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Biomedical subjects

I Varsano

Publications and source records attributed to I Varsano.

At least 19 recordsLinked to original sources
PubMed1992

Rambam-Hasharon syndrome of psychomotor retardation, short stature, defective neutrophil motility, and Bombay phenotype.

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.

ABO Blood-Group System↗
PubMed1992

Transverse myelitis following mumps in children.

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.

Acute Disease↗
PubMed1992

Recurrent parotitis.

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.

Anti-Bacterial Agents↗
PubMed1992

Postcircumcision urinary tract infection.

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.

Age Factors↗
PubMed1992

Prevalence of asthma in Israeli schoolchildren. A comparative study of Jewish and Arab populations.

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.

Asthma↗
PubMed1991

Autosomal recessive Peters anomaly, typical facial appearance, failure to thrive, hydrocephalus, and other anomalies: further delineation of the Krause-Kivlin syndrome.

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.

Abnormalities, Multiple↗
PubMed1991

Breast mild jaundice: natural history, familial incidence and late neurodevelopmental outcome of the infant.

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.

Bilirubin↗
PubMed1991

Comparative efficacy of ceftriaxone and ampicillin for treatment of severe shigellosis in children.

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.

Adolescent↗
PubMed1991

Childhood protein-losing enteropathy associated with Helicobacter pylori infection.

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.

Biopsy↗
PubMed1990

Safety of 1 year of treatment with budesonide in young children with asthma.

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.

Administration, Inhalation↗
PubMed1989

Interstitial deletion 2q14q21.

A girl with multiple congenital anomalies and a tendency to severe pyogenic infections was found to have an interstitial deletion of chromosome band 2q14----q21. Unusual facial manifestations included enophthalmos, long philtrum, micrognathia, narrow forehead, prominent glabella, and depressed nasal bridge. Unilateral corneal clouding, with Peters-like anomaly; agenesis of the corpus callosum; brain atrophy; and heart, kidney, hand, and dermatoglyphic anomalies were additional findings. Eye anomalies were observed in five of 22 patients with deletions of chromosome 2q. In comparing these cases, it seems that deletions of bands 2q21 and 2q31 are variably associated with microphthalmia, corneal clouding, cataracts, and Peters anomaly. Measurement of protein C and interleukin-1 (IL-1) did not show a gene dose effect, but the pyogenic infections and low IgA found in this patient may reflect an abnormality of IL-1 not detectable by our methods.

Abnormalities, Multiple↗
PubMed1989

Experience and training as factors in physician performance in a pediatric emergency room.

The medical records of 463 children seen in a hospital emergency room were studied prospectively to examine the influence of physicians' experience on their performance in the emergency room. The diagnostic and therapeutic approach of three groups of physicians to the following problems were studied: fever lasting less than 24 h or 2-7 days, diarrhea and dyspnea. The results show that less experienced physicians (residents in their first week of practice in the emergency room) tended to request a significantly greater number of laboratory tests than the senior physicians, but tended to prescribe less medications, particularly those used for symptomatic treatment. Following 3 months' training in the emergency room, these residents generally exhibited a pattern of laboratory utilization similar to that of their respective seniors; however, the residents demonstrated a significantly increased propensity to diagnose more patients as having bacterial infections and to prescribe more antibiotics. In addition to the attention paid to young physicians' diagnostic utilization performance, their therapeutic pattern of practice should also be assessed.

Age Factors↗