Chronic Hypocalcemia Research Articles

Chronic asymptomatic hypocalcemia following thyroid surgery

Chronic asymptomatic hypocalcemia following thyroid surgery

Abstract #1176076: Secondary Hypoparathyroidism and Intracranial Calcifications

Hypoparathyroidism is a well-known potential complication of total thyroidectomy and is permanent in 1-3% of cases. One rare complication of chronic hypocalcemia is intracranial calcification, most commonly affecting the basal ganglia. We present a patient with longstanding postsurgical hypoparathyroidism, extensive central nervous system (CNS) calcinosis, but only mild neurologic symptoms.

The role of Teriparatide in post-thyroidectomy permanent hypoparathyroidism: A case report

Hypoparathyroidism is a rare endocrine disease that is commonly found following anterior neck surgery. The case of permanent hypoparathyroidism occurring years after surgery is even more exceptional; therefore, it is highly potential to be left undiagnosed and thus untreated. The goal of treatment in hypoparathyroidism is to maintain the blood calcium level near the low end of the normal range while preventing symptoms of hypocalcemia. The most recent treatment also includes parathyroid hormone therapy. We present a case of a 63-year-old male presenting with vertebral fracture as a complication of hypoparathyroidism diagnosed 3 years after total thyroidectomy. He also had chronic hypocalcemia refractory to calcium and active Vitamin D supplementary. The patient was then treated with routine subcutaneous Teriparatide injection with a satisfying outcome. Serum calcium level was maintained within normal range and other symptoms of hypocalcemia resolved.

The axial spondyloarthritis clinical phenotype in idiopathic hypoparathyroidism: critical review of concept that muscular hypercontractility can induce enthesopathy lesions.

Idiopathic hypoparathyroidism (iH-PoPT) is a rare condition infrequently associated with axial spondyloarthritis (SpA) which may mimic ankylosing spondylitis (AS). Axial SpA is a unifying clinical term for chronic inflammatory spinal disorders, although biomechanical factors may play a role. The primary objective of this review is to critically describe the iHPoPT/SpA phenotype defined by established criteria and its differentiation from AS. Five databases were comprehensively searched without time limit to retrieve 14 (11M, 3F) iH-PoPT/SpA cases. Their demographic, clinical, laboratory, radiographic, and HLA-B27 status were compared to two national series of AS patients. Mean (SD) onset age of musculoskeletal symptoms [32.5 (9.7)] was significantly older than 943 German AS patients [25.1 (8.5), (p=0.004)] and 842 Spanish AS patients [26.1 (9.7), (p=0.030)]. Radiographic lesions of iHPoPT/SpA differ morphologically from skeletal alterations in hyperparathyroid and hypophosphataemic syndromes which often have inadequate bone mineralisation and decreased bone mineral density (BMD). Clinical musculoskeletal manifestations were greater (p<0.001) in iHPoPT/SpA than AS patients at cervical (62 vs. 10%) and hip (85 vs. 22%) localisations, respectively. Typical AS sacroiliac joint structural lesions of erosions and bony bridging were reported in only 1 iHPoPT/SpA case and HLA-B27 was positive in 2 of 10 tested. The iHPoPT/SpA phenotype may be a natural experiment on the novel concept of how chronic hypocalcaemia of iHPoPT causes axial neuromotor hypercontractility and biomechanically induces the rare SpA association. In iHPoPT/SpA, neuromuscular hyper-contractility may predispose to axial radiographic enthesopathy lesions and contribute knowledge on biomechanical contributions and pathways for further research.

Drug-induced hypocalcemia

Hypocalcemia (HCa) is one of the main water-electrolyte disturbances in clinical practice. An acute decrease in serum calcium levels can lead to seizures, ventricular arrhythmias, bronchospasm and laryngospasm. Chronic HCa can result in disorientation and confusion. To prevent these complications, the risk factors for low calcium levels must be carefully evaluated. One of these factors is drugs, in which case we are talking about drug-induced (DI) HCa. The list of drugs-inducers of DI HCa is quite extensive, but the leading role in this disorder is played by drugs for the treatment of osteoporosis, antineoplastic and antiepileptic drugs, as well as drugs for anti-tuberculosis therapy. When taking zoledronic acid, DI HCa is observed with a frequency of up to 39%. When taking imatinib, a targeted anticancer drug, a decrease in calcium levels was observed in 40% of cases. The pathophysiological mechanisms of DI HCa can be a decrease in bone resorption, a decrease in the concentration of vitamin D, inhibition of the action of parathyroid hormone and impaired calcium absorption. Risk factors in most cases of DI HCa are vitamin D deficiency and hypomagnesemia. An acute decrease in calcium levels leads to symptoms of neuromuscular excitability, abnormalities on the electrocardiogram (ECG) and electroencephalogram (EEG). The basis for the treatment of DI HCa is the drug withdrawal and the appointment of calcium. It is also necessary to prescribe vitamin D. The main methods of prevention of DI HCa are to determine the level of calcium and vitamin D before starting therapy with culprit medication, and to correct its level. It is also important to prescribe additional amounts of calcium and vitamin D during therapy with such drugs. Awareness of the attending physicians about the problem of DI HCa, a thorough assessment of its risk factors and the prophylactic administration of calcium and vitamin D preparations will help to effectively prevent those serious complications resulting from a decrease in calcium levels in clinical practice.

B-PO05-213 REVERSAL OF HYPOCALCEMIA-INDUCED DILATED CARDIOMYOPATHY TREATED WITH CRT-D

Hypocalcemia is a rare cause of dilated cardiomyopathy (DCM). Pathophysiology involves decreased myocyte contractility and prolongation of QTc interval. CRT-D is a proven therapy in patients with DCM. We present a novel case of hypocalcemic DCM and syncope that was refractory to GDM, including calcium replacement, that responded to CRT. N/A A 52-year-old woman with DCM and chronic hypocalcemia secondary to hypoparathyroidism presented for biventricular ICD placement. Hypocalcemia began after hemithyroidectomy. She was first diagnosed with heart failure after 10 years of hypocalcemia (mean 6.6 +/- .92 mg/dL). ECG was sinus rhythm with left bundle branch block (QRS 146 ms). TTE showed LVEF of 20% and 4-chamber enlargement. Cardiac catheterization did not show coronary artery disease. After 3 months of GDM and correction of calcium, she continued to have NYHA class III symptoms without change in EF. She was hospitalized for syncope concerning for malignant arrhythmia. QTc was 524 ms (JTc = 378 ms). Cardiac MR did not show evidence of fibrosis or scar. She had class 1b indication for CRT due to LVEF <35% and LBBB and underwent successful placement of Biotronik BiV ICD with LV lead placed into posterolateral branch. At 3-month follow-up she had 100% biventricular pacing. Her LVEF increased from 20% to 35%, LVIDd and LVIDs decreased by 15% (6.76 to 5.73 cc) and 24% (6.10 to 4.64 cc). After prolonged correction of calcium, QTc normalized (474 ms). CRT-D therapy has multiple indications in hypocalcemia-induced DCM, including improvement of LV size and function and prevention of fatal arrhythmias related to QTc prolongation.

A Novel GATA3 Variant Causing Familial Hypoparathyroidism, Renal Agenesis and Sensorineural Deafness Presenting With Atypical Symptoms of Chronic Hypocalcaemia

Introduction: Familial hypoparathyroidism is a rare cause of hypocalcaemia. We report a case of long-standing hypocalcaemia secondary to hypoparathyroidism caused by a novel GATA3 variant resulting in multiple organ involvement. Case: A 20 year old girl was referred to our bone metabolic clinic for hypocalcaemia. Her past medical history included Bechet’s disease, epilepsy and depression. She had bilateral sensorineural hearing loss and encephalitis as a child. She underwent right nephrectomy for an atrophic non-functioning kidney at the age of 16. Current medication included hydroxychloroquine, diazepam, oral calcium and cholecalciferol. It was noted that the hypocalcaemia dated back to 8 years, she denied any typical symptoms of hypocalcaemia but she did report visual and auditory hallucinations, fatigue and had low seizure threshold. She sustained recurrent fractures of her arm, elbow and wrist. Initial investigations: Corrected calcium 1.88 (2.20-2.60mmol/L), Phosphate 1.54 (0.80–1.50mmol/L), PTH 1.2 (1.6–6.9pmol/), 25-OH vitamin D 37 (50-120nmol/L). Myeloma screen, thyroid, renal and liver functions were all within the normal reference range. Other bone markers: Serum Procollagen Type 1 Amino Terminal Peptide was mildly raised at 82 (19-69ug/L), CTX 0.42 (0.1-0.5ug/L), 1,25 OH Vitamin D 29 (55-139pmol/L), 24,25-dihydroxyvitamin D was normal with normal 25:24,25 Dihyroxyvitamin D ratio at 18 normal. Bone density was in the normal range for her age. MRI of the brain was normal with no evidence of calcification. There was a family history of hypocalcemia in her estranged father. Subsequent genetic analysis showed a novel likely pathogenic GATA3 missense variant (c.961T>C p.(Cys321Arg). She was started on alfacalcidol and achieved near normocalcemia with adjusted calcium levels of 2.18nmol/L. Conclusion: Pathogenic variants in the GATA3 gene are responsible for Hypoparathyroidism-deafness-renal dysplasia (HDR) syndrome. In our patient, a novel missense variant in GATA3, p.(Cys321Arg), has been detected. This variant disrupts one of four conserved cysteine residues within a zinc-finger domain, which is involved in DNA binding and is presumed to have a deleterious effect on protein function. Patients may have longstanding asymptomatic hypocalcaemia with atypical features hence genetic testing is recommended in patient with multi-organ involvement. Alfacalcidol successfully restored calcium homeostasis in this case.

Parathyroid Cell Differentiation from Progenitor Cells and Stem Cells: Development, Molecular Mechanism, Function, and Tissue Engineering.

Parathyroid glands are endocrine organs which are located posterior to thyroid glands and control secretion of parathyroid hormone (PTH) in order to regulate blood calcium level. PTH maintains calcium homeostasis by acting on the bone, kidney, and small intestine. PTH deficiency leads to chronic hypocalcemia, organ calcinosis, kidney and heart failure, painful muscle spasms, neuromuscular problems, and memory problems. Since parathyroid cells have inadequate proliferation potential in culture conditions, their utilization as a cellular therapy option is very limited. Although studies conducted so far include parathyroid cell differentiation from various cell types, problems related to successful cellular differentiation and transplantation still remain. Recently, parathyroid tissue engineering has attracted attention as a potential treatment for the parathyroid-related diseases caused by hypoparathyroidism. Although major progression is made in the construction of tissue engineering protocols using parathyroid cells and biomaterials, PTH secretion to mimic its spontaneous harmony in the body is a challenge. This chapter comprehensively defines the derivation of parathyroid cells from various cell sources including pluripotent stem cells, molecular mechanisms, and tissue engineering applications.

Significance of Basal ganglia calcification in Down's syndrome

Purpose There is high incidence of dementia in individuals with Down’s syndrome. Much of the emphasis has been on Alzheimer’s disease as being most prevalent; however, it is apparent that other dementia types are also likely, to which this patient cohort may be predisposed. Specifically, this paper aims to highlight the potential for subcortical dementias in Down’s syndrome, suggesting a role for broader cognitive screening in aging individuals. Design/methodology/approach This paper describes a case of a female with Down’s syndrome and mild intellectual disability who presented with early signs of distinctive cognitive impairment and radiological calcification of the basal ganglia. Findings An active 42-year-old lady, who was mostly independent of activities of daily living and in part-time employment, presented with a three-year history of progressive cognitive deficit, characteristic of subcortical decline. She had no personal or known family history of mental illness, epilepsy or dementia. Routine blood tests showed chronic renal impairment, mild hypocalcaemia and vitamin D deficiency, managed by her GP. CT scan showed only bilateral basal ganglia calcification. Originality/value There is a widespread appreciation for the link between Down’s syndrome and Alzheimer’s disease but lesser consideration of the possibility of subcortical dementias. Given the differential nature and presentation of the two dementias, this case report highlights a need for clinicians to consider both to effectively manage these patients in the longer-term. Screening is discussed as a potential means of achieving this.

MON-352 When Osteoporosis Does Not Make Sense: Tumor-Induced Osteomalacia

Background: Tumor-induced Osteomalacia (TIO) is a rare paraneoplastic disorder that relates to excess FGF-23 secretion from mesenchymal tumors. Typical presentations include weakness, severe bone pain, but more importantly; multiple false or true fractures. Severe Hypophosphatemia with hyperphosphaturia is one of the pathognomonic findings due to FGF-23 action on renal tubules as phosphaturic agent. Multiple imaging modalities usually required as tumor can be in any size and anywhere in the body.Case: 58 yo male with past medical history of multiple fractures: bilateral rib cage, and metatarsal, dextroscoliosis who present to endocrine clinic due to low bone mineral density (BMD). On initial visit, screening bloodwork done was unremarkable except for mild elevation of Alkaline Phosphatase and extremely low phosphorus (1.2). 24 Urine phosphorus ordered which was elevated but it was thought to be nutrition related thus phosphorus replacement was prescribed (4 times per day). On follow up, repeat labs noted again with severe hypophosphatemia with elevated FePhos. DEXA was also performed which noted with T score -4.0 of lumbar spine. During that appointment, he informed also that currently in the process of evaluating right lung mass with biopsy resulted with benign mesenchymal tumor which he was happy to hear. TIO was then suspicious to be the cause of decrease BMD along with history of multiple fractures and height loss. Subsequently, FGF-23 staining was then made to the biopsy sample which was positive thus confirming the diagnosis. Patient then underwent surgical resection of the tumor which post-surgery, patient experienced overall symptoms improvement, resolution of phosphatemia, as well as significant improvement of BMD without additional therapy besides calcium and vitamin D.Discussion: Osteomalacia is a pathological bone disease involving poor mineralization of existing bone during remodeling phrase. It is commonly involved with chronic hypophosphatemia and/or hypocalcemia. Persistent decrease in calcium-phosphate product leads to reduction in bone mineralization activity and bone stiffness with Bowing deformities of the long bone being one of the common findings as well as false fracture.In TIO, FGF-23 is excessively secreted by mesenchymal tumor that is usually difficult to locate due to its small size. When FGF-Receptor is activated, NaPi-2a transcription is reduced in proximal tubule cells which leads to Phosphate excretion and wasting.TIO is a curable disease once tumor is located and surgically removed thus it is important for providers to be aware of it and pursue diagnostic work up when high suspicious. One of the most common reason for misdiagnosis is due to standard metabolic panel on its own does not include phosphorus level which is crucial. TIO has to be distinguish from osteoporosis as Bisphosphonates is not indicated as treatment which is first line for the latter.

MON-LB63 Hypocalcemia-A Clue to Digeorge Syndrome; A Very Rare Case Diagnosed in a 41 Year Male

41-year-old male with history of anxiety, bipolar disorder, depression, cognitive developmental delay, Idiopathic Thrombocytopenic Purpura (ITP), Vit D deficiency, tinnitus, suicidal attempt, auditory hallucination, borderline intellectual functioning comes to the PCP’s office for regular follow-up. The PCP has noted that the patient was hypocalcemic on multiple visits. On exam, vitals were in normal range, height 5 feet 2 inches, BMI 31 kg/m2.Despite the patient being on calcium and Vit D2 pills for a couple of months, his Calcium level were low ranging from 5.8–7.8mg/dl (normal 8.5-10.1) with normal albumin; ionized calcium was low 0.9-0.97mmol/l (normal 1.12-1.23); Magnesium was normal 2mEq/L (normal 1.5-2.5), phosphorus slightly high 5 mg/dL (normal 2.5-4.9), PTH (ParaThyroid Hormone level) was low-normal 18.8pg/ml (normal 11.1-79.5), vitamin D 25 OH low 16ng/ml (normal 30-100); 1,25 di OH vitamin D low 8ng/ml (normal25-40).Thyroid ultrasound showed 2 benign nodules, no further work-up done. TSH and free T4 were normal. FISH (Fluorescence In Situ Hybridization) came positive for DiGeorge syndrome (deletion at 22q11.2). He was treated with calcium 600 mg 4 pills daily, calcitriol 0.25 mcg two pills daily, Vit D2 50000 IU weekly and thiazide diuretic. His labs improved.DISCUSSIONHypocalcemia can be due to low magnesium level, drugs or associated with high or low PTH. The patient had normal magnesium and he was not on any medication that causes hypocalcemia. This rules out first two casues. Hypocalcemia with high PTH (pseudohypoparathyroidism or low vit D levels) doesn’t fall in our differential because our patient had low PTH. For hypocalcemia with low PTH, differentials include post-surgical condition (no surgical history), autoimmune (history and labs not suggestive of), infiltrative diseases (eg hemochromatosis- he had normal iron study, Wilson disease- normal copper level, granulomas- contrary he had low 1,25 Di OH vit D). Hypocalcemia secondary to genetic parthyroid gland anomaly was thought to be most likely in our patient. So FISH was pursued.Conclusion: For patients with cognitive issues, persistence of chronic hypocalcemia (with low PTH) despite treatment should prompt for genetic disorders like DiGeorge. DiGeorge is usually the diagnosis of children. Perhaps this is the first case of DiGeorge diagnosed so late at age of 41.

SAT-354 Isolated Hypoparathyroidism: A Rare Initial Manifestation of Hereditary Hemochromatosis (HH)

Background:We present a case of hypoparathyroidism diagnosed in a patient as initial manifestation of hereditary hemochromatosis. As per our literature search, it is very rarely reported as an isolated abnormality in HH.Case:A 27 year old male with history of seizure disorder, well controlled on Oxcarbazepine, referred for evaluation of chronic hypocalcemia. His PMH includes cerebral palsy, with good functional capacity and mild cognitive impairment. He denied muscle spasm, perioral numbness, bone pain, muscle weakness or fracture. He was taking calcium carbonate 600mg bid and vitamin D 50,000 IU q2monthly.There was no family history of any Ca disorders. Physical exam was unremarkable.Laboratory evaluation revealed Ca levels ranging from 7.6 to 8.5mg/dl with intact PTH values ranging from 11 to 22pg/ml. His ionized calcium was also low. Patient’s 24 hr urinary Ca was 122mg/24hrs. In this case, he did initially have hypomagnesemia and vitamin d deficiency, which could potentially explain low calcium. But even after supplementing Vitamin D and Mg, patient’s Ca remained low. His kidney function was normal. Hemoglobin was in range of 14-15g/dl. To further evaluate the cause of hypoparathyroidism, iron saturation and iron levels were also sent. His iron saturation % was high at 89% with iron level of 286 ug/dl. His ferritin was 224 ng/ml and TIBC was 265 ug/dl. A sample was sent for genetic analysis to rule out hemochromatosis. Homozygous mutations in C282Y gene were found. A diagnosis of hereditary hemochromatosis was made. His other entire hormonal axis was intact. In this case, patient’s hypoparathyroidism is likely an initial manifestation of his HH.Discussion:Hereditary Hemochromatosis (HH) is a genetic disease characterized by an excessive (unregulated) entry of iron into the bloodstream with increased iron deposition in the parenchymal cells of a variety of organs leading to their failure. A defect in the hemochromatosis gene (HFE) is the most common form of HH, also known as the classic form or type 1 HH, where the principal mutation is represented by a substitution of tyrosine for cysteine at position 282 of the HFE gene (C282Y) as seen in our case. According to the genetic forms, the clinical manifestation usually ranges from simple biochemical abnormalities to severe organ damage and disease such as liver cirrhosis, arthritis, DM, cardiomyopathy and hypogonadism.There are reports of hypoparathyroidism from iron overload seen in thalassemia patients and patients who receive long term blood transfusions. In our literature review, this is the first documented case of HH initially manifesting as hypoparathyroidism. As HH is not uncommon in Caucasians, the work up for hemochromatosis as a possible cause of endocrinopathies should be kept in the differential diagnosis. It will help in early diagnosis & treatment which can reverse the effects of the disease leading to better outcomes.

Diagnosis and management of hypocalcemia.

The aim of this clinical narrative review is to summarize and critically appraise the literature on the differential diagnosis of hypocalcemia and to provide its correct management. Calcium is essential for muscle contraction and neurotransmitter release, but clinical manifestations of hypocalcaemia (serum calcium level <8 mg/dl; 2.12 mmol/L) may involve almost any organ and system and may range from asymptomatic to life-threating conditions. Disorders causing hypocalcemia can be divided into parathyroid hormone (PTH) and non-PTH mediated. The most frequent cause of hypocalcemia is postsurgical hypoparathyroidism, while a more comprehensive search for other causes is needed for appropriate treatment in the non PTH-mediated forms. Intravenous calcium infusion is essential to raise calcium levels and resolve or minimize symptoms in the setting of acute hypocalcemia. Oral calcium and/or vitamin D supplementation is the most frequently used as treatment of chronic hypocalcemia. In hypoparathyroidism, providing the missing hormone with the use of the recombinant human (rh) PTH(1-84) has been recently approved both by the Food and Drug Administration (FDA) and the European Medicines Agency (EMA). This new therapy has the advantage of being effective for correcting serum calcium levels and significantly reducing the daily requirements of calcium and active vitamin D supplements. However, due to the high cost, a strict selection of candidates to this therapy is necessary. More challenging is the long-term hypocalcemia treatment, due to its associated complications. The development of long-acting recombinant human PTH will probably modify the management of chronic hypoparathyroidism in the future.

Management of parathyroid disorders: recommendations of the working group of the Bone Section of the Hellenic Endocrine Society.

The Bone Section of the Hellenic Endocrine Society has issued the recommendations herein presented with the aim of providing guidance on optimal management of patients with parathyroid disorders in everyday clinical practice within the Greek health care setting. Although the methodology followed to formulate these recommendations was not strictly based on the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) principles, they were drawn up after an extensive review of the literature and of the currently available guidelines for the management of parathyroid disorders worldwide. Specifically for primary hyperparathyroidism (PHPT), the 2011 guidelines of the Greek National Organization of Medicines were updated accordingly. In particular, definitions, etiologies, and recommended and optional laboratory and imaging examinations are provided both for PHPT and chronic hypoparathyroidism (HypoPT). Finally, treatment algorithms are provided for the management of both PHPT and HypoPT. Specifically for HypoPT, the treatment algorithm describes the recommended steps that should be followed to achieve optimal management of chronic hypocalcemia and the complications of HypoPT through the conventional treatment available in Greece and the use of recombinant human PTH(1-84).

Wavy Triple an Electrocardiographic Sign (Yasser Sign) in Hypocalcemia with Tented T-wave and Chronic Renal Failure; Diverse Management - A Case Report

Rationale: Electrolyte disorders is a noteworthy entity in clinical practice. Hypocalcemia is a well-known serious electrolyte disorder. A Wavy triple sign of hypocalcemia (Yasser sign) is a novel an electrocardiographic sign linked to calcium deficiency. Tented T-wave is frequently identified in chronic kidney disease. Hyperkalemia is a lethal metabolic disturbance that may be a result of kidney failure. Patient concerns: An old-aged male carpenter patient presented to the emergency department with hyperventilation syndrome, chest pain, and tetany. Diagnosis: Chronic renal failure-induced hypocalcemia with subsequent a Wavy triple an electrocardiographic sign or Yasser sign. Interventions: Electrocardiography, arterial blood gases, oxygenation, and echocardiography. Lessons: The dramatic reversal of the wavy triple sign of hypocalcemia (Yasser sign) after calcium gluconate injection interpret that these signs were due to hypocalcemia of chronic renal failure. Tented T-wave is not necessary occurs by hyperkalemia. Outcomes: There was a dramatic improvement of both clinical and electrocardiographic wavy triple sign of hypocalcemia after calcium injection.

CASE REPORT : FAHR'S SYNDROME ON POST THYROIDECTOMY PATIENT

Background: Fahr’s syndrome is slowly progressive, a neurodegenerative disorder, characterized by bilateral symmetrical calcium deposits of ganglia basalis. Fahr’s syndrome is the secondary form of brain calcification that is caused by various metabolic, infectious or degenerative disease. The most common metabolic disorders that cause Fahr’s syndrome is hypoparathyroidism, which is characterized by phosphate and calcium abnormalities. Case Illustration: a 65-year-old female patient who presented with the transient loss of consciousness, It suddenly happened, while she was sitting. The patient tends to be quiet and had no response when she was called. This complaint lasts for 1 minute. Previously, this complaint had happened 30 years ago. The patient had undergone a thyroidectomy 35 years ago. She started having a seizure which progressed 5 years after surgery. In the emergency department, the patient was good oriented and full of consciousness. The neurology examination reveals no abnormal findings. The patient had no motor or sensory symptoms. Serum calcium level was low (4,1 mg/dL) and PTH levels was extremely low (5,53pg/ml). Computed tomography scan of the brain showed intracranial calcification in the basal ganglia, suggestive for Fahr’s syndrome. Discussion: Hypoparathyroidism is a complication that is often found in thyroidectomy. It can lead to increased calcinosis, and it believed that calcium deposition begins in the vessel wall, eventually extending to the neuron. Conclusion: Chronic hypocalcemia due to postoperative hypoparathyroidism can remain subclinical for a long time. Timely recognition of idiopathic and iatrogenic hypoparathyroidism allows appropriate treatment that can prevent the development and clinical manifestations of Fahr’s syndrome and potentially slow its progression.

A Case of Complex Hematoma Formation After Institution of ACS Protocol in a Patient with Untreated Primary Hypoparathyroidism

We report an unusual case of untreated hypoparathyroidism in which the patient presented with a prolonged QT interval and unusual bleeding after the institution of acute coronary syndrome (ACS) protocol. A 53-year-old female presented with sudden pain and diffuse tightness in the abdomen/limbs and profuse sweating for a few hours. Patient was admitted under cardiology services and ACS protocol was instituted. After 2 days of admission, she developed severe abdominal pain and distention, which was due to large hematomas in the bladder wall, rectus sheath, and retroperitoneal area. These hematomas were surgically drained, but the abdominal wall could not be closed due to gut distention and stiffness of the abdominal wall; a Bogota bag was applied for closure. The patient was shifted to oral calcium after 12 days of intravenous calcium and vitamin D replacement. Abdominal closure was done several weeks later as a follow-up procedure. This case illustrates the neglected areas of the impact of hypoparathyroidism; the effects of chronic hypocalcemia on the cardiovascular system and coagulation cascade.

MON-193 RENAL TRANSPLANTATION IN A PATIENT WITH FAMILIAL SYNDROME OF HYPOCALCEMIA WITH HYPERCALCIURIA

The calcium sensing receptor regulates the secretion of parathyroid hormone in response to changes in the extracellular calcium concentration and mutations that result in gain of function of this receptor are associated with with familial hypocalcemia with hypercalciuria. The high urinary calcium excretion leads to nephrocalcinosis and renal insufficiency. We present a case of a patient with end stage kidney disease due to nephrocalcinosis from familial hypocalcemia with hypercalciuria, who subsequently underwent renal transplantation with complete resolution of hypercalciuria. This is the first reported case of renal transplantation in a patient with familial hypocalcemic hypercalciuria. Case report: 58 year old female developed end stage kidney disease due to nephrocalcinosis. Nephrocalcinosis was secondary to familial hypoparathyroidism and she had known mutations in calcium sensing receptors and hence chronic hypocalcemia (1.8-1.9mmol/l) and hypercalciuria( 3.2mmol/l) . She subsequently underwent non directed living donor transplantation. Within 1 week after renal transplantation the urinary calcium concentration was measured and this was completely within normal limits with urine calcium concentration of 1 mmol/l and calcium/creatinine ratio of 0.29 ( normal range 0.06- 0.45mole ratio). Subsequent urine calcium concentrations are less than 0.20mmol/l and her graft function remains excellent. Discussion: This is the first case report of successful renal transplantation in a case of familial hypoparathyroidism with known gain of function or activating mutation of calcium sensing receptor which results in hypocalcemia and hypercalciuria. The calcium sensing receptor gene is expressed in the parathyroid glands and kidneys . This receptor regulates the the secretion of parathyroid hormone and reabsorption of calcium by the renal tubules in response to alterations of serum calcium levels. Gain of function mutation of the calcium sensing receptor gene will suppress the parathyroid hormone and increase the excretion of urinary calcium at inappropriately low serum calcium levels. The high urinary calcium concentration drives polyuria and dehydration and hence subsequently leads to nephrocalcinosis and renal insufficiency. With renal transplantation, we are essential replacing the affected abnormal calcium sensing receptors with normal calcium sensing receptor and hence normalising urinary calcium, thereby eliminating the risk of nephrocalcinosis in the graft. Conclusions: Renal transplantation is a safe and effective way of treating end stage kidney disease in patients with nephrocalcinosis due to familial hypocalcemia and hypercalciuria.

Interrelated role of Klotho and calcium-sensing receptor in parathyroid hormone synthesis and parathyroid hyperplasia

The pathogenesis of parathyroid gland hyperplasia is poorly understood, and a better understanding is essential if there is to be improvement over the current strategies for prevention and treatment of secondary hyperparathyroidism. Here we investigate the specific role of Klotho expressed in the parathyroid glands (PTGs) in mediating parathyroid hormone (PTH) and serum calcium homeostasis, as well as the potential interaction between calcium-sensing receptor (CaSR) and Klotho. We generated mouse strains with PTG-specific deletion of Klotho and CaSR and dual deletion of both genes. We show that ablating CaSR in the PTGs increases PTH synthesis, that Klotho has a pivotal role in suppressing PTH in the absence of CaSR, and that CaSR together with Klotho regulates PTH biosynthesis and PTG growth. We utilized the tdTomato gene in our mice to visualize and collect PTGs to reveal an inhibitory function of Klotho on PTG cell proliferation. Chronic hypocalcemia and ex vivo PTG culture demonstrated an independent role for Klotho in mediating PTH secretion. Moreover, we identify an interaction between PTG-expressed CaSR and Klotho. These findings reveal essential and interrelated functions for CaSR and Klotho during parathyroid hyperplasia.

Life-Threatening Hypocalcemia in A Patient with highly suspected osteomalacia: A Case Report

ABSTRACT Objective: Osteomalacia is a metabolic bone disease characterized by impaired mineralization with increased non-mineralized osteoid tissue, increased frailty, and reduced bone mineral density. A common cause of osteomalacia in adults and the elderly is severe deficiency of vitamin D, which leads to chronic hypocalcemia, hypophosphatemia, and secondary hyperparathyroidism. The objective of this case report is to describe an unusual clinical presentation of osteomalacia, consisting of life-threatening acute hypocalcemia. Methods: Clinical, laboratory, and imaging data are presented. Results: We report the case of a 65-year-old man that showed symptoms and signs of severe and prolonged hypocalcemia due to unrecognized vitamin D deficiency. He presented at the emergency room reporting abdominal pain and vomiting since the evening before. Blood tests showed increased levels of rhabdomyolysis markers, severe hypocalcemia, hypophosphatemia, hypomagnesemia, normal renal function, elevated levels of alkal...