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Biomedical subjects

W B Geven

Publications and source records attributed to W B Geven.

10 recordsLinked to original sources

Absent thumb, immune disorder, and congenital anemia presenting with hydrops fetalis.

A patient is described who presented with severe congenital anemia, hydrops fetalis, immune disorder, and absent thumbs. No toxic, infectious, or metabolic cause was found to explain these symptoms. Immunologic and cytogenetic studies excluded several syndromes that combine radial ray anomalies with hematological involvement. After careful study of the literature, it is concluded that the disorder described here represents a new syndrome that can be added to a growing list of hematological-radial syndromes.

Abnormalities, Multiple

31P nuclear magnetic resonance and zero-point titration compared for measuring free magnesium concentration in erythrocytes.

Intracellular ionized magnesium concentrations ([Mg2+]i) were measured in erythrocytes by 31P nuclear magnetic resonance (NMR) and zero-point titration in 14 controls and seven patients with renal magnesium loss. The mean intracellular ionized magnesium concentration in controls measured by 31P NMR was 0.20 (SD 0.03) mmol/L cell water, compared with 0.55 (SD 0.12) mmol/L cell water by zero-point titration. Total erythrocyte magnesium content measured with the lysate method was 0.63 mmol/L cell water higher than estimated by 31P NMR, probably because not all magnesium complexes are fully visible to the NMR technique. We found a positive correlation between plasma ultrafiltrable magnesium and [Mg2+]i irrespective of the [Mg2+]i assay used. [Mg2+]i measured with 31P NMR correlated modestly but significantly with [Mg2+]i determined by zero-point titration (r = 0.58, P less than 0.02). Washing erythrocytes before the zero-point titration decreased the ATP content and the cell water fraction, which led to overestimation of [Mg2+]i by zero-point titration. Although absolute values for [Mg2+]i differ with the assay used, both methods determined significantly lower values for [Mg2+]i in patients with isolated renal magnesium loss.

Adenosine Triphosphate

[Neonatal dural sinus thrombosis].

Neonatal dural sinus thrombosis is a relatively unknown disease. We recently diagnosed two newborn patients with dural sinus thrombosis. Both patients had associated conditions known to predispose to dural sinus thrombosis. One patient had severe perinatal asphyxia, and the other patient had a deficiency of antithrombin III. Both patients had severe neonatal seizures. The literature on some aspects of this entity is discussed. It is concluded that dural sinus thrombosis may represent an important and underrecognized cause of neonatal seizures.

Antithrombin III Deficiency

[2 successful treatments with extracorporeal membrane oxygenation in neonates with severe respiratory problems].

We report the successful treatment with extracorporeal membrane oxygenation of two Dutch neonates with severe respiratory insufficiency, due to meconium aspiration syndrome and persistent fetal circulation respectively. During this procedure part of the cardiac output is led outside the body via a venous cannula in the right atrium, oxygenated in a membrane oxygenator, rewarmed to the patient's body temperature in a heat exchanger and returned to the patient via a cannula in the carotid artery debouching into the aortic arch.

Extracorporeal Membrane Oxygenation

Reference values of magnesium and potassium in mononuclear cells and erythrocytes of children.

Reference values for magnesium and potassium contents of mononuclear cells and erythrocytes were estimated in cord blood and in children from infancy through adolescence. No differences were detected between results for boys and girls. The mononuclear magnesium content was independent of age and was within the adult range of values. No significant correlation was shown between magnesium in serum and in mononuclear cells. Mononuclear potassium also showed no age-related differences. The correlation between magnesium and potassium contents in mononuclear cells was significant: however, the correlation was lower when the magnesium and potassium contents were expressed in terms of protein potent: micromoles or millimoles per gram of protein, respectively. The concentration of magnesium in erythrocytes was significantly lower in cord blood and during the first month of life, compared with that at older ages, and showed no significant correlation with serum magnesium. The concentration of erythrocyte potassium was independent of age and showed a low but significant correlation with erythrocyte magnesium content.

Adolescent

Combined transcutaneous oxygen, carbon dioxide tensions and end-expired CO2 levels in severely ill newborns.

In 12 newborns combined transcutaneous PO2 and PCO2 levels were measured by means of a Sensor Medics Transend Transcutaneous Gas System simultaneously with the end-tidal PCO2 (PetCo2) values using an Angström Eliza CO2 analyzer. Although the individual correlation coefficients differed greatly, the diagnostic sensitivity of PtcO2 and PtcCO2 was satisfactory within specified limits. The PetCO2 did not agree well with the PaCO2 due to physiologic factors related to lung disorders. We conclude that PtcO2 and PtcCO2 values are very useful in monitoring severely ill newborns although arterial blood gas measurements remain necessary, but not as often as when the transcutaneous technique is not used.

Asphyxia Neonatorum

Renal magnesium wasting in two families with autosomal dominant inheritance.

Hypomagnesemia due to isolated renal magnesium loss was demonstrated in two unrelated families with autosomal dominant mode of inheritance. Magnesium infusions performed in two patients showed not only a reduced renal magnesium threshold but also a lowered renal tubular maximum for magnesium. All members of both families who presented with hypomagnesemia had also a lowered excretion of calcium in the urine, presumably as a consequence of increased reabsorption in Henle's loop.

Calcium

Isolated autosomal recessive renal magnesium loss in two sisters.

A familial autosomal recessive form of isolated renal magnesium loss is presented. Two children in this family suffered from convulsions unrelated to hypomagnesemia. Magnesium infusion studies revealed a lowered threshold but a normal tubular maximum for magnesium. In contrast to two families with the autosomal dominant form of isolated renal magnesium wasting, the calcium excretion in the urine was normal.

Calcium