Cephalometric Evaluation of Patients With Sagliker Syndrome: Uglifying Human Face Appearance in Severe and Late Secondary Hyperparathyroidism in Chronic Renal Failure Patients

Cephalometric craniofacial features of patients with Sagliker syndrome: a primary analysis of our experience

“…Our results revealed that the mandible did not show significant changes in its sagittal direction, rotating backwards and vertically downwards. Overall, the facial features of patients with SS are characterized by a convex profile with excessive vertical growth of the maxilla (vertical maxillary excess) and mandibular retrusion; this is consistent with observations made in a previous study of 4 SS patients (12), and the very strong impression of "lion face" observed in patients.…”

“…As a critical evaluation of the sagittal position of the maxilla to the mandible, the ANB angle showed a statistically significant increase in SS patients, which is well in keeping with observations in other studies. Among these researches, Uzel's lateral cephalometric analysis observed severe skeletal class II malocclusion with mandibular deficiency in the majority of participants with SS (12). Vertical skeletal measurements showed that patients with SS have statistically significantly increased lower and total anterior facial heights, whereas the upper anterior and total posterior facial heights did not show significant differences between the two groups.…”

Sagliker syndrome: A case report of a rare manifestation of uncontrolled secondary hyperparathyroidism in chronic renal failure

“…Sagliker et al[5] reported 25 patients who developed cephalometric skull changes, maxillary and mandibular bone deformations, irregular and misshaped teeth, soft and tumoural tissues inside the oral cavity, a lion-like appearance, short stature, and fingertip changes. Uzel et al[6] and Giray et al[7] reported that SS might result in class II malocclusions or neurologic manifestations, such as headaches, fatigue, and polyneuropathy. Ozenli et al[8] confirmed that psychiatric morbidities were more severe and common in this population.…”

Chromosomal findings and sequence analysis of target exons of calcium-sensingreceptor (CaSR) gene in patients with Sagliker syndrome

“…This syndrome starts and develops particularly before puberty while chronic kidney disease (CKD) reaches the stage III level and as a continuation of secondary hyperparathyroidism. Defining prominent features of CKD, including uglifying human facial appearance, short stature, extremely severe maxillary and mandibulary changes, soft tissues in the mouth, teeth-dental abnormalities, fingertip changes, neurologic manifestations, audiological findings, and severe psychological problems (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13) (Figures 1 and 2), are also important findings for this unique syndrome. The etiology of SS is not known.…”