Complex lumbosacral spinal cord lipomas: A longitudinal study on outcomes from a Singapore children's hospital.

BACKGROUND: Total/near-total resection (TR/NTR) of complex lumbosacral lipomas (CSL) is reported to be associated with better long-term functional outcomes and lower symptomatic re-tethering rates. We report our institutional experience for CSL resection in affected children. METHODS: This is a single-institution, retrospective study. Inclusion criteria consist of patients with CSL with dorsal, transitional and chaotic lipomas based on Pang et al's classification. The study population is divided into 2 groups: asymptomatic patients with a normal preoperative workup referred to as 'prophylactic intent' and 'therapeutic intent' for those with pre-existing neuro-urological symptoms. Primary aims are to review factors that affect post-operative clean intermittent catheterization (CIC), functional outcomes based on Necker functional score (NFS), and re-tethering rates. RESULTS: 122 patients were included from 2000 to 2021. There were 32 dorsal lipomas (26.2 %), 74 transitional lipomas (60.7 %), and 16 chaotic lipomas (13.1 %). 82 % patients achieved TR/NTR. Favourable NFS at 1-year was 48.2 %. The re-tethering rate was 6.6 %. After multivariable analysis, post-operative CIC was associated with median age at surgery (p = 0.026), lipoma type (p = 0.029), conus height (p = 0.048) and prophylactic intent (p < 0.001). Next, extent of lipoma resection (p = 0.012) and the post-operative CSF leak (p = 0.004) were associated with re-tethering. Favourable NFS was associated with lipoma type (p = 0.047) and prophylactic intent surgery (p < 0.001). CONCLUSIONS: Our experience shows that TR/NTR for CSL is a feasible option to prevent functional deterioration and re-tethering. Efforts are needed to work on factors associated with post-operative CIC.

Fibrofatty filum terminale: long-term outcomes from a Singapore children's hospital.

OBJECTIVE: The role of prophylactic detethering a fibrofatty filum terminale (FFT) remains equivocal. Furthermore, long-term studies focusing on urological outcomes are sparse. The aims of this study were to present an institutional experience on the perioperative and long-term outcomes of FFT surgery and to assess for factors that contribute to postoperative clean intermittent catheterization (CIC). METHODS: This was a single-institution, retrospective study conducted over a 20-year period. Patients younger than 19 years of age who underwent surgery for FFT were included. Variables of interest included patient demographics, clinical presentation, radiological findings, postoperative complications, and long-term need for CIC. Outcomes were measured using the Necker functional score and modified Hoffer Functional Ambulation scale score at 3, 6, and 12 months postdischarge. RESULTS: A total of 164 surgeries were performed for FFT from 2000 to 2020. The median age at surgery was 1.1 years, and the mean follow-up duration was 8.3 years. There were 115 patients (70.1%) who underwent prophylactic-intent surgery and 49 patients (29.9%) who underwent therapeutic-intent surgery. The proportion of therapeutic-intent surgeries increased significantly with age percentiles (0-20th, 21.9%; 20th-40th, 9.1%; 40th-60th, 18.2%; 60th-80th, 36.4%; and 80th-100th, 63.6% [p < 0.001]). Thirty patients (18.3%) had an associated syndrome, the most common (n = 19, 11.6%) being VACTERL (vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities). Forty-eight patients (29.3%) had an associated malformation (anorectal anomaly = 37, urogenital anomaly = 16, and sacral anomaly = 3). Cutaneous manifestation was the most common presentation (n = 96, 58.5%), followed by lower-limb neurological deficits (n = 21, 12.8%). A low-lying conus was present in 36.0% of patients (n = 59), and 16.5% had an associated syrinx (n = 27). There were 26 patients (18.8%) with an abnormal preoperative urodynamic study. Three patients (1.8%) had postoperative complications that required repeat surgery. There were no cases of CSF leakage. One patient (0.6%) developed retethering requiring another surgery. Postoperative CIC was required in 11 patients (6.7%). Multivariable analyses showed that an abnormal preoperative urodynamic study (adjusted OR 5.5 [95% CI 1.27-23.9], p = 0.023) and having an intraspinal syrinx (adjusted OR 5.29 [95% CI 1.06-26.4], p = 0.042) were associated with the need for CIC. CONCLUSIONS: The authors' results demonstrate that detethering surgery for FFT is a relatively safe procedure and can be performed prophylactically. Nonetheless, the risks of postoperative CIC should be emphasized during the preoperative counseling process.

A new high-prevalence LW antigen detected by an antibody in an Indigenous Australian homozygous for LW*A c.309C>A variant.

BACKGROUND AND OBJECTIVES: The LW gene encodes the LW glycoprotein that carries the antigens of the LW blood group system. LW antigens are distinct from D antigen, however, they are phenotypically related and anti-LW antibodies are often mistaken as anti-D. An antibody was detected in an Australian patient of Aboriginal descent who consistently typed as LW(a+b-). This study aimed to describe the antibody recognizing a high-prevalence antigen on the LW glycoprotein. STUDY DESIGN AND METHODS: Samples from the patient and her four siblings were investigated. DNA was genotyped by single nucleotide polymorphism (SNP)-microarray and massively parallel sequencing (MPS) platforms. Red blood cells (RBCs) were phenotyped using standard haemagglutination techniques. Antibody investigations were performed using a panel of phenotyped RBCs from adults and cord blood cells. RESULTS: SNP-microarray and MPS genotyped all family members as LW*A/A, (c.299A), predicting LW(a+b-). In addition, a novel LW*A c.309C>A single nucleotide variant was detected in all family members. The patient and one of her siblings (M4) were LW c.309C>A homozygous. Antibody from the patient reacted positive to all reagent panel RBCs and cord blood cells but negative with RBCs from LW(a-b-), Rhnull and sibling M4. Antibody failed to react with RBCs treated with dithiothreitol. CONCLUSION: Antibody detected in the patient recognized a novel high-prevalence antigen, LWEM, in the LW blood group system. LWEM-negative patients who developed anti-LWEM can be safely transfused with D+ RBCs, however, D- is preferred. Accurate antibody identification can help better manage allocation of blood products especially when D- RBCs are in short supply.

The End-to-End Provenance Project.

Data provenance is a machine-readable summary of the collection and computational history of a dataset. Data provenance confers or adds value to a dataset, helps reproduce computational analyses, or validates scientific conclusions. The people of the End-to-End Provenance Project are a community of professionals who have developed software tools to collect and use data provenance.

Appropriateness Criteria for Neuroimaging of Adult Headache Patients in the Emergency Department: How Are We Doing?

The American College of Radiology (ACR) developed Appropriateness Criteria (ACR-AC) for diagnostic imaging to reduce overuse and promote high-yield, cost-effective, evidence-based decision-making. For adult headaches, there are 16 variants with specific imaging recommendations. Headache accounts for 4.5% of emergency department (ED) visits, and 61% are chronic. Imaging for headaches has increased in the past 2 decades, with intracranial pathology diagnoses going down. Evidence suggests that there is poor knowledge of the ACR-AC among advanced practice nurses (APNs) and nonradiologist physicians. The ACR-AC recommendations were examined using the Health Care Cost and Utilization Project State Emergency Department Data (HCUP SEDD) from Maryland in 2013. Imaging proportions were examined, as well as differences between residency program hospitals and hospitals that have APNs in the ED. Of the 11,109 chronic headache visits, a quarter underwent computed tomography ([CT]; 26.9%) and 3.6% underwent magnetic resonance imaging (MRI); the ACR-AC does not recommend use of either of these in patients with chronic headache. There were significant practice differences related to hospital teaching and whether APNs were employed in the ED or not. For patients with posttraumatic headache, there were no significant differences in practice. Computed tomography was used in 76.4% of posttraumatic headache visits. It is unknown whether the ACR-AC are being used in the ED, and there is variability in following the recommendations. Posttraumatic headache protocol is well established in the ED, but chronic headache continues to be a problem in imaging overuse despite recommendations. Radiological education, including the ACR-AC, as well as radiation dosing and exposure information should be part of APN, physician, and registered nurse education, as well as continuing education. Continuing education is critical for adherence to the ACR-AC, as the recommendations are complex and continuously evolving. In addition, to minimize overuse of CT in headaches, the ACR-AC should be integrated into clinical decision support to promote best imaging practices.

Reducing the use of inappropriate coagulation testing in emergency general surgical patients.

Background and aims Indiscriminate coagulation testing in emergency general surgical patients can lead to inappropriate delay in surgery, cause unnecessary concern and is associated with significant cost. The British Committee for Standards in Haematology recommends against coagulation testing to predict peri-operative bleeding risk in unselected patients. Our aim was to assess the appropriateness of coagulation tests performed in emergency general surgical patients and evaluate the effect of a series of educational interventions on clinical practice. Methods and results Appropriate indications for performing coagulation testing included a positive bleeding history, the presence of liver disease/cholestasis, sepsis or use of anticoagulants. Initial data on 142 patients were collected over 2 weeks of receiving. Following analysis, indications for appropriate coagulation testing were highlighted and data were collected on a further 190 patients. Comparing the audit cycles, we observed a decrease in the proportion of patients who underwent routine testing (49.3% vs 32.6%; p = 0.002) and inappropriate testing (67% of tests vs 34% of tests; p < 0.001). Despite being highlighted, there was no evidence of improved documentation of bleeding histories on admission. Conclusions This observational study suggests that simple educational messages can reduce the inappropriate use of coagulation screening tests in general surgical emergencies. This seems to result from clarification of the appropriate surgical indications for coagulation testing in this group.

ABO serology in a case of persistent weak A in a recipient following a group O-matched unrelated bone marrow transplant.

CONCLUSIONS: HLA-matched hematopoietic stem cell transplantation (HSCT) from red blood cell (RBC)-incompatible donors is not uncommon. The engraftment process following ABO-incompatible allogeneic HSCT results in the transition from patient blood group to donor blood group in the recipient. In contrast, most non-hematopoietic tissues retain expression of the patient's original blood group for life, and these antigens may adsorb from the plasma onto the donor-derived RBCs. Correct serologic interpretation of the ABO blood group during this engraftment process can be difficult. We present the serologic findings of a 15-year-old girl of Maori descent, who was diagnosed with acute myeloid leukemia and transplanted with an HLA-matched unrelated group O, D+ bone marrow. Despite engraftment, her RBCs showed persistence of weak A. This case report showcases the importance of awareness and correct serologic interpretation of weak persistence of recipient ABH substance on the patient's RBCs for clinical decision-making, blood component support, and patient wellbeing.

Developing the Cultural Awareness Skills of Behavior Analysts.

All individuals are a part of at least one culture. These cultural contingencies shape behavior, behavior that may or may not be acceptable or familiar to behavior analysts from another culture. To better serve individuals, assessments and interventions should be selected with a consideration of cultural factors, including cultural preferences and norms. The purpose of this paper is to provide suggestions to serve as a starting point for developing behavior analysts' cultural awareness skills. We present strategies for understanding behavior analysts' personal cultural values and contingencies and those of their clients, integrating cultural awareness practices into service delivery, supervision, and professional development, and becoming culturally aware in everyday practice.

Software Assurance Using Structured Assurance Case Models.

Software assurance is an important part of the software development process to reduce risks and ensure that the software is dependable and trustworthy. Software defects and weaknesses can often lead to software errors and failures and to exploitation by malicious users. Testing, certification and accreditation have been traditionally used in the software assurance process to attempt to improve software trustworthiness. In this paper, we examine a methodology known as a structured assurance model, which has been widely used for assuring system safety, for its potential application to software assurance. We describe the structured assurance model and examine its application and use for software assurance. We identify strengths and weaknesses of this approach and suggest areas for further investigation and testing.

Postpartum anti-D: can we safely reduce the dose?

OBJECTIVE: To assess the potential for dose-reduction of prophylactic anti-D postpartum. DESIGN: Retrospective audit of fetomaternal haemorrhage (FMH) quantitation by flow cytometry. PARTICIPANTS AND SETTING: 5148 consecutive Rhesus D-negative women aged 15-45 years who had FMH estimation by flow cytometry at a central laboratory in Western Australia in the 65 months between 1 August 1999 and 31 January 2005. MAIN OUTCOME MEASURES: Quantitation of FMH volume for adequate prophylactic anti-D administration in a timely fashion. RESULTS: 90.4% (4651/5148) of the women had an FMH volume of 1.0 mL or less of Rh D-positive red cells, and 98.5% (5072/5148) had a volume of less than 2.5 mL. Only 0.4% of cases had an FMH volume of 6.0 mL or greater (range, 6.0-92.4 mL). CONCLUSIONS: This large retrospective audit shows that a currently available dose of 250 IU (50 mg) of anti-D would have been sufficient for 98.5% of the 5148 Rh D-negative women. On the basis of this evidence, a reduction in the recommended routine postpartum dose of anti-D from 625 IU to 250 IU when flow cytometric quantitation for FMH is available should be considered. Adopting such a strategy would ensure the ongoing provision of a valuable human blood product currently in limited supply.