ABSTRACT
Purpose: To describe a patient receiving maintenance hemodialysis complicated with calcific uremic arteriolopathy (calciphylaxis) in whom ulcer pain control was successfully achieved by buprenorphine. Case: A 75-year-old man was admitted due to intractable, extreme pain, which was accompanied by skin ulcers of the lower extremities. By a series of examinations including skin biopsy, the lesion was diagnosed to be calcific uremic arteriolopathy. The pain was not controllable with non-steroidal anti-inflammatory drugs and even by the nerve block. Buprenorphine, a partial agonist for the opioid receptor, markedly alleviated the mixed pain which was attenuated from Numerical Rating Scale (NRS) 10/10 to 0-2/10. Conclusion: Buprenorphine was very effective for the refractory pain control in a patient with skin ulcer due to calcific uremic arteriolopathy.
ABSTRACT
La arteriolopatía cálcica urémica se define como un síndrome constituido por úlceras cutáneas isquémicas debido a calcificación de la pared de las arteriolas del tejido celular subcutáneo como consecuencia principalmente de hiperparatiroidismo en pacientes urémicos. Se analizaron las correlaciones clínico patológicas de 3 pacientes con enfermedad renal crónica terminal, hiperparatiroidismo secundario y arteriolopatía cálcico urémica, todos particularmente con lesiones en pene. En la bioquímica sanguínea la paratohormona fue superior a 2,122 pg/dL, así como también se demostró hipercalcemia e hiperfosfatemia, con producto Ca²+xPO³-4 mayor de 70,2. Se realizó paratiroidectomía total con autoimplante en el primer caso y sin autoimplante en el tercer paciente. Las ulceras del pene fueron tratadas con curas locales diarias. La evolución clínica fue tórpida, con desenlace fatal. La ateriolopatía cálcica urémica o calcifilaxis, es una enfermedad compleja, variable, difícil de diagnósticar y de manejo muy complicado. La gangrena del pene es una consecuencia de las calcificaciones vasculares metastásicas asociadas a enfermedad renal crónica terminal y es un marcador de pronóstico sombrío.
The calcific uremic arteriolopathy is defined as a syndrome consisting of cutaneous ischemic ulcers due to calcification of the wall of the arterioles of the subcutaneous tissue as a result mainly of hyperparathyroidism in uremic patients. The clinical pathological correlations of 3 patients were analyzed with chronic renal terminal disease, secondary hyperparathyroidism and calcific uremic arteriolopathy all particularly with injuries in penis. In the blood biochemistry parathyroid hormone was greater 2.122 pg/dL, as well as it was demostrated hypercalcemia and hyperphosphatemia, with greater Ca2+xPO³-4 product of 70.2. Total parathyroidectomy with auto implant was realised in first patient, and without auto implant in third patient. Penis ulcers were dealt with local daily cure. The clinical evolution was poor, with fatal autcome. The calcific uremi arteriolopathy or calciphylaxis, is a complex disease, variable, difficult to diagnose and complicated management. Penile gangrene is a manifestation of widespread vascular calcifications associated with end-stage renal disease and is a marker of poor prognosis.
Subject(s)
Humans , Male , Adult , Middle Aged , Lower Extremity/physiopathology , Hemorrhagic Fever with Renal Syndrome , Hyperparathyroidism, Secondary/etiology , Renal Insufficiency, Chronic/mortality , Renal Insufficiency, Chronic/pathology , Penis/pathology , Skin Ulcer/pathology , Skin Ulcer/therapy , Renal Dialysis/methods , Gangrene/etiology , Uremia/bloodABSTRACT
El hiperparatiroidismo terciario ha sido reconocido como una hiperfunción autónoma de las glándulas paratiroides. Cuando esta enfermedad se instaura, la única opción terapéutica efectiva es la paratiroidectomía. Estos pacientes frecuentemente tienen hiperplasia paratiroidea por lo que la exploración cervical debe ser bilateral. Presentación de casos: Presentamos 3 pacientes, cada uno con una complicación diferente a propósito del hiperparatiroidismo terciario. La primera paciente con arteriolopatía cálcico-urémica, la segunda con calcinosis tumoral de la cadera, y la tercera paciente con tumor pardo bimaxilar. A todos los pacientes los tratamos con paratiroidectomía total con auto trasplante de la glándula más sana en el músculo recto anterior del abdomen. El estudio anatomopatológico reveló hiperplasia de las glándulas paratiroideas resecadas en todos los pacientes. La complicación principal de este procedimiento fue la hipocalcemia sintomática, requiriendo infusión de calcio endovenoso. Dos pacientes presentaron síndrome de hueso hambriento, que se resolvió progresivamente y en el seguimiento se observó normocalcemia. Hubo desenlace fatal en 2 pacientes como consecuencia de complicaciones sistémicas. El hiperparatiroidismo terciario es una enfermedad rara que el clínico debe reconocer para tratarla oportunamente. La paratiroidectomía total es terapéuticamente efectiva en esta condición, pero la hipocalcemia profunda posoperatoria es frecuente y necesita de un manejo cuidadoso.
Tertiary hyperparathyroidism has been recognized as an autonomous hyperfunction of the parathyroid glands. When this disease is established, the only effective therapeutic option is parathyroidectomy. These patients often have parathyroid hyperplasia so that the the neck exploration must be bilateral. Cases presentation: We report 3 patients, each with a different complication as a consequence of tertiary hyperparathyroidism. The first patient with calcic uremic arteriolopathy, the second with tumoral calcinosis of the hip, and the third patient with bimaxillary brown tumor. We treated all this patients with total parathyroidectomy, followed by healthy gland autotransplantation in the anterior rectus abdominal muscle. The anatomopathological study revealed hyperplasia of the resected parathyroid glands, in all the specimens. The main complication of this procedure were the symptomatic hypocalcemia, requiring intravenous calcium infusion. Two patients had the hungry bone syndrome, which was solved progressively. Two parients died due to systemic complications. Tertiary hyperparathyroidism is a rare disease that the clinician should recognize in order to treat it promptly. Total parathyroidectomy is therapeutically effective in this condition, but the postoperative profound hypocalcemia is frequent and needs a careful management.
Subject(s)
Humans , Female , Hyperparathyroidism/complications , Hyperparathyroidism/diagnosis , Parathyroidectomy/methods , Calcinosis/pathologyABSTRACT
Es una vasculopatía caracterizada por calcificación de la capa media de los vasos y proliferación de la íntima de los mismos, asociado a fibrosis y trombosis luminal, llevando a necrosis de los tejidos circundantes, en especial de los tejidos blandos. La fisiopatología de la calciflaxis está relacionada con múltiples factores, siendo una condición que lleva a la calcificación vascular acelerada y que se ha asociado a una serie de factores de riesgo como la enfermedad renal crónica en terapia de reemplazo y en trasplante renal. Se han relacionado otros factores no urémicos como son anormalidades en el metabolismo del calcio, hiperparatiroidismo primario, estados de hipercoagulabilidad como malignidad, déficit de proteína C y S, hepatopatía alcohólica, terapia con warfarina, hipoalbuminemia, terapias con calcio y vitamina D, obesidad y producto calcio/fósforo superior a 70. A continuación se reporta el caso de una paciente a quien se le documenta un cuadro clínico de calciflaxis, describiendo la presentación clínica de esta patología, los factores de riesgo identificados y las complicaciones presentadas durante su atención (Acta Med Colomb 2011; 36: 149-152).
Calciphylaxis is a vasculopathy characterized by calcification of the tunica media and proliferation of the tunica intima of blood vessels, in association with fibrosis and thrombosis of the lumen, which lead to necrosis of surrounding (especially soft) tissues. The pathophysiology of calciphylaxis is related with multiple factors. This condition leads to accelerated vascular calcification and has been associated with a number of risk factors, such as chronic renal disease, kidney replacement therapy, and renal transplantation. It has also been related with other, non-uremic, factors, such as calcium deregulation, primary hyperparathyroidism, hypercoagulability states (such as malignancy and deficiency of proteins C and S), alcoholic hepatopathy, warfarin therapy, hypoalbuminemia, therapy with calcium and vitamin D, obesity, and a Calcium/Phosphorus product above 70. We report the case of a patient with calciphylaxis by describing the clinical presentation, the associated risk factors, and the complications seen during the care of the patient (Acta Med Colomb 2011; 36: 149-152).