Severe Renal Osteodystrophy in a Pediatric Patient With End-Stage Renal Disease: Sagliker Syndrome?

Yavascan O., Kose E., Alparslan C., Sirin Kose S., Bal A., Kanik A., ...Daha Fazla

JOURNAL OF RENAL NUTRITION, cilt.23, sa.4, ss.326-330, 2013 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 23 Sayı: 4
  • Basım Tarihi: 2013
  • Doi Numarası: 10.1053/j.jrn.2012.07.002
  • Dergi Adı: JOURNAL OF RENAL NUTRITION
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.326-330
  • Ankara Üniversitesi Adresli: Hayır

Özet

Renal osteodystrophy (ROD) is a multifactorial disorder of bone metabolism in chronic kidney disease (CKD). As CKD progresses, ensuing abnormalities in vitamin D metabolism and parathyroid hormone (PTH) secretion result in distortions in trabecular microarchitecture, thinning of the cortical shell, and increased cortical porosity. The recently described Sagliker syndrome (SS) might be an exaggerated version of ROD and is a very striking and prominent feature of secondary hyperparathyroidism in patients with end-stage renal disease (ESRD). It includes a distorted facial appearance, short stature, extremely severe maxillary and mandibulary changes, soft tissue tumors in the mouth, teeth/dental abnormalities, fingertip changes, knee and scapula deformities, hearing abnormalities, and neurologic and psychological problems.