Maternal inheritance in cyclic vomiting syndrome.
Boles RG, Adams K, Li BU.
Division of Medical Genetics and the Saban Research Institute, Childrens Hospital Los Angeles, Los Angeles, California, USA. firstname.lastname@example.org
Cyclic vomiting syndrome (CVS), characterized by severe discrete episodes of nausea, vomiting, and lethargy, is a fairly common, disabling, predominately-childhood condition most often associated with migraine and dysautonomic features. Our group recently reported that children with CVS and additional neuromuscular disease manifestations demonstrate strong maternal inheritance of multiple disease manifestations and abnormal urine organic acids, suggesting the presence of predisposing mitochondrial DNA (mtDNA) sequence variants. In order to determine if maternal inheritance is present in CVS in general, a clinical interview was administered regarding 80 unrelated individuals with CVS ascertained randomly from the database of the Cyclic Vomiting Syndrome Association (CVSA). Disease manifestations consistent with potential mitochondrial dysfunction were far more common in matrilineal (sharing the same mtDNA sequence) versus in non-matrilineal relatives, including mothers versus fathers (P = 3 x 10(-9)) and maternal versus paternal grandmothers (P = 2 x 10(-6)). Maternal inheritance is suggested in 52% of the 23 subjects with two or more neuromuscular abnormalities ("CVS+") and in 54% of the 44 subjects without any neuromuscular abnormalities ("CVS-"). In both the CVS+ and CVS- sub-groups, subjects, and affected matrilineal relatives of all ages suffer at a far higher incidence from several dysautonomic-related conditions, including migraine and irritable bowel, as well as depression and hypothyroidism, while neuromuscular and cognitive disorders such as hypotonia and ADHD are common only in affected children. We conclude that mtDNA sequences predispose towards the development of protean disease manifestations in CVS patients ascertained through a disease-specific association, as well as among their matrilineal relatives, whether or not neuromuscular disease is present in the proband. Since CVS was absent in all but one matrilineal relative of our probands, CVS is apparently a rare clinical presentation in individuals carrying the predisposing mtDNA sequences. The four conditions reported most frequently among the matrilineal relatives of our cases, migraine, depression, irritable bowel, and hypothyroidism, are known to segregate together in families, and our findings suggest that a common predisposing genetic factor is likely present on the mtDNA. (c) 2005 Wiley-Liss, Inc.
Every summer I carry a very long bucket with us everywhere we go. The bucket has to be long enough to handle the g force of Zach's projectile vomiting. He threw up so much in one of my cars that we had to get rid of it. It was disgusting! So long to the Saturn! Now I have learned. You must have tinted windows. You much have a powerful airconditioner. You must have pro sport quality sunglasses. Cold water near by. Limit the glare. Glare will set it off. One you vomit or get a headache you will have more trouble getting stable again than the trouble it takes to prevent the start of vomiting. Sometimes it 's a smell or a picture os something and you can't always control everything. I do everything EVERYTHING I can to prevent the vomiting. Taking the Co Q 10 and L Carnitor can help you ward off the vomiting. Zachary likes a cool cloth on his neck. When I fail to prevent vomiting, I sometimes use Zofran disolvable to help stop the cycle.
Zachary has become more aware of his cyclic vomiting syndrome. He knows when he might be sick he might get the bucket or try to tell me he's sick. Instead of just letting it fly like a baby would. It's always worth the extrea effort to prevent stressing the body and setting off a migraine or a cycle of vomiting.