Outcome and quality of life in paediatric home parenteral nutrition

In this review the current status of home parenteral nutrition is analysed, with respect to the predictability of weaning from nutritional support and the risk of developing major complications associated with the technique, the loss of vascular access and liver disease. These two complications were evaluated because they represent the more important indication for intestinal transplantation, the availability of which has changed the perspectives of patients and of physicians. Analysis of outcomes from the largest series allows the identification of patients who could be weaned from parenteral nutrition. Important prognostic factors in patients affected by short bowel syndrome are the length and type of the remnant and the time to tolerate enteral feeding. The main complications of therapy are sepsis, thrombosis, nutrient imbalances and liver disease. Sepsis and thrombosis could lead to line replacement and the loss of vascular access. Sepsis no longer represents a major cause of death, but it is a frequent complication. In some patients, it is difficult to assess the risk factors for sepsis, which is possibly related to a poorer outcome. The care of gut failure appears to be the best preventative measure for the occurrence of cholestatic liver disease, but further studies are needed to define the eventual role of lipid emulsion and of specific nutrient deficiency. The quality of life still remains to be studied: because home parenteral nutrition in children has a longer duration, its analysis is mandatory.     

Correction: Bismuthene nanosheets produced by ionic liquid assisted grinding exfoliation and their use for oxygen reduction reaction

Correction for ‘Bismuthene nanosheets produced by ionic liquid assisted grinding exfoliation and their use for oxygen reduction reaction’ by Manila Ozhukil Valappil et al., RSC Adv., 2020, 10, 43585–43591, DOI: 10.1039/D0RA09763B.

The authors regret that Pagona Papakonstantinou’s email address was shown incorrectly in the original article. The corrected email address is as shown above.The Royal Society of Chemistry apologises for these errors and any consequent inconvenience to authors and readers.     

Kidney aging: from phenotype to genetics

Aging is a physiological process that causes structural and functional changes in human body systems, sometimes leading to various organ failure. As far as the kidney is concerned, both genetic factors and environmental agents may influence the tissues damage in elderly people and the related loss of function. On the other hand, functional adaptations to structural changes appear to be compromised by co-morbid conditions that are frequently found in elderly people, such as atherosclerosis and hypertension. It is not yet known whether physiological aging is inevitably accompanied by a decline in renal function or how rapidly it might happen. The discovery of molecular mechanisms responsible for tissue damage in aging could offer new perspectives on interventions. The role of nitric oxide, oxidative stress, the renin-angiotensin system, changes in length of telomeres, and klotho gene expression are important subjects for further in-depth studies about aging. A better understanding of physiological renal aging could improve the clinical approach to this process and widen the therapeutic possibilities offered by transplantation.     

Late graft dysfunction and autoantibodies after liver transplantation in children: preliminary results of an Italian experience.

Late graft dysfunction (GD) associated with the development of autoantibodies is a common event after pediatric liver transplantation (OLTx) and can present in 2 clinicohistological subsets: de novo autoimmune hepatitis (DNAH) and early chronic rejection (ECR). Sixty out of 247 children developed autoantibodies after OLTx. GD was demonstrated in 22 (37%); based on histology, patients were divided in a DNAH and an ECR group. Portal/periportal inflammatory infiltrate with interface/lobular hepatitis was suggestive for DNAH. Pericentral hepatocytes confluent dropout with a variable degree of central vein endothelitis, but not with ductopenia (loss of >50% of interlobular bile ducts), was diagnosed as ECR. Nine patients had DNAH and 13 ECR. Five out of 9 in the DNAH group were on cyclosporin (CsA) and 4/9 were on tacrolimus (Tac). In the ECR group, 11 children were treated with CsA and 2 with Tac. All DNAH patients had normal liver function tests on steroids and azathioprine (AZA). Five patients with ECR recovered by increasing calcineurin inhibitors (CNIs) dosage, but in 8/13, including 7 switched from CsA to Tac, AZA and steroids were added to obtain remission of disease. Two patients developed late chronic rejection. DNAH and ECR associated with autoantibodies are forms of late GD after OLTx. DNAH improves after standard treatment of autoimmune hepatitis. ECR has a good response to increased doses of CNIs, although ductopenic chronic rejection may occur. In conclusion, the early differential diagnosis of these conditions and an appropriate treatment seem to allow good overall results reflected by a graft survival of more than 90%.     

Polycystin-1 induces resistance to apoptosis through the phosphatidylinositol 3-kinase/Akt signaling pathway

Polycystin-1 (PC-1), the PKD1 gene product, is a large receptor whose expression in renal epithelial cells results in resistance to apoptosis and tubulogenesis, a model consistent with the phenotype observed in patients. This study links PC-1 expression to a signaling pathway that is known to be both antiapoptotic and important for normal tubulogenesis. This study found that PC-1 expression results in phosphorylation of Akt and downstream effectors and that phosphatidylinositol 3-kinase (PI3-K) inhibitors prevent this process. In addition, it is shown that dominant negative Akt can revert PC-1-induced protection from apoptosis. Furthermore, it was observed that increased PI3-K beta activity in PC-1-expressing MDCK cells seems to be dependent on both tyrosine-kinase activity and heterotrimeric G proteins. It also was found that PC-1-induced tubulogenesis is inhibited by PI3-K inhibitors. Taken together, these data suggest that the PI3-K/Akt cascade may be a central modulator of PC-1 function and that its deregulation might be important in autosomal dominant polycystic kidney disease.     

Chordoid meningioma: a retrospective series of seven consecutive cases

Chordoid meningioma is a rare variant of meningioma characterized by a more aggressive behavior. The present study documents the histological, radiological and clinical features of seven cases treated at the Policlinico Umberto I of Rome from 1999 to 2010. There were five males and two females. Most of the cases were located in the supratentorial space, especially the convexity. Surgical gross total resection was achieved in four cases. Of the remaining three cases, two relapsed and underwent further surgeries and adjuvant treatment. The MIB-1 index had a mean value of 7.5 (range 0.3–25.8). Tumors were composed of epithelioid cells or plump to spindle cells, forming cords, cribriforms or nests, in a mucoid matrix. All tumors showed diffuse positive immunoreactivity to vimentin and epithelial membrane antigen. Surgery is the first line of treatment for this kind of lesion. Gross total resection guaranteed a survival free from recurrences in our series. On the other hand, radiation therapy must be considered in patients submitted to a subtotal resection.     

HSV-1 as well as HSV-2 is frequent in oral mucosal lesions of children on chemotherapy

Purpose

Prevalence data of herpes simplex virus (HSV) in oral mucositis in children on treatment for cancer is limited. Quantitative polymerase chain reaction (PCR) has been seldom utilized for detection of HSV-1/2 in oral mucosa.

Methods

Children on treatment for cancer with oral mucositis were enrolled as cases and healthy children as controls. An oral swab from the lesion in cases and mucosal scraping in controls were obtained. Both qualitative and real-time quantitative PCR for HSV-1/2 were performed. Serum ELISA-IgG/IgM for HSV-1/2 antibodies (NovaLisa?-Dietzenbach-Germany) were measured.

Results

Thirty-two cases (Age, 6.3?±?3.4 years) and 30 controls were enrolled. Majority (69 %) of cases had ALL. All patients had febrile neutropenia, except two. ELISA-IgM-HSV-1/2 was not positive in any case or control. ELISA-IgG-HSV-1/2 was positive in 11 (34 %) cases and nine (30 %) controls (p?=?1.0). Qualitative PCR for HSV-1 detected the virus in eight (25 %) cases and nil controls (p?=?0.009). HSV-2 was not detected in any case/control by qualitative PCR. Quantitative PCR detected HSV-1 in 21 (66 %) and HSV-2 in 22 (69 %) cases. In controls, quantitative PCR detected HSV-1 in three (10 %) and HSV-2 in none. In patients, the mean viral load of HSV-1 (5,500?±?15,987?×?10⁴ copies/nanogram DNA) was more than HSV-2 (4.03?±?8.5?×?10⁴) (p?=?0.11). There was no correlation of HSV-1/2 with grading of mucositis.

Conclusions

Both HSV-1/2 are commonly shed from oral mucosal lesions in children receiving chemotherapy. In a novel finding, real-time PCR detected copies of HSV-2 in 69 % cases, all missed by conventional PCR. Implication for morbidity, if any, or treatment needs to be determined.