Literature DB >> 18446747

Is survival enough for quality of life in Sagliker Syndrome-uglifying human face appearances in chronic kidney disease?

Yahya Sagliker1, Vidya Acharya, Ovidia Golea, Alaa Sabry, Musa Bali, Kamil Eyupoglu, Dhananjay Ookalkar, Shruti Tapiawala, Santosh Durugkar, Prakash Khetan, Cristina Capusa, Ramazan Univar, Ismail Yildiz, Kuddisi Cengiz, Harun Akar, Yavuz Yenicerioglu, Piril Sagliker Ozkaynak, Hasan Sabit Sagliker, Nuray Paylar.   

Abstract

BACKGROUND: It is known that secondary hyperparathyroidism (SH) and particularly skeletal changes is a severe condition in chronic kidney disease (CKD). Sagliker syndrome (SS) is a very prominent feature in CKD including uglifying human face appearances, short stature, extremely severe maxillary and mandibulary changes, soft tissues in the mouth, teeth-dental abnormalities, finger tip changes and severe psychological problems.
METHODS: In the last 8 years we have confronted 36 extremely incredible SS cases in CKD by performing an international study in Turkey, India, Malaysia, Romania and Egypt.
RESULTS: In addition to the uglifying human face appearance, we found extremely severe X-ray and tomographical, pantomographical, histo-pathological changes in the head and whole body. Finally, we compared previous face pictures with recent ones. Just a few years earlier they had been pretty and good-looking young boys and girls. By investigating their history, we understood they had not received proper therapy and were in the late-irreversible period.
CONCLUSION: SS is a serious and severe complication of CKD. Late and improper treatment leads to abnormalities throughout skeleton particularly in the skull and face. Changes particularly in children and teens become irreversible-disastrous for appearance and psychological health. Appropriate treatment must begin as early as possible in specialized centers. It is possible that SS patients may survive long-term with dialysis, but with all those particular changes could anyone claim this type of life would continue in an acceptable way without extending their height, correcting all the changes in the skull and face, remodeling new faces and most particularly convincing the patients to deal with all those tragi-dramatic psychological problems?

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Year:  2008        PMID: 18446747

Source DB:  PubMed          Journal:  J Nephrol        ISSN: 1121-8428            Impact factor:   3.902


  3 in total

1.  Sagliker syndrome in a patient with end-stage renal disease with secondary hyperparathyroidism.

Authors:  Muhammad Ajmal Panezai; Sana Ahmed; Gates B Colbert
Journal:  Proc (Bayl Univ Med Cent)       Date:  2019-07-15

2.  Sagliker Syndrome in a Patient with Secondary Hyperparathyroidism and Chronic Renal Insufficiency: A Case Report.

Authors:  Sara Shakeri; Soroush Zarehparvar Moghadam; Ramin Sadeghi; Narjess Ayati
Journal:  Asia Ocean J Nucl Med Biol       Date:  2018

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

Authors:  Jiao-Ping Mi; Peng He; Kai Shi; Shao-Yan Feng; Xian-Zhen Chen; Qing-Qing He; Ming-Yue Zhao; Ping-Jiang Ge; Yun-Ping Fan
Journal:  Ann Transl Med       Date:  2021-06
  3 in total

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