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Biomedical subjects

A L Baker

Publications and source records attributed to A L Baker.

At least 37 records · Page 2Linked to original sources
PubMed1998

Coronary artery dimensions may be misclassified as normal in Kawasaki disease.

BACKGROUND: Current American Heart Association guidelines indicate that patients with Kawasaki disease and no coronary artery abnormalities on echocardiography at any stage of illness may be discharged from cardiologic follow-up 1 year after onset of illness. METHODS AND RESULTS: To determine whether coronary artery dimensions in patients with Kawasaki disease whose vessels are classified as "normal" by Japanese Ministry of Health criteria have a distribution similar to expected population norms when adjusting for body surface area, we studied 125 patients during 4 intervals from onset of illness: (1) 10 days or less, (2) 2 weeks (11 to 21 days), (3) 6 weeks (22 days to 3 months), and (4) 1 year (4 months to 1.5 years). Using two-dimensional echocardiography, we measured the internal lumen diameter of the left main, proximal left anterior descending, and proximal right coronary arteries. Mean body surface area-adjusted dimensions of the proximal left anterior descending and right coronary arteries were significantly larger (P < .01) in patients with Kawasaki disease than those in subjects in all periods, except for a marginal difference at 6 weeks for the proximal right coronary artery (P = .02); for the left main coronary artery, this difference achieved statistical significance in the period of 10 days or less, with a trend at 2 weeks (P = .02). Among patients classified as having normal coronary arteries on all echocardiograms by the Japanese Ministry of Health criteria, 27% had at least 1 body surface area-adjusted coronary dimension more than 2 standard deviations above the expected mean. CONCLUSIONS: Coronary artery dilation in Kawasaki disease is thus more prevalent than previously reported, highlighting the need for systematic long-term surveillance of this population.

Adolescent↗
PubMed1998

Cerebrospinal fluid profile in patients with acute Kawasaki disease.

BACKGROUND: Kawasaki disease (KD) is an acute vasculitis of infancy and early childhood for which there is currently no diagnostic test. The clinical presentation of KD may initially resemble other infectious diseases, including bacterial or viral meningitis. For this reason lumbar puncture (LP) is sometimes performed during the evaluation of these patients. To understand the range of cerebrospinal fluid (CSF) changes that may be associated with acute KD, a retrospective review of unselected KD patients from three pediatric centers was performed. METHODS: Retrospective chart review was performed on KD patients evaluated during the first 10 days of illness who had an LP performed before the administration of intravenous gamma-globulin. RESULTS: During the 6.5-year study period, 46 KD patients underwent LP as part of their clinical evaluation. Of these patients 18 (39.1%) had CSF pleocytosis, 1 (2.2%) had a CSF glucose <45 mg/dl and 8 (17.4%) had an elevated CSF protein. Of the patients with CSF pleocytosis, the median white blood cell count was 22.5 cells (range, 7 to 320 cells), with a median of 6.0% neutrophils (range, 0 to 79%) and 91.5% mononuclear cells (range, 11 to 100%). CONCLUSIONS: In the present series approximately one-third of KD patients who underwent an LP had CSF pleocytosis with a mononuclear cell predominance. No patient had significant hypoglycorrhachia, and elevation of the CSF protein was uncommon. CSF abnormalities were similar between US and Japanese KD patients. The basis for the CSF pleocytosis in acute KD patients remains unknown.

Acute Disease↗
PubMed1997

Passive cigarette smoking and reduced HDL cholesterol levels in children with high-risk lipid profiles.

BACKGROUND: HDL cholesterol levels are known to be lower in smokers than in nonsmokers. Previous studies have demonstrated an association of decreased HDL cholesterol with passive smoking in children but have not adjusted for potential confounding factors. METHODS AND RESULTS: In a cross-sectional, pilot-scale study, we examined the relationship of HDL cholesterol levels to passive smoking in children and adolescents referred to a tertiary hyperlipidemia clinic. Eligibility criteria included (1) first visit to a lipid clinic, (2) LDL cholesterol >95th percentile for age or HDL cholesterol <5th percentile, (3) age between 2 and 18 years, and (4) absence of secondary causes of hyperlipidemia. Sociodemographic information, diet record, medical history, and fasting lipid profiles were obtained. Of 109 eligible patients, 103 (94%) were studied. Twenty-seven percent came from households with cigarette smokers. HDL cholesterol levels were 38.7+/-1.2 mg/dL (mean+/-SEM) in passive smokers versus 43.6+/-1.2 mg/dL in children without smoke exposure (P=.005). Smoking exposure was not significantly associated with other lipid values. The effect of smoking on HDL cholesterol was minimally affected by potential confounders. In multivariate regression adjusting for body mass index, age, sex, exercise, and dietary fat intake, passive smoking remained a significant risk factor for decreased HDL cholesterol (P=.012). CONCLUSIONS: Mean HDL cholesterol levels are lower in dyslipidemic children from households with smokers than in those without household smoke exposure. Passive smoking may worsen the risk profile for later atherosclerosis among high-risk young persons.

Child↗
PubMed1997

Candida (Torulopsis glabrata) liver abscesses eight years after orthotopic liver transplantation.

The authors report the case of a 48-year-old man in whom candida (Torulopsis glabrata) liver abscesses developed 8 years after liver transplantation. After a week of fever, computed tomography and Doppler ultrasonography showed several fluid-filled loculations in the left lobe of the liver and hepatic arterial stenosis. Aspirates from the abscesses contained T. glabrata organisms. This complication probably developed because hepatic arterial stenosis resulted in bile infarcts (bilomas), which were contaminated via the biliary tract with candida from the biliary-enteric anastomosis. Catheter drainage and administration of amphotericin B for 10 weeks permitted successful retransplantation. T. glabrata liver abscesses, a life threatening complication that can occur long after liver transplantation, can be successfully managed by aggressive medical treatment followed by retransplantation.

Candidiasis↗
PubMed1996

Tacrolimus for primary treatment of steroid-resistant hepatic allograft rejection.

Twelve patients who experienced steroid-resistant rejection after primary liver transplantation while receiving cyclosporine-based therapy were converted to tacrolimus without receiving OKT3 or additional steroids. The indications for conversion were ongoing biopsy-confirmed rejection. All patients had received one course of high-dose intravenous steroids, which failed to reverse the rejection episode. No other antirejection therapy was given. Tacrolimus was initiated to reverse rejection and for maintenance therapy. The tacrolimus target level was 15-20 ng/ml (whole blood, IMX). All 12 patients had rapid reversal of the rejection episode and did not experience recurrent rejection (mean follow-up: 8.2 +/- 1.2 months). The mean bilirubin level dropped from 6.1 mg/dl at the initiation of tacrolimus therapy to 4.4 mg/dl by day 7 of therapy, 2.5 mg/dl by day 14, and 1.5 mg/dl by day 21 (P < 0.003). Serum glutamic pyruvic transaminase demonstrated a similar response. The serum creatinine level was unchanged at 1.5 mg/dl. No major adverse reactions were noted in this group of patients. Patient and graft survival rates were 100%. Four of the eight patients with a follow-up of >4 months are no longer receiving steroid therapy. Tacrolimus is effective as the primary therapy for the treatment of steroid-resistant rejection and provides a rapid and sustained biochemical response. Patients with mild to moderate rejection may be safely converted from cyclosporine to tacrolimus without an additional steroid bolus or OKT3 therapy. Early "preemptive" conversion to tacrolimus prior to the use of additional steroids or OKT3 may decrease overall rejection therapy requirements. This approach has promise for improved graft survival and fewer infectious and immunologic complications.

Adult↗
PubMed1996

Cyclosporin A: drug discontinuation for the management of long-term toxicity after liver transplantation.

After liver transplantation, long-term cyclosporin A (CsA) administration is commonly complicated by renal insufficiency and other side effects. To manage these problems, 1.5 to 2.0 mg x kg(-1) x day(-1) of azathioprine for at least 6 weeks was prescribed; CsA was then discontinued or reduced to < or = 2.5 mg x kg(-1) x day(-1) for several months. The dose of prednisone was kept constant. CsA was discontinued in 14 patients because of nephrotoxicity (three or more serum creatinine levels of > or = 1.5 mg/dL), in 1 patient because of headaches and in 1 patient because of a generalized sensory neuropathy; 1 patient refused to continue taking the drug. The CsA dose was reduced in 13 patients, 12 because of nephrotoxicity and 1 because of headaches. One patient in whom administration of CsA was stopped developed azathioprine hepatotoxicity, whereas 1 patient whose dose was reduced developed acute cellular rejection. These complications were controlled by discontinuing azathioprine and reinitiating CsA. In the patients in whom CsA was discontinued, the mean serum creatinine level decreased from 2.42 +/- 0.48 to 1.72 +/- 0.39 mg/dL (P = .00004); in the patients in whom CsA was reduced, the mean serum creatinine level decreased less markedly. This report suggests that discontinuation of CsA along with increased doses of azathioprine is safe for some patients and may be effective in managing CsA-related nephrotoxicity and other side effects after liver transplantation. Additional studies are needed to determine whether this approach is safe and effective for the growing number of post-transplantation patients who may be candidates for such therapy.

Adult↗
PubMed1996

Splenic arteriovenous fistula. A rare lesion causing bleeding esophageal varices, ascites, and diarrhea.

We report the case of a 39-year-old woman with portal hypertension caused by a splenic arteriovenous fistula that was diagnosed by Doppler ultrasound and splenic arteriography. She presented with esophageal variceal hemorrhage and was initially treated with sclerotherapy. Ascites and secretory diarrhea then developed. At laparotomy portal pressure was 60 cm H2O but fell to 26 cm H2O after the fistula was resected with a splenectomy. All symptoms disappeared shortly thereafter, and the patient has remained well for the past two years.

Adult↗
PubMed1995

Chaparral ingestion. The broadening spectrum of liver injury caused by herbal medications.

Unconventional medical practices, including the use of herbal remedies, are prevalent in the United States. Chaparral is an herbal preparation made from a desert shrub and used for its antioxidant properties. We report the case of a 60-year-old woman who took chaparral for 10 months and developed severe hepatitis for which no other cause could be found. Despite aggressive supportive therapy, the patient deteriorated and required orthotopic liver transplantation. She is now well, more than 1 year after her transplant. This case suggests that chaparral can cause serious liver injury and fulminant hepatic failure. Herbal medications should be considered as potential causes of liver toxicity.

Chemical and Drug Induced Liver Injury↗
PubMed1995

Rapidly progressive sclerosing cholangitis following surgical treatment of pancreatic pseudotumor.

Two patients with sclerosing cholangitis presented with a distal stricture of the common bile duct in association with pancreatic pseudotumors. Jaundice resolved following surgery to correct biliary obstruction, but diffuse cholangiographic abnormalities and clinical evidence of sclerosing cholangitis became evident 2 and 4 months later. Rapid progression of symptomatic disease necessitated liver transplantation in one patient, but the other had a complete response to methotrexate therapy. The rapid disease progression in these two patients may have been triggered by surgery that resulted in a generalized fibroproliferative response of the biliary tree, already affected with localized sclerosing cholangitis contiguous to a pancreatic pseudotumor. We suggest that localized sclerosing cholangitis associated with pancreatic pseudotumors may be a unique variant that can progress rapidly but respond dramatically to antiinflammatory therapy.

Adult↗
PubMed1995

Dyslipidemias in childhood. An overview.

This article reviews the current screening and treatment guidelines for dyslipidemias in the pediatric and adolescent populations. In addition, the components of a comprehensive lipid program are reviewed.

Adolescent↗
PubMed1995

Liver transplantation at the University of Chicago.

Over the past 5 years, we have employed several strategies to increase the donor pool for both the pediatric and adult populations. The innovative expansion of the donor pool with the use living-related donors for children and cadaveric, high-risk donors for adults has increased our ability to serve our recipients and transplant them at an earlier stage in the disease process, thereby improving survival. As Hepatitis C is now the leading indication for liver transplantation in the adult population, the investigation of the natural history of Hepatitis C prior to and after transplantation provides a major challenge and is currently a focus of both laboratory and clinical efforts. For those recipients of Hepatitis C-positive-donor livers, determining the role of recipient and donor genotypes in the progression of recurrent hepatitis should help define the proper utilization of these organs. For patients on CsA-based immunosuppression regimens who experience steroid-resistant rejection, tacrolimus has proved to be extremely effective in reversing the rejection episodes and maintaining normal graft function. The long-term results of this therapy appear to be superior to OKT3 therapy. The recipients of living-related liver transplantation continue to have a survival advantage in comparison to recipients of cadaveric grafts. The donor operation can be routinely performed with minimal risk. Because of the superior results achieved and minimal donor risks, we feel that providing the option of living-donor transplantation is ethically justified, and medically necessary. Despite the encouraging results from living-donor transplantation, unexpected complications including portal vein complications and hepatic artery thrombosis have forced technical modifications of the original technique which may have implications to pediatric liver transplantation in general. As the volume of pediatric liver transplants and the number of immmunosuppressive regimens have increased over the years, posttransplant lymphoproliferative disease has been identified as a problem which requires more inspection. We have determined that the severity of rejection and the subsequent treatment, and primary Epstein-Barr virus are the primary risk factors for developing of PTLD. Identification of the risk factors and early detection may provide some hope for treatment of the disease while allowing long-term graft function. Results of our preliminary data show that, following transplantation, 3/4 of the children or parents report minimal impairment with regard to developmental or physical milestones. Patients and their families, however, continue to report significant levels of stress in their lives and occasional pain. Further research on outcome needs to be performed on our pediatric recipients to ensure the long-term benefit of our efforts.

Academic Medical Centers↗
PubMed1994

Performance of four computer-based diagnostic systems.

BACKGROUND: Computer-based diagnostic systems are available commercially, but there has been limited evaluation of their performance. We assessed the diagnostic capabilities of four internal medicine diagnostic systems: Dxplain, Iliad, Meditel, and QMR. METHODS: Ten expert clinicians created a set of 105 diagnostically challenging clinical case summaries involving actual patients. Clinical data were entered into each program with the vocabulary provided by the program's developer. Each of the systems produced a ranked list of possible diagnoses for each patient, as did the group of experts. We calculated scores on several performance measures for each computer program. RESULTS: No single computer program scored better than the others on all performance measures. Among all cases and all programs, the proportion of correct diagnoses ranged from 0.52 to 0.71, and the mean proportion of relevant diagnoses ranged from 0.19 to 0.37. On average, less than half the diagnoses on the experts' original list of reasonable diagnoses were suggested by any of the programs. However, each program suggested an average of approximately two additional diagnoses per case that the experts found relevant but had not originally considered. CONCLUSIONS: The results provide a profile of the strengths and limitations of these computer programs. The programs should be used by physicians who can identify and use the relevant information and ignore the irrelevant information that can be produced.

Analysis of Variance↗
PubMed1994

Gamma-globulin treatment of acute myocarditis in the pediatric population.

BACKGROUND: Myocardial damage in myocarditis is mediated, in part, by immunological mechanisms. High-dose intravenous gamma-globulin (IVIG) is an immunomodulatory agent that is beneficial in myocarditis secondary to Kawasaki disease, as well as in murine myocarditis. Since 1990, the routine management of presumed acute myocarditis at Children's Hospital, Boston, and Children's Hospital, Los Angeles, has included administration of high-dose IVIG. METHODS AND RESULTS: We treated 21 consecutive children presenting with presumed acute myocarditis with IVIG, 2 g/kg, over 24 hours, in addition to anticongestive therapies. A comparison group comprised 25 recent historical control patients meeting identical eligibility criteria but not receiving IVIG therapy. Left ventricular function was assessed during five time intervals: 0 to 7 days, 1 to 3 weeks, 3 weeks to 3 months, 3 to 6 months, and 6 to 12 months. At presentation, the IVIG and non-IVIG groups had comparable left ventricular enlargement and poor fractional shortening. Compared with the non-IVIG group, those treated with IVIG had a smaller mean adjusted left ventricular end-diastolic dimension and higher fractional shortening in the periods from 3 to 6 months (P = .008 and P = .033, respectively) and 6 to 12 months (P = .072 and P = .029, respectively). When adjusting for age, biopsy status, intravenous inotropic agents, and angiotensin-converting enzyme inhibitors, patients treated with IVIG were more likely to achieve normal left ventricular function during the first year after presentation (P = .03). By 1 year after presentation, the probability of survival tended to be higher among IVIG-treated patients (.84 versus .60, P = .069). We observed no adverse effects of IVIG administration. CONCLUSIONS: These data suggest that use of high-dose IVIG for treatment of acute myocarditis is associated with improved recovery of left ventricular function and with a tendency to better survival during the first year after presentation.

Acute Disease↗
PubMed1994

Penicillamine hypersensitivity: successful desensitization of a patient with severe hepatic Wilson's disease.

We report a 16-yr-old patient who developed a severe allergic reaction to penicillamine which was prescribed for newly diagnosed hepatic Wilson's disease. The patient's name was placed on the liver transplant waiting list because of the severity of her liver disease. We describe successful desensitization to penicillamine, so that medical therapy alone was sufficient for management of her condition.

Adolescent↗
PubMed1993

Effects of nutritional counseling on lipoprotein levels in a pediatric lipid clinic.

OBJECTIVE: To determine the impact of nutritional counseling on lipoprotein profiles in dyslipoproteinemic children. DESIGN: Retrospective case review. SETTING: An academic hospital-based pediatric lipid clinic in Boston, Mass. PARTICIPANTS: One hundred four newly referred children with primary dyslipoproteinemia. INTERVENTIONS: Nutritional recommendations were adapted from the National Cholesterol Education Program's step 2 diet. Three-day diet records were used to assess baseline and follow-up diets. RESULTS: Two thirds of the children continued to have excellent diets or improved their diets after counseling, but low-density lipoprotein cholesterol (LDL-c) values decreased by 15% or more in only 19% of children. The observed change in LDL-c was not significantly associated with nutritional counseling. However, a strong correlation was evident between dietary interventions and concentration of high-density lipoprotein cholesterol (HDL-c) values in serum. Marked fat restriction lowered HDL-c levels, while liberalization of use of fat, with emphasis on monounsaturates, in a subset of children following an excessively fat-restricted diet on presentation, appeared to improve HDL-c levels. CONCLUSIONS: After nutritional counseling, LDL-c levels decreased by 15% or more in only 19% of dyslipoproteinemic children referred for treatment. There were no clear predictors of LDL-c responsiveness, but changes in dietary fat intake appeared to significantly influence HDL-c levels.

Child↗
PubMed1993

Diagnosis of hepatic allograft rejection: role of liver biopsy.

The role of the liver biopsy for establishing a diagnosis of liver allograft rejection was investigated by examining under code 151 liver biopsies from 32 recipients and correlating the results with the clinical diagnosis determined by chart review. One-hundred-and-four biopsies were obtained to evaluate the cause of liver dysfunction and 47 were performed on a weekly protocol basis independent of the clinical status. A diagnosis of "definite histologic rejection" was made if a biopsy contained portal triaditis, bile duct injury, and endothelialitis, while biopsies were said to be suspicious for rejection if portal triaditis and bile duct injury were evident. A diagnosis of rejection using clinical and biochemical findings and response to therapy was made in association with 37 biopsies, of which 16 had definite histologic rejection and 13 were suspicious for rejection. Of the 114 biopsies performed in patients without clinical rejection, 71 had histology suspicious for rejection. Thus, biopsy was sensitive for the detection of rejection (78%), but was not very specific (33%). The positive predictive value was low (28%), while the negative predictive value was high (83%). These results indicate that liver biopsy is useful to evaluate graft dysfunction, to exclude rejection, to confirm clinically suspected rejection, and to diagnose other problems that can affect allograft function. Protocol biopsies appear to have little value.

Adolescent↗