[Alcoholic rhabdomyolysis with myocardial involvement]. / Rabdomiolisis alcohólica con compromiso miocárdico.

A case of severe rhabdomyolysis with extensive myocardial involvement in a 32 year old alcoholic man is reported. He referred effort dyspnea for the last eight months which worsened thirteen days before his death. Admitted at the institute he was obnubilated and dyspneic. Massive pulmonary thromboembolism was suspected and heparin was given. He was then transferred to our intensive care unit in circulatory collapse. A chest Rx showed cardiomegaly. A Swan-Ganz catheter was introduced and abnormal parameters corrected. Evolution was complicated with acute cholecystitis, nosocomial bronchopneumonia and hypoxic brain damage. A net reduction of cardiac size as judged by a chest roentgenogram was noted in the final days. Necropsy showed skeletal and cardiac rhabdomyolysis, early alcoholic cirrhosis, diffuse peritonitis and bronchopneumonia. To our knowledge, this case is the first in the literature to provide anatomical evidence of cardiac rhabdomyolysis, a fact which was suspected on clinical grounds but had not been proven.

[POEMS syndrome: report of a case and review of the literature]. / Síndrome de POEMS: presentación de un caso y revisión de la literatura.

A case of POEMS Syndrome of six years of evolution is reported. This syndrome is characterized by Raynaud phenomenon, polyneuropathy, edema, anasarca, papilledema, osteosclerosis and lymphadenopathy with the histopathology of Castleman's disease, hypothyroidism, hypogonadism, cutaneous sclerosis, hyperpigmentation, axillary alopecia and the presence of urinary lambda light chains. A bone marrow biopsy did not show plasmocytic infiltration and there was no evidence of extramedullary plasmocytoma. Methylprednisone was given at the dose of 1 mg/kg/day and subjective and objective improvement was observed. The edema and anasarca disappeared as well as the lymphadenopathies; muscle strength improved and the patient was able to walk without aid. Papilledema persisted. The pathogenesis of this syndrome remains unknown; some of the symptoms have been attributed to paraprotein deposits in peripheral nerves, high capillary permeability due to vascular alterations, accelerated conversion of androgen to estrogen, or to the production by plasma cells of a toxic substance. Mortality is related to complications of the polyneuropathy. Some patients in whom POEMS syndrome was associated, or not, with myeloma were treated with chemotherapy and/or radiotherapy with different responses; in others, corticosteroids were of short lived benefit. Our patients remains well after 42 months treatment with 20 mg methylprednisone every other day.

Rabdomiolisis alcohólica con compromiso miocárdico. / [Alcoholic rhabdomyolysis with myocardial involvement]

A case of severe rhabdomyolysis with extensive myocardial involvement in a 32 year old alcoholic man is reported. He referred effort dyspnea for the last eight months which worsened thirteen days before his death. Admitted at the institute he was obnubilated and dyspneic. Massive pulmonary thromboembolism was suspected and heparin was given. He was then transferred to our intensive care unit in circulatory collapse. A chest Rx showed cardiomegaly. A Swan-Ganz catheter was introduced and abnormal parameters corrected. Evolution was complicated with acute cholecystitis, nosocomial bronchopneumonia and hypoxic brain damage. A net reduction of cardiac size as judged by a chest roentgenogram was noted in the final days. Necropsy showed skeletal and cardiac rhabdomyolysis, early alcoholic cirrhosis, diffuse peritonitis and bronchopneumonia. To our knowledge, this case is the first in the literature to provide anatomical evidence of cardiac rhabdomyolysis, a fact which was suspected on clinical grounds but had not been proven.

Síndrome de POEMS: presentación de un caso y revisión de la literatura. / [POEMS syndrome: report of a case and review of the literature]

A case of POEMS Syndrome of six years of evolution is reported. This syndrome is characterized by Raynaud phenomenon, polyneuropathy, edema, anasarca, papilledema, osteosclerosis and lymphadenopathy with the histopathology of Castlemans disease, hypothyroidism, hypogonadism, cutaneous sclerosis, hyperpigmentation, axillary alopecia and the presence of urinary lambda light chains. A bone marrow biopsy did not show plasmocytic infiltration and there was no evidence of extramedullary plasmocytoma. Methylprednisone was given at the dose of 1 mg/kg/day and subjective and objective improvement was observed. The edema and anasarca disappeared as well as the lymphadenopathies; muscle strength improved and the patient was able to walk without aid. Papilledema persisted. The pathogenesis of this syndrome remains unknown; some of the symptoms have been attributed to paraprotein deposits in peripheral nerves, high capillary permeability due to vascular alterations, accelerated conversion of androgen to estrogen, or to the production by plasma cells of a toxic substance. Mortality is related to complications of the polyneuropathy. Some patients in whom POEMS syndrome was associated, or not, with myeloma were treated with chemotherapy and/or radiotherapy with different responses; in others, corticosteroids were of short lived benefit. Our patients remains well after 42 months treatment with 20 mg methylprednisone every other day.

Rabdomiolisis alcohólica con compromiso miocárdico / Alcoholic rhabdomyolysis with myocardial involvement

Presentamos un paciente de 32 años con antecedentes de alcoholismo, que refirió disnea de esfuerzo desde 8 meses antes, con peoría en los días previos a su internación. En otro hospital se diagnosticó tromboembolismo pulmonar masivo. Ingresó al Instituto en colapso circulatorio. La radiografía de tórax mostró cardiomegalia. Se lo ventiló artificialmente y se efectuó monitoreo hemodinámico, corrigiéndose los parámetros anormales. La evolución se complicó con colecistitis gangrenosa y bronconeumonia falleciendo a los 13 días de internado. La necropsia mostró rabdomiolisis esquelética y cardíaca, estigmas anatómicos de alcoholismo, peritonitis y bronconeumonia. El interés de esta presentación reside en la comprobación anatómica de rabdomiolisis cardíaca, hecho previamente supuesto (AU)

Síndrome de POEMS: presentación de un caso y revisión de la literatura / POEMS syndrome: report of a case and review of the literature

Comunicamos un síndrome de Poems con 6 años de evolución, diagnosticado y tratado desde hace un año y medio y caracterizado por: síndrome de Reynaud, polineuropatía, anasarca, edema de papila, osteoesclerosis, poliadenopatias con hiperplasia angiofolicular (enfermedad de Gatleman), hipotiroidismo, hipogonadismo, esclerodermia, hiperpigmentación, hirsutismo, alopecia axilar y trazas de cadenas ivianas lambda en la orina. La normalidade de las biopsias óseas excluye la presencia de un mieloma múltiple y no se encontraron evidencias de un pasmocitoma extramedular. Con metilprednisoma se observó una mejoría clínica consistente en desaparición de la anasarca y de la adenopatías, recuperación de la fuerza muscular y de la marcha. El edema de papila persistió. La fisiopatogenia se desconoce, si bien alguno de los síntomas han sido atribuidos al depósito de paraproteina en los nervios periféricos, a cambios en la permeabilidad vascular por "vasculopatía" y al aumento en la producción de estrógenos o a una mayor conversión de andrógenos. Se discute el tratamiento; en los casos asociados a mieloma se han utilizado la quimioterapia y/o la radioterapia con resultados variables. En algunos pacientes con corticosteroides, se ha logrado una mejoria transitoria. La causa de la muerte de estos enfermos está relacionada con las complicaciones de la polineuropatía. Por el momento es difícil clasificar a este síndrome, pero su original polimorfismo lo hace fácilmente reconocible (AU)

Rabdomiolisis alcohólica con compromiso miocárdico / Alcoholic rhabdomyolysis with myocardial involvement

Presentamos un paciente de 32 años con antecedentes de alcoholismo, que refirió disnea de esfuerzo desde 8 meses antes, con peoría en los días previos a su internación. En otro hospital se diagnosticó tromboembolismo pulmonar masivo. Ingresó al Instituto en colapso circulatorio. La radiografía de tórax mostró cardiomegalia. Se lo ventiló artificialmente y se efectuó monitoreo hemodinámico, corrigiéndose los parámetros anormales. La evolución se complicó con colecistitis gangrenosa y bronconeumonia falleciendo a los 13 días de internado. La necropsia mostró rabdomiolisis esquelética y cardíaca, estigmas anatómicos de alcoholismo, peritonitis y bronconeumonia. El interés de esta presentación reside en la comprobación anatómica de rabdomiolisis cardíaca, hecho previamente supuesto

Síndrome de POEMS: presentación de un caso y revisión de la literatura / POEMS syndrome: report of a case and review of the literature

Comunicamos un síndrome de Poems con 6 años de evolución, diagnosticado y tratado desde hace un año y medio y caracterizado por: síndrome de Reynaud, polineuropatía, anasarca, edema de papila, osteoesclerosis, poliadenopatias con hiperplasia angiofolicular (enfermedad de Gatleman), hipotiroidismo, hipogonadismo, esclerodermia, hiperpigmentación, hirsutismo, alopecia axilar y trazas de cadenas ivianas lambda en la orina. La normalidade de las biopsias óseas excluye la presencia de un mieloma múltiple y no se encontraron evidencias de un pasmocitoma extramedular. Con metilprednisoma se observó una mejoría clínica consistente en desaparición de la anasarca y de la adenopatías, recuperación de la fuerza muscular y de la marcha. El edema de papila persistió. La fisiopatogenia se desconoce, si bien alguno de los síntomas han sido atribuidos al depósito de paraproteina en los nervios periféricos, a cambios en la permeabilidad vascular por "vasculopatía" y al aumento en la producción de estrógenos o a una mayor conversión de andrógenos. Se discute el tratamiento; en los casos asociados a mieloma se han utilizado la quimioterapia y/o la radioterapia con resultados variables. En algunos pacientes con corticosteroides, se ha logrado una mejoria transitoria. La causa de la muerte de estos enfermos está relacionada con las complicaciones de la polineuropatía. Por el momento es difícil clasificar a este síndrome, pero su original polimorfismo lo hace fácilmente reconocible

Giant fibrous pleural mesothelioma associated with myocardial restriction and hypoglycemia.

A giant pleural fibrous mesothelioma was the cause of severe nonobstructive ventilatory incapacity, myocardial restriction with congestive failure and hypoglycemic episodes in a 58-year-old man. Surgical resection resulted in complete recovery of respiratory function and disappearance of hypoglycemic episodes.