Refractory livedoid vasculitis responding to PUVA: a report of four cases.

Livedoid vasculitis is a chronic disease characterized by recurrent painful irregularly shaped ulcers, which heal with scars, most commonly located on feet or lower extremities. This condition is often resistant to the therapy. We report four cases with refractory livedoid vasculitis that responded to systemic psoralens and ultraviolet A radiation therapy.

Fixed solar urticaria induced by UVA and visible light: a report of a case.

Fixed solar urticaria (FSU) is a rare and less severe subgroup of solar urticaria. It is characterized by urticarial eruptions, which occurs on the same parts of the body following sun exposure. The lesions are reproducible at the same sites with similar morphology and distribution pattern after repeated sun exposure. The action spectrum of FSU is broad (300-700 nm). We reported a case of FSU induced by UVA and visible light. The patient responded well to cetirizine treatment.

Increasing prevalence of type 2 diabetes mellitus in Thai children and adolescents associated with increasing prevalence of obesity.

Type 2 diabetes mellitus (DM) is being diagnosed more frequently in children and adolescents. Thailand has a low incidence of childhood DM. This study reviewed patients with DM in the Division of Pediatric Endocrinology, Faculty of Medicine, Siriraj Hospital compared to our previous study. The results demonstrate that type 2 DM in Thai children and adolescents has increased from 5% during 1986-1995 to 17.9% during 1996-1999. Mean age was 11.6 years. Mean BMI was 27.8 kg/m2. Fifty-six percent were diagnosed on routine examination. The period of increase in type 2 DM is associated with an increase of obesity prevalence from 5.8% in 1990 to 13.3% in 1996. This result emphasizes the importance of encouraging daily physical activity and healthy diet in our populations and also alerts our pediatricians and endocrinologists to the possibilities of type 2 DM in these age groups.

Automated cardiac auscultation for detection of pathologic heart murmurs.

Experienced cardiologists can usually recognize pathologic heart murmurs with high sensitivity and specificity, although nonspecialists with less clinical experience may have more difficulty. Harsh, pansystolic murmurs of intensity grade > or = 3 at the left upper sternal border (LUSB) are likely to be associated with pathology. In this study, we designed a system for automatically detecting systolic murmurs due to a variety of conditions and examined the correlation between relative murmur intensity and likelihood of pathology. Cardiac auscultatory examinations of 194 children and young adults were recorded, digitized, and stored along with corresponding echocardiographic diagnoses, and automated spectral analysis using continuous wavelet transforms was performed. Patients without heart disease and either no murmur or an innocent murmur (n = 95) were compared to patients with a variety of cardiac diagnoses and a pathologic systolic murmur present at the LUSB (n = 99). The sensitivity and specificity of the automated system for detecting pathologic murmurs with intensity grade > or = 2 were both 96%, and for grade > or = 3 murmurs they were 100%. Automated cardiac auscultation and interpretation may be useful as a diagnostic aid to support clinical decision making.

Cardiac auscultatory recording database: delivering heart sounds through the Internet.

The clinical skill of cardiac auscultation, while known to be sensitive, specific, and inexpensive in screening for cardiac disease among children, has recently been shown to be deficient among residents in training. This decline in clinical skill is partly due to the difficulty in teaching auscultation. Standardization, depth, and breadth of experience has been difficult to reproduce for students due to time constraints and the impracticality of examining large numbers of patients with cardiac pathology. We have developed a web-based multimedia platform that delivers complete heart sound recordings from over 800 different patients seen at the Johns Hopkins Outpatient Pediatric Cardiology Clinic. The database represents more than twenty significant cardiac lesions as well as normal and innocent murmurs. Each patient record is complete with a gold standard echo for diagnostic confirmation and a gold standard auscultatory assessment provided by a pediatric cardiology attending.

Incidence of type 1 diabetes in children under 15 years in northern Thailand, from 1991 to 1997.

The annual incidence of Type 1 diabetes in children under 15 years in northern Thailand from 1991 to 1997 was a retrospective study by analyzing the data gathered from the Pediatric Endocrine Division, Faculty of Medicine, Chiang Mai University and through a mail survey to 202 hospitals in the government and private sector in northern Thailand. The response to the mail survey from 1991 to 1992 and 1993 to 1997 was 90.1 per cent and 94.5 per cent respectively. During the seven year period, 76 new cases of Type 1 diabetes were identified. The crude annual incidence ranged from 0.31-0.56/100,000 per year, with an average incidence of 0.37/100,000 per year (95% C.I. = 0.29-0.46). This very low figure had risen 2.2 fold (over 100%) from that reported in 1984. The annual incidence was relatively constant from 1991 to 1996, although there was a moderate rise in 1997 (0.56/100,000 per year). There was no statistically significant difference between the annual incidence of 1996 and 1997. It remains for further studies to confirm the trend of increased incidence. The median age of onset was 11 years, whereas, the peak age of onset occurred in the 10-14 year age group of both sexes. There was a greater incidence among female in this study. These date confirm the need to develop a national registry of Type 1 diabetes for further epidemiological research.

Incidence of diabetes mellitus type 1 in children of southern Thailand.

The study was aimed to determine the incidence of diabetes mellitus in children under fifteen years old in southern Thailand. The data of newly diagnosed diabetic patients from 1992 to 1996 were collected by questionaire. The incidence of diabetes mellitus type 1 was 0.52/100,000 population under fifteen years old. There was an increasing trend of this disease over the study period. The peak incidence occurred in the age group 11-15 years. It was more common in females than males.

Incidence of childhood type 1 (insulin dependent) diabetes mellitus in northeastern Thailand.

The incidence of type 1 (insulin dependent) diabetes mellitus in children under 15 years from northeastern Thailand was reported. Data of new cases from 1991 to 1995 were collected retrospectively by a mail survey from 257 government hospitals in the northeastern part of Thailand. The incidence rate of type 1 (insulin dependent) diabetes mellitus was 0.3/100,000 (95% confidence interval [CI] = 0.24-0.38). The incidence rate in girls was 2 fold that of boys. More than half of the cases were diagnosed between the ages of 10 and 14. These data indicated that incidence of childhood type 1 diabetes mellitus in Thailand is one of the lowest in the world.

Type 1 diabetes in Thai children aged 0-14 years.

Fifty-nine patients were diagnosed with diabetes in the ten years from 1987 to 1996 in the Division of Endocrinology and Metabolism, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Bangkok, Thailand. All patients were less than fifteen years old. Fifty-five patients (93.3%) were type 1 diabetes, three (5%) were type 2 diabetes and MODY, and one (1.7%) was diabetes secondary to beta thalassemia major. Patients with type 2 diabetes, MODY, and secondary diabetes were excluded from this study, and fifty-five patients with type 1 diabetes were analysed. The aims of this study were to determine some of the general characteristics of Thai childhood type 1 diabetes and to see whether a seasonal variation is present. The results showed a female to male ratio of 1.39:1. The peak age at diagnosis was from 9 to 12 years. Seventy-seven per cent had diabetic ketoacidosis at the time of diagnosis. The majority of patients (93.9%) had a BMI of less than 20 kg/m2. A family history of diabetes was reported in 38 per cent but only 2 per cent were type 1 diabetes. We found a high prevalence of patients in the summer and winter seasons (35-48% and 37-50% respectively) and a lower prevalence in the rainy season (14.8-15%). These results are different from a previously reported study in 1984-1985 which found no differences in summer, winter, and rainy seasons. Further research study into Thai childhood type 1 diabetes is needed, especially the influence of seasonal factors, the incidence of the disease, and the significance of family history.

Serum IGF-I and IGFBP-3 levels for normal Thai children and their usefulness in clinical practice.

Serum IGF-I and IGFBP-3 levels are growth hormone (GH) dependent and reflect the endogenous GH secretion. Two hundred and forty-four healthy children were evaluated for serum IGF-I and IGFBP-3 levels and then the age-defined normal values for Thai children were constructed. The results showed that the serum IGF-I and IGFBP-3 levels were age dependent, gradually increased from birth and reached the peak values around the age of 14-16 years. In addition, we studied the IGF-I and IGFBP-3 values in 28 GH deficient children and 26 normal variant short stature (NVSS) by using our normal constructed values as the reference. To minimize the influence of age, both IGF-I and IGFBP-3 values were transformed to standard deviation score (SDS). In clinical practice, we recommend using the IGF-I SDS and IGFBP-3 SDS of -1 and -1.3 respectively as a cut-off point to discriminate between GH deficiency and NVSS to avoid risky GH provocative tests and unnecessary GH replacement with the sensitivity of 71 per cent and the specificity of 92 per cent.

Growth hormone therapy update in Thailand.

There were 841 children in Thailand with growth hormone deficiency (GHD) from January 1992 to 1996. Idiopathic isolated GHD was the major diagnosis. Only 40.19% received recombinant DNA human growth hormone (rhGH) treatment. Also reported here is a 1-2 year study of rhGH therapy in 30 GH-deficient children (21 males, 9 females), aged (mean +/- SD) 10.41+/-3.16 years, and bone age 7.37+/-3.34 years. The height velocity 1 and 2 years posttreatment were 8.17+/-1.9 and 7.36+/-2.8 cm/year respectively, which were statistically significant compared to pretreatment values of 3.91+/-1.09 cm/ year. Improved height SDS was observed at the end of each treatment period. Thyroid function and glycosylated hemoglobin tests were normal during the treatment period. There were no reports of side effects.

Growth response in Turner syndrome with recombinant human growth hormone therapy.

Ten Turner syndrome girls whose mean age was 10.9 +/- 2.7 years were treated with recombinant human growth hormone (rhGH), dose 0.6 U/kg/week. Five of them had classical 45, XO karyotype. The mean height velocity increased from 2.8 +/- 1.3 cm/year before treatment to 6.1 +/- 2.06 cm/year after treatment for a period of 1.4 years. The response of treatment correlated well with pretreatment height velocity (<3 cm/year) but not with karyotype. However, the response has been decreasing and an increased dose after the first year of treatment is recommended.

Molecular examination of GH gene deletion in familial growth hormone deficiency.

The human growth hormone gene (GH gene) from nine members of a family with familial growth hormone deficiency was examined. The patients were diagnosed as having growth hormone deficiency clinically and by response to hormonal treatment. PCR amplification was carried out using total DNA extracted from leukocytes. The flanking regions of the GH gene which are highly homologous were amplified by one pair of primers. PCR products of 1900 bp and 1919 bp were obtained. By using the combination of restriction enzymes BgII, HaeII and SmaI to digest these PCR products, the various sizes of GH gene deletion can be detected. None of the possible deletions was found in these patients and their relatives by either PCR or Southern blot analysis.

Congenital generalized lipodystrophy, a case report.

Generalized lipodystrophy is a rare condition which can be divided into congenital and acquired types, based on the age at presentation and pattern of inheritance. The congenital type of generalized lipodystrophy or Lawrence-Seip syndrome presents in first two years of life and is inherited in an autosomal recessive pattern. The diagnosis is made on the basis of loss of body fat, muscular hypertrophy, acanthosis nigricans, hirsutism, hepatomegaly with fatty liver, hyperlipidemia and hyperglycemia with insulin resistance. A 2 1/2-year-old Thai girl with the clinical features of Lawrence-Seip syndrome is reported. Abnormal platelet function was detected in this girl.

HLA class II polymorphism in Thai insulin-dependent diabetes mellitus.

Fifty-seven Thai IDDM patients were studied for HLA class I by LCT and HLA class II by LCT and PCR-RFLP. It was found that DRB1*0301, DR3, DQB1*0201, DRB3*0202, DQA1*0501 and DQ2 were significantly increased with R.R. = 10.0, 6.6, 4.2, 3.7, 3.5 and 3.2 and Pc < 0.005, 0.001, 0.01, 0.005, 0.01 and 0.005, respectively. In contrast, DQA1*0101, DRB3*0301, DR5 and DQ1 were significantly decreased with R.R. = 0.2, 0.2, 0.3 and 0.5 and Pc < 0.01, 0.05, 0.01 and 0.05, respectively. The primary factor for IDDM susceptibility is probably DRB1. The homozygous Asp57/Asp57 DQB1 genotype appears to determine resistance to IDDM while Arg52-DQA1, non-Asp57-DQB1 haplotype confers susceptibility to IDDM. The common haplotypes in Thai IDDM cases were DRB1*0301, DRB3*0202, DQA1*0501, DQB1*0201, DPB1*0401 and DRB1*0405, DQA1*0301, DQB1*0402 (or 0401 or 0302), DPB1*0401 (or 0301 or 1501). The less common haplotypes were DRB1*0406, DQA1*0301, DPB1*0302, DPB1*0501 and DRB1*1202, DRB1*0301, DQA1*0601, DQB1*0301, DPB1*0501. DR3 was increased in both gender groups with early onset (< 10 years) regardless of a family history of DM. However, DR3/DR4 genotype was increased only in female patients with a family history of DM and early onset. In conclusion, DRB1, DRB3, DQA1 and DQB1, but not DPB1 are involved in the occurrence of IDDM. The cooperation of HLA class II and X-chromosome may contribute to the development of IDDM in addition to other factors such as other genetic (chromosomes 11, 19, 14, 7), immunologic and environmental factors which require further study.

The epidemiology of insulin-dependent diabetes mellitus (IDDM): report from Thailand.

The epidemiology of diabetes mellitus in Thai children aged 0-15 years was studied in 1985 and compared with a previous study done in 1984. Four hundred and seventy-six questionnaires were sent each year to hospitals in Thailand. In 1984, thirty-six cases of newly diagnosed diabetes mellitus were found of which 35 were IDDM and one was NIDDM. In 1985, twenty-seven cases of new IDDM were found, no case of NIDDM was reported. Two cases of MRD were reported from the Northeastern and Southern part of Thailand. The incidence of IDDM in the whole kingdom of Thailand was 0.19/100,000/year in 1984 and 0.14/100,000/year in 1985. The male to female ratio was 1:1.5 in 1984 and 1:2 in 1985. The peak age at diagnosis showed the main peak at 14 years old in boys. The peak age of girls preceded boys by 1-2 years in 1984 and 1985. Similar findings in 1984 and 1985 were the onset of symptoms showing a seasonal variation with highest frequency in winter with a slight change of increased incidence in the rainy season of 1985. There was an increased incidence of IDDM in families with lower educational and socioeconomic levels. The newly diagnosed IDDM with DKA was 16.2, and 19.5 per cent in 1984 and 1985. The incidence of IDDM in Thai children, aged 0-15 years seems to be the lowest compared to other countries previously described which might be due to some genetic and environmental including diet, micronutrient, eating habits and life-style which might play a role in the difference.

Effect of recombinant DNA human growth hormone in Thai children with growth hormone deficiency.

We conclude that recombinant DNA methionine-free hGH treatment of GH-deficient Thai children is very effective in the first year of treatment. No patients reported any side effects or resistance to treatment.

Diabetic education, special consideration of Oriental patients.

The important of patient education program in the management of diabetes has been widely recognized. We studied to find out in general what the patients and their parents know about diabetes and their self-care by using a questionnaire. Then, the diabetic education was given by one-to-one basis to every patient. Thirty four insulin-dependent diabetes mellitus attended the diabetic the clinic at Siriraj Hospital, Bangkok age ranged from 4 to 22 years with peak age at 11 to 15 years. Male to female ratio was l:i. Majority came from low socioeconomic families. 23.5 percent were from separated families, one patient lived with neither her mother nor father. Only one patient had home glucose monitoring. Fourteen cases (41.2%) had been hospitalized with diabetic ketoacidosis (DKA) over the past year, however, there was no statistically significant difference between admission with DKA and low socioeconomic status. In addition to insulin, there were 8 patients taking herbs to cure diabetes. Only 6 patients were able to follow their meal plan and only one case ever used the food exchange list. Most patients accepted being diabetic and attended the clinic regularly mainly to get financial supports. The situation in our country is different from that in the western countries as the patients are low in literacy and socioeconomic status. A well-planned educational programme is essential to cater to the need to the oriental patients.