Hsin-An Kao

Association of CTLA4 gene A–G polymorphism with type 1 diabetes in Chinese children†

The CTLA4 (cytotoxic T lymphocyte associated antigen‐4) gene encodes the T cell receptor involved in the control of T cell proliferation and mediates T cell apoptosis. Thus it is a strong candidate gene for T cell‐mediated autoimmune disease. There is polymorphism at position 49 in exon 1 of the CTLA4 gene, providing a A–G exchange. This polymorphism is reportedly associated with type 1 diabetes in Caucasians but not in a small data set of Chinese. We wished to test this polymorphism in a larger and more homogeneous data set of Chinese children with type 1 diabetes and normal adult controls.

High cytomegalovirus load and prolonged virus excretion in breast milk increase risk for viral acquisition by very low birth weight infants.

Background: Breast milk is the main source of postnatal human cytomegalovirus (HCMV) infection. The aim of this study was to assess the relationship between HCMV load in breast milk and viral transmission to very low birth weight (VLBW) infants. Methods: Breast-fed VLBW infants who were born to HCMV-seropositive mothers and who were managed in a neonatal intensive care unit were enrolled in the study. Blood from mothers and infants was tested for HCMV antibodies after birth. Breast milk was collected for viral culture and HCMV load measurement. Urine from the babies was obtained for HCMV-DNA detection. Symptoms of HCMV infection were recorded and evaluated by neonatologists. Results: Of the 23 evaluated mothers during a 1-year period, 19 were HCMV seropositive; 17 of the women had detectable HCMV-DNA in their breast milk whey. Of the 23 infants born to the 19 seropositive mothers, 8 infants of 8 mothers had HCMV-DNA detected in the urine, indicating that they were infected, even though the breast milk was always frozen prior to feeding. Three infected infants had symptoms. At 4 weeks after delivery, the median viral load in breast milk from mothers of the 8 infected infants was significantly higher than that from mothers of the 15 noninfected infants (P = 0.04). HCMV was detectable in breast milk for a significantly longer period in mothers of infected infants (7.5 vs. 2.6 weeks P = 0.03). Conclusions: High HCMV load and prolonged virus excretion in breast milk are maternal risk factors for viral transmission to VLBW infants.

Outcome of preterm infants with postnatal cytomegalovirus infection via breast milk: A two-year prospective follow-up study

AbstractApproximately 15% of preterm infants may develop postnatal cytomegalovirus (CMV) infection from seropositive mothers via breast milk and are at risk for neurological sequelae in childhood. The aims of this study were to assess the effects and outcomes on growth, neurodevelopmental status, and hearing in very low birth weight (VLBW) premature infants with postnatal CMV infection via breast milk at the corrected age of 12 and 24 months.The prospective follow-up study population comprised all living preterm children (nu200a=u200a55) with a birth weight ⩽1500u200ag and gestational age of ⩽35 weeks, who had been participated in our “postnatal CMV infection via breast milk” studies in 2000 and 2009, respectively. The cohort of children was assessed at 12 and 24 months. Clinical outcomes were documented during hospitalization and after discharge. Long-term outcomes included anthropometry, audiologic tests, gross motor quotient, Infant International Battery, and neurodevelopmental outcomes; all were assessed at postcorrected age in 12 and 24 months during follow-up visits.Of the 55 infants enrolled in the study (4 noninfected infants were excluded because their parents did not join this follow-up program later), 14 infants postnatally acquired CMV infection through breast-feeding (infected group) and were compared with 41 infants without CMV infection (control group). No significant differences were observed between the groups with regard to baseline characteristics, clinical outcomes, anthropometry, or psychomotor and mental development on the Bayley scale of infant development. None of the infants had CMV-related death or permanent sensorineural hearing loss.Transmission of CMV from seropositive mother via breast milk to preterm infants does not appear at this time to have major adverse effects on clinical outcomes, growth, neurodevelopmental status, and hearing function at 12 and 24 months corrected age.

Lack of association of the vascular endothelial growth factor gene polymorphisms with Kawasaki disease in Taiwanese children.

IntroductionKawasaki disease (KD) is an acute febrile vasculitis of unknown etiology that mainly occurs in infants and children. Clinical and histopathologic findings suggest that vascular endothelial growth factor (VEGF) is involved in the coronary artery lesions (CALs) development in KD. This study hypothesized that specific VEGF gene polymorphisms and their haplotypes are associated with KD susceptibility and CAL development in Taiwanese children.Subjects and MethodsThe VEGF −2578 A/C, −634 G/C, and +936 C/T single-nucleotide polymorphisms (SNPs) were genotyped in 156 children with KD and 672 ethnically matched healthy controls using the Pre-Developed TaqMan Allelic Discrimination Assay.ResultsNo significant differences in genotype, allele, carrier, and haplotype frequencies of the three SNPs were found between healthy controls and children with KD or between patients with and without CAL.ConclusionOur data suggest that VEGF −2578 A/C, −634 G/C, and +936 C/T SNPs do not confer increased susceptibility to KD or to CAL development.

Prader-Willi syndrome in Taiwan

Background: Prader–Willi syndrome (PWS) is a congenital disorder caused by absent expression of paternal genes in 15q11‐13 affecting multiple systems. The information concerning the clinical features of this genetic disorder is incomplete in Taiwan.

Small Versus Large Volume Dilute Surfactant Lavage for Meconium Aspiration Syndrome

BACKGROUNDnSurfactant lavage has been used to treat neonatal meconium aspiration syndrome (MAS).nnnOBJECTIVEnTo assess the effects of lavage with a small volume of dilute surfactant in neonates with MAS, and compare the results with those of historical controls treated with larger volumes.nnnMETHODSnFrom August 2002 to June 2005, we treated 11 newborns with MAS using 20 ml of dilute surfactant at a phospholipid concentration of 10 mg/ml (SVL group). We compared the results with those of 9 infants previously treated with large-volume lavage (LVL group), using 40 ml of dilute surfactant, 5 mg/ml.nnnRESULTSnBoth groups were similar at baseline except for a higher mean birth weight in the LVL group (3.29+/-0.36 vs 2.92+/-0.4 kg, P = 0.04). The lavage procedure was longer in the LVL than the SVL group (48.89 +/-7.41 vs. 30.91+/-5.83 mins, P <0.001). Measures of oxygenation, including mean PaO2, oxygenation index, and arterial/alveolar 02 ratio, showed no significant difference between the two groups. Adverse events in the LVL group included transient hypoxemia in 3 infants and white- out on chest x-ray in 5 cases. None of the patients in the SVL group had these findings. The peak mean airway pressure in.the LVL group was higher than that in the SVL group (16.0+/-2. 65 vs 13.3+/-3.01 cmH2O, P = 0.046).nnnCONCLUSIONSnSVL has the same benefits in neonatal MAS as LVL. However, SVL appears to be associated with fewer adverse events.

Thyroid function in children with newly diagnosed type 1 diabetes mellitus

Evaluation of thyroid hormone indices was performed in 138 children with newly diagnosed type 1 diabetes, with their siblings serving as controls. The DKA group consisted of 76 children who had diabetic ketoacidosis (DKA) at initial diagnosis. The non-DKA group consisted of 62 children and the control group of 35. The thyroid function tests of the patients were measured within 3 days of the initial diagnosis of diabetes and at least one follow-up test one month to two years after adequate treatment of diabetes. The DKA group had significantly lower levels of T3, T4, free T4 and FTI than did the other two groups (p < 0.0001, p < 0.0001, p < 0.0001, and p < 0.0001, respectively). T3 concentration was lower in non-DKA subjects than in controls (p = 0.0003), but the two groups did not significantly differ in terms of T4, free T4, and FTI. The TSH level did not differ among the three groups. We conclude that DKA changes thyroid function measurements. In the absence of true thyroid disease, abnormal thyroid function tests are reversible after institution of good diabetic control. We suggest that thyroid function tests should be restricted to those patients suspected of having thyroid disorders at the initial diagnosis of type 1 diabetes.

Final height of children with type 1 diabetes: the effects of age at diagnosis, metabolic control, and parental height.

Normal growth is one of the major goals in the treatment of children with type 1 diabetes. We prospectively monitored the linear growth and metabolic control of 44 children (13 boys) with type 1 diabetes from the time of diagnosis to the attainment of adult height and analyzed the relationship between the height and the age at diagnosis, metabolic control, and genetic target height. At diagnosis, girls at puberty were taller (height in standard deviation score: 0.60 +/- 0.94, p = 0.022), while boys (-0.03 +/- 0.67) and prepubertal girls (0.24 +/- 0.86) were similar to the age-controlled children. During the following years, they lost height compared to their height at diagnosis (p = 0.009), but they still attained an average final height (-0.13 +/- 0.66 in boys, -0.05 +/- 0.86 in girls) correlated with their height at diagnosis (r = 0.37, p = 0.014), as well as their genetic target height (r = 0.78, p < 0.005). The final height as well as the reduction in height was not linearly correlated with the age at diagnosis. The mean HbA1c level of the 44 children was 10.33 +/- 1.74%, boys had better control compared with girls (mean HbA1c 9.45 +/- 1.28 v.s. 10.71 +/- 1.78%, p = 0.013). The final height or the reduction in height was not linearly correlated with the mean HbA1c level.

Continuous Infusion of Vasopressin in Comatose Children with Neurogenic Diabetes Insipidus

Three comatose children with neurogenic diabetes insipidus were treated with intravenous infusion of vasopressin. The infusion of vasopressin was started at a dose of 1.3 to 2.7 mU/kg/h as soon as diabetes insipidus was diagnosed. The effect (urine flow < 2 ml/kg/h with increased specific gravity) was noted in 1 to 6 hours. The infusion rate of vasopressin was adjusted according to urine flow rate which was usually kept around 65 ml/100 kcal metabolized/day. Hypernatremia was corrected 17 to 53 hours after the initiation of infusion of vasopressin. The levels of sodium stayed between 127 and 151 mmol/l during a period of 2.5 to 22 days until the patients death due to the termination of respiratory support or cardiac decompensation. A continuous infusion of vasopressin offered the advantage of rapid onset and termination of effect and therefore could be easily titrated. It seems a rational therapy for comatose children with neurogenic diabetes insipidus.

Bacterial tracheitis in pediatrics: 12 year experience at a medical center in Taiwan

Background:u2002 Bacterial tracheitis may cause life‐threatening airway obstruction.