Endocrine Disorders in Pediatric and Adolescent Liver-transplant Recipients.

Background: Particular requirements of pediatric and adolescent liver-transplant (LT) recipients necessitate the evaluation of such population from the endocrine viewpoint.

Objective: To determine the endocrine disorders among LT recipients.

Methods: 129 LT recipients younger than 18 years, and at least 6 months post-LT with no pervious history of endocrine disorders were included in the study.

The frequency of hypothyroidism and its relationship with HCV positivity in patients with thalassemia major in southern Iran.

Introduction: Hypothyroidism is one the most complication due to iron overload in patients with β-thalassemia major (TM). On the other hand these patients are prone to Hepatitis C virus (HCV) infection that can cause  thyroid dysfunction by itself or as the side effect of treatment with interferon (INF) or IFN plus ribavirin. The aim of this study is to evaluate the association of hypothyroidism with HCV positivity and serum ferritin levels in patients with TM.

Prevalence of Postoperation Metabolic Syndrome in Pediatric Liver Transplant Patients: A Single Center Experience.

Objectives: Metabolic syndrome components, such as being overweight or having hypertension, hyperlipidemia, or diabetes mellitus, are common complications after liver transplant in pediatric patients with probable multifactorial causes and increase the risk of cardiovascular complications in adulthood. In this study, our aim was to evaluate the prevalence of these components both before and after transplant surgery.

Materials And Methods: Our study included all children having liver transplant at our institution over a period of 20 years who were under 18 years old and had at least 6 months of posttransplant follow-up.

Bruck syndrome - a rare syndrome of bone fragility and joint contracture and novel homozygous FKBP10 mutation.

Bruck syndrome is an autosomal recessive syndrome consisting of bone fragility and congenital joint contractures. According to the genotype, it has been classified into types 1 and 2. Recently, mutations in FKBP10, localised to chromosome 17q21, have been identified in some patients of Bruck syndrome.

Correlation of hypocalcemia with serum parathyroid hormone and calcitonin levels in pediatric intensive care unit.

Objectives: To investigate factors involved in causing hypocalcemia in critically ill patients.

Methods: The patients aged 1 mo to 18 y, admitted to PICU at Nemazee Hospital, from May through November 2012, were reviewed. Those with impaired calcium hemostasis or on vitamin-D supplement were excluded.

Measuring agreement between cervical vertebrae and hand-wrist maturation in determining skeletal age: reassessing the theory in patients with short stature.

Introduction: The objective of this study was to determine the degree of agreement between hand-wrist radiography and cervical vertebral maturation analysis in patients diagnosed with short stature.

Methods: A cross-sectional study was designed; 178 patients (90 girls, 88 boys) diagnosed with short stature and seeking treatment were selected. The patients were divided into 2 groups (76 with familial short stature, 102 with nonfamilial short stature).

Skin manifestation of methylmalonic acidemia: case report and review of the literature.

Skin manifestations, including scalded skin, desquamation, and chronic periorificial dermatitis, are rare clinical signs in patients with methylmalonic acidemia. This condition may be due to enzyme deficiency or multi-nutrient deficiency because of nutritional restriction. Bullous skin lesion is very rare in these patients and consequently, this type of skin lesion can be the presenting sign of methylmalonic acidemia.

Cardiovascular involvement in children with osteogenesis imperfecta.

Objective: Osteogenesis imperfecta is a hereditary disease resulting from mutation in type I procollagen genes. One of the extra skeletal manifestations of this disease is cardiac involvement. The prevalence of cardiac involvement is still unknown in the children with osteogenesis imperfecta.

Investigation of osteopontin levels and genomic variation of osteopontin and its receptors in Type 1 diabetes mellitus.

Background: Failure in self-tolerance towards β-cells in diabetes mellitus (DM) pathogenesis involves a series of complex events that are governed by environmental and genetic factors. Considering the importance of osteopontin (OPN) in T-helper-1 (Th1) cells development, the aim of this study was to evaluate the serum level and gene polymorphism of OPN in Iranian Type 1 diabetic (T1DM) children.

Methods: In this case-control study, 87 T1DM children and 86 healthy ones were enrolled.

Adiponectin is a Good Marker for Metabolic State among Type 1 Diabetes Mellitus Patients.

Objective: Adiponectin is secreted from adipose tissue. This hormone has a fundamental role in pathogenesis of insulin resistance, and has anti-inflammatory and anti-atherogenic effects. The objectives of this study were to compare serum adiponectin level between type 1 diabetics and healthy people and to assess its related factors, and also to determine the relationship between adiponectin and metabolic state.

The side effects of gonadotropin releasing hormone analog (diphereline) in treatment of idiopathic central precocious puberty.

Treatment of central precocious puberty (CPP) is the administration of GnRH analogs. Metabolic syndrome comprised metabolic disturbances that confer increased risk of (CVD) diabetes mellitus (DM) and cardiovascular disease. This study is a longitudinal prospective study in pediatric endocrinology clinic.

Thyroid hormone levels in children with liver cirrhosis awaiting a liver transplant.

Objectives: Evidence exists that decreased in triiodothyronine (T3) and thyroxine (T4) levels are associated with the severity of liver disease, and these hormones could be used as disease prognostic factors, but there are paradoxes in this regard in the literature. This study aimed at evaluating the correlation between thyroid hormone levels and severity of liver disease.

Materials And Methods: We measured thyroid hormone levels in 83 children with liver cirrhosis using radioimmunoassay techniques.

Evaluation of congenital hypothyroidism in fars province, iran.

Objective: In Iran thyroid-stimulating hormone (TSH) based neonatal screening program is included in health care services from 2005 for detection of patients with primary congenital hypothyroidism (CH). This study was performed for a critical evaluation of the screening program primary congenital hypothyroidism in Fars province, Iran.

Methods: From November 2006 to September 2007, TSH serum concentrations of 63031 newborns, 3 to 5 days old born in Fars province, were measured by heel prick.

Growth parameters in children with dyspepsia symptoms and Helicobacter pylori infection.

Controversy exists about relationship of H. pylori infection and somatic growth retardation of children. The aim of this study was to evaluate the relationship between H.

Serum insulinlike growth factor-1 and its binding protein-3 levels in children with cirrhosis waiting for a liver transplant.

Objectives: Investigate the prognostic value of serum insulinlike growth factor-1 (IGF-1) and its binding protein 3 (IGFBP-3) in pediatric patients with liver cirrhosis, and investigate the correlation between these parameters and other available prognostic factors including Child-Pugh scoring, Pediatric End-Stage Liver Disease, and Mayo End-Stage Liver Disease scoring.

Materials And Methods: This prospective, case-controlled study was done at the Nemazee hospital for 12 months from August 2009 to August 2010. It included 45 pediatric patients (< 18 years) diagnosed with liver cirrhosis and 38 healthy age and sex-matched controls.

Ambulatory blood pressure monitoring in children and adolescents with type-1 diabetes mellitus and its relation to diabetic control and microalbuminuria.

Diabetes mellitus (DM) is now considered as the major cause of end-stage kidney failure, and hypertension (HTN) is one of the main determinants of progression of renal disease. The aim of this study was to assess the role of blood pressure (BP) by ambulatory blood pressure monitoring (ABPM) in children and adolescents with type-1 DM and its correlation with micro-albuminuria (MA) and diabetic control. Eighty-one patients with type-1 DM (mean age 13 ± 4 years), whose duration of DM was at least two years, were enrolled in this study.

Serum resistin level in obese male children.

Objectives. Resistin is a member of cysteine-rich molecules. Several studies have been carried out to determine the biological effect of resistin, nevertheless a significant number are animal studies.

Autoimmune polyglandular syndrome type 2: an unusual presentation.

Autoimmune polyglandular syndrome (APS) type 2 is characterized by the presence of Addison's disease, in association with autoimmune thyroid disease and/or type 1 diabetes mellitus. APS type 2 occurs most often in middle aged females and is rare in children. Here an 11 year old boy is reported with Addison's disease who developed symptom's of diabetes mellitus, goiter, malabsorption, macrocytic anemia and keratitis.

Incidence of neonatal hyperphenylalaninemia in fars province, South iran.

Objective: Phenylalanine hydroxylase or its cofactor, tetrahydrobiopterin (BH(4)), deficiency causes accumulation of phenylalanine in body fluids and central nervous system. Considering the fact that hyperphenylalaninemia is a preventable cause of mental retardation in infants, the objective of this study was to determine the incidence of congenital hyperphenylalaninemia in Fars province, south of Iran.

Methods: In a period of one year from November 2007 to November 2008 blood samples were withdrawn from all newborns born in Fars province for measurement of serum phenylalanine.

The role of insulin like growth factor (IGF)-1 and IGF-binding protein-3 in diagnosis of Growth Hormone Deficiency in short stature children.

Objective: The purpose of this study was to evaluate the role of IGF-1 and IGFBP-3 in diagnosis of short stature children and adolescents in whom Growth Hormone Deficiency (GHD) was found.

Methods: In this cross sectional study the referred short stature children and adolescents to Namazi Hospital in Shiraz- Iran, in 2003-2005 were studied. The inclusion criteria were proved short stature based on the physical examination, weight, height, standard deviation score (SDS) of height < -2, with considering stage of puberty and predicted height in children without any genetic or chronic disorders.

Insulin-like growth factor-1 levels in children with Beta-thalassemia minor.

Objective: Growth retardation in children with b-thalassemia major is multifactorial. Some etiologies described for this condition are hemochromatosis, disturbed growth hormone (GH) / insulin growth factor-1 (IGF-1) axis, undernutrition and hypermetabolism. It has also been proven that growth retardation is present in b-thalassemia major children despite regular transfusion and chelation.

Evaluation of insulin like growth factor-1 (IGF-1) in children with different stages of chronic renal failure.

Growth retardation in children with chronic kidney disease (CKD) is multifactorial that include inadequate protein and calorie intake, persistent metabolic acidosis, calcitriol deficiency, renal osteodystrophy, drug toxicity, uremic toxins and growth factor abnormalities such as insulin-like growth factor (IGF) and IGF binding proteins. In this study, we compare the IGF-1 levels in normal and growth retarded CKD children. Serum IGF-1 levels were determined in 22 children with end-stage renal disease, 26 children with CKD at different stages, 23 children with normal height and weight for age and 23 children with constitutionally short stature.

Endocrine function in thalassemia intermedia.

Unlabelled: Thalassemias are the most common genetic disorder on a wordwide basis. β-thalassemia is a severe hemolytic anemia which results from genetic defects in the synthesis of the hemoglobin β-chain. Various endocrine abnormalities have been described in patients with thalassemia major.

Reduced insulin growth factor I concentrations in iron-overloaded beta thalassaemic patients with normal growth hormone secretion and liver function.

We selected 92 subjects (46 females and 46 males), aged 10-15 years, from the Haematology and Endocrine Clinic of Shiraz University, Iran. Forty-six were beta thalassaemia patients (beta-Th) with short stature, 23 had idiopathic short stature (ISS) and 23 were healthy children with a standing height between the 10th and 95th percentile. Growth hormone (GH) secretion was normal in 23 beta-Th patients and reduced in the remaining 23 patients.