International study on Sagliker syndrome and uglifying human face appearance in severe and late secondary hyperparathyroidism in chronic kidney disease patients.
OBJECTIVE
It is known that skeletal changes due to secondary hyperparathyroidism (SH) can be severe in chronic kidney disease (CKD). Recently described Sagliker syndrome (SS) is a very striking and prominent feature of SH in CKD, including an uglifying appearance to the face, short stature, extremely severe maxillary and mandibulary changes, soft tissue in the mouth, teeth/dental abnormalities, fingertip changes, knee and scapula deformities, hearing abnormalities, and neurological and, more important, severe psychological problems.


METHODS
In the past 8 years, we have encountered 40 cases of SS in SH and CKD by performing an international study in Turkey, India, Romania, Egypt, Maleysia, Tunis, and China.


RESULTS
The medical history of these patients showed that they did not receive proper therapy. Changes, particularly in children and teenagers, become irreversible, which was disastrous for the patients both aesthetically and psychologically.


CONCLUSIONS
Treatment must begin early and be the appropriate treatment given in centers with sophisticated skills. Otherwise, the inability to correct all the changes in the skull and face, to remodel a new face, to extending the height, and, most important, to convince the patients to face the dramatic psychological problems can be catastrophic for those patients.
Sagliker
Yahya
Y
Nephrology Division, Cukurova University, Adana, Turkey. ysagliker@yahoo.com
Acharya
Vidya
V
Ling
Zhang
Z
Golea
Ovidia
O
Sabry
Alaa
A
Eyupoglu
Kamil
K
Ookalkar
Dhananjay S
DS
Tapiawala
Shruti
S
Durugkar
Santosh
S
Khetan
Prakash
P
Capusa
Cristina
C
Univar
Ramazan
R
Yildiz
Ismail
I
Cengiz
Kuddisi
K
Bali
Musa
M
Ozkaynak
Piril Sagliker
PS
Sagliker
Hasan Sabit
HS
Paylar
Nuray
N
Adam
Siddik Momin
SM
Balal
Mustafa
M
Paydas
Saime
S
Demirhan
Osman
O
Tasdemir
Deniz
D
Ben Maiz
Hedi
H
Redulescu
Dana
D
Garneata
Liliana
L
Mircescu
Gabriel
G
Hong-Liang
Rui
R
Lun
Lide
L
Yildizer
Kasim
K
Emir
Idris
I
Yuksekgonul
Musa
M
Yenicerioglu
Yavuz
Y
Akar
Harun
H
Sagliker
Cemal
C
Esenturk
Mustafa
M
Kiralp
Necati
N
D000328:Adult; D001827:Body Height; D005145:Face / Q000002:abnormalities*; D005147:Facial Bones / Q000002:abnormalities; D005260:Female; D006801:Humans; D006962:Hyperparathyroidism, Secondary / Q000453:epidemiology / Q000523:psychology*; D007676:Kidney Failure, Chronic / Q000150:complications* / Q000523:psychology; D008297:Male; D001523:Mental Disorders / Q000453:epidemiology*; D035781:Siblings; D012886:Skull / Q000033:anatomy & histology; D013131:Spine / Q000002:abnormalities