Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This case report describes a 72-year-old woman who developed an acute postmyocardial infarction ventricular septal defect (VSD) with consequent cardiogenic shock. Intra-aortic balloon pump (IABP) counter-pulsation was urgently initiated in the cardiac catheterisation laboratory, with neither clinical nor haemodynamic improvement, prompting immediate removal of the IABP and the insertion of an Impella 2.5 heart pump (AbioMed Inc; Danvers, Massachusetts, USA), a temporary ventricular assist device. Thereafter, the patient improved clinically and was admitted to the cardiovascular intensive care unit (ICU). While in the cardiovascular ICU, the patient developed worsening mechanical haemolysis of blood cells, stable but persistent cardiogenic shock and a transient ischaemic attack. A consensus decision was made to proceed with percutaneous repair of the VSD as she was deemed at high risk for surgical repair. She underwent successful percutaneous VSD repair on day 4 of hospitalisation, using a single 18 mm Amplatzer muscular VSD occluder (AGA Medical, Plymouth, Minnesota, USA) with trace residual flow across the occluder. Adequate systolic blood pressure and cardiac output was maintained postprocedure with the Impella 2.5 device. The patient, however, succumbed to multiorgan dysfunction occasioned by sepsis.
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PMID:Impella-assisted transcatheter closure of an acute postinfarction ventricular septal defect. 2703 Apr 53

BACKGROUND Chromosome 22q11.2 deletion syndrome (22q11.2 DS) currently includes DiGeorge syndrome, conotruncal anomaly face syndrome, and velocardiofacial syndrome. We present the case of a male infant with 22q11.2 DS exhibiting generalized skin rash and dermatopathic lymphadenitis. CASE REPORT The patient was born at 40 weeks of gestation with interruption of aortic arch, ventricular septal defect, and thymic defect. Fluorescence in situ hybridization method performed on buccal smears detected the deletion of 22q11.2. On day of life 33, diffuse erythema appeared on the entire body. A skin biopsy detected vacuolar interface dermatitis with superficial perivascular infiltration. Laboratory examinations revealed eosinophilia and hypocalcemia. Clinically, cutaneous inflammation was correlated with the abnormal immune response in 22q11.2 DS. On day of life 210, the patient died due to sepsis caused by Pseudomonas aeruginosa. An autopsy revealed lymph nodes swellings in the bilateral axillar and subclavicular areas and around the bilateral iliac arteries. Histology of the lymph nodes demonstrated sparse distribution of atrophic germinal centers surrounded by wide zones of proliferating spindle cells, as well as macrophages, Langerhans cells, and interdigitating dendritic cells. Fontana-Masson staining revealed abundant melanin particles in the macrophages. Accordingly, we diagnosed this case as dermatopathic lymphadenitis. Interestingly, CD123 and CD56 double-positive spindle cells also proliferated around the germinal center. CONCLUSIONS This case had an unusual histological feature of dermatopathic lymphadenitis. Considering the wide variety of unusual immune conditions in 22q11.2 DS, the lymph nodes in the systemic skin inflammation may exhibit an extraordinary histology of spindle cells proliferation.
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PMID:A Patient with 22q11.2 Deletion Syndrome Presenting with Systemic Skin Rash and Dermatopathic Lymphadenitis of Unusual Histology. 3284 11


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