Food Insecurity Classification in Rural Areas of Mozambique: An Integrated Analysis of Survey-Based Indicators.

National food insecurity early warning systems and food policy interventions need reliable information concerning the classification of food insecurity. The aim of this paper was to produce an acute food insecurity classification in Mozambique, by: i) analyzing food insecurity indicators individually; ii) comparing it with a new integrated analysis of survey-based indicators called the "Matrix Analysis." The Matrix results show more severe classifications than the single indicators for the analyzed districts. The matrix novelty consists on a cross-tabulation of all indicators, allowing a less subjective analysis. Further research is needed on how the Matrix approach could complement national classification systems.

Management of Hyperosmolar Hyperglycaemic State (HHS) in Adults: An updated guideline from the Joint British Diabetes Societies (JBDS) for Inpatient Care Group.

Hyperosmolar Hyperglycaemic State (HHS) is a medical emergency associated with high mortality. It occurs less frequently than diabetic ketoacidosis (DKA), affects those with pre-existing/new type 2 diabetes mellitus and increasingly affecting children/younger adults. Mixed DKA/HHS may occur. The JBDS HHS care pathway consists of 3 themes (clinical assessment and monitoring, interventions, assessments and prevention of harm) and 5 phases of therapy (0-60 min, 1-6, 6-12, 12-24 and 24-72 h). Clinical features of HHS include marked hypovolaemia, osmolality ≥320 mOsm/kg using [(2×Na+ ) + glucose+urea], marked hyperglycaemia ≥30 mmol/L, without significant ketonaemia (≤3.0 mmol/L), without significant acidosis (pH >7.3) and bicarbonate ≥15 mmol/L. Aims of the therapy are to improve clinical status/replace fluid losses by 24 h, gradual decline in osmolality (3.0-8.0 mOsm/kg/h to minimise the risk of neurological complications), blood glucose 10-15 mmol/L in the first 24 h, prevent hypoglycaemia/hypokalaemia and prevent harm (VTE, osmotic demyelination, fluid overload, foot ulceration). Underlying precipitants must be identified and treated. Interventions include: (1) intravenous (IV) 0.9% sodium chloride to restore circulating volume (fluid losses 100-220 ml/kg, caution in elderly), (2) fixed rate intravenous insulin infusion (FRIII) should be commenced once osmolality stops falling with fluid replacement unless there is ketonaemia (FRIII should be commenced at the same time as IV fluids). (3) glucose infusion (5% or 10%) should be started once glucose <14 mmol/L and (4) potassium replacement according to potassium levels. HHS resolution criteria are: osmolality <300 mOsm/kg, hypovolaemia corrected (urine output ≥0.5 ml/kg/h), cognitive status returned to pre-morbid state and blood glucose <15 mmol/L.

Cementless femoral neck endoprosthesis SPIRON in men in aspects of clinical status and quality of life in an average 7-year follow-up.

BACKGROUND: We report the clinical evaluation, quality of life and pain assessment in patients who had a femoral neck SPIRON endoprosthesis. METHODS: The study group consisted of 27 men in whom 35 femoral neck endoprosthesis were implanted (8 on the left side, 12 on the right side and 7 bilateral) due to idiopathic osteoarthritis of the hip (20 patients) or avascular femoral osteonecrosis (7 patients) in a mean 7-year follow-up. RESULTS: The median pre-operative Harris Hip score (HHS) was 35.5 and post-operative 98.5 (p < 0.001). The median WOMAC HIP score was pre-operatively 57 and post-operatively 0 (p < 0.001). The median SF-12 score was pre-operatively 4 and post-operatively 33 (p < 0.001). The median pain assessment in VAS scale was 7 pre-operatively and 0 post-operatively (p < 0.001). CONCLUSIONS: The results of all examined patients have changed significantly in every category showing that SPIRON endoprosthesis improved their quality of life and statistically reduced pain ailments. Moreover we have proved that higher BMI (> 30) is associated with worse operation outcomes.

[A geriatric case of hyperosmolar hyperglycemic state with osmotic demyelination syndrome that manifested with dysphagia].

The patient was an 84-year-old man who had been on insulin therapy for type 2 diabetes mellitus for 55 years. He had undergone bile duct stenting to avoid obstruction due to adenocarcinoma of the bile duct. The patient had suffered from fever and anorexia for two weeks, and had subsequently stopped insulin therapy. Since he showed signs of impaired consciousness, he was taken to the emergency room, and was diagnosed with a hyperosmotic hyperglycemic state (HHS) based on the following laboratory findings: blood glucose, 632 mg/dL; plasma osmolality, 391 mOsm/kg·H2O; and serum Na, 163 mEq/L, with urine ketone bodies±and sepsis (Klebsiella pneumoniae). He was therefore admitted to the hospital. His blood glucose and serum Na levels slowly improved following the administration of fluids, insulin, and antibiotics. The patient's consciousness disturbance also improved. However, on the third day after admission, dysphagia was newly observed when the patient resumed eating, and swallowing endoscopy revealed a delayed gag reflex and pharyngeal retention of saliva. Cranial magnetic resonance imaging showed a high-intensity area in the central pontine, which was considered to be caused by osmotic demyelination syndrome (ODS). The patient's oral intake ability recovered with swallowing rehabilitation. ODS is a rare complication of HHS. We report a case of HHS with ODS, in which the patient's chief complaint was dysphagia, which should be distinguished from other diseases.

Internal fixation of acetabular quadrilateral plate fractures in elderly patients: Could the fracture reduction quality affect their functional recovery?

BACKGROUND: Osteoporotic acetabular fractures frequently involve the quadrilateral plate (QP), a flat and thin bone constituting the medial wall of the acetabulum. This study aims to assess the impact of the quality of osteoporotic QP fractures reduction on the patients' functional recovery, at 24 months follow-up. METHODS: Patients referring with osteoporotic QP fractures to our Level I trauma centre were prospectively recruited. INCLUSION CRITERIA: patients aged 60 years old or older; osteoporosis, defined as Dual-energy X-ray Absorptiometry (DXA) T-score ≤ - 2.5; acute acetabular fracture; anatomic or good fracture reduction according to Matta on postoperative CT. EXCLUSION CRITERIA: moderate cognitive impairment (defined as Mini-Mental State Examination < 19); a history of malignant neoplasm; concomitant fractures in other sites; traumatic head injury; lower limb joint prostheses; patient not able to walk independently before trauma; poor fracture reduction, according to Matta, on postoperative CT. All the QP fractures were surgically managed. After surgery, the reduction of each QP fracture was classified as anatomical (displacement 0-1 mm), good (displacement 2-3 mm) and poor (displacement > 3 mm) on postoperative CT. Based on this classification: patients with a poor fracture reduction were excluded from this study, patients with an anatomical reduction were recruited in Group-A and patients with a good reduction in Group-B. All the patients underwent a clinical and radiographic 24-months follow-up. RESULTS: 68 patients (males 38; females 30; mean age 68.6 years old; range 60-79) were finally included in in the study. No cases of open fractures or concomitant pelvic ring fractures were observed. Based on the post-operative CT, 39 patients showed an anatomic fracture reduction (Group-A) while the remaining 29 patients revealed a good fracture reduction (Group-B). Complication rates and mean clinical scores showed no significant differences between groups, at 24-months follow-up. CONCLUSIONS: In this study, the functional recovery at 24 months follow-up showed no significant differences in elderly patients with QP fracture undergoing anatomical reconstruction (displacement 0-1 mm) compared to patients receiving a good QP fracture reconstruction (displacement ≤ 3 mm).

Efficacy of various core decompression techniques versus non-operative treatment for osteonecrosis of the femoral head: a systemic review and network meta-analysis of randomized controlled trials.

BACKGROUND: Various Joint-preserving therapy (JPT) methods have been performed and tried in recent decades, but their results and efficacy were inconsistent and controversial. The purpose of this study is to evaluate its effectiveness and whether there are statistical differences in treatment between different interventions based on published RCT studies. METHODS: Following the PRISMA-NMA checklist, Medline, EMBASE, Web of Science, and Cochrane Library databases were searched and collected related RCT studies. The sources were searched from inception up to October 30, 2020. The primary outcomes including the rate of radiographic progression and conversion to THA and the secondary outcome -Harris Hip Scores (HHS) were extracted and compared in a Network meta-analysis. RESULTS: Seventeen RCT studies involving 784 patients (918 hips) with seven interventions including CD (core decompression), CD + BG (bone graft), CD + TI (tantalum rod implantation), CD + CT (Cell therapy), CD + BG + CT, VBG (vascularized bone graft), and nonsurgical or conservative treatment for ONFH were evaluated. In the radiographic progression results, CD + CT showed a relatively better result than CD, CD + BG and non-surgical treatment, the surface under the cumulative ranking curve (SUCRA) plot displayed that CD + CT (96.4%) was the best, followed by CD (64.1%).In conversion to THA results, there were no significant differences between the JPT methods and non-surgical treatment. In HHS, there was also no significant difference, other than CD + BG showed a statistical difference than non-surgical treatment only in terms of Cis, but the SUCRA was highest in non-surgical treatment (80.5%) followed by CD + CT (72.8%). CONCLUSIONS: This Net-work meta-analysis demonstrated that there was no statistical difference in the outcome of radiographic progression and conversion to THA, also in HHS, other than CD + CT showed a relatively superior result in radiographic progression than nonsurgical treatment, namely, it's maybe an effective method for delaying disease progression or reducing disease development based on current evidence.

Clinical and epidemiological features of heart-hand syndrome, an updated analysis in China.

BACKGROUND: The purpose of this study was to prospectively recruit patients treated with limb malformation and to explore the prevalence and the clinical and epidemiological features of Heart-Hand Syndrome (HHS) in China. METHODS: The consecutive patients treated for congenital upper limb malformation in Beijing Ji Shui Tan Hospital from October 1st, 2016 to October 1st, 2019 were prospectively recruited. We reviewed the patients' medical records and identified patients with abnormal electrocardiogram (ECG) and/or abnormal ultrasonic cardiogram as well as their basic demographic and clinical characteristics. RESULTS: A total 1653 (1053 male and 600 female) patients with congenital upper extremity malformations were prospectively recruited. Among them, 200 (12.1%) had abnormal ultrasonic cardiogram (181patients, 10.9%) and/or abnormal ECG (19 patients, 1.1%). The commonest type of abnormal heart structure was atrial septal defect (69/181 38.1%), and the commonest abnormal ECG was wave patterns (7/19, 36.8%). HHS patients had a higher comorbidity rate (11%) than non-HHS patients (6.9%). Patients with HHS were classified into four groups by the types of congenital upper extremity malformations, among which the most common group was thumb type (121/200, 60.5%). CONCLUSIONS: HHS occurred frequently among patients with congenital upper extremity malformation in China, particularly for those with multiple congenital malformations. The commonest type of hand malformations of HHS patients was thumb malformation.

Results of 198 primary total hip arthroplasties using the Delta PF-FIT system with ceramic-on-ceramic articulating surfaces with average seven years follow up.

BACKGROUND: The lifetime implants is a key parameter that the surgeon should take into account at the time of the primary total hip arthroplasty (THA). The aim of this study was a clinical and radiographical evaluation of the Delta PF-FIT (LimaCorporate, Italy) THA system with ceramic-on-ceramic articulations. We have not found a clinical or radiographical assessment of this implant in available published literature. METHODS: A total of 197 (F = 94, M = 103) primary THAs were evaluated in 163 patients with a mean follow-up of 7.7 years (range 5.1-11.2 years (SD ± 1.5)) Harris hip Score (HHS) and the Western Ontario and McMaster Universities Arthritis index (WOMAC) were used for the clinical evaluation. The statistical evaluation was processed by standard statistical methods. The study was approved by Ethic Committee of the University Hospital Motol (Reference No. EK-73/19). RESULTS: The mean HHS score was found to be 97.59 points (61-100 range with a ± 5.13 SD, preoperative HSS was 51.21, range 28-73 with a ± 4,77 SD). 186 THAs were evaluated as excellent (90-100 points), 9 THAs rated as good (80-89 points), 1 THA was rated as fair (70-79) points and 1 THA rated as poor (less than 70 points). The mean WOMAC score was 97.38 points (65-100 range with a ± 5.18 SD, preoperative was 50,12, range 27-69 with a ± 4.85 SD). We documented an overall 99.49% Kaplan-Meier survival with a mean follow-up of 7.7 years with the FIT (LimaCorporate) stem revision and any component revision as the endpoint. With the Delta PF (LimaCorporate) cup revision as the endpoint, the survival was 100%. We have not found a previously published clinical or radiographical review of this THA system, the study shows a comparison with other THA implants. CONCLUSION: Evaluation of the Delta-PF-FIT (LimaCorporate, Italy) THA system with the use of ceramic-on-ceramic BIOLOX®Delta articulation surfaces shows very good outcomes.

Proximal femoral nailing is superior to hemiarthroplasty in AO/OTA A2 and A3 intertrochanteric femur fractures in the elderly: a systematic literature review and meta-analysis.

INTRODUCTION: Unstable intertrochanteric femoral fractures in the elderly require either fixation or joint sacrificing surgery; proximal femoral nail (PFN) and bipolar hemiarthroplasties (BPH) are the most common interventions. PFN is considered to be the ideal construct for these fractures; however, the usage of hemiarthroplasties to facilitate earlier mobilization has been on a rise. Currently there is no consensus on the superiority of one of these two techniques over the other and the present review was done to determine this. RESEARCH QUESTION: Is PFN a better alternative to BPH for unstable intertrochanteric femur fractures in the elderly? OBJECTIVE: The present systematic review and meta-analysis was conducted to determine the superiority of PFN over BPH by comparing the primary outcomes like mortality, Harris Hip scores (HHS), complications, and re-operations. Additionally, secondary outcomes like blood loss, duration of surgery, and period of hospital stays were also compared. METHODOLOGY: Three databases of PubMed, EMBASE, and SCOPUS were searched for relevant articles that directly compared PFN and BPH in unstable intertrochanteric femur fractures in the elderly. RESULTS: We analyzed a total of seven studies published between the years 2005 to 2017. There were four retrospective and three prospective randomized controlled studies. The number of patients in these studies ranged from 53 to 303. PRIMARY OUTCOMES: There was a significant difference in HHS between two groups with standard mean difference of - 0.51 (range - 0.67 to -0.36), favouring the PFN group. The rate of mortality was higher in the BPH group with odds ratio of 2.07 (range 1.40-3.08). Implant-related complications like fractures and subsidence were more in BPH group but this was not significant. SECONDARY OUTCOMES: Mean surgical time (standard mean difference 2.19) and blood loss (3.75) were significantly less in the PFN group. The duration of hospital stay was also found to be significantly less in the PFN group (2.66). CONCLUSION: Proximal femoral nails are superior to bipolar hemiarthroplasties for unstable intertrochanteric femoral fractures in the elderly. PFN imparts better functional outcomes and has lower rates of overall mortality. Additionally it is faster surgery, with lesser blood loss contributing to better results.

Transcriptome analysis of Valsa mali reveals its response mechanism to the biocontrol actinomycete Saccharothrix yanglingensis Hhs.015.

BACKGROUND: Apple canker is a devastating branch disease caused by Valsa mali (Vm). The endophytic actinomycete Saccharothrix yanglingensis Hhs.015 (Sy Hhs.015) can effectively inhibit the growth of Vm. To reveal the mechanism, by which Vm respond to Sy Hhs.015, the transcriptome of Vm was analyzed using RNA-seq technology. RESULTS: Compared with normal growing Vm in the control group, 1476 genes were significantly differentially expressed in the Sy Hhs.015's treatment group, of which 851 genes were up-regulated and 625 genes were down-regulated. Combined gene function and pathway analysis of differentially expressed genes (DEGs) revealed that Sy Hhs.015 affected the carbohydrate metabolic pathway, which is utilized by Vm for energy production. Approximately 82% of the glycoside hydrolase genes were down-regulated, including three pectinase genes (PGs), which are key pathogenic factors. The cell wall structure of Vm was disrupted by Sy Hhs.015 and cell wall-related genes were found to be down-regulated. Of the peroxisome associated genes, those encoding catalase (CAT) and superoxide dismutase (SOD) which scavenge reactive oxygen species (ROS), as well as those encoding AMACR and ACAA1 which are related to the ß-oxidation of fatty acids, were down-regulated. MS and ICL, key genes in glyoxylate cycle, were also down-regulated. In response to the stress of Sy Hhs.015 exposure, Vm increased amino acid metabolism to synthesize the required nitrogenous compounds, while alpha-keto acids, which involved in the TCA cycle, could be used to produce energy by deamination or transamination. Retinol dehydrogenase, associated with cell wall dextran synthesis, and sterol 24-C-methyltransferase, related to cell membrane ergosterol synthesis, were up-regulated. The genes encoding glutathione S-transferase, (GST), which has antioxidant activity and ABC transporters which have an efflux function, were also up-regulated. CONCLUSION: These results show that the response of Vm to Sy Hhs.015 exposure is a complicated and highly regulated process, and provide a theoretical basis for both clarifying the biocontrol mechanism of Sy Hhs.015 and the response of Vm to stress.

Expression and characterization of a novel chitinase with antifungal activity from a rare actinomycete, Saccharothrix yanglingensis Hhs.015.

Saccharothrix yanglingensis Hhs.015, a new type of rare actinomycete, was isolated from the roots of cucumber. A novel chitinase gene was cloned from S. yanglingensis Hhs.015 and overexpressed as a soluble protein Chi6769 (77.9 kDa) in Escherichia coli. Chi6769 was purified by HisTrap HP affinity chromatography with optimal pH of 7.0. The enzymatic hydrolysis assay revealed that Chi6769 was capable of hydrolyzing chitin to (GlcNAc)3, (GlcNAc)2 and GlcNAc. (GlcNAc)2 was the main hydrolyzate. The antifungal activity result showed that Chi6769 exhibited strong antifungal activity toward Valsa mali 03-8. Overall, Chi6769 was potential to be a novel biofunctional chitinase that could be used as a biological agent in the control of plant diseases.

Comparison of direct anterior, lateral, posterior and posterior-2 approaches in total hip arthroplasty: network meta-analysis.

Total hip arthroplasty (THA) is generally considered to be one of the most successful orthopedic surgical procedures. THA patients continue to experience symptoms, most commonly pain, which prevent their return to full function and activity. Possible causes include failure of fixation, instability and damage to soft tissues, associated with the trauma of the surgical procedure. Choosing the optimal surgical approach can minimize these risks and therefore improve the outcome of THA. Surgical approaches in THA include anterior, lateral [anterolateral (Hardinge) and direct lateral (Watson-Jones)], posterior (posterolateral and posterior) and posterior-2 techniques. However, there is no current consensus regarding which approach is the most suitable. Therefore, we conducted a systematic review and network meta-analysis to compare the postoperative outcomes and complications among THA approach and identify which approach is the best for THA. We searched all RCT studies that compared intra-operative and postoperative outcomes of anterior, lateral [anterolateral (Hardinge) and direct lateral (Watson-Jones)], posterior (posterolateral and posterior) and posterior-2 approaches for THA from the PubMed and Scopus databases up to February 1, 2017. Data were independently extracted by two reviewers. A network meta-analysis was applied to assess treatment outcomes. Probability of being the best treatment was estimated using surface under the cumulative ranking curves (SUCRA). Fourteen RCTs (N = 1017 patients) met inclusion criteria. Interventions were anterior (N = 233 patients), lateral (N = 334 patients), posterior (N = 405 patients) and posterior-2 (N = 45 patients) approaches. A network meta-analysis showed that effects of anterior approach were higher to lateral, posterior and posterior-2 approaches with the pooled mean postoperative within 1 month and last follow-up of HHS of 2.56 (95% CI - 0.79, 5.91), 4.80 (95% CI 1.33, 8.26), 10.80 (95% CI 2.10, 19.49) and 6.40 (95% CI 0.72, 12.09), 2.22 (95% CI - 3.21, 7.66), 4.22 (95% CI - 6.81, 15.25), respectively. For VAS, lateral approach was lower to anterior, posterior and posterior-2 approaches. In terms of complication, posterior approach was the lowest risk with RR of 0.39 (95% CI 0.19, 0.81), 0.57 (95% CI 0.21, 1.57) and 1.74 (95% CI 0.36, 8.33) when compared to anterior, followed by lateral and posterior-2 approaches. Results of SUCRA indicated anterior and lateral approaches were the first and second ranks for postoperative HHS and VAS score, while posterior and lateral approaches were the first and second ranks for postoperative complications. We recommended using lateral approach that has an acceptable postoperative pain, function and complications (second rank for all outcomes) as a surgical technique for THA.

Background characteristics and clinical features of elderly patients with hyperglycemic hyperosmolar syndrome.

AIM: Few reports have described the characteristics of hyperglycemic hyperosmolar syndrome (HHS) in the elderly. We investigated the background characteristics and clinical features of 14 elderly patients with HHS. METHODS: HHS was diagnosed based on a blood glucose level of >600 mg/dL and an effective plasma osmolality [2 (Na) + glu/18] of >320 mOsm/kg. For 14 cases of HHS, we investigated the medical and social backgrounds of the patients, their clinical findings, and the outcomes. RESULTS: The mean patient age was 83 years, and the mean body mass index was 17.8 kg/m2. Half had a history of either cerebral infarction or hip fracture. The mean duration of diabetes was 14 years, but 4 diabetes cases were newly diagnosed. There was a high prevalence of acute infection (79%) in HHS patients, especially urinary tract infection and pneumonia, with a seasonal peak in winter. Patients who had been treated with steroids, tube feeding, or both numbered 1, 2, and 1, respectively. Most HHS patients had a history of dementia. More than half of such patients were living alone or only with their spouse, and their activities of daily living showed marked deterioration. The mean blood glucose level, HbA1c, effective serum osmolality, and pH were 881 mg/dL, 10.3%, 353 mOsm/kg, and 7.39, respectively. One patient died during hospitalization, and 9 were discharged to nursing homes or other hospitals. The mean length of hospitalization was 55 days. In most cases, the insulin secretion capacity was preserved, and 9 patients were treatable with oral hypoglycemic agents alone. CONCLUSIONS: Many cases of HHS in the elderly are associated with infection, a shortage of social support, cognitive impairment, or ADL decline. Although the survival rate in our series was high, the functional prognosis was impaired.

Will Catholic hospitals survive without government reimbursements?

This brief essay will begin to address the feasibility of operating a Catholic healthcare system without reimbursement from government healthcare programs such as Medicare and Medicaid. This question stems from the recent ACA/HHS "Nondiscrimination in Health Programs and Activities" final rule. Summary : The average hospital in the United States receives 40-50 percent of its net revenues from governmental sources. Participation in Medicare is contingent upon the hospital having a Medicare provider agreement. Participation in other governmental programs (Medicaid) as well as most commercial insurance is also contingent upon that agreement. Hospitals, including "Catholic" hospitals, cannot survive without a Medicare provider agreement. That agreement may be terminated for non-compliance with Medicare and other governmental regulations such as the recent ACA/HHS "Nondiscrimination in Health Programs and Activities" final rule, which could require "Catholic" hospitals to provide services which violate moral principals of the Church.

Are Range of Motion Measurements Needed When Calculating the Harris Hip Score?

BACKGROUND: Often the patient-reported outcome (PRO) component of the Harris Hip Score (HHS) is completed, but the physician-assessed range of motion (ROM) component is not. The PRO component only is called a modified Harris Hip Score (mHHS). The purpose of this study was to determine if a statistically significant or clinically meaningful difference existed when calculating the HHS with and without the physician-reported ROM portion. METHODS: Included patients had complete HHS data (both physician and PRO components). Surgical procedure (primary or revision) was recorded for each subject. American Society of Anesthesiologists score was divided into low and high groups. Body mass index was divided into 4 categories. The study used a repeated measures design. RESULTS: Data on 483 patients were collected between 12 and 60 months postoperatively (mean follow-up: 32.5 months, mean age: 55.9 ± 13.5 years). A mean difference of 4 points existed between the 2 groups: HHS group average score was 84.56 ± 13.18, and mHHS group average score was 88.74 ± 13.77. American Society of Anesthesiologists score, body mass index, and surgical type demonstrated a significant interaction with the HHS calculation method (P < .001). Primary total joint patients demonstrated a greater difference between the 2 scoring methods compared with revision patients. CONCLUSION: No clinically meaningful difference in outcomes was found between the mHHS and the HHS. The calculation of the HHS is dependent on the inclusion of the ROM measurement. However, the small point difference between the HHS and mHHS indicates that the mHHS is still useful as an accurate determinant of patient clinical outcome, and ROM assessment is not essential.

The Essential Need for Research Misconduct Allegation Audits.

Nearly 90 % of allegations of biomedical research misconduct in the United States are dismissed by responsible institutions without any faculty assessment or auditable record. Recently, members of the U.S. Congress have complained that the penalties for those against whom findings of research misconduct are made are too light and that too few grant funds associated with research misconduct have been recovered for use by other researchers and taxpayers. Here we discuss the laws that empower federal agencies that can oversee investigations of biomedical research misconduct: the Office of Research Integrity (ORI) and the Office of the Inspector General (OIG), both located within the Department of Health and Human Services (HHS). Research misconduct investigations pertaining to U.S. physical sciences funded through the National Science Foundation (NSF) are overseen by the NSF's OIG. While OIGs may provide some improvement over the ORI in the handling of research misconduct, we have found that a much more serious flaw exists which undermines an ability to conduct performance audits of the effectiveness by which allegations of research misconduct are handled in the United States. Specifically, sufficient data do not need to be retained by U.S. research institutions funded by HHS or NSF to allow effective audit of why allegations of research misconduct are dismissed before being seen by faculty inquiry or investigative committees. U.S. federal Generally Accepted Government Auditing Standards (GAGAS/Yellow Book), if applied to the research misconduct oversight process, would allow a determination of whether the handling of allegations of biomedical research misconduct actually functions adequately, and if not, how it might be improved. In particular, we propose that independent, external peer review under GAGAS audit standards should be instituted without delay in assessing the performance of ORI, or any other similarly tasked federal agency, in handling allegations of research misconduct.

Effect of heterotopic ossification on hip range of motion and clinical outcome.

The utility of heterotopic ossification (HO) classification systems is debatable. The range of motion and Harris hip score (HHS) were calculated in 104 patients with known HO after total hip arthroplasty and 208 matched controls without HO. The patients with HO were radiographically divided into high and low grade HO groups. There was no statistically significant association of HHS with high or low grade HO. High grade HO had a statistically significant 6° loss of terminal hip flexion, 4° loss of abduction, and 6° loss of internal rotation at the hip. The small changes in terminal hip range of motion and lack of association with HHS may be the result of false radiographic continuity resulting in an overestimation of the disability in high grade HO.

Building health care system capacity to respond to disasters: successes and challenges of disaster preparedness health care coalitions.

INTRODUCTION: This research aimed to learn from the experiences of leaders of well-developed, disaster preparedness-focused health care coalitions (HCCs), both the challenges and the successes, for the purposes of identifying common areas for improvement and sharing "promising practices." HYPOTHESIS/PROBLEM: Little data have been collected regarding the successes and challenges of disaster preparedness-focused HCCs in augmenting health care system preparedness for disasters. METHODS: Semi-structured interviews were conducted with a sample of nine HCC leaders. Transcripts were analyzed qualitatively. RESULTS: The commonly noted benefits of HCCs were: community-wide and regional partnership building, providing an impartial forum for capacity building, sharing of education and training opportunities, staff- and resource-sharing, incentivizing the participation of clinical partners in preparedness activities, better communication with the public, and the ability to surge. Frequently noted challenges included: stakeholder engagement, staffing, funding, rural needs, cross-border partnerships, education and training, and grant requirements. Promising practices addressed: stakeholder engagement, communicating value and purpose, simplifying processes, formalizing connections, and incentivizing participation. CONCLUSIONS: Strengthening HCCs and their underlying systems could lead to improved national resilience to disasters. However, despite many successes, coalition leaders are faced with obstacles that may preclude optimal system functioning. Additional research could: provide further insight regarding the benefit of HCCs to local communities, uncover obstacles that prohibit local disaster-response capacity building, and identify opportunities for an improved system capacity to respond to, and recover from, disasters.

Succinylacetone as primary marker to detect tyrosinemia type I in newborns and its measurement by newborn screening programs.

Tyrosinemia type I (TYR I) is caused by autosomal recessive fumarylacetoacetate hydrolase deficiency and is characterized by development of severe liver disease in infancy and neurologic crises. If left untreated, most patients die of liver failure in the first years of life. Intervention with medication is effective when initiated during the first month of life. This improvement in the treatment of TYR I patients influenced the decision to include TYR I in the US Secretary of the Department of Health and Human Services' (HHS) Recommended Uniform Screening Panel. However, while tyrosine is routinely measured in newborn screening (NBS) by tandem mass spectrometry (MS/MS), elevated tyrosine levels are not specific to TYR I. To improve the specificity of NBS for TYR I, several assays were developed to measure succinylacetone (SUAC) in dried blood spots (DBS). SUAC is a pathognomonic marker of TYR I, and its detection by NBS MS/MS is possible. This review of the current status of NBS for TYR I in the US is the result of discussions at the HHS Secretary's (Discretionary) Advisory Committee on Heritable Disorders in Newborns and Children about the inconsistent implementation of effective NBS for TYR I in the US. We sought to understand the different TYR I screening practices in US NBS programs. Results indicate that 50 out of 51 NBS programs in the US screen for TYR I, and a successful SUAC performance evaluation scheme is available from the Centers for Disease Control and Prevention. Programmatic and methodological barriers were identified that prevent widespread adoption of SUAC measurements in NBS laboratories. However, since SUAC detection is currently the best approach to NBS for TYR I, a further delay of the addition of SUAC measurement into NBS procedures is discouraged. SUAC measurement should improve both the false positive and false negative rate in NBS for TYR I thereby yielding the desired benefits for affected patients at no expense to the overall population served.

Three Siblings With a Rare Familial Hyperphosphatemia Syndrome: A Case Series.

Hyperphosphatemia familial tumoral calcinosis (HFTC) and hyperphosphatemia hyperostosis syndrome (HHS) are rare autosomal recessive disorders caused by mutations in the polypeptide N-acetylgalactosaminyltransferase 3 (GALNT3), fibroblast growth factor 23 (FGF23), or klotho (KL) genes. They are characterized by hyperphosphatemia and recurrent episodes of bone lesions with hyperostosis and/or soft tissue calcinosis. Management options include phosphate-lowering therapies, anti-inflammatory medications, and surgical excision of the calcified masses in significantly disabled cases. We describe three cases from a consanguineous family who were found to have the same genetic mutation caused by a homozygous mutation in intron eight of GALNT3 c.1524+1 G>A (IVS8+1). The first case had a presentation similar to chronic osteomyelitis, while the second one presented with a calcified mass in her gluteal area. The third case presented with left leg pain. Being a rare disease, the findings of tumoral calcinosis/ bony abnormalities, along with elevated phosphate levels, should raise the possibility of this entity. Family history and biochemical findings can help reach the diagnosis.