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.

Clinical evaluation of the addition of lyophilized, heat-killed Lactobacillus acidophilus LB to oral rehydration therapy in the treatment of acute diarrhea in children.

BACKGROUND: Addition of a medication to the World Health Organization protocol for treatment of acute diarrhea in children is controversial. In this trial, the clinical efficacy of a medication (Lactéol Fort sachets; Laboratoire du Lactéol du Docteur Boucard, Houdan France) containing lyophilized heat-killed Lactobacillus acidophilus LB was assessed as an adjunct to oral rehydration therapy. METHODS: Children aged 3 to 24 months with acute diarrhea and mild or moderate dehydration were enrolled in the study. Children received oral rehydration therapy for the first 4 hours. After this first rehydration phase, undiluted milk formula or breast milk was fed alternately with oral rehydration solution. Children were fed rice gruel as tolerated. They received either one sachet containing 10 billion of lyophilized heat-killed L. acidophilus LB or placebo at admission and at 12-hour intervals for five doses. RESULTS: Seventy-three children (37 L. acidophilus LB, 36 placebo) were enrolled, of whom 40 (17 L. acidophilus LB, 23 placebo) received an antibiotic before inclusion. Rotavirus was identified in approximately 50% of the children in each group. After 24 hours of treatment, the number of rotavirus-positive children with watery stools was significantly lower (p = 0.012) in the L. acidophilus LB group. Mean duration of diarrhea was decreased (p = 0.034) with L. acidophilus LB (43.4 hours) versus placebo (57.0 hours). This decreased duration was particularly marked in children with no antibiotic therapy before inclusion (31.1 hours): 42.9 hours for the L. acidophilus LB group versus 74.0 hours for the placebo group (p = 0.016). CONCLUSIONS: Addition of L. acidophilus LB to oral rehydration therapy was effective in the treatment of children with acute diarrhea by decreasing the duration of diarrhea.

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.

Transfusion-transmitted AIDS with blood negative for anti-HIV and HIV-antigen.

A four-year-old boy who was diagnosed with Glanzmann's thrombasthenia received supportive treatment and desmopressin for his bleeding episodes. He seldom received blood components, except for platelet concentrates, due to severe bleeding. He was exposed to 18 routine donors and 2 plateletpheresis donors who were negative for human immunodeficiency virus (HIV) antigen and anti-HIV upon screening. At the age of 3 years and 9 months (4.5 months after the transfusion of platelet concentrate), he developed full-blown AIDS and died from circulatory failure 3 months later. The source of HIV transmission was identified as I donor who developed anti-HIV 3 months after the last donation. The rather short incubation time of AIDS in this case was attributed to a large inoculum of HIV virus in the 'window period' of infectivity of the newly infected donor. This case illustrates transfusion-transmitted HIV leading to AIDS, even though HIV antigen and anti-HIV screening tests were negative.

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.

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.

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.

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.

Childhood diarrhoea in a low-income urban community in Bangkok: incidence, clinical features, and child caretaker's behaviours.

A one-year surveillance study of childhood diarrhoea in a low-income urban community in Bangkok revealed an annual incidence of 2.2 episodes per child among infants, and that the overall annual incidence among children under five years of age was 0.9 per child. Rotavirus, Salmonella and Campylobacter jejuni were common aetiologic agents. In children less than one year, diarrhoea was caused mostly by rotavirus and Salmonella. In 1-2 year old children, the major causative agent was rotavirus while E. coli, Campylobacter jejuni and Shigella were subsequent aetiologic agents. In grown up children (aged 2-5 years), the more common diarrhoeal pathogens were Shigella and E. coli. The clinical characteristics of diarrhoeal illness due to different pathogens were shown. The sources of drugs and the usage of available facilities in treating diarrhoea are also described. Caretakers treated childhood diarrhoea with ORS (53%), antibiotics (10%), and a combination of these in 15% of cases. These findings imply that the available facilities in this community can be better utilised to obtain more effective control of diarrhoeal morbidity and mortality.

Oral rehydration therapy for invasive diarrhea.

Current guidelines for the management of diarrheal diseases focus on the use of oral rehydration therapy (ORT). Clinically evident dehydration of a moderate degree is not uncommon with invasive diarrhea, and when it occurs, the response to ORT is satisfactory. Studies from hospitals and the community each document the effectiveness of ORT for rehydration of patients with invasive diarrhea. This has been confirmed in a clinical trial of oral rehydration solution (ORS) for the treatment of diarrheal diseases in children less than 5 years of age. Children with moderate dehydration benefited most from ORS, especially those who had culture-proven invasive diarrhea. Significant early weight gain was observed for this group of patients alone after rehydration with ORS. In developing countries, there is no reason to withhold ORT at the first signs of watery or dysenteric diarrhea regardless of the cause and independent of the decision to treat the patient with antimicrobial agents.

Importance of salmonellae and Campylobacter jejuni in the etiology of diarrheal disease among children less than 5 years of age in a community in Bangkok, Thailand.

The etiology of diarrhea in children less than 5 years of age in a low-income housing project in Bangkok, Thailand, was determined over 1 year. Nontyphoidal salmonellae (13%), Campylobacter jejuni (12%), rotavirus (12%), enterotoxigenic Escherichia coli (7%), shigellae (6%), E. coli that hybridized with the enteropathogenic E. coli adherence factor probe (3%), and enteroinvasive E. coli (1%) were identified in 345 episodes of diarrhea in children less than 5 years of age. Salmonellae were identified in 17% and C. jejuni was identified in 15% of 54 children less than 6 months of age with diarrhea. Shigellae, enteroinvasive E. coli, enteropathogenic E. coli adherence factor, and enterotoxigenic E. coli were not isolated from children less than 6 months of age. Since salmonellae and C. jejuni were the most common bacterial pathogens identified in children less than 6 months of age, efforts to prevent transmission of salmonellae and campylobacter to young children should be a public health priority in Bangkok.

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.

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.

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.

PIP: From June 8-17, 1987, researchers observed oral rehydration therapy (ORT) practices of 200 mothers of children 5 years old and ill with diarrhea in a rural area of northern Thailand. 206 of 1619 children (12.7%) had at least 1 episode of diarrhea during the study period (223 diarrheal episodes). Researchers estimated the number of episodes of diarrhea/year/child to be 3.4 46.1% of the cases experienced mild diarrhea and no child had severe diarrhea. 70.9% of children 2 years old experienced diarrhea during the study period. These children had the highest proportion of diarrheal incidence. 25.2% of the mothers treated the diarrhea with drugs, while 67.8% went to health providers for treatment. 47.5% of the mothers did not treat their children's diarrhea with ORT. 19.7% of those that did treat their children with ORT used WHO's ORS packets, 16.6% used commercial electrolyte mixtures, and 14.4% used home available fluid. Researchers checked the accuracy dilution of ORS and commercial electrolyte packets in 80 cases of diarrhea. Only 31.8% of ORS was correctly prepared. Further, 13.6% of ORS preparations were 3 times too concentrated--a potentially harmful concentration. Only 27.8% of the commercial packets was correctly diluted. The rest were too diluted. Researchers theorized that these mothers either did not read the instructions on the packets and/or did not receive any instructions from the health providers on how to correctly prepare ORT. No child had any adverse effects as a result of improper preparation, however.

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.

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.

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.