The Outcome of Infantile Onset Pompe Disease in South of Iran.

Background: Infantile Onset Pompe Disease (IOPD) is a rare autosomal recessive neuromuscular disorder. It is associated with cardiomegaly, hypotonia, paresis, and death in the first year of life. Since 2006, following the use of Alglucosidase alfa as Enzyme Replacement Therapy (ERT), the patients' survival is improved to a noticeable extent.

Human growth hormone induced cholestatic hepatitis in a growth hormone deficient patient with short stature.

Case Description: We report a patient with growth hormone deficiency that developed cholestatic hepatitis during treatment with recombinant human growth hormone (HGH). The patient developed jaundice and pruritus during treatment with growth hormone. She did not use any other medications.

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.

Thyroid function and stress hormones in children with stress hyperglycemia.

The purpose of the study is to determine the prevalence of stress hyperglycemia and to investigate how thyroid and stress hormones alter during stress hyperglycemia in children admitted to pediatric emergency wards. A prospective cross-sectional study was conducted in children, less than 19 years old, who were admitted to pediatric emergency wards of Nemazee and Dastgheib Hospitals, Shiraz, Southern Iran. Those patients taking steroids, beta-agonists or intravenously administered glucose before venipuncture, and patients with diabetes mellitus (DM) or thyroid diseases were excluded.

Does congenital hypothyroidism have different etiologies in iran?

Objective: To determine the prevalence of congenital hypothyroidism (CH), permanent and transient CH.

Methods: From November 2006 to September 2007, 63031 newborns were screened by measuring serum TSH obtained by heel prick. The neonates who had a TSH≥5mU/L were recalled for measurement of serum T(4), thyroid stimulating hormone (TSH) and TSH receptor blocking antibodies (TRBAb) in venous samples.