Molecular analysis of mucopolysaccharidosis type VI in Iranian patients; the influence of founder effect and consanguinity.

Background: Mucopolysaccharidosis type VI (MPS VI), also known as Manteaux-Lamy syndrome, is an autosomal recessive lysosomal storage disorder caused by deficiency of the enzyme arylsulfatase B(ARSB). This syndrome is progressive and affects many tissues and organs, leading to inflammation and scarring. The classic clinical features of Maroteaux-Lamy syndrome are significant impairment of the osteoarticular system with dysostosis multiplex, short stature and motor dysfunction.

Diabetic Ketoacidosis in Children Before and During COVID-19 Pandemic: A Cross-sectional Study.

Background: Coronavirus disease 2019 (COVID-19) has spread quickly. Comorbidities, such as diabetes, have been determined as critical risk factors for COVID-19.

Objectives: This study aimed to determine the frequency and severity of diabetic ketoacidosis (DKA) in children before and during the COVID-19 pandemic.

Mutational and bioinformatics analysis of the NKX2.1 gene in a cohort of Iranian pediatric patients with congenital hypothyroidism (CH).

Congenital hypothyroidism (CH) occurs with a relatively alarming prevalence in infants, and if not diagnosed and treated in time, it can have devastating consequences for the development of the nervous system. CH is associated with genetic changes in several genes that encode transcription factors responsible for thyroid development, including mutations in the NK2 homeobox 1 (NKX2.1) gene, which encodes the thyroid transcription factor-1 (TTF-1).

New onset of diabetes in a child infected with COVID-19: a case report.

Background: Diabetic ketoacidosis (DKA) is a serious complication of type 1 diabetes. A few studies have reported that COVID-19 is associated with the development of new-onset diabetes. Here, we present an infected child with new onset diabetes leading to DKA.

The prevalence of zinc deficiency in morbidly obese patients before and after different types of bariatric surgery.

Background: The prevalence of obesity is considered to be increased worldwide. Lack of mineral elements is one of the essential side effects of bariatric surgery as a trending treatment for obesity. We aimed to assess zinc deficiency among morbidly obese patients before and following different types of bariatric surgical procedures.

The Effect of Synbiotic Supplementation on Growth Parameters in Mild to Moderate FTT Children Aged 2-5 Years.

Synbiotic (probiotic bacteria and prebiotic) has beneficial effects on the gastrointestinal tract. This study was designed to investigate the effect of synbiotic supplementation on the growth of mild to moderate failure to thrive (FTT) children. A randomized, triple-blind, placebo-controlled trial was conducted involving 80 children aged 2-5 years with mild to moderate FTT, who were assigned at random to receive synbiotic supplementation (10 colony-forming units) or placebo for 30 days.

Mutation analysis of the NKX2.5 gene in Iranian pediatric patients with congenital hypothyroidism.

Background: The embryonic development of the thyroid gland is regulated by the expression of several candidate genes which are related to congenital hypothyroidism. These genes include the numerous critical thyroid transcription factors such as NKX2.1, NKX2.

Identification and biochemical characterization of a new antibacterial and antifungal peptide derived from the insect Sphodromantis viridis.

Antimicrobial peptides are members of the immune system that protect the host from infection. In this study, a potent and structurally novel antimicrobial peptide was isolated and characterized from praying mantis Sphodromantis viridis. This 14-amino acid peptide was purified by RP-HPLC.

Cognitive outcomes for congenital hypothyroid and healthy children: a comparative study.

Objective: Early diagnosis and treatment of congenital hypothyroidism (CH) and the prevention of developmental retardation is the main goal of public health national screening programs. This study compares the cognitive ability of children with CH diagnosed by neonatal screening with a healthy control group (2007) in Yazd, Iran.

Materials & Methods: In a case-controlled study, the intelligent quotient (IQ) of 40 five-year-old children with early treated CH and good compliance were evaluated by the Wechsler preschool and primary scale of intelligent test and compared to 40 healthy age and gender matched children as controls.

Effect of vitamin D on HbA1c levels of children and adolescents with diabetes mellitus type 1.

Background: Studies show the relationship between vitamin D deficiency and glucose metabolism disorders. The aim of this study was the evaluation of the effect of vitamin D administration in management of diabetes mellitus type 1 (T1D).

Methods: We evaluated the effect of supplementation with vitamin D on HbA1c levels of children and adolescents with T1D.

Prevalence of Permanent Congenital Hypothyroidism in Children in Yazd, Central Iran.

Background: Congenital hypothyroidism is a condition of thyroid hormone deficiency. Approximately 1 in 4000 newborn infants has a deficiency of thyroid function. The aim of this study is determination of the prevalence of permanent and transient congenital hypothyroidism (CH) in Yazd, Iran.

Efficacy of levothyroxine in migraine headaches in children with subclinical hypothyroidism.

Objective: Hypothyroidism may be an exacerbating factor for primary headaches and migraine is one of the most common primary headaches in childhood. The purpose of this study was to evaluate the effect of treatment of subclinical hypothyroidism on children with migraine headache.

Materials & Methods: In a quasi-experimental study, the severity and monthly frequency of headache of 25 migraineur children with subclinical hypothyroidism who were referred to the pediatric neurology clinic of Shahid Sadoughi University of Medical Sciences,Yazd, Iran between January 2010 and February 2011 and were treated with levothyroxine for two months were evaluated.