Prevalence of Idiopathic Hypoparathyroidism and Pseudohypoparathyroidism in Japan.

Abstract

A nationwide epidemiologic survey of idiopathic hypoparathyroidism and pseudohypoparathyroidism was conducted in 1998 to clarify the prevalence of the two disorders in Japan. From a total of 14,100 departments of pediatrics, internal medicine, neurology, and endocrinology in whole Japan, 2952 (20.9%) study departments were selected at random. Of these departments receiving the first questionnaire, 1855 (62.8%) responded. From these departments 390 patients with idiopathic hypoparathyroidism and 203 with pseudohypoparathyroidism who visited the hospitals in 1997 were reported. The total numbers of patients were estimated to be 900 (690-1100) for idiopathic hypoparathyroidism and 430 (330-520) for pseudohypoparathyroidism (95% confidence intervals in parentheses). Using these data, the period prevalence of the diseases were 7.2 (5.5-8.8) per million population in idiopathic hypoparathyroidism, and 3.4 (2.6-4.2) in pseudohypoparathyroidism (95% confidence intervals in parentheses).

Nationwide hospital‐based survey of idiopathic normal pressure hydrocephalus in Japan: Epidemiological and clinical characteristics

“…The departments, lists of medical institutions, and special stratified hospitals were selected for the first and second surveys by the method standardized by the Research Committee on Epidemiology of Intractable Diseases in Japan, following previous nationwide surveys of other diseases (Fukushima et al., 2010; Nakamura et al., 2000). …”

“…Since the questionnaire was sent in February 2013, it was theoretically possible to collect the data for the total number of patients who visited for iNPH between January 1 and December 31, 2012. The methodology of the surveys published in the previous reports was followed exactly (Fukushima et al., 2010; Nakamura et al., 2000). Reminder letters were mailed to the departments that did not respond to the first survey.…”

“…Additionally, the 95% confidence interval (CI) was calculated with the assumption of a multinomial hypergeometric distribution (Fukushima et al., 2010; Nakamura et al., 2000). For the analysis of treatment by shunting, good responders were defined as those who showed improved modified Rankin Scale (mRS) scores.…”

Hypocalcemic myopathy without tetany due to idiopathic hypoparathyroidism: case report

“…IHP is an infrequent disease of unknown aetiology, caused by insufficient secretion of parathyroid hormone (PTH). Its estimated prevalence in Japan is 7.2 cases per million individuals 5 . The reduced secretion of this hormone leads to hypocalcemia and hyperphosphatemia, due to the inhibition in calcium reabsorption in distal tubules and bone matrix, and also to the deficiency in the synthesis of 1,25-dihydroxyvitamin D. The clinical manifestations of IHP are multiple and affect mainly tissues of ectodermic origin.…”

Diagnosis and management of pseudohypoparathyroidism and related disorders: first international consensus statement

“…1). 6,7,20,48 * Abstract | This Consensus Statement covers recommendations for the diagnosis and management of patients with pseu do hy popar ath yroi dism (PHP) and related disorders, which comprise metabolic disorders characterized by physical findings that variably include short bones, short stature, a stocky build, early-onset obesity and ectopic ossifications, as well as endocrine defects that often include resistance to parathyroid hormone (PTH) and TSH. The presentation and severity of PHP and its related disorders vary between affected individuals with considerable clinical and molecular overlap between the different types.…”