In porphyria

Emergence delirium Anti-emetic Safe in porphyria... 

Thiopental Rapid onset and rapid recovery (bolus dose) —slow recovery following infusion Standard induction agent causes cardiovascular depression avoid in porphyrias... 

Neither vigabatrin nor gabapentin caused porphyrin accumulation in chicken embryo cultured liver cells, whereas felbamate, lamotrigine, and tiagabine were por-phyrinogenic in this model (129). For gabapentin, safety in porphyrias has been confirmed in preliminary clinical observations (SEDA-19, 70) (SEDA-20, 62). 

D Alessandro R, Rocchi E, Cristina E, Cassanelli M, Benassi G, Pizzino D, Baldrati A, Baruzzi A. Safety of valproate in porphyria cutanea tarda. Epilepsia 1988 29(2) 159-62. 

Mrs JC s acute abdominal pain should be managed symptomatically with analgesics known to be safe in porphyria. For severe pain morphine is non-porphyrogenic. 

Paracetamol and opioid analgesics are safe to use in porphyria, however diclofenac is one of the drugs known to be unsafe to prescribe in porphyria. There are other NSAIDs such as ibuprofen or naproxen which are not listed as unsafe and the medical team should be advised to amend the prescription accordingly. However if Mrs JC is in severe pain it may be necessary to prescribe a strong opiate. These are safe in porphyria. 

Localized hypertrichosis is found in Chvostek s body type in the form of noticeably bushy eyebrows. It is also, however, found in porphyria cutanea tarda in the area around the zygomatic arch and lateral to the eyes, as well as on the forearm and lower leg, mostly in the form of short, compact hairs. 

Fig. 7.10 Red fluorescent liver specimen in porphyria cutanea tarda (typ>e D)...

Low prevalence of hepatitis C virus infection in porphyria cutanea tarda in Germany. Hepatology 1995 21 1500-1503... 

Fig. 31.16 Skin changes (face, front part of the neck) in porphyria cutanea tarda (s. fig. 4.13)...

Fig. 31.17 Chronic active hepatitis in porphyria cutanea tarda. Dispersed light reflection, capsular fibrosis with pronounced net-like fibrosis. Spider-like subcapsular neovascularization...

In porphyria cutanea tarda, iron deposition in the liver is generally low to moderate, predominantly in the hepa-tocytes of the acinar periphery. The cause is thought to be reduced activity of uroporphyrinogen decarboxylase resulting in decreased synthesis of porphyrin and haem. However, this variant of hepatic siderosis only becomes manifest as a result of various triggering factors. Treatment is also based on venesection. 

Bruguera, M. Liver involvement in porphyria. Semin. Dermatol. 1986 5 178-185... 

Gisbert, J.P., Garcia-Buey, L., Pajares, J.M., Moreno-Otero, R. Prevalence of hepatitis C virus infection in porphyria cutanea tarda systematic review and meta-analysis. J. Hepatol. 2003 39 620-627... 

A 75-year-old man with prostatic carcinoma took flutamide for 18 months and developed blisters on the back of the hands and fingers after exposure to the sun (4). The bullae were associated with skin fragility and atrophic scarring. Histopathology and direct immunofluorescence showed ultrastructural features similar to those described in porphyria cutanea tarda. However, porphyrin concentrations in the urine and blood were normal. Flutamide was withdrawn and the lesions healed, without relapse after 11 months. 

Deybach JC, Da Silva V, Nordman Y. The inherited enzymatic defect in porphyria variegate. Hum Genet 1981 58 425-8. 

---