rifampin and Cholangitis

Topics: Biliary Tract Diseases; Cholangitis; Cholecystitis; Clinical Trials as Topic; Humans; Rifampin; Staphylococcal Infections; Sulfobromophthalein; Urinary Tract Infections

Biallelic variants in the USP53 gene have recently been reported to segregate with normal gamma glutamyltransferase (GGT) cholestasis. Using whole-exome sequencing (WES), we detected two USP53 homozygous variants (c.951delT; p. Phe317fs and c.1744C>T; p. Arg582*) in five additional cases, including an unpublished cousin of a previously described family with intractable itching and normal GGT cholestasis. Three patients, a child and two adults, presented with recurrent episodes of normal GGT cholestasis, consistent with a diagnosis of benign recurrent intrahepatic cholestasis (BRIC). Cholangiopathic changes, possibly autoimmune in origin, were recognized in some patients. Additional phenotypic details in one patient included an enlarged left kidney, and speech/developmental delay. Notably, two patients exhibited a complete response to rifampicin, and one responded to ursodeoxycholic acid (UDCA). Two adult patients were suspected to have autoimmune liver disease and treated with steroids. This report describes new cases of USP53 disease presenting with normal GGT cholestasis or BRIC in three children and two adults. We also describe the novel finding of a dramatic response to rifampicin. The association of cholangiopathy with normal GGT cholestasis provides a diagnostic challenge and remains poorly understood.

Topics: Adolescent; Adult; Child; Cholangitis; Cholestasis; Exome Sequencing; Female; gamma-Glutamyltransferase; Homozygote; Humans; Infant; Male; Mutation; Nucleic Acid Synthesis Inhibitors; Pedigree; Prognosis; Rifampin; Ubiquitin-Specific Proteases

Clinical efficacy of rifampicin, a semisynthetic broad spectrum antibiotic was estimated in 247 patients with purulent inflammations. It was shown advisable to use rifampicin intravenously in treatment of severe bronchopulmonary pathology, disorders of the bile excretion system, osteomyelitis, severe wound infections and in prophylaxis of postoperative purulent complications in cardiovascular surgery and other cases. High rifampicin sensitivity of staphylococci and streptococci belonging to various species was revealed. Rifampicin was found to be less active against gramnegative pathogens. The isolation frequency of rifampicin sensitive strains of E. coli, Proteus spp., Klebsiella spp. and P. aeruginosa amounted to 88.4, 52.1, 58.8 and 49.3 per cent respectively.

Topics: Bacteria; Bacterial Infections; Cholangitis; Drug Resistance, Microbial; Hepatitis; Humans; Osteomyelitis; Respiratory Tract Infections; Rifampin; Wound Infection

Topics: Adult; Aged; Animals; Chemical and Drug Induced Liver Injury; Cholangitis; Cholecystitis; Evaluation Studies as Topic; Humans; Liver; Liver Function Tests; Male; Middle Aged; Rifampin

Determination of the minimum inhibitory concentrations of rifamide necessary to inhibit organisms isolated from the biliary tract showed that the organisms were almost invariably sensitive to concentrations which are readily attainable in the biliary tract. Three cases of severe acute inflammation of the biliary tract were treated and this led to rapid clinical improvement. In 61 patients undergoing biliary surgery a random group was given rifamide 150 mg twice daily, beginning 24 hours before operation and continuing for three days afterwards. In the untreated group eight patients had infected bile at operation and five subsequently developed a wound infection. In the rifamide group three had infected bile at operation and only one developed a wound infection. A similar number of postoperative chest infections occurred in each group of patients. There is some evidence of reduction in length of hospital stay in the treated patients.

Topics: Acute Disease; Aged; Bile; Cholangitis; Cholecystitis; Cholelithiasis; Female; Gallbladder; Humans; Length of Stay; Male; Microbial Sensitivity Tests; Postoperative Complications; Respiratory Tract Infections; Rifampin; Surgical Wound Infection

Topics: Ampicillin; Anti-Bacterial Agents; Bile; Chloramphenicol; Cholangitis; Humans; Rifampin; Streptomycin; Tetracycline

Topics: Bile; Bile Duct Neoplasms; Cholangitis; Cholelithiasis; Cholestasis; Colitis, Ulcerative; Humans; Rifampin

Topics: Anti-Bacterial Agents; Biliary Tract Diseases; Cholangitis; Humans; Pancreatitis; Postoperative Complications; Rifampin

Topics: Cholangitis; Escherichia coli Infections; Humans; Rifampin

Topics: Adult; Cholangitis; Cholecystitis; Female; Humans; Male; Middle Aged; Rifampin