Increased peroxidase activity in Pendred's syndrome with hypothyroidism.

نویسندگان

  • M Yamamoto
  • S Saito
  • T Sakurada
  • K Yoshida
  • K Yoshinaga
چکیده

An 8-year-old boy with goiter and bilateral nerve deafness had a 46% discharge of radioiodine after thiocyanate administration. He was clinically euthyroid. Although the serum total T4 was low (2.4 mug/100 ml) and TSH was significantly high (181 muU/ml), the serum total T3 was normal (152 ng/100ml). It was considered that the increased release of TSH by the feedback mechanism in response to the low T4 resulted in a quite normal level of serum T3. The thyroid gland demonstrated a low stable iodine content, an increase in MIT/DIT ration and a decrease in iodothyronine. The thyroglobulin behaved normally in Sephadex G-200 chromatography and immunoreaction. Thyroid tissue exhibited increased peroxidase activity as measured by I3 formation. Increased peroxidase activity may be related to the observed increase in serum level of TSH.

برای دانلود رایگان متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Pendred Syndrome: A Case Report

In this article Pendred's syndrome, which is the combination of - congenital goitre with deafmutism, is discussed. This type of goitre is due to a special enzymatic defect, involving  a process of hormonogenesis, which is respon-· sible for the incorporation of iodide in the tyrosine molecule.  A case of pendred's syndrome is presented  in a 16-year-old girl. This girl has never been able to...

متن کامل

Genetics of congenital hypothyroidism.

Congenital hypothyroidism is the most common neonatal metabolic disorder and results in severe neurodevelopmental impairment and infertility if untreated. Congenital hypothyroidism is usually sporadic but up to 2% of thyroid dysgenesis is familial, and congenital hypothyroidism caused by organification defects is often recessively inherited. The candidate genes associated with this genetically ...

متن کامل

Congenital Goitrous Hypothyroidism, Deafness and Iodide Organification Defect in Four Siblings: Pendred or Pseudo−Pendred Syndrome?

Pendred syndrome (PDS) is an autosomal recessive disorder characterized by congenital deafness, goiter and iodide organification defect. Presence of inner ear malformations is essential for the clinical diagnosis. Most individuals with PDS are clinically and biochemically euthyroid. Mutations in the PDS gene encoding pendrin protein have been shown to be associated with PDS. It has been recentl...

متن کامل

Antioxidant Enzymes Status and Thyroid Stimulating Hormone Level of down Syndrome Patient in West Bengal, India: a Case-control Study

Objective: The copper-zinc superoxide dismutase gene resides on chromosome 21 and is over expressed in Down syndrome patients. Reactive oxygen species can initiate lipid peroxidation and DNA damage leading to mutagenesis, carcinogenesis and cell death, if the antioxidant system is impaired. Down syndrome is associated with various forms of thyroid dysfunction, hypothyroidism being the most comm...

متن کامل

Mustafa Abdalla

Pendred's syndrome was described in a Sudanese family and its affected members were studied in details. They were a boy (aged 14 years) and a girl (11 years of age) each showing a goitre, a positive perchlorate discharge test, marked sensorineural deafness and evidence of mild hypothyroidism. This study adds an entity to the congenital dyshormonogenesis recognised so far in the Sudan.

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

عنوان ژورنال:
  • The Tohoku journal of experimental medicine

دوره 119 2  شماره 

صفحات  -

تاریخ انتشار 1976