RESUMEN
OBJECTIVES: Secondary hyperparathyroidism (sHPT) is an important contributor to bone disease and cardiovascular calcifications in children with chronic kidney disease (CKD). When conservative measures are ineffective, parathyroidectomy is indicated. The aim of our study was to evaluate the efficacy and safety of subtotal parathyroidectomy (sPTX) in pediatric and adolescent patients, and to provide a rationale for considering this aggressive treatment in CKD patients with uncontrolled sHPT. METHODS: We retrospectively analyzed the medical records of 19 pediatric CKD patients on dialysis with refractory sHPT who underwent sPTX at our institution between 2010 and 2020. All patients had clinical, radiological, and biochemical signs of renal osteodystrophy. RESULTS: One year after sPTX, parathyroid hormone (PTH) levels (median and interquartile range (IQR)) dropped from 2073 (1339-2484) to 164 (93-252) pg/mL (p=0.0001), alkaline phosphatase (ALP) levels from 1166 (764-2373) to 410 (126-421) IU/L (p=0.002), and the mean (±SDS) calcium-phosphate (Ca*P) product from 51±11 to 41±13 mg2/dL2 (p=0.07). Postoperatively, all patients presented with severe hungry bone syndrome (HBS) and required intravenous and oral calcium and calcitriol supplementation. None of them had other postoperative complication. Histological findings had a good correlation with preoperative parathyroid ultrasound imaging (n: 15) in 100â¯% and with technetium-99m (99mTc) sestamibi scintigraphy (n: 15) in 86.6â¯%. Clinical and radiological signs of bone disease improved in all patients. CONCLUSIONS: Pediatric sPTX is effective and safe to control sHPT and calcium-phosphate metabolism in children with CKD on dialysis and may mitigate irreversible bone deformities and progression of cardiovascular disease.
Asunto(s)
Enfermedades Óseas , Hiperparatiroidismo Secundario , Insuficiencia Renal Crónica , Adolescente , Humanos , Niño , Calcio , Estudios Retrospectivos , Hiperparatiroidismo Secundario/cirugía , Hiperparatiroidismo Secundario/complicaciones , Insuficiencia Renal Crónica/complicaciones , Insuficiencia Renal Crónica/terapia , Hormona Paratiroidea , Paratiroidectomía/efectos adversos , Paratiroidectomía/métodos , Calcio de la Dieta , FosfatosRESUMEN
PURPOSE: Hyperuricemia is common among patients with chronic kidney disease (CKD). In the general population, hyperuricemia is associated with secondary hyperparathyroidism (SHPT), in a mechanism that involves vitamin D metabolism. Data for patients with CKD, however, are scarce. We aimed to evaluate the relationship between hyperuricemia and mineral and bone metabolism, particularly hyperparathyroidism. METHODS: This is a retrospective study that included 922 adult patients with stages 3, 4, or 5 CKD, not on dialysis. Clinical, demographic, and biochemical data were collected from charts and included uric acid, parathyroid hormone (PTH), 25(OH)-vitamin D, calcium, phosphate, renal function (estimated glomerular filtration rate-eGFR), and medications such as allopurinol, furosemide, and cholecalciferol. SHPT was defined as PTH > 65 pg/ml. RESULTS: Our patients were mostly Caucasian women, with a mean age of 64 ± 16 years. SHPT and hyperuricemia were observed in 70% and 62.4% of patients, respectively. Patients with SHPT presented higher levels of uric acid (7.2 ± 1.8 vs. 6.6 ± 1.7 mg/dL, p = 0.0001) and a higher frequency of hyperuricemia (66% vs. 33%, p = 0.0001). Patients with hyperuricemia were mostly female, with lower eGFR, higher phosphate, and higher PTH. The risk of hypovitaminosis D was higher among patients with SHPT (69.7% vs. 53.1%, p = 0.0001). Hyperuricemia remained independently associated with hyperparathyroidism, (p = 0.033) even after adjustments for eGFR, calcium, phosphate, hypovitaminosis D, and use of allopurinol, calcitriol, furosemide, and cholecalciferol. CONCLUSION: Hyperuricemia seems to be a contributing factor for SHPT in patients with CKD. The mechanisms behind this finding have yet to be elucidated.
Asunto(s)
Hiperparatiroidismo Secundario , Hiperuricemia , Insuficiencia Renal Crónica , Deficiencia de Vitamina D , Adulto , Anciano , Anciano de 80 o más Años , Alopurinol/uso terapéutico , Calcio/uso terapéutico , Colecalciferol/uso terapéutico , Femenino , Furosemida/uso terapéutico , Humanos , Hiperparatiroidismo Secundario/complicaciones , Hiperparatiroidismo Secundario/epidemiología , Hiperuricemia/complicaciones , Hiperuricemia/epidemiología , Masculino , Persona de Mediana Edad , Hormona Paratiroidea , Fosfatos , Insuficiencia Renal Crónica/complicaciones , Estudios Retrospectivos , Ácido Úrico , Vitamina D/uso terapéutico , Deficiencia de Vitamina D/complicacionesRESUMEN
Abstract Introduction: Vascular calcification related to severe secondary hyperparathyroidism (SHPT) is an important cause of cardiovascular and bone complications, leading to high morbidity and mortality in patients with chronic kidney disease (CKD) undergoing hemodialysis (HD). The present study aimed to analyze whether ankle-brachial index (ABI), a non-invasive diagnostic tool, is able to predict cardiovascular outcomes in this population. Methods: We selected 88 adult patients on HD for at least 6 months, with serum iPTH>1,000pg/mL. We collected clinical data, biochemical and hormonal parameters, and ABI (sonar-Doppler). Calcification was assessed by lateral radiography of the abdomen and by simple vascular calcification score (SVCS). This cohort was monitored prospectively between 2012 and 2019 for cardiovascular outcomes (death, myocardial infarction (MI), stroke, and calciphylaxis) to estimate the accuracy of ABI in this setting. Results: The baseline values were: iPTH: 1770±689pg/mL, P: 5.8±1.2 mg/dL, corrected Ca: 9.7±0.8mg/dL, 25(OH)vit D: 25.1±10.9ng/mL. Sixty-five percent of patients had ABI>1.3 (ranging from 0.6 to 3.2); 66% had SVCS≥3, and 45% aortic calcification (Kauppila≥8). The prospective evaluation (51.6±24.0 months), provided the following cardiovascular outcomes: 11% of deaths, 17% of nonfatal MI, one stroke, and 3% of calciphylaxis. After adjustments, patients with ABI≥1.6 had 8.9-fold higher risk of cardiovascular events (p=0.035), and ABI≥1.8 had 12.2-fold higher risk of cardiovascular mortality (p=0.019). Conclusion: The presence of vascular calcifications and arterial stiffness was highly prevalent in our population. We suggest that ABI, a simple and cost-effective diagnostic tool, could be used at an outpatient basis to predict cardiovascular events in patients with severe SHPT undergoing HD.
Resumo Introdução: A calcificação vascular relacionada ao hiperparatireoidismo secundário (HPTS) grave é uma causa importante de complicações cardiovasculares e ósseas, levando a alta morbidade e mortalidade em pacientes com doença renal crônica (DRC) em hemodiálise (HD). O presente estudo objetivou analisar se o índice tornozelo-braquial (ITB), uma ferramenta diagnóstica não invasiva, pode predizer desfechos cardiovasculares nesta população. Métodos: Selecionamos 88 adultos em HD há pelo menos 6 meses, com PTHi sérico>1.000pg/mL. Coletamos dados clínicos, parâmetros bioquímicos e hormonais, e ITB (sonar-Doppler). A calcificação foi avaliada por radiografia lateral do abdome e por escore de calcificação vascular simples (ECVS). Esta coorte foi monitorada prospectivamente entre 2012 e 2019 para desfechos cardiovasculares (óbito, infarto do miocárdio (IM), acidente vascular cerebral e calcifilaxia) para estimar a precisão do ITB neste cenário. Resultados: Os valores basais foram: PTHi: 1770±689pg/mL, P: 5,8±1,2 mg/dL, Ca corrigido: 9,7±0,8mg/dL, 25(OH)vit D: 25,1±10,9ng/Ml; 65% dos pacientes apresentaram ITB>1,3 (variando de 0,6 a 3,2); 66% tiveram ECVS≥3, e 45% calcificação da aorta (Kauppila≥8). A avaliação prospectiva (51,6±24,0 meses) forneceu os seguintes desfechos cardiovasculares: 11% de óbitos, 17% de IM não fatal, um AVC, 3% de calcifilaxia. Após ajustes, pacientes com ITB≥1,6 tiveram risco 8,9 vezes maior de eventos cardiovasculares (p=0,035), e ITB≥1,8 apresentaram risco 12,2 vezes maior de mortalidade cardiovascular (p=0,019). Conclusão: A presença de calcificações vasculares e rigidez arterial foi altamente prevalente em nossa população. Sugerimos o ITB, uma ferramenta diagnóstica simples e econômica, para ser usada em ambulatório para prever eventos cardiovasculares em pacientes com HPTS grave em HD.
Asunto(s)
Humanos , Adulto , Enfermedades Cardiovasculares/etiología , Hiperparatiroidismo Secundario/complicaciones , Infarto del Miocardio , Factores de Riesgo , Diálisis Renal , Índice Tobillo BraquialRESUMEN
INTRODUCTION: Vascular calcification related to severe secondary hyperparathyroidism (SHPT) is an important cause of cardiovascular and bone complications, leading to high morbidity and mortality in patients with chronic kidney disease (CKD) undergoing hemodialysis (HD). The present study aimed to analyze whether ankle-brachial index (ABI), a non-invasive diagnostic tool, is able to predict cardiovascular outcomes in this population. METHODS: We selected 88 adult patients on HD for at least 6 months, with serum iPTH>1,000pg/mL. We collected clinical data, biochemical and hormonal parameters, and ABI (sonar-Doppler). Calcification was assessed by lateral radiography of the abdomen and by simple vascular calcification score (SVCS). This cohort was monitored prospectively between 2012 and 2019 for cardiovascular outcomes (death, myocardial infarction (MI), stroke, and calciphylaxis) to estimate the accuracy of ABI in this setting. RESULTS: The baseline values were: iPTH: 1770±689pg/mL, P: 5.8±1.2 mg/dL, corrected Ca: 9.7±0.8mg/dL, 25(OH)vit D: 25.1±10.9ng/mL. Sixty-five percent of patients had ABI>1.3 (ranging from 0.6 to 3.2); 66% had SVCS≥3, and 45% aortic calcification (Kauppila≥8). The prospective evaluation (51.6±24.0 months), provided the following cardiovascular outcomes: 11% of deaths, 17% of nonfatal MI, one stroke, and 3% of calciphylaxis. After adjustments, patients with ABI≥1.6 had 8.9-fold higher risk of cardiovascular events (p=0.035), and ABI≥1.8 had 12.2-fold higher risk of cardiovascular mortality (p=0.019). CONCLUSION: The presence of vascular calcifications and arterial stiffness was highly prevalent in our population. We suggest that ABI, a simple and cost-effective diagnostic tool, could be used at an outpatient basis to predict cardiovascular events in patients with severe SHPT undergoing HD.
Asunto(s)
Enfermedades Cardiovasculares , Hiperparatiroidismo Secundario , Infarto del Miocardio , Adulto , Índice Tobillo Braquial , Enfermedades Cardiovasculares/etiología , Humanos , Hiperparatiroidismo Secundario/complicaciones , Diálisis Renal , Factores de RiesgoRESUMEN
We present the case of a 65-year-old man with brown tumors due to secondary hyperparathyroidism. Magnetic resonance imaging of the pelvis showed multiple lesions with expansive bone appearance. Additionally, prostate cancer was diagnosed during this time. For this reason, differential diagnosis was performed through biopsy of the right iliac bone lesion. Brown tumors are caused by osteoclastic activity and fibroblast proliferation; the differential diagnosis of these bone lesions includes giant tumors, metastases, Paget's disease, and paraneoplastic syndrome with high levels of parathyroid hormone-related peptide (PTHrP). This case report describes the coexistence of two pathologies that could explain these images. In this report, we present a case of a 65-year-old man with brown tumors due to secondary hyperparathyroidism and prostate cancer. In this setting, histologic confirmation is recommended.
Asunto(s)
Hiperparatiroidismo Secundario , Osteítis Deformante , Osteítis Fibrosa Quística , Neoplasias de la Próstata , Anciano , Diagnóstico Diferencial , Humanos , Hiperparatiroidismo Secundario/complicaciones , Masculino , Osteítis Deformante/complicaciones , Osteítis Fibrosa Quística/complicaciones , Osteítis Fibrosa Quística/diagnóstico , Neoplasias de la Próstata/complicacionesRESUMEN
Mineral and bone metabolism disorders are relatively common among patients with end-stage renal disease on maintenance hemodialysis. Corneal and conjunctival calcification is the main extravascular site for calcification. Recently, this form of calcification has been linked to vascular calcification. Secondary hyperparathyroidism can lead to high levels of calcium and phosphorus and increase the risk of calcification. Here, we report a case of a 38-year-old female with severe hyperparathyroidism who underwent eye examination before and after parathyroidectomy. Anterior segment optical coherence tomography showed an improvement in the number and size of ocular calcifications 6 months after surgery. This case calls attention to the importance of eye examination in patients on dialysis and brings the possibility of recovery of calcification in a short-term follow-up.
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Calcinosis/terapia , Conjuntiva/patología , Córnea/patología , Hiperparatiroidismo Secundario/complicaciones , Fallo Renal Crónico/complicaciones , Paratiroidectomía , Adulto , Calcinosis/patología , Femenino , Humanos , Hiperparatiroidismo Secundario/patología , Hiperparatiroidismo Secundario/terapia , Fallo Renal Crónico/patología , Fallo Renal Crónico/terapia , Diálisis RenalRESUMEN
Introducción: El síndrome de Sagliker es una enfermedad rara que requiere manejo de la vía aérea por personal experimentado, fue descrito en 2004 por Sagliker. Presentación del caso: Mujer de 30 años de edad, hipertensa con enfermedad renal crónica de 10 años de evolución, trasplante renal derecho con rechazo al año y manejada con hemodiálisis. Se le realizó paratiroidectomía bilateral, el manejo de la vía aérea fue con la paciente despierta y uso de fibroscopio. La evolución transoperatoria fue buena, se extubó sin incidentes y después de 3 días fue egresada a su domicilio. Conclusiones: El manejo de la vía aérea en pacientes con Síndrome de Sagliker requiere de experiencia ya que un manejo inadecuado compromete la vida de los pacientes.
Sagliker syndrome is a rare disease that requires airway management by experienced personnel, it was described in 2004 by Sagliker. Case presentation: 30-year-old woman, hypertensive with chronic kidney disease of 10 years of evolution, right kidney transplant with one-year rejection and managed with hemodialysis. A bilateral parotidectomy was performed, the airway was managed with the patient awake and using a fibroscope. The intraoperative evolution was good, she was extubated without incident and after 3 days she was discharged to her home. Conclusions: The management of the airway in patients with Sagliker Syndrome requires experience, since inadequate management compromises the lives of patients.
Asunto(s)
Humanos , Femenino , Adulto , Paratiroidectomía , Insuficiencia Renal Crónica/cirugía , Insuficiencia Renal Crónica/terapia , Manejo de la Vía Aérea/métodos , Hiperparatiroidismo Secundario/complicacionesRESUMEN
La paratiroidectomía (PTX) es la terapia de elección en el hiperparatiroidismo secundario a enfermedad renal crónica (HPT-ERC) resistente al tratamiento médico. El objetivo del presente estudio fue evaluar el resultado de la PTX a largo plazo y sus factores predictores. Métodos: estudio unicéntrico retrospectivo observacional. Se incluyeron 92 pacientes con HPT-ERC en diálisis, en quienes se realizó la primera PTX en el Hospital Italiano de Buenos Aires entre 2006 y 2015 con seguimiento ≥ 6 meses. Se consideró persistencia del HPTERC con PTH > 300 pg/ml en el semestre posoperatorio, y recidiva con PTH > 500 pg/ml luego. Resultados: edad: 43,6±12,8 años, 50% mujeres, mediana 4,6 años de diálisis, PTH preoperatoria mediana 1639 pg/ml. A 39 se les realizó PTX subtotal (PTXS) y a 53 total con autoimplante (PTXT+AI). Se observó persistencia en 16 pacientes (17,4%). Presentaron recidiva 30 de 76 pacientes con adecuada respuesta inicial (39,5%; IC 95 28,5-50,5). La mediana de tiempo hasta la recidiva fue de 4,7 años (RIC 2,3-7,5). Los pacientes con recidiva presentaron mayor calcemia preoperatoria (mediana 9,9 vs. 9,3 mg/dl, p=0,035; OR ajustado 2,79) y menor elevación de fosfatasa alcalina en el posoperatorio (333 vs. 436 UI/l, p=0,031; OR ajustado 0,99). La recidiva se presentó más frecuentemente luego de la PTXT+AI (48,9%; OR ajustado 4,66), que en la PTXS (25,8%). Conclusiones: el tiempo en diálisis con inadecuado control metabólico constituye el principal factor para la recurrencia del HPT. Se postula que la mayor calcemia preoperatoria está relacionada con un HPT más severo y se asocia a recurrencia. Llamativamente, hallamos menores elevaciones de la fosfatasa alcalina durante el posoperatorio en pacientes con recurrencia. Hipotetizamos que esto pueda asociarse con menor mineralización en el posoperatorio e hiperfosfatemia sostenida, con consecuente estímulo paratiroideo. La menor recurrencia del HPT luego de la PTXS se vincula al sesgo generado en la selección del tipo de cirugía. (AU)
Parathyroidectomy is an effective therapy for refractory secondary hyperparathyroidism (sHPT). Continued dialysis represents risk for recurrent sHPT. The aim of this study was to estimate the proportion of recurrence and determine its predictors. Methods: We conducted a retrospective observational study of 92 adults in chronic dialysis, who underwent their first parathyroidectomy in this center between 2006 and 2015. We considered persistence of sHPT if PTH was > 300 pg/ml during the first postoperative semester, and recurrence if it was > 500 pg/ml afterwards. Results: Age 43.6+-12 y/o, 50% female, 4.6 years on dialysis, median preoperative PTH 1636 pg/ml (IQR 1226-2098). Subtotal parathyroidectomy (sPTX) was performed in 39, Total with autotransplantation (TA-PTX) in 53 patients. Persistence of sHPT occurred in 16 patients; relapse in 30 out of 76 with adequate initially response (39.5%; 95CI 28,5-50,5). Median time to recurrence: 4.7 y. Recurring patients had higher preoperative calcemia (9.9 vs 9.3 mg/dl; adj OR 2.79) and lower postoperative elevation of ALP (333 vs 436 UI/ml; adj OR 0.99). Recurrence presented more frequently in TA-PTX (48.9%; adj OR 4.66) than sPTX (25.8%). Conclusions: Time on dialysis with inadequate metabolic control remains the most important risk factor for sHPT recurrence. Higher preoperative levels of calcemia, related to sHPT severity, are associated with recurrence. Lower elevations of ALP during postoperative period in recurring patients are an interesting finding. We hypothesize that patients with less significant postoperative mineralization may have chronically higher levels of phosphatemia, stimulating parathyroid glands. Fewer recurrence in sPTX is associated to a bias in the procedure selection. (AU)
Asunto(s)
Humanos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Anciano , Paratiroidectomía/estadística & datos numéricos , Hiperparatiroidismo Secundario/complicaciones , Recurrencia , Vitamina D/uso terapéutico , Calcitriol/análogos & derivados , Calcitriol/uso terapéutico , Calcio/sangre , Estudios Retrospectivos , Diálisis Renal , Fosfatasa Alcalina/sangre , Insuficiencia Renal Crónica/etiología , Insuficiencia Renal Crónica/terapia , Hiperparatiroidismo Secundario/cirugía , Hiperparatiroidismo Secundario/diagnóstico , Hiperparatiroidismo Secundario/terapiaRESUMEN
BACKGROUND: Secondary refractory hyperparathyroidism (SHPT) is usually resolved with total parathyroidectomy. On the other hand, SHPT is associated with persistent hyperparathyroidism as a result of inadequate resection of the parathyroid tissue or by a fifth gland not previously detected. The objective of this work is to present the case of a patient with persistent SHPT and the surgical strategy used for the resection of a hyperfunctional ectopic parathyroid in the thorax. CLINICAL CASE: 48-year-old female, with a history of chronic kidney disease with persistent SHPT, as well as subtotal parathyroidectomy, subsequent resection of parathyroid remnants twice. She was treated with medical management without obtaining adequate response. Previous location of hypercaptant lesion by single photon emission computed tomography, the patient underwent a sternotomy with 3 × 2 cm mediastinal ectopic parathyroid fixed to aortic root. The patient evolved satisfactorily with a decrease in parathyroid hormone levels, with a 18-month follow-up of the procedure with normal paratohormone values and entered the renal transplant protocol while waiting for a donor. CONCLUSION: Sternotomy is a safe approach to perform parathyroidectomy of the ectopic gland, provided that it is well identified the site of the lesion and there are specific conditions appropriate to perform the procedure.
INTRODUCCIÓN: El hiperparatiroidismo secundario (HPS) refractario suele resolverse con paratiroidectomía total. Por otro lado, el HPS se asocia a hiperparatiroidismo persistente como resultado de una resección inadecuada del tejido paratiroideo o por una quinta glándula no detectada previamente. Se presenta el caso de una paciente con HPS persistente con la estrategia quirúrgica utilizada para la resección de una paratiroides ectópica hiperfuncionante en el tórax. CASO CLÍNICO: Mujer de 48 años con antecedente de enfermedad renal crónica con HPS persistente, paratiroidectomía subtotal y posterior resección de remanente de paratiroides en dos ocasiones. Se mantuvo con manejo médico sin obtener adecuada respuesta. Previa localización de una lesión hipercaptante por tomografía computarizada por emisión de fotón único se sometió a esternotomía con resección de paratiroides ectópica mediastinal de 3 × 2 cm, fija a la raíz aórtica. La paciente evolucionó satisfactoriamente, con descenso de la hormona paratiroidea. En seguimiento a 18 meses del procedimiento se mantiene con valores normales de hormona paratiroidea e ingresó a protocolo de trasplante renal a la espera de donador. CONCLUSIÓN: La esternotomía es un abordaje seguro para realizar la paratiroidectomía de la glándula ectópica, siempre y cuando se tenga bien identificado el sitio de la lesión y existan condiciones clínicas adecuadas para realizar el procedimiento.
Asunto(s)
Hiperparatiroidismo Secundario , Femenino , Humanos , Hiperparatiroidismo Secundario/complicaciones , Hiperparatiroidismo Secundario/cirugía , Persona de Mediana Edad , Glándulas Paratiroides/diagnóstico por imagen , Glándulas Paratiroides/cirugía , Hormona Paratiroidea , ParatiroidectomíaRESUMEN
Introducción: El hiperparatiroidismo secundario (HPTS) es una complicación de la enfermedad renal crónica terminal (ERCT). A pesar de nuevas terapias médicas como calcimiméticos, en HPTS refractarios la paratiroidectomía (PTX) continúa siendo necesaria. Una complicación frecuente en estos pacientes posterior a la PTX es el síndrome de hueso hambriento (SHH), caracterizado por una profunda y prolongada hipocalcemia asociada a hipofosfatemia, secundaria a un excesivo aumento de su captación ósea. Una complicación menos descrita, pero con consecuencias graves e incluso fatales, es la hiperkalemia. El propósito de este trabajo consiste en enfatizar el riesgo de hiperkalemia por SHH a partir de un caso clínico, señalar los mecanismos fisiopatológicos, factores de riesgo y consideraciones terapéuticas. Caso clínico: Mujer de 35 años, con ERCT de causa desconocida, HPTS refractario con PTX total e implante de glándulas en antebrazo hace 9 años. Ingresa por recurrencia de HPTS. Cintigrama MIBI SPECT/CT® evidenció implante hiperfuncionante, indicándose PTX del injerto. Exámenes preoperatorios: calcemia 8.6 mg/dL, fosfatasas alcalinas 1115 UI/L (VN <100), PTH intacta (PTHi) 3509 pg/ml y kalemia 4.8 mEq/L. Biopsia: hiperplasia paratiroidea nodular. En postoperatorio inmediato presentó hiperkalemia de 7.1 mEq/L con cambios electrocardiográficos, requiriendo hemodiálisis de urgencia. Posteriormente desarrolló hipocalcemia, hipofosfatemia e hipomagnesemia, de difícil control. Discusión: El SHH post HPTS puede coexistir con hiperkalemia postoperatoria inmediata grave, incluso fatal si no se identifica y corrige a tiempo. El mecanismo fisiopatológico aún no está bien dilucidado. Varios factores pudieran intervenir, incluyendo aumento del metabolismo celular, traumatismo tisular, fármacos anestésicos, fluidos perioperatorios y flujo de iones transmembrana. El nivel de potasio previo a la cirugía, menor edad, género masculino, tiempo entre la última hemodiálisis y la cirugía, y duración de la PTX, son factores de riesgo para hiperkalemia postoperatoria. El conocimiento de esta grave complicación permitirá estar preparado para monitorizar y eventualmente tratar.
Introduction: Secondary Hyperparathyroidism (SHPT) is a complication of End-Stage Renal Disease (ESRD). Although new medical therapies (i.e.calcimimetics,) parathyroidectomy (PTX) continues to be necessary in refractory cases. A well-known complication after PTX is an entity called Hungry Bone Syndrome (HBS), characterized by deep and prolonged hypocalcemia associated with hypophosphatemia, secondary to an excessive increase in bone formation. A less reported complication, but with severe or even fatal consequences, is hyperkalemia. The purpose of this work consists of emphasizing the risk of hyperkalemia in HBS, reporting a clinical case that points out the physiopathological mechanisms, risk factors, and therapeutic considerations. Clinical case: 35-year-old woman with ESRD of unknown cause with refractory SHPT with total PTX and forearm gland grafts nine years ago. She presented SHPT recurrency. MIBI SPECT/CT® scan showed a hyperfunctioning implant, indicating graft PTX. Preoperative tests: calcemia 8.6 mg/dL, phosphatemia 7.3 mg/dL, alkaline phosphatases 1115 UI/L (VN<100), intact PTH (iPTH) 3509 pg/ml and kalemia 4.8 mEq/L. Biopsy: parathyroid nodular hyperplasia. In the immediate postoperative period, she presented hyperkalemia at 7.1 mEq/L with electrocardiographic changes, requiring emergency hemodialysis. Later she developed hypocalcemia, hypophosphatemia, and hypomagnesemia of difficult control. Discussion: HBS post PTX can coexist with severe immediate postoperative hyperkalemia, which can be even fatal if not detected and corrected. The physiopathological mechanism is still not entirely elucidated. Various factors could interfere, including an increase in cell metabolism, tissue traumatism, anesthetic drugs, intraoperative fluids, and transmembrane ion flow. Preoperative potassium levels, younger age, male gender, the time elapsed between last hemodialysis and surgery, and duration of PTX are risk factors for post-surgical hyperkalemia. Knowing this severe complication will allow the medical team to be prepared for monitoring and eventually treating it.
Asunto(s)
Humanos , Femenino , Adulto , Enfermedades Óseas Metabólicas/etiología , Paratiroidectomía/efectos adversos , Hiperpotasemia/etiología , Hiperparatiroidismo Secundario/cirugía , Insuficiencia Renal Crónica/complicaciones , Hiperparatiroidismo Secundario/complicacionesRESUMEN
El tumor pardo, también conocido como osteoclastoma ó como osteítis fibrosa quística, es un tumor lítico, que se presenta en hiperparatiroidismo (primario, secundario y terciario), aunque su presentación habitual es altamente invasiva, no tiene potencial de malignidad. Los tumores pardos en la mano son muy poco frecuentes y existen solo algunos reportes de casos. Presentamos un paciente masculino de 18 años con una tumoración dura, no móvil, adherida a planos profundos en región dorsal de la mano derecha sobre el cuarto metacarpiano, que además limita la flexión y extensión del cuarto dedo sin alterar su función neurovascular. El paciente fue sometido a resección de la tumoración que involucraba por completo al cuarto metacarpiano derecho, además se realizó un abordaje lateral directo en miembro pelvico izquierdo para tomar un injerto autólogo de peroné no vascularizado. Es importante la detección temprana de este tipo de tumores y se debe dar un adecuado seguimiento, ya que, al progresar, generan una destrucción ósea importante y el tratamiento se vuelve de mayor complejidad. En etapas tempranas, el manejo agresivo con resección y aporte óseo puede evitar secuelas funcionales. El uso de injerto no vascularizado de peroné de seis centímetros para la sustitución del cuarto metacarpiano por osteolísis secundaria a un tumor pardo es una alternativa adecuada de tratamiento que permite la preservación estético funcional de la mano.
The brown tumour, also known as osteoclastoma, or as osteitis fibrosa cystica, is a lytic tumour, which occurs in hyperparathyroidism (primary, secondary, and tertiary), although its usual presentation is highly invasive, has no potential for malignancy. Brown tumours of the hand are sporadic, and there are only few case reports. The case is presented of an 18-year-old male patient with a solid, non-mobile tumour, adhered to deep planes, in the dorsal region of the right hand over the fourth metacarpal. This also limited the flexion and extension of the fourth finger, but did not show alterations in the neurovascular function of the finger. The patient underwent a tumour resection that completely involved the right fourth metacarpal. A direct lateral approach was made in the left pelvic limb to perform a non-vascularised autologous fibular graft. Early detection of this type of tumour is important, and an adequate follow-up must be carried out, since when they progress, they generate significant bone destruction and the treatment becomes more complex. In early stages, aggressive management of resection and bone support can prevent functional sequelae.
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Humanos , Masculino , Adolescente , Osteítis Fibrosa Quística/cirugía , Huesos del Metacarpo/cirugía , Osteítis Fibrosa Quística/etiología , Osteítis Fibrosa Quística/diagnóstico por imagen , Trastorno Mineral y Óseo Asociado a la Enfermedad Renal Crónica/complicaciones , Trasplante Óseo , Huesos del Metacarpo/diagnóstico por imagen , Peroné/cirugía , Hiperparatiroidismo Secundario/complicacionesAsunto(s)
Humanos , Femenino , Embarazo , Recién Nacido , Fracturas Óseas/etiología , Fracturas Óseas/diagnóstico por imagen , Hiperparatiroidismo Secundario/complicaciones , Hiperparatiroidismo Secundario/etiología , Síndrome de Dificultad Respiratoria del Recién Nacido/etiología , Diagnóstico Diferencial , Intercambio Materno-Fetal , Hipotonía MuscularRESUMEN
PURPOSE OF REVIEW: Although we have seen tremendous advances in the comprehension of CKD-MBD pathophysiology during the last few years, this was not accompanied by a significant change in mortality rate and quality of life. This review will address the traditional and updated pathophysiology of CKD-MBD along with the therapeutic limitations that affect CKD-MBD and proposed alternative treatment targets. RECENT FINDINGS: An innovative concept brings the osteocyte to the center of CKD-MBD pathophysiology, in contrast to the traditional view of the skeleton as a target organ for disturbances in calcium, phosphate, parathyroid hormone, and vitamin D. Osteocytes, through the synthesis of FGF-23, sclerostin, among others, are able to interact with other organs, making bone an endocrine organ. Thus, osteocyte dysregulation might be an early event during the course of CKD. This review will revisit general concepts on the pathophysiology of CKD-MBD and discuss new perspectives for its treatment.
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Trastorno Mineral y Óseo Asociado a la Enfermedad Renal Crónica/terapia , Manejo de la Enfermedad , Hiperparatiroidismo Secundario/complicaciones , Animales , Trastorno Mineral y Óseo Asociado a la Enfermedad Renal Crónica/etiología , Suplementos Dietéticos , Factor-23 de Crecimiento de Fibroblastos , HumanosRESUMEN
OBJECTIVE: To analyze the association between the occurrence of pruritus and adherence to the prescribed diet, biochemical indicators of renal function and the quality of hemodialysis in chronic renal patients. METHOD: A cross-sectional study performed at a dialysis clinic in the Northeast of Brazil, with 200 patients undergoing hemodialysis in the first half of 2015.To analyze the data, inferential statistics were used, using Chi-Square and Fisher's Exact tests; and Mann Whitney U test. RESULTS: The pruritus was present in 51% of the sample, being associated statistically with phosphorus consumption (P = 0.024) and elevation of serum calcium (P = 0.009). CONCLUSION: Pruritus in chronic renal patients undergoing hemodialysis is influenced by adequate nonadherence to the prescribed diet, in addition to the elevation of biochemical indicators of renal function.
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Calcio/sangre , Fallo Renal Crónico/complicaciones , Fósforo Dietético/efectos adversos , Fósforo/sangre , Prurito/etiología , Diálisis Renal , Adulto , Anciano , Terapia Combinada , Estudios Transversales , Dieta con Restricción de Proteínas , Dieta Hiposódica , Exantema/sangre , Exantema/etiología , Femenino , Humanos , Hipercalcemia/complicaciones , Hiperparatiroidismo Secundario/complicaciones , Fallo Renal Crónico/sangre , Fallo Renal Crónico/dietoterapia , Fallo Renal Crónico/terapia , Masculino , Persona de Mediana Edad , Cooperación del Paciente , Prurito/sangre , Calidad de Vida , Diálisis Renal/enfermería , Factores SocioeconómicosRESUMEN
Hyperparathyroidism (HPT) is an endocrine metabolic disorder characterized by increased secretion of parathyroid hormone. Untreated secondary HPT leads to renal osteodystrophy (ROD). Facial skeletal abnormalities in patients with ROD are rare. The purpose of this paper is to report a conservative surgical approach of exuberant osteitis fibrosa lesions in patient with chronic kidney disease. A 24-year-old female was referred to maxillofacial surgery department with giants ROD affecting palate, maxilla, and mandible, resulting in esthetic and functional impairment. The pathogeneses and multidisciplinary management of ROD are discussed with a brief literature review. Eight years after the conservative treatment of exuberant jaw lesions, no noticeable bone changes were observed in the patient. A multidisciplinary therapy is essential for correct diagnosis of ROD and optimal multimodality treatment. The conservative management was an efficient alternative for the success of the case reported.
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Trastorno Mineral y Óseo Asociado a la Enfermedad Renal Crónica/etiología , Hiperparatiroidismo Secundario/complicaciones , Fallo Renal Crónico/complicaciones , Enfermedades Mandibulares/etiología , Enfermedades Mandibulares/cirugía , Enfermedades Maxilares/etiología , Enfermedades Maxilares/cirugía , Procedimientos Quirúrgicos Orales/métodos , Osteítis Fibrosa Quística/etiología , Osteítis Fibrosa Quística/cirugía , Hueso Paladar/patología , Hueso Paladar/cirugía , Diagnóstico Diferencial , Estética Dental , Femenino , Humanos , Imagenología Tridimensional , Fallo Renal Crónico/cirugía , Trasplante de Riñón , Enfermedades Mandibulares/diagnóstico por imagen , Enfermedades Maxilares/diagnóstico por imagen , Hueso Paladar/diagnóstico por imagen , Colgajos Quirúrgicos , Tomografía Computarizada por Rayos X , Adulto JovenAsunto(s)
Médula Ósea/patología , Hiperparatiroidismo Secundario/complicaciones , Mielofibrosis Primaria/diagnóstico , Mielofibrosis Primaria/etiología , Adolescente , Biopsia , Humanos , Hiperparatiroidismo Secundario/diagnóstico , Hiperparatiroidismo Secundario/etiología , Hiperparatiroidismo Secundario/cirugía , Fallo Renal Crónico/complicaciones , Fallo Renal Crónico/diagnóstico , Fallo Renal Crónico/terapia , Masculino , Periodo PosoperatorioRESUMEN
BACKGROUND: Restless leg syndrome (RLS) is a sleep disorder with high prevalence among patients on hemodialysis. It has been postulated that high phosphate and high parathyroid hormone may be implicated in its pathogenesis. Standard international criteria and face-to-face interview are not always applied. METHODS: this was an interventional prospective study in which 19 patients (6 men, aged 48±11 years) with severe hyperparathyroidism were evaluated. RLS diagnosis and rating scale were accessed based on the International RLS Study Group pre- and post-parathyroidectomy. Patients also underwent standard polysomnography. RESULTS: At baseline, RLS was present in 10 patients (52.6%), and pain was the most reported symptom associated with the diagnosis. Patients with RLS had higher serum phosphate (p = 0.008) that remained independently associated with RLS in a logistic regression model, adjusted for hemoglobin, age and gender (HR = 7.28;CI = 1.14-46.3, p = 0.035). After parathyroidectomy, there was a reduction of serum parathyroid hormone, phosphate, calcium and alkaline phosphatase, and an increase of 25(OH)-vitamin D, and Fetuin-A. Parathyroidectomy alleviated RLS (from 52% to 21%; p = 0.04), which was accompanied by a decrease in severity scale, in association with relief of pain and pruritus. Polysomnography in these patients showed an improvement of sleep parameters as measured by sleep efficiency, sleep latency and percentage of REM sleep. CONCLUSION: RLS is associated with high levels of phosphate in patients with severe secondary hyperparathyroidism on hemodialysis. Pain is most reported complain in these patients. Parathyroidectomy provided an opportunity to relief RLS. Whether the reduction of serum phosphorus or parathyroid hormone contributed to this improvement merits further investigation.
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Hiperparatiroidismo Secundario/complicaciones , Paratiroidectomía , Diálisis Renal , Insuficiencia Renal/complicaciones , Síndrome de las Piernas Inquietas/complicaciones , Síndrome de las Piernas Inquietas/cirugía , Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Hormona Paratiroidea/sangre , Fosfatos/sangre , Polisomnografía , Estudios Prospectivos , Índice de Severidad de la Enfermedad , SueñoRESUMEN
Doenças cardiovasculares são frequentes no curso da insuficiência renal crônica, constituem importante causa de óbito, e causam 1/3 das hospitalizações de doentes dialíticos. Hiperparatireoidismo secundárioé o distúrbio metabólico mais comum na insuficiência renal, cuja fisiopatologia envolve alterações no equilíbriodo cálcio, fósforo, calcitriol e paratormônio. Objetivo: Avaliar a prevalência de alterações ecocardiográficas em pacientes renais crônicos com hiperparatireoidismo secundário, de acordo com níveis plasmáticos de paratormônio.Métodos: Estudo retrospectivo, realizado com base em dados registrados em prontuários entre 2005 e 2007,incluindo 52 indivíduos de ambos os sexos, com doença renal crônica em programa regular de diálise, estratificados com base nos níveis plasmáticos de paratormônio em três grupos: Grupo I ≤ 299pg/mL (n=10); Grupo II entre 300-499 pg/mL (n=21); e Grupo III ≥500 pg/mL (n=21). Foram avaliados os seguintes parâmetros ecocardiográficos: diâmetros da raiz da aorta, do átrio esquerdo e dos ventrículos; espessuras do septo e da parede posterior; fração de ejeção; e volumes diastólico e sistólico finais.Resultados: A análise comparativa dos achados ecocardiográficos nos três grupos revelou que a única variávelque apresentou significância estatística (p 0,009) foi a espessura diastólica da parede posterior. Conclusão: Doentes renais crônicos com hiperparatireoidismo secundário podem apresentar alteraçõesecocardiográficas, algumas das quais apresentam correlação com níveis circulantes de paratormônio....
Background: Cardiovascular diseases are frequent in the course of chronic kidney disease, are an important cause of death, and cause 1/3 of hospitalizations of patients on dialysis. Secondary hyperparathyroidism is the most common metabolic disorder in kidney failure. Its pathophysiology involves changes in the balance of calcium, phosphorus, calcitriol and parathyroid hormone. Objective: To assess the prevalence of echocardiographic abnormalities in chronic kidney disease patients with secondaryhyperparathyroidism, according to plasma levels of parathyroid hormone.Methods: Retrospective study conducted based on data recorded in medical records between 2005 and 2007, including 52 individuals of both sexes with chronic kidney disease on a regular dialysis program, stratified based on plasma levels of parathyroid hormoneinto three groups: Group I ≤ 299pg/mL (n=10); Group II between 300-499 pg/mL (n=21); and Group III ≥500 pg/mL (n=21).We evaluated the following echocardiographic parameters: aortic root diameter, left atrial and ventricular diameter; septal and posteriorwall thickness; ejection fraction; and end diastolic and systolic volumes. Results: The comparative analysis of the echocardiographic findings in the three groups revealed that the only variable presenting statistical significance (p 0.009) was diastolic posterior wall thickness. Conclusion: Patients with chronic kidney disease with secondary hyperparathyroidism may present echocardiographic changes, some of which correlate with circulating levels of parathyroid hormone...
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Humanos , Masculino , Femenino , Persona de Mediana Edad , Ecocardiografía/métodos , Hiperparatiroidismo Secundario/complicaciones , Hiperparatiroidismo Secundario/fisiopatología , Insuficiencia Renal Crónica/fisiopatología , Insuficiencia Renal Crónica/mortalidad , Pacientes , Enfermedad Crónica , Calcio/análisis , Calcitriol/análisis , Diálisis Renal/métodos , Enfermedades Cardiovasculares/complicaciones , Enfermedades Cardiovasculares/diagnóstico , Fósforo/análisis , Prevalencia , Estudios Retrospectivos , Factores de Riesgo , Riñón/fisiopatología , Interpretación Estadística de DatosRESUMEN
Abstract Bartter syndrome comprises a group of rare autosomal-recessive salt-losing disorders with distinct phenotypes, but one unifying pathophysiology consisting of severe reductions of sodium reabsorption caused by mutations in five genes expressed in the thick ascending limb of Henle, coupled with increased urinary excretion of potassium and hydrogen, which leads to hypokalemic alkalosis. Bartter syndrome type IV, caused by loss-of-function mutations in barttin, a subunit of chloride channel CLC-Kb expressed in the kidney and inner ear, usually occurs in the antenatal-neonatal period. We report an unusual case of late onset presentation of Bartter syndrome IV and mild phenotype in a 20 years-old man who had hypokalemia, deafness, secondary hyperparathyroidism and erythrocytosis.
Resumo A síndrome de Bartter compreende um grupo raro de doenças autossômicas recessivas perdedoras de sal, decorrentes de mutações em genes expressos na porção ascendente espessa da alça de Henle, com fenótipos distintos, porém fisiopatogenia única, que consiste em redução severa da reabsorção de sódio, e aumento da excreção urinária de hidrogênio e potássio, levando à alcalose hipocalêmica. A síndrome de Bartter tipo IV, causada por mutações com perda de função da bartina, uma subunidade do canal de cloro CLC-Kb expressa no rim e ouvido interno, geralmente se apresenta nos períodos ante e neonatal. No presente relato, descreve-se um caso não usual de síndrome de Bartter tipo IV com apresentação tardia e fenótipo atenuado, diagnosticado por análise molecular, em um homem adulto de 20 anos que se apresentava com hipocalemia, surdez, hiperparatireoidismo secundário e eritrocitose.