Intestinal lymphangiectasia: value of double-contrast radiographic study

Intestinal lymphangiectasia is a disorder presenting as enteric protein loss through the dilated lymphatics without mucosal ulceration. To determine the double-contrast radiographic features and to assess the significance of them, five patients with intestinal lymphangiectasia were examined using single- and double-contrast small intestinal studies. The double-contrast examinations demonstrated clearly the main radiographic findings of smooth nodular protrusions, thickening of the mucosal folds, with no evidence of mucosal ulceration. Compared with the single-contrast study, smooth nodular protrusions were seen more often and in more widespread segments, particularly in the duodenum, on double-contrast study. Thickening of the mucosal folds was revealed similarly by both methods. Double-contrast study appears to be worthwhile to demonstrate the characteristic radiographic findings of this disease.     

Results of surgical therapy of ovarian carcinomas in 1948-68]

Bisalbuminaemia in pancreatitis is a transient abnormality related to the presence, on electrophoresis of the serum, of a fast-moving albumin; this abnormal form is also found, in large amounts, in the ascitic or pleural effusions of the patients. Experiments reported here indicate clearly that the fast albumin can be produced by a degradation of normal serum albumin by the proteolytic enzymes of the pancreas (chymotrypsin or elastase in association with carboxypeptidases A and B). Stuctural analysis of the isolated fast albumin of the patients shows that the C-terminal end of the molecule is different from normal serum albumin, which can be understood by a limited enzymatic degradation by chymotrypsin or elastase followed by the action of carboxypeptidases. The discovery of bisalbuminaemia in a patient affected by pancreatitis is suspicious of the presence of an ascitic or pleural effusion and of a pancreatic pseudo-cyst with a fistula emerging in the effusion.     

Hennekam syndrome

Hennekam syndrome is a disorder comprising intestinal lymphangiectasia, facial anomalies and moderate mental retardation. Eight cases have been previously reported. CASE REPORT: A 17-month-old girl was admitted to hospital for peripheral edema. On physical examination, she presented with a normal mental development. Facial anomalies were noted including a flat face, depressed and broad nasal bridge, puffy eye lids, mild down-slanting palpebral fissures, hypertelorism, epicanthal folds, bulbous nasal tip, small mouth, and low set ears. A simian line and haemangiomas on the arms, trunk and left limb were also noted. There was no organomegaly. Laboratory investigations showed iron deficiency anemia, hypoproteinemia, hypogammaglobulinemia and an elevated level of alpha-1 antitrypsin excreted in the feces. Endoscopic investigation and the small bowel biopsy showed findings consistent with lymphangiectasia. The patient did well on 24 hour enteral nutrition including medium-chain triglyceride rich diet and infusion of human albumin. CONCLUSION: We have aimed to remind that Hennekam syndrome should be included in differential diagnosis when intestinal lymphangiectasia are associated with facial anomalies.     

Intestinal mucosal inflammation associated with human immunodeficiency virus infection

The role of the human immunodeficiency virus type-1 (HIV) in producing intestinal disease was studied prospectively in 74 HIV-infected individuals with (43) or without (31) the acquired immunodeficiency syndrome (AIDS). Thirty-one subjects had enteric infections; all but one had AIDS. Alteration in bowel habits was the most common symptom and occurred independently of enteric infections. Abnormal histopathology was present in 69% of cases, and the finding was associated with altered bowel habits. An HIV-associated protein, p24, was detected in 71% of biopsies by ELISA assay. Tissue p24 contents varied with disease stage and were highest in HIV-infected individuals without AIDS (Walter Reed classes 3 and 4). Tissue p24 detection was associated with both altered bowel habits and histologic mucosal abnormalities. Tissue contents of the cytokines, tumor necrosis factor-alpha and interleukin-1 beta, were higher in HIV-infected individuals than in controls and their elevations were independent of enteric infection. We conclude that HIV reactivation in the intestinal mucosa may be associated with an inflammatory bowel syndrome in the absence of other enteric pathogens.     

Primary congenital pulmonary lymphangiectasia--a case report

We report on a 22 year-old women with recurrent pleural effusions and shadowing of the right lower lobe, which was refractory to antibiotic treatment. Histologic examination (open lung biopsy) was interpretated as indicating an early stage of lymphangioleiomyomatosis. Because of progression of the pulmonary changes and development of a pericardial effusion in spite of antiestrogen treatment to achieve pharmacological castration, and in view of the atypical findings in high resolution computed tomography (lack of cysts) a second open lung biopsy was performed, confirming the diagnosis of pulmonary lymphangiectasia. The patient was given oral corticosteroids postoperatively and showed almost complete resolution of the pleural and pericardial effusions.     

Pleural exudates and transudates: diagnosis with contrast-enhanced CT

PURPOSE: To determine the accuracy of computed tomography (CT) in enabling differentiation of pleural exudates from transudates. MATERIALS AND METHODS: Eighty consecutive patients (86 effusions) underwent contrast-enhanced CT. Thoracentesis was performed to measure pleural and serum total protein and lactate dehydrogenase (LDH) values. Effusions were classified as exudates with accepted criteria. CT scans were evaluated for the presence and appearance of parietal pleural and extrapleural fat thickening. RESULTS: Fifty-nine effusions were exudates and 27 were transudates. Thirty-six of the 59 exudates (61%) were associated with parietal pleural thickening. All cases of empyema and 56% of the parapneumonic exudative effusions had pleural thickening. The specificity of this finding in diagnosing the presence of an exudate is 96%. CONCLUSION: Parietal pleural thickening at contrast-enhanced CT almost always indicates the presence of a pleural exudate. A pleural exudate in the absence of pleural thickening occurs most frequently in patients with malignancy or uncomplicated parapneumonic effusion.     

Octreotide for treatment of postoperative alimentary tract fistulas

Eighteen patients with postoperative fistulas of the gastrointestinal tract were treated with the somatostatin analog octreotide between November 1989 and November 1992. Fourteen patients had enterocutaneous fistulas: seven from the duodenum and seven from the ileum. Another three patients had pancreatic fistulas, and one patient had a biliary fistula. Within 24 hours of octreotide treatment, a mean reduction of 52% in the intestinal fistulas' output, 40% in the pancreatic fistulas, and 30% in the biliary fistula was noted. In the intestinal fistulas group the closure rate was 72% after a mean of 11 days. Early closure (mean 6 days) was achieved in all three pancreatic fistulas. In the patient with the biliary fistula a 30% reduction was observed twice following the administration of octreotide, and an increase occurred when it was withheld. The reduction rate of the secretions in high-output intestinal fistulas (> 500 ml/day) was higher than in the low-output fistulas (63 +/- 8% versus 39 +/- 4%, p < 0.05). Fistula output and the initial response to octreotide treatment had no value in predicting spontaneous healing. In conclusion, octreotide is a valuable tool for the conservative treatment of fistulas of the digestive tract. It is especially valuable for management of high-output enteric fistulas and pancreatic fistulas.     

Prenatal diagnosis of a fetal intracranial tumor

Cap polyposis is a rare intestinal disease that can be difficult to differentiate from inflammatory bowel disease. When cap polyposis is suspected, it is important to confirm protein loss. A 54-year-old woman who had been treated for ulcerative colitis for 7 years had severe hypoproteinemia. Scintigraphy with Tc-99m-labeled DTPA complexed with human serum albumin showed protein loss from the descending colon. Left hemicolectomy and sigmoid colectomy were performed. Cap polyposis was diagnosed on the basis of histologic findings from an operative specimen. The patient's diarrhea resolved after surgery and her hypoproteinemia improved. Scintigraphy with this label gave information helpful in the diagnosis of cap polyposis.     

The distribution and enteric loss of 51Cr-labelled lymphocytes in normal subjects and in patients with coeliac disease and other disorders of the small intestine

Peripherally harvested lymphocytes have been labelled with 51Cr, reinjected into human subjects and their distribution then studied. Evidence is presented which suggests faecal loss of 51Cr represents loss of T lymphocytes and that there is normally a pathway of lymphocyte removal into the gut of probable importance in lymphocyte migration streams. In 9 normal subjects, without structural intestinal disease, faecal loss of lymphocytes over 5 days was 0.20% (SEM +/- 0.06) whereas in 5 patients with untreated coeliac disease faecal loss was 1.13 +/- 0.34%, in 7 with Crohn's disease it was 1.01 +/- 0.21% and in 5 with intestinal lymphangiectasia loss was 0.61 +/- 0.10%. In 1 patient with acute tropical sprue, enteric loss was 0.97%. By contrast, faecal loss was normal in 3 coeliac patients in remission on a gluten-free diet. Measurements were also made using an external counter. In contrast to the normals, where count rates steadily diminished, an increasing activity was recorded over the umbilicus over 7 days after dose administration in all the disease categories studied with the exception of the treated coeliacs. The finding of an increased enteric loss of lymphocytes may explain many of the immunological abnormalities in the conditions studied.     

Pleural effusions in the medical ICU: prevalence, causes, and clinical implications

OBJECTIVE: To determine the prevalence and causes of pleural effusions in patients admitted to a medical ICU (MICU). DESIGN: Prospective. SETTING: MICU in a tertiary care hospital. PATIENTS: One hundred consecutive patients admitted to the MICU at the Medical University of South Carolina whose length of stay exceeded 24 h had chest radiographs reviewed daily and chest sonograms performed within 10 h of their latest chest radiograph. RESULTS: The prevalence of pleural effusions in 100 consecutive MICU patients was 62%, with 41% of effusions detected at admission. Fifty-seven of 62 (92%) pleural effusions were small. Causes of pleural effusions were as follows: heart failure, 22 of 62 (35%); atelectasis, 14 of 62 (23%); uncomplicated parapneumonic effusions, seven of 62 (11%); hepatic hydrothorax, five of 62 (8%); hypoalbuminemia, five of 62 (8%); malignancy, two of 62 (3%); and unknown, three of 62 (5%). Pancreatitis, extravascular catheter migration, uremic pleurisy, and empyema caused an effusion in one instance each. Heart failure was the most frequent cause of bilateral effusions (13/34 [38%]). When compared with patients who never had effusions during their MICU stay, patients with pleural effusions were older (54+/-2 years, mean+/-SEM, vs 47+/-2 years [p=0.04]), had lower serum albumin concentration (2.4+/-0.1 vs 3.0+/-0.01 g/dL [p=0.002]), higher acute physiology and chronic health evaluation II scores during the initial 24 h of MICU stay (17.2+/-1.1 vs 12+/-1.2 [p=0.010]), longer MICU stays (9.8+/-1.0 vs 4.6+/-0.7 days [p=0.0002]), and longer mechanical ventilation (7.0+/-1.3 vs 1.9+/-0.7 days [p=0.004]). No patient died as a direct result of his or her pleural effusion. Chest radiograph readings had good correlation with chest sonograms (p<0.0001). CONCLUSION: Pleural effusions in MICU patients are common, and most are detected by careful review of chest radiographs taken with the patient in erect or semierect position. When clinical suspicion for infection is low, observation of these effusions is warranted initially, because most are caused by noninfectious processes that should improve with treatment of the underlying disease.     

Behavioral alterations in male golden hamsters exposed to chlorodibromomethane

STUDY OBJECTIVE: Carcinoembryonic antigen (CEA) is the most frequently used tumor marker in pleural fluid. Nevertheless, little is known about the causes of false-positive results. The aim of the study was to analyze the frequency, etiologies, and characteristics of the nonmalignant pleural effusions associated with elevated levels of CEA in pleural fluid. PATIENTS: Two hundred seventy-three consecutive patients with pleural effusions were evaluated, 91 (33%) associated with malignancy, and 182 (67%) due to benign diseases (51 transudates, 38 tuberculosis, 37 parapneumonic, 56 other). RESULTS: A level of CEA in pleural fluid above 10 ng/mL was found in 47% of pleural effusions associated with malignancy. Elevated levels of CEA were also found in 17 of the 182 (9%) nonmalignant pleural effusions: all five empyemas, one of the 23 typical parapneumonic (4%), two of the six borderline complicated (33%), and four of the eight complicated parapneumonic effusions (50%), one of the 38 tuberculous pleurisy (3%), one of the 11 hepatic transudates (9%), in the only patient with urinothorax, in the only patient with acute pancreatitis, and in one patient with postsurgery pleural effusion but with esophageal carcinoma and elevated CEA level in serum. CONCLUSIONS: Although an elevated level of CEA in pleural fluid is suggestive of malignancy, CEA can be elevated in 9% of pleurisy owing to benign diseases, especially in empyemas and in complicated parapneumonic effusions. Identifying the most frequent causes of false-positive results of CEA helps to correctly interpret the findings of this tumor marker.     

Diagnostic value of tests that discriminate between exudative and transudative pleural effusions. Primary Study Investigators

STUDY OBJECTIVE: To (1) determine appropriate decision thresholds and diagnostic accuracies for pleural fluid (PF) tests that discriminate between exudative and transudative pleural effusions, and (2) evaluate the quality of the primary investigations. DESIGN: Formal meta-analysis of studies that report the diagnostic value of pleural fluid tests. SETTING: Data collected from international academic medical centers. PATIENTS: Hospitalized patients undergoing thoracentesis for pleural effusions. INTERVENTIONS: Primary investigators were requested to transmit original data from patients described in their studies. MEASUREMENTS AND RESULTS: Eight primary studies described 1,448 patients with one or more of the following tests: protein (P)-PF, P-PF/serum ratio (R), bilirubin (BILI)-R, lactate dehydrogenase (LDH)-PF, LDH-R, cholesterol (C)-PF, C-R, and albumin gradient. We found that all eight tests had similar diagnostic accuracies when evaluated by receiver operating characteristic (ROC) analysis except for BILI-R, which was less diagnostically accurate. Decision thresholds determined by ROC analysis differed from previously reported values for LDH-PF (>0.45 upper limits of normal) and C-PF (>45 mg/dL). Paired and triplet test combinations tended to have higher diagnostic accuracies compared with individual tests, but examination of the odds ratios with 95% confidence intervals did not identify a clearly superior test combination. Limitations of the primary studies presented a high likelihood of bias affecting their results. CONCLUSIONS: Several strategies exist for clinicians in utilizing PF tests to classify effusions as exudates or transudates but accurate interpretations of these test results will require better designed studies.     

Congenital enterocyte heparan sulphate deficiency with massive albumin loss, secretory diarrhoea, and malnutrition

BACKGROUND: The molecular basis of protein-losing enteropathy is unknown. However it has been shown that sulphated glycosaminoglycans may be important in regulating vascular and renal albumin loss. METHODS: We describe three baby boys who presented within the first weeks of life with massive enteric protein loss, secretory diarrhoea, and intolerance of enteral feeds. All required total parenteral nutrition and repeated albumin infusions. No cause could be found in any case despite extensive investigations, including small intestinal biopsy sampling, which were repeatedly normal. FINDINGS: By specific histochemistry, we detected gross abnormality in the distribution of small intestinal glycosaminoglycans in all three infants, with complete absence of enterocyte heparan sulphate. The distribution of vascular and lamina propria glycosaminoglycans was, however, normal. INTERPRETATION: The presentation of these infants suggests that enterocyte heparan sulphate is important in normal small intestinal function.     

Nephrotic syndrome during captopril therapy

Captopril, an angiotensin-converting enzyme inhibitor, is being evaluated as an antihypertensive agent. We report on a patient who developed the nephrotic syndrome while on captopril 450 mg/d. Her urinary protein excretion was 5-7 g/24 h, plasma albumin concentration was 25 g/l, plasma cholesterol was 16,2 mmol/l, and she had oedema. Renal biopsy showed subepithelial deposits on the basement membrane.     

Diagnostic value of ferritin, haptoglobin, alpha 1-antitrypsin, lactate dehydrogenase and complement factors C3 and C4 in pleural effusion differentiation

Searching to define diagnostic criteria for malignant and non-malignant pleural effusions, the differential diagnostic value of ferritin (FRT), haptoglobin (Hp), alpha 1-antitrypsin (alpha 1-AT), lactate dehydrogenase (LDH) and complement factors C3 and C4 were investigated prospectively in 100 consecutive patients with pleural effusions of various aetiologies. Pleural effusion FRT, C3 and C4 concentrations were found to be useful in differentiating exudates from transudates, so that transudates practically could be excluded in pleural effusion: serum FRT ratio lower than 0.5 and/or in pleural effusion values for C3 and C4 higher than 300 mg dl-1 and 70 mg dl-1, respectively. A pleural effusion: serum C3 ratio greater than 2 is seen only in malignant effusions. No discriminative pleural: serum ratio could be found in FRT and C4 values capable of differentiating malignant from non-malignant effusions. Pleural effusion alpha 1-AT and LDH values were elevated in exudates, as compared with transudates, and had an excellent sensitivity and predictive value, but low specificity, in differentiating malignant from non-malignant effusions. Finally, the sensitivity, specificity and positive predictive value of pleural effusion Hp concentrations were lower than those of FRT and complement factors C3 and C4, respectively.     

Pleural fluid characteristics in hantavirus pulmonary syndrome

Hantavirus pulmonary syndrome (HPS), is a rodent-borne, acute, often fulminant cardiorespiratory illness. Noncardiogenic pulmonary edema is prominent in HPS as is cardiac dysfunction. Pleural effusions are commonly noted in patients with HPS and have been thought to be exudative. This report describes the prevalence and characteristics of pleural effusions by an assessment of chest radiographs for the presence of pleural fluid and reviews all pleural fluid specimens obtained from patients with HPS. Of 23 patients treated at the University of New Mexico Hospital for HPS, 22 had evidence of pleural fluid while 4 had sampling of their pleural fluid. Two samples met criteria for an exudate by pleural fluid protein to serum protein ratio of more than 0.5; one was clearly a transudate and the other had inconsistent characteristics. The two exudative samples were obtained 7 days after admission, while the other 2 were obtained within 1 day of admission. Pleural fluid cultures were sterile, and the total of nucleated cells was less than 170/mm3, and predominately mononuclear. A hypothesis may be formulated that the pleural fluid in HPS is initially transudative, consistent with the observed cardiopulmonary dysfunction. However, following aggressive resuscitative efforts and as the acute illness resolves, fluid shifts occur as cardiac function normalizes; the pleural fluid may take on characteristics of an exudate.     

ASK1 mediates apoptotic cell death induced by genotoxic stress

It is well known that scorpion venom induces lung lesions and respiratory distress which are usually classified as pulmonary oedema (PO). Tityus discrepans is a scorpion that lives in the north-central area of Venezuela, is the most common source of human envenomation here and produces PO. We studied the action of the venom of Tityus discrepans on whole rabbits and on their isolated lungs perfused with Krebs saline with 1 g/l of bovine serum albumin (Krebs-BSA saline). Two milligram of venom were diluted in 250 ml of solution (approximately the rabbit's total blood volume) and used to perfuse isolated lungs. Lung oedema occurred in rabbits which received 1 mg/kg of scorpion venom i.p., heparin prevented the production of this lung oedema. T. discrepans venom produced PO, in rabbits pretreated with 15 mg/kg of ajoene. Yet, Tityus venom had no effects on isolated lungs perfused with citrated or heparinized blood, and in lungs perfused with Krebs-BSA with normal Ca2+. These result show that Tityus venom does not act directly on lungs. Otherwise, we have observed that abundant microthrombi occurred in all rabbit lungs exposed to venom in vivo, suggesting that these clotting alterations are fundamental to produce PO. The presence of intravascular microthrombi is not characteristic of the usual PO hinting that scorpion venom induced pulmonary alterations are a different clinical entity. We thus propose that the use of the term pulmonary oedema in scorpionism should abandoned in favor of scorpion venom respiratory distress syndrome.     

Results of selective goiter resection in functional autonomy

BACKGROUND: Active colitis in patients with inflammatory bowel disease is associated with mucosal vasodilation, increased intestinal permeability and abnormal colonic motility. Nitric oxide is a messenger molecule with many functions, including regulation of local blood flow, vasomotor tone, and inflammation. Increased nitric oxide production and inducible nitric oxide synthase activity have been demonstrated in experimental models of colitis. This study was designed to determine the relationship between nitric oxide production and colonic inflammation in children with active colitis and in control subjects and whether expression of inducible nitric oxide synthase protein is demonstrable in the intestinal epithelium of these patients. METHODS: Nitrate + nitrite were measured in urine, stool, and plasma using the Griess assay. Expression of inducible nitric oxide synthase protein in intestinal tissue was determined by immunohistochemical localization. RESULTS: Urinary nitrate + nitrite levels were not significantly different in patients and control subjects. In contrast, stool and plasma nitrate + nitrite concentrations were significantly higher in children with inflammatory bowel disease compared with levels in control children (stool: 162.4 +/- 31.0 mumol/l versus 77.2 +/- 22.1 mumol/l; plasma: 65.2 +/- 9.9 mumol/l versus 38.1 +/- 6.6 mumol/L; p < 0.05). Stool nitrate + nitrite levels significantly correlated with plasma values. Immunohistochemical staining of colonic tissue from children with inflammatory bowel disease demonstrated inducible nitric oxide synthase protein located exclusively in epithelial cells. CONCLUSION: Increased nitric oxide production and enhanced intestinal epithelial cell expression of inducible nitric oxide synthase protein are associated with active colonic inflammation.     

Evidence for an independent role of metabolic acidosis on nutritional status in haemodialysis patients

BACKGROUND: Malnutrition in haemodialysis (HD) patients has been referred to underdialysis with low protein intake, and to acidosis. However, the separate effects of underdialysis and acidosis on nutrition have not been clearly demonstrated. To evaluate the role of the dialysis dose and of metabolic acidosis on nutrition, we measured the predialysis serum HCO3, pH, serum albumin, PCRn, Kt/V, and BMI in 81 uraemic patients on maintenance bicarbonate HD for 93+/-80 months. Patients with chronic liver diseases, malignancies, and cachexia were excluded. RESULTS: Mean age was 59+/-17 years, Kt/V was 1.29+/-0.21, PCRn 1.06+/-0.22 g/kg/day, serum albumin 4.07+/-0.28 g/dl, BMI 23+/-4 kg/m2, HCO3 21.1+/-1.9 mmol/l, pH 7.36+/-0.04. Serum albumin showed a significant direct correlation with: PCRn (P=0.001), HCO3 (P=0.001), pH (P=0.002), but no correlation with Kt/V and BMI. Serum HCO3 correlated inversely with PCRn (P=0.027). Multiple regression analysis confirmed the significant role of serum bicarbonate and age, but not of Kt/V, on serum albumin concentrations. The role of PCRn appeared to be marginal compared to serum bicarbonate in determining serum albumin levels. Dividing patients into two groups, serum albumin was 3.96+/-0.22 g/dl with HCO3 < or = 20 mmol/l and 4.18+/-0.31 g/dl in those with serum HCO3 > or = 23 mmol/l (P=0.002). PCRn in the same groups was respectively 1.14+/-0.24 g/kg/day and 1.01+/-0.23 g/kg/day (P=0.03). Most importantly, serum albumin levels did not appear to be affected by the dialysis dose, with Kt/V ranging from 0.90 to 1.88. CONCLUSIONS: In HD patients with adequate Kt/V, metabolic acidosis exerts a detrimental effect on serum albumin concentrations partially independently of the protein intake, as evaluated by PCRn. In the presence of moderate to severe metabolic acidosis, PCRn does not reflect the real dietary protein intake of the patients, probably as a result of increased catabolism of endogenous proteins. For this reason PCRn should be considered with caution as an estimate of the dietary protein intake in HD patients in the presence of metabolic acidosis.