RADIOLOGICAL FINDINGS IN X-LINKED HYPOPHOSPHATEMIA

Öz

Radiological findings in X-linked hypophosphatemia are similar to, albeit less severe than, nutritional rickets. Loss of distinctness of the zone of provisional calcification, widening of the growth plate first in the long axis, followed by widening in the short axis, “cupping” of the metaphysis and irregularity (fraying) at the metaphyseal border are seen on X-rays of long bones. The severity of the findings can be assessed from bilateral anteroposterior X-rays of the wrists and knees using the “Rickets Severity Score” (RSS). RSS can be used at the time of diagnosis as well as for assessing response to treatment. The “Radiographic Global Impression of Change Scale” (RGI-C) is another scoring method which can be used during follow-up of patients.

Anahtar Kelimeler

• X-ray,rickets,scoring

X’E BAĞLI HİPOFOSFATEMİDE RADYOLOJİK BULGULAR

Öz

X’e bağlı hipofosfatemide radyolojik olarak, daha hafif olmakla birlikte, nütrisyonel raşitizme benzer bulgular izlenir. Grafilerde uzun kemiklerin kemikleşme bölgesinde opak hattın kaybolması, büyüme plağında önce uzun aksta, daha sonra transvers planda genişleme, metafizde “kadehleşme” ve fizis ile metafiz arasında “fırçalaşma” diye tabir edilen düzensizlik izlenir. Bulguların şiddeti bilateral el bilek ve diz anteroposterior grafileri kullanılarak “Rickets Severity Score” (RSS) (Raşitizm Ağırlık Skoru) ile değerlendirilmektedir. RSS tanı ve takipte kullanılabilir. Takipte ayrıca “Radiographic Global Impression of Change Scale” (RGI-C) (Genel Radyografik Değişim Ölçüsü) de kullanılabilir.

Anahtar Kelimeler

• Grafi,raşitizm,skorlama

Kaynakça

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2. 2. Shore RM, Chesney, RW. Rickets: Part II. Pediatric Radiology 2013;43:152-72.
3. 3. Haffner D, Emma F, Eastwood DM, Duplan MB, Bacchetta J, Schnabel D, et al. Clinical practice recommendations for the diagnosis and management of X-linked hypophosphataemia. Nature Reviews Nephrology 2019;15:435-55.
4. 4. Thacher TD, Fischer PR, Pettifor JM, Lawson JO, Manaster BJ, Reading JC. Radiographic Scoring Method for the Assessment of the Severity of Nutritional Rickets. Journal of Tropical Pediatrics 2000;46;132-9.
5. 5. Thacher TD, Pettifor JM, Tebben PJ, Creo AL, Skrinar A, Mao M, et al. Rickets severity predicts clinical outcomes in children with X-linked hypophosphatemia: Utility of the radiographic Rickets Severity Score. Bone 2019;122:76-81.
6. 6. Whyte MP, Fujita KP, Moseley S, Thompson DD, McAlister WH. Validation of a Novel Scoring System for Changes in Skeletal Manifestations of Hypophosphatasia in Newborns, Infants, and Children: The Radiographic Global Impression of Change Scale. Journal of Bone and Mineral Research 2018;33:868-74.