Sindrome de Netherton / Netherton Syndrome

Presentamos dos pacientes con la triada disgnostica del Sindrome de Netherton:ictiosis lineal circunfleja,tricorresis invaginada y diatesis atopica.En ambos casos la biopsia de las lesiones de piel y la microscopia por barrido electronico,fueron decisivas para el diagnostico.Los hallazgos histopatologicos del borde externo de las lesiones fueron compatibles con dermatitis psoriasiforme.La tricorresis invaginada o pelo en caña de bambu,se encontro presente a lo largo del pelo.Las manifestaciones atopicas fueron inconstantes.La edad de los pacientes exigio el planteo de terapeuticas poco agresivas.

Sindrome de Netherton / Netherton Syndrome

Presentamos dos pacientes con la triada disgnostica del Sindrome de Netherton:ictiosis lineal circunfleja,tricorresis invaginada y diatesis atopica.En ambos casos la biopsia de las lesiones de piel y la microscopia por barrido electronico,fueron decisivas para el diagnostico.Los hallazgos histopatologicos del borde externo de las lesiones fueron compatibles con dermatitis psoriasiforme.La tricorresis invaginada o pelo en caña de bambu,se encontro presente a lo largo del pelo.Las manifestaciones atopicas fueron inconstantes.La edad de los pacientes exigio el planteo de terapeuticas poco agresivas.AU

[Liver transplantation, vascular complications, role of imaging, percutaneous therapeutic possibilities]. / Transplantation hépatique complications vasculaires place de l'imagerie possibilités thérapeutiques per-cutanées.

Duplex Doppler ultrasound should be the first investigation when a vascular complication is suspected. Serial examinations may increase sensitivity. Color imaging enhances identification of small vessels and increases specificity. Angiography is not necessary unless an pseudoaneurysm is suspected. When duplex Doppler ultrasound is abnormal, angiography is performed to evaluate the possibility of treatment. Endovascular therapeutic procedures appear to have a limited field of application in comparison to percutaneous biliary procedures. Hemostatic embolization or dilatation of vascular strictures can be used either as definitive treatment or while waiting for a new graft to be performed under better conditions.

[Use of a constant-flow 2-phase injector for abdominal scanners in 60 patients]. / Utilisation d'un injecteur biphasique à débit constant pour les scanners abdominaux chez 60 patients.

The intravenous injection of a contrast medium is carried out in more than 80% of all the patients undergoing abdominal CT. The use of a constant-flow two-phase injector has several advantages in practice: possibility to vary the injections, safety, easy use, reproducibility, overall quality of the results and possibility to reduce the dose of contrast medium. The routine use of such equipment seems to be indispensable at present.

[Imaging in the diagnosis of vascular complications after hepatic transplantation]. / Imagerie dans le diagnostic des complications vasculaires après transplantation hépatique.

Twenty-nine vascular complications which occurred after 388 hepatic transplantations performed over a 5 year period (14 arterial thromboses, 4 aneurysms and ruptures of the hepatic artery, 8 portal thromboses and 3 peri-anastomosis portal stenoses) were investigated retrospectively in order to determine the role of imagery in diagnosing these vascular complications. The best screening examination for the diagnosis of hepatic artery thrombosis appeared to be pulsed Doppler coupled with echography. It provides a means of selecting candidates for arteriography, the only investigation allowing certain diagnosis of thrombosis. False aneurysms, suspected in cases of severe post-operative sepsis should be investigated with arteriography even if CAT scans and echo-pulsed Doppler imagery is normal. The diagnosis of portal thrombosis and stenosis relies on echo-pulsed Doppler imagery. In these cases, arteriography is carried out before treatment to evaluate the extent of vascular involvement. Thus echography coupled with pulsed Doppler is the best first intention screening examination to be performed whenever a vascular complication is suspected after hepatic transplantation. Nevertheless, arteriography remains the key examination for the diagnosis and evaluation of these complications.

[Treatment of uncontrollable hemorrhage caused by esophageal varices in cirrhotics. Transhepatic embolization or emergency portal shunt]. / Traitement des hémorragies incontrôlables dues aux varices oesophagiennes chez le cirrhotique. Embolisation transhépatique ou dérivation portale d'urgence.

The purpose of this study was to compare the results of two therapeutic methods used in an emergency to control oesophageal variceal bleeding resistant to medical treatment in cirrhotic patients: portal diversion (116 patients) or transhepatic embolization of the varices (84 patients). Portal diversion proved more effective in stopping the haemorrhage than embolization but the overall mortality rate was about the same with the two procedures. An analysis of the results taking into account the patient's condition failed to show any difference in mortality between the two methods. Indeed, the very high mortality rate in the most severely ill patients may oblige to question their usefulness in such cases. In the least severe patients, we prefer portal diversion which is quicker and can be performed at any time. In patients of the intermediate group transhepatic embolization seems to be less hazardous than portal diversion.