Avascular Necrosis Of Hip Research Articles

Foetal haemoglobin and disease severity in sickle cell anaemia patients in Kampala, Uganda.

BackgroundSickle cell anaemia (SCA) is a major chronic health problem in Uganda. In patients with SCA, the level of foetal haemoglobin (HbF) has been found to be important in influencing the clinical course of the disease. Thus populations with high levels of HbF like those in Saudi Arabia have been described as having a milder clinical course with fewer complications as compared to populations with lower levels. Disease modifying drugs can increase the Hb F levels and modify the presentation of SCA.MethodsThis was a cross sectional study in which we determined foetal haemoglobin levels and examined the relationship between HbF levels and disease severity in SCA patients in Mulago Hospital, Kampala, Uganda. We consecutively enrolled 216 children aged 1 year to 18 years with SCA attending the Sickle Cell Clinic at Mulago Hospital whose guardians had given consent. The history included age at onset of initial symptoms and diagnosis, number of hospitalisations and blood transfusions and other complications of SCA (cardiovascular accidents, avascular hip necrosis and priapism). A detailed physical examination was performed to assess the current state and help describe the disease severity for each patient. Blood samples were drawn for HbF levels. HbF levels ≥10% was defined as high.ResultsOf the 216 children, (80) 37% had HbF levels ≥10%. Significant correlations were observed between HbF level and several clinical parameters independent of age including age at diagnosis (p value 0.013), number of hospitalisations (p value 0.024) and transfusions (p value 0.018) since birth.ConclusionA third of the children with SCA attending the Sickle cell clinic in Mulago Hospital have high HbF levels. Higher HbF level is associated with later onset of symptoms and presentation, and less severe disease characterised by fewer hospitalisations and blood transfusions. We suggest HbF levels should be determined at initial contact for patients with SCA to guide counselling and identify those who may need closer follow up and consideration for disease modifying drugs.

The descending branch of the lateral circumflex femoral artery: a reliable and robust alternative blood supply in the free fibular transfer for avascular necrosis of hip

Avascular necrosis (AVN) of the femoral head is a disastrous condition and even more tragic when affecting the young adult. The fact that AVN has a number of aetiological trigger conditions underpins the fact that ultimately there is loss of vascularity to the caput. Early aggressive treatment through core decompression and vascularised bone grafting has been shown to successfully arrest disease progression. We describe here in a patient series the descending branch of the lateral circumflex femoral artery and vein as reliable alternative vascular recipient vessels to convention for receiving the bone flap. Further refinements are described to expedite the procedure and promote success. It is believed that this is the first described experience of this technique for AVN of the hip.

OA01.07. A clinical study on role of panchatiktaksheerbasti and aabha guggula in the management of avascular necrosis of hip joint

Purpose: WAvascular necrosis is newly emerging disease with increasing incidence involving one or both hip joints. It is progressive disease however no drug is found yet in modern medicine to stop its progression. Hence to evaluate the effect of certain Ayurvedic treatment on the progression of this disease, this clinical study was done. Method : 1)10 patients having Avascular necrosis of either of hip joint less than grade III were selected irrespective of age, sex, religion and occupational status. 2) Two major complains i.e. Vankshan sandhishool (Pain) and Vankshan sandhigraha (Stiffness) were considered. 3) These two complains were scored as per severity before and after treatment. 4) Vidhivat Panchatikta ksheerbasti 120 ml in morning after breakfast and Aabha guggulu 500 mg in Pragbhakta kala (15 min. before lunch and dinner) with Goghrita orally was given daily for 1 month. 5) Pachan and Anuloman was given as per requirement during treatment. 6) The score before treatment was compared with the score after treatment. 7) The total relief in each complains was calculated in percentage. Result: 1) Total score of 10 patients for Vankshan sandhishool (pain) before treatment was 24 and after treatment was 10. The difference was of 14 and hence relief was 58.33%. 2) For Vankshan sandhigraha (stiffness), score was 24 before treatment and 12 after treatment. The difference was of 12 and the relief was 50%. 3) The combine score of 10 patients was 48 before treatment and it was 22 after treatment. The difference was of 26, the total relief was 54.166% Conclusion: The Ayurvedic treatment of Vidhivat Panchatiktaksheerbasti and Abha guggula was found to be effective in preventing progression of avascular necrosis of hip joint.

Magnetic resonance imaging in children with sickle cell disease—detecting alterations in the apparent diffusion coefficient in hips with avascular necrosis

Avascular necrosis (AVN) is a common morbidity in children with sickle cell disease (SCD) that leads to pain and joint immobility. However, the diagnosis is often uncertain or delayed. To examine the ability of apparent diffusion coefficient (ADC) measurements on diffusion-weighted imaging to detect AVN in children with SCD. ADC values were calculated at the hips of normal children (n = 19) and children with SCD who were either asymptomatic with no known previous hip disease (n = 13) or presented for the first time with clinical symptoms of hip pathology (n = 12). ADC values were compared for differences among groups with and without AVN using non-parametric statistical methods. The ADC values were elevated in the hips of children with AVN (median ADC = 1.57 × 10(-3) mm(2)/s [95% confidence interval = 0.86-2.10]) and differed significantly in pairwise comparisons (all P < 0.05) from normal children (0.74 [0.46-0.98]), asymptomatic children with SCD (0.55 [0.25-0.85]), and SCD children who had symptoms referable to their hips but did not show findings of hip AVN on conventional MRI or radiographs (0.46 [0.18-0.72]). Children with sickle cell disease have elevated apparent diffusion coefficient values in their affected hips on initial diagnosis of avascular necrosis.

Staging of hip avascular necrosis: is there a need for DWI?

No comprehensive study has been performed to stage avascular necrosis of the hip using diffusion-weighted imaging (DWI). To determine apparent diffusion co-efficient (ADC) alterations in hip avascular necrosis (AVN) and to determine variations of ADC values according to stages of disease. The study is approved by our institutional review board and local ethical committee. Written informed consent was present for each subject. Thirty-five femoral heads of 21 cases affected by AVN were included in the study. Control group consisted of both femoral heads of 10 healthy volunteers. The hips affected by AVN were staged according to Ficat and Arlet classification system from I to IV. All cases underwent to routine hip magnetic resonance imaging (MRI) and DWI performed with a single-shot fast spin echo sequence at a b value of 600 s/mm(2). The ADC values were calculated automatically by placing ROIs on AVN lesions in affected patients and both femoral heads of control group. The median ADC value obtained from femoral heads of control group and that from AVN lesions were compared by Mann-Whitney U test. The median ADC values of AVN lesions at different stages were compared by Kruskal-Wallis test. The median ADC value of normal bone measured in control group was 185.5 ± 133.2 x 10(-6) mm(2)/s. The median ADC value measured in hip avascular necrosis lesions was 988.0 ± 332.7 x 10(-6) mm(2)/s. ADC values in hip AVN lesions were statistically significantly higher than normal bone marrow (P<0.01). The median ADC values of hips with avascular necrosis at stage I, II, III, IV were 817.5 ± 172.1 x 10(-6) mm(2)/s, 902.0 ± 181.0 x 10(-6) mm(2)/s, 1200.0 ± 363.2 x 10(-6) mm(2)/s and 1024.0 ± 324.0 x 10(-6) mm(2)/s, respectively. There was no statistically significant difference among AVN lesions at stages I, II, III and IV (P>0.05). Although DWI is a promising imaging tool that provides valuable diagnostic information in hip AVN, it fails to distinguish between different stages, and therefore is of limited value.

Influence of Chronic Therapies on Bone Mineral Density In Children with Sickle Cell Anemia.

AbstractAbstract 1650 Introduction:Children with sickle cell anemia (SCA) have increased risk of developing osteoporosis at a young age. Neither the etiology nor the prevalence of osteoporosis in this population is well understood. Chronic therapies, such as hydroxyurea (HU) or chronic red blood cell transfusions (CRBCT) are used to prevent and/or treat acute and chronic complications in children with SCA. However, little is known about the effects of these treatment modalities on bone mineral density (BMD). The current study aimed to explore in detail, the patho-physiology of bone mineral metabolism in 3 groups of pediatric patients; those receiving either HU or PRBC or supportive care only. Aims and Objectives:The main objectives of this study were 1) to determine the prevalence of osteoporosis in children with SCA, 2) to determine if differences exist between the 3 groups with regards to bone mineral density. Other aims were to assess trends in markers of bone metabolism and other inflammatory markers in relation to treatment modalities and bone mineral density. Methods:Children with SCA ≥ age 10 years receiving HU or CRBCT or supportive care were considered for eligibility for this cross sectional study. Patients were excluded if they were on chronic daily steroids, had a history of avascular hip necrosis, or were non-ambulatory. Data collected included demographic information, body composition, pubertal staging, clinical markers of disease severity (pain crises, episodes acute chest syndrome etc), bone mineral density (DEXA z-scores, Hologic), and biochemical markers of bone metabolism (calcium, phosphorous, alkaline phosphatase, parathyroid hormone, urine Ca/Cr ratio, osteocalcin, osteoprogerin, and urine N-telopeptide and RANK-L ligand). Results:Thirty five children with SCA (Hb-SS) were included in the study. The mean age was 14 years (range 10–21 years) and 31% (11) were males. Fifteen children were on CRBCT, 12 patients were taking HU and 8 were receiving supportive care only. There were no differences in age, sex, body mass index and Tanner staging between these groups. All patients were found to be vitamin D deficient (serum 25(OH) vitamin D < 30ng/ml). Overall 11 (31%) patients had osteoporosis as defined by z-score of less than -2.0. Patients on CRBCT had a lower prevalence rate of osteoporosis (20%) and increased BMD than both the supportive group (38%) and the HU (42%) group (Table 1). However, there was no significant difference in vitamin D status, markers of bone turnover and hyperresorption among these three groups. In addition, there was no correlation between BMD and disease severity (pain crises, episodes acute chest syndrome etc), Vitamin D status and markers of bone resorption. Discussion and Conclusions:The prevalence of osteoporosis in our cohort of children with SCA is 31%. To our knowledge, this is the first study to evaluate BMD in children with SCA according to various modalities of treatment. CRBCT group had a lower prevalence of osteoporosis and increased BMD compared to the other 2 groups. This; however, was not statistically significant which could be due to a small sample size. The decrease in BMD in our cohort was not related to advancing age, pubertal status, vitamin D deficiency, and bone hyperresorption or disease severity suggesting abnormal bone formation as the underlying mechanism. Larger studies are needed to identify the difference in BMD amongst these treatment groups.Table 1Bone mineral content and osteoporosis in children with sickle cell anemiaGroupBone Mineral DensityNo. of Children with Osteoporosis (%)Lumbar SpineFemoral NeckWhole BodySupportive care (n=8)-1.675 (1.392)-1.100 (0.920)-0.925 (1.269)3 (37.5%)CRBCT (n=15)-0.707 (1.295)0.173 (1.280)0.247 (1.255)3 (20%)HU (n=12)-1.267 (1.697)-0.892 (1.120)-0.717 (1.245)5 (41.7%) Disclosures:No relevant conflicts of interest to declare.

Diagnostic value of SPECT versus SPECT/CT in femoral avascular necrosis

With the advent of single-photon emission computer tomography/computed tomography (SPECT/CT), its applications and indications have to be evaluated clinically for a more efficient and cost-effective use in patient management. This retrospective study investigated the clinical value of conventional Tc MDP SPECT against SPECT/CT in diagnosing hip avascular necrosis (AVN). This retrospective study evaluated all patients who underwent SPECT/CT during the period from 1 March 2008 to 31 July 2009 for possible femoral AVN for which MRI was contraindicated. The SPECT and SPECT/CT images were reviewed separately by two radiologists with different levels of experience in a double-blinded manner. The likelihood of AVN for each symptomatic hip was graded according to the level of confidence. Clinical outcome was considered as the gold standard. The sensitivity, specificity, and accuracy for SPECT and SPECT/CT by each radiologist was obtained and compared with the gold standard. The corresponding receiver operating characteristic (ROC) curve and area under the curve (AUC) were used to evaluate the diagnostic power of SPECT against SPECT/CT. A total of 22 patients and 24 symptomatic hips were analyzed. Seven hips (29%) were confirmed to have AVN. The AUCs obtained from ROC for trainee radiologist for SPECT vs. SPECT/CT were 0.828 and 0.916, respectively. The AUC for specialist radiologist increased from 0.916 to 0.941 with CT. We concluded that SPECT/CT is beneficial for the improvement of AUC in ROC on the diagnostic of hip AVN compared with SPECT alone.

Meningococcal purpura fulminans in children. II: Late orthopedic sequelae management

Purpura fulminans is a rare and extremely severe infection, mostly due to Neisseria meningitidis. Nineteen patients were followed up immediately after the initial multivisceral failure in order to diagnose late-onset orthopedic sequelae. We report our experience with these 19 patients, in light of our medical follow-up protocol and surgical management. Nineteen patients were referred for acute purpura fulminans between 1987 and 2005 to our institution and followed up prospectively until the present. We collected information on all diagnosed orthopedic sequelae, all surgical procedures performed, and the actual orthopedic outcome. Fourteen patients developed at least one orthopedic sequel after a mean of 2years delay, with a mean of 8.65years follow-up (range 3-22years). The most common presentation was lower limb physeal growth plate arrest in eight patients involving 18 growth plates, leading to five limb length discrepancies and 12 significant knee and/or ankle deviations. Patients were treated by completing epiphysiodesis in addition to limb lengthening and/or reaxation osteotomies, except for two patients, in which epiphysiolysis was performed. All outcomes are, to date, satisfactory, with both knee and ankle axes within the physiological range. Among the seven patients who underwent below-knee amputation, six needed stump revision because of skin conflict (4) or prosthetics misadaptation due to upper tibial varus (2). Regarding the upper limb, three patients presented with four cicatricial scar bands, one located on a ring finger, two at the first commissure, and one at the wrist (all were successfully treated by enlargement Z-plasties). Two patients developed hip avascular necrosis. It is important for children diagnosed with meningococcal purpura fulminans to be followed up closely starting from the very beginning by a pediatric orthopedic surgeon. It ensures that late-onset orthopedic sequelae will be diagnosed early. In accordance to the literature, this study highlights the high rate of lower limb epiphysiodesis, above all other types of sequelae. This study reports a possible link between purpura fulminans and avascular necrosis of the hip.

Percutaneous innominate pelvic osteotomy without the use of bone graft for femoral head coverage in children 2–8 years of age

Percutaneous innominate pelvic osteotomy without the use of bone graft as treatment for hip diseases is proposed as another tool to handle this pathology, which occurs frequently in our area, without changing postural correction at the level of the pelvis by secondary transiliac lengthening through an unilateral iliac bone graft at the lower extremity. This technique was developed by biomechanical studies in corpses and was later performed on children. This was a prospective, multicentric, longitudinal study of a case series in children between 2 and 8 years of age with developmental hip dysplasia, Legg-Calve-Perthes disease, aseptic or avascular necrosis, congenital or acquired lesions of the femoral head having obtained previous consent from the parents. In 121 patients with increases in femoral head coverage in a concentric hip joint, the average follow-up period was 4.1 years (range 6 months to 5 years). The vertical-center-anterior angle of Lequesne and the center-edge angle of Wiberg, both of which showed significant improvement in the coverage of the femoral head with an average of acetabular presurgical index of 38.2 degrees (P=0.002) and a postsurgical average of 19.8 degrees (P=0.003), angles of lateral uncovering of the femoral head of 12.3 degrees (P=0.0019) and a postsurgical angle of 23.2 degrees (P=0.004). The registered complications were 2.7% (P=0.047). The results offer many advantages over the current treatments on handling congenital diseases or acquired diseases of the hip and can indicate an alternative to the other methods of treatment which is possibly less aggressive and with a better future for the patient.

Stroke in Sickle Cell Anemia After Excellent Response to Hydroxyurea.

Abstract 4622 IntroductionStrokes occur in about 10% of children with sickle cell anemia (SS) less than 10 years old. These strokes are usually ischemic in nature. Stroke due to SS in adults is less common and is usually hemorrhagic in nature. We wish to report an unusual adult patient with SS and excellent response to HU who developed a stroke that was unrelated to SS. Case Report and ResultsA 35-year-old female with sickle cell anemia had mitral valve prolapse and migraine headaches presented 2 days after developing abrupt bilateral blurry vision, left facial numbness and weakness of her left leg. Her SS has been complicated by acute chest syndrome, bilateral hip avascular necrosis and frequent painful crises prior to hydroxyurea (HU) therapy. She was enrolled in the multicenter study of hydroxyurea (MSH) in SS and has been on 2500mg of HU per day for the past 13 years. She had an excellent response to HU with no recurrent acute chest syndrome and decreased need for blood transfusion. After starting HU, the frequency of crises requiring hospital admission decreased from 1 admission every 1 to 2 months to less than 1 admission per year except when hydroxyurea was discontinued for pregnancy. Her fetal hemoglobin increased from 6.1 % before HU to a maximum of 45%. Her MCV increased from 96 fl to a maximum of 132 fl and Hb from 8.0 g/dl to 9.8 g/dl Her exam was remarkable for left lower extremity weakness which was more pronounced proximally. All cranial nerves were intact and there was normal sensation bilaterally. CT scan of the brain showed three foci of hypodensity and MRI of the brain showed increased signal on T2, FLAIR and diffusion weighted images within the frontoparietal deep white matter consistent with infarction in the border zone of the middle cerebral artery (MCA)-anterior cerebral artery (ACA). MR angiography of the intracerebral and extracerebral vessels demonstrated focal narrowing of the right MCA at the trifurcation suggesting an embolic cause. Common causes of stroke were ruled out with routine studies. Her hemoglobin electrophoresis after admission but before blood exchange transfusion showed HbS of 55% and HbF of 45%. She underwent exchange transfusion 2 days after admission and was started on chronic blood exchange transfusions with the assumption that she had ischemic stroke due to SS. Initial transthoracic echocardiogram with contrast injection did not show an atrial shunt. Follow-up transesophageal echocardiogram after discharge showed a secundum atrial septal defect with a defect size of 1.4 cm. Right heart catheterization was performed and the pulmonary flow to systemic flow (Qp/Qs) was 1.7:1. An Amplatzer atrial septal defect (ASD) closure device was deployed with transesophageal echocardiographic guidance and a large thrombus was removed from the right atrium. At the patient's request exchange red cell transfusions were discontinued. The patient has continued treatment with hydroxyurea and aspirin. ConclusionsYoung patients with cryptogenic stroke have a much higher prevalence of atrial shunts and in particular patent foramen ovale than patients with other forms of stroke and therefore a cause-effect association is suggested. Young adults with stroke should be evaluated for common and reversible causes of stroke including paroxysmal emboli. Transesophageal echocardiography is the gold standard for diagnosing atrial shunts. Strokes in patients younger than age 55 are related to paroxysmal emboli and have a risk of recurrence of approximately 30% within one year. High risk features for stroke recurrence with an atrial shunt include hypercoagulable states, large opening and presence of an atrial septal aneurysm. Optimal management of patients with a stroke and an atrial shunt is unknown. Options include surgical closure, percutaneous device closure, anticoagulation and antiplatelet therapy. Patients with sickle cell disease and stroke should receive long term blood transfusions to reduce HbS below 30% if the stroke is felt to be related to sickle cell disease vasculopathy. The patient described with SS had a stroke and had an atrial septal defect that was repaired. The MRI/MRA findings are consistent with paroxysmal emboli. The patient is receiving treatment with hydroxyurea and aspirin having discontinued red cell exchange transfusions and at two years of follow-up has not had a recurrent stroke. Disclosures:No relevant conflicts of interest to declare.

Humoral immunodeficiency in congenital insensitivity to pain with anhidrosis

Congenital insensitivity to pain with anhidrosis (CIPA) is an autosomal recessive disorder caused by mutations in the neurotrophic tyrosine receptor kinase 1 (NTRK1) gene, which encodes the receptor for nerve growth factor. We report the clinical course of a 7-year-old girl with CIPA and proven NTRK1 mutation. In addition to recurrent dislocation of the left hip joint and avascular necrosis of the left talus, the patient also presented with recurrent infections secondary to hypogammaglobulinemia, a feature not previously known to be associated with CIPA. The patient was treated with regular administration of intravenous immunoglobulins. Conservative treatment of the recurrent left hip dislocation by cast immobilization and bracing was implemented to stabilize the joint. The implication of the immune system of the reported patient broadens the clinical phenotype associated with NTRK1 mutations.

Avascular necrosis after a steroid injection

I read with interest the Clinical Vista Brief about bilateral hip avascular necrosis.[1][1] I note that the corticosteroid injection was given 8 months before the condition was diagnosed, but the patient had complained of hip pain for 13 months. Cortisone injections (particularly bursa and tendon

Developmental dislocation of the hip is still important problem: Therapeutic guidelines

The authors are describing currently important problem--developmental dislocation of the hip. Guidelines for the treatment have been given according to literature date and upon their own experience. Therapeutic suggestions for the first twelve months of life are based on the ultrasound typing--it is advised to perform nonoperative treatment (abduction devices, "over head" traction, Pavlik harnesses). During the second year of life a pause in the treatment should be adviced in order to avoid postreduction avascular hip necrosis as a very important complication. After that period surgical treatment has to be done (open reduction, pelvic and femoral osteotomies). Special suggestions have been given for the treatment of consecutive leg length inequality and the deformities caused by postreduction avascular hip necrosis.

Comparison of metal-on-metal, ceramic-on-ceramic and XL-PE on metal articulations in total hip arthroplasty

Revision rates of total hip arthoplasty have decreased after introducing total hip arthroplasty using ceramic component due to reduce wear and osteolysis.29 year old male case of bilateral hip avascular necrosis operated for bilateral hip THA presented 30 months after with complaint of squeaking, x-ray showing left ceramic liner fracture without any history of trauma. Revision of ceramic liner and head with ceramic on polyethylene was done.Ceramic on ceramic articulation has the lowest wear rate among various articulations. However, there is a concern about ceramic implant fracture; therefore ceramic with polyethylene bearing could be a good choice.

Diffusion‐weighted MR study of femoral head avascular necrosis in severe acute respiratory syndrome patients

PurposeTo evaluate the apparent diffusion coefficient (ADC) of femoral head avascular necrosis (AVN) in severe acute respiratory syndrome (SARS).Materials and MethodsSeventy‐nine SARS patients with hip pain underwent both conventional and diffusion‐weighted MRI (b‐value = 0–1000 seconds/mm2). The abnormal regions on the diffusion‐weighted images were outlined by using the conventional images as guides, and the ADCs were calculated. The ADC differences between normal and AVN femoral heads were compared.ResultsOf the 158 hips examined, 28 had AVN (11 with bilateral hip AVN, three with right hip AVN, and three with left hip AVN). The mean ADC was markedly greater in the AVN femoral head (1.66 × 10–3 mm2/second ± 0.20) than in the normal femoral head (0.47 × 10–3 mm2/second ± 0.082; P < 0.0001). There was no overlap between the normal and AVN femoral heads.ConclusionDWI can provide valuable information regarding the diffusion properties of femoral head AVN, and markedly increased diffusion was identified in AVN. J. Magn. Reson. Imaging 2005. © 2005 Wiley‐Liss, Inc.

Acetabular Stress Fracture In A 69 Year Old Recreational Runner

HISTORY A 69 year-old female recreational runner who runs 3 miles a day presents to Sports Medicine clinic for left hip pain that radiates to the groin and has been progressively worsening for three weeks. She also attends a high-impact aerobics class three times per week. She has a previous history of sciatica but describes this pain as markedly different. She also describes left lower quadrant abdominal pain but has no GI symptoms. She denies fevers, chills, or other systemic symptoms of recent illness. PHYSICAL EXAM This is a well developed well nourished 69 year old who appears younger than the stated age. Examination of the hip reveals equal limb lengths, no tenderness to palpation of bony prominances of the hip, although there is tenderness to internal and external rotation of the hip. Pain to hip flexion and adduction against resistance but no pain to hip extension or abduction. Range of motion is bilaterally symmetric and equal. No catching or clicking with examination. Negative piriformis stretch test. She has severe inguinal tenderness. Negative straight leg raise. Negative FABER exam. Normal skin. She is neurovascularly intact. She has good lower extremity strength. Her gait is normal. Abdomen-benign, no bruits. DIFFERENTIAL DIAGNOSIS Hip flexor strain Osteoarthritis Stress fracture of the hip Acute fracture of the hip Avascular necrosis of the hip Transient osteoporosis of the hip Lumbar radiculopathy Gout/Pseudogout Tumor- TESTS AND RESULTS CBC: Hb-9.6 g/dL-stable for her IHT Hct: 27.9 % WBC 6.7*1000/cmm MCV 106.0. ((Secondary to hydrea) Platelets 592 *1000/cmm (stable for this patient) 24 hr urine studies for creat clearance- 102cc/minute, calcium clearance 202mg/24hrs Sed Rate-49 High CRP-2.02 High CPK 16 Normal CMP Normal Uric Acid-normal Colonoscopy- normal 2001, 2004 Imaging- Plain film 2 views femur-normal. Two views of hip-mild hip DJD, mild SI arthritis,-mild osteitis pubis 5 views L Spine-DJD L5-S1 otherwise normal MRI L SpineNo focal abnormalities of the lumbar spine. No disk extrusion or spinal stenosis Hip-Anterior acetabular stress fracture CT Scan Hip: Mild DJD posterior aspect of the hip CT Abdomen/pelvis-splenomegaly size of spleen larger than liver Dexa Scan-Mild Osteopenia-Femur/Hip T-score −1.1 Osteopenia-L Spine T-score −1.9 FINAL WORKING DIAGNOSIS Anterior Acetabular Stress Fracture Left Hip-No displacement by CT: TREATMENT Nonweight bearing crutch use for 4 weeks. Active ROM in all planes. No Tylenol/NSAID use to mask pain Fosamax for Osteopenia in addition to current use of estrogen, calcium and vitamin D Further treatment plan will be determined

CD14 promoter polymorphism in Chinese alcoholic patients with cirrhosis of liver and acute pancreatitis

To investigate the relationship between genetic polymorphism of the CD14 promoter and the occurrence of alcoholic cirrhosis and alcoholic pancreatitis, and to challenge the conclusion made earlier that the patients with acute alcoholic pancreatitis and patients with alcoholic cirrhosis of liver are two different subpopulations. Using the polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) method, we determined the polymorphism of CD14 gene and aldehyde dehydrogenase gene 2 (ALDH 2) in 335 alcoholic patients with different organ complications i.e., cirrhosis of liver (n = 100), acute pancreatitis (n = 100), esophageal cancer (n = 82) and avascular necrosis of hip joint (AVN) (n = 53) and 194 non-alcoholic controls in a Chinese group. The results showed that the carriage of T allele was not different among alcoholic patients with cirrhosis of liver, alcoholic patients with other complication and non-alcoholic controls. On the other hand, the carriage of the C allele was significantly more prevalent for alcoholic pancreatitis than for esophageal cancer (0.79 vs 0.60, P<0.001), alcoholic AVN (0.79 vs 0.65, P<0.025) and non-alcoholic controls (0.79 vs 0.68, P<0.025). Furthermore, when only subjects with ALDH2 1-1 genotype were examined, the C allele frequency was significantly more prevalent for alcoholic pancreatitis than for alcoholic liver cirrhosis (0.82 vs 0.69, P<0.025), esophageal cancer (0.82 vs 0.61, P<0.01), alcoholic AVN (0.82 vs 0.64, P<0.01) and non-alcoholic controls (0.82 vs 0.69, P<0.05). The C allele may be associated with some mechanism, which is important in the pathogenesis of alcoholic pancreatitis, and that alcoholic patients with acute pancreatitis and cirrhosis of liver are probably two different subpopulations.

Three-Dimensional Conformal Radiotherapy for Localized Prostate Cancer in Kidney Transplant Recipients

This is the first report of graft function and prostate cancer control in renal transplant recipients subjected to modern conformal radiotherapy. Eight kidney transplant recipients were treated with three-dimensional conformal radiotherapy. All patients but one were subjected to transitory hormonal deprivation. A three-dimensional radiotherapy-planning system (Pinnacle, Philips Medical System, Bothell, WA) was used to delineate anatomic contours on pretreatment computed tomography and for dose computation. The clinical target volume encompassed the prostate and was expanded with a 10-mm wide margin in all directions to obtain the planning target volume. The irradiation technique consisted of a nine-field arrangement delivering 70 Gy in 2-Gy fractions, with 18-MV photon beams. Biochemical recurrence was defined as two consecutive increases in prostate-specific antigen (>1.5 ng/mL). Graft function was monitored by creatinine clearance. Excretory profiles were assessed by furosemide-stimulated diethylenetriaminepentaacetic acid renography. All patients were subjected to hip magnetic resonance imaging to assess for avascular hip necrosis. After a mean follow-up of 28 months, two patients showed isolated biochemical recurrence and six patients remained free of recurrence. In seven patients with functional allografts, the creatinine clearance was unimpaired by treatment. However, significant obstruction of the terminal ureter was revealed in two patients by furosemide-stimulated diethylenetriaminepentaacetic acid renograms. The doses delivered to the uretero-neocystostomy were calculated to range from less than 20 Gy to more than 45 Gy depending on bladder repletion. Adequate cancer control was achieved at the expense of infraclinical ureteral obstruction. The doses delivered to the uretero-neocystostomy may be reduced by having a full bladder at the time of irradiation. No avascular hip necrosis was observed.

The 1226g (N370S) Gaucher mutation among patients with Legg–Calve–Perthes disease

Legg–Calve–Perthes disease (LCPD) is an avascular necrosis of the femoral head with an annual incidence of 5–15/100,000. The estimated incidence of Gaucher disease, a lysosomal recessive storage disease, is 1:850, with a carrier rate of 1:17.5 for the 1226G (N370S) mutation among Ashkenazi Jews in whom there is a predilection. Since clinical and radiological findings of avascular hip necrosis due to either Gaucher disease or LCPD may be indistinguishable, misdiagnosis may occur. The purpose of this study was to evaluate the incidence of 1226G Gaucher mutation in a cohort of radiologically confirmed LCPD patients (diagnosed 1986–2000) in Israel. Enzyme assay was performed for confirmation of affected versus carrier status in patients with the 1226G mutation. In all, 78 LCPD patients, 86% males, 51% with severe bone disease, were studied. Family history was negative for Gaucher disease. Ethnic origin was 39% Ashkenazi Jewish, 6% Arab, and 55% other ethnicities. One Ashkenazi Jewish LCPD patient was homozygous for the 1226G mutation, and 4 LCPD patients were carriers: 3 Ashkenazi Jewish and 1 Arab patient. The frequency of the 1226G mutation among the LCPD patients was increased relative to historical Ashkenazi Jewish Israeli controls ( P = 0.01). Since Gaucher disease may be misdiagnosed as LCPD, glucocerebrosidase enzyme testing is recommended among Ashkenazi Jewish children diagnosed with LCPD.

Femoral head perfusion and composition: MR imaging and spectroscopic evaluation of patients with systemic lupus erythematosus and at risk for avascular necrosis.

To measure fat content and relative marrow perfusion of the femoral head in patients at risk for avascular necrosis. Thirteen patients with systemic lupus erythematosus (SLE) without avascular necrosis and with long-term corticosteroid treatment and 12 healthy subjects were evaluated. Hip perfusion was assessed with gadolinium-enhanced T1-weighted magnetic resonance (MR) imaging. Marrow composition in the femoral head and neck was measured with hydrogen-1 MR spectroscopy. Percentage fat content in the femoral head was similar between the two groups (SLE patients, 77% +/- 5 [standard deviation]; healthy subjects, 78% +/- 6; P = .74). Rate of enhancement of the femoral head was significantly higher in the SLE than the healthy group (11%/min +/- 6 vs 6.9%/min +/- 3, respectively; P = .05), was inversely related to fat content in healthy subjects (r = -.74, P = .02), and was more variable in the SLE group (r = -.53, P = .11). Perfusion of the femoral head is inversely related to marrow fat content in healthy and is higher in SLE patients without hip avascular necrosis.