Recurrent myoglobinuria as a presenting manifestation of very long chain acyl coenzyme A dehydrogenase deficiency.
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Biomedical subjects
Publications and source records attributed to J Amir.
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We describe two children who presented with an acute encephalopathy preceded by a prodromal illness. The disease was marked by an active phase of coma or confusion with abnormal motor movements, followed by a recovery phase with a rapid return of motor function and a gradual improvement in speech and social behavior. No cause was found. These may be additional representative cases of a new syndrome of encephalopathy which is characterized by a distinctive course and a relatively good prognosis.
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Pachygyria is a rare development disorder resulting from impaired neuronal migration. Usually, it is a sporadic phenomenon, but rare dominant or autosomal recessive syndromes are known. This report describes a family in which the parents are first cousins and three of the siblings suffer from moderate mental retardation, pachygyria and strabismus. It is suggested that this is a distinct type of autosomal recessive pachygyria.
We evaluated the anxiety state of 57 parents whose children required lumbar puncture (LP) as part of a diagnostic workup. We also examined parental attitude about their presence during the procedure. The parents were randomly divided into two groups: 29 (51%) were present during the LP (group A) and 28 (49%) remained outside (group B). There were no differences in the anxiety scores between the two groups. All parents in group A and seven in group B (25%) reported a preference for staying with the child should he/she need LP in the future. The results also showed that allowing the parents to be present during LP does not aggravate their anxiety compared with controls.
Preliminary results have recently shown that an early switch from parenteral antimicrobials to an oral substitute provides an effective means of treating pneumonia in pediatric patients. In a controlled randomized study, 62 children with community-acquired lobar/segmental pneumonia were selected to receive 8 days of cefixime or amoxicillin-clavulanate after an initial therapy of two doses of parenteral ceftriaxone. Enrollment criteria included: age 6 months to 5 years, fever > 38.5 degrees C, white blood cell (WBC) count > or = 15,000/ mm3, and lobar/segmental pneumonia on chest radiograph. Twenty-nine patients were randomized to receive oral cefixime and 33 to oral amoxicillin-clavulanate. The two groups were comparable in the following pretreatment parameters: age, duration of illness, temperature, mean WBC count, erythrocyte sedimentation rate, C-reactive protein, and need for hospitalization. Days of resolution of high fever, tachypnea, cough, grunting, and laboratory test abnormalities were similar in the two groups. Clinical response at the end of treatment showed cure, improvement, and failure in 97%, 3%, and 0%, respectively, in the cefixime group and in 88%, 6%, and 6%, respectively in the amoxicillin-clavulanate group (P = NS). We conclude that young children with community-acquired lobar/segmental pneumonia can be successfully treated with 2 days of parenteral ceftriaxone followed by 8 days of oral cefixime or amoxicillin-clavulanate.
OBJECTIVE: To describe a serum-sickness-like reaction in five adolescents treated with minocycline. CASE SUMMARY: Five adolescents developed a rash and arthralgias/arthritis after taking minocycline for 10-30 days. Symptoms resolved gradually after the medication was stopped. DISCUSSION: Serum sickness is not described in the pharmacology literature as an adverse effect of minocycline, and in the English literature there are only two case reports. The migration inhibitory factor assay and mast cell degranulation test were positive in four of the five patients. The results of these assays were consistent with a role for minocycline in causing these reactions. CONCLUSIONS: Clinicians should be aware of the possibility of serum-sickness-like reaction as an adverse effect of minocycline.
In a 3-year nationwide prospective study on pediatric meningitis caused by Haemophilus influenzae type b, Streptococcus pneumoniae, and Neisseria meningitidis in Israel, 1258 invasive infections with a known focus were observed. Meningitis was found in 482 (38%): 56%, 16%, and 76% of all infections by H. influenzae type b, S. pneumoniae, and N. meningitidis, respectively. The incidence of meningitis during the first year of life was 67.1, 17.5, and 9.5/100,000 for H. influenzae type b, S. pneumoniae, and N. meningitidis, respectively, and in children < 5 years old it was 18.5, 5.3, and 5.2. Extrapolated for a population in which 100,000 live births occur yearly, 2097 hospital days were required. The case fatality rate was 2.2%, 5.9%, and 6.3% for H. influenzae type b, S. pneumoniae, and N. meningitidis, respectively. Boys were affected significantly more often than girls, but mortality was higher among girls. On the basis of the observed serotypes and age distribution, even with optimal vaccine development in the next 5 years, it is not likely that > 50% of all cases will be prevented.
In phase I of a 2-phase study, 56 evaluable children (0.8 to 5 years) with lobar or segmental pneumonia received intravenous or intramuscular ceftriaxone 50 mg/kg/day for 2 days followed by oral cefetamet pivoxil 20 mg/kg/day in 2 divided doses to complete 7 days of treatment. All patients achieved a clinical cure. In phase II, a randomised open multicentre study, 62 children with pneumonia received an identical regimen to phase I (arm A), and 59 children received ceftriaxone 50 mg/kg/day for 1 day followed by 6 days' treatment with cefetamet pivoxil 20 mg/kg/day (arm B). Patients from phase I and arm A were combined giving a total of 118 evaluable patients in arm A. At the end of treatment, 100% of patients in arm A and 96% in arm B achieved a clinical cure; cure was maintained in 99 and 98% of patients, respectively. Two (4%) patients in arm B failed therapy; in both cases, factors other than treatment failure may have accounted for the poor response. 11 and 12% of patients in treatment arms A and B, respectively, experienced adverse events; gastrointestinal events (nausea and/or vomiting) were reported in 9 and 8% of patients, respectively. In conclusion, 1 or 2 days' treatment with parenteral ceftriaxone before switching to oral cefetamet pivoxil was safe and effective in the treatment of childhood pneumonia. Therefore, parenteral-oral switch is a feasible treatment option in the treatment of serious paediatric community-acquired pneumonia.
Administration of theophylline to asthmatic children is frequently associated with an adverse influence on their behavior. The efficacy and behavioral effects of the administration of high-dose theophylline (T) and ketotifen (K) in various combinations were evaluated prospectively in a double-blind, placebo controlled study in 55 children with moderately severe perennial asthma. During a baseline period of 2 weeks, theophylline (serum level of 10-20 micrograms/ml) was administered to all the children. After this period the patients were randomly allocated into four comparable groups. The children were treated during a 12-week period with: T+K-Placebo (T group); T+K (T+K group); half-dose T+K (T/2 + K group); or placebo of both T and K (P group). During the 12-week treatment period, as compared to the baseline period, only the three groups of children who received active therapy (T+P, T+K, T/2 + K) showed a similar reduction in the number of days with asthmatic symptomatology, improvement of the total asthmatic symptoms score, and increased PEFR. The behavioral activity of the children (assessed by the Conner's rating scale) improved significantly only in the groups receiving placebo or T/2 + K. The results of this study suggest that a combination therapy of half the recommended therapeutic dose of theophylline with ketotifen can be clinically as effective as therapy with a full dose of theophylline, but with significantly less adverse behavioral effects.
Corticosteroids are known to affect the number and function of circulating lymphocytes in humans. The effect of 2 years administration of inhaled budesonide (200 micrograms/day) on the number and function of B and T lymphocytes and T cell subsets was evaluated in 16 young children with severe asthma. The number of T and B cells and T suppressor/cytotoxic cells (CD8 T cell) and T helper/inducer cells (CD4 T cell) before therapy was found to be comparable to the number of cells observed in the healthy control group. Two years administration of inhaled budesonide did not significantly alter the percentage and absolute number of all these cells. The functional activity of T lymphocytes was evaluated by the "local xenogeneic graft versus host reaction" (GVHR). A positive (normal) GVHR was observed in only 5 of the 16 children (31%) in the budesonide group before therapy, compared to 15 of 16 children (94%) in the healthy control group. During the 2 years treatment with inhaled budesonide, the percentage of patients having positive GVHR increased from 31% before therapy to 69% and 77% after 1 and 2 years of therapy (P = 0.05 and 0.02), respectively. The data observed in this study indicate that 2 years administration of inhaled budesonide did not alter the number of B and T lymphocytes and T cell subsets. However, it was associated with improvement in the GVHR function of T cells.
Rapid tests for the detection of group A beta-hemolytic streptococcus (GABHS) directly from a throat swab have become very popular. Previous studies have reported an antigen test sensitivity of 55-95% and a specificity of 88-100%. The present study evaluates the reliability of one rapid test in detecting GABHS (PathoDx). A total of 164 throat swab specimens was taken. GABHS was isolated on the culture in 37 (22.5%), and the rapid test was positive in 60 (36.6%). The sensitivity of the rapid test was 86.5% and the specificity 80%. Of the 60 positive rapid test results, 28 (47%) were false positive and the positive predicted value was 53%. We conclude that results obtained using rapid test kits should be compared to throat cultures in order to determine the reliability of such kits in specific clinical settings.
We report on a prospective study of 152 children aged 3 months to 5 years, from a community pediatric clinic, who had signs of pharyngitis, temperature > or = 38 degrees C and were not treated by antibiotics during the preceeding week. Nose and throat cultures were taken from each child. Blood antistreptolysin (ASO) was examined. If the cultures were positive for group A beta hemolytic Streptococcus (GABHS), a second blood sample for ASO was obtained later. True streptococcal infection was defined in a case of a positive culture and an increase in the ASO titer of at least two tubes, while cases of positive cultures without significant changes in ASO titer were defined as carriers. Positive GABHS cultures were found in 23 cases. True group A Streptococcus infection was found only in patients > 2 years old. The carriers of GABHS increased gradually from 3% during the 1st year to 22% by the 5th year. This study demonstrated that in the population evaluated, the incidence of true GABHS infection in children < 2 years of age is low, as was observed in the past.
BACKGROUND: The increased use of inhaled corticosteroids in the management of asthma raises concern about the safety of these drugs in children. We sought to determine the safety of long-term administration of inhaled budesonide in young children with asthma. METHODS: We studied 15 children 2 to 7 years old who had severe perennial asthma. They inhaled 100 micrograms of budesonide twice daily for three to five years. Efficacy was assessed by serial evaluation of respiratory symptoms and the need for other medications, and safety by serial evaluation of height, height velocity, weight, bone age, and pituitary-adrenal function. RESULTS: The severity of asthma decreased within the first month after the initiation of therapy, as demonstrated by a 58 percent reduction in the number of days with symptoms of asthma and a 75 percent decrease in the use of bronchodilators. This improvement was maintained thereafter. The growth pattern of all patients, including their height, weight, and bone age, was normal (as compared with standard normal values) throughout the treatment period. Pituitary-adrenal function was not adversely affected by the treatment, as demonstrated by normal serum cortisol concentrations in the morning and 60 minutes after stimulation with corticotropin, normal 24-hour serum cortisol concentrations (mean [+/- SD] of samples collected at 30-minute intervals for 24 hours, 8.4 +/- 4.2 micrograms per deciliter [232 +/- 116 nmol per liter]), and normal urinary cortisol excretion (34 +/- 9 micrograms [95 +/- 25 nmol] per day). CONCLUSIONS: Prolonged administration of 200 micrograms of inhaled budesonide daily to young children with severe asthma does not impair growth or pituitary-adrenal function.
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The reliability of midstream urine culture from circumcised male infants was studied in 60 infants aged 1 to 21 weeks. A midstream urine specimen was collected after cleansing the penis. When the bladder was full again, a suprapubic bladder aspiration (SPA) was also performed. The results of the urine cultures were almost identical in specimens obtained midstream and by SPA. In 37 infants the cultures were sterile and in 13 positive, with the same microorganism being cultured in both instances. In one case, few colonies of Staphylococcus epidermidis grew only from the midstream culture. In nine infants, only midstream specimens were obtained because three attempts at SPA were unsuccessful. These results suggest that in circumcised male infants, the midstream method of obtaining urine for a culture is as reliable as SPA.