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1.
BMC Neurol ; 24(1): 139, 2024 Apr 25.
Article in English | MEDLINE | ID: mdl-38664714

ABSTRACT

BACKGROUND: Non-motor symptoms in myasthenia gravis (MG) are rarely confirmed. Although there are some small cohort studies, a large-systemic survey has not yet been performed. METHODS: We investigated the incidence and clinical characteristics of patients with MG who had taste disorders and alopecia using data of 1710 patients with MG enrolled in the Japan MG Registry 2021. RESULTS: Among them, 104 (6.1%) out of 1692 patients and 138 (8.2%) out of 1688 patients had histories of taste disorders and alopecia, respectively. Among the patients with MG, taste disorders were significantly more common in women, those with severe symptoms, refractory MG, or thymoma-associated MG, and were less common in those with ocular MG. The taste disorders often occurred after the onset of MG and often responded to MG treatments. Alopecia was more common in MG patients with a history of bulbar palsy and thymoma, and it often occurred before the onset of MG and sometimes responded to MG treatments. Multivariate logistic regression analysis revealed taste disturbance was associated with worst quantitative MG score and thymoma-associated MG; and alopecia was associated with thymoma-associated MG. CONCLUSION: Clinicians should be aware of the non-motor symptoms in MG, especially in patients with severe myasthenic symptoms and thymoma-associated MG.


Subject(s)
Alopecia , Myasthenia Gravis , Taste Disorders , Humans , Myasthenia Gravis/epidemiology , Myasthenia Gravis/complications , Myasthenia Gravis/diagnosis , Alopecia/epidemiology , Alopecia/diagnosis , Female , Male , Taste Disorders/epidemiology , Taste Disorders/etiology , Middle Aged , Adult , Aged , Japan/epidemiology , Registries , Thymoma/complications , Thymoma/epidemiology , Incidence
2.
Neurol Sci ; 43(4): 2775-2783, 2022 Apr.
Article in English | MEDLINE | ID: mdl-34677704

ABSTRACT

BACKGROUND: The incidence of myasthenia gravis (MG) is increasing, and its characteristics in elderly patients are believed to differ from those in younger patients. However, only a few studies have focused on elderly patients with MG. OBJECTIVE: To review the characteristics of MG in elderly patients and evaluate whether older age is an independent factor associated with achieving minimal manifestation status (MMS). METHODS: This retrospective cohort study included 367 patients (319 non-elderly and 48 elderly patients) with MG enrolled at Xiangya Hospital from September 1, 2016, to December 31, 2018. We collected demographic data and information regarding comorbidities, antibody status, Myasthenia Gravis Foundation of America classification, affected muscle groups, thymoma, and treatment. MMS was defined as the primary outcome. RESULTS: Comorbidities were more common in elderly than in younger patients with MG. Anti-acetylcholine receptor antibody was the dominant subtype, whereas anti-muscle-specific tyrosine kinase antibody was rare and detected only in non-elderly patients. Elderly patients were more likely than younger patients to have generalized MG, but the frequency of thymoma was lower (28.5% vs. 10.4%, p = 0.0078). MMS or better was achieved in 154 (48.3%) and 13 (27.1%) non-elderly and elderly patients, respectively. Older age did not appear to be an independent factor associated with MMS (hazard ratio = 0.625; 95% confidence interval, 0.345-1.131). CONCLUSIONS: Older age was not an independent factor for a worse prognosis in patients with MG. The treatment of elderly patients with MG should be individually tailored.


Subject(s)
Myasthenia Gravis , Thymoma , Thymus Neoplasms , Aged , Autoantibodies , Humans , Middle Aged , Myasthenia Gravis/complications , Retrospective Studies , Thymoma/complications , Thymoma/epidemiology
3.
J Assoc Physicians India ; 70(2): 11-12, 2022 Feb.
Article in English | MEDLINE | ID: mdl-35436830

ABSTRACT

BACKGROUND: The possibility of recurrence in COVID-19 is very rare and hence mostly underdiagnosed. In the face of pandemic, this can lead to circulation of the virus like a hidden iceberg. Better understanding about this topic can improve our knowledge of the COVID-19 pathogenesis and ways to control the transmission. CASE PRESENTATION: A 41 year old male with no known comorbidities was admitted five times during a period of 7 months each time after being detected RTPCR positive for SARS-CoV-2 and more symptomatic than previously. He had no contact with other COVID-19 patients and was asymptomatic in between admissions. Despite this, he did not develop antibodies against SARSCoV-2. Later on, he was diagnosed with thymoma on biopsy of the anterior mediastinal mass. Patient's condition deteriorated on last hospitalization and he died, despite the treatment. Here we present an interesting report on multiple times recurrent COVID-19 infection, probably a case of reactivation and different plausible explanations on the role of thymoma.;Conclusion: Acknowledging the potential of SARS-CoV-2 to cause recurrence is very important during the pandemic as a part of the long term transmission mitigation. The case report shows that previous infection does not guarantee complete immunity from COVID-19, especially in immuno-compromised patients. Hence, despite the status of prior infection, vulnerable individuals who recovered from COVID-19 should be under surveillance.


Subject(s)
COVID-19 , Thymoma , Thymus Neoplasms , Adult , Humans , Male , Pandemics , SARS-CoV-2 , Thymoma/complications , Thymoma/diagnosis , Thymoma/epidemiology , Thymus Neoplasms/complications , Thymus Neoplasms/diagnosis , Thymus Neoplasms/epidemiology
4.
Muscle Nerve ; 63(2): 209-216, 2021 02.
Article in English | MEDLINE | ID: mdl-33205437

ABSTRACT

INTRODUCTION: The Duke Myasthenia Gravis (MG) Clinic Registry is a disease-specific database containing physician-derived data from patients seen in the Duke MG Clinic since 1980. METHODS: Data from 1060 MG patients initially seen between 1980 and 2008 were reviewed. RESULTS: Fifty-four percent were male. Symptoms began after age 50 in 66% of males and 42% of females. Peak onset age in males was in their 60's; females had no predominant onset age. Onset age for both sexes increased from 1980 to 2008. Thymoma was present in 8.5%. Weakness was limited to ocular muscles for at least 2 y in 22% and became generalized later in 8.3% of these. Acetylcholine receptor antibodies were present in 78% overall, 82% with generalized MG and 52% with ocular MG (OMG). The distribution of MG disease class was similar in males and females, except that a greater proportion of women experienced myasthenic crisis and men were more likely to have OMG. DISCUSSION: Data in the Registry permit comprehensive and longitudinal analysis of a validated MG population. Analysis of Registry data shows that the frequency of AChR antibody negative MG, ocular MG, and thymoma are similar to other reports, but the onset age and proportion of males have progressively increased compared to studies published more than 20 y ago. These observations demonstrate the value of collecting comprehensive clinical information and comparing historic and contemporary populations. Other potential uses of Registry data include comparison of outcome measures in different disease subgroups and the response to specific treatments.


Subject(s)
Autoantibodies/immunology , Muscle Weakness/physiopathology , Myasthenia Gravis/epidemiology , Oculomotor Muscles/physiopathology , Receptors, Cholinergic/immunology , Thymoma/epidemiology , Thymus Neoplasms/epidemiology , Adult , Age of Onset , Aged , Cohort Studies , Disease Progression , Female , Humans , Male , Middle Aged , Myasthenia Gravis/classification , Myasthenia Gravis/immunology , Myasthenia Gravis/physiopathology , Registries , Sex Distribution , Young Adult
5.
Eur J Neurol ; 28(6): 2083-2091, 2021 06.
Article in English | MEDLINE | ID: mdl-33721382

ABSTRACT

BACKGROUND AND PURPOSE: Prognosis of myasthenia gravis (MG) in patients with thymoma is not well established. Moreover, it is not clear whether thymoma recurrence or unresectable lesions entail a worse prognosis of MG. METHODS: This multicenter study was based on data from a Spanish neurologist-driven MG registry. All patients were aged >18 years at onset and had anti-acetylcholine receptor antibodies. We compared the clinical data of thymomatous and nonthymomatous patients. Prognosis of patients with recurrent or nonresectable thymomas was assessed. RESULTS: We included 964 patients from 15 hospitals; 148 (15.4%) had thymoma-associated MG. Median follow-up time was 4.6 years. At onset, thymoma-associated MG patients were younger (52.0 vs. 60.4 years, p < 0.001), had more generalized symptoms (odds ratio [OR]: 3.02, 95% confidence interval [CI]: 1.95-4.68, p < 0.001) and more severe clinical forms according to the Myasthenia Gravis Foundation of America (MGFA) scale (OR: 1.6, 95% CI: 1.15-2.21, p = 0.005). Disease severity based on MGFA postintervention status (MGFA-PIS) was higher in thymomatous patients at 1 year, 5 years, and the end of follow-up. Treatment refractoriness and mortality were also higher (OR: 2.28, 95% CI: 1.43-3.63, p = 0.001; hazard ratio: 2.46, 95% CI: 1.47-4.14, p = 0.001). Myasthenic symptoms worsened in 13 of 27 patients with recurrences, but differences in long-term severity were not significant. Fifteen thymomatous patients had nonresectable thymomas with worse MGFA-PIS and higher mortality at the end of follow-up. CONCLUSIONS: Thymoma-associated MG patients had more severe myasthenic symptoms and worse prognosis. Thymoma recurrence was frequently associated with transient worsening of MG, but long-term prognosis did not differ from nonrecurrent thymoma. Patients with nonresectable thymoma tended to present severe forms of MG.


Subject(s)
Myasthenia Gravis , Thymoma , Thymus Neoplasms , Humans , Myasthenia Gravis/complications , Myasthenia Gravis/epidemiology , Neoplasm Recurrence, Local , Retrospective Studies , Thymectomy , Thymoma/complications , Thymoma/epidemiology , Thymus Neoplasms/complications , Thymus Neoplasms/epidemiology
6.
Acta Neurol Scand ; 143(1): 96-102, 2021 Jan.
Article in English | MEDLINE | ID: mdl-32762063

ABSTRACT

OBJECTIVES: To summarize the clinical features of thymomatous myasthenia gravis (T-MG), examine the association between MG and thymoma, and identify the related factors or predictors for long-term prognosis of T-MG. METHODS: A retrospective, observational study was conducted on 100 patients with T-MG and 96 patients with non-T-MG (NT-MG) between January 1, 2009 and December 31, 2019. The baseline characteristics were recorded for each patient. Logistic regression was used to measure the association between all clinical variables and T-MG prognosis. RESULTS: Between the T-MG and NT-MG groups, age at onset (45.66 ± 11.53 years vs 39.06 ± 14.39 years); age >40 years (72.0% vs. 40.6%); AChR-Ab positive rate (100.0% vs. 83.3%); Myasthenia Gravis Foundation of America (MGFA) classification at the worst condition (≥grade III, 61.0% vs. 33.0%); thyroid dysfunction (7.0% vs. 20.8%); and outcome (complete stable remission + pharmacologic remission + improvement, 74.0% vs. 93.7%) were statistically significant (P < .05). Presence of thymoma (OR = 0.196, 95%CI = 0.076-0.511, P = .001) was a risk factor for MG. Male sex, post-operative complications, higher grade of MGFA classification, and thymoma Masaoka-Koga pathological stage were risk predictors for long-term prognosis of T-MG (P < .1). Use of preoperative anticholinesterase drugs (OR = 5.504, 95%CI = 1.424-21.284, P = .013) was identified as an independent predictor for T-MG. CONCLUSION: T-MG is clinically different from NT-MG, and thymoma is considered a risk factor for MG. Preoperative anticholinesterase drug use is a protective factor for long-term prognosis of T-MG. A comprehensive understanding of the characteristics of T-MG will likely help improve its prognosis.


Subject(s)
Myasthenia Gravis/diagnosis , Myasthenia Gravis/epidemiology , Thymoma/diagnosis , Thymoma/epidemiology , Thymus Neoplasms/diagnosis , Thymus Neoplasms/epidemiology , Adult , Aged , Cholinesterase Inhibitors/therapeutic use , Female , Humans , Male , Middle Aged , Myasthenia Gravis/therapy , Retrospective Studies , Thymectomy/trends , Thymoma/therapy , Thymus Neoplasms/therapy , Time Factors
7.
Jpn J Clin Oncol ; 50(4): 434-439, 2020 Apr 07.
Article in English | MEDLINE | ID: mdl-32134448

ABSTRACT

BACKGROUND: Although thymic carcinoma is a rare epithelial neoplasm that tends to be aggressive and metastasize widely, its incidence in Japan remains unclear. This study was to examine the incidence and initial treatment of thymic carcinoma in the Japanese population using data from a hospital-based cancer registry. METHODS: Using data from the national database of hospital-based cancer registries, we examined the incidence and initial treatment of thymic carcinoma diagnosed and treated in designated and non-designated cancer care hospitals between 2009 and 2015. Based on Japanese population estimates, we calculated the incidence rate of thymic cancer in Japan. RESULTS: A total of 2587 thymic carcinoma cases were diagnosed between 1 January 2009 and 31 December 2015. These patients consisted of 1705 (66%) men and 882 (34%) women, with a median age of 65.5 years (range, 16-96 years). The number and proportion of thymic carcinoma to all registered cancer cases per year increased each year. The incidence rate was estimated to be 0.29/100000 during the observation period, with an annual onset incidence of 0.38/100000 in 2015. Almost half of all cases of thymic carcinoma were treated surgically, while the others were treated with non-surgical therapy consisting of chemotherapy with or without radiotherapy. CONCLUSIONS: We estimated the incidence rate of thymic carcinoma in Japan based on the designated cancer care hospital-based cancer registry. The half of all patients with thymic carcinoma was unfit for multimodality therapy, including thoracic surgery.


Subject(s)
Hospitals , Registries , Thymoma/epidemiology , Thymoma/therapy , Adolescent , Adult , Age Distribution , Aged , Aged, 80 and over , Female , Humans , Incidence , Japan/epidemiology , Male , Middle Aged , Thymoma/pathology , Time Factors , Young Adult
8.
Clin Exp Immunol ; 195(1): 132-138, 2019 01.
Article in English | MEDLINE | ID: mdl-30216434

ABSTRACT

Good's syndrome (thymoma and hypogammaglobulinaemia) is a rare secondary immunodeficiency disease, previously reported in the published literature as mainly individual cases or small case series. We use the national UK-Primary Immune Deficiency (UKPID) registry to identify a large cohort of patients in the UK with this PID to review its clinical course, natural history and prognosis. Clinical information, laboratory data, treatment and outcome were collated and analysed. Seventy-eight patients with a median age of 64 years, 59% of whom were female, were reviewed. Median age of presentation was 54 years. Absolute B cell numbers and serum immunoglobulins were very low in all patients and all received immunoglobulin replacement therapy. All patients had undergone thymectomy and nine (12%) had thymic carcinoma (four locally invasive and five had disseminated disease) requiring adjuvant radiotherapy and/or chemotherapy. CD4 T cells were significantly lower in these patients with malignant thymoma. Seventy-four (95%) presented with infections, 35 (45%) had bronchiectasis, seven (9%) chronic sinusitis, but only eight (10%) had serious invasive fungal or viral infections. Patients with AB-type thymomas were more likely to have bronchiectasis. Twenty (26%) suffered from autoimmune diseases (pure red cell aplasia, hypothyroidism, arthritis, myasthenia gravis, systemic lupus erythematosus, Sjögren's syndrome). There was no association between thymoma type and autoimmunity. Seven (9%) patients had died. Good's syndrome is associated with significant morbidity relating to infectious and autoimmune complications. Prospective studies are required to understand why some patients with thymoma develop persistent hypogammaglobulinaemia.


Subject(s)
Autoimmune Diseases/epidemiology , B-Lymphocytes/immunology , Immunologic Deficiency Syndromes/immunology , Infections/epidemiology , Thymoma/epidemiology , Agammaglobulinemia , Aged , Cohort Studies , Female , Humans , Immunologic Deficiency Syndromes/epidemiology , Immunologic Deficiency Syndromes/mortality , Male , Middle Aged , Prognosis , Prospective Studies , Registries , Severity of Illness Index , Survival Analysis , United Kingdom/epidemiology
9.
Muscle Nerve ; 60(2): 188-191, 2019 08.
Article in English | MEDLINE | ID: mdl-31050006

ABSTRACT

INTRODUCTION: Some myasthenia gravis (MG) patients are refractory to conventional treatments. METHODS: To describe the clinical features of refractory MG (RMG) and explore the association with human leukocyte antigen HLA-DRB1 alleles, a cohort study of 114 consecutive MG patients was performed. Patients were classified as RMG based on predefined criteria. RESULTS: Twenty-two patients were found to have RMG (19.3%). There were no differences between non-RMG and RMG patients with respect to sex, age of onset, abnormal 3-Hz repetitive nerve stimulation, anti-acetylcholine receptor antibody positivity, thymectomy, thymoma or thymic hyperplasia, and polyautoimmunity. HLA-DRB1*03 was more frequent in the non-RMG vs. control population (P = 3 × 10-6 ). The HLA-DRB1*13 allele was less frequent in non-RMG patients compared with controls (P = 0.002), and less frequent in the non-RMG group compared with the RMG group (P = 0.003). DISCUSSION: HLA-DRB1*03 was more common in non-RMG, and the HLA-DRB1*13 allele appeared to have a protective role, as reported previously in other autoimmune disorders. Muscle Nerve 60: 188-191, 2019.


Subject(s)
HLA-DRB1 Chains/genetics , Myasthenia Gravis/genetics , Adult , Age of Onset , Autoantibodies/immunology , Case-Control Studies , Cohort Studies , Female , Genetic Predisposition to Disease , Humans , Male , Middle Aged , Myasthenia Gravis/epidemiology , Myasthenia Gravis/immunology , Portugal/epidemiology , Protective Factors , Receptors, Cholinergic/immunology , Thymectomy/statistics & numerical data , Thymoma/epidemiology , Thymus Hyperplasia/epidemiology , Thymus Neoplasms/epidemiology , Young Adult
10.
Toxicol Pathol ; 47(7): 833-841, 2019 10.
Article in English | MEDLINE | ID: mdl-31394971

ABSTRACT

Thymomas from 277 Fischer 344/N (F344/N), 10 Sprague Dawley (HSD:Sprague Dawley SD) (SD), 129 Wistar Han [Crl:WI(Han)] (WH), and 4 Wistar Outbred (WO) rats were reviewed from long-term studies in the National Toxicology Program (NTP) database. The incidence of thymomas in F344/N rats was slightly higher in males than in females, while the incidences in SD and WH rats were higher in females than in males. Only male WO rats were used in NTP studies. Of the 277 thymomas in F344/N rats, 235 (84.8%) were benign and 42 (15.2%) malignant, 14 of which exhibited metastasis. Of the 10 thymomas in SD rats, 5 (50%) were benign and 5 (50%) were malignant, one of which exhibited metastasis. Of the 129 thymomas in WH rats, 126 (98%) were benign and 3 (2%) were malignant, 1 with metastasis. Of the 4 thymomas in WO rats, 3 (75%) were benign and 1 (25%) was malignant, with no metastases. Malignant thymomas in F344/N and WH rats showed a propensity to be the cause of death and to result in early mortality, whereas the benign thymomas were associated less often with decreased survival. No occurrences of this neoplasm were reported to be related to exposure to any test articles.


Subject(s)
Rodent Diseases/epidemiology , Thymoma/veterinary , Thymus Neoplasms/veterinary , Animals , Female , Incidence , Male , Rats , Rats, Inbred F344 , Rats, Sprague-Dawley , Rats, Wistar , Thymoma/epidemiology , Thymus Neoplasms/epidemiology
11.
Indian J Med Res ; 150(2): 153-160, 2019 08.
Article in English | MEDLINE | ID: mdl-31670270

ABSTRACT

Background & objectives: Thymomas are rare, but most common anterior mediastinal lesions. The histomorphologic spectrum of thymic epithelial tumours (TETs) in Indian population has not been explored in depth. This study was aimed to assess the histomorphology of TETs in the Indian patients and correlate clinical parameters with pathological features. Methods: It was a retrospective study conducted in a tertiary referral hospital in north India. All morphologically confirmed cases of TETs since 2009 were included. Clinical details and histology slides were reviewed using the Modified Masaoka-Koga staging system and WHO 2015 classification. Clinicopathological correlation and survival analysis were done. A comparative review from other published Indian studies was performed. Results: A total of 219 cases of TETs (138 resections and 81 biopsies) were identified. The most common histomorphologic type was B2, and the most frequent stage was I. Types A/AB were common in older age (P<0.01). Clinically, higher stage tumours were found mostly in men (P<0.01), and these were Type B thymomas (P<0.01). Myasthenia gravis was more common in women (P<0.02) and in lower stages (P<0.05). Survival analysis revealed significant association between recurrence and tumour stage. Although thymic carcinoma was diagnosed on biopsy, no resectable case was identified. Interpretation & conclusions: Our findings showed that the thymomas in Indian patients were most commonly Stage I tumours of B2 and AB histotypes. Resected thymic carcinomas were conspicuously absent in our study. More studies need to be done to establish the frequency and biology of TETs from India.


Subject(s)
Neoplasm Recurrence, Local/pathology , Neoplasms, Glandular and Epithelial/pathology , Thymoma/pathology , Thymus Neoplasms/pathology , Adult , Female , Humans , India/epidemiology , Male , Middle Aged , Neoplasm Recurrence, Local/epidemiology , Neoplasm Staging , Neoplasms, Glandular and Epithelial/epidemiology , Retrospective Studies , Thymoma/epidemiology , Thymus Neoplasms/epidemiology
12.
Asian Pac J Allergy Immunol ; 37(2): 109-115, 2019 Jun.
Article in English | MEDLINE | ID: mdl-29888926

ABSTRACT

BACKGROUND: Good syndrome (GS) is an adult-onset immunodeficiency characterized by coexisting thymoma and hypogammaglobulinemia. Clinical course after treatment with intravenous immunoglobulin (IVIg) has rarely been reported. OBJECTIVE: To investigate and report the clinical course and outcomes of GS patients after treatment with IVIg at Thailand's largest national tertiary referral hospital METHODS: This retrospective chart review included patients diagnosed with GS and treated with IVIg during the 1 January 2005 to 31 December 2015 study period. RESULTS: Nine GS patients with a median age at diagnosis of 53 years were included. Pneumonia and sepsis were the most common clinical manifestations. Six infectious organisms suggestive of cell-mediated immunity defect occurred in six patients, including cytomegalovirus (CMV), Mycobacterium tuberculosis, Mycobacterium abscessus, Herpes simplex virus (HSV), Pneumocystis jirovecii, and Aspergillus. Mean serum IgG level was 317 mg/dL. Eight patients had very low to undetectable B-cells. Five patients had either low CD4 number or impaired T-cell function, and one patient had both. All patients received IVIg replacement therapy monthly at a dose of 0.4 g/kg. The mean trough IgG level was 881 mg/dL. After treatment with IVIg replacement, seven patients had favorable clinical outcomes. However, two patients expired due to septicemia. CONCLUSION: Clinical outcomes of patients with GS are more dependent on the severity of infections and associated hematologic and autoimmune diseases than on the severity of thymoma itself. Therefore, early recognition and prompt IVIg replacement may change the natural course of this condition and may be successful in keeping the patient infections free.


Subject(s)
Agammaglobulinemia/diagnosis , Agammaglobulinemia/therapy , Immunization, Passive , Thymoma/diagnosis , Thymoma/therapy , Adult , Agammaglobulinemia/epidemiology , Age of Onset , Aged , Biopsy , Female , Humans , Immunoglobulins, Intravenous/therapeutic use , Lymph Nodes/pathology , Male , Middle Aged , Retrospective Studies , Tertiary Care Centers , Thailand/epidemiology , Thymoma/epidemiology , Thyroid Gland/pathology , Treatment Outcome
13.
Acta Neurol Scand ; 138(4): 338-343, 2018 Oct.
Article in English | MEDLINE | ID: mdl-29845611

ABSTRACT

OBJECTIVE: To provide an estimated prevalence and describe the clinical features of myasthenia gravis (MG) in Chile. METHOD: We carried out (i) a prevalence study of MG using the capture-recapture method and the hospital register of pyridostigmine prescription in South-East Santiago (ii) a nationwide survey of MG patients. RESULTS: Prevalence in adults in South-East Santiago was estimated to be 8.36/100 000 inhabitants (CI: 95%, 7.98-8.80). From the nationwide survey, 405 questionnaires were analysed, there was a female/male ratio of 2.2:1. The mean age of onset of symptoms was 38.7 years (range 1-89). The onset was ocular in 46.4%, oculobulbar in 11.6%, bulbar in 8.9%, limbs in 11.6% and generalized in 21.4%. Of the 13.3% of patients who had had a diagnosis of thymoma, only four of these patients were >60 years old at onset. Thymomas were commoner in patients living in mining counties. Patients ≥60 years old at onset of MG formed 19.5% of the sample, female/male ratio 0.97:1. Associated autoimmune diseases were reported in 14% of patients and in family members of 31.8% of patients. A total of 78 patients had to change work due to MG and 68 needed help in carrying out daily activities. CONCLUSIONS: This study reduces the gap in information about MG in South America. The prevalence of MG in Chile is within the range described worldwide. We did not see an increase in male frequency in the older age of onset group and thymoma was more frequent in the fifth and sixth decades.


Subject(s)
Myasthenia Gravis/diagnosis , Myasthenia Gravis/epidemiology , Population Surveillance , Surveys and Questionnaires , Adolescent , Adult , Aged , Aged, 80 and over , Child , Child, Preschool , Chile/epidemiology , Female , Humans , Infant , Male , Middle Aged , Population Surveillance/methods , Prevalence , Registries , Thymoma/diagnosis , Thymoma/epidemiology , Thymus Neoplasms/diagnosis , Thymus Neoplasms/epidemiology , Young Adult
14.
Future Oncol ; 14(19): 1943-1951, 2018 Aug.
Article in English | MEDLINE | ID: mdl-30081670

ABSTRACT

BACKGROUND: The risk of developing secondary cancers (SCs) among patients with malignant thymoma in the US has not been estimated in the more recent time period. Methods: We extracted demographic and treatment data from the SEER database to estimate the standardized incidence ratios (SIRs). Results: Of 1570 patients with thymoma 211 (13.4%) had SCs. The overall risk of developing SCs was higher among patients with thymoma (SIR: 1.54, 95% CI: 1.34-1.76). The SIRs for cancers of lung, esophagus, sigmoid colon, soft tissue and heart, kidney, NHL and leukemia was significantly higher as compared to the general US population. Conclusions: Patients with thymoma are at modestly elevated risk of developing SCs as compared to the general US population. Although the overall risk has not changed after 14 additional years of follow up, the distribution of SCs has significantly broadened, with increased diversity across type and anatomic location of SCs.


Subject(s)
Neoplasms, Second Primary/epidemiology , Risk Assessment , Thymoma/epidemiology , Aged , Female , Humans , Male , Middle Aged , Neoplasms, Second Primary/etiology , Neoplasms, Second Primary/pathology , Risk Factors , SEER Program , Thymoma/complications , Thymoma/pathology , United States/epidemiology
15.
Muscle Nerve ; 55(6): 819-827, 2017 06.
Article in English | MEDLINE | ID: mdl-27641227

ABSTRACT

INTRODUCTION: Multiethnic studies can provide etiological clues toward the genetic and environmental influence of a disease. The aim of this study was to determine prevalence and clinical features of myasthenia gravis (MG) in immigrants compared with native patients in 2 population-based cohorts. METHODS: This cross-sectional study included 843 MG patients (375 from Norway and 468 from the Netherlands). Ethnic background was defined by questionnaires. RESULTS: Among the participating MG patients, 163 of 843 (19.3%) were first or second generation immigrants, mainly from Europe, Asia, and South America. No marked prevalence differences were found between immigrants and native ethnic groups. MG with muscle specific kinase antibodies and MG with thymoma were more frequent in Asian MG immigrants compared with other ethnic groups (8% vs. 0-4%; P < 0.001 and 21% vs. 6-10%; P < 0.001), respectively. CONCLUSIONS: Our findings indicate that Asian immigrant MG patients carry genetic factors or environmental/lifestyle factors which contribute to their specific phenotype, even after migration. Muscle Nerve 55: 819-827, 2017.


Subject(s)
Emigrants and Immigrants/statistics & numerical data , Myasthenia Gravis/epidemiology , Adult , Age of Onset , Aged , Community Health Planning , Cross-Sectional Studies , Ethnicity , Europe/epidemiology , Female , Humans , Male , Middle Aged , Myasthenia Gravis/ethnology , Myasthenia Gravis/therapy , Prevalence , Receptor Protein-Tyrosine Kinases/immunology , Receptors, Cholinergic/immunology , Thymectomy , Thymoma/epidemiology , Thymoma/surgery , Young Adult
16.
Clin Immunol ; 171: 12-17, 2016 Oct.
Article in English | MEDLINE | ID: mdl-27497628

ABSTRACT

Good syndrome (GS) or thymoma-associated immunodeficiency, is a rare condition that has only been studied in retrospective case series. General consensus was that GS has a worse prognosis than other humoral immunodeficiencies. In this study, physicians of GS patients completed two questionnaires with a two year interval with data on 47 patients, 499 patient years in total. Results on epidemiology, disease characteristics, and outcome are presented. Mean age at diagnosis was 60years and median follow-up from onset of symptoms was 9years. There was a high frequency of respiratory tract infections due to encapsulated bacteria. Median survival was 14years. Survival was reduced compared to age-matched population controls (5-year survival: 82% versus 95%, p=0.008). In this cohort survival was not associated with gender (HR 0.9, 95% CI 0.3-3.0), autoimmune diseases (HR 2.9, 95% CI 0.8-10.1) or immunosuppressive use (HR 0.3, 95% CI: 0.1-1.2).


Subject(s)
Immunologic Deficiency Syndromes/epidemiology , Thymoma/epidemiology , Thymus Neoplasms/epidemiology , Aged , Aged, 80 and over , Autoimmune Diseases/diagnostic imaging , Autoimmune Diseases/epidemiology , Child , Female , Humans , Immunologic Deficiency Syndromes/diagnostic imaging , Infections/epidemiology , Male , Middle Aged , Prognosis , Surveys and Questionnaires , Thymoma/diagnostic imaging , Thymus Neoplasms/diagnostic imaging
18.
Muscle Nerve ; 49(1): 30-4, 2014 Jan.
Article in English | MEDLINE | ID: mdl-23625360

ABSTRACT

INTRODUCTION: Acetylcholine receptor (AChR) and striated muscle antibodies (StrAbs) are found frequently in myasthenia gravis (MG) patients with thymoma. In this study we aimed to determine the positive predictive value (PPV) and negative predictive value (NPV) of these antibodies for thymoma in patients with MG. METHODS: Antibody findings, thymic histology, and onset age were reviewed for 1141 patients with MG. PPV and NPV of these antibodies for thymoma were determined. RESULTS: The PPV of AChR binding antibodies plus StrAbs was highest (50.0%) with onset before the age of 40 years. The PPV of all antibodies was low (<9%) after age 40. Higher StrAb levels did not increase the PPV. The NPV of AChR binding antibodies was high (99.7%) for all ages. CONCLUSIONS: Patients without AChR binding antibody are not likely to have a thymoma. StrAbs and AChR binding antibodies are not diagnostic for thymoma, but in early-onset MG their presence should raise the clinical suspicion for thymoma.


Subject(s)
Antibodies/blood , Muscle, Striated/immunology , Myasthenia Gravis/epidemiology , Receptors, Cholinergic/immunology , Thymoma/epidemiology , Thymus Neoplasms/epidemiology , Adolescent , Adult , Age Factors , Age of Onset , Aged , Aged, 80 and over , Antibodies/immunology , Autoantibodies/blood , Biomarkers/blood , Child , Child, Preschool , Comorbidity , Female , Humans , Infant , Infant, Newborn , Male , Middle Aged , Retrospective Studies , Thymoma/diagnosis , Thymoma/immunology , Thymus Neoplasms/diagnosis , Thymus Neoplasms/immunology , Young Adult
19.
Surg Endosc ; 28(4): 1202-8, 2014 Apr.
Article in English | MEDLINE | ID: mdl-24232134

ABSTRACT

BACKGROUND: The accuracy of a three-dimensional robotic-assisted videothoracoscopic approach may favor a radical resection of thymomas. The aim of this study was to demonstrate the feasibility of the robotic approach by reporting 8 years experience in a single referral center of surgical treatment of thymomas. METHODS: We retrospectively analyzed all consecutive patients who underwent a thymectomy from April 2004 to April 2012. We analyzed the procedure time, morbidity, mortality, conversions, hospitalization, freedom from recurrence, time to progression, and overall survival. RESULTS: From 2004 until 2012, a total of 138 robotic procedures for mediastinal tumors were performed in our center, of which 37 patients with a mean age of 57.3 years underwent a thymectomy for a thymoma. Histological analysis revealed four type A thymomas (10.8 %), seven type AB thymomas (18.9 %), seven type B1 thymomas (18.9 %), fourteen type B2 thymomas (37.8 %), four type B3 thymomas (10.8 %), and one thymus carcinoma (2.7 %). The Masaoka­Koga stages were as follows: stage I in twenty patients (54 %), stage IIA in five patients (13.5 %), stage IIB in eight patients (21.6 %), stage III in three patients (8.1 %), and stage IVa in one patient (2.7 %). The mean overall procedure time was 149 min (range 88­353). No surgical mortality was reported, and there were no peri-operative complications. No conversions were needed for surgical complications. In three cases, a conversion to sternotomy was preferred by the surgeon because tumor invasion in greater vessels was suspected. Two patients (5.4 %) suffered from a myasthenic crisis postoperatively and required prolonged mechanical ventilation. One patient (2.7 %) underwent a procedure for a thoracic herniation 6 months following thymectomy. The median hospitalization was 3 days. The follow-up analysis showed an overall survival of 100 % and tumor recurrence in one patient (2.7 %). CONCLUSIONS: Robotic thymectomies are safe in patients with early-stage thymomas. Robotic surgery may also be feasible for some selected advanced thymomas.


Subject(s)
Imaging, Three-Dimensional , Robotics/methods , Thymectomy/methods , Thymoma/surgery , Thymus Neoplasms/surgery , Adult , Aged , Aged, 80 and over , Biopsy , Feasibility Studies , Female , Follow-Up Studies , Humans , Male , Middle Aged , Morbidity/trends , Netherlands/epidemiology , Retrospective Studies , Survival Rate/trends , Thymoma/diagnosis , Thymoma/epidemiology , Thymus Neoplasms/diagnosis , Thymus Neoplasms/epidemiology , Time Factors , Tomography, X-Ray Computed
20.
Allergy ; 68(7): 829-35, 2013 Jul.
Article in English | MEDLINE | ID: mdl-23742015

ABSTRACT

Several lines of evidence suggest that deficiency of eosinophils is not associated with any characteristic abnormality. Patients lacking eosinophils, in the setting of immunodeficiency or as a consequence of IgG-mediated eosinophil precursor destruction, do not display any distinguishing abnormalities related to eosinophil reduction. The observation that eosinophil-deficient mice do not display any distinctive syndrome or failure of their health is evidence that, under ordinary laboratory conditions, the eosinophil does not play a critical role in the well-being of mammals. Observations that monoclonal antibodies to interleukin-5 (IL-5) are well tolerated appear unsurprising in light of these findings. For example, patients with the hypereosinophilic syndrome have received mepolizumab, an anti-IL-5 monoclonal antibody, for as long as 6 years and have not developed any characteristic set of adverse events. Safety data for reslizumab, another anti-IL-5 monoclonal antibody, and benralizumab, a monoclonal antibody to the IL-5 receptor α-chain, are comparatively limited, especially for benralizumab, although reports of administration of these antibodies to humans suggest that they are well tolerated. Thus, data to the present suggest that reduction of eosinophils appears to have no characteristic ill effects on normal health, and monoclonal antibodies that deplete eosinophils have the potential to be widely employed in the treatment of eosinophil-associated diseases.


Subject(s)
Antibodies, Monoclonal, Humanized/therapeutic use , Eosinophils/immunology , Immunologic Deficiency Syndromes/blood , Immunologic Deficiency Syndromes/immunology , Thymoma/immunology , Thymus Neoplasms/immunology , Animals , Antibodies, Monoclonal, Humanized/adverse effects , Cohort Studies , Disease Models, Animal , Eosinophils/metabolism , Female , Humans , Immunologic Deficiency Syndromes/drug therapy , Incidence , Interleukin-5/adverse effects , Interleukin-5/immunology , Interleukin-5/therapeutic use , Leukocyte Count , Male , Mice , Prognosis , Risk Assessment , Thymoma/blood , Thymoma/epidemiology , Thymus Neoplasms/blood , Thymus Neoplasms/epidemiology
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