Is the Routine Check Nephrostogram Following Percutaneous Antegrade Ureteric Stent Placement Necessary?

Our aim was to review our experience with percutaneous antegrade ureteric stent (PAUS) placement and to determine if the routinely conducted check nephrostogram on the day following ureteric stent placement was necessary. Retrospective review of patients who had undergone PAUS placement between January 2004 and December 2005 was performed. There were 83 subjects (36 males, 47 females), with a mean age of 59.9 years (range, 22–94 years). Average follow-up duration was 7.1 months (range, 1–24 months). The most common indications for PAUS placement were ureteric obstruction due to metastatic disease (n = 56) and urinary calculi (n = 34). Technical success was 93.2% (96/103 attempts), with no major immediate procedure-related complications or mortalities. The Bard 7Fr Urosoft DJ Stent was used in more than 95% of the cases. Eighty-one of 89 (91.0%) check nephrostograms demonstrated a patent ureteric stent with resultant safety catheter removal. Three check nephrostograms revealed distal stent migration requiring repositioning by a goose-snare, while five others showed stent occlusion necessitating permanent external drainage by nephrostomy drainage catheter reinsertion. Following PAUS placement, the serum creatinine level improved or stabilized in 82% of patients. The serum creatinine outcome difference between the groups with benign and malignant indications for PAUS placement was not statistically significant (p = 0.145) but resolution of hydronephrosis was significantly better (p = 0.008) in patients with benign indications. Percutaneous antegrade ureteric stent placement is a safe and effective means of relief for ureteric obstruction. The check nephrostogram following ureteric stent placement was unnecessary in the majority of patients.     

血浆置换显示干细胞移植后环孢素A引起的微血管病溶血性贫血和利福平的相应颜色反应

患者为1例39岁女性,患T/NK细胞淋巴瘤,在外周血异基因干细胞移植后15天,发生环孢素A(CSA)毒性相关的微血管病溶血性贫血(MAHA).因血清肌酐从移植前的0.4mg/dL,上升至移植后第9和15天的1.0和2.9 mg/dL.故停用CSA.     

Pharmacokinetics of busulfan: correlation with veno-occlusive disease in patients undergoing bone marrow transplantation

Summary Busulfan is an alkylating agent that is widely used in preparative regimens for bone marrow transplantation (BMT). We developed a high-performance liquid chromatographic (HPLC) assay for the determination of plasma busulfan concentrations in 30 patients who received oral doses of 1 mg/kg. Concentrations were fit by a one-compartment pharmacokinetic model with first-order absorption. The pattern of absorption and elimination varied widely between patients, with peak concentrations ranging from 1.2 to 10.4 mol/l (mean, 4.25±2.49). The climination half-life ranged from 58 to 433 min (harmonic mean, 140 min). The AUC contributed by a single oral dose ranged from 606 to 5,144 mol-min/l (mean, 2,012±1,223). Patients were evaluated for the development of veno-occlusive disease (VOD), a treatment complication that occurs in 20% of patients undergoing BMT and causes 10% of transplantation-related deaths. All six patients who developed VOD had an AUC greater than the mean, and five of them had an AUC that was >1 SD above the mean. The occurrence of VOD was highly correlated with an increased AUC (>1 SD above the mean) (X²=18;P<0.0001). Using multivariate logistic regression, no other statistically significant pharmacokinetic predictor of VOD was found. The tenfold variability in the busulfan AUC and the statistical association of increased AUC with the development of VOD suggest a possible role for therapeutic monitoring in this setting.Abbreviations VOD veno-occlusive disease of the liver - BMT bone marrow transplantation - k_a absorption rate constant - k elimination rate constant - t_lag absorption lag time - V volume of distribution Supported in part by NCI PO1-CA15396     

Kabuki syndrome: description of dental findings in 8 patients

The cardinal features of Kabuki (Niikawa-Kuroki) syndrome (KS) include characteristic facial dysmorphic features, mild to moderate mental deficiency, skeletal abnormalities, dermatoglyphic abnormalities, and postnatal growth retardation. We identified 8 patients with KS in a genetics clinic over the past 5 years. All were Caucasians, except for 2 who were of mixed Aboriginal and Caucasian descent. All had the facial gestalt, the dermatoglyphic abnormalities characteristic of the syndrome, and developmental delay. Dental abnormalities of permanent teeth were seen in all 8 cases; 6 had missing lower incisors. Five patients had uniquely abnormal upper incisor teeth shape; the upper incisors had a 'flat head' screwdriver-shaped appearance. Other dental abnormalities included missing lower lateral incisors, missing second premolars, and ectopic upper 6-year molars. We believe the presence of the unique dental findings will prove useful in the diagnostic assessment of individuals with KS.