Holt Oram syndrome mistaken for thalidomide embryopathy--embryological considerations.
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Biomedical subjects
Publications and source records attributed to C Kirkpatrick.
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The adenosine-3',5'-monophosphate (cAMP) level in human amnion has been measured in vitro during short-term incubations, with or without prostaglandin E1 (PGE1) or isoproterenol. Concentration-response and kinetic curves were obtained. Isoproterenol and PGE1 stimulated cAMP production by amnion in a time-dependent manner. The stimulating effect of isoproterenol and noradrenalin on cAMP level was abolished by the addition of propranolol. Our study, demonstrating cAMP production by amniotic membranes and its stimulation by PGE1 and beta-agonists, suggests that hormones could modulate the amniotic cell metabolism through the cAMP system.
Biparietal diameter and femoral and humeral length were measured by ultrasonography and correlated with age in 450 normal pregnancies. The resulting data may be helpful in detecting fetal limb malformation.
Seven patients with threatened premature labor and six nonpregnant female control subjects were studied for 12 to 24 hours during ritodrine infusion in order to re-evaluate the effects of this beta-adrenergic tocolytic agent on blood anions, electrolytes, and acid-base balance. Dexamethasone was given intramuscularly in all patients and three control subjects at the beginning of ritodrine infusion. Lactate, pyruvate, betahydroxybutyrate, sodium, potassium, chloride, bicarbonate, pH, glucose, and insulin levels were measured in blood samples obtained at intervals of 2 to 6 hours. While pH remained in the normal range, increases in lactate and pyruvate levels and decreases in bicarbonate values resulted in a significant increase in anion gap 4 to 6 hours after the beginning of treatment. The blood potassium level fell simultaneously. These effects were transient, lasting less than 10 hours, despite continuation of ritodrine infusion and maintenance of tocolysis and tachycardia. Although larger increases in the blood glucose level were observed with combined therapy in control subjects, dexamethasone had no additional influence on potassium concentrations. Monitoring of patients treated by intravenous ritodrine should include routine measurements of blood glucose and electrolyte levels in order to detect its potentially harmful metabolic side effects for mother and fetus.
Serum unconjugated estriol levels were measured in 44 pregnant women treated with corticoids for the prevention of respiratory distress syndrome (RDS) in premature infants. Intravenous administration of cortisol (1 g every 6 hours for 24 hours) decreased the level of circulating unconjugated estriol to 40% of the initial value after 6 hours and to 27% after 24 hours. During the 2 succeeding days, all the results remain below the pretreatment values. On the third day, the mean value is no longer different from the control value, but in some patients recovery takes more than 3 days.
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Circulating thymic-hormone activity was assayed by measuring Thy 1-2 antigen induction on null lymphocytes from athymic mice incubated with human plasma or serum. Plasma from 19 normal children aged under 10 had inductive activity equivalent to 10-6-16-2 ng thymopoitin/ml. Plasma from 15 infants were severe combined immuno-deficiency, 2 of whom had appreciable immunoglobulin synthesis, and from 2 infants with DiGeorge syndrome had little or no inductive activity. Successful reconstitution with thymus or bone-marrow grafts and with red-cell infusions (if adenosine-deaminase deficiency is present) was followed by a rise in circulating thymic-hormone activity.
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Substantial evidence has accumulated to indicate not only that mycosis fungoides and the Sézary syndrome are closely related malignancies, but to suggest that they are part of a larger spectrum of cutaneous lymphomas. The neoplastic cells of these disorders have membrane features of thymus-derived (T) lymphocytes, a characteristic tissue distribution (skin infiltration, marrow sparing, localization in T-cell regions of lymphoid tissue), and distinctive morphology. For these reasons, we suggest that these lymphoproliferative disorders be grouped together as "cutaneous T-cell lymphomas". The anergy noted in patients of this group with leukemia probably is related to both decreased percentages of normal T cells and presence in the serum of macrophage migration inhibitory activity. Leukapheresis has been particularly effective in the management of selected patients. The homogeneous T-cell populations in the patients with leukemia also provide important opportunities to study many aspects of lymphocyte physiology that are of broad biologic significance.
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