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Characteristics of Good's Syndrome in China: A Systematic Review.
Chin Med J (Engl). 2017 Jul 05; 130(13):1604-1609.CM

Abstract

BACKGROUND

Good's syndrome (GS) is a rare disease characterized by thymoma, hypogammaglobulinemia, low or absent B-cells, decreased T-cells, an inverted CD4+/CD8+ T-cell ratio and reduced T-cell mitogen proliferative responses. GS is difficult to diagnose preoperatively due to its rarity and lack of typical symptoms, the characteristics of Chinese GS patients are still lacking. This study aimed to systematically review all the clinical, laboratory, and immunologic findings of reported cases of Chinese patients with GS.

METHODS

We searched for case reports and articles up to January 2017 using PubMed, China National Knowledge Infrastructure, Wangfang database and China Science and Technology Journal Database with the following words in combinations as key words: "thymoma," "hypogammaglobulinemia," and "Good's syndrome." The text words and MeSH terms were entered depending on the databases characteristics. The reference lists from retrieved articles were also screened for additional applicable studies. The authors were restricted to Chinese. There was no language restriction.

RESULTS

Forty-seven patients were reported in 27 studies. We found that GS has a nationwide distribution and that most cases (83%) have been described on the mainland of China. The initial clinical presentation is varied, ranging from symptoms related to the thymoma to infections resulting from immunodeficiency. Type AB (50%) is the most common histologic type of thymomas in Chinese GS patients according to the World Health Organization classification of thymomas. With respect to infection, sinopulmonary infection (74%) is the most common type, followed by skin infection (10%) and intestinal tract infection (10%). Diarrhea was presented in 36% of patients, and autoimmune manifestations were presented in 36% of patients.

CONCLUSIONS

GS is a rare association of thymoma and immunodeficiency with a poor prognosis. Astute clinical acumen and increased awareness of the clinical and immunological profile of GS are needed to increase early diagnosis, that would benefit improved therapeutic effects.

Authors+Show Affiliations

Department of Gastroenterology, Peking University First Hospital, Beijing 100034, China.Department of Gastroenterology, Peking University First Hospital, Beijing 100034, China.Department of Gastroenterology, Peking University First Hospital, Beijing 100034, China.Department of Gastroenterology, Peking University First Hospital, Beijing 100034, China.Department of Gastroenterology, Peking University First Hospital, Beijing 100034, China.

Pub Type(s)

Journal Article
Review
Systematic Review

Language

eng

PubMed ID

28639577

Citation

Dong, Jin-Pei, et al. "Characteristics of Good's Syndrome in China: a Systematic Review." Chinese Medical Journal, vol. 130, no. 13, 2017, pp. 1604-1609.
Dong JP, Gao W, Teng GG, et al. Characteristics of Good's Syndrome in China: A Systematic Review. Chin Med J. 2017;130(13):1604-1609.
Dong, J. P., Gao, W., Teng, G. G., Tian, Y., & Wang, H. H. (2017). Characteristics of Good's Syndrome in China: A Systematic Review. Chinese Medical Journal, 130(13), 1604-1609. https://doi.org/10.4103/0366-6999.208234
Dong JP, et al. Characteristics of Good's Syndrome in China: a Systematic Review. Chin Med J. 2017 Jul 5;130(13):1604-1609. PubMed PMID: 28639577.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Characteristics of Good's Syndrome in China: A Systematic Review. AU - Dong,Jin-Pei, AU - Gao,Wen, AU - Teng,Gui-Gen, AU - Tian,Yu, AU - Wang,Hua-Hong, PY - 2017/6/23/entrez PY - 2017/6/24/pubmed PY - 2018/4/18/medline SP - 1604 EP - 1609 JF - Chinese medical journal JO - Chin. Med. J. VL - 130 IS - 13 N2 - BACKGROUND: Good's syndrome (GS) is a rare disease characterized by thymoma, hypogammaglobulinemia, low or absent B-cells, decreased T-cells, an inverted CD4+/CD8+ T-cell ratio and reduced T-cell mitogen proliferative responses. GS is difficult to diagnose preoperatively due to its rarity and lack of typical symptoms, the characteristics of Chinese GS patients are still lacking. This study aimed to systematically review all the clinical, laboratory, and immunologic findings of reported cases of Chinese patients with GS. METHODS: We searched for case reports and articles up to January 2017 using PubMed, China National Knowledge Infrastructure, Wangfang database and China Science and Technology Journal Database with the following words in combinations as key words: "thymoma," "hypogammaglobulinemia," and "Good's syndrome." The text words and MeSH terms were entered depending on the databases characteristics. The reference lists from retrieved articles were also screened for additional applicable studies. The authors were restricted to Chinese. There was no language restriction. RESULTS: Forty-seven patients were reported in 27 studies. We found that GS has a nationwide distribution and that most cases (83%) have been described on the mainland of China. The initial clinical presentation is varied, ranging from symptoms related to the thymoma to infections resulting from immunodeficiency. Type AB (50%) is the most common histologic type of thymomas in Chinese GS patients according to the World Health Organization classification of thymomas. With respect to infection, sinopulmonary infection (74%) is the most common type, followed by skin infection (10%) and intestinal tract infection (10%). Diarrhea was presented in 36% of patients, and autoimmune manifestations were presented in 36% of patients. CONCLUSIONS: GS is a rare association of thymoma and immunodeficiency with a poor prognosis. Astute clinical acumen and increased awareness of the clinical and immunological profile of GS are needed to increase early diagnosis, that would benefit improved therapeutic effects. SN - 2542-5641 UR - https://www.unboundmedicine.com/medline/citation/28639577/Characteristics_of_Good's_Syndrome_in_China:_A_Systematic_Review L2 - http://dx.doi.org/10.4103/0366-6999.208234 DB - PRIME DP - Unbound Medicine ER -