Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is defined as an acute and clinically significant respiratory deterioration characterized by evidence of new, widespread alveolar abnormality. In the past, AE-IPF was considered to be idiopathic, which was hard to be prevented and its prognosis was hard to be obviously improved; the latest researches have shown that AE-IPF can be triggered by known causes, including pulmonary infection, aspiration, etc. This review summarizes the etiology or risk factors, treatment and prevention of AE-IPF according to the latest researches.
Objective To improve the awareness of acute exacerbation of idiopathic pulmonary fibrosis ( AEIPF) and discuss its clinical characteristics, diagnosis, treatment and outcome. Methods The clinical data of patients with AEIPF from June 2006 to June 2011 in 11 hospitals in Jiangsu were collected and analyzed. Resluts There were 18 males and 3 females in the AEIPF patients with mean age of ( 67.4 ± 8.1) years. The duration from IPF diagnosis was ( 7.4 ±8.2) months. The duration of acute symptom before admission was ( 7.0 ±5.3) days. The distribution pattern of new groud-glass opacity was peripheral in 3 patients,multifocal in 5 patients, and diffuse in13 patients. All patients were treated with corticosteroid pulse therapy. Nine patients survived and 12 patients died. The mortality rate was 57.1% . Conclusions AEIPF progresses quickly and the mortality rate is very high. Corticosteroid pulse therapy is the mainstay of therapy in AEIPF patients.
目的:探讨基因重组人生长激素(recombinant human growth hormone, rhGH)对特发性矮小儿童促身高增长的疗效。方法:ISS儿童60例,每晚睡前接受rhGH治疗0.15～0.18 IU/(kg·d),疗程3～9个月,并对其疗效进行观察。结果:ISS患儿经生长激素治疗后,生长速率明显增快,由治疗前4.21±0.36 cm/年提高到治疗后8.29±4.72 cm/年,差异有显著性(Plt;0.05)。而骨龄和体重无明显变化,差异不显著(Pgt;0.05)。治疗期间除少数肝功能轻度异常,注射部位轻度反应外,未发现明显副作用。结论:rhGH对ISS儿童有增快生长速度作用。
ObjectiveTo observe the children with idiopathic epilepsy who received AED therapy and seizure-free, withdrawal at least 1 year, and analyze the risk factors of seizure recurrence. In order to give better instructions for AED withdrawal of children with idiopathic epilepsy in pediatric department. MethodsChildren with idiopathic epilepsy who were seizure-free and had stopped AED treatment at least 1 year before the deadline of observation were collected from pediatric outpatient and inpatient department of the First Affiliated Hospital of Guangxi Medical University from January 2011 to January 2014. The following variables, derived from the case records were analyzed: sex, age at seizure onset, type of seizure, time period between the onset of the first seizure and start of treatment, number of seizures in the time period between the start of AED therapy and the last seizure, number of AEDs before remission, time period between the start of AED therapy and the last seizure, time period between the last seizure and withdrawal of AED treatment (seizure-free period, timing of AED withdrawal), time period between the start of AED withdrawal and total discontinuation(speed of withdrawal), EEG registered at the time of diagnosis, before AED withdrawal, during and after AED withdrawal, and the condition of recurrence, data analysed by multi-variate step-wise Cox regression model analysis. ResultsA total of 16(12.8%) children relapsed in a total number of 125 children with idiopathic epilepsy we collected; date evaluated by multi-variable analysis showed that the children suffering from seizure type of focal seizure, starting withdrawal after a seizure-free period of less than 3 years and with abnormal post-withdrawal EEG recording was associated with a higher risk of seizure recurrence. ConclusionsChildren with idiopathic epilepsy have a low risk of recurrence after AED withdrawal. Those who suffer from seizure type of focal seizure, start withdrawal after a seizure-free period of less than 3 years and with abnormal post-withdrawal EEG recording are associated with a higher risk of seizure recurrence.
Gastroparesis is a gastrointestinal motility disorder that refers to delayed gastric emptying in the absence of mechanical obstruction. Clinical manifestations include postprandial fullness and epigastric discomfort, abdominal distension, nausea, and vomiting. According to its etiology, it can be divided into three categories: surgery-related gastroparesis, non-surgery-related gastroparesis and idiopathic gastroparesis. Non-surgery-related gastroparesis is common clinically. At present, the exact pathogenesis of gastroparesis remains to be unclear. The intestinal flora is huge and abundant. It participates in a variety of physiological functions of the host. Studies have confirmed that the intestinal flora is related to perioperative treatment measures, surgical stress, and various system diseases (endocrine and metabolic system diseases, nervous system diseases, and immune system diseases), especially the weakening of gastrointestinal motility, and gastrointestinal motility. Attenuation can further promote the occurrence and development of gastroparesis. Based on the current research, this article reviews the research on the correlation between gastroparesis and intestinal flora.
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 investigate the impact and mechanisms of periostin (POSTN), Krebs von den Lungen-6 (KL-6), pulmonary surfactant protein A (SP-A), and pulmonary surfactant protein D (SP-D) on the diagnosis and disease assessment of idiopathic pulmonary fibrosis (IPF), and conduct a comparative analysis. Methods From October 2022 to October 2023, a total of 55 patients diagnosed with IPF and treated at the Third Affiliated Hospital of Anhui Medical University were enrolled as an IPF group. Additionally, 30 patients with bacterial pneumonia and 30 healthy individuals undergoing concurrent health examinations during the same period were selected as a pneumonia control group and a healthy control group, respectively. All participants underwent enzyme-linked immunosorbent assay to measure serum levels of POSTN, KL-6, SP-A, and SP-D, along with pulmonary function tests. The IPF patients also underwent high-resolution computed tomography (HRCT) and echocardiography to quantify HRCT scores and pulmonary artery systolic pressure (PASP). Receiver operating characteristic (ROC) curves were plotted to analyze the significance of serum POSTN, KL-6, SP-A, and SP-D levels in IPF diagnosis. Pearson and Spearman correlation tests were used to analyze the relationships between these biomarkers and pulmonary function, PASP, and HRCT scores. Results Serum concentrations of POSTN, KL-6, SP-A, and SP-D were significantly elevated in the IPF group compared with the pneumonia group and the healthy controls (P<0.05), while serum levels of SP-A and SP-D were notably higher in the pneumonia group compared with the healthy control group (P<0.05). Within the IPF group, serum POSTN levels were negatively correlated with forced expiratory volume in the first second as a percentage of predicted value (FEV1%pred) and diffusion capacity of the lung for carbon monoxide as a percentage of predicted value (DLCO%pred) (P<0.05); KL-6 and SP-D levels were also negatively correlated with FEV1%pred, forced vital capacity as a percentage of predicted value (FVC%pred), and DLCO%pred (P<0.05); and the concentration of SP-A was negatively correlated with DLCO%pred and positively correlated with PASP (P<0.05). Additionally, serum levels of POSTN, KL-6, and SP-A in the IPF group showed significant positive associations with HRCT scores (P<0.01). Conclusions POSTN is a valuable serum biomarker for IPF, exhibiting the highest sensitivity and specificity among the four serum markers, with diagnostic performance superior to KL-6, SP-A, and SP-D. POSTN, KL-6, SP-A, and SP-D can all be used for the diagnosis and assessment of IPF.