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Congenital ascites as a presenting sign of lysosomal storage disease.

Neonatal ascites is usually attributed to hematologic, genitourinary, gastrointestinal tract, or congenital heart disease. When these lesions have been excluded, metabolic storage disorders should be considered in the differential diagnosis. We report eight cases of neonatal ascites associated with different types of lysosomal storage disease: infantile sialidosis, Salla disease, GM1 gangliosidosis, and Gaucher disease. In each case there was a history of sibling of perinatal death resulting from the disease. In three cases the diagnosis of ascites was made in utero by ultrasound examination. These diseases are characterized by excretion in the fetal urine of abnormal catabolic products or by measurement of decreased activity of specific lysosomal hydrolases in cultured amniocytes. Thin-layer chromatography of the oligosaccharides in amniotic fluid may be indicated when a diagnosis of persistent fetal ascites has been established.

Adult↗

Postmenarchal development of chylous ascites in acrocephalosyndactyly with congenital lymphatic dysplasia.

BACKGROUND: Acrocephalosyndactyly is a syndrome characterized by congenital malformation of the skull with craniosynostosis, midface hypoplasia, and symmetrical webbed fusion of the fingers and toes. We describe a possible pathophysiologic mechanism for chylous ascites that developed several months after menarche in a woman with acrocephalosyndactyly and congenital lymphatic dysplasia. CASE: A 25-year-old nulligravid woman with acrocephalosyndactyly, at 18 months after menarche, developed persistent abdominal distension at age 18 years. Laparoscopy at age 25 years revealed chylous ascites with marked chronic peritoneal inflammation, and lymphatic dysplasia with lymphocysts. With hormone manipulation, the chylous ascites fluctuated. CONCLUSION: After menarche in a woman with acrocephalosyndactyly, ovarian steroid hormones might have increased lymph production and hydrostatic pressure, causing rupture of congenitally dysplastic lymph vessels resulting in chylous ascites.

Acrocephalosyndactylia↗

Relationship between broiler chicken haematocrit-selected parents and their progeny, with regard to haematocrit, mortality from ascites and bodyweight.

A previous work of this group demonstrated that the relative haematocrit value of broilers is inherited and may serve as an indicator to susceptibility to the ascites syndrome in cold-stressed broilers. In this study, a full-pedigreed population was produced from male and female grandparent breeding stock that was selected by haematocrit and by normal selection parameters. Matings were made between low (L), medium (M) and high (H) haematocrit parents: L x L, M x M, and H x H. In their progeny, both before and after cold exposure, there was a statistically linear relationship between actual haematocrit and their H, M and L grouping (P<0.0001); heritability of the haematocrit was high (0.46-0.81). Both the low haematocrit parent and progeny groups showed an increased bodyweight. Exposure of the progeny from all the parental groups to an ascites-predisposing cold environment caused similar losses from ascites in the progeny of all three groups. Although this finding was not the same as in the previous trial where the H haematocrit group was associated with high ascites mortality, it is hypothesized that other factors, such as arterial blood saturation with oxygen, interacted in these birds at genetic or environmental levels.

Animals↗

Evidence for an unknown component of pancreatic ascites that induces adult respiratory distress syndrome through an interleukin-1 and tumor necrosis factor-dependent mechanism.

BACKGROUND: The development of acute respiratory distress syndrome (ARDS) during acute pancreatitis is associated with interleukin (IL)-1 and tumor necrosis factor (TNF) gene expression within the pulmonary parenchyma. Although activated pancreatic enzymes have been thought to mediate pancreatitis-induced ARDS, they are not capable of inducing cytokine production in vitro. We hypothesized that IL-1 and TNF production in the lungs is essential to the development of ARDS and is induced by a mediator released from the inflamed pancreas. METHODS: Pancreatic ascites was obtained from rats after induction of bile-salt pancreatitis, cultured, and assayed for IL-1, TNF, IL-6, IL-8, IL-10, interferon-gamma, and endotoxin. Sterile, cytokine-free ascites or saline (control) was subsequently administered intravenously (20 ml/kg) to healthy rats and to IL-1 R1 or TNF R1 knockout mice. RESULTS: Animals administered intravenous ascites had a 30-fold rise in pulmonary IL-1 and TNF mRNA, as well as increased alveolar leukocytes and protein. Knockout animals devoid of active IL-1 or TNF receptors failed to develop increased alveolar protein or leukocytes. CONCLUSIONS: A component of pancreatic ascites other than activated enzymes, bacteria, or inflammatory cytokines is capable of inducing ARDS in healthy animals. The mechanism appears to be directly attributable to the activity of pulmonary IL-1 and TNF.

Acute Disease↗

Comparative microsatellite analysis in discerning origin of disseminated tumor: the case of a patient with malignant ascites and a history of multiple tumors.

The origin of metastatic carcinoma is now always easily resolved on the basis of conventional dinical and pathological parameters, particularly in patients with more than 1 primary tumor. When 1 of the tumors is a renal cell carcinoma, the clinical picture is further confounded by the tendency of these tumors to be locally silent, to metastasize to unusual sites, and to disseminate long after removal of the primary tumor. We compared tumors for loss (ie, deletion) of loci on chromosomal arms 3p, 5q, 11q, and 18q in a patient with a malignant ascites fluid, a remote history of renal and colonic neoplasms, and a strong clinical suspicion of disseminated gastrointestinal adenocarcinoma. DNA from microdissected tumors and normal tissues was subjected to polymerase chain reaction-based microsatellite analysis. Even though the clinical picture suggested a gastrointestinal origin, comparison of genetic alterations clearly showed that the malignant ascites represented recurrence of the renal cell carcinoma. The malignant ascites and the primary renal cell carcinoma showed identical patterns of allelic loss at all loci tested. In contrast, the malignant ascites and colonic adenoma showed discordant patterns of allelic loss. Comparative microsatellite analysis provides a rapid genetic approach for discerning the origin of metastatic tumor spread. This may be a useful diagnostic adjunct when tumor origin is not clear on clinical or morphological grounds. In some instances, it may even provide a reasonable alternative to an extensive and costly conventional work-up.

Adenoma↗

Peritoneal venous shunting for the treatment of lymphatic ascites following retroperitoneal lymph node dissection.

We report a case of refractory lymphatic ascites following retroperitoneal lymph node dissection and venacavectomy. Placement of a Denver peritoneal venous shunt resulted in resolution of the ascites and marked improvement in the patient's nutritional parameters. Shunt occlusion 2 months following placement demonstrated no recurrence of the ascites. This technique may prove useful in the management of lymphatic ascites associated with radical retroperitoneal surgery.

Adult↗

Nadolol is superior to isosorbide mononitrate for the prevention of the first variceal bleeding in cirrhotic patients with ascites.

BACKGROUND/AIMS: beta-blockers effectively prevent first variceal bleeding (FVB) in cirrhotic patients. In patients with ascites, however, their use might be precluded by a high rate of contraindications and side effects. We compared the efficacy and applicability of nadolol and isosorbide-mononitrate (IsMn) in preventing FVB in a population of cirrhotic patients at high risk of variceal bleeding with ascites, who can be frequently intolerant to beta-blockers. METHODS: A total of 80 consecutive cirrhotic patients with ascites and esophageal varices (25% average risk of bleeding at 1 year) were considered, 28 were excluded due to contraindications and 52 were randomly assigned to receive nadolol (n=25) or IsMn (n=27). RESULTS: Frequency of contraindications was greater for beta-blockers than IsMn (35 versus 0%, P=0.001). During 21.3+/-11.6 months of follow-up, side effects forced six patients taking nadolol and four taking IsMn to stop treatment. Bleeding occurred in two patients taking nadolol and ten taking IsMn. The probability of bleeding was significantly lower in the nadolol group (P<0.05), whereas overall survival was similar (seven patients on IsMn and eight on nadolol died, P=0.3). CONCLUSIONS: In patients with ascites IsMn is tolerated but ineffective while nadolol is effective but less tolerated.

Adolescent↗

Medical treatment of ascites in cirrhosis.

Medical treatment of cirrhotic ascites is essentially supportive, dictated by the patient's discomfort, impaired cardiovascular or respiratory function and potential for infection. Treatment of 'simple' ascites (moderate fluid accumulation, serum albumin > 3.5 g/dl, serum creatinine < 1.5 mg/dl, no electrolyte disturbance) is implemented sequentially. Only 10% of patients respond to dietary sodium restriction and bed rest; most require pharmacotherapy consisting of spironolactone, which increases the proportion of responding patients to 65% and loop diuretics, which may produce clinical improvement in an additional 20% (85% in all); in the remaining 15% of refractory patients, use of novel adjunctive therapies may be attempted. Patients with tense ascites, impaired renal function and electrolyte disturbances merit special consideration before diuretics are introduced. Spironolactone has long been a standard for the treatment of cirrhotic ascites because it directly antagonizes aldosterone. The loop diuretic most frequently added to spironolactone has been furosemide. However, there is preliminary evidence that torasemide may be more effective in some patients. Other investigational agents that may play a role in treatment of patients resistant to conventional drugs include ornipressin (a vasopressin analogue) and atrial natriuretic factor.

Ascites↗

Effect of large-volume paracentesis on pulmonary function in patients with cirrhosis and tense ascites.

The effect of large-volume paracentesis on lung function was evaluated in 12 male patients with cirrhosis. All underwent pulmonary function tests including spirometry, plethysmography and single-breath carbon-monoxide diffusing capacity 1 day before and after paracentesis. The amount of ascitic fluid removed ranged from 3.6 to 131 (mean +/- SD, 7.4 +/- 3.01). After paracentesis, forced vital capacity, forced expiratory volume at 1 s, total lung capacity, functional residual capacity, inspiratory capacity, expiratory reserve volume, diffusing capacity and alveolar volume increased significantly. In contrast, Kco (diffusing capacity corrected by alveolar volume) decreased significantly. After paracentesis, the increase in diffusing capacity was highly correlated with lung volumes and the amount of removed ascitic fluid. Nevertheless, a significantly negative correlation was found between the change of Kco before and after paracentesis and that of lung volumes. The increase in lung volumes and ventilation to the lower lungs with unfavorable ventilation-perfusion matching might explain the discrepancy between changes in diffusing capacity and Kco after large-volume paracentesis. In conclusion, these results suggest that pulmonary function in patients with cirrhosis and tense ascites is partly improved by large-volume paracentesis. Large-volume paracentesis might be useful for symptomatic relief in selected patients with tense ascites.

Ascites↗

Daily profile of plasma endothelin-1 and -3 in pre-ascitic cirrhosis: relationships with the arterial pressure and renal function.

BACKGROUND/AIMS: Measurements of plasma endothelin-1 and -3 in pre-ascitic cirrhosis have provided controversial results. Similarly, the role of the endothelin system in the pathogenesis of volume and hemodynamic disturbances of cirrhosis is still debated. To provide a further insight into this issue, we assessed the daily fluctuations of plasma endothelins and their relationship with arterial pressure and renal function in pre-ascitic cirrhosis. METHODS: Endothelin-1 and -3, plasma renin activity, atrial natriuretic peptide, noradrenaline and mean arterial pressure were measured at 11 pm, 7 am, 9 am and 6 pm in 10 patients with pre-ascitic cirrhosis and in 10 healthy subjects on normal sodium diet and carrying on their usual activities (supine from 10 pm to 7 am, standing and mobile after 7 am). Glomerular filtration rate and daily renal sodium excretion were assessed during the supine period, and from 7 am to 12 am and from 12 am to 10 pm during the standing period. RESULTS: Endothelin-1 was higher in patients than in control subjects (p=0.000) and did not change during the study. Endothelin-3 was also higher in patients (p=0.002) and showed slight fluctuation in control subjects. The mean daily level of plasma renin activity was lower (p=0.016) and that of atrial natriuretic peptide higher (p=0.000) in patients with cirrhosis. Norepinephrine and mean arterial pressure did not differ significantly between the two groups. No correlations were found between endothelins and either hemodynamic or neuro-hormonal and renal function parameters in the two groups. CONCLUSIONS: Despite the presence of increased effective volemia (as suggested by the reduced plasma renin activity and elevated atrial natriuretic peptide) and normal adrenergic tone, patients with pre-ascitic cirrhosis show elevated levels of endothelin-1 and endothelin-3 throughout the day. In early cirrhosis circulating endothelins, although elevated, do not appear to play a more prominent role in setting arterial pressure than in normal subjects, and endothelin elevation is not detrimental to renal function.

Adult↗

Natriuretic response to the combination of atrial natriuretic peptide and terlipressin in patients with cirrhosis and refractory ascites.

BACKGROUND/AIMS: Refractory ascites, which occurs in certain patients with cirrhosis, is associated with a blunted natriuretic response to exogenous atrial natriuretic peptide (ANP). Since this blunting seems to be related to ANP-induced arterial hypotension, a vasoconstrictor, such as terlipressin (a vasopressin analogue), may restore natriuresis to exogenous ANP. Moreover, since cirrhosis-elicited vasodilation is thought to play a role in sodium retention, a vasoconstriction caused by terlipressin alone may lead to an increase in sodium excretion. This study aimed to evaluate the natriuretic response to either a combination of ANP with terlipressin or terlipressin alone in patients with cirrhosis and refractory ascites. METHODS: Sixteen consecutive patients with cirrhosis and refractory ascites were randomly assigned to receive either a combination of terlipressin (1-2 mg, i.v. bolus) with ANP (35 ng/kg, i.v. bolus followed by 15 ng x kg(-1) x min(-1) for 60 min) (n=8) or terlipressin alone (1-2 mg, i.v. bolus) (n=8). Sodium excretion and urine output, systemic, splanchnic and renal hemodynamics and renal oxygen consumption were measured before and during treatments. RESULTS: Combined therapy did not change arterial pressure but significantly increased urinary sodium excretion and urine output. These effects were associated with a significant increase in glomerular filtration rate and a decrease in renal oxygen consumption. Terlipressin alone significantly increased arterial pressure but did not change urinary sodium excretion or urine output. Moreover, terlipressin did not change either glomerular filtration rate or renal oxygen consumption. CONCLUSIONS: The combination of exogenous ANP with terlipressin, but not terlipressin alone, increases sodium excretion in patients with cirrhosis and refractory ascites.

Adult↗

Norfloxacin primary prophylaxis of bacterial infections in cirrhotic patients with ascites: a double-blind randomized trial.

BACKGROUND/AIMS: Norfloxacin is useful to prevent infections in hospitalized cirrhotic patients with low ascitic fluid protein concentrations. It is also effective in preventing the recurrence of spontaneous bacterial peritonitis. The aim of our study was to determine the efficacy of norfloxacin in the primary prophylaxis of gram-negative bacilli infections in cirrhotic patients with low ascitic fluid protein levels (<15 g/l). METHODS: One hundred and seven patients were randomized to receive norfloxacin (400 mg/day; n=53) or placebo (n=54) for 6 months. The patients had no history of infection since cirrhosis diagnosis and no active infection. RESULTS: The probability of gram-negative infection was significantly lower among patients treated with norfloxacin than among those treated with placebo. Six gram-negative bacilli infections occurred in the placebo group and none in the treatment group. Severe infections (spontaneous bacterial peritonitis, neutrocytic ascites and bacteremia) developed in nine patients in the placebo group (17%) and in one patient in the norfloxacin group (2%; p<0.03). There was no between-group difference in the overall rate of infection or in survival. In ten patients from the norfloxacin group, gram-negative bacilli not present in baseline stool cultures were transiently isolated in follow-up cultures. CONCLUSIONS: These data show that primary prophylaxis with norfloxacin for 6 months is effective in the prevention of infections caused by gram-negative bacilli in cirrhotic patients with low ascitic fluid total protein levels.

Adult↗

Early effects of total paracentesis and albumin infusion on muscle sympathetic nerve activity in cirrhotic patients with tense ascites.

BACKGROUND/AIMS: Cirrhotic patients with ascites are characterized by a marked activation of the sympathetic and the renin-angiotensin-aldosterone system. Total paracentesis is associated with a short-lived suppression of the renin-angiotensin-aldosterone system. Little information exists as to whether this favourable effect is parallelled by sympathoinhibition. METHODS: In 16 Child C cirrhotic patients (age: 57.1+/-6.2 years, mean+/-SEM) with tense ascites we assessed the time course of the effects of total paracentesis followed by intravenous albumin (6-8 g/l of ascites) on beat-to-beat mean arterial pressure (Finapres), heart rate, plasma norepinephrine, epinephrine (high performance liquid chromatography) and muscle sympathetic nerve activity (microneurography, peroneal nerve). Measurements were obtained under baseline conditions, during staged removal of ascitic fluid (250 ml/min) and 24 h later. The patient remained supine throughout the study period. RESULTS: Total paracentesis (10.6+/-1.3 l) induced a decrease in mean arterial pressure (from 95.0+/-2.6 mmHg to 88.2+/-3.2 mmHg, p<0.01), in heart rate (from 82.5+/-3.3 beats/min to 77.1+/-2.8 beats/min, p<0.01) and a reduction in plasma norepinephrine values (from 782+/-133 pg/ml to 624+/-103 pg/ml, p<0.01), which were substantially maintained 24 h later. In eight patients muscle sympathetic nerve activity did not change during paracentesis (from 65+/-7.1 bursts/min to 65+/-7.4 bursts/min, p=NS), but a marked reduction was observed 24 h later (48.4+/-5.6 bursts/min, p<0.01). CONCLUSIONS: These data provide the first evidence that total paracentesis exerts an acute marked sympathoinhibitory effect. Whether this is a long-lasting phenomenon and to what extent plasma expansion with albumin contributes to this effects need to be further addressed.

Adult↗

Chylous ascites after radical nephrectomy and inferior vena cava thrombectomy. Successful conservative management with somatostatin analogue.

Postoperative chylous ascites is a rare complication of retroperitoneal surgery. The treatment of postoperative chylous ascites is primarily conservative, consisting of repeated paraceteses, medium chain triglyceride (MCT) diet, salt restriction, diuretics and bowel rest with total parenteral nutrition. Occasionally, chylous ascites may take a protracted course which may necessitate insertion of peritoneo-venous shunts or direct surgical lymphostasis. Recently, Somatostatin was shown to be highly effective in closure of refractory lymphatic fistulas. We present a case of refractory chylous ascites following radical nephrectomy with inferior vena caval thrombectomy that failed to respond to conventional conservative measures and resolved rapidly following the administration of Somatostatin.

Chylous Ascites↗

Effects of recombinant human serum albumin on ascites in rats with puromycin aminonucleoside-induced nephropathy.

1. Recombinant human serum albumin (rHSA) (1 g/kg) significantly decreased the weight of ascites in rats with puromycin aminonucleoside-induced nephropathy. 2. Furosemide (1-30 mg/kg) did not significantly reduce the weight of ascites in this model. 3. A combination of rHSA (1 g/kg) with furosemide (5 mg/kg) significantly decreased the weight of ascites in this model compared with furosemide alone. 4. In consideration of these results, rHSA can be a substitute for human serum albumin products prepared from human plasma in therapy for ascites or edema in furosemide-resistant nephrotic syndrome.

Animals↗

Effectiveness of recombinant human serum albumin in the treatment of ascites in liver cirrhosis: evidence from animal model.

1. To investigate the effectiveness of recombinant human serum albumin (rHSA) in the treatment of ascites in liver cirrhosis, we examined its effect on rats with carbon tetrachloride-induced liver cirrhosis. 2. Twenty-five percent rHSA was administered intravenously at a dose of 0.25 to 1.0 g/kg for 2 days to rats with liver cirrhosis accompanied by ascites retention and hypoalbuminemia. 3. rHSA dose dependently decreased abdominal circumference, a clinical index of ascites, with significant difference at a dose of 1.0 g/kg. 4. Although there was no significant difference, rHSA increased blood colloid osmotic pressure (b-COP) and urine volume (UV) in a nearly dose-dependent manner, with significant negative correlation between changes from baseline value in these parameters and in abdominal circumference. 5. These findings suggest that rHSA has abdominal circumference-decreasing action associated with b-COP improvement and UV increase and that it could be effective as a therapeutic drug for ascites in patients with liver cirrhosis accompanied by hypoalbuminemia.

Animals↗

Comparative pilot study of repeated large volume paracentesis vs the combination on clonidine-spironolactone in the treatment of cirrhosis-associated refractory ascites.

OBJECTIVES: To study the usefulness of the combination of clonidine--spironolactone in refractory ascites. METHODS: Twenty cirrhotic patients with refractory ascites were randomly assigned to receive repeated large volume paracentesis plus intravenous albumin (group 1), or a combination of clonidine (0.075 mg twice daily) and spironolactone (200 to 400 mg daily) (group 2). RESULTS: During the first hospitalisation,, the mean weight loss in group 1 was higher than in group 2 (12.4 +/- 3.2 versus 4.3 +/- 1.1 kg, P < or = 0.01). Mean stay in hospital was shorter in group 2 (20 +/- 1.5 versus 10 +/- 2.8 days; P < or = 0.01). Paracentesis did not induce changes in neuro-hormonal measurements. Oppositely, clonidine induced a decreased sympathetic activity, an increased glomerular filtration rate and a delayed reduction of the renin-aldosterone levels. During the follow-up in group 1, the number of rehospitalisations for ascites was higher than in group 2 (37 versus 3; P < or = 0.01), and the mean time to the first readmission was shorter (10 +/- 2.7 versus 23.7 +/- 5.6 days; P < or = 0.01). The total duration spent in hospital were similar in both groups. CONCLUSION: Paracentesis is more effective for short-term treatment of ascites but clonidine-spironolactone association might provide better long-term control.

Adrenergic alpha-Agonists↗

Malignant ascites: a 2-year review from a teaching hospital.

In this retrospective review of 164 consecutive patients with malignant ascites it has been shown that ovarian ascites accounts for 28% of the total and is associated with a significantly improved survival compared with other groups (P<0.001). Non-ovarian ascites is associated with a very poor prognosis and many patients in this group are unsuitable for aggressive treatment. In 49% of patients, ascites will be the presenting feature requiring further investigation to ascertain the primary tumour. Thorough investigation of female patients should be performed in order to identify all patients with an ovarian primary so that appropriate chemotherapy can be given. However, thorough investigation in male patients is not justifiable.

Adult↗