Menkes disease: report of two cases.
نویسندگان
چکیده
Menkes disease is a rare, autosomal recessive disorder characterized by neuronal degeneration, abnormal hair, malformed connective tissue, mental retardation, and a life span of three years. Previously reported dental findings include a high arched palate, delayed eruption of secondary dentition, and open bite. The case of twin seven-year-old males with Menkes disease is presented, along with previously unreported dental findings of spindle-shaped root resorption patterns on the primary maxillary central and lateral incisors.
منابع مشابه
MENKES\' SYNDROME: REPORT OF A CASE
An 8 month old boy is presented with clinical and laboratory features of Menkes' kinky hair syndrome. A brief discussion ensues.
متن کاملMenkes kinky hair disease: characteristic MR angiographic findings.
We report two cases of Menkes kinky hair disease in which MR and MR angiography were performed. The clinical and imaging features are reviewed. MR demonstrated characteristic cerebrovascular tortuousity and thus may be a valuable aid in diagnosis and follow-up.
متن کاملPapillon-Lefevre syndrome: A report of two cases in a family
Palmoplantar keratodermas are a heterogenous group of diseases, one of them is Papillon-Lefevre Syndrome (PLS). This rare disease is inherited as autosomal recessive and characterized by focal hyperkeratotic plaques on elbows and knees, severe periodontal disease resulting in premature loss of teeth. We report two brothers with PLS who did not have a history of this disease in their famil...
متن کاملA report of two cases of sarcoidosis in a family(brother and sister):case report
sarcoidosis is a granulomatius disease of unknown etiology involving multiple body systems.approximately 10-35% of patients with systemic disease have cutaneous involvement.skin lesions commonly observed in sarcoidosis include:erythema nodosum,macular or parpular rashes,nodules,hypopigmented or hyperpigmented patches,scarsarcoid,lupus pernio,plaques,and rarely mocusal lesions.this report descri...
متن کاملMenkes kinky hair disease (Menkes syndrome). A case report.
Menkes disease (MD) is a rare genetic neurodegenerative disorder. It is caused by a mutation in the ATP7A gene, which codes for the copper-transporting ATPase in the cell organelles. Dysfunction of many copper-dependent enzymes results in low concentrations of copper in some tissues and accumulation of copper in others. We report on a boy that at the age of 2 months presented with encephalopath...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- ASDC journal of dentistry for children
دوره 69 1 شماره
صفحات -
تاریخ انتشار 2002