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ACP Best Practice No 165: front line tests for the investigation of suspected porphyria. J Clin Pathol (2001) 1.50

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Structural basis of hereditary coproporphyria. Proc Natl Acad Sci U S A (2005) 1.08

Erythropoietic and hepatic porphyrias. J Inherit Metab Dis (2000) 1.00

Variegate porphyria in Western Europe: identification of PPOX gene mutations in 104 families, extent of allelic heterogeneity, and absence of correlation between phenotype and type of mutation. Am J Hum Genet (1999) 0.93

Guide to drug porphyrogenicity prediction and drug prescription in the acute porphyrias. Br J Clin Pharmacol (2007) 0.85

Self-rated psychosocial consequences and quality of life in the acute porphyrias. J Inherit Metab Dis (2001) 0.84

Anxiety and depression in the acute porphyrias. J Inherit Metab Dis (2005) 0.83

Quantitative structural insight into human variegate porphyria disease. J Biol Chem (2013) 0.80

A retrospective analysis of outcome of pregnancy in patients with acute porphyria. J Inherit Metab Dis (2010) 0.78

Role of aspartate 400, arginine 262, and arginine 401 in the catalytic mechanism of human coproporphyrinogen oxidase. Protein Sci (2007) 0.78

Psychiatric signs and symptoms in treatable inborn errors of metabolism. J Neurol (2014) 0.78

Inborn errors of metabolism as a cause of neurological disease in adults: an approach to investigation. J Neurol Neurosurg Psychiatry (2000) 0.77

Clinic and genetic evaluation of variegate porphyria (VP) in a large family from the Balearic Islands. J Inherit Metab Dis (2009) 0.76

Acute intermittent porphyria associated with hypoganglionosis in a young adult. Gut (2006) 0.75

Metoclopramide and acute porphyria. Lancet (1997) 0.75

Mechanistic insights into the substrate recognition of PPO: toward the rational design of effective inhibitors. Future Med Chem (2014) 0.75

Heme deficiency in Alzheimer's disease: a possible connection to porphyria. J Biomed Biotechnol (2006) 0.75

Anaesthesia for the porphyric patient. Anaesthesia (1993) 3.12

A R59W mutation in human protoporphyrinogen oxidase results in decreased enzyme activity and is prevalent in South Africans with variegate porphyria. Nat Genet (1996) 1.78

The prevention of hepatitis. S Afr Med J (1994) 1.67

Liver disease in erythropoietic protoporphyria: insights and implications for management. Gut (2007) 1.31

A structured assessment of newly qualified medical graduates. Med Educ (2005) 1.28

Porphyrias. Br J Anaesth (2000) 1.19

Purification of and kinetic studies on a cloned protoporphyrinogen oxidase from the aerobic bacterium Bacillus subtilis. Arch Biochem Biophys (1998) 0.95

Molecular characterization of erythropoietic protoporphyria in South Africa. Br J Dermatol (2008) 0.92

Hepatic porphyrias in children. J Inherit Metab Dis (1997) 0.91

Protoporphyrinogen oxidase and porphobilinogen deaminase in variegate porphyria. Eur J Clin Invest (1986) 0.89

Homozygous variegate porphyria: an evolving clinical syndrome. Postgrad Med J (1993) 0.88

A systematic study of the clinical and biochemical expression of variegate porphyria in a large South African family. Br J Dermatol (2004) 0.87

Homozygous variegate porphyria in South Africa: genotypic analysis in two cases. Mol Genet Metab (2000) 0.86

Variegate porphyria. Semin Liver Dis (1998) 0.84

Liver disease in erythropoietic protoporphyria: insights and implications for management. Postgrad Med J (2007) 0.82

The effect of oral activated charcoal on the course of congenital erythropoietic porphyria. Br J Dermatol (1993) 0.82

Treatment of acute porphyria. Hosp Med (2001) 0.82

Hepatoerythropoietic porphyria precipitated by viral hepatitis. Gut (1993) 0.81

Identification of the first variegate porphyria mutation in an indigenous black South African and further evidence for heterogeneity in variegate porphyria. Mol Genet Metab (2001) 0.81

Alcohol intake and porphyria cutanea tarda. Clin Dermatol (1999) 0.80

Propofol as an i.v. anaesthetic induction agent in variegate porphyria. Br J Anaesth (1991) 0.80

Inhibition of mammalian protoporphyrinogen oxidase by acifluorfen. Biochem Mol Biol Int (1994) 0.79

Drugs in the acute porphyrias--toxicogenetic diseases. Cell Mol Biol (Noisy-le-grand) (1997) 0.78

Safety and efficacy of interferon alpha-2b following prednisone withdrawal in the treatment of chronic viral hepatitis B. A case-controlled, randomised study. S Afr Med J (1992) 0.77

Purification of human erythrocyte porphobilinogen deaminase. S Afr Med J (1991) 0.77

Porphyrinogenicity of etomidate and ketamine as continuous infusions. Screening in the DDC-primed rat model. Br J Anaesth (1985) 0.77

Detection of four mutations in six unrelated South African patients with acute intermittent porphyria. Mol Cell Probes (1996) 0.77

Identification and characterisation of a deletion (537delAT) in the protoporphyrinogen oxidase gene in a South African variegate porphyria family. Hum Mutat (1998) 0.77

Variegate porphyria in South Africa, 1688-1996--new developments in an old disease. S Afr Med J (1997) 0.77

Acute intermittent porphyria: the in vitro expression of mutant hydroxymethylbilane synthase. Mol Cell Probes (1997) 0.76

Update on enzyme and molecular defects in porphyria. Photodermatol Photoimmunol Photomed (1998) 0.76

Safety of vancomycin in acute porphyria. Br J Clin Pharmacol (1990) 0.75

A jaundiced look at the management of hepatitis? S Afr Med J (1997) 0.75

Primary biliary cirrhosis. A retrospective survey at Groote Schuur Hospital, Cape Town. S Afr Med J (1990) 0.75

Porphyria cutanea tarda: the etiological importance of mutations in the HFE gene and viral infection is population-dependent. Cell Mol Biol (Noisy-le-grand) (2002) 0.75

The expression of protoporphyrinogen oxidase in human tissues. Cell Mol Biol (Noisy-le-grand) (2009) 0.75

Diagnosis of variegate porphyria at birth by demonstration of the R59W mutation. S Afr Med J (1997) 0.75

Primary pyogenic psoas abscess. A case report. S Afr J Surg (1986) 0.75

Acute intermittent porphyria--an underdiagnosed cause of abdominal pain. S Afr Med J (1989) 0.75

Mapping of the variegate porphyria (VP) gene: contradictory evidence for linkage between VP and microsatellite markers at chromosome 14q32. Hum Genet (1996) 0.75

Porphyria cutanea tarda: pathogenesis in relation to therapy. Curr Probl Dermatol (1991) 0.75

Uroporphyrinogen decarboxylase and protoporphyrinogen oxidase in dual porphyria. S Afr Med J (1989) 0.75

Carbamazepine and haem biosynthesis. Lancet (1983) 0.75

Prophylaxis and treatment of malaria in people with the acute porphyrias. S Afr Med J (1988) 0.75

Acute liver failure. S Afr Med J (1992) 0.75

Administration of oral activated charcoal in variegate porphyria results in a paradoxical clinical and biochemical deterioration. Br J Dermatol (2003) 0.75

The clinical diagnosis, prevention and management of the hepatic porphyrias. Trop Gastroenterol (1997) 0.75

Identification of two novel mutations in the hydroxymethylbilane synthase gene in three patients from two unrelated families with acute intermittent porphyria. Hum Hered (1998) 0.75