Review of the Diagnoses Predisposing Infants to Intestinal Failure on Hospitalized Parenteral Nutrition

During the last 3 decades the use of parenteral nutrition (PN) and the aggressive introduction of enteral feeding in daily practice have transformed the outcome for even the sickest of these infants. More than 90% of infants and children now survive after extensive small bowel resection in the neonatal period. During the last 3 decades the use of parenteral nutrition (PN) and the aggressive introduction of enteral feeding in daily practice have transformed the outcome for even the sickest of these children. The aim of this study was to review the diagnoses (other than infants purely premature) that predispose infants to intestinal failure (IF) and dependency on PN as well as their outcomes. A total of 63 children less than 1 year old received PN for more than 28 days including 35 (56%) boys; 29% of cases were preterm infants with a median gestational age of 26.5 weeks (range, 24-33 weeks). The median age at the start of PN was 0.25 years or 3 months. Median duration of PN treatment was 62 days and median duration of hospitalization was 128 days. Twenty-three (36.5%) children had a primary nondigestive disorder (PNDD) and 40 (63.5%), a primary digestive disorder (PDD). Forty (63.5%) children with severe intestinal failure were successfully weaned off PN; whereas 8 (13%) infants with severe gastrointestinal diseases remained dependent on IV nutrition. Fourteen (22%) patients died. Infants less than 1 year of age with severe intestinal failure have up to a 75% survival rate, with a 65% chance of achieving intestinal autonomy. For children presenting with PDD in infancy, there is a high risk of needing long-term PN.     

Late Effects of Early Surgery on Lipoma and Lipomeningocele in Children Less Than 1 Year Old

Purpose

We attempted to assess the usefulness of urodynamic testing for diagnosing lipoma and lipomeningocele, and the late effects of early neurosurgical intervention in children less than 1 year old.

Materials and Methods

A total of 28 patients in whom spinal surgery was performed before age 1 year for correcting lipoma and lipomeningocele underwent neurological and urodynamic evaluation preoperatively, and within 3 months and up to 14.5 years postoperatively.

Results

Preoperatively 15 patients (54%) had an abnormal neurological examination and 7 had an abnormal urodynamic study. Of the 13 patients with a normal neurological examination 3 had an abnormal urodynamic study. Overall 10 patients (37%) had an abnormal urodynamic examination preoperatively. Immediately after surgery 8 patients (29%) had improvement (2 of 15 with an abnormal neurological examination and 6 of 10 with an abnormal urodynamic study), whereas the condition of 9 (32%) worsened, including 4 of the 13 who were neurologically and 6 of the 18 who were urodynamically normal (1 in both categories). With time an additional 7 patients (25%) had progressive deterioration, including 6 urodynamically and 5 neurologically (4 in both categories), 2 (7%) had improvement (1 each urodynamically and neurologically) and the condition of 19 (68%) remained stable. Six patients (21%) underwent secondary spinal surgery for recurrent tethering.

Conclusions

Early surgical repair seems to provide a degree of protection against later spinal cord tethering, and subsequent neurological and/or urodynamic deterioration (25% of our patients versus a reported 80% of those followed expectantly).     

All-polyethylene and Metal-backed Tibial Components Are Equivalent With BMI of Less Than 37.5

Background  

Modular, metal-backed tibial (MBT) components are associated with locking mechanism dysfunction, breakage, backside wear, and osteolysis, which compromise survivorship. All-polyethylene tibial (APT) components eliminate problems associated with MBTs, but, historically, APT utilization has generally been limited to older, less active patients. However, it is unclear whether APT utilization can be expanded to a nonselected patient population.     

住院肺炎患儿家长维护医护安全行为调查

目的 了解肺炎住院患儿家长维护医护安全行为的状况.方法 采用住院肺炎患儿家长维护医护安全行为问卷对118名患儿家长进行调查.结果 患儿家长5个因子的得分由高到低分别是:积极参与孩子的健康护理(2.73±0.96)分、药物(2.73±0.56)分、手术(2.71±0.70)分、可采取的其他措施(2.69±0.49)分、住院(2.16±0.40)分.患儿家属做的相对较好的前5个条目中,药物因子4条,手术因子1条;做的相对较差的后5个条目中,药物因子2条,可采取的其他措施2条,住院因子1条.结论 住院肺炎患儿家长维护医护安全行为较差,尤其是"可采取的其他措施"和"住院"方面.应对惠儿家长开展多种形式的儿童保健服务指导等教育活动.     

住院肺炎患儿家长维护医护安全行为调查

目的了解肺炎住院患儿家长维护医护安全行为的状况。方法采用住院肺炎患儿家长维护医护安全行为问卷对118名患儿家长进行调查。结果患儿家长5个因子的得分由高到低分别是：积极参与孩子的健康护理（2．73±0．96）分、药物（2．73±0．56）分、手术（2．71±0．70）分、可采取的其他措施（2．69±0．49）分、住院（2．16±0．40）分。患儿家属做的相对较好的前5个条目中，药物因子4条，手术因子1条；做的相对较差的后5个条目中，药物因子2条，可采取的其他措施2条．住院因子1条。结论住院肺炎患儿家长维护医护安全行为较差，尤其是“可采取的其他措施”和“住院”方面。应对患儿家长开展多种形式的儿童保健服务指导等教育活动。     

Muscle and Bone Impairment in Children With Marfan Syndrome: Correlation With Age and FBN1 Genotype

Marfan syndrome (MFS) is a rare connective tissue disorder caused by mutation in the gene encoding the extracellular matrix protein fibrillin‐1 (FBN1), leading to transforming growth factor‐beta (TGF‐β) signaling dysregulation. Although decreased axial and peripheral bone mineral density (BMD) has been reported in adults with MFS, data about the evolution of bone mass during childhood and adolescence are limited. The aim of the present study was to evaluate bone and muscle characteristics in children, adolescents, and young adults with MFS. The study population included 48 children and young adults (22 girls) with MFS with a median age of 11.9 years (range 5.3 to 25.2 years). The axial skeleton was analyzed at the lumbar spine using dual‐energy X‐ray absorptiometry (DXA), whereas the appendicular skeleton (hand) was evaluated using the BoneXpert system (with the calculation of the Bone Health Index). Muscle mass was measured by DXA. Compared with healthy age‐matched controls, bone mass at the axial and appendicular levels and muscle mass were decreased in children with MFS and worsened from childhood to adulthood. Vitamin D deficiency (<50 nmol/L) was found in about a quarter of patients. Serum vitamin D levels were negatively correlated with age and positively correlated with lumbar spine areal and volumetric BMD. Lean body mass (LBM) Z‐scores were positively associated with total body bone mineral content (TB‐BMC) Z‐scores, and LBM was an independent predictor of TB‐BMC values, suggesting that muscle hypoplasia could explain at least in part the bone loss in MFS. Patients with a FBN1 premature termination codon mutation had a more severe musculoskeletal phenotype than patients with an inframe mutation, suggesting the involvement of TGF‐β signaling dysregulation in the pathophysiologic mechanisms. In light of these results, we recommend that measurement of bone mineral status should be part of the longitudinal clinical investigation of MFS children. © 2015 American Society for Bone and Mineral Research.     

儿童慢性胰腺炎的病因及内镜在治疗中的价值

目的探讨儿童慢性胰腺炎的病因和十二指肠镜在治疗中的价值。方法回顾性分析2006～2011年18例18岁以下因慢性胰腺炎行ERCP 40次的临床资料。年龄4～17岁,平均10.3岁。男8例,女10例,均存在上腹痛,影像学检查提示胰腺有异常表现。气管插管全麻下行ERCP。行狭窄括约肌切开、胰管狭窄段扩张、碎石、取石、胰管支架放置/鼻胰引流管放置等治疗。结果 18例40次ERCP操作,胰管插管成功39次（97.5%）。不明原因慢性胰腺炎11例（61.1%,其中2例以胰腺胸膜瘘发病）,胰腺结构异常致慢性胰腺炎7例。全部患者症状消失。ERCP无严重并发症。9例结束治疗者随访1～39个月,均无腹痛发作。结论儿童慢性胰腺炎病因主要为特发性及结构异常,经十二指肠镜治疗微创、安全、有效。     

Impact of Early Parenteral Nutrition on Metabolism and Kidney Injury

A poor nutritional state and a caloric deficit associate with increased morbidity and mortality, but a recent multicenter, randomized controlled trial found that early parenteral nutrition to supplement insufficient enteral nutrition increases morbidity in the intensive care unit, including prolonging the duration of renal replacement therapy, compared with withholding parenteral nutrition for 1 week. Whether early versus late parenteral nutrition impacts the incidence and recovery of AKI is unknown. Here, we report a prespecified analysis from this trial, the Early Parenteral Nutrition Completing Enteral Nutrition in Adult Critically Ill Patients (EPaNIC) study. The timing of parenteral nutrition did not affect the incidence of AKI, but early initiation seemed to slow renal recovery in patients with stage 2 AKI. Early parenteral nutrition did not affect the time course of creatinine and creatinine clearance but did increase plasma urea, urea/creatinine ratio, and nitrogen excretion beginning on the first day of amino acid infusion. In the group that received late parenteral nutrition, infusing amino acids after the first week also increased ureagenesis. During the first 2 weeks, ureagenesis resulted in net waste of 63% of the extra nitrogen intake from early parenteral nutrition. In conclusion, early parenteral nutrition does not seem to impact AKI incidence, although it may delay recovery in patients with stage 2 AKI. Substantial catabolism of the extra amino acids, which leads to higher levels of plasma urea, might explain the prolonged duration of renal replacement therapy observed with early parenteral nutrition.The development of AKI is a frequent and devastating condition in patients admitted to the intensive care unit (ICU). Short-term mortality is high and increases with worsening AKI stages.¹ In AKI survivors, renal recovery is often incomplete, progression to ESRD may be accelerated, and longer-term mortality rates are increased compared with non-AKI patients.^2,3 Patient management consists of maximal prevention of additional renal damage by hemodynamic stabilization and prevention of (iatrogenic) nephrotoxicity. A curative strategy for established AKI is currently unavailable.⁴Observational studies, finding associations between a poor nutritional state and increased morbidity and mortality of AKI patients⁵ and between accumulation of a caloric deficit and poor renal and survival outcome of ICU patients,^6,7 have led to the hypothesis that feeding could ameliorate kidney injury and improve survival of ICU patients. However, nutrition, especially parenteral nutrition (PN), also has potential complications.^8–11 Because of the lack of adequately designed studies, nutritional guidelines are largely based on expert opinion.^12–14 These opinions invariably recommend the early initiation of enteral feeding but substantially differ in their recommendation on when to start supplemental PN.The Early Parenteral Nutrition Completing Enteral Nutrition in Adult Critically Ill Patients (EPaNIC) study was the first large, multicenter, randomized controlled trial (RCT) addressing this important question. The study showed that early initiation of PN increased dependency on intensive care compared with withholding supplemental PN for 1 week (hereafter labeled early PN and late PN, respectively).¹⁵ Indeed, early PN prolonged the ICU and hospital length of stay (LOS), increased the incidence of new infections, and prolonged the need for mechanical ventilation. Renal harm was suggested by a clear prolongation of the duration of renal replacement therapy (RRT) in ICU and a trend for more AKI (defined as a doubling or more of ICU admission plasma creatinine). However, the number of patients requiring RRT was unaltered, and recovery to premorbid kidney function was not investigated.It was preplanned to study the detailed impact of early versus late PN on the incidence and recovery of AKI and the time course of blood/urine markers of renal function during ICU stay.¹⁶ A priori, we hypothesized that early PN would attenuate kidney injury. However, the original study findings suggested that AKI incidence and renal recovery could be aggravated by increased macronutrient provision in the acute phase of critical illness.     

Home Parenteral Nutrition Program and Referral of Potential Candidates for Intestinal and Multivisceral Transplantation in a Single Brazilian Center

Intestinal failure is a multifaceted condition that may require high-complexity treatment and a multidisciplinary program, including home parenteral nutrition therapy (HPNT) and intestinal transplantation. In this article, we profile a Brazilian single-center experience with 128 cases of HTPN followed for the last 30 years and appraise the referral for potential intestinal and multivisceral transplantation.     

Molecular Alterations of Nucleocytoplasmic Transport in Patients on the Heart Transplantation Waiting List and Its Correlation With the Severity and Etiology of Heart Failure

Objectives

To evaluate whether the levels of some molecules implicated in nucleocytoplasmic transport in human cardiomyocytes are related to the severity of heart failure (HF) in patients on the heart transplantation (HT) waiting list, and to determine whether there is a differential pattern of molecular alteration between ischemic cardiomyopathy (ICM) and non-ischemic dilated cardiomyopathy (DCM).

Brain Natriuretic Peptide and Its Relationship to Left Ventricular Hypertrophy in Patients on Peritoneal Dialysis or Hemodialysis Less Than 3 Years

An increase of brain natriuretic peptide (BNP) levels is commonly observed in patients on dialysis. Increased circulating levels of BNP are related to future cardiac events and associated with shorter survival in patients on chronic hemodialysis (HD). During the first 1 or 2 years on dialysis, patients on peritoneal dialysis (PD) have been shown to have an improvement in left ventricular hypertrophy, blood pressure, and volume status. This study compares BNP levels and cardiac status of PD and HD patients without cardiovascular disease and on dialysis for less than 36 months. The correlation between plasma BNP concentration and findings of echocardiography before HD scans were examined and compared with findings of PD. Twenty-two HD patients (15 men, 7 women; mean age, 52.5 ± 13.9 years) and 19 PD patients (10 men, 9 women; mean age, 47.6 ± 11.3 years) were studied. There were no significant differences between HD and PD patients with regard to age, gender, duration of dialysis, left ventricular mass, left ventricular mass index (p > 0.05). Plasma BNP levels were markedly greater in HD patients (467.8 ± 466.5 pg/mL) than those of PD patients (143.1 ± 165.2 pg/mL). Urine output was significantly higher in PD patients compared with HD patients (p < 0.05). A positive correlation between systolic blood pressure, diastolic blood pressure, and plasma BNP in HD patients (r: 0.653, p: 0.001; r: 0.493, p: 0.023, respectively) was detected. Additional studies are needed to investigate whether lower BNP level in PD patients is an advantage.     

Less Than 1-Year Quiescent Period After Septic Arthritis of the Hip is Associated With High Risk of Periprosthetic Joint Infection Following Total Hip Arthroplasty

BackgroundApproximately 20,000 patients are diagnosed with septic arthritis annually, with 15% specifically affecting the hip joint. These cases exacerbate arthritic changes, often warranting a total hip arthroplasty (THA). Given their prior history of infection, these patients are predisposed to subsequent periprosthetic joint infections (PJIs). Multiple studies suggest delaying THA after a native septic hip, but no study utilizing a large cohort examined the specific timing to mitigate post-THA PJI risk within a short (<1 year) quiescent period after septic arthritis. We sought to compare patients who were diagnosed with septic hip arthritis at time intervals (0-6, or 6-12 months) prior to an ipsilateral primary THA to a cohort of THA patients who never had a septic hip history. Specifically, we assessed: from 90 days to 2 years (1) revisions due to PJI and (2) associated risk factors for PJI at 2-years.MethodsA national, all-payer database was queried to identify all patients who underwent a primary THA between 2010 and 2021 and patients who had prior ipsilateral septic hip arthritis were characterized using International Classification of Disease and Current Practice Terminology codes (n = 1,052). A randomized sample of patients who never had a history of septic arthritis prior to undergoing THA was used as a nonseptic group comparison (n = 5,000). The incidences of PJI at 90 days through two years were then identified and compared using bivariate chi-square analyses. Risk factors for post-THA PJIs were then analyzed using multivariate regression models.ResultsThe septic arthritis cohorts were more likely to require revisions due to PJIs, as compared to the non-septic group at 90 days, 1 year, and 2 years (all P < .0001). Patients who were diagnosed with septic arthritis between 0 and 6 months prior to THA were at greater PJI risk at both one-year (odds ratio (OR) of 43.1 versus 29.6, P < .0001) and two years (OR of 38.3 versus 22.1, P < .0001) compared to patients who had diagnoses between 6 and 12 months. Diabetes mellitus, obesity, and tobacco use were associated risk factors for PJIs at 2 years in the septic hip cohort in comparison to the cohort without a septic hip history.ConclusionLess than a 1-year quiescent period after septic arthritis is associated with a 38 times increased risk and a 22 times risk for post-THA PJI, at 0 and 6 months and 6 and 12 months, respectively. Though patients who undergo THA greater than 6 months after their septic arthritis treatment have a decreased risk compared to those between 0 and 6 months the risks are still high. Orthopaedic surgeons should be aware of the increased risks of PJIs when considering performing a THA in patients with a history of septic arthritis.     

Association of Age,Sex, and Heart Failure Etiology in Continuous Flow Left Ventricular Assist Device Patients

BackgroundAdvanced heart failure studies demonstrate that ischemic factors increase in prevalence with age and are more prominent in men. Ejection fraction (EF) cannot be preserved in these patients, and ischemic cardiomyopathy develops. Non-ischemic factors are more prominent in female heart failure patients, where the EF is preserved. Although an age-associated increase in the rate of heart failure is acknowledged in both sexes, etiologic classifications by sex-based age groups are still lacking. This study examined the etiology of heart failure according to age and sex in ventricular assist device patients.MethodsThe patient population included 457 end-stage heart failure patients who received a continuous flow–left ventricular assist device at Ege University Hospital between 2010 and 2017. Age, sex, and cardiomyopathy etiology data were obtained from the hospital database. The Mann-Whitney U test was applied to test the statistical significance among subgroups (95% CI, P < .05 for statistical significance).ResultsThe prevalence of ischemic cardiomyopathy was significantly lower in male patients aged 18 to 39 years compared to older patients. Conversely, no difference was seen among female patients. The prevalence of dilated cardiomyopathy was higher in male patients who were 18 to 39 years of age compared to older patients, but no difference was present among the female patients.ConclusionsAge and heart failure etiology were demonstrated to be interrelated in men but not in women. The fact that etiologic factors of advanced heart failure in women have a wider range than in men makes the current classification systems insufficient for use in female populations.     

护士对住院患儿家属的护理支持及家属满意度调查

目的 了解护士对患儿家属的护理支持情况及家属对护理支持行为的满意度.方法 采用护士对患儿家属的支持量表(NPST)及满意度调查量表对125名患儿家属进行问卷调查.结果 护士对患儿家属的总体支持评分为(3.32±0.45)分,家属对护理支持行为的总体满意度评分为(3.06±0.26)分,其中优质护理支持及其满意度得分最高,而情感支持、尊重支持及其满意度得分偏低.结论 护士对患儿家属的支持情况总体较好,患儿家属基本满意.但在注重疾病护理的同时,需加强对患儿家属的情感支持和尊重支持.