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M J Krowka

Publications and source records attributed to M J Krowka.

At least 37 records · Page 2Linked to original sources
PubMed1999

Alpha1-antitrypsin deficiency allele carriers among lung cancer patients.

Lung cancer (LC) and chronic obstructive pulmonary lung diseases (COPDs; including emphysema and chronic bronchitis) share a common etiology. Despite the known associations of alpha1-antitrypsin deficiency (alpha1AD) with COPD and COPD with LC, few studies examined the association of alpha1AD alleles and LC. We hypothesize that heterozygous individuals who carry a deficient allele of the alpha1AD gene Pi (protease inhibitor locus) are at an increased risk of developing LC. The Pi locus is highly polymorphic with >70 variants reported. There are at least 10 alleles associated with deficiency in alpha1-antitrypsin. Using an exact binomial test, we compared the alpha1AD carrier rate in 260 newly diagnosed Mayo Clinic LC patients to the reported carrier rate in Caucasians in the United States (7%). alpha1AD carrier status, determined by isoelectric focusing assay, was examined with respect to the history of cigarette smoking, COPD, and histological types. Thirty-two of the 260 patients (12.3%; 95% confidence interval, 8.6-16.9%) carried an alpha1AD allele, which was significantly higher than expected (P = 0.002). Twenty-four of the 32 carriers had allele S, 6 had allele Z, and 2 had allele I. Patients who never smoked cigarettes were three times more likely to carry a deficient allele (20.6%; P = 0.008), although smokers had a higher carrier rate (11.1%; P = 0.025) when compared with the 7% rate. Patients with squamous cell or bronchoalveolar carcinoma had a significantly higher carrier rate than expected (15.9% and 23.8%, P < or = 0.01, respectively). Our preliminary findings suggest that individuals who carry an alpha1AD allele may have an increased risk for developing LC, specifically squamous cell or bronchoalveolar carcinoma.

Adenocarcinoma, Bronchiolo-Alveolar↗
PubMed1998

Use of macroaggregated albumin lung perfusion scan to diagnose hepatopulmonary syndrome: a new approach.

BACKGROUND & AIMS: We have reported that contrast echocardiography is a sensitive screening test for the hepatopulmonary syndrome (HPS). However, contrast echocardiography lacks specificity because many cirrhotic patients have positive study results with normal arterial blood gases and therefore do not fulfill criteria for HPS. The aim of this study was to assess the role of macroaggregated albumin lung perfusion scans (MAA scans) in the diagnosis of HPS. METHODS: MAA scans were performed in 25 patients with HPS, 25 cirrhotic patients without HPS, and 15 hypoxemic subjects with intrinsic lung disease alone. An MAA shunt fraction was calculated from brain and lung counts. RESULTS: MAA scan results were positive in 21 of 25 patients with HPS and negative in all controls. All 21 patients with positive MAA scans had PO2 values of <60 mm Hg. There was a strong inverse correlation between the degree of the MAA shunt fraction and arterial hypoxemia (r = -0.726). CONCLUSIONS: A positive MAA scan result in cirrhosis is specific for the presence of moderate to severe HPS. We speculate that MAA scans may be particularly useful in evaluating the contribution of HPS to the hypoxemia in cirrhotic patients with intrinsic lung disease.

Adolescent↗
PubMed1997

Hepatopulmonary syndrome with progressive hypoxemia as an indication for liver transplantation: case reports and literature review.

In the hepatopulmonary syndrome (HPS), a pulmonary vascular complication of liver disease, severe hypoxemia due to pulmonary vascular dilatation can be extremely debilitating. Determining whether patients with advanced liver disease and HPS should be considered for liver transplantation is difficult. We describe three patients with progressive and severe hypoxemia who underwent successful liver transplantation and had resolution of their arterial hypoxemia. In these patients, the progressive pulmonary deterioration accelerated the need and was considered an indication for liver transplantation rather than being considered an absolute or relative contraindication. In addition, we review the literature on 81 pediatric and adult patients with HPS who underwent liver transplantation and specifically highlight mortality, morbidity, syndrome resolution, and prognostic factors. Posttransplantation mortality (16%) was associated with the severity of hypoxemia (mean arterial oxygen tension [PaO2] in 68 survivors was 54.2 +/- 13.2 mm Hg and in 13 nonsurvivors was 44.7 +/- 7.7 mm Hg; P<0.03). Patients with a pretransplantation PaO2 of 50 mm Hg or lower had significantly more frequent mortality (30%) in comparison with those with a PaO2 greater than 50 mm Hg (4%; P<0.02). Pulmonary recommendations that address the severity of hypoxemia and candidacy for liver transplantation are discussed.

Adult↗
PubMed1996

Hepatopulmonary syndrome. A pulmonary vascular complication of liver disease.

Hepatopulmonary syndrome is part of the spectrum of pulmonary vascular disorders seen in advanced liver disease. The pathophysiology of these entities likely is dependent on the degree of pulmonary vasoconstriction or vasodilation that occurs. Our understanding of hepatopulmonary syndrome has helped further our knowledge of the interaction of the liver and the lung. Advances in the management of this disorder, especially liver transplantation, finally have allowed us to offer some hope to patients with this disease.

Hemodynamics↗
PubMed1996

Recent pulmonary observations in alpha 1-antitrypsin deficiency, primary biliary cirrhosis, chronic hepatitis C, and other hepatic problems.

Patients with metabolic, immunologic, viral, and other types of hepatic disorders can have a spectrum of complicating pulmonary abnormalities. The natural history of these associations is poorly understood. Significant reversibility in hepatic and pulmonary dysfunction, however, has been well documented in the era of organ transplantation. The continued relationship among pulmonologists, hepatologists, and transplant surgeons hopefully will provide enlightening data on these interesting clinical associations, their natural histories, and their response to evolving therapeutic approaches.

Chronic Disease↗
PubMed1996

Frequency and clinical implications of increased pulmonary artery pressures in liver transplant patients.

OBJECTIVE: To characterize the pulmonary hemodynamics and identify predictors of pulmonary hypertension in a group of patients before liver transplantation and to determine whether pulmonary hypertension in these patients is related to survival. MATERIAL AND METHODS: In 362 patients before their first liver transplantation (between 1985 and 1993), the clinical history, laboratory data, and results of pulmonary function tests were recorded. Pulmonary artery (PA) catheterization was performed after induction of anesthesia at the time of transplantation. Monthly follow-up was maintained. RESULTS: A hyperdynamic circulation was often present -- an increased mean cardiac output (7.6 L/min), increased mean PA pressure (20.9 mm Hg), correlation of mean PA pressure with cardiac output (r = 0.25; P<0.001), and decreased mean pulmonary vascular resistance (60 dynes times s/cm5). Mean PA pressures were more than 25 mm Hg in 72 patients (20%). Pulmonary hypertension (defined as mean PA pressure of more than 25 mm Hg and pulmonary vascular resistance in excess of 120 dynes times s/cm5) occurred in 15 patients (4%). Pulmonary function tests revealed obstruction in 7%, restriction in 18%, and low diffusing capacity in 46%. By univariate analysis, lower forced expiratory volume in 1 second, forced vital capacity, and total lung capacity were the only preoperative factors associated with pulmonary hypertension (P<0.05). Survival was significantly lower in patients with acute fulminant hepatitis (P<0.001), the group with the highest mean PA pressure, than in those with other diagnoses. Increased PA pressures or mild to moderate pulmonary hypertension was not found to be associated with a worse survival by univariate or multivariate analysis. CONCLUSION: Increased PA pressure is common in liver transplant patients (20%). "True" pulmonary hypertension occurred in only 4% of our patients and was not associated with an adverse outcome.

Adolescent↗
PubMed1995

Hepatopulmonary syndrome: what are we learning from interventional radiology, liver transplantation, and other disorders?

Interventional radiology (pulmonary angiography, coil embolotherapy, and TIPS) in patients with HPS seems to provide both diagnostic data and therapeutic results of clinical importance. The case report by Riegler et al. is instructive and emphasizes the need for further prospective study to analyze the successes and failures in terms of measurable clinical variable in a procedure such as TIPS. The resolution of hypoxemia following liver transplantation and careful patient selection offers hope to patients with severe, debilitating oxygenation abnormalities caused by HPS.

Dilatation, Pathologic↗
PubMed1995

Failure of hepatopulmonary syndrome to resolve after liver transplantation and successful treatment with embolotherapy.

The hepatopulmonary syndrome is an uncommon accompaniment of chronic liver disease. The outcome of this disorder after orthotopic liver transplantation is variable. We describe a patient with the hepatopulmonary syndrome who underwent orthotopic liver transplantation for autoimmune hepatitis. Her platypnea and orthodeoxia failed to improve postoperatively. Pulmonary angiography showed large pulmonary arteriovenous shunts that were successfully treated with coil embolotherapy.

Adult↗
PubMed1994

Is severe arterial hypoxaemia due to hepatic disease an indication for liver transplantation? A new therapeutic approach.

Severe arterial hypoxaemia or deteriorating pulmonary oxygenation due to HPS appears to be reversible within months of orthotopic liver transplantation. Due to the apparently poor prognosis in these patients, such abnormal oxygenation may, indeed, be considered an indication for liver transplantation in selected clinical situations. The pretransplant distinction between HPS and pulmonary hypertension as a reason for arterial hypoxaemia is essential, however, in that different outcomes appear to exist. Further prospective studies of patients with pulmonary vascular complications of liver disease are desperately needed.

Adolescent↗
PubMed1993

Pulmonary complications of orthotopic liver transplantation.

We retrospectively reviewed the pulmonary complications and associated morbidity and mortality of 44 consecutive patients who underwent 52 orthotopic liver transplantations (OLTs) at the Mayo Clinic during 1987. All survivors participated in follow-up for 1 year after OLT. Of the five deaths in the study group, three were associated with pulmonary infections. On postoperative chest roentgenograms, 24 cases of pulmonary infiltrates were noted; 12 were caused by infections. Ten opportunistic pulmonary infections developed in nine patients: four cytomegalovirus, three Pneumocystis carinii pneumonia, and one each of Cryptococcus, Aspergillus, and Candida. All except one of the opportunistic infections were diagnosed after the sixth postoperative week. Fiberoptic bronchoscopy was helpful for diagnosing opportunistic pulmonary infections in six patients. One Aspergillus pulmonary infection was diagnosed by transthoracic needle aspiration. Bacterial pneumonia occurred in five patients. Preoperative pulmonary function tests, performed in 40 patients, revealed a restrictive ventilatory defect in 28% and impaired gas transfer in 52%. Pleural effusion was present in 18% of patients preoperatively and in 77% during the first week after OLT. Preoperative severity of liver disease and results of arterial blood gas determinations, pulmonary function tests, and chest roentgenography were not associated with postoperative mortality and pulmonary infections. Infectious and noninfectious pulmonary complications are common in liver transplant recipients. Attempts to decrease the frequency and severity of pulmonary complications by early diagnosis and effective treatment may diminish the morbidity and mortality associated with OLT.

Adult↗
PubMed1993

Hepatopulmonary syndrome. Clinical observations and lack of therapeutic response to somatostatin analogue.

We retrospectively studied 22 patients with hepatopulmonary syndrome (HPS) evaluated at the Mayo Medical Center from 1984 to 1991. All patients had hepatic cirrhosis with clinical evidence of portal hypertension; 13 (59 percent) had severe hypoxemia while breathing room air in the supine position (PaO2 < 60 mm Hg), and 14 of 16 (88 percent) had orthodeoxia breathing room air. On the basis of angiographic observations, we defined type 1 and type 2 patterns of pulmonary vascular abnormalities in HPS. Response to 100 percent oxygen and therapeutic regimens may differ in the angiographic patterns. Substantial deterioration in PaO2 associated with clinically stable hepatic dysfunction was documented in five of seven patients studied with sequential arterial blood gas testing; four subsequently died within 48 months. Overall mortality was 41 percent, occurring a mean of 2.5 years after diagnosis. In 7 of the 22 patients, we prospectively studied the effect of somatostatin analogue given subcutaneously for 4 consecutive days. No significant improvement in PaO2 was documented while breathing room air or 100 percent oxygen (p < 0.05). We conclude that in selected patients with clinically stable hepatic dysfunction and deteriorating oxygenation, the prognosis is poor. Our data in combination with recent surgical reports suggest that liver transplantation may be the treatment of choice in patients with HPS and worsening oxygenation.

Adult↗