We are presenting a case of a 36-year-old patient with idiopathic hypoparathyroidism and reversible dilated cardiomyopathy
as a result of hypocalcaemia. Twelve years later, the patient presented a picture of nephrogenic diabetes insipidus, which
according to available literature has so far not yet been described

DUBRAVKA BARTOLEK HAMP

DUNJA KURBEGOVIĆ

GORDANA CAVRIĆ

INGRID PRKAČIN

KLARA JURIĆ

KRISTINA NJERŠ

English

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 93www.signavitae.comIdiopathic hypoparathyreoidism, reversible cardiomyopathy and nephrogenic diabetes insipidus - case report ABSTRACTWe are presenting a case of a 36-year-old patient with idiopathic hypoparathyroidism and reversible dilated cardiomyopathy as a result of hypocalcaemia. Twelve years later, the patient presented a picture of nephrogenic diabetes insipidus, which according to available literature has so far not yet been described. GORDANA CAVRIĆ (  ) •DUNJA KURBEGOVIĆ •INGRID PRKAČINUniversity Hospital Merkur Zajčeva 19, Zagreb, CroatiaPhone: +38512253207 Fax: +38512431393 E-mail: gordana.cavric1@gmail.comKLARA JURIĆDepartment of Internal Medicine University Hospital Dubrava Zagreb, CroatiaKRISTINA NJERŠEmergency Medicine Department Slavonsko-brodska County Hospital, Croatia DUBRAVKA BARTOLEK HAMPSv. Katarina, Special Hospital for Orthopaedics Surgery, Neurology and Physical Medicineand Rehabilitation, Zabok, CroatiaPETAR PEKIĆ • GORDANA CAVRIĆ • KLARA JURIĆ • KRISTINA NJERŠ • INGRID PRKAČIN •  DUBRAVKA BARTOLEK HAMP • DUNJA KURBEGOVIĆ   SIGNA VITAE 2014; 9 (Suppl 1): 93 - 94Key words: hypoparathyreoidism, cardiomyopathy, nephrogenic diabe-tes insipidusIntroductionIdiopathic hypoparathyroidism manife-sts with signs of hypocalcaemia. Dilated cardiomyopathy caused by hypocalcae-mia is rare. Association between idiopa-thic hypoparathyroidism and nephroge-nic diabetes insipidus is also rare. Case reportA thirty-six–year-old patient was tran-sferred to the intensive care unit from the department of cardiology because of deterioration of cardiac decompen-sation. This was a patient who was trea-ted for alcoholism seven years ago and has been abstinent since. Four years ago he was hospitalized for acute psyc-hosis. He was already treated at the Department of Cardiology 2.5 months earlier for recurrent pulmonary oedema and dilated cardiomyopathy.The patient was of medium height, con-scious, afebrile and eupneic. Lung aus-cultation showed rales on both sides of the lungs. The action of the heart was rhythmic with quiet tones, and blood pressure was 135/80 mm Hg. The extre-mities were without oedema. A phe-nomenon of gear and “gynecological hand” were found in neurological status with the tongue and left hand tremor and increased muscle tone. The psychiatrist stated that the patient had signs of psyc-hotic anxiety that occasionally had the proportions of panic and fear of imme-diate death. Attacks were mostly pre-sent at night, lasting about five minutes, accompanied by hyperventilation with the worsening of extrapyramidal side effects. He was treated with biperiden, clozapine, fluvoxamine, furosemide 1 tablet at 40 mg every other day with 500 mg of potassium chloride.Arterial blood gas measurements and acid-base status showed metabo-lic alcalosis and hypoxemia. Correc-ted calcium was 0.1 mmol/l (normal values  2.25-2.75 mmol/l), phosphorus 3.5 mmol/l (normal values  0.65-1.62 mmol/l), potassium 3.0 mmol/l (normal values 4.1-5.5 mmol/l), parathyroid hor-mone (PTH) 4.4 pg / ml (normal values 20 - 90 pg/ml). Magnesium, as well as T3, T4 and TSH, were within limits. The X-ray of the heart and the lungs showed an enlarged cardiac shadow with severe intrapulmonary stasis. The electrocardi-ogram revealed sinus rhythm, frequen-cy of 108/min and prolonged corrected QT interval (QTc) of 517 ms. An ultrasound of the heart showed dila-ted cardiomyopathy (left ventricular-diastolic diameter-LVDd) with reduced ejection fraction (EF) of 48% and severe interventricular septum (IVS) and apex hypocontractility, as well as increased atriums - left atrium (LA) 50 mm with severe degree tricuspid and mitral regur-gitation (both 3-4 +). IVS thickness was 12 mm, and the posterior LV wall (LVPW) 10 mm. A brain CT scan pointed out almost symmetrical calcifications in the area of the basal ganglia (in the heads nn. caudati, nn. lentiform as both thala-mus pulvinars). An elongated calx sha-dow was visible in the left hemisphere of the cerebellum. The ocular fundus examination was normal.Considering the test results, a diagnosis of primary idiopathic hypoparathyroidi-sm was made. The therapy involved the preparation of calcium and calcitriol with furosemide, spironolactone, biperiden, 94 www.signavitae.comREFERENCES1. Goltzman D. Clinical manifestations of hypocalcemia. [last updated November 4, 2013., cited May 5, 2014.]. Available from: http://www.uptodate.com/contents/clinical-manifestations-of hypocalcemia?source=search_result&search=clinical+manifestations+of+hypocalcemia&selectedTitle=1~150. 2. Ballane GT, Sfeir JG, Dakik HA, Brown EM, Fuleihan GEH. Use of recombinant human parathyroid hormone in hypocalcemic cardiomyo-pathy. Eur J Endocrinol 2012; 1113- 1120.3.   Jung YJ, Kim SE, Hong JY, Lee JH, Park DG, Han KR at al. Reversible dilated cardiomyopathy caused by idiopathic hypoparathyroidsm. Korean J Intern Med 2013; 28:605- 608. 4.   Benzarouel D, Hasni K, Ashab H, El Hattaoui M. A reversible cause of cardiomyiopathy: hypocalcemia. Ann Cardiol Angeiol (Paris) 2014; 63 (2): 102- 106.5. Bichet DG. Clinical manifestations and causes of nephrogenic diabetes insipidus. [last updated November 12, 2012., cited May 5, 2014.]. Avai-lable from: http://www.uptodate.com/contents/clinical-manifestations-and-causes-of-nephrogenic-diabetes insipidus?source=search_result&search=Clinical+manifestations+and+causes+of+nephrogenic+diabetes+insipidus&selectedTitle=1~77.6. Hunter AGW, Heick H, Poznanski WJ, McLaine PN. Autosomal dominant hypoparathyreoidsm: a proband with concurrent nephrogenic diabetes inspidus. J Med Genet 1981;18:431- 435.sulpiride, nitrazepam. The therapy lead to an improvement of the general condi-tion of the patient, and the patient’s heart compensated. Control tests showed the following values: corrected calcium 1.7 mmol/l, phosphorus 2.36 mmol/l, pota-ssium 4.5 mmol/l, the electrocardiogram monitored the reduction in QTc - 395 ms. A neurological examination was made after a few days and it showed no dis-turbances other than the patient`s slow thought process and incomplete space orientation. The patient was discharged after 32 days in the hospital with the recommendation of further outpatient follow-ups with a cardiologist, an endo-crinologist and a psychiatrist.Twelve years later, the patient was hospi-talised in the nephrology department for a diagnostic treatment of hypertension, polyuria and polydipsia that had been present for two years. The patient was monitored for psychosis by a psychiatrist. A cardiologist monitored the reversibility of dilated cardiomyopathy.  The patient was on fluphenazine, biperiden, risperi-done, zolpidem, atorvastatin, lisinopril, atenolol, CaCO3 and calcitriol therapy. A cardiac ultrasound was done and showed left ventricular concentric hypertrophy, incomplete diastolic relaxation without regional contractility disturbances, functi-onal valves, moderate tricuspidal regurgi-tation with no indirect signs of pulmonary hypertension (LVDd 3.9 cm, right ventric-le 1.0 cm, LA  4.8 cm, IVS 1.7 cm, LVPW 1.3 cm, EF 73 %). The values  of urea, cre-atinine, calcium, phosphorus, potassium, magnesium, TSH, T3 and T4 were within normal limits. PTH was < 6 pg/ml, the creatinine clearance was 65.9 ml/min and the protein in 24 h urine 0.36g. QTc was 400 ms. The kidneys were examined by ultrasound and were of normal size. Hol-ter blood pressure recorded an average value of 135.2/97 mm Hg and the brain CT scan showed no significant changes compared to the scan made  12 years ago. Aldosterone and plasma renin valu-es were within normal limits as was the value of antidiuretic hormone (ADH) in the urine. Urine concentration test indicated the isosthenuria (plasma osmolality 290 mOsm/kg, urine osmolality 150 mOsm/kg) with diuresis 3000-5500 ml for 24 h. Renal biopsy found a 20% altered glo-meruli (connective tissue) without signi-ficant interstitial fibrosis, tubular atrophy and without significant changes in the blood vessels. Based on these results, we concluded that the patient suffered from nephrogenic diabetes insipidus, which required further monitoring. The patient was discharged in good general condition with the recommendation for further follow-ups.DiscussionIdiopathic hypoparathyroidism manife-sts with signs of hypocalcaemia such as tetany, epileptic seizures, hypotension (in acute hypocalcaemia), prolongation of the QT interval in the electrocardi-ogram, papilloedema and psychiatric manifestations including psychosis. A few cases of congestive heart failure and dilated cardiomyopathy are also descri-bed. Although the mechanism remains undefined, calcium plays a critical role in excitation-contraction coupling and is required for epinephrine-induced glyco-genolysis in the heart. (1) As a result of long-term hypoparathyroidism, calcifi-cations of the basal ganglia may occur, which may or may not be associated with extrapyramidal symptoms. (1)According to available data to date, the literature has described 29 cases of hypocalcemic cardiomyopathy in patients of all ages. (2-4)Our case concerned hypoparathyroi-dism with a distinct clinical picture of hypocalcaemia, including dilated cardi-omyopathy that withdrew after treatment with preparations of calcium and vita-min D. The psychiatric and neurological disorders that were present at hospital admission were also significantly impro-ved due to calcium correction.Twelve years later, the patient was dia-gnosed with idiopathic nephrogenic diabetes insipidus. Theoretically, tran-sient hypercalcaemia could have been the cause, possibly as a consequence of hypoparathyroidism treatment. (5) To our knowledge, one case of autoso-mal dominant hypoparathyroidism and nephrogenic diabetes insipidus has been described so far. (6) There are no data so far indicating that the drugs that the patient took could be associated with the development of nephrogenic diabetes insipiduss.ConclusionWe have presented a case of a 36-year-old patient with idiopathic hypoparat-hyroidism and reversible dilated cardio-myopathy as a result of hypocalcaemia. Twelve years later, the patient presented a picture of nephrogenic diabetes insipi-dus, which according to available litera-ture has so far not yet been described.

Idiopathic hypoparathyreoidism, reversible cardiomyopathy and nephrogenic diabetes insipidus - case report

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Idiopathic hypoparathyreoidism, reversible cardiomyopathy and nephrogenic diabetes insipidus - case report

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