Posterior fossa tumors presenting as meningoencephalitis.
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Biomedical subjects
Publications and source records attributed to I Varsano.
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BACKGROUND: Sudden onset of daytime urinary frequency of a small amount of urine in a previously toilet-trained child can be a disturbing problem. Psychosocial problems, problems at school, or problems within the family have to be taken into consideration. METHODS: Fifteen children with sudden onset of isolated daytime urinary frequency were evaluated and followed for a period of 12 to 18 months. A thorough medical and family history was obtained for each child, with special attention given to any psychosocial problems the child had experienced. All the children underwent a physical examination, complete urinalysis, and ultrasonography of the kidneys and bladder. RESULTS: In all of the patients, the urinalysis and ultrasonographic findings were within normal limits. A trigger factor was identified as the cause of urinary frequency in each case. CONCLUSIONS: Because urinary frequency is usually a benign self-limited condition, an extensive urological evaluation is not indicated. In most cases, providing reassurance to the parents and the child is the only intervention necessary.
We describe 2 Arab patients, both offspring of unrelated consanguineous matings, with unusual facial appearance, severe mental retardation, microcephaly, cortical atrophy, seizures, hypotonia, dwarfism, and recurrent infections with neutrophilia. Neutrophil motility was markedly decreased but the opsonophagocytic activity was normal. Both patients lack the red blood cell (RBC) H antigen and manifest the Bombay (hh) phenotype. Familial endocardial fibroelastosis and familial tetralogy of Fallot segregated independently in one family. The occurrence of the same syndrome in 2 unrelated families suggests that the various aspects of the disorder are the pleiotropic effects of a single mutation. Homozygosity-by-descent for a deletion involving contiguous genes may explain the findings in this syndrome. Alternatively, a mutation which involves an ubiquitous GDP fucose donor rather than the enzyme (alpha 2-L-fucosyltransferase) or its substrate (glcNAc) may account for the pleiotropic manifestations in this syndrome.
Although acute transverse myelitis is a rare complication of mumps, it is relatively well documented. We describe a child who developed mumps associated acute transverse myelitis and who subsequently recovered completely. To our knowledge, only 13 cases have been reported in children. This case is compared with 13 previously reported patients.
Recurrent parotitis in children is a well described but rare condition of unknown cause. The clinical features of 11 children with recurrent parotitis are described.
The possible association of urinary tract infection (UTI) with ritual circumcision on the eighth day of life was studied by analyzing the epidemiology of urinary tract infections during the first year of life in 169 children with UTI (56 males and 113 females) born in Israel from 1979 to 1984. Forty-eight percent of the episodes of UTI occurring in males appeared during the 12 days following circumcision, and the increased incidence during that period was highly significant. The median age of the males at the time of the UTI was 16 days, compared with seven months in females. Ritual Jewish circumcision as practiced in Israel may be a predisposing factor for UTI during the 12-day period following that procedure.
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The prevalence of asthma in 2,160 schoolchildren from Arab and Jewish rural and urban communities in central Israel, was determined by means of questionnaire. For the purposes of the study, asthmatic children were defined as those suffering from two or more asthmatic episodes which had been diagnosed by their physicians and had necessitated anti-asthmatic therapy. Doubtful cases were classified after personal interviewing and physical examination. The overall prevalence of asthma was 6.38%. In the Arab rural sample, it was 2.86%, significantly lower than the other groups studied: Arab urban 6.63% (P < 0.001), Jewish urban 9.2% and Jewish rural 8.66% (P < 0.001). The relatively low prevalence in the Arab rural sample is in accordance with the rarity of asthma reported from less advanced societies in other countries. The reasoning for the low asthmatic morbidity found in this group is not clear.
Two cousins and an unrelated patient, all offspring of consanguineous parents, presented with Peters anomaly, unusual facial appearance, disproportionate short stature, retarded skeletal maturation, and a variable degree of mental retardation. Variable digital, cardiac, CNS, and urogenital anomalies were present. The inheritance is probably autosomal recessive. The condition is a distinct clinical entity for which we suggest the eponym Krause-Kivlin syndrome. Peters anomaly is thought to result from abnormal migration of neural crest cells. A similar mechanism was implicated in the pathogenesis of other disorders of the anterior chamber. The presence of Peters anomaly, and possibly of other corneal endothelial disorders in a newborn infant, should alert the clinician to the possibility of this syndrome. Communicating hydrocephalus (or brain atrophy) and polyhydramnios were documented in two patients, potentially allowing prenatal diagnosis in secondary familial cases.
Jaundice associated with breast feeding is a frequent problem facing the paediatrician. Despite numerous reports on this subject, the natural history, familial occurrence and late neurodevelopment of children with breast milk jaundice remain unclear. The follow up of 60 infants with breast milk jaundice showed that there are two bilirubin peaks, on the 4th and 5th day and on the 14th-15th day of life. In the infants with uninterrupted breast feeding, the hyperbilirubinaemia disappeared slowly and could still be detected 12 weeks after birth. The familial incidence of 13.9%, indicating that in some cases a unique genetic factor is expressed. Late neurodevelopment or hearing defects were not observed, thus enabling the paediatrician to encourage continuation of breast feeding in most cases of healthy infants with breast milk jaundice.
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In a prospective randomized open study, ceftriaxone, 50 mg/kg per day, was compared with ampicillin, 100 mg/kg per day, both given for a period of 5 days, for the treatment of 40 children whose mean (+/- SD) age was 4.5 +/- 3.2 years and who had severe dysentery caused by Shigella organisms. Twenty patients were treated with ceftriaxone and 20 with ampicillin. Both drugs were initially given intravenously for a period of 1 to 2 days and were continued intramuscularly, in the case of ceftriaxone, or orally, in the patients receiving ampicillin. All Shigella organisms isolated were susceptible to ceftriaxone; 28% were resistant to ampicillin. The diarrhea persisted for a mean (+/- SD) period of 2.5 +/- 1.0 days in the ceftriaxone-treated patients versus 6.8 +/- 6.3 days in the ampicillin-treated patients (p less than 0.005). At the end of the 5 days of therapy, stool cultures for Shigella organisms were negative in 12 (60%) of the 20 patients from the ampicillin group and in all the children (100%) from the ceftriaxone group (p less than 0.001). Bacteriologic relapses were observed in eight (40%) of the patients treated with ampicillin but in none of the children treated with ceftriaxone (p less than 0.001). In instances of clinical or bacteriologic failure in the ampicillin group, retreatment was instituted in most of the cases with ceftriaxone; persistent clearing of the Shigella organisms from the stool was finally achieved after a mean (+/- SD) period of 11.75 +/- 9.4 days after therapy was started, as compared with 1.85 +/- 0.6 days in the ceftriaxone-treated patients (p less than 0.001). We conclude that in children with severe shigellosis, treatment with ceftriaxone for 5 days is effective and better than use of ampicillin for clinical cure and eradication of the Shigella organisms from the stool.
Helicobacter pylori was found in a gastric biopsy specimen of a child with protein-losing gastropathy. Through erythromycin therapy, clinical and ultrasonographic recovery was associated with the disappearance of the pathogen on repeated biopsy. The association of H. pylori with protein-losing gastropathy has been reported only once in children, to our knowledge. Although the causative correlation between the pathogen and the disease has not been proven yet, we suggest that future cases of protein-losing gastropathy be studied for the presence of H. pylori and followed up by ultrasonographic investigation and urea breath test. Appropriate treatment should be given to suspected cases.
Inhaled budesonide has been demonstrated to be effective and safe when it is used in the prophylaxis of severe asthma in adults and school-age children but has not been studied in younger patients with asthma. Sixteen children, aged 3 1/2 to 7 years, with severe asthma, were treated with budesonide aerosol, 200 micrograms daily, via a spacer for 1 year in an open study. One month after starting budesonide therapy, a significant decrease in the number of days with asthmatic symptomatology, mean symptom scores, use of concomitant antiasthmatic medications, and increase in the peak expiratory flow rates were observed compared with the 1 month run-in period. This improvement was maintained throughout the year with budesonide therapy. During the study period, the height and weight of the children were not significantly deviated from the expected, and their bone age advanced normally. Adrenal function, as evaluated by fasting 8 AM blood cortisol levels and after adrenocorticotropic hormone stimulation, demonstrated no adverse effects with budesonide therapy. The suspectibility to severe infections was low, as evidenced by infrequent use of antibiotics. We conclude that a 12-month administration of inhaled budesonide to preschool-age children is safe and efficient and can be useful in the management of severe asthma in this young age group.
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