Henoch Schonlein purpura in childhood: epidemiological and clinical analysis of 150 cases over a 5-year period and review of literature.

OBJECTIVE: To examine epidemiological, clinical, and outcome in Italian children affected with Henoch Schönlein purpura (HSP). METHODS: Retrospective study of children discharged with a diagnosis of HSP from the Meyer Children's Hospital, between 1998 and 2002. Epidemiological, clinical, laboratory data, treatment, and outcome were collected by reviewing medical charts. One year after data collection, the children's parents were interviewed by telephone about the outcome. RESULTS: 150 children entered the study: M:F=1.8:1; mean age 6.1+/-2.7 years. At onset, purpura was present in all cases, arthritis/arthralgias in 74%, abdominal involvement in 51%, scrotal edema in 13%, renal involvement in 54%, severe nephropathy in 7%, acute renal insufficiency in 2%, and intussusception in 0.6%. Purpura was the presenting symptom in 74%, arthritis in 15%, and abdominal pain in 12%. The most frequent laboratory abnormalities were high-erythrocyte sedimentation rate (ESR) (57%), hyper-IgA (37%), and proteinuria (42%). All patients recovered within 2 months. Recurrences, verified in 35%, were correlated with high ESR values and corticosteroid (CS) treatment, independently from other variables. After a mean 2.5-years follow-up, 2 patients had hematuria with normal renal function. CONCLUSION: Epidemiological and clinical findings in our cohort are similar to those in the literature, even though the mean disease duration was shorter than previously reported. Relapses occurred significantly more frequently in children treated with CS. This finding supports the recommendation to limit the use of steroids to a carefully selected group of HSP children. The prognosis was excellent; although severe nephropathy was found in a small percentage of the children, at follow-up all had normal renal function. Thus, our study confirms the benignity of HSP in Italian children, especially regarding renal outcome.

Very late nonfatal consequences of fractionated TBI in children undergoing bone marrow transplant.

PURPOSE: To describe long-term late consequences in children who received total body irradiation (TBI) for hematopoietic stem cell transplantation 10 years earlier. METHODS AND MATERIALS: A cohort of 42 children treated with TBI between 1985 and 1993, still alive at least 10 years after fractionated TBI (FTBI), was evaluated. Twenty-five patients received FTBI at 330 cGy/day for 3 days (total dose 990 cGy), whereas 17 children were administered fractions of 200 cGy twice daily for 3 days (total dose 1200 cGy). Twenty-seven patients received autologous and 16 allogeneic hematopoietic stem cell transplantation. Median age at TBI was 6.3 years, and 18.4 years at most recent follow-up. RESULTS: Cataract was diagnosed in 78% of patients after a median of 5.7 years. Hypothyroidism was detected in 12%, whereas thyroid nodules were observed in 60% of our population after a median interval of 10.2 years. Patients treated with 990 cGy developed thyroid nodules more frequently than those treated with 1200 cGy (p = 0.0002). Thyroid carcinoma was diagnosed in 14% of the total population. Females who received FTBI after menarche more frequently developed temporary ovarian dysfunction than those treated before menarche, but cases of persistent ovarian dysfunction did not differ between the two groups. Indirect signs of germinal testicular dysfunction were detected in 87% of males. Restrictive pulmonary disease was observed in 74% of patients. Osteochondroma was found in 29% of patients after a median interval of 9.2 years. This latter complication appeared more frequently in patients irradiated before the age of 3 years (p < 0.001). CONCLUSIONS: This study shows that late effects that are likely permanent, although not fatal, are frequent in survivors 10 years after TBI. However, some of the side effects observed shortly after TBI either disappeared or remained unchanged without signs of evolution. Monitoring is recommended to pursue secondary prevention strategies and counseling on family planning.

Role of active follow-up for early diagnosis of relapse after elective end of therapies.

OBJECTIVE: To evaluate the role of active follow-up for the detection of relapses occurring after completion of therapy in children with cancer. METHODS: The clinical records of all children who had a cancer relapse more than 3 months after the end of therapies in the period 1985-2000 were reviewed. Relapses were defined "diagnosed at a scheduled visit" or "at an unscheduled visit" based upon how the visit that lead to the suspected diagnosis was scheduled. Information was collected on how the first suspicion of relapse was made. Survival after relapse was calculated, by type of visit and tumor type. RESULTS: Among 739 children who completed therapy for a malignant tumor in first complete remission (CR), 101 relapses [74 after solid tumors (ST), 27 after leukemia/lymphoma (L)] occurred after a median time of 12 months (range 3-87). Fifty-one (50.5%) first relapses were diagnosed during a visit scheduled because of symptoms (36 ST, 15 L), and 50 relapses (49.5%) at a regularly scheduled visit (38 ST, 12 L). Overall, 75% of relapses were first suspected on clinical basis, 16% via imaging, and only 9% via lab tests. Survival more than 10 years from first relapse was 25.7% (SE: 0.05%), with no significant differences between relapses diagnosed at a scheduled visit (20.5%), or at an unscheduled visit (32.1%; P = 0.826). Children with L had a better overall survival (OS, 70.6%) as compared to those with ST (9.2%, P < 0.001), probably because of a more extensive use of stem cell transplantation (SCT) as part of the salvage regimens. CONCLUSIONS: Scheduled follow-up programs failed to detect relapses in 50% of cases presented here. Survival after relapse is not affected by whether relapse was detected at a scheduled or an unscheduled visit.