Biomedical subjects

R F Labbe

Publications and source records attributed to R F Labbe.

At least 37 records · Page 2Linked to original sources

PubMed1980

Chronic vitamin A intoxication in infants fed chicken liver.

Twin female infants were fed 120 gm of chicken liver homogenate daily for four months. They developed irritability, vomiting, and bulging anterior fontanelles. Computed tomograms of the brain revealed enlarged ventricles in both infants and dilated subarachnoid spaces in one. Plasma vitamin A concentrations were elevated. After all sources of vitamin A intake were stopped, the infants recovered without sequelae. The chicken liver homogenate contained 36,000 IU of vitamin A per 120 gm. Since infants often receive 4,000 units of vitamin A daily from fortified milk and vitamin supplements, they probably cannot be fed 60 gm of chicken liver safely more often than once weekly.

Animals↗

PubMed1979

Inherited deficiency of delta-aminolevulinic acid dehydratase.

Delta-aminolevulinic acid dehydratase (ALA-D) is the second enzyme in the porphyrin-heme pathway and converts delta-aminolevulinc acid (ALA) to porphobilinogen (PBG). A family is reported with an inherited deficiency of red cell ALA-D activity occurring over three generations in an autosomal dominant pattern. Intial experiments support the hypothesis that the mutation in this family may affect a regulatory gene, but enzyme purification and further study are required. Although no clinical manifestations of deficient ALA-D activity have been found in affected persons, families such as this may be at increased risk for the serious consequences of lead poisoning, which produces marked inhibition of ALA-D activity.

Adolescent↗

PubMed1977

Effect of blood donation on iron stores as evaluated by serum ferritin.

Serum ferritin was measured in 2982 blood donors. First-time male donors had a geometric mean of 127 microgram/liter and female donors 46 microgram/liter. While values were essentially constant in the women between the ages of 18 and 45, there was a rapid increase in the men between 18 and 30 years of age consistent with the establishment of iron stores during that time. Blood donation was associated with a decrease in serum ferritin. One unit per year, equivalent to an increased requirement of 0.65 mg/day, halved the serum ferritin level in the male. More frequent donations were associated with further decreases. From the data obtained it would appear that male donors, while depleting their iron stores, were able to donate 2-3 U/yr without an appreciable incidence of iron deficiency. Women could donate only about half that amount, and more frequent donations were associated with a high incidence of iron deficiency and donor dropout. These data have provided information on the effect of graded amounts of iron loss through bleeding on iron balance.

Adolescent↗

PubMed1976

Extracorporeal treatment with cyanate in sickle cell disease: preliminary observations in four patients.

The effects of extracorporeal carbamylation were determined in four patients with sickle cell anemia. Treatments were carried out in clusters every 4 to 6 weeks over a 6-month period. Mean carbamylation achieved in vivo was 1.3 moles cyanate per mole of hemoglobin. The mean in vitro P50 during treatment was 22.1 mm. Hg as compared to the pretreatment value of 30 mm. Hg. Hemoglobin concentration was increased over the 6 months by 24 per cent. The treatment resulted in the stimulation of erythropoiesis. Treated cells had a prolonged survival of about 40 days, but bilirubin did not fall. Blood volume and heart size remained unchanged. Carbamylation did not appear to alter the degree of sickling at any given O2 saturation. Painful attacks continued through the treatment period with a frequency of 84 per cent and a severity-frequency index of 77 per cent of that in the control period. There was no evidence of cyanate toxicity associated with extracorporeal therapy.

Adult↗

PubMed1975

Feline congenital erythropoietic porphyria associated with severe anemia and renal disease. Clinical, morphologic, and biochemical studies.

A feline erythropoietic porphyria was studied in an affected female Siamese cat and 2 male offspring. The principal elevated porphyrins were Type I isomers of uroporphyrin and coproporphyrin; the porphyrin precursors, porphobilinogen and sigma-aminolevulinic acid, were also detected. Porphyrins were present in the blood and in all the viscera, teeth, bones, and excreta. There was severe macrocytic hypochromic anemia, hepatomegaly, splenomegaly, and uremia associated with a renal disease characterized by mesangial hypercellularity and proliferation (resulting in narrowing of glomerular capillaries) and ischemic tubular injury. There was thickening of tubular basement membranes and tubular epithelial lipidosis, degeneration, and necrosis. Electron microscopic studies of bone marrow and kidney revealed the presence of membrane-enclosed lamellar bodies 150 to 1000 nm in diameter in cytoplasmic and extracellular locations.

Anemia, Hypochromic↗

PubMed1974