M. Ries

Paravertebral and intraspinal malposition of transfemoral central venous catheters in newborns.

We report permanent tetraplegia in a newborn resulting from intraspinal malposition of a transfemoral catheter. In 2 other neonates paravertebral malposition of indwelling Silastic lines was detected. We suggest that left-sided transfemoral catheterization and conditions enhancing collateral flow through the vertebrolumbar pathway may predispose to inadvertent paravertebral catheter placement.

Intestinal perforations in a premature infant caused by Bacillus cereus.

Abstract.Although Bacillus cereus is a ubiquitous bacterium, the incidence of neonatal infections is very low with only a few cases of B. cereus infections in neonates reported in the literature. We report the case of a premature infant with multiple intestinal perforations and an abdominal B. cereus infection. The initial course was characterized by severe cardiovascular shock, anemia, thrombocytopenia and disseminated intravascular coagulation, leading to periventricular leukomalacia, alopecia capitis and toxic epidermal necrolysis. The possible role of B. cereus-associated enterotoxins for the clinical manifestations are discussed. Our case confirms previous reports of severe clinical symptoms in B. cereus infection in premature neonates. We speculate that the systemic complications of B. cereus infection are at least partly related to the effect of B. cereus-associated enterotoxins.

In Vitro Fibrinolysis After Adding Low Doses of Plasminogen Activators and Plasmin Generation with and without Oxidative Inactivation of Plasmin Inhibitors in Newborns and Adults

Purpose The aim of this study was to investigate in vitro fibrinolysis after adding low doses of plasminogen activators and to determine the functional role of plasmin inhibitors in newborns and adults. Patients and Methods We have studied the kinetics of in vitro fibrinolysis after adding low doses of urokinase (UK) and recombinant tissue plasminogen activator (rt-PA) by use of a microtiter clot lysis assay. Additionally, we have determined plasmin generation with and without oxidative inactivation of plasmin inhibitors in newborns and adults. Results The 50% lysis time in the clot lysis assay correlated with the activator dose and was significantly shorter in newborns at rt-PA concentrations of <0.2l μg/ml. When UK was used as an activator, the 50% lysis time was slightly but significantly prolonged in newborns at concentrations of 140–200 lU/ml, whereas we could find lower values (nonsignificant) at 110 and 80 lU/ml. Plasmin generation after oxidative inactivation of plasmin inhibitors was significantly lower in newborns, even when compared with adult plasma, which was diluted 50%. However, in a physiological plasma milieu (containing natural inhibitors), there were no differences in plasmin generation when streptokinase (SK) was used as an activator and only minor differences when UK was used. Conclusions Our data indicate a more rapid clot lysis at low UK and rt-PA concentrations in newborns despite significantly reduced plasminogen levels. The results of the plasmin generation experiments suggest a diminished effect of plasmin inhibitors towards fetal plasmin. which raises an explanation for the concentration-related differences in the clot lysis assay. The experience with thrombolytic agents in newborns is limited. Most dosage regimens for thrombolytic therapy in children or adults consist of an initial bolus infusion, followed by low-dose continuous treatment. Based on the results of our clot lysis experiments, we think that especially the continuous infusion of plasminogen activators after bolus administration should not be enhanced in newborns compared to older children or adults.

AGE-RELATED REFERENCE VALUES FOR ACTIVATION MARKERS OF THE COAGULATION AND FIBRINOLYTIC SYSTEMS IN CHILDREN

The physiology of the hemostatic system in infancy and childhood is different from that in adulthood. Despite differences in several components of the coagulation and fibrinolytic systems, there is no evidence that children are at greater risk for hemorrhagic or thrombotic problems compared with adults (1,2). Advances in understanding of the biochemistry of the hemostatic mechanism have led to the development of sensitive immunochemical methods for measuring peptides or enzyme-inhibitor complexes that are liberated with the activation of the coagulation and fibrinolytic systems in vivo (3). Activation markers of coagulation and fibrinolysis have been measured in newborns, infants and children with a variety of underlying disorders (4-16). However, reference ranges for children of different age groups have hitherto not been established. The aim of the present study was to document thrombin-antithrombin III-complex (TAT), prothrombin fragment 1 + 2 (F1 + 2), plasmin-alpha 2-antiplasmin-complex (PAP) and D-dimer in healthy children and to determine whether age-related differences can be demonstrated.

Age-related differences in a clot lysis assay after adding different plasminogen activators in a plasma milieu in vitro

Purpose The fibrinolytic system is involved in a wide variety of biological phenomena and differs physiologically in newborns compared to older children or adults. Because the new born has hypoplasminogenemia and a possible existence of a dysfunctional plasminogen with normal adult levels of plasminogen activator inhibitor type 1 and α2-antiplasmin and elevated levels of plasminogen activator inhibitor type 2, it could be expected that the response to standard concentrations and doses of plasmonogen activators would be reduced. Patients and Methods We have studied the Kinetics of in vitro fibrinolysis after adding different concentrations of streptokinase(SK), urokinase (UK), and recombinant tissue plasminogen activator(rt-PA) by use of a microtiter clot lysis assay. Results Geometrical dilution rows showed characteristic dose response curves. After clot formation, a rapid lysis was seen with all plasminogen activators. The 50% lysis time correlated to the plasminogen activator dose and showed no diferences among normal adults, children aged 1–6 years, and children aged 7–14 years. Newborns demonstrated a significantly prolonged 50% lysis time with all urokinase concentrations. The 50% lysis time with recombinant tissue plasminogen activator and streptokinase was significantly prolonged only at high concentrations, whereas we could not see any differences at lower concentrations. Conclusion The experience with thrombolytic agents in newborns is limited, and no controlled investigations have been reported. Our results of the fibrinolytic potential in a plasma milieu in vitro after adding different plasminogen activators can be helpful to establish dosage guidelines for thrombolytic therapy in newborns and older children.

Brain abscesses in neonates — report of three cases

We report three newborns with brain abscesses. Two infants suffered fromSerratia marcescens meningitis and one infant had enterococcal sepsis and meningitis. Brain abscesses were detected by cerebral sonography. Outcome in one infant withS. marcescens infection was poor. This patient developed multicystic encephalomalacia and severe developmental retardation. In the other patient withS. marcescens infection surgical drainage of the abscess was performed. The outcome was good both in this infant and in the patient with enterococcal brain abscess.

Demonstration of perivascular echogenicities in congenital cytomegalovirus infection by colour Doppler imaging

Two children with congenital cytomegalovirus infection and intracerebral echogenicities were investigated by computer sonography and colour Doppler imaging (CDI). By simultaneous imaging of brain tissue and CDI, blood flow within the stripe-like echogenicities of the basal ganglia was demonstrated. Using CDI the echogenicities were identified as the walls of thalamostriate vessels.

Polyposis of the gallbladder associated with metachromatic leukodystrophy

We report on two children with metachromatic leukodystrophy and polyposis of the gallbladder. In both patients ultrasound examination revealed a small gallbladder with a thickened echogenic wall and multiple polypoid masses. In one patient diagnosis of gallbladder polyposis was made 6 months before the first neurological symptoms occurred. As gallbladder polyposis is a rare phenomenon in childhood, metachromatic leukodystrophy should be excluded.

Colour Doppler imaging of intracranial vasculopathy in severe infantile sialidosis

Neonatal ascites is usually attributed to prenatal infections, lysosomal storage disease and anomalies of the genitourinary tract, gastrointestinal tract or cardiovascular system. We report one case of neonatal ascites associated with infantile sialidosis. Cerebral sonography showed stripe-like intracerebral echogenicities in the region of the basal ganglia. Colour Doppler imaging demonstrated blood flow within the echogenicities confirming the suspected diagnosis of intracranial vasculopathy.

Congenital intracerebral teratoma : a rare differential diagnosis in newborn hydrocephalus

Abstract Congenital hydrocephalus is caused by a broad spectrum of underlying disorders. In the majority of cases it is due to aqueductal stenosis and other distinct congenital anomalies, like Arnold-Chiari malformation. Nevertheless, in the differential diagnosis rare conditions such as cerebral malignancies must also be considered. We present two cases of congenital intracerebral teratoma as a differential diagnosis in congenital obstructive hydrocephalus. A teratoma is suggested when a rapidly growing hydrocephalus with a central calcified and vascularized mass is found sonographically. Regular cerebral structures usually cannot be detected. Early diagnosis in such cases is of clinical importance as the prognosis of congenital intracerebral teratoma is generally very poor.