Alveolar rhabdomyosarcoma with massive disseminated intravascular coagulopathy treated with systemic chemotherapy / 소아과

It is uncommon for pediatric patients with rhabdomyosarcoma to present with clinical and/or laboratory features of disseminated intravascular coagulation (DIC). We report a case of metastatic alveolar rhabdomyosarcoma with severe bleeding because of DIC in a 13-year-old boy. He experienced persistent oozing at the site of a previous operation, gross hematuria, and massive epistaxis. Two weeks after initiating combination chemotherapy consisting of vincristine, doxorubicin, and cyclophosphamide, the patients' laboratory indications of DIC began to resolve. During this period, the patient received massive blood transfusion of a total of 311 units (26 units of red blood cells, 26 units of fresh frozen plasma, 74 units of platelet concentrates, 17 units of single donor platelets, and 168 units of cryoprecipitate), antithrombin-III and a synthetic protease inhibitor. Despite chemotherapy and radiation therapy, he died 1 year later because of disease progression. In children with metastatic rhabdomyosarcoma and massive DIC, prompt chemotherapy and aggressive supportive care is important to decrease malignancy-triggered procoagulant activities.

Long-term follow-up of Fanconi anemia: clinical manifestation and treatment outcome / 소아과

PURPOSE: The aim of this study was to characterize Korean patients with Fanconi anemia (FA), which is a rare but very challenging genetic disease. METHODS: The medical records of 12 FA patients diagnosed at Chonnam National University Hospital from 1991 to 2012 were retrospectively reviewed. RESULTS: The median age at diagnosis was 6.2 years. All patients showed evidence of marrow failure and one or more physical stigmata. Chromosome breakage tests were positive in 9 out of 11 available patients. The median follow-up duration was 69.5 months. The Kaplan-Meier (KM) survival of all patients was 83.3% at 10 years and 34.7% at 20 years, respectively. Seven patients underwent 9 stem cell transplantations (SCTs). Among them, 5 were alive by the end of the study. Ten-year KM survival after SCT was 71.4% with a median follow-up of 3.4 years. All 5 patients treated with supportive treatment alone died of infection or progression at the median age of 13.5 years, except for one with short follow-up duration. Acute leukemia developed in 2 patients at 15.4 and 18.1 years of age. Among 6 patients who are still alive, 3 had short stature and 1 developed insulin-dependent diabetes mellitus. CONCLUSION: We provide information on the long-term outcomes of FA patients in Korea. A nation-wide FA registry that includes information of the genotypes of Korean patients is required to further characterize ethnic differences and provide the best standard of care for FA patients.

Sedative Effect of Chlorpheniramine in Children Failed to sedate with Chloral hydrate and Midazolam / 대한소아신경학회지

PURPOSE: To evaluate the sedative effect of add-on chlorpheniramine in children with neurologic diseases failed to sedate with chloral hydrate and midazolam. METHODS: Thirty three patients who had not been successfully sedated with oral chloral hydrate and intravenous midazolam for diagnostic examinations were attempted for sedation with intravenous chlorpheniramine at Chonnam National University Hospital from September 2007 to September 2012. The sedative effects were compared on the aspects of age, sex, body weight, dosage of drug and underlying neurologic conditions with the retrospective review of medical records. RESULTS: Among 33 patients, 26(78.7%) were successfully sedated and 7(24.2%) failed to sedate. The success rates were different by age and were decreased with age: 100%(0-4y), 84.6%(5-9y), 50%(10-14y). The effectiveness of chlorpheniramine was not significantly different in terms of ages, sex, body weight, dosage of drug and the underlying neurologic conditions-developmental delay, seizures or organic brain lesions. Children with ADHD(attention-deficit hyperactivity disorder), however, showed a significantly lower success rate than the non-ADHD patient group (28.5%, P=0.002). No serious side effects were reported except for one case with transient perioral cyanosis. CONCLUSION: Chlorpheniramine appeared highly effective in children with neurologic diseases who had not been sedated with chloral hydrate and midazolam. The efficacy seemed to be higher in the younger age groups and lower in children with ADHD.

Growing Teratoma Syndrome of the Pineal Gland Recognized Very Early during Chemotherapy in a Child with a Non-Germinomatous Germ-Cell Tumor / 임상소아혈액종양

The growing teratoma syndrome (GTS) is defined as the development of mature low-grade elements in the absence of a recurrent non-germinomatous germ-cell tumor (NGGCT) after partial response to multimodal treatment. It is uncommon and may occur in intracranial NGGCTs. Here, we report that a 7-year-old boy with intracranial NGGCT presented with precocious puberty and developed growing teratoma syndrome only 2 weeks after the first cycle of chemotherapy.

Growing Teratoma Syndrome of the Pineal Gland Recognized Very Early during Chemotherapy in a Child with a Non-Germinomatous Germ-Cell Tumor / 임상소아혈액종양

The growing teratoma syndrome (GTS) is defined as the development of mature low-grade elements in the absence of a recurrent non-germinomatous germ-cell tumor (NGGCT) after partial response to multimodal treatment. It is uncommon and may occur in intracranial NGGCTs. Here, we report that a 7-year-old boy with intracranial NGGCT presented with precocious puberty and developed growing teratoma syndrome only 2 weeks after the first cycle of chemotherapy.