Carey-Fineman-Ziter (CFZ) syndrome: report on affected sibs.
نویسندگان
چکیده
We describe a sib pair with craniofacial anomalies, micrognathia, Mobius sequence, generalised myopathy, relative macrocephaly, and developmental delay. They appear to have the Carey-Fineman-Ziter syndrome (MIM 254940), which has been reported in only four children, a sib pair and two sporadic cases. This report on an additional affected brother and sister pair supports autosomal inheritance as the likely cause. These cases also confirm that scoliosis, talipes equinovarus, and a non-specific primary myopathy are important manifestations of Carey-Fineman-Ziter syndrome.
منابع مشابه
Pontine hypoplasia in Carey-Fineman-Ziter (CFZ) syndrome.
We describe an infant with multiple congenital anomalies including cleft palate and micrognathia, Möbius sequence, developmental delay, myopathy, hydronephrosis, and bilateral clubfeet. These features are consistent with Carey-Fineman-Ziter (CFZ) syndrome (MIM 254940), which has been previously reported in six children (including two sibling pairs). Cranial magnetic resonance imaging (MRI) reve...
متن کاملSevere congenital myopathy with Möbius, Robin, and Poland sequences: new aspects of the Carey-Fineman-Ziter syndrome.
We report a boy with severe congenital myopathy, Möbius-Poland sequence, Robin sequence, and severe developmental delay. We consider this patient to have Carey-Fineman-Ziter syndrome. Since this is only the seventh case reported, this case helps to define further the consistent manifestations of this recognizable phenotype. Additionally our patient shows laryngostenosis, intermittent episodes o...
متن کاملThe Carey-Fineman-Ziter syndrome: follow-up of the original siblings and comments on pathogenesis.
In 1981 at the Festschrift honoring the many contributions of David W. Smith, I presented a unique pair of siblings with a distinctive pattern of malformation. The paper from this presentation was published as part of a series in the Journal of Pediatrics [Carey et al., 1982], and this condition has come to be called the Carey–Fineman–Ziter syndrome (CFZ). The described disorder included the fo...
متن کاملA defect in myoblast fusion underlies Carey-Fineman-Ziter syndrome
Multinucleate cellular syncytial formation is a hallmark of skeletal muscle differentiation. Myomaker, encoded by Mymk (Tmem8c), is a well-conserved plasma membrane protein required for myoblast fusion to form multinucleated myotubes in mouse, chick, and zebrafish. Here, we report that autosomal recessive mutations in MYMK (OMIM 615345) cause Carey-Fineman-Ziter syndrome in humans (CFZS; OMIM 2...
متن کاملTHE SYNDROME OF DIABETES INSIPIDUS, DIABETES MELLITUS, OPTIC ATROPHY, DEAFNESS, AND ATONIA OF THE URINARY TRACT (DIDMOAD SYNDROME). TWO AFFECTED SIBS AND A SHORT REVIEW OF THE LITERATURE
Two brothers with DIDMOAD syndrome are reported. The older brother has diabetes mellitus (type I), diabetes insipidus, optic atrophy, deafness and atonia of the urinary tract with severe symptoms such as diabetic ketoacidosis and frequent urinary tract infections. His younger brother had the same manifestations but with less severity. We report the findings of our two patients and compare ...
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:
- American journal of medical genetics
دوره 82 2 شماره
صفحات -
تاریخ انتشار 1999