Cases reported "Kidney Diseases"

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1/10. Idiopathic multicentric osteolysis with nephropathy.

    Idiopathic multicentric osteolysis is a rare syndrome that manifests with progressive loss of carpal and tarsal bones in childhood. Affected children have arthritic-like episodes, followed by progressive deformities, radiographic osteolytic changes, and variable degrees of disability. A rare form of this disease (type III, sporadic) is associated with serious nephropathy. We present the first reported case of type III idiopathic multicentric osteolysis in a Chinese woman. The patient, a 34-year-old woman with normal mental development and no family history of bone or kidney disease, presented with a 4-day history of nausea and vomiting. She had shortening and swelling of the hands, which had occurred in childhood and persisted at the time of admission. X-ray studies showed disappearance of the carpal bones, and multiple osseous erosions of the tarsal bones. hypertension, severe azotemia, and metabolic acidosis were also noted. Advanced renal disease was documented after a series of investigations, including renal biopsy. She is now dialysis-dependent. This case illustrates the importance of early diagnosis and management of idiopathic multicentric osteolysis with nephropathy.
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keywords = osteolysis
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2/10. Idiopathic carpotarsal osteolysis with nephropathy.

    Idiopathic carpotarsal osteolysis is characterized by gradual lysis and resorption of bones, occurring mainly on hands and feet. It may be sporadic or hereditary and either form can manifest renal involvement. Nephropathy is seen more frequently and is more severe in the sporadic form. We present herein two new cases of unrelated boys with the sporadic form associated with nephropathy. One of the patients had focal segmental glomerulosclerosis. The other patient revealed isolated proteinuria.
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ranking = 0.71428571428571
keywords = osteolysis
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3/10. Carpal and tarsal osteolysis.

    A 19-year-old girl with carpal and tarsal osteolysis has been presented. The clinical course was characterized by normal early growth and motor development followed by the insidious onset in early childhood of a progressive, peripheral joint destruction, especially in the tarsal and carpal bones. The patient's course was complicated by acquired spasticity from platybasia, scoliosis, generalized muscle weakness, mild growth failure and corneal clouding.
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ranking = 0.71428571428571
keywords = osteolysis
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4/10. Idiopathic multicentric osteolysis.

    A 59 year old while female with striking carpal and tarsal osteolysis is reported. The process primarily involves the carpals and tarsals but includes other sites. The pertinent literature is reviewed and a simplified classification of idiopathic multicentric osteolysis is presented. Idiopathic multicentric osteolysis may be sporadic but can demonstrate either dominant or recessive inheritance. There may or may not be associated nephropathy. The underlying mechanism appears similar in all variations of this condition. Gorham's disease, the Winchester syndrome, and other osteolytic conditions which may predominantly affect the tarsal and carpal bones most likely are totally different processes, unrelated to idiopathic multicentric osteolysis.
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ranking = 1.1428571428571
keywords = osteolysis
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5/10. Essential osteolysis associated with nephropathy, corneal opacity, and pulmonary stenosis.

    We report on 5-year-old girl with essential osteolysis, nephropathy, corneal opacity, and valvular pulmonary stenosis. The patient was initially seen for evaluation of flexion contractures at wrists, elbows, and knees. Radiographic examination showed osteolytic changes primarily involving the hands and feet. She had persistent proteinuria; renal biopsy disclosed focal glomerulosclerosis in 1/3 of glomeruli. Electron microscopic study of skin fibroblast showed dilated and vacuolated rough endoplasmic reticulum. To our knowledge essential osteolysis associated with the aforementioned disorders has not been previously reported.
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ranking = 0.85714285714286
keywords = osteolysis
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6/10. Idiopathic multicentric osteolysis with facial anomalies and nephropathy.

    Idiopathic osteolysis denotes a group of rare bone disorders differentiated on the basis of clinical, radiological, and genetic criteria. Idiopathic multicentric osteolysis (IMO) is one form of osteolysis that can occur as an autosomal dominant condition. In childhood, affected individuals have arthritic-like episodes, followed by progressive deformities, radiological osteolytic changes, and variable degrees of disability. A peculiar face and variable renal involvement have been described as associated manifestations. We report on a family with three members affected by IMO in two generations showing variable bone changes and persistent proteinuria. A sporadic case with severe renal damage is also described in which a de novo dominant mutation is suggested. All patients had peculiar facial manifestations including triangular shape, protruding eyes, and micrognathia. These manifestations may be part of the syndrome of IMO.
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ranking = 1
keywords = osteolysis
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7/10. Carpal and tarsal osteolysis.

    This report describes the symptoms, clinical course and radiological features in three cases of idiopathic carpotarsal osteolysis. Before signs of extensive osteolysis were noted, one of our patients showed flattening, loss of harmonious curvature and minimal osteoporosis of carpal and tarsal bones, all early radiological signs which have not been described previously. We discuss the findings, classification and our ideas as to the cause of the disorder.
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ranking = 0.85714285714286
keywords = osteolysis
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8/10. Carpal and tarsal osteolysis.

    A 34-year-old male is reported with carpal and tarsal osteolysis syndrome. In addition he presented a fluctuating proteinuria and a slightly reduced renal function. The relevant literature on the subject is reviewed and the combination of carpal and tarsal osteolysis with nephropathy is discussed.
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ranking = 0.85714285714286
keywords = osteolysis
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9/10. Nephropathy of idiopathic multicentric osteolysis.

    Progressive osteolysis with disappearance of the carpal and tarsal bones occurs as a rare syndrome associated with a serious nephropathy. The characteristic skeletal changes have an onset in early childhood and precede renal failure by 10--15 years. The present case, the ninth reported in the world literature, was a 22-year-old, white female who presented with carpal and tarsal swelling at age 2. Severe hypertension and azotemia were present when the patient died of pneumonia and extracellular fluid volume depletion. At necropsy, the kidneys weighed 55 g each. microscopy showed proliferation of vascular intima and medial hypertrophy of renal vessels of all sizes. Tubulointerstitial and glomerular changes are probably secondary to the severe vascular disease. Although similar skeletal lesions can be seen in kindred with autosomal dominant and recessive inheritance, all reported cases of nephropathy including the present one are sporadic.
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ranking = 0.71428571428571
keywords = osteolysis
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10/10. Idiopathic multicentric osteolysis: family report and review of the literature.

    A mother and son affected by idiopathic multicentric osteolysis are reported. This condition usually manifests in early childhood and is characterized by progressive destruction of the carpal and tarsal bones, with or without renal anomalies. Unusual facies might be the clinical features of the syndrome. review of the literature shows that osteolysis can occur in isolation or may be associated with renal and/or facial anomalies.
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ranking = 0.85714285714286
keywords = osteolysis
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