Atypical presentation of distal renal tubular acidosis in two siblings

Primary distal renal tubular acidosis (dRTA) is an inherited disease characterized by the inability of the distal tubule to lower urine pH <5.50 during systemic acidosis. We report two male siblings who presented with severe hyperchloremic metabolic acidosis, high urinary pH, nephrocalcinosis, growth retardation, sensorineural hearing loss, and hypokalemic paralysis. Laboratory investigations revealed proximal tubular dysfunction (low molecular weight proteinuria, generalized hyperaminoaciduria, hypophosphatemia with hyperphosphaturia, and hypouricemia with hyperuricosuria). There was significant hyperoxaluria and laboratory evidence for mild rhabdomyolysis. Under potassium and alkali therapy, proximal tubular abnormalities, muscular enzymes, and oxaluria normalized. A homozygous mutation in the ATP6V1B1 gene, which is responsible for dRTA with early hearing loss, was detected in both siblings. In conclusion, proximal tubular dysfunction and hyperoxaluria may be found in children with dRTA and are reversible under appropriate therapy.     

Intraoperative single-shot “3-in-1” femoral nerve block with ropivacaine 0.25%, ropivacaine 0.5% or bupivacaine 0.25% provides comparable 48-hr analgesia after unilateral total knee replacement

PURPOSE: To compare analgesia after intraoperative single shot "3-in-1" femoral nerve block (FNB) in combination with general anesthesia using ropivacaine 0.25%, ropivacaine 0.5% with bupivacaine 0.25% for total knee replacement (TKR). METHODS: We performed a randomized, double-blind study in 48 patients for elective TKR under general anesthesia. Patients were randomized to one of four groups (C: sham block, R1: "3-in-1" FNB using 30 mL of ropivacaine 0.25%, R2: "3-in-1" FNB using 30 mL of ropivacaine 0.5%, B: "3-in-1" FNB using 30 mL of bupivacaine 0.25%). Verbal pain score (VPS) both at rest and movement were assessed for 48 hr after TKR (0=none; 1=mild; 2=moderate; 3=severe). Total morphine consumption and its associated side effects, duration of hospitalization after operation were also compared. RESULTS: There were no differences in patients' physical characteristics, intraoperative morphine usage, operation time, tourniquet time or length of hospitalization between the four groups. When compared with group C, the VPS was significantly lower in groups R1, R2 and B at one, four, eight, 24 and 48 hr after TKR (P <0.05). The morphine requirement of groups R1, R2 and B were also significantly lower when comparing with group C up to 48 hr postoperatively (P <0.05). There were no significant differences in VPS and postoperative morphine requirement at any time between groups R1, R2 and B. CONCLUSION: "3-in-1" FNB with ropivacaine provided analgesia that was clinically comparable to that of bupivacaine up to 48 hr after TKR. Increasing the concentration of ropivacaine from 0.25% to 0.5% failed to improve the postoperative analgesia of "3-in-1" FNB.     

Clinics in diagnostic imaging (165)

Oesophageal rupture is a life-threatening complication of balloon tamponade for bleeding oesophageal varices. We herein describe the clinical course and imaging findings in a 33-year-old Indian man who had a Sengstaken-Blakemore (SB) tube inserted for uncontrolled haematemesis, which was unfortunately complicated by malposition of the gastric balloon with resultant oesophageal rupture. The inflated SB tube gastric balloon was visualised within the right hemithorax on chest radiography after the SB tube insertion. Further evaluation of the thorax on computed tomography confirmed the diagnosis of oesophageal rupture associated with right-sided haemopneumothorax. It is crucial for both the referring clinician and reporting radiologist to recognise early the imaging features of an incorrectly positioned SB tube gastric balloon, so as to ensure prompt intervention and a reduction in patient morbidity and mortality.     

Immunohistochemical localization of membrane and alpha-granule proteins in human megakaryocytes: application to plastic-embedded bone marrow biopsy specimens

Using a new technique for antigen localization, we have demonstrated platelet proteins in megakaryocytes in plastic-embedded biopsy specimens of normal human bone marrow. In a series of 25 specimens, megakaryocytes showed labeling with antibodies to the integral membrane glycoproteins IIIa, IIb, and the IIb-IIIa complex; granule membrane protein 140; and five alpha-granule matrix proteins: thrombospondin, factor VIII-related antigen, beta-thromboglobulin, platelet factor 4, and fibrinogen. The antibodies to the membrane glycoproteins IIIa, IIb, and IIb-IIIa produced diffuse cytoplasmic staining and heavier staining on the plasma membrane, whereas the antibodies to the alpha-granule matrix proteins produced a distinct granular staining within the cytoplasm. Staining for granule membrane protein 140 was also granular in distribution. Rare mononuclear cells consistent with megakaryocyte precursors were labeled with these markers. Other enzyme histochemical and lectin-binding studies showed that the enzyme alpha-naphthyl acetate esterase, the lectin Ulex europaeus I, and the periodic-acid Schiff reaction were consistent, but not specific, markers of megakaryocytes. This immunohistochemical technique should facilitate the examination of qualitative and quantitative changes in megakaryocytes in a variety of physiologic and pathologic processes.     

Excel97在药物分析中的应用

目的：在药物分析中,电子表格软件ＭｉｃｒｏｓｏｆｔＥｘｃｅ１９７ｆｏｒ Ｗｉｎｄｏｗｓ。方法：利用Ｅｘｃｅｌ的数据处理功能,进行药物的图表绘制、数据计算和统计处理,回归分析,特别是计算分析,并可建立分析数据库。结果和结论：Ｅｘｃｅｌ操作简单,功能强大,数据分析工作直观。     

Robotic-assisted mesh pelvic closure for prevention of small bowel descent after surgery for recurrent rectal cancer

Techniques in Coloproctology -