Dengue hemorrhagic fever in patients with thalassemia.

Dengue hemorrhagic fever (DHF) causing by dengue viral infection is endemic in Thailand and Southeast Asian countries where thalassemias are prevalent. Thalassemic patients are also at risk to acquire dengue viral infections and to develop DHF. However, they can have different clinical manifestations and complications as well as more severity than general population requiring special awareness for proper diagnosis and management. We reported 20 thalassemic patients (10 boys and 10 girls) with DHF admitted to Department of Pediatrics, Siriraj Hospital during 1977 to 2001. Their ages ranged from 2-16 years (average 9.5 years). These cases included 5 cases of Hb H disease, 5 cases of Hb H with Hb Constant Spring (CS), 9 cases of beta-thalassemia/Hb E disease and 1 case of beta-thalassemia major. Two cases were in Grade I, 10 cases in grade II, 7 cases in Grade III and one case in grade IV severity of DHF. Though there were evidences of plasma leakage, instead of hemoconcentration, eighteen patients (90 percent) had hematocrit dropped at the range of 11-66% of the initial level. Fifteen patients (75 percent) required at least one packed red cell transfusion. Nine patients (45 percent) had mild bleeding symptoms, one of them had upper gastrointestinal hemorrhage requiring platelet concentrate transfusion. Two patients (10 percent) had serious complications including one with infection-associated hemophagocytic syndrome (IAHS) requiring intravenous immunoglobulin (IVIG) and packed red cell transfusion and the other had generalized seizure due to hyponatremia and hypotension. No mortality was observed among this group of patients. Early recognition of the DHF in thalassemic patients and appropriate packed red cell transfusion in patients with anemic symptoms is warranted to reduce morbidity and mortality in these patients.

Treatments of severe aplastic anemia in children.

We carried out a retrospective analysis of the outcome of treatment in patients with severe aplastic anemia who attended the Department of Pediatrics, Siriraj Hospital, during 1972- 1998. There were 31 patients, 17 boys and 14 girls, by Camitta’s criteria for severe aplastic anemia. All of them were idiopathic. Twenty patients were treated conventionally with steroid and androgen compounds, 5 with immunosuppressive therapy and 6 with bone marrow transplantation. In the conventional treatment group, after 1 year to 19 years of follow up, 64.7% achieved complete response, 11.8% achieved partial response and 23.5% died. The response rate in the immunosuppressive therapy group was only 40% after 3 months to 2 years of follow up. In the bone marrow transplantation group, bone marrow engraftment was achieved in all cases (means 29.8, range 19 - 42 days), and the patients yielded the highest complete response rate (100%). However, 1 case relapsed after complete response for 1 year, but he was successfully cured after second transplantation. The overall survival rate and cure rate were also 100% after 1 year and a half to 10 years of follow up. Bone marrow transplantation, when compared to other treatments, resulted in the highest response or cure rate with the shortest treatment duration. The only disadvantages of bone marrow transplantation are the high cost and the limited availability of HLA compatible donors.

Nutritional support for children underwent bone marrow transplantation.

Nutritional support for children underwent bone marrow transplantation was studied by comparing parenteral nutritional support and oral intakes. During 1988-1991 a total of 15 recipients, 10 boys and 5 girls, ages ranging 1-12 years from the Department of Pediatrics, Faculty of Medicine, Siriraj Hospital were studied. The patients were classified according to underlying diseases into 3 groups; Group I (thalassemia), Group II (aplastic anemia) and Group III (malignancy). The results indicate that Group I required less parenteral support than the other groups. Group III required the most parenteral support. Complications from bone marrow transplantation support among the 3 groups were similar. Therefore the requirement for nutritional support depend on the type of hematologic disease from which the patient is suffering.

Abdominal mass in children.

A retrospective study of abdominal mass was carried out among 87 patients admitted to the Department of Paediatrics, Siriraj Hospital, from October 21, 1980 to October 31, 1984. These patients were the pre-school and the school age groups with the age ranged from 1 day to 12 years with the mean of 3.3 years. Among the commonly diseases found were the lymphoma, the neuroblastoma and the Wilms’ tumour which were encountered in 19.5, 17.3 and 14.9 percent respectively. These patients presented with abdominal mass and fever in 60.9 and 35.6 percent of cases respectively. The most commonly organ involved was the liver with malignant disease in the majority of cases. The abdominal masses characterized by well-defined border with firm to hard consistency and nodular surface were found to be malignant while those did with ill-defined border and cystic consistency were benign in nature. The intravenous pyelography was carried out in 68 patients disclosing the abnormal pyelograms in 60 (88.2%). The accuracy of ultrasonography in disclosing the nature of the abdominal mass was 61.5 percent (16/26 patients).

Autoimmune haemolytic anemia in children: A review of 17 cases.

Seventeen children with autoimmune haemolytic anaemia (AIHA) seen at the Department of Paediatrics, Siriraj Hospital during 1977 to 1986 were analysed. The patients were devided into 2 groups, acute and chronic types, according to their response to treatment. In acute type (3 cases) the disease occured within the first year of life, with abrupt onset and severe clinical symptoms but dramatic response to prednisolone. In chronic type (14 cases), the disease appeared mostly at 9-11 years of age, associated with underlying diseases such as systemic lupus erythematosus, thalassaemia or Hodgkin’s disease and poorly responded to prednisolone. Two patients died, from septicaemia in one case and from the uncontrolled underlying disease in another case.