Myasthenia gravis is an autoimmune disease that results from an antibody-mediated reaction and occurs with thymoma in 15+ACU- of patients. It is very rarely associated with autoimmune hepatitis. Four cases of myasthenia gravis with autoimmune hepatitis have been reported in the world. We recently experienced a case of 30-year-old man with myasthenia gravis associated with thymoma and autoimmune hepatitis. This condition is the first case that has not been reported previously in Korea. We report this rare condition along with a brief review of the literature.
Adult ; Case Report ; HLA-DR Antigens/genetics ; Hepatitis, Autoimmune/etiology+ACo- ; Human ; Male ; Myasthenia Gravis/complications+ACo- ; Thymoma/etiology+ACo- ; Thymus Neoplasms/etiology+ACo-

ACTH Syndrome, Ectopic ; etiology ; Adult ; Carcinoid Tumor ; pathology ; secretion ; Cushing Syndrome ; etiology ; Humans ; Male ; Pro-Opiomelanocortin ; genetics ; Reverse Transcriptase Polymerase Chain Reaction ; Thymus Neoplasms ; pathology ; secretion

We previously determined that AKR/J mice housed in a low-dose-rate (LDR) (137Cs, 0.7 mGy/h, 2.1 Gy) gamma-irradiation facility developed less spontaneous thymic lymphoma and survived longer than those receiving sham or high-dose-rate (HDR) (137Cs, 0.8 Gy/min, 4.5 Gy) radiation. Interestingly, histopathological analysis showed a mild lymphomagenesis in the thymus of LDR-irradiated mice. Therefore, in this study, we investigated whether LDR irradiation could trigger the expression of thymic genes involved in the DNA repair process of AKR/J mice. The enrichment analysis of Gene Ontology terms and Kyoto Encyclopedia of Genes and Genomes pathways showed immune response, nucleosome organization, and the peroxisome proliferator-activated receptors signaling pathway in LDR-irradiated mice. Our microarray analysis and quantitative polymerase chain reaction data demonstrated that mRNA levels of Lig4 and RRM2 were specifically elevated in AKR/J mice at 130 days after the start of LDR irradiation. Furthermore, transcriptional levels of H2AX and ATM, proteins known to recruit DNA repair factors, were also shown to be upregulated. These data suggest that LDR irradiation could trigger specific induction of DNA repair-associated genes in an attempt to repair damaged DNA during tumor progression, which in turn contributed to the decreased incidence of lymphoma and increased survival. Overall, we identified specific DNA repair genes in LDR-irradiated AKR/J mice.
Animals ; DNA Repair/*radiation effects ; Dose-Response Relationship, Radiation ; Female ; Gene Expression Regulation/*radiation effects ; Gene Regulatory Networks/radiation effects ; Lymphoma/etiology/*genetics ; Mice ; Mice, Inbred AKR ; Oligonucleotide Array Sequence Analysis ; *Radiation, Ionizing ; Reverse Transcriptase Polymerase Chain Reaction ; Thymus Gland/*radiation effects ; Thymus Neoplasms/etiology/*genetics