The effects of thyroid function on retinopathy of prematurity.

OBJECTIVES: To assess whether TSH and fT4 have a role in the angiogenesis of vaso-obliteration and neovascularization which are the basic pathophysiology of ROP. METHODS: In this retrospective case-control study, the control group (n = 56) included preterm newborns with risk for ROP while the laser group (n = 63) was recruited from cases who developed severe neovascularization and needed laser photocoagulation therapy. Considering the first (vaso-obliteration) and second (neovascularization) phases of the disease, in this study we researched the distribution of thyroid function tests between groups. RESULTS: With regard to the first phase of the disease, TSH and fT4 showed no significant differences between the control and laser groups accordingly (P > 0.05). Likewise, in the second phase of ROP, there was no significant difference between the control and laser groups with respect to TSH and fT4 levels (P > 0.05). CONCLUSION: We found that between the study groups, the levels of thyroid function tests did not have any significant differences, either in the first or the second phases of ROP which are the principal pathophysiology of the disease. Therefore, it was concluded that thyroid hormone values were not informative markers in the course of the disease in preterm babies at risk of developing ROP.

The relation between childhood obesity and adenotonsillar hypertrophy.

Childhood obesity is a common and significant public health problem all over the world. As a well-known fact obese children have an increased risk of obesity-associated comorbidities, including obstructive sleep apnea, diabetes, and cardiovascular disorders at an earlier age compared to their normal weight peers. They also have an increased risk of poor self-esteem, greater body dissatisfaction, and increased peer teasing that lead to a lower health-related quality of life. While the presence of adenoid hypertrophy and increased rate of obstructive sleep apnea frequently co-exists in majority of cases. We have limited knowledge about the effect of adenotonsillar hypertrophy on development of childhood obesity. In this study, we aimed to investigate the association between obesity, presence of adenotonsillar hypertrophy and the quality of life parameters in obese children as measured by the OSA-18 quality of life questionnaire. Fifty obese children aged between 3 and 18 years and 50 age- and gender-matched otherwise children were enrolled to the study. All subjects were routinely examined by the otolaryngologist before enrollment. The size of adenoid hypertrophy was measured using lateral cephalometric radiographs. The tonsils were also graded using the schema recommended by Brodsky et al. We used OSA-18 questionnaires to evaluate the subjects' quality of life issues. We found, 34 % of obese group had tonsillar hypertrophy while the rate was 6 % in control group. Similarly 16 % of obese group had tonsillar hypertrophy compared to only 4 % in non-obese group. It was also noted that total OSA-18 scores of obese group were significantly higher than those of non-obese group. In subgroup analysis of obese group, total OSA-18 score of obese subjects with either adenoid and/or tonsillar hypertrophy was significantly higher than that of obese subjects without adenoid or tonsillar hypertrophy. As the related literature suggests that the impact of adenotonsillar size on OSA symptoms is prominent especially in children under 7 years of age, but its impact on the development of childhood obesity is still controversial. Our results revealed a possible relation between adenotonsillar hypertrophy and obesity rates. Further studies on larger populations should be planned to better define the real impact of adenotonsillar hypertrophy in obese children.

Association between the corrected QT interval, carotid artery intima-media thickness, and hepatic steatosis in obese children.

OBJECTIVE: Childhood obesity is related to subclinical atherosclerosis. Carotid intima-media thickness (CIMT) and hepatosteatosis are parameters that reflect subclinical atherosclerosis and are shown to be associated with obesity. However, their relation with the corrected QT interval (QTc) has not been thoroughly studied in children. Here, we aimed to research the relation between QTc, hepatic steatosis, and CIMT among obese children. METHODS: Fifty-three obese and 53 age- and sex-matched non-obese children aged 6-16 years were included in this prospective cross-sectional study. The QTc of each subject was accordingly obtained from lead II on a 12-lead resting electrocardiogram. Thus, CIMT measurement and abdominal ultrasonographic examination were performed. The data for obese and non-obese children were analyzed and compared. RESULT: The age and gender distribution of the subjects were statistically similar. The CIMT value of the obese group was higher than that of the non-obese group (p<0.001). The obese group had a higher frequency of hepatosteatosis at grade 1 or 2 than the non-obese group (p<0.001). The QTc values were also found to be more prolonged in the obese group than in the other group (p<0.001). With Student's t-test and Mann-Whitey U test accordingly. CONCLUSION: We demonstrated that obese children had higher CIMT and QTc values as well as more frequent hepatosteatosis, and that the presence of hepatosteatosis or increased CIMT had an association with prolonged QTc values in obese children. Therefore, with the aim of detecting cardiovascular effects of obesity, it may be beneficial to perform the measurements of QTc in the presence of hepatosteatosis and/or increased CIMT among obese children.

Vitamin B12, folate and iron levels in primary nocturnal enuresis.

OBJECTIVE: Folate, vitamin B12 and iron are important vitamin and minerals which play role in the development of nervous system. The aim of this study was looking at the presence of folate, vitamin B12 and iron deficiency among patients with Primary nocturnal enuresis (PNE) and possible relation between the delay of central nervous system (CNS) development, PNE and folate, vitamin B12 and iron states. METHODS: Consecutively applied forty patients with PNE (23 girls and 17 boys) and otherwise normal thirty control subjects (17 girls and 13 boys) were included in the study. Average ages (in range) of PNE and the control group were 9.2(6-12) years and 9.3 (6-12) years accordingly. Age, height, weight, complete blood count, blood vitamin B12, folate, ferritin and iron values of both groups were recorded and compared to each other. RESULTS: Average vitamin B12 and folate levels of patients with PNE were significantly and statistically lower compared to those of the control group. Average blood iron of patients with PNE was significantly higher than that of the control group and also average ferritin level of the PNE group was detected to be higher than the control group but this relation was statistically insignificant. CONCLUSION: Primary nocturnal enuresis is related to the delay in CNS maturation so it was thought that low vitamin B12 and folate which were found in patients with PNE may have role in the delay of CNS maturation. Additionally, further studies are needed to investigate the role of vitamin B12 and folate either alone or as combination in treatment of patients with PNE who have low vitamin B12and folate level.

Unusual course of congenital hypothyroidism and route of the L-thyroxine treatment in a preterm newborn.

Congenital hypothyroidism (CH) is the most common endocrine pathology in neonates. Inappropriate treatment of CH is complicated by irreversible brain damage or low IQ score. Hormone replacement therapy with L-thyroxine (L-T4) is sufficient for a very large proportion of patients. However, during treatment, the patient needs to be carefully monitored for presence of factors which might affect the absorption or bio-availability of the drug as well as its dose. Herein, we report a preterm newborn with CH who presented with gastrointestinal problems mimicking necrotizing enterocolitis. The clinical course was also complicated by cholestasis. The L-T4 replacement treatment was switched from oral route to parenteral. After resolution of the cholestasis, L-T4 treatment was continued successfully by the oral route.

Effect of iodine loading on the thyroid hormone level of newborns living in Kayseri province.

INTRODUCTION: Excessive iodine exposure during the fetal and neonatal periods can lead to neonatal hypothyroidism. This study was conducted to evaluate the level of iodine loading among newborns living in Kayseri province. A total of 59 newborns, who were admitted due to disorders in thyroid hormone levels, were included in the study. Materials and METHODS: Among the patients who applied with thyroid hormone dysfunction, newborns with a spot urine iodine level ≥ 20 µg/dL were included in the study between the years 2003 and 2013. Free T3 (fT3), free T4 (fT4), thyroid stimulating hormone (TSH), thyroglobulin (Tg), breast milk iodine, thyroid ultrasonography, and control measurements of fT3, fT4, TSH, and Tg levels were obtained accordingly from both groups of patients who received or did not receive treatment. RESULTS: The average age of the patients was 15 days with a 36/23 girl to boy ratio. Statistically, no significant difference was noticed between all the girls and boys with respect to all the measured values. The etiologic search showed that out of 59 cases, in 18 cases (30.5%) only the mother and in 19 cases only the newborns (32.2%) had a history of povidone iodine exposure; in 8 cases both mothers and their babies had exposure to povidone iodine (13.6%). In 14 cases (23.7%), the source of iodine loading could not be determined. Levothyroxine (L-thyroxine) treatment was initiated in 56% of the patients (n = 33). Out of 33 patients who were under treatment with L-thyroxine, in 13 cases only the mother had history of povidone iodine exposure; in 12 cases, only the baby had a history of povidone iodine exposure; in 1 case, both mother and her baby had a history of povidone iodine exposure, but the etiology could not be found in 7 cases. CONCLUSION: The use of antiseptics-containing iodine for mothers before and after birth and for newborns, especially for umbilical cleansing, can lead to iodine loading and hypothyroidism. If transient hypothyroidism develops within this period, then it may not be detected promptly. This can later lead to retardation in psychomotor development and disorder in learning skills during the childhood period.

The comparison of thyroxine versus thyroxine plus oral iodine in the treatment of congenital hypothyroidism due to iodine deficiency.

AIM: Iodine deficiency is one of the most important causes of congenital hypothyroidism. In addition to thyroid hormone replacement, iodine supplementation is also given to newborns with congenital hypothyroidism due to iodine deficiency. We aimed to determine whether it is beneficial to administer iodine supplementation in addition to the L-thyroxine (L-T4) treatment of newborns with congenital hypothyroidism due to iodine deficiency. MATERIALS AND METHODS: Of 51 newborns, 26 who were diagnosed with congenital hypothyroidism due to iodine deficiency were treated with L-T4. The remaining 25 cases were given L-T4 plus 100 µg/day of oral iodine. Free triiodothyronine (fT3), free thyroxine (fT4), thyroid-stimulating hormone (TSH), thyroglobulin (TG), thyroid volume, urine iodine and breast milk iodine levels were measured in the first and third months of treatment, and the data were compared between the two groups. RESULTS: First- and third-month values of fT3, fT4, TSH, TG and thyroid volume for both groups were statistically similar. There was no significant difference between the two groups in respect to falling levels of fT3 and TSH, the rate of increase of fT4 levels or the shrinkage rate of thyroid volume. CONCLUSION: In this study, the addition of oral iodine to L-T4 treatment provided no benefit compared to treatment with L-T4 alone.

Neonatal diabetes in an infant of diabetic mother: same novel INS missense mutation in the mother and her offspring.

Neonatal diabetes is defined as an uncontrolled hyperglycemic state occurring within the first 6 months of life. It is a rare disease with an incidence of 1 to 90,000-250,000. It is usually a disease of genetic origin in which insulin gene mutations play the main role in the disease process. A baby, born to a mother who had previously been diagnosed with type 1 diabetes mellitus at 14 months of age, had a high blood sugar level within the first few hours after birth and was subsequently diagnosed as having neonatal diabetes mellitus. Baby and mother were identified as having a novel heterozygous insulin missense mutation, p.C109R. Difficulties occurred in both follow-up and feeding of the baby. Without the addition of the mother's milk, an appropriate calorie milk formula and isophane insulin were used for the baby during follow-up. Multiple mechanisms are responsible in the pathogenesis of neonatal diabetes mellitus. Insulin gene mutations are one of the factors in the development of neonatal diabetes mellitus. If a resistant hyperglycemic state persists for a long time among babies, especially in those with intrauterine growth retardation whose mothers are diabetic, the baby concerned should be followed-up carefully for the development of neonatal diabetes mellitus.

Congenital hypothyroidism due to maternal radioactive iodine exposure during pregnancy.

Radioactive iodine (RAI) is used effectively in the treatment of hyperthyroidism and thyroid cancer, but it is contraindicated during pregnancy. RAI treatment during pregnancy can lead to fetal hypothyroidism, mental retardation and increased malignancy risk in the infant. Pregnancy tests must be performed before treatment in all women of reproductive age. However, at times, RAI is being used before ruling out pregnancy. We herein present a male newborn infant with congenital hypothyroidism whose mother was given a three-week course of methimazole therapy for her multiple hyperactive nodules and subsequently received 20 mCi RAI during the 12th week of her pregnancy. The patient was referred to our neonatology unit at age two weeks when his thyrotropin (TSH) level was reported to be high in the neonatal screening test. Physical examination was normal. Laboratory investigations revealed hypothyroidism (free triiodothyronine 1.55 pg/mL, free thyroxine 2.9 pg/mL, TSH 452 mU/L, thyroglobulin 20.1 ng/mL). The thyroid gland could not be visualized by ultrasonography. L-thyroxine treatment was initiated.

Fetal adrenal suppression due to maternal corticosteroid use: case report.

During pregnancy, steroids are usually used in maternal diseases such as adrenal failure or other autoimmune diseases, e.g. idiopathic thrombocytopenic purpura (ITP), Crohn's disease, systemic lupus erythematosus, dermatomyositis, scleroderma, Addison's disease and hyperemesis gravidarum, HELLP syndrome. Endogenous or exogenousmaternal steroids are metabolized by the placental enzyme 11 beta-hydroxy steroid dehydrogenase type 2. Prednisolone and methylprednisolone are highly sensitive to this enzyme, while dexamethasone and betamethasone are less well metabolized. Steroids which can cross the placental barrier are administered in cases like fetal lupus, congenital adrenal hyperplasia and for enhancement of fetal lung maturation, whereas steroids used in maternal diseases are usually the ones with low affinity to the placenta; however, in case of long-term use or in high doses, placental enzyme saturation occurs and thus, resulting in fetal adrenal suppression. Antenatal steroids can lead to low birth weight, as observed in our patient. Here, we report a case with fetal adrenal suppression due to maternal methylprednisolone use presenting with early hypoglycaemia and late hyponatremia in neonatal period and requiring three-month replacement therapy.

Internal iliac vein thrombosis in pediatric Crohn's disease.

Thromboembolic events are one of the important extraintestinal manifestations of inflammatory bowel diseases that are associated with considerable morbidity and mortality. Iliac vein thrombosis is rarely reported in inflammatory bowel diseases. A 9.5 year-old girl was presented with joint pain, nausea, vomiting and weight loss. She was diagnosed with Crohn's disease and right internal iliac vein thrombosis. With the implementation of immunosuppressive and anticoagulant therapies clinical picture has improved and thrombosis has resolved. Timely diagnosis and early treatment of extraintestinal complications of inflammatory bowel diseases might be lifesaving.

Hematopoietic stem cell transplantation from a donor with Klinefelter syndrome for Wiskott-Aldrich syndrome.

WAS is a rare X-linked recessive disorder characterized by primary progressive T cell immunodeficiency, impaired antipolysaccharide antibody response, thrombocytopenia with small platelet, and eczematoid dermatitis. Untreated patients with typical WAS have poor prognosis with the major causes of death being infection, bleeding, lymphoproliferative disorders, and malignancy. Due to the increased risk of infectious and hemorrhagic episodes the best results with HSCT are achieved in patients less than five yr of age and are recommended as early as possible. Here, we report a three-yr-old boy with WAS who underwent UCB and BMT from his genotypically identical brother with Klinefelter syndrome.