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ObjectiveTo evaluate associations between neighborhood income and burden of hospitalizations for children with short bowel syndrome (SBS).Study designWe used the Pediatric Health Information System (PHIS) database to evaluate associations between neighborhood income and hospital readmissions, readmissions for central line-associated bloodstream infections (CLABSI), and hospital length of stay (LOS) for patients

IntroductionSevere forms of short bowel syndrome (SBS) resulting in chronic intestinal failure (IF) have limited therapeutic options, all of which are associated with significant morbidities. Spring-mediated distraction enterogenesis (SMDE) uses an intraluminal self-expanding spring to generate mechanical force to induce intestinal stretching and sustained axial growth, providing a promising novel approach for patients with SBS. Previous studies have established this method to be safe and effective in small and large animal models. However, SMDE has previously not been implemented in a large, clinically relevant animal model.MethodsJuvenile mini-Yucatan pigs with 75% of their small intestine resected had intraluminal springs placed after an initial adaptive period. Morphological and histological assessments were performed on SMDE segments compared to the control region of the intestine undergoing normal adaptive responses to resection.ResultsWhile the initial histologic adaptive response observed following resection was attenuated after a month, the SMDE segments instead augmented these adaptive changes. Specifically, intestinal length increased 2-fold in SMDE segments, and the widths of the epithelial, muscularis, and serosal layers were enhanced in SMDE compared with control segments of the same animal. This data suggests that morphologic intestinal adaptation may be enhanced with SMDE in the setting of SBS.DiscussionHere we demonstrate the successful and reproducible implementation of SMDE in a large animal model in the setting of prior intestinal resection, making SMDE a viable and novel approach for SBS to be explored further.

The destruction of red cells during blood collection or with the processing of the sample continues to occur at a high rate, especially among emergency department (ED) patients. This can produce pre-analytical laboratory errors, particularly for potassium. We determined the incidence of hemolyzed samples and discuss the potential medical impact for hypokalemic patients who potassium level is artificially normal (pseudoeukalemia). Potassium results were obtained for a 6-month period. Using a measured hemolysis index (HI), hemolysis was present in 3.1 % for all potassium ordered (n=94,783) and 7.5 % for ED orders (n=22,770). Most of these samples were reported as having high normal result or were hyperkalemic. There were 22 hemolytic samples with a potassium of <3.5 mmol/L, and 57 hemolytic samples with a potassium in lower limit of normal (3.5–3.8 mmol/L). From this group, we examined the medical histories of 8 selected patients whose initially normal potassium levels were subsequently confirmed to have a potassium values that were below, at, or just above the lower limit of normal due to hemolysis. The primary complaint for these patients were: necrotizing soft tissue infection, pancreatitis, volume overload from heart failure with reduced ejection fraction, hypertension treated with hydrochlorothiazide, and presence of a short bowel syndrome. A subsequent non-hemolyzed sample was collected demonstrating hypokalemia in all of these patients. Within these cases, there was a potential for harm had hemolysis detection not been performed. We demonstrate the medical importance of detecting hemolysis for patients who have pseudoeukalemia. This is relevant because the HI cannot be obtained when electrolytes are tested using whole blood samples, and a normal potassium may lead to inappropriate patient management. [ABSTRACT FROM AUTHOR]

Pediatric chronic intestinal failure (PIF) is a rare and heterogeneous condition characterized by the inability of the patient's intestine to adequately absorb the required fluids and/or nutrients for growth and homeostasis. As a result, patients will become dependent on home parenteral nutrition (HPN). A MEDLINE search was performed in May 2024 with keywords "intestinal failure", "parenteral nutrition" and "pediatric". Different underlying conditions which may result in PIF include short bowel syndrome, intestinal neuromuscular motility disorders and congenital enteropathies. Most common complications associated with HPN are catheter-related bloodstream infections, catheter-related thrombosis, intestinal failure-associated liver disease, small intestinal bacterial overgrowth, metabolic bone disease and renal impairment. Treatment for children with PIF has markedly improved with a great reduction in morbidity and mortality. Centralization of care in specialist centers and international collaboration between centers is paramount to further improve care for this vulnerable patient group. A recently promising medical therapy has become available for children with short bowel syndrome which includes glucagon-like peptide 2, a naturally occurring hormone which is known to delay gastric emptying and induce epithelial proliferation. Despite advances in curative and supportive treatment, further research is necessary to improve nutritional, pharmacological and surgical care and prevention of complications associated with parenteral nutrition use. [ABSTRACT FROM AUTHOR]

A 4-year-old neutered male English bulldog was referred to the veterinary teaching hospital for presumed short bowel syndrome following an ileocecal resection to remove a tumor involving the caudal third of the jejunum, ileum, and proximal colon. Histopathology of the tumor revealed chronic transmural lipogranulomatous and necrotizing lymphangitis. After surgery, mixed-bowel diarrhea developed and the patient lost weight. When the diarrhea and weight loss did not respond to medical management, the patient was referred to the university nutrition service for nutritional management of presumed short bowel syndrome and lymphangitis. Diet modification in conjunction with medical management led to improved body weight and fecal consistency. [ABSTRACT FROM AUTHOR]

Objective: To compare the phase angle (PhA) through bioelectrical impedance (BIA) of children with intestinal failure (IF) using prolonged parenteral nutrition (PN) followed by an Intestinal Rehabilitation Program, with a control group. Methods: Children under 10 years of age with IF using prolonged PN for >60 days (study group) were included. The control group consisted of healthy children without chronic pathologies, matched by sex and age. Anthropometric parameters evaluated were: weight, height, weight/ age z-score (W/A), height/age z-score (H/A), BMI, BMI/A z-score, arm circumference, triceps skinfold, subscapular skinfold, mid-arm muscle circumference. BIA parameters were resistance (R), reactance (Xc), and phase angle (PhA). Results: Twenty-eight children were included in the study group, median (IQR) age was 11 (8(27) months, 53.6 % were male. In the control group, 28 children were included, median (IQR) age was 12.5 (8(24.7) months, 50 % were male. Children from the study group had W/A z-scores and H/A z-scores significantly lower than controls. There was no significant difference between PhA in the study group and controls, [median (IQR) 4.3° (3.8;4.6) vs 4.0° (3.8;5.4) respectively, p = 0.980]. Prematurity was significantly higher in the study group than in the controls, but there was no significant correlation between gestational age at birth and PhA of the children from the study group. [ABSTRACT FROM AUTHOR]

Purpose of Review: Intestinal failure (IF), a rare organic dysfunction, is characterized by a reduction in intestinal function, necessitating intravenous supplementation to sustain health and/or growth due to inadequate absorption of macro and micronutrients, water, and electrolytes. This condition is distinct from "Intestinal Insufficiency," where intravenous replacement is not immediately necessary. The purpose of this review is to evaluate the literature published in the last 5 years on drug treatment in the management of patients with intestinal failure (IF) and short bowel syndrome (SBS) and to identify strategies to minimize fluid and electrolyte loss by increasing nutrient-enterocyte contact time, improving intestinal transit time, replenishing micronutrients, and enhancing nutrient absorption. Recent Findings: We describe current treatments for rehabilitation aimed at optimizing absorption of the intestinal mucosa. We also outline recommended pharmacological adjustments of some drugs used in the treatment of commonly found comorbidities in these patients. Recent articles have demonstrated that GLP-2 analogs have become an important tool in the treatment of SBS, improving intestinal secretory control, gastrointestinal transit time, nutrient absorption, and, most importantly, positively impacting quality of life. Summary: IF is a rare disease defined by need for intravenous hydration and nutrients delivery and associated with malnutrition and high mortality rate. Medical management and treatment of IF can increase survival time and quality of life. [ABSTRACT FROM AUTHOR]

Background: Intestinal failure-associated liver disease (IFALD) is a complication of long-term PN use, attributed to the use of ω-6 injectable lipid emulsions (ILE). Fish oil (FO) ILE have been successful in reversing liver injury in neonates. Evidence for pure FO ILE use in adult patients is limited. Methods: Case series of the use of FO lipid emulsions in adults with IFALD from the University of Chicago PN registry. Analysis of medical charts and PN formulations was performed. Results: Three cases of IFALD treated with FO ILE were identified. The first case was a 30-year-old man with short bowel syndrome (SBS), hyperbilirubinemia, and biopsy-proven IFALD. Following a change from a soy lipid emulsion to FO lipid emulsion, his liver tests rapidly improved and remained stable over 202 weeks of use. The second case was a 76-year-old woman with intestinal failure (IF) due to a frozen bowel. A change from a soy ILE to a composite lipid and later to a pure FO ILE did not result in improvement in her liver tests. The third case was a 28-year-old man with SBS and biopsy-proven IFALD. Change to a composite ILE and subsequently FO lipid emulsion resulted in a gradual improvement in liver tests. No clinical essential fatty acid (EFA) deficiencies were identified during treatment. Conclusion: FO ILE may be effective in the treatment of adult patients with cholestatic IFALD. Use is safe with no EFA deficiencies detected in up to 4 years of use. [ABSTRACT FROM AUTHOR]

Intestinal failure‐associated liver disease (IFALD) is a serious complication of long‐term parenteral nutrition in patients with short bowel syndrome (SBS), and is the main cause of death in SBS patients. Prevention of IFALD is one of the major challenges in the treatment of SBS. Impairment of intestinal barrier function is a key factor in triggering IFALD, therefore promoting intestinal repair is particularly important. Intestinal repair mainly relies on the function of intestinal stem cells (ISC), which require robust mitochondrial fatty acid oxidation (FAO) for self‐renewal. Herein, we report that aberrant LGR5+ ISC function in IFALD may be attributed to impaired farnesoid X receptor (FXR) signaling, a transcriptional factor activated by steroids and bile acids. In both surgical biopsies and patient‐derived organoids (PDOs), SBS patients with IFALD represented lower population of LGR5+ cells and decreased FXR expression. Moreover, treatment with T‐βMCA in PDOs (an antagonist for FXR) dose‐dependently reduced the population of LGR5+ cells and the proliferation rate of enterocytes, concomitant with decreased key genes involved in FAO including CPT1a. Interestingly, however, treatment with Tropifexor in PDOs (an agonist for FXR) only enhanced FAO capacity, without improvement in ISC function and enterocyte proliferation. In conclusion, these findings suggested that impaired FXR may accelerate the depletion of LGR5 + ISC population through disrupted FAO processes, which may serve as a new potential target of preventive interventions against IFALD for SBS patients. [ABSTRACT FROM AUTHOR]

Avian coccidiosis, a common disease caused by Eimeria species, results in significant losses in global poultry production. Mycotoxins are low-molecularweight natural products (i.e., small molecules) produced as secondary metabolites by filamentous fungi and they have the potential to economically and significantly affect global poultry production. Little is known about the relationship between mycotoxins and avian coccidiosis, although they often co-occur in the field. This comprehensive review examines the intricate relationship between mycotoxins and avian coccidiosis, in particular how mycotoxins, including aflatoxins, ochratoxins, trichothecenes as well as Fusarium mycotoxins, compromise the health of the poultry flock and open the door to Eimeria parasites in the gut. In addition, this review sheds light on the immunosuppressive effects of mycotoxins, their disruption of cellular signaling pathways, and the consequent exacerbation of coccidiosis infections. The mechanisms of mycotoxin toxicity are also reviewed, emphasizing direct damage to intestinal epithelial cells, impaired nutrient absorption, inflammation, oxidative stress, and changes in the gut microbiota. Finally, the consequences for the prevention and treatment of coccidiosis when mycotoxins are present in the feed are discussed. This review emphasizes the need for effective management strategies to mitigate the combined risks of mycotoxins and coccidiosis and highlights the complexity of diagnosing and controlling these interrelated problems in poultry. The review advocates a holistic approach that includes strict feed management, disease prevention measures and regular monitoring to maintain the health and productivity of poultry against these significant challenges. [ABSTRACT FROM AUTHOR]

By introducing the healing of many distinctive anastomoses by BPC 157 therapy, this review practically deals with the concept of the resection and reconnection of the hollow parts of the gastrointestinal tract as one of the cornerstones of visceral surgery. In principle, the healing of quite distinctive anastomoses itself speaks for applied BPC 157 therapy, in particular, as a way in which the therapy of anastomoses can be successfully approached and carried out. Some of the anastomoses implicated were esophagogastric, colocolonic, jejunoileal, and ileoileal anastomoses, along with concomitant disturbances, such as esophagitis, sphincter dysfunction, failed intestinal adaptation, colitis, short bowel syndrome, major vessel occlusion, NO-system, and prostaglandins-system dysfunction, which were accordingly counteracted as well, and, finally, findings concerning other anastomoses healing (i.e., nerve and vessel). Moreover, the healing of fistulas, both external and internal, colocutaneous, gastrocutaneous, esophagocutaneous, duodenocutaneous, vesicovaginal, colovesical, and rectovaginal in rats, perceived as anastomoses made between two different tissues which are normally not connected, may also be indicative. This may be a particular reconnection of the parts of the gastrointestinal tract to re-establish adequate integrity depending on the tissue involved, given that both various intestinal anastomoses and various fistulas (intestinal and skin were accordingly healed simultaneously as the fistulas disappeared) were all healed. [ABSTRACT FROM AUTHOR]

To evaluate the influence of early nutritional intake on the growth pattern of very preterm infants. This was an observational study including 109 newborns (< 32 weeks gestational age). Perinatal morbidities, nutritional therapy (first four weeks of life), and weight, length, and head circumference (HC) growth at term-equivalent age were evaluated. Growth restriction was defined as a difference > 1.2 SD between the birth and term age measurements. Growth restriction at term-equivalent age: 52.3% (weight), 42.9% (length), and 22% (HC). Morbidities were positively correlated with nutrition therapy and negatively correlated with the total energy provision: protein ratio. The duration of parenteral nutrition, the time to reach full enteral feedings, and the total energy provision: protein ratio were significantly correlated. Nutrient intake influenced weight, length, and HC growth, and cumulative energy deficit was significantly associated with HC growth restriction. Conclusion: Perinatal morbidities interfere with nutritional therapy and early nutrient intake, leading to insufficient energy and energy provision: protein ratio for growth. What is Known: • The intake of macronutrients early in life, mainly protein, is important for the optimal growth of pretem infants. • The severity of morbidities and low gestational ages impact the nutritional management of preterm infants. What is New: • The number of morbidities, reflecting the severity of the neonatal clinical course, had a detrimental effect on the nutritional therapy and nutrients intake. • The inadequate energy provision per gram of protein ratio was significantly associated with growth restriction in all growth measures at the second week of life, persisting for head circumference up to the fourth week, highlighting the importance of its measurement, as it could be a precocious sign of development risk. [ABSTRACT FROM AUTHOR]

Teduglutide is a glucagon-like-peptide-2 analogue that reduces the need for parenteral support in patients with short bowel syndrome (SBS). Nevertheless, data about long-term therapy with teduglutide in children are still scarce. Our objective was to describe the real-life experience with teduglutide in children with SBS over the last 5 years in Spain. This was a national multicentre and prospective study of paediatric patients with intestinal failure (IF) treated with teduglutide for at least 3 months. The data included demographic characteristics, medical background, anthropometric data, laboratory assessments, adverse events, and parenteral nutrition (PN) requirements. Treatment response was defined as a > 20% reduction in the PN requirement. The data were collected from the Research Electronic Data Capture (REDCap) database. Thirty-one patients from seven centres were included; the median age at the beginning of the treatment was 2.3 (interquartile range (IQR) 1.4–4.4) years; and 65% of the patients were males. The most frequent cause of IF was SBS (94%). The most common cause of SBS was necrotizing enterocolitis (35%). The median residual bowel length was 29 (IQR 12–40) cm. The median duration of teduglutide therapy was 19 (IQR 12–36) months, with 23 patients (74%) treated for > 1 year and 9 treated for > 3 years. The response to treatment was analysed in 30 patients. Twenty-four patients (80%) had a reduction in their weekly PN energy > 20% and 23 patients (77%) had a reduction in their weekly PN volume > 20%. Among the responders, 9 patients (29%) were weaned off PN, with a median treatment duration of 6 (IQR 4.5–22) months. The only statistically significant finding demonstrated an association between a > 20% reduction in the weekly PN volume and a younger age at the start of treatment (p = 0.028). Conclusions: Teduglutide seems to be an effective and safe treatment for paediatric patients with IF. Some patients require a prolonged duration of treatment to achieve enteral autonomy. Starting treatment with teduglutide at a young age is associated with a higher response rate. What is Known: • Glucagon-like peptide-2 (GLP-2) plays a crucial role in the regulation of intestinal adaptation in short bowel syndrome (SBS). Teduglutide is a GLP-2 analog that reduces the need for parenteral support in patients with SBS. • Data about long-term therapy with teduglutide in children in real life are still scarce. What is New: • Most pediatric patients with SBS respond in a satisfactory manner to teduglutide treatment. The occurrence of long-term adverse effects is exceptional. • Starting treatment with the drug at a young age is associated with a greater response rate. [ABSTRACT FROM AUTHOR]

This article discusses a case report of a 14-year-old male with PERCC1-associated enteropathy, a rare disorder characterized by severe diarrhea and intestinal failure. The patient had low levels of enteroendocrine hormones, including glucagon-like peptide-2 (GLP-2), which plays a crucial role in intestinal function. The researchers hypothesized that GLP-2 deficiency could be treated with teduglutide, a synthetic analog of GLP-2. After starting teduglutide therapy, the patient experienced significant improvements in weight, stool frequency, and calorie intake, leading to the discontinuation of parenteral nutrition. This case suggests that teduglutide may be an effective treatment for PERCC1-associated enteropathy and other conditions characterized by enteroendocrine dysfunction. [Extracted from the article]

Background: Patients receiving home parenteral nutrition (HPN) are known to experience psychological distress and have profoundly disrupted sleep. The aim of this analysis was to examine the relationship between sleep patterns with depressive symptoms and HPN characteristics. Methods: The study was a secondary analysis of cross‐sectional data examining sleep patterns using subjective and objective measures. Sleep was assessed by surveys and 7‐day actigraphy. The Patient Health Questionnaire‐8 was used to evaluate depressive symptoms. Participants provided information on HPN. Spearman correlations were calculated between sleep measures with depressive symptoms and HPN characteristics. Correlations were further examined in multivariable linear regression models. Results: Thirty‐two adults (age = 53 years; 75% female; 94% White) were included. Lower sleep quality (r = 0.54–0.60; P < 0.001) and later sleep timing (r = −0.35; P = 0.049) were correlated with higher depressive symptoms. Sleep patterns were also correlated with several HPN characteristics (r = −0.47 to 0.51). In linear regression models, rate of infusion was associated with sleep duration (β = −0.004 [0.002] h; P = 0.046) in which each 100 mL/h was associated with 24‐min shorter duration. Higher total energy was associated with lower sleep quality (β = 0.0004 [0.0002] log‐unit; P = 0.042), and higher volume was associated with longer sleep onset latency (β = 0.0006 [0.0003] log‐min; P = 0.049). Conclusions: We provide evidence supporting the link between poor and later sleep with higher depressive symptoms and identify potentially modifiable infusion characteristics (notably, slower rate of infusion and lower total energy and volume) that, on further verification, may support sleep among those receiving HPN. [ABSTRACT FROM AUTHOR]

The Short Bowel Syndrome (SBS) Registry (NCT01990040) is a multinational real-world study evaluating the long-term safety of teduglutide in patients with SBS and intestinal failure (SBS-IF) in routine clinical practice. This paper describes the study methodology and baseline characteristics of adult patients who have (ever-treated) or have never (never-treated) received teduglutide. A total of 1411 adult patients (679 ever-treated; 732 never-treated) were enrolled at 124 sites across 17 countries. The mean (standard deviation [SD]) age at enrollment was 55.4 (15.46) years, and 60.2% of patients were women. Crohn's disease was the most common cause of major intestinal resection in both ever-treated (34.1%) and never-treated patients (20.4%). A similar proportion of ever-treated and never-treated patients had a prior history of colorectal polyps (2.7% vs. 3.6%), whereas proportionally fewer ever-treated patients reported a history of colorectal cancer (1.8% vs. 6.2%) or any malignancy (17.7% vs. 30.0%) than never-treated patients. Never-treated patients received a numerically greater mean (SD) volume of parenteral nutrition and/or intravenous fluids than ever-treated patients (12.4 [8.02] vs. 10.1 [6.64] L/week). Ever-treated patients received a mean teduglutide dosage of 0.05 mg/kg/day. This is the first report of patient baseline characteristics from the SBS Registry, and the largest cohort of patients with SBS-IF to date. Overall, ever-treated and never-treated patients had similar baseline characteristics. Differences between treatment groups may reflect variations in patient selection and degree of monitoring. [ABSTRACT FROM AUTHOR]

Purpose: This study aimed to investigate the impact of hepatocyte growth factor (HGF) on colonic morphology and gut microbiota in a rat model of short bowel syndrome (SBS). Methods: SD rats underwent jugular vein catheterization for total parenteral nutrition (TPN) and 90% small bowel resection [TPN + SBS (control group) or TPN + SBS + intravenous HGF (0.3 mg/kg/day, HGF group)]. Rats were harvested on day 7. Colonic morphology, gut microflora, tight junction, and Toll-like receptor-4 (TLR4) were evaluated. Results: No significant differences were observed in the colonic morphological assessment. No significant differences were observed in the expression of tight junction-related genes in the proximal colon. However, the claudin-1 expression tended to increase and the claudin-3 expression tended to decrease in the distal colon of the HGF group. The Verrucomicrobiota in the gut microflora of the colon tended to increase in the HGF group. The abundance of most LPS-producing microbiota was lower in the HGF group than in the control group. The gene expression of TLR4 was significantly downregulated in the distal colon of the HGF group. Conclusion: HGF may enhance the mucus barrier through the tight junctions or gut microbiome in the distal colon. [ABSTRACT FROM AUTHOR]

Malnutrition poses a critical challenge in inflammatory bowel disease, with the potential to detrimentally impact medical treatment, surgical outcomes, and general well-being. Parenteral nutrition is crucial in certain clinical scenarios, such as with patients suffering from short bowel syndrome, intestinal insufficiency, high-yielding gastrointestinal fistula, or complete small bowel obstruction, to effectively manage malnutrition. Nevertheless, research over the years has attempted to define the potential effects of parenteral nutrition on the intestinal barrier and the composition of the gut microbiota. In this narrative review, we have gathered and analyzed findings from both preclinical and clinical studies on this topic. Based on existing evidence, there is a clear correlation between short- and long-term parenteral nutrition and negative effects on the intestinal system. These include mucosal atrophic damage and immunological and neuroendocrine dysregulation, as well as alterations in gut barrier permeability and microbiota composition. However, the mechanistic role of these changes in inflammatory bowel disease remains unclear. Therefore, further research is necessary to effectively address the numerous gaps and unanswered questions pertaining to these issues. [ABSTRACT FROM AUTHOR]

Introduction: It is unknown how intestinal B cell populations and B cell receptor (BCR) repertoires are established and maintained over time in humans. Following intestinal transplantation (ITx), surveillance ileal mucosal biopsies provide a unique opportunity to map the dynamic establishment of recipient gut lymphocyte populations in immunosuppressed conditions. Methods: Using polychromatic flow cytometry that includes HLA allele groupspecific antibodies distinguishing donor from recipient cells along with high throughput BCR sequencing, we tracked the establishment of recipient B cell populations and BCR repertoire in the allograft mucosa of ITx recipients. Results: We confirm the early presence of naïve donor B cells in the circulation (donor age range: 1-14 years, median: 3 years) and, for the first time, document the establishment of recipient B cell populations, including B resident memory cells, in the intestinal allograft mucosa (recipient age range at the time of transplant: 1-44 years, median: 3 years). Recipient B cell repopulation of the allograft was most rapid in infant (<1 year old)-derived allografts and, unlike T cell repopulation, did not correlate with rejection rates. While recipient memory B cell populations were increased in graft mucosa compared to circulation, naïve recipient B cells remained detectable in the graft mucosa for years. Comparisons of peripheral and intra-mucosal B cell repertoires in the absence of rejection (recipient age range at the time of transplant: 1-9 years, median: 2 years) revealed increased BCR mutation rates and clonal expansion in graft mucosa compared to circulating B cells, but these parameters did not increase markedly after the first year post-transplant. Furthermore, clonal mixing between the allograft mucosa and the circulation was significantly greater in ITx recipients, even years after transplantation, than in deceased adult donors. In available pan-scope biopsies from pediatric recipients, we observed higher percentages of naïve recipient B cells in colon allograft compared to small bowel allograft and increased BCR overlap between native colon vs colon allograft compared to that between native colon vs ileum allograft in most cases, suggesting differential clonal distribution in large intestine vs small intestine. Discussion: Collectively, our data demonstrate intestinal mucosal B cell repertoire establishment from a circulating pool, a process that continues for years without evidence of stabilization of the mucosal B cell repertoire in pediatric ITx patients. [ABSTRACT FROM AUTHOR]

The article examines malnutrition in adults, detailing its definition as an imbalance between the growth and breakdown of body tissues and nutrient stores, leading to muscle loss, decreased physical and mental functioning, and impaired clinical outcomes. It also discusses the three main types of malnutrition: disease-related with inflammation, disease-related without inflammation, and starvation due to inadequate food access, along with the pathophysiology of energy.

Trichohepatoenteric syndrome (THES), also known as phenotypic diarrhea or syndromic diarrhea, is a rare autosomal recessive genetic disorder caused by mutations in SKIC2 (THES-type 2) or SKIC3 (THES-type 1) and is characterized by early onset diarrhea, woolly brittle hair, facial dysmorphic features and liver disease. We report the case of a 24-month-old girl who presented with chronic diarrhea since the neonatal period along with intrauterine growth restriction (IUGR), developmental delay, dysmorphic features, congenital heart defects, liver disease, and recurrent infections. The diagnosis was made through whole-exome sequencing analysis, which detected a homozygous variant (c.4070del, p.Pro1357Leufs*10) in the SKIC3 gene. The patient required parenteral nutrition and was hospitalized for the first 10 months of life and then discharged on PN after showing improvement. She remained stable on PN after discharge despite a few admissions for central line infections. Recent follow-up at the age of 2 years revealed that she was stable on long-term parenteral nutrition and that she had advanced chronic liver disease. [ABSTRACT FROM AUTHOR]

The role of long‐term parenteral support in patients with underlying benign conditions who do not have intestinal failure (IF) is contentious, not least since there are clear benefits in utilising the oral or enteral route for nutritional support. Furthermore, the risks of long‐term home parenteral nutrition (HPN) are significant, with significant impacts on morbidity and mortality. There has, however, been a recent upsurge of the use of HPN in patients with conditions such as gastro‐intestinal neuromuscular disorders, opioid bowel dysfunction, disorders of gut–brain interaction and possibly eating disorders, who do not have IF. As a result, the European Society of Clinical Nutrition and Metabolism (ESPEN), the European Society of Neuro‐gastroenterology and Motility (ESNM) and the Rome Foundation for Disorders of Gut Brain Interaction felt that a position statement is required to clarify – and hopefully reduce the potential for harm associated with – the use of long‐term parenteral support in patients without IF. Consensus opinion is that HPN should not be prescribed for patients without IF, where the oral and/or enteral route can be utilised. On the rare occasions that PN commencement is required to treat life‐threatening malnutrition in conditions such as those listed above, it should only be prescribed for a time‐limited period to achieve nutritional safety, while the wider multi‐disciplinary team focus on more appropriate biopsychosocial holistic and rehabilitative approaches to manage the patient's primary underlying condition. [ABSTRACT FROM AUTHOR]

Aim Methods Results Conclusion To investigate the clinical characteristics and course of parenteral nutrition‐associated cholestasis (PNAC) in very low birth weight (VLBW) infants.The charts of VLBW infants were retrospectively reviewed. The clinical characteristics of infants with and without PNAC were compared, trends in liver enzymes were investigated, and the characteristics of infants with PNAC were analysed based on age of onset.PNAC was observed in 53 (13.2%) of 403 infants who survived and completed follow‐up and was associated with significantly lower gestational age, birth weight, and adverse neonatal outcomes. PNAC started at a median 32 (interquartile range 23–47) days, PN was applied for 53 (34.5–64.5) days, the maximum direct bilirubin (DB) was observed at 63 (50–76) postnatal days, and PNAC resolved at 94 (79–122) postnatal days postnatal age. PNAC lasted 61 (38–89.5) days. AST and ALT normalised at 111 (100.3–142.0) and 109.5 (97–161.3) postnatal days. Infants with early‐onset PNAC had significantly longer PN duration, higher maximum DB, and higher maximum AST than those with late‐onset PNAC.Elevated DB, AST, and ALT persist for a long period after discontinuing PN. We suggest a cautious approach that involves waiting and reducing the frequency of additional repetitive examinations. [ABSTRACT FROM AUTHOR]

Objective Health care-associated infections (HAIs) are important causes of morbidity and mortality in neonatal intensive care units (NICUs). This study aimed to evaluate the frequency of HAIs and related factors in the NICU. Methods HAIs detected and monitored by daily active surveillance by the Infection Control Team at Necmettin Erbakan University Faculty of Medicine NICU between January 1, 2017 and December 31, 2022 were evaluated retrospectively. There were a total of 43 incubators in our hospital's NICU (19 secondary level units and 24 tertiary level). Between 2017 and 2022, there was an average of 982 admissions to the NICU per year. Results A total of 5,895 newborns and 74,726 patient days were monitored in the study. The average HAIs rate for all study years was 3.4% and the incidence density was 2.68 per thousand patient days. The highest HAI rate was in newborns with birth weights < 750 g. A total of 201 HAIs were detected in 172 patients. Bloodstream infection (BSI) was the most frequent HAI. The most frequent pathogens were Klebsiella spp. (44.8%), methicillin-resistant coagulase-negative staphylococci (CoNS) (24.4%), and Acinetobacter spp. (11.6%). Note that 88.5% of Enterobacterales were extended-spectrum beta-lactamase producers, and 26% of Klebsiella spp. were carbapenem-resistant. No colistin resistance was detected in Pseudomonas aeruginosa and Acinetobacter spp. Methicillin resistance was detected in 86.5% of CoNS and 50% of Staphylococcus aureus. The vancomycin resistance rate in Enterococcus spp. was 40%. Note that 16.7% of Candida spp. were fluconazole-resistant; no resistance to caspofungin was found. The most common risk factors for development of HAI were prematurity, umbilical catheter use, total parenteral nutrition, and mechanical ventilation. The mortality rate in patients with HAIs was 20.9%. Conclusion HAIs, including those caused by multidrug-resistant Gram-negative bacteria, are an important problem in our hospital, and also globally. Active surveillance should be continued, and changes over the years evaluated. Infection control programs should be executed by determining the risk and mortality factors attributed to infection and their implementation should be closely monitored. These practices will increase success in the fight against HAIs and antimicrobial resistance. [ABSTRACT FROM AUTHOR]

Aim: to assess the prescription of oral nutritional supplements (ONS) in the Northern Area of Gran Canaria in the period 2016-2021. Materials and methods: based on electronic prescription data, the first ONS prescription during 2016-2021 was analyzed considering age, gender, nutritional requirements (NR), body mass index (BmI), percentage of weight loss (%WL), albumin and number of prescribed ONS per patient. Results: 10,595 prescriptions were identified corresponding to 6661 patients with the following characteristics: 46.3 % men, mean age 72.84 ± 15.93 years, BMI 20.60 ± 3.98 kg/m², %WL 11.89 ± 8.32 %; albumin 3.08 ± 0.63 g/dl. The most frequent etiologies of DRE were: neoplasms 42.6 %; degenerative processes of the CNS 28.9 %; stroke 3.9 %; short bowel syndrome 6.9 %, and inflammatory bowel disease (IBD) 5.5 %. The percentages of NR covered by the prescribed ONS were: 100 % in 8.9 % of cases, 50 % in 36.9 %, and 25 % in 54.2 %; 40.4 % of patients received 1 unit of ONS daily, 36.3 % took 2 units of ONS, and 23 % received > 3 units of ONS per day. Greater NR were associated with a greater number of ONS (p < 0.001), but 40.8 % of patients who needed to cover > 50 % of NR received only one unit of ONS. Conclusion: a significant percentage of patients with disease-related malnutrition (DRM) do not receive a number of ONS according to their NR. [ABSTRACT FROM AUTHOR]

Background: Complex gastroschisis is a rare variant characterized by intrauterine closure of the abdominal defect, which can be accompanied by atresia, necrosis, and, in most cases, lead to short bowel syndrome. This is the first case of gastroschisis presenting as a whirl pattern of loops and raises suspicion of intestinal ischemia and atresia. Case Report: A newborn at 38 weeks of gestation with a prenatal diagnosis of complex gastroschisis presented with a whirl pattern of gastroschisis and jejunal atresia. The newborn was treated with staged closure of the defect and underwent a second surgical procedure for correction of jejunal atresia, with a successful outcome observed during 2 years of follow-up. Conclusion: Whirl pattern gastroschisis is a rare presentation of complex gastroschisis, and its diagnosis can be made prenatally, allowing for the preparation of medical and surgical management at birth. It is necessary to actively search for the presence of intestinal atresia or ischemia in this type of presentation. [ABSTRACT FROM AUTHOR]

Jejunocolic anastomosis (JCA) is a surgical procedure that can result in various complications. Depending on how much of the jejunum remains, these patients are often defined as having intestinal failure (IF) because of short bowel syndrome (SBS). One of the significant complications among these patients is the formation of calcium oxalate renal stones. It has been estimated that 24% of patients with a JCA develop symptomatic renal stones. This narrative review aims to explore the nursing and dietary management strategies for individuals with oxalate stones following JCA. This includes providing adequate hydration, limiting dietary intakes of fat and oxalate, and optimising dietary intake of fluid and calcium whilst discussing the evidence for dietary magnesium, vitamin C and citric acid. [ABSTRACT FROM AUTHOR]

Objective: To compare the phase angle (PhA) through bioelectrical impedance (BIA) of children with intestinal failure (IF) using prolonged parenteral nutrition (PN) followed by an Intestinal Rehabilitation Program, with a control group. Methods: Children under 10 years of age with IF using prolonged PN for >60 days (study group) were included. The control group consisted of healthy children without chronic pathologies, matched by sex and age. Anthropometric parameters evaluated were: weight, height, weight/age z-score (W/A), height/age z-score (H/A), BMI, BMI/A z-score, arm circumference, triceps skinfold, subscapular skinfold, mid-arm muscle circumference. BIA parameters were resistance (R), reactance (Xc), and phase angle (PhA). Results: Twenty-eight children were included in the study group, median (IQR) age was 11 (8–27) months, 53.6 % were male. In the control group, 28 children were included, median (IQR) age was 12.5 (8–24.7) months, 50 % were male. Children from the study group had W/A z-scores and H/A z-scores significantly lower than controls. There was no significant difference between PhA in the study group and controls, [median (IQR) 4.3° (3.8;4.6) vs 4.0° (3.8;5.4) respectively, p = 0.980]. Prematurity was significantly higher in the study group than in the controls, but there was no significant correlation between gestational age at birth and PhA of the children from the study group. Conclusion: Children with IF using prolonged PN showed lower W/A and H/A compared to the control group, but without significant difference between the PhA of children with IF compared to controls.

Background: Short bowel syndrome is a devastating malabsorptive condition and the most common cause of chronic intestinal failure (CIF). Patients need parenteral support for months or years. Ideally, it should be delivered at home, reducing limitations in everyday life activities. Summary: The Portuguese Health Directive 017/2020 was the first step in the regulation of home CIF management, and more patients are now being treated in an ambulatory setting. However, much work still needs to be performed in this area. Our country lacks a network of units capable of providing home parenteral nutrition (HPN), and only a few centers have expertise to take care of these complex patients: fluid support, oral, enteral, and parenteral nutrition; disease/HPN-related complications; pharmacologic treatment; and surgical prevention/treatment. Providing adequate transition from pediatric to adult care is a mandatory issue that should only be addressed by expert centers. Key Messages: Implementation of a national network, as well as the creation of an intestinal failure registry, with an initial focus on adult patients, will start a new era in the identification and management of these complex CIF patients.