Severe respiratory distress syndrome unresponsive to intensive care treatment--diagnostic and therapeutic considerations.

Respiratory Distress Syndrome (RDS) is a common complication in preterm neonates. If RDS is not responding to conventional treatment modalities (surfactant therapy, ventilatory support, etc.), an underlying pathology (pulmonary lymphangiectasia, capillary alveolar dysplasia, alpha-1 antitrypsin deficiency, etc.) other then prematurity should be taken into consideration.Here, we report on a preterm neonate with the unusual simultaneous occurrence of pulmonary and systemic lymphangiectasia and homozygous alpha-1 antitrypsin deficiency who developed severe RDS that was refractory to conventional treatment. The diagnostic and therapeutic approach in this patient is presented.

Severe valproic acid intoxication is associated with atrial tachycardia: secondary detoxication by hemoperfusion.

Valproic acid is an anticonvulsant drug which is associated with serious toxicity including fatal outcome in case of severe intoxication. Secondary detoxication by hemodialysis or hemoperfusion has been employed successfully in valproic acid intoxication. Cardiac arrhythmias have only been described rarely in valproic acid intoxication in humans. We report on a 15 year-old boy with severe valproic acid intoxication (valproic acid plasma level on admission: 1 150 mg/l) who presented with coma, hypernatremia and atrial tachycardia. The patient was successfully treated with hemoperfusion and intensive supportive care without implementation of a specific antiarrhythmic therapy. We conclude that patients with severe valproic acid intoxication may benefit from secondary detoxication. In addition to generally known symptoms valproic acid intoxication may also be associated with cardiac arrhythmias.

[Arterial tortuosity syndrome]. / Das Arterial-Tortuosity-Syndrom.

The arterial tortuosity syndrome is a rare congenital disorder characterized by elongation and generalized tortuosity of the major arteries including the aorta. Associated clinical features consist of excessively stretchable skin and joint laxity which is indicative of a connective tissue disorder such as Ehlers-Danlos or Cutis laxa syndrome. The gene locus of the arterial tortuosity syndrome has recently been localised on chromosome 20q13; inheritance ist autosomal recessive. - We report on a newborn with arterial tortuosity syndrome and hiatal hernia, bilateral hip dislocation, inguinal hernias and diffuse tortuosity of the great arteries including the aorta. Known gene loci involved in Ehlers-Danlos syndrome, cutis laxa syndrome and other connective tissue disorders were excluded by specific DNA markers. By homozygosity mapping with polymorphic microsatellite markers it was possible to confirm the gene locus for the ATS on chromosome 20q13. In addition to the presentation of this patient, a review of the literature is presented.

[Reverse flow in fetal vessels and perinatal events]. / Reverse Flow in den fetalen Gefässen - Klinische Bedeutung und perinatale Auffälligkeiten.

UNLABELLED: BACKGROUND/PATIENTS: A reverse flow in the umbilical artery and/or fetal aorta is associated with a higher perinatal and neonatal mortality. 30 fetuses showed a reverse flow using pulsed wave Doppler sonography (group I). A matched-pair control group including 30 fetuses with the same gestational age as well as a normal Doppler flow pattern in the umbilical artery and/or fetal aorta was taken for comparison (group II). RESULTS: In the group with reverse flow the rates of pregnancies with pre-eclampsia (n = 19/30, p < 0.0001), intrauterine growth retardation (n = 25/30, p < 0.0001), oligohydramnios (n = 21/30, p < 0.0001) and nicotine abuse (n = 15/30, p < 0.01) were significantly higher compared to the control group. Postnatal data showed significantly lower pH values in group I (p < 0.01). 40 % of the fetuses with reverse flow died in utero whereas in 67 % the reverse flow was accompanied by an insufficiency of the placenta (IUGR, oligohydramnios, histopathological abnormalities of the placenta). None of the fetuses in the control group died in utero. The incidence of IUGR (< 5ht percentile) was 83 % in group I but only 3 % in group II. The perinatal and overall mortality (including neonatal mortality 7 - 28 days after birth) amounted to 27 % and 53 % in group I, respectively, compared to 3 % and 0 % in the control group (p < 0.001). In addition cerebral anomalies could be found by ultrasound in 50 % of the neonates who presented a reverse flow prenatally. In 28 % of the surviving newborns an intracerebral hemorrhage (ICH) could be detected. None of the newborns of group II developed an ICH. CONCLUSIONS: Pregnancies with a reverse flow in the umbilical artery and/or fetal aorta have to be considered as a high risk group with a poor prognosis. The reverse flow is mainly caused by chronic placental insufficiency with IUGR. With respect to the further neuromotor development the incidence and severity of cerebral lesions in affected fetuses should be considered when discussing the perinatal situation with the parents.

[Ventricular tachycardia after erythromycin administration in a newborn with congenital AV-block]. / Ventrikuläre Tachykardie nach Erythromycin-Gabe bei einem Neugeborenen mit angeborenem AV-Block.

UNLABELLED: This report is on a newborn with congenital complete av-block due to a maternal collagenosis. The intravenous application of erythromycin produced premature ventricular beats and non sustained ventricular tachycardias by prolongation of the QT interval. After discontinuation of the erythromycin application, the QT-interval normalised. CONCLUSION: In atrioventricular conduction disorders with severe bradycardia and prolongation of the QT interval, the application of erythromycin--if unavoidable--should be managed by slow intravenous infusion and with permanent ECG monitoring.

Dose reduction in thorax radiography in simulated neonates with additional filtration and digital luminescence radiography.

PURPOSE: To determine the minimum acceptable radiation dose for an adequate image quality in thorax a.p. radiographs of neonates using mobile X-ray equipment. MATERIAL AND METHODS: The influence of additional filtration (1.0 mm Al + 0.1 mm Cu) on image quality and radiation dose was determined for the speed class 400 screen-film system (SFS) and digital luminescence radiography (DLR) by making radiographs of a test phantom. Conventional and digital thorax a.p. radiographs of a rabbit were produced using various tube current-time products. The quality of the rabbit radiographs was judged by eight radiologists applying image quality criteria according to the German guidelines and the recommendations of the European Community. RESULTS: The added filter resulted in a dose reduction of 39% at 66 kV. DLR gave a further dose reduction of 25% in comparison to the speed class 400 SFS while maintaining adequate image quality, i.e. the radiographs were clinically acceptable with regard to quality criteria. CONCLUSION: The radiation dose resulting from thorax a.p. radiographs of neonates can be reduced by approximately 50% with the use of additional filtration and DLR.

The influence of gastroschisis on plasmatic coagulation, humoral immunity, and C-reactive protein.

Around the 30th week of gestation, patients with gastroschisis (GS) develop chronic inflammatory reactions on the serosal surface of the eventrated loops of intestine that lead to severe hyperfibrinogenemia and hypercoagulability, particularly if they are born prematurely. This result was found on comparison of coagulation studies from 12 patients with GS with those of 4 patients with omphalocele and of healthy premature babies and term infants. Furthermore, the patients with GS showed marked hypogammaglobulinemia, which might also be responsible for the increased numbers of infections complicating the course of this disease. The hypercoagulability increases the risk of thromboembolic complications and disseminated intravascular coagulation, particularly in combination with the surgical closure of the abdominal wall that is undertaken postnatally or with perinatal complications such as asphyxia. Prophylactic therapy with low-dose heparin is indicated.

[Congenital tuberculosis in a premature infant]. / Beitrag zur angeborenen Tuberkulose eines Frühgeborenen.

5 days after birth a premature baby of 1,300 g (31st week of gestation) was examined, because a congenital tuberculosis was suspected. In the stomach secretion mycobacterium tuberculosis could in fact be found. Abdominal symptoms as well as a post-natal anemia could be lead back to the infection. For three months a double parenteral therapy with INH (10 mg/kg/d) and Rifampicin (10 mg/kg/d) was carried through, then a mono therapy with INH for another 6 months. The bacillus had become resistant to streptomycin. At the beginning of the chemotherapy a cholestasis developed. The tuberculosis could be cured.

Plasmapheresis in newborns with hyperbilirubinemia.

With a miniaturized plasmapheresis system consisting of a double head pump and balancing pump, extracorporeal detoxification treatments such as plasmapheresis, hemofiltration, and hemodialysis were carried out. In total 15 premature infants and newborns with Rherythroblastosis and hyperbilirubinemia were treated using this miniaturized system. The average birth weight of the 15 patients was 2,386 g. With a main blood flow of 5.3 ml/min and a filtrate flow of 1.3 ml/min, an average of 192.4 ml of plasma per treatment was exchanged. In all patients 1 to 10 plasmapheresis treatments were necessary to reduce the mean serum bilirubin from 15.6 +/- 4.8 to 7.3 +/- 3.2 mg/dl, and after several days the serum bilirubin subsequently normalized in 12 patients, who improved. Three patients died 5 to 18 days after birth as a result of their primary disease and immaturity. For vascular access small catheters were inserted in the umbilical or the femoral vein. All treatments were well tolerated by the patients.

[Mediastinal cystic lymphangioma as a cause of hydrops fetalis]. / Mediastinales zystisches Lymphangiom als Ursache eines Hydrops fetalis.

This report is upon a case with fetal hydrops on the base of a paracardiac cystic lymphangioma in the mediastinum. The newborn which had hypoplastic lungs and multiple pneumatothoraces died because of a pulmonary insufficiency. An overview is given about the most common causes of the nonimmune fetal hydrops, and the pathomechanisms are discussed.

[Kawasaki syndrome in infants in the first 6 months of life]. / Das Kawasaki-Syndrom bei Säuglingen im ersten Lebenshalbjahr.

Diagnosis in Kawasaki disease usually is made on the so called main symptoms and on laboratory findings. The analysis of three cases in the first six months of life demonstrated that the typical symptoms concerning the mucosa and the lymphatic nodes are rare in this age group, whereas drug resistant fever and polymorphic exanthema was present in all patients. Besides that accessory symptoms like abacterial meningitis and pathologic urine analysis seemed to be frequent signs. Laboratory findings such as pathologic blood sedimentation rate, elevated CRP and thrombocytosis on the other hand were helpful in making the diagnosis.

Hemofiltration and plasmapheresis in newborns using a small blood pump system.

Extracorporeal detoxification methods in newborns and premature infants are unavailable and contraindicated because of technical problems. The authors' experiences with a blood pump unit that was miniaturized for hemodialysis, hemofiltration, and plasmapheresis in newborns are reported. In 28 newborns and premature infants, 32 hemofiltrations, 3 hemodialyses, and 57 plasmaphereses were carried out for different diseases. Thirteen of 28 patients died despite hemofiltration and/or plasmapheresis as a result of their primary disease and/or immaturity, and 15 patients improved.

Hemofiltration and plasmapheresis in premature infants and newborns.

Extracorporal detoxication methods in newborns are most unavailable, contraindicated because of technical problems. Herein are reported the experiences with a blood pump unit that was miniaturized for hemodialysis, hemofiltration, and plasmapheresis in newborns. In three premature infants with acute renal failure of different etiology (two newborns with severe Rh-erythroblastosis, 1 premature infant with hypoproteinemia) 13 single-needle hemofiltrations and 6 single-needle plasmaphereses were performed with double head pump, special tube systems, and small modules. The age of the patients ranged from 1 to 14 days, body weight was between 800 and 2,800 g. Four umbilical veins and two femoral veins were used as vascular access. The ultrafiltration rate during the treatment averaged 0.3 ml/min in single-needle hemofiltration and the plasma filtrate flow rate 1.3 ml/min in single-needle plasmapheresis. All treatments were well tolerated. Four patients died due to complications unrelated to the treatment, two patients recovered. These preliminary results show that both hemofiltration and plasmapheresis may be carried out without major problems in premature infants and newborns.

[Single-needle diafiltration and single-needle plasmapheresis in premature infants]. / Single-needle-Diafiltration und Single-needle-Plasmapherese bei Frühgeborenen.

Extracorporal detoxication methods in newborns are most unavailable, contraindicated because of technical problems. We report our experiences with a blood pump unit developed by our-selves for diafiltration and plasmapheresis in newborns. In 6 newborns with acute renal failure, Rh-ery-throblastosis and hypoproteinemia, 13 single-needle-diafiltrations and 6 single-needle-plasmapheresis were performed with the self-constructed double head pump, special tube systems and small models. Our results show that all extracorporal detoxication methods can be carried without problems.