Myelodysplastic syndromes in the adolescent.

Myelodysplastic syndromes (MDS) are a group of acquired blood diseases that are the result of abnormal bone marrow function. The ineffective production of red cells, platelets, and white blood cells can lead to symptomatic anemia, bruising, infections, and the likelihood of evolution into acute myelogenous leukemia. While MDS is uncommon in the adolescent patient, a surprising number of affected individuals are also affected with a predisposing constitutional syndrome. The treatment of MDS in the adolescent patient is in part determined by symptoms and also by the historical outcomes associated with each of five morphologic categories of presentation. Improved supportive care has allowed for increasingly more children with MDS to survive into the second decade of life. The management of MDS in affected adolescents presents a number of interesting and worthwhile challenges to health care professionals.

Ultrasound screening of healthy infants for urinary tract abnormalities.

The purpose of the study was to determine the incidence of silent but significant urinary tract abnormalities that might be detected by screening renal ultrasound studies of apparently healthy infants. Of 437 babies studied, six were found to have uropathology severe enough to warrant surgery for an incidence of 1.37% or one of every 73 babies studied.

Chromatographic behavior of cyclic AMP dependent protein kinase and its subunits from Dictyostelium discoideum.

An adenosine cyclic 3',5'-monophosphate (cAMP) dependent protein kinase has recently been shown to exist in Dictyostelium discoideum and to be developmentally regulated. In this report we have followed the chromatographic behavior of both the holoenzyme and its subunits. A cAMP-dependent holoenzyme could be obtained from the 100000 g soluble fraction after passage through DE-52 cellulose (pH 7.5) and Sephacryl S300. Under conditions of low pH the holoenzyme could be further purified by flat-bed electrofocusing (pI = 6.8). Application of the holoenzyme to electrofocusing at high pH resulted in dissociation of the holoenzyme into a cAMP binding component (pI = 6.1) and a cAMP-independent catalytic activity (pI = 7.4). Dissociation of the holoenzyme into subunits also occurred during histone affinity chromatography and gel filtration chromatography (S300) in the presence of a dissociating buffer. Although the subunit structure was clearly evident during chromatography, the holoenzyme could not be dissociated by simple addition of cAMP to the extract. The catalytic subunit could be purified further by CM-Sephadex, DE-52 cellulose (pH 8.5), histone affinity, and hydrophobic chromatography. The regulatory subunit was further purified by DE-52 cellulose (pH 8.5) and cAMP affinity chromatography. Proof that the cAMP binding activity and the cAMP-independent catalytic activity were in fact the regulatory and catalytic subunits was shown by reconstitution of the cAMP-dependent holoenzyme from the purified subunits. By using these separation procedures, one can obtain from extracts of Dictyostelium the subunits that are free of each other as well as free of any endogenous protein substrates.

Transport of the neonate.

A regionalization program, initiated in 1973 at Vanderbilt University Medical Center, has included community hospital site visits, formal nursing courses, and neonatal transport. The neonatal transport program was designed not only to give initial medical care to the sick newborn, but also to develop the educational aspects of the regionalization program. Analysis of data from the first two years (614 transports) revealed significant educational interactions occurring during transport. Improved perinatal care the second year was reflected in decreased regional neonatal mortality and improved neonatal management.