Pancreatitis aguda en el escenario de hipercalcemia por hiperparatiroidismo primario / Acute pancreatitis in the setting of hypercalcemia due to primary hyperparathyroidism

Introducción: describir el caso de un paciente con pancreatitis aguda secundaria a hipercalcemia por hiperparatiroidismo prImario. Esta es una causa poco frecuente de pancreatitis, asociada a morbimortalidad significativa en caso de no ser diagnosticada oportunamente Caso clínico: un hombre de 44 años, con antecedente de pancreatitis de presunto origen biliar que había requerido previamente colecistectomía, consultó por dolor abdominal y náuseas. Los estudios complementarios fueron compatibles con un nuevo episodio de pancreatitis aguda. Presentaba hipercalcemia y hormona paratiroidea (PTH) elevada, configurando hiperparatiroidismo primario. La gammagrafía informó hallazgos compatibles con adenoma paratiroideo. Se inició tratamiento con reanimación hídrica y analgesia con adecuada disminución de calcio sérico y resolución de dolor abdominal. Después de la paratiroidectomía se logró normalizar los niveles de calcio y PTH. Discusión: la pancreatitis aguda es una condición potencialmente fatal, por lo que la sospecha de causas poco frecuentes como la hipercalcemia debe tenerse en cuenta. El tratamiento de la hipercalcemia por adenoma paratiroideo se basa en reanimación hídrica adecuada y manejo quirúrgico del adenoma, con el fin de evitar recurrencia de pancreatitis y mortalidad. (AU)

Introduction: we describe the case of a patient with acute pancreatitis secondary to hypercalcemia due to primary hyperparathyroidism. This is a rare cause of pancreatitis associated with significant morbidity and mortality if not diagnosed in time. Clinical case: a 44-year-old man with a history of pancreatitis of presumed biliary origin, which had previously required cholecystectomy, consulted for abdominal pain and nausea. The laboratory findings were compatible with a new episode of acute pancreatitis. He presented hypercalcemia and an elevated parathyroid hormone (PTH), configuring primary hyperparathyroidism. Scintigraphy was performed, yielding findings compatible with parathyroid adenoma. Treatment with fluid resuscitation and analgesia was started, resulting in an adequate decrease in serum calcium and resolution of abdominal pain. After parathyroidectomy, calcium and PTH levels were normalized. Discussion: acute pancreatitis is a potentially fatal condition; therefore the suspicion of rare causes, such as hypercalcemia, should be considered. The treatment of hypercalcemia due to parathyroid adenoma is based on adequate fluid resuscitation and surgical management of the adenoma, to avoid recurrence of pancreatitis and death. (AU)

Hiperparatiroidismo primario por carcinoma paratiroideo: caso problema y revisión / Primary hyperparathyroidism by parathyroid cancer: case report and review

El carcinoma paratiroideo (CP) es una neoplasia maligna con una incidencia de 0,015 cada 100.000 habitantes por año. Representa el 1% de los diagnósticos de hiperparatiroidismo primario y se manifiesta entre la 4.a y 5.a década de la vida, con una incidencia similar entre hombres y mujeres. La etiología del CP es incierta, ha sido asociada a formas esporádicas o familiares. Está caracterizado por altos niveles séricos de calcio y PTH y el desafío clínico-quirúrgico es el diagnóstico diferencial con otras entidades benignas como el adenoma o la hiperplasia de paratiroides. Aunque el diagnóstico de certeza es anatomopatológico, la sospecha clínica y el uso de métodos de baja complejidad (ecografía) con operadores avezados permite una correcta localización y abordaje pertinente del paciente para dirigir el tratamiento quirúrgico adecuado (resección en bloque) evitando persistencias y recurrencias de enfermedad. Se presenta el caso clínico de un paciente masculino que ingresa por síndrome de impregnación asociado a hipercalcemia, su abordaje diagnóstico, tratamiento y manejo interdisciplinario con discusión y revisión bibliográfica. (AU)

Parathyroid carcinoma (CP) is a malignant disease with an incidence of 0.015 per 100,000 inhabitants per year. It accounts for 1% of primary hyperparathyroidism diagnoses and occurs between the 4th and 5th decade of life, with a similar incidence between men and women. The etiology of CP is uncertain and has been associated with sporadic or family forms. CP is characterized by high serum calcium and PTH levels and the clinical-surgical challenge is the differential diagnosis with other benign entities such as parathyroid adenoma or hyperplasia. Although the diagnosis of certainty is achieved by pathological anatomy examination, the clinical suspicion and the use of low complexity methods (ultrasound) by experienced operators allows a correct localization and a patient-specific approach to direct the appropriate surgical treatment (block resection), avoiding persistence and recurrences of disease. The clinical case of a male patient admitted for severe hypercalcemia with multiple organ disfunction, the diagnostic approaches, treatment, and interdisciplinary management, together with review and discussion of the current literature are presented. (AU)

Pancreatitis aguda por hipercalcemia en el embarazo: caso clínico / Acute pancreatitis due to hypercalcemia during pregnancy: report of one case

Acute pancreatitis during pregnancy is uncommon and usually associated with gallstones. However other etiologies must be considered. We report a 24 years old woman with a 32 weeks pregnancy consulting for abdominal pain, nausea and vomiting. She had elevated lipase and amylase levels, a corrected serum calcium of 13.1 mg/dl and a serum phosphate of 1.6 mg/dl. A magnetic resonance colangiopancreatography showed an enlarged pancreas with inflammatory changes and a normal Wirsung duct. A parathyroid nodule was found on cervical ultrasonography. The patient was treated initially with cinacalcet with partial response. A parathyroidectomy was performed at 39 weeks of pregnancy with a good maternal and fetal evolution.

Síndromes de neoplasia endocrina múltiple: revisión radiológica / Multiple Endocrine Neoplasia Syndromes: Radiological Review

Los síndromes de neoplasia endocrina múltiple (MEN), incluyen una serie de enfermedades con alteraciones genéticas que se caracterizan por la presencia de tumores que afectan a dos o más glándulas endocrinas. Son síndromes con una herencia autosómica dominante e incluyen tres patrones: MEN 1 (síndrome de Wermer), MEN 2 (que incluye MEN 2A o síndrome de Sipple y MEN 2B o síndrome de Wagenmann-Froboese) y MEN 4. Los adenomas paratiroideos y el carcinoma medular tiroideo, son los tumores más frecuentes del MEN tipo 1 y 2 respectivamente. Esos síndromes son más comunes en pacientes jóvenes, con patología de afectación bilateral, múltiple o multifocal y, sobre todo, en pacientes con antecedentes familiares. Es necesario el trabajo en equipo de endocrinólogos, cirujanos, oncólogos y radiólogos para optimizar el tratamiento de esos pacientes.

Multiple endocrine neoplasia (MEN) encompasses a serial of familial genetically disorders in wich tumors simultaneusly occur in two or more endocrine organs. MEN síndromes are autosomal-dominant disorders categorized into three main patterns: MEN 1 (Wermer syndrome), MEN 2 (includes MEN 2A o Sipple syndrome and MEN 2B o Wagenmann-Froboese syndrome) and MEN 4. Parathyroid adenomas and medullary thyroid carcinoma are the most frecuent tumors in MEN 1 and MEN 2 respectively. These entities will be suspected in younger patients, bilateral, multiple or multifocal disease and, specially, in patients with family background. Cooperation between endocrinologist, surgeons, oncologists and radiologists is pivotal for optimizing patient treatment.

Crisis hipercalcémica causada por adenoma paratiroideo: reporte de un caso clínico / Hypercalcemic crisis due to parathyroid adenoma: report of a clinical case

La hipercalcemia es un trastorno común que representa aproximadamente el 0,6% de todas las admisiones médicas agudas. El hiperparatiroidismo primario (HPTP) y las neoplasias malignas son las dos causas más comunes de elevación de los niveles séricos de calcio; constituyen, en conjunto, alrededor del 90% de todos los casos. La presentación sintomática clásica de la hipercalcemia se observa con relativa poca frecuencia en el mundo desarrollado; la presentación más común es la detección asintomática en las pruebas bioquímicas. Sin embargo, en casos raros, el HPTP puede desarrollar hipercalcemia aguda, grave y sintomática, llamada crisis hipercalcémica (CH). Esta condición se asocia a alteraciones profundas en el estado mental y las funciones cardíaca, renal y gastrointestinal en presencia de concentraciones marcadamente elevadas de calcio sérico y paratohormona (PTH). Mientras que algunas elevaciones en el calcio sérico pueden ser bien toleradas, los síntomas de la CH son severos. Si el tratamiento se retrasa, la CH puede provocar la muerte. Describimos el caso de un paciente masculino que ingresa en la unidad de cuidados críticos por una CH secundaria a un HPTP por adenoma paratiroideo. (AU)

Hypercalcaemia is a most common disorder, accounting for approximately 0,6% of all acute medical admissions. Primary hyperparathyroidism (PHPT) and malignancy are the two most common causes of increased serum calcium levels, together accounting for about 90% of all cases. The classical symptomatic presentation of hypercalcaemia is seen relatively rarely in the developed world, the most common presentation being asymptomatic and detected following on biochemical testing. However, in rare cases HPTP can result in acute, severe and symptomatic hypercalcemia, called hypercalcemic crisis (HC). This condition is associated with profound disturbances in mental status, and cardiac, renal, and gastrointestinal function in the presence of markedly increased serum calcium and parathyroid hormone (PTH) concentrations. While some elevations in serum calcium can be well tolerated, symptoms of HC are severe. If treatment is delayed, HC can result in death. We describe herein a case of a male patient who was admitted to the intensive care unit as a consequence of HC resulting from elevated PTH, secondary to a parathyroid adenoma. We describe the case of a male patient who was admitted to the critical care unit for a HC mediated by PTH secondary to a parathyroid adenoma. (AU)

Análisis de falsos negativos en la cintigrafía SPECT de paratiroides con sestamibi en pacientes con hiperparatiroidismo primario sometidos a cirugía entre 2008-2015 en hospital universitario / False negative spect parathyroid scintigraphy with sestamibi in patients with primary hyperparathyroidism

Background: 99mTc-sestamibi parathyroid SPECT scintigraphy is a useful tool in the pre-operative study of hyperparathyroidism. False negatives (FN) have been reported in 5.7-14% of the examinations. Aim: To characterize 99mTc-sestamibi FN in cases referred for primary hyperparathyroidism (PHP) to a university hospital. Material and Methods: Descriptive retrospective analysis. We included patients with PHP, studied with SPECT scintigraphy, operated at our center between 2008 and 2015. Clinical and surgical data were recorded; biopsies of the FN were blindly reviewed by one pathologist. Results: One hundred twenty one scintigraphies fulfilled the inclusion criteria. Seven (5.8%) were negative and 114 positive. There was no difference in age, sex and PTH levels between FN and true positive scintigraphies. At surgery, one FN case had two hyperplasic glands and two cases had ectopic glands. Pathology reported adenoma in three cases, hyperplasia in three and carcinoma in one. The largest diameter of the lesion was lower in FN (1.3 and 2.1 cm respectively, p = 0.02) and the proportion of adenomas was higher in true positive cases (29% and 75% respectively; p < 0.01). The interval between scintigraphy and parathyroidectomy was greater in FN with a median of 92 days (range 20 days-3.2 years, p < 0.01). The percentage of oxyphilic cells observed was similar in both groups. Conclusions: FN parathyroid SPECT scintigraphies in PHP are uncommon. They corresponded to lesions under the equipment's resolution limit and resulted in longer time lags between scintigraphy and surgery.

Hipercalcemia hipocalciurica familiar en una paciente con mutación del receptor de calcio: forma atípica de presentación y tratamiento con cinacalcet / Familial hypocalciuric hypercalcemia in a patient with calcium-sensing receptor mutation: atypical clinical presentation and treatment with cinacalcet

El hiperparatiroidismo familiar y la hipercalcemia hipocalciúrica familiar (HHF) constituyen un subgrupo heterogéneo de trastornos con herencia mendeliana, que representan en conjunto el 5% de las causas de hipercalcemia PTH dependiente. La HHF se asocia con mutaciones del gen del receptor sensor de calcio (CaSR). Esta entidad se manifiesta, en la mayoría de los casos, con la presentación asintomática y familiar de hipercalcemia e hipocalciuria y valores elevados o normales de hormona paratiroidea (PTH). Los avances en la biología molecular han contribuido al diagnóstico, evaluación del fenotipo de cada entidad y elección del tratamiento. Se describe el caso de una paciente con hipercalcemia estudiada a partir de una tumoración de cuello asociada con una glándula paratiroides quística. Luego de un exhaustivo proceso diagnóstico se halló en el estudio genético una mutación inactivante en el gen CaSR. Teniendo en cuenta la presencia de la relación clearance calcio/clearance creatinina <0,01 y la falta de respuesta al tratamiento quirúrgico, se consideró la entidad de HHF con forma de presentación atípica. La paciente, sin tratamiento, presentaba un progresivo incremento de la calcemia luego de la cirugía de las glándulas paratiroides, que no se controló con el uso de bifosfonatos y evolucionó con episodios de mareos y desmayos frecuentes sin causa neurológica o cardiovascular detectada. Por lo tanto, se inició el tratamiento con cinacalcet, con el cual se obtuvo una buena respuesta terapéutica: descenso de la calcemia y mejoría de la sintomatología luego de un año de su comienzo. El cinacalcet es una herramienta terapéutica de importancia en estos raros casos de HHF. (AU)

Familial hyperparathyroidism including familial hypocalciuric hypercalcemia (FHH) is an heterogeneous subgroup of disorders with Mendelian inheritance, that account for 5% of PTH dependent hypercalcemia. FHH is associated with mutations of the calcium receptor (CaSR) gene. This entity is manifested by hypercalcemia with hypocalciuria and high or normal levels of parathyroid hormone (PTH) generally asymptomatic and with familial presentation. Advances in molecular biology have contributed to the diagnosis, evaluation of the phenotype of each entity and the choice of treatment. We describe a patient with hypercalcemia diagnosed following the finding of a neck tumor associated with cystic parathyroids. After an exhaustive diagnostic process, an inactivating mutation in the CaSR gene was found. Considering the presence of a ratio clearance calcium / clearance creatinine <0.01 and the lack of response to surgical treatment, HHF entity with atypical presentation was considered. The patient exhibited progressive increase in serum calcium following parathyroid surgery, which was not controlled with the use of bisphosphonates and evolved into episodes of frequent dizziness and fainting, without neurological or cardiovascular causes. Treatment with cinacalcet was initiated, with a good therapeutic response. The use of cinacalcet is a useful therapeutic tool in these rare cases of FHH. (AU)

Evaluation of preoperative ultrasonographic and biochemical features of patients with aggressive parathyroid disease: is there a reliable predictive marker?

ABSTRACT Objective Parathyroid cancer (PC) represents < 1% of cases of PHPT. Tumors demonstrating atypical histopathologic features and don’t fulfill criteria for carcinoma are classified as atypical adenomas (APA). The purpose of this study was to determine a biochemical or ultrasonographic feature that can predict aggressive disease requiring more extensive surgery and closer follow-up. Subjects and methods Twenty eight patients operated for PHPT and diagnosed with atypical adenoma (23 patients) or carcinoma (5 patients) were enrolled in this study. The control group consisted of 102 patients operated between the same dates and diagnosed with classical PA. Classical adenomas, atypical adenomas, and carcinomas were compared according to their biochemical and ultrasonographic parameters. Results Serum Ca levels were significantly higher in the PC group compared with the APA and classical PA groups. Serum median PTH, Serum ALP and UCa was significantly higher in the APA and carcinoma groups compared to the classical PA group. ROC analysis was made to determine the best cut off values for predicting aggressive disease were 12.45 mg/dL, 265.05 pg/mL, 154.5 IU/l, 348.5 mg/day and 21.5 mm for Ca, PTH, ALP, UCa and the adenoma diameter, respectively. Multivariate analysis showed that serum Ca, ALP and isoechoic/cystic appearance were independent predictors for aggressive disease. Conclusion Preoperatively high PTH, ALP, and UCa levels and large lesions with isoechoic or cystic appearances may be predictive of atypical adenoma or carcinoma in patients being evaluated for PHPT. In such cases, surgeons may prefer en bloc parathyroidectomy to minimally invasive surgery.

Periapical radiolucency not requiring endodontic therapy: an unusual case.

The article is a case report of an unusual manifestation of primary hyperparathyroidism seen in the mandible. Primary hyperparathyroidism is a rare disorder that can present its first symptoms in the jaws. The pre- and post-treatment radiographic features of such cases have only rarely been reported in dental literature. This case report highlights the importance of careful clinical and radiographic examination before commencing root canal treatment.

Dual phase MIBI scintigraphy in diagnosis of parathyroid adenoma followed by ultrasound guided percutaneous alcoholic ablation

Primary Hyperparathyroidism [HPT] is an inappropriate hyper secretion of parathyroid hormone [PTH]. Primary HPT is caused by parathyroid adenoma in 80-85% of patients. Clinical manifestations are kidney stones, abdominal groans, painful bones, psychic moans, and fatigue overtones. Ultrasonography is widely used in suspected cases for localization of parathyroid adenoma. There is considerable intra-observer variation and it is difficult for ultrasound alone to differentiate parathyroid lesion form that of thyroid. Dual phase Tc-99m MIBI scinitigraphy for detection of parathyroid adenomas has sensitivity and specificity values ranging from 82% to 100% and from 89% to 100%, respectively. Percutaneous ethanol injection for parathyroid glands can be applied effectively in selected cases when surgery is unadvisable either for technical reasons e.g., recurrence of hyperplastic glands in the neck after subtotal surgery or intrathyroideal parathyroid tumors or the poor clinical state of the patient

Hyperfunctioning intrathyroidal parathyroid carcinoma

Intrathyroidal parathyroid carcinoma is an exceedingly rare cause of primary hyperparathyroidism with difficulties in the diagnosis and management. We report a case of hypercalcemia from intrathyroidal parathyroid carcinoma in a 63-year-old Saudi female. She was diagnosed 2 years earlier with osteoporosis in a primary care clinic and was on alendronate since then. This year she was noted to have hypercalcemia, but in retrospect she had more than 10 years history of multiple medical problems related to hypercalcemia. Parathyroid 99mTc-SestaMIBI scintigraphy revealed parathyroid adenoma in the left inferior parathyroid gland. She had successful video-assisted parathyroidectomy that relieved most of her symptoms. The extreme rarity of such a case, the interesting clinical presentation and review of the literature are discussed

[Evaluation of diagnostic role of 99mTc-terofosmin scintigraphy in cold thyroid nodules]

Various radiopharmaceuticals, including 67Ga, 201Tl, and 99mTc-sestamibi have been used to differentiate benign from malignant thyroid nodules. 99mTc-Tetrofosmin, a lipophilic cationic radiotracer, and 99mTc-sestamibi have also been reported to accumulate in thyroid tumors. In this study, we evaluated the role of 99mTc-Tetrofosmin in the differentiation of malignant from benign thyroid nodules. We prospectively studied 108 patients with solitary cold thyroid nodule on 99mTc-pertechnetate scintigraphy [33 malignant and 75 benign] to investigate the diagnostic value of 99mTc-Tetrofosmin scintigraphy. 99mTc-Tetrofosmin scintigraphy was performed 15, 60, and 120 minutes following IV injection of 20 mCi [740 MBq] of radiotracer in the anterior planar mode with a gamma camera equipped with LEAP collimator. The scans were visually analyzed by two experienced nuclear physician. The nodules with late tracer retention [activity more than adjacent thyroid tissue] were classified as positive and nodules without late retention were interpreted as negative for malignancy. Fine needle aspiration [FNA] was performed in all patients in 3-7 days interval. 52 patients were subsequently operated on while 56 patients refused surgery. These 56 patients, however, had at least two negative FNA results. 45 out of 108 nodules show high 99mTc-Tetrofosmin uptake on delayed images; 27 of them were malignant. Sensitivity, specificity, positive predictive value, and negative predictive value were calculated to be 81.8%, 76.0%, 54.0%, and 90.4% respectively. Accuracy of the test was also determined to be 77.7%. We concluded that 99mTc-Tetrofosmin scintigraphy is a relatively sensitive, but not enough specific, method in diagnosing malignant thyroid nodules. This agent could be of value in the presence of FNA limitations

Double-phase technetium 99m setamibi scintigraphy for the localization of parathyroid adenoma

The purpose of the present study was to evaluate the accuracy of double phase Tc99m sestamibi scintigraphy in the assessment of parathyroid glands in patients with hyperparathyroidism and to compare this method with the high-resolution ultrasonography. From August-1998 through August-2000, we performed Tc99m -MIBI scintigraphy [MIBI] and neck ultrasonography in 35 patients who were clinically and biochemically suspected of having hyperparathyroidism. All patients underwent surgical neck exploration. Before surgery, blood intact parathyroid hormone and serum calcium level were measured peripherally. Early and delayed cervical images of MIBI scans and neck ultrasonography were interpreted by two radiologists. The findings were compared to ultrasonography and scintigraphic results. The sensitivity and positive predictive values of both modalities for localizing the diseased glands were determined by comparing scan findings with pathological findings, which were considered the gold standard. All resected glands were abnormal. They belonged to 25 patients with a single adenoma, two patients with ectopic glands, two patients with double adenomas and 6 patients with hyperplastic parathyroid glands. The sensitivity and positive predictive values for localizing the diseased glands in patients with hyperparathyroidism were 91% and 97%, respectively for Tc 99m MIBI. The values were 74% and 100%, respectively, for ultrasonography. Tc 99m MIBI sensitivity was higher in patients with parathyroid adenomas [96%] than that in patients with parathyroid hyperplasia [67%]. Our results demonstrate that Tc99m sestamibi scintigraphy is highly accurate in identifying parathyroid adenoma when performed by an experienced radiologist. It is helpful in preoperative localization of parathyroid adenoma in patients with suspected ectopic glands. Nevertheless, because of the greater accessibility, lower costs and simplicity, ultrasonography could be used as a screening modality for parathyroid adenoma localization

Causes of superscan picture on bone scintigraphy

To identify causes of the superscan picture on 99mTc-MDP bone scintigraphy in our clinical environment. Design: A descriptive study. Place and Duration of Study: The study was conducted over a period of two years [January 1997 to December 1998] at the Nuclear Medicine Department [NMD], Institute of Radiotherapy and Nuclear Medicine [IRNUM], Peshawar. Subjects and A total of 27 cases [23 females and 4 males] having super-scan pictures of metabolic bone disease were prospectively evaluated. After three-observer confirmation of bone scan, serum calcium and parathyroid hormone [PTH] estimation was done. The patients having serum PTH greater than 250 pg /ml underwent two-phase parathyroid MIBI scintigraphy [2PP MIBI scan] for the detection of parathyroid adenoma. The patients having positive scans for parathyroid adenoma were subjected to surgery and histopathological confirmation was obtained. Selected cases [N=19] underwent a trial of depot-preparation of vitamin D3 and calcium supplementation. The final diagnosis of 16 patients was osteomalacia [59%], six were histopathologically confirmed cases of parathyroid adenoma [22.2%], One case had toxic adenoma thyroid [3.7%] and one had chronic renal failure [3.7%]. In three cases, final diagnosis could not be made [11.1%]. Osteomalacia and parathyroid adenoma are the two most common causes for the superscan picture on bone scintigraphy