Intravenous gamma globulin for treatment of chronic idiopathic thrombocytopenic purpura in children.

A prospective and descriptive study was carried out in 17 children with chronic ITP. Five-day course of Intraglobin (400 mg/kg/d x 5) was given intravenously to 10 children with the age of 4-16 years (5 males and 5 females). Two-day course of Venoglobulin-I (1 g/kg/d x 2) was given intravenously to 7 children with the age of 3-15 years (3 males and 4 females). Intraglobin and Venoglobulin-I were effective in treating children with chronic ITP. All of the patients had transient increased in their platelet counts during the first 2 weeks. The two-day course of Venoglobulin-I was superior to the five-day course of Intraglobin. Mild adverse effects were observed in a greater percentage of patients treated by Venoglobulin-I than in patients treated by Intraglobin. Intravenous immunoglobulin was one of the choices of treatment in children with chronic ITP, but the cost of immunoglobulin or gamma globulin is quiet high.

Molecular basis of beta-thalassemia in Thai Muslim patients in the the south of Thailand.

Among a sample of 29 unrelated Thai Muslim children, a total of 37 beta thalassemia genes was identified and 33 out of 37 mutations (89%) were characterized giving 6 different mutations. Four mutations [IVS-1 nt 5 (G-C), codon 19 (A-G), codons 41/42 (-CTTT) and IVS-1 nt 1 (G-T)] account for 86%. IVS-1 nt 5 (G-C) is the most common mutation found in Thai Muslim patients. Thai Muslim patients share the four most common mutations with Malays.

Clinical, hematological and molecular features in Thais with beta-Malay/beta-thalassemia and beta-Malay/HbE.

A total of 50 patients and relatives were studied comprising 12 cases of compound heterozygosity of beta-Malay and beta + thalassemia, 10 cases of compound heterozygosity of beta-Malay and beta degree thalassemia, 10 cases of beta-Malay and HbE and 18 cases of beta-Malay heterozygosity. Patients with beta-Malay and HbE had very mild clinical symptoms or were asymptomatic of thalassemia disease in the absence of blood transfusion. Homozygosity of beta-Malay produce mild clinical symptoms of thalassemic disease with normal facial characteristics and were not transfusion dependent. Patients with beta-Malay and IVS 1 nt 5 (G-C) had severe clinical symptoms, and were transfusion dependent. Patients with beta-Malay and beta degree thalassemia had severe clinical symptoms, delayed weight and height in relation to age, were transfusion dependent and had classical features of thalassemic diseases.

The prevalence of hepatitis C virus antibodies in thalassemic patients in the south of Thailand.

One hundred and one thalassemic patients, 37 with homozygous beta-thalassemia, 60 with beta-thalassemia Hb E and 4 with hemoglobin H disease with Hb Constant Spring were studied. Twenty-four of 101 (23.8%) tested positive for antibody to hepatitis C virus (anti-HCV). Anti-HCV positivity among those with homozygous beta-thalassemia was significantly higher than anti-HCV positivity among the beta-thalassemic Hb E group. The number of blood transfusions received by anti-HCV positive thalassemic patients was significantly higher than that by anti-HCV negative thalassemic patients. Ninety per cent of anti-HCV positive thalassemic patients had persistently or intermittently raised SGPT levels.

Acarboxy prothrombin (PIVKA-II) in cord plasma in the south of Thailand.

Acarboxy prothrombin or PIVKA-II (protein induced by vitamin K absence or antagonist-II) was used to determine the presence of vitamin deficiency in newborn infants. Of 230 cord blood samples assayed by using ELISA method, 34.8 per cent were positive for PIVKA-II 0.13-17 AU/ml. The positive rate for PIVKA-II was greater in infants of primigravida (50.7%) than in those of multigravida (27.9%). All infants received prophylactic vitamin K, and no infant with positive PIVKA-II in cord blood subsequently had clinical bleeding. Because of the high prevalence of vitamin K deficiency in newborn infants in the South of Thailand, all newborn infants should receive prophylactic vitamin K at birth.

Hemoglobin H disease in children.

One hundred and ten children with hemoglobin H (Hb H) disease who attended the hematology unit of the Department of Pediatrics at Songklanagarind Hospital from 1982 to 1988 were studied. Hb Constant spring (Hb CS) was found in 55 patients. Four patients, two with Hb CS, were diagnosed during the newborn period. Anemia and jaundice were the main symptoms in three neonates, while the fourth one was found to have anemia with hepatosplenomegaly. Nine infants were diagnosed in the first year of life with the chief symptoms of anemia with or without fever. Two of them needed blood transfusions. Hb H was found in only three infants, while Hb Bart's was the constant finding in every infant. The Hb H children with Hb CS had a more severe clinical course than the group without Hb CS. The levels of Hb at steady state were found to be lower and the reticulocyte counts, red cells with inclusion bodies and Hb H were higher in patients with Hb CS. The clinical picture of acute hemolysis in Hb H children can be found in the neonatal period and the difference in clinical severity between the two genotypes of Hb H disease seems to develop from the first year of life.

Spontaneous platelet aggregation in thalassemic children and adolescents.

Spontaneous platelet aggregation was studied in 51 children and adolescents, comprising 30 nonsplenectomized thalassemic patients, 12 splenectomized thalassemic patients and 9 normal children. Spontaneous platelet aggregation was significantly increased in whole blood and platelet-rich plasma of splenectomized thalassemic patients but not in nonsplenectomized cases.

Langerhans cell histiocytosis of the spinal cord: a case report.

A 14-year-old boy with Langerhans cell histiocytosis of the spinal cord who presented with progressive enlarged bilateral inguinal masses, difficulty in urination, walking and paresthesia in the left lower extremity and perineum is reported. Radiographic studies and computerized axial tomography of T12 to L2 spines showed destruction of the vertebral body of L1 and left-sided paravertebral soft tissue mass extending into the vertebral canal with complete block of the dural sac at the level of L1 spine. The operative finding was an extradural mass at the anterolateral aspect of L1 to L2 spines. After surgical removal of the lesion and chemotherapy with vincristine, prednisolone, 6-MP and methotrexate for 15 months, his neurological deficit improved and there has been no evidence of recurrent disease during his two years follow-up. Langerhans cell histiocytosis is a possible cause of spinal cord compression or cauda equina syndrome and should be considered after excluding other more common causes.

Congenital factor XIII deficiency: report of a case and literature review.

We described a five-year-old boy with congenital deficiency of factor XIII. He had bled from the umbilical and circumcision sites during the first week of life. He had frequent ecchymoses and hematomas within 12-24 hours after trauma. His parents are first cousins. The screening hemostatic tests of this patient and his parents were within normal limits. Factor XIII, known as "fibrin-stabilizing factor", is a transpeptidase that produces strong covalent bonds between soluble fibrin monomers formed during coagulation. Presumptive diagnosis of factor XIII deficiency was made with a clot solubility screening test and confirmation was accomplished by demonstrating the absence of factor XIII by latex agglutination.