ABSTRACT
BACKGROUNDS: Team leadership skills of physicians working in high-performing medical teams are directly related to outcome. It is currently unclear how these skills can best be developed. Therefore, in this multi-national cross-sectional prospective study, we explored the development of these skills in relation to physician-, organization- and training characteristics of Helicopter Emergency Medicine Service (HEMS) physicians from services in Europe, the United States of America and Australia. METHODS: Physicians were asked to complete a survey regarding their HEMS service, training, and background as well as a full Leader Behavior Description Questionnaire (LBDQ). Primary outcomes were the 12 leadership subdomain scores as described in the LBDQ. Secondary outcome measures were the association of LBDQ subdomain scores with specific physician-, organization- or training characteristics and self-reported ways to improve leadership skills in HEMS physicians. RESULTS: In total, 120 HEMS physicians completed the questionnaire. Overall, leadership LBDQ subdomain scores were high (10 out of 12 subdomains exceeded 70% of the maximum score). Whereas physician characteristics such as experience or base-specialty were unrelated to leadership qualities, both organization- and training characteristics were important determinants of leadership skill development. Attention to leadership skills during service induction, ongoing leadership training, having standards in place to ensure (regular) scenario training and holding structured mission debriefs each correlated with multiple LBDQ subdomain scores. CONCLUSIONS: Ongoing training of leadership skills should be stimulated and facilitated by organizations as it contributes to higher levels of proficiency, which may translate into a positive effect on patient outcomes. TRIAL REGISTRATION: Not applicable.
Subject(s)
Leadership , Humans , Prospective Studies , Cross-Sectional Studies , Male , Female , Surveys and Questionnaires , Patient Care Team/organization & administration , Adult , Clinical Competence , Emergency Medical Services/organization & administration , Middle Aged , Emergency Medicine/education , Emergency Medicine/organization & administration , Air Ambulances/organization & administration , United States , EuropeABSTRACT
Early haemorrhage control and minimizing the time to definitive care have long been the cornerstones of therapy for patients exsanguinating from non-compressible haemorrhage (NCH) after penetrating injuries, as only basic treatment could be provided on scene. However, more recently, advanced on-scene treatments such as the transfusion of blood products, resuscitative thoracotomy (RT) and resuscitative endovascular balloon occlusion of the aorta (REBOA) have become available in a small number of pre-hospital critical care teams. Although these advanced techniques are included in the current traumatic cardiac arrest algorithm of the European Resuscitation Council (ERC), published in 2021, clear guidance on the practical application of these techniques in the pre-hospital setting is scarce. This paper provides a scoping review on how these advanced techniques can be incorporated into practice for the resuscitation of patients exsanguinating from NCH after penetrating injuries, based on available literature and the collective experience of several helicopter emergency medical services (HEMS) across Europe who have introduced these advanced resuscitation interventions into routine practice.
Subject(s)
Balloon Occlusion , Endovascular Procedures , Balloon Occlusion/methods , Endovascular Procedures/methods , Hemorrhage/etiology , Hemorrhage/therapy , Hospitals , Humans , Resuscitation/methodsABSTRACT
The conduct and benefit of pre-hospital advanced airway management and pre-hospital emergency anaesthesia have been widely debated for many years. In 2011, prehospital advanced airway management was identified as a 'top five' in physician-provided pre-hospital critical care. This article summarises the evidence for and against this intervention since 2011 and attempts to address some of the more controversial areas of this topic.
Subject(s)
Airway Management , Anesthesia , Critical Care , Emergency Medical Services , HumansABSTRACT
BACKGROUND: On-scene management of pre-hospital emergencies is often inter-disciplinary, involving ground-emergency medical services (EMS), police- and fire services, and in Norway general practitioners on-call. This can also be supplemented by physician-staffed EMS (P-EMS), utilizing helicopters or rapid response vehicles. We hypothesized that P-EMS cooperates extensively with other emergency services, and therefore the primary aim of this study was to investigate the fraction of inter-disciplinary cooperation between P-EMS and other emergency services. METHODS: Retrospective, observational study of primary pre-hospital missions with patient contact performed at a Norwegian P-EMS base from 01.01.06 to 31.12.15. Descriptive statistics, comparisons using Student`s t-test, and chi-squared test for trend were applied. RESULTS: Inter-disciplinary cooperation occurred in 94.3% of the 8580 missions, of which physician-staffed EMS cooperated with ground EMS in 92.4%, general practitioner 32.9%, police service 11.6% and fire service 11.8%. Trauma constituted 34.4 and cardiac arrest 14.1% of missions. The mean National Advisory Committee for Aeronautics score was 4.21 (95% Confidence Interval 4.18-4.24). There was an overall decrease in cooperation with general practitioners and the police service (P < 0.001). During helicopter missions, we reported a decrease in general practitioner cooperation compared to an increase during rapid response car missions (P < 0.001). In cardiac arrest cases, cooperation with both general practitioners and the fire service increased (P < 0.001). CONCLUSION: Physician-staffed EMS cooperates extensively with other professional emergency services, especially ground-EMS. On-scene cooperation with general practitioners decreased, whereas there was an increased cooperation with the fire service in a "first-responder" role during cardiac arrest missions.
Subject(s)
Emergency Medical Services , Intersectoral Collaboration , Physicians , Adolescent , Adult , Aged , Aged, 80 and over , Child , Child, Preschool , Female , General Practitioners , Humans , Infant , Infant, Newborn , Male , Middle Aged , Retrospective Studies , Young AdultABSTRACT
BACKGROUND: The benefits of pre-hospital emergency anaesthesia (PHEA) are controversial. Patients who are hypovolaemic prior to induction of anaesthesia are at risk of severe cardiovascular instability post-induction. This study compared mortality for hypovolaemic trauma patients (without major neurological injury) undergoing PHEA with a patient cohort with similar physiology transported to hospital without PHEA. METHODS: A retrospective database review was performed to identify patients who were hypotensive on scene [systolic blood pressure (SBP) < 90 mmHg], and GCS 13-15. Patient records were reviewed independently by two pre-hospital clinicians to identify the likelihood of hypovolaemia. Primary outcome measure was mortality defined as death before hospital discharge. RESULTS: Two hundred and thirty-six patients were included; 101 patients underwent PHEA. Fifteen PHEA patients died (14.9%) compared with six non-PHEA patients (4.4%), P = 0.01; unadjusted OR for death was 3.73 (1.30-12.21; P = 0.01). This association remained after adjustment for age, injury mechanism, heart rate and hypovolaemia (adjusted odds ratio 3.07 (1.03-9.14) P = 0.04). Fifty-eight PHEA patients (57.4%) were hypovolaemic prior to induction of anaesthesia, 14 died (24%). Of 43 PHEA patients (42.6%) not meeting hypovolaemia criteria, one died (2%); unadjusted OR for mortality was 13.12 (1.84-578.21). After adjustment for age, injury mechanism and initial heart rate, the odds ratio for mortality remained significant at 9.99 (1.69-58.98); P = 0.01. CONCLUSION: Our results suggest an association between PHEA and in-hospital mortality in awake hypotensive trauma patients, which is strengthened when hypotension is due to hypovolaemia. If patients are hypovolaemic and awake on scene it might, where possible, be appropriate to delay induction of anaesthesia until hospital arrival.
Subject(s)
Anesthesia , Emergency Medical Services , Hypotension/complications , Wounds and Injuries/complications , Adult , Hemodynamics , Hospital Mortality , Humans , Hypotension/physiopathology , Retrospective Studies , Wakefulness , Wounds and Injuries/physiopathologyABSTRACT
INTRODUCTION: The current management of severely injured patients includes damage control resuscitation strategies that minimise the use of crystalloids and emphasise earlier transfusion of red blood cells (RBC) to prevent coagulopathy. In 2012, London's air ambulance (LAA) became the first UK civilian pre-hospital service to routinely carry RBC to the trauma scene. OBJECTIVE: To investigate the effect of pre-hospital RBC transfusion (phRTx) on overall blood product consumption. METHODS: A retrospective trauma database study compares before implementation with after implementation of phRTx in exsanguinating trauma patients transported directly to one major trauma centre. Pre-hospital deaths were excluded. Univariate and multivariate Poisson regression analyses on data subject to multiple imputation were conducted. RESULTS: We included 137 and 128 patients in the before and after the implementation of phRTx groups, respectively. LAA transfused 304 RBC units (median 2, inter quartile range 1-3). We found a significant reduction in total RBC usage and reduced early use of platelets and fresh-frozen plasma (FFP) after the implementation of phRTx in both univariate (P < 0·001) and multivariate analyses (P < 0·001). No immediate adverse transfusion reactions were identified. CONCLUSION: Pre-hospital trauma transfusion practice is feasible and associated with overall reduced RBC, platelets and FFP consumption.
Subject(s)
Blood Coagulation Disorders/therapy , Erythrocyte Transfusion , Plasma , Platelet Transfusion , Wounds and Injuries/therapy , Adult , Blood Coagulation Disorders/blood , Female , Humans , London , Male , Retrospective Studies , Trauma Centers , Wounds and Injuries/bloodABSTRACT
BACKGROUND: Pre-hospital basic airway interventions can be ineffective at providing adequate oxygenation and ventilation in some severely ill or injured patients, and advanced airway interventions are then required. Controversy exists regarding the level of provider required to perform successful pre-hospital intubation. A previous meta-analysis reported pre-hospital intubation success rates of 0.849 for non-physicians versus 0.991 for physicians. The evidence base on the topic has expanded significantly in the last 10 years. This study systematically reviewed recent literature and presents comprehensive data on intubation success rates. METHODS: A systematic search of MEDLINE and EMBASE was performed using PRISMA methodology to identify articles on pre-hospital tracheal intubation published between 2006 and 2016. Overall success rates were estimated using random effects meta-analysis. The relationship between intubation success rate and provider type was assessed in weighted linear regression analysis. RESULTS: Of the 1838 identified studies, 38 met the study inclusion criteria. Intubation was performed by non-physicians in half of the studies and by physicians in the other half. The crude median (range) reported overall success rate was 0.969 (0.616-1.000). In random effects meta-analysis, the estimated overall intubation success rate was 0.953 (0.938-0.965). The crude median (range) reported intubation success rates for non-physicians were 0.917 (0.616-1.000) and, for physicians, were 0.988 (0.781-1.000) (p = 0.003). DISCUSSION: The reported overall success rate of pre-hospital intubation has improved, yet there is still a significant difference between non-physician and physician providers. The finding that less-experienced personnel perform less well is not unexpected, but since there is considerable evidence that poorly performed intubation carries a significant risk of morbidity and mortality careful consideration should be given to the training and experience required to deliver this intervention safely.
Subject(s)
Emergency Medical Services , Health Personnel/standards , Intubation, Intratracheal/standards , Emergency Medical Services/methods , Emergency Medical Services/standards , Humans , Intubation, Intratracheal/methods , WorkforceABSTRACT
BACKGROUND: The Scandinavian society of anaesthesiology and intensive care medicine task force on pre-hospital airway management was asked to formulate recommendations following standards for trustworthy clinical practice guidelines. METHODS: The literature was systematically reviewed and the grading of recommendations assessment, development and evaluation (GRADE) system was applied to move from evidence to recommendations. RESULTS: We recommend that all emergency medical service (EMS) providers consider to: apply basic airway manoeuvres and airway adjuncts (good practice recommendation); turn unconscious non-trauma patients into the recovery position when advanced airway management is unavailable (good practice recommendation); turn unconscious trauma patients to the lateral trauma position while maintaining spinal alignment when advanced airway management is unavailable [strong recommendation, low quality of evidence (QoE)]. We suggest that intermediately trained providers use a supraglottic airway device (SAD) or basic airway manoeuvres on patients in cardiac arrest (weak recommendation, low QoE). We recommend that advanced trained providers consider using an SAD in selected indications or as a rescue device after failed endotracheal intubation (ETI) (good practice recommendation). We recommend that ETI should only be performed by advanced trained providers (strong recommendation, low QoE). We suggest that videolaryngoscopy is considered for ETI when direct laryngoscopy fails or is expected to be difficult (weak recommendation, low QoE). We suggest that advanced trained providers apply cricothyroidotomy in 'cannot intubate, cannot ventilate' situations (weak recommendation, low QoE). CONCLUSION: This guideline for pre-hospital airway management includes a combination of techniques applied in a stepwise fashion appropriate to patient clinical status and provider training.
Subject(s)
Airway Management/methods , Emergency Medical Services/methods , Practice Guidelines as Topic , Humans , Scandinavian and Nordic Countries , Societies, MedicalABSTRACT
BACKGROUND: A mutation found in the BRCA1 or BRCA2 gene of a breast tumor could be either germline or somatically acquired. The prevalence of somatic BRCA1/2 mutations and the ratio between somatic and germline BRCA1/2 mutations in unselected breast cancer patients are currently unclear. PATIENTS AND METHODS: Paired normal and tumor DNA was analyzed for BRCA1/2 mutations by massively parallel sequencing in an unselected cohort of 273 breast cancer patients from south Sweden. RESULTS: Deleterious germline mutations in BRCA1 (n = 10) or BRCA2 (n = 10) were detected in 20 patients (7%). Deleterious somatic mutations in BRCA1 (n = 4) or BRCA2 (n = 5) were detected in 9 patients (3%). Accordingly, about 1 in 9 breast carcinomas (11%) in our cohort harbor a BRCA1/2 mutation. For each gene, the tumor phenotypes were very similar regardless of the mutation being germline or somatically acquired, whereas the tumor phenotypes differed significantly between wild-type and mutated cases. For age at diagnosis, the patients with somatic BRCA1/2 mutations resembled the wild-type patients (median age at diagnosis, germline BRCA1: 41.5 years; germline BRCA2: 49.5 years; somatic BRCA1/2: 65 years; wild-type BRCA1/2: 62.5 years). CONCLUSIONS: In a population without strong germline founder mutations, the likelihood of a BRCA1/2 mutation found in a breast carcinoma being somatic was â¼1/3 and germline 2/3. This may have implications for treatment and genetic counseling.
Subject(s)
BRCA1 Protein/genetics , BRCA2 Protein/genetics , Breast Neoplasms/genetics , Adult , Aged , Breast Neoplasms/epidemiology , Breast Neoplasms/pathology , Female , Genetic Predisposition to Disease , Germ-Line Mutation , Humans , Middle Aged , Mutation , Sweden/epidemiologyABSTRACT
Objective: The increasing prevalence of obesity is having an impact on morbidity worldwide. Since young mature women are equally affected by the general increase in weight, the aim of the study was to evaluate the prevalence of obesity together with associated maternal risk factors, complications during pregnancy, and fetal outcomes in a local cohort for the years 2006 and 2011. Study Design: Maternal and fetal records of women who delivered at the University of Würzburg, with a 5-year interval (2006 and 2011) between investigations, were retrospectively analyzed. Descriptive statistics included prevalence of obesity, maternal weight gain, as well as several complications during pregnancy and fetal characteristics. The association between maternal or fetal complications and extent of maternal obesity was analyzed. Results: Our analysis included 2838 mothers with singleton pregnancies who delivered in 2006 (n = 1293) or 2011 (n = 1545) in our department. We found that neither pre-pregnancy body mass index (23.77 ± 4.85 vs. 24.09 ± 5.10 kg/m2, p = 0.25) nor weight gain (14.41 ± 5.77 vs. 14.78 ± 5.65 kg; p = 0.09) increased significantly over time. But the majority of all overweight (71â%) or obese (60.4â%) mothers gained more weight than generally recommended. The prevalence of gestational diabetes, gestational hypertension, and preeclampsia increased significantly and was associated with high pre-pregnancy body mass index, as was delivery by cesarean section. However, obesity was not associated with prolonged pregnancy and did not seem to negatively affect fetal outcome. Conclusion: There is a trend to increasing weight gain during pregnancy, and the majority of mothers, especially those with a high pre-pregnancy body mass index, exceeded the weight gain recommendations. Associated risk factors such as gestational diabetes, hypertension, and delivery by cesarean section are increasing.
ABSTRACT
Significant placental insufficiency, indicated by Doppler ultrasound findings of absent or reverse end-diastolic flow velocities (AREDV), is associated with increased morbidity and mortality. Analysis of blood flow in the ductus venosus should assist in early intrauterine recognition of threatened foetuses. 58 high-risk pregnancies with umbilical AREDV were repeatedly examined (n=364). Doppler findings were correlated with neonatal signs of deterioration (ratio of normoblasts to leukocytes, pH, base excess, Apgar score), as well as short-term morbidity [need for intubation, duration of assisted respiration, evidence of respiratory distress syndrome (RDS), bronchopulmonary dysplasia (BPD), necrotising enterocolitis (NEC), intraventricular haemorrhage (IVH grade III+IV)] against the analysis of the blood flow findings (normal or increased pulsitility, absence or reverse end-diastolic flow) in the umbilical arteries (AU), the middle cerebral arteries (ACM) and ductus venosus (DV) relating these to birth weight and the duration of the pregnancy. The median period of observation was 12.8 days, 48% of the foetuses showed an abnormal ductus venosus flow and 26% an absent venous or reverse end-diastolic flow. The median date of delivery was 30 weeks, with a mean birth weight of 816 g. 93% were live births with 12% dying postnatally. Although the criteria for postnatal morbidity (BPD, NEC, IVH III+IV) and mortality did not correlate with changes in arterial and venous Doppler parameters in our group, there was a significant relationship between the normoblast count, known to be a marker of chronic hypoxia. The Apgar 10 minte score, umbilical arterial pH and base excess were correlated with changes in the DV flow curves. Healthy survival started, irrespective of arterial or venous blood flow criteria, from 27+0 weeks of pregnancy. If born between 27.0 and 30+6 weeks, the infants were more likely to be healthy the less the blood flow had been compromised. A birth weight of 590 g (sensitivity 62.5%; specificity 93.5%) and gestational age of 28+5 weeks (sensitivity 87.5%; specificity 90.3%) were shown to be cut-off points between healthy survival and survival with serious neonatal complications.
Subject(s)
Fetomaternal Transfusion/diagnostic imaging , Fetomaternal Transfusion/mortality , Placental Insufficiency/diagnostic imaging , Placental Insufficiency/mortality , Pregnancy Outcome/epidemiology , Ultrasonography, Doppler/statistics & numerical data , Female , Fetal Death , Germany/epidemiology , Humans , Infant, Newborn , Perinatal Mortality , Pregnancy , Prognosis , Reproducibility of Results , Risk Assessment , Sensitivity and Specificity , Stroke Volume , Ultrasonography, Prenatal/statistics & numerical data , Umbilical Arteries/diagnostic imagingABSTRACT
To overcome the limitations of the state-of-the-art influenza surveillance systems in Europe, we established in 2008 a European-wide consortium aimed at introducing an innovative information and communication technology approach for a web-based surveillance system across different European countries, called Influenzanet. The system, based on earlier efforts in The Netherlands and Portugal, works with the participation of the population in each country to collect real-time information on the distribution of influenza-like illness cases through web surveys administered to volunteers reporting their symptoms (or lack of symptoms) every week during the influenza season. Such a large European-wide web-based monitoring infrastructure is intended to rapidly identify public health emergencies, contribute to understanding global trends, inform data-driven forecast models to assess the impact on the population, optimize the allocation of resources, and help in devising mitigation and containment measures. In this article, we describe the scientific and technological issues faced during the development and deployment of a flexible and readily deployable web tool capable of coping with the requirements of different countries for data collection, during either a public health emergency or an ordinary influenza season. Even though the system is based on previous successful experience, the implementation in each new country represented a separate scientific challenge. Only after more than 5 years of development are the existing platforms based on a plug-and-play tool that can be promptly deployed in any country wishing to be part of the Influenzanet network, now composed of The Netherlands, Belgium, Portugal, Italy, the UK, France, Sweden, Spain, Ireland, and Denmark.
Subject(s)
Epidemiological Monitoring , Influenza, Human/epidemiology , Influenza, Human/prevention & control , Internet , Public Health Surveillance , Community-Based Participatory Research , Disease Outbreaks/statistics & numerical data , Europe/epidemiology , Health Surveys , HumansABSTRACT
We present the case of a female infant born prematurely at 34 weeks of gestation. Prenatally a midsized ventricular septal defect was diagnosed. Due to marked respiratory distress intubation was attempted but failed, since the tube could not be placed beyond the glottis. Oxygenation could be improved by nasopharyngeal bag ventilation. The clinical course as well as radiographic imaging was suggestive for a complete tracheal agenesis with broncho-oesophageal fistula which was confirmed at autopsy. Tracheal agenesis (TA) is a rare differential diagnosis of postnatal respiratory distress and the obstetrician or neonatologist will regularly be surprised by this malformation. Partial or complete absence of the trachea without associated malformations will be rarely diagnosed antenatally. In the case of the absence of an oesophageal fistula to the remaining airway a congenital high airway obstruction syndrome (CHAOS) ensues, leading to enlarged hyperechogenic lungs, dilated and fluid-filled trachea and bronchi and an absent tracheal flow during foetal breathing. Aetiology of TA is unknown, therapeutic options are limited thus making TA a usually fatal disorder.
Subject(s)
Bronchial Fistula/complications , Bronchial Fistula/diagnosis , Constriction, Pathologic/complications , Constriction, Pathologic/diagnosis , Infant, Premature, Diseases/diagnosis , Infant, Premature, Diseases/therapy , Respiratory Distress Syndrome, Newborn/etiology , Trachea/abnormalities , Bronchial Fistula/therapy , Constriction, Pathologic/therapy , Delivery Rooms , Diagnosis, Differential , Fatal Outcome , Female , Humans , Infant, Newborn , Infant, Premature , Neonatology/methods , Obstetrics/methods , Respiratory Distress Syndrome, Newborn/diagnosis , Respiratory Distress Syndrome, Newborn/therapyABSTRACT
Free-living wild birds worldwide act as reservoir for Chlamydia psittaci, but the risk of transmission to humans through contact with wild birds has not been widely documented. From 12 January to April 9 2013, a total of 25 cases of psittacosis were detected in southern Sweden, about a threefold increase compared with the mean of the previous 10 years. A matched case-control study investigating both domestic and wild bird exposure showed that cases were more likely than controls to have cleaned wild bird feeders or been exposed to wild bird droppings in other ways (OR: 10.1; 95% CI: 2.1-47.9). We recommend precautionary measures such as wetting bird feeders before cleaning them, to reduce the risk of transmission of C. psittaci when in contact with bird droppings. Furthermore, C. psittaci should be considered for inclusion in laboratory diagnostic routines when analysing samples from patients with atypical pneumonia, since our findings suggest that psittacosis is underdiagnosed.
Subject(s)
Bird Diseases/epidemiology , Bird Diseases/transmission , Chlamydophila psittaci/isolation & purification , Psittacosis , Adult , Aged , Aged, 80 and over , Animals , Animals, Domestic/microbiology , Animals, Wild/microbiology , Birds , Case-Control Studies , Chlamydophila psittaci/pathogenicity , Cluster Analysis , DNA, Bacterial/analysis , Disease Reservoirs/microbiology , Disease Reservoirs/veterinary , Feces/microbiology , Female , Humans , Male , Middle Aged , Psittacosis/epidemiology , Psittacosis/transmission , Psittacosis/veterinary , Risk Factors , Sweden , ZoonosesABSTRACT
Trisomy 22 is a common trisomy in spontaneous abortions. In contrast, live-born trisomy 22 is rarely seen due to severe organ malformations associated with this condition. Here, we report on a male infant with complete, non-mosaic trisomy 22 born at 35 + 5 weeks via caesarean section. Peripheral blood lymphocytes and fibroblasts showed an additional chromosome 22 in all metaphases analyzed (47,XY,+22). In addition, array CGH confirmed complete trisomy 22. The patient's clinical features included dolichocephalus, hypertelorism, flattened nasal bridge, dysplastic ears with preauricular sinuses and tags, medial cleft palate, anal atresia, and coronary hypospadias with scrotum bipartitum. Essential treatment was implemented in close coordination with the parents. The child died 29 days after birth due to respiratory insufficiency and deterioration of renal function. Our patient's history complements other reports illustrating that children with complete trisomy 22 may survive until birth and beyond.
ABSTRACT
The perinatal morbidity and mortality risk in monochorionic twin pregnancies are 3-5-fold increased compared to those of dichorionic twin pregnancies. Partially, this is due to the higher rate of preterm delivery but also to the twin-to-twin transfusion syndrome (TTTS). Caused by unidirectional blood flow via placental anastomoses, the TTTS leads to weight differences of more than 20% between monochorial twins. The blood donor often shows oligohydramnios, whereas the recipient shows polyhydramnios. Lewi et al. demonstrated, in a study with 202 monochorionic twin pregnancies, a 9% rate of severe TTTS. The mortality of this complication is about 90% when untreated. In contrast to the chronic TTTS, little is known about the acute intrapartal one, which is characterised by anaemia and hypovolaemia of the donor and polyglobulia of the recipient without significant weight differences between the two. In most cases, anaemia occurred after normal delivery of the first twin. Still, there are no means or signs for early detection. We describe the case of a 30-year-old primigravida with a monochorionic diamniotic twin pregnancy. During pregnancy, no evidence of TTTS could be detected. At 37 + 1 weeks gestation labour was induced with prostaglandin-containing gel. Both foetuses showed cephalic presentation. The CTG of the first twin showed a conspicuous heart rate. After labour the first twin presented with anaemia and hypovolaemic shock, the APGAR was 2/7/8. The infant's haemoglobin was 13.7 g/dL. After delivery, the second twin with APGAR 10/10/10 showed a haemoglobin of 19.6 g/dL, which is in the upper normal range. Their birth weights differed by merely 10.4%. Acute TTTS is frequently characterised by anaemia and hypovolaemia of the second twin. In our case of a monochorionic twin delivery with acute TTTS the donor was born first. Early diagnosis and neonatal intervention is essential for reducing postnatal morbidity and mortality.
Subject(s)
Fetofetal Transfusion/diagnosis , Adult , Diagnosis, Differential , Early Diagnosis , Female , Humans , Male , PregnancyABSTRACT
Biallelic CEBPA mutations and FMS-like tyrosine kinase receptor 3 (FLT3) length mutations are frequently identified in human acute myeloid leukemia (AML) with normal cytogenetics. However, the molecular and cellular mechanisms of oncogene cooperation remain unclear because of a lack of disease models. We have generated an AML mouse model using knockin mouse strains to study cooperation of an internal tandem duplication (ITD) mutation in the Flt3 gene with commonly observed CCAAT/enhancer binding protein alpha (C/EBPα) mutations. This study provides evidence that FLT3 ITD cooperates in leukemogenesis by enhancing the generation of leukemia-initiating granulocyte-monocyte progenitors (GMPs) otherwise prevented by a block in differentiation and skewed lineage priming induced by biallelic C/EBPα mutations. These cellular changes are accompanied by an upregulation of hematopoietic stem cell and STAT5 target genes. By gene expression analysis in premalignant populations, we further show a role of FLT3 ITD in activating genes involved in survival/transformation and chemoresistance. Both multipotent progenitors and GMP cells contain the potential to induce AML similar to corresponding cells in human AML samples showing that this model resembles human disease.