Spontaneous twin anemia polycythemia sequence: diagnosis, management, and outcome in an international cohort of 249 cases

Transfusió intrauterina; Cirurgia làser; Seqüència anemia-policitemia bessonaIntrauterine transfusion; Laser surgery; Twin anemia polycythemia sequenceTransfusión intrauterina; Cirugía láser; Secuencia anemia-policitemia gemelarBackground Twin anemia polycythemia sequence is a chronic form of unbalanced fetofetal transfusion through minuscule placental anastomoses in monochorionic twins, leading to anemia in the donor and polycythemia in the recipient. Owing to the low incidence of twin anemia polycythemia sequence, data on diagnosis, management, and outcome are limited. Objective This study aimed to investigate the diagnosis, management, and outcome in a large international cohort of spontaneous twin anemia polycythemia sequence. Study Design Data from the international twin anemia polycythemia sequence registry, retrospectively collected between 2014 and 2019, were used for this study. A total of 17 fetal therapy centers contributed to the data collection. The primary outcomes were perinatal mortality and severe neonatal morbidity. Secondary outcomes included a risk factor analysis for perinatal mortality and severe neonatal morbidity. Results A total of 249 cases of spontaneous twin anemia polycythemia sequence were included in this study, 219 (88%) of which were diagnosed antenatally and 30 (12%) postnatally. Twin anemia polycythemia sequence was diagnosed antenatally at a median gestational age of 23.7 weeks (interquartile range, 9.7–28.8; range, 15.1–35.3). Antenatal management included laser surgery in 39% (86 of 219), expectant management in 23% (51 of 219), delivery in 16% (34 of 219), intrauterine transfusion (with partial exchange transfusion) in 12% (26 of 219), selective feticide in 8% (18 of 219), and termination of pregnancy in 1% (3 of 219) of cases. Perinatal mortality rate was 15% (72 of 493) for the total group, 22% (54 of 243) for donors, and 7% (18 of 242) for recipients ( P <.001). Severe neonatal morbidity occurred in 33% (141 of 432) of twins with twin anemia polycythemia sequence and was similar for donors (32%; 63 of 196) and recipients (33%; 75 of 228) ( P =.628). Independent risk factors for spontaneous perinatal mortality were donor status (odds ratio, 3.8; 95% confidence interval, 1.9–7.5; P <.001), antenatal twin anemia polycythemia sequence stage (odds ratio, 6.3; 95% confidence interval, 1.4–27.8; P =.016 [stage 2]; odds ratio, 9.6; 95% confidence interval, 2.1–45.5; P =.005 [stage 3]; odds ratio, 20.9; 95% confidence interval, 3.0–146.4; P =.002 [stage 4]), and gestational age at birth (odds ratio, 0.8; 95% confidence interval, 0.7–0.9; P =.001). Independent risk factors for severe neonatal morbidity were antenatal twin anemia polycythemia sequence stage 4 (odds ratio, 7.9; 95% confidence interval, 1.4–43.3; P =.018) and gestational age at birth (odds ratio, 1.7; 95% confidence interval, 1.5–2.1, P <.001). Conclusion Spontaneous twin anemia polycythemia sequence can develop at any time in pregnancy from the beginning of the second trimester to the end of the third trimester. Management for twin anemia polycythemia sequence varies considerably, with laser surgery being the most frequent intervention. Perinatal mortality and severe neonatal morbidity were high, the former especially so in the donor twins

Spontaneous twin anemia polycythemia sequence: diagnosis, management, and outcome in an international cohort of 249 cases.

BACKGROUND: Twin anemia polycythemia sequence is a chronic form of unbalanced fetofetal transfusion through minuscule placental anastomoses in monochorionic twins, leading to anemia in the donor and polycythemia in the recipient. Owing to the low incidence of twin anemia polycythemia sequence, data on diagnosis, management, and outcome are limited. OBJECTIVE: This study aimed to investigate the diagnosis, management, and outcome in a large international cohort of spontaneous twin anemia polycythemia sequence. STUDY DESIGN: Data from the international twin anemia polycythemia sequence registry, retrospectively collected between 2014 and 2019, were used for this study. A total of 17 fetal therapy centers contributed to the data collection. The primary outcomes were perinatal mortality and severe neonatal morbidity. Secondary outcomes included a risk factor analysis for perinatal mortality and severe neonatal morbidity. RESULTS: A total of 249 cases of spontaneous twin anemia polycythemia sequence were included in this study, 219 (88%) of which were diagnosed antenatally and 30 (12%) postnatally. Twin anemia polycythemia sequence was diagnosed antenatally at a median gestational age of 23.7 weeks (interquartile range, 9.7-28.8; range, 15.1-35.3). Antenatal management included laser surgery in 39% (86 of 219), expectant management in 23% (51 of 219), delivery in 16% (34 of 219), intrauterine transfusion (with partial exchange transfusion) in 12% (26 of 219), selective feticide in 8% (18 of 219), and termination of pregnancy in 1% (3 of 219) of cases. Perinatal mortality rate was 15% (72 of 493) for the total group, 22% (54 of 243) for donors, and 7% (18 of 242) for recipients (P<.001). Severe neonatal morbidity occurred in 33% (141 of 432) of twins with twin anemia polycythemia sequence and was similar for donors (32%; 63 of 196) and recipients (33%; 75 of 228) (P=.628). Independent risk factors for spontaneous perinatal mortality were donor status (odds ratio, 3.8; 95% confidence interval, 1.9-7.5; P<.001), antenatal twin anemia polycythemia sequence stage (odds ratio, 6.3; 95% confidence interval, 1.4-27.8; P=.016 [stage 2]; odds ratio, 9.6; 95% confidence interval, 2.1-45.5; P=.005 [stage 3]; odds ratio, 20.9; 95% confidence interval, 3.0-146.4; P=.002 [stage 4]), and gestational age at birth (odds ratio, 0.8; 95% confidence interval, 0.7-0.9; P=.001). Independent risk factors for severe neonatal morbidity were antenatal twin anemia polycythemia sequence stage 4 (odds ratio, 7.9; 95% confidence interval, 1.4-43.3; P=.018) and gestational age at birth (odds ratio, 1.7; 95% confidence interval, 1.5-2.1, P<.001). CONCLUSION: Spontaneous twin anemia polycythemia sequence can develop at any time in pregnancy from the beginning of the second trimester to the end of the third trimester. Management for twin anemia polycythemia sequence varies considerably, with laser surgery being the most frequent intervention. Perinatal mortality and severe neonatal morbidity were high, the former especially so in the donor twins

Anomalies du rythme cardiaque foetal et analgésie péridurale du travail obstétrical

L analgésie péridurale est actuellement la méthode la plus efficace pour soulager les douleurs du travail obstétrical. Cependant elle est associée dans près de 15% des cas à la survenue d anomalies du rythme cardiaque fœtal, le plus souvent en rapport avec une hypotension artérielle maternelle. L utilisation concomitante d ocytociques au cours de l installation d une analgésie péridurale pourrait ainsi induire une augmentation du tonus utérin, qui, associée ou non à une baisse de la pression artérielle, pourrait compromettre le débit utéro-placentaire et ainsi précipiter ces anomalies du rythme cardiaque foetal. Nous avons donc réalisé une étude observationnelle analysant les rythmes cardiaques fœtaux dans l heure suivant la réalisation de l analgésie péridurale obstétricale, en présence ou non d ocytocine. L incidence de survenue d anomalies du rythme cardiaque foetal, ainsi que leurs facteurs prédictifs ont été analysés. Les conséquences obstétricales et néonatales ont été colligées. Enfin, une revue de la littérature sur les conséquences fœtales de l anesthésie péridurale au cours du travail a été réalisée.PARIS6-Bibl.Pitié-Salpêtrie (751132101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Linear IgA dermatosis in association with angioimmunoblastic T-cell lymphoma infiltrating the skin: A case report with literature review.

Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive form of peripheral T-cell lymphoma, characterized by systemic symptoms, diffuse lymphadenopathy, hepatosplenomegaly and immunodysregulation. Half of AITL is associated with cutaneous symptoms, but only few cases with bullous eruption have been described. Association with a linear IgA dermatosis is extremely rare. Linear IgA dermatosis can be idiopathic, or linked with drug intake or neoplastic disorders. Some cases of linear IgA dermatosis presenting as toxic epidermal necrolysis (TEN) have been described, most of them being drug induced. We here present the case of a 72-year-old man recently diagnosed with AITL who developed a bullous eruption, presenting as TEN. Histopathology showed deep cutaneous involvement of the lymphoma with a sub-epidermal blistering and direct immunofluorescence revealed a heavy IgA linear deposit on the dermal-epidermal junction. A diagnosis of linear IgA dermatosis associated with cutaneous involvement of an angioimmunoblastic T-cell lymphoma was made. Chemotherapy and corticosteroids allowed cutaneous improvement but the patient died of his lymphoma shortly after

Prenatal diagnosis of isochromosome 20q in a fetus with vertebral anomaly and rocker-bottom feet

Objective: Isochromosome of the long arm of chromosome 20 (i(20q)) is a rare structural abnormality in prenatal diagnosis. Thirty prenatal cases of mosaic i(20q) have been reported, among which only four are associated with fetal malformations. We describe a new prenatal case of i(20q) with fetal malformations. Materials and methods: We also observed a discrepancy between uncultured and cultured amniotic fluid cells by using conventional cytogenetic, fluorescence in situ hybridization and array-SNP analysis. Results: The short arm deletion of chromosome 20 arising from the isochromosome encompassed two candidate genes PAX1 and JAG1 involved in cranio-facial and vertebral development. Conclusion: The data would allow establishing a phenotype–genotype correlation. Thus, we proposed to define a recognizable syndrome combining cranio-facial dysmorphism, vertebral bodies' anomalies, feet and cerebral malformations

Eruption bulleuse associée à un lymphome T angio-immunoblastique

INTRODUCTION : Le lymphome T angio-immunoblastique (LTAI) est une forme rare et agressive de lymphome T périphérique. Il est caractérisé par des symptômes généraux, des adénopathies diffuses, une hépatosplénomégalie et une hypergammaglobulinémie polyclonale. 50% des LTAI sont accompagnés de symptômes cutanés. Quelques cas d’éruption bulleuse paranéoplasique associée à un LTAI ont été décrits. OBSERVATION : Un homme de 70 ans est admis aux urgences en raison d’une éruption eczématiforme du corps associée à des nécroses des ailes du nez. Le lendemain, il développe des bulles tendues du scrotum et de la langue. Les lésions s’étendent rapidement à l’ensemble du corps. Ce patient est connu des hématologues pour un LTAI stade IVBb, traité par une cure de polychimiothérapie de type CHOP associée au lénalidomide. Plusieurs biopsies sont réalisées : Un infiltrat dermique et hypodermique dense de lymphocytes atypiques à disposition péri-vasculaire et interstitielle est présent à l’histologie des lésions eczématiformes et nécrotiques ; le même type d’infiltrat, surmonté par un décollement bulleux sous-épidermique, est mis en évidence à l’histologie des lésions bulleuses ; d’importants dépôts d’IgA le long de la jonction dermo-épider- mique (JDE), en signal intense, épais et continu, sont constatés à l’immunofluorescence en peau péribulleuse. Ces éléments permettent d’éliminer le diagnostic de toxidermie bulleuse initialement suspecté, et orientent vers une atteinte cutanée du LTAI avec dermatose à IgA linéaire paranéoplasique. L’absence de réponse à la première cure de chimiothérapie impose un changement de traitement du lymphome (gemcitabine associée à des corticoïdes) qui améliore les symptômes cutanés malgré l’absence d’effet bénéfique sur l’état général du patient. DISCUSSION : Les manifestations cutanées accompagnent 50% des LTAI. Outre l’éruption maculopapuleuse la plus souvent décrite, des placards érythémato-squameux, des lésions papulovésiculeuses prurigineuses, du purpura ou encore des lésions urticariennes sont également possibles. Quatre images histologiques sont décrites : infiltrat dense, pléomorphe, de lymphocytes atypiques du derme superficiel et profond avec des hyperplasies vasculaires ; infiltrat périvasculaire léger avec quelques lymphocytes atypiques ; infiltrat superficiel aspécifique ; vasculite leucocytoclasique. La première dominait largement chez ce patient. Les cas de manifestations cutanées avec dépôts d’IgA ne sont que rarement décrits, et seulement 2 cas de décollement sous-épidermique avec dépôts linéaires d’IgA au niveau de la JDE ont, à notre connaissance, été rapportés. CONCLUSION : La dermatose à IgA linéaire surmontant un infiltrat dermique de lymphocytes atypiques est une forme rare de manifestation cutanée associée à un LTAI. Notre patient a présenté une forme à début brutal et extension rapide, améliorée par la chimiothérapie et les soins locaux, malgré la progression du lymphome sous-jacent

Evaluations CE2-sixieme Reperes nationaux septembre 1998

SIGLEAvailable from INIST (FR), Document Supply Service, under shelf-number : 25880, issue : a.1999 n.111 / INIST-CNRS - Institut de l'Information Scientifique et TechniqueFRFranc

Evaluations CE2 - sixieme Reperes nationaux septembre 1997

Available from INIST (FR), Document Supply Service, under shelf-number : 25880, issue : a.1998 n.100 / INIST-CNRS - Institut de l'Information Scientifique et TechniqueSIGLEFRFranc