Two cases of preceded aortic valve replacement for severe aortic stenosis before the cancer operations

We report two cases of aortic valve replacement (AVR) for severe aortic stenosis (AS) before the cancer operations. Severe AS poses a great risk for noncardiac surgery. In the ACC/AHA 2007 Guideline on Perioperative Cardiovascular Evaluation and Care for Noncardiac Surgery, if the AS is symptomatic, elective noncardiac surgery should generally be postponed or canceled. Such patients require AVR before elective noncardiac surgery. On the other hand, in patients with severe AS who refuse cardiac surgery, noncardiac surgery can be performed with a mortality risk of approximately 10%. In our cases, severe AS was found in the preoperative examination. We informed them about necessary AVR before noncardiac surgery, and patients consented to our suggestion. AVR was performed around 7 days after this consent, and cancer operation was performed around 30 days after the AVR. However, there are no clear guidelines for this interval between AVR and cancer operation. In our cases the patients underwent the cardiac surgery and noncardiac surgery in a short period without serious complication in the perioperative management. It is very important to discuss among surgeon, cardiovascular surgeon, cardiologist and anesthesiologist. Especially anesthesiologist should take an important role in organizing these departments for such patients.     

Risk factors for kidney impairment and differential impact of liver transplantation on renal function

Background

Chronic kidney disease (CKD) is a common problem in long-term survivors after liver transplantation (LT). It is important to identify and correct risk factors that negatively affect kidney function. The purpose of this study was to delineate the risk factors associated with progressive kidney dysfunction after OLT.

Methods

We analyzed 50 recipients (10 female, 40 male) of overall age of 44 ± 13 year who were all ≥18 years old and underwent first LT between 1999 and 2005. Patient-related risk factors were evaluated for renal failure at 3 and 5 years after transplantation. We evaluated parameters of demographic data, laboratory values, daily proteinuria, and renal resistive index (RRI) by Doppler ultrasonography. CKD was defined as a sustained decrease in estimated glomerular filtration rate (eGFR). Patients were divided into 3 groups according to the change in eGFR from the baseline value: group 1, stable eGFR (no change from baseline); group 2, <50% decreased eGFR; and group 3, ≥50% decrease from baseline. eGFR was calculated by using Modification of Diet in Renal Disease (MDRD) formula.

Results

At 3 years after LT, GFR negatively correlated with initial Child-Pugh score (r = −0.42; P < .01); microalbuminuria (r = −0.28; P < .01), and RRI (r = −0.36; P < .01). After 5 years, GFR negatively correlated with initial gamma glutamyl transferase (r = −0.21; P < .05), PT (r = −0.29; P < .05), and RRI (r = −0.32; P < .01). Pretransplantation direct bilirubin levels were significantly correlated with GFR decrease at 3 years (P = .05). At 5 years of follow-up, smoking (P < .05), baseline alanine aminotransferase (P = .03) and serum triglyceride (P < .01) levels significantly correlated with eGFR decrease. Pretransplantation serum creatinine levels were stratified into normal versus high groups. Patients with increased basal serum creatinine levels displayed shorter survivals than those with normal creatinine levels, namely, median values of 21 ± 3.9 months versus 14 ± 2.4 months, log rank test: P < .05).

Conclusion

Renal function after liver transplantation show sustained impairment in certain patients. In the short term the main risk factors for renal detoriation were severity of liver disease before LT, microalbuminuria, and renal perfusion. In the long term, smoking and dyslipidemia were the main predictors of CKD. Patients with high basal serum creatinine values were at increased risk of mortality.     

Effects of atorvastatin and ezetimibe on endothelial function in dyslipidemic patients with chronic kidney disease

Backgroud

Chronic kidney disease (CKD) is a staple risk factor not only for renal failure but also for cardiovascular diseases. In addition, because dyslipidemia facilitates atherosclerosis and renal dysfunction, antihyperlipidemic treatment is important to prevent cardiac and renal events in CKD patients.

Methods

We compared the effects of a statin and an intestinal cholesterol transporter inhibitor in 20 dyslipidemic patients with CKD presenting with proteinuria and/or glomerular filtration rate <60 mL/min/1.73 m². Either 5–10 mg atorvastatin or 10 mg ezetimibe was given for 3 months each in a randomized crossover manner and the parameters of oxidative stress, inflammation and endothelial function were compared.

Results

Atorvastatin lowered serum low-density lipoprotein (LDL) cholesterol more prominently than ezetimibe (103 ± 38 vs 130 ± 45 mg/dL, p < 0.001), while serum γ-glutamyl transpeptidase was higher in atorvastatin than in ezetimibe (29 ± 16 vs 25 ± 11 U/L, p = 0.013). On the other hand, serum oxidized LDL and high-sensitivity C-reactive protein were lower in the atorvastatin treatment period than in the ezetimibe treatment period (109 ± 38 vs 146 ± 67 U/L, p = 0.002; 1.02 ± 1.46 vs 1.47 ± 1.77 µg/mL, p = 0.003). Although serum adiponectin was not significantly different between the two drugs, the reactive hyperemia index, an index of endothelial function, was higher in atorvastatin than in ezetimibe (1.94 ± 0.58 vs 1.60 ± 0.44, p = 0.023).

Conclusion

It is concluded that atorvastatin is more potent than ezetimibe in improving the serum lipid profile, reducing oxidative stress, suppressing inflammation and preserving endothelial function, while ezetimibe may be advantageous in reducing the hepatic lipid load.     

Outcomes of liver transplantation for Alagille syndrome after Kasai portoenterostomy: Alagille Syndrome with agenesis of extrahepatic bile ducts at porta hepatis

BackgroundAlagille syndrome (ALGS) is an autosomal dominant disorder, characterized by a paucity of intrahepatic bile ducts, resulting in significant cholestasis, and peculiar extrahepatic features. Some ALGS patients show a considerable overlap with biliary atresia (BA), and they can undergo Kasai procedure. The purpose of this study is to show the manifestations of BA overlapped ALGS cases in our institution, and to compare the outcomes of ALGS patients following liver transplantation (LT) between those who previously underwent Kasai surgery (ALGS-Kasai group) and those who did not (ALGS-non-Kasai group).MethodsMedical records of ALGS patients who underwent LT in Kyoto University Hospital, Japan from January 1992 to March 2018 were analyzed. ALGS diagnosis was determined according to physical, radiologic, and histopathological findings.ResultsThirty-one patients were ascertained (ALGS-Kasai: 4 males and 5 females vs. ALGS-non-Kasai: 14 males and 8 females, p = 0.43). Of 31 ALGS patients, 96.8% of children had pulmonary artery stenosis, 54.8% showed facial features, 29% revealed skeletal anomalies and 9.7% demonstrated ocular anomalies. The age at LT was significantly younger in ALGS-Kasai than ALGS-non-Kasai group (1.47 [interquartile range (IQR), 0.75–1.92] vs. 5.1 [IQR, 1.4–9.29] years; p = 0.038). Overall patient survival did not significantly differ between ALGS-Kasai (88.9%) and ALGS-non-Kasai patients (86.4%) (p = 0.84). Furthermore, the 1-year, 5-year, and 10-year patient survival rates for ALGS-Kasai group were 100%, 88.9%, and 88.9%, respectively, whereas those for ALGS-non-Kasai group were 90.9%, 90.9%, and 86.4%, respectively, with p-values of 0.36, 0.90, and 0.84, respectively.ConclusionsBA overlapped ALGS cases had neonatal progressive cholestasis which prompted Kasai procedure, and early liver dysfunction after Kasai led to performing LT. The ALGS-Kasai patients undergo LT at earlier ages than the ALGS-non-Kasai patients, however, overall patients' survival rates are similar between groups. Overall ALGS patients' survival rate after LT is considered high.Levels of EvidenceLevel III; Case–control study or Retrospective comparative study.     

Clinical usefulness of the frontal assessment battery (FAB) for elderly in memory clinic]

To investigate the usefulness of the frontal assessment battery (FAB) as a brief assessment for diagnosis in patients with dementia, we examined cognitive function using the FAB, Mini-mental state examination (MMSE), Kana-hiroi test, and Raven's colored progressive matrices (RCPM). Subjects were 82 senile patients having amnesia as a complaint including 28 Alzheimer's disease (AD), 14 frontotemporal dementia (FTD), 17 vascular dementia (VD), 13 mild cognitive impairment (MCI), and 10 healthy persons. We discussed the difference of scores in different types of dementia. In the FAB, the scores of healthy persons and MCI were much higher compared with the scores of all types of dementia. There were high correlation between FAB and MMSE, Kana-hiroi test, and RCPM. Although some subjects showed normal score in MMSE and/or RCPM, they showed low score in FAB. Based on this, FAB is efficient for the differential diagnosis of dementia as brief screening tool.     

Kanji-predominant alexia with agraphia in opticospinal multiple sclerosis]

Alexia with agraphia is very rare symptom in multiple sclerosis. We present a patient of opticospinal multiple sclerosis with kanji-predominant alexia with agraphia. A 55-year-old, right-handed man was admitted to our hospital because of difficulty in reading and writing in August 2001. The patient had been diagnosed as having relapsing-remitting opticospinal multiple sclerosis eight years prior to admission. Language examination showed alexia with agraphia predominantly affecting kanji and also mild naming difficulties, but a good comprehension and a normal repetition. T2-weighted MRI demonstrated hyperintensity area in the left temporo-parietal lobe, involving the white matter beneath the postero-inferior temporal lobe and inferior parietal lobule. On brain SPECT, low blood perfusion was observed in the left temporo-parietal regions. Although agraphia for kana and alexia for both kana and kanji improved after steroid therapy, agraphia for kanji did not improve. After the treatment, high intensity area of inferior parietal lobule was disappeared on MRI, and the hypoperfusion of inferior parietal lobule on brain SPECT was also improved, but the lesion of left postero-inferior temporal lobe did not show any remarkable changes. We considered that the kanji-predominant alexia with agraphia was due to the lesions of left inferior parietal lobule and postero-inferior temporal lobe, and agraphia for kanji was due to the lesion of left postero-inferior temporal lobe.     

Postoperative improvement of executive function and adaptive behavior in children with intractable epilepsy

IntroductionAn alteration in postoperative cognitive function varies according to the patients’ background characteristics, such as etiology, focus, and seizure duration. Accurate prediction and assessment of postoperative cognitive function is difficult in each patient. Adaptive behavior could describe the typical performance of daily activities and represents the ability to translate cognitive potential into real-world skills. We examined the relationship between alterations of executive function (EF) and adaptive behavior in school children undergoing surgery for intractable epilepsy.MethodologyWe enrolled 31 children with focal resection or corpus callosotomy for intractable epilepsy [mean age at surgery, 12.5 years; 16 boys; mean intellectual quotient, 73.3]. We surveyed answered questionnaires on attention-deficit hyperactivity disorder (ADHD), autism spectrum disorder (ASD), and adaptive behavior using the Vineland Adaptive Behavior Scale, 2nd edition (VABS-II), and performed continuous performance tests (CPTs) on children pre- and postoperatively.ResultADHD and ASD symptoms improved after epilepsy surgery. The omission error (OE) in the CPT variable improved after epilepsy surgery, especially in children with a shorter preoperative period. Improved ASD symptoms led to an increased score of the coping skills subdomain. The reduced OE observed after surgery also increased the score of the community skills subdomain.ConclusionImprovement in EF and ASD symptoms resulted in better adaptive behavior postoperatively. These results were important for the pre- and postoperative evaluation and re-evaluation of children with epilepsy requiring special education and related services.     

Risk factors for hyperammonemia associated with valproic acid therapy in adult epilepsy patients

Hyperammonemia is one of the side effects of treatment with valproic acid (VPA), but the risk factors and mechanisms involved remain obscure. This study analyzed the risk factors for hyperammonemia associated with VPA therapy in adult epilepsy patients. A retrospective analysis of 2724 Japanese patients (1217 males and 1507 females aged from 16 to 76years) treated with VPA between January 2006 and December 2010 were analyzed. The ammonia level increased markedly in a VPA dose-dependent manner, and was significantly elevated in patients who also used hepatic enzyme inducers such as phenytoin (PHT), phenobarbital (PB), carbamazepine (CBZ), and combinations of these drugs. When a blood ammonia level exceeding 200μg/dl was defined as hyperammonemia, the risk factors for hyperammonemia according to multiple regression analysis were a VPA dose >20mg/kg/day (odds ratio (OR): 4.1; 95% confidence interval (CI): 1.6-10.8) and concomitant use of PHT (OR: 11.0; 95% CI: 3.1-38.7), concomitant PB (OR: 4.3; 95% CI: 1.0-17.9), concomitant CBZ (OR: 2.8; 95% CI: 0.6-11.9), and concomitant topiramate (OR: 2.8; 95% CI: 1.2-6.5). Regimens containing multiple inducers were associated with an increased risk of hyperammonemia. Identification of risk factors for hyperammonemia associated with VPA therapy can help to minimize side effects during its clinical use.