Results 81 to 90 of about 1,646 (102)
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Familial Isolated Congenital Asplenia: A Rare, Frequently Hereditary Dominant Condition, Often Detected Too Late as a Cause of Overwhelming Pneumococcal Sepsis. Report of a New Case and Review of 31 Others

European Journal of Pediatrics, 2002
Congenital isolated asplenia may arise as a minor form of situs abnormalities or result from an unrelated specific defect of spleen development. It is a rare life-threatening condition and pneumococcal sepsis is often the first sign of the disease. We report on the case of a deceased 11-month-old girl and her father who developed recurrent pneumococcal
Brigitte, Gilbert   +5 more
exaly   +4 more sources

Isolated Congenital Asplenia: An Occult Case of Overwhelming Sepsis

Archives of Pediatrics & Adolescent Medicine, 1979
Overwhelming bacterial infections occur more frequently in children in whom a splenectomy was performed, in children with a condition of autosplenectomy who have sickle cell disease, and in children who have the asplenia syndrome (Ivermark's syndrome: congenital absence of the spleen, cardiovascular anomalies, and other malformations).
R, Honigman, P, Lanzkowsky
openaire   +2 more sources

Isolated congenital asplenia? Screen the relatives

The Journal of Pediatrics, 2011
nuary 2011 Isolated congenital asplenia (ICA) (ie, in the absence of heterotaxy or cardiac anomalies) is thought to be rare and sporadic. Mahlaoui et al from France performed a national survey of French Pediatric Societies’ members and collected 10 index cases of ICA; the evaluation of relatives revealed an additional 10 cases.
openaire   +1 more source

[Isolated congenital asplenia--a rare cause of severe pneumococcal sepsis].

Harefuah, 2011
Invasive disease caused by Streptococcus pneumoniae (SPn) is common among young children. Severe sepsis can be seen among those with primary or secondary immunodeficiency states. The absence of a spleen is a well-known risk factor for severe and overwhelming pneumococcal sepsis, as well as other encapsulated bacteria (e.g. Haemophilus influenzae type b
Yael, Shachor-Meyouhas   +2 more
openaire   +1 more source

[Connecting isolated congenital asplenia to the ribosome].

Biologie aujourd'hui, 2015
Isolated congenital asplenia is characterized by the absence of a spleen at birth without any other developmental defect. Isolated congenital asplenia is a rare and life-threatening disease that predisposes patients to severe bacterial infections. The first and main genetic etiology of isolated congenital asplenia was discovered in 2013.
openaire   +1 more source

Congenital Asplenia Study: Clinical and Laboratory Characterisation of Adults With Congenital Asplenia

Annals of Hematology, 2022
Erica Wood   +2 more
exaly  

Congenital Asplenia Interrupts Immune Homeostasis and Leads to Excessive Systemic Inflammation in Zebrafish

Frontiers in Cellular and Infection Microbiology, 2021
Lang Xie, Zhe-Yu Chen
exaly  

A Rare Case of Isolated Congenital Asplenia Presenting in Septic Shock: Howell-Jolly Bodies a Clue to Early Diagnosis

Clinical Pediatrics, 2017
Pradeep, Bangalore Prakash   +3 more
openaire   +2 more sources

Isolated Congenital Asplenia: A Rare Case of Small Bowel Arteriovenous Malformation Associated Bleeding and Literature Review

American Journal of Gastroenterology, 2016
Jelena Arnautovic   +2 more
openaire   +1 more source

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