Hybrid SPECT/CT and PET/CT imaging: the next step in noninvasive cardiac imaging

The past few years have witnessed impressive advances in the field of noninvasive cardiac imaging. For example, computed tomography (CT) coronary angiography has been adopted into daily clinical routine and, at least in some patient populations, is challenging the role of invasive angiography as the anatomic standard of reference. This is because the latter is associated with a nonnegligible periprocedural morbidity and mortality, which suggests confining its use to patients who will benefit from a revascularization procedure. Many factors that are beyond the quantification of anatomic narrowing and therefore cannot be fully appreciated with morphologic assessment will eventually determine whether or not a given lesion produces stress-induced ischemia. Myocardial perfusion imaging with single-photon emission computed tomography (SPECT) is among the most widely used and well-established noninvasive tools for the diagnosis of ischemic coronary disease. It allows assessment of the physiological relevance of coronary lesions and offers a high prognostic predictive value. Although positron emission tomography (PET) may achieve a higher accuracy than SPECT, its use has so far often been limited to large centers. Recent advances in image processing software and the advent of hybrid scanners have paved the way for fusion of image datasets from different modalities. An ideal, noninvasive technique for the diagnosis of coronary artery disease should provide complementary information on coronary anatomy as well as on pathophysiologic lesion severity. SPECT/CT and PET/CT hybrid imaging can provide such unique information, which not only improves diagnostic assessment and risk stratification but may also guide decision making with regard to revascularization in patients with coronary artery disease

Cardiofaciocutaneous syndrome (CFC) with congenital peripheral neuropathy and nonorganic malnutrition: An autopsy study

Many phenotypic manifestations have been reported in cardiofaciocutaneous (CFC) syndrome, but none, to date, are pathognomonic or obligatory. Previous histopathological studies reported findings in skin and hair; no autopsy studies have been published. We report the clinical and autopsy findings of a 7-year-old boy with severe CFC syndrome and malnutrition of psychosocial origin. Manifestations of CFC, reported previously, included macrocephaly and macrosomia at birth; short stature; hypotonia; global developmental delays; dry, sparse thin curly hair; sparse eyebrows and eyelashes; dilated cerebral ventricles; high cranial vault; bitemporal constriction; supraorbital ridge hypoplasia; hypertelorism; ptosis; exophthalmos; depressed nasal bridge; anteverted nostrils; low-set, posteriorly-rotated, large, thick ears; decayed, dysplastic teeth; strabismus; hyperelastic skin; wrinkled palms; keratosis pilaris atrophicans faciei; ulerythema ophryogenes; hyperkeratosis; gastroesophageal reflux; and tracheobronchomalacia. Additional findings, not previously reported, include islet cell hyperplasia, lymphoid depletion, thymic atrophy and congenital hypertrophy of peripheral nerves with onion bulb formations. Although the islet cell hyperplasia, lymphoid depletion, and thymic atrophy are nonspecific findings that may be associated with either CFC or malnutrition, the onion bulb hypertrophy is specific for a demyelinating-remyelinating neuropathy. These findings implicate congenital peripheral neuropathy in the pathogenesis of the developmental delays, feeding difficulties, respiratory difficulties, ptosis and short stature in this case. Additional studies of other cases of CFC are needed. (c) 2005 Wiley-Liss, Inc.Univ S Alabama, Womens & Childrens Hosp, Mobile, AL 36688 USAUniversidade Federal de São Paulo, São Paulo, BrazilUniversidade Federal de São Paulo, São Paulo, BrazilWeb of Scienc