Kimura disease in children: a case report and a summary of the literature in Chinese.

Kimura disease is a chronic inflammatory disease,which frequently affects middle-aged Asian men, although children are seldom affected by it. Therefore, the characteristics of the Kimura disease of childhood have not been well illustrated. In this report, we have described a case of Kimura disease and have summarized 29 childhood cases reported in Chinese people from 1988 to 2009 by using 3 Chinese journal search engines. Most of these cases were from provinces near the eastern and southern coast of China. Boys were predominantly affected. Head and neck are the most frequently involved sites of subcutaneous masses (24 of the 29, 82.8%). On laboratory investigations, peripheral blood eosinophilia (25 of 26, 96.2%) and elevated immunoglobulin E level (13 of 13, 100%) were common. Kimura disease was associated with high incidence of nephritic syndrome (8 of the 29, 27.6%) and eczema rash (13 of 29, 44.8%). Surgery excision, steroids, and chemotherapy were the main therapy methods in this cohort. For patients with follow-up data, 12 experienced recurrence, with a recurrence rate of 60.0%. Pediatricians need to be aware of this disease when dealing with patients with lymphadenopathy. A standard and effective treatment protocol would improve the outcome of the Kimura disease.

Sarcoidosis among US Hispanics in a Nationwide Registry.

BACKGROUND: Sarcoidosis has been well studied in multiple races and ethnic groups. However, there is a paucity of data that describes sarcoidosis in Hispanics. We aimed to determine the prevalence of Hispanic ethnicity, clinical characteristics and impact of sarcoidosis among Hispanics from a US based national registry. METHODS: We conducted a national registry-based study investigating 3835 respondents to the Sarcoidosis Advanced Registry for Cures questionnaire. This registry is a web-based, self-reported questionnaire that provides data related to demographics, diagnostics, organ involvement, treatment modalities, and the physical and psychosocial impact of sarcoidosis. We compared Hispanic patients to non-Hispanics. We performed multivariate logistic regression analysis adjusting for age, gender, education, income and insurance status and looked at the association between Hispanic ethnicity with depression, chronic pain syndrome, chronic fatigue syndrome, impact on family finances, employment-based disability and job termination. RESULTS: Nine percent of the patients reported a Hispanic ethnicity and the majority of these patients self-identified as white women. The most common organs involved were the lungs (74.9%), central lymph nodes (53.8%), and peripheral lymph nodes (37.1%). Hispanics reported more peripheral nerves and peripheral lymph nodes involvement than non-Hispanics. Hispanics experienced more depression, sleep apnea, and chronic pain syndrome than non-Hispanics. The use of mobility assistive devices was more common among Hispanics, as well as employment-based disability, and disease-related job termination compared to non-Hispanics. The majority of Hispanics reported significantly more pain that interfered with the enjoyment of life than non-Hispanics. On multivariate logistic regression analysis, Hispanic ethnicity was associated with depression (adjusted odds ratio (aOR) = 1.5; 95% CI: 1.01-2.2), chronic pain syndrome (aOR = 1.7; 1.1-2.6), job termination due to sarcoidosis (aOR = 1.7; 1.1-2.7) and higher impact on family finances (aOR = 1.7; 1.1-2.5). CONCLUSION: The clinical presentation of sarcoidosis in Hispanic patients differs from that in non-Hispanic patients living in the United States. These differences should be considered when managing Hispanic patients with sarcoidosis. We encourage more studies that investigate phenotyping among Hispanics with sarcoidosis.

Disparities in Cardiovascular Disease Risk Among Hispanic Breast Cancer Survivors in a Population-Based Cohort.

Background: Breast cancer is the leading cause of cancer death among Hispanic women. The aim of our study was to estimate cardiovascular disease (CVD) risk among Hispanic and non-Hispanic White (NHW) breast cancer survivors compared with their respective general population cohorts. Methods: Cohorts of 17 469 breast cancer survivors (1774 Hispanic and 15 695 NHW) in the Utah Cancer Registry diagnosed between 1997 and 2016, and 65 866 women (6209 Hispanic and 59 657 NHW) from the general population in the Utah Population Database were identified. Cox proportional hazards models were used to estimate hazard ratios (HRs) for CVD. Results: The risk of diseases of the circulatory system was higher in Hispanic than NHW breast cancer survivors 1-5 years after cancer diagnosis, in comparison with their respective general population cohorts (HRHispanic = 1.94, 99% confidence interval [CI] = 1.49 to 2.53; HNHW = 1.38, 99% CI = 1.33 to 1.43; 2-sided P heterogeneity = .01, respectively). Increased risks were observed for both Hispanic and NHW breast cancer survivors for diseases of the heart and the veins and lymphatics, compared with the general population cohorts. More than 5 years after cancer diagnosis, elevated risk of diseases of the veins and lymphatics persisted in both ethnicities. The CVD risk due to chemotherapy and hormone therapy was higher in Hispanic than NHW breast cancer survivors but did not differ for distant stage, higher baseline comorbidities, or baseline smoking. Conclusions: We observed a risk difference for diseases of the circulatory system between Hispanic and NHW breast cancer survivors compared with their respective general population cohorts but only within the first 5 years of cancer diagnosis.

The profile of lymphadenopathy in adults and children.

Surgical biopsies of lymphnodes of 315 patients (273 adult and 42 children) were reviewed microscopically and analysed. The sex ratio was similar in adults but in children males were twice than female (2:1). Ethnically Malay patients predominated in both the groups. The commonest cause of biopsied lymphadenopathy in adults were malignancy (47%) followed by chronic nonspecific lymphadenitis (20%) and granulomatous lymphadenitis (9%) while in children chronic nonspecific lymphadenitis was the commonest (46%) followed by granulomatous lymphadentitis (21%) and malignancy (14%). Twenty percent of adults and 4% of children had normal lymphnodes. Secondary deposits in adult males and females were of squamous cell carcinoma and adenocarcinoma type respectively. In our study 100% of clildren and 96% of adults with granulomatous lymphadenitis had tubercular lesions.

[Adult T-cell leukemia and non-malignant adenopathies associated with HTLV I virus. Apropos of 17 patients born in the Caribbean region and Africa]. / Leucémies à cellules T de l'adulte et adénopathies non malignes associées au virus HTLV I. A propos de 17 malades originaires des Caraïbes et d'Afrique.

Adult T-cell leukaemia is the first blood disease caused by a retrovirus: HTLV-1. The authors report the first French series of 15 patients, of whom 9 came from the classical endemic areas--the Antilles and outer Caribbean Islands--and 6 from Africa where the serological prevalence of HTLV-1 is high but few cases of adult T-cell leukaemia have been reported. Emphasis is laid on the importance of immunodeficiency (refractory strongyloidiasis, Pneumocystis carinii pneumonia, polyclonal B lymphoproliferative syndrome) and of other pathologies associated with the retrovirus (polyarthritis, lymphocytic interstitial pneumonia). The authors also describe the presence of adenopathy in healthy carriers: either adenitis suggestive of retroviral infection, or Castelman's disease adenopathy. These clinical presentations are similar to those described in lymphadenopathy syndromes due to the human immunodeficiency viruses. Aggressive lymphomas require chemotherapy, but sooner or later resistance develops, and the prognosis is very poor. The indications for allogeneic bone marrow transplantation are still to be determined. The diagnosis of adult T-cell leukaemia must be considered in all patients with blood disease coming from the endemic areas.

Pseudomediastinal fibrosis caused by massive lymphadenopathy in domestically acquired particulate lung disease.

In this report, we describe a case of domestically acquired particulate lung disease (DAPLD) or "hut lung" in a 59-year-old woman of Moroccan descent who emigrated to the Netherlands, having lived in an rural area for most her life. She presented with obstructive lung disease and with signs of mediastinal fibrosis which were shown to be caused by massive enlargement of mediastinal lymph nodes. To the best of our knowledge, this is the first case of DAPLD from Morocco and the first report of a case of DAPLD mimicking mediastinal fibrosis.

Acute-onset sarcoidosis with erythema nodosum and polyarthralgia (Löfgren's syndrome) in Japan: a case report and a review of the literature.

Löfgren's syndrome is an acute form of sarcoidosis that is characterized by erythema nodosum (EN), bilateral hilar lymphadenopathy (BHL), and polyarthralgia or polyarthritis. This syndrome is common among white people, but is considered rare among Japanese people. We present the case of a 26-year-old Japanese woman with Löfgren's syndrome. The patient complained of polyarthritis and EN of the lower extremities that lasted for 3 months. A chest radiograph revealed BHL and nodular shadows. The angiotensin-converting enzyme (ACE) level was within the normal range. Transbronchial lung biopsy revealed a noncaseating granuloma with giant cells. Six Japanese cases of Löfgren's syndrome have been reported previously. Five of the seven Japanese patients with Löfgren's syndrome had normal ACE levels; all of them exhibited BHL. Löfgren's syndrome should be considered as a possibility when examining a patient with EN and articular symptoms, even if the patient is Japanese.