Deborah Freese | Mayo Clinic (original) (raw)

Papers by Deborah Freese

Pediatrics in review / American Academy of Pediatrics, 2004

Transplantation, 1987

... Hepatitis-Associated Aplastic Anemia After Liver Transplantation. STOCK, PETER G.; STEINER,MA... more ... Hepatitis-Associated Aplastic Anemia After Liver Transplantation. STOCK, PETER G.; STEINER,MARIE E.; FREESE, DEBORAH; SHARP, HARVEY; ASCHER, NANCY L. Collapse Box Abstract. An abstract is unavailable. This article is available as a PDF only. Close Window. ...

New England Journal of Medicine, 1988

Aplastic anemia developed in 9 of 32 patients (28 percent) undergoing orthotopic liver transplant... more Aplastic anemia developed in 9 of 32 patients (28 percent) undergoing orthotopic liver transplantation for acute non-A, non-B hepatitis, at one to seven weeks after the procedure. No patient previously had evidence of hematologic dysfunction or conditions known to be associated with aplastic anemia. No other cases of aplastic anemia were identified among 1463 patients undergoing liver transplantation for all other indications at the four centers participating in the study (chi-square = 415, P less than 0.001; 95 percent confidence interval for the incidence of aplastic anemia after transplantation for non-A, non-B hepatitis, 13 to 44 percent, vs. 0.00 to 0.13 percent for all other indications). The operative and postoperative treatment of these patients was not otherwise different, indicating that the aplastic anemia was a complication of the hepatitis, not of the transplantation procedure. Four of the nine patients died of complications due to infections. Three of the surviving patients have been followed for less than six months, one for one year, and one for two years. The two patients followed the longest have recovered marrow function to an appreciable degree, and two of the others have evidence of early recovery. We conclude that patients undergoing orthotopic liver transplantation for non-A, non-B hepatitis are at a high risk for the development of aplastic anemia.

The Journal of Pediatrics, 1987

Three patients with hereditary tyrosinemia type I were examined before and after liver transplant... more Three patients with hereditary tyrosinemia type I were examined before and after liver transplantation to assess the role of extrahepatic tissues in the biochemical disorders of this disease. Before transplantation the three patients excreted excessive amounts of succinylacetoacetate (SAA), succinylacetone (SA), tyrosyl acidic compounds, and 5-aminolevulinate (ALA). The activity of 5-aminolevulinate dehydratase (ALA-D) in red blood cells was markedly inhibited (1% to 5% of control) in the three patients. Successful liver transplantation resulted in decreased excretion of urinary SAA plus SA, tyrosyl acidic compounds, and ALA. Two of the patients continued to excrete significant amounts of SAA plus SA, whereas those compounds were undetectable in the urine of the third patient. Tyrosine loading resulted in increased excretion of SAA plus SA in two patients, but those compounds remained undetectable in the third. All three patients continued to excrete higher than normal amounts of ALA, but the activity of ALA-D in red blood cells returned to normal after transplantation, indicating marked clearance of SA from the blood. Liver transplantation may not totally correct the biochemical abnormalities of hereditary tyrosinemia. It is likely that the kidney is the source of persistent biochemical aberrations in the urine without significant effects on the blood. Our results suggest the existence of heterogeneity for renal involvement in hereditary tyrosinemia.

The Journal of Pediatrics, 2003

Objective To assess the prevalence of abnormal gastric emptying and small bowel transit in childr... more Objective To assess the prevalence of abnormal gastric emptying and small bowel transit in children with functional dyspepsia at a tertiary care center, and the relationship between abnormal gastric and small bowel transit and symptoms in pediatric patients with functional gastrointestinal disorders.

Journal of Inherited Metabolic Disease, 1985

A liver transplant was performed on a 4-year-old female in liver failure caused by hereditary tyr... more A liver transplant was performed on a 4-year-old female in liver failure caused by hereditary tyrosinaemia, with hepatocellular carcinoma following a negative evaluation for metastases. However, serum alpha-fetoprotein levels never returned to normal after the surgery. Urinary succinylacetone (SA) was detected in her urine prior to transplantation despite strict adherence to a low-tyrosine diet. Other patients with severe liver disease awaiting liver transplantation do not excrete SA in the urine. She continued to excrete SA during the postoperative period despite normal liver functions. Oral tyrosine loading resulted in significant elevation of SA excretion. Possible explanations for this observation and clinical and therapeutic relevance are discussed.

Journal of Hepatology, 2002

Aims: To assess the frequency of CTLA-4 genotypes in Brazilian patients with AIH types 1 and 2. P... more Aims: To assess the frequency of CTLA-4 genotypes in Brazilian patients with AIH types 1 and 2. Patients e Methods: Determination of CTLA-4 genotypes were carried out in 106 patients with AIH type 1, 26 subjects with AIH type 2, diagnosed according to international criteria, and 67 healthy controls by PCR-based techniques. Results: The frequencies of AA, AG and GG genotypes of CTLA-4 gene were respectively 41%, 44% and 15% in patients with AIH-1; 46%, 35% and 19% in subjects with AIH-2 and 43%, 45% and 12% in healthy controls (p = NS). When compared to healthy controls, no difference in the distribution of CTLA-4 genotypes was observed in adult and pediatric patients with AIH-1 (35%, 54% and 11% respectively with AA, AG and GG alleles in adults vs. 42%, 42% and 16% in children). Conclusions: Susceptibility to AIH types 1 and 2 in not influenced by exon 1 CTLA-4 gene polymorphism at position 49. These findings highlight the genetic differences observed in susceptibility to AIH in different ethnic groups.

Hepatology, 1984

The histopathological features of orthotopic liver transplants were evaluated in 63 serial biopsy... more The histopathological features of orthotopic liver transplants were evaluated in 63 serial biopsy specimens from 17 patients. Biopsies were taken at the time of insertion of the liver (six biopsies), at the time of development of liver function abnormalities (11 biopsies) and as follow-up to previously abnormal biopsies (46 biopsies). The biopsies taken at the time of insertion all showed diffuse hepatocellular ballooning with confluent areas of necrosis in one case. Biopsies taken at the time of onset of rejection (nine cases) all showed a mixed portal inflammatory infiltrate, bile duct damage and central or portal vein endothelialitis (i.e., attachment of lymphocytes to the vein endothelium). Follow-up biopsies showed several patterns including: (i) resolution of changes of acute rejection with subsequent development of recurrent acute or chronic rejection (four cases); (ii) prolonged acute rejection simulating extrahepatic biliary obstruction (four cases); (iii) prolonged acute rejection with predominance of eosinophils simulating a drug reaction (one case); and (iv) rapidly progressive acute rejection leading to death (one case). Biopsy of the transplanted liver at the time of transplantation is useful to provide a baseline for comparison with later biopsies. Biopsy at the time of onset of changes in liver function is essential to confirm the presence of rejection prior to alteration of immunosuppression.

Gastroenterology, 2003

Is gastrointestinal transit abnormal in children with functional dyspepsia and upper gastrointest... more Is gastrointestinal transit abnormal in children with functional dyspepsia and upper gastrointestinal symptoms? ... No abstract is available. To read the body of this article, please view the PDF online. ... Visit SciVerse ScienceDirect to see if you have access via your institution.

CHEST Journal, 1993

A major complication of hepatic cirrhosis is arterial hypoxemia, often the result of intrapulmona... more A major complication of hepatic cirrhosis is arterial hypoxemia, often the result of intrapulmonary arteriovenous shunting. While previously such hypoxemia was thought to preclude successful hepatic transplantation, more recent studies have suggested that hepatic transplantation should be considered if the hypoxemia is corrected by supplemental oxygen. We report the findings in a cirrhotic patient with severe hypoxemia associated with intrapulmonary arteriovenous shunting. The patient did not respond to supplemental oxygen (PaO2 < 40 mm Hg on O2 at 4 L/min). The patient underwent successful hepatic transplantation, with complete resolution of intrapulmonary shunting. We believe that patients with cirrhosis-associated intrapulmonary shunting, even with hypoxemia resistant to supplemental oxygen, are acceptable candidates for hepatic transplantation.

The American Journal of Surgical Pathology, 1987

Two-hundred-seventy biopsy specimens from 47 patients undergoing liver transplants at the Univers... more Two-hundred-seventy biopsy specimens from 47 patients undergoing liver transplants at the University of Minnesota were analyzed to determine if histological features could predict the eventual outcome of rejection episodes. Thirty-six patients (76.6%) rejected the transplant. Of these, five either suffered acute liver failure due to rejection (two cases) or developed chronic rejection (three cases). Features of significance in predicting such a bad outcome were arteritis, bile duct paucity, or simultaneous hepatocellular ballooning and hepatocellular dropout and necrosis. Other features, such as type and intensity of infiltrate, degree of bile duct damage, or simple presence of hepatocellular necrosis, were not predictive of outcome. Our conclusion is that biopsy is useful in predicting outcome. Since many of the histologic findings of predictive value were not present in initial pretreatment biopsy specimens, follow-up biopsies of patients being treated for rejection are recommended to assess efficacy of therapy.

The American Journal of Medicine, 1992

It has been stated that arteriohepatic dysplasia is a form of biliary paucity with a good prognos... more It has been stated that arteriohepatic dysplasia is a form of biliary paucity with a good prognosis. We wished to determine the long-term morbidity and mortality associated with arteriohepatic dysplasia. The charts of all patients with arteriohepatic dysplasia followed by the pediatric gastroenterologists of the University of Minnesota into adulthood were reviewed. Over the last 33 years, the pediatric gastroenterologists have followed 16 children with syndromic paucity, six of whom are now beyond age 18 years. Although five of six patients responded to medical therapy with improvement in their cholestasis and appeared stable clinically through childhood, five of six patients had complications of arteriohepatic dysplasia after age 16 years that resulted in severe morbidity (three) or death (two). These complications included hepatic failure (two), renal failure (one), cerebellar herniation (one), and hepatocellular carcinoma (one). In only one patient were symptoms of the complications present prior to the age of 18 years. As more patients with arteriohepatic dysplasia reach adulthood, it appears that this syndrome may be accompanied by long-term manifestations extending beyond childhood. It is important that physicians assuming management of these patients from pediatricians be aware that new abnormalities may appear without warning and that the hepatic disease may deteriorate despite apparent stability through childhood.

American Journal of Medical Genetics, 1987

Case reports in pediatrics, 2014

A 17-year-old male was transferred to the pediatric intensive care unit for evaluation of acute l... more A 17-year-old male was transferred to the pediatric intensive care unit for evaluation of acute liver failure. He was recently released from an alcohol treatment center with acute onset of chest pain. Cardiac workup was negative but he was found to have abnormal coagulation studies and elevated liver transaminases. Other evaluations included a normal toxicology screen and negative acetaminophen level. Autoimmune and infectious workups were normal providing no identifiable cause of his acute liver failure. He initially denied any ingestions or illicit drug use but on further query he admitted taking niacin in an attempt to obscure the results of an upcoming drug test. Niacin has been touted on the Internet as an aid to help pass urine drug tests though there is no evidence to support this practice. Niacin toxicity has been associated with serious multisystem organ failure and fulminant hepatic failure requiring liver transplantation. Pediatric providers should be aware of the risks a...