ABSTRACT
Juvenile ossifying fibroma is an osseous tumor encased in a sclerotic shell. We report findings of juvenile ossifying fibroma in the orbit of a six-year-old boy. The bony aspects of this tumor may be suspected on physical examination. Magnetic resonance imaging was not helpful in the diagnosis of this fibroma. In fact, the technology was misleading because it did not define clearly the bony aspects of the tumor. In this case, the less costly computerized tomography revealed the bony encasement of the tumor and best defined the borders. With the trend in health care of cost containment, it may be that computerized tomography is the appropriate primary imaging study for some suspected orbital tumors based on thorough clinical examination.
Subject(s)
Fibroma, Ossifying/diagnosis , Orbital Neoplasms/diagnosis , Child , Diagnosis, Differential , Fibroma, Ossifying/physiopathology , Fibroma, Ossifying/surgery , Humans , Magnetic Resonance Imaging , Male , Orbital Neoplasms/physiopathology , Orbital Neoplasms/surgery , Tomography, X-Ray Computed , Visual AcuityABSTRACT
The ethical issues concerning the use of fetal tissue as a source for organ transplantation has focused interest on anencephaly. For reasons that are not entirely clear, the incidence of anencephaly has been declining. As anencephaly is easily recognized and invariably fatal, mortality figures provide an excellent reflection of incidence. In the United States, between 1968 and 1987, infant mortality rates per 100,000 live births due to anencephaly declined from 22.4 to 12.1 (46.0%) for male infants and from 32.7 to 16.6 (49.2%) for female infants. However, when separated by race, the mortality rate declined for white male infants from 25.0 to 13.3 (46.8%) but only decreased from 9.0 to 7.7 (14.4%) for nonwhite male infants. The mortality rate declined for white female infants from 36.7 to 17.6 (52.0%) and actually increased slightly from 12.8 to 13.2 (3.1%) for nonwhite female infants. Thus, the declining incidence of anencephaly reflects a declining incidence of affected white infants but not of affected nonwhite infants.
Subject(s)
Anencephaly/mortality , Anencephaly/etiology , Anencephaly/prevention & control , Cross-Sectional Studies , Humans , Incidence , Infant, Newborn , United States/epidemiologyABSTRACT
We report two siblings with a mitochondrial encephalomyopathy. The syndrome was characterized by ataxia, intellectual impairment, myoclonic jerks, rare seizures, and small stature. Muscle biopsy specimens showed abnormal accumulations of mitochondria and lipid droplets. Biochemical studies on muscle demonstrated decreased succinate-cytochrome c reductase activity in the mitochondrial respiratory chain.
Subject(s)
Brain Diseases/pathology , Mitochondria, Muscle/ultrastructure , Muscular Diseases/pathology , Brain Diseases/enzymology , Child , Female , Humans , Male , Mitochondria, Muscle/enzymology , Muscular Diseases/enzymology , Succinate Cytochrome c Oxidoreductase/metabolismABSTRACT
Serial motor conduction velocities and distal motor latencies were determined in two pairs of dizygotic twins, each born to a parent with dominant hypertrophic neuropathy of the Charcot-Marie-Tooth type (HMSN-I). Motor nerve conduction velocities could not distinguish between the normal and affected twin of the first pair studied at birth. Distal motor latency in the affected twin at birth, however, was borderline prolonged. The affected twin of the second pair had slowed motor velocities at age 17 months, but the extent of conduction slowing had not yet fully developed. Studies of these patients and the affected family members showed that maximal slowing of motor nerve conduction velocities evolved over the first 3-5 years of life in HMSN-I. Prolongation of distal motor latency may be the earliest abnormality observed in HMSN-I and this abnormality evolves over 10 or more years.
