Lipoprotein abnormalities in patients with asymptomatic acute porphyria

Fernández‐Miranda, Consuelo; Calle, María de la; Larumbe, Sagrario; Gómez-Izquierdo, Teresa; Porres, Amelia; Gómez-Gerique, J.A.; Salamanca, Rafael Enrı́quez de

doi:10.1016/s0009-8981(99)00257-0

Cited by 11 publications

(4 citation statements)

References 19 publications

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“…It has been reported that hypercholesterolaemia may be present in patients with porphyria [26] although without atherogenic potential [24]; however, our results demonstrate a good lipid profile and cholesterol in patients with porphyria according to recommendations [14].…”

Section: Discussioncontrasting

confidence: 63%

Assessing nutritional status of acute intermittent porphyria patients

García‐Diz

Murcia

Gris

et al. 2012

Eur J Clin Investigation

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Section: Discussioncontrasting

confidence: 63%

Assessing nutritional status of acute intermittent porphyria patients

García‐Diz

Murcia

Gris

et al. 2012

Eur J Clin Investigation

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“…Patients affected by acute porphyrias show increased incidence of hepatocellular carcinoma, hypertension and chronic renal failure [37][38][39] . On the other hand, it has been reported that hypercholesterolemia and/or hypertriglyceridemia may be present in patients with porphyria but results are contradictory [40][41][42] . Dietary practices may have positive or negative effects on the evolution of such diseases.…”

Section: Discussionmentioning

confidence: 99%

Nutritional Assessment of Patients Affected by Porphyria Variegata

Romaguera

Puigros²,

Palacín³

et al. 2006

Ann Nutr Metab

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Aims: To describe the nutritional status and dietary practices of patients affected by porphyria variegata, paying special attention to the consumption of nutrients that may help or hinder the condition, and to assess the compliance with prevalent nutritional recommendations. Methods: Cross-sectional study. 24 individuals affected by porphyria variegata (16 females and 8 males; mean age 46.8 (SD 19.5) years) from the Balearic Islands (Spain) recruited through the Balearic Porphyria Association. Dietary questionnaires (7-day dietary record and a semi-quantitative food frequency questionnaire), socio-demographic, lifestyle and health status interviews and anthropometric measurements were carried out. Results: According to current recommendations for the Spanish population and to specific recommendations for porphyria patients, the daily intake of saturated fat (13.2% of total energy), sugars (16.9% of total E), cholesterol (387 mg) and alcoholic beverages (1.1 servings per day) were too high whereas the consumption of total carbohydrates (43.5% of total E), vitamin E (69.5% of RDI), beta-carotene (63.1% of RDI) and vitamin D (42.4% of RDI) was lower than recommended. Conclusions: Dietary pattern observed among porphyria variegata patients was in line with current dietary trends in the Balearic Islands. Nutritional recommendations to these individuals for the management of porphyria are poorly met. It is necessary to translate these recommendations into food-based dietary guidelines based on prevailing dietary patterns.

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“…The factors associated with hypercholesterolemia are as follows. First, hypercholesterolemia may be related to porphyria itself and acute AIP attacks (105)(106)(107)(108)(109). Second, hypercholesterolemia is associated with an increase in low-density lipoprotein (LDL) (105,106).…”

Section: Metabolically Related Changesmentioning

confidence: 99%

Acute intermittent porphyria: focus on possible mechanisms of acute and chronic manifestations

Teng

Zhang

et al. 2020

IRDR

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acute intermittent porphyria, mechanisms, aminolevulinic acid (ALA), attack Porphyrias are a group of inherited metabolic diseases that include eight types, each of which is caused by a mutation that affects an enzyme of the heme biosynthetic pathway. When an enzyme defect has physiological significance, it leads to overproduction of pathway precursors prior to the defective step. The partial absence of the third enzyme in the heme biosynthetic pathway, porphobilinogen deaminase (PBGD) also known as hydroxymethylbilane synthase (HMBS), results in acute intermittent porphyria (AIP), which affects mainly women. Subjects who had AIP symptoms were deemed to have manifest AIP (MAIP). Clinical manifestations are usually diverse and non-specific. Acute AIP episodes may present with abdominal pain, nausea, and vomiting, and repeated episodes may result in a series of chronic injuries. Therefore, studying the mechanisms of acute and chronic manifestations of AIP is of great significance. This review aims to summarize the possible mechanisms of acute and chronic manifestations in patients with AIP.

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Lipoprotein abnormalities in patients with asymptomatic acute porphyria

Cited by 11 publications

References 19 publications

Assessing nutritional status of acute intermittent porphyria patients

Assessing nutritional status of acute intermittent porphyria patients

Nutritional Assessment of Patients Affected by Porphyria Variegata

Acute intermittent porphyria: focus on possible mechanisms of acute and chronic manifestations

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