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Biomedical subjects

G W Moore

Publications and source records attributed to G W Moore.

At least 163 records · Page 9Linked to original sources
PubMed1980

The liver in sickle cell disease. A clinicopathologic study of 70 patients.

In clinical studies, frequent hepatic dysfunction associated with crises in sickle cell disease has been noted, but whether irreversible morphologic changes arise from these transient episodes is uncertain. We studied 70 patients with sickle cell disease (57 SS, 12 SC and one S-thalassemia (S-thal) hemoglobin) autopsied at The Johns Hopkins Hospital. They ranged in age from five months to 75 years (average 21 years) and 35 (50 percent) were female, In 64 patients (91 percent), livers were enlarged and had distention of Kupffer cells with phagocytized sickled red cells; this was massive in 10. In 19 patients (27 percent) the sinusoids were markedly distended with sickled red cells and appeared obstructed. Focal parenchymal necroses were present in 24 patients (34 percent) and were explained in 12, eight by cardiac dysfunction and four by sepsis. Reparative changes, portal fibrosis and regenerative nodules were each found in 14 patients (20 percent), only one of whom had a known history of viral hepatitis despite the frequency of transfusions. Cirrhosis of unknown cause was present in seven patients and cardiac cirrhosis in one. Cirrhosis with hemochromatosis was present in three patients and 30 others had parenchymal iron accumulation. Thus, unexplained hepatic necroses, portal fibrosis, regenerative nodules and cirrhosis were frequently encountered in these patients. This spectrum of liver disease appears to be best understood as a consequence of recurrent vascular obstruction, necrosis and repair arising as a component of sickle cell disease.

Adolescent↗
PubMed1980

Gonadal dysfunction in adult men with congenital adrenal hyperplasia.

Two adult males are described with congenital adrenal hyperplasia (21-hydroxylase deficiency). Patient 1 was receiving therapy with cortisone acetate and presented with clinical features of glucocorticoid excess and uncontrolled adrenal androgen activity. It was established that the short-acting steroid which the patient was receiving was cleared so rapidly that endogenous ACTH secretion was not inhibited. Patient 2 presented with enlarged and painful testes in association with poor compliance with corticosteroid therapy. The histologic picture of the testis was compatible with 'Leydig cell hyperplasia'. However, successful response to dexamethasone therapy suggests that the testes harboured an adrenal rest. These observations highlight the need for careful follow-up and treatment of adult male patients with congenital adrenal hyperplasia.

Adrenal Hyperplasia, Congenital↗
PubMed1979

Role of the left interventricular sulcus in formation of interventricular septum and crista supraventricularis in normal human cardiogenesis.

Serial sections of normal human embryos were studied and three-dimensional images reconstructed to determine the early development of the interventricular septum. The position of the interventricular septum is determined in stage 9 of normal development by the formation of the left interventricular sulcus. As a result of unknown properties of the cells of the myocardial layer, the left interventricular sulcus persists while the right disappears, producing the initial lateral asymmetry of the primary heart tube. By stage 14, the left interventricular sulcus forms a spiral which is continuous with the developing interventricular septum. The dorsal limb of the spiral passes to the right between the atrioventricular canal and the origin of the outflow tract, and is lost in the wall of the trabeculated right ventricle. It appears that this dorsal limb of the spiral is the precursor of part of the cirsta supraventricularis. The midportion of the sulcus, the bulboventricular groove, becomes the so-called fibrous continuity between the aortic and mitral valves. The ventral limb of the spiral passes caudally in the anterior interventricular groove and then dorsally and cranially toward the dorsal cushion of the atrioventricular canal. The ventral limb of the spiral is continuous with the crest of the muscular interventricular septum, which develops by apposition of tissue from the expanding right and left ventricles. From stage 14 to stage 19, the muscular interventricular septum, the atrioventricular endocardial cushions, and the ventricular end of the spiral ridges of the outflow tract appose and fuse. Subsequent formation of the membranous interventricular septum completes the physical separation of the right and left ventricles.

Endocardium↗
PubMed1979

Reversed septal curvature. Association with primary pulmonary hypertension and Shone syndrome.

In the normal heart the interventricular septum is curved concave to the left and functions as a component of the left ventricular wall. We have studied the hearts of five patients at autopsy, after postmortem angiography and fixation in distention, in which the septum was concave to the right ventricle. These patients ranged in age from eight months to 25 years (average, 16 years); three had primary pulmonary hypertension, and two had coarctation of the aorta and congenital aortic stenosis. Catheterization studies in the latter two patients had demonstrated subaortic stenosis. At autopsy there was no evidence of asymmetric septal hypertrophy or of abnormal septal myocardial fiber disarray. The right ventricles were markedly hypertrophied. In each, the septal curvature was reversed leading to protrusion of septal muscle into the left ventricular outflow tract with marked narrowing of the outflow tract. Since the septal configuration is determined early in life, this abnormality of ventricular topography may develop primarilary or it may develop in hearts subject to early as well as long-standing right ventricular overload.

Adolescent↗
PubMed1979

Reduction of serum cholesterol in two patients with homozygous familial hypercholesterolemia by dichloroacetate.

Dichloroacetate is known to reduce plasma cholesterol and triglyceride in patients with Fredrickson Types IIb or IV hyperlipoproteinemia. We now report the effects of chronic, oral dichloroacetate administration (as the sodium salt) in two patients with severe homozygous familial hypercholesterolemia. Dichloroacetate markedly reduced serum total and low density lipoprotein cholesterol levels and lowered the low density lipoprotein to high density lipoprotein cholesterol ratio. One patient developed a polyneuropathy while receiving dichloroacetate which resolved following discontinuation of the drug. Because of its apparent toxicity, dichloroacetate cannot be recommended for chronic oral use. Investigation of the mechanism of its lipid-lowering effect, however, may provide insight into the pathogenesis and treatment of hypercholesterolemic disorders.

Acetates↗
PubMed1979

Atrioventricular canal malformation interpreted as secondary to reduced compression upon the developing heart.

This study was undertaken to evaluate the nature and pathogenesis of malformations of the atrioventricular canal in relation to normal cardiogenesis. Serial histologic sections of normal human embryos and fetuses were made, from which three-dimensional images were reconstructed to show the relationship between the developing heart and its surrounding structures, and the course of development of the atrial septum and atrioventricular valves. Based on these reconstructions and on examination of the hearts of 59 patients with atrioventricular canal malformations, it is suggested that the spectrum of atrioventricular malformations may arise as a result of reduced compression of the developing atria by surrounding structures during embryonic Stages 13 through 18. Comparison of hearts with atrioventricular canal defects with normal embryos indicated that the malformations may be classified as primitive canals, complete canals, or partial canals, corresponding to failure of completion of normal development in Stages 14 through 18. In primitive canal the atrial septum was absent or had only a portion of septum primum. In complete canal both atrial septums were present, but the atrioventricular valve material was not subdivided and the four chambers were in communication. In partial canal, the atrioventricular valve was divided, but atrial and ventricular septal defects and valve clefts were present in varying degrees of severity. It is proposed that the spectrum of cardiac abnormalities which constitutes atrioventricular canal malformations may be understood as arising from varying degrees of lack of normal compression of the developing heart by surrounding structures. (Am J Pathol 95.579-598, 1979)

Adolescent↗
PubMed1978

On investigating the statistical properties of the populous path algorithm by computer simulation. Counterconclusions to those of Tateno and Nei.

Goodman et al.'s (1974) populous path algorithm for estimating hidden mutational change in protein evolution is designed to be used as an adjunct to the maximum parsimony method. When the algorithm is so used, the augmented maximum parsimony distances, far from being overestimates, are underestimates of the actual number of nucleotide substitutions which occur in Tateno and Nei's (1978) computer simulation by the Poisson process model, even when the simulation is carried out at two and a half times the sequence density. Although underestimates, our evidence shows that they are nevertheless more accurate than estimates obtained by a Poisson correction. In the maximum parsimony reconstruction, there is a bias towards overrepresenting the number of shared nucleotide identities between adjacent ancestral and descendant nodal sequences with the bias being stronger in those portions of the evolutionary tree sparser in sequence data. Because of this particular property of maximum parsimony reconstructed sequences, the conclusions of Tateno and Nei concerning the statistical properties of the populous path algorithm are invalid. We conclude that estimates of protein evolutionary rates by the maximum parsimony--populous path approach will become more accurate rather than less as larger numbers of closely related species are included in the analysis.

Biological Evolution↗
PubMed1978

Metabolic effects of dichloroacetate in patients with diabetes mellitus and hyperlipoproteinemia.

Dichloroacetate is known to reduce plasma glucose and triglycerides in diabetic and starved animals and to lower plasma lactate under various experimental conditions. To investigate its metabolic effects in man, we administered oral doses (3 to 4 g) of dichloroacetate as the sodium salt to patients with diabetes mellitus or hyperlipoproteinemia or both for six to seven days. Dichloroacetate significantly reduced fasting hyperglycemia an average of 24 per cent (P less than 0.01) from base line and produced marked, concomitant falls in plasma lactate (73 per cent; P less than 0.05 to less than 0.01) and alanine (82 per cent; P less than 0.01 to less than 0.001). In addition, it significantly decreased plasma cholesterol (22 per cent; P less than 0.01 to less than 0.001) and triglyceride (61 per cent; P less than 0.01) levels while increasing (71 per cent; P less than 0.01) plasma ketone-body concentrations. Plasma insulin, free fatty acid and glycerol levels were not affected. Serum uric acid rose, whereas excretion and renal clearance fell. Some patients experienced mild sedation, but no other laboratory or clinical evidence of adverse effects was noted during or immediately after the treatment phase.

Acetates↗
PubMed1978

Association of interrupted aortic arch with malformations producing reduced blood flow to the fourth aortic arches.

Interrupted aortic arch is a rare congenital malformation of the great arteries with loss of continuity between the proximal portion of the aortic arch and the descending aorta, which is usually supplied by a patent ductus arteriosus. The hearts of 12 patients with interrupted aortic arch listed in the autopsy files of The Johns Hopkins Hospital were evaluated. Four categories were identified. Seven hearts with normal outflow tract relations had significant pulmonary arterial override of a ventricular septal defect and thus recevived part of left ventricular and all of right ventricular ejection flow; flow into the aorta was correspondingly reduced. Two hearts had mitral valve obstruction with decreased left heart flow. Two hearts had double inlet left ventricle and transposed great arteries with the aorta originating from a small anterior conal chamber. One heart had a truncus malformation with a disproportionately large flow entering the pulmonary arterial division. In each of these hearts, the pattern of the outflow tracts and associated malformations suggested that there was a preferential flow of blood into the sixth arches and correspondingly a reduced flow into the fourth arches at the time of normal disappearance of components of the embryonic aortic arch system. Thus, interrupted aortic arch may simply be disappearance of the normally persisting connection between the left fourth and sixth arches as a result of flow imbalance in early cardiogenesis.

Aorta, Thoracic↗