bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinicopathological Study of Xeroderma Pigmentosa: A Series of Eight Cases
Authors: Roy Kakali , Raju Sahana P. , Dhar Subhra , Dhar Sandipan 
Published: March 2024

Abstract

Background:
Xeroderma pigmentosa (XP) is a rare inherited (autosomal recessive) disease, resulting from impairment in DNA repair that involves recognition and repair of ultraviolet radiation (UVR)-induced DNA damage in the nucleotide excision repair pathway. This results in increased photosensitivity, UVR-induced damage to skin and eye, increased susceptibility to cutaneous and ocular cancers, and progressive neurodegeneration in some patients.

Aims and Objective:
The objective of the study was to describe the clinicopathological spectrum of eight cases of XP.

Materials and Methods:
An ambispective case series was conducted in a pediatric tertiary care hospital in eastern India during a 10-year period from 2013 to 2022.

Results:
All the children of our cohort were born of consanguineous marriage. The mean age of presentation was 1.2 years (range: 7 months–3 years), whereas three children presented during their infancy. The male-to-female ratio was 5:3. The most common findings were cutaneous (100%), followed by ophthalmic (75%) and/or neurological symptoms (25%). Patients had normal skin at birth but soon developed extreme photosensitivity followed by abnormal skin pigmentation and subsequently progressive xerosis, atrophy, wrinkling, and poikiloderma over time. Six patients had varied degrees of ocular involvement, whereas three of them had severe manifestations including madarosis, tylosis, ectropion, lagophthalmos, phthisis bulbi, clouding and scarring of the cornea with complete or partial loss of vision, and ophthalmic malignancies. Fifty percent (n = 4) of cases had cutaneous and ocular premalignant (actinic keratosis) and malignant lesions including melanoma and nonmelanoma skin cancer such as squamous cell carcinoma (SCC) and basal cell carcinoma (BCC) in their early childhood. One patient had simultaneous occurrence of multiple malignancies together (SCC, BCC, and melanoma). Neurological abnormalities (subnormal intelligence) were found in two cases. There was no evidence of sensory neural hearing loss, microcephaly, neuroregression, or neurodeficits.

Conclusion:
Although XP is associated with increased mortality and morbidity, early diagnosis followed by persistent vigorous photoprotection and regular screening for early detection of malignancies along with psychological support can drastically improve patients’ quality of life and life expectancy. Besides genetic counseling, further research is required on formulating optimal management of XP, specifically the role and possibilities of gene therapy in XP.
Topical Therapies of Pyogenic Granuloma in Children: A Systematic Review of Case Reports, Case Series, and Cohort Studies
Authors: Preclaro Ivan Arni C. , Jamisola Lian C. , Yang Chin-Yi 
Published: March 2024

Abstract
Pyogenic granuloma (PG) is a benign vascular tumor removed surgically or by physical modalities, such as electrocautery and cryotherapy. Other treatment options may be warranted, especially in children, using nonsurgical approach. The study aims to determine the effectiveness and safety of topical treatments on PG in children in the clearance of lesions. Systematic literature search were done in February 2023 in the following database: PubMed, HERDIN, Cochrane Library, Hindawi, Directory of Open Access Journals, ScienceDirect, and Google Scholar. The search included studies involving PG treated with topical medications in the pediatric age group. Twenty-five studies (9 cohort studies, 6 case series, and 10 case reports) were included and utilized the following medications: timolol, propranolol, corticosteroids, common table salt, imiquimod, and ingenol mebutate. Among the medications, timolol was the most reported drug to provide partial to complete resolution in 62 out of 149 patients. All the reviewed topical medications provided partial to complete resolution with minimal adverse effects. Among them, topical timolol, propranolol, and table salt have demonstrated high response rates. Topical therapy in children with PG may be considered as a nonsurgical option. However, further clinical trials are warranted to determine its effectiveness.
A study on the outcome of preoperative pulmonary function tests on a patient undergoing rheumatic mitral valve surgery
Authors: Khurana Harneet S. , Kamath Sushmit , Ghosh Kakali , Biswas Arunava , Dasgupta Chaitali Sen 
Published: March 2024

Abstract

Background and Aims:
Evaluation of pulmonary function by spirometer in adult patients undergoing cardiac surgery is a simple test to assess pulmonary reserve that has important implications in operative morbidity. However, there is no established consensus regarding which patients should undergo preoperative pulmonary function tests (PFTs), including forced expiratory volume in the first second (FEV1) and forced vital capacity (FVC). The primary aim was to evaluate the outcome of preoperative PFTs on patients undergoing rheumatic mitral valve surgery.

Material and Methods:
One hundred patients undergoing rheumatic mitral valve surgery, meeting the inclusion criteria, were included in this prospective observational study. The pulmonary function of the patients was assessed using a spirometer before surgery. Preoperative pulmonary artery systolic pressure (PASP) and arterial blood gas (ABG) analyses were also performed. The correlation of PFTs with early postsurgical outcome, comprising mortality within 30 days of surgery and morbidity defined as the existence of at least one of the postoperative complications, such as low cardiac output state indicated by increased requirement of inotropes, prolonged ventilation (>24 hours), postoperative new-onset hemodynamically significant arrhythmias, renal dysfunction, and post-op infection, was assessed.

Results:
With the increasing New York Heart Association (NYHA) class of the patient, there was a decline of FEV1% and FVC%, which was statistically significant (P < 0.0001). There was a significant positive correlation of FVC% with preoperative saturation of peripheral oxygen (SpO2) and preoperative saturation of peripheral carbon dioxide (PaO2) and FEV1% with preoperative SpO2 and preoperative PaO2. Patients who had postoperative prolonged ventilation had lower values of FVC% and FEV1%, which was statistically significant (P < 0.001). The patients who expired had significantly lower values of FVC% and FEV1%.

Conclusions:
Preoperative lung function has an implication on intraoperative morbidity during cardiac surgery although a common consensus on its application is lacking. Preoperative spirometry can be one of the parameters for predicting postoperative morbidity and mortality in patients undergoing rheumatic mitral valve surgery. Spirometry might have a role to play in predicting patient outcomes in rheumatic mitral valve surgeries; however, larger well-powered studies are needed.
Antimicrobial peptides as new-generation antibiotics against Mycobacterium
Authors: Eslami Parisa , Khosravi Adnan 
Published: March 2024

Abstract
More than 10 million cases of tuberculosis (TB) in 2019 have made TB a global health problem. The increase in infection has caused an increase in the use of antibiotics for treatment, but with the spread of antibiotic use and the emergence of antibiotic resistance, the treatment has become difficult. This issue highlights the need to find a suitable alternative more than in the past. This matter emphasizes the urgency of discovering a viable substitute more than ever before. These obstacles underscore the necessity of developing innovative approaches that are not only cost-effective and efficient but also possess reduced levels of toxicity. Antimicrobial peptides (AMPs) possess a wide range of characteristics, which positions them as highly promising alternatives for a future era that follows the use of antibiotics. Here, we will focus on investigating AMPs from different sources, such as animal, nonanimal, and synthetic, with their inhibitory activity against TB as a therapeutic strategy. The aim of this study was to investigate different aspects of using AMPs to deal with Mycobacterium tuberculosis as a new strategy in its treatment. For this purpose, we studied more than 50 articles with the keywords peptide, AMP, and M. tuberculosis and investigated the importance of AMP against TB by searching valuable data sources such as PubMed, Scopus, Google Scholar, and Web of Science. In this study, the effect of antimicrobial peptides (AMPs) against mycobacteria was investigated. With the review done and considering all the advantages and challenges of AMPs, we concluded that this class of new antibiotics can be used as a long-term strategy to fight TB. As a result, we suggest research priorities to discover and optimize the challenges of these peptides as TB treatment and diagnostic methods in different countries.
Bacterial Contamination of Blood Products Stored at Room Temperature on Agitator in Lagos State, Nigeria
Authors: Davies-Folorunso Tesleemah Oluwakemi , Muhibi Musa Abidemi , Folorunso Jamiu Bello , Olaniyi Olufemi David , Ademosun Abdulwaheed Adewale , Abdulganiy Nuryn , Kosamat Yekeen Adebisi , Kanikwu Phoebe Nwamaka , Osikomaiya Bodunrin Ibitola , Olaniyan Mathew Folaranmi 
Published: March 2024

Abstract

BACKGROUND:
The relevance of some plasma-based medicinal products, especially platelet concentrates in modern medical care, is confirmed by their inclusion in the World Health Organization Essential Medicines. Therefore, this study characterized bacterial contamination in blood donor’s platelet concentrates in Lagos State.

METHODS:
A total number of 10 units of screened blood were collected and platelet concentrates were serially sampled following an aseptic procedure and stored for a period of 8 days (day 1 to day 8). The samples were incubated at 37°C on a daily basis for 7 days in brain–heart infusion growth medium and thioglycolate broth, respectively. The identities of the isolated organisms were determined by following standard microbiological techniques. The resistance pattern of isolates to selected antimicrobial agents was also determined by disc diffusion method.

RESULTS:
The results obtained showed that the overall prevalence of bacterial contamination in platelet concentrates stored at room temperature was 20% (2/10), with the growth on the 1st day of culture. The pattern of the bacterial isolates was Staphylococcus aureus – 10% (1/10) and Staphylococcus epidermidis – 10% (1/10) from 10 platelet concentrates with agitation for 8 days with no evidence of co-bacterial contamination. The resistance pattern of the bacterial isolates to different selected antibiotics ranged from 50% to 100%. There was no significant difference in P value obtained in the comparative analysis of the proportions of S. aureus and S. epidermidis in platelet concentrates (P = 1.000; P > 0.05). The findings of this study also confirmed the presence of multidrug-resistant Gram-positive bacteria in the blood component for transfusion studied in Lagos; the organisms isolated were S. aureus and S. epidermidis. The resistance pattern of the bacteria to the antibiotics ranged from 50% to 100%.

CONCLUSION:
This work revealed evidence of bacterial contamination in platelet concentrates prepared for transfusion with an overall bacterial contamination of 20% (S. aureus – 10%; S. epidermidis – 10%) with no evidence of co-bacterial contamination but with evidence of S. aureus and S. epidermidis multidrug resistance, hence the need for proper storage and screening of platelet concentrates for bacterial contamination before transfusion to avoid platelet aggregation that may lead to fatal complications in the recipient.
Xiao Tan San Jie Fang Hampers the Growth of Colon Cancer Stem Cells through the Wnt/β-catenin Signaling Pathway
Authors: Zhou Yu-Qi , Yuan Jun-Fei , Shen Hong-Shi , Wang Ya-Ping , Shi Han-Fei , Pan De-Jian , Ye Min 
Published: March 2024

Abstract

Objective:
The purpose of this research was to examine the potential anticancer properties of Xiao Tan San Jie Fang (XTSJF) and its potential mechanism of action against colorectal cancer.

Materials and Methods:
HCT116 cells were induced into HCT116 spheres in DMEM/F12 medium by treatment with epidermal growth factor + fibroblast growth factor + leukemia inhibitory factor + B27. The proliferation ability and stemness of HCT116 spheres was examined. Various concentrations of XTSJF were used to treat HCT116 spheres to observe the impact on proliferation, apoptosis, and expression of stem cell markers. Next, Wnt/β-catenin pathway-related factor proteins were detected.

Results:
The findings revealed that XTSJF suppressed cell growth and induced cell death in HCT116 cells in a dosage-dependent manner. Similarly, XTSJF promotes apoptosis, inhibits cell proliferation, prolongs survival, and maintains the expression of stem cells through the Wnt/-catenin/TCF4 axis. XTSJF also inhibits AKT activity and subsequently activates glycogen synthesis kinase-3β expression, inhibiting Wnt/beta-catenin pathway activity and downstream target gene transcript expression. The Wnt/β-catenin signaling pathway is inhibited by the XTSJF, leading to the suppression of colon cancer stem cell proliferation.

Conclusion:
Xiaotan Sanjie Prescription inhibited colon cancer stem cell growth through Wnt/β-catenin signaling pathway.
Predictive value of angiopoietin-like protein 8 in metabolic dysfunction-associated fatty liver disease and its progression: A case-control study
Authors: Gan Lu-Lu , Xia Can , Zhu Xuan , Gao Yue , Wu Wen-Chang , Li Qi , Li Ling , Dai Zhe , Yan Yi-Min 
Published: March 2024

BACKGROUND
The prevalence of metabolic dysfunction-associated fatty liver disease (MAFLD) is rapidly increasing, currently affecting approximately 25% of the global population. Liver fibrosis represents a crucial stage in the development of MAFLD, with advanced liver fibrosis elevating the risks of cirrhosis and hepatocellular carcinoma. Simple serum markers are less effective in diagnosing liver fibrosis compared to more complex markers. However, imaging techniques like transient elastography face limitations in clinical application due to equipment and technical constraints. Consequently, it is imperative to identify a straightforward yet effective method for assessing MAFLD-associated liver fibrosis.
AIM
To investigate the predictive value of angiopoietin-like protein 8 (ANGPTL8) in MAFLD and its progression.
METHODS
We analyzed 160 patients who underwent abdominal ultrasonography in the Endocrinology Department, Xiaogan Central Hospital affiliated to Wuhan University of Science and Technology, during September 2021-July 2022. Using abdominal ultrasonography and MAFLD diagnostic criteria, among the 160 patients, 80 patients (50%) were diagnosed with MAFLD. The MAFLD group was divided into the liver fibrosis group (n = 23) and non-liver fibrosis group (n = 57) by using a cut-off fibrosis-4 index ≥ 1.45. Logistical regression was used to analyze the risk of MAFLD and the risk factors for its progression. Receiver operating characteristic curves were used to evaluate the predictive value of serum ANGPTL8 in MAFLD and its progression.
RESULTS
Compared with non-MAFLD patients, MAFLD patients had higher serum ANGPTL8 and triglyceride-glucose (TyG) index (both P < 0.05). Serum ANGPTL8 (r = 0.576, P < 0.001) and TyG index (r = 0.473, P < 0.001) were positively correlated with MAFLD. Serum ANGPTL8 was a risk factor for MAFLD [odds ratio (OR): 1.123, 95% confidence interval (CI): 1.066-1.184, P < 0.001). Serum ANGPTL8 and ANGPTL8 + TyG index predicted MAFLD [area under the curve (AUC): 0.832 and 0.886, respectively; both P < 0.05]. Compared with MAFLD patients without fibrosis, those with fibrosis had higher serum ANGPTL8 and TyG index (both P < 0.05), and both parameters were positively correlated with MAFLD-associated fibrosis. Elevated serum ANGPTL8 (OR: 1.093, 95%CI: 1.044-1.144, P < 0.001) and TyG index (OR: 2.383, 95%CI: 1.199-4.736, P < 0.013) were risk factors for MAFLD-associated fibrosis. Serum ANGPTL8 and ANGPTL8 + TyG index predicted MAFLD-associated fibrosis (AUC: 0.812 and 0.835, respectively; both P < 0.05).
CONCLUSION
The serum levels of ANGPTL8 are elevated and positively correlated with MAFLD. They can serve as predictors for the risk of MAFLD and liver fibrosis, with the ANGPTL8 + TyG index potentially exhibiting even higher predictive value.
Comparative efficacy of sodium glucose cotransporter-2 inhibitors in the management of type 2 diabetes mellitus: A real-world experience
Authors: Islam Lubna , Jose Dhanya , Alkhalifah Mohammed , Blaibel Dania , Chandrabalan Vishnu , Pappachan Joseph M 
Published: March 2024

BACKGROUND
Sodium glucose cotransporter-2 inhibitors (SGLT-2i) are a class of drugs with modest antidiabetic efficacy, weight loss effect, and cardiovascular benefits as proven by multiple randomised controlled trials (RCTs). However, real-world data on the comparative efficacy and safety of individual SGLT-2i medications is sparse.
AIM
To study the comparative efficacy and safety of SGLT-2i using real-world clinical data.
METHODS
We evaluated the comparative efficacy data of 3 SGLT-2i drugs (dapagliflozin, canagliflozin, and empagliflozin) used for treating patients with type 2 diabetes mellitus. Data on the reduction of glycated hemoglobin (HbA1c), body weight, blood pressure (BP), urine albumin creatinine ratio (ACR), and adverse effects were recorded retrospectively.
RESULTS
Data from 467 patients with a median age of 64 (14.8) years, 294 (62.96%) males and 375 (80.5%) Caucasians were analysed. Median diabetes duration was 16.0 (9.0) years, and the duration of SGLT-2i use was 3.6 (2.1) years. SGLT-2i molecules used were dapagliflozin 10 mg (n = 227; 48.6%), canagliflozin 300 mg (n = 160; 34.3%), and empagliflozin 25 mg (n = 80; 17.1). Baseline median (interquartile range) HbA1c in mmol/mol were: dapagliflozin - 78.0 (25.3), canagliflozin - 80.0 (25.5), and empagliflozin - 75.0 (23.5) respectively. The respective median HbA1c reduction at 12 months and the latest review (just prior to the study) were: 66.5 (22.8) & 69.0 (24.0), 67.0 (16.3) & 66.0 (28.0), and 67.0 (22.5) & 66.5 (25.8) respectively (P < 0.001 for all comparisons from baseline). Significant improvements in body weight (in kilograms) from baseline to study end were noticed with dapagliflozin - 101 (29.5) to 92.2 (25.6), and canagliflozin 100 (28.3) to 95.3 (27.5) only. Significant reductions in median systolic and diastolic BP, from 144 (21) mmHg to 139 (23) mmHg; (P = 0.015), and from 82 (16) mmHg to 78 (19) mmHg; (P < 0.001) respectively were also observed. A significant reduction of microalbuminuria was observed with canagliflozin only [ACR 14.6 (42.6) at baseline to 8.9 (23.7) at the study end; P = 0.043]. Adverse effects of SGLT-2i were as follows: genital thrush and urinary infection - 20 (8.8%) & 17 (7.5%) with dapagliflozin; 9 (5.6%) & 5 (3.13%) with canagliflozin; and 4 (5%) & 4 (5%) with empagliflozin. Diabetic ketoacidosis was observed in 4 (1.8%) with dapagliflozin and 1 (0.63%) with canagliflozin.
CONCLUSION
Treatment of patients with SGLT-2i is associated with statistically significant reductions in HbA1c, body weight, and better than those reported in RCTs, with low side effect profiles. A review of large-scale real-world data is needed to inform better clinical practice decision making.
Dietary fiber intake and its association with diabetic kidney disease in American adults with diabetes: A cross-sectional study
Authors: Jia Xin-Hua , Wang Sheng-Yan , Sun Ai-Qin 
Published: March 2024

BACKGROUND
Dietary fiber (DF) intake may have a protective effect against type 2 diabetes (T2D); however, its relationship with diabetic kidney disease (DKD) remains unclear.
AIM
To investigate the potential association between DF intake and the prevalence of DKD in individuals diagnosed with T2D.
METHODS
This cross-sectional study used data from the National Health and Nutrition Examination Survey collected between 2005 and 2018. DF intake was assessed through 24-h dietary recall interviews, and DKD diagnosis in individuals with T2D was based on predefined criteria, including albuminuria, impaired glomerular filtration rate, or a combination of both. Logistic regression analysis was used to assess the association between DF intake and DKD, and comprehensive subgroup and sensitivity analyses were performed.
RESULTS
Among the 6032 participants, 38.4% had DKD. With lower DF intake-T1 (≤ 6.4 g/1000 kcal/day)-as a reference, the adjusted odds ratio for DF and DKD for levels T2 (6.5-10.0 g/1000 kcal/day) and T3 (≥ 10.1 g/1000 kcal/day) were 0.97 (95%CI: 0.84-1.12, P = 0.674) and 0.79 (95%CI: 0.68-0.92, P = 0.002), respectively. The subgroup analysis yielded consistent results across various demographic and health-related subgroups, with no statistically significant interactions (all P > 0.05).
CONCLUSION
In United States adults with T2D, increased DF intake may be related to reduced DKD incidence. Further research is required to confirm these findings.
Jianpi Gushen Huayu decoction ameliorated diabetic nephropathy through modulating metabolites in kidney, and inhibiting TLR4/NF-κB/NLRP3 and JNK/P38 pathways
Authors: Ma Zi-Ang , Wang Li-Xin , Zhang Hui , Li Han-Zhou , Dong Li , Wang Qing-Hai , Wang Yuan-Song , Pan Bao-Chao , Zhang Shu-Fang , Cui Huan-Tian , Lv Shu-Quan 
Published: March 2024

BACKGROUND
Jianpi Gushen Huayu Decoction (JPGS) has been used to clinically treat diabetic nephropathy (DN) for many years. However, the protective mechanism of JPGS in treating DN remains unclear.
AIM
To evaluate the therapeutic effects and the possible mechanism of JPGS on DN.
METHODS
We first evaluated the therapeutic potential of JPGS on a DN mouse model. We then investigated the effect of JPGS on the renal metabolite levels of DN mice using non-targeted metabolomics. Furthermore, we examined the effects of JPGS on c-Jun N-terminal kinase (JNK)/P38-mediated apoptosis and the inflammatory responses mediated by toll-like receptor 4 (TLR4)/nuclear factor-kappa B (NF-κB)/NOD-like receptor family pyrin domain containing 3 (NLRP3).
RESULTS
The ameliorative effects of JPGS on DN mice included the alleviation of renal injury and the control of inflammation and oxidative stress. Untargeted metabolomic analysis revealed that JPGS altered the metabolites of the kidneys in DN mice. A total of 51 differential metabolites were screened. Pathway analysis results indicated that nine pathways significantly changed between the control and model groups, while six pathways significantly altered between the model and JPGS groups. Pathways related to cysteine and methionine metabolism; alanine, tryptophan metabolism; aspartate and glutamate metabolism; and riboflavin metabolism were identified as the key pathways through which JPGS affects DN. Further experimental validation showed that JPGS treatment reduced the expression of TLR4/NF-κB/NLRP3 pathways and JNK/P38 pathway-mediated apoptosis related factors.
CONCLUSION
JPGS could markedly treat mice with streptozotocin (STZ)-induced DN, which is possibly related to the regulation of several metabolic pathways found in kidneys. Furthermore, JPGS could improve kidney inflammatory responses and ameliorate kidney injuries in DN mice via the TLR4/NF-κB/NLRP3 pathway and inhibit JNK/P38 pathway-mediated apoptosis in DN mice.