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City Louisville / Deerfield / New York / / Company Ovation Pharmaceuticals / ADP / Trace American/Trace Diagnostics / HCP Yes Yes (bullae fragility) No / / Country United States / / / Event Product Issues / / Facility University of Connecticut / / / IndustryTerm heme products / Online Mendelian Inheritance / chemicals / end-product / degradation products / / MedicalCondition Major Clinical Manifestations Abdominal pain / Vomiting Constipation Diarrhea / central neurologic manifestation / seizures / arterial hypertension / hereditary coproporphyria / coma / hallucinations / ficiency porphyria / acute intermittent porphyria / congenital erythropoietic porphyria / leukocytosis / cardiac arrhythmia / chronic debilitating neurovisceral attacks / hyponatremia / starvation / otherwise unexplained abdominal pain / much more severe deficiency / bullae / end-stage liver disease / Paresis / paranoia / dehydratase deficiency / neurologic manifestations / severe neurologic manifestations / lead poisoning / Due to nausea / abdominal pain / paralysis / precise disorder / Porphyrias The porphyrias / systemic arterial hypertension / Hereditary / hypoventilation / ALA dehydratase deficiency porphyria / pneumonias / hepatocellular carcinoma / variant hereditary coproporphyria / acute porphyria / endometriosis / phlebitis / congenital erythorpoietic porphyria / fever / vomiting / acid-dehydratase deficient porphyria / alcoholic cirrhosis / serious bradycardia / diseases / erythropoietic protoporphyria / irritation / Neurovisceral Porphyrias / deficiency / depression / disorders / individual porphyrias / Gastrointestinal Abdominal pain / hereditary tyrosinemia type I. The diagnosis / homozygous acute porphyria / Neuro- Clinical Manifestation Porphyria / tachycardia and systemic arterial hypertension / ALA dehydratase deficiency / hepato-erythropoietic porphyria / progressive paralysis / Nausea / Muscle weakness / bladder paresis / basal ganglion / deficient porphyria / inherited enzyme deficiency / X-linked sideroblastic anemia / intermittent porphyria / Peripheral neuropathy / Gunther’s disease / severe hyponatremia / inherited disorders / acute hepatic porphyrias / severe ALA dehydratase deficiency / porphyrias / porphyria cutanea tarda / tachycardia / dominant Variegate porphyria / porphyria / hepatoma cell line LMA / hepatoerythrocytic porphyria / biochemically active disease / Hepatic porphyrias / very rare porphyria / enzyme deficiency / muscles and bulbar paralysis / namely porphyria cutanea tarda / hypotension / insomnia / disease / inherited partial enzymatic deficiency / intercurrent infections / severe constipation / disorientation / coughing / erythema / hepatoerythropoietic porphyria / aminolaevulinate dehydratase deficiency / Pain / usually lower / abdominal pain / acute porphyrias / urticaria / hereditary tyrosinemia type I / inducible hepatic porphyrias / severe homozygous dominant disease / syndrome / / MedicalTreatment surgery / birth control / / MusicGroup Nausea / / Organization American Society of Hematology Table / Mt. Sinai School of Medicine / American Society of Hematology Diagnosis of ALA / American Society of Hematology / deAmerican Society of Hematology Figure / Liver-BiliaryPancreatic Center / National Institute of Health / University of Connecticut / Department of Human Genetics / / Person Yes Yes / Ann Intern Med / Arch Biochem Biophys / Gene Locus Cytosolic / Arch Neurol / Herbert L. Bonkovsky / Jean Clark / Ann Intern / Kenneth Astrin / / / Position VP Chronic PCT / Major / VP / VP Abbreviations / AIP HCP VP CEP EPP HCP HEP PCT VP / / Product nadolol / gabapentin / glycine / Bang & Olufsen Form 2 Headphone/Headset / rifampin / sterile water / gene / Enzymes / porphyria / / ProvinceOrState Alabama / New York / Colorado / / Technology diagnostic tests / ultrasound / transplantation / condensation / / URL www.ncbi.nlm.nih.gov/entrez / www.hgmd.org / / SocialTag 
Neurovisceral Porphyrias: What a Hematologist Needs to Know Herbert L. Bonkovsky The acute or inducible hepatic porphyrias comprise four inherited disorders of heme biosynthesis. They