Clinical significance of human neutrophil antigen-1 antibodies in children with neutropenia.

Primary autoimmune neutropenia in young children is characterized by chronic neutropenia and positivity for antibodies against human neutrophil antigens (HNAs). This study analyzed the clinical characteristics of 402 children with neutropenia to identify differences between those with and without HNA-1 antibodies (HNA1abs). HNAabs in sera were detected by granulocyte immunofluorescence testing using flow cytometry. Relative fluorescence intensity (RFI) values were used to divide patients into positive (PG, n = 302), borderline (BG, n = 34), and negative (NG, n = 66) groups. The antibodies reacted to HNA-1a alone (59%), HNA-1b alone (1%), and HNA-1a/1b (40%). The PG had a significantly lower absolute neutrophil count before definitive diagnosis and a 1.6- to 2-times greater risk of hospitalization during neutropenia than the other groups. The median duration of neutropenia was longest in the PG at 25 months, followed by 20 months in the BG and 14 months in the NG. This large-scale cohort characterizes clinically distinct groups using the RFI value for HNA1abs in young children with neutropenia. Detection of HNA1abs may aid in understanding the clinical characteristics of children with neutropenia.

Age-specific incidence of joint disease in paediatric patients with haemophilia: A single-centre real-world outcome based on consecutive US examination.

INTRODUCTION: Joint health is one of the most important factors contributing to a healthy life in patients with haemophilia. Recent study revealed that starting early prophylaxis was not enough to prevent joint disease in most paediatric patients with haemophilia. AIM: In this study, we aimed to determine the age-specific incidence of acute joint disease during childhood at single haemophilia treatment centre (HTC). METHOD: The joint health in 48 patients was evaluated based on consecutive US testing for 5 years at annual multidisciplinary comprehensive care. RESULTS: During the study period, 23 patients (47.9%) had no joint disease since the initial examination, whereas 13 patients (27.0%) showed development from negative to positive findings. The incidence of joint disease increased with age: 0% in preschool, 5.3% in elementary school, 14.3% in junior high school and 35% beyond high school age. Among the 13 patients who developed joint disease, two experienced acquired synovitis that resolved during the follow-up period. Statistical analysis revealed that the patients who routinely underwent follow-up by the HTC exhibited a significantly lower incidence of joint disease than did those followed up at other institutions (p < .001). CONCLUSION: These results indicated that close check-up, including routine joint examination using US as well as frequent assessment of pharmacokinetic profile at the HTC, might play an important role in avoiding joint disease among paediatric patients with haemophilia.

Correction: Modeling the natural history of fatty liver using lifestyle-related risk factors: Effects of body mass index (BMI) on the life-course of fatty liver.

[This corrects the article DOI: 10.1371/journal.pone.0223683.].

Modeling the natural history of fatty liver using lifestyle-related risk factors: Effects of body mass index (BMI) on the life-course of fatty liver.

BACKGROUND: Incident fatty liver increases the risk of non-alcoholic fatty liver disease (NAFLD), which may lead to end-stage liver diseases, and increase the risk of cardiovascular disease and diabetes. For its prevention, modeling the natural history of fatty liver is useful to demonstrate which lifestyle-related risk factors (e.g. body mass index and cholesterol) play the greatest role in the life-course of fatty liver. METHODS: Model predictors and their predictive algorithms were determined by prospective regression analyses using 5-year data from approximately 2000 Japanese men aged 20-69 years. The participants underwent health examinations and completed questionnaires on their lifestyle behaviors annually from 2012 to 2016. The life-course of fatty liver was simulated based on this participant data using Monte Carlo simulation methods. Sensitivity analyses were performed. The validity of the model was discussed. RESULTS: The body mass index (BMI) and low-density/high-density lipoprotein cholesterol (LDL-C/HDL-C) ratio significantly aided in predicting incident fatty liver. When the natural history of fatty liver was simulated using the data of participants aged 30-39 years, the prevalence increased from 20% to 32% at 40-59 years before decreasing to 24% at 70-79 years. When annual updates of BMI and LDL-C/HDL-C ratio decreased/increased by 1%, the peak prevalence of fatty liver (32%) changed by -8.0/10.7% and -1.6/1.4%, respectively. CONCLUSIONS: We modeled the natural history of fatty liver for adult Japanese men. The model includes BMI and LDL‒C/HDL‒C ratio, which played a significant role in predicting the presence of fatty liver. Specifically, annual changes in BMI of individuals more strongly affected the life‒course of fatty liver than those in the LDL-C/HDL-C ratio. Sustainable BMI control for individuals may be the most effective option for preventing fatty liver in a population.