Peripartum Management of Women with Suspected Hereditary Thrombocytopenia.
Notice bibliographique
Résumé
Introduction: Hereditary macrothrombocytopenic disorders are rare syndromes characterized by mild to moderate thrombocytopenia, large platelets, and a variable bleeding phenotype. In general, the diagnosis must be made clinically and management is empiric. Because of the autosomally inherited nature of these disorders, peripartum management must take into account the risk of bleeding for both mother and baby, during the pregnancy and at delivery. Study Purpose: To describe the peripartum management and bleeding complications in pregnant women with suspected inherited macrothrombocytopenic disorders and their children. Study Design: We performed a retrospective review of all mothers referred to our tertiary care hospital from 2004 to 2007 for evaluation of macrothrombocytopenia in pregnancy where hereditary thrombocytopenia was suspected. The diagnosis was confirmed if at least 2 of the following clinical features were present: life-long thrombocytopenia; family history of thrombocytopenia that spanned at least 2 generations; and the lack of a platelet count response to IVIG or corticosteroids. Data relating to bleeding and therapies used to treat or to prevent bleeding before or during delivery for mother and child were extracted from medical charts. Blood films were reviewed with experts in morphology, and diagnostic testing was performed when possible. Results: A total of 5 mothers and 8 babies were included. The median platelet count of mothers at delivery was 54 x 109/L (range 15–83 x 109/L) and the median MPV was 9.8 fL (range 7.4–11.3 fL). Of the 8 babies, 4 were thrombocytopenic with a median platelet count at birth of 50 x 109/L (range 9–93 x 109/L) and a median MPV of 9.4 fL (range 8.9–9.6fL). One mother and her baby had neutrophilic Dohle body inclusions, and none had skeletal, neurologic or renal abnormalities. Three mothers were previously treated with IVIG, including one who also received prednisone, with no platelet count response. During pregnancy, fetal blood sampling for platelet count measurements was not done. None of the mothers had epidural anesthesia, all delivered vaginally and 7 of 8 labours were induced. Two mothers received prophylactic tranexamic acid at the time of active labour and 1 received DDAVP. Prophylactic platelet transfusions were not given. One mother had bleeding associated with spontaneous rupture of membranes, and one had a post partum hemorrhage associated with uterine atony and vaginal laceration. The latter was repaired surgically and treated with platelet transfusions and DDAVP. None of the babies bled, but 1 was given a platelet transfusion because of severe thrombocytopenia at birth (platelet count = 9 x 109/L) with persistent platelet clumping. Conclusions: The frequency of pregnancy-related bleeding in mothers with hereditary macrothrombocytopenia was low in this cohort even in the absence of prophylaxis. There is a need for improved diagnosis and risk stratification of mothers with hereditary macrothrombocytopenia. A multicentre prospective study would assist in determining optimal peripartum management.
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Comment cette classification a été obtenuedéplier
Prédiction machine sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Le volet Gemma est une étiquette directe du modèle pour chaque travail de la base, lue sur la notice réduite au titre. Le volet Codex est un classifieur appris des 10 348 étiquettes directes de Codex et calibré sur les taux pondérés de l'échantillon; les champs sans appui suffisant ne portent aucun appel Codex. Le mode candidate est l'union des deux volets; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont pas des étiquettes humaines.
Scores du classifieur distillé par catégorie (deux têtes)
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,000 | 0,002 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,000 | 0,000 |
| Bibliométrie | 0,000 | 0,000 |
| Études des sciences et des technologies | 0,001 | 0,000 |
| Communication savante | 0,000 | 0,000 |
| Science ouverte | 0,000 | 0,000 |
| Intégrité de la recherche | 0,001 | 0,000 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,002 | 0,000 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule source (Gemma direct ou Codex distillé), pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».