A Preliminary Study Chinese Sagliker Syndrome

Sagliker syndrome (SS) was described in2004as a very prominent feature of this kind of change and included CKD, SH, uglifying appearance to the face in varying degrees from mild to extremely severe skull changes and other very severe maxillary and mandibular jaw changes, soft and pathologically demonstrated benign mucosal hyperplasia tissues in the mouth, severe teeth-dental, cephalometric skull changes, short stature, fingertip changes, knee and scapula deformities, hearing abnormalities, severe neurological and disturbingly severe psychological problems, and depression, among other features. However, the patients suffering from this syndrome have not yet been investigated in China.This study included two parts:one is the craniofacial morphologies of Sagliker syndrome, the other is the etiology of Sagliker Syndrome. Part Ⅰ:16patients were selected in this study that was diagnosed by the Department of Nephrology and the Center of Dentistry of China-Japan Friendship Hospital. They were divided into2groups:the Sagliker syndrome group (SS) included8patients,the secondary hyperparathyroidism group (SHPT) included8patients. Cephalometric radiographs were taken from all the16patients.29skeletal and dental cephalometric measurements were carried out for each patient to determine their craniofacial morphologies. The results showed the whole body of maxillary increased obviously at the horizontal and vertical directions. The anterior of the mandibular increased obviously at the vertical direction. The other part of the mandibular had no obvious change. The measurement of the soft tissue had the similar changes to the bone. The dental measurement of the dentist had no obvious change.Part Ⅱ:Select of8patients of Sagliker Syndrome and26patients of simple SHPT who were diagnosed by the Department of Nephrology and the Center of Dentistry of China-Japan Friendship Hospital. All the patients had detail information and data on gender, age, the cause of SHPT, dialysis age and several related blood parameters, and all of the data of the two different groups were compared, and liner regression analysis was done between the related blood parameters. Gender, age, dialysis age might be risk factors of Sagliker Syndrome. If patients was female, young and had a long dialysis age, the possibility to be a Sagliker Syndrome patient was high. For Sagliker Syndrome patient, the increasing of phosphate concentration could not result in the increasing of PTH concentration directly, so there might be some inhibition of forward conduction mechanism of the phosphate concentration increases-PTH concentration increasing. In addition, there might be some mechanism in Sagliker Syndrome patients, which making the average ALP concentration of the Sagliker Syndrome patient was far higher than that of the simple SHPT patient when the PTH concentration was at a high level, and at the same time making the ALP concentration of the Sagliker Syndrome patient was more sensitive to changes in PTH concentration than that of the simple SHPT patient.