Effects of hemin and hemodialysis in a patient with acute intermittent porphyria and renal failure

Attarian, Shirin; Yu, Chunli; Anderson, Karl E.; Friedman, Ellen

doi:10.1182/bloodadvances.2017005660

Cited by 6 publications

(3 citation statements)

References 9 publications

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“…Murine models have suggested a role of melatonin in drug-induced porphyria,26 but it is yet to be investigated in humans. Haemodialysis has also been used with and without human hemin to decrease the levels of PBG and ALA in blood 27 28. In some cases where the disease progresses to liver cirrhosis, the only treatment option is liver transplant 20.…”

Section: Discussionmentioning

confidence: 99%

Paediatric porphyria and human hemin: a treatment challenge in a lower middle income country

et al. 2020

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Here, we report a case of a 15-year-old girl who presented to the emergency department with symptoms of abdominal pain, nausea, vomiting and seizures. She was diagnosed with acute intermittent porphyria. Treatment was started by removing all porphogenic drugs, providing high glucose intake (oral and intravenous), which initially resulted in good clinical outcomes. However, she deteriorated again and also developed neurological manifestation (paraplegia) for which she required mechanical ventilation because of acute respiratory failure. This time she was initiated on human hemin for four consecutive days. After 2 days of therapy, her porphobilinogen levels decreased to 50% of the initial raised value. Increased lactic acid and blood urea nitrogen were the two side effects observed after the treatment, with no apparent signs of acute kidney injury. To the best of our knowledge, in paediatric population, this is the first reported case of treatment of acute intermittent porphyria with human hemin in Pakistan.

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Section: Discussionmentioning

confidence: 99%

Paediatric porphyria and human hemin: a treatment challenge in a lower middle income country

et al. 2020

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“…In 2007 Kuo et al, (Taiwan) described the improvement of neurological complications upon hematin infusions [ 76 ]. In one patient in New York hemin treatment was combined with hemodialysis [ 77 ].…”

Section: Therapymentioning

confidence: 99%

Therapy Follows Diagnosis: Old and New Approaches for the Treatment of Acute Porphyrias, What We Know and What We Should Know

Petrides

2022

Diagnostics

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Heme, iron protoporphyrin IX, is one of life’s most central molecules. Hence, availability of the enzymatic machinery necessary for its synthesis is crucial for every cell. Consequently, inborn errors of porphyrin metabolism that compromise normal synthesis, namely the family of porphyrias, undermine normal cellular metabolism given that heme has functions in catalytic centers, signal transduction and functional regulation and its synthesis is fully integrated into the center of intermediary metabolism. Very often, diagnosis of porphyrias is difficult and therefore delayed. Therapy can be as complicated. Over the last 50 years, several strategies have been developed: because of its integration with other parts of intermediary metabolism, the infusion of glucose (glucose effect) was one of the first attempts to counterbalance the dysregulation of porphyrin synthesis in porphyrias. Since heme synthesis is impaired, infusional replacement of heme was the next important therapeutic step. Recently, siRNA technology has been introduced in order to downregulate 5-ALA-synthase 1, which contributes to the patho-physiology of these diseases. Moreover, other novel therapies using enzyme protein replacement, mRNA techniques or proteostasis regulators are being developed.

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“…Our diamond magnetic microscope is capable of imaging paramagnetic substances other than hemozoin. Of possible interest are hemin microcrystals, which are used as a pharmacological agent for treating porphyria [56]. Hemin crystals have magnetic susceptibility similar to that of hemozoin but are much larger (up to 30 μ m).…”

Section: Magnetostatic Modeling Of S1–s3mentioning

confidence: 99%

Diamond Magnetic Microscopy of Malarial Hemozoin Nanocrystals

et al. 2019

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Magnetic microscopy of malarial hemozoin nanocrystals is performed by optically detected magnetic resonance imaging of near-surface diamond nitrogen-vacancy centers. Hemozoin crystals are extracted from Plasmodium falciparum –infected human blood cells and studied alongside synthetic hemozoin crystals. The stray magnetic fields produced by individual crystals are imaged at room temperature as a function of the applied field up to 350 mT. More than 100 nanocrystals are analyzed, revealing the distribution of their magnetic properties. Most crystals (96%) exhibit a linear dependence of the stray-field magnitude on the applied field, confirming hemozoin’s paramagnetic nature. A volume magnetic susceptibility of 3.4 × 10 −4 is inferred with use of a magnetostatic model informed by correlated scanning-electron-microscopy measurements of crystal dimensions. A small fraction of nanoparticles (4/82 for Plasmodium falciparum –produced nanoparticles and 1/41 for synthetic nanoparticles) exhibit a saturation behavior consistent with superparamagnetism. Translation of this platform to the study of living Plasmodium -infected cells may shed new light on hemozoin formation dynamics and their interaction with antimalarial drugs.

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Effects of hemin and hemodialysis in a patient with acute intermittent porphyria and renal failure

Abstract: Key Points 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.

Cited by 6 publications

References 9 publications

Paediatric porphyria and human hemin: a treatment challenge in a lower middle income country

Paediatric porphyria and human hemin: a treatment challenge in a lower middle income country

Therapy Follows Diagnosis: Old and New Approaches for the Treatment of Acute Porphyrias, What We Know and What We Should Know

Diamond Magnetic Microscopy of Malarial Hemozoin Nanocrystals

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