Performance of Subcutaneous Continuous Glucose Monitoring in Adult Critically Ill Patients Receiving Vasopressor Therapy.

Subcutaneous continuous glucose monitoring (CGM) may facilitate glucose control in the ICU. We aimed to assess the accuracy of CGM (Dexcom G6) against arterial blood glucose (ABG) in adult critically ill patients receiving intravenous insulin infusion and vasopressor therapy. We also aimed to assess feasibility and tolerability of CGM in this setting.

Investigation of deletions at 7q11.23 in 44 patients referred for Williams-Beuren syndrome, using FISH and four DNA polymorphisms.

Williams syndrome (WS) is associated with a submicroscopic deletion of the elastin gene (ELN) at 7q11.23. The deletion encompasses closely linked DNA markers.

Noonan syndrome with café-au-lait spots and multiple lentigines syndrome are not linked to the neurofibromatosis type 1 locus.

Noonan syndrome, multiple lentigines syndrome (LEOPARD syndrome), Watson syndrome and neurofibromatosis type 1 share certain clinical manifestations. We present a linkage analysis using microsatellite markers located in the neurofibromatosis type 1 region at 17q11 in a family with Noonan syndrome and café-au-lait spots and in another family with multiple lentigines syndrome. No linkage of the disease to the neurofibromatosis type 1 locus was found in the families investigated.

Clinical experience of pacemaker treatment in children.

Pacemaker treatment is more complicated in children than in adults, mainly because of electrode problems. A pacemaker system was implanted in 23 children aged 2 days-14 years (mean 4.8 years, with 11 less than or equal to 3 years) at the Karolinska Hospital since 1983.

Total anomalous pulmonary venous connection in siblings. Report on three families.

Three pairs of siblings with total anomalous pulmonary venous connection (TAPVC) are reported. The types of the anomalous venous return (supra- or infracardial connections) varied within the families indicating that genetic regulation deals with the left atrial connection to the intrapulmonary veins. Having failed to establish this connection the intrapulmonary veins can attach themselves to any adjacent venous structure; hence the variety of connections at birth.

The "cat eye syndrome": dicentric small marker chromosome probably derived from a no.22 (tetrasomy 22pter to q11) associated with a characteristic phenotype. Report of 11 patients and delineation of the clinical picture.

Eleven patients with the so-called Cat Eye syndrome are reported including a more detailed description of the original cases reported by Schmid and Fraccaro. All cases had, in addition to a normal karyotype, a small extra G-like chromosome which appeared to be an isochromosome for the juxtacentromeric region (pter to q11) of an acrocentric chromosome. None were mosaics.

Ventricular dynamics after surgical closure of VSD.

Twenty patients varying in age between 5 and 20 years at the time of surgical closure of VSD were studied 2 to 9 years postoperatively. Ventricular function was studied by echocardiography and measurement of systolic time intervals for the left and right ventricles and the findings were related to clinical and haemodynamic results of operation. The VSD was closed in all instances and the haemodynamic situation was normalized in all but 2 patients who had persisting pulmonary vascular disease.

What is a shunt? Concepts of central shunts and effective flow and a method for graphical presentation of circulatory quantities and their interrelations.

The terminologies commonly used in descriptions of abnormal patterns of blood flow in patients with congenital heart defects are valid only in the case of simple abnormal communications in an otherwise essentially normal cardiac anatomy. In more complex cases the expressions are unambiguous and elucidatory only when qualified further, but then the terminology becomes cumbersome. Much might be gained in clarity by considering the combined ventricular output as having four components: two are essential, and equal in size; they constitute the effective flows to the systemic and pulmonary arterial trees of blood from the pulmonary and systemic veins, respectively.

IV. Evaluation of dichromatic earpiece densitometry in children with left-to-right shunts.

Earpiece densitometry was performed in 60 infants and children with left-to-right cardiovascular shunts. The method was found to possess a high diagnostic sensitivity with respect to identification of the left-to-right shunt. Earpiece densitometry was in this respect clearly superior to conventional method using oxygen saturation figures.

II. Cardiac output studies in infants and children undergoing right heart catheterization.

Cardiac output figures from 97 patients were computed by earpiece densitometry and correlated to various dimensional parameters. Stroke volume was better correlated to body dimensions than cardiac output indicating that patients undergoing right heart catheterization were not in basal state.

V. Exercise studies in children with congenital heart disease. An earpiece densitometric investigation.

Twenty-three patients have been studied by exercise testing during right heart catheterization. Cardiac output was measured by the earpiece densitometric technique before, during and after the exercise period. It is concluded that the earpiece densitometric technique in conjunction with exercise testing offers an attractive alternative for the study of pressure and flow characteristics in children with congenital heart disease.

I. Validity of dichnomatic earpiece densitometry for quantitative studies in children with shunt-free cardiovascular malformations.

Cardiac output was assessed in 16 children without evidence of cardiovascular shunts from simultaneously recorded earpiece and cuvette densitometric tracings. The earpiece set-up was calibrated by the end-tail method. Both instruments gave linear response to dye concentrations used.