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  • 1
    ISSN: 1432-1076
    Keywords: Key words: Kallmann syndrome – X-recessive inheritance – Hypoplastic olfactory sulci – Rhinencephalon – MR-imaging
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. A 3-month-old infant presented with hypogonadism, a small penis and bilateral cryptorchidism. He showed an insufficient response of luteotropic hormone (LH) and follicle stimulating hormone (FSH) to luteotropic hormone releasing hormone (LHRH) and of testosterone to human chorionic gonadotrophin. The maternal uncle had hypogonadism and anosmia and also showed an impaired LH and FSH response to LHRH. MRI examination showed hypoplasia of the rhinencephalon in both cases. These findings in the son and brother of the clinically unaffected mother suggest X-linked recessive inheritance.
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  • 2
    ISSN: 1432-1076
    Keywords: Indomethacin ; Patent ductus arteriosus ; Pharmacokinetics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We present pharmacokinetic data of prolonged, intra-arterial indomethacin treatment (i.e. induction plus maintenance dose) for symptomatic patent ductus arteriosus (sPDA) in 26 ventilated premature infants. sPDA was assessed by two-dimensional and pulsed Doppler echocardiography. Permanent ductal closure occurred in 20 (76%) infants. Plasma levels of indomethacin were 1.18±0.74; 1.8±1.0; 1.51±0.93 and 1.25±0.98 μg/ml (mean±SD) at 12, 24, 48 and 72 h after initial dose administration. All except one patient who responded with ductal closure, showed plasma levels above 0.25 μg/ml throughout the 3 day treatment period and no case of sPDA reopening was noted. Although target concentrations over time were not defined, the data indicate that the maintenance levels measured were within the therapeutic range. A negative correlation was found for plasma drug levels and postnatal age (r=0.52;P〈0.01). Volume of drug distribution was 0.23±0.18l/kg, total clearance 0.1±0.11 ml/min and elimination constant 0.06±0.05h−1 (mean±SD). The great variation in pharmacokinetic data reflects the heterogeneity of the population studied with respect to extracellular fluid space, cardiovascular status, serum protein and other parameters.
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  • 3
    ISSN: 1432-1076
    Keywords: Kallmann syndrome X-recessive inheritance ; Hypoplastic olfactory sulci ; Rhinencephalon MR-imaging
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 3-month-old infant presented with hypogonadism, a small penis and bilateral cryptorchidism. He showed an insufficient response of luteotropic hormone (LH) and follicle stimulating hormone (FSH) to luteotropic hormone releasing hormone (LHRH) and of testosterone to human chorionic gonadotrophin. The maternal uncle had hypogonadism and anosmia and also showed an impaired LH and FSH response to LHRH. MRI examination showed hypoplasia of the rhinencephalon in both cases. These findings in the son and brother of the clinically unaffected mother suggest X-linked recessive inheritance.
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  • 4
    ISSN: 1432-1076
    Keywords: Key words Kinking ; Ischaemic brain infarction ; Cyclosporin A
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We present a 3-year-old patient with stenotic kinking of the left internal carotid artery (ICA) who developed an ischaemic infarction of the left brain hemisphere followed by severe neurological sequelae after a prolonged generalized seizure. At time of the seizure the boy was in biological remission of a nephrotic syndrome and received prednisolone and cyclosporin A (CsA) treatment. The haemodynamic consequences of inborn kinking of the ICA is discussed controversely in the literature. The presented case shows that stenotic kinking of the ICA may significantly impair the blood flow towards the homolateral hemisphere and therefore may result in an ischaemic infarction. The influence of CsA on seizure activity is discussed. Conclusion This case provides clinical and radiological evidence supporting an association between stenotic kinking of the carotid artery and homolateral hemispheric brain infarction.
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  • 5
    ISSN: 1432-1076
    Keywords: Key words 3-hydroxy-3-methyl-glutaryl-coenzyme A (HMG-CoA) lyase deficiency ; Organic aciduria ; Stroke-like encephalopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 2.5-year-old boy presented with acute metabolic decompensation in whom 3-hydroxy-3-methyl-glutaryl-coenzyme A (HMG-CoA) lyase deficiency was diagnosed. Four days after metabolic decompensation, a stroke-like encephalopathy with tonic clonic convulsion of the left arm and leg and coma developed. Brain oedema and subsequent demarcation and atrophy were observed mainly within the supply areas of the right anterior and middle cerebral artery and to a lesser extent in various sites within the right hemisphere. Residual neurological deficits included spastic paresis of the left arm and leg, and left supranuclear facial palsy and aphasia, indicating bilateral diffuse brain affection. Conclusion In the presented patient with HMG-CoA lyase deficiency, stroke-like encephalopathy occurred days after metabolic decompensation indicating ongoing (intracerebral) metabolic derangement. Monitoring of the intracerebral accumulation of toxic metabolites by magnetic resonance spectroscopy and of cerebral haemodynamics might be useful for a better understanding of the pathogenetic mechanisms of stroke-like encephalopathy and to identify patients at risk.
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  • 6
    ISSN: 1432-2102
    Keywords: Schlüsselwörter Staging thorakoabdominaler Tumoren ; Kinder ; Computertomographie (CT) ; Magnetresonanztomographie (MRT) ; Malignome ; Key words Staging of tumors ; Thoracoabdominal tumors ; Childhood ; Computed tomography ; Magnetic resonance imaging
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Thoracoabdominal tumors in childhood can arise from all organs and affect children from the neonate to the adolescent. Better prognosis of childhood tumors, due to better biological understanding and improvement of chemotherapy, require sufficient radiological staging. Sufficiency in radiological staging needs modern cross-sectional techniques with high resolution, good availability and lower radiation dose. In general computed tomography (CT) is being used for radiological staging; increasingly MR imaging is being used because of multiplanar imaging techniques. Replacement of invasive techniques such as myelography and lymphography and modern cross-sectional techniques create painless staging conditions. Nevertheless, scintigraphy will always be a major examination technique, especially for evaluation of metastases and postoperative control examinations. The most common thoracoabdominal tumors in childhood and their staging are described.
    Notes: Zusammenfassung Thorakale und abdominale Tumoren im Kindesalter nehmen ihren Ausgang von allen Organsystemen und können vom Neugeborenen bis zum Adoleszenten auftreten. Verbesserte Prognose, bedingt durch das bessere biologische Verständnis der Tumorerkrankung, sowie der effiziente Einsatz von Chemotherapeutika machen ein radiologisches Staging unumgänglich. Üblicherweise wird zum Staging die Computertomographie (CT) eingesetzt. Durch die Möglichkeit der multiplanaren Schnittführung und die bessere Auflösung bei Tumoren mit Beteiligung des ZNS wird in zunehmendem Maße die Magnetresonanztomographie (MRT) eingesetzt. Diese modernsten Schnittbildverfahren haben invasive Methoden wie Lymphographie und Angiographie in den Hintergrund gedrängt. Im weiteren werden die häufigsten thorakalen und abdominalen Tumoren im Kindesalter besprochen und deren Stagingsysteme erläutert.
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  • 7
    ISSN: 1432-2102
    Keywords: Schlüsselwörter ; Zentrale Katheter ; Neonatologie ; Radiologie ; Key words ; Central catheter ; Neonatology ; Radiology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Background: Central catheters in neonatological intensive care patients are used for the prolonged application of medicataion or parenteral infusions. Dislocations and septic and thromboembolic complications may occur. Control of position: Radiologically, the correct position of the catheter must be proven. Dislocations or complications associated with central catheters must be diagnosed. For catheter monitoring plain film radiographs are the first line of investigation; however, sonography may be of additional assistance. Angiographic techniques should only be performed when conventional noninvasive methods do not supply satisfactory results. Special knowledge is necessary for monitoring catheters that are set via the umbilical artery or vein. The radiological applications for catheter monitoring in the neonate intensive care unit are discussed in this article.
    Notes: Zusammenfassung Hintergrund: Die im neonatologischen Krankengut am häufigsten verwendeten Zugänge sind Katheter, die eine prolongierte Verabreichung von Antibiotika, höher osmolarer Lösungen oder evtl. Chemotherapeutika gewährleisten. Komplikationen ergeben sich durch Fehllagen oder sind septischer und thrombembolischer Genese. Lagekontrolle: Für den Radiologen gilt es, eine adäquate Lagekontrolle des Katheters durchzuführen, um Fehllagen zu vermeiden, jedoch auch Komplikationen zu diagnostizieren. Für dieses Kathetermonitoring steht zur Lagekontrolle das Nativbild im Vordergrund, des Weiteren erweist sich jedoch die Sonographie als wesentlicher Pfeiler in der Katheterdiagnostik, insbesondere im Hinblick auf die katheterassoziierten Komplikationen. Angiographische Techniken sollten erst nach Ausschöpfen sämtlicher nichtinvasiver diagnostischer Möglichkeiten durchgeführt werden. Besondere Anforderungen ergeben sich durch die in der Neonatologie verwendeten Zugänge über den Nabel, wobei sowohl Kenntnis der exakten Topographie der Katheterlage als auch der speziellen potentiellen Komplikationen von großer Bedeutung ist. Im Folgenden sollen die radiologischen Aufgaben und Möglichkeiten beim Kathetermonitoring unter besonderer Berücksichtigung spezieller Anforderungen bei neonatologischen Intensivpatienten vorgestellt werden.
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  • 8
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Over a two year period 74 consecutive Duplex Doppler scans were performed in 23 children with renal allografts and were compared to the Doppler sonographic findings in orthotopic kidneys of 25 age matched healthy controls. The Doppler waveforms of renal arterial flow were analysed qualitatively assessing systolic and diastolic flow amplitudes, for quantitation the Pourcelot index (PI) was used. There was no variation between the Doppler waveforms in recipients with normal allograft function and healthy controls. In 12 patients with biopsy proven acute rejection a decrease or absence of the diastolic flow amplitude was noted, resulting in increased pulsatility of the Doppler wave-form. The mean PI in acute rejection differed significantly from the mean PI in normal allograft function. Duplex Doppler sonography is a useful imaging modality in the differentiation between acute rejection and normal allograft function and should therefore be integrated in the screening of children after renal transplantation.
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  • 9
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Changes of portal venous hemodynamics were investigated in 32 patients with cystic fibrosis (CF) with a mean age of 11.6 years. Hepatic profile of these patients included total bilirubin and albumin together with determination of size and echogenicity of the liver and spleen, determination of the diameter of the portal vein by real-time sonography and quantitation of flow volume of the portal vein using Duplex Doppler sonography. As a control, 35 age matched healthy children were also examined. Diameter of the portal vein was significantly increased in CF patients versus controls. Comparison of the mean flow volume of the portal vein showed a significant increase in CF-patients over 12 y old versus controls. In patients less than 12 y no significant difference of flow volume of the portal vein between CF-patients and controls was noted. The increase of diameter and flow volume of the portal vein suggest an adaptive mechanism in the pressure-volume relationship of the portal venous system in patients with CF.
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  • 10
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The influence of mechanical ventilation with low mena airway pressure (MAP) on cerebral blood flow (CBF) veolocity in newborn infants was assessed in fifteen ventilated infants by Duplex Doppler Sonography (Duplex DS). As a control, CBF velocities were examined in 15 age and weight matched non-ventilated infants. For quantitation, maximal systolic velocity, enddiastolic velocity and the semiquantitative Pourcelot index were determined as representative flow variables. There was no significant difference of these flow variables between ventilated and non-ventilated infants. The pH, pO2 and pCO2 did not differ significantly between the two groups and there was no correlation between the flow variables, pH, pO2, pCO2 or MAP. Mechanical ventilation with low MAP is not associated with adverse effects on cerebral hemodynamics in newborn infants when significant alterations of the blood gases are avoided.
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