Extra-pulmonary tuberculosis in BCG-vaccinated patients.

Six patients with extra-pulmonary tuberculosis who presented within a 2-year period to the Paediatric Department of the Faculty of Medicine, Galle, Sri Lanka, are presented. In spite of the BCG vaccination being given to all of them at birth, five patients subsequently developed miliary tuberculosis and one patient bone tuberculosis. The likely reasons for failure of BCG vaccination to prevent extra-pulmonary complications developing are discussed.     

Infections in patients with neutropenia.

We review published reports and our experience in regard to the causative organisms of infections in patients with neutropenia. The organisms isolated from infected patients were almost exclusively pyogenic and enteric bacteria, and our patients had no history of serious viral or fungal infections, documenting the importance of the neutrophil in normal host defense against extracellular but not intracellular pathogens. Staphylococcus aureus was the single most commonly cultured organism; however, Pseudomonas and Escherichia coli were almost as commonly isolated. Positive cultures were obtained primarily from the blood or cutaneous, subcutaneous, or deep tissue abscesses. In patients with congenital neutropenia, pneumonia, otitis media, and abscesses were the most frequent infections diagnosed clinically. There is a suggestion that recent antibiotic therapy shifted the spectrum of infecting organisms toward enteric bacteria.     

Liver replacement for pediatric patients.

Between March 1963 and January 1978, 74 patients 18 years of age or younger have had liver replacements at the University of Colorado Medical Center, Denver. The most common cause of native liver failure was biliary atresis (48/74, 65%); the second most common cause was chronic aggressive hepatitis (12/74, 16%). Twenty-nine patients (39%) lived for at least one year, and 16 are still alive one to nine years after transplantation. Technical surgical problems, rejection, and infection were the main causes of death. Improved immunosuppression is needed; nevertheless, the quality of life in the long-term survivors has encouraged continuation of this difficult work.     

Tetracycline-induced esophagitis in adolescent patients.

Ulcerative esophagitis may be caused by corrosive agents and by commonly prescribed medications. We report severe esophagitis in five adolescents after ingestion of tetracycline preparations with minimal water immediately before going to bed.     

Hypocitraturia in patients with urolithiasis.

The urinary citrate/creatinine ratio was evaluated in 25 children with idiopathic calcium urolithiasis and 24 controls. The mean (SD) urinary citrate/creatinine ratio in controls and patients was 0.510 (0.205) and 0.181 (0.076), respectively, a statistically significant difference. In neither group was there a relation between age and urinary citrate excretion.     

Testing the psychogenic vomiting diagnosis. Four pediatric patients.

We treated four patients with chronic vomiting during childhood in whom a tentative diagnosis of psychogenic vomiting was made after an extensive evaluation. In each case, the diagnosis was reconsidered during the course of treatment, as observations about the patients and their response to interventions accumulated. In three instances, these observations did not fit those expected if the diagnosis of psychogenic vomiting was correct. This led to a reexamination of the organic evaluation and the discovery of an undiagnosed organic contribution to the vomiting. In the fourth patient, gastric emptying studies confirmed that there was a strong psychological contribution to the vomiting, and helped to more carefully define this contribution. Family and individual psychotherapy and treatment were aided by the greater clarity in diagnosis.     

Anthropometry of patients with osteogenesis imperfecta.

Standing height, sitting height, armspan, subischial leg length, head circumference, and growth hormone-insulin-like growth factor I (IGF-I) axis were determined in 86 patients with osteogenesis imperfecta. The aim of this study was to determine standing height and body proportions and their variability among osteogenesis imperfecta types and collagen defects. Mean standing height was reduced in all groups of patients, to the greatest extent and variability in osteogenesis imperfecta type III/IV and in those with qualitative collagen defects. The mean standing height of patients with osteogenesis imperfecta was lower than that of their unaffected first degree family members. Truncal height of patients with osteogenesis imperfecta was reduced; head size was increased, and this was more pronounced in patients with osteogenesis imperfecta type III/IV and qualitative collagen defects than in patients with osteogenesis imperfecta type I and quantitative collagen defects. Mean concentrations of IGF-I and IGF binding protein 3 (IGFBP-3) were low, but most values were within age specific reference values. The reduction of standing height appears to correlate with osteogenesis imperfecta type and the type of collagen defect. A relatively short trunk is typical and head circumference and body length are disproportionate.     

Cranberry use among pediatric nephrology patients.

INTRODUCTION: Recurrent urinary tract infections are common in children, and the use of complementary therapies is common in other children with recurrent illnesses. However, little is known about the use of cranberry products by children with renal disease. We hypothesized that, because cranberry is often used to prevent urinary tract infections (UTI) in adult women, many parents would give it to their children, particularly to children prone to recurrent UTI (rUTI). METHODS: Anonymous, cross-sectional, self-administered survey of parents of children seen in the pediatric nephrology clinic at Brenner Children's Hospital between June 1, 2004, and August 13, 2004. RESULTS: Of the 117 parents surveyed, the patients' average age was 10.3 years, and 15% reported rUTI as a problem. Overall, 29% of surveyed parents gave cranberry products therapeutically; as expected, use was higher among those with rUTI (65%) than among those with other renal conditions (23%); odds ratio = 6.1 (2.0, 18.4, P < .001); many parents gave cranberry to treat as well as prevent diverse renal problems. Most felt it was beneficial and only 1 parent reported a side effect (nausea). Only 23% of those who used it had discussed cranberry use with their physician. CONCLUSION: Cranberry is commonly used therapeutically among patients seen in a pediatric nephrology clinic and is perceived as useful by parents, though uncommonly discussed with physicians. Randomized controlled trials are needed to determine the effectiveness of cranberry juice therapy for rUTI in children.     

Gastrointestinal symptoms in patients with asthma.

AIMS: Minor gastrointestinal abnormalities have been reported in children with asthma, but the prevalence of gastrointestinal symptoms in these children has not been studied. METHODS: 75 children with bronchial asthma and an age and sex matched control group were recruited. Parents completed a questionnaire on gastrointestinal symptoms and on asthma. Weight and height were measured; a clinical evaluation of asthma was undertaken and skin prick tests were performed. RESULTS: Children with asthma had a significantly greater frequency of gastrointestinal symptoms, particularly diarrhoea, vomiting, and abdominal pain, than did controls. Gastrointestinal symptoms were slightly more common in children with atopic symptoms other than asthma, or with positive skin prick tests to foods. There was no association between current gastrointestinal symptoms and medications or attacks of asthma. CONCLUSIONS: The occurrence of gastrointestinal symptoms appears to be common in children with asthma. These symptoms might be caused by an atopic gastroenteropathy, which might play a part in the pathogenesis of asthma in some cases.     

Silver-Russell syndrome. Observations in 20 patients

The growth and development data of 20 patients with the Silver-Russell syndrome (14 boys, 6 girls) were analyzed. Family history, pregnancy and delivery did not reveal any significant anomalies. Birth length was 44.0 +/- 3.0 cm (boys) and 43.8 +/- 2.1 cm (girls), birth weight 2.0 +/- 0.4 kg and 2.05 +/- 0.3 kg, respectively. At the time of diagnosis (mean age 4.1 +/- 2.2 years), height was -4.4, bone age -1.9, weight -3.7, and head circumference -1.5 standard deviations below the normal mean for age. Calculated or reached adult height corresponded to 82--94% of target height. Intelligence was normal in most patients. 8 had asymmetrical extremities, 3 an asymmetrical face. 7 of 14 boys had cryptorchidism (3 uni-, 4 bilateral), 2 incomplete masculinization, and 2 of 6 girls hypertrophy of the clitoris. Development of secondary sex characters was appropriate for bone age with exception of one boy, whose puberty was early. In 3 boys with completed pubertal development, testicular volume was small and gonadotropins (before and after LHRH) high. It is concluded that 1. the growth pattern in Silver-Russell syndrome is quite homogeneous, and rather accurate predictions are possible; 2. Intersexual genitalia do not seem to be related to endocrine factors, and 3. hypergonadotropic hypogonadism appears to be frequent in males.