The Dietary Patterns of Japanese Hemodialysis Patients: A Focused Ethnography.

The relationship between diet and kidney disease outcomes has been established, and as a result, hemodialysis patients are required to modify their diet. However, there is a gap in understanding how diet is influenced by social factors, particularly among Japanese patients whose diet is known to be high in salt. The purpose was to explore the dietary patterns of Japanese hemodialysis patients and better understand the challenges they face in adhering to dietary restrictions. A focused ethnography, employing observational methods with six Japanese hemodialysis patients was undertaken. Fieldwork included observations, informal interviews, and weekly formal interviews for 6 weeks. Five categories were identified during the process of data analysis. This study highlights the strong influence of culture, tradition, and family on the dietary patterns of Japanese hemodialysis patients, which has rarely been acknowledged by Japanese health professionals. The translated abstract was prepared by the author based on the accepted English version of the original abstract. Only light additional review of the translation has been completed by the journal.

Percutaneous endoscopic gastrostomy for enteral nutrition: a 5-year clinical experience with 324 patients.

BACKGROUND: Percutaneous endoscopic gastrostomy (PEG) is considered the route of choice for long-term enteral nutrition. In this study, we evaluated the short term outcomes of PEG and aimed to identify the risk factors for in-hospital mortality as well as adverse events such as aspiration pneumonia. METHODS: Three hundred and twenty-four patients who received PEG for enteral nutrition between January 2007 and December 2011 were included in this study. Data regarding baseline characteristics, preoperative biomarkers and clinical outcomes were collected and analyzed. RESULTS: The average patient age was 82.3±10.9 (SD) years old. 149 patients (46%) were men and 199 patients (61%) had stroke as a comorbidity. Postoperatively, feeding-related aspiration pneumonia was observed in 72 patients (22%), diarrhea in 40 patients (12%) and peristomal infection in 34 patients (11%). The average postoperative length of stay was 34.6 days. 51 patients (16%) died before discharge, 22 (6.8%) of them within 30 days after PEG. Two deaths (0.6%) were considered procedure-related (bleeding). Multivariate analysis revealed the male gender, lower serum albumin levels and higher blood urea nitrogen (BUN) levels as significant prognostic factors for in-hospital mortality. Higher C-reactive protein levels and BUN levels were found to be independently associated with the occurrence of postoperative aspiration pneumonia. CONCLUSIONS: Our experience reconfirmed PEG as a minimally invasive and safe procedure for long-term enteral nutrition in patients with impaired oral intake. However, significant prognostic factors should be taken into account during the preoperative evaluation of patients. Deferral of procedure or specialized postoperative care may be indicated in high risk patients.

Semi-solid feeds may reduce the risk of aspiration pneumonia and shorten postoperative length of stay after percutaneous endoscopic gastrostomy (PEG).

Background and study aims: Feeding-related adverse events after percutaneous endoscopic gastrostomy (PEG) such as aspiration pneumonia may result in prolonged hospitalization and postoperative mortality. This study evaluated the efficacy of using semi-solid feeds to reduce feeding-related adverse events and improve clinical outcomes. Patients and methods: Patients who received PEG for enteral nutrition at our hospital between January 2014 and December 2015 were allocated to a postoperative feeding protocol that used either liquid feed or semi-solid feed. Baseline characteristics, postoperative feeding-related adverse events and clinical outcomes in the 2 groups were prospectively analysed and compared. Results: One hundred and seventeen PEG patients (age range: 59 - 97 years, male: 53) were enrolled with 72 patients given liquid feed and 45 patients receiving semi-solid feed. Baseline characteristics were similar in both groups. The semi-solid feed group experienced fewer incidence of feeding-related aspiration pneumonia (2.2 % vs. 22.2 %, P < 0.005) and shorter postoperative hospital length of stay (12.7 days vs. 18.8 days, P < 0.01). Significant differences were not observed in the frequency of peristomal infection (11.1 % vs. 12.5 %, P = 0.82), feeding-related diarrhea (2.2 % vs. 12.5 %, P = 0.09) and 30-day mortality rates (2.2 % vs. 8.3 %, P = 0.25). Conclusions: Semi-solid feeding may reduce the risk of aspiration pneumonia and shorten postoperative hospital length of stay after PEG. Semi-solid feeds are safe to use and can be employed either as a first line feeding protocol or an alternative when liquid feeding is unsuccessful.

Percutaneous endoscopic gastrostomy (PEG) using a novel large-caliber introducer technique kit: a retrospective analysis.

BACKGROUND AND STUDY AIMS: Percutaneous endoscopic gastrostomy (PEG) using the introducer technique is not only useful in patients with upper digestive tract stenosis but has been shown to reduce peristomal infection. In this study, we evaluated the safety and utility of a novel large-caliber introducer PEG kit (using 20 Fr size tube) compared with a push kit of similar size. PATIENTS AND METHODS: One hundred and thirty-six patients who received PEG at our hospital between January 2014 and December 2015 were retrospectively analyzed. Baseline characteristics, laboratory biomarkers, hemodynamic changes, postoperative adverse events and clinical outcomes with both kits were compared. RESULTS: The new introducer PEG kit was used in 61 patients while the remaining 75 patients received tube placement using a push technique PEG kit. Except for the prevalence of dementia, which was lower in the introducer PEG kit group, baseline characteristics were similar in both groups. Tube placements were 100 % successful with both PEG kits and there were no significant differences in the change of postoperative hemodynamic or laboratory biomarkers. The Introducer PEG kit group experienced fewer incidence of feeding-related aspiration pneumonia (8.2 % vs. 24 %, P = 0.02), lower peristomal infection scores (1.2 vs. 1.6, P < 0.01), shorter postoperative length of stay (16 days vs. 23.7 days, P = 0.01) and fewer deaths at day 60 (3.3 % vs. 16 %, P = 0.02). CONCLUSIONS: Gastrostomy using the new large-caliber introducer PEG kit is safe and produced non-inferior (with some favourable) results when compared to the push technique using similar size tubes.

Percutaneous endoscopic transgastric jejunostomy (PEG-J): a retrospective analysis on its utility in maintaining enteral nutrition after unsuccessful gastric feeding.

BACKGROUND/AIMS: Although percutaneous endoscopic gastrostomy (PEG) is the method of choice for long-term enteral nutrition, feeding-related adverse events such as aspiration pneumonia and peristomal leakage can impede the use of PEG. Percutaneous endoscopic transgastric jejunostomy (PEG-J) using large-bore jejunal tubes may help by circumventing gastric passage during enteral nutrition and improving drainage of gastric secretions. METHODS: 20 patients (12 males and 8 females) who received PEG-J after unsuccessful PEG feeding during a 6-year period in our institution were analysed retrospectively to evaluate the efficacy of large-bore jejunal feeding tubes in maintaining enteral nutrition. RESULTS: The median age was 83.5 (71-96) years. The median period between PEG and PEG-J was 33 (14-280) days. Indications were aspiration due to gastro-oesophageal reflux in 18 patients and severe peristomal leakage in 2 patients. Tube placements were successful in all patients. There were 6 (30%) in-hospital mortalities, with 3 (15%) occurring within 30 days after procedure. CONCLUSIONS: PEG-J can be performed safely in patients with PEG and may facilitate the maintenance of enteral nutrition in most of the patients. Patients with unsuccessful PEG feeding can be offered the option of jejunal feeding before considering termination of enteral nutrition.

Reduced INF2 expression in nephrotic syndrome is possibly related to clinical severity of steroid resistance in children.

AIM: Mutations of the inverted formin 2 gene (INF2), which encodes a member of the formin family, cause autosomal dominant focal segmental glomerulosclerosis (FSGS) and Charcot-Marie-Tooth (CMT) disease-associated FSGS. However, their role in idiopathic FSGS remains unclear. This study investigated INF2 localization in the normal adult kidney and its expression in children with idiopathic nephrotic syndrome. METHODS: We generated a rabbit polyclonal antibody against the conjugated peptide from human INF2 and studied the glomerular expression of INF2 and synaptopodin using normal human adult kidney tissues and tissues from children with glomerular diseases such as minimal change disease (MCD), FSGS, IgA nephropathy (IgAN), non-IgA mesangial proliferative glomerulonephritis (non-IgAN), and Henoch-Schönlein purpura nephritis (HSPN). RESULTS: The anti-INF2 antibody detected an approximately 140-kD fragment isolated from adult mature glomeruli by western blotting. Immunohistochemically, INF2 was detected in podocytes and renal arteries. Among 56 patients, INF2 in glomeruli was expressed at a similar level in patients with MCD, IgAN, non-IgAN, or HSPN and controls. In FSGS patients, INF2 expression in glomeruli was either decreased or absent. There was a relationship between decreased INF2 expression and the clinical severity of steroid resistant nephrotic syndrome (SRNS). CONCLUSION: We propose that examination of INF2 expression may help to differentiate MCD from FSGS and evaluate the clinical severity of SRNS in children.

Hypophosphatemic rickets developed after treatment with etidronate disodium in a patient with generalized arterial calcification in infancy.

Ectonucleotide pyrophosphatase/phosphodiesterase 1 (ENPP1) was originally reported as a responsible gene for generalized arterial calcification in infancy (GACI). Though the prognosis of GACI patients is poor because of myocardial infarction and heart failure in relation to medial calcification of the coronary arteries, some patients rescued by bisphosphonate treatment have been reported. Recently, ENPP1 is also reported as responsible for autosomal recessive hypophosphatemic rickets type 2. We show here a boy with homozygous ENPP1 mutations diagnosed as having GACI in early infancy. After the diagnosis, he was treated with etidronate disodium (EHDP) in combination with antihypertensive drugs. The calcification of major arteries was diminished and disappeared by the age of eight months. He also showed mild hypophosphatemia (2.6-3.7 mg/dl) from the age of one year. After the treatment with EHDP for five years, he showed genu valgum with hypophosphatemia (2.6 mg/dl). He was diagnosed as having hypophosphatemic rickets at the age of seven years. The findings that hyper-mineralization of the arteries and hypo-mineralization of the bone observed in the same patient are noteworthy. ENPP1 could be regarded as a controller of the calcification of the whole body at least in part.

HPGCD outperforms HPBCD as a potential treatment for Niemann-Pick disease type C during disease modeling with iPS cells.

Niemann-Pick disease type C (NPC) is a lysosomal storage disease characterized by abnormal accumulation of free cholesterol and glycolipids. Here, we established induced pluripotent stem cell (iPSC) lines from NPC patients. Hepatocyte-like cells (HLCs) and neural progenitors derived from the iPSC lines accumulated cholesterol and displayed impaired autophagy and ATP production. A molecular signature related to lipid metabolism was also impaired in the NPC-iPSC-derived HLCs. These findings indicate that iPSC-derived cells can phenocopy human NPC. We also newly found that 2-hydroxypropyl-γ-cyclodextrin (HPGCD) could reduce the cholesterol accumulation and restore the functional and molecular abnormalities in the NPC patient-derived cells, and do so more effectively than 2-hydroxypropyl-ß-cyclodextrin treatment. In addition, NPC model mice showed an improved liver status and prolonged survival with HPGCDs. Thus, iPSC lines derived from patient cells are powerful tools to study cellular models of NPC, and HPGCD is a potential new drug candidate for future treatment of this disease.