Intensive diabetes education program and multidisciplinary team approach in management of newly diagnosed type 1 diabetes mellitus: a greater patient benefit, experience at Siriraj Hospital.

It is accepted worldwide that an effective multidisciplinary management team is essential for providing comprehensive self-management training to type I diabetics and their families. Therefore, the authors developed an intensive multidisciplinary education team that included pediatric endocrinologists, a dietitian, a psychologist, nurses, scientists and volunteers in the Department of Pediatrics, Siriraj Hospital in August 1996. This study aimed to analyze twenty-four newly diagnosed diabetics who underwent this specified program and multidisciplinary team approach in comparison to twenty-eight diabetic patients who were diagnosed before the program and team were established in order to see whether the length of hospitalization had been reduced and to compare the readmission rates of recurrent DKA with previous patients. The results demonstrated that by using the intensive program and multidisciplinary team the average length of admission was reduced from 36.04 days to 17.63 days (p value = 0.03). The readmission rate in the first year after diagnosis was also reduced from 17.8 per cent to 4 per cent. Concerning diabetes control, the average HbA1c level showed significantly better control. Therefore, this study demonstrated a successful team and program for newly diagnosed Thai childhood and adolescent diabetics and also emphasized that a multidisciplinary team approach with an effectively intensive education program is important in helping diabetics and families cope with their emerging problems and receive the long-term benefits of effective self-care.

Ambiguous genitalia: an overview of 22 years experience and the diagnostic approach in the Pediatric Department, Siriraj Hospital.

The newborn with abnormal genital development presents a difficult diagnostic and treatment challenge for the pediatrician providing care. It is important that a definitive diagnosis be determined as quickly as possible so that the appropriate treatment plan can be established to minimize medical, psychological and social complications. The purpose of this study was to provide an extensive review of the clinical characteristics of a patient cohort with ambiguous genitalia, from 22 years' experience in the Division of Endocrinology and Metabolism, Department of Pediatrics, Siriraj Hospital, and to classify them into diagnostic categories. Moreover, a cascade of diagnostic tools in approaching sexual ambiguity in the authors' institution, starting with history and physical examination and leading to further radiographic and laboratory investigations is demonstrated and can be adopted as a guideline for the clinical management of these disorders. From 1979 to 2001, care was provided to a total of 109 patients with ambiguous genitalia, of whom 104 patients were reviewed. Among these individuals, 52 patients (50.0%) belonged to the diagnosis of female pseudohermaphroditism, 5 patients (4.8%) were in the true hermaphroditism group and the remaining 47 patients (45.2%) were in the male pseudohermaphroditism group. All female pseudohermaphrodites carried a diagnosis of congenital adrenal hyperplasia (CAH) and were reared as girls. 21 hydroxylase deficiency CAH accounted for all except one (98%) in this group. Among the 47 male pseudohermaphrodites, 9 (19.1%) had dysgenetic male pseudohermaphroditism, 7 (14.9%) had either testosterone biosynthetic defects or hCG unresponsiveness, 22 (46.8%) had either androgen insensitivity syndrome or 5 alpha-reductase deficiency, 4 (8.5%) had ambiguous genitalia in a 46,XY male associated with multiple anomalies and 5 (10.6%) had an unidentifiable cause. Sex reassignment occurred, not uncommonly, in 4 cases of female pseudohermaphrodites (7.7%) and at least 2 cases (3.9%) in the combined group of male pseudohermaphrodites and true hermaphrodites. The scope of the ambiguous genitalia problem is definitely not minor. An inappropriate approach to this problem poses an undue risk to the integrity of the physical and psychosexual health in the future for these children.

Diabetes insipidus in Thai children.

The medical record of 43 patients who presented with diabetes insipidus (DI) at Endocrine Unit, Department of Pediatric, Siriraj Hospital during 1986-1995 were retrospectively reviewed in order to determine the causes of diabetes insipidus in Thai children. The results demonstrated male to female ratio of 1.9:1, mean age of 6.1 (4 years. Patients had central diabetes insipidus of 90.7% and remaining 9.3% had nephrogenic diabetes insipidus (P<0.05). The causes of central DI were hematology and oncology disease (28.2%), brain tumor (25.6%), post CNS infection (10.25%) and indiopathic cause (35.8%). Hematology and oncology disease included Langerhan cell histiocytosis (23%), non-Hodgkin lymphoma (2.5%), ALL (2.5%). Brain tumor included germinoma (15%), craniopharygioma (7.5%), astrocytoma (2.5). The results of morning urine and serum osmolarity ratio showed less than 2 in 88% of cases. The remaining 12% had the ratio more than 2 and all patients of this group had organic causes of central DI. A water deprivation test did not demonstrate a difference between organic and non organic causes of central DI. The most common hormone insuffiencies after stimulation test were growth hormone and ACTH. In summary, children who present with central DI need investigations including complete physical examination and investigations for hemato-oncology disease. If there is no hematologic causes, a CT or MRI brain is a further investigation. An anterior pituitary function evaluation is also necessary foe further management.

Precocious puberty.

Sixty-five patients who presented with clinical features of precocious puberty during 1987-1993 at pediatric endocrinology unit, Siriraj ?Hospital, were studied. Female to male ratio was 6:1. Idiopathic true precocious puberty was the most frequent diagnosis in girls (56%) but 40 percent in boys had CNS lesions. Discriminant analysis showed that the difference between none age and chronological age (BA-CA) and height standard deviation score (htSDS) could discriminate between premature thelarche and precocious puberty with 92 percent accuracy, but not serum baseline FSH, LH and estradiol. Twenty seven percent of premature thelarche had remission in two years. Seventy eight percent of congenital adrenal hyperplasia were salt-losing type.

Three years of growth hormone therapy in children with growth hormone deficiency.

The objects is to study the long-term therapeutic effect of recombinant DNA hGH by administration of biosynthetic hGH for 2 ½ - 3 years to 12 children with growth hormone (GH) deficiency, consisted of 7 males, 5 females; whose ages ranged from 3 years 4 months to 17 years old. All patients fulfilled the criteria of GH deficiency namely; growth rate less than 7 ng/ml after clonidine and dopa-propranolol stimulation test. Ten patients had idiopathic GH deficiency. Two had hypopituitarism as a result of brain tumors and cranial surgery. The growth rate was observed over a pretreatment period of 12 months. The patients received 0.1 µ/kg of recombinant DNA hGH subcutaneously 6 days per week. In males with GH-deficiency, pre-treatment mean height was 2.6 cm per year. The mean height increment at 6th, 12th, 24th, 30th, and 36th month of treatment were 6, 11.2, 14.1, 17.6, 20.6 and 23.4 cm respectively. The females had mean height increment during pre-treatment period of 2.2 cm per year. The mean height increment on 6th, 12th, 18th, 24th, and 30th month of treatment were 4.7, 7.8, 10.3, 14.2 and 14.4 cm respectively. All patients have a significant satisfactory response to recombinant DNA hGH in term of height increment. There was no report of either side effect or complication. Every patient has had improved sense of physical, mental and psycho-social well-being.