[Nutcracker syndrome: a difficult case of recurrent gross hematuria]. / Sindrome di nutcracker: una macroematuria intermittente di difficile diagnosi.

BACKGROUND: The Nutcracker Syndrome (NS) is an uncommon clinical condition caused by compression and entrapment of the renal vein (LRV) as it passes through the angle between the superior mesenteric artery (SMA) and the aorta (meso-aortic angle). Intermittent macrohematuria, left peripelvic and gonadal vein varices and aspecific abdominal pain may be common manifestations of this syndrome. CASE-REPORT: A 15-year-old white boy developed recurrent macrohaematuria in June 2002. He had a history of upper respiratory infection prior the first episode of gross hematuria followed by 4 other episodes of macrohematuria 'sine causa'. Blood pressure and renal function were normal. Routine laboratory tests showed only an increase in serum LDH levels (901 IU/L) with negative Coombs' test, both direct and indirect, and absence of schistocytes in the blood smear. Renal ultrasonography showed normal kidneys while an intravenous pyelography showed a 'minus' in the right ureter. For this reason, a cystoscopy and a retrograde pielography were performed but resulted normal. A renal biopsy was carried out because of the presence of one episode of post-infective macrohaematuria, but light microscopy and immunofluorescence examinations were found to be normal. Renal ultrasonography and Color Doppler ultrasonography (CD-USG) oriented our diagnosis towards NS. An abdominal computerised tomography (CT) scan confirmed that the LRV was compressed between the aorta and the SMA. CONCLUSIONS: NS cannot be diagnosed with routine diagnostic methods. Endoscopic and radiological methods may provide some clues for the presence of NS, while CD-USG may be considered to be the first level non-invasive method for diagnosis. The sensitivity and specificity of this test for diagnosing NS is reported as being 78% and 94%, respectively. The best treatment available for this syndrome is still being debated.

Haemodynamic effects of propranolol, octreotide and their combination during fasting and post-prandial splanchnic hyperaemia in patients with cirrhosis.

BACKGROUND/AIMS: This double-blind study was designed to evaluate the haemodynamic effect of two drugs, propranolol and octreotide, and their combination in patients with cirrhosis. METHODS: Fifteen patients with cirrhosis were randomly assigned to two groups receiving either octreotide subcutaneously at 100 microg ('octreotide' group, n = 9) or propranolol orally at 40 mg followed by a subcutaneous dose of octreotide (100 microg) after 1 h ('propranolol + octreotide' group, n = 6); then, after 30 min, a standard meal was administered to both groups. The hepatic vein pressure gradient by hepatic vein catheterization, portal and superior mesenteric artery blood flow velocity, superior mesenteric artery pulsatility index by the echo-Doppler duplex system were recorded at baseline, 1 h after propranolol in the 'propranolol + octreotide' group, and in both groups 30 min after octreotide and 30 min after meal. RESULTS: At fast, propranolol was more active in decreasing portal pressure (from 16 +/- 2.2 to 12.7 +/- 3.8 mmHg, -20%, P < 0.05) as compared to octreotide (from 18.6 +/- 4.8 to 16.6 +/- 4.3 mmHg, -11%, P < 0.05). Conversely, octreotide was more active on the mean blood flow velocity of superior mesenteric artery (from 22.8 +/- 5 to 19 +/- 4.5 cm/ s, -17%; P< 0.05). Octreotide administration in patients receiving beta-blockers showed, also, a trend to increase the mesenteric vascular resistances (pulsatility index from 3.14 +/- 0.69 to 3.68 +/- 1.29, +17%, not significant (NS)) which had not been affected by previous treatment with propranolol. After the meal, a reduction of the expected hyperaemic response occurred in both groups. CONCLUSIONS: The combined acute haemodynamic effect of this association suggests the possible combination of these two drugs in critical situations, such as variceal bleeding in patients receiving beta-blockers. The simultaneous use of echo-Doppler and hepatic vein catheterization permitted us a more complete analysis of the acute haemodynamic events.

Octreotide blunts postprandial splanchnic hyperemia in cirrhotic patients: a double-blind randomized echo-Doppler study.

The effect of octreotide, a long-acting synthetic analog of somatostatin, on fasting and postprandial splanchnic hemodynamics was investigated in cirrhotic patients. Splanchnic hemodynamics were assessed using an echo-Doppler duplex system in a prospective, double-blind, placebo-controlled, crossover study performed on 2 separate days, 1 week apart, in 30 cirrhotic patients. Measurements of portal vein (PV) cross-sectional area (PV-A) and mean velocity (PV-V), and of superior mesenteric artery (SMA) mean velocity (SMA-V) and pulsatility index (SMA-PI) (an index of vascular resistance) were performed at baseline, 30 minutes after octreotide (200 micrograms subcutaneously) or placebo administration, and 30 and 60 minutes after the ingestion of a liquid meal. In the fasted state, octreotide induced a significant decrease in PV-V (-7%) and in SMA-V (-10%) and an increase in PI (+16%). On the day of placebo administration, meal ingestion induced a significant increase in PV-V (+21%) and in SMA-V (+43%) and a decrease in PI (-21%). In contrast, meal ingestion on octreotide day induced significantly smaller increases in PV-V (+10%) and in SMA-V (+18%) and a significantly smaller decrease in PI (-10%). Octreotide, although producing a mild reduction in PV-V and SMA-V in the fasted state, markedly blunts postprandial splanchnic hyperemia in cirrhotic patients.

Comparison between the effect of L-propionylcarnitine, L-acetylcarnitine and nitroglycerin in chronic peripheral arterial disease: a haemodynamic double blind echo-Doppler study.

The haemodynamic effects on the peripheral vascular bed of L-acetylcarnitine, L-propionylcarnitine, and nitroglycerin were tested by echo-Doppler in a double blind cross-over study. Eleven male patients suffering from peripheral arterial obliterative disease (PAOD) in the second stage of Fontaine's classification, and 11 matched control subjects were enrolled in the study. Each subject received one of three different treatments on each day of the study in a different order following a random assignment. The treatments were either 30 mg x kg of L-acetylcarnitine (LAC) or 30 mg x kg of L-propionylcarnitine (LPC) or nitroglycerin (NTG) 1.25 mg given as a single i.v. bolus injected over 3 min. Echo-Doppler measurements of blood flow velocity, and cross-sectional area of the femoral artery were performed at baseline and 10, 20, and 30 min after injection of the drugs. Pulsatility Index (an index derived from the blood flow velocity and related to vascular resistance: PI = Vmax - Vmin/Vmean) was also obtained each time. Results were analysed using a Student's t-test for paired data. L-acetylcarnitine and L-propionylcarnitine showed no haemodynamic effects in either group of subjects (controls and PAOD patients) whether blood flow or vascular resistance was considered. There were haemodynamic changes (a decrease in blood flow velocity and an increase in arterial systemic resistance) only after NTG administration. The changes were more evident in controls than in PAOD patients. Femoral artery cross-sectional area showed no statistically significant effect as regards treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

[Echo-color Doppler in the study of portal hypertension]. / Eco color-Doppler nello studio dell'ipertensione portale.

Color-Doppler US is currently an extremely valuable tool in the study of abdominal circulation, cirrhosis of liver and its complications, different kinds of portal hypertension, vascular/avascular liver malformations and, finally, in the evaluation of liver transplants. Moreover, its use in experimental studies has yielded good results. Even spontaneous porto-systemic shunts can be demonstrated in most cases (up to 88%). Color-Doppler allows partial obstructions to be demonstrated and permits qualitative and quantitative dynamic evaluations the presence/absence of flow, vascular masses--e.g., mean velocity measurement and blood flow assessment. The interobserver variability of the method was calculated with a double-blind study carried out by our laboratory team cooperating with a study group from the University of Yale, USA. The study evidenced how color-Doppler US cannot be used to follow the single patient unless the variations to measure are higher than variability itself. Color-Doppler limitations depend on the subjectivity of the results and on its difficult application to obese patients or to those with severe intestinal meteorism.

Echo-Doppler evaluation of acute flow changes in portal hypertensive patients: flow velocity as a reliable parameter.

In order to evaluate the behavior of the portal vein cross-sectional area during changes in portal flow, two groups of subjects were analyzed in two blinded cross-over studies using echo-Doppler flowmetry. The first group (I) consisted of 21 patients with cirrhosis and 16 controls. They received a standardized meal which is known to increase portal flow. The second group (II) consisted of 31 patients with cirrhosis who received a dose of propranolol which is known to decrease portal flow. In Group I, 30 min after the meal, the portal vein blood velocity increased by 35 +/- 6% (p less than 0.01) in cirrhotic patients and by 55 +/- 5% (p less than 0.01), in normal subjects. The portal vein cross-sectional area increased significantly in normal subjects (22 +/- 2%, p less than 0.01) but not in cirrhotic patients (4 +/- 2%, n.s.). In Group II, 2 h after propranolol, there was a significant decrease in portal blood velocity (-14 +/- 2%), whereas the portal vein cross-sectional area did not show any significant changes. These data demonstrate that, in portal hypersensitive patients, the portal area measured by echo-Doppler flowmetry can be assumed to be constant and hence its calculation to estimate changes in portal blood flow can be omitted. Therefore, the use of blood velocity alone is suggested to monitor acute changes in flow in portal hypertension using Doppler flowmetry. The elimination of the portal vein cross-sectional area measurement simplifies the quantitative calculation of portal hemodynamics and increases the reliability of the technique by avoiding a source of error.

Acute systemic and splanchnic haemodynamic effects of L-carnitine in patients with cirrhosis.

L-carnitine (L-C) is a naturally occurring substance in mammalian tissues that has recently been proposed as a therapeutic agent in hepatic encephalopathy and liver steatosis. L-C also produces some acute, non-metabolic, haemodynamic effects that have not previously been studied in patients with cirrhosis. Therefore, the authors evaluated the acute effect of i.v. administration of L-C (30 mg/kg) on systemic and splanchnic haemodynamics in ten patients (L-C group) with chronic liver disease (Child-Pugh's class A: 4, B: 3, C: 3 patients) and a control group composed of ten patients with similar clinical characteristics (Child-Pugh's class A: 5, B: 2, C: 3 patients) who received placebo. Heart rate, cardiac index and pulmonary arterial pressure (measured by right heart catheterization) decreased slightly but significantly in the L-C group and the changes observed in stroke volume were highly correlated to the Pugh's score. Moreover, the hepatic venous pressure gradient (measured by hepatic vein catheterization) decreased significantly in the L-C group, whereas no changes occurred in the placebo group. The overall response to L-C was contradictory to that previously observed in animals and humans with normal liver function, and the extent seemed to depend on the severity of liver disease. The effect of the drug on cardiac index, heart rate and hepatic venous gradient could possibly be beneficial for patients with hyperdynamic circulatory condition and portal hypertension.