RESUMEN
Peripheral blood progenitor cells (PBPCs) are a predominant graft source in allogeneic hematopoietic cell transplantation. Citrate-induced hypocalcemia remains the most frequent side effect of PBPC apheresis. Although the method for preventing severe adverse events is established, more efficient prophylaxis is required so that volunteer donors can donate PBPCs without pain and anxiety. We studied 80 healthy donors who underwent PBPC harvest between February 2014 and June 2020. Of these, 23 donors who underwent apheresis between February 2014 and December 2015 received only the standard prophylaxis of intravenous calcium gluconate. Oral calcium drinks were provided to 57 donors who underwent apheresis from January 2016 to June 2020 to supplement intravenous calcium gluconate prophylaxis. The ionized calcium (ICa) levels at multiple time intervals and the hypocalcemic symptoms were evaluated. Oral supplementation with a calcium drink maintained significantly higher ICa levels. Analysis using the inverse probability weighted regression adjustment method suggested that calcium drinks reduced the frequency of citrate-related reactions by 39.2 %. Administering a prophylactic oral calcium drink before apheresis with intravenous administration of calcium gluconate is promising to further reduce citrate-induced hypocalcemia in volunteer donors.
Asunto(s)
Gluconato de Calcio/administración & dosificación , Ácido Cítrico , Suplementos Dietéticos , Movilización de Célula Madre Hematopoyética , Células Madre de Sangre Periférica/metabolismo , Donantes de Tejidos , Administración Oral , Adulto , Eliminación de Componentes Sanguíneos , Calcio/administración & dosificación , Ácido Cítrico/efectos adversos , Ácido Cítrico/farmacología , Femenino , Trasplante de Células Madre Hematopoyéticas , Humanos , Masculino , Persona de Mediana EdadRESUMEN
Introducción. El hipoparatiroidismo es una enfermedad caracterizada por la ausencia o concentraciones inadecuadamente bajas de hormona paratiroidea (PTH), que conduce a hipocalcemia, hiperfosfatemia y excreción fraccional elevada de calcio en la orina. Las calcificaciones del sistema nervioso central son un hallazgo frecuente en estos pacientes. Caso clínico. Mujer de 56 años con antecedente de hipotiroidismo, que ingresó por un cuadro de 6 días de evolución caracterizado por astenia, parestesias periorales y movimientos anormales de manos y pies. Las pruebas de laboratorio demostraron hipocalcemia, hiperfosfatemia y niveles bajos de hormona paratiroidea. Se realizó una tomografía computarizada de cráneo que mostró áreas bilaterales y simétricas de calcificaciones en hemisferios cerebelosos, ganglios basales y corona radiata. No se evidenciaron trastornos en el metabolismo del cobre y hierro. Se estableció el diagnóstico del síndrome de Fahr secundario a hipoparatiroidismo y se inició tratamiento con suplementos de calcio y vitamina D con evolución satisfactoria. Discusión. El síndrome de Fahr es un trastorno neurológico caracterizado por el depósito anormal de calcio en áreas del cerebro que controlan la actividad motora. Se asocia a varias enfermedades, especialmente, hipoparatiroidismo. La suplementación con calcio y vitamina D con el objetivo de normalizar los niveles plasmáticos de estos cationes es el tratamiento convencional. (AU)
Introduction. Hypoparathyroidism is a disease characterized by absence or inappropriately low concentrations of circulating parathyroid hormone, leading to hypocalcaemia, hyperphosphataemia and elevated fractional excretion of calcium in the urine. Central nervous system calcifications are a common finding in these patients. Case report. 56-year-old woman with a history of hypothyroidism who was admitted for a 6-day course of illness characterized by asthenia, perioral paresthesias, and abnormal movements of the hands and feet. Laboratory tests showed hypocalcemia, hyperphosphatemia, and low parathyroid hormone levels. A cranial computed tomography was performed. It showed bilateral and symmetrical areas of calcifications in the cerebellar hemispheres, basal ganglia, and radiata crown. No disorders of copper or iron metabolism were evident. The diagnosis of Fahr syndrome secondary to hypoparathyroidism was established and treatment with calcium and vitamin D supplements was started with satisfactory evolution. Discussion. Fahr's syndrome is a neurological disorder associated with abnormal calcium deposition in areas of the brain that control motor activity. It is associated with various diseases, especially hypoparathyroidism. The conventional treatment is supplementation with calcium and vitamin D, with the aim of normalizing their plasma levels. (AU)
Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , Calcinosis/diagnóstico por imagen , Hipoparatiroidismo/diagnóstico , Enfermedades del Sistema Nervioso/diagnóstico por imagen , Hormona Paratiroidea/sangre , Calcinosis/complicaciones , Calcinosis/tratamiento farmacológico , Calcitriol/administración & dosificación , Carbonato de Calcio/administración & dosificación , Gluconato de Calcio/administración & dosificación , Calcio/administración & dosificación , Hiperfosfatemia/sangre , Hipocalcemia/sangre , Hipoparatiroidismo/etiología , Hipoparatiroidismo/tratamiento farmacológico , Enfermedades del Sistema Nervioso/complicaciones , Enfermedades del Sistema Nervioso/tratamiento farmacológicoRESUMEN
Hypoparathyroidism patients present with features of hypocalcemia like carpopedal spasm, numbness and paresthesias but hypocalcemic cardiomyopathy leading to congestive heart failure (CHF) is a rare presentation. We present here a case of 55-year-old Asian man who was a known case of dilated cardiomyopathy for 6 months, presented with the chief complaints of shortness of breath on exertion and decreased urine output. On general physical examination, features suggestive of CHF were seen. Chvostek and Trousseau's sign was positive. The patient had a history of cataract surgery of both eyes 15 years ago. Further investigations revealed hypocalcemia. Echo showed severe global hypokinesia of left ventricle with left ventricle ejection fraction 15%. This CHF was refractory to conventional treatment, though, with calcium supplementation, the patient improved symptomatically. On follow-up after 3 months, an improvement was seen in the echocardiographic parameters with ejection fraction improving to 25%.
Asunto(s)
Cardiomiopatía Dilatada/complicaciones , Insuficiencia Cardíaca/etiología , Hipocalcemia/complicaciones , Hipoparatiroidismo/complicaciones , Gluconato de Calcio/administración & dosificación , Gluconato de Calcio/uso terapéutico , Cardiomiopatía Dilatada/diagnóstico , Ecocardiografía/métodos , Insuficiencia Cardíaca/tratamiento farmacológico , Insuficiencia Cardíaca/fisiopatología , Humanos , Hipocalcemia/diagnóstico , Hipocalcemia/tratamiento farmacológico , Hipocalcemia/fisiopatología , Hipoparatiroidismo/tratamiento farmacológico , Masculino , Persona de Mediana Edad , Volumen Sistólico , Resultado del Tratamiento , Disfunción Ventricular Izquierda/diagnóstico por imagenRESUMEN
Abdominal wall pain (AWP) is a common and underrecognized cause of chronic abdominal pain. The etiology of AWP varies. History and physical examination are critical to an accurate diagnosis of AWP. Trigger point injection using either a corticosteroid, a local anesthetic, or a combination of both often gives relief of pain and is of diagnostic and therapeutic value. Increased awareness of AWP as a cause of chronic, nonvisceral abdominal pain can prevent fruitless searches for intra-abdominal pathology and reduce medical costs.
Asunto(s)
Dolor Abdominal/etiología , Hiperpotasemia/complicaciones , Insuficiencia Renal Crónica/complicaciones , Dolor Abdominal/sangre , Dolor Abdominal/diagnóstico , Pared Abdominal , Anciano , Inhibidores de la Enzima Convertidora de Angiotensina/uso terapéutico , Gluconato de Calcio/administración & dosificación , Diagnóstico Diferencial , Diuréticos/administración & dosificación , Furosemida/administración & dosificación , Humanos , Hiperpotasemia/sangre , Hiperpotasemia/terapia , Infusiones Intravenosas , Lisinopril/uso terapéutico , Masculino , Potasio/sangre , Insuficiencia Renal Crónica/sangre , Insuficiencia Renal Crónica/terapiaRESUMEN
Clinically relevant hypocalcemia is a well-documented complication of glucocorticoid administration in people with hypoparathyroidism. The current report describes the phenomenon in a dog. A 7 yr old neutered male Pomeranian was diagnosed with immune-mediated thrombocytopenia, immune-mediated hemolytic anemia, and primary hypoparathyroidism. This dog required long-term parenteral calcium gluconate to prevent clinical hypocalcemia despite appropriate doses of oral calcitriol and calcium carbonate. This is the first description of clinically significant presumptive glucocorticoid induced hypocalcemia in a dog with primary hypoparathyroidism.
Asunto(s)
Anemia Hemolítica/veterinaria , Enfermedades de los Perros/inducido químicamente , Glucocorticoides/efectos adversos , Hipocalcemia/veterinaria , Hipoparatiroidismo/veterinaria , Trombocitopenia/veterinaria , Anemia Hemolítica/tratamiento farmacológico , Anemia Hemolítica/inmunología , Animales , Calcitriol/administración & dosificación , Calcitriol/uso terapéutico , Carbonato de Calcio/administración & dosificación , Carbonato de Calcio/uso terapéutico , Gluconato de Calcio/administración & dosificación , Gluconato de Calcio/uso terapéutico , Hormonas y Agentes Reguladores de Calcio/administración & dosificación , Hormonas y Agentes Reguladores de Calcio/uso terapéutico , Suplementos Dietéticos , Enfermedades de los Perros/tratamiento farmacológico , Enfermedades de los Perros/inmunología , Perros , Esquema de Medicación , Glucocorticoides/administración & dosificación , Hipocalcemia/inducido químicamente , Hipocalcemia/prevención & control , Hipoparatiroidismo/tratamiento farmacológico , Hipoparatiroidismo/inmunología , Masculino , Trombocitopenia/tratamiento farmacológico , Trombocitopenia/inmunologíaRESUMEN
Using calcium salts in management of amlodipine overdose is challenging. A 25-year-old male with known history of adult polycystic kidney disease presented with hypotension, tachycardia, and intact neurological status after ingestion of 450 mg of amlodipine. Immediately, normal saline infusion and norepinephrine were initiated. Two grams of calcium gluconate was injected, followed by intravenous infusion of 1.16 mg/kg/h. The patient was put on insulin-glucose protocol to maintain euglycemia and hyperinsulinemia. Electrocardiography demonstrated junctional rhythm. Serum creatinine was 2.5 mg/dL with metabolic acidosis. By the end of 24 h post-admission, his consciousness, blood pressure, and urine output were normal. Almost 32 h post-admission, he became disoriented and his oxygen saturation decreased and therefore was mechanically ventilated. Second chest X-ray showed pulmonary edema. Serum calcium level increased to 26.1 mg/dL. Calcium was discontinued, and furosemide infusion and calcitonin were intravenously administrated. Urine output increased and hemodialysis improved pulmonary edema and serum calcium level with no change in consciousness. Three days after admission, the patient became anuric and developed multi-organ failure and died 5 days post-admission. To avoid the consequences of excessive infusion of calcium in renal failure patients, the minimum calcium dose with close monitoring is recommended.
Asunto(s)
Amlodipino/envenenamiento , Bloqueadores de los Canales de Calcio/envenenamiento , Gluconato de Calcio/efectos adversos , Hipercalcemia/inducido químicamente , Hipotensión/tratamiento farmacológico , Enfermedad Iatrogénica , Taquicardia/tratamiento farmacológico , Adulto , Amlodipino/administración & dosificación , Bloqueadores de los Canales de Calcio/administración & dosificación , Gluconato de Calcio/administración & dosificación , Electrocardiografía , Resultado Fatal , Humanos , Hipercalcemia/sangre , Hipercalcemia/diagnóstico , Hipercalcemia/fisiopatología , Hipotensión/inducido químicamente , Hipotensión/diagnóstico , Hipotensión/fisiopatología , Riñón/fisiopatología , Fallo Renal Crónico/complicaciones , Fallo Renal Crónico/fisiopatología , Masculino , Insuficiencia Multiorgánica/inducido químicamente , Factores de Riesgo , Suicidio , Taquicardia/inducido químicamente , Taquicardia/diagnóstico , Taquicardia/fisiopatologíaRESUMEN
BACKGROUND: To prevent hypocalcemia, this study examined the efficiency of a calcium supplement formula guided by predicted calcium requirement in patients with renal failure after parathyroidectomy with autotransplantation. METHODS: In the first phase, a protocol was followed whereby intravenous calcium gluconate was repeatedly titrated based on serum calcium levels in 22 patients with parathyroidectomy with autotransplantation. In the second phase, the first equation protocol was applied in 74 patients with parathyroidectomy with autotransplantation and revised. RESULTS: There is a significant correlation between the postoperative amount of calcium requirement and preoperative serum alkaline phosphatase level (r = 0.442; P < .001) and parathyroid hormone level (PTH; r = 0.889; P < .001). For prediction of insufficient calcium supplement, the cutoff point of PTH and alkaline phosphatase level values were calculated by receiver operating characteristic (ROC) curve analysis and modified equations were developed by regression analysis. CONCLUSION: The preoperative PTH and alkaline phosphatase levels can predict postoperative calcium requirements, such that equations of calcium supplement allow the management of hypocalcemia efficiently in patients on dialysis after parathyroidectomy with autotransplantation.
Asunto(s)
Fosfatasa Alcalina/sangre , Gluconato de Calcio/administración & dosificación , Hiperparatiroidismo Secundario/cirugía , Hipocalcemia/cirugía , Hormona Paratiroidea/sangre , Paratiroidectomía , Administración Intravenosa , Adolescente , Adulto , Anciano , Biomarcadores/sangre , Calcio/sangre , Femenino , Humanos , Hipocalcemia/sangre , Hipocalcemia/tratamiento farmacológico , Masculino , Persona de Mediana EdadRESUMEN
Preterm infants often have a reduced bone mineral content (BMC) with increased risk of metabolic bone disease. After birth it is difficult to supply calcium (Ca) and phosphorus (P) comparable to the high fetal accretion rate. It is not known whether high supplementation of minerals in the early postnatal period improves growth and bone mineralization. The aim of this study was to evaluate growth and bone mineralization at term corrected age (TCA) in very and extremely preterm infants who received different enteral Ca and P intakes during the first 10 days of life. Infants (n = 109) with birth weights below 1500 g were randomly assigned to one of three groups that differed in the nutritional protocols delivered until day 10: Group A, mother's own milk (MOM) and donor milk (unfortified); Group B, MOM (unfortified) and preterm formula; Group C, MOM (start fortification >50 mL/day) and preterm formula. Due to the earlier commencement of fortification, Group C received higher intakes of calcium and phosphorus and protein (p < 0.001) until day 10. At TCA weight, length, BMC and bone mineral density (BMD), measured by dual-X-ray absorptiometry, were not different between the groups. Nutritional intake of P was positively associated with length (ß; (95% confidence interval (CI): 0.20 (0.001; 0.393); p-value = 0.048), whereas Ca intake was negatively associated with BMC (-1.94 (-2.78; -1.09); p-value < 0.001). A small interaction between Ca and P intake was only found for BMD (0.003 (0.00002; 0.00006); p-value = 0.036). The volume of human milk per kg provided during the first 10 days was positively associated with BMC (ß; (95% CI): 0.013 (0.002; 0.023); p < 0.017). Higher intakes of Ca and P during the first 10 days, as provided in this study, did not improve bone mineralization at term corrected age.
Asunto(s)
Desarrollo Óseo/efectos de los fármacos , Nutrición Enteral , Recien Nacido Prematuro/crecimiento & desarrollo , Nutrición Parenteral , Absorciometría de Fotón , Gluconato de Calcio/administración & dosificación , Femenino , Glicerofosfatos/administración & dosificación , Humanos , Fórmulas Infantiles/análisis , Fenómenos Fisiológicos Nutricionales del Lactante , Recién Nacido , Masculino , Vitamina DRESUMEN
Nutritional rickets remains a significant public health issue for children worldwide. Although it has almost disappeared in industrialized countries following routine vitamin D supplementation, recent evidence suggests an increasing incidence, especially in young children. In addition to the classical clinical consequences on bone and the growth plate, rickets may also be associated with life-threatening neurological and cardiac complications in the most severe forms. Consequently, early screening and treatment are required. Here, we report the case of a 2-year-old child who presented with severe nutritional rickets associated with seizure and cardiomyopathy. Family screening revealed rickets in all the siblings. This case report emphasizes the importance of being aware of this disease, notably in population with sociocultural risk factors.
Asunto(s)
Conservadores de la Densidad Ósea/administración & dosificación , Gluconato de Calcio/administración & dosificación , Colecalciferol/administración & dosificación , Raquitismo/tratamiento farmacológico , Raquitismo/etiología , Cardiomiopatías/complicaciones , Preescolar , Humanos , Masculino , Raquitismo/complicaciones , Raquitismo/diagnóstico , Convulsiones/complicaciones , Resultado del TratamientoRESUMEN
RATIONALE: Fluoroacetamide poisoning is the acute and severe disease of human, which leads to nervous, digestive, and cardiovascular system damage or even death in a short period of time. PATIENT CONCERNS: We report a case of a 65-year-old woman with loss of consciousness, nausea, and vomiting who was sent to the hospital by passers-by. DIAGNOSIS: She was diagnosed with severe fluoroacetamide poisoning with combined multiple organ dysfunction syndrome. INTERVENTIONS: When the diagnosis was unclear, we gave gastric lavage, support and symptomatic treatment, and closely with the vital sign. When the diagnosis was clear, based on the evidence of retrieved, muscle injection of acetamide, calcium gluconate, and vitamin C. Traditional Chinese medicine aspect, oral administration of mung bean soup of glycyrrhizae and Da-Cheng-Qi decoction enema. OUTCOMES: By setting reasonable treatment for patients, she had no special discomfort and complications after treatment. Besides, through 1-month follow-up, it was confirmed that the treatments were effective. LESSONS: Evidence-based integrated Chinese and Western medicines can effectively improve the therapeutic effects in severe fluoroacetamide-poisoned patients with combined MODS.
Asunto(s)
Antídotos/administración & dosificación , Medicamentos Herbarios Chinos/administración & dosificación , Fluoroacetatos/envenenamiento , Lavado Gástrico , Medicina Tradicional China , Insuficiencia Multiorgánica/terapia , Acetamidas/administración & dosificación , Anciano , Ácido Ascórbico/administración & dosificación , Gluconato de Calcio/administración & dosificación , Diagnóstico Diferencial , Medicina Basada en la Evidencia , Femenino , HumanosRESUMEN
BACKGROUND/AIMS: To prevent hypocalcemia after parathyroidectomy (PTX), parenteral calcium is required in addition to oral calcitriol and calcium. After switching to oral calcium, patients can be discharged from the hospital. The aim of this study was to analyze the clinical characteristics and outcomes of PTX performed at a single Korean center and to investigate the associated laboratory factors used to analyze the total amount of postoperative calcium required. METHODS: We enrolled 91 hemodialysis patients undergoing PTX from November 2003 to December 2011. We collected clinical and laboratory data preoperatively, 12 and 48 hours postoperatively, at discharge, and 3 and 6 months postoperatively. RESULTS: In total, 59 patients underwent PTX with autotransplantation (AT), 6 underwent total PTX without AT, 11 underwent subtotal PTX, and 15 underwent limited PTX. Total PTX without AT showed the lowest recurrence rate. At all postoperative time points, the mean levels of serum calcium, phosphorus, and intact parathyroid hormone (iPTH) decreased significantly, compared with preoperative levels; however, alkaline phosphatase (ALP) increased significantly from 48 hours postoperatively to discharge (p < 0.001). On multiple linear regression analysis, the total amount of injected calcium during hospitalization showed a significant correlation with preoperative ALP (p < 0.001), preoperative iPTH (p = 0.037), and Δphosphorus at 48 hours (p < 0.001). We developed an equation for estimating the total calcium requirement after PTX. CONCLUSIONS: Preoperative ALP, preoperative iPTH, and Δphosphorus at 48 hours may be significant factors in estimating the postoperative calcium requirement. The formula for postoperative calcium requirement after PTX may help to predict the duration of postoperative hospitalization.
Asunto(s)
Carbonato de Calcio/administración & dosificación , Compuestos de Calcio/administración & dosificación , Gluconato de Calcio/administración & dosificación , Técnicas de Apoyo para la Decisión , Suplementos Dietéticos , Hiperparatiroidismo Secundario/cirugía , Hipocalcemia/prevención & control , Lactatos/administración & dosificación , Paratiroidectomía/efectos adversos , Administración Intravenosa , Administración Oral , Adulto , Anciano , Biomarcadores/sangre , Calcio/sangre , Femenino , Humanos , Hiperparatiroidismo Secundario/sangre , Hiperparatiroidismo Secundario/diagnóstico , Hipocalcemia/diagnóstico , Hipocalcemia/etiología , Modelos Lineales , Masculino , Persona de Mediana Edad , Modelos Biológicos , Análisis Multivariante , Hormona Paratiroidea/sangre , Fósforo/sangre , Recurrencia , República de Corea , Estudios Retrospectivos , Factores de Riesgo , Factores de Tiempo , Resultado del Tratamiento , Adulto JovenRESUMEN
The case of a patient with clinical symptoms, laboratory and imaging findings of hypoparathyroidism, sensorineural deafness, renal dysplasia HDR, or Barakat syndrome (hypoparathyroidism, deafness, renal dysplasia), and vitamin D deficiency, is presented. A Caucasian man aged 51 years with a history of chronic hypocalcaemia since childhood, was admitted with hypertonia of the body and extremities, and loss of consciousness. On admission, he was found to have severe hypocalcaemia, hyperphosphataemia, severe hypoparathyroidism, low serum magnesium and mild renal insufficiency. Calcium gluconate was administered intravenously supplemented with magnesium, and the patient recovered consciousness while clinical and laboratory findings improved. Evaluation revealed left renal aplasia and sensorineural deafness affecting both ears. Vitamin D deficiency was also present. He was given calcium and vitamin D supplements orally, and the hypocalcaemia was corrected. This case is described as it is an extremely rare case of HDR syndrome with concurrent vitamin D deficiency.
Asunto(s)
Pérdida Auditiva Sensorineural/etiología , Hipoparatiroidismo/etiología , Nefrosis/diagnóstico , Insuficiencia Renal/etiología , Deficiencia de Vitamina D/complicaciones , Gluconato de Calcio/administración & dosificación , Fluidoterapia , Pérdida Auditiva Sensorineural/complicaciones , Pérdida Auditiva Sensorineural/diagnóstico , Pérdida Auditiva Sensorineural/tratamiento farmacológico , Humanos , Hipoparatiroidismo/complicaciones , Hipoparatiroidismo/diagnóstico , Hipoparatiroidismo/tratamiento farmacológico , Riñón/anomalías , Masculino , Persona de Mediana Edad , Nefrosis/complicaciones , Nefrosis/tratamiento farmacológico , Insuficiencia Renal/diagnóstico , Resultado del Tratamiento , Vitamina D/administración & dosificación , Deficiencia de Vitamina D/diagnóstico , Deficiencia de Vitamina D/tratamiento farmacológico , Vitaminas/administración & dosificaciónRESUMEN
Seizures during the neonatal period have a broad differential diagnosis. Unlike in developing countries where hypovitaminosis D and hypocalcemia constitutes a major cause of infantile seizures, the number of neonatal seizures attributed to hypocalcemia in developed countries has decreased dramatically due to the improvement of infant formulas and vitamin D supplementation. In these countries, most infants that present with hypocalcemic seizures have underlying endocrinological etiologies rather than dietary insufficiencies. Here, we describe 3 cases of neonatal seizures due to hypocalcemia. Although the symptoms and calcium concentrations at presentation were similar in all 3 cases, the course of the disease and the final diagnosis for each were distinct. The cases are presented along with a brief review of the pathophysiology, differential diagnosis, and treatment of neonatal hypocalcemia.
Asunto(s)
Gluconato de Calcio/administración & dosificación , Calcio/sangre , Hipocalcemia/complicaciones , Convulsiones/etiología , Diagnóstico Diferencial , Electroencefalografía , Femenino , Estudios de Seguimiento , Humanos , Hipocalcemia/sangre , Hipocalcemia/tratamiento farmacológico , Lactante , Recién Nacido , Inyecciones Intravenosas , Masculino , Convulsiones/diagnóstico , Convulsiones/tratamiento farmacológicoRESUMEN
Mutations in sorting nexin 10 (Snx10) have recently been found to account for roughly 4% of all human malignant osteopetrosis, some of them fatal. To study the disease pathogenesis, we investigated the expression of Snx10 and created mouse models in which Snx10 was knocked down globally or knocked out in osteoclasts. Endocytosis is severely defective in Snx10-deficient osteoclasts, as is extracellular acidification, ruffled border formation, and bone resorption. We also discovered that Snx10 is highly expressed in stomach epithelium, with mutations leading to high stomach pH and low calcium solubilization. Global Snx10-deficiency in mice results in a combined phenotype: osteopetrosis (due to osteoclast defect) and rickets (due to high stomach pH and low calcium availability, resulting in impaired bone mineralization). Osteopetrorickets, the paradoxical association of insufficient mineralization in the context of a positive total body calcium balance, is thought to occur due to the inability of the osteoclasts to maintain normal calcium-phosphorus homeostasis. However, osteoclast-specific Snx10 knockout had no effect on calcium balance, and therefore led to severe osteopetrosis without rickets. Moreover, supplementation with calcium gluconate rescued mice from the rachitic phenotype and dramatically extended life span in global Snx10-deficient mice, suggesting that this may be a life-saving component of the clinical approach to Snx10-dependent human osteopetrosis that has previously gone unrecognized. We conclude that tissue-specific effects of Snx10 mutation need to be considered in clinical approaches to this disease entity. Reliance solely on hematopoietic stem cell transplantation can leave hypocalcemia uncorrected with sometimes fatal consequences. These studies established an essential role for Snx10 in bone homeostasis and underscore the importance of gastric acidification in calcium uptake.
Asunto(s)
Densidad Ósea/genética , Ácido Gástrico/metabolismo , Osteoclastos/metabolismo , Osteopetrosis/genética , Nexinas de Clasificación/genética , Secuencia de Aminoácidos , Animales , Calcio/administración & dosificación , Calcio/metabolismo , Gluconato de Calcio/administración & dosificación , Endocitosis/genética , Técnicas de Silenciamiento del Gen , Homeostasis , Humanos , Ratones , Mutación , Osteoclastos/efectos de los fármacos , Osteoclastos/patología , Osteopetrosis/metabolismo , Osteopetrosis/patología , Nexinas de Clasificación/metabolismo , Deficiencia de Vitamina D/genética , Deficiencia de Vitamina D/metabolismo , Deficiencia de Vitamina D/patologíaRESUMEN
BACKGROUND/AIMS: To prevent hypocalcemia after parathyroidectomy (PTX), parenteral calcium is required in addition to oral calcitriol and calcium. After switching to oral calcium, patients can be discharged from the hospital. The aim of this study was to analyze the clinical characteristics and outcomes of PTX performed at a single Korean center and to investigate the associated laboratory factors used to analyze the total amount of postoperative calcium required. METHODS: We enrolled 91 hemodialysis patients undergoing PTX from November 2003 to December 2011. We collected clinical and laboratory data preoperatively, 12 and 48 hours postoperatively, at discharge, and 3 and 6 months postoperatively. RESULTS: In total, 59 patients underwent PTX with autotransplantation (AT), 6 underwent total PTX without AT, 11 underwent subtotal PTX, and 15 underwent limited PTX. Total PTX without AT showed the lowest recurrence rate. At all postoperative time points, the mean levels of serum calcium, phosphorus, and intact parathyroid hormone (iPTH) decreased significantly, compared with preoperative levels; however, alkaline phosphatase (ALP) increased significantly from 48 hours postoperatively to discharge (p < 0.001). On multiple linear regression analysis, the total amount of injected calcium during hospitalization showed a significant correlation with preoperative ALP (p < 0.001), preoperative iPTH (p = 0.037), and Deltaphosphorus at 48 hours (p < 0.001). We developed an equation for estimating the total calcium requirement after PTX. CONCLUSIONS: Preoperative ALP, preoperative iPTH, and Deltaphosphorus at 48 hours may be significant factors in estimating the postoperative calcium requirement. The formula for postoperative calcium requirement after PTX may help to predict the duration of postoperative hospitalization.
Asunto(s)
Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto Joven , Administración Intravenosa , Administración Oral , Biomarcadores/sangre , Calcio/sangre , Carbonato de Calcio/administración & dosificación , Compuestos de Calcio/administración & dosificación , Gluconato de Calcio/administración & dosificación , Técnicas de Apoyo para la Decisión , Suplementos Dietéticos , Hiperparatiroidismo Secundario/sangre , Hipocalcemia/diagnóstico , Lactatos/administración & dosificación , Modelos Lineales , Modelos Biológicos , Análisis Multivariante , Hormona Paratiroidea/sangre , Paratiroidectomía/efectos adversos , Fósforo/sangre , Recurrencia , República de Corea , Estudios Retrospectivos , Factores de Riesgo , Factores de Tiempo , Resultado del TratamientoRESUMEN
CASE DESCRIPTION: A 4-year-old castrated male domestic ferret (Mustela putorius furo) was examined because of a 3-week history of intermittent seizures, signs of depression, hypocalcemia, and hyperphosphatemia. CLINICAL FINDINGS: Plasma biochemical analysis confirmed hyperphosphatemia (17.7 mg/dL) and low concentrations of total (4.3 mg/dL) and ionized (0.49 mmol/L) calcium. Serum parathyroid hormone concentration (2.30 pmol/L) was low or in the low part of the reference interval. TREATMENT AND OUTCOME: Calcium gluconate was administered (2.0 mg/kg/h [0.9 mg/lb/h], IV), followed by a transition to administration of calcium carbonate (53 mg/kg [24.1 mg/lb], PO, q 12 h) and dihydrotachysterol (0.02 mg/kg/d [0.009 mg/lb/d], PO). Attitude of the ferret improved and seizures ceased as blood calcium concentrations increased. The ferret was reexamined because of seizures approximately 1 year after oral maintenance administration of dihydrotachysterol and calcium was initiated. The ferret responded well to emergency and long-term treatment but then was lost to follow-up monitoring. The ferret died approximately 2 years after the initial evaluation and treatment. Hypertrophic cardiomyopathy was diagnosed during necropsy, but the parathyroid glands could not be identified. CLINICAL RELEVANCE: To the authors' knowledge, primary hypoparathyroidism has not previously been reported in a ferret. The condition should be considered for ferrets with hypocalcemia and hyperphosphatemia without azotemia. Treatment with dihydrotachysterol and oral supplementation of calcium appeared to be a viable option for long-term management.
Asunto(s)
Carbonato de Calcio/uso terapéutico , Gluconato de Calcio/uso terapéutico , Dihidrotaquisterol/uso terapéutico , Hurones , Hipoparatiroidismo/veterinaria , Vitaminas/uso terapéutico , Animales , Carbonato de Calcio/administración & dosificación , Gluconato de Calcio/administración & dosificación , Dihidrotaquisterol/administración & dosificación , Hipocalcemia/veterinaria , Hipoparatiroidismo/sangre , Hipoparatiroidismo/tratamiento farmacológico , Masculino , Vitaminas/administración & dosificaciónRESUMEN
Hypocalcaemia due to hypoparathyroidism following thyroidectomy is a relatively common occurrence. Standard treatment is with oral calcium and vitamin D replacement therapy; lack of response to oral therapy is rare. Herein we describe a case of hypoparathyroidism following thyroidectomy unresponsive to oral therapy in a patient with a complex medical history. We consider the potential causes in the context of calcium metabolism including: poor adherence, hungry bone syndrome, malabsorption, vitamin D resistance, bisphosphonate use and functional hypoparathyroidism secondary to magnesium deficiency. Malabsorption due to intestinal hurry was likely to be a contributory factor in this case and very large doses of oral therapy were required to avoid symptomatic hypocalcaemia.
Asunto(s)
Calcitriol/administración & dosificación , Carbonato de Calcio/administración & dosificación , Hipocalcemia/tratamiento farmacológico , Hipoparatiroidismo/tratamiento farmacológico , Tiroidectomía/efectos adversos , Administración Intravenosa , Administración Oral , Adulto , Citrato de Calcio/administración & dosificación , Gluconato de Calcio/administración & dosificación , Sustitución de Medicamentos , Quimioterapia Combinada , Femenino , Humanos , Hidroxicolecalciferoles/administración & dosificación , Hipocalcemia/sangre , Hipocalcemia/diagnóstico , Hipocalcemia/etiología , Hipoparatiroidismo/sangre , Hipoparatiroidismo/diagnóstico , Hipoparatiroidismo/etiología , Factores de Riesgo , Factores de Tiempo , Insuficiencia del TratamientoAsunto(s)
Lesión Renal Aguda/etiología , Gluconato de Calcio/administración & dosificación , Suplementos Dietéticos , Hipocalcemia/tratamiento farmacológico , Rabdomiólisis/complicaciones , Lesión Renal Aguda/diagnóstico , Lesión Renal Aguda/terapia , Adulto , Biomarcadores/sangre , Calcio/sangre , Esquema de Medicación , Humanos , Hipocalcemia/diagnóstico , Hipocalcemia/etiología , Masculino , Diálisis Renal , Rabdomiólisis/diagnóstico , Rabdomiólisis/terapia , Factores de Riesgo , Índice de Severidad de la Enfermedad , Factores de Tiempo , Resultado del Tratamiento , Procedimientos InnecesariosRESUMEN
INTRODUCTION: Hypocalcemia is common in critically ill patients. However, its clinical course during the early days of admission and the role of calcium supplementation remain uncertain, and the assessment of calcium status is inconsistent. We aimed to establish the course of hypocalcemia during the early days of critical illness in relation to mortality and to assess the impact of calcium supplementation on calcium normalization and mortality. METHODS: Data were collected on 1,038 admissions to the critical care units of a tertiary care hospital. One gram of calcium gluconate was administered intravenously once daily to patients with adjusted calcium (AdjCa)<2.2 mmol/L. Demographic and outcome data were compared in normocalcemic (ionized calcium, iCa, 1.1-1.3 mmol/L) and mildly and severely hypocalcemic patients (iCa 0.9-1.1 mmol/L and <0.9 mmol/L, respectively). The change in iCa concentrations was monitored during the first four days of admission and comparisons between groups were made using Repeated Measures ANOVA. Comparisons of normalization and outcome were made between hypocalcemic patients who did and did not receive calcium replacement according to the local protocol. The suitability of AdjCa to predict low iCa was determined by analyzing sensitivity, specificity and receiver operating characteristic (ROC) curves. Multivariate logistic regression was performed to determine associations of other electrolyte derangements with hypocalcemia. RESULTS: 55.2% of patients were hypocalcemic on admission; 6.2% severely so. Severely hypocalcemic patients required critical care for longer (P=0.001) compared to normocalcemic or mildly hypocalcemic patients, but there was no difference in mortality between groups (P=0.48). iCa levels normalized within four days in most, with no difference in normalization between those who died and survived (P=0.35). Severely hypocalcemic patients who failed to normalize their iCa by day 4 had double the mortality (38% vs. 19%, P=0.15). Neither iCa normalization nor survival were superior in hypocalcemic patients receiving supplementation on admission. AdjCa<2.2 mmol/L had a sensitivity of 78.2% and specificity of 63.3% for predicting iCa<1.1 mmol/L. Low magnesium, sodium and albumin were independently associated with hypocalcemia on admission. CONCLUSIONS: Hypocalcemia usually normalizes within the first four days after admission to ICU and failure to normalize in severely hypocalcemic patients may be associated with increased mortality. Calcium replacement appears not to improve normalization or mortality. AdjCa is not a good surrogate of iCa in an ICU setting.
Asunto(s)
Gluconato de Calcio/administración & dosificación , Enfermedad Crítica/terapia , Hipocalcemia/diagnóstico , Hipocalcemia/tratamiento farmacológico , Admisión del Paciente/tendencias , Anciano , Femenino , Humanos , Hipocalcemia/sangre , Inyecciones Intravenosas , Masculino , Persona de Mediana Edad , Estudios RetrospectivosRESUMEN
Desde el inicio de la era industrial, el raquitismo ha sido una enfermedad endémica. Con el descubrimiento de la vitamina D y el aporte de suplementos, sobre todo en las fórmulas lácteas infantiles, prácticamente había desaparecido, pero durante los últimos años parece haberse convertido de nuevo en un problema de salud pública. La carencia de vitamina D se asocia no sólo a problemas óseos, sino también a un importante incremento del riesgo de enfermedades cardiovasculares, autoinmunes, infecciosas y tumorales. Comunicamos un caso de tetania neonatal por hipocalcemia secundaria a hipovitaminosis D en un neonato de menos de 2 días de vida. La precocidad de la aparición y la gravedad clínica son excepcionales. Además, advierte del problema emergente que constituye el déficit de vitamina D y de la necesidad de instaurar la suplementación con ésta vitamina, siguiendo las últimas recomendaciones de la Asociación Española de Pediatría (AU)
Since industrial revolution, rickets has been an endemic disease. Since the discovery of vitamin D and its supplements, above all in milk formulates, practically it was disappear, but in the last few years it seems to be again a public health problem. Vitamin D deficiency contributes, not only to bone problems, but also to an important increase in the risk of cardiovascular, autoimmune, infectious diseases and cancer. We communicate a case of neonatal hypocalcemic tetany secondary to a vitamin D deficiency that appeared in a neonate before the second day of life. The precocity of its appearance and its clinical severity are exceptional. It also warn of the emergent problem that it suppose the vitamin D deficiency and the necessity of set up the supplementation with this vitamin, following the last recommendations of the Spanish Paediatrics Association (AU)