摘要:

BackgroundThe frequency of vaso-occlusive crises correlates with mortality in patients with sickle cell disease (SCD). We examined the degree to which a high number of hospitalization days for these events affected survival.Patients and MethodsWe reviewed data for 58 adult patients with SCD (mean age, 29.9 ± 7.3 years) treated at our hospital between 1986 and 1994 who had at least 100 hospitalization days during any of these years. Their mean follow up period was 6.65 years (median, 6.84 years; range, 0.15–14.51 years).ResultsThirty-five patients (60.3%) died during follow-up. In multivariate analysis that included age, gender, and numbers of transfusions and hospitalization days, only age was significantly associated with mortality. The National Cooperative Study of Sickle Cell Disease (CSSCD) estimates the 10-year mortality at 15% for all 20-year old SS patients and also for all 30-year-old women. For 30-year-old men, the CSSCD estimated a 10-year mortality of about 28%. Thus, the 60.3% mortality of our patients after a median follow-up of only 6.84 years was substantially higher. Fifty-one patients were still alive after 1992, when hydroxyurea became available for SCD. The median survival of 15 hydroxyurea-treated patients was 7.3 years, compared with 4.3 years in 36 patients who did not take the drug.ConclusionsMortality for patients with SCD with a high number of hospitalization days was much higher than that expected for patients with SCD in general. There was a (nonsignificant) trend for longer survival in these severely ill patients if they took hydroxyurea.

ISSN:0002-9629    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

BackgroundNephropathy has long been recognized as a potential complication of congenital cyanotic heart disease (CCHD). The present study was undertaken to investigate some aspects of glomerular function by measuring urinary total protein, microalbumin, and tubular function by assessing urinary &agr;-1-microglobulin. The structural integrity of the renal proximal tubules was also studied by measuring urinary activities of the brush-border enzyme leucine-aminopeptidase and the lysosomal enzymeN-acetyl-&bgr;-d-glucosaminidase. The levels of hematocrit (Hct) and oxygen saturation were also investigated as predisposing factors for renal impairment in CCHD.MethodsThese investigations were done by recruiting 86 children who were grouped as follows: the control group (G1) consisted of 14 children (aged 4–12 years); the other 72 children with CCHD were divided according to age (ie, duration of cyanosis) into 4 equal groups, each containing 18 patients: G2(age <1 year), G3(age ≥1 year and <5 years), G4(age ≥5 years and <10 years), and G5(age ≥10 years). In addition, 10 of the 72 patients underwent a palliative surgery and were included as G6(regardless of age: 2 from G3, 4 from G4, and 4 from G5) to study the effect of the palliative surgery on the above-mentioned parameters.ResultsResults of the present work showed that with increasing duration of cyanosis (ie, on going from G2to G5) among the studied children with CCHD, there was a significant elevation in the urinary excretion of the investigated functional and structural parameters of the glomeruli and proximal tubules compared with the control children. The data also showed a significant increase in Hct, whereas oxygen saturation was significantly decreased. Results of G6after the palliative surgery demonstrated a significant decrease in the urinary excretion of the investigated parameters of the kidney, with a significant decrease in Hct and increase in oxygen saturation levels, compared with the results of the patients of this group before the palliative surgery.ConclusionsThese results suggest impairment of both glomerular and tubular functions as well as structure of the proximal tubules among children with CCHD and that the palliative surgery has significantly improved this impairment.

ISSN:0002-9629    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

ObjectivesDelay in maturation or failure of maturation of Cimino-Brescia fistulae contributes to the significant vascular access-related morbidity of chronic hemodialysis patients. Increased size and capacitance of native veins before the formation of vascular access has been considered an important variable in the success rate of native fistulae. We evaluated whether a formal exercise program might alter the size of native veins.MethodsThe effect of exercise on venous size was evaluated in 5 patients with severe chronic renal failure [glomerular filtration rate, 30.6 ± 5.3 mL/min (mean ± SD)]. Five male patients with a mean age of 57 ± 9 years underwent a 6-week forearm exercise training program, involving nondominant arms, that included isometric hand-grip contractions to 25 to 35% of MVC lasting 40 to 120 seconds and repetitive squeezing of squash and racquet balls. Both the volume and intensity of exercise training was increased weekly based on strength measured by hand-grip dynamometer and on the patients’ indicated level of comfort. Cephalic vessel size in both the nondominant (trained) and dominant (control) arms, with and without a tourniquet, were obtained using Doppler ultrasound before and after the 6-week exercise training program.ResultsThe size of the cephalic vein of the exercised arm increased significantly (P< 0.05) compared with the control arm when measured in both the absence (048 ± 0.016 versus 0.024 ± 0.023 cm2) and the presence of a tourniquet (0.056 ± 0.022 versus 028 ± 0.027 cm2).ConclusionsThese findings indicate that a simple, incremental resistance, exercise-training program can cause a significant increase in the size of the cephalic vein commonly used in the creation of an arteriovenous fistula. The increase in size and resultant probable increase in blood flow might accelerate the maturation of native arteriovenous fistulae, thereby lessening the morbidity associated with vascular access.

ISSN:0002-9629    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

A proportion of cases of breast and colon cancers are caused by inherited mutations that confer a greatly increased susceptibility to malignancy. Certain clinical features may help distinguish patients with a genetic cause for their cancer from the larger number of patients with sporadic tumors. A thorough family medical history is also necessary to identify those at high risk for developing cancer. Many of the normal functions of these genes are understood, and mutation analysis for patients and their families is now available as a clinical service. Presymptomatic detection of mutations allows the patient to pursue preventive measures to reduce the probability of developing a malignancy. Evidence is now available that some prophylactic measures do reduce the incidence of cancer and reduce mortality in mutation carriers, and the standard of care is evolving rapidly. The essential elements necessary to provide accurate interpretations of molecular genetics test results to patients are described.

ISSN:0002-9629    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

Peripheral arterial disease (PAD) is a prevalent illness that most commonly affects patients older than 60 years. As the population ages, the prevalence of PAD and its associated adverse outcomes will also increase. Adverse outcomes in PAD are either systemic (ie, cardiovascular events such as myocardial infarctions or strokes) or localized to the legs (ie, bypass surgery or amputation). Although much research has focused on adverse systemic outcomes in patients with PAD, less is known about those factors related to adverse limb events. The Centers for Disease Control and Prevention estimate that more than 100,000 amputations are performed in the United States each year. What remains to be determined is the association between how physicians manage patients with PAD (ie, process of care) and the development of adverse limb outcomes. Determining the association of the management of PAD with adverse limb outcomes will highlight those areas in which to focus to improve the quality of care for patients with this disease. Understanding the relationship between risk factors, process of care, and outcomes will be of importance to patients, clinicians, and policy makers. The purpose of this article is to review the burden of PAD and to discuss the association of process of care with adverse limb outcomes in patients with PAD.

ISSN:0002-9629    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

Hepatitis C virus (HCV) affects 1.8% of the American population, and approximately 38% of patients with HCV will manifest symptoms of at least 1 extrahepatic manifestation during the illness. Renal disease, neuropathy, lymphoma, and Sjögren syndrome with or without mixed cryoglobulinemia are all strongly associated with HCV infection. Porphyria cutanea tarda and diabetes have also been linked to HCV. Most extrahepatic manifestations of chronic HCV infection are immunological, and the chronic infection seems to be necessary for their development. The molecular study of the unique way in which the HCV virus interacts with the human immune system is beginning to provide plausible explanations of the pathogenic role of HCV in some of these syndromes, but many pathogenetic links remain completely obscure.

ISSN:0002-9629    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

We report 2 patients with polycythemia vera who were demonstrated to bebcr/abl-negative and were unable to tolerate either hydroxyurea or interferon-&agr; but who had excellent clinical responses to imatinib mesylate (STI-571). This effect is consistent with the inhibitory effect of imatinib mesylate on c-kit’s tyrosine kinase activity as demonstrated by its effectiveness in patients with gastrointestinal stromal tumors.

ISSN:0002-9629    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

Long-term licorice ingestion is a well-known cause of secondary hypertension and hypokalemia. Nevertheless, its initial presentation with a very severe degree of hypokalemia and paralysis is exceedingly rare. We report an elderly Asian man who presented to the emergency department with marked muscle weakness that progressed to paralysis. His blood pressure was 160/96 mm Hg. The major biochemical abnormalities were hypokalemia (plasma K+concentration, 1.8 mmol/L) and metabolic alkalosis (HCO3−, 36 mmol/L). His renal potassium excretion was higher (transtubular potassium gradient of 9). Plasma renin activity and aldosterone concentration were suppressed and cortisol concentration was normal. A detailed history revealed that he had ingested tea flavored with 100 g of natural licorice root containing 2.3% glycyrrhizic acid daily for 3 years. Note that renal potassium wasting and hypertension persisted for 2 weeks after discontinuing licorice consumption along with KCl supplement and spironolactone. Long-term licorice ingestion should be kept in mind as a cause of paralysis with an extreme degree of hypokalemia to avoid missing this recognizable and curable medical disorder.

ISSN:0002-9629    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

We present a rare case of malignant melanoma of unknown primary origin presenting as cardiac and cholecystic masses. In this 72-year-old man, a cardiac lesion caused right ventricular outlet obstruction, and gallbladder lesions led to hemobilia. Right atrial and gallbladder metastases were resected. After 2 years of disease-free survival, the patient had recurrent skin lesions, which were treated with local resection and dacarbazine. The patient has survived for more than 5 years after initial diagnosis, with continued complete clinical remission. This case illustrates the importance of aggressive surgical resection and chemotherapy in the treatment of metastatic malignant melanoma.

ISSN:0002-9629    

出版商:OVID    

年代:2003

数据来源: OVID