Clinical Observation of Multisystem Langerhans Cell Histiocytosis / 대한소아혈액종양학회지

PURPOSE: Langerhans cell histiocytosis (LCH) has a wide spectrum of clinical features. Especially, disseminated disease has been associated with a chronic course, high rate of morbidity and possible mortality. The purpose of our study was to investigate the clinical features, subsequent disease course, survival and late sequelae in multisystem LCH (ms-LCH). METHODS: Fourteen cases diagnosed to histologically proven ms-LCH at Pusan National University Hospital between January 1991 and December 1997 were enrolled in this study. All patients received combination chemotherpy. The medical records were retrospectively reviewed for organ involvement at diagosis, disease course, and late sequelae. RESULTS: 1) The peak incidence was between 6 months and 2 years and sex distribution revealed female predominance with the ratio of 1.8:1. 2) Mean number of involved organs was 4.4 and the most frequently involved organ was liver (85.7%) followed by bone, middle ear, skin and spleen. 3) The mean duration of follow up was 41.6 27.5 months. And the overall estimated survival rate at 5 years was 75.9%, with an estimated disease free survival rate of only 40.8% at 5 years. 4) Three patients died and the causes of death were respiratory failure due to pneumonia, gastrointestinal bleeding due to hepatic failure and septicemia. 5) Late sequelae were seen in 42.8% among 14 patients. The most common sequelae were skeletal defects in 21.4% and diabetes insipidus in 21.4%. 6) Among the late sequelae, 3 patients had vertebra plana. Conservative treatment was done and long term follow up of 28.7 7.0 months demonstrated partial healing of the vertebra plana in two cases and no improvement in one. 7) The diabetes insipidus developed in 3 cases, at diagnosis, at 14 months and 20 months after diagnosis respectively. None of the cases received radiation therapy. All of them responded to anti-diuretic CONCLUSION: These data show that, despite the favorable survival, about half of ms-LCH patients had further dissemination of disease or late sequelae. Further treatment needs to be designed to prevent disease progression and late sequelae. hormone replacement therapy.

Hemostatic Changes and Effect of Antithrombin III Replacement Therapy in Children with Acute Lymphoblastic Leukemia

PURPOSE: We evaluated plasma coagulation parameters sequentially and the influence of antithrombin III adjuvant treatment in children with acute lymphoblastic leukemia. METHODS: Twenty-nine cases with acute lymhoblastic leukemia who had been treated with L-asparaginase at Pusan National University Hospital were enrolled in this study. The coagulation parameters were checked before and on every week of L-asparaginase administration. The patients whose level of antithrombin III was below normal range were given antithrombin III done adjuvant treatment. RESULTS: In the twenty-nine cycles of chemotherapies, nine cycles were treated with antithrombin III adjuvant therapy and others were not treated with the therapy. The PT and aPTT levels showed no changes during L-asparaginase treatment. After one week of L-asparaginase treatment, the fibrinogen and antithrombin III levels were decreased markedly. Antithrombin III level in patients under antithrombin III adjuvant treatment had increased significantly. On the other hand, antithrombin III level in patients not on antithrombin III adjuvant treatment showed low levels until finishing L-asparaginase. We observed only one case of sudden cerebral thrombosis in the non-antithrombin III adjuvant treatment. CONCLUSION: Antithrombin III adjuvant treatment can correct hemostatic parameters partially and may prevent thromboembolic events in patients with L-asparaginase.

A Case of Hepatoblatoma with Intraatrial Extension / 대한소아혈액종양학회지

Hepatoblastoma is an uncommon malignant tumor of the liver in children, moreover its intracaval extension to the right atrium is extremely rare. Current data indicate that combined hepatectomy and resection of intraatrial extension is necessary for cure. However 40% of the patients present with tumor that would make primary complete resection impossible or extremely hazardous. In these case preoperative chemotherapy is recommanded. We recently experienced a rare case of hepatoblastoma which metastasized to lung and to right atrium via inferior vena cava. We report this case with brief review of literature.

A Case of Glucose-6-Phosphate Dethydrogenase Deficiency with Chronic Hepatitis B / 대한소아내분비학회지

Glucose-6-phosphate dehydrogenase(G6PD) deficiency is the most common X- linked inherited disorder and is estimated to affect 400 million people worldwide. But the incidence of this disease is very rare in far-east Asia, especially in Korea. Many drugs and infections cause hemolytic anemia in patients with G6PD deficiency. We experienced a case of G6PD deficiency with chronic hepatitis B. The diagnosis was made by clinical symptoms, laboratory data including serologic test and bone marrow findings. We report a case of G6PD with chronic hepatitis with a brief review of related literatures.

A Case of Tracheal Bronchus / 소아알레르기및호흡기학회지

Tracheal bronchus is an aberrant bronchus that arises most often from right tracheal wall above the carina. It is a rare congenital anomaly, which is usually asymptomatic but occasionally associated with recurrent pneumonia, chronic bronchitis and bronchiectasis. Anomalies found in association with tracheal bronchi include respiratory(tracheal hypoplasia, tracheal stenosis, cystic lung lesion), gastrointestinal and musculoskeletal systems. Tracheal bronchus has been diagnosed by conventional tomography, bronchography and bronchoscopy in the past. Technical advances have greatly enhanced the utility of this diagnostic modality. So chest CT, even three-dimensional reconstruction, is of particular importance in the evaluation of mediastinal, pleural and lung parenchymal lesions. We experienced a case of duodenal atresia and tracheal bronchus in 6-month-old child who suffered from recurrent pneumonia and dyspnea. So we report with a brief review and its related literatures.