Download Citation on ResearchGate | Encondromatosis múltiple, enfermedad de Ollier | Multiple enchondromatosis (Ollier’s disease) is a rare disease. PDF | Ollier disease (Spranger type I) is a rare bone disease that is characterized by multiple Encondromatosis múltiple, enfermedad de Ollier. Article. Enchondromatosis, also known as Ollier disease (see Terminology section), is a non-hereditary, sporadic, skeletal disorder characterised by multiple.

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Lifelong monitoring is required in olliers patients given the risk of malignant transformation [ 6 ]. Ollier disease X-ray image showing enchondromas localized in the lower part of the radius of a 7-year-old girl with Ollier disease.


In enchondromas and chondrosarcomas from 31 enchondromatosis patients Ollier disease or Maffucci syndrome, lacking platyspondyly from 3 different European countries, Rozeman et al.

The mutation RC was heterozygous and was found in enchondroma specimens from 2 of 6 individuals with Ollier enchondromatosis.

About Blog Go ad-free. Ollier disease enchondromatosis associated with ovarian juvenile granulosa cell tumor and precocious pseudopuberty. Achondrogenesis type 1B Autosomal recessive multiple epiphyseal dysplasia Atelosteogenesis, type II Diastrophic dysplasia.

Nara Sobreira – updated: You can change the settings or obtain more information by clicking here. Enchondromas are common benign cartilage tumors of bone. SNIP measures contextual citation impact by wighting citations based on the total ollie of citations in a subject field.

Encondromatosis múltiple, enfermedad de Ollier | Anales de Pediatría (English Edition)

In distinction from solitary lesions, enchondromas of enchondromatosis are more likely to be hypercellular in nature, yet are still considered benign in the absence of other aggressive findings Two peculiar types of enchondromatosis.

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Generalized enchondromatosis in a boy with only platyspondyly in the father. Additional information Further information on this disease Classification s 4 Gene s 3 Clinical signs and symptoms Publications in PubMed Other website s ollief. J Bone Joint Surg Am. Related Radiopaedia articles Ollief tumours The differential diagnosis for bone tumours is dependent on the age of the patient, with a very different set of differentials for the pediatric patient.

Olliers disease and maffucci syndrome are usually non-familial encondromatlsis [ 3 ]. This rare combination is known as metachondromatosis. Please review our privacy policy. Samples from multiple tumors range, ; mean, 2.

Osteochondrodysplasia Q77—Q78 Find articles by Soumya Cicilet. Most patients have bilateral involvement but often significant asymmetry 4. Professionals Summary information Greekpdf Review article English You can help by adding to it.

The documents contained in this web site are presented for information purposes only. The Spanish Association of Pediatrics has as one of its main objectives the dissemination of rigorous and updated scientific information on the different areas of pediatrics. In patients with limb discrepancy distraction osteogenesis with the use of ilizarov instrument is indicated encondrommatosis 2 ].

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Case 2 Case encondromatowis. Dominant inheritance with reduced penetrance is possible. The authors thus suggested that the mutations occurred during development, resulting in genetic mosaicism in these individuals. However routine use of magnetic resonance imaging is not recommended because plain radiographs provide adequate information [ 2 ].

Ollier disease

Otro caso de discondrosteosis generalizada congenita, tipo Ollier. Case 3 Case 3. This article has been cited by other articles in PMC. The patients become symptomatic usually in the first decade of life. Disease definition Enchondromatosis is a rare primary bone dysplasia disorder characterized by the development of multiple mainly unilateral or asymmetrically distributed enchondromas throughout the metaphyses of the long bones.


There was no such history in her family. If involvement of the lower limbs is significant, leg length discrepancy may occur. However, a differential of hereditary exostosis needs to be ruled out. The Impact Factor measures the average number of citations received in a particular year by papers published in the journal during the two receding years.

C ] – Ovarian juvenile granulosa cell tumor with precocious pseudopuberty. Review of the literature with an emphasis on the clinical behaviour, radiology, malignant transformation and the follow up. From Wikipedia, the free encyclopedia. There are a few instances of familial occurrence.

Due to its rarity literature focusing on olliers disease is limited. When malignancy is suspected, the histopathology investigation is used for grading because different grade requires different management [ 2 ]. Si continua navegando, consideramos que acepta su uso. Check for errors and try again.

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