Association Between Dietary Phytochemical Index and Neonatal Thyroid Function.

Background: Thyroid hormones regulate fetal growth and differentiation of several tissues. Maternal dietary patterns may be correlated with changes in the level of neonatal thyroid-stimulating hormone (TSH). We hypothesized that since maternal nutrition affects birth weight and offspring growth, it may also impact endocrine patterns in offspring. This study is aimed at assessing the relationship between maternal dietary phytochemical index (DPI) in the first trimester of pregnancy and neonatal cord blood thyroid hormone levels. Methods: This cross-sectional study is a substudy of a birth cohort. Overall, 216 mothers, aged 16-45 years, were recruited in their first trimester of pregnancy. To calculate DPI, the daily energy percentage of phytochemical-rich foods was divided by the total daily energy intake. At delivery time, TSH and free thyroxine (FT4) levels were measured in cord blood samples using chemiluminescence immunoassay. Results: The mean (standard deviation (SD)) age of mothers was 29.56 (5.50) years, and 47% of newborns were girls. The mean (SD) of DPI in the first, second, third, and fourth quartiles was 25.03 ± 4.67, 33.87 ± 2.18, 40.64 ± 2.10, and 51.17 ± 4.98, respectively. There was not any significant correlation between DPI score with cord serum TSH and FT4 levels in crude and adjusted analysis. Conclusion: No significant relationship between maternal DPI with cord serum TSH and FT4 levels was shown. Limited experience exists about the effect of maternal diet quality indices on neonatal thyroid function, and further studies are needed in this regard.

Evaluation of cardiac findings in mucopolysaccharidosis.

PURPOSE: Mucopolysaccharidoses (MPS) are a group of rare genetic diseases and heart involvement is one of the important conflicts in most types, which may cause serious complications. We used M-Mode and two-dimensional speckle tracking echocardiography (2D-STE) to explore cardiovascular involvements in MPS patients. METHOD: The present cross-sectional study investigated the frequency of cardiac involvements in MPS patients. Included participants were MPS types I, II, III, IV, and VI who underwent specialized echocardiography exams to assess valvular function, systolic and diastolic function, left ventricular ejection fraction (LVEF), and global longitudinal strain (GLS). RESULTS: 35 patients were enrolled in this study. The total mean age of patients was 9.58 ± 5.11 years and 71.4% were male. Type IV (40%) and type III (31.4%) were the most frequent MPS. Although LVEF did not differ notably among MPS types, GLS was significantly different (p = 0.029). Mitral regurgitation was observed remarkably more in MPS type III (p = 0.001) while mitral stenosis was more common in type III (p = 0.007). There was a significant association between LVEF and GLS (ß= -0.662; p = 0.025) and between LVEF and MPS type (ß = 1.82; p = 0.025) when adjusted for GLS. CONCLUSION: Cardiac complications are very common and are one of the most important causes of death in MPS patients. 2D-STE seems to be superior to M-Mode for detection of early and subclinical cardiac dysfunction in MPS patients.

Predicting childhood overweight status by accelerated weight gain from neonatal period to infancy.

Background: The increased prevalence of obesity in early childhood is a public health problem. Childhood obesity may affect cardiorespiratory fitness and can induce obesity and its comorbidities in adulthood. We aimed to assess childhood overweight status by accelerated weight gain during infancy. Materials and Methods: This is a historical cohort that was conducted on 637 7-year-old students of Guilan province, north of Iran. Data were collected, including demographic characteristics, weight at 4, 6, 12, and 18 months, and clinical examination. The ROC curve was designated based on the standardized z-scores, and the most appropriate cutoff point by sensitivity and specificity was noted for predicting obesity at 7 years. Rapid weight gain (RWG) was also assessed. Results: Among participants, 334 (53.3%) were female. In this study, the mean and standard deviation of RWG in 0-4 months, 0-6 months, 0-12 months, and 0-18 months were 3.50 ± 0.89, 4.64 ± 1.02, 6.54 ± 1.21, and 8.00 ± 1.46 kg, respectively. The highest AUC was dedicated to 0-18 months (0.7 ± 0.05) and the suitable cut-off for RWG in this interval was 8.55 kg with 65.5% and 72.0% sensitivity and specificity, respectively. Conclusion: Although in the previous investigations, the changes in the first 3 years of life had a significant role in further complications, regarding our results, it seems that even earlier consideration of excess weight gain may be necessary.

Trends in incidence rates of childhood type 1 diabetes mellitus: A retrospective study in Isfahan province, Iran.

AIMS/INTRODUCTION: We aimed to determine the incidence trend of childhood type 1 diabetes mellitus in Isfahan province over a period of 12 years. MATERIALS AND METHODS: In this retrospective study, children aged <20 years at the time of type 1 diabetes mellitus diagnosis, from March 2007 to March 2019, were included. The crude and adjusted incidence rate of type 1 diabetes mellitus is calculated as the number of cases per 100,000 person-years by the period. The cumulative, age- and sex-specific incidence rates were also calculated. Age-specific incidence rates were calculated for age and sex groups. RESULTS: A total of 1,954 (983 boys and 971 girls) cases of type 1 diabetes mellitus were identified. The mean age at diagnosis in all studied populations was 9.89 (standard deviation 4.76). There were no significant differences between the proportion of boys and girls in different years (P = 0.12) and different age groups (P = 0.19). The average annual percent change of incidence rate for the total population, for girls and boys, was 6.9%, 6.7% and 6.3% respectively. The type 1 diabetes mellitus incidence rate had a significant trend to be increased from 2007 to 2019 (P < 0.001, t = 3.6). CONCLUSION: Our findings showed that currently our region is considered a region with a high incidence rate of type 1 diabetes mellitus. Although we have had fluctuations in the incidence rate over the 12 years, the overall trend is increasing.

Evaluation of Growth Hormone Deficiency in Children with Cystic Fibrosis.

Background: Due to chronic respiratory and gastrointestinal problems, growth failure is a common issue in patients with cystic fibrosis (CF). The present study aimed to investigate the prevalence of growth hormone deficiency (GHD) in CF children with stable gastrointestinal and respiratory conditions. Materials and Methods: In this study, the growth indicators of all 4-16-year-old children referred to two CF clinics were monitored over 3 years. Children without severe gastrointestinal or pulmonary symptoms with weight <3% percentile or whose height increase were two standard deviations below their expected height growth over 6 months were selected for the growth hormone (GH) stimulation test by clonidine and L-dopa test. Some of the children without CF, who were also referred for height growth disorders and matched the CF group, were considered the control group. They underwent the GH stimulation test, and the results were compared. Results: From 150 patients with CF, growth failure was observed in 24 patients with stable gastrointestinal and respiratory conditions; in 10 of them, the GH stimulation test was deficient. The prevalence of GHD was 6.6% in CF patients. In the control group of 30 children without CF, but with growth failure, the GH was deficient in nine cases, implying no significant difference with the case group (P = 0.37). Conclusion: In our study, the prevalence of GHD was 6.6% in CF patients, whereas the prevalence GHD in the normal population of childhood is <1%. Therefore, further studies should be designed to investigate the cause of GHD in CF patients.

Prevalence of Nonalcoholic Fatty Liver Disease in Children with Renal Failure Underwent Treatment with Dialysis.

Background: In this study, we aimed to investigate the prevalence of nonalcoholic fatty liver disease (NAFLD) in children with renal failure under treatment with dialysis and its association with biochemical measurements. Methods: In this cross-sectional study, children aged less than 18 years with chronic kidney disease (CKD) who were under treatment with dialysis at least 3 months ago were enrolled. To evaluate fatty liver in those patients who had no recent liver ultrasonography (last 6 months), liver ultrasonography was performed. The characteristics of patients with renal failure with and without NAFLD based on the ultrasonographic evaluation were compared. The association between NAFLD and the studied variables was evaluated. Results: In this study, 39 children (31 males and 8 females) with renal failure who underwent treatment with dialysis were included. From the studied population, six (19.4%) had NAFLD based on ultrasonographic evaluation. There were no differences between renal failure patients with and without NAFLD regarding the biochemical and anthropometric characteristics (P > 0.0). Conclusions: The prevalence of NAFLD in our studied children with renal failure who underwent treatment with dialysis was like the general population and it was not associated with the biochemical and anthropometric characteristics of the patients. Given the importance of NAFLD in renal failure patients as well as its subtle nature, it is recommended to screen patients with CKD for NAFLD.

Prevalence of nephrocalcinosis in children with congenital adrenal hyperplasia.

Background: We aimed to investigate the prevalence of nephrocalcinosis (NC) among children with diagnosed congenital adrenal hyperplasia (CAH). Our findings would be helpful for earlier diagnosis, management, and prevention of NC-related complications. Materials and Methods: In this cross-sectional study, children with CAH, aged <18 years old who were regularly referred for follow-up, were included. The information of the patients was extracted from their medical files, and they underwent renal ultrasonography for evaluation of the presence of NC. Results: From 120 studied patients with CAH, four patients (3.3%) had NC. The prevalence of NC was higher in males than females (P = 0.05). Mean age and age of CAH diagnosis had a trend to be lower in CAH patients with NC than those without. Regression analysis indicated significant association between NC and sex (P = 0.027, r = 2.24). Conclusion: The results of this study indicated a 3.3% prevalence rate of NC for children with CAH. NC had a trend to be more prevalent in male children with CAH. Though it was not significantly different but given that the mean age and age at diagnosis of CAH in children with CAH and NC was lower that CAH patients without NC, it is suggested that in patients with CAH and NC other factors such as genetic background or unknown disease related factors are associated with hypercalcemia and NC.

Effect of group hope therapy on self-efficacy of adolescents with type 1 diabetes.

OBJECTIVE: This study aims to determine the effect of group hope therapy on the self-efficacy of adolescents with type 1 diabetes. METHODS: This randomized clinical trial was conducted on 45 adolescent patients with type 1 diabetes in Iran. The subjects were randomly assigned into the intervention and control groups. The intervention group received eight 90-min sessions of group hope therapy using Snyder's method. RESULTS: The mean score of self-efficacies in the intervention group was significantly higher than the control group (p<0.05). CONCLUSION: The results of the study suggested that group hope therapy had a significant impact on self-efficacy of adolescents with type 1 diabetes but had no effect on academic self-efficacy and self-efficacy in blood glucose management.

Cardiovascular Risk Factors in Children and Adolescents with Congenital Adrenal Hyperplasia.

BACKGROUND: Congenital adrenal hyperplasia (CAH) is an autosomal recessive disorder caused by impaired steroidogenesis. Glucocorticoid treatment with increased androgens may lead to cardiovascular and metabolic effects in these patients. In this study, we investigated the relationship between cardiovascular risk factors and androgen levels in children and adolescents with CAH due to 21 hydroxylase deficiency. MATERIALS AND METHODS: A cross-sectional study of 78 patients (37 boys and 41 girls) with CAH aged 3-17 years. Anthropometric, body mass index (BMI), systolic (SBP), and diastolic (DBP) blood pressure were measured. Fasting blood glucose with plasma insulin and lipids were measured, and insulin resistance (HOMA-IR) calculated using the homeostasis assessment model. Furthermore, testosterone, Dehydroepiandrosterone sulfate (DHEAS), and 17-Hydroxyprogesterone (17OHP) were investigated. RESULTS: The mean SBP and DBP were 112.01 ± 19.13 and 69.77 ± 7.56, respectively. The mean of HOMA-IR in patients was 2.25 ± 1.46. The frequency of patients with overweight and High HOMA index were, respectively, 33.3% and 29.3%. The correlation analysis between clinical characteristics and androgen serum levels showed that DBP and BMI had a significant positive correlation with 17OHP. The median regression analysis showed, only DBP in the adjusted model had a significant positive effect with 17OHP level (P < 0.05), and no significant relationship was observed for other characteristics. CONCLUSION: A significant association was found between BMI and DBP with serum concentrations of 17-OHP, suggesting that elevated 17-OHP can lead to an increased risk of cardiovascular disorders in children and adolescents with CAH.

Correlation between Stable Hyperglycemia and Mortality in Children Admitted to the Pediatric Intensive Care Unit of Imam Hossein Hospital.

BACKGROUND: Stress-induced hyperglycemia is an important issue among pediatrics admitted in the pediatric intensive care unit (PICU). Former studies have declared that hyperglycemia has a high prevalence rate and could increase the risks of mortality among pediatrics. Here, we aimed to investigate the prevalence rate of hyperglycemia and its effects on mortality among pediatrics in the PICU of the hospital. MATERIALS AND METHODS: This cross-sectional study was performed in 2018-2019 on 88 patients admitted in PICU. Data regarding blood sugar (BS) and other clinical and laboratory parameters were collected. Hyperglycemia was accounted for as BS of >126 mg/dl. Hyperglycemia was divided into: mild (126 200). The pediatric risk of mortality (PRISM) score was also calculated for each patient during the first 24 h. RESULTS: Thirty patients (34.1%) had persistent hyperglycemia and 58 patients (65.9%) had normal glycemic indexes. Eleven patients (12.5%) had mild, 9 patients (10.2%) had moderate, and 10 patients (11.4%) had severe hyperglycemia. The prevalence of mortality was 5.7% among hyperglycemic patients and 6.8% among normal glycemic pediatrics. There were no statistically significant differences regarding mortality rate (P = 0.499). The mean PRISM score for normal glycemic patients was 7.03 ± 5.18 and for patients with hyperglycemia was 7.36 ± 6.37. CONCLUSION: Hyperglycemia has no significant effects on mortality and PRISM score of pediatrics in PICU, despite of the previous studies. The frequency of hyperglycemia was also 5.7% among the patients admitted in PICU.

The impact of poor glycemic control on lipid profile variables in children with type 1 diabetes mellitus.

BACKGROUND: Type 1 diabetes mellitus (T1DM) and its related comorbidities are considered an important health issue. This study aimed to evaluate the impact of glycemic control on lipid profile variables in children with T1DM. MATERIALS AND METHODS: This study included 274 children (≤19 years of age) with T1DM who had referred to the outpatient clinics of endocrinology in Emam-Hossein Hospital of Isfahan, Iran. Based on American Diabetes Association criteria, patients were divided into two groups including optimal glycemic control (OGC) and poor glycemic control (PGC). Mean lipid level and frequencies of lipid profile abnormalities between the two studied groups were compared. RESULTS: Mean age of the studied population was 13 ± 5.9 years and 133 (48.5%) were boys. A total of 162 (59.1%) and 112 (40.9%) patients had PGC and OGC, respectively. Hypercholesterolemia was the most common dyslipidemia in both groups (33 [29.1%] of OGC and 63 [39.1%] of PGC patients). The frequency of high low-density lipoprotein (LDL) was significantly higher in patients with PGC than those with OCG (P = 0.007). The frequencies of hypercholesterolemia, hypertriglyceridemia, and low levels of high-density lipoprotein were also higher in PGC group, but did not reach the significant threshold. CONCLUSION: It is suggested that glycemic control is in association with lipid profile abnormality in patients with T1DM. High LDL was significantly more frequent in patients with PGC than those with OGC. It is recommended to investigate the role of glycemic control on other cardiometabolic risk factors of T1DM patients. Our findings could be used for planning preventative strategies for reducing T1DM-related cardiovascular disease.

SOFT Syndrome: The First Case in Iran.

Primordial dwarfism (PD) is a group of rare genetically heterogeneous disorders consisted of disorders with intrauterine growth retardation continued through the life. SOFT syndrome with characteristics of short stature, onychodysplasia, facial dysmorphism, and hypotrichosis has been presented as a subtype of PD. Only 20 cases of SOFT syndrome have been reported in world to date, but none of them were not in Iran. Our case was 6.5-year-old girl with a complaint of growth retardation including height of 97 cm (Z = -4.6 standard deviation [SD]) and weight of 14 kg (Z = -4 SD) referred to growth clinic. She had a prominent forehead, triangular face, short limbs, malformed nails, and crowded teeth and her psychomotor function was normal. Laboratory and karyotype tests were normal while she was homozygous for c.G491A mutation of POC1A gene thus SOFT syndrome diagnosis was confirmed for her and recombinant growth hormone therapy was discontinued.

Twin Brothers with Carnitine Membrane Transporter Deficiency: A Case Study.

Carnitine membrane transporter deficiency or primary carnitine deficiency (PCD) is an autosomal recessive disorder of fatty acid oxidation, in which the transport of carnitine into cells is impaired. Carnitine plays an important role in transporting fatty acids into the mitochondria and carnitine deficiency block oxidation of long-chain fatty acids in the mitochondria that leads to heart and hepatic disease, myopathy, nonketotic hypoglycemia, and neurological complications. PCD has a wide range of symptoms and can reveal itself as symptomatic cardiomyopathy or even asymptomatic. In this study, we reported twin brothers with PCD. One of them had symptoms of disease and cardiomyopathy and was under treatment with carnitine. Another twin was asymptomatic and was diagnosed during follow-up period of his brother.

Thrombotic Thrombocytopenic Purpura in a Child with Diabetic Ketoacidosis.

Thrombotic thrombocytopenic purpura (TTP) secondary to diabetic ketoacidosis has been rarely reported and is considered as a rare complication. If left untreated, this condition could be life threatening with considerable morbidity and mortality. Herein, we report a 6-year-old girl with reduced consciousness and respiratory distress with a history of polydipsia and polyuria in the 2 weeks before hospitalization. The patient was initially diagnosed as diabetic ketoacidosis based on clinical and laboratory findings and treated accordingly. After treatment and during hospitalization although she had gained relative consciousness, she experienced seizure and reduced consciousness again. Considering laboratory and clinical findings and the patient's underlying conditions (thrombocytopenia, renal failure, and high lactate dehydrogenase), TTP was suspected although ADAMTS13 test could not be done. Treatment with plasmapheresis was initiated, and after 48 h, the patient was conscious, and laboratory indices became normal within a few days. The patient was discharged after full recovery. TTP should be considered as a rare complication of diabetic ketoacidosis in patients with thrombocytopenia, renal failure, and reduced consciousness and should be immediately treated.

A Case of Tyrosinemia Type III with Status Epilepticus and Mental Retardation.

Tyrosinemia type III is an autosomal recessive disorder caused by the deficiency of 4- hydroxyphenylpyruvate dioxygenase (4-HPPD). It is characterized by elevated levels of blood tyrosine and massive excretion of its derivatives into the urine. Clinical findings of tyrosinemia type III include neurological symptoms and mental retardation. Only a few patients presenting with this disease have been described, and the clinical phenotype remains variable and unclear. We present a case, who was admitted to the hospital at the age of 4 months for recurrent seizures. Two months later, she was admitted again with status epilepticus. Laboratory data showed increased level of tyrosine in the blood. She was treated with a diet low in tyrosine and phenylalanine and anamix formula that leading to catch-up growth and improvement of her symptoms. Plasma tyrosine level dropped to normal values. In any child who presents with the neurologic symptom, some rare diagnosis like tyrosinemia type III should be considered.

Homocystinuria with Stroke and Positive Familial History.

Homocystinuria is the second most common treatable aminoacidopathy. Clinically, affected patients present with eye, skeleton, central nervous system, and most importantly, vascular system abnormalities. This autosomal recessive disorder leads to accumulation of homocysteine and its metabolites in the blood and urine. In this report, we present a case with clinical and biochemical findings of homocystinuria with stroke and a positive familial history of the disease in her brother. A 4-year-old girl was admitted to pediatric emergency ward because of acute onset of right hemiparesis and subsequent generalized tonic-clonic seizures. Cranial magnetic resonance imaging revealed acute infarct areas in the left cerebral hemisphere. Metabolic screening revealed elevated concentrations of serum homocysteine and methionine and a normal serum concentration of vitamin B12. These findings, along with a positive familial history led to the diagnosis of homocystinuria. In any child who presents with stroke, some rare condition such as homocystinuria should be considered in diagnosis.

Demographic and Clinical Findings in Pediatric Patients Affected by Organic Acidemia.

OBJECTIVE: Metabolic disorders, which involve many different organs, can be ascribed to enzyme deficiency or dysfunction and manifest with a wide range of clinical symptoms. This study evaluated some of the demographic and clinical findings in pediatric patients affected by organic acidemia. MATERIALS & METHODS: This cross-sectional study was part of a larger study conducted in patients with metabolic disorders during a period of 7 years from 2007 to 2014 in Isfahan Province, Iran. Our study covered a wide range of cases from newborn infants (one-week old) to adolescents (children up to the age of 17 years). This study evaluated patients' demographic information, history of disease, developmental and educational status, clinical and general conditions. Phone and in-person interviews were used to gather information. RESULTS: Out of 5100 patients screened in this study, 392 patients were affected by one of the different metabolic disorders and 167 individuals were diagnosed as organic acidemia. Propionic acidemia/methyl malonic acidemia (PA/MMA) was the most prevalent form of this metabolic disorder. The frequency of consanguinity was 84.7% in the group of patients. The mortality rate was 18.8% in patients with organic academia. CONCLUSION: Each of the metabolic diseases, as a separate entity, is rare; nevertheless, in aggregate they have a somewhat high overall prevalence. These diseases result in mental and developmental disorders in the absence of quick diagnosis and initiation of treatment. Furthermore, more mutations should be identified in societies affected by consanguinity. Further research should also be conducted to determine worthwhile and more-efficient screening methods as well as long term neurological prognosis.

Effect of long-term Vitamin C intake on vascular endothelial function in diabetic children and adolescents: A pilot study.

BACKGROUND: This study attempted to determine the effects of long-term use of Vitamin C on vascular endothelial function. MATERIALS AND METHODS: During a pilot clinical trial study conducted at Imam Hussein Hospital (Isfahan) in 2014-2015, a total of forty diabetic patients were selected and then assigned randomly into two twenty-subject groups receiving Vitamin C and placebo tablets. The patients were treated with Vitamin C or placebo for 6 months. All patients were examined through echocardiography in terms of cardiac function before and after treatment. To evaluate the endothelial function (flow-mediated dilatation [FMD], intima-media thickness), they underwent arterial Doppler. Moreover, the chemical indices of vascular function were tested through intercellular adhesion molecule and vascular cell adhesion molecule (VCAM). Finally, the results were compared between the two groups. RESULTS: Based on the results, the mean left ventricular mass significantly reduced after the intervention in the group treated with Vitamin C (from 76.35 ± 25.6-68.62 ± 22.66; P = 0.015) while there was no significant difference observed in the control group (from 67.58 ± 25.38-71.63 ± 26.84; P = 0.19) but no statistically difference between the two groups-based repeated measures ANOVA test (P = 0.6). In addition, the mean of VCAM changes was significantly difference between the two groups (P < 0.001). CONCLUSION: Long-term use of Vitamin C in diabetic patients can improve certain echocardiographic parameters such as ejection fraction, fractional shortening, and FMD, which in turn enhances vascular endothelial function.

The role of maternal thyroid stimulating hormone receptor blocking antibodies in the etiology of congenital hypothyroidism in isfahan, iran.

BACKGROUND: Considering the role of maternal thyroid stimulating hormone (TSH) receptor blocking antibody (TRAb) in the etiology of congenital hypothyroidism (CH), this study aimed to determine TRAb among patients with CH in Isfahan, Iran. METHODS: In this case-control study, patients with CH and their mothers were compared with a group of healthy neonates and their mothers. Venous blood samples were obtained for measurement of TRAb using enzyme-linked immunosorbent assay (ELISA) method among mothers and their neonates. TSH of mothers was also determined. RESULTS: The case group consisted of 65 patients with CH and their mothers; controls were 148 healthy neonates and their mothers. The prevalence of positive TRAb in patients with CH and their mothers was higher than in the control group (81.5% vs. 1.3% in mothers and 80% vs. 0% in neonates, respectively, P<0.05). The relationship between the TRAb and occurrence of CH was significant (P<0.05), whereas the corresponding figure was not significant for TRAb and the level of maternal and neonatal TSH in case and control groups (P>0.05). CONCLUSION: It seems that autoimmunity has an important role in the etiology of CH. Further studies are necessary to determine other autoantibodies in CH patients.