History Of Avascular Necrosis Research Articles

Outcomes following total ankle total talus replacement: A systematic review

BackgroundProsthetic substitution of the talus presents a significant challenge to the foot and ankle surgeon. The shear and compressive forces on the talus and its tenuous blood supply lead to high rates of avascular necrosis and eventual talar collapse. The purpose of this systematic review is to evaluate whether total ankle total talus replacement (TATTR) leads to improved clinical and radiographic outcomes with appropriate safety metrics in patients with a history of avascular necrosis or significant trauma. MethodsWe searched the concepts of talus, prosthesis, and arthroplasty in MEDLINE (PubMed), Embase (Elsevier), CINAHL Complete (EBSCOhost), and Scopus (Elsevier) from the database's inception through March 9, 2023. Inclusion Criteria were 1) previous trauma to the talus, 2) post-traumatic arthritis to the tibiotalar joint, 3) avascular necrosis of talus, 4) multiple failed prior interventions, 5) degenerative osteoarthritis to the tibiotalar joint, and 6) inflammatory arthropathy to tibiotalar joint. Patients less than 18 years of age and manuscripts in non-English languages were excluded. ResultsOf the 7625 references, 16 studies met the inclusion criteria, yielding data from 136 patients (139 ankles). The studies varied in design, with case reports and retrospective case series being predominant. The overall weighted average modified Coleman Methodology Score (mCMS) was 70.4 out of 100, indicating moderate flaws in study design that may be subject to various forms of bias and possible confounders. Demographics showed a diverse range of etiologies, with alumina ceramic being the primary prosthesis material. Functional scores demonstrated improvements in dorsiflexion and plantarflexion, although patient-reported outcome measures (PROs) were inconsistently reported. Complications included fractures, heterotopic ossification, prolonged wound healing, and infections. Revision details were sparsely reported. ConclusionTATTR is a promising treatment modality for improving short-term functional outcomes for patients with avascular necrosis or trauma-related issues. However, this systematic review underscores the need for standardized reporting, longer-term follow-ups, and further research to establish the procedure’s efficacy and safety, particularly in comparison to other treatment modalities. Level of EvidenceIII, Systematic Review of Level IV Studies

Sex-Related Differences in Fetal Hemoglobin Levels in Sickle Cell Patients

Sickle cell disease (SCD) continues to be the most common inherited blood disorder in the United States, characterized by a mutated HBB gene in hemoglobin subunit β (Kato 2018; Sedrak 2023). Currently, hydroxyurea is the gold standard treatment for SCD due to its mechanism of raising fetal hemoglobin (HbF) levels, ultimately lessening pain crises and need for blood transfusions (Acharya 2023). Prior to widespread usage of hydroxyurea, significant differences were found regarding HbF levels between males and females. In many cases, females were found to have, on average, higher HbF levels (El-Hazmi 1994; Ceglie 2019; Solovieff 2010; Sutnick 1968). The aim of this study is to determine whether sex-related differences presently exist in HbF levels in patients with SCD after Hydroxyurea use. This is a cross-sectional study conducted at the Center for Blood Disorders in Augusta, Georgia, consisting of a total of 496 patients diagnosed with SCD. Variables analyzed from this database included sex (male v. female), levels of HbF, and common complications of SCD: pulmonary hypertension, retinopathy, avascular necrosis, and thrombosis. Levels of HbF were calculated based on percentage and averaged. Two-sided t-test was used to identify differences between male and female HbF levels. Correlation of common complications of SCD with sex was observed using Pearson's coefficient and Fisher's z-test using R version 4.2.3. P value <0.05 was taken as significant. To control for confounding factors, HbF levels and age were included as covariates in the logistic regression when exploring the relationship between sex and complications of SCD. The 496 SCD patients consist of 233 males and 263 females. The males had a mean HbF of 8.71 (SD 9.84), and the females had a mean HbF of 9.83 (SD 10.4). A two-sided t-test resulted in a p-value of 0.21. 85 patients (45 female) had a history of pulmonary hypertension. The correlation between female sex on likelihood of developing pulmonary hypertension controlling for age and HbF was -0.24 (SE 0.22).121 patients (66 female) had a history of retinopathy. The correlation between female sex and the likelihood of developing pulmonary hypertension controlling for age and HbF retinopathy was -0.0066 (SE 0.21).177 patients (86 female) had a history of avascular necrosis. The correlation between female sex and likelihood of AVN controlling for age and HbF was -0.43 (SE 0.19).131 patients (72 female) had a prior thrombotic event. The correlation between female sex and likelihood of thrombotic event controlling for age and HbF was -0.12 (SE 0.19). The findings reveal no significant relationship between sex and HbF levels. We posit that this may likely be due to the effect of hydroxyurea treatment for SCD, as prior to treatment, there were significantly higher HbF levels in females (El-Hazmi 1994; Ceglie 2019; Solovieff 2010; Sutnick 1968). Adjusting for age and HbF levels, we found that there were no significant differences between the sexes in terms of risk for developing complications of pulmonary hypertension, sickle cell retinopathy, and thrombotic events. We did find that there was a significant difference between the sexes in terms of risk for developing avascular necrosis, where females were shown to have a decreased risk compared to males. Due to our limitation with sample size, next steps would entail analyzing these relationships with a larger database. With a more extensive data set of patients with SCD, further relationships may be elucidated regarding sex differences in the progression of disease that could aid in informed treatment decisions.

Contemporary Obstetric and Neonatal Outcomes in Sickle Cell Disease: A Retrospective Cohort Study.

Sickle cell disease is associated with adverse perinatal outcomes. Aspects of sickle cell disease in pregnancy, such as health care utilization and neonatal abstinence syndrome, are understudied. We aimed to describe contemporary sickle cell disease outcomes in a U.S. hospital system to improve perinatal counseling. We conducted a retrospective cohort study of patients with sickle cell disease who delivered at >20 weeks' gestation at two sites within the University of Pennsylvania Health System from May 1, 2017 to August 30, 2020. Descriptive statistics were utilized. Over the study period, 48 patients with sickle cell disease had 52 deliveries of 53 neonates. Sickle cell disease-related morbidity was prevalent prior to pregnancy; 27% had a history of avascular necrosis, and 58% had experienced acute chest syndrome. In the year prior to pregnancy, 52% used daily opioids. During pregnancy, more than half of patients were admitted at least once for sickle cell disease-related complications, spending a median 3 days admitted interquartile range (0-23); >10% spent >70 days of pregnancy admitted. New daily opioids were prescribed during pregnancy for 10% to manage pain crises. Acute chest syndrome was experienced by 23% of patients during pregnancy, and 8% required placement of long-term intravenous access. Preterm delivery <37 weeks occurred in 48%. The primary cesarean rate in nulliparas was 43%. Additionally, 50% experienced a hypertensive disorder of pregnancy, 35% underwent transfusion during delivery admission, and 10% had a perinatal venous thromboembolism. Finally, 53% of neonates were admitted to the intensive care unit. Low birth weight was noted in 34%, severe respiratory distress in 15% of infants, and neonatal abstinence syndrome in 21%. Sickle cell disease remains associated with significant perinatal morbidity and need for hospitalization. These data provide contemporary outcomes to target improvements in the care of patients with sickle cell disease. · SCD was associated with significant perinatal morbidity and healthcare utilization.. · Most patients with SCD required hospitalization during pregnancy.. · Neonates of patients with SCD experienced preterm birth, NICU admission, and neonatal abstinence syndrome..

Characterizing Frailty in Adults with Sickle Cell Disease Using Frailty Phenotype

Background As individuals with sickle cell disease (SCD) age, they acquire both SCD and age-related complications early in life leading to premature functional decline. Chronologic age does not accurately represent health status. Frailty is a syndrome that frequently occurs with aging, characterized by decreased physiologic reserve and increased vulnerability to stressors. It leads to adverse outcomes such as loss of independence and death. Individuals with SCD experience stressors such as frequent hospitalizations for vaso-occlusive painful events and organ damage; however, frailty has not been defined in people with SCD. This study seeks to assess the feasibility of characterizing frailty in older adults with SCD using the Fried Frailty Phenotype (FP). Methods We enrolled younger (age 18-39) and older (age ≥ 40 years) adults with SCD in a single-center cross-sectional study. Frailty was defined as the presence of ≥ 3 of the following criteria: unintentional weight loss (≥ 10 pounds in the last year), exhaustion (by 2-item questionnaire), weakness (seated grip strength < 27 kg for males and < 16 kg for females), slowness (usual gait speed ≤ 0.8 m/s), and low physical activity by questionnaire (energy expenditure < 383 kcal/week for men and < 270 kcal/week for women). Individuals with 1 or 2 criteria present were categorized as 'prefrail' and individuals with none of the criteria were categorized as 'robust'. We summarized demographic, medical history, and scores for each FP criterion. Feasibility end points were the proportion of participants who completed all portions of the FP assessment, acceptability of the assessments, and adverse events. We described the characteristic of participants in each FP group. Results We assessed FP at steady state in 28 participants. 93% (n=26) of participants had complete data (1 did not complete all surveys and 1 did not complete grip strength assessment both due to limited time in clinic). There were no adverse events related to performing FP. No participants reported that FP measures were difficult to understand or upsetting. The mean age of all participants was 46.3 (range 19-77 years). The mean age was 28.8 (± 5.1) for younger adults and 54.6 (± 8.7) for older adults. Most (68%) were aged ≥ 40 years and female (71%). Most (57%) had HbSS, 32% had HbSC, 7% had HbSβ0-thalassemia, and 4% had HbSβ+-thalassemia. Out of the 26 participants with complete data, 1 participant met criteria for being frail, 58% (n=15) were prefrail, and 38% (n=10) were robust. The proportion of participants positive for each FP criterion is shown in Figure 1. Out of the 27 participants who completed survey data, 7% (n=4) met criteria for weight loss. Several (41%) participants met criteria for exhaustion with 11 participants reporting that they felt that "everything was an effort" and 3 participants reported that they also felt that they "could not get going" at least a moderate amount of the time. The median grip strength was 30 kg (IQR 25-37) with median of 26 kg (IQR 21-32) for women and 53 kg (IQR 42-51) for men. No participants met criteria for weakness. On usual gait speed assessment, the median gait speed was 1.17 m/s (IQR 1.02-1.22). No participants met criteria for slowness. When we compared characteristics between prefrail/frail versus robust participants (Table 1), prefrail/frail participants had a significantly shorter six-minute walk distance (6MWD) (414 vs 492 meters; p=0.03) and had more participants hospitalized in the last year that approached significance (38% vs 0%; p=0.053). There was no difference in age, income, employment status, hemoglobin, hydroxyurea use, opioid use, history of avascular necrosis, or history of stroke. Conclusion We demonstrated that measuring frailty using Fried Frailty Phenotype was feasible, acceptable, and safe in both younger and older adults with SCD. Most participants met criteria for being prefrail due to exhaustion. Prefrail/frail participants in our study had a shorter 6MWD and more participants hospitalized in the last year, which suggests there is clinical significance in identifying prefrail individuals with SCD. This may facilitate early initiation of interventions to prevent frailty. Regular exercise has been shown to reduce exhaustion and frailty in older adults in the general population. In future studies we will assess the prevalence of frailty in a large sample of adults with SCD and determine the appropriate cut points for each criterion. Figure 1View largeDownload PPTFigure 1View largeDownload PPT Close modal

Outcomes Following Total Talus Replacement: A Systematic Review

Category: Ankle; Hindfoot; Trauma Introduction/Purpose: Prosthetic substitution of the talus presents a unique challenge to the foot and ankle surgeon. The shear and compressive forces on the talus and its tenuous blood supply lead to high rates of avascular necrosis with possible talar collapse. The purpose of this systematic review is to evaluate whether total talus replacements (TTR) lead to improved clinical outcomes with appropriate safety metrics in patients with a history of avascular necrosis or significant trauma. Methods: Concepts of talus and arthroplasty were searched in MEDLINE (PubMed), Embase (Elsevier), CINAHL Complete (EBSCOhost), and Scopus (Elsevier) from the database's inception through November 4, 2021. Inclusion Criteria were 1) previous trauma to the talus, 2) post-traumatic or degenerative arthritis to the tibiotalar joint, 3) avascular necrosis of talus, 4) multiple failed prior interventions, 5) degenerative osteoarthritis to the tibiotalar joint, and 56) inflammatory arthropathy to tibiotalar joint. Manuscripts in non-English languages or those with concomitant total ankle arthroplasty or revision arthroplasty were excluded. All study designs were eligible according to the Journal of Bone and Joint Surgery criteria level I-IV. The modified Coleman Methodology Score was used to evaluate study quality and the Methodological Index for Non-Randomized Studies criteria was used to assess risk of bias. Results: Twenty-two studies of 191 patients (196 tali) at an average age of 50 (14-80) years were included (Figure 1a). Nineteen studies utilized third generation implants, two studies used first generation (n=9), and one study used second generation implants (n = 14) made of ceramic (n=82), cobalt chrome (n=49), or titanium (n=23) (Figure 1b). Radiographic and patient-reported outcomes are outlined in Figure 1c. The most common adverse outcome was osteosclerosis of the tibia (n=24), calcaneus (n=19), and navicular (n=5). Additionally, hindfoot varus (n=3), ankle discomfort (n = 3), decreased subtalar motion (n=7), periprosthetic fracture (n=1), calcaneal fracture (n=1), delayed wound healing (n=3), hindfoot valgus (n=1), prosthetic stem sunken into the talar neck (n=1), superficial peroneal nerve neuroma (n=1), loosening (n=2) were reported. Eight revisions were reported. Conclusion: TTR is an appealing option to maintain range of motion through the tibiotalar joint and allow for maintenance of more normal foot and ankle biomechanics. Given promising early- and mid-term outcomes, TTR is becoming more widely used in the setting of talar collapse. Still, high complication rates, especially adjacent joint osteoarthritis, may remain under-reported in the literature given inadequate long-term follow-up. Future research should aim to expand upon the indications of TTR, elucidate survivorship and complication rates, and directly compare TTR to existing forms of salvage options for advanced talar avascular necrosis.

An Empirical Classification of Chronic Pain Subgroups in Pediatric Sickle Cell Disease: A Cluster-Analytic Approach

Introduction: Youth with sickle cell disease (SCD) and chronic pain are a heterogeneous group with variability in their daily pain experience and physical and psychosocial functioning. We aimed to 1) empirically derive chronic pain subgroups based on sensory pain characteristics using cluster analysis within a sample of youth with chronic SCD pain, and 2) investigate derived subgroups for differences in sociodemographics, clinical characteristics, and psychosocial and functional outcomes. We hypothesized that chronic SCD pain subgroups with higher sensory pain experiences would be associated with poorer functional and psychosocial outcomes.Methods: Children and adolescents receiving care at comprehensive SCD clinics at three tertiary care locations within a southeast children's hospital were included if they were aged 10-18 years, any SCD genotype, reported chronic pain (i.e., pain on most days per month for a duration of at least 3 months), and had English fluency. Youth were excluded if they had comorbid medical conditions typically associated with pain but unrelated to SCD or had significant cognitive or developmental limitations that would interfere with study procedures. Patients completed a battery of patient-reported outcomes including pain characteristics (i.e., intensity, frequency, and the Adolescent Pediatric Pain Tool to assess number of pain locations and pain quality descriptors), PROMIS Pediatric Short Forms for pain interference, anxiety, and depressive symptoms, the Adolescent Sleep Wake Scale for sleep quality, and the Pain Catastrophizing Scale. Clinical characteristics and healthcare utilization outcomes were abstracted from electronic medical records including number of inpatient admissions for pain and emergency department visits for pain in the prior 12 months.Chronic SCD pain subgroups were based on sensory pain characteristics including pain intensity ratings, pain frequency, number of body sites affected by pain, and pain quality descriptors. Hierarchical cluster analysis informed the number of clusters at the patient level. K-means cluster analysis was used to assign patients to clusters once the number of clusters was established. Clusters were compared on sociodemographics, clinical characteristics, healthcare utilization, and child psychosocial and functional outcomes.Results: Youth (n=62) were on average (M) 13.9 years old (SD=2.5), 56% female, 95% Black or African American, and 85% Non-Hispanic/Latinx. Most (75%) had HbSS or HbSβ 0 and 67% were prescribed hydroxyurea. Hierarchical cluster analysis and k-means clustering supported a 2-cluster solution (see Figure 1). Cluster 1 (n=35; Frequent, Moderate Pain) was distinguished by significantly lower scores on worst pain intensity (M=6.4, SD=0.4), lower number of pain days per month (M=12.1, SD=2.8), fewer number of body sites affected by pain (M=8.9, SD=0.9), and lower pain quality ratings (M=15.9, SD=1.3). Cluster 2 (n=27; Almost Daily, High Pain) represented patients who reported high ratings of worst pain intensity (M=8.2, SD=0.3), daily to almost daily pain (M=20.3, SD=1.7), higher number of body sties affected by pain (M=12.5, SD=1.5), and higher ratings of pain quality (M=40.8, SD=1.9) (all p's <.05). There were no differences between chronic SCD pain subgroups by sociodemographics (e.g., age, sex, family income), clinical characteristics (e.g., genotype, history of avascular necrosis, disease-modifying treatments, prescribed long-acting opioids, neuropathic medications, or antidepressants), or healthcare utilization. Patients in the Almost Daily High Pain subgroup reported significantly higher pain interference, depressive symptoms, and pain catastrophizing compared to patients in the Frequent, Moderate Pain subgroup (see Table 1). There were no differences between subgroups on anxiety or sleep quality.Conclusions: Two subgroups of chronic SCD pain were identified based on pain, psychosocial, and functional outcomes. Beyond sensory pain characteristics, pain interference, depressive symptoms, and pain catastrophizing were the only variables that best differentiated the chronic SCD pain subgroups. These empirically derived subgroups are comparable to other non-SCD chronic pain subgroups in pediatrics and adults. Identifying homogenous chronic SCD pain subtypes can inform tailored assessment and management of chronic pain. [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Risk of Complications After THA Increases Among Patients Who Are Coinfected With HIV and Hepatitis C.

Individuals coinfected with both hepatitis C virus (HCV) and HIV represent a unique and growing population of patients undergoing orthopaedic surgical procedures. Data regarding complications for HCV monoinfection or HIV monoinfection are robust, but there are no data available, to our knowledge, on patients who have both HCV and HIV infections. We sought to determine whether patients with coinfection differed in terms of baseline demographics and comorbidity burden as compared with patients without coinfection and whether these potential differences were translated into varying levels of postoperative complications, mortality, and hospital readmission risk. Specifically, we asked: (1) Are there demonstrable differences in baseline demographic variables between patients infected with HCV and HIV and those who do not have those infections (age, sex, race, and insurance status)? (2) Do patients with HCV and HIV infection differ from patients without those infections in terms of other medical comorbidities? (3) Do patients with HCV/HIV coinfection have a higher incidence of early postoperative complications and mortality than patients without coinfection? (4) Is the frequency of readmission greater for patients with HCV/HIV coinfection than those without? The New York Statewide Planning and Research Cooperative System (SPARCS) database was used to identify patients undergoing THA between 2010 and 2014. The SPARCS database is particularly useful because it captures 100% of all New York State inpatient admissions while providing detailed demographic and comorbidity data for a large, heterogeneous patient population with long-term followup. Patients were stratified into four groups based on HCV/HIV status: control patients without disease, HCV monoinfection, HIV monoinfection, and coinfection. We sought to determine whether patients coinfected with HCV and HIV would differ in terms of demographics from patients without those infections and whether patients with HCV and HIV would have a greater risk of complications, longer length of stay, and hospital readmission. A total of 80,722 patients underwent THA between 2010 and 2014. A total of 98.55% (79,554 of 80,722) of patients did not have either HCV or HIV, 0.66% (530 of 80,722) had HCV monoinfection, 0.66% (534 of 80,722) HIV monoinfection, and 0.13% (104 of 80,722) were coinfected with both HCV and HIV. Multivariate analysis was performed controlling for age, sex, insurance, residency status, diagnosis, and comorbidities to allow for an equal comparison between groups. Patients with coinfection were more likely to be younger, male (odds ratio [OR], 2.90; 95% confidence interval [CI], 2.20-3.13; p < 0.001), insured by Medicaid (OR, 6.43; 4.41-7.55; p < 0.001), have a history of avascular necrosis (OR, 8.76; 7.20-9.53; p < 0.001), and to be homeless (OR, 6.95; 5.31-7.28; p < 0.001) as compared with patients without HIV or HCV. Additionally, patients with coinfection had the highest proportion of alcohol abuse, drug abuse, and tobacco use along with a high proportion of psychiatric disorders, including depression. HCV and HIV coinfection were independent risk factors for increased length of stay (OR, 1.97; 95% CI, 1.29-3.01; p < 0.001), having two or more in-hospital complications (OR, 1.64; 1.01-2.67; p < 0.001), and 90-day readmission rates (OR, 2.97; 1.86-4.77; p < 0.001). As the prevalence of HCV and HIV coinfectivity continues to increase, orthopaedic surgeons will encounter a greater number of these patients. Awareness of the demographic and socioeconomic factors leading to increased complications after THA will allow physicians to consider interventions such as in-hospital psychiatric counseling, advanced discharge planning, and coordination with social work and collaboration with HCV/HIV infectious disease specialists to improve patient health status to improve outcomes and reduce costs. Level III, therapeutic study.

Patterns of opioid use in sickle cell disease.

Pain, the hallmark complication of sickle cell disease (SCD), is largely managed with opioid analgesics in the United States, but comprehensive data regarding the long-term use of opioids in this patient population is lacking. The pain medication prescription records from a cohort of 203 SCD patients were analyzed. Twenty-five percent were not prescribed opioid medications while 47% took only short-acting opioids, 1% took only long-acting opioids, and 27% took a combination of short-acting and long-acting opioids. The median (interquartile range) daily opioid dose was 6.1 mg (1.7-26.3 mg) of oral morphine equivalents, which is lower than the published opioid use among patients with other pain syndromes. The dose of opioids correlated with the number of admissions due to vaso-occlusive crisis (VOC) (r = 0.53, P < 0.001). When the patients were grouped into quartiles based on daily dose opioid use, a logistic regression model showed that history of avascular necrosis (AVN) (OR: 2.87, 95% CI: 1.37-6.02, P = 0.005), 25-OHD levels (OR: 0.59, 95% CI: 0.38-0.93, P = 0.024) and total bilirubin concentration (OR: 0.64, 95% CI: 0.42-0.99, P = 0.043) were independently associated with opioid use quartiles. In conclusion, doses and types of opioid medications used by adult SCD patients vary widely. Our findings implicate AVN and lower vitamin D levels as factors associated with higher opioid use. They also suggest an association of higher bilirubin levels, possibly suggesting higher hemolytic rate, with lower opioid use. Am. J. Hematol. 91:1102-1106, 2016. © 2016 Wiley Periodicals, Inc.

Chronic Opioid Use Pattern in Adult Patients with Sickle Cell Disease

Background: Pain, the hallmark complication of sickle cell disease (SCD), is largely managed with opioid analgesics in the United States (1, 2). There is a common perception that SCD patients tend to use high dose of opioids chronically (3, 4), but comprehensive data regarding the long-term use of opioids in this patient population is lacking.Methods: A cohort of 359 adults (age ≥ 18 years old) with the diagnosis of SCD followed at University of Illinois Hospital in FY 2010-2013 was enrolled in a prospective natural history study. An outpatient clinic visit with comprehensive laboratory closest to the enrollment date was selected as the focal point of this report. A total of 140 patients had consistent outpatient follow-up defined as ³4 visits between 6 months prior to and 6 months after this clinic visit (the study period). Pain medication prescribing records, number of hospital admissions, and other clinical variables during the study period were collected and evaluated with descriptive statistics and Spearman correlation.Results: Among the 140 patients analyzed, 74% took short-acting opioid medications and 31% took long-acting opioid medications (Table 1). The median daily opioid dose was 4.8 mg oral morphine equivalents (OME) with an interquartile range (IQR) of 0 to 14.7 mg. Sixty-six percent of patients used less than 10 mg OME daily whereas 11% used more than 50 mg OME daily (Figure 1). Among the short-acting opioids, acetaminophen-hydrocodone was the most commonly used medication (34% of patients) followed by immediate-release morphine (23%) and acetaminophen-codeine (20%) (Table 2). Extended-release morphine was used by 24% of the patients. Twelve patients (8.6%) were prescribed with nonsteroidal anti-inflammatory drugs (NSAIDs), and 16% used medications treating neuropathic pain. The Spearman correlation test with Bonferroni correction showed that the dose of opioid usage was significantly associated with the number of hospital admissions due to vaso-occlusive crisis (VOC) (p<0.0001) (Table 3). Hydroxyurea use, hemoglobin genotype, history of avascular necrosis (AVN) and 25-OHD levels did not correlate with opioid dose.Summary: The doses and types of opioid medications used by adult SCD patients vary widely. The majority of patients use a relatively low dose (<10 mg OME daily). The dose of chronic opioid use has a strong correlation with the frequency of hospital admissions due to VOC, demonstrating the necessity of finding an alternative pain management approach in treating sickle cell pain.1. L. R. Solomon, J Natl Med Assoc102, 1025 (Nov, 2010).2. P. Tanabe, Z. Martinovich, B. Buckley, A. Schmelzer, J. A. Paice, J Emerg Nurs41, 227 (May, 2015).3. W. T. Zempsky, JAMA302, 2479 (Dec 9, 2009).4. B. S. Shapiro, L. J. Benjamin, R. Payne, G. Heidrich, J Pain Symptom Manage14, 168 (Sep, 1997).The relative frequency = the number of patients taking certain dose of opioids/total number of patients. [Display omitted] [Display omitted] [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Phenotype in patients with Gaucher disease and Parkinson disease

Previous reports have shown an increased risk for Parkinson disease (PD) among type 1 Gaucher disease (GD) patients. However, the number of the reported cases of GD/PD is small and it is unknown whether certain GD phenotypes are associated with an increased PD risk. To present GD and PD characteristics of adults affected by both diseases from a large Israeli GD clinic, and assess whether certain GD characteristics are associated with a higher risk for PD. Medical files of patients >18years between 1990 and 2010 were reviewed for PD signs or symptoms. Available patients with PD underwent an additional neurological examination. Demographic and GD characteristics were compared between GD patients with and without PD using t-test and Fisher exact. Kaplan-Meier survival curves were used to estimate age-specific risk for PD among males and females. Age-specific risk for PD was compared between males and females using Cox Hazard ratio model. 510 type 1 GD adults (233 males; 45.7%), were evaluated. 11 PD patients were identified (2.2%). Among those with GD/PD cognitive impairment was common (7/11). Two patients underwent successful deep brain stimulation (DBS). PD diagnosis was associated with male gender (81.8% versus 44.9% male, p=0.027) and older age (mean age, PD=62.8, non-PD=47.1, p=0.004). GD phenotype and severity did not differ between the two groups, including mean Zimran Severity Score index (7.7 versus 8.3), percent splenectomized (15.8% versus 27.3%), history of avascular necrosis (13.0% versus 27.3%) and percent ever treated with enzyme replacement (49.4% versus 45.5%). Male gender and older age are risk factors for PD among GD patients, but GD severity is not associated with increased risk for PD. Further research is required to assess which GD patients are at a higher risk for PD, and why.

Pulmonary hypertension associated with sickle cell disease: Clinical and laboratory endpoints and disease outcomes

Screening for pulmonary hypertension (pHTN) has not yet become routine in sickle cell disease (SCD), despite clinical evidence of its high prevalence and associated mortality. Our objectives are to identify clinical conditions and laboratory findings predictive of/or associated with pHTN. One hundred twenty-five adult outpatients with Hb SS, SC, SOArab, Sbeta(0), or Sbeta(+) thalassemia, who underwent echocardiography and/or right heart catheterization due to cardiorespiratory symptoms, were studied. pHTN was identified in 36% (28/77) of SS/Sbeta(0) and in 25% (12/48) of SC/SOArab/Sbeta(+) patients studied. In SS/Sbeta(0) patients, pHTN was associated with low hemoglobin, low GFR, increasing age, no history of treatment with hydroxyurea and a history of leg ulcers, with trends for associations with higher total bilirubin, LDH levels, systolic systemic blood pressure, history of avascular necrosis, seizures, and cerebrovascular events. Twelve (40%) of the SS/Sbeta(0) patients with pHTN had >or= 1+ proteinuria. (P<0.039). The presence of proteinuria correlated with lower GFR and had a high positive predictive value (0.60) for pHTN in subjects with SS/Sbeta(0). The data also provided evidence that pHTN in this population is associated with right heart failure, with echocardiographic evidence of right ventricle enlargement and pericardial effusion. This study confirmed that even relatively mild elevations in pulmonary pressure are associated with high prospective mortality (hazard ratio: 15.9). We concluded that pHTN has a high prevalence in all Hb S related syndromes and is associated with increased mortality in SS/Sbeta(0). Kidney dysfunction, as indicated by proteinuria or decreased GFR, also represents sufficient reason to screen for pHTN.

MRI-Negative, Bone Scintigram-Positive in Early Osteonecrosis of the Knees

Nontraumatic avascular necrosis (AVN) of bone is a well-reported complication of glucocorticoid therapy for immunologic and malignant disease. We present the case of a 13-year-old girl with no history of trauma who presented with a 5-day history of increasing pain in both knees after cord blood transplantation for acute lymphoblastic leukemia. Plain film and magnetic resonance imaging (MRI) were reported as normal. Bone scintigraphy revealed evidence of bilateral avascular necrosis in the distal femora. MRI subsequently became abnormal several weeks later. The case illustrates the natural history of AVN, in which changes that are detected by MRI can take several weeks to develop. The scintigraphic findings influenced early management of the condition.

Avascular necrosis after open reduction for congenital dislocation of the hip: analysis of causative factors and natural history.

We analysed the causative factors and natural history of avascular necrosis (AVN) after open reduction of 87 dislocated hips. AVN was observed in 37% of the hips, yet open reduction did not appear to contribute to the development of AVN and its prevalence was similar in hips treated by open or closed reduction in our institution. Only 45% of hips with AVN had a good late result. More containment procedures were required if AVN had occurred. Premature physeal closure was one late manifestation of this complication, which occurred following apparent partial AVN changes.