The use of TachoSil for haemostasis after primary haemostatic treatment appears to be safe and effective in children undergoing liver resection.
Focal nodular hyperplasia is a benign liver tumour that is rare in children. We report the second case of a child with sickle ceil disease presenting with symptomatic focal nodular hyperplasia. The possible pathogenesis of focal nodular hyperplasia and the association with sickle ceil disease are discussed.Focal nodular hyperplasia of the liver is a rare benign lesion found predominantly in women during their reproductive years.' It consists of a well circumscribed area of hyperplastic liver parenchyma that may contain a stellate fibrous scar. The lesion is usually asymptomatic and is an incidental finding. We describe a patient with sickle cell disease presenting with a symptomatic lesion of focal nodular hyperplasia. This is the second description of such an association2 and we discuss whether sickle cell disease is a pathogenetic mechanism for the development of focal nodular hyperplasia.expanding portal tracts that contained dilated vascular channels and numerous bile ductules was typical of focal nodular hyperplasia (fig 3). DiscussionFocal nodular hyperplasia is a rare benign liver tumour with fewer than 20% of cases presenting in children.3 The majority are asymptomatic and usually arise on the surface of the liver beneath the capsule. Their aetiology is unknown, although an association with oral contraceptives has been reported.4The radiological diagnosis depends on identification by ultrasound or computed tomography of a central scar.
Background: Whole body hyperthermia induced by radiative systems has been used in therapy of malignant diseases for more than ten years. Von Ardenne and co-workers have developed the 'systemiche Krebs-Mehrschritt-Therapic' (sKMT), a combined regime including whole body hyperthermia of 42°C, induced hyperglycaemia and relative hyperoxaemia with additional application of chemotherapy. This concept has been employed in a phase I/II clinical study for patients with metastatic colorectal carcinoma at the Virchow-Klinikum since January 1997. Methods: The sKMT concept was performed eleven times under intravenous general anaesthesia, avoiding volatile anaesthetics. Core temperatures of up to 42°C were reached stepwise by warming with infrared-A-radiation (IRATHERM 2000®). During the whole procedure blood glucose levels of 380-450 mg/dl were maintained as well as PaO 2 levels above 200 mmHg. Extensive invasive monitoring was performed in all patients including measurements with the REF-Ox-Pulmonary artery catheter with continuous measuring of mixed venous saturation (Baxter Explorer®) and invasive monitoring of arterial blood pressure. Data for calculation of hemodynamic and gas exchange parameters were collected four times, at temperatures of 37°C, 40°C, 41.8-42°C and 39°C, during measurements FiO 2 was 1.0 at all times. Fluids were given in order to keep central-venous and Wedge pressure within normal range during the whole procedure. Statistics were performed using the Wilcoxon Test. Results: Statistically significant differences were found between heart rate, cardiac index and systemic vascular resistance comparing data at 37°C and 42°C. Heart rate and cardiac index increased to a maximum at 42°C (P < 0.0001) whereas systemic vascular resistance had its minimum at 42°C (P < 0.0001). Mean arterial pressure dropped with increasing temperature, differences were not significant. Calculation of stroke volume index and ventricular volumes showed only a slight decrease in endsystolic volumes with increasing temperature, the resulting differences in right ventricular ejection fraction were marginally significant (P = 0.038) comparing 42°C to baseline. Right ventricular stroke work index as well as mean pulmonary arterial pressure increased at 42°C (P = 0.0115 and P = 0.0037), pulmonary vascular resistance only dropped little compared to systemic vascular resistance, left ventricular stroke work index even dropped with increasing temperature, though showing no significant difference. Values for mixed venous oxygen saturation did not vary during therapy, pulmonary right-left shunt showed a temperature associated increase (P = 0.0323) to a maximum at 42°C. Conclusion: Under the procedure of sKMT cardiac function in patients, who do not have any pre-existing cardiac impairment, can be maintained almost unchanged, ie with normal right and left ventricular pressure, despite an increase in right ventricular stroke work Acknowledegment: Supported by Deutsche Krebshilfe.
Pili annulati (ringed hair) is a rare hair anomaly characterized by alternating light and dark bands along the hair shaft. The light bands are due to air‐filled cavities which scalier the light. It is inherited as an autusomal‐dominant trait, although sporadic cases have been reported. The authors report the occurrence of three cases of piti annulati in a mother and two children. In both children, a buy and a girl 12 and IS years old, respectively, (he anomaly had been present since the first year of life. In the young girl, alopecia areata was also present from the age of 7; this unusual association seems to be fortuitous. The mother and one of her brothers were also affected less severely. All the patients clinically showed an increased brittleness of the hair that appeared otherwise normal. The nails and teeth were unaffected. Ammo‐acid analysis of the hair was performed and showed low levels of serine and glycine and high levels of glutamic acid. The typical light, polarized and scanning‐electron microscopy changes in the hair shaft are described. The inheritance of the disease is discussed.
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