Diagnosis Of Idiopathic Hypoparathyroidism Research Articles

Idiopathic Hypoparathyroidism Presenting with Erythrodermic Psoriasis and Fahr’s Syndrome: A Case Report

Idiopathic hypoparathyroidism may remain asymptomatic or present with hypocalcaemic neuromuscular manifestations. Dermatological manifestations like exfoliative dermatitis, pustular psoriasis and symmetrical brain calcification known as Fahr’s syndrome are uncommon in clinical practice. Even in absence of neuromuscular symptoms, hypocalcaemia associated with exfoliative skin lesions and symmetrical brain calcification should prompt investigation for hypoparathyroidism. A 40-year-old male presented with diffuse erythematous pruritic plaques and fever of 10 days duration. Auspitz sign was positive on examination. Investigations showed leucocytosis with neutrophilic predominance. He was admitted with a diagnosis of erythrodermic psoriasis with secondary infection. On the second day of admission he developed an episode of generalised tonic clonic seizure for the first time. Investigations into the cause of seizure revealed low serum calcium while computerised tomography of head revealed symmetrical calcification of bilateral basal ganglia. Further, he was found to have a low level of intact Parathyroid Hormone (PTH) level and hyperphosphataemia. Based on clinical, biochemical and radiological findings, a diagnosis of idiopathic hypoparathyroidism, psoriasis and Fahr’s syndrome was made. Serum calcium was corrected with infusions of calcium gluconate followed by oral calcium carbonate. The patient was kept under observation for seizure recurrence. Calcium and vitamin D supplements and hydrochlorothiazide were started and psoriasis treatment was continued. His skin lesions resolved over the next two weeks, serum calcium levels returned to normal level and no seizure episodes were reported over three months of follow-up.

Basal ganglia calcifications is not inconsequential in pediatric cases

Introduction. Basal ganglia calcification (BGC) in pediatric population is rare and is considered as a pathological finding. Various causes may be responsible for BGC including hypoparathyroidism, various infectious, toxicities or hereditary disorders. Aim. We aimed to present a 8 year old boy presented with generalized seizure and bilateral small amount of globus pallidum calcifications on neuroimaging studies leading to the diagnosis of idiopathic hypoparathyroidism, which is a treatable cause of seizure. Description of the case. A 8-year-old boy presented to our emergency department with generalized seizure for the first time in his life. There was no history of previous head trauma and his family history was unremarkable. Neurological examination revealed no pathological findings. Radiological imaging studies revealed only bilateral small amount of globus pallidus calcifications. He was referred to the pediatric endocrinology department for further evaluation of the hypocalcemic convulsion, where laboratory investigations revealed idiopathic hypoparathyroidism as the cause of hypocalcemic convulsion with exclusion of the other causes. Conclusion. Even a small amount of BGC in pediatric patients may be the sign of primary hypoparathyroidism and should be evaluated with serum electrolyte levels for early diagnosis and for the prevention of multisystemic complications of hypoparathyroidism.

Hip Pain and Gait Disturbance Associated with Idiopathic Hypoparathyroidism.

Idiopathic Hypoparathyroidism (IHP) is a disease characterized by diminishes in production of parathyroid hormone due to dysfunction of the parathyroid glands [1]. IHP is characterized by soft-tissue ectopic calcifications and skeletal abnormalities. Subcutaneous calcifications around the hips and shoulders may be seen in the course of this disease [2]. Muscle cramps involving the back and legs, paresthesias, and convulsions are common complaints. The clinical manifestations of this condition are varied and nonspecific. Consequently, IHP is usually diagnosed many years after its onset [3]. Back and hip pain and stiffness are is commonly overlooked in these patients [1]. We hereby report a case of a 40-year-old man who presented with bilateral hip pain that had hindered his normal walking for 6 months. He also had a 30-year history of epilepsy and cataract surgery 10 years earlier. His family history was non-contributory. He had no history of surgical excision or damage to the parathyroid glands, radiation, or neoplastic/granulomatosis infiltration. He had no muscle weakness or cranial nerve involvement and no difficulty with speech, apathy, or mental slowness. The FADIR (flexion, abduction, and internal rotation) and FABERE (flexion, abduction, external rotation, and extension) tests were positive and painful at 15° of flexion due to hip contractures. Laboratory results demonstrated the typical findings of hypoparathyroidism: parathormone value 2 (normal level = 15–65) pg/mL, serum calcium 4.6 (normal level= 8.4–10.6) mg/dL, and phosphate 7.2 (normal level = 2.3–4.7) mg/dL. Pelvis radiography revealed bilateral ossification extending along the acetabulum [Table/Fig-1]. Computed tomography of the brain showed calcifications in the pallidum and caudate nuclei bilaterally [Table/Fig-2]. The electroencephalogram was normal. [Table/Fig-1]: Pelvis radiography shows bilateral ossification around the acetabulum. Note the preservation of the joint space. [Table/Fig-2]: Head computed tomography shows bilateral symmetrical calcifications in the pallidum and caudate nuclei. No other cause of hypoparathyroidism was found. Tests for polyendocrinopathy were normal. A diagnosis of idiopathic primary hypoparathyroidism was made. Calcium, vitamin D supplementation, paracetamol 500 mg tid, diclofenac sodium 50 mg bid, and physical therapy with range of motion and strengthening exercises were started and continued until the pain limit was reached. These therapies reduced the severity of his complaints within 1 week. IHP causes hypocalcaemia by hindering calcium reabsorption in the renal tubules and bone, and to a insufficiency in the production of 1,25-dihydroxyvitamin D. The most frequent osseous findings of IHP are localized or generalized sclerosis. Extraosseous calcification is typically seen in the basal ganglia, periarticular tissue, blood vessels, and ocular lens. The development of calcification may be associated not only parathyroid hormone deficiency but also to the duration of hypocalcaemia and hyperphosphataemia [3]. The imaging findings of IHP closely resemble those of diseases such as diffuse idiopathic skeletal hyperostosis and ankylosing spondylitis [1–5]. Our patient’s main complaint was pain and gait difficulty and the diagnosis of IHP was delayed until the age of 40 years. Delaying diagnosis and treatment resulted in cataracts, speech difficulty, pain, limited range of motion of the hip and gait disturbance. Disclosures: Financial disclosure statements have been obtained, and no conflicts of interest have been reported by the authors or by any individuals in control of the content of this article.

Dental findings in the diagnosis of idiopathic hypoparathyroidism

Idiopathic hypoparathyroidism (IHP) is a rare endocrinopathy, characterized by the disturbances in calcium and phosphorous metabolism, owing to deficiency in parathyroid hormone, which leads to tetanic manifestations. Onset of the clinical features occurs early in life, and the severity depends on the extent of chemical imbalance. This article describes a case of 22-year-old patient undiagnosed for 12 years with this endocrinopathy (IHP). Over retained deciduous teeth, delayed eruption, impacted tooth and short roots probably resulting from untreated hypocalcemia during the developmental phase of dentition enabled us to unearth this endocrinopathy through a series of investigations. Thus the article emphasizes the importance of dental findings of this endocrinopathy.

Idiopathic hypoparathyroidism as a cause of extensive intracranial calcification.

A 60-year-old male was referred to radiology department for head CT for evaluation of seizures of recent onset. CT showed extensive symmetric bilateral calcification in caudate nucleus, putamen, thalami, cerebellum and centrum semiovale (Fig. A,B). A radiological diagnosis of Fahr’s disease was made. During hospital stay, patient developed muscular tetany. Serum calcium was found to be low (7.2 mg/dl; normal range 8.4-10.2 mg/dl). Further tests revealed low serum parathyroid hormone (PTH) level (3.60 pg/ml; normal range 15.0-68.0 pg/dl) and elevated phosphorus level. All possible causes of hypoparathyroidism were evaluated with no positive results. On the basis of clinical, hormonal and radiological findings a diagnosis of idiopathic hypoparathyroidism was made. Patient was started calcium and 1,25-dihydroxy vitamin D. Patient reported no episode of seizures during next two days of hospital stay. He was last seen at 3 months follow up with significant improvement of clinical symptoms and serum calcium level returning to normal range.

Role of Dental Findings in the Diagnosis of Idiopathic Hypoparathyroidism

Idiopathic hypoparathyroidism (IHP) is a rare endocrinopathy, characterized by the disturbances in the calcium and phosphorous metabolism, owing to deficiency in parathyroid hormone, which leads to tetanic manifestations. Onset of the clinical features occurs early in the life and the severity depends on the extent of chemical imbalance. This article describes a case of 22-year-old male patient undiagnosed for 12 years with this endocrinopathy (IHP). Overretained deciduous teeth, delayed eruption, impacted tooth and short roots probably resulting from untreated hypocalcemia during the developmental phase of dentition enabled us to unearth this endocrinopathy through a series of investigations. Thus, the article emphasizes the importance of dental findings of this endocrinopathy.

Idiopathic Hypoparathyroidism Mimicking Diffuse Idiopathic Skeletal Hyperostosis

Idiopathic hypoparathyroidism is an uncommon disease caused by insufficient secretion of parathyroid hormone. Idiopathic hypoparathyroidism may cause various musculoskeletal findings, including diffuse ligamentous and entheseal ossifications. Diffuse idiopathic skeletal hyperostosis is a disorder of the elderly characterized by ossification of the anterior longitudinal ligament of the spine and various extraspinal ligaments. We present a 50-year-old man with idiopathic hypoparathyroidism who had been diagnosed as having DISH at 40 years of age. Resistant neck, left shoulder, and left hip pain did not improve with calcium and calcitriol treatment after diagnosis of idiopathic hypoparathyroidism.

A Case of Idiopathic Hypoparathyroidism Complicated with Chronic Thyroiditis

It has been suggested that autoimmunity and genetic factors may play a specific role in the development of idiopathic hypoparathyroidism. We reported a case of idiopathic hypoparathyroidism complicated with chronic thyroiditis. The patient, a woman 40 years old, visited our clinic because of tetany of both hands and dizziness. She was of short stature with a round face. She also had a goiter, hypocalcemia, hyperphosphatemia and decreased parathyroidal function, but renal function was normal. Her TSH level was slightly high with a positive microsome test (x 1600), and the levels of thyroid hormones tended to be low. Based on Ellsworth-Howard test findings, a diagnosis of idiopathic hypoparathyroidism was made, with the complication of chronic thyroiditis confirmed by the thyroidal biopsy. Administration of l alpha-OH-D3 normalized the level of serum calcium. No special treatment was given for the chronic thyroiditis in order to observe its natural course. Her TSH returned to normal, and the level of thyroid hormones was increased to normal ranges. Tests were positive for anti-adrenal antibody and anti-gastric antibody. The complication of chronic thyroiditis, an autoimmune disease, and a positive finding for every antibody suggested the possible involvement of autoimmunity in the mechanism of development of idiopathic hypoparathyroidism. The administration of 1 alpha-OH-D3 resulted in an increase in the serum calcium level and also normalization of levels of TSH and thyroid hormones. Thus, it is likely that the elevation of the calcium ion or immunoregulation by active vitamin D may have induced the increase in thyroid hormone secretion.

Response of plasma 1,25-dihydroxyvitamin D in the human PTH(1–34) infusion test: An improved index for the diagnosis of idiopathic hypoparathyroidism and pseudohypoparathyroidism

Synthetic human parathyroid hormone (1-34) (hPTH(1-34] infusion test has been utilized in the differential diagnosis of hypoparathyroidism by examining the incremental response of urinary phosphate and cyclic adenosine monophosphate (AMP). The response of plasma levels of 1,25-dihydroxyvitamin D (1,25(OH)2D) in parathyroid hormone (PTH) infusion test was studied as a new criterion for the differential diagnosis of idiopathic hypoparathyroidism (IHP) and pseudohypoparathyroidism (PHP). Fourteen patients with IHP, 4 patients with PHP, and five control subjects were studied. All subjects received an intravenous infusion of 30 micrograms hPTH(1-34) over 5 minutes. The basal levels of plasma 1,25(OH)2D in patients with IHP and PHP were significantly lower than those in control subjects, but there was no significant difference between the levels in patients with IHP and in patients with PHP. The plasma levels of 1,25(OH)2D increased after the infusion of hPTH(1-34) and reached a peak 6 to 24 hours afterward. The 1,25(OH)2D increase at 24 hours afterward the infusion (delta 1,25(OH)2D) in control subjects and in patients with IHP were 18.1 +/- 3.91 (mean +/- SEM) and 24.1 +/- 2.80 pg/ml, respectively. There was no significant increase in patients with PHP (delta 1,25(OH)2D = 4.9 +/- 1.97 pg/ml). From these results, the measurement of delta 1,25(OH)2D in hPTH(1-34) infusion test is useful as a criterion for the differential diagnosis of hypoparathyroidism.

Idiopathic hypoparathyroidism: A report of four cases

FOUR cases of idiopathic hypoparathyroidism are presented. In three of these cases there was evidence of familial endocrinopathy (cases 2, 3, and 4) and in two (cases 3 and 4) there was evidence of multiple autoimmune endocrine disease. The diagnosis of idiopathic hypoparathyroidism and some interesting features of the disease and its treatment are discussed.

An unusual case of idiopathic hypoparathyroidism, adrenal insufficiency, hypothyroidism and metastatic calcification.

ABSTRACT A case of idiopathic hypoparathyroidism, adrenal insufficiency and hypothyroidism is presented. The patient, a female, was 3½ years old when the diagnosis of idiopathic hypoparathyroidism was first made. At the age of 5½ the first evidence of adrenal insufficiency developed, and the picture was complete one and a half years later. At this time hypothyroidism had developed, which proved to be primary in nature. This patient demonstrated roentgenographic calcification of the lungs, in the absence of abnormally high serum calcium levels or calcium-phosphorus concentration products, and without evidence of local tissue damage due to a chronic inflammatory process.

Idiopathic hypoparathyroidism and pseudohypoparathyroidism; observations on the electroencephalogram in hypocalcemia.

IDIOPATHIC hypoparathyroidism is considered a rare disease. 1 In the American literature, 34 cases had been recorded up to February, 1945. 2 Lachmann 3 reviewed the American and foreign literature, covering the period from 1926 to 1939, and found 48 cases in which the diagnosis of idiopathic hypoparathyroidism seemed justified. He reported an additional 22 cases from the records of various hospitals in Denmark between 1924 and 1939. However, his criteria for the diagnosis seem questionable, since 8 of the 22 patients recovered spontaneously. The cases enumerated by Winer 2 do not include any instances of pseudohypoparathyroidism, which are even rarer. Albright and Reifenstein 1 observed four such cases at the Massachusetts General Hospital, three of which had previously been reported. 4 Six additional cases have been reported subsequently. 5 Some further idea of the rarity of these two conditions may be gained from the following data: Kings County Hospital,

STUDIES ON THE PHYSIOLOGY OF THE PARATHYROID GLANDS

In order to determine, if possible, the exact train of events which lead to a rise in the serum calcium following the injection of a potent parathyroid extract, we undertook the present series of investigations. The subject of this study was an Italian boy on whom the diagnosis of idiopathic hypoparathyroidism was made. The criteria on which such a diagnosis can be based will be discussed below. The injection of a potent parathyroid extract results in four wellestablished changes and any theory as to the action of a parathyroid extract will have to take cognizance of these four cardinal points. They are: a. Rise in serum calcium (1). b. Rise in urinary calcium excretion (2) (3) (4). c. Fall in serum phosphorus (5). d. Rise in urinary phosphorus excretion (2) (3). Removal of the parathyroid glands results in the converse changes, i.e., a. Fall in the serum calcium. b. Fall in the urinary calcium excretion. c. Rise in the serum phosphorus. d. Fall in urinary phosphorus excretion. In addition Albright, Bauer, Ropes, and Aub (3) emphasize the fact that on administration of parathormone, the increase in urinary phosphorus excretion tends to precede the increase in calcium excretion. They further point out that the increased phosphorus appearing in