Clinical experiences with low-molecular weight heparins in pediatric patients.

The courses of 79 children (2 weeks to 19 years old) treated with two different low-molecular weight heparins (LMWHs)--nadroparin (n=66) and enoxaparin (n=13)--were retrospectively analysed. In 62 patients, LMWHs were given for short-term prophylaxis (1-2 weeks) during immobilization after surgery or trauma. Thirteen children with thromboembolic events received long-term prophylaxis with LMWHs for 2-18 months--six after thrombolytic therapy and seven after therapy with unfractionated heparin (UFH). Because of thromboembolic events, four patients were initially treated with LMWHs. In all patients with short-term prophylaxis, no thrombosis occurred. After thrombolytic therapy, three children had no reocclusion, two had no thrombus apposition and one had complete recanalization. In the seven patients treated with LMWHs after UFH, four had no reocclusion, two had recanalization and one had reocclusion. In all patients receiving LMWHs for initial treatment of thrombosis, no thrombus apposition, but also no recanalization, occurred. For short-term prophylaxis, nadroparin was used independent of the body weight and without determination of anti-factor Xa (anti-FXa) activity. Long-term prophylaxis was given mainly as doses of 45-100 anti-FXa U/kg resulting in anti-FXa activities between 0.2 and 0.4 U/ml. For treatment of thrombosis, doses of 200-300 anti-FXa U/kg corresponded to 0.5-1.0 anti-FXa U/ml. Side effects--slight gastrointestinal bleeding and temporary reversible hair loss--were seen in two patients. In conclusion, LMWHs proved to be efficacious and safe especially in prophylaxis of thromboembolic events in children.

[Late complications after transcatheter closure of atrial septal defect]. / Spätkomplikation nach katheterinterventionellem Vorhofseptumdefektverschluss.

The transcatheter closure of atrial septal defects is generally accepted as an alternative to surgical therapy in selected patients. Potential complications of these devices are a matter of concern, as well as the very complicated implantation technique required for some systems. We report on a 17 year old patient in whom embolization of a fractured umbrella arm into a peripheral branch of the left pulmonary artery occurred. A 35 mm-ASDOS-Device (protected trademark of the Dr. Osypka GmbH/Grenzach-Wyhlen, Germany) had been implanted 4 years ago. The perfusion scintigraphy of the left lung showed normal pulmonary blood flow. Echocardiography failed to demonstrate instability of the double-umbrella device. The patient was always free of any symptoms. Due to the permanent stresses between device and heart, late fractures of the device and embolizations of fragments are possible. It can be concluded that patients who underwent transcatheter closure of an ASD require lifelong follow-up.

Thrombolytic therapy in children--clinical experiences with recombinant tissue-plasminogen activator.

For the improvement of thrombolytic therapy with recombinant tissue-plasminogen activator (rt-PA) in children, more clinical data are needed. We retrospectively analyzed the clinical course of 20 patients (age ranging from 1 day to 16 years) with venous thrombosis (n = 16), arterial thrombosis (n = 2), and purpura fulminans by meningococcosis (n = 2). The venous thromboses were localized in the iliac-femoral veins (n = 9), brachiocephalic-jugular-subclavian veins (n = 6), and the superior caval vein (n = 1). The arterial occlusions were localized in the abdominal aorta and in the left pulmonary artery. Central venous catheters were of pathogenetic importance in seven cases. The patients were treated with rt-PA for 3 hours to 13 days. The dose ranged between 0.2 and 0.5 mg/kg for the initial bolus and 1.0 to 2.0 mg/kg/d for the continuous infusion. Nineteen patients received simultaneously low-dose unfractionated heparin. Complete clot lysis was detected in 11 cases, a partial lysis in 1, and in 8 patients thrombolytic therapy was not successful. An episode of hematemesis in one patient represented the only serious side effect observed in our study. A systemic decrease in fibrinogen concentration was also rare. In conclusion, thrombolysis with rt-PA represents an effective and safe therapy for children at the dosage used.

Clinical importance of prothrombotic risk factors in pediatric patients with malignancy--impact of central venous lines.

UNLABELLED: To evaluate the role of inherited thrombophilia in the development of central venous line (CVL)-related thrombosis, the following parameters were determined in 77 pediatric-oncologic patients with CVL: activated protein C (APC)-ratio, factor V (FV) G1691A and prothrombin G20210A mutation, protein C, protein S, antithrombin, coagulation factor XII, lipoprotein (a) and homocysteine. An inherited prothrombotic risk factor was found in 17 patients (23%). Four out of 14 patients with a single detect (hyperlipoproteinemia, heterozygous FV G1691A and prothrombin G20210A mutation, protein C deficiency type I) and all three patients with combined defects (heterozygous FV G1691A mutation combined with heterozygous prothrombin G20210A variant, protein S deficiency or hyperlipoproteinemia) suffered from CVL-related thrombosis. In 11 out of 77 patients (14%) a CVL-related thrombosis was detected. In 2 children thrombosis occurred a few days after asparaginase therapy and in another three thrombosis was associated with CVL-related septicemia caused by Staphylococcus epidermidis. After removal of CVL, thrombosis was detected in 5 children, in 2 without clinical symptoms but in the presence of inherited prothrombotic risk factors. CONCLUSION: The present study demonstrates the clinical importance of CVL in combination with inherited thrombophilia in the development of thrombosis in pediatric-oncologic patients. Before or shortly after insertion of CVL, patients should be tested for the presence of factor V G1691A mutation, prothrombin G20210A variant and increased lipoprotein (a) values.

[Lethal meningeal encephalitis from Cryptococcus neoformans var. neoformans in a girl without serious immunodeficiency]. / Tödliche Meningoenzephalitis durch Cryptococcus neoformans var. neoformans bei einem Mädchen ohne schwerwiegende Immundefekte.

Case report on a lethal meningo-encephalitis due to Cryptococcus neoformans in a 14-year-old girl without serious immunodeficiency inclusive HIV-infection. The detection of high quantities of cells of Cryptococcus neoformans (about 10,000/ml) and high levels of Cryptococcus antigen (up to 1:2048) in the cerebrospinal fluid are remarkable. The patient was treated with a triple combination of amphotericin B, flucytosine and fluconazole. After 18 days the cerebrospinal fluid was sterile. Nevertheless considerable lesions of the brain arised. The patient died from the Cryptococcus infection on day 74 of the antimycotic therapy. Cryptococcosis should be included into the differential diagnosis of the chronic lymphocytic pleocytosis of the cerebrospinal fluid connected with symptoms of intracranial pressure and ocular symptoms.

[The acrocallosal syndrome. Report of an additional case]. / Das akrocallosale Syndrom. Bericht über einen weiteren Fall.

The acrocallosal syndrome is a rare congenital malformation syndrome with the main findings: agenesis of the corpus callosum, craniofacial dysmorphisms, mental retardation, polydactyly of the hands and/or feet. We report a patient with partial agenesis of the corpus callosum, dolichocephalus, broad and short nose, low-set and posteriorly rotated ears, pre- and postaxial polydactyly of feet, duplication with syndactyly of big toes, and postaxial hexadactyly of the left hand. Differential diagnosis and the genetic counseling are discussed.

[Vein of Galen malformation in a newborn infant with clinical symptoms of a cardiologic emergency]. / Vena-Galeni-Malformation bei einem Neugeborenen mit der klinischen Symptomatik eines kardiologischen Notfalls.

We report on the management of one term newborn infant with a malformation of the Vena Galeni. The baby suffered from cyanosis and intractable congestive heart failure without evidence of congenital heart malformation at his second day of life. The diagnosis was established by two-dimensional sonography and Doppler examination. However, we thought it necessary additionally to perform an angiography of the intracerebral vessels before surgical intervention. We conclude that a real time two-dimensional imaging system and a range-gated pulsed Doppler velocimeter should be available in newborn intensive care units. Otherwise neonatologists are forced to transport these infants to pediatric heart centers because of inability to detect such an extracardiac origin of heart and circulatory failure.

[Pneumothorax and subvesical urinary tract obstruction in newborn infants--a pathogenically related symptom complex]. / Pneumothorax und subvesikale Harnwegsobstruktion beim Neugeborenen--ein ursächlich im Zusammenhang stehender Symptomenkomplex.

Within a period of 3 years 4 mature male neonates were observed with an unusual complex of symptoms: subvesical obstruction (valve) with spontaneous pneumothorax/pneumomediastinum. All infants showed immediately postnatal respiratory insufficiency. There are pathogenetical relations between renal and urinary tract malformations and pulmonary changes, which are recognized in Potter's sequence with pulmonary hypoplasia. The authors postulate a deranged dynamic of pulmonary fluid by oligohydramnios, causing increased vulnerable lungs also in such cases without typical x-ray signs of pulmonary hypoplasia. A spontaneous pneumothorax/pneumomediastinum in newborn infants should be the sign to perform nephro-urologic (sonographic) examination.

[Milk proteins for food. I. Some aspects of the use of milk proteins for the protein supply to the population of the GDR]. / Milcheiweiss für Nahrungszwecke. 1. Mitt. Einige Aspekte des Einsatzes von Milchproteinen bei der Eiweissversorgung der Beväolkerung in der DDR.

Milk protein accounts for approximately 21% of the protein supply to the population in the GDR. All milk protein fractions have a high nutritional-physiological value and favourable functional properties. This notwithstanding, only 50% of the milk protein processed by the dairy industry are at present employed in human nutrition, the remainder is used as a provender. The reasons for this situation are discussed. Principles of procedures for the industrial production of milk protein concentrates are presented.

[Mass transfer in production of milk protein coprecipitates]. / Stoffübergangsbilanzen bei der Herstellung von Milcheiweisscopräzipitaten

During the last twenty years heat coagulation has become a more important technical procedure for the isolation of milk proteins because of its very efficient utilization of raw material. Experiments are reported to get approximation equations for the mass transfer in the production of milk protein coprecipitates from skim milk and mixtures of skim milk with rennet and acid whey by means of statistical planning and interpreting of experiments. Good exactness was reached. The coagula consist of casein fractions, whey protein fractions, calcium, and phosphates. The products are thermically rather stable, sensorically indifferent and contain water insoluble protein. They are suitable to improve the fat: protein ration of sausages, but also for the protein enrichment of other foods and for dietetic purposes.