Radiographic osteoarthritis of the hip joint in Turkey

The prevalence of hip osteoarthritis (OA) varies greatly across different geographic areas. Limited data exist about the prevalence of radiographic hip OA in the Turkish population. The aim of this study was to estimate the prevalence of radiographic hip OA in Turkey utilizing a random sample. That sample consisted of radiographs filed at the Department of Urology, Gazi University Hospital, Ankara, and included patients aged 25 years and older (range 25-97 years). Plain supine abdominal radiographs and intravenous pyelographies were evaluated using the Kellgren and Lawrence (K&L) grading system. Evaluations were made of 1,248 hips in 682 patients (205 women and 477 men). Overall, 8.8% of the hips evaluated had radiographic hip OA (K&L grade 2 or higher). Both hips were visible in 566 patients. Of those patients 11.7% had radiographic hip OA in either one or both hips (9.4% of women and 12.6% of men), and 51.5% of whom had bilateral findings. Prevalence increased with age and was 1.9%, 16.1%, and 21.5% for age ranges 25-39, 40-54, and 55 years and older, respectively. Patients aged 40 years and older had significantly more radiographic hip OA than those younger than 40 years (P<0.001). Men had a higher frequency than women after the age of 40 years, but this did not reach statistical significance. In most patients radiographic findings indicated only mild disease. Three patients had grade 3 disease and only one patient (aged 68 years) had grade 4 disease. Among patients aged 40 years or older. 1.1% had grade 3 or 4 disease. These findings suggest that K&L grade 2 OA changes emphasizing osteophytes at hip joints are not uncommon in the Turkish population, especially among those aged 40 years and older. However, frequency of moderate or severe radiographic hip OA may be low.     

Avascular osteonecrosis after treatment of SARS: a 3-year longitudinal study

Objective  To investigate the relationship between avascular osteonecrosis (AVN) and corticosteroid treatment given to patients with severe acute respiratory syndrome (SARS).
Methods  Longitudinal study of 71 former SARS patients (mainly health care workers) who had been treated with corticosteroids, with an observation time of 36 months. Magnetic resonance images (MRI) and X-rays of hips, knees, shoulders, ankles and wrists were taken as part of the post-SARS follow-up assessments.
Results  Thirty-nine per cent developed AVN of the hips within 3–4 months after starting treatment. Two more cases of hip necrosis were seen after 1 year and another 11 cases of AVN were diagnosed after 3 years, one with hip necrosis and 10 with necrosis in other joints. In total, 58% of the cohort had developed AVN after 3 years of observation. The sole factor explaining AVN in the hip was the total dose of corticosteroids received.
Conclusion  The use of corticosteroids in SARS has been debated; opinions conflict about whether the immediate benefits in terms of saving lives compensate for the adverse effects, including AVN.     

Use of Direct Oral Anticoagulants in Patients with Sickle Cell Disease and Venous Thromboembolism: A Prospective Cohort Study of 12 Patients

Abstract

Patients with sickle cell disease have an increased risk of venous thromboembolism (VTE) and with a mortality 2-fold higher. The anticoagulation of VTE in a young population is an important question. Indeed, hemorrhagic complications of anticoagulation may occur more frequently than in the general population. The use of a direct oral anticoagulant (DOAC) is not recommended for VTE in patients with sickle cell disease because those patients were not included in the clinical studies. We aimed to study the safety of using DOACs in a prospective cohort of patients with sickle cell disease and VTE. We prospectively followed the cohort of all sickle cell disease patients undergoing recent DOAC treatment for VTE at a sickle cell disease reference center. Twelve patients received rivaroxaban for VTE (eight women and four men). The median age was 27?years (20–45). The sickle cell disease variants included homozygous Hb SS (HBB: c.20A>T) in eight patients, Hb S-β⁺-thalassemia (Hb S-β⁺-thal) in two, Hb S-β⁰-thal in one and Hb S-Hb C (HBB: c.19G>A) in one. The cumulative duration of follow-up was 3134?days under rivaroxaban treatment. There were two thrombotic events, including a patient with a double positivity of antiphospholipid antibodies. No major bleeding was observed, and 6/12 patients presented minor bleeding (epistaxis: n?=?4; anal fissure bleeding: n?=?1; menorrhagia n?=?4). Of these, 3/6 required their treatment to be switched to apixaban, which stopped the bleeding. Direct oral anticoagulants may be an alternative treatment for VTE in patients with sickle cell disease, except for an associated antiphospholipid syndrome.     

Acute septic arthritis in chronic osteonecrosis of the hip

Osteonecrosis is not well documented as a predisposing factor of septic arthritis despite such a relationship having obvious clinical significance. We report 4 patients with involvement of 5 hips with septic arthritis in established osteonecrotic joints. The etiologies of the osteonecrosis in our study included corticosteroid therapy, sickle cell disease and one case of idiopathic osteonecrosis. Osteonecrosis appears to render the hip more susceptible to hematogenously derived bacterial infection. In some cases, removal of the necrotic tissue may be necessary to cure the infection.     

Pulse oximetry and factors associated with hemoglobin oxygen desaturation in children with sickle cell disease

The observation of low transcutaneous arterial oxygen saturation (SaO2) in otherwise well sickle cell patients has lead to questions about the interpretation of pulse oximetry values in these patients. We undertook a prospective study of children with sickle cell disease to (1) determine the prevalence of, and factors associated with, low transcutaneous SaO2 in clinically well patients, (2) develop an algorithm for the use of pulse oximetry in acutely ill patients, and (3) assess the accuracy of pulse oximetry in these patients. Eighty-six clinically well children with hemoglobin (Hb) SS had a lower mean transcutaneous SaO2 than 22 Hb SC patients and 10 control subjects (95.6% v 99.1% v 99.0%, respectively; p < .001). In Hb SS patients, a history of acute chest syndrome and age greater than 5 years were associated with lower transcutaneous SaO2 (mean 93.8% for those with a history of acute chest syndrome v 97.8% for those without a history of acute chest syndrome, and 94.0% for patients > 5 years old v 97.2% for those < or = 5 years old; P < .001). These associations were not seen in Hb SC patients. During acute illness, Hb SS patients with acute chest syndrome had transcutaneous SaO2 values that were less than 96% and at least 3 points lower than measurements made when they were well. A nomogram was designed to aid in the interpretation of transcutaneous SaO2 in acutely ill Hb SS patients when a comparison value is not available. The accuracy of pulse oximetry was shown by the correlation between SaO2 measured by pulse oximetry and calculated by using the patient's oxygen dissociation curve and PaO2 (r = .97). This study provides evidence that Hb oxygen desaturation is not a universal finding among children with sickle cell disease and identifies factors associated with Hb oxygen desaturation. We conclude that pulse oximetry may be useful to assess whether progressive pulmonary dysfunction begins at an early age in Hb SS patients, and to assess acutely ill patients for the presence of hypoxemia associated with acute chest syndrome.     

Early treatment of avascular necrosis in systemic lupus erythematosus.

Avascular necrosis (AVN) of the hips is associated with significant disability, and the majority of established cases require major surgery. In a retrospective analysis of 185 patients with systemic lupus erythematosus (SLE) 13 (7%) were found to have AVN. Of these, six had Raynaud's phenomenon, all had been on corticosteroids, and one had digital vasculitis. The mean duration of corticosteroid therapy was two years (range four months to five years). Five patients developed AVN two to 10 years after discontinuing steroids. The mean duration of disease at the onset of AVN was 6.85 years (range 1-19 years), and the mean age at onset of AVN was 31 years. Ten patients had severe multisystem involvement. None of the patients abused alcohol. Surgery was performed on 11 hips. Three had total hip replacement for stages 3 and 4 and seven had core decompression for stages 1 and 2. AVN progressed in two (28%) of these patients. In another patient core decompression failed for technical reasons. She subsequently required total hip replacement. The early detection of AVN to avoid the need for major surgery is stressed.     

Limitations of Hb F as a phenotypic modifier in sickle cell disease: study of Kuwaiti Arab patients

Sickle cell disease is characterized by phenotypic heterogeneity and many genetic modifiers have been identified with elevated Hb F being the most recognized ameliorating factor. Kuwaiti sickle cell disease patients carry the India/Arab chromosomal haplotype, which is associated with elevated Hb F (on average ~22%) on account of the Xmn1 site in the (G)γ-globin gene promoter. Most patients had either Hb SS or Hb S-β(0)-thalassemia (β(0)-thal) and there are a few Hb SD compound heterozygotes. We have carried out longitudinal clinical studies of these patients to document the pattern of morbidity, spleen function, brain and hip magnetic resonance imaging (MRI) for prevalence of silent brain infarcts and avascular necrosis of the femoral head (AVNFH), respectively. In addition, pulmonary function, SPECT (single photon emission computerized tomography) brain cerebral blood flow and response of selected patients to hydroxyurea (HU) treatment were also studied. The Hb SS and Hb S-β-thal patients have a generally mild phenotype compared to sickle cell disease in other populations and most patients do not have their first pain crisis until about the age of 4 years. Spleen function is retained till late childhood; pneumococcemia and other severe bacterial infections are rare. Overt stroke and silent brain infarcts are uncommon in childhood (~3% prevalence) although SPECT reveals cerebral blood flow deficits in ~30%. Avascular necrosis of the femoral head is, however, common with a prevalence of ~26% in children and 50% in adults. There is brisk response to HU in patients with frequent pain crises, with marked increases in Hb F levels. Patients who are compound heterozygotes for Hbs S and D-Los Angeles, have the most severe phenotype despite Hb F levels of >20% and Hb S <30%. In conclusion, although the patients have a uniformly elevated Hb F level, there are still considerable phenotypic heterogeneity and other modulating genetic factors that require further studies.     

Limitations of Hb F as a Phenotypic Modifier in Sickle Cell Disease: Study of Kuwaiti Arab Patients

Sickle cell disease is characterized by phenotypic heterogeneity and many genetic modifiers have been identified with elevated Hb F being the most recognized ameliorating factor. Kuwaiti sickle cell disease patients carry the India/Arab chromosomal haplotype, which is associated with elevated Hb F (on average ～22%) on account of the Xmn1 site in the ^Gγ-globin gene promoter. Most patients had either Hb SS or Hb S-β⁰-thalassemia (β⁰-thal) and there are a few Hb SD compound heterozygotes. We have carried out longitudinal clinical studies of these patients to document the pattern of morbidity, spleen function, brain and hip magnetic resonance imaging (MRI) for prevalence of silent brain infarcts and avascular necrosis of the femoral head (AVNFH), respectively. In addition, pulmonary function, SPECT (single photon emission computerized tomography) brain cerebral blood flow and response of selected patients to hydroxyurea (HU) treatment were also studied. The Hb SS and Hb S-β-thal patients have a generally mild phenotype compared to sickle cell disease in other populations and most patients do not have their first pain crisis until about the age of 4 years. Spleen function is retained till late childhood; pneumococcemia and other severe bacterial infections are rare. Overt stroke and silent brain infarcts are uncommon in childhood (～3% prevalence) although SPECT reveals cerebral blood flow deficits in ～30%. Avascular necrosis of the femoral head is, however, common with a prevalence of ～26% in children and 50% in adults. There is brisk response to HU in patients with frequent pain crises, with marked increases in Hb F levels. Patients who are compound heterozygotes for Hbs S and D-Los Angeles, have the most severe phenotype despite Hb F levels of >20% and Hb S <30%. In conclusion, although the patients have a uniformly elevated Hb F level, there are still considerable phenotypic heterogeneity and other modulating genetic factors that require further studies.     

Phenotypic Diversity of Sickle Cell Disease in Patients with a Double Heterozygosity for Hb S and Hb D-Punjab

Phenotypic heterogeneity for sickle cell disease is associated to several genetic factors such as genotype for sickle cell disease, β-globin gene cluster haplotypes and Hb F levels. The coinheritance of Hb S (HBB: c.20A?>?T) and Hb D-Punjab (HBB: c.364G?>?C) results in a double heterozygosity, which constitutes one of the genotypic causes of sickle cell disease. This study aimed to assess the phenotypic diversity of sickle cell disease presented by carriers of the Hb S/Hb D-Punjab genotype and the Bantu [–?+?– – – –] haplotype. We evaluated medical records from 12 patients with sickle cell disease whose Hb S/Hb D-Punjab genotype and Bantu haplotype were confirmed by molecular analysis. Hb S and Hb D-Punjab levels were quantified by chromatographic analysis. Mean concentrations of Hb S and Hb D-Punjab were 44.8?±?2.3% and 43.3?±?1.8%, respectively. Painful crises were present in eight (66.7%) patients evaluated, representing the most common clinical event. Acute chest syndrome (ACS) was the second most prevalent manifestation, occurring in two individuals (16.7%). Three patients were asymptomatic, while another two exhibited greater diversity of severe clinical manifestations. Medical records here analyzed reported a significant clinical diversity in sickle cell disease ranging from the absence of symptoms to wide phenotypic variety. The sickle cell disease genotype, Bantu haplotype and hemoglobin (Hb) levels did not influence the clinical diversity. Thus, we concluded that the phenotypic variation in sickle cell disease was present within a specific genotype for disease regardless of the β-globin gene cluster haplotypes.     

Non-pharmacologic management of sickle cell pain

Patients with sickle cell disease (SCD) suffer from both acute and chronic pain. The latter includes avascular necrosis of the hip joints mostly in the adult population. It has a negative impact on the quality of life of affected individuals and is often associated with depression, disability, unemployment and dependence on opioid analgesics. In this study, we show that a non-pharmacologic approach to management with deep tissue/deep pressure massage therapy technique, including neuromuscular trigger point treatment with acupressure, in patients with SCD has a salutary effect on pain relief and quality of life.     

Non-pharmacologic Management of Sickle Cell Pain

Patients with sickle cell disease (SCD) suffer from both acute and chronic pain. The latter includes avascular necrosis of the hip joints mostly in the adult population. It has a negative impact on the quality of life of affected individuals and is often associated with depression, disability, unemployment and dependence on opioid analgesics. In this study, we show that a non-pharmacologic approach to management with deep tissue/deep pressure massage therapy technique, including neuromuscular trigger point treatment with acupressure, in patients with SCD has a salutary effect on pain relief and quality of life.     

Hemoglobin S/O(Arab): thirteen new cases and review of the literature

Hemoglobin S/O(Arab) (Hb S/O(Arab)) is a rare compound heterozygous hemoglobinopathy characterized by the presence of two variant beta-globin chains: beta6Glu --> Val (Hb S) and beta121Glu --> Lys (Hb O(Arab)). The diagnosis of Hb S/O(Arab) requires electrophoresis on both cellulose acetate and citrate agar, since Hb O(Arab) co-migrates with Hb C at alkaline pH and close to Hb S at acidic pH. To date only case reports and small series of patients with Hb S/O(Arab) have been described. To better characterize the clinical and laboratory aspects of this unusual disorder, we reviewed the Duke University Medical Center experience. We identified 13 African-American children and adults with Hb S/O(Arab) ranging in age from 2.7 to 62.5 years. All patients had hemolytic anemia with a median Hb of 8.7 gm/dL (range 6.1-9.9 gm/dL), and a median reticulocyte count of 5.8% (range 1.2-10.3%). The peripheral blood smear typically showed sickled erythrocytes, target cells, polychromasia, and nucleated red blood cells. All 13 patients have had significant clinical sickling events including acute chest syndrome (11), recurrent vasoocclusive painful events (10), dactylitis (7), gallstones (5), nephropathy (4), aplastic crises (2), avascular necrosis (2), leg ulcers (2), cerebrovascular accident (CVA) (1), osteomyelitis (1), and retinopathy (1). Four patients have died, including two from pneumococcal sepsis/meningitis at ages 5 and 10 years, one of acute chest syndrome at age 14 years, and one of multiorgan failure at age 35 years. We conclude that Hb S/O(Arab) disease is a severe sickling hemoglobinopathy with laboratory and clinical manifestations similar to those of homozygous sickle cell anemia.     

The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease

The acute chest syndrome (ACS), a pneumonia-like illness in sickle cell patients, is one of the most frequent causes of their morbidity and hospitalizations. Repeated ACS events may predict the development of chronic lung disease. ACS is reported as a frequent cause of death in these patients. We examine here the incidence and risk factors of ACS in 3,751 patients with sickle cell disease who were observed prospectively for at least 2 years (19,867 patient-years [pt-yrs]) as part of a multicenter national study group. The ACS, defined by a new pulmonary infiltrate on x-ray, occurred at least once in 1,085 patients (2,100 events). ACS incidence was higher in patients with homozygous sickle cell disease (SS; 12.8/100 pt-yrs) and in patients with sickle cell-beta(0) -thalassemic (9.4/100 pt-yrs), and lower in patients with hemoglobin (Hb) SC disease (5.2/100 pt-yrs) and patients with sickle cell-beta(+) thalassemia (3.9/100 pt-yrs). alpha-Thalassemia did not affect the rate of ACS incidence in SS patients. Within each Hb type the incidence was strongly but inversely related to age, being highest in children 2 to 4 years of age (25.3/100 pt-yrs in SS) and decreasing gradually to its lowest value in adults (8.8/100 pt-yrs in SS). In SS children (< 10 years of age), we documented an age-related within- person reduction in ACS attack rates. Adults with a higher ACS rate had a higher rate of mortality (from all causes) than those with low ACS rates. This increased rate of mortality might also have contributed to the decline in ACS rate with age. In multivariate analysis, other factors affecting incidence in SS patients were degree of anemia (lower ACS rates in patients with lower steady-state Hb levels) and fetal Hb (lower rates in patients with high fetal Hb). There was also a positive association between ACS rate and steady-state leukocyte count. The relationship of ACS rate to higher steady-state Hb levels in SS patients is unexplained but might be caused by increased blood viscosity.     

Clinical,hematological, and biochemical features of Hb SC disease

Twenty-seven patients were seen and followed at our Sickle Cell Center over a period of seven years. Their clinical, hematological, and biochemical features were determined and compared to those of patients with sickle cell anemia who were concurrently investigated. The data indicate that the mild anemia of hemoglobin (Hb) SC disease is slightly microcytic and hyperchromatic. Parameters of hemolysis and the complications of chronic hemolytic anemia (cholelithiasis, leg ulcers, hepatomegaly, and cardiomegaly) are milder in Hb SC disease than in sickle cell anemia. Asplenia and its sequelae (increased platelet count and reduced serum IgM levels) are less frequent in Hb SC disease. Cerebrovascular accidents and the decreased leukocyte alkaline phosphatase scores are similar in both diseases. Thromboembolic complications, retinopathy, and renal papillary necrosis are more frequent in Hb SC disease.     

Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease [see comments]

Within the Cooperative Study of Sickle Cell Disease, 694 infants with confirmed sickle cell disease were enrolled at less than 6 months of age. Information about the nature and frequency of complications was collected prospectively over a 10-year period. Painful crises and acute chest syndrome were the most common sickle cell-related events in homozygous sickle cell anemia (SS), hemoglobin SC disease (SC), and S beta thalassemia patients (overall incidence in SS patients of 32.4 and 24.5 cases per 100 person-years, respectively). Bacteremia occurred most frequently in SS children under 4 years of age and in SC patients less than 2 years of age. The mortality rate was low in this cohort compared with that found in previous reports. Twenty children, all with Hb SS, died (1.1 deaths per 100 person-years among SS patients). Infection, most commonly with Streptococcus pneumoniae and Hemophilus influenzae, caused 11 deaths. Two children died of splenic sequestration, 1 of cerebrovascular accident, and 6 of unclear causes. Two patients underwent cholecystectomies, and 17 underwent splenectomies after one or more splenic sequestration crises. The experience of this cohort should reflect closely the true clinical course of those children with Hb SS and Hb SC disease who are observed in sickle cell centers in the United States.     

Avascular necrosis of the hip in children with sickle cell disease and high Hb F: magnetic resonance imaging findings and influence of alpha-thalassemia trait

Avascular necrosis (AVN) of the hip is a common cause of morbidity in sickle cell disease (SCD). Its prevalence increases with age and predisposing factors include coexistent alpha-thalassemia trait, frequent vaso-occlusive crisis and a high hematocrit (Hct). SCD is relatively mild among Kuwaiti patients because of their elevated Hb F levels, but a subset exists with severe recurrent vaso-occlusive crises. We carried out a prospective magnetic resonance imaging (MRI) study of the hip in a group of patients being followed in the Pediatric Hematology clinics of Al-Mubarak and Al-Amiri Hospitals. The association of AVN with age, frequency of hospitalization, alpha-thal trait, steady-state Hb, Hct, Hb F, WBC and platelet counts was investigated. MRI was carried out with a 1.5-tesla GE unit with a super-conducting magnet. Thirty patients (19 males, 11 females) (23 SS and 7 SbetaThal) were studied. Their ages ranged from 6 to 17 years, with a mean of 9.8 +/- 3.5 years, and Hb F from 11 to 35% with a mean of 22.8 +/- 5.7%. Among the SS patients, 11 (47.8%) had coexistent alpha-thal trait (-3.7-kb deletion). A total of 8 (26.7%) patients (6 SS and 2 SbetaThal) had varying degrees of osteonecrosis of the hip. Four (36.4%) of the 11 SS patients with alpha-thal trait and 2 (16.7%) of those without alpha-thal trait had osteonecrosis. This difference is, however, not statistically significant (chi(2) = 0.3, p = 0.5). While there was also no significant difference in the mean age and hematological parameters (Hb, Hct, Hb F, WBC, platelets), the SS patients with osteonecrosis had a significantly higher number of hospitalizations for vaso-occlusive crisis in the preceding 3 years than those without osteonecrosis.     

The perioperative complication rate of orthopedic surgery in sickle cell disease: report of the National Sickle Cell Surgery Study Group.

Orthopedic disease affects the majority of sickle cell anemia patients of which aseptic necrosis of the hip is the most common, occurring in up to 50% of patients. We conducted a multicentered study to determine the perioperative complications among sickle cell patients assigned to different transfusion regimens prior to orthopedic procedures: 118 patients underwent 138 surgeries. The overall serious complication rate was 67%. The most common of these were excessive intraoperative blood loss, defined as in excess of 10% of blood volume. The next most common complication was sickle cell-related events (acute chest syndrome or vaso-occlusive crisis), which occurred in 17% of cases. While preoperative transfusion group assignment did not predict overall complication rates, higher risk procedures were associated with significantly higher rates of overall complications. Transfusion complications were experienced by 12% of the patients. Two patients died following surgery. Both deaths were associated with an acute pulmonary event. The 52 patients undergoing hip replacements experienced the highest rate of complications with excessive intraoperative blood loss occurring in the majority of patients. Sickle cell-related events occurred in 19% of patients, and surgical complications occurred after 15% of hip replacements and included postoperative hemorrhage, dislocated prosthesis, wound abscess, and rupture of the femoral prosthesis. There were twenty-two hip coring procedures. Acute chest syndrome occurred in 14% of the patients. Overall, decompression coring was a safer, shorter operation. A randomized prospective trial to determine the perioperative and long-term efficacy of core decompression for avascular necrosis of the hip in sickle cell disease is needed. In conclusion, this study demonstrates a high rate of perioperative complications despite compliance with sickle cell perioperative care guidelines. Pulmonary complications and transfusion reactions were common. This study supports the results previously published by the National Preoperative Transfusion in Sickle Cell Disease Group. These results stated that a conservative preoperative transfusion regimen to bring hemoglobin concentration to between 9 and 11 g/dl was as effective as an aggressive transfusion regimen in which the hemoglobin S level was lowered to 30%.     

ASEPTIC NECROSIS OF THE FEMORAL HEAD IN SICKLE-CELL DISEASE

Aseptic necrosis of the femoral head accounted for 75 (28.2%)of the 266 major skeletal complications seen in 207 patientswith sickle-cell disease in a 66-month period. Forty-five (60%)of the 75 patients were males. The onset of symptoms occurredbetween the ages of 10 and 29 years in 60 (80.0%) of the patients,and the mean age at onset was 20.8 (range 8–54) years.There were 37 patients with sickle-cell anaemia (SS) with 46hips affected by necrosis, and 38 patients with sickle-cellhaemoglobin C with 40 affected hips. Perthes-like changes occurredin 40 hips, osteochondritis dissecans-like lesion in one hipand severe hip deformity in 45 hips. Four of the five hips withPerthes-like necrosis which were treated by rotation upper femoralosteotomy had partial reconstitution of the femoral head, andall five were symptom-free. The other hips were treated conservativelywith generally poor results. KEY WORDS: Femoral head necrosis, Osteonecrosis, Anaemia, sickle-cell     

The Assessment and Sustainable Management of Sickle Cell Disease in the Indigenous Tharu Population of Nepal

Sickle cell disease is an inherited hemoglobinopathy associated with significant morbidity and mortality. Reports suggest a high sickle cell disease burden among the indigenous Tharu population of Nepal, who for centuries have inhabited regions where malaria is endemic. Unfortunately, health care resources are limited and often inaccessible for Tharu individuals suffering from sickle cell disease. We conducted a large-scale screening effort to estimate the prevalence of Hb S (HBB: c.20A>T) among the Tharu population and delivered community-based education sessions to increase sickle cell disease awareness. A total of 2899 Tharu individuals aged 6 months to 40 years in the rural district of Dang in Western Nepal were screened using a sickling test, of whom, 271 [9.3%; 95% confidence interval (95% CI): 8.3–10.4%] screened positive for Hb S. Those who screened positive were offered diagnostic gel electrophoresis testing. Of the 133 individuals who underwent diagnostic testing, 75.9% (n?=?101) were confirmed to be Hb AS heterozygotes, 4.5% (n?=?6) were confirmed to be Hb SS homozygotes and 19.5% (n?=?26) were false positives. These findings support a large burden of sickle cell disease among the Tharu population and highlight the importance of appropriate resource allocation and management. With a positive predictive value of 80.0% (95% CI: 73.0-87.0%), the sickling test in conjunction with raising local sickle cell disease awareness may be a simple and sustainable way to promote access to health resources.     

Bone marrow edema syndromes of the hip: MRI features in different hip disorders

The objectives of this study were to describe the essential magnetic resonance imaging (MRI) features of bone marrow edema syndromes affecting the hip joint. In addition, to evaluate the role of MRI in the assessment of hip joint involvement in different clinical settings that may share similar clinical findings. Thirty-four patients who complained of hip pain were studied consecutively. Of these, 21 were men (61.8%) and 13 were women (38.2%). After clinical assessment of possible hip disease, plain radiograph and MRI study of both hips were performed. The literature was searched using keywords: bone marrow edema, hip, and MRI. All patients had antalgic gait and limping. Initial clinical examination revealed painful limited internal and external rotation of the affected hip/hips suspect for hip disease. Unilateral hip involvement was identified in 31 patients (91.2%), and bilateral hip involvement was found in three patients (8.8%), with a total of 37 hips evaluated by MRI. The final diagnoses in our patients were: reactive arthritis (1), transient osteoporosis (7), avascular necrosis (10), osteoarthritis (2), tuberculous arthritis (4), septic arthritis (2), osteomyelitis (2), sickle cell anemia (2), lymphocytic leukemia (1), and femoral stress fracture (3). Bone marrow edema affecting the hip is neither a specific MR imaging finding nor a specific diagnosis and may be encountered in a variety of hip disorders due to different etiologies. MR imaging is the modality of choice when clinical examination is suspect for hip disease and plain radiographs are normal or equivocal. Early diagnosis and treatment is important in many of the disorders. The literature is reviewed regarding bone marrow edema of the hip.