Short-term outcome of total knee replacement in a patient with hemophilia:A case report and review of literature

BACKGROUND Hemophilia A is a rare inherited bleeding disorder caused by mutations in the factor Ⅷ gene. This clotting factor plays an intrinsic role in the blood coagulation pathway. Patients with hemophilia may develop orthopedic manifestations such as hemarthrosis, but multiple malunion of fractures over the knee is rare and difficult to treat.CASE SUMMARY We report a patient with hemophilia A who developed severe knee osteoarthritis along with fracture malunion and nonunion. Total knee replacement was performed using a custom-made modular hinged knee prosthesis(cemented) equipped with extended distal and proximal stems. At 3 years’ follow-up, the patient exhibited excellent clinical function and remained satisfied with the surgical outcome. Surgical intervention was accompanied by rigorous coagulation factor replacement.CONCLUSION This case highlights various unique scenarios specific to individuals with hemophilia and fracture deformity.

Enzyme replacement therapy in two patients with classic Fabry disease from the same family tree:Two case reports

BACKGROUND The pathophysiology of Fabry disease(FD)-induced progressive vital organ damage is irreversible.Disease progression can be delayed using enzyme replacement therapy(ERT).In patients with classic FD,sporadic accumulation of globotriaosylceramide(GL-3)in the heart and kidney begins in utero;however,until childhood,GL-3 accumulation is mild and reversible and can be restored by ERT.The current consensus is that ERT initiation during early childhood is paramount.Nonetheless,complete recovery of organs in patients with advanced FD is challenging.CASE SUMMARY Two related male patients,an uncle(patient 1)and nephew(patient 2),presented with classic FD.Both patients were treated by us.Patient 1 was in his 50s,and ERT was initiated following end-organ damage;this was subsequently ineffective.He developed cerebral infarction and died of sudden cardiac arrest.Patient 2 was in his mid-30s,and ERT was initiated when the patient was diagnosed with FD,during which the damage to vital organs was not overtly apparent.Although he had left ventricular hypertrophy at the beginning of this treatment,the degree of hypertrophy progression was limited to a minimal range after>18 years of ERT.CONCLUSION We obtained discouraging ERT outcomes for older patients but encouraging outcomes for younger adults with classic FD.

Persistent left superior vena cava in right hemiarch replacement under deep hypothermic circulatory arrest:A case report

BACKGROUND Persistent left superior vena cava(PLSVC),a relatively rare thoracic vascular malformation,can inconvenience perfusionists and operators when encountered during deep hypothermic circulatory arrest(DHCA).CASE SUMMARY Herein,we describe the case of a patient with concurrent giant aortic arch aneurysm,aortic stenosis,and PLSVC.To treat these conditions,we performed right hemiarch and aortic valve replacements under DHCA.Notably,we applied“bilateral superior vena cava retrograde cerebral perfusion(RCP)”for cerebral protection,which significantly optimized the surgical procedure and reduced the risk of postoperative complications.The patient was discharged 14 d after surgery with no complications.CONCLUSION Surgical intervention for PLSVC under DHCA can be performed using the bilateral superior vena cava RCP approach.

An Optimal Algorithm for the Retrieval of Chlorophyll,Suspended Sediments and Gelbstoff of Case Ⅱ Waters in the Pearl River Estuary

An optimal algorithm for the retrieval of chlorophyll, suspended sediments and gelbstoff of case Ⅱ waters in the Pearl River estuary was established with the optical parameters derived from the in-situ data obtained in Jan. 2003 in the same area. And then, the chlorophyll, suspended sediments and gelbstoff of the SeaWiFS pixels on Jan. 29, 2003 corresponding to the in-situ sites of Jan. 25 and 26, 2003 were synchronously retrieved, with average relative errors of 14.9%, 12.1% and 13.6% for chlorophyll, suspended sediments and gelbstoff, respectively. The research results indicated that the optimal retrieval algorithm established here was relatively fit for the retrieval of the chlorophyll, suspended sediments and gelbstoff of case Ⅱ waters in the Pearl River estuary, and had quite good retrieval accuracy.

Dynamic Case Retrieval Method With Subjective Preferences and Objective Information for Emergency Decision Making

Case-based reasoning(CBR) is one of the best methods for generating an effective solution in an emergency. In recent years, some methods for generating emergency alternatives have been included in practical CBR applications, but there have been no in-depth studies of these processes. In this study,we propose a new method for dynamic case retrieval with subjective preferences and objective information, which considers the personal preferences of the decision makers and changes in the attributes of the emergency as the situation develops. First,we present a formula for calculating the case similarity and changing trends in the case considered, where similar cases are obtained. Next, we describe a method for measuring the overall assessment value with respect to similar historical cases, which is obtained by aggregating the case similarity, the utility case similarity, the first response time, and the implementation effect.The subjective preferences and objective information are also integrated in the decision-making process. Finally, we present a case study based on the emergency response to a fire in a highrise building, which illustrates the applicability and feasibility of the proposed method.

Therapeutic plasma exchange and continuous renal replacement therapy for severe hyperthyroidism and multi-organ failure:A case report

BACKGROUND Severe hyperthyroidism is a life-threatening exacerbation of thyrotoxicosis,characterized by high fever and multiorgan failure. The most common medical treatments are administration of antithyroid drugs and radioactive iodine, and thyroidectomy. In some patients, antithyroid therapy is limited due to serious adverse effects or failure to control disease progression. In some extreme cases,such as thyroid storm, conventional therapy alone does not yield effective and rapid improvement before the development of multiorgan failure.CASE SUMMARY This report describes a Chinese patient with severe hyperthyroidism accompanied by multiorgan failure, who was transferred to the medical intensive care unit of our hospital. The patient presented with palpitations, vomiting,diarrhea, and shortness of breath for a week. Laboratory tests showed elevation of thyroid hormones. Hepatic failure occurred with high aminotransferase levels and jaundice. Given her abnormal liver function and medication history, we could not exclude diagnosis of propylthiouracil-induced hepatic failure.Moreover, she also suffered from heart failure. Therapeutic plasma exchange(commonly known as TPE) and continuous renal replacement therapy(commonly known as CRRT) were used as life-saving therapy, which resulted in notable improvement of clinical symptoms and laboratory tests.CONCLUSION Combined TPE and CRRT are safe and effective for patients with hyperthyroidism and multiorgan failure.

An Approach to Improving the Retrieval Accuracy of Oceanic Constituents in Case Ⅱ Waters

In the present paper, a method is proposed to improve the performance of Artificial Neural Network (ANN) based algorithms for the retrieval of oceanic constituents in Case Ⅱ waters. The ANN-based algorithms have been developed based on a constraint condition, which represents, to a certain degree, the correlation between suspended particulate matter (SPM) and pigment (CHL), coloured dissolved organic matter (CDOM) and CHL, as well as CDOM and SPM, found in Case Ⅱ waters. Compared with the ANN-based algorithm developed without a constraint condition, the performance of ANN-based algorithms developed with a constraint conditions is much better for the retrieval of CHL and CDOM, especially in the case of high noise levels; however, there is not significant improvement for the retrieval of SPM.

Total femur replacement with 18 years of follow-up:A case report

BACKGROUND Osteosarcoma is one of the most common primary malignant bone tumors and is more common in adolescents.The femur is the most common site of osteosarcoma,and many patients require total femur replacement.We reviewed the relevant literature and case findings,summarized and analyzed this case in combination with relevant literature,and in doing so improved the understanding of the technology.CASE SUMMARY The case we report was a 15-year-old patient who was admitted to the hospital 15 days after the discovery of a right thigh mass.The diagnosis was osteosarcoma of the right femoral shaft.After completion of neoadjuvant chemotherapy and preoperative preparation,total right femoral resection+artificial total femoral replacement was performed.Then,chemotherapy was continued after surgery.The patient recovered well after treatment,and the function of the affected limb was good.No recurrence,metastasis,prosthesis loosening,dislocation,fracture or other complications were found during 18 years of follow-up.At present,the patient can still work and lives normally.The results of the medium-and longterm follow-up were satisfactory.CONCLUSION Artificial total femur replacement is a feasible limb salvage operation for patients with femoral malignant tumors,and the results of medium-and long-term follow-up are satisfactory.

Borderline form of empty follicle syndrome treated with a novel dual trigger method combined with delayed oocyte retrieval: A case report

BACKGROUND Borderline form of empty follicle syndrome is a condition in which only a few mature or immature oocytes are recovered after meticulous follicular aspiration,despite adequate ovarian response to stimulation.It is a rare phenomenon with an unclear cause.Currently,the condition still lacks effective treatment.CASE SUMMARY A patient with secondary infertility who had undergone three cycles of assisted reproductive technique(ART)is described.With regard to good follicular response,two oocytes were obtained in the first two ART cycles,but no embryo was formed.In the third ART cycle,which is the subject of this study,ovulation was induced by dual trigger of a supernormal dose of human chorionic gonadotropin(HCG)combined with a delayed oocyte retrieval approach.The method involved administration of gonadotropin-releasing hormone agonist,recombinant HCG,and urinary HCG 39 h before ovum pick-up.Ten oocytes were recovered,two out of three mature eggs were fertilized after intracytoplasmic sperm injection,resulting in two embryos that were subsequently cryopreserved.The case report guidelines have been used herein to present the first case of this novel dual trigger method.CONCLUSION This approach provides a new treatment option for patients with a similar condition in the future.This study can also inspire further investigation on the effects of variousβ-HCG serum levels 36 h after intramuscular HCG administration.

A Retrieval Matching Method Based Case Learning for 3D Model

The similarity metric in traditional content based 3D model retrieval method mainly refers the distance metric algorithm used in 2D image retrieval. But this method will limit the matching breadth. This paper proposes a new retrieval matching method based on case learning to enlarge the retrieval matching scope. In this method, the shortest path in Graph theory is used to analyze the similarity how the nodes on the path between query model and matched model effect. Then, the label propagation method and k nearest-neighbor method based on case learning is studied and used to improve the retrieval efficiency based on the existing feature extraction.

Surgical strategy used in multilevel cervical disc replacement and cervical hybrid surgery:Four case reports

BACKGROUND Multilevel artificial cervical disc replacement and anterior hybrid surgery have been introduced as reliable treatments for multilevel cervical degenerative disc disease.Surgical techniques are important for resolving patients’symptoms and maintaining the normal functioning of cervical implants.However,the use of inappropriate surgical strategies could lead to complications such as implant migration and neurological deficit.In this paper,we summarize our surgical strategies used in multilevel cervical disc replacement and hybrid surgery into five major notes.CASE SUMMARY We share the key notes and our surgical procedures in the form of four typical case presentations.All patients were diagnosed with cervical degenerative disc disease with myelopathy or radiculopathy and needed multilevel cervical spine surgery.The first case demonstrated that index levels indicating the presence of highly serious spinal cord compression required a prioritized decompression.The second case demonstrated that the disc replacement should be performed before fusion in cervical hybrid surgery.The third and forth cases demonstrated that a top-down implantation sequence was needed in continuous two-level cervical disc replacement.The symptoms of all patients were significantly relieved after surgery.CONCLUSION We hope that our surgical strategies can help improve the performance and outcomes of multilevel cervical spine surgery.

A Case Retrieval Projection Pursuit Model for Configuration Design of a Product Family

The paper studies on case-based reasoning of uncertain product attributes in configuration design of a product family. Interval numbers characterize uncertain product attributes. By interpolating a number of certain values randomly to replace interval numbers and making projection pursuit analysis on source cases and target cases of expanded numbers, we can get a projection value in the optimal projection direction. Based on projection value, we can construct a case retrieval model of projection pursuit that can handle coexisting certain and uncertain product attributes. The application examples of chainsaw configuration design show that case retrieval is highly sensitive to reliable results.

Successful minimal approach transcatheter aortic valve replacement in an allograft heart recipient 19 years post transplantation for severe aortic stenosis: A case report

BACKGROUND Aortic stenosis is one of the rare valvular complications in a transplanted heart.Over the past 8 years,transcatheter approach for aortic valve replacement(TAVR)has been slowly evolving to be the preferred approach in these patient population when compared to the surgical approach.We report a second case in the United States with successful transfemoral minimal approach with minimal sedation for TAVR in a heart transplant recipient 19 years post transplantation for severe symptomatic calcified aortic stenosis.CASE SUMMARY We present a case of 73-year-old male who has undergone successful minimal approach transcatheter aortic valve replacement in an allograft heart.Patient had received orthotopic heart transplantation 19 years ago for non-ischemic cardiomyopathy.Follow up transthoracic echocardiograms as per routine protocol did not show any aortic valve disease until 15 years post transplantation.Aortic valve was noted to be mildly sclerotic at that time and gradually progressed to severe symptomatic aortic stenosis over the next 4 years.Patient had complaints of worsening shortness of breath that limited his functional capacity.Overall his post heart transplantation period has been mostly uneventful except for allograft non occlusive vasculopathy and aortic stenosis.His Society of Thoracic Surgery risk score was 12.205%and he was considered to be a high-risk surgical candidate by surgeon.Decision was made to undergo transcatheter aortic valve replacement.CONCLUSION With the improved survival of these patients,we think it is time to look intopathophysiology of valvular disease in transplant heart recipients.Some other unanswered questions include,underlying donor and recipient risk factors for valvular diseases in heart transplant recipients.

Patellar dislocation following distal femoral replacement after extraarticular knee resection for bone sarcoma:A case report

BACKGROUND For the treatment of bone sarcoma in the distal femur,wide-margin resection and knee reconstruction with tumor endoprosthesis are standard therapies.Extraarticular knee resection is required in cases of tumor invasion of the knee joint;however,the incidence of complications,such as aseptic loosening,prosthesis infection,and implant failure,is higher than that following intra-articular knee resection.To the best of our knowledge,there are three reports of patellar dislocations after replacement of a tumor endoprosthesis.CASE SUMMARY A 36-year-old man with no significant past medical history was admitted to our institution with continuous pain in his left knee for 4 mo.An open biopsy was performed,and the patient was diagnosed with a left distal femoral malignant bone tumor.Extra-articular knee resection and knee reconstruction with a tumor endoprosthesis were performed.Although the alignment of the tumor prosthesis was acceptable,knee instability was noticed postoperatively.The axial radiographic view of the patellar and computed tomography showed lateral patellar dislocation at 4 wk postoperatively.The patient had to undergo a lateral release and proximal realignment.He could perform his daily activities at 9 mo postoperatively.Radiography revealed no patellar re-dislocation.CONCLUSION Proximal realignment may be considered during primary surgery if there is an imbalance in the forces controlling the patellar tracking.

Case Retrieval Method of Multi-parameter for Customized Product

The CBR (Case-Based Reasoning) usually is been used to accomplish customized products by variant design or reusable design.In the CBR,retrieve is very important.A simple case retrieval method is been brought forward to retrieve a opti- mal prototype based on using inputted multi-parameters,it can be programmed easily.An example has been proved this method can find optimal prototype for new design task efficiently.

Early diagnosis and treatment of pulmonary embolism after total joint replacement: report of five cases

Objective To study the early diagnosis and treatment of pulmonary embolism (PE) after total joint replacement (TJR). Methods From April 1987 to December 2001, we performed 1 336 total knee replacements (TKR) in 926 patients and 1 745 total hip replacements (RHRs) in 1 566 patients. In this group there were 5 PE patients after operation. Two patients died (all after TKR), and 3 patients salvaged successfully (2 after TKR, 1 after THR). Results The total incidence of PE after TJR was 0. 2% (5/2 492), the incidence of PE after TKR was 0. 4% (4/926), and the incidence of PE after THR was 0.06% (1/1 556). In the patients who suffered PE, 2 died. The incidence of PE before 2000 was 0%. Conclusion Death after total joint replacement is due to pulmonary embolism ( PE), especially massive PE. The incidence of PE after TKR is higher than that after THR. Pulmonary angiography is the gold standard for the diagnosis of PE. With more understanding on PE and more popularity of joint replacement, the diagnosis of PE

Isolated mitral valve replacement with Guangdong-Ⅰ porcine bioprostheses in rheumatic heart valve disease: analysis of 166 cases with long-term follow-up

Objective To study the long-term effects of mitral valve replacement with bioprostheses in rheumatic heart valve disease. Methods 166 patients with rheumatic heart valve disease underwent isolated mitral valve replacement from Jan. 1978 to Dec. 1985. 79 Patients were male and 87 female. Patients’ age ranged from 11 to 53 years [mean (29.4 ± 9.9) years]. The patients were classified into two groups: group 1 (age【 30 years) included 84 patients, group 2 (age≥30 years) 82 cases. Mean CTR is 0.68 *0.08. Most patients were in NYHA function class Ⅲ-Ⅳ. 76. 3% of patients had atrial fibrillation. 6 patients had pre-operative cerebral vascular accidents. Postoperative deaths were excluded. Follow-up extended from 0.4 year to 19.4 years. Results Actuarial estimate of survival rate at 14 years is (52.5 ± 7.0)%. Heart function was improved to class Ⅰ - Ⅱ in most of patients. Late structural valve deterioration occurred in 89 patients. Among them, 59 were re-operated. Actuarial estimate of freedom from

Rapid progression of heart failure secondary to radioactive iodine treatment of hyperthyroidism:A case report

BACKGROUND The deterioration of thyroid health is involved in the progression of heart failure(HF).This is usually a lengthy process,so there are almost no reports on its rapid development.Here we report a case of a young male who rapidly developed hypothyroid cardiomyopathy secondary to radioactive iodine treatment,suggesting that severe HF might occur even after a short period of hypothyroidism.CASE SUMMARY A 26-year-old man was referred to our hospital for HF presenting with dyspnea on exertion and chest discomfort lasting for 1 mo.He received radioactive iodine treatment for hyperthyroidism 1 year ago and had an almost normal echocardiogram 6 mo ago.Admission echocardiogram and cardiac magnetic resonance(CMR)revealed left ventricle(LV)global hypokinesia and severely depressed systolic function.In addition,late gadolinium enhancement indicated no obvious changes in the myocardium.Thyroid function tests showed decreased serum levels of thyroid hormone(TH)and elevated thyroid-stimulating hormone.Based on an exclusionary examination,the patient was diagnosed with hypothyroid cardiomyopathy and was started on replacement therapy.His HF symptoms were completely relieved during the six-month follow-up,and echocardiogram and CMR revealed recovered LV size and ejection fraction.CONCLUSION This report demonstrates that severe fluctuations in TH levels may lead to acute HF,which can completely recover with timely thyroid hormone replacement.In addition,our findings highlight the importance of routinely detecting cardiac function in patients treated with radioactive iodine.

Management of Rhabdomyolysis and Acute Renal Failure Following Strenuous Exercise in Young Adult: A Case Report

Introduction: Rhabdomyolysis is severe and life threating condition in which skeletal muscles are damaged by dissolution of striped muscles. Acute kidney injury (AKI) has been widely reported (13% - 50%) as a complication of Rhabdomyolysis in which the main pathophysiological mechanisms are intra-renal vasoconstriction, intraluminal cast formation (Tamm-Horsefall) and direct myoglobin toxicity. In this report we are going to describe the management of Acute Kidney Injury due to Rhabdomyolysis that is not responding to vigorous rehydration. Objective: Reporting about acute renal failure induced by Rhabdomyolysis due to Excessive Exercise and dehydration in young patient. Case Report: A 20 years male came to the outpatient clinic complaining of sever lower limb pain, back pain and vomiting. He was anuric and hypertensive (BP = 150/90 mmHg) with serum creatinine and urea levels of 15.72 mg/dl and 235 mg/dl, respectively. The diagnosis was based on the laboratory finding of creatine kinase = 3127 IU/l. The patient, then, has been referred to the emergency department. The Management plan was based on two arms: Emergency Management with Urgent Hemodialysis for AKI and high fluid replacement therapy. Patient started to recover after three hemodialysis sessions but the peak of recovery was noted after starting manual fluid replacement therapy with a target urine output of greater than 2 ml/kg, a urine pH of greater than 6. Manual fluid replacement therapy consisted of loop diuretics, intravenous fluids and intravenous sodium bicarbonate 1.26%. Full recovery was noted after one month of hospital admission with inpatient care and regular follow-up. A follow-up after one month has been set to assess the patient progression and monitor his kidney functions. Relevance and Impact: Home messages and lessons are;Firstly, young adults are vulnerable to Rhabdomyolysis, second, the diagnosis of Rhabdomyolysis can be made on the clinical bases but a confirmatory laboratory test of Creatine Kinase is mandatory, and lastly’ acute kidney injury needs to be treated urgently. Also, reducing the risk of infection is one of the management objectives to achieve recovery.

Blood purification for treatment of non-liquefied multiple liver abscesses and improvement of T-cell function:A case report

BACKGROUND Non-liquefied multiple liver abscesses(NMLA)can induce sepsis,septic shock,sepsis-associated kidney injury(SA-AKI),and multiple organ failure.The inability to perform ultrasound-guided puncture and drainage to eradicate the primary disease may allow for the persistence of bacterial endotoxins and endogenous cytokines,exacerbating organ damage,and potentially causing immunosuppression and T-cell exhaustion.Therefore,the search for additional effective treatments that complement antibiotic therapy is of great importance.CASE SUMMARY A 45-year-old critically ill female patient presented to our hospital’s intensive care unit with intermittent vomiting,diarrhea,and decreased urine output.The patient exhibited a temperature of 37.8℃.Based on the results of liver ultrasonography,laboratory tests,fever,and oliguria,the patient was diagnosed with NMLA,sepsis,SA-AKI,and immunosuppression.We administered antibiotic therapy,entire care,continuous renal replacement therapy(CRRT)with an M100 hemofilter,and hemoperfusion(HP)with an HA380 hemofilter.The aforementioned treatment resulted in a substantial reduction in disease severity scores and a decrease in the extent of infection and inflammatory factors.In addition,the treatment stimulated the expansion of the cluster of differentiation 8^(+)(CD8^(+))Tcells and led to the complete recovery of renal function.The patient was discharged from the hospital.During the follow-up period of 28 d,she recovered successfully.CONCLUSION Based on the entire therapeutic regimen,the early combination of CRRT and HP therapy may control sepsis caused by NMLA and help control infections,reduce inflammatory responses,and improve CD8^(+)T-cell immune function.