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Background The main purpose was to determine basic epidemiological data on CKD among hospitalized pediatric patients in China. Methods Data from pediatric inpatients with CKD hospitalized from June 1, 2013 to May 31, 2017 were extracted from the electronic records of HQMS database, which includes over 14 million inpatients. Codes from the 10th revision of the International Classification of Diseases (ICD-10) were used to search the database. Results A total of 524 primary diseases of CKD were included in this study. In all, there were 278 231 pediatric inpatients with CKD, which accounted for 1.95 % of the 14 250 594 pediatric inpatients registered in the HQMS database. The number of pediatric inpatients with CKD was 67 498 in 2013, 76 810 in 2014, 81 665 in 2015 and 82 649 in 2016, which accounted for 1.93 %, 1.93 %, 1.99 and 2.09 %, respectively, of the total population of pediatric inpatients. The etiology of CKD was secondary nephrosis in 37.95 % of cases, which ranked first and followed by CAKUT with a percentage of 24.61 %. Glomerular diseases and cystic kidney disease accounted for 21.18 and 5.07 %, respectively. Among all 278 231 patients, 6 581 (2.37 %) had a primary discharge diagnosis of CKD. The renal pathology findings of CKD showed that IgA accounted for 51.17 %. Conclusions This study provides a descriptive analysis of the hospitalized population of pediatric CKD patients. Our study provides important, fundamental data for policy making and legislation, registry implementation and the diagnosis, treatment and prevention of CKD in China.

Background No therapeutics have yet been proven effective for the treatment of severe illness caused by SARS-CoV-2. Methods We conducted a randomized, controlled, open-label trial involving hospitalized adult patients with confirmed SARS-CoV-2 infection, which causes the respiratory illness Covid-19, and an oxygen saturation (Sao2) of 94% or less while they were breathing ambient air or a ratio of the partial pressure of oxygen (Pao2) to the fraction of inspired oxygen (Fio2) of less than 300 mm Hg. Patients were randomly assigned in a 1:1 ratio to receive either lopinavir–ritonavir (400 mg and 100 mg, respectively) twice a day for 14 days, in addition to standard care, or standard care alone. The primary end point was the time to clinical improvement, defined as the time from randomization to either an improvement of two points on a seven-category ordinal scale or discharge from the hospital, whichever came first. Results A total of 199 patients with laboratory-confirmed SARS-CoV-2 infection underwent randomization; 99 were assigned to the lopinavir–ritonavir group, and 100 to the standard-care group. Treatment with lopinavir–ritonavir was not associated with a difference from standard care in the time to clinical improvement (hazard ratio for clinical improvement, 1.31; 95% confidence interval [CI], 0.95 to 1.80). Mortality at 28 days was similar in the lopinavir–ritonavir group and the standard-care group (19.2% vs. 25.0%; difference, −5.8 percentage points; 95% CI, −17.3 to 5.7). The percentages of patients with detectable viral RNA at various time points were similar. In a modified intention-to-treat analysis, lopinavir–ritonavir led to a median time to clinical improvement that was shorter by 1 day than that observed with standard care (hazard ratio, 1.39; 95% CI, 1.00 to 1.91). Gastrointestinal adverse events were more common in the lopinavir–ritonavir group, but serious adverse events were more common in the standard-care group. Lopinavir–ritonavir treatment was stopped early in 13 patients (13.8%) because of adverse events. Conclusions In hospitalized adult patients with severe Covid-19, no benefit was observed with lopinavir–ritonavir treatment beyond standard care. Future trials in patients with severe illness may help to confirm or exclude the possibility of a treatment benefit. (Funded by Major Projects of National Science and Technology on New Drug Creation and Development and others; Chinese Clinical Trial Register number, ChiCTR2000029308. opens in new tab.)

Background To investigate the repeatability of superficial retinal vessel density measurements in healthy eyes with long axial length (AL) using optical coherence tomography angiography (OCTA). Methods There were 60 eyes of 31 volunteers enrolled in this cross-sectional observational study. All subjects underwent OCTA, AL and refraction test. The enrolled eyes were divided into the long AL group (26 mm ≤ AL

Purpose To compare the anatomical outcomes of different extents of internal limiting membrane (ILM) peeling in idiopathic macular hole surgery. Methods Prospective, parallel‐group, randomized clinical trial. A total of 121 eyes of 121 patients with idiopathic macular hole underwent pars plana vitrectomy, and peeling of the ILM with a diameter of two disk diameters (DD) or 4DD based on randomization. The main outcome was the proportion of eyes with complete hole closure at 12 months. The second outcome was the hole closure grading stratified by macular hole closure index (MHCI) at each visit. Results At 12 months, there was no significant difference in anatomical outcomes with complete closure achieved in 52 (82.5%) of 63 eyes in the 2DD group and 53 (91.4%) of 58 eyes in the 4DD group (p = 0.15). For subjects with MHCI ≤0.5 (n = 24), complete closure rate was significantly lower in the 2DD group compared to the 4DD group (p = 0.012; 18.2% versus 75.9%, respectively). Average BCVA was lower in 2DD group than 4DD group (p = 0.014). By contrast, when MHCI was >0.5, the complete closure rate between the two groups showed no significant difference: 96.2% (50 patients) versus 95.6% (43 patients), respectively (p = 0.185). Conclusion In patients with idiopathic full‐thickness macular hole and MHCI ≤0.5, a larger ILM peel of 4DD tends to achieve better anatomical outcomes than a more limited 2DD peel.

Background There are many public health issues to resolve regarding rare diseases, including a lack of data from large-scale studies. The objective of this study was to explore fundamental data for a list of rare diseases in China, based on a hospitalization summary reports (HSRs) database. The Target Rare Diseases List (TRDL) 2017 was generated using an expert consensus method in which experts listed diseases according to research priorities. Using codes of the 10th revision of the International Statistical Classification of Diseases and Related Health Problems (ICD-10) and key search terms of rare diseases in English and Chinese, data were obtained from HSRs of 96 hospitals, covering a population of over 15 million in China from 2014 to 2015. We extracted and analyzed information on demographics, hospitalizations, and readmissions. Results A total 281 rare diseases were included in the TRDL 2017. Altogether, 106,746 hospitalizations for a rare disease were captured from 1 January 2014 to 31 December 2015, accounting for 0.69% of inpatients during the same period. The top 10 rare diseases with most cases on the TRDL 2017 were thalassemia, idiopathic pulmonary arterial hypertension, pulmonary Langerhans cell histiocytosis, moyamoya disease, motor neuron disease, idiopathic pulmonary fibrosis, systemic sclerosis, hepatolenticular degeneration, coarctation of the aorta, and transposition of the great arteries. Among the 24 cities in the database, the five cities with the most types of the rare disease were Beijing, Changsha, Guangzhou, Shanghai, and Chengdu, with 191, 162, 143, 141, and 133 types, respectively. The five cities with most cases of the 281 rare diseases were Beijing, Guangzhou, Shanghai, Nanning, and Chengdu. The age distribution of rare diseases was 52% for the age group 25–64 years, and 27% of cases in the age group of 0–14 years were among children. The 10 highest readmission rates ranged from 35 to 65%. Conclusions This study provided the TRDL 2017 and descriptive analysis of 281 rare diseases in a hospitalized population. Our study reveals important fundamental information that will be useful in national policy making and legislation; registry implementation; and diagnosis, treatment, and prevention of rare diseases in China. Electronic supplementary material The online version of this article (10.1186/s13023-019-1137-y) contains supplementary material, which is available to authorized users.

Background This study examined the association between interaction of retinoid-related orphan receptor alpha (RORA) (rs8042149) genotype x trauma exposure and post-traumatic stress disorder (PTSD) symptoms. Methods A total of 1196 Chinese adults who suffered from a deadly 2008 Wenchuan earthquake participated in this study. PTSD symptoms were measured with the PTSD Checklist for DSM-5 (PCL-5). A custom-by-design 2 × 48-Plex SNPscan™Kit were used to genotype the RORA rs8042149 SNP. Results Our results showed that the interaction of rs8042149 genotype x trauma exposure was associated with total PTSD symptoms in males. Moreover, the rs8042149 genotype x trauma exposure interaction was only associated with severity of the negative affect, anhedonia and dysphoric arousal symptoms in males. Limitations A moderate sample exposed to a specific event was assessed with a self-reported PTSD measure. Conclusions This study provides preliminary evidence supporting the functional role of RORA in PTSD, and adds to the knowledge for understanding the genetic underpinnings of PTSD. Moreover, this study carries implications for understanding the comorbidity of PTSD and the sex-specific expression of PTSD’s symptoms.

To investigate whether a new macular hole closure index (MHCI) could predict anatomic outcome of macular hole surgery. A vitrectomy with internal limiting membrane peeling, air-fluid exchange, and gas tamponade were performed on all patients. The postoperative anatomic status of the macular hole was defined by spectral-domain OCT. MHCI was calculated as (M+N)/BASE based on the preoperative OCT status. M and N were the curve lengths of the detached photoreceptor arms, and BASE was the length of the retinal pigment epithelial layer (RPE layer) detaching from the photoreceptors. Postoperative anatomical outcomes were divided into three grades: A (bridge-like closure), B (good closure), and C (poor closure or no closure). Correlation analysis was performed between anatomical outcomes and MHCI. Receiver operating characteristic (ROC) curves were derived for MHCI, indicating good model discrimination. ROC curves were also assessed by the area under the curve, and cut-offs were calculated. Other predictive parameters reported previously, which included the MH minimum, the MH height, the macular hole index (MHI), the diameter hole index (DHI), and the tractional hole index (THI) had been compared as well. MHCI correlated significantly with postoperative anatomical outcomes (r = 0.543, p = 0.000), but other predictive parameters did not. The areas under the curves indicated that MHCI could be used as an effective predictor of anatomical outcome. Cut-off values of 0.7 and 1.0 were obtained for MHCI from ROC curve analysis. MHCI demonstrated a better predictive effect than other parameters, both in the correlation analysis and ROC analysis. MHCI could be an easily measured and accurate predictive index for postoperative anatomical outcomes.

Stathmin (STMN1) has been demonstrated as a regulator of fear processing across species, which implicates that it may be important in the etiopathogenesis of fear-related psychiatric disorders such as posttraumatic stress disorder (PTSD). This study examined the association between STMN1 rs182455 genotype, a single nucleotide polymorphism (SNP) located within or close to the putative transcriptional control region of STMN1 gene, and PTSD symptoms. A total of 326 Chinese adults who suffered from a deadly 2008 Wenchuan earthquake and unexpectedly lost their children during the disaster participated in this study. PTSD symptoms were measured with the PTSD Checklist (PCL). The Sequenom iPlex chemistries and the MassARRAY system were used to genotype the STMN1 rs182455 SNP. Our results indicated that the STMN1rs182455 genotype was not associated with severity of total PTSD symptoms in either females or males; however, it could significantly predict severity of PTSD's reexperiencing symptoms in females. The findings provide preliminary evidence supporting the important role of STMN1 in the development of PTSD, and expand extant knowledge on the genetic underpinnings of PTSD and the sex-specific expression of PTSD's symptoms.

Importance A randomized clinical trial is needed to evaluate what is the best photodynamic therapy (PDT) protocol to use for acute central serous chorioretinopathy. Objective To compare the efficacy and safety of a 50% dose of verteporfin (a method of PDT) with the efficacy and safety of a 30% dose for acute central serous chorioretinopathy. Design, Setting, and Participants A multicenter, noninferiority, double-masked, randomized, controlled, clinical trial in which 131 patients (131 eyes) with acute central serous chorioretinopathy for less than 6 months were recruited with a follow-up of 12 months from university-based ophthalmology practices. Interventions Patients were randomly assigned to either a 50% dose of verteporfin (the 50%-dose PDT group) or a 30% dose (the 30%-dose PDT group). Main Outcomes and Measures The 2 primary outcome measures were the proportion of eyes with complete absorption of subretinal fluid and the proportion of eyes with complete disappearance of fluorescein leakage at 6 and 12 months. The secondary outcome measures included the subretinal fluid recurrent rate, the fluorescein leakage recurrent rate at 12 months, the mean best-corrected visual acuity, the retinal thickness of the foveal center, and the maximum retinal thickness at each scheduled visit. Results The noninferiority of the 30%-dose PDT compared with the 50%-dose PDT for the primary outcomes was not demonstrated. The optical coherence tomography–based improvement rate in the 30%-dose PDT group was less than that in the 50%-dose PDT group both at 6 months (73.8% vs 92.9%; α = 0.0125, P = .006) and at 12 months (75.4% vs 94.6%; α = 0.0125, P = .004). The fluorescein angiography–based improvement rate in the 30%-dose PDT group was less than that in the 50%-dose PDT group both at 6 months (68.9% vs 91.1%; α = 0.0125, P = .003) and at 12 months (68.9% vs 92.9%; α = 0.0125, P = .001). The subretinal fluid recurrence rate in the 30%-dose PDT group was greater than that in the 50%-dose PDT group (24.0% vs 5.7% at 12 months; P = .010, determined by use of the log-rank test). The fluorescein leakage recurrent rate in the 30%-dose PDT group was significantly higher than that in the 50%-dose PDT group (16.7% vs 3.8% at 12 months; P = .03, determined by use of the log-rank test). No ocular adverse event was encountered in the study. Conclusions and Relevance A 50% dose of verteporfin may be more effective at resolving subretinal fluid and fluorescein leakage, and with better visual outcomes, than a 30% dose for acute central serous chorioretinopathy. Trial Registration clinicaltrials.gov Identifier:NCT01574430