02 Nov 2021
Vitamin B6 and Oxalic Acid in Clinical Nephrology
Click on the link to read the full study.
28 Sept 2021
Severe porphyric neuropathy – importance of screening for porphyria in Guillain-Barré syndrome
Clara Schutte is Professor and Head of the Department of Neurology in the School of Medicine, Faculty of Health Sciences, University of Pretoria, South Africa.
Kees van der Meyden is Emeritus Professor and at the time of writing was working part-time in the Department, in which Linette van Niekerk is a senior registrar and Mandisa Kakaza is Professor and senior consultant. Riaan van Coller is a neurologist in private practice in Pretoria who has session appointments in the Department.
Veronica Ueckermann is a physician in charge of the intensive care unit in the Department of
Internal Medicine, Steve Biko Academic Hospital and University of Pretoria, and Nicky Oosthuizen is a senior pathologist in the Department of Chemical Pathology, University of Pretoria.
Corresponding author: C-M Schutte (firstname.lastname@example.org)
19 May 2021
Porphyria News Opens Survey to Gain More Insight Into Disease Community
Porphyria News is conducting an online survey with the goal of gaining greater insight into the characteristics of the porphyria community, their experiences in managing the disease, and their needs.
It takes an all-comer approach, meaning everyone is invited to participate, including patients, caregivers, family members, researchers, and healthcare professionals.
The survey was designed to take no more than 15 minutes to complete, and includes up to 35 questions focused on demographics, socioeconomics, disease symptoms, treatments, side effects, and the desire to participate in clinical trials. For those who would like to participate, the survey is available by clicking the link below.
12 Jun 2017
Effects of hemin and hemodialysis in a patient with acute intermittent porphyria and renal failure
Hemin and hemodialysis had an additive effect in decreasing ALA and PBG in our patient with acute intermittent porphyria and renal failure.
The time course of ALA and PBG reaccumulation after hemodialysis is not known.
Link to the article - https://ashpublications.org/bloodadvances/article/1/14/915/16181/Effects-of-hemin-and-hemodialysis-in-a-patient
02 Nov 1999
Biochemical Differentiation of The Porphyrias
Urine ALA & PGB - Elevated In AIP but were usually found to be "Normal" in VP and HCP
Studies documented by the above research team state categorically that the usual standards for testing carried out on VP and HCP patients show that not all those with these Porphyrias will show elevated levels with the current testing requirements.
Excerpt taken from the research paper:
Objectives: To differentiate the porphyrias by clinical and biochemical methods.
Design and methods:
We describe levels of blood, urine, and faecal porphyrins and their precursors in the porphyrias and present an algorithm for their biochemical differentiation. Diagnoses were established using clinical and biochemical data. Porphyrin analyses were performed by high performance liquid chromatography.
Results and conclusions: Plasma and urine porphyrin patterns were useful for diagnosis of porphyria cutanea tarda, but not the acute porphyrias. Erythropoietic protoporphyria was confirmed by
erythrocyte protoporphyrin assay and erythrocyte fluorescence.
Acute intermittent porphyria was diagnosed by increases in urine delta-aminolevulinic acid and porphobilinogen and confirmed by reduced erythrocyte porphobilinogen deaminase activity and normal or near-normal stool porphyrins. Variegate porphyria and hereditary coproporphyria were diagnosed by their characteristic stool porphyrin patterns. This appears to be the most convenient diagnostic approach until molecular abnormalities become more extensively defined and more widely available. Copyright © 1999 The Canadian Society of Clinical Chemists
Reference the full content of the article - see link above