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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Fifty-nine children with congenital asplenia were reviewed for episodes of severe infection. Seven children had isolated asplenia and 52 had asplenia associated with complex congenital heart disease (asplenia syndrome). A control group of eusplenic children with comparable cardiac lesions were assembled and used for comparative statistical analysis. There were 16 instances of documented sepsis among 59 children (27%). In those less than six months of age, the invading organism was usually gram-negative (Escherichia coli or Klebsiella). In children six months of age or older, the infecting organism was usually a pneumococcus or H. influenzae. When those with asplenia syndrome were compared to the control population, the former group had a significantly greater incidence of sepsis. Children with asplenia syndrome who survived the first month of life were at greater risk of dying from sepsis than from their heart disease. It is recommended that prophylactic antibiotics be administered to children with congenital absence of the spleen, commencing at three months of age, to be continued indefinitely.
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PMID:Sepsis and congenital asplenia. 32 Feb 99

Complete and unselected data concerning the postoperative pathology of congenital heart disease are presented for the first time. This study was based on 2,365 autopsies performed at the Children's Hospital Medical Center, Boston, in the 9 years from 1966 through 1974. Of these, 586 autopsies (25 percent) revealed congenital heart disease--238 performed in medically treated patients (41 percent) and 348 in surgically treated patients (59 percent). Tetralogy of Fallot, including cases with pulmonary outflow tract atresia and other associated malformations, was the congenital heart disease most often encountered in the postoperative autopsy series (88 cases, 25 percent of that series). D-transposition of the great arteries, including cases with other associated anomalies, was second (54 cases, 15.5 percent). Early death (hospital mortality) accounted for 320 (92 percent) of the 348 surgical cases; late death occurred in 28 patients (8 percent). Causes of late postoperative death included arrhythmias, excessively small ventricular septal defect with tricuspid atresia, massive hemoptysis, rupture of the pulmonary artery, cyanotic spell, congestive heart failure and infection. Prophylactic penicillin is recommended for patients with the asplenia syndrome because of their probably enhanced vulnerability to fulmfulminating septicemia by encapsulated bacteria such as the pneumococcus. Completeness and lack of selection in reporting data are essential in the interests of perspective and comparability of findings.
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PMID:Postoperative pathology of congenital heart disease. 95 66

Congenital asplenia has been traditionally described in association with cardiopulmonary anomalies (Ivemark syndrome). A case of congenital asplenia with duplication of the hindgut and genitourinary tract, myelomeningocele, situs inversus abdominis, and imperforate anus generated interest in review of the gastrointestinal anomalies of 36 patients who died of the disease in Childrens Hospital of Los Angeles. A significant number were found to have situs inversus, malrotation of the bowel, esophageal varices, duplication and hypoplasia of the stomach, Hirschsprung's disease, imperforate anus, and duplication of the hindgut. These patients present with symptoms referable to their gastrointestinal anomalies and may not draw attention to the concomitant cardiopulmonary anomalies or to the absence of the spleen. It becomes important, therefore, to establish the presence of the spleen in infants suffering from various anomalies of the gastrointestinal tract because of the overwhelming septicemia that is known to complicate the clinical management of these patients.
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PMID:Congenital asplenia and anomalies of the gastrointestinal tract. 705 5

Eight children with congenital asplenia syndrome have been studied for their cardiac and immunologic status. All patients were greater than 2 years of age and had severe complex cyanotic heart disease. All eight patients had abnormalities of cardiac and/or visceral situs. All patients had evidence of pulmonary stenosis or atresia and a common atrium or large atrial septal defect. Five patients required palliative cardiac surgery. All patients were given prophylactic antibiotics; there were no documented episodes of sepsis. One patient had an isolated deficiency of IgM; two patients had an isolated deficiency of IgE. Seven of eight patients were immunized with a dodecavalent pneumococcal vaccine. Four of the seven patients failed to have a twofold or greater antibody response. Our findings suggest that prophylactic antibiotics may reduce the incidence of sepsis in the asplenia syndrome. Because the prognosis for these patients must be optimistic, we recommend early documentation of splenic function in children suspected of having the asplenia syndrome, prophylactic antibiotics, and parent education. Children immunized with bacterial vaccines should have their antibody responses monitored.
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PMID:Congenital asplenia: immunologic assessment and a clinical review of eight surviving patients. 725 76

Two families have been presented. In one, asplenia occurred with and without gastrointestinal malformations. In the other, cardiovascular malformations occurred in one member and Ivemark syndrome in the other. All three children with asplenia died in infancy, two with evidence of sepsis. Sudden death in infancy may be due to sepsis in a child with isolated asplenia syndrome and impaired resistance to infection. Both isolated asplenia and some cases of congenital heart disease without asplenia may be related to Ivemark syndrome. New infants born in families with a history of congenital cardiovascular malformations, visceral heterotaxy, or other malformations associated with Ivemark syndrome should be tested for asplenia, primarily by searching for Howell-Jolly bodies. Infans with asplenia should be protected with prophylactic antibiotics. Pneumococcal polysaccharide vaccine is indicated at the age of 2 years. It would appear worthwhile for pediatric surgical or pediatric cardiologic services to perform a screening program for Howell-Jolly bodies.
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PMID:Familial asplenia, other malformations, and sudden death. 736 May 56

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 (Hib), or Neisseria meningitidis). The most common cause of asplenia is surgical removal of the spleen (after trauma or as a treatment for various hematological conditions). Dysfunction of the spleen can also be seen in sickle cell disease, with high risk of invasive bacterial diseases. Primary congenital asplenia is rare. Congenital asplenia can be a part of Ivemark syndrome which is accompanied by other malformations. Isolated congenital asplenia is exceedingly rare. These patients can present with severe sepsis, meningitis or even sudden death. Overall, 31 cases have been reported, among them 17 were familial and the rest sporadic. The genetic bases for this rare life-threatening malformation are not known. The authors describe a one year old patient, presented with severe pneumococcal sepsis and multisystem organ failure. Isolated congenital asplenia was demonstrated. The child recovered and is being treated according to the recommendations for adults with asplenia.
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PMID:[Isolated congenital asplenia--a rare cause of severe pneumococcal sepsis]. 2134 24

This case report describes a diagnosis of right-sided isomerism and specifically right-sided bronchial isomerism in a patient who was being investigated for deteriorating bronchiectasis. Right-sided bronchial isomerism is a variation of the normal bronchial anatomy (situs solitus) consisting of a left lung that is identically configured at the bronchial and lobar level to the right. It is sometimes referred to as bilateral right lung and is usually associated with congenital asplenia and therefore impaired immunity with susceptibility to pneumococcal sepsis and cardiac abnormalities which may be severe and result in a high mortality in infancy. Ivemark syndrome (also known as right atrial isomerism) combines these associations with malrotation of the gut and a midline liver.2 Interestingly, left-sided isomerism is associated with polysplenia as well as midline liver, malrotation of the gut, partially anomalous pulmonary venous drainage and cardiac septal defects.3 To the best of our knowledge cases of right-sided isomerism are sufficiently rare in adulthood that there are only two other reports in the literature and only one of the patients had bronchial isomerism.4,5.
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PMID:Right-sided bronchial isomerism diagnosed in adulthood. 2269 94