Hipoparatiroidismo y calcificaciones cerebrales: reporte de caso / Hypoparathyroidism and brain calcifications: a case report

Introducción. El hipoparatiroidismo es una enfermedad caracterizada por la ausencia o concentraciones inadecuadamente bajas de hormona paratiroidea (PTH), que conduce a hipocalcemia, hiperfosfatemia y excreción fraccional elevada de calcio en la orina. Las calcificaciones del sistema nervioso central son un hallazgo frecuente en estos pacientes. Caso clínico. Mujer de 56 años con antecedente de hipotiroidismo, que ingresó por un cuadro de 6 días de evolución caracterizado por astenia, parestesias periorales y movimientos anormales de manos y pies. Las pruebas de laboratorio demostraron hipocalcemia, hiperfosfatemia y niveles bajos de hormona paratiroidea. Se realizó una tomografía computarizada de cráneo que mostró áreas bilaterales y simétricas de calcificaciones en hemisferios cerebelosos, ganglios basales y corona radiata. No se evidenciaron trastornos en el metabolismo del cobre y hierro. Se estableció el diagnóstico del síndrome de Fahr secundario a hipoparatiroidismo y se inició tratamiento con suplementos de calcio y vitamina D con evolución satisfactoria. Discusión. El síndrome de Fahr es un trastorno neurológico caracterizado por el depósito anormal de calcio en áreas del cerebro que controlan la actividad motora. Se asocia a varias enfermedades, especialmente, hipoparatiroidismo. La suplementación con calcio y vitamina D con el objetivo de normalizar los niveles plasmáticos de estos cationes es el tratamiento convencional. (AU)

Introduction. Hypoparathyroidism is a disease characterized by absence or inappropriately low concentrations of circulating parathyroid hormone, leading to hypocalcaemia, hyperphosphataemia and elevated fractional excretion of calcium in the urine. Central nervous system calcifications are a common finding in these patients. Case report. 56-year-old woman with a history of hypothyroidism who was admitted for a 6-day course of illness characterized by asthenia, perioral paresthesias, and abnormal movements of the hands and feet. Laboratory tests showed hypocalcemia, hyperphosphatemia, and low parathyroid hormone levels. A cranial computed tomography was performed. It showed bilateral and symmetrical areas of calcifications in the cerebellar hemispheres, basal ganglia, and radiata crown. No disorders of copper or iron metabolism were evident. The diagnosis of Fahr syndrome secondary to hypoparathyroidism was established and treatment with calcium and vitamin D supplements was started with satisfactory evolution. Discussion. Fahr's syndrome is a neurological disorder associated with abnormal calcium deposition in areas of the brain that control motor activity. It is associated with various diseases, especially hypoparathyroidism. The conventional treatment is supplementation with calcium and vitamin D, with the aim of normalizing their plasma levels. (AU)

Calcinosis cutis idiopática circunscripta: comunicación de un caso / Idiopathic calcinosis cutis circumscripta: report of a case

La calcinosis cutis comprende una serie de manifestaciones caracterizadas por el depósito de calcio a nivel cutáneo, con o sin alteración del metabolismo fosfocálcico. Actualmente se clasifica de acuerdo con el mecanismo fisiopatológico en cinco grandes grupos: distrófica, metastásica, iatrogénica, idiopática y calcifilaxis. El abordaje terapéutico de esta afección, tanto médico como quirúrgico, es difícil y de resultados dispares. Presentamos el caso de una paciente con calcinosis cutis idiopática, que evolucionó favorablemente con la administración oral de diltiazem.

Calcinosis cutis includes a group of disordes characterized by cutaneous deposition of calciumon the skin, with or without alteration of phosphocalcic metabolism. It is classified according topathophysiological mechanisms into five major categories: dystrophic, metastatic, iatrogenic,idiopathic and calciphilaxys. The therapeutic approach to this condition, both medical and surgical,is difficult and outcomes often disappointing. We present a patient with idiopathic calcinosis cutiswho evolved favorably after oral administration of Diltiazem.

Statins for progression of aortic valve stenosis and the best evidence for making decisions in health care / Estatinas para a progressão da estenose da valva aórtica e a melhor evidência para tomar decisões em saúde

In the Western world, calcified aortic valve stenosis is the most common form of valvular heart disease, affecting up to 3 percent of adults over the age of 75 years. It is a gradually progressive disease, characterized by a long asymptomatic phase that may last for several decades, followed by a short symptomatic phase associated with severe restriction of the valve orifice. Investigations on treatments for aortic valve stenosis are still in progress. Thus, it is believed that calcification of aortic valve stenosis is similar to the process of atherosclerosis that occurs in coronary artery disease. Recent studies have suggested that cholesterol lowering through the use of statins may have a salutary effect on the progression of aortic valve stenosis.

No mundo ocidental, a estenose da valva aórtica calcificada é a forma mais comum de doença cardíaca valvar, afetando até 3 por cento dos adultos com idade acima de 75 anos. É uma doença gradual e progressiva, caracterizada por uma longa fase assintomática, podendo durar várias décadas, seguida de uma curta fase sintomática associada à restrição grave do orifício da valvar. A investigação sobre o tratamento da estenose da valva aórtica ainda está em curso. Desta forma, acredita-se que a calcificação da estenose da valva aórtica seja semelhante ao processo de aterosclerose que ocorre na doença arterial coronariana. Estudos recentes têm sugerido que a redução do colesterol pelo do uso das estatinas pode ter um efeito salutar sobre a progressão da estenose da valva aórtica.

Calcinosis cutis extensa en pacientes adultos con dermatomiositis: a propósito de dos casos / Extensive calcinosis cutis in adult patients with dermatomyositis: two case reports

La calcinosis cutis es el término empleado para describir una serie de trastornos caracterizados por presentar depósitos de sales de calcio a nivel subcutáneo o de la dermis, ocurriendo tanto en hipercalcemia como en normocalcemia y en ocasiones se asocia a un trastorno subyacente. El pronóstico suele ser benigno y el tratamiento, tanto médico como quirúrgico, presenta resultados variables. Presentamos dos casos de pacientes adultos con diagnóstico de dermatomiositis que desarrollaron extensas zonas de calcificación subcutánea.

Calcinosis cutis is the term used to describe a group of disorders characterized by calcium deposits in the skin, occurring in hypercalcemia or normocalcemia and sometimes associated to an underlying disorder. It has a benign evolution and the treatment, both medical and surgical, present variable outcomes. We report the cases of two adult patients with diagnosis of dermatomyositis that developed extensive areas of calcium deposit.

Calcinosis cutis / Calcinosis cutis

Se presenta el caso de un paciente de 2 años de edad, de la raza blanca, sexo femenino, que fue valorada en su provincia de procedencia por presentar nódulos cutáneos de localización múltiple en los miembros inferiores y abdomen, y fue remitida al Hospital Pediátrico Juan M Márquez. En las radiografías de las extremidades inferiores se observaron calcificaciones en partes blandas y la biopsia informó nódulos subepidérmicos calcificados. En la anamnesis no se encontraron antecedentes de traumatismos en la zona ni de consumo de fármacos ricos en calcio o fósforo. La exploración física fue normal, salvo las lesiones previamente descritas. Se realizaron estudios para determinar alguna enfermedad subyacente que pudiera justificar este cuadro clínico, pero no se constataron causas evidentes. Dado que la calcicosis cutánea de la paciente no es secundaria a afecciones sistémicas, ni se evidencian alteraciones metabólicas ni de otro tipo, se estableció el diagnóstico de calcinosis cutánea localizada idiopática

This is the case of a white female patient aged 2, assessed in its origin province due to presence of cutaneous nodules of multiple localizations in lower limbs and in abdomen, referred to Juan Manuel Márquez Children Hospital. Lower limbs X-rays showed soft tissues calcifications and a biopsy confirmed the presence of calcified subepidermal nodules. In anamnesis there were neither traumata backgrounds in the area nor consumption of drugs rich in calcium and phosphorus. The physical examination was normal except the above mentioned lesions. Studies to determine some underlying disease that could justify this clinical picture were conducted but without obvious causes. Since the cutaneous Calcinosis is not secondary to systemic affections and there is not metabolic alterations or of other type, diagnosis con idiopathic localized cutaneous calcinosis was made

Fatores de risco associados à calcinose na dermatomiosite juvenil / Risk factors associated with calcinosis of juvenile dermatomyositis

OBJETIVO: Identificar fatores de risco associados à calcinose em crianças e adolescentes com dermatomiosite juvenil. MÉTODOS: Prontuários de 54 pacientes com dermatomiosite juvenil foram estudados. Foram avaliados dados demográficos; características clínicas: grau de força muscular (I a V do Medical Research Council), presença de comprometimentos pulmonar (distúrbio ventilatório restritivo com presença ou ausência do anticorpo anti-Jo-1), gastrointestinal (refluxo gastroesofágico) e cardíaco (pericardite e/ou miocardite); exames laboratoriais: elevação de enzimas musculares (creatinoquinase, aspartato aminotransferase, alanina aminotransferase e desidrogenase lática) e terapias utilizadas: corticoterapia isolada ou associada à cloroquina e/ou imunossupressor. Os pacientes foram divididos em dois grupos de acordo com a presença ou ausência de calcinose e foram avaliados através de análise univariada e multivariada. RESULTADOS: Calcinose foi evidenciada em 23 (43 por cento) pacientes, sendo em seis (26 por cento) antes do diagnóstico e em 17 (74 por cento) após. A análise univariada revelou que comprometimentos cardíaco (p = 0,01) e pulmonar (p = 0,02) e necessidade da utilização de um ou mais imunossupressores (metotrexato, ciclosporina A e/ou pulsoterapia com ciclofosfamida endovenosa) no tratamento da dermatomiosite juvenil (p = 0,03) foram associados com uma maior incidência de calcinose. A análise multivariada mostrou que comprometimento cardíaco (OR = 15,56; IC95 por cento 1,59-152,2) e uso de um ou mais imunossupressores (OR = 4,01; IC95 por cento 1,08-14,87) foram as únicas variáveis independentes associadas à presença de calcinose. CONCLUSÕES: O aparecimento da calcinose foi freqüente na dermatomiosite juvenil, habitualmente na evolução da doença. A calcinose foi associada aos casos mais graves, que apresentaram envolvimento cardíaco e necessitaram da utilização de imunossupressores no seu tratamento.

OBJECTIVE: To identify risk factors associated with calcinosis in children and adolescents with juvenile dermatomyositis. METHODS: A review was carried out of the medical records of 54 patients with juvenile dermatomyositis. Data were collected on demographic characteristics, clinical features: muscle strength (stages I to V of the Medical Research Council scale), pulmonary involvement (restrictive pulmonary disease with presence or absence of anti-Jo1 antibodies), gastrointestinal problems (gastroesophageal reflux) and/or heart disease (pericarditis and/or myocarditis); laboratory tests: elevated muscle enzyme levels in serum (creatine phosphokinase, aspartate aminotransferase, alanine aminotransferase and/or lactate dehydrogenase); and on the treatments given: corticoid therapy in isolation or associated with hydroxychloroquine and/or immunosuppressants. The patients were divided into two groups, depending on presence or absence of calcinosis and data were evaluated by both univariate and multivariate analyses. RESULTS: Calcinosis was identified in 23 (43 percent) patients, and in six (26 percent) patients it had emerged prior to diagnosis while in 17 (74 percent) it was post diagnosis. The univariate analysis revealed that cardiac (p = 0.01) and pulmonary (p = 0.02) involvement and the need for one or more immunosuppressor (methotrexate, cyclosporine A and/or pulse therapy with intravenous cyclophosphamide) to treat juvenile dermatomyositis (p = 0.03) were all associated with an increased incidence of calcinosis. The multivariate analysis then demonstrated that only cardiac involvement (OR = 15.56; 95 percentCI 1.59-152.2) and the use of one or more immunosuppressor (OR = 4.01; 95 percentCI 1.08-14.87) were independently associated with the presence of calcinosis. CONCLUSIONS: Calcinosis was a frequent development among these juvenile dermatomyositis cases, generally emerging as the disease progressed. Calcinosis was associated with...

Effects of atorvastatin on warfarin-induced aortic medial calcification and systolic blood pressure in rats / 华中科技大学学报（医学）（英德文版）

The effect of atorvastatin on warfarin-induced aortic medial calcification and systolic blood pressure (SBP) of rats induced by warfarin was studied. Thirty healthy and adult rats were randomly divided into Warfarin group (n=10), Atorvastatin group (n=10) and normal control group (n=10). Caudal arterial pressure of rats was measured once a week, and 4 weeks later, aorta was obtained. Elastic fiber, collagen fiber and calcium accumulation in tunica media of cells were measured by Von Kossa staining. The results showed that warfarin treatment led to elevation of systolic blood pressure and aortic medial calcification. The chronic treatment also increased collagen, but decreased elastin in the aorta. However, the atorvastatin treatment had adverse effects. It was concluded that treatment with atorvastatin presented evidence of blood pressure lowing and calcification reducing. These data demonstrate that atorvastatin protected aortic media from warfarin-induced calcification and elevation of systolic blood pressure.

Familial calcific band-shaped keratopathy: report of two new cases with early recurrence.

We report two siblings with the rare entity of familial calcific band-shaped keratopathy (BSK). Detailed ophthalmic and systemic investigations failed to reveal any underlying causative pathology. Topical disodium ethylenediamine-tetraacetate (EDTA) was applied for 30 min to all four eyes. In addition the right eye of the younger sibling required a superficial keratectomy. An improvement in corneal clarity was seen in the immediate postoperative period in both siblings. Histopathology of the keratectomy specimen revealed linear extracellular sub-epithelial granular calcium deposits. However, an early recurrence was noted in all four eyes at four weeks postoperatively. We report the second instance in the English literature of this entity. Band-shaped keratopathy presenting without an obvious etiology merits a complete systemic and ophthalmic workup. Patients with familial idiopathic BSK could be cases with poor prognosis for treatment with EDTA due to an early recurrence of the disease.

Aspectos clínicos e neurorradiológicos do pseudo-hipoparatireoidismo: relato de caso / Clinical and neuroradiologic aspects of pseudohypoparathyreoidism: case report

Os autores relatam o caso de um paciente do sexo masculino, de 18 anos, com baixa estatura, epilepsia e deficiencia mental, alem de calcificacoes dos glanglios da base. Os achados clinicos e laboratorias sugeriram psdeudo-hipoparatireoidismo. Trata-se de patologia rara, de base genetica, secundaria a resistencia periferica ao hormonio da paratireoide e que evolui com excitabilidade neuromuscular, baixa estatura e calcificacoes dos glanglios da base, entre outros achados. Sao discutidas as formas de diagnosticas e a terapeutica empregada, seguidas de preve revisao da literatura sobre calcificacao dos glanglios da base.