Successful allogeneic peripheral blood stem cell transplantation in Wiskott-Aldrich Syndrome Patients: first report in Thailand.

Wiskott-Aldrich syndrome (WAS), an X-linked recessive disorder, is characterized by primary progressive T cell immunodeficiency, impaired antipolysaccharide antibody production, eczema, and thrombocytopenia. Stem cell transplantation is the only curative therapy. To evaluate the use of allogeneic peripheral stem cell transplantation (PBSCT) in this group of patients, we performed allogeneic PBSCT in two WAS patients (3 and 12 years old). The conditioning regimen consisted of busulfan 4 mg/kg/day for 4 days, and cyclophosphamide 50 mg/kg/day for 4 days. Graft-versus-host disease prophylaxis was consistent with cyclosporin A and methotrexate. Peripheral blood stem cells were collected from their brother donors (6 and 16 years old) by continuous flow leukapheresis after mobilization with granulocyte-colony-stimulating factor at a dose of 7.5 microg/kg/day for 5 days. Both recipients achieved neutrophils engraftment on days 11 and 12. The first patient achieved platelets engraftment on day 30. The second patient did not have platelet count below 20.0 x 10(9)/l during PBSCT procedure. Both did not develop acute or chronic graft-versus-host disease. At present, they are healthy after PBSCT. The follow up time after transplantation is 1,170 days and 269 days, respectively. Allogeneic PBSCT is economically feasible for WAS. The cost of PBSCT in Thailand is 20 to 30% less than bone marrow and cord blood stem cell transplantation. The cost of the transplant procedure for each patient in Thailand is US $ 12,000. This study is the first report of a successful stem cell transplantation in WAS patients in Thailand.

Reduction of rotavirus infection in children receiving bifidobacteria-supplemented formula.

This study was conducted at Pakkred Babies Home, Bangkok, Thailand; with the hypothesis that children receiving probiotic-supplemented milk-based formula may be protected from developing diarrheal diseases. Salivary rotavirus-specific IgA antibody was used as an indicator of rotavirus infection. One hundred and seventy-five children, aged 6-36 months, were enrolled in the study. They were divided into 3 groups according to the type of formula given. There were 81 episodes of diarrhea during an 8-month study period, most of which were caused by bacterial enteropathogens. Ninety-seven pairs of salivary samples were adequate for the analysis of rotavirus antibody. Among 23 children receiving milk-based follow-up formula and serving as control group, 30.4 per cent of them had > or = 4-fold increase in the antibody titre, indicating subclinical rotavirus infection. The majority of children in the other 2 study groups, receiving the same formula supplemented with either Bifidobacterium Bb12 alone or together with Streptococcus thermophilus, had no significant change in the antibody titres between the two time points. The results of this study support our hypothesis that children receiving bifidobacteria-supplemented milk-based formula may be protected against symptomatic rotavirus infection.

Cow milk protein allergy during the first year of life: a 12 year experience at the children's hospital, Bangkok.

CMA should be suspected for patients aged less than one year who had persistent diarrhea and/or hematemesis with no enteric pathogen found. Confirmed diagnosis could be made by Goldman challenge test. Patients with confirmed CMA should be treated by changing the cow milk feeding to soy milk feeding. However, in our study, 17% of CMA patients were also allergic to soy protein. Thus the soy milk was replaced by the elemental formula for successful treatment of this group of patients. Beside persistent diarrhea, hematemesis, anemia and hypoalbuminemia were other possible findings among patients with CMA with or without soy protein allergy.

Nocturnal physiological and biochemical changes in sudden unexplained death syndrome: a preliminary report of a case control study.

Sudden nocturnal deaths among "healthy" workers in Southeast Asia have been termed "sudden unexplained nocturnal death syndrome (SUNDS)" or "sudden unexplained death syndrome (SUDS)". The pathogenesis is still unknown. The paucity of publications on nocturnal monitoring and scientific data stimulated us to perform this study, which included biochemical tests and physiological monitoring during the night in 11 males north-eastern Thai workers. Group 1 (G1) consisted of 5 subjects with neither a previous history of near-SUDS (NSUDS) nor a familial history of SUDS (FHSUDS). Group 2 (G2) consisted of 6 subjects with a family history of either SUDS or NSUDS. Two subjects in G2 presented with NSUDS. Two-day nocturnal monitoring included blood sugar, electrolytes, and respiratory parameters. 24-hour Holter ECGs were monitored for 2 days. The subjects underwent exercise stress tests on the 2nd day of this study. Significant nocturnal hypoxia was more common in G2 than G1 and this abnormality was aggravated by exercise. There were no significant findings in sleep apnea (apnea indices) or in nocturnal biochemical changes, eg blood sugar, electrolytes, thiamine. The recordings of the Holter-ECGs were within normal limits in both groups. We conclude that nocturnal hypoxia might be the primary abnormality in SUDS, and this abnormality was aggravated by the day-time exercise. The cause of nocturnal hypoxia requires further studies.

Comparison of efficacy of peptilose-base ORS (ORALNU) and WHO-ORS.

We examined whether replacing glucose with Peptilose into standard ORS would be advantageous over WHO-ORS. A study was carried out on 134 diarrheal children with mild to moderate dehydration. They received either WHO-ORS or Peptilose-ORS by randomized selection. In only two cases in each group, diarrhea was caused by Vibrio cholerae non 0-1. Significant per cent weight gain was observed in patients with Peptilose-ORS compared to those treated with WHO-ORS (P = 0.046). The patients could voluntarily take a higher amount of Peptilose-ORS and had significantly less stool output in the combined mildly and moderately dehydrated patients. It is concluded that Peptilose-ORS is more advantageous and acceptable than the standard WHO glucose-ORS for treatment of non cholera and 2 cases of cholera dehydrating diarrhea in children.

Risk factors for childhood diarrhea in an urban community, Bangkok, Thailand.

A follow-up study for diarrheal disease was carried out for a period of one year in children aged 0-5 yrs who lived in a government housing project in Din Daeng community of Bangkok metropolitan area during 1988-1989. The overall incidence was 0.9 episode per child per year with the higher episode of 2 per child per year in children less than 2 yrs. The risk factors of diarrheal occurrence were studies within different age groups. For children less than 6 months the major risk factors were low family income, low education level and unemployed parents. In children 6-11 months the other risk factors added to low income and education in mothers were children who did not live with parents. The risk factors for children 1-2 yrs were non-working mothers, and unhygienic behavior of the child care takers such as no hand washing after toilet use and the method of cleaning milk bottles for children 2-5 yrs. Method of faecal disposal, handwashing, day care and food protection were major sources of diarrheal risk.

Impact of an educational program on the treatment practices of diarrheal diseases among pharmacists and drugsellers.

The impact of an intervention program, measured by changes in the prescription of ORS, antibiotics and antidiarrheal drugs by those pharmacists and drugsellers after administering the proposed educational package was assessed. The results of the study indicated that, before the educational program started, ORS was prescribed by pharmacists for 31.4% and 15.7% of watery diarrhea and dysentery episodes, respectively. Only 18.9% and 13.3% of drugsellers gave ORS to assessors in case of watery diarrhea and dysentery. Antibiotics and antidiarrheal agents were prescribed extensively, watery and dysenteric diarrhea (84% and 56% for watery diarrhea by pharmacists and drugsellers; 92% and 60% for dysentery). Antidiarrheal drugs were used as frequently. After the educational program, the assessment of the prescription behavior of the pharmacists showed no change in ORS, antibiotics and antidiarrheal drugs prescribed to treat watery diarrhea. In dysentery, the effective percent change in prescribing ORS between pre- and post- intervention program was much higher in intervention group than the control group. For drugsellers, effective percent change in ORS usage in treatment of watery diarrhea was 11.8% compared with -7.7% in the control group. No such change was observed in treatment of dysentery. There was a slight significant change in behavior concerning use of antibiotics among subjects getting information by mail, compared to those who got full intervention, when the pre-intervention behavior, store type and treatment type was taken into account.

Cryptosporidium in 100 Australian children.

Over a consecutive 36 month period, 100 patients out of 6,980 (1.4%) from whom stool samples were examined had oocysts of Cryptosporidium detected on fecal microscopy. Three patients were receiving cytotoxic chemotherapy and adequate clinical information was available from 84 of the remainder. Fifty per cent of patients were aged 1 to 2 years, 29% were less than 12 months and the rest were more than 2 years of age. More than one-quarter of the patients were Australian Aborigines. Isolations of cryptosporidia were most prevalent in late summer, autumn and early winter. Most patients (89%) had diarrhea, more than 75% had vomiting and 44% were dehydrated, mostly to a mild degree. Other symptoms included fever (11%) and abdominal pain (11%). Ten percent of patients were apparently symptomless. Cryptosporidium sp. is the second most commonly identified intestinal parasite, after Giardia intestinalis, in fecal specimens examined in our children's hospital. The high rate of isolation in Aboriginal children emphasized the importance of enteric pathogens in this group and the role of inadequate hygiene in relation to diarrheal disease in young Aborigines.

Behavior of urban based child caretakers in the home treatment of diarrheal diseases.

Behaviors of low income urban mothers and child caretakers in the treatment of childhood less than 5 year diarrhea were analysed from a surveillance study conducted between August 1988 and July 1989. Help seeking behaviors of mothers and caretakers for 412 episodes of child diarrhea were as follows: investigators 37.1%, drug stores 18.2%, wait and see or self treatment 17.0%, private clinics 12.6%, near by hospital 10.2%, and local health center 4.9%. Major treatment practices included ORT alone (54%) and ORT plus antibiotics and/or antidiarrheal drug (22%). Overall ORT usage was 76%. Twelve percent of diarrheal episodes no treatment was given to the children. Antimicrobials were believed to be essential in addition to ORT especially when diarrhea was associated with fever, vomiting and bloody stools. Thirty-six percent of invasive diarrhea cases (Shigella, Salmonella, Campylobacter) were treated with antibiotics. Only 18.2% of noninvasive diarrhea received antibiotics, most of this antibiotic use being in rotavirus diarrhea where vomiting and some fever are prominent. Availability of oral rehydration salts (ORS) and good experience with ORT were the key to the extensive use or ORT in this study. A surprisingly small number of mothers and child caretakers (4.9%) sought help from the local health center when their children had diarrhea.

Etiology of diarrhea in measles.

Thirty-six measles cases with diarrhea were studied with two age and sex matched control groups, measles without diarrhea (75 cases) and acute diarrhea (70 cases). Bacterial pathogens were isolated from 5 out of 36 (13.9%) in measles with diarrhea but rotavirus, coronavirus and parasites were not detected in any case. The bacterial and viral etiology of measles with diarrhea were statistically significant different from the acute diarrhea group (p less than 0.005) and p less than 0.01 respectively) but not from the measles without diarrhea group (p greater than 0.05). 83.3 per cent of cases had diarrhea during 4 days before and after the appearance of rash. Watery diarrhea was frequently observed in this study (63.9%). Although measles virus was not identified in stools, data from this study suggested that measles may be the viral agent causing diarrhea. Watery stools are often observed and the presence of faecal white blood cell may be seen.

Liver size and serum alkaline phosphatase in normal Thai school-aged children.

The purpose of this study is to determine liver size in normal Thai school children aged 6 to 11 years, using both the clinical and ultrasonographic methods. Normal value of serum alkaline phosphatase in this age-group is also obtained. To confirm that liver function is normal, serum alanine transaminase and hepatitis B virus profile were performed. One hundred and fifty-nine normal children from Phyathai School were included in the study. They were divided into 5 age-groups: 6-7, 7-8, 8-9, 9-10 and 10-11 years. Six children were found to be asymptomatic HBsAg carriers (3.8%) and were excluded from the analysis. Mean liver sizes examined separately by two pediatricians were not significantly different, they were 8.47, 9.32, 10.06, 9.64 and 9.38 cm respectively, whereas those obtained by the ultrasonogram were 9.37, 9.17, 9.71, 10.57 and 10.21 cm respectively. Liver was palpable in 15.7 per cent of the children and liver edge did not exceed 2 cm below right costal margin. The mean value of serum alkaline phosphatase, obtained by the automate method, in these children was 223.1 unit/litre and in 97.3 per cent of them, the value did not exceed 350 unit/litre. The results of this study showed that liver size obtained clinically is comparable to size determined by ultrasonogram. Liver size in school children does not increase with age but tends to increase with body weight and height of the children.

HBsAg carrier rate among institutionalised children from Phyathai Institute.

To identify the evidence supporting the horizontal transmission of hepatitis B virus, HBsAg carrier rate among institutionalised children is determined and compared to children of the same age-group from the well baby clinic, Ramathibodi hospital. The results of this study show that HBsAg is detected four times more frequently in institutionalised children than in children from a well baby clinic. The chance of becoming an HBsAg carrier increases with age and duration of stay in the institution. Repeated study one year later shows that the HBsAg carrier rate among the same group of institutionalised children had increased by 35 per cent. The evidence supports the hypothesis that hepatitis B virus can be transmitted nonparenterally. To prevent this mode of transmission in both institutions and the community, hepatitis B vaccine should be given to these children.

Severe hepatitis B in infants born to HBsAg carrier mothers.

Five infants admitted consecutively with severe hepatitis B were treated with exchange transfusion, correction of coagulation defects and supportive measures. All were born to HBsAg carrier mothers and one had received hepatitis B immune globulin (HBIG) within 24 hours after birth. All of them presented with nonspecific symptoms such as vomiting, loose stools, low grade fever and progressed to acute liver failure. Three of them survived with full recovery and two died from hepatic encephalopathy one and two weeks after admission. Early recognition of hepatic failure and prompt exchange transfusions with intensive supportive treatment may save these infants.

Oral rehydration therapy in a rural area, northern Thailand.

A cross-sectional sampling survey of mothers' practice of ORT in Amphoe Bunpotphesai in the northern part of Thailand included 1,619 children under five. Two hundred and six children were reported to have 223 diarrheal episodes. The incidence of diarrhea in children under five was 3.4 episodes per child per year. When children had diarrhea 65.5 per cent of mothers sought help from health providers, 25.2 per cent treated their children with drugs bought from stores, 2.3 per cent used herbal medicine and 6.1 per cent did not treat their children. 50.7 per cent of diarrheal episodes mother gave ORT, using ORS 19.7, commercial electrolytes mixture 16.6 and home available fluid 14.4 per cent. The accuracy of dilution of electrolytes powder from the packets was checked in 80 incidences. 31.8 and 27.8 per cent of mothers made correct dilution of ORS and commercial electrolytes products respectively. Health providers carried both ORS and commercial electrolytes packets. ORS added to a glass of water was found in 13.6 per cent which was 3 times concentrated. Commercial electrolytes products were too dilute in 72.2 per cent. 17.5 per cent of mothers divided electrolytes powder to add in one spoon of water to treat their children as one drug dose. Data showed that the ORT use rate was 50.7 per cent. Home available fluid was used by 14.4 per cent. ORT should be further promoted to control diarrheal diseases and health providers should give instructions to every mother or child minder on how to dispense ORS or electrolytes packets for appropriate dilution and use.

Aluminium hydroxide and cholestyramine in the treatment of acute diarrhea.

A randomized, double blind control study of the treatment of acute diarrhea with aluminium hydroxide and cholestyramine in comparison with a control group demonstrated the effectiveness of cholestyramine in shortening the hospital stay, and the diarrhea course was better than that of aluminium hydroxide. However, aluminium hydroxide was superior to intravenous fluid plus early feeding.