Intrapleural nitrogen mustard for treating chylous effusion of pulmonary lymphangioleiomyomatosis.
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A 54-year-old woman, presenting with chyluria and progressive dyspnea, is reported. Necropsy revealed co-existent lymphangiomyomatosis and pneumoconiosis in the lungs. Retroperitoneal lymphangiomyomas surrounded the distal ureters, and cystoscopy revealed pyelolymphatic backflow into renal lymphatic sinuses. The finding of chyluria with atypical smooth muscle hyperplasia within the ureters is extremely rare. Previously reported cases of lymphangiomyomatosis are discussed and compared with our own. Electron micrographs of involved lymph nodes are presented and the findings briefly evaluated.
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Accurate staging as per National Wilms' Tumor Study (NWTS) criteria is essential for therapy of Wilms' tumor. We report a unique case of a 9-mo-old white male in whom the diagnosis based upon clinical, radiological, surgical, and initial histopathologic findings was Stage I Wilms' tumor without regard to a positive cytologic examination of a small amount (5 ml) of chylous ascitic fluid obtained intraoperatively. This problem was clarified later at the NWTS panel review when the deeper recuts of a renal hilar lymph node revealed a single cluster of Wilms' tumor cells in capsular sinus, resulting in upstaging of tumor to Stage III. The significance of these findings in relation to staging will be discussed.
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We report a case of mesenteric chylous cyst diagnosed preoperatively by ultrasonography (US) and computed tomography (CT). Both demonstrated a unilocular cystic mass with a fluid-fluid level. The CT number of the two components indicated fat density and water density and with shaking or positional changes, the contents displayed miscibility. The tumor changed its position during hospitalization. Both features are considered to be quite diagnostic of this condition.
Patients with chyluria, postoperative chylothorax, or surgical thoracic duct drainage are depleted of lymphocytes, IgA, and C3 as a result of the loss of lymphatic cellular and humoral elements, but the specific defects have not been well characterized. Therefore, we investigated the immunologic status of three patients with longstanding chyluria who were lymphopenic but did not have evidence of opportunistic infections. All patients had normal levels of immunoglobulins except for a moderate decrease in the IgA fraction, a normal antibody response to viral agents, and a normal hypersensitivity response to at least one of six recall antigens. Their complement levels were essentially normal. All were leukopenic because of decreased numbers of circulating lymphocytes. Two had depressed relative and absolute numbers of OKT4 (helper/inducer) T cells and normal or increased relative numbers of OKT8 (cytotoxic/suppressor) T cells, resulting in a marked reversal of the OKT4 to OKT8 ratio. The third was severely depleted of both OKT4 and OKT8 cells. The response of their peripheral blood mononuclear cells to mitogen stimulation was reduced, but they all had normal or increased absolute and relative numbers of natural killer (NK) cells as defined by HNK-1 or OKM-1. Their NK activity was less than that of normal controls unless stimulated by interferon, suggesting that many of the NK cells were immature. Normal delayed hypersensitivity, reduced but effective mitogen stimulation, and a normal NK response after interferon may be factors that protect these patients against opportunistic infections and neoplasms.
The presence of lipids in postprandial urine was assessed in 116 patients with a history of filariasis and 70 normal individuals using a biochemical autoanalyzer. Urinary triglycerides (TGs) ranging from 10 to 1955 mg/dl were detected in 13 individuals with a history of chyluria, including 3 with TG levels ranging from 233 to 1955 mg/dl and cholesterol levels of 6-35 mg/dl. Three patients who had a filarial history but without chyluria were also found to have urinary TGs (13-15 mg/dl) without detectable cholesterol. Neither TGs nor cholesterol were detected in the urine of normal individuals. Fluctuations in postprandial urine lipid contents were measured by time course determinations of urinary TG and cholesterol in 17 patients with filariasis and a history of chyluria, 16 patients with filariasis and hydrocele and 16 normal individuals. The level of urine lipid excretion was found to increase within 1-4 h postprandially, with urinary TG levels ranging between 7.8 and 1284 mg/h in eight patients and urinary cholesterol levels between 1.2 and 138 mg/h in seven patients with a history of chyluria. To evaluate the origin of the urine lipids in hematochyluria, fish oil containing 360 mg eicosapentaenoic acid (EPA) and 240 mg docosahexaenoic acid (DHA) was prescribed to a patient with hematochyluria. The excretion of EPA and DHA in urine was increased postprandially in the patient, similar to the elevation of urinary TG and cholesterol. The profile of fatty acids from urine samples showed it was of dietary origin. Our results suggest that postprandial urine lipids, especially TG, might be used as markers for the clinical evaluation of chyluria.
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A 10-year-old boy with chyluria due to a congenital fistulous communication between the lymphatic system and the bladder is described. Chyluria can be parasitic or non-parasitic. Many causes of non-parasitic chyluria have been reported. Lymphography is the preoperative imaging procedure of choice since it demonstrates the site, the calibre and the number of the fistulous communications. Lymphoscintigraphy shows very well the site of the fistula but is not as precise as lymphography. However, it has the advantage to be less invasive and is an excellent alternative in the non-surgical cases. The prognosis of non-parasitic chyluria is usually very good and the treatment is mostly conservative.
A 47-year-old woman with acute necrotizing pancreatitis developed sudden cardiorespiratory arrest and needed resuscitation. A pericardial effusion was found, and 350 ml of a white nontransparent milky fluid was aspirated that contained 1020 mg triglycerides/100 ml. The diagnosis of chylous cardiac tamponade was made. Absence of amylase in the chylous effusion militates against the popular hypothesis that lymphatic transport of exocrine digestive enzymes from the inflamed pancreas produces the frequent intrathoracic serosal effusions in acute pancreatitis. The data of our patient rather suggest that these effusions result from the leakage of pancreatic inflammatory exudates through the diaphragm which, apparently, may even result in the loss of pericardial and adjacent thoracic lymph vessel integrity. Although pericardial tamponade is a rare complication, it should be considered if otherwise unexplained circulatory deterioration occurs in a patient with acute pancreatitis.