We aimed to identify all patients with postsurgical hypoparathyroidism (HypoPT) and to evaluate their risks of renal complications and cardiovascular disease in relation to their disease and its treatment. We identified possible patients through the Danish National Patient Registry and a prescription database. Case status was adjudicated by review of individual patients' hospital records. For each patient with postsurgical HypoPT due to surgery for nonmalignant diseases between 1988 and 2012, three age-matched (±?2 years) and gender-matched controls were selected from the general background population. The prevalence of postsurgical HypoPT was 22 per 100,000 inhabitants. We identified 688 patients who had undergone neck surgery since 1988 with subsequent hypocalcaemia and inappropriate low parathyroid hormone (PTH) levels that necessitated treatment with calcium and/or vitamin D supplementation for more than 6 months. The average age at diagnosis was 49 years (range, 17-87 years), and 88% were women. Sixteen percent of all patients had had neck surgery prior to the operation causing HypoPT. Compared with controls, patients with HypoPT had an increased risk of renal complications (hazard ratio [HR], 3.67; 95% confidence interval [CI], 2.41-5.59) and hospitalization due to seizures (HR, 3.82; 95% CI, 2.15-6.79), whereas there was no increased risk of cardiac arrhythmias (HR, 1.11; 95% CI, 0.79-1.57) or cardiovascular disease or death (HR, 0.89; 95% CI, 0.73-1.09). In conclusion, although risk of seizures and renal complications is increased, mortality and risk of cardiovascular diseases or arrhythmias is not increased in patients with HypoPT. Further study should try to determine how to reduce the risk of seizures and renal complications in HypoPT.
Hypoparathyroidism is a disorder characterized by hypocalcemia due to insufficient secretion of PTH. Pseudohypoparathyroidism is a less common disorder due to target organ resistance to PTH. This report summarizes the results of the findings and recommendations of the Working Group on Epidemiology and Diagnosis of Hypoparathyroidism.
Each contributing author reviewed the recent published literature regarding epidemiology and diagnosis of hypoparathyroidism using PubMed and other medical literature search engines.
The prevalence of hypoparathyroidism is an estimated 37 per 100 000 person-years in the United States and 22 per 100 000 person-years in Denmark. The incidence in Denmark is approximately 0.8 per 100 000 person-years. Estimates of prevalence and incidence of hypoparathyroidism are currently lacking in most other countries. Hypoparathyroidism increases the risk of renal insufficiency, kidney stones, posterior subcapsular cataracts, and intracerebral calcifications, but it does not appear to increase overall mortality, cardiovascular disease, fractures, or malignancy. The diagnosis depends upon accurate measurement of PTH by second- and third-generation assays. The most common etiology is postsurgical hypoparathyroidism, followed by autoimmune disorders and rarely genetic disorders. Even more rare are etiologies including parathyroid gland infiltration, external radiation treatment, and radioactive iodine therapy for thyroid disease. Differentiation between these different etiologies is aided by the clinical presentation, serum biochemistries, and in some cases, genetic testing.
Hypoparathyroidism is often associated with complications and comorbidities. It is important for endocrinologists and other physicians who care for these patients to be aware of recent advances in the epidemiology, diagnosis, and genetics of this disorder.
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Nonsurgical hypoparathyroidism (HypoPT) is a rare disorder most often caused by mutations in different genes. It is characterized by hypocalcaemia with inappropriately low PTH levels. Knowledge about this group of patients, including their mortality and morbidity, is very sparse. The aim was to identify all patients diagnosed with nonsurgical HypoPT in Denmark and assess their mortality and risk of complications. Through registers and review of individual patient hospital charts we identified all patients diagnosed with nonsurgical HypoPT in Denmark between 1977 and 2012. We assessed their mortality and morbidity by comparing them with a group of age- and gender-matched population-based controls. We identified a total of 180 patients with nonsurgical HypoPT among whom 123 (68%) were alive at the date of follow-up (prevalence of 2.3/100,000 inhabitants). Compared with controls, mortality was not increased, but patients had a significantly increased risk of renal insufficiency (hazard ratio [HR] 6.01), cardiovascular diseases (HR 1.91), neuropsychiatric complications (HR 2.45), infections (HR 1.94), seizures (HR 10.05), cataract (HR 4.21), and fractures at the upper extremities (HR 1.93). In contrast patients had significantly reduced risk of malignant diseases (HR 0.44). In conclusion, nonsurgical HypoPT is a rare disease associated with a number of complications that should be considered when taking care of these patients.
A 4-day-old neonate presented with respiratory distress owing to chest wall deformity associated with metabolic bone disease. He was found to have congenital hyperparathyroidism and his mother was suffering from post-surgical hypoparathyroidism and vitamin D deficiency. The patient was given calcium lactate and maintenance doses of vitamin D. The respiratory distress subsided, the parathyroid hormone level returned to normal and by 4 weeks of age bone mineral content had improved. Congenital hyperparathyroidism should be considered in neonates presenting with respiratory distress and chest deformity.
A 59-year-old woman with idiopathic hypoparathyroidism who had tetany and zonular cataract is described. Twelve years ago she had had a period with cramps and convulsions, followed by an absence of symptoms for several years. Judging from the distance between the opacity of cataract and the surface of the lens, the onset of the cataract was estimated to have occurred 11 years ago and the apparent cessation of cataract development 10 years ago. It is suggested that the absence of hypocalcaemic symptoms during the last 10 years was associated with an increase in serum calcium levels, possibly connected with the onset of menopause. The occurrence of hypocalcaemia was analyzed in a health-screened population of 15 903 persons. Nine of the subjects were found to have a serum calcium level of less than 2.10 mmol/l, giving a prevalence of 0.6%0. None had primary hypoparathyroidism, which illustrates the rarity of this condition.
BACKGROUND AND OBJECTIVE: Postoperative hypoparathyroidism is a rare, but complex endocrine disorder. The purpose of this case series study was to evaluate the symptoms, signs and sequelae of the disease in a major homogeneous patient group after the Chernobyl accident in the Ukraine. PATIENTS AND METHODS: This study is based on a random sample of 25 patients with symptomatic permanent hypoparathyroidism after surgical procedures on the thyroid for carcinoma following the Chernobyl accident in May 1986. They underwent physical examination and their parathyroid hormone and calcium levels in serum were determined. The signs, symptoms and sequelae of their disease were systematically assessed by a validated specific questionnaire. The extent of individual distress by the symptoms and signs were analysed by using a Likert scale (points between 1 = no and 5 = maximal distress). RESULTS: In 14 patients (56%) (age at time of operation: 18.24 +/- 4.26 years) paresthesias occurred at least every third day. Very frequent general symptoms were joint pains and hair loss (in 17 patients / 68%). 19 patients (76%) had enamel defects, 17 (68%) increasingly dry skin since the operation. Psychical symptoms like excitability (20 patients / 80%) and depressed mood since the operation (16 patients / 64%) were strikingly frequent. The patients were affected more by dry skin (2,76 on the Likert scale) than by paraesthesias (2,56) or joint pains (2,68) and most of all by depressive mood (2.96) and excitability (3.38). 4 patients (16%) had kidney stones, while osteoporosis and osteosclerosis occurred in 7 (28%) respectively 3 persons (12%). 6 patients (24%) had cataracts and 3 (12%) had basal ganglia calcification. Interestingly, 1/3 of all patients had only non-characteristic symptoms of the disease consistent with a latent form of hypoparathyroidism. CONCLUSIONS: Our data partly show a severe form of hypoparathyroidism of very young persons in the Chernobyl region, a finding which strongly supports the need of exact diagnosis and interdisciplinary treatment options of this postoperative disorder.