bluuebunny/crossref_metadata_2025_split huggingface dataset:
High Mycoplasma pneumoniae loads and persistent long-term Mycoplasma pneumoniae DNA in lower airway associated with severity of pediatric Mycoplasma pneumoniae pneumonia
Authors: Liu Jinrong , Zhao Fei , Lu Jie , Xu Hui , Liu Hui , Tang Xiaolei , Yang Haiming , Zhang Jianzhong , Zhao Shunying 
Published: December 2019

Abstract
Background
An increased number of refractory mycoplasma pneumoniae (MP) pneumonia (MPP) cases have been reported. However the duration of MP infection in lower airway and the course of anti-MP treatment remains unclear.

Methods
We retrospectively reviewed the medical records of 94 MPP children. Patients were classified into two groups. The long-term group (Group LT) was defined as bronchoalveolar lavage fluid (BALF) remained MP-positive by PCR after 30 days of the disease course. The non-long-term group (Group NLT) was defined as BALF became MP-negative by PCR within 30 days of disease and patients who only needed one bronchoscopy lavage therapy. MP loads, clinical outcomes were analyzed along with other clinical measurements.

Results
The average levels of inflammatory markers such as C reactive protein and lactate dehydrogenase in Group LT were significantly higher than those in Group NLT. Airway and lung damage in Group LT were more severe than Group NLT. 28 patients developed necrotizing pneumonia and 8 patients developed pulmonary embolism in Group LT. Mean maximum MP loads in BALF were 107.46 ± 0.93 and 104.86 ± 0.93 in Groups LT and NLT, respectively. There was persistent MP DNA in Group LT, even lasted for 120 days. One severe MPP patient in Group LT had MP-associated bloodstream infection. After 3 months of follow-up, chest imaging revealed incomplete absorption of pulmonary consolidation in 33 patients of Group LT [including 13 airway obliterans (AO) patients] and in 7 patients of Group NLT (including 2 AO patients).

Conclusion
MP loads of BALF were associated with the subsequent duration of MP DNA in lower airway. High MP loads and persistent long-term MP DNA in lower airway were associated with severity of pediatric MPP.
Novel gene mutation in von Hippel-Lindau disease – a report of two cases
Authors: Wang Jitian , Cao Wenjie , Wang Zhaoxia , Zhu Hong 
Published: December 2019

Abstract
Background
Von Hippel-Lindau (VHL) syndrome is a familial autosomal dominant hereditary neoplastic disease caused by mutations in the VHL gene. Approximately 503 kinds of VHL gene mutations have been reported. Different types of mutations manifest various clinical phenotypes, from benign to malignant tumours or coexisting cysts. Thus, a gene mutation test is essential in the diagnosis of VHL syndrome.

Case presentation
We reported two cases in which a novel mutation site in the c530-536delGACTGGA region in exon 3 of the VHL gene resulted in the development of VHL syndrome. According to the ACMG guidelines, this variation is pathogenic and consistent with autosomal dominant inheritance. This variation has not been reported anywhere in the databases or literature.

Conclusion
This report will add a new mutation site to VHL gene databases. The newly added gene mutation and its associated clinical phenotypes will help improve the accuracy of VHL diagnosis and benefit the community of VHL gene mutation carriers.
Monoclonal immunoglobulin mediates complement activation in monoclonal gammopathy associated-C3 glomerulonephritis
Authors: Li Lin-Lin , Li Zhi-Ying , Wang Su-Xia , Yu Xiao-Juan , Tan Ying , Wang Yu , Yu Feng , Zhao Ming-Hui 
Published: December 2019

AbstractBackgroundC3 glomerulonephritis (C3GN) is a rare disease caused by inherited or acquired complement alternative pathway (CAP) dysregulation, which could also be secondary to monoclonal gammopathy of undetermined significance (MGUS). Herein, we described a patient presenting with C3GN and monoclonal gammopathy, and the pathogenic association between the two diseases was further explored in vitro.Case presentationA 76-year-old Chinese man presented with low serum C3 level, haematuria and nephrotic syndrome, and experienced rapid worsening of renal function over a period of 10 months. His serum and urine immunofixation electrophoresis both revealed a monoclonal IgGλ. A bone marrow puncture showed plasma cell dyscrasias with the highest plasma cell count of 5.25%. Kidney biopsy showed the presence of C3 glomerulonephritis, with exclusive deposits of C3 visible on immunofluorescence, a membranoproliferative pattern on light microscopy and electron dense deposits in sub-epithelial, intramembranous, sub-endothelial and mesangial regions by electron microscopy. The patient was positive for C3 nephritic factor (C3NeF) activity and anti-CFH autoantibodies, and all became negative during disease remission. The anti-CFH autoantibodies purified from the patient’s plasma exchange fluids were proven to be a monoclonal IgGλ, and could inhibit CFH binding to C3b and accelerate the formation of C3 convertase indirectly by interfering with the formation-impeding activity of CFH. No deficiency of candidate genes, especially variants in CFH, was detected in our patient. Based on the pathological and laboratory findings, the diagnosis of monoclonal gammopathy of renal significance (MGRS)-associated C3GN was finally made.ConclusionsThis is the first demonstration that intact monoclonal immunoglobulin (IgGλ) could act as an anti-CFH antibody and lead to MGRS-associated C3GN by activating the CAP.
Malnutrition is common in children with cerebral palsy in Saudi Arabia – a cross-sectional clinical observational study
Authors: Almuneef Abdul Rahman , Almajwal Ali , Alam Iftikhar , Abulmeaty Mahmoud , Bader Bader Al , Badr Mohamed Farouq , Almuammar May , Razak Suhail 
Published: December 2019

AbstractBackgroundCerebral palsy (CP) is considered as the main cause of severe physical impairment and malnutrition in children. This cross-sectional study intended to survey the nutritional status of children cerebral palsy in Riyadh, Saudi Arabia.MethodsWe examined 74 children (age: 1–10 yrs) with CP, who attended Sultan Bin Abdulaziz Humanitarian City (SBAHC), Riyadh Saudi Arabia. Data on age, general demographics, nutritional status, and dietary intake were collected. A child was considered underweight, wasted, stunted or thin if the standard deviation scores for his/her weight for age, weight for height, height for age and body mass index for age were ≤ −2.0 standard deviation (SD) using WHO growth standards. Multivariable logistic regression identified the factors associated with nutritional indicators.ResultsMore than half (56.4%) of the children with cerebral palsy were malnourished as they had z-score below <−2 SD in at least one of the four indicators. Thinness (50%) was the most common form of malnutrition, followed by underweight, stunting, and wasting. Arm anthropometrics gave similar results on the percent number of malnourished children. Factors that were independently associated with malnutrition with an adjusted OR (aOR) were as follow: age ≤ 5 yrs. (aOR: 4.29); presence of cognitive impairment (aOR: 4.13); presence of anemia (aOR: 3.41) and inadequate energy intake (aOR: 4.86) (p, for all trends <0.05).ConclusionChildren with cerebral palsy of the current study have impaired growth and nutritional status as assessed by all four common nutritional status indicators. Further large-scale community-based studies for in-depth evaluation of nutritional status and growth patterns in children with CP are needed.
Effects of pre-pregnancy body mass index on pregnancy and perinatal outcomes in women with PCOS undergoing frozen embryo transfer
Authors: Lin Jiaying , Huang Jialyu , Wang Ningling , Kuang Yanping , Cai Renfei 
Published: December 2019

AbstractBackgroundPrevious studies have shown that among women with polycystic ovary syndrome who have difficulties conceiving, frozen-embryo transfer resulted in increased live birth rates and decreased ovarian hyperstimulation syndrome risk than did fresh-embryo transfer. In the present retrospective analysis, we sought to determine the effect of body mass index (BMI) on pregnancy and perinatal outcomes in women with PCOS undergoing FET.MethodsWomen with PCOS (n = 1556) undergoing FET were divided into groups based on weight, with those with normal weight having a BMI of 18.5–24.9 kg/m2,those who were overweight having a BMI of 25–29.9 kg/m2, and those who were obese having a BMI ≥30 kg/m2. Both pregnancy and perinatal outcomes were compared among these groups.ResultsThe normal-weight, overweight, or obese groups exhibited similar pregnancy outcomes, including clinical pregnancy rate, miscarriage rate, ongoing pregnancy rate and live birth rate. In singletons, birth characteristics regarding newborn gender, gestational age, birthweight and length at birth were comparable between the three groups. For adverse neonatal outcomes, the three groups showed no significant differences on the rates of low birthweight, very low birthweight, preterm birth, and very preterm birth after adjustment. In addition, the obstetric complications and the frequencies of live-birth defects were also comparable between the three groups except that overweight and obese women were more likely than women of normal weight to have delivered via cesarean section.ConclusionBMI did not affect the pregnancy or perinatal outcomes in women with PCOS undergoing FET.
Pregnancy outcomes among 31 patients with tetralogy of Fallot, a retrospective study
Authors: Wang Kana , Xin Junguo , Wang Xiaodong , Yu Haiyan , Liu Xinghui 
Published: December 2019

Abstract
Background
Tetralogy of Fallot (TOF) is a severe type of congenital heart disease (CHD) and it confers substantial risk to mother and fetus for pregnant women. However, the outcome of pregnancy in women with TOF has not been well studied.

Methods
Women with TOF who have been seen and/or delivered at our tertiary-care hospital between April 2008 and January 2018 were retrospective reviewed.

Results
A total of 31 pregnant women with TOF were identified during ten-year period. Among these patients, cardiac defects remained uncorrected in 12 women and were surgically repaired in 19 women. The frequency of miscarriages, premature birth, and the percentage of neonates of small for gestational age (SGA) were greater in the uncorrected group than the surgically repaired group (16.67% vs 0, 50% vs 5.26, 41.67% vs 10.53% respectively). The neonatal mortality and fetal mortality were not observed in the surgically repaired group, but were observed in the uncorrected group [3.23% (1/31) and 6.45(2/31) respectively]. Furthermore, the obstetric and cardiac complications in the two groups were stratified and analyzed.

Conclusions
Surgical correction of TOF is associated with improved maternal and perinatal outcome. However, pregnancy in women with uncorrected TOF was still seen and it was observed at a rate of 1.4/10,000 in our medical center during ten year period. The high degree of ventricular dilatation heart, high functional classifications, serious cardiac arrhythmias and pulmonary hypertension appeared to be associated with maternal and neonatal risks.
Quality of maternal and newborn healthcare services in two public hospitals of Bangladesh: identifying gaps and provisions for improvement
Authors: Biswas Taposh Kumar , Sujon Hasnat , Rahman M. Hafizur , Perry Henry B. , Chowdhury Mahbub Elahi 
Published: December 2019

Abstract
Background
Healthcare service delivery systems need to ensure standard quality of care (QoC) for achieving expected health outcomes. Although Bangladesh has a good healthcare service delivery system, there are major concerns about the quality of maternal and newborn health (MNH) care services, which is imperative for achievements in health. The study aimed to measure the QoC for different MNH services in two selected public health facilities of Bangladesh. This study also documented the specific areas of each care which needs intervention.

Methods
The study was conducted in two district-level public health facilities—a district hospital (DH) and a mother and child welfare centre (MCWC). A total of 228 cases of MNH services were observed by using contextualized checklist ‘Standards-based Management and Recognition (S-BMR)’ for 8 selected MNH care services. For scoring, performed activities were calculated as percentages of the total recommended activities and categorized as high (> 80%), moderate (50 to 80%), and low (< 50%).

Results
Overall QoC scores were moderate for each DH (54.8%), and MCWC (56.1%). In DH, the QoC score was high for blood transfusion (80.3%); moderate for maternal complications management (77.0%), caesarean section (CS) (65.6%), infection prevention (64.3%), sick newborn care (54.1%), and normal vaginal delivery (NVD) (52.6%); and low for antenatal care (ANC) (25.6%) and postnatal care (PNC) (19.0%). In MCWC, the QoC scores were high for infection prevention (83.0%); moderate for CS (76.5%) and NVD (59.8%); and low for ANC (36.9%) and PNC (24.5%).

Conclusions
In the study facilities, the QoC for MNH services is found to be unsatisfactory, particularly for ANC and PNC. Urgent initiative needs to be taken by introducing contextualized quality monitoring tools at health facilities, along with training of the care providers and introducing a quality monitoring system.
Predictive factors for the presence of invasive components in patients diagnosed with ductal carcinoma in situ based on preoperative biopsy
Authors: Lee Kwan Ho , Han Jeong Woo , Kim Eun Young , Yun Ji Sup , Park Yong Lai , Park Chan Heun 
Published: December 2019

Abstract
Background
In patients diagnosed with ductal carcinoma in situ (DCIS) with needle biopsy before surgery, invasive component (IC) is often found in the postoperative tissue, which results in altered post-surgical care. However, there are no clinically available factors to predict IC, and few MRI studies are available for the detection of IC in DCIS patients. The purpose of this study was to evaluate which risk factors can predict IC preoperatively.

Methods
Patients with a DCIS diagnosis based on preoperative biopsy, who underwent breast surgery Kangbuk Samsung Hospital between Jan 2005 and June 2018, were retrospectively evaluated. Clinico-pathological and breast MRI factors were compared between DCIS and DCIS with IC in postsurgical specimens.

Results
Of the 431 patients with a preoperative diagnosis of DCIS, 34 (7.9%) showed IC during the postoperative pathological investigations, and 217 (50.3%) underwent breast MRI. Among MRI-related factors, Mass-like enhancement on MRI was the sole but significant predictor of IC (HR = 0.26, C.I. = 0.07–0.93, p = 0.038), while nipple-areolar complex invasion, enhancement peak and pattern were not statistically significant. Nuclear grade was the only significant predictor of IC in the analysis of other clinico-pathological factors (HR = 2.39, C.I. = 1.05–5.42, p = 0.038 in univariate analysis, HR = 2.86, C.I. = 1.14–7.14, p = 0.025 in multivariate analysis).

Conclusions
Mass-like enhancement on MRI and high nuclear grade were associated with IC in patients with preoperative diagnosis of DCIS. Considering the high sensitivity of breast MRI for IC, further evaluation of the predictive value of MRI in preoperative DCIS patients is desirable.
Serum levels of the chemokine CCL2 are elevated in malignant pleural mesothelioma patients
Authors: Kishimoto Takumi , Fujimoto Nobukazu , Ebara Takeshi , Omori Toyonori , Oguri Tetsuya , Niimi Akio , Yokoyama Takako , Kato Munehiro , Usami Ikuji , Nishio Masayuki , Yoshikawa Kosho , Tokuyama Takeshi , Tamura Mouka , Yokoyama Yoshifumi , Tsuboi Ken , Matsuo Yoichi , Xu Jiegou , Takahashi Satoru , Abdelgied Mohamed , Alexander William T. , Alexander David B. , Tsuda Hiroyuki 
Published: December 2019

Abstract
Background
Malignant pleural mesothelioma (MPM) is a debilitating disease of the pleural cavity. It is primarily associated with previous inhalation of asbestos fibers. These fibers initiate an oxidant coupled inflammatory response. Repeated exposure to asbestos fibers results in a prolonged inflammatory response and cycles of tissue damage and repair. The inflammation-associated cycles of tissue damage and repair are intimately involved in the development of asbestos-associated cancers. Macrophages are a key component of asbestos-associated inflammation and play essential roles in the etiology of a variety of cancers. Macrophages are also a source of C-C motif chemokine ligand 2 (CCL2), and a variety of tumor-types express CCL2. High levels of CCL2 are present in the pleural effusions of mesothelioma patients, however, CCL2 has not been examined in the serum of mesothelioma patients.

Methods
The present study was carried out with 50 MPM patients and 356 subjects who were possibly exposed to asbestos but did not have disease symptoms and 41 healthy volunteers without a history of exposure to asbestos. The levels of CCL2 in the serum of the study participants was determined using ELISA.

Results
Levels of CCL2 were significantly elevated in the serum of patients with advanced MPM.

Conclusions
Our findings are consistent with the premise that the CCL2/CCR2 axis and myeloid-derived cells play an important role in MPM and disease progression. Therapies are being developed that target CCL2/CCR2 and tumor resident myeloid cells, and clinical trials are being pursued that use these therapies as part of the treatment regimen. The results of trials with patients with a similar serum CCL2 pattern as MPM patients will have important implications for the treatment of MPM.
Repeatability of ocular surface vessel density measurements with optical coherence tomography angiography
Authors: Cai Sijie , Zhao Fengping , Du Chixin 
Published: December 2019

Abstract
Background
To determine the repeatability of measurements of ocular surface vessel density in normal and diseased eyes using optical coherence tomography angiography (OCTA).

Methods
Ten normal eyes, 10 pinguecula eyes, and 10 pterygium eyes of 30 volunteers were subjected to OCTA (AngioVue Imaging System, Optovue, Inc.). For scanning, we used the corneal adapter module. Each eye was scanned three times in the nasal and temporal directions, separately. AngioVue software was used to generate the ocular surface vessel density. Ocular surface vessel density was defined as the proportion of vessel area with blood flow to the total measurement area (3 × 3 mm2). Intersession repeatability of the measurement was summarized as the coefficient of variation (CV), and intraclass correlation coefficients (ICC) were calculated by variance component models.

Results
The CVs were less than 5% in all subjects, and the ICCs exceeded 0.9; thus, all measurements showed good repeatability. The nasal vessels densities differed significantly between healthy eyes and eyes with pterygium (P < 0.05); however, there was no significant difference between healthy eyes and eyes with pinguecula (P = 0.466).

Conclusions
These results suggest that measurement of ocular surface vessel density by OCTA in normal eyes and eyes with pterygium and pinguecula is repeatable. This preliminary research describes a quantitative and visual method for assessing vessel density of the ocular surface with a high level of consistency.
Internal limiting membrane insertion technique combined with nerve growth factor injection for large macular hole
Authors: Zhang Luyi , Li Xiaoxia , Yang Xiaoli , Shen Yu , Wu Miaoqin 
Published: December 2019

Abstract
Background
The study was proposed to determine whether nerve growth factor (NGF) combined with an internal limiting membrane (ILM) insertion was effective in the large idiopathic full-thickness macular hole (iFTMH) therapy.

Methods
A subset of 18 eyes (July 2015–October 2017) diagnosed as the large iFTMH were enrolled in this study. The subjects were treated using ILM insertion technique alone (ILM group) or ILM combined with NGF injection (NGF group) and the follow-up period was 6 months. Macular hole closure rates, best-corrected visual acuity (BCVA, improvements using ETDRS), and optical coherence tomography (OCT) findings were analyzed at 1st, 3rd, and 6th months postoperatively.

Results
We found that macular holes in both groups fully closed. In comparison to ILM insertion group, the NGF group had better BCVA at the 3rd month (48.00 ± 2.392 vs 58.22 ± 2.957, 95% confidence interval (CI): 2.159 to 18.29). The mean external limiting membrane (ELM, 422.2 ± 96 vs 674.9 ± 103.6, 95% CI: − 47.26 to 552.8) and ellipsoid zone (EZ, 496.7 ± 101.6 vs 766.7 ± 111.8, 95% CI: − 50.29 to 590.4) defects were significantly smaller in the NGF group at the 6th month in the follow-up examination. Complete recovery of ELM and EZ was observed in the NGF group in one eye of a patient and two eyes of two patients, respectively. In comparison, one eye’s ELM and another eye’s EZ were completely recovered in the ILM insertion group.

Conclusion
Our results indicated that ILM insertion with NGF injection might be an effective technique for the initial surgical treatment of eyes with large MHs. The proposed approach yielded better recovery of the photoreceptor layers and consequently might have superior postoperative visual acuity.

Trial registration
chiCTR1900021711. Retrospectively registered 5 March 2019.
Analysis of factors related to recurrence of paediatric hepatoblastoma - a single Centre retrospective study
Authors: Yang Wei , Chen Yiwei , Huang Yijin , Wang Huanmin 
Published: December 2019

Abstract
Background
This study was performed to identify risk factors associated with recurrence of hepatoblastoma.

Methods
A retrospective study was conducted on 56 patients with hepatoblastoma from 2012 to 2015 in Beijing Children’s Hospital. Pretreatment extension stage (PRETEXT), serum alpha fetoprotein (AFP) value, change trend of tumors after treatment and some other clinical characteristics were collected and analyzed. The comparison of independent variables that were not distributed normally was performed with the log-rank test.

Results
Twenty-eight patients with tumour recurrence and 28 patients without recurrence were included in this study, and the median age at presentation was 46.5 (26, 71.5) months. There was a significant difference in the 3-year recurrence-free survival (RFS) probability between patients aged over 54 months and those younger than 54 months (p = 0.007). After neoadjuvant chemotherapy, the chance of recurrence in partial response (PR) patients was significantly lower than that in stable disease (SD) patients (p = 0.004). The 3-year RFS rate of patients with a reduction in AFP of more than 60% after neoadjuvant chemotherapy was significantly higher than that of patients with a reduction of less than 60% (p = 0.005). The postoperative follow-up revealed that patients whose postoperative AFP fell to normal levels within 6 months of the start of treatment had a 3-year RFS rate of 68.6%, which is higher than that of patients whose AFP fell below the normal range after 6 months (p = 0.0005). Finally, the multivariate analysis by Cox regression showed that AFP decreased by less than 60% and tumour size decreased by less than 50% after neoadjuvant chemotherapy were significant independent prognostic risk factors for the 3-year RFS rate. The other clinical features were not significantly associated with tumour recurrence in this study.

Conclusions
Through this study, we concluded that the prognosis of childhood HB is related to the age at presentation and the response of chemotherapy. The results of the multivariate analysis showed that AFP decreased by less than 60% and tumour size decreased by less than 50% after neoadjuvant chemotherapy were significant independent prognostic risk factors. These findings can be helpful to evaluate therapeutic effects and predict prognosis.