Results 81 to 90 of about 129 (113)

Treatment of acute hepatic porphyria with hematin

Journal of Hepatology, 1988
The efficacy of hematin has been evaluated in eight patients with acute intermittent porphyria: six with acute attacks and two with chronic subacute symptoms. Hematin suppressed the chemical signs of porphyria in all patients and the symptoms in those with acute attacks.
openaire   +2 more sources

Acute hepatic porphyrias—A guide for hepatologists

Hepatology
The acute hepatic porphyrias (AHPs) are a group of rare, inherited disorders of the heme biosynthesis pathway, usually manifesting with attacks of acute abdominal pain and other neurovisceral symptoms, with or without cutaneous manifestations. AHPs are characterized by the accumulation of porphyrin precursors, porphobilinogen, and/or aminolevulinic ...
Akshata Moghe, Brendan M. McGuire, Cynthia Levy   +2 more
openaire   +2 more sources

Acute hepatic porphyrias: pathophysiology and pathogenesis of acute attacks

Clinical Neurology
Heme is an iron-containing molecule essential for virtually all living organisms. However, excessive heme is cytotoxic, necessitating tight regulation of intracellular heme concentration. The acute hepatic porphyrias (AHPs) are a group of rare inborn errors of heme biosynthesis that are characterized by episodic acute neurovisceral attacks that are ...
Makiko Yasuda
exaly   +3 more sources

Novel treatment options for acute hepatic porphyrias

Current Opinion in Gastroenterology, 2021
Purpose of review Acute hepatic porphyrias (AHP) are a group of rare diseases that are characterized by episodic acute neurovisceral pain episodes caused by abnormal accumulation of the neurotoxic porphyrin precursor delta-aminolevulinic acid (ALA).
openaire   +2 more sources

Unusual Manifestation of Acute Hepatic Porphyria in Pregnancy

Fetal Diagnosis and Therapy, 2006
A 22-year-old para I/gravida II developed psychiatric symptoms at 8 weeks of gestation. Subsequently neurological symptoms with seizures developed leading to a status epilepticus with continuing seizures at week 14. Anticonvulsive therapy had little effect in alleviating the seizures and the condition of the patient rapidly deteriorated.
A, Weinzierl, C, Brezinka, K, Engelhardt
openaire   +2 more sources

[Anesthesia and acute hepatic porphyrias].

Anasthesiologie, Intensivmedizin, Notfallmedizin, Schmerztherapie : AINS, 1992
Acute hepatic porphyrias are inherited disorders of heme biosynthesis. A characteristic feature of acute porphyrias is the occurrence of acute attacks which are often precipitated by drugs. Many of the commonly used anaesthetic agents may trigger such a potentially lethal attack.
H, Böhrer, H, Schmidt, E, Martin
openaire   +1 more source

ACUTE HEPATIC PORPHYRIA IN POLAND

The Lancet, 1982
E, Kostrzewska, A, Gregor
openaire   +2 more sources

Therapeutic approach to acute crises of hepatic porphyrias

Revista Clínica Española (English Edition)
Acute hepatic porphyria is a genetic disorder affecting enzymes involved in heme biosynthesis. The most common subtype is acute intermittent porphyria, accounting for 80% of cases. Other types include hereditary coproporphyria, variegate porphyria, and delta-aminolevulinic acid dehydratase deficiency. Attacks in acute hepatic porphyria are triggered by
M, Garrido Montes, R, Pertusa Mataix, J S, Garcia Morillo   +2 more
openaire   +2 more sources

Treatment of acute hepatic porphyria.

Lancet (London, England), 1978
C.A. Pierach, C.J. Watson, A.A.-B. Badawy   +2 more
  +7 more sources

Acute Hepatic Porphyrias: “Purple Flags”—Clinical Features That Should Prompt Specific Diagnostic Testing

American Journal of the Medical Sciences, 2022
Robert Desnick, , Paolo Ventura
exaly