A report of two cases of pyknodysostosis in two children

pyknodysostosis is a rare bone disease characterzed with short stature,wide fontaneles,small chin,hyperdensity of bones,erosion of tufts of the disatl phalanges,and narrowing of the mandibular angle. pyknodysostosis is inheritted as an autosomal recessive disease and may be seen in both sexes at any age.the patients have normal life span.short stature and bone fractures are their major problems.in this report two children ۱.۵ and ۴ years old are presented.the former was referred due to Gastroenteritis and tha latter because of delayed closure of the anterior fontanele,and both were admitted in the Kerman university of medical sciences-hospitalNo۱.pyknodysostosis was suggested as the probanle disease indicated for these patients and later confirmed by clinical and paraclinical investigations.

کلیدواژه ها:

pyknodysostosis ، wide fontanele ، Short Stature ، hyperdensity of bones