Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disease caused by abnormal expression of glycosylphosphatidylinositol (GPI) on the cell membrane due to mutations in the phosphatidylinositol glycan class A(PIGA) gene. It is commonly characterized by intravascular hemolysis, repeated thrombosis, and bone marrow failure, as well as multiple systemic involvement symptoms such as renal dysfunction, pulmonary hypertension, swallowing difficulties, chest pain, abdominal pain, and erectile dysfunction. Due to the rarity of PNH and its strong heterogeneity in clinical manifestations, multidisciplinary collaboration is often required for diagnosis and treatment. Peking Union Medical College Hospital, relying on the rare disease diagnosis and treatment platform, has invited multidisciplinary clinical experts to form a unified opinion on the diagnosis and treatment of PNH, and formulated the Expert Consensus of Multidisciplinary Diagnosis and Treatment for Paroxysmal Nocturnal Hemoglobinuria (2024), with the hope of promoting the standardization of PNH diagnosis and treatment.

Objectives:To investigate the relationship between visceral adipose index and glomerular filtration rate in patients with type 2 diabetes mellitus.Methods:A total of 1 036 patients with type 2 diabetes mellitus who received treatment in The Second Affiliated Hospital of Xi'an Jiaotong University from May 2017 to May 2018 were included in this study. The visceral adipose index was detected using a bioresistance assay. These patients were divided into four groups using the quartile method: Visceral adipose index < 8.10 (q1 group, n = 246), 9.60 > visceral adipose index ≥ 8.10 (q2 group, n = 64), 11.10 > visceral adipose index ≥ 9.60 (q3 group, n = 423), visceral adipose index ≥ 11.10 (q4 group, n = 233). One-way analysis of variance was performed to compare the differences among groups. Partial correlation and multiple regression were used to analyze the correlation between body mass index, waist circumference, waist-to-height ratio, waist-to-hip ratio, body fat content, visceral adipose index, and urinary microalbumin and glomerular filtration rate. Results:With the increase in the visceral adipose index, the glomerular filtration rate gradually decreased. The glomerular filtration rate in the q1, q2, q3, q4 groups was (112.19 ± 31.74) mL·min -1·1.73 m -2, (106.14 ± 28.26) mL·min -1·1.73 m -2, (104.73 ± 23.63) mL·min -1·1.73 m -2, (103.40 ± 27.51) mL·min -1·1.73 m -2, respectively. In the female group, with the increase in visceral adipose index, the glomerular filtration rate decreased gradually. After controlling for age, sex, diabetes, and hypertension, the visceral adipose index was significantly correlated with the glomerular filtration rate ( r = -0.10, P < 0.001). Multiple regression analysis showed that visceral adipose index and waist-to-height ratio were closely related to glomerular filtration rate ( F = 6.00, P < 0.001). Conclusion:With the increase of visceral adipose index, body mass index, waist circumference, waist-to-height ratio, waist-to-hip ratio, body fat content, and urinary microalbumin increased gradually. When the visceral adipose index is greater than 9.60, the glomerular filtration rate is significantly decreased. Therefore, it is suggested to adopt various methods to evaluate obesity in clinical work, and visceral fat index should be paid more attention, especially when the visceral fat index is greater than 9.60.

Objective: To investigate the relationship between hypertriglyceridemic waist (HTW) phenotype and abnormal blood pressure in children and adolescents in Yinchuan City, Ningxia, and to provide scientific basis for early identification and prevention of hypertension. Methods: A cluster sampling method was adopted to select 1 566 children and adolescents in Yinchuan. All participants completed questionnaire survey, physical measurement and biochemical examination. Results: In general, after adjusting for age, smoking, drinking, and family history of hypertension, compared with the normal triglyceride normal waist (NWNT) phenotype, the enlarged waist (EW) and HTW phenotypes both increased the risk of abnormal blood pressure ( OR EW =2.62, 95% CI =1.87-3.67; OR HTW =3.97, 95% CI =2.30-6.86); the risk of abnormal blood pressure in boys with EW and HTW phenotypes was that of boys with NTNW phenotype, respectively 3.80 times (95% CI =2.35-6.15) and 3.32 times (95% CI =1.59-6.92) of girls EW and HTW phenotypes. The risk of abnormal blood pressure in girls with EW and HTW phenotypes was that of girls with NTNW phenotype, respectively 1.72 times (95% CI =1.06-2.82) and 4.62 times(95% CI =1.97-10.85). Conclusion The hypertriglyceridemic waist phenotypes among children and adolescents in Yinchuan is significantly correlated with increased risk of abnormal blood pressure. More attention should be paid to triglyceride level and waist circumference among children and adolescents.

Objective: To explore the relationship between the muscle mass index (MMI) and the aggregation of cardiovascular risk factors (CVRFs) in children and adolescents, and to provide a scientific basis for the prevention and treatment of cardiovascular diseases in children and adolescents. Methods: The current situation study design was adopted, and 1 622 children and adolescents aged 12-17 years old in Yinchuan City were selected by a cluster random sampling method. All subjects were subjected to questionnaire survey, physical examination, body composition determination and laboratory index testing. Results: When other variables were not adjusted, MMI was a risk factor for the aggregation of cardiovascular risk factors ( P <0.01). After adjusting for age, gender and BMI, MMI became a protective factor for CVRFs≥1 ( OR =0.74, 95% CI =0.62-0.89), compared with insufficient MMI, the risk of developing CVRFs≥1 with good MMI and sufficient MMI was 0.60(95% CI =0.46-0.79), 0.56(95% CI =0.37- 0.85 ) times. The risk of CVRFs≥2 was 0.54(95% CI =0.37-0.79), 0.51(95% CI =0.30-0.87) times, and similar results were found in boys ( P <0.05). Conclusion Under the same BMI level, muscle mass index is a protective factor for cardiovascular risk factor aggregation in children and adolescents. Physical exercise of children and adolescents should be emphasized to maintain the best muscle mass and weight.

Objective:To analyze the clinical and pathological characteristics, treatment and prognosis of renal changes in patients with Kimura disease and improve the clinicians′ understanding on renal manifestations of Kimura disease.Methods:The clinical data of Kimura disease patients with definite diagnosis and detailed data in Peking Union Medical College Hospital from January 1980 to August 2020 were retrospectively analyzed. The patients were divided into renal impairment group and non-renal impairment group according to whether the kidney was involved or not and the related clinical data between the two groups were compared. The patients presenting with nephrotic syndrome were followed up.Results:There were 60 patients with Kimura disease confirmed by pathological diagnosis with 48 males. The median age was 33(3, 62) years old, and the median duration was 36(12, 111) months. There were 18 cases complicated with renal injury in 49 patients with complete routine urine and renal function examination and the main manifestations of renal injury were proteinuria and/or microscopic hematuria. There was no significant difference at age, sex and absolute value of eosinophils between the two groups (all P>0.05). Compared with the renal inpairment group, patients in non-renal inpairment group had longer course of disease, higher levels of hypersensitive C-reactive protein and erythrocyte sedimentation rate, and lower median values of total eosinophils and total IgE, but there was no statistically significant difference (all P>0.05). Among the patients with renal involvement, 6 patients met the diagnostic criteria for nephrotic syndrome, and 5 of them completed renal biopsies. The renal pathological diagnosis was membranous nephropathy in 2 cases and minimal change disease in 3 cases, and no interstitial eosinophil infiltration was found in renal biopsy tissues. These patients had a good response to glucocorticoids and/or immunosuppressive therapy, and achieved complete remission of nephrotic syndrome; at the same time, lymphadenopathy caused by Kimura disease could be well controlled. Conclusions:Kimura disease can combine with various renal lesions, and the pathology of nephrotic syndrome can be membranous nephropathy or minimal change nephropathy. After energetic treatment of glucocorticoids and/or immunosuppressive therapy, nephrotic syndrome can be completely relieved, and lymphadenopathy can be well controlled. The relationship between Kimura disease and renal disease needs further study.

Objective: To elucidate age and sex specific characteristics in fat free mass (FFM) among middle and high school adolescents in Yinchuan City, China, and to provide a scientific basis for healthy development, chronic disease prevention and intervention in children and adolescents. Methods: Using a convenient cluster sampling method, 1 175 middle and high school adolescents, aged 10 to 20 years old, from Yinchuan, China, were selected to participate in a questionnaire survey and physical examination, which involved body composition by bioelectrical impedance analysis(BIA). Results: For adolescents aged 12 to 18 years, FFM and the fat free mass percentage (FFMP) of boys increased with age(from 39.78 to 52.63 kg; 77.51% to 83.80%, respectively), the age trends of the FFM and FFMP of body parts were in the similar pattern, and were significantly higher than those of girls(all P <0.05). In girls, the FFM reached a peak of 40.96 kg at 15 years old, and decreased slightly between the ages of 15 and 18 years, the age trends of the FFM of body parts were in the similar pattern, and the FFMP decreased with age (from 75.63% to 71.91 %). In addition, in girls, the age trends of the FFMP of left and right legs were the same as the general trend, while the FFMP of left and right arms increased with age. The FFMP of the trunk reached a minimum of 29.93% at 15 years old and increased from 15 to 18 years old(all P <0.05). Conclusion For middle and high school adolescents aged 12 to 18 years old in Yinchuan City, China, the distribution of FFM changed in accordance with age and gender differences, in accordance with the characteristics of the adolescents growth and development.

Objective: To analyze the interaction of fat mass (FM) and fat free mass (FFM) on the aggregation of cardiovascular disease risk factors (CVRFs) among children and adolescents in Yinchuan City, China, so as to provide scientific basis for the prevention of CVRFs and cardiovascular disease in children and adolescents. Methods: A total of 1 822 children and adolescents aged 12 to 18 years in Yinchuan City were randomly selected for questionnaire survey, physical examination, body composition assessment and laboratory tests, through a cluster sampling method from 2017 to 2020. Binary Logistic regression was used to analyze the relationship between FM, FFM and the aggregation of CVRFs, and their interaction was analyzed. Results: After adjusting for sex and age, the risk of having CVRFs aggregation ≥1 in high FM and low FFM group and high FM and high FFM group was 2.01(95% CI =1.46-2.77) and 3.64(95% CI =2.66-4.98) times higher than that in low FM and low FFM group, and the risk of having CVRFs aggregation ≥2 was 1.67(95% CI =1.06-2.63) and 4.20 (95% CI =2.76-6.38) times, respectively( P <0.05). There was a multiplicative interaction between FM and FFM, which increased the risk of CVRFs aggregration ≥1 and ≥2. The adjusted OR and 95% CI were 1.58(1.04-2.40) and 1.95(1.12-3.42), respectively( P <0.05). Conclusion The results indicated the additive and multiplicative interactions between high levels of fat mass and fat free mass on the aggregation of cardiovascular risk factors, which increased the risk of aggregation of cardiovascular risk factors.

Objective: To analyze the relationship between body mass index (BMI), waist circumference (WC), waist to height ratio (WHtR) and bone metabolism markers. Methods: By using the method of cluster sampling, a total of 1 084 adolescents aged 12 to 18 from one middle school in Yinchuan were investigated by questionnaire, physical examination and laboratory examination during sept. to Dec., 2018. Results: BMI, WC and WHtR were significantly negatively correlated with Ca (r=-0.10, -0.15, -0.15, P<0.05), and WC was negatively correlated with β-CTX(r=-0.06 P<0.05). After adjusting for age, sex and vitamin D supplementation, WC had significant effects on Ca and β-CTX(β=-0.33, -0.22, P<0.05). Logistic regression analysis showed that there was no significant relationship between different types of obesity and the level of OC(P>0.05). The level of serum β-CTX in obese students was 1.93 times higher than that in normal students, Ca serum levels of obese students was 0.31 times higher than that in normal students that in abdominal obese students (defined as WC) was 0.54 times higher than that in normal students. Conclusion BMI, WC and WHtR were correlated with OC, β-CTX and Ca in adolescents in Yinchuan city, and WC was more closely related to Ca and β-CTX, suggesting that obesity has a negative effect on bone absorption.

Severe and critically ill patients with coronavirus disease 2019 (COVID-19) were usually with underlying diseases, which led to the problems of complicated drug use, potential drug-drug interactions and medication errors in special patients. Based on ( 6), and -19: , we summarized the experience in the use of antiviral drugs, corticosteroids, vascular active drugs, antibacterial, probiotics, nutrition support schemes in severe and critically ill COVID-19 patients. It is also suggested to focus on medication management for evaluation of drug efficacy and duration of treatment, prevention and treatment of adverse drug reactions, identification of potential drug-drug interactions, individualized medication monitoring based on biosafety protection, and medication administration for special patients.
Adrenal Cortex Hormones ; adverse effects ; therapeutic use ; Anti-Bacterial Agents ; therapeutic use ; Antiviral Agents ; adverse effects ; therapeutic use ; Betacoronavirus ; isolation & purification ; Coronavirus Infections ; drug therapy ; Critical Illness ; Drug Therapy ; Humans ; Nutritional Support ; Pandemics ; Pneumonia, Viral ; drug therapy ; Probiotics ; administration & dosage

Objective: Attentional biases toward emotional scenes may represent vulnerability and maintenance factors in depression. Antidepressant therapy may improve cognitive function and reduce depression, and is considered as the mechanism of action of antidepressants. Therefore, we conducted an eye-tracking test to examine whether selective serotonin re-uptake inhibitor (SSRI) antidepressants can reduce negative attentional biases and elicit clinical responses in depression. Methods: Twenty first-episode depressive patients freely viewed three types of pictures that depicted different emotional scenes (i.e., positive-control, neutral-control, and negative-control) for 4,000 ms while their eye movements were monitored. The attentional bias to different emotional scenes was assessed before and after eight weeks of SSRI treatment using the eye-tracking method. The control group included a group of healthy individuals. Results: The results revealed that first-episode depressive patients oriented their gaze more frequently to negative images and less to happy images, compared to controls. Importantly, the attentional bias in depressive patients was regulated after eight weeks of SSRI treatment. Patients showed an increased tendency to fixate on positive images and a decreased tendency to focus on negative images. Conclusion This suggests that SSRI antidepressants decrease vulnerability to negative images, while having an effect on attention in respect to positive images.