Bilateral Avascular Necrosis Of Femoral Head Research Articles

Twenty-Three-Year-Old Collegiate Soccer Athlete With Bilateral Femoral Head Avascular Necrosis: A Case Report

A 23-year-old collegiate club soccer player reported to his athletic trainer right hip flexor pain and no recalled mechanism. Diagnostic imaging revealed bilateral avascular necrosis (AVN) of both femoral heads. Medical intervention included bilateral core decompression surgery. The majority of reported AVN cases are pediatric patients and adults over 50 years old, following trauma (i.e., femoral fracture) being the main risk factor. Although not seen often in athletic populations, athletic trainers should be aware of the possibility of AVN in patients. AVN is a life-altering condition and accurately documenting all patient encounters, and proper interprofessional collaboration is imperative for positive outcomes.

Avascular Necrosis and Minimal Trauma Bone Fractures in Patients with Dyskeratosis Congenita

Introduction: Dyskeratosis congenita (DC) and related telomere biology disorders (TBD) result from germline variants in genes required for telomere maintenance. DC is traditionally characterized by a triad of nail dysplasia, oral leukoplakia, and reticular skin pigmentation. Significant complications include bone marrow failure (BMF), pulmonary fibrosis, liver disease, and hematologic and solid organ malignancies. Previous reports described avascular necrosis (AVN) of the femoral head or humerus in DC/TBD. However, these findings were limited to a few individual case reports. Herein, we present a study of AVN and minimal trauma fractures (MTF) in a large cohort of patients with DC/TBD. Methods: We reviewed medical records of all patients with a confirmed diagnosis of DC/TBD enrolled in the IRB-approved Inherited Bone Marrow Failure Syndromes study (NCT00027274) between 2002 - 2019 for the history of AVN and/or MTF. Data were compiled on DC/TBD characteristics, treatments, as well as body mass index (BMI), lipid profile, bone mineral density (BMD), and 25-hydroxyvitamin D (25[OH]D) levels. We used stratified random sampling to match DC/TBD patients with AVN and/or MTF by age, gender, inheritance pattern and genotype with a control group of DC/TBD patients with no AVN/MTF. Distributional variance was assessed for all t-tests and two-sided p values <0.05 were considered significant. Statistical analysis was carried out using RStudio (Ver 1.4.1106). Results: Forty-two of 233 patients (18%) with DC/TBDs experienced at least one AVN and/or MTF at a median age of 21.9 years (range 4 to 58.7); 15/42 were females (Table 1). Twenty-three patients had AVN alone, 6 had MTF alone, and 13 developed both AVN and MTF (Figure 1). The median age at first AVN was 24.1 years (range 6.3 - 58.7) and at first MTF 13.6 years (range 4 - 44.3). AVN was more common in patients with autosomal recessive/X-linked recessive (AR/XLR)/TINF2 DC (n=20, 21.3%) and developed at a younger age (median 18.8 years, range 6.3 - 47.8) than in patients with autosomal dominant (AD) disease (12.5%; median age 29.9 years, range 11.1 - 58.7); p=0.013. MTF occurred almost exclusively in patients with AR/XLR/TINF2 DC (n=17, 19%) compared with AD DC/TBD (n=1, .9%); p<.0001. There were 68 AVN events in 36 patients (median 2 events/patient, range 1 - 5). The most frequent sites were femoral heads (33/36) followed by humeral heads (5/36) and knees (3/36); 22/33 had bilateral femoral head AVN (Figure 1). Twenty-two of 26 patients with severe BMF received androgen/steroids within 5 years of AVN and 8 received hematopoietic cell transplantation (HCT). Compared with the matched control group, significantly more DC patients with AVN had severe BMF and received androgen/corticosteroids (p<0.02). Importantly, 12/36 DC/TBD patients (33%) developed AVN without prior HCT, corticosteroid, or androgen treatment. 24/36 patients received treatment for AVN, including total hip arthroplasty (n=15) and core decompression (n=5) or other medical intervention (n=4). Median time from diagnosis with AVN to treatment was 0.81 years (range 0.03 - 2.7). We identified 38 MTF events in 19 DC patients; the most frequent fracture site was the femoral diaphysis (11 events in 8 patients) (Figure 1). Fractures occurred spontaneously or during routine activities such as entering a vehicle, running in the playground, or low-impact dancing as per patient reports. Seventeen patients (89%) with MTF had severe BMF, 12 (63%) had received HCT (7 had taken androgens/steroids). Compared with matched controls, significantly more DC patients with MTF had received HCT (p=0.02). Other factors such as BMD, elevated cholesterol, overweight/obesity, and 25[OH]D levels were not significantly associated with AVN or MTF in our cohort. Conclusions: AVN and MTF are highly prevalent in patients with DC/TBDs. AVN developed across the entire DC/TBD spectrum but was significantly associated with severe BMF and AR/XLR/TINF2 DC. MTFs occurred almost exclusively in young patients with severe disease indicated by AR/XLR/TINF2 mode of inheritance. Improved screening and effective clinical management of symptoms for patients at higher risk of developing AVN/MTF is needed. Furthermore, these findings signal the need for future research exploring the mechanism underlying AVN and MTF in patients with DC/TBDs. Figure 1View largeDownload PPTFigure 1View largeDownload PPT Close modal

PSAT066 Adrenal Insufficiency Associated with Amyloidosis and Sickle Cell Disease

Abstract Background Amyloidosis causes chronic inflammation by the deposition of amyloid protein fragments in multiple organs, such as kidneys, adrenal, and pituitary glands. It is a challenge to access adrenocortical function in renal amyloidosis patients due to heavy proteinuria induced hypoproteinemia and low corticosteroid-binding globulin (CBG) which may cause false positive adrenocorticotropin (ACTH) test and the reduced cortisol level uncorrelated with free cortisol. On the other hand, adrenal insufficiency also frequently occurs in Sickle Cell Disease (SCD) patients. The hypothalamic-pituitary-adrenal (HPA) axis can be affected by tissue hypoxia during recurrent small vessel occlusion, chronic anemia, and iron-overload related oxidative stress. We report a SCD patient with nephrotic syndrome due to amyloidosis, who has adrenal insufficiency. Case Presentation A 41 years old female with Nephrotic syndrome secondary to Amyloid A (AA) amyloidosis, Adrenal insufficiency, Sickle cell disease (HbSC), Graves’ Hyperthyroidism, CVA, and bilateral femoral head avascular necrosis, presented with sickle cell pain crisis and anasarca with hypotension. She was on Hydrocortisone for 12 years but was not compliant for 2 months. She was on Methimazole for several years before it was discontinued one year ago. She has amenorrhea, severe edema with anasarca from the lower extremities to the abdomen. Labs showed morning cortisol 5.46 ug/dL, low albumin 2.4 g/dL, high TSH 5.43 uIU/mL, normal FT4 1.60 ng/dL, high urine protein 996.2 mg/dL, and eGFR 21.2. CT revealed pericardial effusion, free pelvic fluid, splenomegaly, edema, and thyroid goiter. Previous labs showed persistent low cortisol 0.9 to 3.5 ug/dL, high ferritin 1007 ng/mL, and pituitary workup was normal for Prolactin 4.6 ug/L, LH 5.02 mIU/mL, FSH 9.9 mIU/mL, IGF1 216 ng/mL, and ACTH 13.1 pg/mL. NM thyroid uptake elevated 51.7% and positive TPO 79.7 IU/mL. CT head was unremarkable. The patient was managed with Lasix and Albumin infusion for anasarca, Cyclosporine for amyloidosis, and Midodrine 10 mg TID for hypotension. Hydrocortisone stress dose was given followed by Prednisone 30 mg BID. Her symptoms improved. Discussion In Amyloidosis patients with heavy proteinuria, the low serum CBG levels affect total cortisol level. Therefore, the proper assessment for the adrenal function should be used with albumin-calculated free cortisol or cortisol/CBG ratio in addition to the ACTH stimulation tests. It is essential to differentiate between adrenal insufficiency and amyloidosis-related symptoms to prevent adrenal crisis and refrain from glucocorticoid side effects by unnecessary steroid use. On the other hand, endocrine manifestations of SCD may include hypogonadism, thyroid, adrenal, and pituitary dysfunction due to vaso-occlusive episodes induced multi-organ ischemic damage. This patient has amenorrhea, adrenal insufficiency, and normalized thyroid function test in Graves’ disease. It is crucial to avoid iron-overload induced oxidative stress which would worsen endocrine dysfunctions as well. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

P8.031: Outcome of Simultaneous Pancreas Kidney Transplantation A Single Center Experience

Introduction: Simultaneous simultaneous pancreas-kidney (SPK) transplantation is one of the treatment options for type 1 diabetes mellitus (T1DM) patients with chronic kidney disease stage 5. The SPKT outcomes are reported to be excellent with a 5 year patient, kidney and pancreas graft survival of 88%, 77% and 69% respectively. Here, we present data regarding outcome of the SPKT done at our institution. Method: We studied patients who underwent SPKT in our institution. 11 T1DM patients who were CKD-5 underwent SPKT from 2014 to 2022. Their graft function and complications were analysed. Results: A total of 12 patients were studied. 50 % were males and 50% were females. Mean age at diagnosis was 14 ± 4 years. Mean age at transplantation was 32 ± 6 years. Mean hemodialysis (HD) vintage was 22 ± 14 months. Mean age of donor was 31 ± 10 years. Induction was with Basiliximab in 1 patient, Alemtuzumab in 5 patients and ATG in 6 patients. Maintenance immunosuppression was tacrolimus and mycophenolate in 8 patients; and, tacrolimus, mycophenolate and everolimus in 2 patients Mean duration of hospital stay was 30 ± 18 days. Mean graft kidney function at 1 month was 1.38 ± 0.48 mg/dl. Mean graft kidney function at 6 months was 1.30 ± 0.47 mg/dl. At 1 year, one patient had severe renal graft dysfunction requiring HD. Among the other 10 patients who completed one year, mean kidney graft function at 1 year was 1.25 ± 0.23 mg/dl. Three (25%) patients had renal allograft loss with need for initiation of HD after 30, 21 and 6 months. Two patients with renal allograft loss required insulin after 19 and 25 months. Three (25%) patients had delayed graft function. Five (41.7%) patients had renal allograft rejection at 1 month. Among the five, three had another episode of rejection at 17, 11 and 4 months. Three (25%) patients had suspected pancreatic graft rejection at POD 13, 13 and 14, which resolved with intravenous steroids. Eight (66.6%) patients had infections in the first year following transplant which included urosepsis, LRTI, tuberculosis, varicella, CMV, cellulitis, periodontal abscess, dental caries and esophagial candidiasis. Seven (58.3%) had surgical complications including pseudo aneurysm of graft pancreas, urinoma, perinephric hematoma, abdominal bleed and DIC, jejujno-jejunal anastomotic site bleed, kinked renal vein requiring re-exploration, graft pancreas fat necrosis with impending GB rupture and peritonitis, and splenic vein thrombosis requiring re-exploration. Other complications noted were incisional hernia, subacute intestinal obstruction, bilateral femoral head avascular necrosis and acute myeloid leukemia. Conclusion: The graft survival and outcomes in our institution is comparable to that of international standards. Surgical complications were noted to be higher compared to renal transplantation. SPK remains to be treatment of choice in T1DM patients with ESRD.

Idiopathic Bilateral Femoral Head Avascular Necrosis and Bilateral Humeral Head Avascular Necrosis

Idiopathic Bilateral Femoral Head Avascular Necrosis and Bilateral Humeral Head Avascular Necrosis

Bilateral Femoral Head Avascular Necrosis with Physiological Doses of Steroids.

Bilateral Femoral Head Avascular Necrosis with Physiological Doses of Steroids.

Avascular necrosis in neuronopathic Gaucher despite high-dose enzyme replacement therapy

Neuronopathic Gaucher disease presents a clinical spectrum of neurological features from eye findings to seizures and in severe cases brain and central nervous system damage. Treatment via enzyme replacement therapy (ERT) is recommended, with excellent response for the visceral manifestations including bone health, but no impact on neurological disease. Recommendations suggest ERT doses upwards of 120 U/kg every two weeks for pediatric patients with neuropathic Gaucher and 60 U/kg every 2 weeks for adults (Altarescu et al., 2001; Vellodi et al., 2001). We report two patients with neuronopathic Gaucher (c.L444P homozygotes) maintained on high dose ERT who developed avascular necrosis (AVN) at 4.5 and 6 years post high dose ERT initiation, respectively. Patient 1 is a 13 year old Hispanicmalewho presented at age 14 months and began ERT at 60 U/kg every twoweeks at age 17 months. ERT was increased to 120 U/kg every two weeks at age 2. He had normalization of visceral manifestations at the increased dose. At age 6.5 years, he presented with left-sided bone pain; X-rays revealed sclerosis, and flattening and widening of the left femoral epiphysis, consistentwith AVN. At age 7 years, he presented with rightsided pain; X-rays revealed a femoral neck stress fracture, repaired by Spica cast. Bone density scanwaswithin two standard deviations of the mean onemonth prior to the onset of pain. Biomarkers were previously stable; chitotriosidase level increased from 4511 to 8771 nmol/h/ml approximately one month before onset of pain. Patient 1 initiated weekly ERT dose of 60 U/kg after surgical repair and showed improvement. Routine MRI at age 13 noted worsening bilateral femoral head AVNs. Patient 2 is an 18 year old Caucasianmale who presented at 18 months and began ERT at 22 months at a dose of 35 U/kg at an outside institution. He began care at Duke at age 4 years and continued on 35 U/kg. At 6.5 years, he was noted to have horizontal and vertical supranuclear gaze palsy, slow saccades, and difficulties with downward gaze. ERT dosewas increased andmaintained at 60 U/kg every 2 weeks; patient showed a good response, was growing and developing well without bone pain or symptoms. Routine surveillance MRI at age 17 noted a left femoral head AVN and bilateral femoral lytic lesions. Patient 2 was not reporting pain at this time. Previous surveillance MRI at age 15was normal. We present 2 cases of c.L444P homozygotes developing bone crises and avascular necrosis in spite of optimum ERT dosing for neuropathic Gaucher. Registry data will be queried to identify any additional cases. Both cases demonstrate the need for regular surveillance imaging as AVNs can present in a short amount of time. These cases additionally exhibit current challenges in the ability to prevent manifestations in bone tissue of patients with Gaucher disease.

Avascular osteonecrosis mechanism – between osteoporosis and antiphospholipid syndrome

HIV infected patients receiving antiretroviral therapy (ART) can develop avascular osteonecrosis, 45 times greater than the general population. Avascular osteonecrosis increased in the last few years in patients with HIV infection. The most important mechanisms for avascular osteonecrosis in HIV-infected patients are: changes in bone metabolism, especially osteoporosis correlated with protease inhibitors and coagulopathy and antiphospholipid syndrome, frequently described in HIV infection. Aim: to describe different mechanisms of avascular osteonecrosis We present two HIV-infected patients under antiretroviral therapy who developed avascular osteonecrosis. First patient, male, 38 year-old, with AIDS-C3, with good immuno-virological outcome under AZT-3TC and lopinavir/ritonavir, developed severe osteoporosis after 5 years of ART, diagnosed by DXA test with a T-score <-2,5. The patient had: C4-C5-C6 severe osteoporosis with high fracture risk, with segmental instrumentation applied at those levels, and then bilateral avascular necrosis of femoral head (ANFH) with bilateral hip arthroplasty. ART regimen was changed and the patient received 3TC-ABC and Nevirapine. The patient didn’t have other risk factors for avascular osteonecrosis or osteoporosis: nonsmoker, normal CD4, undetectable viral load, without dyslipidemia. Despite ART changes, the patient developed bilateral osteonecrosis of the right knee and of both humeral heads. The second patient, male, 23 year-old with HIV-B3, was treated in 2007 with AZT-3TC and lopinavir/ritonavir. After 9 months of ART the patient had normal CD4 count and undetectable viral load but developed right ANFH stage II, diagnosed by MRI, without surgery recommendation. DXA didn’t show signs of osteoporosis or osteopenia. The ART regimen was changed and the patient received 3TC-ABC and raltegravir with good ANFH outcome. After one year the patient discontinued the ART. One year later symptoms related with avascular osteonecrosis reappeared and bilateral ANFH was diagnosed by MRI. The patient had low CD4 count (<200/cmm), high HIV viral load and positive antiphospholipid antibodies. The patient had risk factors for coagulopathies: smoker, recreational drug user, alcohol consumer, uncontrolled HIV infection with low CD4. We restarted the same regimen with 3TC-ABC and raltegravir with a good outcome for bone affliction. When HIV infection was well-controlled, the antiphospholipid antibodies became negative and bone affliction was improved. We emphasize the importance of metabolic disturbances in HIV-infected patients, among them avascular osteonecrosis with different mechanisms. Both, ART and uncontrolled HIV infection can affect bone metabolism and vascularization.

Steroid Induced Bilateral Femoral Head Avascular Necrosis in a Patient of Minimal Change Glomerulonephritis: Detection by Radionuclide Bone Scan

One of the commonest causes of avascular necrosis of bones in patients with renal transplants are glucocorticoids. Early detection and management of this complication is crucial to the progression and prevention of collapse of femoral heads. In the early diagnosis of avascular necrosis radionuclide bone scintigraphy provides an useful information on the aetiology of the painful hip before changes appear on X ray examination. We present a case of proved glomerulonephritis taking glucocorticoids with difficulty in walking and pain around hip joints. A planar whole body and a SPECT Technetium 99-m (Tc99-m) bone scans were done which revealed avascular necrosis of both femoral heads. JMS 2012;15(1):61-64.

Perioperative nursing of total hip replacement surgery for patient with systemic lupus erythematosus and bilateral femoral head avascular necrosis: A case report

To report the perioperative nurse management of total hip replacement surgery for a patient with systemic lupus erythematosus and bilateral femoral head avascular necrosis.Before the surgery,giving the patient a psychological care and doing preoperative prepare cooperated with the doctors.After the surgery,observing vital signs in general and lower limbs closely,emphasizing on basic nurse care and functional exercise.After effective treatment and careful nursing,the patient recovered well without pressure sores,venous thrombosis,joint dislocation and other complications.The patient was discharged from the hospital with the primary healing. Key words: Lupus erythematosus,Systemic; Femur head necrosis; Arthroplasty,Replacement,Hip; Nursing

Avascular Necrosis of Bilateral Femoral Heads in a Patient with Fabry's Disease

The underlying cause of avascular necrosis (AVN) of the femoral head is often not apparent. We report the case of a 26 year old builder with a four month history of bilateral hip pain, and a diagnosis of bilateral femoral head avascular necrosis. Fabry's disease was identified as the probable cause. Since 2001, enzyme replacement therapy for Fabry's disease has become available, with a potential to influence the disease process, and this is of potential importance to clinicians treating AVN.