A newborn with moderate hemophilia A with severe intracranial and extracranial hemorrhage: A case report

Intracranial hemorrhage among term newborns is a rare clinical condition with high morbidity and mortality. Although major bleeding is relatively uncommon, the incidence of intracranial hemorrhage in hemophilic children is higher during the first few days of life than at any other stage in childhood, which relates to the trauma of delive ry. Here, we reported a newborn case diagnosed with moderate hemophilia A, without the presence of a positive family history of hemophilia and presenting with intracranial and extracranial hemorrhage and we aimed to emphasize that the early diagnosis and replacement therapy carries an essential importance

Aggressive Neuroblastoma in a Pediatric Patient with Severe Hemophilia A

Despite the extensive information regarding hemophilia's hemorrhagic complications, the literature on cancer in hemophilia is scarce, especially in pediatric patients. Many uncertainties remain concerning diagnosis and workup. We report a rare case of two severe diseases (neuroblastoma and hemophilia A (HA)) concomitantly present in the same pediatric patient. We highlight that the diagnosis of severe HA did not have a negative impact on the patient's oncologic course. This case also illustrates the significance of the cooperation among different specialties and hospitals when caring for the same patient.info:eu-repo/semantics/publishedVersio

Large subgaleal hematoma producing turban head in 10 year boy with cerebral palsy: Rare case report with review of literature

Subgaleal hematomas (SGHs) are not uncommon. Because the subgaleal space has no anatomical boundaries, SGHs usually involve a large space and are typically limited to the parietal region. Cases of SGHs involving whole of head are relatively rare. In this study we report a rare case of massive enlargement of head after SGH causing severe pain and giving an appearance of turban. A 10 year old, male patient with cerebral palsy presented with progressive enlargement of head attaining a size of turban due to habitual head banging and self-punching overhead. SGH drainage and hematoma aspiration were performed and the patient’s head size was restored

Women with inherited bleeding disorders and their offspring - the unresolved issues

The past few decades have seen major advances in management of gynaecological conditions and multidisciplinary obstetric care in women with inherited bleeding disorders (IBDs) and their offspring. However, there remain many unresolved issues within the field. A series of observational studies were conducted to address these issues with an overarching aim of improving patient care. A case-control study determined if there was an association between IBDs and endometriosis. Women with a surgically confirmed diagnosis of endometriosis (n = 84) and controls (n = 30) underwent investigations of haemostasis. Women with endometriosis had significantly more platelet aggregation defects to one and multiple agonists compared to controls (31% vs 4%, p = 0.005 and 15% vs 4%, p < 0.05, respectively). Reduced von willebrand factor (VWF) activity correlated with increased laparoscopic stage of endometriosis (r = -0.35, p = 0.01). A 10-year review and questionnaire study was carried out in carriers of haemophilia to determine their attitudes towards prenatal diagnosis. Sixty-one carriers of haemophilia had obstetric care in 73 pregnancies. Forty-one out of 61 women responded to the questionnaire. The uptake for invasive prenatal diagnosis of haemophilia was reduced compared to previous studies published at the Royal Free Hospital (15% versus 20% in 2008 [1], and 35% in 1997 [2]). Invasive testing to confirm the haemophilia status of the fetus was used to guide management decisions of labour and delivery. The rate of termination of pregnancy (TOP) for haemophilia was lower than in previous case series [1]. Non-invasive determination of fetal gender using free fetal DNA (ffDNA) was carried out in 58 pregnancies (79%). Fifty-nine deliveries were managed at the Royal Free Hospital over 10-years. The majority of women (66%) in this series underwent elective caesarean section (CS). The primary indication for CS was for haemophilia in 59% of deliveries. A literature review and meta-analysis assessed the incidence of cranial bleeding at birth in newborns with haemophilia. The incidence of symptomatic intracranial haemorrhage (ICH) was determined by mode of delivery (MOD). Newborns with haemophilia were 44 times (95%CI 34.7-57.1, p < 0.01) more likely to experience symptomatic ICH, and 8 times (95%CI 5.38-12.6, p < 0.01) more likely to experience extracranial haemorrhage (ECH) at birth, compared to the general population. The OR of experiencing ICH following an assisted vaginal delivery was 4.4 (95%CI 1.46-13.7, p = 0.008) compared to vaginal delivery in newborns with haemophilia. The OR of experiencing ICH following CS was 0.34 (95%CI 0.14-0.83, p = 0.018) compared to vaginal delivery. CS was associated with the lowest risk of ICH in newborns with haemophilia. A prospective MRI screening study in term newborns with severe IBDs was undertaken to determine feasibility and incidence of asymptomatic ICH. Cranial MRI within 72 hours of delivery excluded asymptomatic ICH in affected infants. No cases of ICH were reported among eight participants. One newborn experienced cephalohaematoma following an emergency CS. Two of the eight newborns experienced spontaneous ICH in early infancy. A case-control study analysed the differences in rotational thromboelastometry (ROTEM®) parameters between parturient women with FXI deficiency, and parturient and non-parturient controls. Women with FXI deficiency achieved a hypercoagulable status during the third trimester of pregnancy; however, the changes were not as pronounced as in pregnant controls. Women with prolonged clotting time and clot formation time were considered to have an increased risk of bleeding. A prospective cohort study evaluated the role of ROTEM® analysis in assessment of bleeding risk in women with FXI deficiency. Pregnancy outcomes and haemostatic cover was reviewed in 57 deliveries in women with FXI deficiency. ROTEM® enabled treatment decision and reduced the need for treatment with factor concentrate in women with severe FXI deficiency. A cohort study assessed the correlation between bleeding score, haemostatic and prothrombotic variables in women with VWD and carriers of haemophilia. The presence of a thrombotic marker (anticoagulant deficiency or prothrombotic gene mutation) altered the bleeding score for a given VWF:RCo level in women with VWD (p = 0.015). Co-inheritance of thrombophilia reduces bleeding severity in women with IBDs, and thrombotic risk must be considered in these women

Inherited bleeding disorders in obstetrics and gynaecology

The aim of this thesis is to investigate the obstetric and gynaecological problems and their management in women with inherited bleeding disorders, as well as the role of such disorders in obstetric and gynaecological haemorrhage. The uptake of prenatal diagnosis and termination of an affected pregnancy is low in carriers of haemophilia. Fetal gender determination has important implications in the management of labour in carriers who do not wish to have specific prenatal diagnosis. The attitude of women towards reproductive choices is influenced by ethnic and cultural issues and family experience with the disease. Haemostatic response to pregnancy is variable in different types and subtypes of inherited bleeding disorders and in the same patient in different pregnancies. Haemorrhagic complications are confined to post-abortal and post-partum period. The incidence of primary and secondary post-partum haemorrhage was 22% and 11% in carriers of haemophilia, 18.5% and 20% in vWD and 16% and 24% in FXI deficient women, respectively. Women with low factor levels (<50 iu/dl) and no prophylactic treatment for labour and puerperium are especially at risk. There are great inter- and intra-individual variations in coagulation markers in women due to different physiological conditions including age, ethnicity, blood group and hormonal changes during different phases of the menstrual cycle. Women with inherited bleeding disorders suffer from heavy and prolonged menstruation which adversely affects their quality of life. Objectively confirmed menorrhagia is significantly higher in these women (67%) compared with the control group (29%). On the other hand, undiagnosed inherited bleeding disorders can be the underlying cause in a significant proportion (17%) of women presenting with unexplained menorrhagia. The DDAVP nasal spray was shown not to be superior to placebo in the treatment of menorrhagia. Increased awareness among clinicians responsible for women's health of these disorders and their morbidity and the availability of management guidelines are essential for optimal care and improvement of the quality of life of these patients

Developing a labour and birth orientation program

Purpose and Background: The purpose of this practicum was to re-develop the labour and birth orientation program at the Queen Elizabeth Hospital in Charlottetown, PE. The need for this practicum was identified following a substantial staff turnover. This potentiated a need for orientation of a number of registered nurses to labour and birth. During orientation, presentation of program content consisted of two days of traditional classroom style teaching followed by a six-week preceptorship, where the majority of labour and birth knowledge and content would be acquired during this time and facilitated by the preceptor. This led to inconsistencies in information provided to new learners. Evaluation was determined by a written exam and preceptor feedback; inconsistencies were noted by unit leaders and preceptors with these methods of evaluation. Methods: A literature review was undertaken to explore the theoretical underpinnings for the program, Kolb’s Experiential Learning theory, and to explore the literature on the benefits and orientations programs. Also, a consultation plan and report was conducted, which provided a theoretical and evidence-based framework program development. Conclusion: The final program describes methods by which labour and birth content is presented to orientees, and includes 16 learning modules; 15 of which were developed. Additionally, the formal orientation process, method for evaluation of the orientee, and methods for remediation for individuals who are having difficulties with the orientation process are described throughout the program

PRELIMINARY FINDINGS OF A POTENZIATED PIEZOSURGERGICAL DEVICE AT THE RABBIT SKULL

The number of available ultrasonic osteotomes has remarkably increased. In vitro and in vivo studies have revealed differences between conventional osteotomes, such as rotating or sawing devices, and ultrasound-supported osteotomes (Piezosurgery®) regarding the micromorphology and roughness values of osteotomized bone surfaces. Objective: the present study compares the micro-morphologies and roughness values of osteotomized bone surfaces after the application of rotating and sawing devices, Piezosurgery Medical® and Piezosurgery Medical New Generation Powerful Handpiece. Methods: Fresh, standard-sized bony samples were taken from a rabbit skull using the following osteotomes: rotating and sawing devices, Piezosurgery Medical® and a Piezosurgery Medical New Generation Powerful Handpiece. The required duration of time for each osteotomy was recorded. Micromorphologies and roughness values to characterize the bone surfaces following the different osteotomy methods were described. The prepared surfaces were examined via light microscopy, environmental surface electron microscopy (ESEM), transmission electron microscopy (TEM), confocal laser scanning microscopy (CLSM) and atomic force microscopy. The selective cutting of mineralized tissues while preserving adjacent soft tissue (dura mater and nervous tissue) was studied. Bone necrosis of the osteotomy sites and the vitality of the osteocytes near the sectional plane were investigated, as well as the proportion of apoptosis or cell degeneration. Results and Conclusions: The potential positive effects on bone healing and reossification associated with different devices were evaluated and the comparative analysis among the different devices used was performed, in order to determine the best osteotomes to be employed during cranio-facial surgery