Hepatic porphyria: A narrative review

AbstractPorphyrias are a group of metabolic disorders, which result from a specific abnormality in one of the eight enzymes of the heme biosynthetic pathway. These have been subdivided based on the predominant site of enzyme defect into hepatic and erythropoietic types and based on clinical presentation into acute neurovisceral and cutaneous blistering porphyrias. This review focuses on hepatic porphyrias, which include acute intermittent porphyria (AIP), variegate porphyria (VP), hereditary coproporphyria (HCP), aminolevulinic acid dehydratase deficiency porphyria (ADP), and porphyria cutanea tarda (PCT). Of these, AIP and ADP are classified as acute porphyria, PCT as cutaneous, while VP and HCP present with both acute and cutaneous clinical manifestations. Porphobilinogen levels in a spot urine sample is the initial screening test for the diagnosis of acute hepatic porphyria, and plasma with spot urine porphyrin levels is the initial screening test to approach patients suspected of cutaneous porphyria. Specific biochemical porphyrin profile for each porphyria helps in determining the specific diagnosis. Pain relief and elimination of triggering agents are the initial steps in managing a patient presenting with an acute attack. Intravenous glucose administration terminates the mild episode of acute porphyria, with intravenous hemin needed for management of moderate to severe episodes. Liver transplantation is curative and may be needed for patients with a life-threatening ac...
Source: Indian Journal of Gastroenterology - Category: Gastroenterology Source Type: research

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Authors: Brignardello-Petersen R PMID: 32245571 [PubMed - as supplied by publisher]
Source: Journal of the American Dental Association - Category: Dentistry Tags: J Am Dent Assoc Source Type: research
I'm a current fellow scheduled to graduate in July. After that, I'm joining a private practice located>1000 miles away from where I'm training. Until the pandemic hit, the plan was to fly out there next month to start looking for housing. In addition to the inherent risk of travel right now, my hospital just implemented a policy that if you leave the state you have to self-quarantine for 2 weeks (and they count that as vacation time). Not sure what I'm going to do to find housing in time... Finding housing during pandemic
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In mid-March the CEO of BuzzBallz/Southern Champion saw that the COVID-19 pandemic was causing hand sanitizer supplies to dwindle in North Texas and all over the country, and her company reacted to help fill the need. In a few days, Merrilee Kick, CEO of Carrolton-based BuzzBallz/Southern Champion, said she was producing something she never dreamed of — BuzzBallz hand sanitizer. And the company is donating it to hospitals, health care providers, grocery stores and more. “It’s the right…
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Publication date: Available online 4 April 2020Source: Journal of EthnopharmacologyAuthor(s): Jin-Biao Xu, Guang-Chun Gao, Ming-Jing Yuan, Xuan Huang, Hong-Yu Zhou, Yang Zhang, Ya-Xin Zheng, Zhe Wu, Jun-Miao Feng, Ji-Ming Wu
Source: Journal of Ethnopharmacology - Category: Drugs & Pharmacology Source Type: research
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Source: Guardian Unlimited Science - Category: Science Authors: Tags: Coronavirus outbreak Medical research Infectious diseases Science World news Drugs Flu pandemic Health Society UK news Epidemics France Europe Source Type: news
This study delves into the mechanisms by which a short period of fasting can accelerate wound healing. Fasting triggers many of the same cellular stress responses, such as upregulated autophagy, as occur during the practice of calorie restriction. It isn't exactly the same, however, so it is always worth asking whether any specific biochemistry observed in either case does in fact occur in both situations. In particular, the period of refeeding following fasting appears to have beneficial effects that are distinct from those that occur while food is restricted. Multiple forms of therapeutic fasting have been repor...
Source: Fight Aging! - Category: Research Authors: Tags: Newsletters Source Type: blogs
CONCLUSIONS: In EUA, physical and mood disorders are associated with new and old inflammatory serological, clinical and imaging biomarkers. HLA-DRB1*11-positivity may be protective against these disease-related features. PMID: 32242813 [PubMed - as supplied by publisher]
Source: Clinical and Experimental Rheumatology - Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research
CONCLUSIONS: Gadolinium-enhanced MRI could display capsulitis, rotator cuff tendonitis and focal bone oedema in humerus heads that was sensitive and specific to patients with PMR, improving diagnostic accuracy in PMR. Rotator cuff tendonitis and synovial hypertrophy on MRI could help predict recurrence in PMR. PMID: 32242812 [PubMed - as supplied by publisher]
Source: Clinical and Experimental Rheumatology - Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research
Authors: Moreno-Fresneda P, Triguero-Martínez A, Olivas-Martínez I, Ortiz-Aljaro P, González-Escribano MF, Nuño-Nuño L, Ortiz AM, González-Álvaro I Abstract OBJECTIVES: To investigate the role of body mass index (BMI) in the phenotypic and genotypic characteristics of early arthritis patients. METHODS: We analysed the clinical and laboratory parameters from the baseline visit of patients (670 patients [78.51% women]) included in the PEARL study. The WHO definition for low weight, normal weight, overweight and obesity (BMI
Source: Clinical and Experimental Rheumatology - Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research
CONCLUSIONS: PMR patients with long-term normal APR seem to be a milder subset of PMR in clinical presentation and prognosis. Additionally, our data also suggest there is a subgroup with normal APR who are caught early in the disease. Misdiagnosis does not appear to play a role. PMID: 32242804 [PubMed - as supplied by publisher]
Source: Clinical and Experimental Rheumatology - Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research
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