No abstract available.
Porphyrias, Hepatic*

No abstract available.
Leg* ; Porphyria, Acute Intermittent* ; Porphyrias

No abstract available.
Alcoholism* ; Porphyria Cutanea Tarda* ; Porphyrias*

No abstract available.
Humans ; Interferon-beta ; Interferons ; Multiple Sclerosis ; Porphyria Cutanea Tarda ; Porphyrias

The Porphyria are a group of inherited and acquired disorders characterized by partial defects in the heme biosynthetic pathway. Among the hepatic forms, acute intermittent porphyria(AIP) is the most severe and common type in western hemisphere. Though its association with pregnancy is rare, it presents the obstetrician with challenging problems in diagnosis and management and it is probable that pregnancy had some deleterious effect in acute porphyria. The authors present a cae of AIP in pregnancy with a review of literature.
Biosynthetic Pathways ; Diagnosis ; Heme ; Porphyria, Acute Intermittent* ; Porphyrias ; Pregnancy*

No abstract available.
Humans ; Kidney Failure, Chronic ; Porphyria Cutanea Tarda* ; Porphyrias* ; Renal Dialysis*

Porphyria cutanea tarda(PCT) is a photocutaneous disorder due to excessive porphyrins in the skin. We experienced 4 male patients who showed typical clinical manifestations. Histologic findings revealed subepidermal bullae with festooned derrnal papi11ae, and typical porphyrin excretion pattern of PCT was detected. One case had on 500mg daily of chloroquine with r.linical and biochemical improvement 4 months later without any adverse effects. Other 2 cases had on 125mg of chloroquine twice a week and the other one had perforrned phlebotomy.
Chloroquine ; Humans ; Male ; Phlebotomy ; Porphyria Cutanea Tarda* ; Porphyrias* ; Porphyrins ; Skin

A case of porphyria cutanca tarda is reported, in which the photosensitive cutaneous symptoms developed in association with impaired hepatic function possibly related to chronic consumption of alcohol. Wood's light examination of the urine revealed coral red fluorescence, and qualitative chemical urinalysis was strong positive for uroporphyrin and coproporphyrin. Biopsy of the skin showed subepidermal bulla with festooning arrangement of the naked papillae and basophilic degeneration of the dermis. The patient's favorable response to alkalinization therapy by sodium bicarbonate per os is encouraging, even for a short period's observation. Clinical, histopathological, and biochemical features of this disease are also discussed.
Anthozoa ; Basophils ; Biopsy ; Dermis ; Fluorescence ; Porphyrias ; Skin ; Sodium Bicarbonate ; Urinalysis

The porphyrias are inherited or acquired disorders of specific enzymes in the heme biosynthetic pathway. Porphyrias have diverse neurologic manifestations with unknown mechanism. Some authors have proposed ischemic vasculopathy as a cause of neurologic deficits, which was supported by reversible MRI lesions in porphyrias. We report a rare case with reversible MRI findings in porphyria. A 27-year-old woman was presented with seizures and hallucinations. MRI revealed multiple lesions of both hemispheres. After treatment, MRI lesions showed marked resolution of signal abnormalities.
Adult ; Biosynthetic Pathways ; Female ; Hallucinations ; Heme ; Humans ; Magnetic Resonance Imaging* ; Neurologic Manifestations ; Porphyrias* ; Seizures

Porphyrias are a group of diseases characterized by an enzyme deficiency in the heme biosynthesis pathway, resulting in accumulation of precursor molecules in the tissue. Some porphyric patients develop progressive liver disease that requires liver transplantation. This case report describes special anesthetic challenges, including careful selection of drugs and the use of special filters that can exclude harmful wavelengths of ultraviolet, in a patient with porphyria who underwent living donor liver transplantation. Understanding the patient's status and disease process, and avoiding triggering factors of porphyria attacks, are important for successful liver transplantation anesthesia in patients with porphyria.
Anesthesia ; Heme ; Humans ; Liver ; Liver Diseases ; Liver Transplantation ; Living Donors ; Porphyrias