SAT-335 New Onset Adult Idiopathic Primary Hypoparathyroidism Concomitant with Severe Aplastic Anemia
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Abstract Introduction: Primary hypoparathyroidism is a relatively rare cause of hypocalcemia with cases of primary hypoparathyroidism in the US estimated at 24–37 per 100,000 with 75% being due to neck surgery and 25% due to non-surgical causes. The clinical presentation depends on the acuity of development of hypocalcemia and the absolute level of serum calcium. Here is a case of severe hypocalcemia secondary to hypoparathyroidism of unknown etiology followed by the development of severe aplastic anemia.Case report: A 60-year-old Caucasian male presented to our ED with fatigue, tingling, numbness in extremities and was found to have severe hypocalcemia at 6.8 mg/dl and decreased PTH at 11 pg/mL. Calcium levels 8 months prior to presentation were normal. No history of neck surgery, radiation exposure or family history of autoimmune disorders. Initial workup included creatinine, magnesium and TSH were normal. Autoimmune panel (including PTH Abs and CaSR Abs), HIV test, hepatitis panel, serum protein electrophoresis were also negative. Infiltrative causes of hypoparathyroidism including hemochromatosis, malignancy and granulomatous diseases like sarcoidosis were ruled out with tissue sampling and lab workup. Sestamibi scan obtained showed no parathyroid activity in all four glands. Patient was initially treated with IV calcium to improve serum calcium to more than 7.5mg/L and then switched to oral calcium carbonate 500mg TID and calcitriol 0.5mcg BID until the calcium level was brought up to the lower limit of normal. Patient was seen in follow up and was doing well without any adverse effects. Consequently, the patient developed severe aplastic anemia which was treated with steroids and interestingly, has caused a gradual but consistent increase in PTH levels.Discussion: Idiopathic hypoparathyroidism (IHP) is a rare condition with an incidence of 0.02%. IHP can occur sporadically or as part of a familial condition with autosomal dominant, recessive and X-linked recessive patterns. Certain autosomal forms of hypoparathyroidism have mutations in the PTH gene and Calcium-Sensing Receptor (CaSR) gene. The challenging nature of this case is due to the subacute nature of the patient’s presentation along with the lack of a definitive etiology. The patient’s negative family history and older age makes genetic causes less likely, and Abs against PTH and CaSr were also negative. The patient’s diagnosis of severe aplastic anemia has made the case more fascinating, especially since its management with steroids has causes an improvement in the patient’s PTH status. Regardless of etiology, primary hypoparathyroidism is treated with lifelong supplementation of calcium and calcitriol to a goal serum calcium level at the lower limit of normal. Reference: Abate EG,Clarke BL. Review of Hypoparathyroidism. Front Endocrinol (Lausanne). 2017; 7:172. Published 2017 Jan 16Keywords:
Hypoparathyroidism
Aplastic anemia
Hypoparathyroidism following neck surgery, mainly thyroidectomy, is not a rare event, well known since many years. Post-surgical hypoparathyroidism may occur in form of two clinical syndromes of different etiology and prognosis. The first disease is a transitory hypoparathyroidism that might spontaneously recover within a few weeks or months. The second disease is a permanent hypoparathyroidism needing a definitive opoterapic treatment. Anyhow in both cases, hypocalcemic symptoms begin always within a short time from surgery, usually after an asymptomatic period elapsing from days to months. Only few cases of hypoparathyroidism clinically conclamate after many years from surgery have been reported. Description of a patient with hypoparathyroidism that became clinically evident thirty years after the thyroid surgery is herewith described. Our findings and review of a few cases reported by medical literature, can suggest a third form of post-surgical hypoparathyroidism with the distinctive feature of a very late beginning, probably following a long period of a latent parathyroid insufficiency.
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Nephrocalcinosis
Hypocalcaemia
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Hypoparathyroidism
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Hypoparathyroidism is one of the most common complications after total or completion thyroidectomy. The reported incidence rate of hypoparathyroidism in the literature is highly variable. Data that provide a better understanding of the magnitude of this postoperative complication are warranted and can provide a stepping stone for further collaborations that aim to reduce complication rates and establish uniform treatment protocols.To evaluate the incidence of postoperative, persistent hypoparathyroidism after total or completion thyroidectomy in patients who were referred to university hospital centers and assess the association of different definitions with the incidence of hypoparathyroidism.This retrospective multicenter cohort study conducted throughout 2016 in 7 Dutch university hospital centers included 200 patients who were undergoing a total or completion thyroidectomy. Data analysis was conducted in January 2021.We report on the incidence of persistent hypoparathyroidism, defined as the need for active vitamin D with or without calcium supplementation longer than 1 year after surgery.A total of 200 patients (143 women [71.5%]; mean [IQR] age, 49.0 [37.0-62.0] years) were included and 30 patients (15.0%) developed persistent hypoparathyroidism. The incidence of persistent hypoparathyroidism varied between 14.5% (calcium and active vitamin D 1 year postsurgery) to 28.5% (calcium and/or active vitamin D 6 months postsurgery) depending on the definition used.In this cohort study, the risk of persistent hypoparathyroidism after total or completion thyroidectomy was 15% in patients who were referred to university hospital centers. The high rate of persistent hypoparathyroidism warrants efforts to reduce this complication rate. There is discrepancy in the definition and treatment of persistent hypoparathyroidism, and use of uniform evidence-based treatment guidelines enables comparison of interventions.
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Post-operative hypoparathyroidism is a complication in patients who undergo thyroid surgery. Our study aimed to evaluate the incidence and causes of post-operative transient and permanent hypoparathyroidism in patients undergoing thyroid surgery.The data of 933 consecutive patients who underwent total thyroidectomy in a single center were retrospectively evaluated. The rate of post-operative hypoparathyroidism, clinicopathological features, and laboratory parameters during the post-operative first day, first month, and first year of patients with and without hypoparathyroidism were analyzed. Patients with hypoparathyroidism were classified as transient or permanent cases.The incidence of post-operative hypoparathyroidism was 22.7%, including transient (20.6%) and permanent (2.1%). In multivariable analysis, independent predictors of permanent hypoparathyroidism were as follows: surgery due to malignant thyroid disease, tumor multifocality, and pre-operative vitamin-D deficiency (VDD) (p<0.001, 0.047, and 0.002, respectively). During the post-operative first month, the mean serum PTH levels were found to be 7.58 pg/mL, and they remained low on the post-operative first year in patients with permanent hypoparathyroidism.Surgery due to thyroid malignancy and VDD should be considered risk factors for permanent hypoparathyroidism in patients who undergo thyroid surgery. The post-operative first month is important in the prediction of permanent hypoparathyroidism.Hypoparathyroidism, Permanent, Transient.L’ipoparatiroidismo postoperatorio è una complicanza possibile della chirurgia della tiroide, e ci siamo proposti di studiarne incidenza transitoria o permanente, e cause, in 933 pazienti della nostra casistica di operati alla tiroide controllati retrospettivamente. Si tratta della casistica dei un singolo unico centro, e di una valutazione retrospettiva riguardante l’incidenza dell’ipoparatiroidismo postoperatorio, le caratteristiche clinico- patologiche del pazienti, ed i parametri di laboratorio del primo giorno postoperatorio, del primo mese e del primo anno di questi pazienti, senza o con ipoparatiroidismo, distinguendo in quest’ultimo gruppo l’ipoparatiroidismo transitorio o permanente. Come risultato abbiamo riscontrato un’incidenza di ipoparatiroidismo postoperatorio del 22,7% - rispettivamente 20,6% transitorio e 2,1% permanente. Con l’analisi multivariata i fattori indipendenti di previsione dell’ipoparatiroidismo sono risultati: interventi per patologia maligna, multifocalità del tumore, carenza preoperatoria di vitamina D (VDD) – rispettivamente p<0,001; 0.047; 0.002). Durante il primo mese postoperatorio i livelli medi del PTH nel siero sono stati trovati essere 7,58 pg/ml, rimanendo bassi per tutto il primo anno postopertorio in caso di iperparatiroidismo permanente. In conclusione la chirurgia per patologia maligna e la carenza preoperatoria di vit D dovrebbero essere considerato fattori di rischio di ipoparatiroidismo postoperatorio nel pazienti candidati ad intervento chirurgico. Il primo mese postoperatorio è importante per la previsione dell’ipoparatiroidismo permanente.
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A family is described in which 2 boys developed idiopathic hypoparathyroidism when 11 and 21 yr old. A sister, who died when 19 yr old, may also have had the disease. The remaining sibling, a brother age 30, and the parents are alive and well. Hypoparathyroidism in this family has apparently been transmitted by an autosomal recessive gene. This is the seventh family to be reported in which more than one member had idiopathic hypoparathyroidism and provides additional evidence that some cases of hypoparathyroidism are of genetic origin. In addition, the literature on familial idiopathic hypoparathyroidism and the classification of hypoparathyroidism are reviewed.
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Endocrine surgery
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The incidence of hypoparathyroidism following thyroid surgery in the series reported was 7.8 per cent. Temporary hypoparathyroidism was present in 15 patients (4.6%) and permanent hypoparathyroidism in 10 (3.2%). Persistently low levels of serum calcium and high levels of serum phosphorus at six to 10 weeks after thyroid operation in patients with temporary hypoparathyroidism, with no tendency toward normalization, suggests permanent hypoparathyroidism. Serum calcium below 5 mg per cent suggests permanent hypoparathyroidism.
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Objective:The most common complication following a bilateral thyroidectomy is hypoparathyroidism.The aim of this study was to analysis of the clinical factors of hypoparathyroidism after bilateral thyroidectomy and the medical treatment to prevent hypocalcaemia.Methods:We studied 193 bilateral thyroidectomy patients between January 2011 and November 2011.PTH and serum calcium level were assayed 1 day before,1 day,2 days,1 month,3 months and 6 months after thyroidectomy.Results:Among these 193 cases,hypoparathyroidism was found performed in 25 cases(13.0%),19 cases(9.8%)developed transient hypoparathyroidism,6 cases(3.1%)were permanent hypoparathyroidism.The patients with malignancy thyroid disease(24.7%) had significantly(P0.05)higher hypoparathyroidism than those with benign thyroid disease(5.2%,P0.001).The patients with lymphadenectomy(20.9%)had significantly higher hypoparathyroidism than those without lymphadenectomy(8.7%,P0.05).There were no significent difference in different age and sex groups(P0.05).The permanent hypoparathyroidism group had higher cervical lymphadenectomy proportion(100%)than transient hypoparathyroidism group(42.1%,P0.05).The rate of the unintentional parathy oidect omy was significant higher in permanent hypoparathyroidism group(83.3%)than transient hypoparathyroidism group(21.0%,P0.01).Selective postoperative oral calcium and calcitriol supplementation was performed.Among 25 hypoparathyroidism cases only 1 case develpoed hypocalcaemia.Conclusion:Hypoparathyroidism after bilateral thyroidectomy was related with operation range.Unintentional parathyroidectomy is a risk factor for permanent hypoparathyroidism.Selective suppliment of calcium and calcitriol can reduce hypocalcaemia effectively.
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