Nail patella syndrome (NPS) is a skeletal dysplasia with patellofemoral
dysfunction as a key symptom. We present the first in-depth radiological
evaluation of the knee in a large series of NPS patients and describe
the typical malformations. Conventional radiological examination of 95 skeletally mature
patients with NPS was performed. Patellar morphology was classified
according to the Wiberg classification as modified by Baumgartl
and Ficat criteria, and trochlear shape was classified according
to the Dejour classification.Aim
Patients and Methods
Haemophilia is an x-linked inherited bleeding disorder which can cause severe arthropathy. We have reviewed the results of 70 primary total knee replacements (TKR) performed in 57 haemophilic patients between 1983 and 2007. The functional results were assessed using the Hospital for Special Surgery (HSS) knee scoring system and Kaplan-Meier survivorship analysis. Six patients died. HSS scores were available for 60 TKRs at a mean follow-up of 9.2 years (2 to 23); 57 (95%) had good or excellent results. Deep infection was recorded in one patient. Kaplan-Meier analysis using infection and aseptic loosening as endpoints showed the survival rate at 20 years to be 94.0%. A reduction in infection, spontaneous haemarthrosis and improvement in the quality of life were noted to justify surgery in our series of patients with a mean age of 43 (25 to 70). We have found that using the latest techniques of continuous infusion of clotting Factor have significantly helped to reduce the complication rates and have achieved results which match those of the non-haemophilic population undergoing TKR.
