Medical complications of achondroplasia: a multicentre patient review.

نویسندگان

  • A G Hunter
  • A Bankier
  • J G Rogers
  • D Sillence
  • C I Scott
چکیده

Achondroplasia is the most prevalent chondrodysplasia and numerous authors have documented the varied social and medical complications that may compromise a full and productive life. Complications include cervicomedullary compression, spinal stenosis, restrictive and obstructive lung disease, otitis media, and tibial bowing, among others. These known complications have led to recommendations for the anticipatory management of such patients. There are relatively few data on the actual rates and timing of these problems. This paper reports data on the rates and age of occurrence of several of these complications based on a review of recorded chart information of 193 patients ascertained from several well established genetic centres with a known interest in the chondrodysplasias. The length of follow up varied and the rates of occurrence at specific age intervals were used to estimate the cumulative percentage affected for each complication. The report includes information on otitis media, ventilation tubes, hearing loss, tonsillectomy, speech problems, tibial bowing and osteotomy, ventricular shunting, apnoea, cervicomedullary decompression, and neurological signs attributable to spinal stenosis.

برای دانلود رایگان متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Optimal management of complications associated with achondroplasia

Achondroplasia is the most common form of skeletal dysplasia, resulting in disproportionate short stature, and affects over 250,000 people worldwide. Individuals with achondroplasia demonstrate a number of well-recognized anatomical features that impact on growth and development, with a complex array of medical issues that are best managed through a multidisciplinary team approach. The complexi...

متن کامل

Clinical management of achondroplasia.

Achondroplasia, one of the skeletal dysplasias and the commonest form of disproportionate short stature, has a different developmental and growth profile compared to average stature children. In addition, a specific group of complications occur more frequently in children with achondroplasia. These include common but usually relatively minor complications such as recurrent otitis media, and rar...

متن کامل

Current Care and Investigational Therapies in Achondroplasia

PURPOSE OF REVIEW The goal of this review is to evaluate the management options for achondroplasia, the most common non-lethal skeletal dysplasia. This disease is characterized by short stature and a variety of complications, some of which can be quite severe. RECENT FINDINGS Despite several attempts to standardize care, there is still no widely accepted consensus. This is in part due to abse...

متن کامل

بی‌حسی کامل نخاعی در بیمار مبتلا به آکندروپلازی: گزارش موردی

Background: Total spinal anesthesia is a complication of lumbar epidural anesthesia following undiagnosed subarachnoid or subdural injection of local anesthetic. Although many achondroplastic dwarfs have a normal spine, catheter insertion may be more problematic with a narrow epidural space making a subarachnoid tap more probable.  Other malformations associated with achondroplasia, such as pro...

متن کامل

Polycystic kidney disease in a patient with achondroplasia: case report.

Autosomal dominant polycystic kidney disease is a multisystem disease involving many organs. An association with other diseases such as tuberous sclerosis, von Hippel-Lindau disease and Marfan syndrome have been previously described. We describe a 35 year old female with achondroplasia who developed polycystic kidney disease involving both kidneys and progressing to end-stage renal disease. To ...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

عنوان ژورنال:
  • Journal of medical genetics

دوره 35 9  شماره 

صفحات  -

تاریخ انتشار 1998