BACKGROUND: Mitochondrial diseases (MDs) are systemic disorders that can affect multiple organs. Renal manifestations, including renal tubular acidosis, are common because kidneys are particularly vulnerable to energy deprivation. Treatment of MDs is often complex and electrolyte replacement can be difficult especially in pediatric patients, because large and repeated amounts of oral supplements are needed but are not well tolerated.
METHODS: We describe the case of a girl affected by Kearns-Sayre disease with severe renal tubular acidosis. The management of her metabolic acidosis was challenging because she showed persistent low levels of serum bicarbonates despite a progressive incrementation of oral bicarbonates. Furthermore, as a result to the ingestion of large amounts of alkali, the girl developed an aversion to oral supplementation. After positioning a percutaneous gastrostomy (PEG) and starting enteral administration of bicarbonates (with daily boluses and continuous nocturnal infusion), she finally obtained an adequate electrolyte control, with a significant increase in her quality of life.
CONCLUSIONS: In MDs, the combination of nocturnal continuous enteral administration of alkali plus diurnal boluses may represent a valid solution to correct metabolic acidosis. It can also result in an improved patients\' quality of life, particularly in pediatric settings, where compliance to oral therapy is often lacking due to the large and repeated amounts of unpalatable bicarbonates solutions required.

UNASSIGNED: Although percutaneous endoscopic gastrostomy (PEG) placement is still widely practiced in Japan, studies from Western countries report that it is less beneficial for patients in end-of-life care with cognitive decline. Decisions regarding PEG placement are largely influenced by physician judgment.
UNASSIGNED: The aim of this study was to investigate the background and perceptions of Japanese physicians regarding PEG for older adults in end-of-life care and to identify the factors associated with differences in physician judgment regarding PEG.
UNASSIGNED: The study employed a cross-sectional design.
UNASSIGNED: A questionnaire on PEG for older adults in end-of-life care was sent to Japanese physicians. Logistic regression analysis was used to calculate the odds ratios (ORs) and confidence intervals (CIs) of the association between PEG recommendations and each factor.
UNASSIGNED: PEG placement was advised for bedridden patients and older adults with cognitive decline by 26% of the physicians who responded to the survey. Differences in physician perceptions of PEG feeding were associated with the recommendation for PEG, benefits of preventing aspiration pneumonia (OR: 4.9; 95% CI: 3.1-8.2), impact on post-discharge accommodation decisions (OR: 6.1; 95% CI: 1.9-30.9), and hesitancy to recommend a PEG placement (OR: 1.9; 95% CI: 1.3-4.5). Working in a facility with PEG placement (OR: 2.0; 95% CI: 1.2-3.5) was an associated background factor.
UNASSIGNED: Differences in Japanese physicians\' attitudes toward using PEG feeding for older adults in end-of-life care were significantly associated with differences in their perceptions of the impact of PEG feeding and working in a facility with PEG placement.

Percutaneous endoscopic gastrostomy (PEG) is widely used for long-term enteral nutrition in patients unable to maintain adequate oral intake. Despite advancements in PEG techniques, complications remain a concern. We report a case of a 94-year-old bedridden man who developed significant complications after PEG placement using the pull method. Initially, minor bleeding at the puncture site was managed using traction compression. However, the patient later experienced hemorrhagic shock owing to pulsatile bleeding around the gastrostomy site. Despite attempts to control the bleeding through traction and transfusions, a pseudoaneurysm adjacent to the PEG button was identified. The patient underwent successful transcatheter arterial embolization (TAE). Post-TAE, no further bleeding or hematoma was observed, and imaging confirmed the resolution of the pseudoaneurysm and hematoma. Methicillin-resistant Staphylococcus aureus (MRSA) infection was detected at the gastrostomy site, which contributed to complications. Despite successful management of the bleeding, the patient\'s overall condition deteriorated, and he died on postoperative day 66. This case underscores the importance of vigilant monitoring and management of PEG-related complications, particularly infections that may precipitate severe vascular events.

BACKGROUND: Initiation of feeding after percutaneous endoscopic gastrostomy (PEG) placement has been debated. Randomized controlled trials (RCTs) have been performed on early feeding compared with delayed feeding after PEG placement with varying results. Therefore, a meta-analysis was conducted examining early vs delayed feeding after placement of a PEG.
METHODS: A comprehensive search of databases was conducted in January 2024. Peer-reviewed published RCTs comparing early feeding (≤4 h) with delayed feeding (>4 h) were identified and included in the meta-analysis. Meta-analysis was completed using pooled estimates of overall complications, individual complications, mortality ≤72 h, and number of day 1 significant gastric residual volumes.
RESULTS: Six RCTs (n = 467) were included in the analysis. Comparison of early feeding with delayed feeding after PEG showed no statistically significant differences for overall complications (P = 0.18), mortality ≤72 h (P = 0.3), and number of day 1 significant gastric residual volumes (P = 0.05). No differences were also noted for individual complications, including vomiting, wound infection, bleeding, or diarrhea.
CONCLUSIONS: Feeding ≤4 h after PEG have no differences in minor and major complications compared with that of delayed feeding. Early feeding ≤4 h is safe and should be recommended in future guidelines.

UNASSIGNED: When commencing enteral feeding, patients and families will want to know the likelihood of returning to an oral diet. There is a paucity of data on the prognosis of patients with gastrostomies. We describe a large dataset of patients, which identifies factors influencing gastrostomy removal and assesses the likelihood of the patient having at home enteral nutrition.
UNASSIGNED: Retrospective data was collected on patients from Sheffield Teaching Hospitals who had received a gastrostomy and had outpatient enteral feeding between January 2016 and December 2019. Demographic data, indication and outcomes were analysed.
UNASSIGNED: A total of 451 patients were assessed, median age: 67.7. 183/451(40.6%) gastrostomies were for head and neck cancer, 88/451 (19.5%) for stroke, 28/451 (6.2%) for Motor Neuron Disease, 32/451 (7.1%) for other neurodegenerative causes, 120/451 (26.6%) other. Of the 31.2% who had their gastrostomy removed within 3 years, head and neck cancer was the most common indication (58.3%) followed by stroke (10.2%), Motor Neuron Disease (7.1%) and other neurodegenerative diseases (3.1%). Gastrostomy removal was significantly influenced by age, place of residence, and having head and neck cancer (p < 0.05). There was the greatest likelihood of removal within the first year (24%). 70.5% had enteral feeding at home.
UNASSIGNED: This large cohort study demonstrates 31.2% of patients had their gastrostomy removed within 3 years. Head and neck cancer patients, younger age and residing at home can help positively predict removal. Most patients manage their feeding at home rather than a nursing home. This study provides new information on gastrostomy outcomes when counselling patients to provide realistic expectations.

BACKGROUND: Clinically assisted nutrition and hydration via percutaneous endoscopic gastrostomy (PEG) is a therapeutic option to ameliorate the difficulties associated with enhanced catabolism, weight loss, and dysphagia in Huntington\'s disease (HD).
OBJECTIVE: The objective is to provide insights into demographics, staging (Shoulson-Fahn), complications, weight trajectories, and survival rates in people with HD (pwHD) who underwent PEG.
METHODS: This retrospective study included 705 consecutive pwHD who attended our HD clinic between July 2006 and March 2024, of whom 52 underwent PEG. A control group (n = 52), comprising pwHD without PEG, were closely matched for sex, stage, age, CAG length, and disease burden score at PEG. The study was registered as a service evaluation at the National Hospital for Neurology and Neurosurgery.
RESULTS: PEG prevalence was 15.0% (n = 52/347) among manifest pwHD: 4.8% (n = 3/62) for Stage 3; 33.3% (n = 16/48) for stage 4; and 44.1% (n = 30/68) for stage 5. Commonest indications were dysphagia, weight loss, and inadequate oral intake. Complications included chest infection, tube dislodgement, and peristomal and skin infections. Modeling of weight trajectories after PEG found no difference between PEG and non-PEG groups. Mortality rate was 34.6% (n = 18/52) in the PEG and 36.5% (n = 19/52) in the non-PEG groups (P = 0.84). Treatment duration (until study endpoint or death) was 3.48 years (interquartile range = 1.71-6.02; range = 0.23-18.8), with 65.4% (n = 34/52) alive at the study endpoint.
CONCLUSIONS: PEG in pwHD at-risk for weight loss may help slow weight loss. Prospective studies are required to strengthen PEG decision-making in pwHD. PEG survival was much longer than other dementias, highlighting the need to consider PEG independently in pwHD.

BACKGROUND: Percutaneous radiological gastrostomy (PRG) and percutaneous endoscopic gastrostomy (PEG) are minimally invasive gastrostomy techniques for individuals requiring prolonged enteral feeding. Recent meta-analyses concerning their efficacy and safety mainly included retrospective studies and yielded conflicting results. This meta-analysis of randomized controlled trials (RCTs) aimed to compare efficacy, safety, and procedure time between PRG and PEG for enteral feeding.
METHODS: MEDLINE, Embase, and the Cochrane Library were searched for eligible RCTs comparing PRG and PEG for enteral feeding through February 23, 2024. The primary outcome was technical success. The secondary outcomes were (1) adverse events (AEs), (2) mortality, and (3) procedure time. We used the random-effects model to calculate pooled risk ratio (RR) and mean difference (MD) with corresponding 95% CIs for dichotomous and continuous outcomes, respectively.
RESULTS: Five RCTs with 544 patients (268 PRG and 276 PEG) were included. There was similar technical success (RR = 1.02; 95% CI = 0.98-1.05; I² = 35%; moderate certainty of evidence because of inconsistency), overall mortality (RR = 1.25; 95% CI = 0.63-2.47; I² = 47%; very low certainty of evidence because of inconsistency, indirectness, and imprecision), and overall AEs risk (RR = 1.06; 95% CI = 0.63-1.76; I² = 81%; low certainty of evidence because of inconsistency and imprecision) between the two groups. However, compared with PEG, the procedure time was longer in the PRG group (MD = 19.35 min; 95% CI = 0.95-37.75 min; I² = 98%; very low certainty of evidence because of inconsistency and imprecision).
CONCLUSIONS: PRG and PEG demonstrate similar efficacy and safety; however, the endoscopic technique may boast a shorter procedure time.

Bacteria can impact the host organism through their metabolites, with short-chain fatty acids (SCFAs) being the most important, including acetate (C2), propionate (C3), butyrate (C4), valerate (C5n), and isovalerate (C5i). This study aimed to identify the impact of enteral nutrition on SCFAs in children with cerebral palsy and to test the hypothesis that the type of nutrition in cerebral palsy affects gut SCFA levels. Cerebral palsy is a heterogeneous syndrome resulting from non-progressive damage to the central nervous system. The study group included 30 children diagnosed with cerebral palsy, receiving enteral nutrition (Cerebral Palsy Enteral Nutrition (CPEN)) via gastrostomy. The first reference group (Cerebral Palsy Controls (CPCs)) consisted of 24 children diagnosed with cerebral palsy and fed orally on a regular diet. The second reference group (Healthy Controls (HCs)) consisted of 24 healthy children with no chronic disease and fed on a regular diet. Isolation and measurement of SCFAs were conducted using gas chromatography. Differences were observed in the median contents of isobutyric acid, valeric acid, and isovaleric acid between the CPC group, which had significantly higher levels of those acids than the HC group. No differences were found between the CPEN and CPC groups nor between the CPEN and HC groups. We conclude that enteral nutrition in cerebral palsy has no influence on the levels of SCFAs.

BACKGROUND: Percutaneous endoscopic gastrostomy (PEG) and laparoscopically inserted gastrostomy have become the gold standard for adult patients and children, respectively, requiring long-term enteral nutrition support. Procedure-related mortality is a rare event, often reported to be zero in smaller studies. National data on 30-d mortality and long-term survival rates after gastrostomy placement are scarce in the literature.
OBJECTIVE: To study the use of gastrostomies in Sweden from 1998-2019 and to analyze procedure-related mortality and short-term (< 30 d) and long-term survival.
METHODS: In this retrospective, population-based cohort study, individuals that had received a gastrostomy between 1998-2019 in Sweden were included. Individuals were identified in the Swedish National Patient Register, and survival analysis was possible by cross-referencing the Swedish Death Register. The cohort was divided into three age groups: Children (0-18 years); adults (19-64 years); and elderly (≥ 65 years). Kaplan-Meier with log-rank test and Cox regression were used for survival analysis.
RESULTS: In total 48682 individuals (52% males, average age 60.9 ± 25.3 years) were identified. The cohort consisted of 12.0% children, 29.5% adults, and 58.5% elderly. An increased use of gastrostomies was observed during the study period, from 13.7/100000 to 22.3/100000 individuals (P < 0.001). The use of PEG more than doubled (about 800 to 1800/year), with a corresponding decrease in open gastrostomy (about 700 to 340/year). Laparoscopic gastrostomy increased more than ten-fold (about 20 to 240/year). Overall, PEG, open gastrostomy, and laparoscopic gastrostomy constituted 70.0% (n = 34060), 23.3% (n = 11336), and 4.9% (n = 2404), respectively. Procedure-related mortality was 0.1% (n = 44) overall (PEG: 0.05%, open: 0.24%, laparoscopic: 0.04%). The overall 30-d mortality rate was 10.0% (PEG: 9.8%, open: 12.4%, laparoscopic: 1.7%) and decreased from 11.6% in 1998-2009 vs 8.5% in 2010-2019 (P < 0.001). One-year and ten-year survival rates for children, adults, and elderly were 93.7%, 67.5%, and 42.1% and 79.9%, 39.2%, and 6.8%, respectively. The most common causes of death were malignancies and cardiovascular and respiratory diseases.
CONCLUSIONS: The annual use of gastrostomies in Sweden increased during the study period, with a shift towards more minimally invasive procedures. Although procedure-related death was rare, the overall 30-d mortality rate was high (10%). To overcome this, we believe that patient selection should be improved.

BACKGROUND: There are no reports of anesthesia use in adult patients with Silver-Russell syndrome (SRS). Here, we report our experience with anesthesia in an adult patient with SRS complicated by chronic respiratory failure.
METHODS: A 33-year-old woman was clinically diagnosed with SRS. She had severe chronic respiratory failure, complicated by superior mesenteric artery syndrome. Percutaneous gastrostomy was scheduled for nutritional management under epidural anesthesia; however, soon after esophagogastroduodenoscopy was started, she lost consciousness and spontaneous respiration. The patient was urgently intubated and converted to general anesthesia. The end-tidal carbon dioxide tension was > 90 mmHg at intubation.
CONCLUSIONS: Adult SRS patients with chronic respiratory failure have a risk of CO2 narcosis. SRS also requires preparation for difficult airway management during the perioperative period.