Biomedical subjects
Publications and source records attributed to S Bahabri.
Transverse myelitis is a rare but serious complication of systemic lupus erythematosus (SLE). We describe the youngest patient with transverse myelitis ever recorded in SLE. Clinical improvement was observed after prednisone and cyclophosphamide therapy. The English language literature from 1966 to the present on the subject are reviewed.
OBJECTIVE: To determine the rate and nature of infection in a cohort of children with SLE. METHODS: Records of 70 children with SLE were reviewed for documentation of infections. All patients with infection seen between 1990 and 1998 were included. Data extracted comprised demographics and clinical features including the disease activity index (SLEDAI), detailed descriptions of therapy, and response to antibiotic therapy. Infections were identified and catagorized as class I (minor) or class II (major). RESULTS: A total of 29 patients (41%) had infections; 73% had class I and 27% had class II infections. The most common cause of class II infection was bacteremia (45%), while urinary tract infection was more frequent (38%) in class I infections. There was no association between the severity of infection and various potential risk factors. CONCLUSION: Our data confirm that infection is common among children with SLE. There were no deaths directly related to infection.
We retrospectively reviewed 19 patients (11 male, 8 female) with infantile systemic hyalinosis (ISH) seen at a tertiary care hospital. Fifteen patients (83.3%) presented in the neonatal period. The referral diagnosis was inaccurate in 14 patients (73.7%). Thirteen patients were products of first-degree cousin marriages (68%) and 5 families had more than one affected child. All patients had painful joint contractures and typical mucocutaneous changes (hyper-pigmented sclerodermatous skin over the knuckles and malleoli, gingival hyperplasia, subcutaneous and perianal fleshy nodules). Growth failure was noted in all of them and 39% had profuse diarrhea, 72% had low serum albumin. Radiological findings included osteopenia, periosteal reaction and osteolytic lesions. Tissue biopsy was consistent with the diagnosis in the 8 patients who had the biopsies. Despite aggressive management with physiotherapy and different medications (including NSAIDs, penicillamine and methotrexate), the disorder was progressive and none of them showed improvement. 16 patients died with a mean age of 11 months and only 3 are alive with a mean age of 20 months. This report represents the largest series of ISH. Our data suggests that ISH is a commonly diagnosed disorder in Saudi Arabia and among Arabs.