RESUMO
Introduction: Long-term data pertaining to childhood-onset lupus nephritis (cLN) remain extremely scarce. Methods: We conducted a retrospective cohort study of biopsy-proven cLN with onset age <18 years diagnosed from 2001 to 2020 to ascertain the long-term patient and kidney survival rates, and the incidence of advanced chronic kidney disease (CKD) (estimated glomerular filtration rate [eGFR] <60 ml/min per 1.73 m2). Results: A total of 92 subjects (78 female; age 13.7 ± 3.3 years; all Chinese) were included, with follow-up duration of 10.3 years (interquartile range, 5.8-15.9). Of these, 83 children (90%) had proliferative lupus nephritis (LN) (Class III/IV ± V). Mycophenolate was used for induction in 36%, whereas 34% received cyclophosphamide (CYC); 55% received mycophenolate as maintenance immunosuppression. The rates of complete remission (CR) and partial remission (PR) at 6 months and 12 months, respectively, were 65% and 20% and 78% and 8%. Two patients died (mortality rate 2.1/1000 patient-years), with a standardized mortality ratio of 22.3. Three patients (3.2%) developed end-stage kidney disease (ESKD), and advanced CKD occurred in 5 patients (5.4%). Survival rates without advanced CKD, ESKD, or death were 96.7%, 94.2%, 92.7%, 83.2% and 83.2% at 1 year, 5 years, 10 years, 15 years, and 20 years, respectively. Multivariate analysis revealed that severe kidney failure necessitating dialysis at presentation (adjusted hazard ratio 37.7, 95% confidence interval [CI] 4.0-355.6, P = 0.002), nonresponse (NR) after 12 months of treatment (adjusted hazard ratio 11.2, 95% CI 2.3-54.9, P = 0.003), and multiple nephritis flares (adjusted hazard ratio 2.6, 95% CI 1.1-6.2, P = 0.03) were predictive of advanced CKD, ESKD, or death. Other adverse outcomes included infections (2.9 episodes/100 patient-years), osteopenia (32%), hypertension (17%), short stature (14%), and avascular necrosis (7%). Conclusion: The long-term outcomes of cLN appeared to have improved in the present era with effective immunosuppression, cautious drug tapering, and assurance of medication adherence. There is still an unacceptably high prevalence of adverse outcomes.
RESUMO
INTRODUCTION: Pertussis, or whooping cough, is a highly contagious respiratory infection that is caused by the bacterium Bordetella pertussis. It is one of the most common causes of death in childhood. It is also a frequent cause of chronic cough in children, adolescents, and adults. METHODS: Global and Hong Kong perspectives of childhood pertussis were described. RESULTS: Hong Kong has prided herself in the city's childhood immunization program. There appear to be no major outbreaks of pertussis since the 1960s. Nevertheless, pediatricians may see isolated cases of pertussis or pertussis-like cases from time to time. Occasionally, infants are severely affected with apneas and managed with ventilator supports in the PICU. Outbreaks of the notifiable disease continue to occur despite a reasonable surveillance system and vaccination program in Hong Kong. Vaccination of mothers, adolescents, and adults are efficacious methods to further reduce the risks of pertussis. Macrolides remain efficacious antibiotics especially used early during the infectious phase. Infants with pertussis may require intensive care support and morbidity is high. CONCLUSIONS: Physicians should be reminded from time to time that outbreaks of pertussis still exist in Hong Kong and in many cities globally.
