The present study was carried out in five cases of hepatic porphyria, including three of acute intermittent porphyria, one of variegate porphyria, and one of porphyria cutanea tarda in clinical remission. In two cases of acute intermittent porphyria (in relapse), a marked lowering effect on serum and urine porphobilinogen and delta-aminolevulinic acic was observed, together with prompt and gratifying clinical improvement. In a third case, in chemical remission but with longstanding psychoneurosis, no significant effects were noted, nor were any observed in the case of porphyria cutanea tarda. Although clinical improvements occurred in the case of variegate porphyria, the results were inconclusive for reasons given. Hematin was generally well tolerated. Preliminary reference is made to a transitory renal injury, without sequelae, where an excess of hematin was given in relation to time. Limits of tolerance are proposed. In the light of these observations the basic mechanism of the acute attack is diccussed.

Download full-text PDF

Source
http://dx.doi.org/10.7326/0003-4819-83-1-20DOI Listing

Publication Analysis

Top Keywords

porphyria
8
hepatic porphyria
8
acute intermittent
8
intermittent porphyria
8
variegate porphyria
8
porphyria cutanea
8
cutanea tarda
8
tarda clinical
8
effects hematin
4
hematin hepatic
4

Similar Publications

Specialty laboratory testing for chronic abdominal pain in irritable bowel syndrome.

Scand J Gastroenterol

January 2025

Department of Gastroenterology, Hepatology, and Nutrition, Digestive Disease Institute, Cleveland Clinic Foundation, Cleveland, OH, USA.

Background: Irritable bowel syndrome (IBS) is a common gastrointestinal disorder seen by both primary care providers (PCPs) and gastroenterologists, and further diagnostic testing is generally discouraged unless red-flag symptoms are present.

Aims: Examine if advanced serologic testing for chronic abdominal pain in IBS patients followed society-specific guidelines and evaluate the diagnostic accuracy of these tests.

Methods: The study involved a retrospective cross-sectional analysis of adults aged 18 and older who were seen at our institution between 2013 and 2018.

View Article and Find Full Text PDF

Hereditary tyrosinemia type 1 (HT-1) is an inborn error of metabolism caused by a defect in tyrosine (tyr) degradation. This defect results in the accumulation of succinylacetone (SA), causing liver failure with a high risk of hepatocarcinoma and kidney injury, leading in turn to Fanconi syndrome with urine loss of phosphate and secondary hypophosphatemic rickets (HR). HT-1 diagnosis is usually made in infants with acute or chronic liver failure or by neonatal screening programs.

View Article and Find Full Text PDF

Cost-Benefit Analysis of in vivo Reflectance Confocal Microscopy for Melanoma Diagnosis in a Real-World Clinical Setting.

Risk Manag Healthc Policy

January 2025

Department of Clinical Internal, Anesthesiological and Cardiovascular Sciences, Dermatology Clinic, Sapienza University of Rome, Rome, Italy.

Background: In a recent prospective, multicenter, two-arm randomized controlled trial (RCT), we demonstrated that adjunctive reflectance confocal microscopy (RCM) in routine clinical practice provides clinical benefits, including safe melanoma detection and a 43.3% reduction in the number needed to excise (NNE).

Methods: A cost-benefit analysis was conducted based on NNEs for standard care (5.

View Article and Find Full Text PDF

The histamine pathway is a target to treat hepatic experimental erythropoietic protoporphyria.

Cell Mol Gastroenterol Hepatol

January 2025

Center for Advanced Biotechnology and Medicine, Rutgers University, Piscataway, NJ, USA. Electronic address:

Background & Aims: Erythropoietic protoporphyria (EPP) is caused by mutations in ferrochelatase which inserts iron into protoporphyrin-IX (PP-IX) to generate heme. EPP is characterized by PP-IX accumulation, skin photosensitivity, cholestasis, and end-stage liver disease. Despite available drugs that address photosensitivity, treatment of EPP-related liver disease remains an unmet need.

View Article and Find Full Text PDF

Background: Acute hepatic porphyria (AHP) is characterized by debilitating and potentially life-threatening neurovisceral attacks, possible chronic symptoms, and long-term complications. In a phase 1/2 open-label extension (OLE) study and the phase 3 ENVISION study, givosiran led to sustained improvement in annualized attack rate and quality of life (QOL) measures. To capture the patient experience of symptoms and impacts of AHP, and any changes experienced during treatment with givosiran, qualitative interviews were conducted with study participants.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!