Combined raw cornstarch and nifedipine as an additional treatment in persistent hyperinsulinemic hypoglycemia of infancy.

This study reports the result of treatment with the combination of raw cornstarch and nifedipine in two infants affected with hyperinsulinemic hypoglycemia of variable severity. The first infant developed hypoglycemia during early neonatal period and required subtotal pancreatectomy. She still developed hypoglycemia after her second operation. The second infant developed hypoglycemia at the age of 7 months. Raw cornstarch and nifedipine efficiently normalized both infants' blood glucose levels. Although they still need frequent feedings, no hypoglycemic episode was reported except when they were sick. Their growth and development were markedly improved after initiation of treatment.

Thyroid function in healthy Thai neonates.

OBJECTIVES: To construct a normative data for serum thyroxine (T4), free T4 (FT4), triiodothyronine (T3) and thyrotropin (TSH) in Thai neonates. STUDY DESIGN: A cross-sectional study of 275 healthy full-term neonates was conducted. Blood samples were obtained from umbilical cords of the neonates and from heel pads of infants aged 1-30 days. Hormone measurements included serum T4, FT4, T3 and TSH. RESULTS: Mean serum T4 and FT4 levels rapidly increased after delivery to the maximum level at 1-3 days of age. Thereafter, they declined to a steady state level within 2-4 weeks. Mean serum T3 level was very low at birth. The concentration increased 3-5 times and reached a steady state levels within 1 week. In contrast, mean serum TSH declined from birth and the level at 1-3 days of age was slightly less than that of the cord blood. It changed little after 3 days of age. Previous studies have shown a transient TSH surge in the first 24-48 hour of life. TSH surge was not apparent in our study because samples were not obtained from infants < 24 hours old. Therefore, if TSH is measured for screening of congenital hypothyroidism, samples should be obtained from umbilical cord or infants aged > 48 hours. CONCLUSIONS: This study provides the normative data for thyroid function tests in Thai full-term neonates. These data are useful for detection and verification of hypothyroidism in a screening program for congenital hypothyroidism.

Gonadotropin-releasing hormone testing in premature thelarche.

Premature thelarche (PT) is characterized by isolated breast development in girls prior to 8 years of age. In addition, there is neither growth spurt nor advanced bone age. It has been suggested that luteinizing hormone (LH) response to gonadotropin-releasing hormone (GnRH) alone is adequate to distinguish central precocious puberty from PT. However, LH response to GnRH is greater in infancy than that in childhood. Therefore, gonadotropin response to GnRH in girls with isolated premature breast development in different age group was studied. Thirty-six girls with isolated PT (aged 0.25-8 years) were evaluated. They were classified into 2 groups; aged < 4 years (group A: mean age 1.57 +/- 0.87 years, n = 13) and > or = 4 years (group B: mean age 6.97 +/- 0.94 years, n = 23). Initial evaluation included X-ray bone age, pelvic sonography and GnRH testing. Patients were followed for at least 1 year to confirm that no patient had progression into puberty. Bone ages in both groups were within mean +/- 2 SD in all patients. Pelvic sonography was performed in all patients which revealed no abnormality of ovaries and uterus. Pubertal response to GnRH stimulation is characterized by peak LH of > 20 IU/L or delta LH of > 15 IU/L which is generally greater than peak follicle stimulating hormone (FSH) or delta FSH, respectively. Mean peak LH and delta LH in group A were 13.0 +/- 6.06 and 11.4 +/- 5.92 IU/L whereas those in the group B were 8.5 +/- 4.10 and 6.3 +/- 3.49 IU/L. Therefore, LH response to GnRH in group A was significantly higher than that in group B (p < 0.05). In addition, the mean peak FSH and delta FSH in group A were 120.5 +/- 45.87 and 109.9 +/- 42.09 IU/L whereas those in the group B were 48.7 +/- 24.05 and 39.9 +/- 23.69 IU/L. Therefore, FSH response to GnRH in group A was significantly greater than that in group B (p < 0.001). LH response to GnRH alone can distinguish prepuberty from puberty in girls > 4 years of age. However, in prepubertal young girls with PT aged < 4 years, pubertal LH response can occur, i.e. peak LH > 20 IU/L. Hence, the greater FSH response to GnRH than that of LH would confirm the diagnosis of premature thelarche in this group. Therefore, the evaluation of FSH response to GnRH is beneficial to distinguish puberty from prepuberty in young girls.

Five-year thyrotropin screening for congenital hypothyroidism in Ramathibodi Hospital.

OBJECTIVES: To detect newborns with congenital hypothyroidism (CH) and to treat the affected infants as early as possible. STUDY DESIGN: Cord blood thyrotropin (TSH) screening for CH in Ramathibodi Hospital began in 1993. From October 1993 to December 1998, 35,390 neonates were screened. The infants with elevated TSH level of greater than 30 mU/L were recalled for verification of CH. Confirmation tests included total thyroxine, free thyroxine and TSH level. Thyroid scan and uptake were performed in some affected infants. RESULTS: Twelve infants with CH were detected resulting in an incidence of one in 2,949 live-births. All affected infants were asymptomatic at birth. Of 12 infants with CH, one premature neonate had a delayed TSH elevation and was diagnosed as having primary hypothyroidism at 2 months of age. The recall rate for validation of CH based on a cut-off value at serum TSH level of greater than 30 mU/L is 1.1 per cent. If the cut-off value of serum TSH level was raised to greater than 40 mU/L, the recall rate would decrease to 0.43 per cent. None of the affected infants had cord blood TSH level of less than 50 mU/L except one premature patient. Therefore, beginning in January 1997, the cut-off value of TSH was raised to 40 mU/L or greater. Pitfalls in this program include incomplete blood-specimen collection and incomplete follow-up. To strengthen the program, improvements were made in the follow-up system from 1996 onward. Therefore, the coverage for blood-specimen collection progressively increased from 84 per cent in 1994 to 96 per cent in 1998. Simultaneously, the patients' return after recalls also increased from 38 per cent to 100 per cent. CONCLUSIONS: The incidence of CH in Ramathibodi Hospital is approximately 1:3,000 live-births. The optimal cord blood TSH level for recall is 40 mU/L or greater. The intensification of follow-up strategy resulted in better response to recall and earlier treatment in the affected infants.

Elevated serum cholesterol levels in Bangkok children and adolescents.

Hypercholesterolemia is a major cardiovascular risk factor. This study aimed to assess serum total cholesterol (TC) levels of children and adolescents living in Bangkok, Thailand. During 1995-1997, nonfasting blood samples were obtained from 570 healthy school children and adolescents aged 9-18 years. The mean TC levels ranged from 143-180 mg/dl in males and from 145-202 mg/dl in females. The prevalences of hypercholesterolemia (TC > or = 200 mg/dl) were 12.2 per cent and 20.3 per cent in males and females, respectively. Twenty-eight per cent of males and 26.9 per cent of females had borderline values (TC 170-199 mg/dl). TC inversely correlated with age (r = -0.16, P < 0.01) in males. The findings indicate that notable percentage of these children had elevated cholesterol levels and warrant additional study concerning risk factors and tracking of lipoprotein levels from childhood into adulthood.

Slipped capital femoral epiphysis in Ramathibodi Hospital.

Slipped capital femoral epiphysis is rare in Asiatic Indonesian-Malays. Seven cases (9 hips) of this condition in Ramathibodi Hospital including five boys (average age, 12.5 years) and two girls (average age, 13 years) were reviewed. Most of the cases (4 out of 7) were acute on chronic and mild slips. No endocrine disorder was observed in all cases. All of the patients had a body weight above the mean of the normal population, four of which were obese. For the treatment, a single screw fixation including one case with cancellous and six cases with cannulated type were used. In the follow-up of average 2.5 years, six cases had satisfactory results. Avascular necrosis occurred in one case with mild and chronic slips in which a cancellous screw was used. It is concluded that obesity is the important factor related to the etiology in this study and probably is the same in other developing countries. The effect of a cancellous screw causing avascular necrosis is still questionable.

Insulin-like growth factor-I (IGF-I) screening for the diagnosis of growth hormone (GH) deficiency.

Growth hormone deficiency (GHD) is a common cause of growth retardation in children and adolescents. Gold standard for the diagnosis of GHD is based upon two standard growth hormone (GH) provocative tests demonstrating a peak serum GH of less than 7 ng/mL. These tests, besides requiring multiple blood samplings, are time-consuming and costly. GH primarily mediates its growth-promoting effect through insulin-like growth factor-I (IGF-I). Hence, basal serum IGF-I level reflects GH status. We studied 47 prepubertal children with or without short stature. Aged ranged between 4.3 and 15.6 years. They were divided into 2 groups based upon 2 standard GH provocative tests. Seventeen Children were classified as having GHD. The remaining 30 were non-GHD. Basal serum IGF-I was obtained before GH testing. The means +/- SE (range) of serum IGF-I concentration were 44.26 +/- 3.19 (19.10-75.63) ng/mL in GHD and 118.42 +/- 10.03 (60.65-235.91) ng/mL in non-GHD which were significantly different (P < 0.001). 88.2 per cent of GHD had serum IGF-I concentration less than 60 ng/mL whereas 100 per cent of non-GHD had serum IGF-I greater than 60 ng/mL. There was no correlation between serum IGF-I and either bone age or chronologic age in children with GHD. These data indicate that serum IGF-I level is a useful screening test to exclude GHD with high sensitivity. We suggest that if serum IGF-I is less than 80 ng/mL in prepubertal children, GH provocative tests should be performed to diagnose GHD. If serum IGF-I is greater than 80 ng/mL, growth rate monitoring is recommended. If growth rate is decreased despite normal IGF-I, GH provocative tests should be obtained to rule out GHD.

Prolactin (PRL) release in normal and growth hormone deficient children after oral metoclopramide.

Studies were done to determine plasma PRL in response to oral MC 0.2 mg/kg in 17 normal children (NC), 12 males and 5 females aged between 4.7-12.8 years and in 26 idiopathic growth hormone deficient children (IGHD), 15 M, 11 F, between 1.5-14.7 years old. Peak serum GH levels above 10 ng/ml after clonidine test and during insulin induced hypoglycemia were used to distinguish these 2 groups. None of the subjects had secondary sex characteristics. Adrenocortical and thyroid disorders were excluded. The subjects were fasted overnight. Blood samples for PRL determination were obtained at 0, 60, 90, 120 min after oral MC. In 17 NC the basal serum PRL values ranged from 0 to 19.2 ng/ml with the mean +/- SE of 7.24 +/- 1.7 ng/ml. The peak serum PRL response to MC ranges were from 33 to 127 ng/ml with the mean +/- SD and +/- SE of 64.45 +/- 24.22 and +/- 5.88 ng/ml respectively giving the cut point-2SD value of 16.01 ng/ml. Among 26I GHD, only 2 patients (7.69%) being all male, had peak PRL response to MC below 16.01 ng/ml, whereas, the rest (92.31%) had peak PRL levels above it. It is concluded that oral MC 0.2 mg/kg is the potent PRL stimulator in children, which can be safely used to test pituitary PRL secretion effectively. The majority (92.31%) of idiopathic GH deficient children had adequate serum PRL response to oral MC, whilst 7.69 per cent disclosed inadequate response which might indicate different etiologies.

Congenital complete absence of GH, TSH and PRL in infants: a consequence of Pit-1 gene deletion.

The patient was the first child of a short mother (140 cm) born at term with a birthweight of 2,700 g. On arrival, she was 1 4/12-year-old, weighed 4,150 g and 47 cm long. Her bone age was at the 6 month-old level. Endocrine investigation revealed undetectable plasma growth hormone (GH), thyrotropin (TSH) and prolactin (PRL) levels. CT scan of ovaries revealed bilateral ovarian agenesis in spite of normal, 46 XX karyotype. MRI of the brain did not demonstrate intracranial tumor or congenital malformation. Peak plasma GH level after oral clonidine provocation, insulin induced hypoglycemia, and I.V. GH-RF stimulation were 0.6, 0, and 0 ng/ml respectively. Peak plasma TSH response after I.V. TRH stimulation was 0.04 microU/ml. The patient could not secrete PRL at any time after insulin induced hypoglycemia, TRH and metoclopramide stimulations. On the other hand the child had elevated basal plasma cortisol (38 micrograms/dl at 8.00 AM) and raised 24 hr urinary 17 OHCS excretion (50 mg/1 g Cr against normal value of 3 mg/1 g Cr) without evidence of Cushing syndrome probably indicate partial glucocorticoid resistance. Peak plasma cortisol responses after intravenous metoclopramide and insulin induced hypoglycemia were 46 and 42.9 micrograms/dl respectively. Dexamethasone administration reduced plasma cortisol to 2.9 micrograms/dl. The child had also elevated basal plasma FSH (36 microU/ml) and LH (5 microU/ml) with further elevation to the peak of 123 and 99 microU/ml respectively after LHRH stimulation. All evidence suggested the diagnosis of congenital complete absence of GH, TSH, and PRL which is characteristic of Pit-1-gene deletion.(ABSTRACT TRUNCATED AT 250 WORDS)

Plasma prostacyclin (PGI2) in dengue hemorrhagic fever.

Dengue hemorrhagic fever (DHF) is an epidemic viral disease. The exact mechanism attributable to platelet and vascular dysfunctions is still obscure. Plasma 6-keto-PGF1a (6KPGF1), the stable metabolite of PGI2 was determined in 60 DHF patients and in 11 non-DHF (NDHF) patients with fever of over 38.5 degrees C to compare with that of 33 normal children (NC) in the same age group (2-15 years). Among 60 DHF patients, 32 had blood obtained during impending shock, whereas blood samples of the remainder were taken during normotension. Their plasma 6 KPGF1 values (mean +/- SE) were 201.06 +/- 12.42 and 132.87 +/- 13.08 pg/ml respectively. All patients had serology positive for acute dengue viral infection. The plasma 6 KPGF1 (mean +/- SE) of 33 NC and 11 - NDHF subjects were 149.82 +/- 4.93 and 108.69 +/- 14.53 respectively. The plasma 6KPGF1 levels of 32 DHF patients with impending shock were significantly higher than those of 28 normotensive DHF patients (p less than 0.005), 33 NC (p less than 0.005) and 11 - NDHF patients (p less than 0.005). However the levels in 28 normotensive DHF patients are not statistically different from the values of 33 NC and 11 - NDHF patients. It is concluded that there is a tendency of excessive PGI2 production in DHF patients during hypotensive crisis.