Tipo de Articulo: Casusticas

Titulo: Ostelisis multicntrica no hereditaria con nefropata: reporte de un caso y revisin de la literatura

Title: No hereditary multicentric osteolysis with nephropathy: report of a case and review of the literature

Actual. Osteol 9(2):217:224, 2013

Autor(es): Leandro Ferreyra- Garrott, Gabriel Meneses, Luisa Plantalech


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La ostelisis idioptica multicntrica (OIM) se caracteriza por el compromiso de carpos y de tarsos, con inflamacin y dolor; se inicia en la infancia y se autolimita en la 2a o 3a dcada de la vida. Segn Hardegger, se describen tipos hereditarios dominantes (tipo I) o recesivos asociados a osteoporosis (tipo II), OIM espordicas con compromiso renal (tipo III) u ostelisis masiva monocntrica (tipo IV). El tipo V se caracteriza por clnica similar al tipo II asociada a lesiones oculares o dermatolgicas y talla baja. En esta oportunidad se presenta el caso clnico de una OIM tipo III. Consulta un varn de 50 aos con antecedentes de dolor, tumefaccin y deformidad en ambos carpos y tarsos y compromiso funcional de inicio en la infancia. A los 34 aos presenta proteinuria, edemas e hipertensin arterial, desarrolla insuficiencia renal crnica (ICR). Se realiz trasplante renal. Sin antecedentes familiares de OIM. Se comprueba: disminucin en la funcin de ambas manos, limitacin en la extensin de mueca y deformidad en los dedos, y distrofia muscular de antebrazos y piernas. Camina con dificultad. En las radiografas se observa ausencia de huesos del carpo y tarso, ostelisis en algunas falanges. Se indica tratamiento con bifosfonatos, vitamina D y rehabilitacin. El paciente presenta OIM asociada a IRC e hipertensin arterial. Por carecer de antecedentes familiares se la considera OIM del tipo III. La OIM es una enfermedad infrecuente; el diagnstico oportuno evita tratamientos innecesarios y permite tratar la enfermedad renal y la hipertensin en estadios ms tempranos.

Palabras clave: ostelisis idioptica multicntrica, nefropata, ostelisis carpo-tarsal, herencia.

Idiopathic Multicentric Osteolysis (IMO) is a rare disease characterized by osteolysis of carpus and tarsus, with inflammation and pain with onset in childhood and arrest y the second or third decade. It is classified by Hardegger in 5 Types, 1: Hereditary IMO with dominant transmission. 2: Hereditary IMO with recessive transmission. 3: Non Hereditary IMO, associated with nephropathy and hypertension. 4: Gorhams Syndrome: massive osteolysis and replacement of bone by lymphatic or blood vessel tissue. 5: Winchester Syndrome: IMO with short stature, contractures, thick skin, corneal opacities and osteoporosis. A 50 year old male with a history of pain, swelling and deformity in both wrists and tarsi evolving from age 4, attends the consultation. He has functional involvement of hands and feet. At age 34 he had referred the presence of proteinuria, edema and hypertension, and developed chronic renal falilure (CRF). Dialysis and renal transplantation were indicated. No family history of IMO. Findings: Reduced function of boths hands, limited range of wrist and finger deformity, muscular forearms and legs dystrophy. He walked with difficulty. It is observed on radiographs, absence of carpal and tarsal bones, phalanges commitment. Physiotherapy rehabilitation and treatment with bisphosphonates and vitamin D was indicated. A patient with IMO associated with CRF and hypertension, was presented. He has absence of family history. It is considered Type III IMO. The IMO is a rare diseases, early diagnosis prevents unnecessary treatment and can treat kidney disease and hypertension at an earlier stage.

Key words: idiopathic multicentric osteolysis, nephropaty, carpal-tarsal osteolysis, inheritance.

Propietario: Asociación Argentina de Osteología y Metabolismo Mineral (AAOMM)
Domicilio legal: 9 de Julio 1324, (2000) Rosario, Santa Fe, Argentina.
Editores responsables: Dra. Virginia Massheimer - Dr. Fernando Saraví

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