Successful treatment of disseminated Aspergillosis in a leukemic child.

The incidence of severe fungal infections in the immunocompromised patient with malignancies has increased in recent years. This appears to be associated to the profound periods of immunosuppression and the extended use of broad spectrum antibiotics. Aspergillosis is the second most common fungal infection reported in the immunocompromised cancer patients. In patients with advanced immunosupression, the mortality due to invasive aspergillosis approaches 100% despite treatment with antifungal agents. Reports of complete or partial response to echinocandins are well demonstrated in adults, but very limited in the pediatric population. This report describes the case of a child with relapsed acute lymphoblastic leukemia (ALL) who developed cutaneous aspergillosis and subsequent multiorgan dissemination during therapeutic induction and was treated successfuly with caspofungin acetate.

Poor response to desmopressin acetate (DDAVP) in children with Hermansky-Pudlak syndrome.

BACKGROUND: Hermansky-Pudlak syndrome (HPS) is a common genetic disorder in Puerto Rico. In children with HPS, bleeding is the most disturbing and incapacitating problem. Desmopressin (1-deamino-8-D-arginine vasopressin, (DDAVP)) has been recommended in the management of bleeding disorders characterized by platelet dysfunction, such as HPS. METHODS: Nineteen pediatric Puerto Rican patients with HPS and prolonged bleeding time (BT) were tested for response to administration of DDAVP. RESULTS: Baseline BT was abnormal in 18 (95%) of the patients. The BT following DDAVP administration improved in two cases (11%): one from 7.2 to 5.6 min and the other from 8 to 6 min (Tables II and III). BT measurements remained very prolonged (>15 min) in 17 (89%) of the patients. Patients with the HPS 1 gene mutation had a statistically significant correlation with the poor response following DDAVP (P = 0.03). CONCLUSIONS: DDAVP seldom improves the BT of Puerto Rican children with HPS. Response to DDAVP should be determined individually and platelet transfusion should remain the treatment of choice for a major bleeding episode or surgical procedure.

Treatment of childhood acute immune thrombocytopenic purpura with high-dose methylprednisolone, intravenous immunoglobulin, or the combination of both

OBJECTIVE: To compare the effectiveness of intravenous immunoglobulin (IVIG) alone, high dose methylprednisolone (HDMP) alone and the combination of IVIG and HDMP in the treatment of childhood immune thrombocytopenic purpura (ITP). BACKGROUND: Acute ITP in children is a self-limited disease with a benign course and low mortality rate. Patients with platelet count less than 20,000 x 10(9)/L are at increased risk of bleeding complications, making them candidates for treatment. METHOD: A 4 year retrospective study of 148 patients hospitalized with acute ITP was conducted to compare the effectiveness of HDMP vs IVIG vs the combination of IVIG/HDMP. Statistical methods used were descriptive statistics and variance analysis utilizing F distribution. RESULTS: The IVIG and the HDMP combination demonstrated to be superior to HDMP alone in raising the platelet count within the first 24 hours. The HDMP and IVIG combination was statistically a superior modality of treatment for patients with platelet count greater than 10,000 x 10(9)/L than was IVIG or HDMP alone. Intravenous immunoglobulin had the least effectiveness in patients with platelet count less then 10,000 x 10(9)/L within the first 24 hours. CONCLUSIONS: IVIG followed by the combination of HDMP and IVIG is the most effective therapeutic modality in rapidly increasing the platelet count to safe levels in children with acute ITP when compared to HDMP alone within the first 24 hours. For borderline low platelet count (> 10,000 x 10(9)/L) HDMP and IVIG was superior to IVIG alone.