High frequency of transient congenital hypothyroidism among infants referred for suspected congenital hypothyroidism from the Turkish National screening program: thyroxine dose may guide the prediction of transients.

PURPOSE: We aimed to determine the frequency of transient congenital hypothyroidism (TCH) in 17 participating centers in Türkiye, evaluate the etiological distribution in permanent congenital hypothyroidism (PCH) cases, and investigate the role of laboratory and clinical findings in predicting TCH. METHODS: This retrospective observational multicenter study included patients from 17 pediatric endocrinology centers identified by "National Newborn Screening Program" (NNSP) who were born in 2015 and followed for 6 years. Demographic, clinical, and laboratory information of the cases were compiled through the database http://cedd.saglik-network.org (CEDD-NET). RESULTS: Of the 239 cases initially treated for CH, 128 (53.6%) were determined as transient in whom a trial of levothyroxine (LT4) withdrawal was performed at a median age of 36 (34-38) months. Among the patients with PCH (n = 111), thyroid dysgenesis was diagnosed in 39.6% (n = 44). The predictive factors for TCH were: LT4 dose at the withdrawal of treatment, and initial newborn blood screening (NBS)-TSH level. Based on the receiver operating characteristic (ROC) curve analysis to predict optimal cut-offs for TCH predictors, LT4 dose < 2.0 µg/kg/day at treatment discontinuation was predictive for TCH and was associated with 94.5% specificity and 55.7% sensitivity, with an area under the curve (AUC) of 0.802. The initial NBS-TSH level value < 45 µIU/mL was predictive for TCH with 93.1% specificity and 45.5% sensitivity, with an AUC of 0.641. In patients with eutopic thyroid gland only LT4 dose < 1.1 µg/kg/day at withdrawal time was predictive for TCH with 84.7% sensitivity and 40.4% specificity, with an AUC of 0.750. CONCLUSION: According to our national follow-up data, the frequency of TCH was 53.6%. We determined the LT4 dose < 2.0 µg/kg/day at discontinuation of treatment and the initial NBS-TSH level < 45 µIU/mL as the best cut-off limits to predict TCH.

A CHILD WITH LARON SYNDROME ASSOCIATED WITH VASCULITIS.

BACKGROUND AND OBJECTIVES: Levels of insulin-like growth factor-I are characteristically low in Laron syndrome which is a factor that has important roles on vascular health and development. Congenital insulin-like growth factor-I deficiency was reported to be associated with some vascular disorders. However, vasculitis diseases and Laron Syndrome association has not been reported in English literature up to date. PATIENT: We report the case of a two and a half years old Turkish girl, who was diagnosed as Laron syndrome when she was 12 months old. She presented with acute vasculitis lesions. Her physical examination and laboratory studies did not reveal a specific infectious agent or also an autoimmune disease was not detected. Her lesions disappeared during hospitalization without a complication. CONCLUSION: Since insulin-like growth factor-I reduces endothelial cell oxidative stress and maintains the structural integrity of vessels, some common mechanisms might be responsible for the occurrence of vasculitis in this patient with Laron syndome. The role of insulin-like growth factor-I and recombinant human insulin-like growth factor-I treatment choice in vasculitis diseases is a matter of investigation.

Utility of ApoB/ApoA1 ratio for the prediction of cardiovascular risk in children with metabolic syndrome.

OBJECTIVE: To assess whether apoB/apoA1 ratio is associated or not with metabolic syndrome in obese children. METHODS: A 198 obese children and 41 healthy control subjects were enrolled in a cross-sectional study. The apoB/apoA1 ratio and other metabolic syndrome components in obese children with/without metabolic syndrome were compared to healthy controls. RESULTS: The apoA1 level did not show significant difference (p = 0.664) but apoB level (p = 0.000) and apoB/apoA1 ratio (p = 0.001) were significantly higher in obese group than in control group. Also, the apoB/apoA1 ratio was significantly higher in obese children with metabolic syndrome when compared to obese children without metabolic syndrome (p = 0.007) and showed positive correlation with triglyceride (r = 0.404, p = 0.000) and negative correlation with high-density lipoprotein cholesterol (r = -0.593, p = 0.000). CONCLUSIONS: The apoB/apoA1 ratio is associated with metabolic syndrome in obese children. An elevated apoB/apoA1 ratio may constitute an important feature of the metabolic syndrome. There is a need for long term follow-up studies concerning cardiovascular risk in obese children with metabolic syndrome and high apoB/apoA1 ratio.