HAVING MULTIPLE RENAL CYSTS IN A YOUNG ADULT IS
NOT ALWAYS A SIGN OF POLYCYSTIC KIDNEY DISEASE Kaynar K, Kayıpmaz S, Çebi AH, Hüseynova S *Corresponding Author: Professor Dr. Kubra Kaynar, Karadeniz Teknik Üniversitesi, Tıp Fakültesi,
Nefroloji Bilim Dalı, 61080 Trabzon, Turkey. Tel.: +90-542-241-58-79. Fax: +90-462-325-22-70. Email:
kkaynar@yahoo.com page: 83 download article in pdf format
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Abstract
Multiple renal cysts in adult patients could have asymptomatic,
benign and a nonprogressive course. However,
these cysts could be renal features of a very rare
hereditary, progressive syndrome defined as cranioectodermal
dysplasia (CED or Sensenbrenner syndrome).
Affected patients show dysmorphic features such as craniosynostosis,
nail dystrophy, cutaneous dyshydrosis, dry
or scaly palmar skin, trichodysplasia, deafness, pectus excavatum,
telecanthus, hypertelorism, low set ears, everted
lower lip, anteverted nares, short neck and height, joint
laxity, inguinal hernia, widely spaced teeth, microdontia,
hypodontia in addition to nephronophthisis. We report a
22-year-old male hypertensive patient with multiple renal
cysts and dental malformations listed as malocclusion,
screwdriver shaped crowns, widely spaced front teeth,
microdontia and hyperdontia. Molecular analysis reported
missense p.(Ala875Thr) and p.(Lys969Asn) variants in
the WDR35 gene. The 1-year follow-up of this case provided
the knowledge that angiotensin II receptor blocker
drug (olmesartan) reduced the microalbuminuria to normal
levels and preserved the renal functions. We suggest
interdisciplinary studies, especially intraoral and genetic
evaluations for patients with cystic renal diseases. For the
first time we report that hyperdontia could be found as a
dental feature of CED.
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