Classic polyarteritis nodosa (c-PAN) is a rare disease in adults and extremely rare in children. We report a 3-year-old girl with c-PAN who presented with disturbances of consciousness and hypertension. Cranial tomography showed a subarachnoid hemorrhage. Subsequent magnetic resonance imaging and magnetic resonance angiography demonstrated subarachnoid hemorrhage and acute ischemic lesions. Renal angiography revealed bilateral multiple aneurysms. Due to her constitutional symptoms and hypertension and radiological findings she was diagnosed as having c-PAN. She was successfully treated with hydralazine followed by angiotensin-converting enzyme inhibitor, calcium channel blocker, intravenous pulse methylprednisolone, and subsequently oral prednisolone and oral cyclophosphamide. To our knowledge this is the youngest patient with c-PAN presenting with subarachnoid hemorrhage. Malign hypertension at this young age deserves a meticulous investigation of the vascular origin. Furthermore, treatment with pulse methyl prednisolone followed by oral prednisolone and oral cyclophosphamide is a successful modality of treatment in such a life-threatening presentation of c-PAN in childhood.
Allogeneic hematopoetic stem cell transplantation (HSCT) is the only radical cure of beta-thalassemia. However, iron overload remains a cause of morbidity and mortality in posttransplant period. The authors present 7 patients as a preliminary report who underwent bone marrow transplant (BMT) and received oral chelating therapy (deferasirox) because of poor compliance to phlebotomy and desferrioxamine. The patients investigated mainly for possible side effects of deferasirox. No negative effect was seen in aspartate aminotransferase (AST), alanine aminotransferase (ALT), hemoglobin (Hb), and donor chimerism of the patients while serum ferritin levels significantly reduced (P = .018). Although serum creatinin significantly increased (P = .034), it was in normal limits in all patients. The authors believe that this report shows promising findings to plan further studies to clarify clinical safety and efficacy of deferasirox in posttransplant period.
A micromethod is described for the separation and quantitative determination of individual bile acids in duodenal content and bile. After a mild saponification procedure for splitting the taurine and glycine conjugates, the products are separated by thin-layer chromatography on silicagel layer with the solvent system //0-octane-ethyl acetate-acetic acid (60 ml + 35 ml + 5 ml) and determined by spectrofluorirrietry. A series of control experiments has been performed on the validation of the procedure. Values obtained for human subjects or experimental applications of the method are presented.Eine Mikromethode zur Trennung und quantitativen Bestimmung der einzelnen Gallensäure inDuodenal-Inhalt und Galle wird beschrieben, Nach milder Verseifung zur Spaltung der Taurin-und Glycinkonjugate erfolgt die Dünnschichtchromatographie auf Silicagel mit dem Laufmittel /jO-Oktan/Äthylacetat/Eisessig 60 ml + 35 ml -f-5 ml. IDie nachfolgende Bestimmung wird spektrofluorimetriseh durchgeführt. Zur Bewertung des Verfahrens wurde eine Serie von Kontrollversuchen durchgeführt. Werte für den Menschen oder Laboratoriumstiere und Beispiele klinischer oder experimenteller Anwendung der Methode werden mitgeteilt.Methods for the separation of individual bile acids from biological fluids include column and paper (1 -4), thin-layer (5-13) or gas-liquid (14-17) chromatography. Quantitative measurements of the metabolites are made by an in situ semi-quantitative approach (6, 7) or densitometry without elution of zones from paper or layer, by colorimetry (1-4, 9), fluorimetry (13, 18, 19), enzymatic methods (10, 12) following elution of the steroids, or gas chromatography using internal standards (14-17). Recently, the introduction of spectrofluorimetry and gas chromatography resulted in a considerable increase in the sensitivity and specificity of the methods. Determination of the individual free and conjugated bile acids in a large number of samples by gas chromatography appears to be rather laborious and the technique cannot be used for routine purposes. A procedure in which the individual conjugated bile acids are separated by thin-layer chromatography and quantitated by spectrofluorimetry overcomes this disadvantage. Methods StandardsAuthentic cholic and deoxycholic acid were obtained from Fluka AG, chenodeoxycholic and lithocholic acid from Koch-Light Ltd., taurochenodeoxycholic, taurodeoxycholic, taurolithocholic and glycolithocholic acid from Calbiochern Ltd., glycochenodeoxycholicandglycodeoxycholic acid were purchased from Sigma Chemical Corp. The purity of the standards was tested by thinlayer chromatography and phosphomolybdic acid reaction.Chromatography procedures Thin-layer chromatography was performed on Silicagel G Merck AG chromatoplates (20 20 cm and layer-thickness 0.2 mm). The plates were activated at 105°C for at least two hours and" were not equilibrated in the chamber before development. The steroids were applied on strips of 1.5-2.0 cm. Solvent systems:A. /r0-Octane-ethyl acetate-acetic acid (50 ml + 40 ml + 10 ml) ...
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