A Case of Juvenile Nephronophthisis Developed to End-stage Renal Failure at Infantile Period.
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Accession number;02A0351401
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| Title;A Case of Juvenile Nephronophthisis Developed to End-stage Renal Failure at Infantile Period. |
| Author;
TERAMOTO SATOSHI
(Tokyojikeikaiidai Kashiwabyoin Shonika)
SAITO YOSHIHIRO
(Tokyojikeikaiidai Kashiwabyoin Shonika)
ONO KYOKO
(Tokyojikeikaiidai Kashiwabyoin Shonika)
KOBAYASHI NAOAKI
(Tokyojikeikaiidai Kashiwabyoin Shonika)
TAMAKI HISASHI
(Tokyojikeikaiidai Kashiwabyoin Shonika)
USUI NOBUO
(Tokyojikeikaiidai Aotobyoin Shonika)
ITO FUMIYUKI
(Tokyojikeikaiidai Kashiwabyoin Shonika)
KUBO MASAKATSU
(Tokyojikeikaiidai Kashiwabyoin Shonika)
ETO YOSHIKATSU
(Tokyojikeikaiidai Byoin Hon'in Shonika)
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Journal Title;Journal of the Japan Pediatric Society
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Journal Code:F0896A
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ISSN:0001-6543
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VOL.106;NO.4;PAGE.504-507(2002)
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| Figure&Table&Reference;FIG.1, TBL.1, REF.13 |
| Pub. Country;Japan |
| Language;Japanese |
| Abstract;We report a case of juvenile nephronophthisis (JN) developed to end-stage renal failure at infantile period. The patient was an 11-month-old female infant presented with dyspnea, anemia, and renal dysfunction. Despite intensive care, she died four hours after admission. Generally, it is characteristic that renal dysfunction developed chronically in JN. But a few cases called "infantile nephronophthisis" (IN) which developed to end-stage renal failure at infantile period have been reported. In Japan, there have been more than 50 reports of JN, but only one report applied to IN. This may be the second report which applies to IN in Japan. There is no positive rational therapy for JN at present. And because renal dysfunction of IN develops rapidly in the infantile period, it is difficult to diagnose at an early stage. Recently, however, gene loci associated with JN and IN are being inferred, respectively. Thus, we expect that elucidation of the pathophysiology may lead to a rational therapy and control of the onset. (author abst.) |
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