Pyoderma gangraenosum, a rare, but potentially fatal complication in paediatric oncology patients.

Pyoderma gangraenosum (PG) is a serious chronic, ulcerative skin disorder afflicting both adults and children. As PG is often associated with systemic diseases (>50%) such as inflammatory bowel disease, rheumatoid arthritis or haematological disorders, it requires a multidisciplinary approach. This disorder is not commonly reported in paediatrics; therefore children with PG represent a particularly difficult diagnostic challenge.

Drotrecogin alpha (activated) in neonatal septic shock.

A 12-d-old neonate suffering from group B streptococcal septic shock was treated with 24 microg/kg/h recombinant human activated protein C [rhAPC, drotrecogin alpha (activated)] for 96 h. The protein C activity increased from 5% to 53% after rhAPC infusion. The patient recovered within 14 d without any adverse effects.

Hepatic failure with neonatal tissue siderosis of hemochromatotic type in an infant presenting with meconium ileus. Case report and differential diagnosis of the perinatal iron storage disorders.

We report on a female preterm infant with hepatic failure and neonatal tissue siderosis of hemochromatotic type diagnosed by using both histochemistry and atomic absorption spectroscopy. The infant presented with meconium ileus, signs of rapidly progressive hepatic failure, and hyperferritinemia (7132 ng/ml). Despite surgery and intensive care the infant died 32 days after birth.

Vascular tracheobronchial compression syndromes-- experience in surgical treatment and literature review.

Between January 1988 and December 1997 a total of 22 patients (age: 8 days-46 years) were operated for vascular airway compression syndromes with respiratory insufficiency. Vascular anomalies in tracheal compression were double aortic arch in 7 patients, (2 previously operated elsewhere), right aortic arch + left ligamentum arteriosum in 1, and pulmonary artery sling in 3. Three of these patients had secondary long-segment tracheomalacia.

Clinical symptoms, biochemical studies and therapeutic approaches in a sibship with a new congenital tubulopathy.

Unlabelled: We report on two siblings suffering from a new congenital tubulopathy. Following normal pregnancies not complicated by polyhydramnios, severe renal losses of potassium, chloride, sodium and magnesium occurred in the first weeks after birth. Calcium metabolism was not affected.

Impaired deformability of erythrocytes and neutrophils in children with newly diagnosed insulin-dependent diabetes mellitus.

Aims/hypothesis: Abnormal rheological properties of erythrocytes, leucocytes and plasma may have a role in the development of diabetic microangiopathy. We hypothesized that changed haemorrheological variables may already be found in children with onset diabetes.

Methods: Erythrocyte deformation (rheoscope), neutrophil deformation (micropipette), erythrocyte aggregation, blood and plasma viscosity were measured in 15 children with insulin-dependent diabetes mellitus before initiation of insulin treatment and 4 to 6 weeks later, 15 diabetic children treated with insulin for 5 to 8 years, 15 healthy children and 15 healthy adults.

Refractory congenital ascites as a manifestation of neonatal sialidosis: clinical, biochemical and morphological studies in a newborn Syrian male infant.

A Syrian newborn with coarse facies, hepato-splenomegaly, and refractory ascites is reported. Examination of the ascitic fluid showed vacuolated lymphocytes and thin-layer chromatography of urinary oligosaccharides revealed an abnormal pattern indicative of sialidosis. Despite intensive care, the baby died of respiratory insufficiency 28 days after birth.

Wolf-Hirschhorn syndrome: case report and review of the chromosomal aberrations associated with diaphragmatic defects.

A female fetus showing severe growth retardation was delivered at 31 weeks of gestation because of fetal distress. At birth, the infant showed bradycardia and no spontaneous breathing. Although high frequency oscillatory ventilation was started, severe asphyxia persisted and the infant died of respiratory insufficiency.

Meconium aspiration syndrome complicated by massive intravascular thrombosis.

Fetal aspiration of meconium in amniotic fluid during fetal distress by newborn infants can induce the meconium aspiration syndrome (MAS), a form of neonatal respiratory distress. Should this event occur, admission to a Neonatal Intensive Care Unit and vigorous airway management and monitoring are required. We present a term gestation resulting in MAS complicated by a massive intravascular thrombosis.

External stabilization of long-segment tracheobronchomalacia guided by intraoperative bronchoscopy.

Background: Symptomatic obstruction of long-segment tracheal or bronchial portions either related to congenital instability or secondary to vascular compression are rare malformations, which remain difficult to manage. A method of external tracheal or bronchial stabilization is described.

Methods: From July 1992 to April 1995, 7 children (age range, 4 months to 4 years; mean age, 19 months) and 1 adult (age, 46 years) were operated on for severe respiratory insufficiency.

Effects of high-frequency oscillatory ventilation on circulation in neonates with pulmonary interstitial emphysema or RDS.

Objective: Mechanical ventilation may impair cardiovascular function if the transpulmonary pressure rises. Studies on the effects of high-frequency oscillatory ventilation (HFOV) on cardiovascular functions have yielded conflicting results. This study was done to compare alterations in left ventricular output and blood flow velocities in the anterior cerebral artery, internal carotid artery, and celiac artery using a Doppler ultrasound device before and 2 h after initiating HFOV in neonates with respiratory distress syndrome (RDS) or pulmonary interstitial emphysema (PIE).

Alternative pathway activation of the complement system in preterm infants with early onset infection.

The increased incidence of infection in preterm neonates has been related in part to their relative deficiency of most complement components, because complement is known to participate in the defense against bacterial and viral infections. In a prospective study, complement activation products were determined in 52 preterm infants. Twenty preterm infants suffered from proven early onset infection, 11 infants were presumed to suffer from infection, which could not be confirmed.

[High frequency jet ventilation during tracheal resection in children and infants].

Between 1986 and 1996, 16 infants and children less than 11 years of age (m = 11, f = 5) underwent resections for acquired or congenital tracheobronchial stenoses. During this period, various techniques of total intravenous anaesthesia (TIVA) were employed (midazolam, fentanyl, pancuronium; propofol, fentanyl, pancuronium). During the phase of dividing the airways, high-frequency-jet ventilation (HFJV) into the trachea or the main bronchi by 8-12Fr catheter(s) was applied for 10-75 min with driving pressures between 0.

[Bronchopulmonary dysplasia. Retrospective analysis of various forms of treatment and development of a staged therapeutic plan].

50 premature infants with bronchopulmonary dysplasia (BPD) were treated in the Perinatal Center of the University of Heidelberg from January 1990 to December 1992. Gestational age was 24-31 weeks and birthweight was 500 to 1430 grams. 27 infants received dexamethasone only and 14 were initially given dexamethasone followed by beclomethasone inhalation.

[Rheologic changes in stored erythrocyte concentrates].

We studied the effect of two different preservation solutions on mean corpuscular volume (MCV), red cell deformability and flow in narrow tubes in red blood cell concentrates. Blood from 10 healthy blood donors was processed in parallel in SAG-M (S-RBC) as well as in PAGGS-M (P-RBC) in identical aliquots. Samples were studied at days 0, 7, 14, 28 and 42 of storage.

Effect of Leboyer childbirth on cardiac output, cerebral and gastrointestinal blood flow velocities in full-term neonates.

The Leboyer birth method requires that the newly born infant is placed on the mother's abdomen and the cord is clamped when it stops pulsating. This investigation was done to study the effect of Leboyer childbirth on neonatal circulation during the first 5 days after birth. Hematocrit, blood viscosity, left and right ventricular output, and cerebral blood flow velocities in the arteria carotis interna, arteria cerebri anterior, and truncus coeliacus were studied in 15 full-term neonates with early (less than 10 seconds) cord clamping and 15 full-term neonates delivered according to Leboyer (cord clamping after 3 minutes) on day 1 (2 to 4 hours after birth) and day 5.

Effects of red cell transfusion on cardiac output and blood flow velocities in cerebral and gastrointestinal arteries in premature infants.

Anaemia may increase the risk of tissue hypoxia in preterm infants. The effect of transfusion on circulation was studied in 33 preterm infants with a mean (SD) gestational age of 29 (5) weeks (range 26-34), birth weight 1153 (390) g (range 520-1840), and postnatal age of 48 (21) days (range 19-100). Packed cell volume, blood viscosity (capillary viscometer), cardiac output, and cerebral blood flow velocities in the internal carotid artery, anterior cerebral artery, and coeliac trunk (Doppler ultrasound) were determined before and after transfusion of 10 ml/kg of packed red blood cells.

Plasmapheresis in severe septic shock with disseminated intravascular coagulation.

An 18-year-old female with CNS relapse of acute lymphoblastic leukemia after previous complete remission of the disease underwent chemotherapy. Due to the therapy she suffered from profound suppression of bone marrow with consecutive thrombocytopenia and leukopenia. Despite prophylactic treatment, severe septicemia occurred with septic shock, hemolysis and disseminated intravascular coagulation (DIC).

Complement activation in newborn infants with early onset infection.

The complement system is an important element in host defense. Quantitative deficiencies of total hemolytic complement activity and decreased C3 levels were reported in sera from normal neonates. However, little is known about complement activation products in the newborn.

The effect of Leboyer delivery on blood viscosity and other hemorheologic parameters in term neonates.

Objective: This study was done to compare postnatal alterations in blood viscosity, hematocrit value, plasma viscosity, red blood cell aggregation, and red blood cell deformability in term neonates undergoing both early umbilical cord clamping and delivery according to the Leboyer method.

Study Design: The umbilical cords of 15 healthy, term infants were clamped within 10 seconds of birth (early cord clamping), and 15 infants delivered according to the Leboyer method were placed on the mother's abdomen, and the umbilical cords were clamped 3 minutes after birth. Hemorheologic parameters were studied in umbilical cord blood at 2 hours, 24 hours, and 5 days from the time of delivery.

The effect of early and late cord-clamping on blood viscosity and other hemorheological parameters in full-term neonates.

This study was done to compare postnatal alterations in blood viscosity (capillary viscometer) and its determinants: hematocrit, plasma viscosity (capillary viscometer), red cell aggregation (Myrenne aggregometer) and red cell deformability (rheoscope) in the first five days of postnatal life in full-term neonates with early (< 10 s) and late (3 min) cord-clamping. The fetal blood volume of the placenta ("residual placental blood volume") decreased from 52 +/- 8 ml/kg of neonatal body weight after early cord-clamping to 15 +/- 4 ml/kg after later cord-clamping. Neonatal blood volume, calculated as the difference between an assumed total feto-placental blood volume of 115 ml/kg and the measured fetal blood volume of the placenta, was 50% higher in the late cord-clamped infants than in the early cord-clamped infants.

Blood viscosity and optimal hematocrit in preterm and full-term neonates in 50- to 500-micrometer tubes.

Blood viscosity is an important determinant of blood flow resistance. Because a substantial part of flow resistance arises in small arteries and arterioles with diameters of 100 microns and less, rheologic properties of blood from preterm infants (24 to 36 wk of gestation), full-term neonates, and adults were measured in glass tubes with diameters of 50, 100, and 500 microns for a wide range of adjusted feed hematocrits (0.15-0.

[The critical hemoglobin value in newborn infants, infants and children].

The optimum and critical hemoglobin concentrations are determined by the oxygen demand of the tissues and several oxygen transport parameters (i.e., blood flow, arterial oxygen saturation, oxygen affinity of hemoglobin, and the critical venous oxygen pressure).