The objective was to evaluate the mortality and the morbidity in neonates operated for persistent ductus arteriosus at Copenhagen University Hospital in the 10-year period from 1 January 1998 to 31 December 2007, and to compare the results with results reported in extant literature.Patient data were collected by retrospective audit of medical charts. Infants with complex heart disease were excluded. The outcomes were mortality, necrotising enterocolitis (NEC), intraventricular haemorrhage (IVH), paresis of the recurrent laryngeal nerve and chylothorax. For statistical analyses, we used Fisher's exact test and Mann-Whitney U test.We included 46 neonates among an initial 60 candidates. Gestational age was from 23 + 6 to 34 + 0 weeks (mean 26 + 6.5) and the birth weight ranged from 535 g to 1,793 g (mean 943.5 g). In 93% of the cases, the left atrium diameter (LAD)/aorta diameter (Ao)-ratio measured by echocardiography was greater than or equal to 1.5, and medical closure had been attempted in 93% of the cases. After surgical ligation, there were three cases of paresis of the recurrent laryngeal nerve, two cases of chylothorax, eight cases of NEC, four cases of IVH and seven deaths (15.2%). The deaths occurred between three and 119 days postoperatively. Those who died were operated at an earlier age than the remaining patients (p < 0.05) and they tended to be of lower gestational age and to have a lower birth weight (p > 0.05) than those who survived.The need for ligation of a patent arterial duct in preterm neonates is subject to considerable risk. Total mortality in this vulnerable group of patients was 15%. Although no obviously procedure-related deaths were observed, severe complications and postoperative morbidity occurred.
The number of congenital heart disease (CHD) patients worldwide is increasing. Eisenmenger syndrome continues developing in patients who, for various reasons, did not undergo early surgery for CHD but may also occur in previously operated patients. When Eisenmenger syndrome develops, repair of the underlying defect is contraindicated. We have previously published the largest to date, international study providing information on contemporary outcomes of Eisenmenger syndrome and identifying factors associated with higher risk of death. We have, herewith, performed further indepth analysis of our data, culminating to an absolute predicted 5-year mortality table for patients with ES, an important aid to optimal care. Selection criteria for lung or heart lung transplantation in Eisenmenger syndrome, in the absence of absolute mortality estimates for conservative management, have been uncertain and this is where our novel risk stratification tool should contribute.
In utero exposure to maternal cancer and cancer treatment might influence the child's cognitive development. This study investigated if exposure to maternal cancer during fetal life impacted school performance and educational achievement as adults.
PURPOSE In utero exposure to maternal cancer and cancer treatment might influence the child's short- and long-term health and development. The objective of the study was to investigate short- and long-term somatic and psychiatric outcomes in children exposed to maternal cancer in utero. METHODS This nationwide cohort study identified all liveborn children in Denmark between January 1978 and December 2018. Exposure was defined as maternal cancer diagnosis during pregnancy, and in a subgroup analysis, exposure to chemotherapy in utero. The main outcomes of interest were overall mortality, somatic diagnoses, and psychiatric diagnoses identified in the National Health Registers. Follow-up started at birth and ended at an event, death, emigration, or end of 2018. Hazard ratios of end points adjusted for potential confounders were estimated using Cox regression analysis. RESULTS Of 2,526,163 included liveborn children, 690 (0.03%) were exposed to maternal cancer in utero. Compared with unexposed fetuses, children exposed in utero had no higher overall mortality, adjusted hazard ratio 0.8 (95% CI, 0.4 to 1.5), nor increased risk of congenital malformations, overall somatic or psychiatric disease. During the period 2002-2018, of 378 (0.03%) children exposed to cancer in utero, 42 (12.5%) were exposed to chemotherapy. Among these 42 children, in utero exposure to chemotherapy was not associated with selected somatic diseases nor to congenital malformations when compared with in utero exposure to maternal cancer without chemotherapy. CONCLUSION Overall, findings did not indicate excess risk of mortality or severe morbidity among children exposed to cancer in utero. Fetal exposure to chemotherapy was not associated with adverse health outcomes in childhood.
We thank Ferrero et al for their interest in our recent publication in Circulation. 1 We aimed in this study to assess mortality and management strategies in contemporary patients with Eisenmenger syndrome (ES).Although the incidence and prevalence of ES seem to decrease in developed countries, it remains worldwide a common complication of congenital heart disease. 2,3There has been considerable progress in the management of ES over the past 1 to 2 decades.Routine phlebotomies, for example, often resulting in iron deficiency, microcytosis, and inappropriately low hemoglobin levels, have been abandoned. 4There is increased awareness of the risks associated with even minor surgery and pregnancy; as a result, fewer patients with ES die of periprocedural complications. 5Last, targeted therapies for pulmonary hypertension have been introduced and helped by improving pulmonary hemodynamics, exercise capacity, quality of life, and seemingly survival.Despite this progress, mortality remains high, as demonstrated in our study, although survival prospects vary significantly between patients.Some patients with ES die in their childhood, whereas others survive into late adulthood.Prognostication in these patients is therefore pivotal and may help in informing patients, optimizing conventional therapy, and, crucially, identifying patients who may benefit from heart and lung or lung transplantation.We elected to use a Cox proportional hazards regression model in our study to test the association of various parameters with mortality.This is currently the most widely used and accepted approach for mortality risk stratification.We have carefully assessed the correlation of parameters tested in the multivariable model to avoid multicollinearity.Although ES is an orphan disease, the multicenter effort enabled analysis of a large cohort of 1098 patients; 278 of them died during the follow-up.We have included in the multivariable model only the parameters available in the vast majority of patients to maintain the ratio of events to parameters at >10 and to avoid overfitting.However, we agree with Ferrero et al that our prognostic model would benefit from external validation.With this in mind, we are currently working on a further study testing our statistical model in a different patient cohort to provide absolute mortality risk estimates in this challenging population.Further progress in mortality risk stratification in patients with congenital heart disease will require multicenter collaboration and analysis of big data, offering sufficient quality and granularity.Although the multivariable model presented in our publication seems promising, in view of future analyses, the results of the univariable analysis require particular attention.This analysis identified a number of parameters significantly associated with mortality that were, however, not available in a significant subset of patients and therefore were not included into the multivariable model.These included 6-minute walk test distance, brain natriuretic peptide, serum albumin, C-reactive protein, and tricuspid plane systolic excursion on echocardiography.We propose that these simple parameters should be incorporated into the routine, periodic assessment of patients with ES both to Response by Kempny et al to Letter
Objectives. To investigate the obstetrical management of cancer in pregnancy and to determine adverse pregnancy and neonatal outcomes. Design. A register-based nationwide historical prospective cohort study. Setting and population. We assessed all pregnancies (N = 4,071,848) in Denmark from 1 January 1973 to 31 December 2018. Methods. We linked data on maternal cancer, obstetrical, and neonatal outcomes. Exposure was defined as pregnancies exposed to maternal cancer (n = 1,068). The control group comprised pregnancies without cancer. The groups were compared using logistic regression analysis and adjusted for potential confounders. Main outcome Measures. The primary outcome was the iatrogenic termination of the pregnancy (induced abortions/labor induction or elective caesarean section). Secondary outcomes were adverse neonatal outcomes. Results. More women with cancer in pregnancy, as compared to the control group, experienced first-trimester induced abortion; adjusted odds ratio (aOR) 3.7 (95% CI 2.8─4.7), second-trimester abortion; aOR 9.0 (6.4─12.6), iatrogenic preterm delivery; aOR 10.9 (8.1─14.7), and iatrogenic delivery below 32 gestational weeks; aOR 16.5 (8.5─32.2). Neonates born to mothers with cancer in pregnancy had a higher risk of respiratory distress syndrome; aOR 1.5 (1.2─2.0), but not of low birth weight; aOR 0.6 (0.4─0.8), admission to neonatal intensive care unit more than seven days; aOR 1.4 (1.1─1.9), neonatal infection; aOR 0.9 (0.5─1.5) nor neonatal mortality; aOR1.3 (0.6─2.6). Conclusion. Cancer in pregnancy implies an increased risk of iatrogenic termination of pregnancy and iatrogenic premature birth. Neonates born to mothers with cancer in pregnancy had no increased risk of severe adverse neonatal outcomes.
INTRODUCTION The objective was to evaluate the mortality and the morbidity in neonates operated for persistent ductus arteriosus at Copenhagen University Hospital in the 10-year period from 1 January 1998 to 31 December 2007, and to compare the results with results reported in extant literature. MATERIAL AND METHODS Patient data were collected by retrospective audit of medical charts. Infants with complex heart disease were excluded. The outcomes were mortality, necrotising enterocolitis (NEC), intraventricular haemorrhage (IVH), paresis of the recurrent laryngeal nerve and chylothorax. For statistical analyses, we used Fisher's exact test and Mann-Whitney U test. RESULTS We included 46 neonates among an initial 60 candidates. Gestational age was from 23 + 6 to 34 + 0 weeks (mean 26 + 6.5) and the birth weight ranged from 535 g to 1,793 g (mean 943.5 g). In 93% of the cases, the left atrium diameter (LAD)/aorta diameter (Ao)-ratio measured by echocardiography was greater than or equal to 1.5, and medical closure had been attempted in 93% of the cases. After surgical ligation, there were three cases of paresis of the recurrent laryngeal nerve, two cases of chylothorax, eight cases of NEC, four cases of IVH and seven deaths (15.2%). The deaths occurred between three and 119 days postoperatively. Those who died were operated at an earlier age than the remaining patients (p 0.05) than those who survived. CONCLUSION The need for ligation of a patent arterial duct in preterm neonates is subject to considerable risk. Total mortality in this vulnerable group of patients was 15%. Although no obviously procedure-related deaths were observed, severe complications and postoperative morbidity occurred.
Glucose transporter 1 deficiency syndrome (GLUT1-DS1) is a rare and complex congenital metabolic encephalopathy characterized by infantile seizures, movement disorder, delayed development and acquired microcephaly. GLUT1-DS1 is most often caused by a
In a patient with primary haemorrhagic thrombocythaemia the initial disappearance of compatible donor platelets from the peripheral blood was markedly increased compared to that of the autologous platelets. Both autologous and heterologous platelets disclosed abnormally short life span. The initial platelet disappearance rate may be used as a supplementary examination for characterisation of platelet abnormalities. In the peripheral blood an unexpected number of cells with structural chromosome aberrations were found. These aberrations were supposedly induced by busulphan therapy 6 years earlier. No clone formation or Ph1-chromosomes were found in the bone marrow.
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