Hepatic porphyrias: diagnosis and management

Chemmanur, Annie T.; Bonkovsky, Herbert L.

doi:10.1016/j.cld.2004.07.001

Cited by 47 publications

(55 citation statements)

References 17 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Different porphyrias are caused by the biosynthesis deficiencies of different enzyme, resulting in an increase in the production of porphyrin metabolites. Hence, analysis of porphyrin metabolites in urine or blood is the main tool for the biochemical diagnosis of the porphyrias [11][12][13][14][15].…”

Section: Introductionmentioning

confidence: 99%

Direct assay of uroporphyrin and coproporphyrin in human urine by reverse-mode field amplified sample injection-sweeping and micellar electrokinetic chromatography

Chen

Huang

Wang

2015

Talanta

View full text Add to dashboard Cite

Section: Introductionmentioning

confidence: 99%

Direct assay of uroporphyrin and coproporphyrin in human urine by reverse-mode field amplified sample injection-sweeping and micellar electrokinetic chromatography

Chen

Huang

Wang

2015

Talanta

View full text Add to dashboard Cite

“…Indeed, as reviewed elsewhere in this book series, acute hepatic heme deficiency is a hallmark of clinical attacks of the genetically inherited disorders stemming from defective heme synthesis known as the hepatic porphyrias, which are characterized by acute abdominal and neurological symptoms that can be fatal but are heme reversible if expediently treated. [8][9][10] Acute heme deficiency in neuronal cells is proposed to disrupt neuronal signaling pathways that may contribute to the neurological symptoms. [5][6][7] Acute hepatic heme depletion in rats has also been documented to alter the serotonergic tone by impairing the expression of a functional hepatic TDO, the enzyme that catalyzes the rate-limiting step in tryptophan catabolism.…”

Section: Introductionmentioning

confidence: 99%

Heme-Regulated Inhibitor (HRI) eIF2α Kinase and the Heme-Regulated Turnover of Cytochromes P450 and Other Hepatic Proteins

Correia¹,

Liu²,

Wang³

et al. 2013

Handbook of Porphyrin Science

View full text Add to dashboard Cite

“…Diazepan sometimes contributes to initial seizure control, then becoming soon ineffective. 10,20,27 HELLP syndrome (hemolysis, elevated liver enzyme levels, low platelet count) and eclampsia belong to a …”

Section: Treating Seizures Caused By Hepatic Failurementioning

confidence: 99%

Treating seizures in renal and hepatic failure

Lacerda

2008

J. epilepsy clin. neurophysiol.

View full text Add to dashboard Cite

Introduction: Renal and hepatic diseases cause seizures and patients with epilepsy may suffer from such diseases which change antiepileptic drugs (AEDs) metabolism. Objectives: To revise how seizures may be caused by metabolic disturbances due to renal or hepatic diseases, by their treatment or by comorbidities and how AEDs choice might be influenced by these conditions. Results: Seizures arise in renal failure due to toxins accumulation and to complications like sepsis, hemorrhage, malignant hypertension, pH and hydroelectrolytic disturbances. Hemodialysis leads to acute dysequilibrium syndrome and to dementia. Peritoneal dialysis may cause hyperosmolar non-ketotic coma. Post-renal transplant immunosupression is neurotoxic and cause posterior leukoencephalopathy, cerebral lymphoma and infections. Some antibiotics decrease convulsive thresholds, risking status epilepticus. Most commonly used AEDs in uremia are benzodiazepines, ethosuximide, phenytoin and phenobarbital. When treating epilepsy in renal failure, the choice of AED remains linked to seizure type, but doses should be adjusted especially in the case of hydrosoluble, low-molecular-weight, low-protein-bound, low apparent distribution volume AEDs. Hepatic failure leads to encephalopathy and seizures treated by ammonium levels and intestinal bacterial activity reductions, reversal of cerebral edema and intracranial hypertension. Phenytoin and benzodiazepines are usually ineffective. Seizures caused by post-hepatic immunosupression can be treated by phenytoin or levetiracetam. Seizures in Wilson's disease may result from D-penicillamine dependent piridoxine deficiency. Porphyria seizures may be treated with gabapentin, oxcarbazepine and levetiracetam. Hepatic disease changes AEDs pharmacokinetics and needs doses readjustments. Little liver-metabolized AEDs as gabapentin, oxcarbazepine and levetiracetam are theoretically more adequate. conclusions: Efficient seizures treatment in renal and hepatic diseases requires adequate diagnosis of these disturbances and their comorbidities besides good knowledge on AEDs metabolism, their pharmacokinetic changes in such diseases, careful use of concomitant medications and AEDs serum levels monitoring.

show abstract

Hepatic porphyrias: diagnosis and management

Cited by 47 publications

References 17 publications

Direct assay of uroporphyrin and coproporphyrin in human urine by reverse-mode field amplified sample injection-sweeping and micellar electrokinetic chromatography

Direct assay of uroporphyrin and coproporphyrin in human urine by reverse-mode field amplified sample injection-sweeping and micellar electrokinetic chromatography

Heme-Regulated Inhibitor (HRI) eIF2α Kinase and the Heme-Regulated Turnover of Cytochromes P450 and Other Hepatic Proteins

Treating seizures in renal and hepatic failure

Contact Info

Product

Resources

About