ABSTRACT
Familial amyloidotic polyneuropathy (FAP) is an autosomal dominant hereditary disorder. In Portugal, it is mainly linked to transthyretin (TTR) mutation, and patients present with length-dependent sensory-motor polyneuropathy, often accompanied by autonomic dysfunction. Treatment options for FAP include liver transplant, and due to the lack of organs, FAP livers began being implanted in patients with severe liver disease in a process known as domino liver transplantation (DLT). We report a case of a 68-year-old Portuguese man, with post-hepatitis C-related cirrhosis liver transplantation, who presented to his family doctor with decreased sensitivity in both feet and weight loss, which were initially attributed to diabetic neuropathy and an adjustment in diabetic medication, respectively. Symptoms evolved to changes in both feet's thermal and painful sensitivity, reduced sensitivity in both hands, diarrhea, and progressive weight loss. At this time, the patient's disclosure of receiving a DLT prompted the correct diagnosis of iatrogenic amyloid polyneuropathy. This case underscores the challenges in diagnosing and managing iatrogenic amyloid polyneuropathy following DLT, highlighting the importance of prompt identification of DLT recipients, active vigilance of these patients via structured monitoring, and increased healthcare providers' awareness of this practice so that early signs of the disease may be recognized.
ABSTRACT
PURPOSE: The purpose of this study was to review retrospectively the cases operated upon in our department in recent years with a Wagner stem and a small socket in cases of Crowe I or II dysplastic hips. METHODS: We conducted a retrospective clinical radiological review of 30 hips diagnosed with hip dysplasia Crowe I or II treated in our centre between 2002 and 2008. All of them were treated with a Wagner cone stem and a small Trilogy acetabulum (Zimmer ®). RESULTS: There were 15 men and 11 women with 13 left and 17 right femurs. Mean follow-up was 43.44 months (range 14-87). Eight patients were Crowe I type and 22 were Crowe II type. Merlé d'Aubigne score pre-operatively was 12.23 and at the last follow-up was 15.54. Mean leg length inequality was 1.79 and after surgery it was 0.69 cm. Complications included three infection and three dislocations (two of them in the same patient). CONCLUSIONS: The use of a Wagner stem is a good option to correct the different deformities in the proximal femur in these cases. A small socket allows a correct relocation of the acetabulum in a dysplastic socket with good bone coverage.
Subject(s)
Arthroplasty, Replacement, Hip/instrumentation , Hip Dislocation, Congenital/surgery , Hip Prosthesis , Osteoarthritis, Hip/surgery , Arthroplasty, Replacement, Hip/adverse effects , Arthroplasty, Replacement, Hip/methods , Cementation , Female , Femur/surgery , Hip Dislocation, Congenital/complications , Hip Dislocation, Congenital/diagnostic imaging , Hip Joint/diagnostic imaging , Hip Joint/physiopathology , Hip Joint/surgery , Humans , Leg Length Inequality , Male , Osteoarthritis, Hip/diagnostic imaging , Osteoarthritis, Hip/etiology , Postoperative Complications , Prosthesis Design , Prosthesis-Related Infections , Radiography , Retrospective StudiesABSTRACT
BACKGROUND AND OBJECTIVE: Preoperative anemia is a major risk factor for perioperative transfusion in orthopedic surgery and aging is associated with an increased risk for developing anemia. The aim of this study was to compare the efficacy and safety of preoperative epoetin alfa in patients older and younger than 65 years in orthopedic surgery. PATIENTS AHD METHOD: This study involved 75 patients scheduled for total joint arthroplasty who had an hemoglobin level between 100 and 130 g/l. Patients were assigned to receive weekly doses of subcutaneous epoetin alfa (40000 IU) on days -21, -14, -7, -1 before surgery. We tabulated age, weight, gender, baseline analytic parameters, perioperative evolution of the mean hemoglobin level, transfusion, side effects and complications. RESULTS: Fifty-four patients were included in group A (> or = 65 years) and 21 in group B (< 65 years). The two study groups did not differ in terms of demographic characteristics and baseline analysis but differed in age and associated diseases. The preoperative increase in mean hemoglobin level (20 and 18 g/l) and transfusion rate (15% and 14.3%) were similar in both groups. There were no complications associated with the use of epoetin alfa. CONCLUSIONS: Preoperative epoetin alfa administration seems to be as effective and safe in patients younger as in those older than 65 years.
