Early splenectomy and polychemotherapy versus polychemotherapy alone in chronic myeloid leukemia

The effect of early splenectomy and of polychemotherapy with hydroxyurea, busulfan, and alternate bimonthly courses of arabinosyl cytosine and vincristine plus prednisone, was evaluated in 139 previously untreated patients with chronic myeloid leukemia (CML), consecutively admitted to 18 hospitals from March 1973 to October 1974. Fifty-six patients were splenectomized and 83 patients were not splenectomized. Splenectomy did not influence the duration of chronic and blastic phase, and did not prolong survival. The prognosis of high risk patients was not improved. During the chronic phase, high platelet counts were more frequent in splenectomy group, and five patients developed thrombotic or thromboembolic complications, 5 to 19 months after the operation. The median survival of the whole group was 50 months, with 32 of 139 patients (actuarial proportion 30%) remaining alive 72 months after diagnosis, but the slope of the survival curve was similar to that of historical controls. The results of this trial suggests that new strategies should be developed for the therapy of CML.     

Aminopenicillin allergy

Eight two children with histories of maculopapular or urticarial rashes during aminopenicillin treatment underwent skin tests, patch tests, radioallergosorbent assays and, in some cases, oral challenges. Hypersensitivity was diagnosed in eight (9.8%): immediate in four with urticarial reactions and delayed (that is cell mediated) in four with maculopapular rashes. In 49 children (38 with maculopapular eruptions, 11 with urticarial/angiooedematous reactions), negative allergologic findings were confirmed using oral challenges with the suspected drug. Maculopapular rashes may reflect delayed hypersensitivity to aminopenicillins, which can be diagnosed on the basis of late intradermal reactions and/or patch test positivity. The allergen panel must include the suspected aminopenicillin itself, as many cases are side chain specific. Most patients with urticarial reactions (more typical of immediate hypersensitivity) will also react to penicilloyl polylysine and minor determinant mixture. The time elapsed between the reaction and testing must be considered if negative results emerge, because IgE mediated sensitivity (unlike cell mediated forms) declines in the absence of antigen exposure.     

Laparoscopic appendectomy for acute and recurrent appendicitis: retrospective analysis of a single-group 5-year experience

BACKGROUND AND PURPOSE: Twenty years after the first report of laparoscopic appendectomy (LA), its role in the treatment of appendicitis is still under debate. We report herein a retrospective analysis of our cases of LA in the last 5 years, during which we adopted a policy of an almost uniform laparoscopic approach on a rather selected population composed mainly of women with acute or recurrent lower quadrant abdominal pain. PATIENTS AND METHODS: Laparoscopic appendectomy was performed on 33 male and 98 female patients. The mean age at operation was 25.7+/-11.4 years (range 11-59 years). Acute appendicitis with localized or diffuse peritonitis was present in 34 cases. In the remaining 97 patients, the operation was performed for acute or recurring symptoms of lower quadrant abdominal pain. RESULTS: There were no conversions to open surgery. The operating time was 45+/-17 minutes (range 30-110 minutes). The pathology examination of the removed appendices showed acute appendicitis in 34 cases and chronic inflammation in the remaining 97 patients. In one case, histology revealed a coexisting mucinous carcinoid that extended to the perivisceral fat, and a completion right hemicolectomy was performed. Complications were minor in most cases. Reoperation for deep abdominal abscess or hematoma was required in three cases. The mean hospital stay was 2.59+/-1.58 days (range 24 hours-11 days). CONCLUSION: In our hands, LA has proven to be safe and effective. The laparoscopic operation has significant advantages in terms of lower invasiveness and better diagnostic capability. It is especially useful in women of child-bearing age, in whom it may be considered the procedure of choice.     

Vault Prolapse II: Restoration of Dynamic Vaginal Supports by Infracoccygeal Sacropexy,an Axial Day-Case Vaginal Procedure

Our objective was to present and critically analyze the first 75 infracoccygeal sacropexy (ICS) operations for cure of vault prolapse. Level 1 reconstruction was achieved by the insertion of a tension-free vaginal tape (nylon) via the ischiorectal fossa into a transversely incised posterior vaginal fornix. Level 2 defects were repaired by cutting a central ‘bridge’ in the posterior vaginal wall and suturing the lateral flaps to the bridge. Level 3 repair reconstituted the perineal body. Postoperative pain was minimized by avoiding excessive tightening of tissues and avoiding surgery on the distal 1 cm of the vagina. Total operating time varied between 30 and 60 minutes. Mean blood loss was 120 ml. All patients were discharged within 24 hours of surgery, with minimal pain and without indwelling catheters. Almost all returned to normal activities within 7–10 days. Of the 75 patients (mean age 54 years), 71 were prospectively followed up between 1 and 4.5 years after surgery. Vault prolapse recurred in 6%. The main complication was tape erosion (5.3%). Infracoccygeal sacropexy is a promising day-case alternative to conventional methods. It has built-in safety, as it avoids pudendal nerves and vessels and surface rectal veins. Areas of future development are identified.     

Gastrointestinal stromal tumours: a broad spectrum of clinical presentations

Gastrointestinal stromal tumours, though rare, are the most common mesenchymal neoplasms affecting the gastrointestinal tract. The most frequent sites of origin are the stomach and the small bowel, but they can occur anywhere in the gastrointestinal tract. Mesenteric and retroperitoneal forms have been described. The Authors present their personal experience with the treatment of gastrointestinal stromal tumours, with particular reference to the broad spectrum of clinical presentations and to the consequent therapeutic implications. We report on a retrospective analysis of the clinical presentations and courses, surgical management and pathological features of 27 patients with such tumours treated in our institution from 1993 to 2005. The variables analysed were the morphological and clinical characteristics of the tumours, demographic data, type of surgical treatment and postoperative course. Long-term survival was evaluated on the basis of clinical and/or telephonic follow-up in all patients. One tumour was located in the oesophagus, 14 in the gastric area, 7 in the small bowel, 2 in the colon-rectum, and 3 in the peritoneum. All patients studied received radical surgical treatment. In 7 patients surgical resection was extended to other organs. No postoperative mortality or major postoperative complications were observed. Twenty-two patients are still alive at follow-up. Three patients died as a result of neoplastic relapse and 2 of other causes. The median survival was 36 months. The actuarial 3- and 5-year survival rates were 89.7% and 67.8%, respectively. Our experience indicates that the site of origin of gastrointestinal stromal tumours with their broad spectrum of clinical presentations may influence both the therapeutic choice (neoadjuvant utilisation of imatinib mesylate) and the surgical treatment (wedge resection vs enlarged operations).     

Disease patterns and causes of death of hospitalized HIV-positive adults in West Africa: a multicountry survey in the antiretroviral treatment era

Objective

We aimed to describe the morbidity and mortality patterns in HIV-positive adults hospitalized in West Africa.

Method

We conducted a six-month prospective multicentre survey within the IeDEA West Africa collaboration in six adult medical wards of teaching hospitals in Abidjan, Ouagadougou, Cotonou, Dakar and Bamako. From April to October 2010, all newly hospitalized HIV-positive patients were eligible. Baseline and follow-up information until hospital discharge was recorded using standardized forms. Diagnoses were reviewed by a local event validation committee using reference definitions. Factors associated with in-hospital mortality were studied with a logistic regression model.

Results

Among 823 hospitalized HIV-positive adults (median age 40 years, 58% women), 24% discovered their HIV infection during the hospitalization, median CD4 count was 75/mm³ (IQR: 25–177) and 48% had previously received antiretroviral treatment (ART). The underlying causes of hospitalization were AIDS-defining conditions (54%), other infections (32%), other diseases (8%) and non-specific illness (6%). The most frequent diseases diagnosed were: tuberculosis (29%), pneumonia (15%), malaria (10%) and cerebral toxoplasmosis (10%). Overall, 315 (38%) patients died during hospitalization and the underlying cause of death was AIDS (63%), non-AIDS-defining infections (26%), other diseases (7%) and non-specific illness or unknown cause (4%). Among them, the most frequent fatal diseases were: tuberculosis (36%), cerebral toxoplasmosis (10%), cryptococcosis (9%) and sepsis (7%). Older age, clinical WHO stage 3 and 4, low CD4 count, and AIDS-defining infectious diagnoses were associated with hospital fatality.

Conclusions

AIDS-defining conditions, primarily tuberculosis, and bacterial infections were the most frequent causes of hospitalization in HIV-positive adults in West Africa and resulted in high in-hospital fatality. Sustained efforts are needed to integrate care of these disease conditions and optimize earlier diagnosis of HIV infection and initiation of ART.     

Residual factor VII activity and different hemorrhagic phenotypes in CRM(+) factor VII deficiencies (Gly331Ser and Gly283Ser)

Two cross-reacting material-positive (CRM(+)) factor VII (FVII) mutations, associated with similar reductions in coagulant activity (2.5%) but with mild to asymptomatic (Gly331Ser, c184 [in chymotrypsin numbering]) or severe (Gly283Ser, c140) hemorrhagic phenotypes, were investigated. The affected glycines belong to structurally conserved regions in the c184 through c193 and c140s activation domain loops, respectively. The natural mutants 331Ser-FVII and 283Ser-FVII were expressed, and in addition 331Ala-FVII and 283Ala-FVII were expressed because 3 functional serine-proteases bear alanine at these positions. The 331Ser-FVII, present in several asymptomatic subjects, showed detectable factor Xa generation activity in patient plasma (0.7% +/- 0.2%) and in reconstituted system with the recombinant molecules (2.7% +/- 1.1%). The reduced activity of recombinant 283Ala-FVII (7.2% +/- 2.2%) indicates that the full function of FVII requires glycine at this position, and the undetectable activity of 283Ser-FVII suggests that the oxydrile group of Ser283 participates in causing severe CRM(+) deficiency. Furthermore, in a plasma system with limiting thromboplastin concentration, 283Ser-FVII inhibited wild-type FVIIa activity in a dose-dependent manner.     

Predictors of survival after liver transplantation for hepatocellular carcinoma associated with Hepatitis C.

The efficacy of orthotopic liver transplantation (OLT) for hepatocellular carcinoma (HCC) associated with hepatitis C virus (HCV) is not well defined. This study examines the variables that may determine the outcome of OLT for HCC in HCV patients. From 1990 to 1999, 463 OLTs were performed for HCV cirrhosis. Of these patients, 67 with concurrent HCC were included in the study. Univariate and multivariate analyses considered the following variables: gender, pTNM stage, tumor size, number of nodules, vascular invasion, incidental tumors, adjuvant chemotherapy, preoperative chemoembolization, alpha-fetoprotein (AFP) tumor marker, lobar distribution, and histological grade. Overall OLT survival of HCV patients diagnosed with concomitant HCC was significantly lower when compared to patients who underwent OLT for HCV alone at 1, 3, and 5 years (75%, 71%, and 55% versus 84%, 76%, and 75%, respectively; P < 0.01). Overall survival of patients with stage I HCC was significantly better than patients with stage II, III, or IV (P < .05). Eleven of 67 patients developed tumor recurrence. Sites of recurrence included transplanted liver (5), lung (5), and bone (1). Twenty-four of 67 patients (36%) died during the follow-up time. Causes of deaths included recurrent HCC in 8 of 24 patients (12%) and recurrent HCV in 3 of 24 patients (4.5%), whereas 13 (19.5%) patients died from causes that were unrelated to HCV or HCC. Both univariate and multivariate analysis demonstrated that pTNM status (I versus II, III, and IV; P < .05) was a reliable prognostic indicator for patient survival. Presence of vascular invasion (P = .0001) and advanced pTNM staging (P = .038) increased risk of recurrence. Multivariate analysis showed that pretransplant chemoembolization and adjuvant chemotherapy reduced risk of death after OLT in HCC recipients. In conclusion, this study demonstrates the effectiveness of OLT for patients with HCC in a large cohort of chronic HCV patients. Advanced tumor stage, and particularly vascular invasion, are poor prognostic indicators for tumor recurrence. Early pTNM stage, adjuvant chemotherapy, and preoperative chemoembolization were associated with positive outcomes for patients who underwent OLT for concomitant HCV and HCC.