The effect of 17 years of increased salt iodization on the prevalence and nature of goiter in Croatian schoolchildren.

Background High goiter prevalence caused by iodine deficiency (medium content 5.6 mg potassium iodide [KI]/kg of salt, median urine iodine concentration [UIC] 68 µg/L) in Croatia was observed in 1991 and 1995 when salt was iodized with 10 mg KI/kg. A new regulation introduced in 1996, specified 25 mg KI/kg of salt resulting in an increase of median UIC to 248 µg/L. Afterwards, goiter prevalence was only assessed in two small studies. Methods In this study, we investigated the prevalence and etiology of goiter in 3594 schoolchildren 17 years after an increase in salt iodization in Croatia. Thyroid size was determined by palpation in 1777 girls and 1817 boys aged 10-18 years. In goitrous children, a thyroid ultrasound and thyroid-stimulating hormone, free thyroxine (fT4), free triiodothyronine (fT3), thyroid peroxidase (TPO) and thyroglobulin (TG) antibody measurements were performed. Results Goiter was found in 32 children (0.89% vs. 2.8% in 1991, p<0.00001 and 27% in 1995, p<0.00001), simple goiter (SG) in 18/32 (56%) goitrous children vs. 126/152 (82.8%) in 1991 p<0.00001, autoimmune thyroiditis (AT) in 13/32 (40.6%) vs. 19/152 (12.5%) in 1991 p<0.0009, nodules in four: two cysts, toxic adenoma and carcinoma (in 1991 two adenomas and one cyst), Graves' disease was not found (four in 1991). Subclinical hypothyroidism was found in three children. Thyroid disease was diagnosed in four of 32 children before the investigation. Increased iodine supply decreased goiter prevalence and SG/AT ratio in goitrous patients. Conclusions As thyroid abnormalities were found in 0.89% of children and some required treatment, thyroid examination is important in apparently healthy children regardless of sufficient iodization.

[Surgical treatment of thyroid gland diseases in childhood--our results]. / Kirursko lijecenje bolesti stitne zlijezde u djecjoj dobi--prikaz nasih bolesnika.

Thyroid gland diseases are the most common endocrinopathies in children. Vast majority of these conditions are treated with medical therapy but in individualised cases surgery is indicated. Decision about surgical treatment is made in cooperation of pediatric endocrinologist and surgeon, treatment options depend on the nature of the disease while the extent of surgical procedure is determined by the pathological changes present in the gland. In this paper we represent the results of surgical treatment of 41 children at our department from 1991 to 2009 and current trends in surgical treatement of thyroid gland diseases.

[Low doses of sulphonyluria as a successful replacement for insulin therapy in a patient with neonatal diabetes due to a mutation of KCNJ11 gene encoding Kir6.2]. / Niske doze sulfonilureje kao uspjesna zamjena za inzulin u lijecenju neonatalnog dijabetesa uzrokovanog aktivirajucom mutacijom Kir6.2.

Neonatal diabetes mellitus is a rare metabolic disorder with an estimated incidence of 1:300.000 to 400.000 newborns, and less than 50% of the neonates have permanent neonatal diabetes mellitus (PNDM). Recently, activating mutation in the KCNJ11 gene encoding Kir6.2 subunit of the adenosin triphosphate-sensitive potassium (K(ATP)) channel has been described as the most frequent cause of PNDM. Under physiological circumstances K(ATP) channel closure plays a central role in glucose-stimulated insulin secretion from pancreatic beta cells. Sulphonylurea drugs stimulate insulin secretion by binding to and closing K(ATP) channels and thus bypassing beta cell metabolism stimulate the same chain of reactions as glucose. We describe a boy diagnosed with PNDM at the age of 3 months when insulin therapy was started, and at the age of 4.5 years KCNJ11 gene was sequenced and found that the boy carried a de novo activating R201H mutation. Insulin therapy was successfully switched to low doses of oral glibenclamide. Accordingly, it is important to emphasize that every person diagnosed with diabetes before six months of life, however old they actually are, should be tested for K(ATP) mutations which is offered via the website www.diabetesgenes.org.

[Primary pigmented nodular adrenocortical disease as cause of Cushing's syndrome associated with Carney complex]. / Primarna pigmentna nodularna adrenokortikalna bolest kao uzrok Cushingova sindroma u sklopu Carneyeva kompleksa.

We report a 11-year-old girl and two 14-year-old boys with Cushing's syndrome due to primary pigmented nodular adrenocortical disease (PPNAD). In these patients, hypercortisolism is a consequence of autonomous cortisol secretion from adrenal glands and is ACTH-independent. Besides PPNAD, the girl had lentigines, spotty pigmentation on her bucal mucosa and lips and she also had schwannoma. One of the reported boys had prolactinoma. Considering this, those two patients fulfill the criteria for Carney complex which is a type of multiple endocrine neoplasia syndromes inherited in an autosomal dominant trait. The other boy had PPNAD but no other obvious signs of Carney complex were noticed. Family study didn't reveal any clinical or laboratory signs of Carney complex in our patients' first relatives. All of our patients underwent bilateral adrenalectomy (in one of the boys laparoscopic surgery was performed). Glucocorticoid and mineralocorticoid substitution has been started. Adrenal glands were macroscopically normal but pathohistological analysis confirmed the diagnosis of PPNAD.