrospinal fluid cells from patients with bacterial, viral, aseptic and partially treated meningitis can be used for diagnostic purposes. METHODS: Cerebrospinal fluid samples of 100 patients with meningitis (15 bacterial, 13 partially treated, 10 viral and 62 aseptic) were compared on the basis of the predefined leukocyte aggregation score (LAS). RESULTS: Mean LAS was 56% in the bacterial meningitis group (range, 15 to 90%), 5.8% in the partially treated meningitis group (range, 0 to 27%), 2% in the proven viral meningitis group (range, 0 to 5%) and 2% in the aseptic meningitis group (range, 0 to 15%). All patients with bacterial meningitis had a LAS of > 15%, whereas all those with viral or aseptic meningitis had a score of < 15%. Although most patients with partially treated meningitis had a low LAS, several had higher scores, which may indicate bacterial infection. There was no statistical correlation between number of cells, type of cells (mononuclear or polymorphonuclear) or cerebrospinal fluid protein and glucose concentration and degree of leukocyte aggregation for the different groups. CONCLUSION: Measurement of the LAS may contribute to the immediate differential diagnosis of bacterial or viral meningitis, especially in patients with very high pleocytosis, as sometimes seen in enteroviral meningitis. It may also serve as a guide for the likelihood of bacterial infection in cases of partially treated meningitis. Additional studies are needed to confirm these observations.
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Acute haemorrhagic oedema of infancy (AHEI) is an acute leucocytoclastic vasculitis affecting infants and young children. It has a striking appearance of large purpuric skin lesions in a target-like pattern and marked oedema mainly on the face, auricles and extremities. In some patients there is mucosal involvement as well. We present five patients with skin lesions characteristic of AHEI with no visceral involvement and complete resolution within 7-14 days. In three of our five cases, histopathological examination was performed, and demonstrated typical leucocytoclastic vasculitis. Although sometimes confused with Schönlein-Henoch purpura, we suggest that AHEI should be regarded as a separate entity. Clinical criteria for diagnosis are proposed.
Acute febrile neutrophilic dermatosis (Sweet syndrome) is rare in children and is regularly associated with underlying malignancies or inflammtory diseases. A 5-year-old girl with glycogen storage disease type Ib, neutropenia, and recurrent infections developed characteristic skin eruption of Sweet syndrome after 2 years of granulocyte colony-stimulating factor (G-CSF) therapy. This association points to a possible role of G-CSF-induced granulopoiesis and granulodyte activation in the pathogenesis of Sweet syndrome.
We studied the prevalence of myopia in 17-year-old Israeli military recruits with neurofibromatosis type 1 (NF1). Twenty-four percent of the youngsters with neurofibromatosis had myopia, compared to 19% of the controls; this difference was statistically significant (P < 0.03). The recruits with NF1 had a slightly lower IQ, fewer years of education, and lower height and weight than the controls. Thus, these factors, which have been reported to be associated with myopia, did not significantly influence the high prevalence of myopia in the neurofibromatosis subjects. A high prevalence of myopia seems to be an additional feature of NF1.
Patients with glycogen storage disease type 1B present the clinical features of glycogen storage disease type 1A. In addition, they are susceptible to recurrent bacterial infection and have recurrent oral ulcers and occasionally Crohn's-like disease. There features are due to immune dysfunction caused by a combination of neutropenia and impaired phagocytic functions.
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We describe 2 sibs (brother and sister) with myopathy, sideroblastic anemia, lactic acidosis, mental retardation, microcephaly, high palate, high philtrum, distichiasis, and micrognathia. Very low levels of cytochromes a, b, and c were detected in the patients' muscle mitochondria. Deposition of iron within the mitochondria of bone marrow erythroblasts was observed on electron microscopy. Irregular and enlarged mitochondria with paracrystalline inclusions were also seen on electron microscopy of the patients' muscle specimen. Examination of DNA from the affected sibs showed no deletions in the mitochondrial DNA nor the mutations identified in the syndromes of mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS) or myoclonus, and epilepsy associated with rugged-red fibers (MERRF). Since the parents were first cousins and 2 of 6 sibs (male and female) were affected, we suggest that the syndrome expressed by our patients represents a previously unknown autosomal recessive disorder that includes mitochondrial myopathy, lactic acidosis, and sideroblastic anemia.
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A 13-year-old boy with hyperimmunoglobulin E (hyper-IgE) syndrome presented with headache, blurred vision, photophobia and bilateral papilledema due to cryptococcal meningitis. Treatment with amphotericin B, and S-fluorocytosine for several weeks and repeated lumbar punctures did not reduce the intracranial pressure, and a myeloperitoneal shunt was performed. The child was maintained on fluconazole for an additional six months. Patients with hyper-IgE syndrome are at increased risk of opportunistic fungal infections such as cryptococcal meningitis.
Zinc modulates the activity of glutamic acid decarboxylase, the rate limiting enzyme in the synthesis of gamma-aminobutyric acid (GABA), which is a major inhibitory neurotransmitter. Low cerebrospinal fluid GABA values have been reported in association with several seizure disorders, including febrile convulsions. It is also known that fever and/or infections may cause a reduction in serum zinc concentrations. In this study the hypothesis that febrile convulsions are related to low cerebrospinal fluid zinc was tested. Cerebrospinal fluid zinc concentrations were measured in 66 febrile children: 32 with febrile convulsions, 18 with fever but without convulsions, and 16 with aseptic (viral) meningitis. There was no statistically significant difference in the cerebrospinal fluid zinc between the three groups of children, and the mean concentration was 26.2 micrograms/l. No significant relationship was found between either age, gender, maximal temperature, type of infection, or time of performance of the lumbar puncture and cerebrospinal fluid zinc concentration. These results do not support the hypothesis that febrile convulsions are related to reduced cerebrospinal fluid zinc concentrations.