Neoplasia endócrina múltipla tipo 2A: relato de caso / Multiple endocrine neoplasia type 2A: a case report / Neoplasia endocrina múltiple tipo 2A: reporte de caso

As neoplasias endócrinas múltiplas (NEM) são síndromes genéticas autossômicas dominantes implicadas no desenvolvimento de neoplasias benignas ou malignas, envolvendo ao menos duas glândulas endócrinas. Entre seus subtipos, está a NEM2A, que consiste em carcinoma medular de tireoide (CMT), feocromocitoma e hiperparatireoidismo. Este texto apresente o relato de caso de um paciente de 40 anos, previamente hígido, que passou a apresentar episódios de cefaleia associada a sudorese profusa, vômitos e taquicardia. Evoluiu com distensão abdominal intensa após alimentação por via oral, perda ponderal, desnutrição, astenia, obstipação, humor deprimido e picos pressóricos. Exames laboratoriais evidenciaram alterações dos hormônios tireoidianos, PTH e hormônios da adrenal. Foi levantada a suspeita clínica de NEM2A, posteriormente corroborada pelos diagnósticos anatomopatológicos de feocromocitoma e CMT, associados à presença de hiperparatireoidismo. Foi possível concluir que, a despeito de sua baixa prevalência na população geral, a NEM é uma síndrome clínica de grande relevância, tendo em vista os impactos para os pacientes e famílias acometidas. Dessa forma, é necessário que os profissionais de saúde tenham conhecimento acerca da síndrome e que o Sistema Único de Saúde (SUS) esteja apto a assistir aos pacientes portadores de NEM, possibilitando diagnóstico precoce e tratamento adequado.

Multiple endocrine neoplasias (MEN) are dominant autosomal genetic syndromes involved in the development of benign or malignant tumors in at least two endocrine glands. MEN2A is one of its subtypes, which consists of medullary thyroid carcinoma (MTC), pheochromocytoma, and hyperparathyroidism. This study reports the case of a healthy 40-year-old male patient presenting with episodes of headache associated with profuse sweating, vomiting, and tachycardia. The patient evolved with severe abdominal distension after oral feeding, weight loss, malnutrition, asthenia, constipation, depressed mood, and pressure peaks. Laboratory tests showed abnormalities in thyroid, parathyroid (PTH), and adrenal hormones ­ thus raising the hypothesis of MEN2A, which was later corroborated by the histological diagnosis of pheochromocytoma and MTC, associated with hyperparathyroidism. The results indicate that, despite its low prevalence in the general population, MEN has a great impact on affected patients and families, thus being a relevant clinical syndrome. For enabling early diagnosis and adequate treatment, health professionals must be familiarized with such a condition, and the Brazilian Unified Health System (SUS) must be able to assist affected patients.

Las neoplasias endocrinas múltiples (NEM) son síndromes genéticos autosómicos dominantes involucrados en el desarrollo de neoplasias benignas o malignas, que afectan al menos dos glándulas endocrinas. Entre sus subtipos se encuentra NEM2A, que consiste en carcinoma medular de tiroides (CMT), feocromocitoma e hiperparatiroidismo. Este es un reporte de caso de un paciente de 40 años de edad, previamente sano, que comenzó a presentar episodios de cefalea asociada a sudoración profusa, vómitos y taquicardia. Evolucionó con distensión abdominal severa después de alimentarse, pérdida de peso, desnutrición, astenia, estreñimiento, estado de ánimo deprimido y picos de presión. Las pruebas de laboratorio mostraron alteraciones en las hormonas tiroideas, PTH y hormonas suprarrenales. Se planteó la hipótesis de MEN2A, posteriormente corroborada por el diagnóstico anatomopatológico de feocromocitoma y CMT, asociado a hiperparatiroidismo. Se pudo concluir que, a pesar de su baja prevalencia en la población general, el NEM es un síndrome clínico de gran relevancia, dado el impacto que tiene en los pacientes y familiares afectados. Por tanto, es necesario que los profesionales sanitarios tengan conocimiento sobre el síndrome y que el Sistema Único de Salud (SUS) sea capaz de asistir a los pacientes con NEM, posibilitándoles diagnóstico precoz y tratamiento adecuado.

Causes of hypercalcemia in renal transplant recipients: persistent hyperparathyroidism and others

Hypercalcemia is common in patients after kidney transplantation (KTx) and is associated with persistent hyperparathyroidism in the majority of cases. This retrospective, single-center study evaluated the prevalence of hypercalcemia after KTx. KTx recipients were evaluated for 7 years after receiving kidneys from living or deceased donors. A total of 301 patients were evaluated; 67 patients had hypercalcemia at some point during the follow-up period. The median follow-up time for all 67 patients was 62 months (44; 80). Overall, 45 cases of hypercalcemia were classified as related to persistent post-transplant hyperparathyroidism (group A), 16 were classified as "transient post-transplant hypercalcemia" (group B), and 3 had causes secondary to other diseases (1 related to tuberculosis, 1 related to histoplasmosis, and 1 related to lymphoma). The other 3 patients had hypercalcemia of unknown etiology, which is still under investigation. In group A, the onset of hypercalcemia after KTx was not significantly different from that of the other groups, but the median duration of hypercalcemia in group A was 25 months (12.5; 53), longer than in group B, where the median duration of hypercalcemia was only 12 months (10; 15) (P<0.002). The median parathyroid hormone blood levels around 12 months after KTx were 210 pg/mL (141; 352) in group A and 72.5 pg/mL (54; 95) in group B (P<0.0001). Hypercalcemia post-KTx is not infrequent and its prevalence in this center was 22.2%. Persistent hyperparathyroidism was the most frequent cause, but other important etiologies must not be forgotten, especially granulomatous diseases and malignancies.

Therapy for persistent hypercalcemic hyperparathyroidism post-renal transplant: cinacalcet versus parathyroidectomy / Tratamento do hiperparatireoidismo hipercalcêmico persistente pós-transplante renal: cinacalcete versus paratireoidectomia

ABSTRACT Background: Persistent hyperparathyroidism post-transplant is associated with increases in the incidence of cardiovascular events, fractures, and deaths. The aim of this study was to compare both therapeutic options available: parathyroidectomy (PTX) and the calcimimetic agent cinacalcet. Methods: A single center retrospective study including adult renal transplant recipients who developed hypercalcemia due to persistent hyperparathyroidism. Inclusion criteria: PTH > 65 pg/mL with serum calcium > 11.5 mg/dL at any time after transplant or serum calcium persistently higher than 10.2 mg/dL one year after transplant. Patients treated with cinacalcet (n=46) were compared to patients treated with parathyroidectomy (n=30). Follow-up period was one year. Clinical and laboratory data were analyzed to compare efficacy and safety of both therapeutic modalities. Results: PTX controlled calcemia faster (month 1 x month 6) and reached significantly lower levels at month 12 (9.1±1.2 vs 9.7±0.8 mg/dL, p < 0.05); PTX patients showed significantly higher levels of serum phosphate (3.8±1.0 vs 2.9±0.5 mg/dL, p < 0.05) and returned PTH to normal levels (45±51 pg/mL). Cinacalcet, despite controlling calcium and phosphate in the long term, decreased but did not correct PTH (197±97 pg/mL). The proportion of patients that remained with PTH above normal range was 95% in the cinacalcet group and 22% in the PTX group. Patients treated with cinacalcet had better renal function (creatinine 1.2±0.3 vs 1.7±0.7 mg/dL, p < 0.05). Conclusions: Surgical treatment was superior to cinacalcet to correct the metabolic disorders of hyperparathyroidism despite being associated with worse renal function in the long term. Cinacalcet proved to be a safe and well tolerated drug.

RESUMO Introdução: O hiperparatireoidismo persistente pós-transplante está associado a aumento na incidência de eventos cardiovasculares, fraturas e óbitos. O objetivo deste estudo foi comparar as opções terapêuticas disponíveis: paratireoidectomia (PTX) e o agente calcimimético cinacalcete. Métodos: Estudo retrospectivo de um único centro incluiu pacientes transplantados renais adultos que desenvolveram hipercalcemia devido a hiperparatireoidismo persistente. Critérios de inclusão: PTH > 65 pg/mL com cálcio sérico > 11,5 mg/dL a qualquer momento após o transplante, ou cálcio sérico persistentemente superior a 10,2 mg/dL um ano após o transplante. Os pacientes tratados com cinacalcete (n = 46) foram comparados aos pacientes tratados com paratireoidectomia (n = 30). O período de acompanhamento foi de um ano. Dados clínicos e laboratoriais foram analisados para comparar a eficácia e a segurança de ambas as modalidades terapêuticas. Resultados: a PTX controlou a calcemia mais rapidamente (mês 1 x mês 6) e atingiu níveis significativamente mais baixos no mês 12 (9,1 ± 1,2 v.s. 9,7 ± 0,8 mg/dL, p < 0,05); pacientes submetidos à PTX apresentaram níveis significativamente mais altos de fósforo sérico (3,8 ± 1,0 v.s. 2,9 ± 0,5 mg/dL, p < 0,05) e retornaram aos níveis normais de PTH (45 ± 51 pg/mL). O cinacalcete, apesar de controlar o cálcio e o fósforo no longo prazo, diminuiu, mas não corrigiu o PTH (197 ± 97 pg/mL). A proporção de pacientes que permaneceram com PTH acima da faixa normal foi de 95% no grupo cinacalcete e 22% no grupo PTX. Os pacientes tratados com cinacalcete apresentaram melhor função renal (creatinina 1,2 ± 0,3 v.s. 1,7 ± 0,7 mg/dL, p < 0,05). Conclusões: O tratamento cirúrgico foi superior ao cinacalcete para corrigir os distúrbios metabólicos do hiperparatireoidismo, apesar de estar associado a pior função renal no longo prazo. Cinacalcete provou ser um medicamento seguro e bem tolerado.

Depleción corporal de magnesio durante el embarazo por nefropatía hipercalcemica. Reporte de caso y revisión de la literatura / Magnesium depletion secondary to hypercalcémie nephropathy during pregnancy: Case report and literatura review

Resumen El magnesio sérico es el "ion olvidado" en la práctica médica: la mayoría de veces no se tiene en cuenta en los estudios clínicos, sus alteraciones tienden a ser ignoradas y su aproximación terapéutica no está definida de forma adecuada. La sintomatologia producto de la hipomagnesemia es inespecífica y su aproximación diagnostica es compleja. Se presenta el caso de una paciente con hipomagnesemia sintomática severa asociada a daño renal por hipercalcemia durante la gestación.

Abstract Serum magnesium is the «forgotten ion¼ in medical practice. Most of the time it is not taken into account in clinical studies, its alterations tend to be ignored and its therapeutic approach is not well defined. The symptomatology produced by hypomagnesemia is nonspecific and its diagnostic approach is complex. We present the case of a pregnant patient with symptomatic hypomagnesemia secondary to renal damage due to hypercalcemia.

Hiperparatiroidismo ectópico: Detección de la localización mediastinal / Ectopic hyperparathyroidism. Detection of mediastinal localization

El tejido paratiroideo ectópico es una causa frecuente de recurrencia del hiperparatiroidismo (HPT) siendo de 16% en primarios y 14% en secundarios. La localización intratorácica representa el 20-35%, pero solo un 2% requiere una cirugía torácica. El objetivo fue analizar una cohorte de pacientes con diagnóstico de HPT mediastinal operados en un hospital de alta complejidad de Argentina. Se realizó un estudio retrospectivo de todos los operados por HPT entre enero de 2006 y julio 2019 en ese hospital. Se incluyeron aquellos que requirieron acceso torácico por HPT ectópico. En este período se trataron 728 pacientes con HPT primario y secundario. Seis con primario y 3 con secundario requirieron cirugía torácica. Se realizaron 6 videotoracoscopias (VATS) y 3 esternotomías, sin complicaciones graves. Se utilizó biopsia por congelación en todos y dosaje de paratohormona intraoperatoria (PTHi) en 8 casos, que descendió en promedio 65% respecto al valor basal. Se confirmaron 5 adenomas y 4 hiperplasias. La enfermedad paratiroi dea mediastinal representó el 1.65% (12/728), mientras que recibieron tratamiento quirúrgico en nuestra institución 1.24% (9/728). La biopsia por congelación y el descenso de PTHi resultan útiles para confirmar el foco y eventualmente disminuir el riesgo de recurrencia. La VATS es segura pero depende del entrenamiento y de la disponibilidad en el medio asistencial. Si bien el sestamibi es el método con mayor sensibilidad, se propone el uso de 18F-colina PET/TC ante la sospecha de HPT ectópico. La posibilidad de obtener conclusiones basadas en la evidencia requiere de estudios con mayor número de pacientes.

The ectopic parathyroid tissue is a frequent cause of recurrent hyperparathyroidism (HPT), accounting 16% in primary HPT and 14% in secondary cases. Although intrathoracic ectopic glands represent 25-35% of all ectopic cases, only 2% requires thoracic surgery. The aim of this study is to report a case series of patients with ectopic mediastinal HPT treated by thoracic approach in a private institution in Argentina. This is a retrospective analysis from January 2006 to June 2019. All patients diagnosed with ectopic hyperparathyroidism who required a thoracic surgical approach were included. During this period, 728 patients with primary HPT and secondary HPT were treated. Six with primary HPT and 3 with secondary HPT required a thoracic approach. Six video-assisted thoracoscopy surgeries (VATS) and 3 sternotomies were performed. None of them presented serious posoperative complications. Frozen section biopsy was used in all cases. iPTH was measured in 8 cases, with a mean drop of 65% after 15 minutes. Final pathology reports confirmed 5 adenomas and 4 hyperplasias. Our case series reported an incidence of 1.65% (12/728) mediastinal parathyroids, while 1.24% (9/728) received surgical treatment at our institution. Intraoperative frozen section and PTHi are useful to confirm the diagnosis and to avoid recurrences. Although VATS is a safe and efficient treatment option, it depends on surgical training and availability. In terms of diagnostic imaging resources, sestamibi remains the current gold standard. However, 18F-choline PET/CT may arise as a new diagnostic tool. The possibility of obtaining evidence-based conclusions requires studies with higher number of patients.

Association between hypocalcemia and rivaroxaban in coagulation disorders: a case report / Associação entre hipocalcemia e rivaroxaban em distúrbios de coagulação: relato de caso

ABSTRACT We describe a patient with tertiary hyperparathyroidism with history of three episodes of deep vein thrombosis and on rivaroxaban. The patient underwent a subtotal parathyroidectomy, developing cervical hematoma with airway compression. Therefore, emergency surgical decompression was necessary. Later, on the ninth postoperative day, the serum ionized calcium levels were low. Medical team knowledge about preexisting diseases and their implication in the coagulation state are essential conditions to reduce morbidity and mortality of surgeries. However, no reports were found in literature about the association of hypocalcemia with the use of the new class of anticoagulants, which act as factor X inhibitors (Stuart-Prower factor), predisposing to increased bleeding in the immediate postoperative period.

RESUMO Descrevemos um paciente com hiperparatireoidismo terciário com história de três episódios de trombose venosa profunda e em uso de rivaroxabana. O paciente foi submetido a uma paratireoidectomia subtotal, desenvolvendo hematoma cervical com compressão das vias aéreas. Foi necessária descompressão cirúrgica de emergência. No nono dia de pós-operatório, os níveis séricos de cálcio iônico estavam baixos. O conhecimento da equipe médica sobre doenças preexistentes e de sua implicação no estado de coagulação é condição indispensável para a redução da morbimortalidade do procedimento cirúrgico. No entanto, não há relatos na literatura associando hipocalcemia com o uso da nova classe de anticoagulantes que atuam como inibidores do fator X (fator de Stuart-Prower), predispondo ao aumento do sangramento no pós-operatório imediato.

Perfil Clínico e Bioquímico de Pacientes em Hemodiálise com Hiperparatireoidismo Secundário / Clinical and Biochemical Profile of Patients on Hemodialysis with Secondary Hyperparathyroidism

Objetivo: Avaliar o perfil clínico e bioquímico de pacientes com e sem HPTS. Metodologia:Estudo transversal e analítico realizado com 93 pacientes com DRC em HD. Os pacientes foram alocados em dois grupos: sem HPTS (Paratormônio: < 300pg/mL) e com HPTS (Paratormônio: ≥ 300pg/mL). Foram verificados os dados socioeconômicos por meio de questioná-rio semiestruturado. Os dados bioquímicos e antropométricos foram obtidos por meio do prontuário de cada paciente. Tam-bém foram coletadas informações quanto ao tempo de HD, tabagismo, diabetes mellitus, hipertensão arterial sistêmica, cardiopatias, atividade física e a razão triglicerídeo/HDL-Coles-terol. Resultados:O gênero feminino apresentou-se prevalente no grupo com HPTS (59,09%, p=0,017). Nos pacientes que não apresentavam HPTS foi detectado maior presença de diabetes mellitus (77,27%, p=0,021). Os pacientes com HPTS diferiram dos pacientes sem HPTS para o hematócrito (p=0,0457), crea-tinina (p=0,0303) e a fosfatase alcalina (p=0,0011). Conclusão:Encontramos diferença ao comparar os grupos quanto aos níveis de hematócrito, creatinina, fósforo e fosfatase alcalina, resultados que podem implicar no estado clínico de indivíduos com HPTS. Essas análises poderão direcionar condutas e estratégias específicas para o acompanhamento da doença e proposta adequada de tratamento, buscando a melhora do quadro clínico e qualidade de vida. (AU)

Objective: To evaluate the clinical and biochemical profile of patients with and without secondary hyperparathyroidism. Methodology: Cross-sectional and analytical study conducted with 93 patients with CKD in HD. The patients were allocated into two groups: without HPTS (Parathyroid hormone: <300pg / mL) and with HPTS (Parathyroid hormone: ≥ 300pg / mL). Socioeconomic data were verified through a semi-structured questionnaire. Biochemical and anthropometric data were obtained from each patient's medical record. Information was also collected regarding HD time, smoking, diabetes mellitus, systemic arterial hypertension, heart disease, physical activity and the triglyceride / HDL-cholesterol ratio. Results: The female gender was prevalent in the group with HPTS (59.09%, p = 0.017). In patients without HPTS, a higher presence of diabetes mellitus was detected (77.27%, p = 0.021). Patients with HPTS differed from patients without HPTS for hematocrit (p = 0.0457), creatinine (p = 0.0303) and alkaline phosphatase (p = 0.0011). Conclusion: We found a difference when comparing the groups regarding hematocrit, creatinine, phosphorus and alkaline phosphatase levels. Results that may imply the clinical status of individuals with HPTS. These analysis may direct specific conducts and strategies for the follow-up of the disease and appropriate treatment proposal, seeking to improve the clinical condition and quality of life. (AU)

Tumor marrom do hiperparatireoidismo: relato de caso / Brown hyperparatireoidism tumor: case report

Introdução: a prevalência de doenças endócrino-metabólicas, como, por exemplo, o hiperparatireoidismo, tem crescido com a melhoria da expectativa de vida da população e pode levar a alterações no sistema estomatognático. Objetivo: relatar um caso clínico de um paciente com diagnóstico de tumor marrom associado ao hiperparatireoidismo. Metodologia: a metodologia adotada neste artigo foi o relato de caso, através da revisão de prontuário de um paciente atendido no ambulatório da disciplina de Estomatologia II na Faculdade de Odontologia da Universidade Federal da Bahia. Resultados: Clinicamente, observou-se um aumento de volume da hemiface esquerda e, radiograficamente, pôde-se observar imagem radiopaca envolvendo processo alveolar e seio maxilar esquerdo. Além disso, os exames laboratoriais mostraram elevados níveis do hormônio paratireoidiano. Conclusão: as características clínicas, radiológicas e histológicas das lesões de tumor marrom devem ser analisadas junto à condição sistêmica do indivíduo, a fim de propiciar correto diagnóstico e intervenção precisa.

Introduction: the prevalence of endocrine-metabolic diseases, such as hyperparathyroidism, has increased with the improvement in life expectancy of the population and may lead to changes in the stomatognathic system.Objective: to report a clinical case of a patient diagnosed with brown tumor associated with hyperparathyroidism. Methodology: methodology adopted in this article was the case report, through the review of the medical records of a patient treated at the outpatient clinic of Stomatology II at the School of Dentistry of the Federal University of Bahia. Results: clinically, an enlarged left hemiface was observed, and radiographically, a radiopaque image involving the alveolar process and left maxillary sinus could be observed. In addition, laboratory tests showed high levels of parathyroid hormone. Conclusion: clinical, radiological and histological characteristics of brown tumor lesions should be analyzed with the individual's systemic condition, in order to provide correct diagnosis and precise intervention.

Serum PTH reference values in an adult Brazilian population: implications for the diagnosis of hyperparathyroidism

ABSTRACT Objective: To define serum parathyroid hormone (PTH) reference values in carefully selected subjects following the recommended pre-analytical guidelines. Subjects and methods: First, 676 adults who would be submitted to thyroidectomy were evaluated. Patients using interfering medications or with malabsorption syndrome, hypomagnesemia, hyper- or hypophosphatemia, hypo- or hypercalcemia, 25-hydroxyvitamin D < 30 ng/dL, estimated glomerular filtration rate < 60 mL/min/1.73 m2, urinary calcium/creatinine ratio ≥ 0.25, thyroid dysfunction, parathyroid adenoma detected during surgery were excluded. The sample consisted of 312 subjects. Results: The median, minimum, maximum, and 2.5th and 97.5th percentiles of the PTH values obtained were 30, 7.2, 78, 10.1, and 52 pg/mL, respectively. Thus, the reference range was 10 to 52 pg/mL. PTH > 65 pg/mL, the upper limit of normal according to the manufacturer of the kit, was observed in only one subject (0.3%). Considering the upper limit proposed by the kit's manufacturer, 1/6 hypercalcemic patients and 4/8 normocalcemic patients with PHPT had normal PTH. Using the upper limit established in this study, only one normocalcemic patient had normal PTH. Thus, the sensitivity of PTH in detecting asymptomatic primary hyperparathyroidism (PHPT) using the values recommended by the kit and established in this study was 64% and 93%, respectively (50% versus 87.5% for normocalcemic PHPT). Conclusion: The upper reference limit of PTH obtained for a rigorously selected sample was 20% lower than that provided by the assay, which increased its sensitivity in detecting PHPT.

Tumor pardo en esqueleto axial de dos pacientes con enfermedad renal crónica en diálisis / Brown tumor in the axial skeleton of two patients with chronic kidney disease in dialysis

Resumen Los tumores pardos son una forma localizada de osteítis fibrosa no neoplásica, secundaria a hiperparatiroidismo (primario o secundario). Hacen parte de las alteraciones del metabolismo mineral y óseo de la enfermedad renal crónica (ERC). Se manifiestan como lesiones líticas, expansivas, asociadas a masas de tejidos blandos, que pueden estar localizadas en cualquier parte del esqueleto, con predilección por las costillas, clavículas, pelvis, fémur, huesos faciales y mandíbula. Reportamos dos casos de paciente con ERC en terapia de reemplazo renal (TRR), con hiperparatiroidismo secundario y tumores pardos localizados en mandíbula, arcos costales y cuerpos vertebrales, con manifestaciones radiológicas atípicas. Conclusión: los tumores pardos hacen parte de las alteraciones óseas de los pacientes con ERC. El aspecto benigno en los estudios de imágenes (lesiones expansivas sin destrucción de la cortical) en el contexto de un paciente con hiperparatiroidismo, pueden sugerir el diagnóstico. (Acta Med Colomb 2018; 43: 221-225).

Abstract Brown tumors are a localized form of non-neoplastic osteitis fibrosa, secondary to hyperparathyroidism (primary or secondary). They are part of the alterations of the mineral and bone metabolism of chronic kidney disease (CKD). They manifest as lytic, expansive lesions associated to soft tissue masses that can be located in any part of the skeleton with predilection for the ribs, clavicles, pelvis, femur, facial bones and jaw. Two cases of patients with CKD in renal replacement therapy (RRT), with secondary hyperparathyroidism and brown tumors located in the jaw, costal arches and vertebral bodies, with atypical radiological manifestations are described. Conclusion: brown tumors are part of the bone disorders of patients with CKD. The benign appearance in imaging studies (expansive lesions without destruction of the cortex) in the context of a patient with hyperparathyroidism, may suggest the diagnosis. (Acta Med Colomb 2018; 43: 221-225).

Disparity between Preoperative and Pre-Excisional Intraoperative Parathyroid Hormone in Parathyroid Surgery

Abstract Introduction Intraoperative parathyroid hormone (ioPTH) testing is a widely accepted standard for assessing the parathyroid gland function. A decline of preoperative parathyroid hormone (PTH) levels by more than 50% is one accepted measure of parathyroid surgery adequacy. However, there may be a variation between preoperative PTH levels obtained at a clinic visit and pre-excisional ioPTH. Objective Our study explores the differences between preoperative PTH and pre-excisional ioPTH levels, and the potential impact this difference has on determining the adequacy of parathyroid surgery. Methods A retrospective study that consisted of 33 patients that had undergone parathyroid resection between September 2009 and March 2016 at a tertiary academic center was performed. Each subject's preoperative PTH levels were obtained from clinic visits and pre-excisional ioPTH levels were recorded along with the time interval between the measurements. Results There was a significant difference between the mean preoperative PTH and the pre-excisional ioPTH levels of 147 pg/mL (95% confidence interval [CI] 11.43 to 284.47; p= 0.0396). The exclusion of four outliers revealed a further significant difference with a mean of 35.09 pg/mL (95% CI 20.27 to 49.92; p< 0.0001). The average time interval between blood draws was 48 days + 32 days. A weak correlation between the change in PTH values and the time interval between preoperative and pre-excision blood draws was noted (r2 = 0.15). Conclusion Our study reveals a significant difference between the preoperative PTH levels obtained at clinic visits and the pre-excisional intraoperative PTH levels. We recommend routine pre-excisional intraoperative PTH levels, despite evidence of elevated preoperative PTH levels, in order to more accurately assess the adequacy of surgical resection.

Experiencia con paratiroidectomía quirúrgica en pacientes con enfermedad renal crónica e hiperparatiroidismo terciario / Experience with surgical parathyroidectomy in patients with chronic kidney disease and tertiary hyperparathyroidism

Resumen Introducción: el hiperparatiroidismo terciario es la consecuencia final de las alteraciones del metabolismo calcio-fósforo en pacientes con enfermedad renal crónica. Aquellos pacientes que no logran controlarlo con el tratamiento médico, requieren paratiroidectomía. Objetivo: describir la evolución clínica y paraclínica en pacientes con enfermedad renal crónica y diagnóstico de hiperparatiroidismo terciario, que fueron llevados a paratiroidectomía. Metodología: estudio prospectivo, que incluyó pacientes con hiperparatiroidismo terciario llevados a paratiroidectomía entre los años 2006 y 2015. Se realizaron estudios bioquímicos pre y post quirúrgicos y se evaluó la presencia y progresión de síntomas. Resultados: se incluyeron 32 pacientes, 68.8% mujeres, con media de edad de 46.2 años. Se identificaron diferencias estadísticamente significativas en el cambio en los valores de calcio, fósforo y PTH pre y post quirúrgicos. El 81.5% de los pacientes reportaron presencia de síntomas. El 34.6, 26.9, y 23% presentaron mejoría total de dolor óseo, dolor articular y prurito, respectivamente. El 28% presentaron hipocalcemia sintomática post operatoria. No hubo casos de muerte durante el seguimiento. Conclusiones: la paratiroidectomía se constituye en un alternativa segura y confiable para los pacientes con hiperparatiroidismo terciario, mejorando significativamente los síntomas y las alteraciones en el metabolismo óseo y mineral. Vigilar los niveles de calcio en el post operatorio es importante para evitar las complicaciones del síndrome de hueso hambriento.

Abstract Introduction: tertiary hyperparathyroidism is the final consequence of alterations in calcium-phosphorus metabolism in patients with chronic kidney disease. Those patients who fail to control it with medical treatment require parathyroidectomy. Objective: go describe the clinical and paraclinical evolution in patients with chronic kidney disease and diagnosis of tertiary hyperparathyroidism, who underwent parathyroidectomy. Methodology: prospective study, which included patients with tertiary hyperparathyroidism taken to parathyroidectomy between 2006 and 2015. Pre and post-surgical biochemical studies were performed and the presence and progression of symptoms was evaluated. Results: 32 patients were included. 68.8 (%) were women with an average age of 46.2 years. Statistically significant differences were identified in the change in calcium, phosphorus and PTH values before and after surgery. 81.5% of patients reported the presence of symptoms. 34.6, 26.9, and 23% presented total improvement of bone pain, joint pain and pruritus, respectively. 28% presented postoperative symptomatic hypocalcemia. There were no deaths during follow-up. Conclusions: parathyroidectomy is a safe and reliable alternative for patients with tertiary hyper-parathyroidism, significantly improving symptoms and alterations in bone and mineral metabolism. Monitoring calcium levels in the postoperative period is important to avoid the complications of the hungry bone syndrome.

Hiperparatiroidismo asociado a la enfermedad renal crónica / Hyperparathyroidism and chronic kidney disease

El hiperparatiroidismo terciario (HPTT) se caracteriza por hipercalcemia en respuesta a la excesiva secreción de hormona paratiroidea (PTH) en pacientes con hiperparatiroidismo secundario (HPTS) que responden de manera ineficaz al tratamiento médico, o como con-secuencia de un mal control del metabolismo fosfo-cálcico que ocasiona estimulación prolongada de las células paratiroideas, hi-perplasia y con frecuencia formación de un adenoma glandular. El HPTT puede manifes-tarse con prurito, dolores óseos, calcificación de tejidos blandos y alteraciones bioquímicas, en especial aumento de los niveles de calcio, fósforo y fosfatasa alcalina. Esta alteración puede darse en pacientes con enfermedad renal crónica en diálisis (ERCD) o en pa-cientes que han recibido un trasplante renal; en este último escenario, puede persistir una excesiva secreción de PTH por la hipertrofia de las glándulas paratiroides; sin embargo, la secreción hormonal en esta población remite hasta en 90% de los casos después de 6 meses de realizado el trasplante.

Lesiones óseas multicéntricas por trastorno paratiroideo: Reporte de un caso clínico / Multicentric Bone Lesions Due to Parathyroid Disease: A Case Report

Resumen Introducción: El hiperparatiroidismo (Hpt) es un desorden endócrino que crea condiciones de hipersecreción de la hormona paratiroidea, propicias para el asentamiento de tumores en el tejido óseo. Si bien no es frecuente, los huesos maxilares, pueden estar involucrados en la patogenia de estas lesiones. Pacientes y métodos: En este trabajo, se presenta un caso clínico de una paciente femenina de 56 años de edad, con lesiones óseas multicéntricas maxilares y extramaxilares a predominio de células gigantes, vinculado a un hiperparatiroidismo. Discusión: El diagnóstico de un Tumor Pardo del Hiperparatiroidismo (TPH) es difícil en la clínica estomatológica, donde la metodología de diagnóstico debe reunir criterios serológicos, clínicos e histopatológicos. La multifocalidad de las lesiones, hacen a este caso un verdadero síndrome endócrino, siendo éste el aspecto más importante. Esta entidad, de incumbencia en la Endocrinología y en la Medicina Bucal, debe ser tratada fundamentalmente de forma interdisciplinar para lograr un tratamiento exitoso.

Abstract Introduction: Hyperparathyroidism is an endocrine disorder that could create parathyroid hormone hypersecretion conditions, propitious for bone tumors development. Although is not frequent, maxillary bones may be involved in the pathogenesis of these lesions. PATIENTS AND METHODS: In this case, a 56-year-old female is reported. An Hyperparathyroidism was diagnosed due to multiple maxillary and extra-maxillary multicentric bone lesions, with a predominance of giant cells, linked to her endocrine disorder. Discussion: The diagnosis of a Hyperparathyroidism Brown Tumor must meet serological, clinical and histopathological criteria. The multifocality of the lesions is the most remarkable clinical aspect in this case. For an adequate treatment of this entity is necessary an interdisciplinary and coordinate work between different areas of healthcare physicians and Oral Medicine specialist.