Hirschsprung's disease in young adults

Hirschsprung's disease is rarely seen in the young adult, and presents unique problems in management because of the massive dilatation and hypertrophy that occur proximal to the aganglionic rectum or the rectosigmoid colon. The diagnosis, which may be suspected by barium enema, is confirmed by suction or full-thickness biopsy of the rectum that may be complemented by anal manometry. Based on our experience with eight patients, a two-stage surgical reconstruction is recommended, with a preliminary sigmoid colostomy through the normally innervated colon and an associated defunctionalized stoma constituting the initial operation. The distal colonic stoma permits cleansing of the caudal colon while the normally innervated proximal colon reverts to near normal caliber, usually within 2 to 6 months. This approach is in accord with the recommendation of Fairgrieve. Reconstruction using a Duhamel or Soave procedure has given good results. The Duhamel procedure seems preferable when a considerable discrepancy remains between the ganglionic and aganglionic segments of rectum.     

Diverticular disease in young adults

Acute diverticulitis of the colon should be considered in the differential diagnosis in young adult (younger than 40 years) patients with pain and tenderness in the lower abdomen. Among young adults, men are afflicted more commonly than women and the disease runs a more virulent course. Complications are commonly encountered, and the key to diagnosis is a high index of suspicion. Barium enema examination without bowel preparation is useful and safe in this age group. In patients with acute diverticulitis with perforation and abscess, the most appropriate therapy is surgical, as demonstrated in the three cases reported here.     

Carotid artery disease in young adults

The angiographic and clinical findings in 17 young adults (14-39 years) with stenosis and/or occlusion of at least one of the common or internal carotid arteries were reviewed to determine the spectrum of carotid artery disease in this age group. Five aetiological groups were identified: atherosclerosis (3 patients), arteritis (4), non-inflammatory arteriopathies (6), compression by neoplasm (1), and occlusions of undetermined cause (3). Over a mean follow-up time of 19.7 months, 2 patients died (12.5%) and further cerebrovascular events occurred in 4 others (25%). We conclude that carotid angiography plays a meaningful role in providing a definitive diagnosis in young adults with carotid artery disease and thus may assist with therapy and prognosis.     

Matrix metalloproteinase expressions in arteriosclerotic aneurysmal disease

Medial degeneration of extracellular matrix (ECM) proteins in the wall of abdominal aortas results in smooth muscle cell destruction, a loss of architectural integrity, and abdominal aortic aneurysm (AAA) formation. It has been theorized that an imbalance between proteinases and their naturally occurring inhibitors is the cause of these observed histologic abnormalities. Therefore, the purpose of this investigation was to determine if differences in the matrix metalloproteinase (MMP) -2 and -9, tissue inhibitor of metalloproteinase-1 (TIMP-1), tissue-type plasminogen activator (tPA), and urokinase-type plasminogen activator (uPA) protein and activity levels existed between infrarenal AAA and normal abdominal aortic tissue specimens. Between November 1995 and January 1997, 10 patients undergoing elective infrarenal AAA repair had a portion of their aneurysm walls snap frozen in liquid nitrogen and processed for subsequent western blot or zymographic analysis. Tissue specimens from 6 normal abdominal aortas obtained from fresh cadaver specimens were similarly processed and served as controls. Protein levels for MMP-2, MMP-9, TIMP-1, uPA, and tPA were analyzed by western blotting. The degree of MMP-2 and MMP-9 gelatinolytic activity was analyzed by zymography. Detection and immunolocalization for MMP-2, MMP-9 and CD68 was performed on tissue sections of AAA and normal infrarenal abdominal aortas fixed in 10% formalin. MMP-9 and tPA protein levels were increased in AAAs compared to controls by western blotting. However, uPA levels were slightly increased in controls. No differences in TIMP-1 protein levels were identified. Similarly, zymography demonstrated increased MMP-2 and MMP-9 gelatinolytic activity in AAAs compared to controls (p < or = 0.05). CD68-positive cells (macrophages) in the adventitia and media demonstrated immunoreactivity to MMP-9. This investigation demonstrated increased MMP-9 proteinase activity and tPA protein levels in the walls of AAAs, as well as inflammatory leukocyte invasion of the adventitia and media compared to controls. These data suggest that leukocyte-derived MMP-9 is associated with aortic wall degeneration and aneurysm formation. Furthermore, activation of MMP-9 may be caused by increased tPA levels in the walls of AAAs.     

358例下肢动脉硬化闭塞症外科治疗效果分析

目的 分析慢性下肢动脉硬化闭塞症外科治疗方法及疗效.方法 回顾性分析2002年1月至2007年7月序贯治疗的358例慢性下肢动脉硬化闭塞症患者接受动脉搭桥或介入治疗后远期疗效.结果 男293例,女65例,年龄(66 ±10)岁.本组接受了包括搭桥手术、介入治疗、截肢(趾)等外科治疗共413例(次).失访48例,随访率86.6%,随访时间6～64个月.髂动脉支架介入治疗术后1、2、3年初次开通率高于腹主-髂-股动脉人工血管搭桥,差异无统计学意义(P＞0.05),也高于股(腘)动脉支架介入治疗术后初次开通率,但差异有统计学意义(P＜0.05).股-腘动脉膝上人工血管搭桥术后1年的初次开通率略高于膝下搭桥,但差异无统计学意义(P＞0.01);术后2、3年初次开通率均高于膝下搭桥,差异有统计学意义(P＜0.01).股浅动脉球囊扩张成形术术后1年通畅率略低于腘动脉以远的球囊扩张术,但差异无统计学意义(P＞0.05).全组截肢率为10.3%(37/358),围术期死亡率3.9%(14/358),随访期死亡率6.4%(23/358).结论 下肢动脉硬化患者根据病情选择适当的外科治疗方式,可以获得较高的术后通畅率.     

Pneumococcal vaccine in children and young adults with chronic renal disease

BACKGROUND.: Pneumococcal vaccination has been recommended for immunocompromisedchildren over 2 years including patients with chronic renaldisease. However, the effect of vaccination and revaccinationis variable and the indication for immunization is a subjectof controversy. METHODS.: Forty children and young adults with chronic renal diseases(including the idiopathic nephrotic syndrome, chronic renalfailure, patients undergoing dialysis and after transplantation)were vaccinated with a 23-valent pneumococcal vaccine. The efficacyof the vaccine was evaluated by measuring antibody titres beforeand 4 weeks, 6 months, and 12 months after vaccination. Twenty-twopatients were submitted to a revaccination 1 year after thefirst vaccination. RESULTS.: A sufficient immune response, defined as an at least fourfoldincrease of postvaccinal antibody titres and an antibody titre>200, was observed in 83% of the patients 4 weeks after vaccination,but only in 68% after 6 months, and in 48% after 1 year. Revaccinationproduced a significant immune response in 11/22 patients (50%)followed by a rapid decline of antibody levels within 6 months.Both vaccinations were well tolerated. CONCLUSIONS.: The currently available vaccine is without major side-effectsand effective in producing a significant immune response. Antibodylevels should be monitored in vaccinated patients with chronicrenal diseases considering the rapid decline as early as 6 monthsafter vaccination. Evaluation of the efficacy of revaccinationin these patients requires further investigations.     

Racial differences in early-onset renal disease among young adults: the coronary artery risk development in young adults (CARDIA) study

Although 11 million people in the United States have chronic renal insufficiency, little is known about ethnic/racial disparities for early-onset renal impairment. This study sought to determine whether there is an independent association between race/ethnicity and early-onset renal impairment and to identify other risk factors that might account for observed disparities. All Coronary Artery Risk Development in Young Adults subjects in which serum creatinine was measured at the year 15 examination were identified (n = 3554), excluding those who were pregnant at year 15. Potential risk factors at study entry (ages 18 to 30 yr, 1985 to 1986) included age, weight, gender, race/ethnicity, glucose, uric acid, and systolic BP. Renal impairment was defined as creatinine > or =1.5 mg/dl for men and > or = 1.2 mg/dl for women at year 15 (ages 33 to 45 yr). Fifty-two (2.7%) women and 39 (2.4%) men had renal impairment at the year 15 examination. In bivariate analyses, the odds of renal impairment among black women was estimated to be 2.4-fold that of white women, and among black men, the odds of renal impairment were 9.0-fold that of white men. In multivariate analysis, the odds of an elevated creatinine among black women compared with white women reduced to a nonsignificant 1.5-fold, whereas among men, the odds of an elevated creatinine among blacks was 11.4-fold that of whites. Although adjustment for baseline glucose levels accounted for much of the association between ethnicity and elevated creatinine among women, adjustment for weight, systolic BP, uric acid, glucose, and socioeconomic status did not account for the association between ethnicity and renal impairment among men. The data suggest that there are ethnic differences in the development of early-onset renal dysfunction. Among women, these differences are modest and largely accounted for by differences in glucose levels early in adult life. Differences in race/ethnicity related risk of early-onset renal impairment are particularly large among men and are not accounted for by the metabolic or socioeconomic factors evaluated.     

Increased arterial stiffness in young adults with end-stage renal disease since childhood

Increased arterial stiffness is a risk factor for mortality in adults over 40 yr of age with end-stage renal disease (ESRD). As no data exist on vascular changes in young adults with ESRD since childhood, a long-term outcome study was performed. All living Dutch adult patients with onset of ESRD between 1972 and 1992 at age 0 to 14 yr were invited for carotid artery and cardiac ultrasound and BP measurements. Data on clinical characteristics were collected by review of all medical charts. Carotid ultrasound data were compared with those of 48 age-matched and gender-matched healthy controls. Carotid artery and cardiac ultrasound was performed in 130 out of 187 eligible patients. Mean age was 29.0 (20.7 to 40.6) yr. Compared with controls, patients had a similar intima media thickness but a reduced mean arterial wall distensibility DC (40.0 versus 45.0 kPa(-1). 10(-3); 95% CI, -9.1 to -0.8; P < 0.001), an increased stiffness parameter beta (4.2 versus 3.8; 95% CI, 0.05 to 0.68; P = 0.02), an increased elastic incremental modulus E(inc) (0.35 versus 0.27 kPa. 10(3); 95% CI, 0.02 to 0.12; P < 0.001). Multiple regression analyses in all subjects revealed that ESRD was associated with an increase in beta and E(inc). Arterial wall properties of patients currently on dialysis and transplanted patients were comparable. In all patients, current systolic hypertension was associated with increased E(inc) and decreased DC. In conclusion, carotid arterial wall stiffness is increased in young adult patients with pediatric ESRD. Hypertension is a main determinant and might be a target for treatment of these potentially lethal arterial wall changes.     

Acquired renal cystic disease in children and young adults on maintenance dialysis

Acquired renal cystic disease (ARCD) is a well-known complication of end-stage renal disease (ESRD). We studied 24 patients, aged 8 – 27 years (mean 19.8±5.3 years), on chronic maintenance dialysis in our service. The duration of dialysis ranged between 13 and 192 months (mean 77.8±44.3 months). High-resolution ultrasonography revealed ARCD in 11 (45.8%) patients. No cysts were seen in 7 (29.1%) patients and solitary cysts in one or both kidneys were seen in 6 (25%) patients. Renal malignancy was diagnosed in 2 patients. One, 15 years old, had renal cell carcinoma after being on dialysis for 6 years. She did well after bilateral nephrectomy, left salpingo-oophorectomy, and regional lymphadenectomy. The second patient, 23 years old, had been on dialysis for 16 years when she developed renal oncocytoma. She died of congestive cardiomyopathy 6 months later. We conclude that ARCD is common in children and young adults with ESRD. Neoplastic transformation, although rare, is a potential complication. Annual follow-up with ultrasonography with selective use of computed tomography or magnetic resonance imaging is advised. Received July 29, 1996; received in revised form and accepted November 15, 1996     

Early coronary calcification in children and young adults with end-stage renal disease

Cardiovascular disease is a major cause of morbidity and mortality in children and young adults with end-stage renal disease. In our study, we retrospectively analyzed the records of 11 patients who had undergone electron beam computerized tomography in our dialysis unit. Our patients, aged 11 to 24 years (median, 19.3 years) were on dialysis or had functioning grafts. Coronary calcification was observed in seven patients (64%) with a mean calcium score of 273.8 +/- 708 (range 0.8 to 1864) in our study population. We compared clinical characteristics like age, gender, duration of end-stage renal disease, time on hemodialysis, body mass index, and blood pressures between the patients with calcifications (group I) and those with out calcification (group II). We also compared the laboratory data including daily calcium and calcitriol intake, lipid profile, serum calcium and phosphorus levels, calcium/phosphorus products, and serum parathyroid hormone levels in the both groups. The mean daily dose of total calcium, triglyceride level, and calcium/phosphorus products were higher in the calcification group though not statistically significant. The mean daily dose of calcitriol was significantly higher in patients with calcification. Using Spearman multivariate correlation, we found a correlation between the coronary calcium scores and mean daily doses of total calcium and calcitriol (r = .750, P =.008 and r = .869, P = .001, respectively). We conclude that coronary calcification, which is a proven predictor of cardiovascular disease, begins at a very early age and that daily doses of elemental calcium and calcitriol seem to be important factors in our study population.