Lower quality of life in young men after pediatric kidney transplantation when compared to healthy controls and survivors of childhood leukemia—a cross‐sectional study

Data about health‐related quality of life (HRQOL) in adult recipients after pediatric kidney transplantation (KTx) are scarce. In this nationwide questionnaire‐based study, HRQOL and social status in young adult men having undergone KTx during childhood (n = 29) were studied and compared to age‐ and gender‐matched healthy controls (n = 56) and survivors of childhood acute lymphoblastic leukemia (n = 52) comprising controls with another chronic disease of childhood. Altogether 41% of the KTx recipients, 50% of the leukemia survivors and 80% of the healthy controls lived in a permanent relationship. When compared with leukemia survivors, the KTx recipients reported significantly more bodily pain and worse general health (RAND‐36). Older age at time of study, longer duration of dialysis, multiple transplantations and diminished graft function correlated with lower scores. The KTx recipients had a significantly higher mean Beck Depression Inventory (BDI) score than the leukemia survivors (P = 0.000) or the healthy controls (P = 0.006). BDI scores were highest in patients who lived without a partner or children had lower educational level or were unemployed. KTx recipients had significantly lower HRQOL scores than their healthy and controls with childhood chronic disease. Early detection of psychosocial problems and poor physical functioning among these patients is warranted.     

Comparative study of complete versus incomplete Kawasaki disease in 59 pediatric patients

ObjectivesTo compare the clinical and laboratory features and the rate of echocardiographic coronary artery abnormalities in patients with complete and incomplete forms of Kawasaki disease (KD) and to determine which additional clinical criteria might support a suspicion of KD.MethodsWe retrospectively reviewed the medical records of patients with KD who were admitted to the general pediatrics department of the Kremlin Bicêtre Teaching Hospital, France, between January 1995 and May 2006. We compared patients with a fever and four or five of the principal criteria (complete KD) to the other patients (incomplete KD). Clinical and laboratory features were abstracted from the records.ResultsWe identified 63 patients with a mean age of 33 months (± 31). The male-to-female ratio was 2.47. Four patients were excluded. Of the remaining 59 patients, 39 had complete KD and 20 incomplete KD. The group with complete KD had significantly higher rates of changes in the extremities, conjunctival injection, exanthem, and enanthem; and a significantly lower rate of coronary artery dilation (48.7% vs. 90% in the incomplete KD group, P = 0.002). Serum levels of alanine aminotransferase and gamma glutamyl transferase were significantly higher in the complete KD group. No significant differences were found between the two groups regarding age, sex, blood cell counts, or laboratory markers for inflammation. Pyuria was found in 45.4% of patients with complete KD and in 30.8% of those with incomplete KD (P = 0.17). Of 14 patients who underwent ophthalmological evaluation, two had uveitis; both of them had complete KD.ConclusionIncomplete KD shares with complete KD a risk of coronary artery disease. The diagnosis of incomplete KD is challenging but can be supported by the presence of features other than the principal criteria, such as acute anterior uveitis or unexplained pyuria.     

Renal function up to the 5th decade of life after nephrectomy in childhood: A literature review

The aim of the present study was to find out if in children ablation of 50% of renal mass may be associated with a progressive renal damage (Brenner’s hypothesis). We collected 1035 adult or adolescent survivors who underwent nephrectomy for unilateral oncological or non‐oncological causes during childhood. Stratification of all survivors for age revealed that the number of subjects with blood hypertension and/or renal dysfunction (glomerular filtration rate < 90 mL/min per 1.73 m²) to be significantly higher in survivors ≥30 years old in comparison with younger patients. Available data on long‐term renal function after nephrectomy during childhood support the Brenner’s hypothesis.     

Long-Term Survivors After Pediatric Liver Transplatation Are at Increased Risk for Development of Cardiovascular Disease Events: Analysis of 30 Cases

BackgroundLiver transplantation (LT) in adult patients is associated with a higher incidence of cardiovascular risk factors (CVRF), chronic kidney disease (CKD), and cardiovascular disease mortality than the general population. Available information about these problems in adult patients with LT from a pediatric age is limited. The aim of this study was to analyze the incidence of CVRF, risk of developing CKD, and risk of 10-year coronary event in adult patients who received LT in childhood.MethodsThirty adult patients (11 female, 19 male; mean age, 29.3 years) who underwent LT in childhood were analyzed, and CVRF, estimated glomerular filtration rate, and current immunosuppressive regimen were recordered. The risk of 10-year coronary event was calculated with the use of validated equations (Framingham and Regicor) and compared with the estimated risk in the general population.ResultsNone of the patients had CVRF before LT, except 1 patient who received a transplant because of familial hypercholesterolemia. Median age of patients at the time of study was 28.6 years (range, 19.3–43.1 y), and mean follow-up after LT was 17.83 ± 5.21 years. Twenty-nine patients (96.7%) were receiving a calcineurin inhibitor (69% tacrolimus, 31% cyclosporine), along with steroids in 13 of them. The average CVRF per patient was 2, and 11 patients (43.33%) had ≥3. Thirteen patients (43.33%) had CKD. The estimated risk of developing a coronary event at 10 years according to the Framingham score was 3%, higher than expected in the general population of same age and sex. With the use of the Regicor equation, adapted to the Spanish population, the estimated cardiovascular risk was 1.6%, corresponding to Spanish men without CVRF aged 50–55 years. None of the patients had cardiovascular events during the follow-up.ConclusionsOur data show a high incidence of CVRF and CKD in young adults who received LT in childhood, resulting in an increased risk of cardiovascular disease.     

Surgical management of the preinfarction syndrome

The indications for coronary reconstruction have been extended to include those patients with the preinfarction syndrome who have failed to respond to medical therapy. Preinfarction syndrome is characterized by: (1) rapidly progressive angina; (2) exacerbation of previously stable angina; and (3) recurrent bouts of coronary insufficiency. During a three-year period 60 patients with this syndrome ranging in age between 30 and 72 years underwent urgent or emergency bypass procedures. Twenty showed electrocardiographic evidence of previous myocardial infarction. Single-vessel disease (> 75% obstruction) was demonstrated by coronary angiography in 18, double-vessel disease in 14, and triple-vessel involvement in 28. The operative technique is described. Eight sustained an early and 5 a late myocardial infarction with 1 and 3 deaths, respectively. A detailed analysis of these patients is presented including restudies and pathological findings. Of the 56 survivors, only 2 continue to have incapacitating angina. From this experience we conclude that the surgical management of a selected group of patients with the preinfarction syndrome is associated with a lower mortality and an improved functional result when compared with the natural history of the syndrome.     

Anaesthesia for abdominal aortic surgery — a review (Part II)

Patients presenting for abdominal aortic surgery have a high incidence of vascular disease elsewhere, manifested primarily by hypertension, coronary and cerebrovascular disease, as well as co-existing respiratory, renal and metabolic disorders. Routine clinical assessment, electrocardiogram, chest roentgenograms, resting and exercise radionuclide ventriculography and echocardiography, dipyrdiamole-thallium scanning are all designed to assess the functional status of the myocardium and to detect the presence of significant coronary artery disease. Patients with no abnormalities on physical examination, routine evaluation and no redistribution on dipyridamole-thallium scanning should proceed to surgery with the expectation of very low perioperative cardiac risk. Patients with evidence of coronary artery disease and significant redistribution on dipyridamole-thallium scan should undergo coronary angiography and possible myocardial revascularization before definitive aortic vascular surgery. For high cardiac risk patients with no bypassable lesions presenting for abdominal aortic aneurysm resection a conservative policy of serial three monthly ultrasound or CT assessment may be adopted, with selective resection for rapid aneurysm expansion or symptom development. A variety of extra-anatomical and angioplastic techniques is available for similar high cardiac risk patients with aortoiliac occlusive disease. The haemodynamic consequences of aortic cross-clamping, especially in aneurysm patients, include a significant reduction in stroke volume, cardiac index, and myocardial oxygen consumption with an increased systemic vascular resistance. Patients with coronary artery disease may respond to aortic cross-clamping by increasing pulmonary capillary wedge pressure and by demonstrating ECG evidence of myocardial ischaemia. Pulmonary artery catheterization is especially indicated in patients with a history of previous myocardial infarction, angina or signs of cardiac failure and in patients with evidence of diminished ejection fraction, abnormal ventricular wall motion or myocardial redistribution on preoperative scanning. The more widespread application of intraoperative transoesophageal two-dimensional echocardiography and radionuclide cardiography monitoring techniques into anaesthetic practice will enable measurement of left ventricular dimensions, myocardial performance and wall motion. Suggested guidelines for anaesthetic management are presented in Table VI. A combined opiate-oxygen-volatile anaesthetic agent technique will best ensure a hypodynamic circulation with preservation of myocardial oxygenation.(ABSTRACT TRUNCATED AT 400 WORDS)     

Isoflurane and coronary heart disease

Early studies indicated that isoflurane caused coronary steal and should therefore be avoided in patients with coronary heart disease. Subsequently, more detailed trials have disputed this and have shown that as long as coronary perfusion pressure is maintained, isoflurane does not cause coronary steal or myocardial ischaemia. There is now growing evidence, initially in animal work but more recently in human studies, that isoflurane has myocardial protective properties, limiting infarct size and improving functional recovery from myocardial ischaemia. The mechanism for this protection mimics ischaemic preconditioning and involves the opening of adenosine triphosphate-dependent potassium channels. The few studies comparing the myocardial protection offered by individual anaesthetic agents indicate that isoflurane represents the anaesthetic agent of choice for patients with coronary heart disease.     

Personality constellations in patients with a history of childhood sexual abuse

Although childhood sexual abuse (CSA) appears to have an impact on personality, it does not affect all survivors the same way. The goal of this study was to identify common personality patterns in women with a history of CSA. A national sample of randomly selected psychologists and psychiatrists described 74 adult female patients with a history of CSA and a comparison group of 74 without CSA using the Shedler-Westen Assessment Procedure-200 (SWAP-200), a Q-sort procedure for assessing personality pathology. Q-factor analysis identified four personality constellations among abuse survivors: Internalizing Dysregulated, High Functioning Internalizing, Externalizing Dysregulated, and Dependent. The four groups differed on diagnostic, adaptive functioning, and developmental history variables, providing initial support for the validity of this classification. The data have potential methodological and treatment implications.     

Cardiac tumors. Clinical spectrum and prognosis of lesions other than classical benign myxoma in 20 patients

Between 1961 and 1983, 20 patients underwent operation at the Texas Heart Institute for primary tumors other than classical benign myxoma. Total follow-up was 70.7 patient years (mean 3.5 years). There were 10 adults and 10 children under 12 years of age. There were two operative deaths and four late deaths. All of the pediatric patients had benign lesions (five fibromas, five rhabdomyomas), and only one patient in this group died (during the operation). All operative survivors are alive between 0.9 and 18 years postoperatively, although in three cases excision was incomplete. Of the 10 adult patients, five had benign lesions; all were completely excised. In the other five adult patients, unresectable malignant tumors were found, and all four operative survivors died of metastatic disease within 8 months. Benign cardiac tumors in childhood have an excellent prognosis when completely excised and appear to have a good short-term prognosis even when excision is incomplete. Although prognosis for benign tumors in adults is good, malignant tumors are associated with very poor survival.     

Blood pressure in children and target-organ damage later in life

The aim of this study was to examine the association between blood pressure (BP) in children and adolescents and cardiovascular and renal disease in adulthood. This was a retrospective study on patients <18 years of age with an elective admission to Landspitali University Hospital in Reykjavik, Iceland, between 1950 and 1967. We recorded baseline variables including BP and invited all patients for a follow-up visit in 2008 for repeat studies. We used χ², Fisher’s exact test, and logistic regression to examine the association between BP in childhood and outcome variables at follow-up. We identified 126 individuals (54 men) for the study. The median age (range) at childhood admission was 15 (10–17) years and the median BP was 125/80 mmHg. Median age at follow-up was 58 (42–68) years, follow-up time 43 (25–52) years, and median BP 133/75 mmHg. Eleven had died (five men) and 49 had been diagnosed with hypertension (23 men) and 12 with coronary artery disease (ten men). There was a significant correlation between the diagnosis of coronary artery disease at follow-up and childhood systolic BP (odds ratio?=?1.052; P?=?0.03) as well as systolic BP?≥?95th percentile (P?=?0.03). Our results suggest that elevated childhood systolic BP may increase the risk of coronary artery disease in adult life. The sample size is a limiting factor, and the study should be carried out in a larger population.     

Evaluation of diagonal earlobe crease as a marker of coronary artery disease: the use of this sign in pre-operative assessment*

In this study, we examined the usefulness of the earlobe crease sign as a marker of coronary artery disease in the pre-operative assessment of patients. We were interested in evaluating this sign for use in emergency patients. We investigated 530 patients, aged > 40 years, undergoing elective surgery. If the electrocardiogram was abnormal or the patient reported symptoms suggesting coronary artery disease, further cardiac examinations were performed. Patients who demonstrated evidence of coronary artery disease in the additional investigations or had a clear history of coronary artery disease were classified as the abnormal coronary group. Other patients with no history and/or normal investigations were classified as the normal coronary group. The assessment of earlobe crease sign was performed prior to anaesthesia, and the sensitivity, specificity and positive predictive value of this sign were calculated. We found that the sensitivity and specificity was high regardless of age, except for specificity in patients > 70 years old. The data suggest that the earlobe crease sign may be a useful marker for the presence of coronary artery disease in patients undergoing emergency operations in which little or no history and investigations are available. However, more work is required to assess the use of this sign in other ethnic groups.     

Evaluation of the premature infant at risk for postoperative complications

We reviewed anaesthetic records of 35 infants with a history of prematurity, who presented for elective herniorrhaphy. We applied a scoring system to help evaluate risk of postoperative complications. Six patients experienced postoperative complications. All six patients had a score of five or more and gave history of either apnoea or a history of moderate bronchopulmonary dysplasia. A preoperative history of apnoea and/or moderate bronchopulmonary dysplasia appear to be valuable markers for postoperative complications. A conceptual age of 40 weeks is an acceptable lower limit of age providing there is no history of apnoea or pulmonary disease.     

A review of choledochal cyst in pediatric and adult patients

A review of 80 pediatric and 13 adult patients with choledochal cyst disease occurring in a multiracial population is presented to define the incidence, clinical pattern, surgical techniques, and complications seen over periods of 30 years (pediatric) and 15 years (adult patients). In pediatric patients, most cases are now being diagnosed at a much earlier age, usually in infancy or early childhood, presumably due to the ready availability of ultrasound. Adult patients, by contrast, present a complex picture of heptobiliary disease, and delay in diagnosis is not uncommon. Jaundice appears to be the predominant symptom and the classical triad (pain, jaundice, and mass) is seldom seen. The surgical technique emphasises complete excision of the cyst, including the distal end, as the critical factor in the prevention of late complications. Avoidance of anastomotic stricture depends on the apposition of gut to healthy duct wall and not on the size of the anastomosis alone. Postoperative results have been excellent with no immediate mortality and no evidence of cholangitis in patients with extrahepatic cysts. The morbidity is related to patients with persistent intrahepatic biliary dilatation, Caroli's disease, and carcinoma. While endoscopic intervention, hepatic resection, and liver transplantation have been utilized in the management of cystic disease in the liver, the outlook for patients with cholangiocarcinoma associated with choledochal cyst remains extremely poor, with no survivors in this series.     

Sexual Abuse Survivors and Psychiatric Hospitalization after Bariatric Surgery

Background Some investigators have postulated that a history of being the victim of childhood sexual abuse may impact outcome of bariatric surgery. Methods In this retrospective chart review, we examined the electronic medical records of 152 adults with morbid obesity who underwent Roux-en-Y gastric bypass and who had a weight recorded in their medical record or reported in a follow-up surgery at 2 years after the RYGBP. The purpose of this retrospective chart review was to examine the relationship between psychosocial factors assessed preoperatively and the percent of excess weight lost (%EWL) at 2 years after bariatric surgery. Results We found a high prevalence of being the victim of childhood sexual abuse (27%), adult sexual trauma (9%), and/or physical abuse (19%) at the initial evaluation. There was no association between these factors and %EWL at 2 years. However, when we examined participants’ medical records for post-operative psychiatric hospitalizations at our medical center, 8 of 11 hospitalized patients reported a history of childhood sexual abuse (73%). Conclusions History of being the victim of childhood sexual abuse is reported frequently by patients seeking bariatric surgery. Our finding that having been the victim of childhood sexual abuse may be associated with increased risk of psychiatric hospitalization after RYGBP has several clinical implications. First, we recommend that clinicians assess carefully for a history of sexual or physical abuse, and secondly, abuse survivors may need to be told that there is an increased risk of psychiatric morbidity after bariatric surgery. Finally, perhaps close monitoring of these patients may prevent psychiatric difficulties after surgery. Further research to verify these preliminary findings is clearly needed.     

Predictors of coronary disease in patients with end stage renal disease.

Patients with end stage renal disease have a high prevalence of cardiovascular disease and coronary arteriography is often routinely performed prior to kidney transplantation. However, the value of the conventional risk factors and non-invasive markers of coronary artery disease (CAD) in triaging patients for coronary arteriography has not been fully examined. 116 patients with end stage renal disease were evaluated. Coronary arteriography was performed in all patients either for a suspicion of CAD or as part of a routine pre-transplant evaluation. Lesions causing > or = 50% luminal diameter stenosis in any of the three major coronary artery systems were considered significant. The mean age was 53.3 +/- 9.3 years. Significant CAD was present in 69 patients (60%). Increasing age, family history of premature ischemic heart disease, the presence of angina, abnormal Q waves on the ECG or abnormal ST segment depression and the presence of coronary calcification were significant markers of coronary artery disease. However male gender, diabetes mellitus and obesity did not correlate with coronary disease. Even though hypertension, hypercholesterolemia and smoking were also not useful predictors these could have been modified by the renal failure. In conclusion increasing age, a family history of premature ischemic heart disease and some non-invasive markers were useful predictors of coronary disease.     

Apolipoprotein E polymorphism is not a predictor for repeated coronary artery bypass surgery

OBJECTIVES: Factors leading to the occlusion of coronary grafts are diverse and may at least partially be inherited. We aimed to study the possible genetic predisposition and especially the role of apoE epsilon4 allele as a risk factor for repeated coronary artery bypass grafting (CABG) in a case-control setting. DESIGN: All patients (n=184) who underwent repeated CABG between 1990 and 1998 were identified in the computed registry of the Department of Cardiothoracic Surgery in Tampere University Hospital. Age, sex and operation date matched controls with first time CABG were selected from the same registry. DNA samples were collected by sample stick sent via the mail for buccal smear. The final analysis included 137 surviving matched pairs. RESULTS: In patients <62 years of age (median age), family history emerged as the only significant (OR=3.4; 95% CI=1.5-7.8, p=0.004) predictor for repeated surgery. Among older patients, repeated CABG was no longer predicted by family history but by hypercholesterolemia (OR=2.1; 95% CI=1.1-4.0, p=0.027), modified by apoE genotype. CONCLUSIONS: Our results suggest that medium-term survivors after redo CABG have a strong genetic predisposition unrelated to hypercholesterolemia or apoE genotype, leading to more severe coronary artery disease at earlier age. In the older age group, redo coronary artery bypass surgery is associated with hypercholesterolemia, which, although modified by apoE genotype, may mainly be due to other genetic or acquired factors.     

PREDICTORS OF CORONARY DISEASE IN PATIENTS WITH END STAGE RENAL DISEASE

Patients with end stage renal disease have a high prevalence of cardiovascular disease and coronary arteriography is often routinely performed prior to kidney transplantation. However, the value of the conventional risk factors and non-invasive markers of coronary artery disease (CAD) in triaging patients for coronary arteriography has not been fully examined. 116 patients with end stage renal disease were evaluated. Coronary arteriography was performed in all patients either for a suspicion of CAD or as part of a routine pre-transplant evaluation. Lesions causing ≥ 50% luminal diameter stenosis in any of the three major coronary artery systems were considered significant. The mean age was 53.3 ± 9.3 years. Significant CAD was present in 69 patients (60%). Increasing age, family history of premature ischemic heart disease, the presence of angina, abnormal Q waves on the ECG or abnormal ST segment depression and the presence of coronary calcification were significant markers of coronary artery disease. However male gender, diabetes mellitus and obesity did not correlate with coronary disease. Even though hypertension, hypercholesterolemia and smoking were also not useful predictors these could have been modified by the renal failure. In conclusion increasing age, a family history of premature ischemic heart disease and some non-invasive markers were useful predictors of coronary disease.     

Cardiovascular disease as a late complication of end-stage renal disease in children

As in older adults, cardiovascular disease is the most important cause of death in adolescents and young adult patients with end-stage renal disease (ESRD) since childhood. This concerns patients on dialysis as well as transplant patients, despite the fact that a long duration of dialysis during childhood is an extra mortality risk factor. Left ventricular hypertrophy (LVH), aortic valve calcification, and increased arterial stiffness, but not increased arterial intima media thickening, are the most frequently observed alterations in young adult survivors with childhood ESRD. In transplanted patients a concentric LVH as a result of chronic hypertension is mostly observed; in dialysis patients a more asymmetric septal LVH is found as a result of chronic volume overload. These results suggest that in children and young adults with ESRD chronic pressure and volume overload, a high calcium-phosphate product, and chronic inflammation, but not dyslipidemia, play a role in the development of cardiovascular disease.This work was presented in part at the IPNA Seventh Symposium on Growth and Development in Children with Chronic Kidney Disease: The Molecular Basis of Skeletal Growth, 1–3 April 2004, Heidelberg, Germany     

The risk of cardiovascular disease in adults who have had childhood nephrotic syndrome

While increased risk of cardiovascular disease (CVD) in patients with hyperlipidemia, chronic kidney disease (CKD), or end-stage renal disease (ESRD) is well documented, transient hyperlipidemia or intermittent renal disease as a consequence of relapsing nephrotic syndrome (NS) has not been studied. To investigate this enigma, 62 patients, between 25 and 53 years of age, who had steroid-responsive/dependent NS during childhood, were identified from the records of the Division of Pediatric Nephrology at Yale School of Medicine. Forty patients were located and contacted to ascertain symptoms or occurrences of CVD via a telephone interview. At the time of follow-up, 23–46 years after cessation of NS, none of these patients had ESRD or CKD. Three patients had experienced a myocardial infarction (MI): a 32-year-old male with a family history of CVD; a 41-year-old male with a history of heavy smoking, hypertension, diabetes mellitus, and elevated cholesterol; a 31-year-old male after a cocaine overdose. The occurrence of events (8%) and mortality from CVD (none) in this cohort of patients is comparable to patients of a similar age in the general population and is lower than that of patients of the same age who are on dialysis. The data suggest that relapsing NS during childhood does not place patients at increased risk for CVD mortality or morbidity compared with the general population. Consequently, it would appear that factors related to persistent proteinuria or renal insufficiency, rather than transient proteinuria and renal disease, contribute to the CVD documented in patients with CKD or ESRD.     

老年女性冠心病人群骨质疏松的患病率及相关因素分析

目的 调查老年女性冠心病人群骨质疏松的患病率及相关影响因素,为两种疾病的协同防治提供更多的理论依据.方法 成功搜集并调查了181例诊断为冠心病并行骨密度检测的老年女性患者,包括年龄、家庭、身高、体重、收入、居住地等基本情况,根据是否存在骨质疏松分为观察组(骨质疏松人群)及对照组(骨量正常人群).通过单因素及多因素Log...