Comment diagnostiquer une porphyrie ?

Le diagnostic repose sur des tests sanguins et urinaires pour mesurer les niveaux de porphyrines.

Quels tests sont utilisés pour les porphyrines ?

Les tests incluent l'analyse des urines, du sang et parfois des biopsies de peau.

Quels symptômes indiquent une porphyrie ?

Les symptômes peuvent inclure des douleurs abdominales, des troubles neurologiques et des photosensibilités.

Les tests génétiques sont-ils nécessaires ?

Oui, des tests génétiques peuvent aider à confirmer certains types de porphyries héréditaires.

Comment interpréter les résultats des tests ?

Des niveaux élevés de porphyrines dans le sang ou l'urine indiquent une porphyrie.

Quels sont les symptômes courants des porphyries ?

Les symptômes incluent des douleurs abdominales, des éruptions cutanées et des troubles mentaux.

Les porphyries causent-elles des douleurs ?

Oui, des douleurs abdominales aiguës sont fréquentes lors des crises de porphyrie.

Y a-t-il des symptômes cutanés ?

Oui, des éruptions cutanées et une photosensibilité peuvent survenir, surtout dans la porphyrie cutanée.

Les porphyries affectent-elles le système nerveux ?

Oui, elles peuvent provoquer des troubles neurologiques, y compris des crises et des hallucinations.

Les symptômes varient-ils selon le type de porphyrie ?

Oui, chaque type de porphyrie présente des symptômes spécifiques et des manifestations cliniques.

Comment prévenir les crises de porphyrie ?

Éviter les déclencheurs comme certains médicaments, l'alcool et le stress peut aider.

Y a-t-il des conseils diététiques pour les patients ?

Une alimentation équilibrée et riche en glucides peut aider à prévenir les crises.

Les patients doivent-ils éviter certains médicaments ?

Oui, certains médicaments peuvent déclencher des crises et doivent être évités.

La gestion du stress est-elle importante ?

Oui, la gestion du stress est cruciale pour prévenir les crises de porphyrie.

Les patients doivent-ils être suivis régulièrement ?

Oui, un suivi médical régulier est essentiel pour surveiller l'évolution de la maladie.

Quel est le traitement principal des porphyries ?

Le traitement principal inclut l'évitement des déclencheurs et des médicaments pour soulager les symptômes.

Les transfusions sanguines sont-elles nécessaires ?

Dans certains cas, des transfusions peuvent être nécessaires pour traiter l'anémie associée.

Y a-t-il des médicaments spécifiques pour les porphyries ?

Des médicaments comme l'hématine peuvent être utilisés pour traiter les crises aiguës de porphyrie.

Comment gérer les crises de porphyrie ?

Les crises peuvent être gérées par des soins d'urgence et des traitements symptomatiques.

La photoprotection est-elle recommandée ?

Oui, les patients doivent éviter l'exposition au soleil et utiliser des écrans solaires.

Quelles sont les complications possibles des porphyries ?

Les complications incluent des lésions nerveuses, des problèmes hépatiques et des infections.

Les porphyries peuvent-elles causer des dommages au foie ?

Oui, certaines porphyries peuvent entraîner des lésions hépatiques graves.

Y a-t-il un risque accru de cancer ?

Certaines porphyries, comme la porphyrie cutanée, peuvent augmenter le risque de cancer de la peau.

Les complications neurologiques sont-elles fréquentes ?

Oui, des complications neurologiques peuvent survenir, affectant la qualité de vie.

Comment gérer les complications des porphyries ?

La gestion des complications nécessite un suivi médical et des traitements adaptés.

Quels sont les facteurs de risque des porphyries ?

Les facteurs incluent des antécédents familiaux, certains médicaments et des conditions hépatiques.

L'hérédité joue-t-elle un rôle ?

Oui, certaines porphyries sont héréditaires et peuvent être transmises dans les familles.

Les femmes sont-elles plus à risque ?

Certaines études montrent que les femmes peuvent être plus touchées par certaines porphyries.

L'alcool augmente-t-il le risque ?

Oui, la consommation d'alcool peut déclencher des crises chez les personnes prédisposées.

Les infections peuvent-elles aggraver la condition ?

Oui, certaines infections peuvent exacerber les symptômes des porphyries.

Porphyrines - Informations médicales vérifiées

How do I/we forecast tomorrows' transfusion: Blood components.

Feb 2023

DOI: 10.1016/j.tracli.2022.07.006 PMID: 35914702

The current implementation of Pathogen Reduction Technologies (PRTs) offers advantages and disadvantages to transfusion medicine. PRT rollout may significantly reduce the incidence of transfusion-tran...

Humans Blood Component Transfusion Blood Platelets Hemorrhage Blood Preservation +2 autres

Balanced blood component resuscitation in trauma: Does it matter equally at different transfusion volumes?

05 2023

DOI: 10.1016/j.surg.2022.11.010 PMID: 36528406

It remains unclear whether the association between balanced blood component transfusion and lower mortality is generalizable to trauma patients receiving varying transfusion volumes. We sought to stud... Adult patients in the 2013 to 2018 American College of Surgeons Trauma Quality Improvement Program database receiving ≥6 red blood cell, ≥1 platelet, and ≥1 fresh frozen plasma within 4 hours were inc... A total of 14,549 patients were included. In patients receiving 6 to 10 units of red blood cells, red blood cell:platelet ratios were not associated with 4-hour mortality, and only red blood cell:fres... The association between balanced blood component transfusion and 4-hour mortality is not homogenous in trauma patients requiring different transfusion volumes and is specifically less evident in patie...

Adult Humans Blood Transfusion Blood Component Transfusion Erythrocyte Transfusion +5 autres

Influence of the leukoreduction moment of blood components on the clinical outcomes of transfused patients in the emergency department.

2024

DOI: 10.1590/0034-7167-2023-0293 PMID: 39194129

to investigate the influence of the leukoreduction moment (preor post-storage) of blood components on the clinical outcomes of patients transfused in the emergency department.... retrospective cohort study of patients aged 18 years or older who received preor post-storage leukoreduced red blood cell or platelet concentrate in the emergency department and remained in the instit... in a sample of 373 patients (63.27% male, mean age 54.83) and 643 transfusions (69.98% red blood cell), it was identified that the leukoreduction moment influenced the length of hospital stay (p<0.009... patients who received pre-storage leukoreduced blood components in the emergency department had a shorter length of hospital stay....

Humans Male Female Emergency Service, Hospital Retrospective Studies +9 autres

Hypocalcemia in Trauma is Determined by the Number of Units Transfused, Not Whole Blood Versus Component Therapy.

09 2023

DOI: 10.1016/j.jss.2023.03.043 PMID: 37148855

Blood component resuscitation is associated with hypocalcemia (HC) (iCal <0.9 mmol/L) that contributes to coagulopathy and death in trauma patients. It is unknown whether or not whole blood (WB) resus... This is a retrospective review of all adult trauma patients who received WB from July 2018 to December 2020. Variables included transfusions, ionized calcium levels, and calcium replacement. Patients ... Two hundred twenty-three patients received WB and met the inclusion criteria. 107 (48%) received WB only. HC occurred in 13% of patients who received more than one WB unit compared to 29% of WB and ot... HC and failure to correct HC are significant risk factors for mortality in trauma. Resuscitations with WB only and WB in combination with other blood components are associated with HC especially when ...

Adult Humans Hypocalcemia Calcium Blood Transfusion +5 autres

Parents' understanding and experiences of blood component transfusion in the neonatal intensive care unit: A qualitative study.

12 2023

DOI: 10.1111/apa.16952 PMID: 37615240

Blood component transfusion is a common intervention in the neonatal intensive care unit (NICU). Parents consent on their babies' behalf. This study aimed to explore parents' understandings and experi... A "low inference" qualitative descriptive semi-structured interview approach was utilised. Grounded theory was employed. Parents described their memories of babies' transfusions, their responses to th... A purposive sample of 17 parents whose babies required blood transfusion in the NICU participated. Parents talked about their initial fears of transfusion, later replaced by confidence in the process ... Parents in our study trust information from the healthcare professionals caring for their baby and would like more specific information about how blood transfusion will impact their baby, in a variety...

Infant, Newborn Infant Humans Intensive Care Units, Neonatal Parents +4 autres

Blood component-associated acute transfusion reactions in pediatric patients: experience of a tertiary care hospital.

2024

DOI: 10.55730/1300-0144.5869 PMID: 39473757

The transfusion of blood products is a life-saving clinical practice in patients with bleeding, hemoglobinopathy, and cancer. It was aimed herein to analyze the frequency and types of blood component-... This retrospective study was conducted at a tertiary care academic pediatric hospital.... During the study period, 30,811 transfusions were administered to 25,448 patients. There were 103 ATRs detected in 81 patients (0.33%; 3.34 reactions per 1000 transfusions, mean age 8.3 ± 5.98 years, ... Within our hospital, pediatric hematology-oncology wards and the stem cell transplantation unit had the most frequent ATR reports; therefore, when transfusions are carried out, increased attention sho...

Humans Child Female Male Retrospective Studies +6 autres

Transfusion-related cost comparison of trauma patients receiving whole blood versus component therapy.

01 07 2023

DOI: 10.1097/TA.0000000000003933 PMID: 36973870

With the emergence of whole blood (WB) in trauma resuscitation, cost-related comparisons are of significant importance to providers, blood banks, and hospital systems throughout the country. The objec... A retrospective review of adult and pediatric trauma patients who received either LTO+WB or CT from time of injury to within 4 hours of arrival was performed. Annual mean cost per unit of blood produc... Prehospital LTO+WB transfusion began at this institution in January 2018. After the initiation of the WB transfusion, the mean annual cost decreased 17.3% for all blood products, and the average net d... With increased use of LTO+WB for resuscitation, cost comparison is of significant importance to all stakeholders. Low titer O+ WB was associated with reduced cost in severely injured patients. Ongoing... Therapeutic/Care Management; Level IV....

Adult Humans Child Blood Transfusion Blood Banks +5 autres

The abrogated role of premedication in the prevention of transfusion-associated adverse reactions in outpatients receiving leukocyte-reduced blood components.

Oct 2022

DOI: 10.1111/vox.13333 PMID: 35843874

Although it remains controversial, premedication before transfusion is a common clinical practice to prevent transfusion-associated adverse reactions (TAARs) in Taiwan. Thus, we aimed to investigate w... Clinical data from outpatients receiving transfusion therapy, including predisposing diseases, histories of transfusion and TAARs, premedication and the occurrence of TAARs in the period April 2017 to... A total of 5018 blood units were transfused to 803 outpatients, with 2493 transfusion events reported in the study interval. The most frequently transfused component was leukocyte-reduced packed red c... Decreased premedication was not associated with increased incidence of TAARs in outpatients; these findings provide important evidence to support the need to revise clinical practices in the era of le...

Blood Component Transfusion Blood Transfusion Humans Leukocytes Outpatients +1 autres

How to improve issuing, transfusion and follow-up of blood components in Southern and Eastern Mediterranean countries? A benchmark assessment.

Apr 2023

DOI: 10.1016/j.transci.2022.103616 PMID: 36470725

To determine the existence of guidelines regarding the appropriate clinical use of blood and blood components, transfusion requests, and blood issuing/reception documents and procedures. The different...

Humans Benchmarking Follow-Up Studies Blood Transfusion Blood Component Transfusion +1 autres

Outcomes of Transfusion With Whole Blood, Component Therapy, or Both in Adult Civilian Trauma Patients: A Systematic Review and Meta-Analysis.

07 2023

DOI: 10.1016/j.jss.2023.02.010 PMID: 36947979

This systematic review and meta-analysis was conducted to compare outcomes, including transfusion volume, complications, intensive care unit length of stay, and mortality for adult civilian trauma pat... A systematic review and meta-analysis were conducted using studies that evaluated outcomes of transfusion of WB, COMP, or WB + COMP for adult civilian trauma patients. A search of PubMed, Embase, and ... This study identified an increased risk of 24-h mortality with COMP versus WB + COMP (relative risk: 1.40 [1.10, 1.78]) and increased transfusion volumes of red blood cells with COMP versus WB at 6 an... Transfusion with WB + COMP is associated with lower 24-h mortality versus COMP and transfusion with WB is associated with a lower volume of red blood cells transfused at both 6 and 24 h. Based on thes...

Humans Adult Blood Transfusion Blood Component Transfusion Resuscitation +3 autres

Porphyrines : Questions médicales fréquentes

Termes MeSH sélectionnés :

Questions fréquentes et termes MeSH associés

Diagnostic 5

Symptômes 5

Prévention 5

Traitements 5

Complications 5

Facteurs de risque 5

Contenu validé par Dr Olivier Menir

Sous-catégories

Coproporphyrines

Deutéroporphyrines

Étioporphyrines

Hématoporphyrines

Mésoporphyrines

Métalloporphyrines

Protoporphyrines

Uroporphyrines

Hématoporphyrine D

Hème

Éther de dihématoporphyrine

Hémine

Auteurs principaux

Sources (10000 au total)