Cardiac Surgery in Patients with Acute Intermittent Porphyria

Benassi, Filippo; Righi, Elena; Cimato, Paolo; Parravicini, R

doi:10.1111/j.1540-8191.2011.01350.x

Cited by 4 publications

(2 citation statements)

References 11 publications

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“…Acute intermittent porphyria (AIP) is an autosomal dominant metabolic disease caused by gene mutations that results in decreasing heme synthase activity and accumulation of metabolic substrates, leading to neurological dysfunction (1). Primary Sjögren's syndrome (PSS) is a chronic inflammatory autoimmune disease that mainly affects exocrine glands such as the lacrimal and salivary glands (2) and that has a definite familial aggregation.…”

Section: Introductionmentioning

confidence: 99%

The challenge of managing comorbidities: a case report of primary Sjogren's syndrome in a patient with acute intermittent porphyria

Teng

et al. 2020

IRDR

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acute intermittent porphyria, primary Sjogren's syndrome, autoimmune disease, treatment Acute intermittent porphyria (AIP) is a rare inherited metabolic disease associated with heme metabolism. Primary Sjogren's syndrome (PSS) is a common autoimmune disease. The combined presence of AIP and PSS complicates treatment. A rare case of concomitant AIP and PSS is reported in this paper. A 30-year-old woman with AIP had recurrent acute abdominal pain, nausea and vomiting, constipation, persistent chest, back, and waist pain, red urine, positivity for porphobilinogen (PBG) in urine and a pathogenic mutation of the HMBS gene. Two and a half years after she was diagnosed with AIP, she was diagnosed with PSS based on dryness of the eyes and mouth, the elevation of immunoglobulins (IgG and IgA) and positive results on an anti-SS-A antibody test, an anti-SS-B antibody test, Schirmer's test and a labial gland biopsy. A mutation in the HMBS gene was detected in the patient and her cousin, but the patient had more severe AIP and more severe symptoms (such as epilepsy and a limp), which may be related to the co-morbidity of PSS. According to her PSS activity score, the patient had an ESSDAI score of 9 and required systemic treatment. However, potential medications were limited by AIP, so mycophenolate mofetil was eventually added to delay the progression of the primary disease.

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Section: Introductionmentioning

confidence: 99%

The challenge of managing comorbidities: a case report of primary Sjogren's syndrome in a patient with acute intermittent porphyria

Teng

et al. 2020

IRDR

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“…Its inheritance is autosomal dominant. [ 1 ] A deficiency of porphobilinogen deaminase is not sufficient by its self to produce AIP, and other activating factors must also be present. These include some drugs, hormones, infection, injury, stress, dehydration, fasting, puberty, and alcohol.…”

Section: Introductionmentioning

confidence: 99%

Anesthetic implication of tricuspid valve replacement in a patient with acute intermittent porphyria

et al. 2016

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Facing a patient with acute intermittent porphyria (AIP), there is narrow safety margin which circumscribe all the therapeutic actions including choice of drugs. This would become even more complicated when it comes to a stressful and drug-dependent process like a cardiopulmonary bypass. According to author's researches, no specific AIP case of tricuspid valve (TV) replacement is reported recently. Furthermore, fast-track anesthesia was safely used in this 37-year-old male known the case of AIP, who was a candidate for TV replacement and removing the port catheter. The patient was extubated subsequently, only 3 h after entering the Intensive Care Unit.

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Ataque agudo de porfiria posterior a cirugía de reemplazo valvular mitral: a propósito de caso

Lara¹,

Villa²,

Rosales

et al. 2022

Acta Colombiana de Cuidado Intensivo

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Cardiac Surgery in Patients with Acute Intermittent Porphyria

Cited by 4 publications

References 11 publications

The challenge of managing comorbidities: a case report of primary Sjogren's syndrome in a patient with acute intermittent porphyria

The challenge of managing comorbidities: a case report of primary Sjogren's syndrome in a patient with acute intermittent porphyria

Anesthetic implication of tricuspid valve replacement in a patient with acute intermittent porphyria

Ataque agudo de porfiria posterior a cirugía de reemplazo valvular mitral: a propósito de caso

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