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


CONCLUSIONS
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?
Sagliker
Yahya
Y
Division of Nephrology, Cukurova University, Ziya Pasa Bulvari, Sun Sinema Yani, Adana, Turkey. sagerya@superonline.com
Acharya
Vidya
V
Golea
Ovidia
O
Sabry
Alaa
A
Bali
Musa
M
Eyupoglu
Kamil
K
Ookalkar
Dhananjay
D
Tapiawala
Shruti
S
Durugkar
Santosh
S
Khetan
Prakash
P
Capusa
Cristina
C
Univar
Ramazan
R
Yildiz
Ismail
I
Cengiz
Kuddisi
K
Akar
Harun
H
Yenicerioglu
Yavuz
Y
Sagliker Ozkaynak
Piril
P
Sabit Sagliker
Hasan
H
Paylar
Nuray
N
D001827:Body Height; D001847:Bone Diseases / Q000209:etiology* / Q000473:pathology / Q000523:psychology; D002508:Cephalometry; D002908:Chronic Disease; D017281:Cost of Illness; D004534:Egypt; D005147:Facial Bones / Q000473:pathology; D005260:Female; D006801:Humans; D006962:Hyperparathyroidism, Secondary / Q000150:complications / Q000209:etiology* / Q000473:pathology / Q000523:psychology; D007194:India; D007674:Kidney Diseases / Q000150:complications* / Q000473:pathology / Q000523:psychology; D008296:Malaysia; D008297:Male; D001523:Mental Disorders / Q000209:etiology* / Q000473:pathology; D011788:Quality of Life; D012383:Romania; D012886:Skull / Q000473:pathology; D017741:Survivors / Q000523:psychology; D014421:Turkey