A case of bilateral renal hypoplasia and neonatal hemochromatosis
Neonatal hemochromatosis is a severe, often fatal multiorgan disorder of iron metabolism. We report a case of neonatal hemochromatosis associated with bilateral renal hypoplasia. The patient also had congenital intestinal atresia and meconium peritonitis. The radical operation was performed for cong...
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| Veröffentlicht in: | Japanese journal of pediatric nephrology 2011/04/15, Vol.24(1), pp.68-73 |
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| container_title | Japanese journal of pediatric nephrology |
| container_volume | 24 |
| creator | Hattori, Kiyoko Nakazato, Hitoshi Kawano, Tomoyasu Satoh, Ayumi Tamura, Hiroshi Matsumoto, Shirou Ichihara, Junko Karashima, Mami Tanaka, Kenichi Iwai, Masanori Itoh, Norihiro Nishi, Kazuhiko Mitsubuchi, Hiroshi Kondo, Yuichi Endo, Fumio |
| description | Neonatal hemochromatosis is a severe, often fatal multiorgan disorder of iron metabolism. We report a case of neonatal hemochromatosis associated with bilateral renal hypoplasia. The patient also had congenital intestinal atresia and meconium peritonitis. The radical operation was performed for congenital intestinal atresia at one-day-old. After surgery, oliguria occurred and renal disorder progressed to chronic renal failure. Subsequently, liver dysfunction and hyperferritinemia appeared. Bilateral hypoplastic kidney was diagnosed by CT scan, and MRI demonstrated abnormal iron deposition in the liver, pancreas and renal cortex at 2-month-old, indicating hemochromatosis. The hemochromatosis slowly improved by withdrawal of blood and oral vitamin E. Peritoneal dialysis was initiated for chronic renal failure at 10-month-old. We conclude that this is a very rare case of renal hypoplasia complicated with neonatal hemochromatosis. |
| doi_str_mv | 10.3165/jjpn.24.68 |
| format | Article |
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We report a case of neonatal hemochromatosis associated with bilateral renal hypoplasia. The patient also had congenital intestinal atresia and meconium peritonitis. The radical operation was performed for congenital intestinal atresia at one-day-old. After surgery, oliguria occurred and renal disorder progressed to chronic renal failure. Subsequently, liver dysfunction and hyperferritinemia appeared. Bilateral hypoplastic kidney was diagnosed by CT scan, and MRI demonstrated abnormal iron deposition in the liver, pancreas and renal cortex at 2-month-old, indicating hemochromatosis. The hemochromatosis slowly improved by withdrawal of blood and oral vitamin E. Peritoneal dialysis was initiated for chronic renal failure at 10-month-old. We conclude that this is a very rare case of renal hypoplasia complicated with neonatal hemochromatosis.</description><identifier>ISSN: 0915-2245</identifier><identifier>EISSN: 1881-3933</identifier><identifier>DOI: 10.3165/jjpn.24.68</identifier><language>jpn</language><publisher>The Japanese Society for Pediatric Nephrology</publisher><subject>neonatal hemochromatosis ; peritoneal dialysis ; renal hypoplasia</subject><ispartof>Japanese journal of pediatric nephrology, 2011/04/15, Vol.24(1), pp.68-73</ispartof><rights>2011 The Japanese Society for Pediatric Nephrology</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c1278-f0a70cd3de7bba719eb892f4dbd69afa2571d2423f7d3abde9fd7204dd1f49033</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,1883,27924,27925</link.rule.ids></links><search><creatorcontrib>Hattori, Kiyoko</creatorcontrib><creatorcontrib>Nakazato, Hitoshi</creatorcontrib><creatorcontrib>Kawano, Tomoyasu</creatorcontrib><creatorcontrib>Satoh, Ayumi</creatorcontrib><creatorcontrib>Tamura, Hiroshi</creatorcontrib><creatorcontrib>Matsumoto, Shirou</creatorcontrib><creatorcontrib>Ichihara, Junko</creatorcontrib><creatorcontrib>Karashima, Mami</creatorcontrib><creatorcontrib>Tanaka, Kenichi</creatorcontrib><creatorcontrib>Iwai, Masanori</creatorcontrib><creatorcontrib>Itoh, Norihiro</creatorcontrib><creatorcontrib>Nishi, Kazuhiko</creatorcontrib><creatorcontrib>Mitsubuchi, Hiroshi</creatorcontrib><creatorcontrib>Kondo, Yuichi</creatorcontrib><creatorcontrib>Endo, Fumio</creatorcontrib><title>A case of bilateral renal hypoplasia and neonatal hemochromatosis</title><title>Japanese journal of pediatric nephrology</title><addtitle>Nihon Shoni Jinzobyo Gakkai Zasshi</addtitle><description>Neonatal hemochromatosis is a severe, often fatal multiorgan disorder of iron metabolism. We report a case of neonatal hemochromatosis associated with bilateral renal hypoplasia. The patient also had congenital intestinal atresia and meconium peritonitis. The radical operation was performed for congenital intestinal atresia at one-day-old. After surgery, oliguria occurred and renal disorder progressed to chronic renal failure. Subsequently, liver dysfunction and hyperferritinemia appeared. Bilateral hypoplastic kidney was diagnosed by CT scan, and MRI demonstrated abnormal iron deposition in the liver, pancreas and renal cortex at 2-month-old, indicating hemochromatosis. The hemochromatosis slowly improved by withdrawal of blood and oral vitamin E. Peritoneal dialysis was initiated for chronic renal failure at 10-month-old. 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We report a case of neonatal hemochromatosis associated with bilateral renal hypoplasia. The patient also had congenital intestinal atresia and meconium peritonitis. The radical operation was performed for congenital intestinal atresia at one-day-old. After surgery, oliguria occurred and renal disorder progressed to chronic renal failure. Subsequently, liver dysfunction and hyperferritinemia appeared. Bilateral hypoplastic kidney was diagnosed by CT scan, and MRI demonstrated abnormal iron deposition in the liver, pancreas and renal cortex at 2-month-old, indicating hemochromatosis. The hemochromatosis slowly improved by withdrawal of blood and oral vitamin E. Peritoneal dialysis was initiated for chronic renal failure at 10-month-old. We conclude that this is a very rare case of renal hypoplasia complicated with neonatal hemochromatosis.</abstract><pub>The Japanese Society for Pediatric Nephrology</pub><doi>10.3165/jjpn.24.68</doi><tpages>6</tpages><oa>free_for_read</oa></addata></record> |
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| subjects | neonatal hemochromatosis peritoneal dialysis renal hypoplasia |
| title | A case of bilateral renal hypoplasia and neonatal hemochromatosis |
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