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John P Bilezikian - One of the best experts on this subject based on the ideXlab platform.
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quality of life in Hypoparathyroidism improves with rhpth 1 84 throughout 8 years of therapy
The Journal of Clinical Endocrinology and Metabolism, 2019Co-Authors: Gaia Tabacco, Natalie E Cusano, John C Williams, Beatriz Omeragic, Rukshana Majeed, Maximo Gomez Almonte, Mishaela R Rubin, John P BilezikianAbstract:CONTEXT: Calcium and vitamin D treatment does not improve reduced quality of life (QOL) in Hypoparathyroidism. Recombinant human (rh) PTH(1-84) therapy improves QOL metrics for up to 5 years. Data on QOL beyond this time point are not available. OBJECTIVES: To evaluate the effects of 8 years of rhPTH(1-84) therapy on QOL and factors associated with long-term benefit. DESIGN: Prospective, open-label trial. SETTING: Referral center. PATIENTS: Twenty patients with hypoparathyoidism. MAIN OUTCOME MEASURES: RAND 36-Item Short Form Health Survey (SF-36). RESULTS: rhPTH therapy led to substantial improvement in five of the eight SF-36 domains [vitality, social functioning (SF), mental health (MH), bodily pain (BP) and general health] and three of these domains (SF, MH, BP) were no longer lower than the reference population. The improvement in the mental component summary (MCS) score was sustained through 8 years, while the physical component summary (PCS) score improved through 6 years. A lower baseline QOL score was associated with greater improvement. A threshold value <238 (MCS) and <245 (PCS) predicted long-term improvement in 90% and 100% of the cohort, respectively. In patients whose calcium supplementation was reduced, MCS and PCS scores improved more than those whose supplementation did not decline to the same extent. Improvement in PCS was greater in patients whose calcitriol dosage was reduced and duration of disease was shorter. CONCLUSIONS: rhPTH(1-84) improves long-term well-being in Hypoparathyroidism. The improvements are most prominent in those with impaired SF-36 at baseline and those whose requirements for conventional therapy decreased substantially.
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Signs and Symptoms of Hypoparathyroidism.
Endocrinology and Metabolism Clinics of North America, 2018Co-Authors: Natalie E Cusano, John P BilezikianAbstract:Hypoparathyroidism is associated with a spectrum of clinical manifestations in the acute and chronic settings, from mild to debilitating. Although the acute symptoms of hypocalcemia are primarily due to neuromuscular irritability, the chronic manifestations of Hypoparathyroidism may be due to the disease itself or to complications of therapy or to both. The chronic complications of Hypoparathyroidism can affect multiple organ systems, including the renal, neurologic, neuropsychiatric, skeletal, and immune systems. Further research is needed to determine the pathophysiology of complications in Hypoparathyroidism and whether interventions can decrease the risk of these complications.
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new directions in treatment of Hypoparathyroidism
Endocrinology and Metabolism Clinics of North America, 2018Co-Authors: Gaia Tabacco, John P BilezikianAbstract:The history of parathyroid hormone (PTH) replacement therapy for Hypoparathyroidism begins in 1929. In 2015, the Food and Drug Administration approved recombinant human PTH(1–84) [rhPTH(1–84)] as a treatment for Hypoparathyroidism. Long-term studies of rhPTH(1–84), up to 6 years, have demonstrated continued efficacy of this replacement agent. Approaches to optimize PTH treatment in Hypoparathyroidism include subcutaneous pump delivery systems, long-lived carrier molecules, and long-acting PTH analogues that show promise to prolong efficacy. Calcilytic compounds have been explored as a treatment for autosomal dominant hypocalcemia. Calcilytics are negative modulators of the calcium-sensing receptor and may present a therapeutic opportunity to increase endogenous PTH synthesis and secretion.
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Hypoparathyroidism
Nature Reviews Disease Primers, 2017Co-Authors: Michael Mannstadt, Rajesh V Thakker, John P Bilezikian, Bart L. Clarke, Fadil M. Hannan, Lars Rejnmark, Karen K Winer, Deborah M. Mitchell, Tamara J. Vokes, Dolores ShobackAbstract:Hypoparathyroidism is a disease characterized by inadequately low circulating levels of parathyroid hormone resulting in low calcium levels and increased phosphate levels in the blood. In this Primer, Mannstadt et al . summarize current knowledge of the prevalence, pathophysiology, clinical presentation and management of Hypoparathyroidism. Hypoparathyroidism is a disease characterized by inadequately low circulating concentrations of parathyroid hormone (PTH) resulting in low calcium levels and increased phosphate levels in the blood. Symptoms of the disease result from increased neuromuscular irritability caused by hypocalcaemia and include tingling, muscle cramps and seizures. The most common cause of the disease is inadvertent removal of, or injury to, the parathyroid glands during neck surgery, followed by genetic, idiopathic and autoimmune aetiologies. Conventional treatment includes activated vitamin D and/or calcium supplements, but this treatment does not fully replace the functions of PTH and can lead to short-term problems (such as hypocalcaemia, hypercalcaemia and increased urinary calcium excretion) and long-term complications (which include nephrocalcinosis, kidney stones and brain calcifications). PTH replacement has emerged as a new treatment option. Clinical trials using human PTH(1–34) and PTH(1–84) showed that this treatment was safe and effective in studies lasting up to 6 years. Recombinant human PTH(1–84) has been approved in the United States and Europe for the management of Hypoparathyroidism; however, its effect on long-term complications is still being evaluated. Clinical practice guidelines, which describe the consensus of experts in the field, have been published and recognize the need for more research to optimize care. In this Primer, we summarize current knowledge of the prevalence, pathophysiology, clinical presentation and management of Hypoparathyroidism.
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presentation of Hypoparathyroidism etiologies and clinical features
The Journal of Clinical Endocrinology and Metabolism, 2016Co-Authors: Dolores Shoback, John P Bilezikian, Aliya Khan, Aline G Costa, David W Dempster, Henning Dralle, Munro Peacock, Marco Raffaelli, Barbara C Silva, Rajesh V ThakkerAbstract:Context: Understanding the etiology, diagnosis, and symptoms of Hypoparathyroidism may help to improve quality of life and long-term disease outcomes. This paper summarizes the results of the findings and recommendations of the Working Group on Presentation of Hypoparathyroidism. Evidence Acquisition: Experts convened in Florence, Italy, in May 2015 and evaluated the literature and recent data on the presentation and long-term outcomes of patients with Hypoparathyroidism. Evidence Synthesis: The most frequent etiology is surgical removal or loss of viability of parathyroid glands. Despite precautions and expertise, about 20–30% of patients develop transient and 1–7% develop permanent postsurgical Hypoparathyroidism after total thyroidectomy. Autoimmune destruction is the main reason for nonsurgical Hypoparathyroidism. Severe magnesium deficiency is an uncommon but correctable cause of Hypoparathyroidism. Several genetic etiologies can result in the loss of parathyroid function or action causing isolated h...
Natalie E Cusano - One of the best experts on this subject based on the ideXlab platform.
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Hypoparathyroidism and Seizure
Hypoparathyroidism, 2019Co-Authors: Barbara C Silva, Natalie E CusanoAbstract:Permanent Hypoparathyroidism is reported as a complication of 1–5% of neck operations, usually involving the thyroid or parathyroids. We report a case of a 29-year-old woman with permanent postsurgical Hypoparathyroidism following total thyroidectomy for a papillary thyroid carcinoma. Despite the standard treatment with oral calcium and active vitamin D, the patient presented with frequent episodes of severe hypocalcemia, three of them associated with generalized tonic-clonic seizures. Several aspects of the physiology, epidemiology, diagnosis, and management of seizures precipitated by hypocalcemia, as well as the treatment of hypocalcemia due to Hypoparathyroidism are reviewed.
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quality of life in Hypoparathyroidism improves with rhpth 1 84 throughout 8 years of therapy
The Journal of Clinical Endocrinology and Metabolism, 2019Co-Authors: Gaia Tabacco, Natalie E Cusano, John C Williams, Beatriz Omeragic, Rukshana Majeed, Maximo Gomez Almonte, Mishaela R Rubin, John P BilezikianAbstract:CONTEXT: Calcium and vitamin D treatment does not improve reduced quality of life (QOL) in Hypoparathyroidism. Recombinant human (rh) PTH(1-84) therapy improves QOL metrics for up to 5 years. Data on QOL beyond this time point are not available. OBJECTIVES: To evaluate the effects of 8 years of rhPTH(1-84) therapy on QOL and factors associated with long-term benefit. DESIGN: Prospective, open-label trial. SETTING: Referral center. PATIENTS: Twenty patients with hypoparathyoidism. MAIN OUTCOME MEASURES: RAND 36-Item Short Form Health Survey (SF-36). RESULTS: rhPTH therapy led to substantial improvement in five of the eight SF-36 domains [vitality, social functioning (SF), mental health (MH), bodily pain (BP) and general health] and three of these domains (SF, MH, BP) were no longer lower than the reference population. The improvement in the mental component summary (MCS) score was sustained through 8 years, while the physical component summary (PCS) score improved through 6 years. A lower baseline QOL score was associated with greater improvement. A threshold value <238 (MCS) and <245 (PCS) predicted long-term improvement in 90% and 100% of the cohort, respectively. In patients whose calcium supplementation was reduced, MCS and PCS scores improved more than those whose supplementation did not decline to the same extent. Improvement in PCS was greater in patients whose calcitriol dosage was reduced and duration of disease was shorter. CONCLUSIONS: rhPTH(1-84) improves long-term well-being in Hypoparathyroidism. The improvements are most prominent in those with impaired SF-36 at baseline and those whose requirements for conventional therapy decreased substantially.
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Signs and Symptoms of Hypoparathyroidism.
Endocrinology and Metabolism Clinics of North America, 2018Co-Authors: Natalie E Cusano, John P BilezikianAbstract:Hypoparathyroidism is associated with a spectrum of clinical manifestations in the acute and chronic settings, from mild to debilitating. Although the acute symptoms of hypocalcemia are primarily due to neuromuscular irritability, the chronic manifestations of Hypoparathyroidism may be due to the disease itself or to complications of therapy or to both. The chronic complications of Hypoparathyroidism can affect multiple organ systems, including the renal, neurologic, neuropsychiatric, skeletal, and immune systems. Further research is needed to determine the pathophysiology of complications in Hypoparathyroidism and whether interventions can decrease the risk of these complications.
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management of Hypoparathyroidism present and future
The Journal of Clinical Endocrinology and Metabolism, 2016Co-Authors: John P Bilezikian, Natalie E Cusano, Mishaela R Rubin, Michael Mannstadt, Maria Luisa Brandi, Lars Rejnmark, Rene Rizzoli, Karen K Winer, Uri A Liberman, John T PottsAbstract:Context: Conventional management of Hypoparathyroidism has focused upon maintaining the serum calcium with oral calcium and active vitamin D, often requiring high doses and giving rise to concerns about long-term consequences including renal and brain calcifications. Replacement therapy with PTH has recently become available. This paper summarizes the results of the findings and recommendations of the Working Group on Management of Hypoparathyroidism. Evidence Acquisition: Contributing authors reviewed the literature regarding physiology, pathophysiology, and nutritional aspects of Hypoparathyroidism, management of acute hypocalcemia, clinical aspects of chronic management, and replacement therapy of Hypoparathyroidism with PTH peptides. PubMed and other literature search engines were utilized. Evidence synthesis: Under normal circumstances, interactions between PTH and active vitamin D along with the dynamics of calcium and phosphorus absorption, renal tubular handing of those ions, and skeletal responsi...
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Use of parathyroid hormone in Hypoparathyroidism
Journal of Endocrinological Investigation, 2013Co-Authors: Natalie E Cusano, Mishaela R Rubin, Dinaz Irani, James Sliney, John P BilezikianAbstract:Hypoparathyroidism is a disorder characterized by hypocalcemia, deficient PTH, and abnormal bone remodeling. Standard treatment of Hypoparathyroidism consists of oral calcium and vitamin D supplementation. However, maintaining serum calcium levels can be a challenge. In addition, concerns exist regarding hypercalciuria and ectopic calcifications that can be associated with such treatment. Hypoparathyroidism is the only classic endocrine deficiency disease for which the missing hormone, PTH, is not yet an approved treatment. This review focuses on the use of PTH in the treatment of Hypoparathyroidism, in the form of teriparatide [PTH(1–34)] and the full-length molecule, PTH(1–84). Studies in hypoparathyroid subjects demonstrate that PTH(1–34) and PTH(1–84) lower or abolish supplemental calcium and vitamin D requirements as well as increase markers of bone turnover. Densitometric and histomorphometric studies in some subjects treated with PTH(1–34) and PTH(1–84) show an improvement in bone-remodeling dynamics and return of bone metabolism toward normal levels. Given the chronic nature of Hypoparathyroidism, and the expectation that PTH will be used for extended periods of time in Hypoparathyroidism, further studies are needed to determine the long-term safety of PTH therapy in this population.
David Karpf - One of the best experts on this subject based on the ideXlab platform.
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Assessing the Patient Experience of Hypoparathyroidism Symptoms: Development of the Hypoparathyroidism Patient Experience Scale-Symptom (HPES-Symptom)
The Patient - Patient-Centered Outcomes Research, 2020Co-Authors: Meryl Brod, Laura Tesler Waldman, Alden Smith, David KarpfAbstract:Background and Objective Hypoparathyroidism is a rare endocrine disorder characterized by absent or inappropriately low levels of circulating parathyroid hormone. Patients with Hypoparathyroidism receiving standard-of-care therapy report debilitating physical and cognitive symptoms, which may indicate a reduced health-related quality of life. The purpose of this study was to develop a new disease-specific measure of the signs and symptoms of Hypoparathyroidism, the Hypoparathyroidism Patient Experience Scale-Symptom (HPES-Symptom), and provide evidence for the content validity of items in the measure based on rigorous qualitative research methodologies for patient-reported outcome development. Methods Semi-structured, individual concept elicitation interviews were conducted with five clinical experts and 42 adults with Hypoparathyroidism in the USA to identify the signs and symptoms of relevance and importance to those with the condition. Transcripts were coded and analyzed using an adapted grounded theory approach. Following item generation, cognitive debriefing interviews of the draft measure were conducted in an independent sample of 16 adults with Hypoparathyroidism. Results One hundred percent of the concept elicitation patient sample reported experiencing physical symptoms that were attributed to Hypoparathyroidism, including tingling/numbness/paresthesia ( n = 37, 88%), muscle cramping ( n = 36, 86%), and physical fatigue ( n = 35, 83%). The majority of patients ( n = 36, 86%) further reported experiencing cognitive dysfunction, including impaired memory ( n = 24, 57%), impaired ability to have a conversation ( n = 21, 50%), and lack of concentration/focus ( n = 18, 43%). Seventeen major signs and symptoms were identified during item generation and included in the preliminary measure. After the cognitive debriefing, the 17-item HPES-Symptom was generated. Conclusions The findings provided evidence of content validity for the HPES-Symptom in US adults with Hypoparathyroidism. Additional research is needed to validate the measure in patients with Hypoparathyroidism to assess its psychometric properties.
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Assessing the Patient Experience of Hypoparathyroidism Symptoms: Development of the Hypoparathyroidism Patient Experience Scale-Symptom (HPES-Symptom).
The Patient: Patient-Centered Outcomes Research, 2019Co-Authors: Meryl Brod, Laura Tesler Waldman, Alden Smith, David KarpfAbstract:Hypoparathyroidism is a rare endocrine disorder characterized by absent or inappropriately low levels of circulating parathyroid hormone. Patients with Hypoparathyroidism receiving standard-of-care therapy report debilitating physical and cognitive symptoms, which may indicate a reduced health-related quality of life. The purpose of this study was to develop a new disease-specific measure of the signs and symptoms of Hypoparathyroidism, the Hypoparathyroidism Patient Experience Scale-Symptom (HPES-Symptom), and provide evidence for the content validity of items in the measure based on rigorous qualitative research methodologies for patient-reported outcome development. Semi-structured, individual concept elicitation interviews were conducted with five clinical experts and 42 adults with Hypoparathyroidism in the USA to identify the signs and symptoms of relevance and importance to those with the condition. Transcripts were coded and analyzed using an adapted grounded theory approach. Following item generation, cognitive debriefing interviews of the draft measure were conducted in an independent sample of 16 adults with Hypoparathyroidism. One hundred percent of the concept elicitation patient sample reported experiencing physical symptoms that were attributed to Hypoparathyroidism, including tingling/numbness/paresthesia (n = 37, 88%), muscle cramping (n = 36, 86%), and physical fatigue (n = 35, 83%). The majority of patients (n = 36, 86%) further reported experiencing cognitive dysfunction, including impaired memory (n = 24, 57%), impaired ability to have a conversation (n = 21, 50%), and lack of concentration/focus (n = 18, 43%). Seventeen major signs and symptoms were identified during item generation and included in the preliminary measure. After the cognitive debriefing, the 17-item HPES-Symptom was generated. The findings provided evidence of content validity for the HPES-Symptom in US adults with Hypoparathyroidism. Additional research is needed to validate the measure in patients with Hypoparathyroidism to assess its psychometric properties.
Yeo Kyu Youn - One of the best experts on this subject based on the ideXlab platform.
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risk factors of Hypoparathyroidism following total thyroidectomy for thyroid cancer
World Journal of Surgery, 2013Co-Authors: Se Hyun Paek, Ki Wook Chung, Yeo Kyu YounAbstract:Background The risk factors responsible for Hypoparathyroidism after total thyroidectomy have not been completely defined. The present study evaluated one surgeon’s personal experience of postoperative Hypoparathyroidism after total thyroidectomy for thyroid cancer and predisposing risk factors of postoperative Hypoparathyroidism.
Meryl Brod - One of the best experts on this subject based on the ideXlab platform.
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Assessing the Patient Experience of Hypoparathyroidism Symptoms: Development of the Hypoparathyroidism Patient Experience Scale-Symptom (HPES-Symptom)
The Patient - Patient-Centered Outcomes Research, 2020Co-Authors: Meryl Brod, Laura Tesler Waldman, Alden Smith, David KarpfAbstract:Background and Objective Hypoparathyroidism is a rare endocrine disorder characterized by absent or inappropriately low levels of circulating parathyroid hormone. Patients with Hypoparathyroidism receiving standard-of-care therapy report debilitating physical and cognitive symptoms, which may indicate a reduced health-related quality of life. The purpose of this study was to develop a new disease-specific measure of the signs and symptoms of Hypoparathyroidism, the Hypoparathyroidism Patient Experience Scale-Symptom (HPES-Symptom), and provide evidence for the content validity of items in the measure based on rigorous qualitative research methodologies for patient-reported outcome development. Methods Semi-structured, individual concept elicitation interviews were conducted with five clinical experts and 42 adults with Hypoparathyroidism in the USA to identify the signs and symptoms of relevance and importance to those with the condition. Transcripts were coded and analyzed using an adapted grounded theory approach. Following item generation, cognitive debriefing interviews of the draft measure were conducted in an independent sample of 16 adults with Hypoparathyroidism. Results One hundred percent of the concept elicitation patient sample reported experiencing physical symptoms that were attributed to Hypoparathyroidism, including tingling/numbness/paresthesia ( n = 37, 88%), muscle cramping ( n = 36, 86%), and physical fatigue ( n = 35, 83%). The majority of patients ( n = 36, 86%) further reported experiencing cognitive dysfunction, including impaired memory ( n = 24, 57%), impaired ability to have a conversation ( n = 21, 50%), and lack of concentration/focus ( n = 18, 43%). Seventeen major signs and symptoms were identified during item generation and included in the preliminary measure. After the cognitive debriefing, the 17-item HPES-Symptom was generated. Conclusions The findings provided evidence of content validity for the HPES-Symptom in US adults with Hypoparathyroidism. Additional research is needed to validate the measure in patients with Hypoparathyroidism to assess its psychometric properties.
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Assessing the Patient Experience of Hypoparathyroidism Symptoms: Development of the Hypoparathyroidism Patient Experience Scale-Symptom (HPES-Symptom).
The Patient: Patient-Centered Outcomes Research, 2019Co-Authors: Meryl Brod, Laura Tesler Waldman, Alden Smith, David KarpfAbstract:Hypoparathyroidism is a rare endocrine disorder characterized by absent or inappropriately low levels of circulating parathyroid hormone. Patients with Hypoparathyroidism receiving standard-of-care therapy report debilitating physical and cognitive symptoms, which may indicate a reduced health-related quality of life. The purpose of this study was to develop a new disease-specific measure of the signs and symptoms of Hypoparathyroidism, the Hypoparathyroidism Patient Experience Scale-Symptom (HPES-Symptom), and provide evidence for the content validity of items in the measure based on rigorous qualitative research methodologies for patient-reported outcome development. Semi-structured, individual concept elicitation interviews were conducted with five clinical experts and 42 adults with Hypoparathyroidism in the USA to identify the signs and symptoms of relevance and importance to those with the condition. Transcripts were coded and analyzed using an adapted grounded theory approach. Following item generation, cognitive debriefing interviews of the draft measure were conducted in an independent sample of 16 adults with Hypoparathyroidism. One hundred percent of the concept elicitation patient sample reported experiencing physical symptoms that were attributed to Hypoparathyroidism, including tingling/numbness/paresthesia (n = 37, 88%), muscle cramping (n = 36, 86%), and physical fatigue (n = 35, 83%). The majority of patients (n = 36, 86%) further reported experiencing cognitive dysfunction, including impaired memory (n = 24, 57%), impaired ability to have a conversation (n = 21, 50%), and lack of concentration/focus (n = 18, 43%). Seventeen major signs and symptoms were identified during item generation and included in the preliminary measure. After the cognitive debriefing, the 17-item HPES-Symptom was generated. The findings provided evidence of content validity for the HPES-Symptom in US adults with Hypoparathyroidism. Additional research is needed to validate the measure in patients with Hypoparathyroidism to assess its psychometric properties.