Scurvy: forgotten but not gone.

Scurvy is still seen sporadically in the developed world. At a time when subclinical vitamin C deficiency in the general population is being recognized increasingly, the need for clinicians to be aware of this disease remains. We present the case of a 9-year-old boy admitted to hospital with musculoskeletal pain, weakness and changes in the skin and gums. After extensive investigation, he was found to have vitamin C deficiency resulting from a restricted eating pattern. Musculoskeletal complaints are a common mode of presentation of scurvy in children. Failure to appreciate this fact and the risk factors for poor vitamin C intake in the paediatric age group can result in unnecessary and invasive investigations for apparent 'multisystem' disease.

Job-sharing in paediatric training in Australia: availability and trainee perceptions.

OBJECTIVE: To examine the current availability of job-sharing in paediatric training hospitals in Australia and to evaluate job-sharing from the trainees' perspective. DESIGN: National survey with structured telephone interviews and postal questionnai res. SETTING: The eight major paediatric training hospitals in Australia. PARTICIPANTS: Directors of Paediatric Physician Training (DPPTs) at each hospital (or a staff member nominated by them) provided information by phone interview regarding job-sharing. All paediatric trainees who job-shared in 1998 (n=34) were sent written questionnaires, of which 25 were returned. RESULTS: Hospitals differed in terms of whether a trainee was required to give a reason for wishing to job-share, and what reasons were acceptable. One hospital stated that two specialty units (Intensive Care and Neonatal Intensive Care) were excluded from job-sharing, and another stated that certain units were unlikely to be allocated job-sharers. The remaining six hospitals said that all units were available for job-sharing, but the majority of their trainees disagreed. Only one hospital had a cap on the number of job-share positions available yearly. Trainees perceived benefits of job-sharing to include decreased tiredness, increased enthusiasm for work, and the ability to strike a balance between training and other aspects of life. Trainees believed job-sharing did not adversely affect the quality of service provided to patients, and that part-time training was not of lower quality than full-time training. CONCLUSIONS: Job-sharing in Australian paediatric training hospitals varies in terms of the number of positions available, eligibility criteria, and which units are available for job-sharing. In our survey, trainees' experience of job-sharing was overwhelmingly positive.

Normal levels of soluble E-selectin, soluble intercellular adhesion molecule-1 (sICAM-1), and soluble vascular cell adhesion molecule-1 (sVCAM-1) decrease with age.

sE-selectin, sICAM-1, sVCAM-1 and von Willebrand factor (vWF) were assayed in 238 samples in a longitudinal study of 81 normal children from 9.5 to 15.5 years old. Multilevel modelling was used to quantify changes with age. sE-selectin, sICAM-1 and sVCAM-1 all fell significantly over the age range (by 17%, 16%, and 10%, respectively). In contrast, levels of vWF were not age-dependent. Our findings highlight the need for age-matched controls when studying cell surface adhesion molecules in disease groups, and may imply developmental changes in expression of these molecules and their shedding from the cell surface.

Anti-neutrophil cytoplasmic antibodies and anti-endothelial cell antibodies are not increased in Kawasaki disease.

We studied anti-neutrophil cytoplasmic antibodies (ANCA) and anti-endothelial cell antibodies (AECA) in 58 children with acute Kawasaki disease (KD) before i.v. gamma globulin treatment, 35 children with infection and fever > 38.5 degrees C, and 48 healthy afebrile children. ANCA were studied by indirect immunofluorescence (IIF) on ethanol-fixed neutrophils and by ELISA with crude neutrophil extract as antigen. AECA were studied using ELISA on resting and activated endothelial cells. ANCA IIF was weakly positive, cytoplasmic, diffuse and homogeneous in all three groups. ANCA IIF, ANCA ELISA and AECA ELISA were no higher in KD than in febrile children. There was no difference between KD with and KD without coronary artery aneurysms. AECA differences between the KD and afebrile group were not significant after correction for total IgM. In contrast with our previous findings, we conclude that ANCA and AECA are not raised in KD compared with febrile controls. It therefore seems unlikely that they are important in the pathogenesis of vasculitis in KD.

Soluble cell adhesion molecules and von Willebrand factor in children with Kawasaki disease.

Fifty-nine children with acute Kawasaki disease (KD), a childhood vasculitis, were compared with 35 children with fever due to infection and 48 healthy children. Levels of soluble E-selectin (sE-selectin), soluble intercellular adhesion molecule-1 (sICAM-1), and soluble vascular cell adhesion molecule-1 (sVCAM-1) in the healthy children were double those found in adults. All three soluble cell adhesion molecules and von Willebrand factor (vWF) were higher in the children with KD than in the healthy children, but only sE-selectin, a marker for activated endothelial cells, and sICAM-1 were higher than in the febrile children. The high levels of vWF in KD appear to reflect the prominent acute-phase reaction. This information can help us to understand further the complex interactions between cytokines, circulating inflammatory cells and the vascular endothelium, and may lead to new therapeutic avenues in KD and other inflammatory diseases and vasculitides.

Anti-neutrophil cytoplasmic antibody-associated glomerulonephritis in children.

Two cases of anti-neutrophil cytoplasmic antibody (ANCA)-associated necrotizing and crescentic glomerulonephritis are reported. A 12-year-old girl and a 10-year-old boy presented with polyarthritis, anaemia, haematuria, proteinuria, impaired renal function, anorexia, nausea, marked loss of weight and lethargy. The boy also had a vasculitic rash and anterior uveitis. Both children had diffuse cytoplasmic ANCA identified by indirect immunofluorescence and confirmed by specific enzyme-linked immunosorbent assay. Renal biopsies showed severe focal and segmental necrotizing glomerulonephritis with 100% crescents. They were treated with plasma exchange, prednisolone, cyclophosphamide and heparin. Within 1 month of commencing treatment, both had normal serum creatinine concentrations and ANCA was not detectable. Renal biopsies 6 weeks following commencement of treatment revealed quiescent disease, although up to 40% of glomeruli were sclerosed or had fibrous crescents. Following cessation of cyclophosphamide and heparin after 7 months and reduction in steroid dose, a biopsy at 10 months in the boy revealed quiescent disease, but the girl had recurrent disease associated with reappearance of a low titre of ANCA and small cellular crescents in 20% of the glomeruli. These cases reflect the potential usefulness of ANCA determination for categorizing paediatric patients, helping in the selection of therapy and as a possible marker of disease activity, similar to the experience in adults.