Paper Information

Journal:   RAZI JOURNAL OF MEDICAL SCIENCES (JOURNAL OF IRAN UNIVERSITY OF MEDICAL SCIENCES)   Fall 2003 , Volume 10 , Number 35; Page(s) 387 To 396.
 
Paper: 

THE STUDY OF PSEUDOHYPOPARATHYROIDISM PATIENTS WHO REFERRED TO THE INSTITUTE OF ENDOCRINOLOGY AND METABOLISM DURING YEARS

 
 
Author(s):  ROOHANI F., KIANI AMIN F.
 
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Abstract: 
Pseudohypoparathyroidism (PHP) refers to a spectrum of disorders, which is characterized by resistance of target organ to parathyroid hormone (PTH) followed by hypocalcemia, hyperphosphatemia and hyper secretion of PTH. Nowadays different types of PHP (1A, 1B, 1C, 2) can be distinguished: the most common type among them is type 1A accompanied by Albright"s phenotype (round faces, shortening of metacarpals & metatarsals...) PHP is an inherited diseas and prevalence ate in different societies is variable. Can Albright"s phenotype be identified in most patients with PHP in our country? Is PHP always accompanied by hypocalcemia? As there has not been andy study on PHP in our country, and considering possible differences among patients with PHP in different geographical regions, and in an effort to answer aforementioned questions, we decided to study clinical manifestations, laboratory findings and frequency of Albrights phenotype in patients with PHP, who referred to the Institute of endocrinology and metabolisms during past 10 years in an observational descriptive manner. 10 patients with PHP including 6 girls (60%) and 4 boys (40 %) with the mean age of 13.8±2.62 years were examined. Clinical manifestations of patients consisted of seizure (70%) paresthesia (30 %), carpopedal spasm (20%) and muscle cramps (20%) Basal ganglion calcification (20%), chvostek"s sign (10%), cataract (10 %), abdominal pain (10 %) and malaise (10%). Laboratory findings consisted of increased plasma PTH level in 100% (mean 172 ± 85.6 pg/ml), hyperphosphatemia in 90 % and hypocalcemia in 70% (mean 7.3 ± 1.23 mg/dl) of patients were observed. Thyroid function tests done in i -% of patients were normal. Albright"s phenotype was identified in one patient (10%). Short stature in 30 % and obesity in 10% of patients was observed. 8 patients (80%) had normal puberty and in 2 prepubertal patients (20%) secondary sexual characters had not appeared. PHP was common in females & adolescents. The most common clinical manifestations & laboratory findings were seizure & increased plasma PTH level respectively. PHP with normocalcemia was identified in 30% of patients. This may be due to the relative response of ones to PTH. Albrights phenotype was identified in just 10% of the patients which can be attributed to the non prevalence of PHP type 1A in our patients and this necessitates conducting more studies in this regard. There was not any case of resistance to TSH and gonadotropines. As seizure is the most common clinical sign in PHP patients. Therefore, measuring the level of serum calcium should be considered as a priority in each patient with seizure and PHP should be subjected to differential is diagnosis.
 
Keyword(s):  PSEUDOHYPOPARATHYROIDISM, HYPOCALCAEMIA, HYPERPHOSPHATEMIA
 
References: 
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