Study on the efficacy of early treatment with pirfenidone on the lung function of patients with idiopathic pulmonary fibrosis

BACKGROUND Idiopathic pulmonary fibrosis(IPF)is classified under fibrotic interstitial pneumonia,characterized by a chronic and progressive course.The predominant clinical features of IPF include dyspnea and pulmonary dysfunction.AIM To assess the effects of pirfenidone in the early treatment of IPF on lung function in patients.METHODS A retrospective analysis was performed on 113 patients with IPF who were treated in our hospital from November 2017 to January 2023.These patients were divided into two groups:control group(n=53)and observation group(n=60).In the control group,patients received routine therapy in combination with methylprednisolone tablets,while those in the observation group received routine therapy together with pirfenidone.After applying these distinct treatment approaches to the two groups,we assessed several parameters,including the overall effectiveness of clinical therapy,the occurrence of adverse reactions(e.g.,nausea,vomiting,and anorexia),symptom severity scores,pulmonary function index levels,inflammatory marker levels,and the 6-min walk distance before and after treatment in both groups.RESULTS The observation group exhibited significantly higher rates than the control group after therapy,with a clear distinction(P<0.05).After treatment,the observation group experienced significantly fewer adverse reactions than the control group,with a noticeable difference(P<0.05).When analyzing the symptom severity scores between the two groups of patients after treatment,the observation group had significantly lower scores than the control group,with a distinct difference(P<0.05).When comparing the pulmonary function index levels between the two groups of patients after therapy,the observation group displayed significantly higher levels than the control group,with a noticeable difference(P<0.05).Evaluating the inflammatory marker data(C-reactive protein,interleukin-2[IL-2],and IL-8)between the two groups of patients after therapy,the observation group exhibited significantly lower levels than the control group,with significant disparities(P<0.05).Comparison of the 6-min walking distance data between the two groups of patients after treatment showed that the observation group achieved significantly greater distances than the control group,with a marked difference(P<0.05).CONCLUSION Prompt initiation of pirfenidone treatment in individuals diagnosed with IPF can enhance pulmonary function,elevate inflammatory factor levels,and increase the distance covered in the 6-min walk test.This intervention is conducive to effectively decreasing the occurrence of adverse reactions in patients.

Ductopenia and cirrhosis in a 32-year-old woman with progressive familial intrahepatic cholestasis type 3: A case report and review of the literature

Progressive familial intrahepatic cholestasis type 3 is caused by a mutation in the ATP-binding cassette, subfamily B, member 4 (ABCB4) gene encoding multidrug resistance protein 3. A 32-year-old woman with a history of acute hepatitis at age 9 years was found to have jaundice during pregnancy in 2008, and was diagnosed as having intrahepatic cholestasis of pregnancy. In 2009, she underwent cholecystectomy for gallstones and chronic cholecystitis. However, itching and jaundice did not resolve postoperatively. She was admitted to our hospital with fatigue, jaundice, and a recently elevated γ-glutamyl transpeptidase level. Liver biopsy led to the diagnosis of biliary cirrhosis with ductopenia. Genetic testing revealed a pathogenic heterozygous mutation, ex13 c.1531G > A (p.A511 T), in the ABCB4 gene. Her father did not carry the mutation, but her mother's brother carried the heterozygous mutation. We made a definitivediagnosis of familial intrahepatic cholestasis type 3. He symptoms and liver function improved after 3 mo o treatment with ursodeoxycholic acid.

Rare spontaneous intrahepatic portosystemic shunt in hepatitis Binduced cirrhosis: A case report

BACKGROUND The portosystemic shunt is the pathway between the portal vein (PV) and systemic circulation.A spontaneous intrahepatic portosystemic shunt (SPISS) is a rare portosystemic shunt type.Here we report an extremely rare type of SPISS,a spontaneous intrahepatic PV-inferior vena cava shunt (SPIVCS).CASE SUMMARY A 66-year-old woman was admitted to our hospital with the complaint of abdominal distention and a decreased appetite for 1 mo.The patient had a 20- year history of hepatitis B surface antigen positivity and a 5-year history of cirrhosis.She had been treated with Chinese herbal medicine for a long time.Liver function tests showed: alanine aminotransferase,35 U/L;aspartate aminotransferase,42 U/L;serum albumin (ALB) 32.2 g/L;and serum ascites ALB gradient,25.2 g/L.Abdominal ultrasonography and enhanced computed tomography showed that the left branch of the PV was thin and occluded;the right branch of the PV was thick and showed a vermicular dilatation vein cluster in the upper pole of the right kidney that branched out and converged into the inferior vena cava from the bare area of the lower right posterior lobe of the liver.We diagnosed her with an extremely rare SPIVCS caused by portal hypertension and provided symptomatic treatment after admission.One week later,her symptoms disappeared and she was discharged.CONCLUSION SPIVCS is a rare portosystemic shunt with a clear history of cirrhosis and portal hypertension.Clarifying the type PV shunt has important clinical significance.