Immunosuppressive drugs and hypertrophic cardiomyopathy.
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Bronchopulmonary Dysplasia↗
Biomedical subjects
F Brus
Publications and source records attributed to F Brus.
Aspirin versus indomethacin treatment of patent ductus arteriosus in preterm infants with respiratory distress syndrome.
Indomethacin (Indo) is commonly used for treatment of patent ductus arteriosus (PDA) but has renal failure as a main side effect. Aspirin (ASA) is an alternative, but there are no controlled trials on its efficacy. We randomly assigned 75 premature infants suffering from respiratory distress syndrome (RDS) (mean gestational age: 29.6 +/- 2.5 wk, mean birth weight: 1295 +/- 464 g) (+/- SD) and on artificial ventilation at the start of the study (mean: 3.4 d of life), to either Indo (3 x 0.2 mg/kg/12 h) or ASA (4 x 15 mg/kg/6 h). PDA and degree of shunting were evaluated by echocardio-Doppler; side effects were carefully recorded. PDA closed in 35/38 patients from the Indo group (92%) and in 16/37 patients from the ASA group (43%) (p < 0.0001). Nineteen patients needed further treatment with Indo or surgery (17 in the ASA group and 2 in the Indo group). The only side effect observed was a decrease of uresis in the Indo group during 4 d post treatment (p < 0.01). Closing of PDA was positively correlated with gestational age, but not with time of starting Indo/ASA or grade of shunting. We conclude that ASA is not as effective in closing PDA as Indo, but has no adverse effect on uresis.
Aspirin↗
Activation of the plasma clotting, fibrinolytic, and kinin-kallikrein system in preterm infants with severe idiopathic respiratory distress syndrome.
We studied the activation pattern of clotting, fibrinolysis, and kinin-kallikrein during the first 5 d of life in 10 preterm infants with signs of severe idiopathic respiratory distress syndrome (IRDS) after birth (IRDS group) and in 12 healthy preterm infants (reference group). We found systemic activation of clotting, fibrinolysis, and kinin-kallikrein in the IRDS infants within 12 to 24 h of birth, represented by increased median thrombin-antithrombin III complex formation (90 ng/mL versus 10 ng/mL in the reference group, p < 0.05), increased mean tissue-type plasminogen activator plasma concentrations (11.8 ng/mL versus 3.5 ng/mL in the reference group, p < 0.05), and increased mean plasma kallikrein activity (182.6% versus 162.0% of maximal activated human plasma in the reference group, p < 0.05), respectively. Clotting activation was accompanied by a significant decrease of the platelet count. Clotting and fibrinolytic activity decreased in the IRDS group during the first 2 to 3 d of life. Kinin-kallikrein activation was accompanied by decreased plasma kallikrein inhibitor activity values and did not change throughout the study period. Plasma factor XII activity was not significantly increased in the IRDS infants during the first 2 d of life but did significantly increase thereafter. The cause of simultaneous activation of clotting, fibrinolysis, and kinin-kallikrein in our IRDS infants has not yet been clarified. However, this activation process may contribute to lung injury such as that described in the adult respiratory distress syndrome.
Antithrombin III↗
Non-immune hydrops fetalis and bilateral pulmonary hypoplasia in a newborn infant with extralobar pulmonary sequestration.
Extralobar pulmonary sequestration was found in a newborn premature infant that presented with non-immune hydrops fetalis, massive bilateral hydrothorax and polyhydramnios in utero. The baby died of severe respiratory insufficiency 15 h after birth. Postmortem examination revealed distended lymphatic vessels in the sequestered lung tissue probably due to impeded lymph drainage. We suggest that not extralobar pulmonary sequestration itself but a subsequent massive unilateral hydrothorax due to severe obstruction of lymph drainage was the cause of the non-immune hydrops fetalis, pulmonary hypoplasia and polyhydramnios. If these symptoms are diagnosed before delivery, a search for extralobar pulmonary sequestration is indicated.
Adult↗
[Malrotation?].
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Digestive System Abnormalities↗
Fatal ureaplasmal pneumonia and sepsis in a newborn infant.
Ureaplasma urealyticum was isolated in pure culture from blood tracheal aspirate and lung tissue in a newborn infant, who died of a severe pneumonia within 48 h after birth. The clinical course was characterized by persistent pulmonary hypertension of the newborn (PPHN). Post-mortem examination revealed extensive hyaline membrane formation combined with signs of inflammation in both lungs. The clinical and histopathological picture resembled that of early onset group B haemolytic streptococcal pneumonia/sepsis.
Humans↗
Streptokinase treatment for femoral artery thrombosis after arterial cardiac catheterisation in infants and children.
Data on 205 children who underwent retrograde arterial catheterisation were studied to assess the frequency of femoral artery thrombosis and the safety and efficacy of systemic streptokinase treatment for this complication. In 29 (14%) a transarterial balloon dilatation was performed. In 15 (7.3%) patients impaired arterial perfusion due to vascular spasm with or without thrombus formation was seen in the cannulated leg after catheterisation. Despite heparinisation, signs of impaired arterial circulation persisted in nine patients (4.4% of the total). In these patients femoral artery thrombosis was strongly suspected. Six (53%) of these had undergone a balloon dilatation. Therefore in this study the risk of femoral artery thrombosis developing was 12 times greater after transarterial balloon dilatation than after arterial catheterisation without dilatation (20.6% v 1.7%). Systemic infusion of streptokinase was started in all patients with femoral artery thrombosis. Arterial perfusion became normal in all patients, though in one this was delayed. Haematological monitoring showed lengthening of the thrombin time and a decrease of the fibrinogen concentration during streptokinase treatment. There were no serious complications. Systemic infusion of streptokinase is a safe and useful treatment in children with persistent femoral artery thrombosis after arterial cardiac catheterisation.
Cardiac Catheterization↗
[Obstructive disorders of the urinary tract in the unborn child].
The history of a patient with signs of urinary tract obstruction in utero is presented. After this presentation pathophysiologic, diagnostic and therapeutic aspects of prenatal detected obstructive malformations of the urinary tract are discussed. Obstructions of the fetal urinary tract may cause damage to the developing renal parenchyma, impairment of the normal lung development and fetal growth retardation. The seriousness of obstruction and the time of appearance during pregnancy determine the severity of the damage to the unborn child. Optimum management in case of prenatal detected obstructive uropathies needs good information about renal function of the fetus, the presence of other structural defects or chromosomal abnormalities and also the maturity and state of development of the lungs. Besides careful ultrasonography of the fetus invasive methods of investigation may be necessary. In case of fetal obstructive uropathy intrauterine decompression or extrauterine decompression after an induced premature delivery can be considered. However a good renal function and the absence of other severe structural or chromosomal malformations are necessary. When the gestational age has come to 32 to 34 weeks and the lungs are mature enough decompression of the urinary tract after an induced premature delivery can be done. When pregnancy is less than 32 weeks of gestational age and the lungs are immature intrauterine decompression is possible. The most used technique of intrauterine decompression so far is insertion of a percutaneous amniotic-bladder catheter. Antenatal detected obstructive abnormalities of the fetal urinary tract need optimum perinatal care besides good information and support of the parents.
Abnormalities, Multiple↗
[Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency in 2 patients with symptoms of Reye syndrome].
Two patients are described, who were submitted to our clinic with signs of the Reye syndrome. In both cases a medium-chain acylcoënzyme A dehydrogenase (MCAD) deficiency was diagnosed. This is an inborn error of the mitochondrial beta-oxidation of fatty acids. Stimulation of the fatty acid oxidation in case of this enzyme deficiency might result in a metabolic crisis presenting clinically as the Reye syndrome. The structure of a fatty acid molecule and the process of beta-oxidation of fatty acids are discussed shortly in this article. The most important clinical, diagnostic and therapeutic aspects of MCAD deficiency are presented next. A MCAD deficiency seems not to be rare in cases presenting as a Reye-like syndrome. Accurate distinction between MCAD deficiency and Reye syndrome can be made by gas chromatographic together with mass spectrometric analysis of urine. Investigation of so called crisis urine is of utmost importance. Confirmation of the diagnosis needs measuring of MCAD enzyme activity in cultured fibroblasts or in leucocytes of the patient.
Acyl-CoA Dehydrogenase↗
[Chlamydia pneumonia in infants].
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Antigens, Bacterial↗
Large volume instillation of surfactant during extracorporeal life support improves lung function in lung lavaged rabbits.
The sometimes limited effect of surfactant therapy in neonates might be explained in part by an non homogeneous distribution of the surfactant after endotracheal instillation. This distribution can be improved significantly by increasing the fluid volume. The aim of this study was to evaluate the effect of two methods for gas exchange during a large volume instillation of surfactant on the outcome of this treatment in lung lavaged rabbits. In the control group (n = 6) gas exchange was maintained with continuous positive pressure ventilation (CV), whereas in the other group gas exchange was established with extracorporeal life support (ECLS) (n = 6) and intermittent sighs. Five hours after surfactant administration, an identical weaning procedure was started in both groups. The authors found significantly higher PaO2 values in the ECLS group than in the control group in the normocarbia state. All animals in the ECLS group could be weaned to room air maintaining normal blood gases, whereas all the animals in the control group died in the course of weaning. The ventilator efficiency index was significantly higher during the weaning period in the ECLS group, indicating better lung function, than in the control group. The authors conclude that a large volume instillation of surfactant is feasible by applying ECLS and intermittent sighs. Additional studies are needed to elucidate if this combined treatment will be an improvement over current surfactant therapy.
Animals↗
Hypertrophic obstructive cardiomyopathy as a side effect of dexamethasone treatment for bronchopulmonary dysplasia.
We report three infants who developed hypertrophic obstructive cardiomyopathy during dexamethasone treatment for bronchopulmonary dysplasia. In all three infants, echocardiography had ruled out cardiac abnormalities prior to the dexamethasone course. The hypertrophic obstructive cardiomyopathy appeared and progressed during dexamethasone therapy and resolved completely after its cessation. This suggests a causative association between the hypertrophic obstructive cardiomyopathy and the exogenous glucocorticosteroid therapy. The mechanism of this dexamethasone-related hypertrophic obstructive cardiomyopathy is unclear. This complication may be encountered more frequently with the increasing use of dexamethasone in infants with bronchopulmonary dysplasia.
Bronchopulmonary Dysplasia↗
The clinical spectrum of post-streptococcal syndromes with arthritis in children.
Acute rheumatic fever (ARF) and post-streptococcal reactive arthritis (PSRA) are well known complications of streptococcal throat infections. We describe four children with arthritis following a streptococcal throat infection. In addition to arthritis, other clinical manifestations included erythema nodosum, livedo reticularis and cutaneous vasculitis. Because of the very diverse clinical manifestations that may appear after a streptococcal throat infection, we suggest a classification and treatment of post-streptococcal syndromes according to the severity of the disease.
Acute Disease↗