Liver transplantation for the management of hepatoblastoma.

INTRODUCTION: Hepatoblastoma (HEP) is the most frequent liver malignancy occurring in childhood. Surgical resection currently represents the gold standard for treatment. In patients with initially unresectable tumors, chemotherapy may induce remarkable reductions in size. In nonresponder patients, liver transplantation (OLTx) may offer a chance of cure. MATERIALS AND METHODS: From 1990 to 2003, a total of 400 OLTx (31 pediatric transplants) have been performed at Padua University. Seven patients (4 males and 3 females) underwent OLTx for hepatoblastoma. All patients presented with bilobar liver involvement and had received chemotherapy according to the SIOPEL-1. In all patients preoperative staging was negative for extrahepatic involvement. RESULTS: The mean age of the pts was 8.2 years (range 6.4 months to 34 years). Mean follow-up after OLTx was 41.4 months (median 36, range 3 to 108 months). Actuarial patient survival rates after OLTx for hepatoblastoma are 83.3%, 83.3%, and 56% at 1, 3, and 5 years, respectively. Five of seven subjects with HEP are alive after transplant at 3, 12, 36, 65, and 108 months. Two patients died owing to recurrent disease after 6 and 60 months, respectively, from transplantation. Another subject, primarily treated with surgical resection, shows HEP recurrence at 40 months after OLTx. The remaining 4 patients are alive and well at a mean follow-up of 28 months (median 24, range 3 to 65 months). CONCLUSIONS: Liver transplantation may represent a valid therapeutic option for patients with unresectable HEP, but it is contraindicated in cases of recurrence following previous resection surgery. Neo-adjuvant chemotherapy is of paramount importance to obtain good long-term results.

Adjuvant chemotherapy for transplanted hepatocellular carcinoma patients: impact on survival or HCV recurrence timing.

INTRODUCTION: Hepatocellular carcinoma (HCC) is one of the most common tumors worldwide. In the Western world the current epidemic of cirrhosis due to the hepatitis C virus (HCV) is increasing the number of new cases. Liver transplantation (OLTx) represents a radical treatment for HCC and the underlying cirrhosis. Whether adjuvant chemotherapy is indicated in the postoperative period to prevent recurrence is controversial. MATERIAL AND PATIENTS: Forty-eight HCC patients underwent liver transplantation during 11 years, including 21 who were chemo-treated (CT) patients. Thirty-one patients (65%) had post-necrotic virus-C cirrhosis (PNC-C). Twenty-one cases (44%) were p-TNM stages III-IV, and 15 cases (31%) incidental HCC detected in the explanted liver. Seven HCV patients (15%) received chemotherapy (before 1998). RESULTS: One-, 3-, and 5-year overall survival rates were 100%, 85%, 79% (CT group), and 89%, 71%, 71% (no CT group), respectively. The HCV recurrence-free survival rates at 3, 6, and 12 months were 29%, 14%, 0% for the CT group, versus 76%, 38%, 25% for the no CT group (P =.005). CONCLUSIONS: Discontinuation of HCV-HCC patients by chemotherapeutic adjuvant protocols after transplantation appears rational due to the early hepatitis C recurrence confirmed in our series. Moreover, few studies have demonstrated that CT prolongs survival of HCC transplanted patients. New pharmacological approaches are necessary to solve these questions.

[2 cases of severe hypokalemia with acute rhabdomyolysis]. / Considerazioni su due casi di grave ipokaliemia con rabdomiolisi acuta.

We report two cases of acute rhabdomyolysis due to severe hypokalemia. The first case appears to be caused by a chronic dietary potassium deficiency, responsible also for the elevation of blood pressure and the impairment of myocardial contractility. The administration of hydrochlorothiazide, albeit associated with captopril, could have decompensated the potassium balance, thus precipitating the acute rhabdomyolysis. More commonly the second case is due to acute potassium losses induced by a trivial enteritis in a hypertensive patient who was taking thiazide diuretics for a long period of time. We emphasize that chronic dietary potassium deficiency may represent a predisposing variable to acute rhabdomyolysis, which is generally precipitated by the superimposition of other potassium lowering factors.

[Prognostic stratification of survivors of lst myocardial infarction: evaluation of regional kinetics and ejection fraction by bidimensional echocardiography]. / Stratificazione prognostica di pazienti sopravvissuti al primo infarto del miocardio mediante valutazione della cinetica regionale e della frazione di eiezione con ecocardiografia bidimensionale.

To determine the prognostic value of some echocardiographic indices of left ventricular function (ejection fraction, wall motion score index, left ventricular dimension) in the first year after acute myocardial infarction, we studied prospectively 162 consecutive patients (mean age: 61 +/- 11) who survived the hospital phase of a first acute myocardial infarction. Two-dimensional echocardiography was performed at hospital discharge (mean: 20 +/- 3 days after admission). For the analysis of wall motion, an 11 segment model of the left ventricle was used; from the scoring system of segmental ventricular function (1 = normal, 2 = hypokinetic, 3 = akinetic, 4 = dyskinetic, 5 = aneurysmal) we derived the wall motion score index (sum of assigned number to each segment/11). The echocardiographic ejection fraction was determined using the monoplane ellipsoid formula for the calculation of end diastolic and end systolic volumes in apical four-chamber and two-chamber views; the assumed ejection fraction was the mean value resulting from values of ejection fraction calculated in the two views. The follow-up was protracted from 13 to 36 months (mean: 22 months). Fourteen patients (9%) died as a result of cardiac events within 13 months of myocardial infarction. Of the patients with ejection fraction greater than or equal to 45% (81/162 = 50%) two died (first year mortality = 2.4%); of those with 35-45% ejection fraction (58/162 = 35%) two died (first year mortality = 3.5%); while of those with less than or equal to 35% ejection fraction (25/162 = 15%) ten died (first year mortality = 40%). Of the patients with wall motion score index less than 1.5 (76/162 = 47%) none died in the follow-up period; of those with score index between 1.5 and 1.9 (61/162 = 37%) four died (first year mortality = 7%); of those with score index greater than or equal to 1.9 (25/162 = 15%) ten died (first year mortality = 40%). Thus, ejection fraction and score index have the same predictive value for mortality in the first year after a first acute myocardial infarction. However, an interesting datum is that in the sub-group of patients with less than or equal to 35% ejection fraction and score index less than 1.9 the first year mortality was 15%, while in the sub-group with less than or equal to 35% but score index greater than or equal to 1.9 the first year mortality was 57%.(ABSTRACT TRUNCATED AT 400 WORDS)