Clinically Important Features of Porphyrin and Heme Metabolism and the Porphyrias

Heme, like chlorophyll, is a primordial molecule and is one of the fundamental pigments of life. Disorders of normal heme synthesis may cause human diseases, including certain anemias (X-linked sideroblastic anemias) and porphyrias. Porphyrias are classified as hepatic and erythropoietic porphyrias based on the organ system in which heme precursors (5-aminolevulinic acid (ALA), porphobilinogen and porphyrins) are chiefly overproduced. The hepatic porphyrias are further subdivided into acute porphyrias and chronic hepatic porphyrias. The acute porphyrias include acute intermittent, hereditary copro-, variegate and ALA dehydratase deficiency porphyria. Chronic hepatic porphyrias include porphyria cutanea tarda and hepatoerythropoietic porphyria. The erythropoietic porphyrias include congenital erythropoietic porphyria (Gűnther’s disease) and erythropoietic protoporphyria. In this review, we summarize the key features of normal heme synthesis and its differing regulation in liver versus bone marrow. In both organs, principal regulation is exerted at the level of the first and rate-controlling enzyme, but by different molecules (heme in the liver and iron in the bone marrow). We also describe salient clinical, laboratory and genetic features of the eight types of porphyria

Liver disease in the Era of Coronavirus Disease 19 (COVID-19) pandemic

Coronavirus infections have caused outbreaks in humans: SARS-COV ((Severe Acute Respiratory Syndrome) and MERS-CoV (Middle East Respiratory Syndrome) resulting in significant mortality and morbidit

Evaluation of socioeconomic factors and achievement of sustained virologic response (SVR) in the treatment of hepatitis C virus (HCV)

Poster presented at: Aurora Scientific Day; May 24, 2017; Milwaukee, WI

Evalation of the Impact of the Implementation of a Specialty Pharmacy Program in the Treatment of Hepatitis C (HCV)

Conclusion: SVR (sustained virologic response) rates are comparable to clinical trials in this real-life clinical setting using a specialty pharmacy program. Those with HCV Genotype 1A had lower SVR rates post-medication completion, though it did not reach statistical significance. There was no difference in SVR rates between previously treated and treatment-naive patients