About dutch-kennedy syndrome
What is dutch-kennedy syndrome?
Trismus-Pseudocamptodactyly Syndrome is a very rare inherited disorder characterized by the inability to completely open the mouth (trismus), causing difficulty with chewing (mastication) and/or the presence of abnormally short muscle-tendon units in the fingers, causing the fingers to curve or bend (camptodactyly) when the hand is bent back at the wrist (dorsiflexion). Because the fingers are not permanently bent or curved, this particular finding is termed "pseudocamptodactyly" (pseudo meaning false). In addition, the muscle-tendon units of the forearms and/or the legs may also be abnormally short, resulting in limited movements and various deformities of the feet. Individuals with this disorder are slightly shorter than would otherwise be expected (mild short stature). The severity of these physical findings varies from individual to individual. Trismus-Pseudocamptodactyly Syndrome is thought to be inherited as an autosomal dominant trait.
What are the symptoms for dutch-kennedy syndrome?
Inability to completely open mouth symptom was found in the dutch-kennedy syndrome condition
The symptoms of TPS vary from one person to another. It causes shortened muscles and tendons. The most common symptom is limited mobility of the mouth, which can cause problems with chewing. Other symptoms can include:
- limited motion of the arms or legs
- clenched fists
- a club foot
- abnormalities of the feet and hands
What are the causes for dutch-kennedy syndrome?
TPS is an inherited disease. A mutation of the MYH8 gene causes TPS. It’s autosomal dominant. This means that a person can inherit the abnormal gene from only one parent. The only known risk factor for this condition is a family history of TPS.
What are the treatments for dutch-kennedy syndrome?
No cure for TPS is available. However, you can have surgery to alleviate some of the symptoms of TPS. Doctors also often suggest physical and occupational therapy for people with TPS who have trouble walking or who have dexterity issues.
What are the risk factors for dutch-kennedy syndrome?
TPS affects females and males equally. However, twice as many affected females have been reported. The current prevalence rate of TPS is unknown. Approximately 11 families and over 300 individual cases have been reported since the disorder was first described in 1969. Of those cases, some have been traced to a Dutch female who came to the United States and settled in Tennessee during the eighteenth century. In addition, other reported cases have included five affected individuals in three generations of one Japanese family, six affected members of a German family. Affected individuals of families from the Netherlands, Canada, United Kingdom, Belgium, and Taiwan have also been reported.
Is there a cure/medications for dutch-kennedy syndrome?
Dutch-kennedy syndrome is a rare inherited disorder characterized by short muscles and tendons that result in limited mobility of the hands, legs, and mouth. The most serious complication of this condition is the inability to completely open the mouth (trismus), which causes difficulty with chewing. Short muscles and tendons in the fingers cause the fingers to bend or curve (camptodactyly) when the hand is bent back at the wrist. However, the fingers are not permanently bent or curved, so this particular finding is called “pseudocamptodactyly”.
DUTCH-KENNEDY SYNDROME is an autosomal dominant disorder caused by changes in the MYH8 gene. All cases of DUTCH-KENNEDY SYNDROME studied to date were found to have the same change in the MYH8 gene. This gene is involved in making the instructions for the development of the limb skeletal muscles and the muscles in the face.
1. The treatment of this disorder is directed toward the specific symptoms seen in each individual.
2. Treatment may require the efforts of a team of specialists who work together to plan a child’s care.
3. Such specialists may include pediatricians, those who diagnose and treat skeletal disorders (orthopedists), orthopedic surgeons, dentists, anesthesiologists, physicians who specialize in disorders of the digestive tract (gastroenterologists), nutritionists, and physical and occupational therapists.
4. In some people, jaw surgery may be performed to help increase the opening of their mouth.
Genetic counseling is recommended for affected individuals and their families.
Short muscles and tendons that result in limited mobility of the hands, legs, and mouth
Inability to completely open the mouth (trismus), which causes difficulty with chewing