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1.
Artigo em Inglês | IMSEAR | ID: sea-40370

RESUMO

The effects on linear growth and development among thalassemic patients under different treatment regimens were compared. Twelve homozygous beta-thalassemia (homozygous beta-thal) and 36 beta-thalassemia/Hb E (beta-thal/Hb E) were studied longitudinally between 1977 and 1998. Eighteen cases (10 homozygous beta-thal and 8 beta-thal/Hb E) received hypertransfusion with iron chelation by desferrioxamine. Another 30 cases (2 homozygous beta-thal and 28 beta-thal/Hb E) were given a low transfusion (depending on their clinical requirement). Their heights were measured serially and are presented as a standard deviation score (SDS). There was no significant difference in initial basic hematological data and ferritin levels between either group. However, the hypertransfused group, seemed to be clinically more severely affected than the other group as evidenced by early age at initial transfusion, the early onset of anemia and diagnosis and also their large acquired iron load after a period of transfusion. The average height SDS of the hypertransfused patients was within the 50th percentile +/- 1 SD during the first decade of life in both sexes and both genotypes. Whereas, in patients who were transfused infrequently, the SDS was always below the -1 SD and decreased gradually. In severe beta-thal/Hb E cases, their growth SDS showed no difference from those with homozygous beta-thal. Normal linear growth in those with homozygous beta thal and severe beta-thal/Hb E was only seen in the group that underwent hypertransfusion and this regimen contributed to normal growth during the first ten years of life. However, adequate iron chelation and hormonal treatment in these patients were also required in order to achieve normal adult height.


Assuntos
Transfusão de Sangue , Estatura , Peso Corporal , Distribuição de Qui-Quadrado , Criança , Desferroxamina/uso terapêutico , Feminino , Transtornos do Crescimento/etiologia , Humanos , Quelantes de Ferro/uso terapêutico , Modelos Lineares , Estudos Longitudinais , Masculino , Puberdade/fisiologia , Estatísticas não Paramétricas , Talassemia beta/complicações
2.
Southeast Asian J Trop Med Public Health ; 2000 Dec; 31(4): 795-800
Artigo em Inglês | IMSEAR | ID: sea-35414

RESUMO

During the period 1984-1992, 2 severe cases (1 male, 1 female) of congenital F VII deficiency with intracranial hemorrhage (ICH) were referred to the Department of Pediatrics, Siriraj Hospital Bangkok, Thailand at the ages of 1 and 3 months old. They both responded very well to fresh frozen plasma (FFP) transfusion therapy. Subsequently, both had repeated episodes of ICH (repeated ICH) 5 and 6 times, despite the 10-14 days of replacement therapy for each episode and eventually died at the ages of 11 and 13 months. Since September 1996, another 2 severe cases (2 females) of congenital F VII deficiency who had ICH within their first month of life were referred to us. In order to prevent repeated ICH, we started a prophylactic regime after the second episode of ICH, by giving FFP 10 ml/kg twice a week. The average duration of follow up was 21 months (at 8 and 34 months). All of them (aged 14, and 38 months old) are doing well at this time and free from repeated ICH. From this observation, if there is FFP available, this regime is an effective way to prevent repeated ICH in infants with severe congenital Factor VII deficiency.


Assuntos
Transfusão de Componentes Sanguíneos , Deficiência do Fator VII/complicações , Feminino , Humanos , Lactente , Recém-Nascido , Hemorragias Intracranianas/etiologia , Masculino , Cooperação do Paciente , Plasma , Tailândia , Resultado do Tratamento
3.
Southeast Asian J Trop Med Public Health ; 1999 ; 30 Suppl 2(): 143-7
Artigo em Inglês | IMSEAR | ID: sea-35973

RESUMO

Gaucher's disease, a lysosomal disorder, is not a common disease in Thailand. During the period 1966-1998 we saw 20 patients with Gaucher's disease at the Department of Pediatrics. Siriraj Hospital. The patients came from different regions of the country but mostly from the central part of Thailand. There were 8 males and 12 females from 13 families of Thai, Thai-Chinese, Thai-Laos and Chinese-Chinese in origin. A history of consanguinity was present in 2 families. The age of onset was 2 months-4 years and the age when they were diagnosed was 4 months-15 years. The most common clinical features included splenomegaly, hepatomegaly, growth retardation, pallor, bleeding disorders and neurological abnormalities. The diagnosis was made by the clinical manifestations, hematologic complications and demonstration of Gaucher cells in the bone marrow and/or other tissues. In one family, the diagnosis was confirmed by evaluation of glucocerebrosidase activities in skin fibroblasts. The management of these patients was symptomatic ie packed red cell and platelet transfusion, splenectomy and other supportive measures. Most patients died of bleeding or infection at an early age.


Assuntos
Adolescente , Criança , Pré-Escolar , Feminino , Doença de Gaucher/diagnóstico , Humanos , Lactente , Masculino , Tailândia/epidemiologia
4.
Southeast Asian J Trop Med Public Health ; 1997 ; 28 Suppl 3(): 64-8
Artigo em Inglês | IMSEAR | ID: sea-33814

RESUMO

In Thailand, the most common cause of chronic hemolytic anemia is thalassemia hemoglobinopathy. We report here a 10-year-old girl with pyruvate kinase (PK) deficiency who was initially diagnosed to have Hb H disease, like her sister. The patient had a history of neonatal jaundice which required blood exchange transfusion twice and phototherapy. She became anemic and regular blood transfusion was required since the age of 2 1/2 months. She was very anemic compared to her sister and was transfusion dependent. Besides, she never had red cell inclusion bodies, thus re-evaluation was performed. The diagnosis of red cell pyruvate kinase deficiency and the exclusion of Hb H disease was achieved after cessation of blood transfusion for 3 months. The family study also confirmed the diagnosis. The patient is now on high transfusion and iron chelation. She is doing well with mild splenomegaly.


Assuntos
Criança , Eritrócitos/enzimologia , Família , Feminino , Humanos , Piruvato Quinase/efeitos dos fármacos , Tailândia/epidemiologia , Talassemia alfa/epidemiologia
5.
Southeast Asian J Trop Med Public Health ; 1995 ; 26 Suppl 1(): 291-5
Artigo em Inglês | IMSEAR | ID: sea-31766

RESUMO

The hematopoietic committed progenitor cells (BFU-E and CFU-GM) in blood and bone marrow were studied in thalassemic patients before and after bone marrow transplantation. Eighteen transplants were performed in 17 patients with thalassemia. Five were homozygous beta-thalassemia and 12 were beta-thalassemia/hemoglobin E disease. The age ranged from 1.2-14 years (median = 3.4 years). The conditioning regimen comprised busulfan 3.5-4 mg/kg/day for 4 days and cyclophosphamide 50 mg/kg/day for 4 days. Cyclosporin in combination with methotrexate were administered post transplant for GVHD prophylaxis. Before transplantation, BFU-E and CFU-GM in the blood of the patients were significantly higher compared with normal (p < 0.05) but were normal in the bone marrow. Only the CFU-GM in the bone marrow of the successful cases after transplantation recovered to the normal level at the first month through the 12th month whereas the BFU-E were low. Both CFU-GM and BFU-E in the blood were lower than normal after follow up to the 12th month. Inspite of the low number of progenitor cells, there was hematological recovery in the blood of the patients. It may be due to the capacity of the hematopoiesis react to stress which could be amplified the differentiation compartment or the shortened-transit time through the stem cell compartment.


Assuntos
Medula Óssea/patologia , Transplante de Medula Óssea , Bussulfano/uso terapêutico , Células Cultivadas , Criança , Pré-Escolar , Ensaio de Unidades Formadoras de Colônias , Ciclofosfamida/uso terapêutico , Ciclosporina/uso terapêutico , Feminino , Doença Enxerto-Hospedeiro/prevenção & controle , Células-Tronco Hematopoéticas/patologia , Hemoglobina E , Hemoglobinúria/patologia , Homozigoto , Humanos , Imunossupressores/uso terapêutico , Lactente , Masculino , Metotrexato/uso terapêutico , Valores de Referência , Talassemia beta/patologia
6.
Southeast Asian J Trop Med Public Health ; 1995 ; 26 Suppl 1(): 271-4
Artigo em Inglês | IMSEAR | ID: sea-30603

RESUMO

Thalassemia hemoglobinopathies and glucose-6-phosphate dehydrogenase (G-6-PD) deficiency are prevalent in Thailand. We studied the prevalence of these disorders from 1,000 cord bloods collected during 14 months period, using EDTA as anticoagulant. Red blood cell G-6-PD quantitative assay was performed in all male subjects. Nine hundred and eighty five specimens were available for hemoglobin (Hb) typing by starch gel electrophoresis. Further evaluation by cellulose acetate electrophoresis and follow up were made in the cases who had Hb E and/or high level of Hb Bart's. It was found that out of 505 males, 61 cases (12.08%) had G-6-PD deficiency. Among 985 cases studied for Hb typing, 61.92% revealed normal Hb type AF while Hb E was present in 18.68% and Hb Bart's designated alpha-thalassemias were present in 25.18% respectively. Of these 985 cases, 18.78% had low Hb Bart's level ie detectable to 8.2% consistent with alpha-thal2, Hb Constant Spring (CS) or alpha-thal1 trait. Ten cases (1.02%) had high levels of Hb Bart's ranging from 16.1-35% without or with Hb CS and E, and further follow-up revealed homozygous Hb CS, Hb A-E-Bart's, Hb H and Hb H with Hb CS disease. The other 53 cases (5.38%) had low level of Hb Bart's with Hb E consistent with alpha-thalassemia trait with Hb E trait. There were 127 cases (12.89%) who had only Hb E trait and 3 cases (0.3%) who had Hb F and E without Hb A initially.


Assuntos
Eritrócitos/enzimologia , Feminino , Sangue Fetal , Seguimentos , Glucosefosfato Desidrogenase/sangue , Deficiência de Glucosefosfato Desidrogenase/epidemiologia , Hemoglobina A/análise , Hemoglobina E/análise , Hemoglobinas Anormais/análise , Hemoglobinúria/epidemiologia , Humanos , Recém-Nascido , Masculino , Prevalência , Tailândia/epidemiologia , Fatores de Tempo , Talassemia alfa/epidemiologia
7.
Southeast Asian J Trop Med Public Health ; 1995 ; 26 Suppl 1(): 137-41
Artigo em Inglês | IMSEAR | ID: sea-33089

RESUMO

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is prevalent in Thailand. This condition can cause acute hemolysis during oxidative stress and also severe hyperbilirubinemia in the newborn in some populations. Our aim was to study the prevalence of G6PD deficiency in relation to neonatal jaundice. We performed quantitative red blood cell (RBC) G6PD assay in the cord blood of 505 male subjects. Observation of jaundice and determination of bilirubin level as well as work up for other causes of jaundice were made in the G6PD deficiency group compared to a G6PD normal group. Questionnaires were also sent for further follow up to both groups. The results of the study were as follows: Sixty-one of 505 male (12.08%) had RBC G6PD deficiency (Group I). The rest (444 cases) had normal G6PD (Group II). In Group I, 49.15% developed neonatal jaundice, of which 28.82% were physiologic and 20.33% were pathologic jaundice. In group II, 23.68% developed jaundice; 16.51% were physiologic and 7.17% were pathologic jaundice, respectively. Onset of jaundice, date of peak bilirubin and peak bilirubin level in Group I and Group II were not statistically different. ABO incompatibility was associated with Group I in 17.24% and with Group II in 9.09%. Hospitalization day in Groups I and II were not statistically different. Other associated diseases were found in both groups, ie infection, congenital malformation, respiratory distress syndrome, but there was no significant difference in terms of jaundice. Phototherapy was required in 18.64% and 10.28% in Group I and II with a duration of 3.91 +/- 1.24 and 3.21 +/- 1.75 days, respectively. One case in Group I who was also premature received one exchange blood transfusion due to severe sepsis but he did not survive. One case in Group II who had polycythemia was successfully treated by partial exchange transfusion with plasma.


Assuntos
Bilirrubina/sangue , Eritrócitos/enzimologia , Sangue Fetal , Glucosefosfato Desidrogenase/sangue , Deficiência de Glucosefosfato Desidrogenase/diagnóstico , Hospitalização , Humanos , Recém-Nascido , Icterícia Neonatal/diagnóstico , Masculino , Prontuários Médicos , Fototerapia , Prevalência
8.
Asian Pac J Allergy Immunol ; 1993 Jun; 11(1): 85-91
Artigo em Inglês | IMSEAR | ID: sea-37067

RESUMO

The immunogenicity and the protective efficacy of a recombinant DNA hepatitis B vaccine, GenHevac B Pasteur with or without passive immunization with hepatitis B immunoglobulin (HBIG) in high risk neonates born from HBsAg and HBeAg positive mothers was evaluated. Twenty-six neonates (group A) received HBIG 100 IU intramuscularly at birth plus GenHevac B Pasteur 20 micrograms at birth, 1, 2 and 12 months of age while another 23 neonates (group B) received only GenHevac B Pasteur vaccine. Forty high risk newborns who received no immunization served as control group. It was found that at months 4, 12, 13 and 24 the seroconversion rate in both group A and B were very high in the range of 95-100% with the GMT ranging from 10-160,000 mlU/ml. In the control group of infants, 85% had HBsAg positive at one year of age but it was only 3.8% and 8.7% in vaccinated groups A and B, respectively. The protective efficacy in neonates group A and B were 95.5% and 89.8% at one year, respectively, with no statistically significant difference. In 46 normal school children (group C) and 48 healthy adults (group D) who received the same dose of GenHevac B Pasteur the seroconversion rates at month 4 after receiving 3 doses of vaccination were 97.8% and 83.3% in group C and group D, respectively. At month 12, the seroconversion rate in group C rose to 100% and was significantly higher than the 89.6% of group D.(ABSTRACT TRUNCATED AT 250 WORDS)


Assuntos
Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Hepatite B/imunologia , Anticorpos Anti-Hepatite B/biossíntese , Vacinas contra Hepatite B/administração & dosagem , Humanos , Imunoglobulinas Intravenosas/imunologia , Lactente , Recém-Nascido , Injeções Intramusculares , Masculino , Fatores de Risco , Vacinas Sintéticas/administração & dosagem
9.
Southeast Asian J Trop Med Public Health ; 1993 ; 24 Suppl 1(): 139-40
Artigo em Inglês | IMSEAR | ID: sea-32468

RESUMO

Prevention of transfusion associated AIDS (TAA) in Thailand began in 1986 when the HIV infection started to be sharply increased among the general population. The retrospective anti-HIV screening in various blood donor populations by The National Blood Center (NBC) revealed a seroconverted prisoner. Then the use of prisoners, prisoners' blood was not recommended from 1986. In April 1987, the first case of TAA was disclosed. Five months later, anti-HIV screening in all units of blood was firstly introduced at Ramathibodi Hospital (RH) and NBC. From 1989, anti-HIV screening in all units of blood is mandatory nationwide by Ministry of Public Health. Despite the anti-HIV screening, TAA cases transmitted by seronegative blood were gradually reported. Among many Medical Centers, there were 9 and 18 cases of TAA recorded from Chiang Mai and Bangkok areas respectively, since 1985. In addition, several new seroconverters were observed among voluntary blood donors. All of this evidence indicates the existence of blood donation during the early stage of infection, the so-called "window period". At present, HIV-P24 antigen ELISA seems to be the only available technique for mass screening. In 1990, NBC successfully performed a retrospective study on HIV-Ag ELISA screening by obtaining the prevalence of 1/10,000 units of blood. At the same period of time, in RH prospective study, a unit of blood with HIV-Ag only was detected when 3432 units of blood were screened. The HIV-Ag ELISA screening was then performed on every unit of blood routinely since Aug 12, 1991 at RH.(ABSTRACT TRUNCATED AT 250 WORDS)


Assuntos
Síndrome da Imunodeficiência Adquirida/epidemiologia , Transfusão de Sangue/efeitos adversos , Soronegatividade para HIV , Humanos , Vigilância da População , Prevalência , Estudos Prospectivos , Estudos Retrospectivos , Fatores de Risco , Tailândia/epidemiologia
10.
Southeast Asian J Trop Med Public Health ; 1993 ; 24 Suppl 1(): 80-5
Artigo em Inglês | IMSEAR | ID: sea-34963

RESUMO

Abstract. Abnormal hemostasis in dengue hemorrhagic fever includes:- 1. Vasculopathy which occurs during the early febrile to pre-shock and shock phase. The evidences support are: 1.1 Increased anaphylatoxin, released by complement activation causing leakage of intravascular fluid in to serous space. 1.2 Positive tourniquet test, some of which occur preceeding thrombocytopenia in the acute phase of DHF. 1.3 Excessive increased in PGI2 which is the most potent vasodilator and platelet aggregation inhibitor. 2. Platelets: 2.1 Thrombocytopenia due to 2.1.1 The bone marrow hypocellularity with increased in all forms of megakaryocytes but the vacuolated and disintegrated ones. 2.1.2 Destruction by the liver and spleen. 2.1.3 Immune-mediated injury as demonstration of dengue antibody complexes on the platelet surface. 2.1.4 The in vitro spontaneous aggregation to vascular endothelial cell pre-infected by dengue virus inducing platelet aggregation, causing lysis and platelet destruction. 2.2 Dysfunction shown by 2.2.1 Increased release of betathromboglobulin (BTG), PF4 and PGI2. 2.2.2 In vitro hypoaggregation stimulated by ADP and defect in ADP-releasing ability. 3. Coagulopathy including: 3.1 Prothrombin complex deficiency due to liver damage. 3.2 Consumptive coagulopathy due to the activation by mononuclear phagocytes, PF3 released from platelet aggregation. DIC is seen in prolonged shock cases of DSS.


Assuntos
Dengue/sangue , Hemostasia/fisiologia , Humanos
11.
Southeast Asian J Trop Med Public Health ; 1993 ; 24 Suppl 1(): 106-12
Artigo em Inglês | IMSEAR | ID: sea-34414

RESUMO

Among 117 cases of hemophilia, there were 7 hemophilia A and 2 hemophilia B with factor VIII and factor IX inhibitors diagnosed at the Department of Pediatrics, Siriraj Hospital, Bangkok, Thailand. The overall incidence of hemophilia with inhibitors was 7.7%. Eight cases (6 hemophilia A. 2 hemophilia B) were severe hemophilia and 1 moderate hemophilia A. The average age of the inhibitor detection was about 5 years. Of the 9 cases, 7 had high inhibitor titers and 2 had low inhibitor titers. The frequency of bleeding problems before and after inhibitor detection were not different. The bleedings included hemarthrosis, mucosal bleed, hematoma, oozing from wound, hematuria and intracranial hemorrhage. The treatment of hemarthrosis in hemophilia A with low inhibitor titers was the combination of short course of prednisolone and single large dose factor VIII. In high inhibitor titer patients with acute hemarthrosis (both hemophilia A and hemophilia B), the treatment consisted of prednisolone short course and single high dose of PCC. For bleeding control in both high and low inhibitor titer with mucosal bleeds, oozing from wounds, central nervous system bleeding and hematuria, the combination was used of high dose factor VIII or factor IX for 2 days, and tranexamic acid, prednisolone, cyclophosphamide were required. In life-threatening hemorrhage and surgical operation, plasmapheresis and large dosage factor VIII or factor IX were the treatment of choice. All supportive measures were also important in every case of mucosal bleeds, wounds and surgical operations. The result of treatment revealed one death from massive intracranial hemorrhage and 8 survivals, with joint contracture in 2 cases. All still have inhibitor detected, but in low titer.


Assuntos
Criança , Pré-Escolar , Fator IX/antagonistas & inibidores , Fator VIII/antagonistas & inibidores , Hemofilia A/sangue , Hemofilia B/sangue , Hemorragia/etiologia , Humanos , Incidência , Lactente , Estudos Retrospectivos , Índice de Gravidade de Doença , Tailândia , Resultado do Tratamento
12.
Southeast Asian J Trop Med Public Health ; 1993 ; 24 Suppl 1(): 116-20
Artigo em Inglês | IMSEAR | ID: sea-35930

RESUMO

The prevalence of vitamin K deficiency in the newborns delivered at Siriraj Hospital was studied. The prolongation of one stage prothrombin time and the presence of PIVKA-II (non carboxylated prothrombin antigen) in cord blood were interpreted as the secondary change from vitamin K deficiency state. The most reliable method to diagnose vitamin K deficiency is the detection of vitamin K level in plasma which is not yet available in Thailand. Although the prevalence of vitamin K deficiency in the newborns from our data is not high, only 0.6%, it is shown that some of the apparently normal newborn infants may have bleeding problem from vitamin K deficiency in both newborn and early infancy periods. So, the correction of this deficiency by administration of vitamin K to all newborns is appropriate and reasonable decision.


Assuntos
Biomarcadores , Fatores de Coagulação Sanguínea/análise , Feminino , Sangue Fetal/química , Sangramento por Deficiência de Vitamina K/sangue , Humanos , Recém-Nascido , Masculino , Valor Preditivo dos Testes , Prevalência , Precursores de Proteínas/análise , Protrombina/análise , Tempo de Protrombina , Sensibilidade e Especificidade , Tailândia , Vitamina K/análise
13.
Artigo em Inglês | IMSEAR | ID: sea-41070

RESUMO

Passive and active immunization for the prevention of hepatitis B virus (HBV) transmission in neonates born to HBeAg positive carrier mothers was studied using different kinds and dosages of combined hepatitis B immunoglobulin (HBIG) and hepatitis B vaccine. The long term efficacy of these various immunoprophylaxis programmes was evaluated up to 3 years of age. Eight groups of neonates, 20 in each group, received HBIG either the usual dose of 100 IU intramuscularly or intravenously or increased dose to 200 IU, combined with different kinds of hepatitis B vaccine either in full dose or half dose given in different schedules of 0, 1, 2 or 0, 1, 6 months, in some groups also received a booster dose at 12 months. After follow-up for 3 years, there were no statistical significant differences of the results between these 8 different immunoprophylaxis programmes, either in the rate of seroconversion or the protective efficacy as well as the antibody response (anti-HBs titer). The seroconversion rates after 3 years of age in all 8 groups were in the range of 63.6 to 92.9 per cent. The protective efficacy rates at 3 years of age were in the range of 69.7 to 100 per cent.(ABSTRACT TRUNCATED AT 250 WORDS)


Assuntos
Portador Sadio , Pré-Escolar , Feminino , Seguimentos , Hepatite B/etiologia , Vacinas contra Hepatite B , Humanos , Esquemas de Imunização , Imunização Passiva , Lactente , Recém-Nascido , Gravidez , Fatores de Risco , Tailândia
14.
Asian Pac J Allergy Immunol ; 1991 Dec; 9(2): 89-93
Artigo em Inglês | IMSEAR | ID: sea-36979

RESUMO

A low dose of recombinant DNA hepatitis B vaccine (HB-VAX II, MSD) was tested for efficacy in the prevention of perinatal hepatitis B virus (HBV) transmission in normal and high-risk neonates born from HBsAg carrier mothers. A dose of 2.5 micrograms recombinant vaccine was injected intramuscularly at 0, 1, 2 and 12 months of age to 30 newborns from HBsAg negative mother (group I), 30 from HBeAg negative/HBsAg carrier mother (group II) and 30 from HBeAg positive/HBsAg carrier mother (group III). The incidence of persistent HBsAg carrier infants at 13 months of age was 0, 0, and 30.4 percent in groups I, II and III, respectively. The protective efficacy in high risk infants in group III was 65.7 percent. The seroconversion at month 4, after the third dose of vaccination was 96.3, 95.7 and 100 percent in group I, group II and group III, respectively. After a booster dose of vaccination at 12 months of age, the seroconversion rose to 100 percent at month 13 in all three groups. The geometric mean titer (GMT) of anti-HBs antibody at 13 months of age were 2,092, 1,657 and 1,938 mIU/ml in group I, group II and group III, respectively. It is concluded that the low dose (2.5 micrograms) recombinant hepatitis B vaccine using alone is effective in prevention of perinatal HBV transmission in low risk infants (groups I and II), but it is less effective in high risk infants (group III).(ABSTRACT TRUNCATED AT 250 WORDS)


Assuntos
Feminino , Hepatite B/prevenção & controle , Antígenos de Superfície da Hepatite B/imunologia , Vacinas contra Hepatite B , Humanos , Incidência , Lactente , Recém-Nascido , Injeções Intramusculares , Gravidez , Complicações Infecciosas na Gravidez/imunologia , Estudos Prospectivos , Fatores de Risco , Vacinas Sintéticas/administração & dosagem , Vacinas contra Hepatite Viral/administração & dosagem
15.
Asian Pac J Allergy Immunol ; 1990 Dec; 8(2): 127-32
Artigo em Inglês | IMSEAR | ID: sea-36652

RESUMO

Three cases of sinus histiocytosis with massive lymphadenopathy are reported in Thai children and is probably a first report in Thailand. They were clinically characterized by bilateral massive cervical lymphadenopathy. Other lymph node groups were also involved but no extranodal manifestations could be detected. Leukocytosis with neutrophilia, hypergammaglobulinemia and polyclonal gammopathy were common features. The characteristic histopathologic findings in the involved lymph nodes included pericapsular fibrosis, markedly dilatation of subcapsular and medullary sinuses which were filled with numerous histiocytes showing active phagocytosis of lymphocytes and lymphoplasmacytoid proliferation in paracortical strands and medullary cord. The disease characteristically has a benign protracted clinical course, and does not respond to either irradiation or chemotherapy. The etiology and pathogenesis of this disorder are unknown. The possible pathologic mechanisms include an abnormal response to a specific infectious process and an immune deficiency status.


Assuntos
Criança , Histiócitos/patologia , Histiocitose Sinusal/patologia , Humanos , Leucocitose/diagnóstico , Doenças Linfáticas/patologia , Masculino , Tailândia
16.
Artigo em Inglês | IMSEAR | ID: sea-39617

RESUMO

Massive chemotherapy and autologous bone marrow transplantation (ABMT) have been successfully used for the treatment of Burkitt's lymphoma. We report our first success with such treatment in two children with Burkitt's lymphoma in Thailand. Both patients had massive abdominal tumors with ascites and minimal bone marrow metastasis at the first presentation. They received induction chemotherapy and intensive treatment including central nervous system prophylaxis with cranial irradiation and intrathecal methotrexate until being in complete remission before starting massive chemotherapy, comprising the combination of BCNU, cytosine arabinoside, cyclophosphamide and 6-thioguanine followed by ABMT. Both patients recovered completely following intensive supportive treatments post ABMT and are still good health without evidence of the disease for 18 and 8 months after transplantation respectively. The role of massive chemotherapy and ABMT for the treatment of non-Hodgkin's lymphoma especially Burkitt's lymphoma was discussed.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Transplante de Medula Óssea , Linfoma de Burkitt/epidemiologia , Carmustina/administração & dosagem , Criança , Terapia Combinada , Ciclofosfamida/administração & dosagem , Citarabina/administração & dosagem , Feminino , Humanos , Masculino , Tailândia/epidemiologia , Tioguanina/administração & dosagem
17.
Artigo em Inglês | IMSEAR | ID: sea-42331

RESUMO

Nineteen children and adolescents in long-term complete remission from acute lymphoblastic leukemia (ALL) were studied for hormonal and growth status after cessation of therapy. Cranial irradiation of 600-2,400 cGy was given for C.N.S. prophylaxis in 18 out of 19 patients, and additional irradiation of 2,000 cGy was given to both testes of a prepubertal boy because of testicular infiltration. The time ranges after cranial irradiation and cessation of therapy at the time of study were 61-137 months and 5-127 months respectively. Thyroid hormone, cortisol and peak cortisol response after ACTH stimulation were normal in every tested children. Basal serum gonadotropin and sex steroid values were appropriate in the majority of patients. A child who received testicular irradiation, had elevated levels of gonadotropins. Glucagon stimulation test (GST) and/or L-Dopa propranolol test (DP test) were used to study growth hormone (GH) response. None had peak GH value less than 7 ng/ml. Ten patients had peak GH values of over 15 ng/ml. Nine female patients had normal puberty and regular menstruation. Eight out of ten male children also had normal puberty. All except two male patients had normal linear growth within 2 standard deviations of the mean. The mean attained final height of 11 children was not significantly different when compared to the mean predicted heights obtained from Bayley-Pinneau and Tanner methods. Excessive weight gain during and after cessation of chemotherapy was observed in the majority of children. Continuing long-term review of these children is essential.(ABSTRACT TRUNCATED AT 250 WORDS)


Assuntos
Adolescente , Criança , Terapia Combinada , Crescimento , Hormônios/sangue , Humanos , Leucemia-Linfoma Linfoblástico de Células Precursoras/sangue
18.
Asian Pac J Allergy Immunol ; 1989 Jun; 7(1): 37-40
Artigo em Inglês | IMSEAR | ID: sea-36570

RESUMO

A half dose recombinant hepatitis B vaccine (HBVax II, MSD, 5 micrograms) was investigated for efficacy in the prevention of perinatal hepatitis B virus (HBV) transmission in high risk neonates born from e-antigen positive HBsAg carrier mothers as compared to the half-standard dose regimen of plasma derived hepatitis B vaccine (HBVax, MSD, 10 micrograms). Forty infants born to carrier mothers were given hepatitis B immune globulin (HBIG) 100 IU intramuscularly immediately after birth, combined with either the recombinant or plasma derived hepatitis B vaccine. The infants were randomly divided into two groups of 20 infants each. The plasma derived vaccine (10 micrograms) was given to group I, while infants in group II received the recombinant vaccine (5 micrograms) at birth, 1 and 6 months of age. There were no statistically significant differences in the efficacy and the seroconversion rate of these two combined prophylaxis regimens. The protective efficacy rate of both kinds of HBV vaccine was found to be 94.6 and 89.2 percent in group I and group II respectively. At twelve months of age, the anti-HBs seroconversion rates were 95.0 percent in group I and 84.2 percent in group II. However, the geometric mean titres in group I (179.55 mIU/ml) was significantly higher than those in group II (42.2 mIU/ml) but the anti-HBs titre was still above protective level (10 mIU/ml) in most of the infants.(ABSTRACT TRUNCATED AT 250 WORDS)


Assuntos
Feminino , Hepatite B/prevenção & controle , Antígenos de Superfície da Hepatite B/imunologia , Vacinas contra Hepatite B , Antígenos E da Hepatite B/imunologia , Humanos , Lactente , Recém-Nascido , Gravidez , Distribuição Aleatória , Fatores de Tempo , Vacinas/administração & dosagem , Vacinas Sintéticas/administração & dosagem , Vacinas contra Hepatite Viral/administração & dosagem
19.
Asian Pac J Allergy Immunol ; 1988 Dec; 6(2): 107-10
Artigo em Inglês | IMSEAR | ID: sea-36991

RESUMO

A reduced dose of plasma derived hepatitis B vaccine (Hevac B) was tested for efficacy in the prevention of perinatal hepatitis B virus (HBV) transmission in high risk neonates born from e-antigen positive HBsAg carrier mothers. Forty newborn infants born of these mothers were given hepatitis B immune globulin (HBIG) 100 IU intramuscularly immediately after birth, combined with either standard or reduced doses of HBV vaccine. The infants were divided into two groups of 20 infants each. The standard dose of HBV vaccine (5 micrograms) was given to group I, while infants in group II received reduced dose (2 micrograms) at birth and at 1, 2 and 12 months of age. There was no statistically significant difference in the efficacy and antibody responses of these two combined prophylaxis regimens. The protective efficacy rate of HBV vaccine was found to be 94.0 and 93.2 percent in group I and group II, respectively. At twelve months of age, the anti-HBs seroconversion rates were 80.0 percent in group I and 86.7 percent in group II, with geometric mean titres of 84.57 mlU/ml and 78.56 mlU/ml, in group I and group II, respectively. One month after a booster at one year of age, anti-HBs could be detected in 86.7 percent of the infants in both groups. The geometric mean titres were 429.04 and 664.81 mlU/ml, in group I and group II, respectively. Anti-PreS2 antibody was detected in high titre as early as 4 months after the first dose of HBV vaccine, with a geometric mean titre of 116.30 mlU/ml and 107.97 mlU/ml, in group I and group II, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)


Assuntos
Portador Sadio/imunologia , Feminino , Seguimentos , Hepatite B/prevenção & controle , Antígenos de Superfície da Hepatite B/análise , Vacinas contra Hepatite B , Antígenos E da Hepatite B/análise , Humanos , Imunoglobulinas/administração & dosagem , Lactente , Recém-Nascido , Injeções Intramusculares , Gravidez , Distribuição Aleatória , Fatores de Risco , Tailândia , Vacinas contra Hepatite Viral/administração & dosagem
20.
Southeast Asian J Trop Med Public Health ; 1988 Dec; 19(4): 649-56
Artigo em Inglês | IMSEAR | ID: sea-31239

RESUMO

A kinetic study of lymphocyte subpopulations was performed in 61 dengue hemorrhagic fever/dengue shock syndrome (DHF/DSS) patients aged 8 months to 12 years and in 59 age-matched normal controls. There were 36 patients in grade 2 and 25 patients in grade 3 of the disease severity. The studies were performed on febrile stage, the day of subsidence of fever or shock stage, 3 subsequent days after subsidence of fever or shock, and once on the recovery stage (approximately 14-18 days after subsidence of fever or shock). The study revealed that the absolute total lymphocytes, CD3+, CD4+, CD8+ and HNK-1+ cells were decreased on febrile stage and their lowest values were noted on the first day of subsidence of fever or shock, while B1+ cells were in the normal range. Thereafter, all lymphocyte subpopulations were increased. The total lymphocytes, B1+ and CD8+ cells were rapidly increased and were above normal value on day 2 after subsidence of fever or shock (early convalescence), then gradually declined to the normal range. In contrast, CD3+, CD4+ and HNK-1+ cells were increased gradually and reached their normal values on day 2 after subsidence of fever or shock. The T4:T8 ratio began to reverse on the day of subsidence of fever or shock, reached its peak on day 2 after shock and returned to normal ratio rapidly thereafter. Thus, the absolute lymphopenia on the day of shock was due to the decrement or T cells (both CD4+ and CD8+ cells) and HNK-1+ cells.(ABSTRACT TRUNCATED AT 250 WORDS)


Assuntos
Criança , Pré-Escolar , Dengue/complicações , Humanos , Lactente , Linfócitos/classificação , Linfocitose/etiologia , Linfopenia/etiologia , Choque/etiologia
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