The long QT syndrome affects heart rhythm by prolonging ventricular repolarisation; it is potentially life-threatening since it can evolve into torsades de pointes (a polymorphic ventricular tachycardia) and/or ventricular fibrillation. The case is presented of a 55-year-old liver transplant recipient with a genetically determined long QT syndrome not detected by the standard preoperative cardiological evaluation. It was mild in the immediate post-operative period but developed into torsades de pointes after discharge, probably as a result of therapy. This case was particularly challenging because the first arrhythmic episodes were short and electocardiographically silent, and thus the related faints were thought to have a neurological basis. When the true cause emerged during a monitored episode of torsades de pointes, electric defibrillation was used to restore sinus rhythm and isoproterenol administered to increase heart rate and thus shorten the prolonged QT interval Long-term control was obtained by means of an implantable intracardiac defibrillator. In orthotopic liver transplant recipients with long QT syndrome, particular attention should be given to post-operative therapy as some routinely used drugs can trigger life-threatening ventricular arrhythmias.

Arrhythmias, Cardiac↗

PubMedDate not supplied

Postoperative adjuvant chemoradiotherapy for rectal cancer: analysis of acute and chronic toxicity.

AIMS AND BACKGROUND: The aim of the study was to evaluate acute and chronic toxicity of combined postoperative standard radiation therapy to the pelvis and 5-fluorouracil plus levamisole in resectable rectal cancer. METHODS: Between July 1990 and September 1993, 58 patients with histologically confirmed adenocarcinoma of the rectum entered the prospective study. The schedule consisted of 5-fluorouracil, 450 mg/m2 i.v. for 5 days, and from day 28 5-fluorouracil, 450 mg/m2 i.v. weekly for 24 weeks, plus levamisole given orally at the dose of 150 mg every day for 3 days every 2 weeks for 6 months; radiotherapy (180 cGy/day) 5 days a week for a total dose of 45 Gy was administered from day 28. RESULTS: After the first cycle of chemotherapy (before radiotherapy), overall toxicity was mild. During chemoradiotherapy, dose-limiting toxicity was grade 3 diarrhea and proctitis, for which the combined treatment was interrupted for more than 7 cumulative days in 28 patients. During the 24 weeks of weekly 5-fluorouracil (after radiotherapy), no severe toxicity was reported. Three-year survival and progression-free survival were 65% and 50-55%, respectively. CONCLUSIONS: Although adjuvant chemoradiotherapy is usually feasible, in our study toxicity was severe in a substantial proportion of patients, probably due to the schedule applied. We are evaluating the feasibility and toxicity of a combined treatment which includes 5-fluorouracil in continuous chronomodulated infusion during radiotherapy.

Acute Disease↗

PubMedDate not supplied

Different expressivity of two adjacent mutations of the APC gene.

AIMS AND BACKGROUND: The phenotypic expression of different APC mutations in familial adenomatous polyposis (FAP) is variable: two to three variants of the disease have been defined based on the severity of colonic manifestations. Age of onset and number of polypectomies per person-year of post-surgical follow-up were compared in two FAP families with very close mutation sites in the APC gene, in order to ascertain mutation-specific variation of expressivity. FAMILIES AND APC MUTATIONS: Family A (5 patients) carried a newly characterized mutation, a four bp deletion at codon 843. Family B (5 patients) carried a previously identified mutation at codon 835. RESULTS: Mean age of onset was 49.7 years in family A and 30.5 years in family B; number of polypectomies per person-year of follow-up was 1.05 for family A and 10.1 for family B (P < 0.001). CONCLUSIONS: There is significant variation of expressivity (allelic heterogeneity) in FAP between two mutations separated by only eight codons, located at the 5' extremity of APC gene exon 15.

Adenomatous Polyposis Coli↗

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Intrahepatic chemotherapy with floxuridine, leucovorin and dexamethasone in continuous infusion and mitomycin-C bolus in unresectable hepatic metastases from colorectal cancer: a phase II study.

BACKGROUND: Intrahepatic continuous infusion FUDR induces a 50% response rate in patients with hepatic metastases from colorectal cancer. Lower rates have been observed in pretreated patients. The combination of floxuridine plus leucovorin has obtained over 70% responses, with high hepatic toxicity. The use of dexamethasone can decrease hepatic toxicity. A randomized study reported an increase in response rate and a decrease in hepatic toxicity in a group of patients treated with floxuridine plus dexamethasone compared to a group receiving only floxuridine. Moreover, the combination of mitomycin C, carmustine and floxuridine is also effective in pretreated patients. METHODS: On such premises, since July 1993 we have treated 39 patients affected by unresectable hepatic metastases from colon carcinoma (26 patients) and rectal carcinoma (13 patients) with the combination continuous infusion of floxuridine (0.20 mg/kg per day) + leucovorin (7.5 mg/m2/day) + dexamethasone (20 mg on days 1 to 14) and bolus mitomycin C (10 mg/m2 on day 1) via the hepatic artery. Cycles were administered every four weeks. There were as 28 males and 11 females, with a median age of 64 years (range, 39-75) and a median PS = 0. Twenty-two patients were pretreated with systemic chemotherapy including 5-fluorouracil plus leucovorin. Total number of cycles was 189, with a median of 6 cycles per patient (range, 1-12). RESULTS: Of 39 patients 37 were assessable for response (2 patients were not assessable because they stopped chemotherapy for occlusion of the catheter after the first cycle). There were 3 complete responses (1 in a naive patient and 2 in pretreated patients), 16 partial responses (11 in pretreated patients and 5 in chemonaive patients), 4 minor responses, 4 stable disease and 10 progressive disease. The overall response rate was 51.3% (95 Cl, 51.3-86.7%). Median time to progression was 6 months (range, 1-34+). Overall survival was 18 months (range, 1-34+). Of 39 patients, 36 were assessable for toxicity (WHO) (3 patients died after the first cycle for progression of disease): diarrhea and nausea-vomiting grade 3-4 occurred respectively in 15 (41%) and 3 patients (8%); hepatic toxicity was mild. CONCLUSIONS: The treatment we used showed an elevated activity in liver metastases from colorectal cancer even in patients pretreated and resistant to systemic chemotherapy, although toxicity grade 3-4 diarrhea occurred in approximately 40% of the patients.

Adult↗

PubMedDate not supplied

Role of ultrasound in renal transplantation.

Ultrasound examination of a renal transplant was performed in 27 patients over a period of 28 months; there were kidneys from 12 living and 15 cadaveric donors. The ultrasonic scans were performed over a period ranging from 2 days to 12 years following transplantation. We were able to observe and describe the echographic findings of the normal evolution of a well functioning renal transplant, acute tubular necrosis, acute and chronic rejection, perirenal fluid collection, and obstructive uropathy. Ultrasound evaluation of renal transplant was accurate in the diagnosis of postoperative complications together with clinical and laboratory findings. Ultrasound imaging is independent of renal function and can be performed quickly as often as necessary. Percutaneous procedures, including fine needle aspiration, biopsy, aspiration of fluid collection, and positioning of the pyelostomy catheter can be performed under ultrasonic guidance.

Adolescent↗

PubMedDate not supplied

Percutaneous cholecystostomy for acute cholecystitis in critically ill patients.

BACKGROUND/AIMS: The aim of this study was to ascertain the therapeutic efficacy of percutaneous cholecystostomy in a selected group of high-risk patients who were physiologically unable to tolerate an open procedure. METHODOLOGY: We reviewed the hospital records of 11 critically ill patients who underwent percutaneous cholecystostomy for acute cholecystitis during the intensive care unit course of major underlying diseases. RESULTS: Percutaneous cholecystostomy was easily performed in all cases (feasibility rate: 100%). No procedure-related death was recorded and minor complications occurred in 2 patients (18%). Percutaneous cholecystostomy led to resolution of the sepsis in all but 1 patient with gangrenous calcolous cholecystitis who required emergent cholecystectomy (success rate: 91%). Percutaneous cholecystostomy was the permanent treatment in all patients with acalcolous cholecystitis. Among patients with calcolous cholecystitis, 4 underwent delayed elective cholecystectomy, 1 required no further treatment, and 2 eventually died from the evolution of their underlying diseases. After a mean follow-up of 25 months (range: 12-32 months), none of the patients managed non-operatively required surgery or re-hospitalization. CONCLUSIONS: Ease of performance, low complication rate, and high success rate make percutaneous cholecystostomy the procedure of choice for critically ill patients with acute cholecystitis. Whenever possible, percutaneous cholecystostomy should be followed by elective cholecystectomy. However, especially in acalcolous cholecystitis, it may constitute the definitive treatment.

Acute Disease↗

PubMedDate not supplied

[Echo-guided percutaneous cholecystostomy in the treatment of acute cholecystitis].

Ultrasound-guided cholecystostomy (UGC) is indicated for high-risk patients with acute cholecystitis (AC). The advantage of this approach is greatest for critically ill patients who develop AC while in the intensive care unit (ICU). Moreover, in ICU patients with unexplained sepsis UGC serves as a diagnostic maneuver since it may allow the identification of a biliary infection. UGC has a high therapeutic efficacy approaching 100% in patients with a well-established diagnosis of AC. Morbidity is low and almost entirely related to catheter dislodgment. Trans-catheter cholecystocholangiograms (TCC), indispensable for planning any further treatment, must be delayed until the resolution of the sepsis. The risk of recurrence depends on AC etiology. Acalculous AC entails a low recurrence risk and may often be managed non-operatively. After the resolution of the sepsis, all calculous AC should be considered for cholecystectomy. However, if the operative risk remains high the possibility of avoiding the operation depends on the TCC demonstration of the patency of the cystic duct. The catheter should remain in place until operation. In case of non-operative management withdrawal should be delayed until the resolution of the sepsis. Laparoscopy is suitable in case of recent inflammation.

Acute Disease↗