THU0274 An Inception Cohort of Chinese Patients with Antineutrophil Cytoplasimic Antibody-Associated Vasculitis in a Tertiary Centre in Hong Kong – a Study of Clinical Manifestations, Survival and Prognostic Factors

Abstract Objective To clarify the epidemiological and clinical features of Birt–Hogg–Dubé syndrome (BHDS) in Chinese patients. Methods We identified reports on Chinese patients with BHDS by searching the China Academic Journals Database, Wanfang Chinese Database, and PubMed databases, either in Chinese or English languages published from January 1, 2008 to December 31, 2020. Studies without sufficient clinical data were excluded and cases under 18 years old were excluded. Results Twenty papers were included and comprised 120 families with 221 cases. Most families with BHDS were reported from institutions in Beijing (66.7%) and Jiangsu Province (15.8%); 80.8% of cases were reported within the past five years. The average duration from clinical presentation to diagnosis was 9.6 years. The average age was 47.0 ± 13.9 years (range, 18–84 years) and the ratio of male to female was 1:1.6. The most common manifestations of BHDS were multiple pulmonary cysts (92.4%), spontaneous pneumothorax (71.0%), skin lesions (18.1%) and renal tumors (3.6%). Pulmonary cysts were predominantly distributed in the lower lobe on chest CT imaging. Family history of spontaneous pneumothorax was identified in 84.7% of the families and average number of pneumothoraxes was 1.8 (range, 1–6). The FLCN gene mutation c.1285dupC/delC in exon 11 was the most frequent mutation observed (17.4% of patients). The recurrence rate of pneumothorax after conservative treatment (including tube thoracostomy) was 29/41 (71%) while the pneumothorax recurred after surgical treatment (pulmonary bullectomy or pleurodesis) in only 4/37 (11%). Conclusions Although BHDS has been increasingly reported in the recent years, only minority of families were reported from institutions outside of Beijing and Jiangsu Province. The dominant clinical manifestations were pulmonary cysts associated with recurrent pneumothorax, while skin lesions and renal tumors were less commonly reported. Delayed diagnosis along with suboptimal management appear to represent critical challenges for Chinese patients with BHDS.

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A novel prognostic model for angioimmunoblastic T-cell lymphoma: A retrospective study of 55 cases

Journal of International Medical Research ◽

10.1177/03000605211013274 ◽

2021 ◽

Vol 49 (5) ◽

pp. 030006052110132

Author(s):

Jie Sun ◽

Sha He ◽

Hong Cen ◽

Da Zhou ◽

Zhe Li ◽

...

Keyword(s):

Prognostic Factors ◽

T Cell ◽

Prognostic Model ◽

Proportional Hazards Model ◽

Cell Lymphoma ◽

Initial Diagnosis ◽

Cox Proportional Hazards Model ◽

T Cell Lymphoma ◽

Chinese Patients ◽

Angioimmunoblastic T Cell Lymphoma

Objective To explore prognostic factors and develop an accurate prognostic prediction model for angioimmunoblastic T-cell lymphoma (AITL). Methods Clinical data from Chinese patients with newly diagnosed AITL were retrospectively analysed. Overall survival (OS) and progression-free survival (PFS) were estimated using Kaplan-Meier method survival curves; prognostic factors were determined using a Cox proportional hazards model. The sensitivity and specificity of the predicted survival rates were compared using area under the curve (AUC) of receiver operating characteristic (ROC) curves. Results The estimated 5-year OS and PFS of 55 eligible patients with AITL were 22% and 3%, respectively. Multivariate analysis showed that the presence of pneumonia, and serous cavity effusions at initial diagnosis were significant prognostic factors for OS. Based on AUC ROC values, our novel prognostic model was superior to IPI and PIT based models and suggested better diagnostic accuracy. Conclusions Our prognostic model based on pneumonia, and serous cavity effusions at initial diagnosis enabled a balanced classification of AITL patients into different risk groups.

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Pathobiology of A/Chicken/Hong Kong/220/97 (H5N1) Avian Influenza Virus in Seven Gallinaceous Species

Veterinary Pathology ◽

10.1354/vp.38-2-149 ◽

2001 ◽

Vol 38 (2) ◽

pp. 149-164 ◽

Cited By ~ 190

Author(s):

L. E. L. Perkins ◽

D. E. Swayne

Keyword(s):

Hong Kong ◽

Avian Influenza ◽

Pulmonary Edema ◽

Multiorgan Failure ◽

Clinical Manifestations ◽

Highly Pathogenic ◽

H5n1 Avian Influenza Virus ◽

Multiple Organs ◽

H5n1 Avian Influenza ◽

Virus Localization

Direct bird-to-human transmission, with the production of severe respiratory disease and human mortality, is unique to the Hong Kong-origin H5N1 highly pathogenic avian influenza (HPAI) virus, which was originally isolated from a disease outbreak in chickens. The pathobiology of the A/chicken/Hong Kong/ 220/97 (H5N1) (HK/220) HPAI virus was investigated in chickens, turkeys, Japanese and Bobwhite quail, guinea fowl, pheasants, and partridges, where it produced 75-100% mortality within 10 days. Depression, mucoid diarrhea, and neurologic dysfunction were common clinical manifestations of disease. Grossly, the most severe and consistent lesions included splenomegaly, pulmonary edema and congestion, and hemorrhages in enteric lymphoid areas, on serosal surfaces, and in skeletal muscle. Histologic lesions were observed in multiple organs and were characterized by exudation, hemorrhage, necrosis, inflammation, or a combination of these features. The lung, heart, brain, spleen, and adrenal glands were the most consistently affected, and viral antigen was most often detected by immunohistochemistry in the parenchyma of these organs. The pathogenesis of infection with the HK/220 HPAI virus in these species was twofold. Early mortality occurring at 1-2 days postinoculation (DPI) corresponded to severe pulmonary edema and congestion and virus localization within the vascular endothelium. Mortality occurring after 2 DPI was related to systemic biochemical imbalance, multiorgan failure, or a combination of these factors. The pathobiologic features were analogous to those experimentally induced with other HPAI viruses in domestic poultry.

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Clinical Features and Prognosis of Adult-onset Still’s Disease: 61 Cases from China

The Journal of Rheumatology ◽

10.3899/jrheum.080365 ◽

2009 ◽

Vol 36 (5) ◽

pp. 1026-1031 ◽

Cited By ~ 48

Author(s):

TING ZENG ◽

YU-QIONG ZOU ◽

MEI-FANG WU ◽

CHENG-DE YANG

Keyword(s):

Prognostic Factors ◽

Clinical Features ◽

Chinese Patients ◽

Adult Onset Still’S Disease ◽

Disease Course ◽

Adult Onset ◽

Still’S Disease ◽

Laboratory Findings ◽

Still's Disease ◽

Adult Onset Still's Disease

Objective.To describe the onset, clinical features, prognostic factors, and treatment of adult-onset Still’s disease (AOSD) in cases from China.Methods.Sixty-one Chinese patients with AOSD were analyzed retrospectively.Results.Common clinical features were fever (100.0%), rash (88.5%), and arthritis (82.0%). The laboratory findings were as follows: leukocytosis (83.6%), increased erythrocyte sedimentation rate (100.0%), elevated transaminase concentrations (23.0%), elevated ferritin levels (79.6%), negative antinuclear antibody (88.5%), and negative rheumatoid factor (88.5%). Of the 61 patients, 44.3% exhibited a monocyclic disease pattern, 29.5% experienced disease relapse at least once, 16.4% exhibited chronic articular course, and 9.8% died; most deaths were due to pulmonary infection and respiratory failure. Based on the disease course, we divided the 61 patients into 2 groups: those with favorable outcome (cyclic disease course, n = 45) and unfavorable outcome (chronic disease course or death, n = 16). We analyzed the prognostic factors for the 2 groups, and found that pleuritis, interstitial pneumonia, elevated ferritin levels, and failure of fever to subside after 3 days of prednisolone at 1 mg/kg/day were unfavorable prognostic factors for patients with AOSD.Conclusion.Patients with AOSD had complex symptoms with no specific laboratory findings. Our results indicate that AOSD is not a relatively benign disease, especially in cases that are refractory to high doses of prednisone.

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Basal Cell Carcinoma of the Head and Neck Region in Ethnic Chinese

International Journal of Surgical Oncology ◽

10.1155/2011/890908 ◽

2011 ◽

Vol 2011 ◽

pp. 1-7 ◽

Cited By ~ 1

Author(s):

Velda Ling Yu Chow ◽

Jimmy Yu Wai Chan ◽

Richie Chiu Lung Chan ◽

Joseph Hon Ping Chung ◽

William Ignace Wei

Keyword(s):

Neck Region ◽

Surgical Excision ◽

Chinese Patients ◽

Skin Closure ◽

Recurrence Rates ◽

Tumour Control ◽

Female Patients ◽

Ethnic Chinese ◽

Elderly Female ◽

Tertiary Centre

Objectives.This study aims to report our experience in the management of HNBCC in ethnic Chinese over a 10-year period.Methods.A retrospective review of all ethnic Chinese patients with HNBCC treated in a tertiary centre from 1999 to 2009.Results.From 1999 to 2009, 225 patients underwent surgical excision for HNBCC. Majority were elderly female patients. Commonest presentation was a pigmented (76.2%) ulcer (64.8%) over the nose (31.6%). Median skin margin taken on tumour excision was 2.0 mm; primary skin closure was achieved in 51.8%. Postresection skin margin was clear in 75.4%. Of those with inadequate skin margins, 56.7% opted for further treatment, 43.4% for observation. Recurrence rates were 2.6% and 13.8%, respectively (). Overall recurrence rate was 5.5%.Conclusions.HNBCC commonly presented as pigmented ulcers over the nose of elderly female patients in our locality. Adequate tumour excision ± reconstruction offered the best chance of cure. Reexcision of those with inadequate skin margins improved local tumour control.

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Acute mastoiditis and intracranial complications in children

Otorinolaryngologie a foniatrie ◽

10.48095/ccorl2021207 ◽

2021 ◽

Vol 70 (4) ◽

pp. 207-213

Author(s):

Andrea Kaliariková ◽

Klára Perceová ◽

Jan Machač ◽

Michal Jurajda ◽

Milan Urík

Keyword(s):

Streptococcus Pneumoniae ◽

Patient Group ◽

Clinical Manifestations ◽

Aetiological Agent ◽

Acute Mastoiditis ◽

Average Value ◽

Tertiary Centre ◽

Number Of Patients ◽

Intracranial Complications ◽

Low Incidence

Objectives: Characterisation of clinical manifestations in children who had acute mastoiditis (AM) -related intracranial complications (ICCs) and to determine the incidence of ICCs in the study group. To define children with a higher risk of ICCs and gain new information on aetiological microbial agents of AM. Methods: A retrospective analysis of 137 paediatric patients with AM treated at a tertiary centre using standard diagnostic and therapeutic protocol between 2002 and 2019. Results: Altogether 137 patients with AM were hospitalised at our centre between 2002 and 2019. During this time, the occurrence of ICC in children with AM was low (n = 3, incidence 2.19%). Due to the low number of patients with ICC with AM in our patient group, we were unable to define a specific group of patients with a higher risk of ICC development in AM. Despite this fact, the average value and median of CRP were considerably higher in patients with AM-associated ICCs. The most frequent aetiological agent was Streptococcus pneumoniae. Conclusions: The low incidence of ICC in patients with AM in our patient group might be caused by routinely performed paracentesis in developed acute otitis, rational antibiotic therapy and the good availability of an ENT specialist in the Czech Republic. The most common aetiological agent of AM was Streptococcus pneumoniae. Keywords: Streptococcus pneumoniae – acute mastoiditis – intracranial complications – aetiological agent – antibiotic therapy – antromastoidectomy

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Birt-Hogg-Dubé Syndrome in Chinese Patients: A Literature Review of 120 Families

10.21203/rs.3.rs-247951/v1 ◽

2021 ◽

Author(s):

Guofeng Zhang ◽

Xianmeng Chen ◽

Kaifeng Xu ◽

Xiaowen Hu

Keyword(s):

Spontaneous Pneumothorax ◽

Delayed Diagnosis ◽

Clinical Manifestations ◽

Lower Lobe ◽

Skin Lesions ◽

Jiangsu Province ◽

Chinese Patients ◽

Renal Tumors ◽

Pulmonary Cysts ◽

Exon 11

Abstract ObjectiveTo clarify the epidemiological and clinical features of Birt-Hogg-Dubé syndrome (BHDS) in Chinese patients. MethodsWe identified reports on Chinese patients with BHDS by searching the China Academic Journals Database, Wanfang Chinese Database, and PubMed databases, either in Chinese or English languages published from January 1, 2006 to December 31, 2020. Studies without sufficient clinical data were excluded and cases under 18 years old were excluded. ResultsTwenty papers were included and comprised 120 families with 221 cases. Most families with BHDS were reported from institutions in Beijing (66.7%) and Jiangsu Province (15.8%); 80.8% of cases were reported within the past five years. The average duration from clinical presentation to diagnosis was 9.6 years. The average age was 47.0±13.9 years (range, 18-84 years) and the ratio of male to female was 1:1.6. The most common manifestations of BHDS were multiple pulmonary cysts (92.4%), spontaneous pneumothorax (71.0%), skin lesions (18.1%) and renal tumors (3.6%). Pulmonary cysts were predominantly distributed in the lower lobe on chest CT imaging. Family history of spontaneous pneumothorax was identified in 84.7% of the families and average number of pneumothoraxes was 1.8 (range, 1-6). The FLCN gene mutation c.1285dupC/delC in exon 11 was the most frequent mutation observed (17.4% of patients). The recurrence rate of pneumothorax after conservative treatment (including tube thoracostomy) was 29/41 (71%) while the pneumothorax recurred after surgical treatment (pulmonary bullectomy or pleurodesis) in only 4/37 (11%).ConclusionsAlthough BHDS has been increasingly reported in the recent years, only minority of families were reported from institutions outside of Beijing and Jiangsu Province. The dominant clinical manifestations were pulmonary cysts associated with recurrent pneumothorax, while skin lesions and renal tumors were less commonly reported. Delayed diagnosis along with suboptimal management appear to represent critical challenges for Chinese patients with BHDS.

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Patient participation

Journal of Asian Pacific Communication ◽

10.1075/japc.20.2.06wei ◽

2010 ◽

Vol 20 (2) ◽

pp. 260-278 ◽

Cited By ~ 1

Author(s):

Wei Zhang

Keyword(s):

Hong Kong ◽

Pregnant Women ◽

Genetic Counselling ◽

Patient Participation ◽

Qualitative Difference ◽

Information Delivery ◽

The Self ◽

Chinese Patients ◽

Chinese Group

This study analyses patient questions in prenatal genetic counselling (PGC) in a Hong Kong hospital. The focus is on the kinds of questions asked by the patients and the sequential environments in which the questions are asked. The ten patients in the study are pregnant women at or above 38 years of age, four local Cantonese-speaking Chinese, and six of Filipina or Thai origin. The PGC is conducted by a nurse who communicates with the Chinese patients in Cantonese and with the non-Chinese patients in English. Two broad types of questions, medical and administrative, in line with the purpose of PGC, are found in both groups. While both groups share a concern on the accuracy of the test, the Chinese group asks more questions on medical details beyond the issue of accuracy. With regard to sequential environments, questions may be either ‘occasioned’ by the nurse’s prior talk or ‘self-motivated’; both may occur in the positions for minimal responses in extended information delivery sequences. Self-motivated questions also occur as a second or later component in the patient’s extended turn. While the two groups of patients ask questions from either of the sequential positions, a qualitative difference lies in the turn design of the self-motivated questions.

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A RETROSPECTIVE STUDY ON AMIODARONE PULMONARY TOXICITY IN CHINESE PATIENTS IN HONG KONG

Respirology ◽

10.1111/resp.13206_47 ◽

2017 ◽

Vol 22 ◽

pp. 23-23

Keyword(s):

Hong Kong ◽

Retrospective Study ◽

Pulmonary Toxicity ◽

Chinese Patients

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Shortened Relative Leukocyte Telomere Length is Associated with Prevalent and Incident Cardiovascular Complications in Type 2 Diabetes- Analysis from the Hong Kong Diabetes Register

10.2337/figshare.12498335 ◽

2020 ◽

Author(s):

Feifei Cheng ◽

Andrea O Luk ◽

Claudia HT Tam ◽

Baoqi Fan ◽

Hongjiang Wu ◽

...

Keyword(s):

Type 2 Diabetes ◽

Hong Kong ◽

Telomere Length ◽

Cox Regression ◽

Quantitative Polymerase Chain Reaction ◽

Hazard Ratio ◽

Chinese Patients ◽

Diabetes Register

Objective: Several studies support potential links between leukocyte relative telomere length (rLTL), a biomarker of biological aging and type 2 diabetes. This study investigates relationships between rLTL and subsequent cardiovascular disease (CVD) in patients with type 2 diabetes. Research design and methods: Consecutive Chinese patients with type 2 diabetes (N=5349) from the Hong Kong Diabetes Register with stored baseline DNA and available follow-up data were studied. rLTL was measured using quantitative polymerase chain reaction. CVD was diagnosed based on ICD-9 code. Results: Mean (SD) follow-up was 13.4(5.5) years. rLTL was correlated inversely with age, diabetes duration, blood pressure, HbA1c, urine ACR and positively with eGFR (all P<0.001). Subjects with versus without CVD at baseline had shorter rLTL (4.3±1.2 vs. 4.6±1.2, P<0.001). Of the 4541 CVD-free subjects at baseline, the 1140 who developed CVD during follow-up had shorter rLTL than those remaining CVD-free after adjusting for age, sex, smoking and albuminuria status (4.3±1.2 vs. 4.7±1.2, P<0.001). In Cox regression models, shorter rLTL was associated with higher risk of incident CVD (hazard ratio (95% CI) for each unit decrease: 1.252 (1.195-1.311), P<0.001), which remained significant after adjusting for age, sex, BMI, SBP, LDL-C, HbA1c, eGFR and ACR (hazard ratio (95% CI): 1.141 (1.084-1.200), P<0.001). Conclusions: rLTL is significantly shorter in type 2 diabetes patients with CVD, is associated with cardiometabolic risk factors, and is independently associated with incident CVD. Telomere length may be a useful biomarker for CVD risk in type 2 diabetes.

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