Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0021051 (immunodeficiency)
71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Good's syndrome is thymoma accompanied by immunodeficiency. A 69-year-old woman presented with recurrent chest infections, hypogammaglobulinemia, and radiological features of a thymoma. Immunoglobulin replacement therapy was not tolerated prior to surgery. Postoperative recovery was uneventful, and a Masaoka stage II type AB thymoma was confirmed on histology. One-year follow-up revealed no recurrence of the thymoma but the patient remained hypogammaglobulinemic and developed collagenous colitis. She declined immunoglobulin replacement therapy but remains under follow-up. Awareness of Good's syndrome to avoid overwhelming infection is emphasized. The finding of thymoma should prompt the thoracic surgeon to test for immunodeficiency.
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PMID:Good's syndrome: Is thymectomy the solution? Case report and literature review. 2735 13

Good's syndrome is a rare condition of immunodeficiency that is characterized by thymoma and hypogammaglobulinemia. A 74-year-old Japanese woman underwent total thymothymectomy for type AB thymoma (2015 WHO classification). She developed recurrent infectious diseases caused by Escherichia coli (bacteremia), Streptococcus pneumoniae (pneumonia and bacteremia) and Pseudomonas aeruginosa (bacteremia) in the year after thymectomy. The serum levels of immunoglobulin were significantly low (IgG 157mg/dL), which suggested that her infectious diseases were associated with Good's syndrome. Although she began receiving intravenous immunoglobulin every four weeks, she died of pneumonia a week after the second administration of immunoglobulin. When physicians encounter patients with recurrent infection who have a medical history of thymoma, the detection of hypogammaglubulinemia can be a key clue to the diagnosis of Good's syndrome.
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PMID:Good's syndrome with clinical manifestation after thymectomy: A case report. 2997 69

A large solitary intrathoracic mass was identified in a patient with concurrent immunodeficiency. The patient was found to have a type AB thymoma and was diagnosed with Good's syndrome. A case report demonstrates the importance of timely surgical intervention for this rare syndrome.
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PMID:An intrathoracic mass behind severe immunodeficiency: a case report of Good's syndrome and large type AB thymoma. 3201 24