Biomedical subjects
Publications and source records attributed to I Varsano.
Four patients with recurrent upper lip and facial swelling and lingua plicata together with peripheral facial nerve palsy involvement were diagnosed as having Melkersson-Rosenthal syndrome. Because of the rarity of this syndrome in children, we present the results of follow-up evaluations of our patients and review the literature.
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.
Nonketotic hyperosmolar diabetic coma is well known in adults, but infrequently reported in children. This syndrome is associated with a 20-70% mortality rate. We report the successful treatment of a 12.5 year old girl with a hyperglycemic (1,800 mg/dl glucose) nonketotic hyperosmolar diabetic coma and summarize the reported cases in the literature.
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.
We report on 2 cousins, offspring of consanguineous matings, with multiple congenital anomalies. Square face, frontal bossing with metopic ridge, small anteverted nose, flat nasal bridge, slanted palpebral fissures, and epicanthal folds contributed to an unusual appearance. Multiple bucco-alveolar frenula and notched inferior alveolar ridges were present at birth and one had a notched uvula and submucous cleft of the hard palate. Both had congenital heart anomalies, micropenis, and cryptorchidism. Persistence of Müllerian structures was documented at necropsy in one patient. The surviving patient was mentally retarded and had unilateral central hexadactyly and partial agenesis of the corpus callosum. Bulimia and episodic hyperthermia were attributed to hypothalamic dysfunction. Results of unstimulated endocrine studies and gonadotropin releasing hormone (GnRH), and human chorionic gonadotropin (HCG) stimulation tests were normal. The manifestations of the 2 patients overlap those reported in the OFD VI and Opitz trigonocephaly syndromes.
In a consanguineous Jewish family originating from Bombay, India, the propositus presented with anal atresia, micropenis, urethral obstruction with secondary prune belly, omphalocele, patent urachus, and cryptorchidism. The kidneys were dysplastic and he had the Potter phenotype with limb deformities. Additional findings included IUGR with microcephaly, congenital heart defects, spinal anomalies, and hypoplastic lungs. The mother and all three sisters had cervical ribs, and she and one sister had 11 pairs of thoracic ribs. The other two sisters had chronic immune thrombopenia. One of those had bilateral Sprengel deformity with homovertebral bones, club feet, and microcephaly and the other sister also had unilateral preaxial hexadactyly. Although familial segregation of cervical ribs and Sprengel deformity has been reported, the association of the findings in this family is unique and may represent a new syndrome. X-linked dominant transmission may explain the severe manifestations in the affected male, but other modes of inheritance may also apply.
Two brothers, aged 15 1/2 and 13 1/2 years, with dwarfism, microcephaly, and advanced sexual and skeletal maturation are described. One patient was mentally retarded. The parents were first cousins. Endocrine studies of these patients documented low growth-hormone levels after clonidine and insulin stimulation and blunted growth-hormone response to growth hormone releasing hormone. Gonadotropin releasing hormone stimulation produced no changes in levels of luteinizing and follicle-stimulating hormones. Basal levels of 17-alpha-hydroxyprogesterone were elevated in the two patients and increased further in response to stimulation with corticotropin. Levels of testosterone, dehydroepiandrosterone sulfate, and androstenedione were variably increased in both patients and showed a proportional increase on stimulation with human chorionic gonadotropin. To our knowledge, this is the first report of a familial association between growth-hormone deficiency and advanced bone and sexual maturation. A pituitary and an independent adrenal defect could account for the observations in these patients, but in view of the familial recurrence, a common underlying defect is possible.
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.
We compared the clinical and bacteriologic response of 5-day treatment with cefixime, 8 mg/kg per day, with the response to trimethoprim-sulfamethoxazole (TMP-SMX), 10-50 mg/kg per day, the currently recommended therapy. Of the assessable children with acute, culture-proven shigellosis, 38 received cefixime and 39 received TMP-SMX. Pretreatment data on the two study groups were similar. In the first group, all isolates were susceptible to cefixime; in the TMP-SMX group, 32 isolates were resistant and 7 were susceptible to TMP-SMX. Clinical response (day 5) showed cure, improvement, and failure in 89%, 8%, and 3%, respectively, of the cefixime group, and in 25%, 44%, and 31%, respectively, of the TMP-SMX-resistant group (p < 0.001). Bacteriologic cure (day 3) occurred in 78% and 23% of the cefixime and TMP-SMX-resistant groups, respectively (p < 0.001). Clinical or bacteriologic relapse (day 12) was infrequent in both groups. The response to treatment of the cefixime and the TMP-SMX-susceptible groups was similar. No significant side effects were noted. We conclude that cefixime is superior to TMP-SMX in the treatment of suspected shigellosis in areas with a high rate of resistance to TMP-SMX.
Administration of ceftriaxone for 5 days has been shown to be highly efficient in the treatment of severe shigellosis in children. Our study, involving 40 children, demonstrated that a 2-day course of ceftriaxone was as beneficial as a 5-day course and constituted effective therapy for children with severe shigellosis.
In three young children a newly acquired clinical sign--the knee-chest position--was associated with increased intracranial pressure. This sign may serve as an additional marker to suggest increased intracranial pressure in young children.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
One hundred and seven chronically wheezing infants, aged 6 months to 3 years, completed a double-blind placebo controlled multicenter study. After a two-week baseline period the patients were randomized into two groups receiving twice daily either ketotifen or placebo for a period of 12 weeks. The ketotifen dosage was 0.5 mg for children younger than one year and 1 mg for the older. During the 12-week treatment period the patients from the two groups demonstrated gradual improvement of the disease severity parameters, as compared with the baseline period. The amelioration was more marked in the ketotifen treated patients and during the 0-4, and 4-8 weeks of treatment a significant decrease was achieved in the percentage of days with a cough (p < 0.04) and in the number of wheezing episodes (p = 0.02). Moreover, a 50% reduction of the days and nights with cough and in the number of wheezing episodes occurred earlier in the ketotifen than in the placebo treated patients, i.e. after 1.2 vs 4.6; 4.4 vs 8.6 and 5.2 vs 7.2 weeks, respectively. It should be stressed that the amelioration of the asthmatic morbidity in the ketotifen group was achieved with a significantly reduced need for the concomitant use of bronchodilators. The principal side effects observed were weight gain and transient sedation. It is concluded that ketotifen may be effective in the amelioration of asthma associated symptomatology and acceleration in the natural tendency for improvement in chronic wheezing infants.
Asthmatic attacks continue to be a major cause of referral to the emergency room (ER), despite currently available effective treatments. We have prospectively evaluated 100 consecutive children who were referred to the ER with acute asthma and were followed by their primary physicians. Only 46% were able to recognize acute attacks, and 26% had the knowledge and skills for self-management. The prophylactic therapy recommended was usually appropriate, but the compliance was poor and mean serum theophylline levels (STL) was 6.8 micrograms/ml, with subtherapeutic values (< 10 micrograms/ml) in 44 (88%). We studied an additional group of 50 consecutive children who were on a routine follow-up in the hospital asthma clinic. All were examined periodically and were instructed on the disease. The compliance of these children was much better and STL were within the therapeutic range in 73%, significantly higher than in the ER patients (p < 0.001). Their need for ER treatment or hospitalization was much lower than the former group. Poor compliance is a major factor causing referral of asthmatic children to ER, and careful education can improve patient compliance with reduced referral and subsequent hospitalization.
Headache, nuchal rigidity, positive Kernig's sign, and even convulsions may be observed during severe bacterial infections such as pneumonia, pyelonephritis, typhoid fever, and bacillary dysentery. In such cases, meningitis can be excluded only by documentation of normal cerebrospinal fluid (CSF). The authors describe four children with lobar pneumonia in whom the clinical signs of meningeal irritation were associated with a mild increase in the white blood cell count in the CSF (pleocytosis) although there was no other evidence of meningeal infection.