ObjectiveTo systematically evaluate the prognostic prediction models for Idiopathic Pulmonary Fibrosis (IPF). MethodsA computer-based search was conducted in the PubMed, Embase, Web of Science, and Cochrane Library databases for literature relevant to the research objective, with the search period ranging from database inception to Jun 2025. Two researchers independently screened the articles. Data were extracted according to the key assessment and data extraction checklist for systematic reviews of prediction models (CHARMS). The risk of bias and applicability of the models were assessed using the PROBAST (Prediction model Risk of Bias Assessment Tool). The quality of model reporting was evaluated using the TRIPOD (Transparent Reporting of a Multivariable Prediction Model for Individual Prognosis or Diagnosis) checklist. ResultsA total of 49 studies were included, of which 26 (53.06%) reported both model development and validation. The most common predictors included gender, age, diffusing capacity for carbon monoxide, forced vital capacity (FVC), and FVC percentage of predicted value. In terms of bias risk, 32 studies (65.31%) were classified as high risk of bias, mainly due to factors related to study subjects and predictors. Regarding applicability, 26 studies (53.06%) were rated as high risk, 11 studies (22.45%) were rated as unclear, and only 12 studies (24.49%) were rated as low risk, suggesting limited clinical applicability of the models. As for reporting quality, existing models showed generally insufficient adherence to the TRIPOD statement, especially in key areas such as research methods and result reporting, where normative issues were prominent. Of the 22 signaling questions in the TRIPOD checklist, most studies achieved only moderate reporting quality, with 8 signaling questions (1, 5c, 6b, 7b, 8, 11e, 13a, 14a) showing key information omissions or vague descriptions. ConclusionExisting prognostic prediction models for IPF generally exhibit high methodological bias risk and reporting deficiencies. Future studies should control for modeling biases based on the PROBAST framework, adhere to the TRIPOD guidelines for transparent reporting, and optimize clinical applicability through external validation.
Objective To explore the accuracy and practicability of bone age assessment for the diagnosis of idiopathic precocious puberty (IPP). Methods According to the “Gold Standard”, we selected 55 girls with IPP for the study group, and 83 normal girls for the control group. We retrospectively analyzed the first left hand-wrist radiographs at the first visit. Bone ages were assessed by using a single-blind method according to the RUS (Radius Ulna and Short bones), carpale and 20 bones method (TW2). Each had 5 decision thresholds (gt;97th percentile, gt;90th percentile, gt;75th percentile, gt;50th percentile and ≤50th percentile). The diagnostic values from RUS, carpale and 20 bones methods assessing bone age were analyzed to identify the best decision threshold. Results ① Both sensitivity and specificity of the four decision thresholds were relatively higher, including gt;90th percentile of RUS (sensitivity 0.836, specificity 0.916), gt;90th percentile of carpale (sensitivity 0.746, specificity 0.916), gt;90th and gt;75th percentile of 20 bone (sensitivity 0.746, specificity 0.964 and sensitivity 0.982, specificity 0.783, respectively). ② Area under receiver operator characteristic curve (AUR): AUR of RUS 0.939 ± 0.019 (95%CI 0.902 to 0.977), AUR of carpale 0.899 ± 0.028 (95%CI 0.845 to 0.954), AUR of 20 bone 0.958 ± 0.014 (95%CI 0.930 to 0.986). No significant difference was found (F=2.03, P=0.13). ③ Agreement assessment within-observer reliability was 89.28%, and between-observer reliability was 80.3% (Kappa 0.68, u=6.87, P＜0.01). Conclusions RUS and 20 bones methods have high accuracy for the diagnosis of idiopathic precocious puberty. Considering sensitivity and specificity, we think that ＞90th percentile of RUS is the best decision threshold.
Objective To analyze the clinical presentations and radiological characteristics of acute exacerbation of idiopathic pulmonary fibrosis ( IPF) . Methods Clinical and radiological data of 2 patients with acute exacerbation of IPF from April 2006 to July 2008 were retrospectively analyzed and literatures were reviewed. Results Both patients were senior male patients over 60 years old. Dyspnea, cough and inspiratory crackles were the major symptoms and signs. Two patients were experiencing an exacerbation of dyspnea for one week and half of month, respectively. PaO2 /FiO2 of both patients was less than225 mm Hg. In both patients, high-resolution computed tomography ( HRCT) scans at the exacerbation showed typical signs of IPF including peripheral predominant, basal predominant reticular abnormality, with honeycombing and traction bronchiectasis and bronchiolectasis, and newly developing alveolar opacity. HRCT scan showed peripheral area of ground-glass attenuation adjacent to subpleural honeycombing in one patient, and diffusely distributed ground-glass opacity in another patient. Two patients had received corticosteroid treatment. For one patient, the symptoms improved, and ground-glass attenuation adjacent to subpleural honeycombing had almostly resolved. The other patient died of respiratory failure. Conclusions Some acute exacerbation in idiopatic pulmonary fibrosis can be idiopathic. The clinical presentations mainly include the worsening of dyspnea within short time. HRCT generally demonstrates new bilateral ground-glass abnormality with or without areas of consolidation, superimposed on typical changes of IPF.
Objective To summarize the clinical characteristics of patients with interstitial pneumonia with positive myeloperoxidase (MPO-IP). Methods The clinical data of 15 patients hospitalized with MPO-IP from June 2013 to January 2016 were analyzed retrospectively, including clinical manifestations, laboratory test, lung function test, chest high resolution computed tomography (HRCT) and management. Simultaneously, 11 patients with IPF were recruited as control. Results The onset age of MPO-IP was older than that in IPF patients (74.07±6.31 yearsvs. 66.73±6.80 years,P＜0.01). There was no manifestation of vasculitis in kidney in all included patients, and the differences were not significant in gender, smoking, cough and expectoration, wheezing, shortness of breath, HRCT manifestations, FVC%pred, TLCO%pred, PaO2 or PaCO2 between the two groups. In 15 MPO-IP patients, HRCT revealed 12 cases of usual interstitial pneumonitis (UIP), 2 cases of nonspecific interstitial pneumonia (NSIP), 1 case of organizing pneumonia (OP), and 5 patients with overlapped emphysema. Eleven IPF patients were all in UIP pattern and 4 with overlapped emphysema. One MPO-IP patients and 5 IPF patients had clubbed-fingers. The differences in clubbed-finger and treatment were significantly different between the two groups. Nine IPF patients received symptomatic treatment and7 MPO-IP patients were administered with glucocorticoids and immunosuppressants. In addition 2 MPO-IP patients were treated with pirfenidone. Conclusions MPO-IP patients have older onset age of disease and lower incidence of clubbed-finger than IPF patients. UIP is the main HRCT change both in MPO-IP and IPF. Beside glucocorticoid and immunosuppressants, pirfenidone is a choice and worthy of further research in management of MPO-IP.
ObjectiveTo understand the genetic polymorphisms of MUC5B and TOLLIP in Chinese patients with idiopathic pulmonary fibrosis (IPF), and to explore whether gene polymorphism variation in Chinese IPF patients can be used as a genetic biomarker for accurate treatment and prognosis judgment.MethodsA total of one hundred and twenty-six patients with IPF were enrolled in this study. The baseline characteristics, total lung capacity (TLC), forced vital capacity (FVC), carbon monoxide diffusion function (DLCO), imaging changes of the patients were followed up. The levels of serum sputum glycosylated antigen-6 (Krebs Von den Lungen-6, KL-6) and B lymphocyte chemotactic factor C-X-C motif chemokine 13 (CXCL13) were detected by chemiluminescent enzyme immunoassay and enzyme-linked immunosorbent assay. The gene MUC5B rs35705950 and TOLLIP rs5743890, rs5743894 single nucleotide polymorphism (SNP) were determined by polymerase chain reaction.ResultsOne hundred and twenty-six patients with IPF were found with AA type by TOLLIP rs5743890 and rs5743894 SNP, accounting for 100.0%; MUC5B rs35705950 SNP was expressed as 116 patients (92.1%) with GG type, and 10 patients (7.9%) with GT type, no TT patients were detected. There was no significant difference in clinical characteristics between the two groups in age and non-smokers (P>0.05). Compared with group G, annual decrease of lung function (FVC, DLCO, and TLC), serum biomarkers (KL-6 and CXCL13), annual increase of reticular and honeycombing lesions, and mortality were significantly lower in group T (P<0.05). The median survival time of IPF patients carrying the MUC5B SNP rs35705950 minor allele (gene phenotype GT) heterozygous was significantly higher than that of homozygous IPF patients with a genetic phenotype of GG.ConclusionsThere are genetic polymorphisms in Chinese patients with IPF. MUC5B rs35705950 and TOLLIP rs5743890, rs5743894 gene subtypes have low mutation rates in the cohort. Compared with homozygous patients of MUC5B SNP rs35705950, heterozygous patients have smaller changes in lung function and radiological image, lower levels of serum KL-6 and CXCL13, and better prognosis.
ObjectiveTo construct a map of cerebral cortex thickness in Idiopathic Generalized Epilepsy (IGE) diagnosed at the first visit, using T1-weighted brain magnetic resonance imaging and advanced image analysis software. MethodsHigh-resolution three-dimensional T1 images were obtained from 27 IGE patients diagnosed at the first visit and 29 normal controls in Shouguang People's Hospital from January 1, 2022 to December 31, 2021. The location recognition calculation system of the Freesurfer software was used to calculate the values of cortical thickness in each brain region, and the cortical thickness values were transformed into a brain atlas using the image analysis software. A differential brain atlas was generated using the two-sample t-test to analyze the difference in cortical thickness between IGE patients and normal controls. Paired t-test was used for within-group comparison to explore changes of cortical thickness laterality. ResultsIn the IGE brain atlas, the brain regions with higher cortical thickness were the right left temporal pole, the right left entorhinal cortex, the head of the right anterior cingulate gyrus, the right and left insular lobe, the right and left middle temporal gyrus, the right inferior temporal gyrus, the head of the left anterior cingulate gyrus, the left tail of the anterior cingulate gyrus, the left inferior temporal gyrus, the left and right fusiform gyrus, and the left frontal pole. The areas with lower cortical thickness were the right and left paracalcaric gyrus, the right and left cuneiform lobe, the left and right lingual gyrus, the left and right posterior central gyrus, the left lateral occipital gyrus, and the right and left superior parietal gyrus. The distribution of cortical thickness of the IGE group was comparable to the cortical thickness atlas of the normal control. Compared with normal control, the areas with changes of cortical thickness in the IGE group were bilateral superior frontal gyrus, bilateral posterior central gyrus, bilateral anterior central gyri, bilateral lingual gyri, left cuneiform lobe, bilateral entorhinal cortex and temporal pole. The brain areas with laterality of cortical thickness between hemispheres in the IGE group were the tail of anterior cingulate gyrus, cuneiform lobe, inferior parietal gyrus, lateral occipital gyrus, posterior central gyrus, head of anterior cingulate gyrus, and superior marginal gyrus. Compared with normal control, the IGE group has decreased number of brain regions with laterality of cortical thickness. ConclusionThe present study revealed the distribution and laterality of cerebral cortical thickness map in early idiopathic generalized epilepsy, which provides imaging structural basis for brain research in the future.
After pirfenidone and nintedanib showed efficacy, drug treatment for idiopathic pulmonary fibrosis began to focused on anti-fibrosis. Current research on idiopathic pulmonary fibrosis mainly focus on the pathogenesis and therapeutic targets, and more targeted drugs are gradually entering clinical trials. This article summarizes the results of recent studies on the treatment of idiopathic pulmonary fibrosis with pirfenidone and nintedanib alone or in combination by searching the literature, and reviews the mechanism and test results of the new target anti-fibrosis drugs based on molecular biology that are currently undergoing clinical research in various phases, and aims to provide a basis for how to choose drugs to treat idiopathic pulmonary fibrosis.