The Roux-en-Y procedure in congenital hepato-biliary disorders

BACKGROUND: The use of the Roux-en-Y procedure is limited in paediatric surgery practice, and is performed mainly in congenital hepatobiliary disorders either as an initial or permanent treatment. In this 18-year retrospective study, we present our experience of the Roux-en-Y procedure in childhood cases of biliary atresia (BA) and congenital choledochal cyst (CCC). METHODS: Twenty-eight children (18 females and 10 males; age 25 days-12 years) with hepatobiliary disorders were treated in our clinics between 1986-2004. Twenty patients suffered from BA (11 females, 9 males) and eight from CCC (seven females, one male). The surgical approach in the patients with BA (mean age 2.1 months) was Roux-en-Y hepatic portoenterostomy (Kasai procedure) and in the patients with CCC (mean age 7.2 years) was cyst excision with Roux-en-Y hepaticojejunostomy. The mean follow up period was 9.3 years. RESULTS: The children with BA developed the follow postoperative complications: 12 cholangitis, 6 portal hypertension and 5 hepatic cirrhosis. Among the children with CCC, two presented post-operative cholangitis, which was treated conservatively, and one developed anastomotic stricture and underwent reoperative reconstruction. At the end of the follow-up period among the children with BA 6 had died, 3 had undergone liver transplantation, and 5 were on a waiting list for transplantation. All children with CCC were alive without sequelae. CONCLUSIONS: Roux-en-Y in BA, with timely diagnosis, is preferred as an initial procedure, followed by liver transplantation in cases with no bile drainage and is the only possible reconstruction in cases of CCC after excision of the biliary cyst.     

Adefovir dipivoxil therapy for lamivudine-resistant hepatitis B in pre- and post-liver transplantation patients

Three-hundred and twenty-four patients were enrolled in an open-label, multicenter, international study in which pre- and post-liver transplantation (LT) patients with recurrent chronic hepatitis B (CHB) and evidence of lamivudine-resistant HBV were treated with adefovir dipivoxil 10 mg once daily. In the pre- and post-LT cohorts, 128 and 196 patients were treated for a median duration of 18.7 and 56.1 weeks, respectively. In patients who received 48 weeks of treatment, 81% of the pre-LT and 34% of the post-LT cohort achieved undetectable serum hepatitis B virus (HBV) DNA (Roche Amplicor Monitor polymerase chain reaction [PCR] assay lower limit of quantification [LLQ] < 400 copies/mL) with a median change in serum HBV DNA from baseline of -4.1 log(10) and -4.3 log(10) copies/mL, respectively. Serum alanine aminotransferase (ALT), albumin, bilirubin, and prothrombin time normalized in 76%, 81%, 50%, and 83% of pre-LT patients and 49%, 76%, 75%, and 20% of post-LT patients. The Child-Pugh-Turcotte (CPT) score improved in over 90% of patients in both cohorts. Genotypic analysis of 122 HBV baseline samples revealed that 98% of these patients had lamivudine-resistant mutant HBV. No adefovir resistance mutations were identified in patients after 48 weeks of therapy. One-year survival was 84% for pre-LT and 93% for post-LT patients (Kaplan-Meier analysis). Treatment-related adverse effects associated with adefovir dipivoxil in this setting were primarily mild to moderate in severity. In conclusion, 48 weeks of adefovir dipivoxil resulted in significant improvements in virologic, biochemical, and clinical parameters in CHB patients pre- and post-LT with lamivudine-resistant HBV.     

Perioperative use of iloprost in cardiac surgery patients diagnosed with heparin‐induced thrombocytopenia‐reactive antibodies or with true HIT (HIT‐reactive antibodies plus thrombocytopenia): An 11‐year experience

Thrombocytopenia and thromboembolism(s) may develop in heparin immune‐mediated thrombocytopenia (HIT) patients after reexposure to heparin. At the Onassis Cardiac Surgery Center, 530 out of 17,000 patients requiring heart surgery over an 11‐year period underwent preoperative HIT assessment by ELISA and a three‐point heparin‐induced platelet aggregation assay (HIPAG). The screening identified 110 patients with HIT‐reactive antibodies, out of which 46 were also thrombocytopenic (true HIT). Cardiac surgery was performed in HIT‐positive patients under heparin anticoagulation and iloprost infusion. A control group of 118 HIT‐negative patients received heparin but no iloprost during surgery. For the first 20 patients, the dose of iloprost diminishing the HIPAG test to ≤5% was determined prior to surgery by in vitro titration using the patients’ own plasma and donor platelets. In parallel, the iloprost “target dose” was also established for each patient intraoperatively, but before heparin administration. Iloprost was infused initially at 3 ng/kg/mL and further adjusted intraoperatively, until ex vivo aggregation reached ≤5%. As a close correlation was observed between the “target dose” identified before surgery and that established intraoperatively, the remaining 90 patients were administered iloprost starting at the presurgery identified “target dose.” This process significantly reduced the number of intraoperative HIPAG reassessments needed to determine the iloprost target dose, and reduced surgical time, while maintaining similar primary clinical outcomes to controls. Therefore, infusion of iloprost throughout surgery, under continuous titration, allows cardiac surgery to be undertaken safely using heparin, while avoiding life‐threatening iloprost‐induced hypotension in patients diagnosed with HIT‐reactive antibodies or true HIT. Am. J. Hematol. 90:608–617, 2015. © 2015 Wiley Periodicals, Inc.     

Comparison between Nageotte and flow cytometric counting of residual leucocytes in freshly prepared leucocyte-reduced red blood cell components

Background

Flow cytometry (FC) and Nageotte hemocytometry represent the most widely accepted methods for counting residual white blood cells (rWBCs) in leucocyte-reduced (LR) blood components. Our aim was to study the agreement between the two methods, under real working blood bank conditions.

Immunophenotypic analysis reveals heterogeneity and common biologic aspects in monoclonal B‐cell lymphocytosis

Monoclonal B‐cell lymphocytosis (MBL) is the presence of small B‐cell clones in the peripheral blood of healthy subjects. Most MBL have the characteristic phenotype of chronic lymphocyte leukemia (chronic lymphocytic leukemia (CLL)‐like MBL), and depending on the number of monoclonal B‐cells, may characterize a preclinical stage of the CLL. However, there are also MBL with an atypical (CD5⁺CD20^+/brightCD23^dim/?) or a CD5^neg phenotype, which remain largely unexplored. We performed an extended immunophenotypic, cytogenetic, and hematologic analysis in 75 CLL‐like, 39 atypical, 50 CD5^neg, and 7 biphenotypic MBL cases to detect differences or similarities among the MBL subsets. The phenotypic analysis showed expression variations in many surface markers and a wide spectrum of disease‐specific phenotypes within each MBL subtype. Interphase fluorescent in situ hybridization analysis showed a different panel of aberrations according to the phenotype. Overall, del(13q14) and +12 were the most common abnormalities (39%), whereas del(11q13), del(17p13), and del(6q23) were detected only in 3, 1, and 0 cases, respectively. A comparison of MBL with overt chronic lymphoproliferations revealed common aspects in the preclinical state, regarding both the kind of cytogenetic aberrations detected and the lymphocyte composition. Our findings highlight not only the heterogeneity among MBL subsets but also indicate common biologic features which differentiate MBL from clinical disease. © 2014 Wiley Periodicals, Inc.     

Assessment of labor using a new type partogram compared to the classical Fisher partogram

Objective: To assess the quality of a new type partogram used to monitor labor.

Methods: We compared efficacy using two types of partograms. The first was the classical WHO partogram (group A) and the second a new type in which we estimated and reported the sub of cervical dilatation and the position of the descending head (group B).

Results: It was noted that there was a statistically significant decrease of the duration between the initiation of active phase of labor and the delivery time (dt1?+?dt2?+?dt3) (p?p?p?Conclusion: The new partogram is more helpful in the recognition of the initiation of the acceleration stage during the active phase of labor and in the timely use of appropriate actions in order to achieve a safer delivery.     

Intensive insulin treatment reduces transient ischaemic episodes during acute coronary events in diabetic patients

OBJECTIVES: This study tested the impact of intensive metabolic treatment with insulin on transient myocardial ischaemia detected with continuous 12-lead ST-segment monitoring during non-ST segment elevation acute coronary syndromes in type 2 diabetic patients. METHODS AND RESULTS: The study included 57 type 2 diabetic patients with non-ST segment elevation acute coronary syndromes.Twenty-eight patients randomized to conventional treatment plus intensive insulin therapy (group A) and twenty-nine to conventional therapy only (group B). Group A patients received insulin by infusion for 48 hours according to a predefined protocol aiming to maintain normoglycaemia. Group B patients received standard coronary care unit treatment. The ST-segment monitoring was performed for 48 hours in the coronary care unit. The two groups were comparable in terms of medical history, clinical and biochemical data. Three patients from both groups were excluded from the analysis because there was objective evidence for evolution in persistent ST-segment elevation acute myocardial infarction. Six patients (24%) from group A vs. twelve from group B (46.2%) had evidence of transient ischaemia (p = 0.098). Group A patients showed significantly lower values in the mean number [group A vs. group B: 0.4 +/- 0.8 vs. 2 +/- 3.1, p < 0.01] and total duration of ST-episodes [group A vs. group B: 2.4 +/- 5.1 vs. 21.2 +/- 31 min, p < 0.01]. Multivariate analysis revealed that the mean plasma glucose during the study period was a powerful predictor of the presence (b:0.377,p < 0.01), the number (b:0.523,p < 0.001) and the total duration (b: 0.686, p < 0.001) of ST-episodes, respectively. CONCLUSIONS; Intensive insulin treatment considerably decreases the number and the total duration of ST-episodes in type 2 diabetic patients suffering from non-ST segment elevation acute coronary syndromes.     

Increased bone resorption is implicated in the pathogenesis of bone loss in hemophiliacs: correlations with hemophilic arthropathy and HIV infection

Osteoporosis has been recently recognized as a severe comorbidity factor in hemophilia. However, its pathogenesis is still obscure. We evaluated the incidence of osteoporosis in 90 hemophilia patients and investigated possible correlations with clinical and laboratory data. Out of the 90 patients, 80 (89%) had severe hemophilia, and 35 (38.9%) were human immunodeficiency virus (HIV)-positive. Hemophilic arthropahty was assessed using World Federation of Hemophilia clinical score and Petterson radiological score. Bone mineral density of the lumbar spine (LS) and femoral neck (FN) were measured using dual-energy X-ray absortiometry. Bone turnover was evaluated by the measurement of: (1) bone resorption markers [N-terminal cross-linking telopeptide of collagen type I (NTX), C-terminal cross-linking telopeptide of collagen type I (CTX), and tartrate-resistant acid phosphatase isoform-5b (TRACP-5b)], (2) bone formation markers [bone-alkaline phosphatase (bALP) and osteocalcin], and (3) osteoclast stimulators (receptor activator of nuclear factor-κB ligand, osteoprotegerin, and tumor necrosis factor-alpha). Osteopenia or osteoporosis was observed in 86% and 65% of the patients in FN and LS, respectively. Osteoporosis was more common among HIV-positive patients in both FN (65.3% vs 41.6%; p = 0.007) and LS (17.86% vs 5.41%, p = 0.004). The severity of osteoporosis in FN correlated with the patients' total clinical and radiological score (p = 0.001). Hemophilia patients showed increased osteoclastic activity (significant increase of TRACP-5b, NTX, and CTX), which was not accompanied by a comparable increased bone formation (reduced osteocalcin and borderline increase of bALP). In multivariate analysis, HIV infection (p = 0.05) and total clinical score (p = 0.001) were independent risk factors for osteoporosis development. We conclude that there is a high prevalence of osteoporosis among hemophiliacs, which is related to the severity of arthropathy and is enhanced by HIV infection. We report for the first time a high bone resorption that seems not to be balanced by a comparable bone formation.