An unusual white blood cell scan in a child with inflammatory bowel disease: a case report.

Technetium-99m-labeled leukocyte (WBC) imaging is a valuable screening method for inflammatory bowel disease, especially in children, because of its high rate of sensitivity, low cost, and ease of preparation. A 14-year-old girl is described who had juvenile arthritis and iritis complicated by inflammatory bowel disease. She was examined for recurrent abdominal pain. A Tc-99m stannous colloid WBC scan was performed, and tracer accumulation was seen in the small bowel in the region of the distal ileum on the initial 1-hour image. Delayed imaging at 3 hours also revealed tracer accumulation in the cecum and ascending colon, which was not seen on the early image. A biopsy of the colon during endoscopy showed no evidence of active inflammation in the colon. The small bowel was not seen. Computed tomography revealed changes suggestive of inflammatory bowel disease in the distal ileum. The appearance on the WBC study was most likely a result of inflammatory bowel disease involving the distal ileum, with transit of luminal activity into the large bowel.

The musculoskeletal complications of cystic fibrosis.

OBJECTIVE: To determine the spectrum of musculoskeletal complications of cystic fibrosis (CF) in a paediatric population in Australia. METHOD: Clinical assessment followed by serology and bone scan on patients attending a specialized CF clinic. RESULTS: Of 125 patients studied, 21 had musculoskeletal complications, 17 attributable to CF. Eleven had joint involvement (six hypertrophic pulmonary osteoarthropathy (HPOA)), one CF arthropathy, two ciprofloxacin induced arthralgia, one joint contracture following long-line placement, one chest infection associated arthralgia), four kyphosis (two also with HPOA) and two thoracic deformity. HPOA was associated with older age, lower average pulmonary function and lower average Shwachman score. Three patients with HPOA died within 12 months of reporting symptoms. Kyphosis was also associated with older age and lower pulmonary function. CONCLUSION: Increasing age with deteriorating clinical and pulmonary function were associated with a higher incidence of musculoskeletal involvement. The development of symptomatic HPOA is a marker of poor prognosis.

Hirschsprung disease, microcephaly, mental retardation, and characteristic facial features: delineation of a new syndrome and identification of a locus at chromosome 2q22-q23.

We have identified six children with a distinctive facial phenotype in association with mental retardation (MR), microcephaly, and short stature, four of whom presented with Hirschsprung (HSCR) disease in the neonatal period. HSCR was diagnosed in a further child at the age of 3 years after investigation for severe chronic constipation and another child, identified as sharing the same facial phenotype, had chronic constipation, but did not have HSCR. One of our patients has an interstitial deletion of chromosome 2, del(2)(q21q23). These children strongly resemble the patient reported by Lurie et al with HSCR and dysmorphic features associated with del(2)(q22q23). All patients have been isolated cases, suggesting a contiguous gene syndrome or a dominant single gene disorder involving a locus for HSCR located at 2q22-q23. Review of published reports suggests that there is significant phenotypic and genetic heterogeneity within the group of patients with HSCR, MR, and microcephaly. In particular, our patients appear to have a separate disorder from Goldberg-Shprintzen syndrome, for which autosomal recessive inheritance has been proposed because of sib recurrence and consanguinity in some families.

Psychosocial functioning in children and young adults with juvenile arthritis.

A questionnaire survey of 363 children and young adults with juvenile arthritis was conducted to assess the relations among disease severity, psychosocial functioning, and adjustment in three age groups--primary school, high school, and young adult. Parents were surveyed separately to determine which characteristics of the ill child at different ages most significantly impact the well-being of the family. Indices of psychologic functioning and disease severity were associated with adjustment in the primary school and high school groups, whereas measures of social relationships were strongly associated with adjustment only in the high school group. Relations among measures of psychologic functioning, social relationships, disease severity, and adjustment in young adults were minimal. Level of disease severity was associated with the presence of financial concerns, emotional problems, and physical strain in parents of high school children and young adults. The results emphasize the importance of using a developmental model for understanding the adjustment of individuals with chronic juvenile arthritis and their families.

Congenital dislocation of the hip. Incidence, and treatment of a local population group.

For a period of three months, the incidence of hip abnormality was determined in 450 consecutive deliveries at the Hornsby and Ku-ring-gai Hospital newborn nursery. Three children were found to have dislocatable hips, and received treatment with a von Rosen splint. In 60 babies, soft tissue clicks could be elicited but the joints were stable. These babies received no treatment, but were reassessed one and six weeks later. No hip abnormality was detected in any of them at the follow-up examination. A retrospective analysis of the records of the previous 450 newborn babies showed that nine had received treatment with double nappies; von Rosen splints had not been used. At the six-month follow-up examination, one of these nine babies had a dislocated hip. "Double nappies" as a treatment modality is still widely practised in Sydney, though neither the literature nor the results of our retrospective analysis support its efficacy.

Pyomyositis tropicans: a diagnostic dilemma.

Pyomyositis tropicans is a rare disease in non-tropical climates and thus presents diagnostic difficulties. Two children with single staphylococcal psoas muscle abscesses were recently successfully treated. Computerized axial tomography was found to be a useful diagnostic aid, allowing exact localization of the lesion. The diagnosis and therapy of these abscesses are discussed.