Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sickle crises frequently manifest as abdominal pain that may simulate intra-abdominal infection. To establish parameters to distinguish these, we retrospectively studied 53 patients with sickle-cell anemia who had abdominal pain (genotype SS 62%, SC 15%, SA 11%, S-other 11%; 30% men and 70% women; mean age 23). A vaso-occlusive crises was responsible for the pain in 57 per cent; 23 per cent had a surgical entity and 20 per cent had a nonsurgical genitourinary disorder. Of the surgical conditions, 9 of 12 patients (95%) had cholecystitis and 4 of 12 patients (33%) had acute appendicitis (one patient had both). Vaso-occlusive crises were diffuse in 15 of 30 patients (50%), compared with proven surgical conditions, and was more often associated with remote pain such as limbs and chest (23 of 30 [77%] P less than 0.005). The pain of vaso-occlusive crises simulated prior crises in 21 of 30 patients (70%) compared with 1 of 12 patients (8%) who had surgical abdominal pain (P less than 0.005). A precipitating event (especially upper respiratory infection) was found in 50 per cent of abdominal vaso-occlusive crises versus 0 per cent of surgical abdomens (P less than 0.010). The pain was relieved with hydration and oxygen in 97 per cent of sickle crises within 48 hours versus 0 per cent of surgical abdomens (P less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The presentation and management of the acute abdomen in the patient with sickle-cell anemia. 281 19

Nonimmune hydrops fetalis (NIHF) or generalized soft tissue edema and cavity effusions may be due to cardiovascular diseases, congenital infections, genitourinary malformations, thoracic masses, placental conditions, chromosomal abnormalities, and idiopathic. We report 32 cases of NIHF from among 429 neonates who underwent autopsies (incidence 7.45%). Sixteen cases (50%) had cardiovascular disease; all were due to low output cardiac failure; 7 had structural congenital heart disease. Three of the children with congenital heart disease also had chromosomal abnormalities: 2 had trisomy 18 and 1 had Noonan syndrome. Among myocardial conditions were five subjects with cardiomyopathies (1 of each of the following types): oncocytic, dilated, endocardial fibroelastosis, cardiac glycogenosis, and carnitine deficiency; 3 had myocarditis, and 1 had cardiac rhabdomyomas. Congenital infections were due to cytomegalovirus in 3 cases, bacteria in 2, and parvovirus in 1. The mechanism of NIHF in these cases might be a combination of decreased myocardial contractility due to myocarditis and fetal anemia. Genitourinary diseases were present in 5 newborns: Two had congenital nephrotic syndrome, 1 had VACTER association, 1 had prune-belly syndrome, and 1 had urogenital sinus malformation. Intrathoracic lesions were found in 2 babies (pulmonary sequestration and diaphragmatic hernia). One twin died of volume overload due to twin transfusion syndrome. Only 2 newborns were classified as idiopathic. Our study shows that cardiovascular diseases that lead to heart failure or impaired venous return are more common in the liveborn (50%), whereas congenital infections are more common in the stillborn with NIHF.
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PMID:Nonimmune hydrops fetalis in the liveborn: series of 32 autopsies. 1601 Apr 81