1.A Case of Acute Hepatic Porphyria.
Young Cheal HAN ; Sin Hang JOO ; Jin Han KANG ; Byung Churl LEE
Journal of the Korean Pediatric Society 1988;31(2):253-257
No abstract available.
Porphyrias, Hepatic*
2.Acute Intermittent Porphyria Presenting with Periodic Leg Pain.
Hye Jin LEE ; Soojeong KIM ; Jeeyoung OH
Journal of the Korean Neurological Association 2017;35(1):53-54
No abstract available.
Leg*
;
Porphyria, Acute Intermittent*
;
Porphyrias
3.Porphyria cutanea tarda precipitated by alcohol abuse.
Hee Yeon KIM ; Ji Hyun KIM ; Seung Hwan LEE ; Ho Young SON
Korean Journal of Medicine 2007;73(6):670-671
No abstract available.
Alcoholism*
;
Porphyria Cutanea Tarda*
;
Porphyrias*
4.Sporadic Porphyria Cutanea Tarda in a Patient with Multiple Sclerosis Treated with Interferon Beta 1-a Therapy: A Case Report.
Pietro CARRIERI ; Maria PETRACCA ; Silvana MONTELLA ; Giovanni CERULLO ; Ilaria CERILLO ; Gianfranco CIMMINO
Journal of Clinical Neurology 2013;9(3):196-197
No abstract available.
Humans
;
Interferon-beta
;
Interferons
;
Multiple Sclerosis
;
Porphyria Cutanea Tarda
;
Porphyrias
5.Acute Intermittent Porphyria and Pregnancy.
Ji Hee RYU ; Eun A CHOI ; Hyung Min CHOI ; Tae Yoon KIM ; Jae Sung CHO ; Yong Won PARK ; Jae Wook KIM
Korean Journal of Obstetrics and Gynecology 1997;40(2):414-418
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*
6.Porphyria Cutanea Tarda in a Hemodialysis Patient.
Kee Suck SUH ; Sang Hwa HAN ; Joon Hee KIM ; Kang Hoon LEE ; Jong Bin PARK ; Sang Tae KIM ; Min Soo JANG
Korean Journal of Dermatology 2016;54(1):81-83
No abstract available.
Humans
;
Kidney Failure, Chronic
;
Porphyria Cutanea Tarda*
;
Porphyrias*
;
Renal Dialysis*
7.Four Cases of Porphyria Cutanea Tarda.
Sung Hwa KIM ; Sang Lip CHUNG ; Sang Won KIM
Korean Journal of Dermatology 1983;21(4):421-427
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
8.Porphytia Cutanea Tarda: Report of a Case.
Soon Taek KIM ; Hou Suk SEONG ; Tae An CHUNG
Korean Journal of Dermatology 1974;12(3):183-186
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
9.A Case of Porphyria with reversible MRI findings.
Suk Yun KANG ; Hyeo Il MA ; Kyu Hwan KWAK ; San Ahm LEE
Journal of the Korean Neurological Association 1998;16(2):241-244
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
10.A case report of anesthesia management in the liver transplantation recipient with porphyria: A case report.
Hye Won SONG ; Young Hee SHIN ; Justin Sangwook KO ; Mi Sook GWAK ; Gaab Soo KIM
Korean Journal of Anesthesiology 2012;62(1):83-86
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