Renal cysts and nephrocalcinosis in a patient deficient in 11 beta-hydroxylase enzyme

2015
journal article
article
4
dc.abstract.enBACKGROUND: Chronic hypokalemia is known to induce renal structural and functional abnormality. The former includes induction of renal cyst formation and interstitial fibrosis while the latter entails urine-concentrating defect. However, these hypokalemia-mediated changes occur in a handful of conditions including primary aldosteronism, distal renal tubular acidosis, Liddle's disease, apparent mineralocorticoid excess syndrome and Bartter's type 3 syndrome. Such a finding has never been described in an 11 beta-hydroxylase deficient individual. CASE REPORT: We describe a case of a 15-year-old male, deficient in 11 beta-hydroxylase enzyme, presenting with hypertensive haemorrhage in basal ganglia and chronic hypokalemia-mediated nephrocalcinosis and renal cysts. To add to the uniqueness, our patient was discovered to harbour bilateral testicular adrenal rests as well. CONCLUSIONS: An early diagnosis could help prevent these sequelae and preserve long-term renal function and safeguard against ill-effects of hypertension. Besides, aetiology of nephrocalcinosis should be sought for and corrected.pl
dc.contributor.authorAswani, Yashantpl
dc.contributor.authorThakkar, Hemanginipl
dc.contributor.authorHira, Priyapl
dc.date.accessioned2017-07-17T11:06:58Z
dc.date.available2017-07-17T11:06:58Z
dc.date.issued2015pl
dc.date.openaccess0
dc.description.accesstimew momencie opublikowania
dc.description.additionalBibliogr. s. 381pl
dc.description.physical379-381pl
dc.description.versionostateczna wersja wydawcy
dc.description.volume80pl
dc.identifier.doi10.12659/PJR.894678pl
dc.identifier.eissn1899-0967pl
dc.identifier.issn1733-134Xpl
dc.identifier.urihttp://ruj.uj.edu.pl/xmlui/handle/item/42852
dc.languageengpl
dc.language.containerengpl
dc.rightsUdzielam licencji. Uznanie autorstwa - Użycie niekomercyjne - Bez utworów zależnych 3.0 Polska*
dc.rights.licenceCC-BY-NC-ND
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/3.0/pl/legalcode*
dc.share.typeotwarte czasopismo
dc.subject.enadrenal hyperplasiapl
dc.subject.encongenitalpl
dc.subject.enhypertensionpl
dc.subject.enhypokalemiapl
dc.subject.ennephrocalcinosispl
dc.subtypeArticlepl
dc.titleRenal cysts and nephrocalcinosis in a patient deficient in 11 beta-hydroxylase enzymepl
dc.title.journalPolish Journal of Radiologypl
dc.typeJournalArticlepl
dspace.entity.typePublication
dc.abstract.enpl
BACKGROUND: Chronic hypokalemia is known to induce renal structural and functional abnormality. The former includes induction of renal cyst formation and interstitial fibrosis while the latter entails urine-concentrating defect. However, these hypokalemia-mediated changes occur in a handful of conditions including primary aldosteronism, distal renal tubular acidosis, Liddle's disease, apparent mineralocorticoid excess syndrome and Bartter's type 3 syndrome. Such a finding has never been described in an 11 beta-hydroxylase deficient individual. CASE REPORT: We describe a case of a 15-year-old male, deficient in 11 beta-hydroxylase enzyme, presenting with hypertensive haemorrhage in basal ganglia and chronic hypokalemia-mediated nephrocalcinosis and renal cysts. To add to the uniqueness, our patient was discovered to harbour bilateral testicular adrenal rests as well. CONCLUSIONS: An early diagnosis could help prevent these sequelae and preserve long-term renal function and safeguard against ill-effects of hypertension. Besides, aetiology of nephrocalcinosis should be sought for and corrected.
dc.contributor.authorpl
Aswani, Yashant
dc.contributor.authorpl
Thakkar, Hemangini
dc.contributor.authorpl
Hira, Priya
dc.date.accessioned
2017-07-17T11:06:58Z
dc.date.available
2017-07-17T11:06:58Z
dc.date.issuedpl
2015
dc.date.openaccess
0
dc.description.accesstime
w momencie opublikowania
dc.description.additionalpl
Bibliogr. s. 381
dc.description.physicalpl
379-381
dc.description.version
ostateczna wersja wydawcy
dc.description.volumepl
80
dc.identifier.doipl
10.12659/PJR.894678
dc.identifier.eissnpl
1899-0967
dc.identifier.issnpl
1733-134X
dc.identifier.uri
http://ruj.uj.edu.pl/xmlui/handle/item/42852
dc.languagepl
eng
dc.language.containerpl
eng
dc.rights*
Udzielam licencji. Uznanie autorstwa - Użycie niekomercyjne - Bez utworów zależnych 3.0 Polska
dc.rights.licence
CC-BY-NC-ND
dc.rights.uri*
http://creativecommons.org/licenses/by-nc-nd/3.0/pl/legalcode
dc.share.type
otwarte czasopismo
dc.subject.enpl
adrenal hyperplasia
dc.subject.enpl
congenital
dc.subject.enpl
hypertension
dc.subject.enpl
hypokalemia
dc.subject.enpl
nephrocalcinosis
dc.subtypepl
Article
dc.titlepl
Renal cysts and nephrocalcinosis in a patient deficient in 11 beta-hydroxylase enzyme
dc.title.journalpl
Polish Journal of Radiology
dc.typepl
JournalArticle
dspace.entity.type
Publication
Affiliations

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