Asplenia Syndrome with Congenital Microgastria and Malrotation
نویسندگان
چکیده
منابع مشابه
Congenital short bowel syndrome with malrotation.
Congenital short bowel syndrome (SBS) associated with malrotation and malabsorption is a very rare condition. We report on an infant girl with congenital SBS associated with malrotation and malabsorption. No polyhydraminos was noted during the regular prenatal examination. Protracted postnatal postprandial vomiting with progressive failure to thrive was noted. A laparotomy showed the small bowe...
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Five patients developed overwhelming infection as a result of congenital asplenia, which was previously unsuspected in all cases. Each illness followed a fulminant course resulting in death within 24 hours. They illustrate the respective roles of Haemophilus influenzae infection (n = 4) and adrenal haemorrhage (n = 4) in this condition. We suggest a management protocol for screening infants wit...
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Congenital asplenia is rare and could occur sporadically or less commonly as part of a syndrome. Sporadic or isolated congenital asphenia is not usually associated with significant morbidity. Congenical isolated asphenia can be present as septicaemia, commonly pneumococcal, hence the importance of screening the peripheral blood for Howell-Jolly bodies. Abdominal imaging becomes necessary if scr...
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BACKGROUND Isolated congenital asplenia is a poorly understood and rare form of primary immunodeficiency, often associated with life-threatening infections. CASE REPORT We encountered a unique case of a 22-year-old asplenic male who presented with severe iron-deficiency anemia secondary to occult gastrointestinal bleeding since age 15. Our extensive work-up confirmed jejunal arteriovenous malfo...
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Microgastria is a rare congenital condition often associated with other anomalies. In the present report we describe the case of a 6-year-old girl with isolated CM who presented with dumping syndrome successfully treated by a Hunt-Lawrence pouch.
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ژورنال
عنوان ژورنال: Journal of Perinatology
سال: 2003
ISSN: 0743-8346,1476-5543
DOI: 10.1038/sj.jp.7211022