bluuebunny/crossref_metadata_2025_split huggingface dataset:
Fabry disease: a functional and anatomical study of cardiac manifestations in 20 hemizygous male patients
Authors: Senechal M , Germain DP 
Published: March 2003

Fabry disease (FD, OMIM 301500) is an X‐linked disorder of glycosphingolipid metabolism resulting from the deficient activity of α‐galactosidase A, a lysosomal acid hydrolase, leading to progressive lysosomal accumulation of incompletely metabolized neutral glycosphingolipids. Cardiac involvement is frequent. The objective of this study was to characterize the cardiac abnormalities in male patients affected with classic Fabry disease and define the context in which the clinicians were able to make the diagnosis.Clinical evaluation, laboratory tests, electrocardiography (ECG) and echocardiography were performed in 20 hemizygous men (mean age 39 years, range 12–65 years). The context of diagnosis was obtained by review of patients' charts.Left ventricular hypertrophy (LVH) and/or concentric remodeling were found in 12 patients (60%). Structural changes in mitral and aortic valves were found in 25% and 10% of cases, respectively. The sensitivity of the ECG Romhilt–Estes score for LVH was high (80%). Short PR interval (40%), ST segment abnormalities and conduction delay were frequent on ECG. Left ventricular mass index, ECG scores for LVH and systolic pulmonary pressure correlated positively with age. There was no relation between classic vascular risk factors and coronary artery disease, transient ischemic attack (TIA) or stroke. Diagnosis of Fabry disease was frequently suggested by nephrologists, dermatologists or geneticists.Echocardiograph and ECG abnormalities are frequently observed in patients with Fabry disease. Cardiologists should be aware of the diagnosis of Fabry disease in patients presenting with LVH, concentric remodeling, mitral and aortic valve thickening on echocardiography, short PR interval and conduction defects on ECG.
Compliance and late acute rejection after kidney transplantation: a psycho‐medical perspective
Authors: Baines Lyndsay S , Joseph John T , Jindal Rahul M 
Published: March 2003

Introduction: We examined the relationship between late acute rejection (LAR) after cadaveric kidney transplantation and medical compliance utilizing a modified version of the Long‐term Medication Behaviour Self‐efficacy Scale (LTMBS‐scale), a validated patient self‐report questionnaire. The original LTMBS‐scale uses a five‐point scale, however, our pilot study showed that patients found it difficult to discriminate between the five options. We therefore modified this to a three‐point scale.Patientsand methods: We carried out a retrospective analysis of all patients who received a kidney transplant in our unit in the cyclosporin (CyA) era. We divided rejections into early and late rejection based on the time interval after transplantation. Graft rejection was confirmed by biopsy; LAR was defined as acute rejection occurring after 90 d. We retrospectively administered the modified LTMBS‐scale to determine individual patient confidence and self‐efficacy in taking their medications in a variety of situations (home, work, leisure, psychological and physical). Individual patient confidence and self‐efficacy was analysed in relationship to compliance behaviour.Results: Twenty‐four questionnaires were distributed, 22 (92%) were returned fully completed. The overall results suggested that our patients surveyed were not particularly confident (mean score 2.17 out of maximum possible 3) in taking their medications in a variety of contexts. They demonstrated significantly less confidence (mean score 1.0) when experiencing physical (brittle bones, feeling `ill') and psychological (`sadness') side‐effects of medication and emotional reactions to the experience of chronic illness.Conclusion: Negative physical and psychological states were related to low self‐efficacy with the taking of immunosuppressive medication, non‐compliance and subsequent LAR in our cohort of patients.
Magnesium depletion in chronic terminal liver cirrhosis
Authors: Koivisto Mirja , Valta Päivi , Höckerstedt Krister , Lindgren Leena 
Published: March 2003

Koivisto M, Valta P, Höckerstedt K, Lindgren L. Magnesium depletion in chronic terminal liver cirrhosis. Clin Transplant 2002: 16: 325–328. © Blackwell Munksgaard, 2002Serum ionized magnesium represents less than 1% of the total body magnesium. The most reliable method to evaluate magnesium status is the magnesium loading test: In magnesium depletion its uptake is increased (20–50%) and is about 6% in normal magnesium status. There are no studies on magnesium status in chronic cirrhotics who may be in depletion. We performed magnesium loading test in 10 chronic cirrhotics listed for liver transplantation and in six healthy control patients. Magnesium sulphate 30 mmol was infused and urine magnesium was determined over 24 h. Serum ionized magnesium increased similarly in all patients. The uptake of magnesium was 8 ± 8% in control patients and 34 ± 26% in cirrhotics (p < 0.01). Chronic terminal cirrhotics are magnesium depleted which should be taken into account in case of liver transplantation and also in other interventions. Spot sampled serum ionized magnesium revealed magnesium depletion poorly.
Factors contributing to long graft survival in non‐heart‐beating cadaveric renal transplantation in Japan: a single‐center study at Kitasato University
Authors: Yoshida Kazunari , Endo Tadao , Saito Takeshi , Iwamura Masatsugu , Ikeda Masae , Kamata Koju , Sato Koshi , Baba Shiro 
Published: March 2003

Yoshida K, Endo T, Saito T, Iwamura M, Ikeda M, Kamata K, Sato K, Baba S. Factors contributing to long graft survival in non‐heart‐beating cadaveric renal transplantation in Japan: a single‐center study at Kitasato University. Clin Transplant 2002: 16: 397–404. © Blackwell Munksgaard, 2002A total of 107 cadaveric kidneys from non‐heart‐beating donors (NHBDs) have been transplanted between 1974 and 2000 at Kitasato University Hospital, Sagamihara, Japan. The patient survival of the 107 recipients of cadaveric renal transplants at 1, 5 and 10 yr was 0.857, 0.770 and 0.746, respectively. The 50% graft survival was 3.8 yr. The 5 and 10‐yr graft survival was 0.457 and 0.337, respectively. Twenty of the 107 recipients of non‐heart‐beating cadaveric renal transplantation had graft survival longer than 10 yr. Of these 20 patients, 14 survivors still maintain functioning renal grafts and two died with functioning graft, although the remaining four reverted to dialysis because of chronic rejection and nephropathy. The average graft survival of these 20 patients at the time of study was 13.3 yr and the longest was 21.4 yr. The average serum creatinine level at 10 yr after transplantation was 1.63 mg/dL, almost identical to that at 5 yr post‐transplant. The donors aged on average 40.2 yr; 13 were male and seven were female. The youngest donor was 9‐yr‐old and the oldest was 66. The graft survival was significantly better in the group with donor age younger than 55 yr (Log‐rank: p=0.007). The average weight of the renal graft was not different between the long and shorter graft survival groups. The average warm ischemic time and total ischemic time were 9.7 and 539.7 min, respectively. The duration of post‐transplant acute tubular necrosis averaged 9.2 days. These parameters tended to be shorter than those in recipients with graft survival >10 yr, but with no statistical significance. The mean numbers of acute rejection (AR) episode within 3 months after transplantation were 0.25 ± 0.66 and 0.92 ± 0.90 (p=0.020) in long survival and shorter survival groups, respectively. Long survivors had a significantly lower incidence of AR. Two of 20 cases received conventional immunosuppression with prednisolone, azathioprine and mizoribin, and 18 had prednisolone and calcineurin inhibitor (CNI). Kaplan–Meier analysis showed a significant contribution of CNI to graft survival (p=0.036). However, the graft survival reduction rate after 1 yr post‐transplant did not differ between conventional and CNI immunosuppression. These data suggest that renal grafts retrieved with proper organ procurement procedures from NHBDs may survive long‐term and help to overcome donor shortage.
Enteric drainage of a pancreas allograft is safe for patients with celiac sprue
Authors: Akoad Mohamed , Giraldo Mauricio , Monaco Anthony P , Hanto Douglas W , Uknis Marc E 
Published: March 2003

Akoad M, Giraldo M, Monaco AP, Hanto DW, Uknis ME. Enteric drainage of a pancreas allograft is safe for patients with celiac sprue. Clin Transplant 2002: 16: 387–388. © Blackwell Munksgaard, 2002Enteric drainage of exocrine secretions in whole organ pancreas transplantation is generally avoided in patients with pre‐existing small bowel disease; however, bladder drainage is associated with a 20% rate of urinary tract‐related complications. This is a case report of a type 1 diabetic patient with celiac sprue and renal failure. We performed a simultaneous cadaveric kidney pancreas transplant enterically draining the exocrine pancreas. There were no complications. The patient is now more than 6 months post‐transplant with excellent function of both renal and pancreas allografts. We conclude that enteric drainage of pancreas allografts in patients with celiac sprue may be performed safely.Whole organ pancreas transplantation is being performed with greater success than ever before, mostly as a result of lessons learned from past experience (1). Enteric drainage of allograft exocrine secretions is preferred for simultaneous pancreas/kidney (SPK) recipients to avoid urinary tract complications associated with bladder drainage. However, most agree that diabetics with pre‐existing bowel disease should have bladder drainage of allograft exocrine secretions, so as to prevent the devastating complication of a bowel leak. We describe here a successful case of enteric drainage of an SPK transplant in a patient with celiac sprue. We believe that, when carefully performed, enteric drainage of pancreas allografts is a safe approach for diabetic patients with celiac sprue, and may avert the serious complications associated with bladder drainage.
Concomitant intramyocardial and epicardial vasculitis in an autopsied heart allograft for cardiac rhabdomyosarcoma
Authors: Chang Yih‐Leong , Lin Chien‐Yu , Wang Shoei‐Shen , Kuo Kuan‐Ting , Lee Yung‐Chie , Wu Chen‐Tu 
Published: March 2003

Chang Y‐L, Lin C‐Y, Wang S‐S, Kuo K‐T, Lee Y‐C, Wu C‐T. Concomitant intramyocardial and epicardial vasculitis in an autopsied heart allograft for cardiac rhabdomyosarcoma. Clin Transplant 2002: 16: 461–464. © Blackwell Munksgaard, 2002Primary cardiac tumours are rare, with only one quarter of the patients being malignant. The vast majority of malignant neoplasms of the heart are sarcomas. We describe a patient of primary cardiac rhabdo‐myosarcoma presented as coronary artery disease and recurrent myocardial infarction. Histopathologic finding of the excised native heart revealed a high grade pleomorphic rhabdomyosarcoma in the myoepi‐cardial portion of the anterior wall with rupture. The accompanying unusual feature was myocardial infarction because of tumour emboli of the left anterior descending and left circumflex coronary arteries. After transplantation, the patient developed mild to moderate acute cellular rejection of the transplanted heart on post‐transplantation day 1, 8, and 44, respectively. Unfortunately, he expired on the post‐transplantation day 47 because of acute rejection, presenting as concomitant intramyo‐cardial and epicardial lymphocytic vasculitis and multifocal myocardial ischaemia. We found that this uncommon medial lymphocytic vasculitis lesion was mediated by T cells and also by antibody directly against smooth muscle cells of small arteries. The consequence of such immune response would be compromised myocardial oxygenation resulting in allograft failure.
The apo A‐I gene promoter region polymorphism determines the severity of hyperlipidemia after heart transplantation
Authors: González‐Amieva Angel , López‐Miranda José , Marín Carmen , Pérez‐Martinez Pablo , Gómez Purificación , Paz‐Rojas Elier , Arizón Josè M. , Jiménez‐Perepérez José A. , Concha Manuel , Pérez‐Jiménez Francisco 
Published: March 2003

Abstract: Background: To study whether the Apolipoprotein A‐I (apo A‐I) promoter region gene polymorphism produces changes in the lipid profile of heart transplant recipients.Methods: One hundred and three heart transplant recipients (93 men and 10 women, with a mean age of 47 ± 13 yr) receiving triple immunosuppressive therapy were submitted to a genetic study of the apo A‐I gene promoter region. Anthropometric and analytical data, including lipid profile, arterial blood pressure, were collected prior to transplantation and 3, 6, 12, and 24 months after transplantation.Results: Sixty‐three subjects had the GG genotype and 40 the GA genotype. Carriers of the GA genotype had higher triglyceride levels at 6 months and 2 yr (2.50 ± 1.20 versus 1.93 ± 0.98 mmol/L and 2.46 ± 1.58 versus 1.60 ± 0.68 mmol/L, respectively, p < 0.001), and a greater rise in LDL‐cholesterol at 1 yr than the GG subjects (4.57 ± 1.16 versus 4.16 ± 1.18 mmol/L, p < 0.05). Multiple regression analyses showed that genetic variants at the apo A‐I promoter region are responsible for 11% of the variability in triglyceride levels at 6 months (p = 0.005).Conclusions: The GA genotype of the apo A‐I promoter region produces a greater rise in plasma triglyceride and LDL‐cholesterol levels in heart transplant patients.
Choline, myo‐inositol and mood in bipolar disorder: a proton magnetic resonance spectroscopic imaging study of the anterior cingulate cortex
Authors: Moore Constance M , Breeze Janis L , Gruber Staci A , Babb Suzann M , DeB Frederick Blaise , Villafuerte Rosemond A , Stoll Andrew L , Hennen John , Yurgelun‐Todd Deborah A , Cohen Bruce M , Renshaw Perry F 
Published: March 2003

Objectives:

Alterations in choline and myo‐inositol metabolism have been noted in bipolar disorder, and the therapeutic efficacy of lithium in mania may be related to these effects. We wished to determine the relationship between anterior cingulate cortex choline and myo‐inositol levels, assessed using proton magnetic resonance spectroscopic imaging (MRSI), and mood state in subjects with bipolar disorder.

Methods: Serial assessments of anterior cingulate cortex choline and myo‐inositol metabolism were performed in nine subjects with bipolar disorder, taking either lithium or valproate, and 14 controls. Each bipolar subject was examined between one and four times (3.1±1.3). On the occasion of each examination, standardized ratings of both depression and mania were recorded.

Results: In the left cingulate cortex, the bipolar subjects’ depression ratings correlated positively with MRSI measures of Cho/Cr‐PCr. In the right cingulate cortex, the Cho/Cr‐PCr ratio was significantly higher in subjects with bipolar disorder compared with control subjects. In addition, bipolar subjects not taking antidepressants had a significantly higher right cingulate cortex Cho/Cr‐PCr ratio compared with patients taking antidepressants or controls. No clinical or drug‐related changes were observed for the Ino/Cr‐PCr ratio.

Conclusions: The results of this study suggest that bipolar disorder is associated with alterations in the metabolism of cytosolic, choline‐containing compounds in the anterior cingulate cortex. As this resonance arises primarily from phosphocholine and glycerophosphocholine, both of which are metabolites of phosphatidylcholine, these results are consistent with impaired intraneuronal signaling mechanisms.
Health care utilization and costs among privately insured patients with bipolar I disorder
Authors: Bryant‐Comstock Lynda , Stender Monika , Devercelli Giovanna 
Published: March 2003

Objective:  This study examined health care resource utilization and direct health care costs among patients diagnosed with bipolar I disorder in a privately insured population.Methods:  Health care claims data for 2883 patients with a primary diagnosis of bipolar disorder were compared over a 1‐year period (1997) with claims data for 2883 randomly selected, age‐ and sex‐matched, non‐bipolar patients, all covered under the same large private insurer in USA. Resource use (i.e. original and refill pharmaceutical dispensing, medical and procedural services received, inpatient hospitalization, outpatient services, physician visits and emergency room treatment) and their costs are described overall, as well as by bipolar disorder diagnosis (based on ICD‐9 codes) and type of care (i.e. mental health versus non‐mental health).Results:  Bipolar patients utilized nearly three to four times the health care resources and incurred over four times greater costs per patient compared with the non‐bipolar group during the 1‐year period ($7663 versus $1962). Inpatient care (hospitalizations) accounted for the greatest disparity between groups, as it was the single‐most costly resource in the bipolar group ($2779 versus $398). Patients with bipolar depression (among the single bipolar diagnostic categories of mixed, manic or depressed) incurred the highest health care costs. While mental health care cost was a significant component of total cost in the bipolar group, it accounted for only 22% of the total per‐patient cost; in comparison, it accounted for only 6% of the total per‐patient cost in the non‐bipolar group.Conclusion:  Treatment of bipolar disorder, particularly inpatient care, is costly to patients and health insurers. Further study is needed to find ways to reduce the overall cost of managing these patients without jeopardizing patient care.
Impact of concurrent alcohol misuse on symptom presentation of acute mania at initial evaluation
Authors: Salloum Ihsan M , Cornelius Jack R , Mezzich Juan E , Kirisci Levent 
Published: March 2003

Objectives:  The aim of this study was to evaluate the impact of current alcohol misuse on symptom presentation of acute mania.Methods:  The impact of concurrent alcohol misuse on symptom presentation of acute mania was examined by comparing comorbid subjects with acute bipolar mania complicated by current alcohol misuse (n=60) with subjects with acute bipolar mania without current alcohol misuse (n=196).Results:  Age‐ and gender‐controlled analysis revealed that the comorbid group presented with more severe psychopathology, as indicated by higher number of total mood‐related symptoms as well as of higher total number of manic symptoms. Specifically, they presented with significantly higher rates of mood lability and impulsivity, and also demonstrated higher rates of violent behavior, and other drug use.Conclusions:  Acute mania complicated by current alcohol misuse is differentiated from acute mania without alcohol misuse by the presence of higher numbers of manic symptoms and increased high risk behavior such as mood lability, impulsivity, violence, and other drug abuse.
Tiagabine in treatment refractory bipolar disorder: a clinical case series
Authors: Suppes Trisha , Chisholm Karrie A , Dhavale Dawn , Frye Mark A , Altshuler Lori L , McElroy Susan L , Keck Paul E , Nolen Willem A , Kupka Ralph , Denicoff Kirk D , Leverich Gabrielle S , Rush A John , Post Robert M 
Published: March 2003

Objectives:  Anticonvulsants have provided major treatment advances for patients with bipolar disorder. Many of these drugs, including several with proven efficacy in bipolar mania or depression, enhance the activity of the γ‐amino butyric acid (GABA) neurotransmitter system. A new anticonvulsant, tiagabine, has selective GABAergic activity and is approved for patients with partial epilepsy. Few reports of its potential effectiveness in bipolar disorder, however, have been published. We sought to evaluate the effectiveness of tiagabine added to ongoing medication regimens in patients with bipolar disorder inadequately responsive to or intolerant of usual treatments.Methods:  Seventeen treatment‐refractory patients participating in the Stanley Foundation Bipolar Network (SFBN) long‐term follow‐up study were offered open treatment with add‐on tiagabine after discussion of the risks, benefits, other treatment options and giving informed consent. Patients’ clinical symptoms and somatic complaints were closely monitored with SFBN longitudinal and cross‐sectional ratings. Four patients discontinued low‐dose tiagabine prior to the second visit and were excluded from data analysis.Results:  Thirteen patients received a mean of 38 days of treatment at a mean dose of 8.7 mg/day of tiagabine. On the Clinical Global Impression Scale for Bipolar Disorder Overall category, three (23%) patients showed much or very much improvement and 10 (77%) patients showed no change or worsening. Three significant adverse events were noted, including two presumptive seizures.Conclusions:  Open add‐on tiagabine for treatment‐refractory patients with bipolar disorder demonstrated limited efficacy with the majority of patients showing no change or worsening of clinical symptoms. In addition, patients experienced serious side‐effects attributed as likely due to the medication, which resolved without lasting consequence when tiagabine was discontinued.
Gabapentin augmentation therapy in bipolar depression
Authors: Wang Po W , Santosa Claudia , Schumacher Matthew , Winsberg Mirène E , Strong Connie , Ketter Terence A 
Published: March 2003

Background:  Gabapentin (GBP) may be useful in bipolar disorders, including as adjunctive therapy for bipolar depression, although controlled studies suggest inefficacy as primary treatment for mania or treatment‐resistant rapid cycling.Methods:  We performed a 12‐week trial of open GBP (mean dose 1725 mg/day) added to stable doses of mood stabilizers or atypical antipsychotics in 22 (10 women, mean age 38.4 years) depressed [28‐item Hamilton Depression Rating Scale (HDRS) >18] bipolar (10 bipolar I, 12 bipolar II) disorder outpatients. Mean illness duration was 18.6 years; current depressive episode duration was 18.0 weeks. Prospective 28‐item HDRS, Young Mania Rating Scale (YMRS) and Clinical Global Impression – Severity (CGI‐S) ratings were obtained.Results:  Overall, HDRS ratings decreased 53% from 32.5 ± 7.7 at baseline to 16.5 ± 12.8 at week 12 (p < 0.0001). Twelve of 22 (55%) patients had moderate to marked improvement (HDRS decrease=50%) with HDRS decreasing 78% from 27.9 ± 6.2 to 6.2 ± 4.5 (p < 0.0001). Eight of 22 (36%) patients remitted (HDRS≥8). In non‐responders, HDRS decreased from 38.0 ± 5.4 to 28.9 ± 6.7 (p=0.005). Ten of 13 (77%) mild to moderately depressed (baseline HDRS >18 and <35) patients responded, while only two of nine patients (22%) with severe depression (HDRS ? 35) responded (p < 0.03). Both groups, however, had similar, statistically significant HDRS decreases. GBP was well tolerated.Conclusion:  Open adjunctive GBP was effective and well tolerated in patients with mild to moderate bipolar depression. This open pilot study must be viewed with caution, and randomized controlled studies are warranted.