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Background/Objectives: One of the rare causes of cholestasis may be hemolytic disease of the fetus and newborn (HDFN). Methods: We retrospectively analyzed 88 medical records of HDFN newborns with cholestasis and 186 records of children with HDFN without cholestasis and conducted an observational, case-control, retrospective study. Results: Factors influencing the risk of cholestasis were lower gestational age at birth (36.83 ± 1.9 vs. 37.57 ± 1.8, p = 0.002), Rh or Kidd HDFN (80.7% vs. 53.2%), and the need for intrauterine transfusion (27.3 vs. 11.8%). The subjects had lower hemoglobin concentrations at birth (14.01 ± 3.8 vs. 16.39 ± 2.8 g/dL) and during whole hospital stay, higher cord blood total bilirubin concentration (4.26 ± 1.8 vs. 2.39 ± 1.4 mg/dL), higher maximum bilirubin concentration (15.27 ± 5.8 vs. 10.24 ± 3.4 mg/dL), and more frequent liver ultrasound abnormalities (19.9 vs. 6.3%). They also required more extended hospitalization due to higher rates of postnatal blood transfusion (33 vs. 3.8%), more frequent need for exchange transfusion (8.8% vs. 2.2%), more extended time and higher risk of phototherapy (94.3 vs. 59.1%), and higher usage of immunoglobulins (55.7 vs. 8.1%), parenteral nutrition (45.5 vs. 12.9%), and antibiotics (14.8 vs. 4.8%). Conclusions: The risk factors for cholestasis in children with HDFN are lower gestational age at delivery, Rh and Kidd serological type of HDFN, and the need for intrauterine transfusions. [ABSTRACT FROM AUTHOR]

Background/Objectives: Crohn's disease is a chronic and debilitating intestinal disorder that alternates between remission and active flare-ups, often leading to hospitalization. Social support is known to enhance adaptation to the disease and modulate stress perception in patients, while stress may exacerbate symptoms. The aim of this study was to examine the roles of perceived stress and social support in Crohn's disease and their impact on the frequency of flare-ups. Methods: A cross-sectional observational study was conducted, assessing stress and social support in a cohort of 91 patients with Crohn's disease during flare-up and remission phases. The Perceived Stress Scale (PSS-14) and a Social Support Questionnaire were utilized for evaluation. We examined the relationship between stress and social support in Crohn's disease. The interaction between the variables studied was also observed, considering the stage of the disease. Finally, we carried out an analysis of the influence of these two variables on the development of flare-ups in Crohn's disease. Results: The study revealed that patients experience higher stress levels during flare-ups and that these levels are amplified by a lack of social support. A significant relationship was identified between the levels of social support and the occurrence of flare-ups, indicating that better social support is associated with fewer flare-ups. Conclusions: Patients with Crohn's disease in the flare-up phase are subject to considerable stress. A deficit in social support is linked to an increase in stress levels. The interaction between social support and stress plays a critical role in the development of flare-ups. [ABSTRACT FROM AUTHOR]

(1) Background: We present the first real-world-data study on teduglutide-treated SBS patients in the Slovak Republic and the first study to enable the comparison of the effects of teduglutide treatment between the adult and pediatric populations. (2) Methods: This was a non-interventional retrospective cohort study of adult and pediatric SBS patients treated with teduglutide. Primary and secondary endpoints were the results of teduglutide use at 12 weeks and 6 months after the initiation of treatment, compared to baseline. (3) Results: Teduglutide treatment led to a statistically significant reduction in the volume of intravenous hydration, HPN caloric intake, HPN and intravenous hydration applications per week and to increased urine output in adult patients. The results in the pediatric population were similar, but not statistically significant. A complete weaning off HPN was achieved in 57.14% of all patients (50.00% of children; 62.50% of adults) after a median of 0.99 years of teduglutide treatment (1.07 and 0.98 years for children and adults, respectively). (4) Conclusions: Teduglutide treatment in SBS patients leads to considerable reduction in or even weaning off PN in both pediatric and adult patients. [ABSTRACT FROM AUTHOR]

Hyperammonemia syndrome is a complication that has been reported to occur in 1–4% of lung transplant patients with mortality rates as high as 60–80%, making detection and management crucial components of post-transplant care. Patients are treated with a multimodal strategy that may include renal replacement therapy, bowel decontamination, supplementation of urea cycle intermediates, nitrogen scavengers, antibiotics against Mollicutes, protein restriction, and restriction of parenteral nutrition. In this review we provide a framework of pharmacologic mechanisms, medication doses, adverse effects, and available evidence for commonly used treatments to consider when initiating therapy. In the absence of evidence for individual strategies and conclusive knowledge of the causes of hyperammonemia syndrome, clinicians should continue to design multimodal regimens based on suspected etiologies, institutional drug availability, patient ability to tolerate enteral medications and nutrition, and availability of intravenous access. [ABSTRACT FROM AUTHOR]

This retrospective study aimed to investigate the impact of microorganisms identified in the reproductive tract on disorders during the early adaptation period in newborns. A cohort of 823 patients and cervical canal cultures were analyzed to identify the presence of microorganisms. Newborns included in the study were divided into two groups due to the number of pathogens identified in the swab from the cervical canal of the mother. The first group consisted of newborns whose mothers had one pathogen identified (N = 637), while the second group consisted of newborns whose mothers had two or more pathogens identified (N = 186). The analysis of disorders of the early adaptation period included the incidence of respiratory distress syndrome, the number of procedures performed with the use of CPAP, oxygen therapy, antibiotic therapy and parenteral nutrition. Respiratory distress syndrome was more common in group II than in group I (85 vs. 31, p = 0.001). In group II, CPAP (63 vs. 21, p = 0.001), oxygen therapy (15 vs. 8, p = 0.02) and antibiotics were used more frequently (13 vs. 8, p = 0.01). The findings of this study revealed that the number of pathogens colonizing the reproductive tract had a significant influence on the early adaptation period in newborns. Multifactorial colonization of the reproductive tract was associated with an increased incidence of infections in newborns and a higher prevalence of acid–base balance disorders. This study highlights the importance of monitoring and addressing the microbial composition of the reproductive tract during pregnancy. [ABSTRACT FROM AUTHOR]

(1) Background: We present the first real-world-data study on teduglutide-treated SBS patients in the Slovak Republic and the first study to enable the comparison of the effects of teduglutide treatment between the adult and pediatric populations. (2) Methods: This was a non-interventional retrospective cohort study of adult and pediatric SBS patients treated with teduglutide. Primary and secondary endpoints were the results of teduglutide use at 12 weeks and 6 months after the initiation of treatment, compared to baseline. (3) Results: Teduglutide treatment led to a statistically significant reduction in the volume of intravenous hydration, HPN caloric intake, HPN and intravenous hydration applications per week and to increased urine output in adult patients. The results in the pediatric population were similar, but not statistically significant. A complete weaning off HPN was achieved in 57.14% of all patients (50.00% of children; 62.50% of adults) after a median of 0.99 years of teduglutide treatment (1.07 and 0.98 years for children and adults, respectively). (4) Conclusions: Teduglutide treatment in SBS patients leads to considerable reduction in or even weaning off PN in both pediatric and adult patients.

Objectives: To investigate the clinical outcomes of ultrasound (US)-detected perforated necrotizing enterocolitis (NEC) without radiographic pneumoperitoneum in very preterm infants. Methods: In this single-center retrospective study, very preterm infants who underwent a laparotomy for perforated NEC during their neonatal intensive care unit stay were classified into two groups according to the absence or presence of pneumoperitoneum on radiographs (the case versus the control groups). The primary outcome was death before discharge, and the secondary outcomes included major morbidities and body weight at 36 weeks postmenstrual age (PMA). Results: Of the 57 infants with perforated NEC, 12 (21%) had no pneumoperitoneum on the radiographs and were diagnosed with perforated NEC on the US. In the multivariable analyses, the primary outcome of death before discharge was significantly lower in infants with perforated NEC without radiographic pneumoperitoneum than in those with perforated NEC and radiographic pneumoperitoneum (8% [1/12] vs. 44% [20/45]; adjusted odds ratio [OR], 0.02; 95% confidence interval [CI], 0.00–0.61; p = 0.025). The secondary outcomes (short bowel syndrome, total parenteral nutrition dependence for 3 months or more, the length of their hospital stay, a bowel stricture requiring surgery, sepsis after the laparotomy, acute kidney injury after the laparotomy, and body weight at 36 weeks PMA) did not differ significantly between the two groups. Conclusions: Very preterm infants with US-detected perforated NEC without radiographic pneumoperitoneum had a lower risk of death before discharge than those with perforated NEC and radiographic pneumoperitoneum. Bowel USs may have a potential role in surgical decision-making in infants with advanced NEC. [ABSTRACT FROM AUTHOR]

The incidences of colorectal cancer and inflammatory bowel diseases are increasing in the developed countries of Western Europe and North America, and consequently, the prevalence rate of temporary or permanent stomas has increased in recent years. Nevertheless, the amount of research in the nutrition field in the case of patients with stoma is rather limited. This review article aims to assess the impact of nutrition on an intestinal stoma and possible sequelae resolved with nutritional therapy. The research analyses conducted thus far indicate a significantly increased risk of developing malnutrition, vitamin deficiency (especially group B), and an increased number of discharges from the stoma and its relevance with abnormalities found in electrolyte concentrations. [ABSTRACT FROM AUTHOR]

Oropharyngeal and esophageal dysmotility can cause serious clinical complications such as aspiration pneumonia, cachexia, and sarcopenia, with a resulting increase in mortality and disability. The current standard of care for the treatment of SSc-associated swallowing dysfunction is mainly supportive, although severe cases are usually refractory to conventional management. Recent studies have shown that the abnormal production of functional autoantibodies such as anti-cholinergic muscarinic receptor III antibodies may participate in the pathogenesis of SSc-associated gastrointestinal dysmotility and may provide a novel target for therapeutic intervention. We describe two patients with severe and rapid onset of SSc-associated severe swallowing dysfunction and esophageal dysmotility who had failed standard of care therapy, requiring complete enteral and parenteral nutrition. Both patients were positive for the presence of circulating antimuscarinic III receptor antibodies. They were treated with IVIG at a dose of 2 g/Kg/month divided in two consecutive days, for six months. Following IVIG therapy, both patients markedly improved their symptoms as shown by a reduction in their UCLA2.0 score, and achieved an improvement of esophageal motility documented radiologically. Both patients resumed oral feeding and had their feeding tubes removed within the treatment period. None of the patients developed severe adverse events attributable to IVIG, except for low-grade fever during IVIG infusion in one of the cases. These results provide support for the role of functional autoantibodies in the development of SSc-associated gastrointestinal dysfunction. [ABSTRACT FROM AUTHOR]

Background: Small bowel perforations are a rare diagnosis compared with esophageal, gastric, and colonic perforations. However, small bowel perforations can be fatal if left untreated. A classification of small bowel perforations or treatment recommendations do not exist to date. Methods: A retrospective, monocentric, code-related data analysis of patients with small bowel perforations was performed for the period of 2010 to 2019. Results: Over a 10-year period, 267 cases of small bowel perforation in 257 patients (50.2% male and 49.8% female; mean age of 60.28 years) were documented. Perforation's localization was 5% duodenal, 38% jejunal, 39% ileal, and 18% undocumented. Eight etiologies were differentiated: iatrogenic (41.9%), ischemic (20.6%), malignant (18.9%), inflammatory (8.2%), diverticula-associated (4.5%), traumatic (4.5%), foreign-body-associated (1.9%), and cryptical (1.5%) perforations. Operative treatment combined with antibiotics was the most commonly used therapeutic approach (94.3%). The mortality rate was 14.23%, with highest rate for patients with ischemic perforations. Discussion: An algorithm for diagnostic and therapeutic steps was established. Furthermore, it was found that small bowel perforations are rare events with poor outcomes. Time to diagnosis and grade of underlying disease are the most essential parameters to predict perforation-associated complications. [ABSTRACT FROM AUTHOR]

Background: Small bowel perforations are a rare diagnosis compared with esophageal, gastric, and colonic perforations. However, small bowel perforations can be fatal if left untreated. A classification of small bowel perforations or treatment recommendations do not exist to date. Methods: A retrospective, monocentric, code-related data analysis of patients with small bowel perforations was performed for the period of 2010 to 2019. Results: Over a 10-year period, 267 cases of small bowel perforation in 257 patients (50.2% male and 49.8% female; mean age of 60.28 years) were documented. Perforation’s localization was 5% duodenal, 38% jejunal, 39% ileal, and 18% undocumented. Eight etiologies were differentiated: iatrogenic (41.9%), ischemic (20.6%), malignant (18.9%), inflammatory (8.2%), diverticula-associated (4.5%), traumatic (4.5%), foreign-body-associated (1.9%), and cryptical (1.5%) perforations. Operative treatment combined with antibiotics was the most commonly used therapeutic approach (94.3%). The mortality rate was 14.23%, with highest rate for patients with ischemic perforations. Discussion: An algorithm for diagnostic and therapeutic steps was established. Furthermore, it was found that small bowel perforations are rare events with poor outcomes. Time to diagnosis and grade of underlying disease are the most essential parameters to predict perforation-associated complications.

Several compounds based on short chain fatty acids and/or probiotics/prebiotics have shown promising results in the therapy of ulcerative colitis (UC), possibly due to its key role in restoring gut homeostasis as well as intestinal barrier integrity. Here, we investigated the efficacy of a patented preparation based on calcium butyrate, Bifidobacterium bifidum, Bifidobacterium lactis, and fructooligosaccharides (FEEDColon®, Princeps, Cuneo, Italy) in maintaining remission and improving subjective symptoms and inflammatory indices in patients with UC receiving 5-ASA therapy. A total of 42 patients were prospectively recruited and randomized in 21 patients receiving combination therapy with mesalamine (5-ASA) plus FEEDColon® and 21 patients treated with standard 5-ASA therapy. Patients were assessed at baseline, at 6-month, and 12-month follow-up (FU). Therapeutic success (defined as Mayo partial score ≤ 2 and faecal calprotectin (FC) < 250 µg/g at 12-month FU) was reached by 32 (76%) patients: 20 (95%) among those treated with 5-ASA + FeedColon®, and 12 (57%) among those treated with 5-ASA only (p = 0.009). Consistently, patients treated with combination therapy improved subjective symptoms (quality of life, abdominal pain, and stool consistency) and reduced FC values, while those treated with 5-ASA alone, improved neither subjective symptoms nor FC during the FU. In conclusion, FEEDColon® supplementation appears to be a valid add-on therapy for the maintenance of remission in patients with UC. Further multicentre, placebo-controlled, double-blind clinical trials are needed to validate our results on larger cohorts of patients with UC. [ABSTRACT FROM AUTHOR]

Malnutrition is a major contributor to muscle loss and muscle dysfunction, known as sarcopenia. Malnutrition is common in patients with inflammatory bowel disease (IBD). IBD includes ulcerative colitis (UC) and Crohn's disease (CD). The number of patients with IBD has recently been increasing. More severe malnutrition is often seen in CD compared to UC, probably due to CD affecting the main site of nutrient absorption, extensive mucosal lesions, fistulas, short bowel syndrome after resection, or obstruction of the gastrointestinal tract. A recent meta-analysis showed the high prevalence of sarcopenia in patients with IBD, and thus sarcopenia is a very important problem for IBD. Although IBD is more common in younger patients, sarcopenia can develop through a variety of mechanisms, including malnutrition, chronic inflammation, increased inflammatory status in adipose tissue, vitamin deficiency, and imbalance of the muscle–gut axis. In addition, sarcopenia has a negative impact on postoperative complications and hospital stay in patients with IBD. Appropriate intervention for sarcopenia may be important, in addition to clinical remission and endoscopic mucosal healing in patients with IBD. Much more attention will thus be paid to sarcopenia in patients with IBD. In this review, we outline IBD and sarcopenia, based on the current evidence. [ABSTRACT FROM AUTHOR]

In recent years, there has been a significant increase in the incidence of Crohn's disease. Despite significant medical progress, the treatment options available today do not meet the needs of all patients. Recent reports indicate that external environmental factors, including diet, are key in the pathomechanism of the disease. It was proven that the so-called Western dietary pattern is associated with an increased risk of disease. In the pediatric population, exclusive enteral nutrition is the only nutritional therapy option recommended today with proven high efficacy in inducing remission. Recent publications that indicate at least comparable efficacy and significantly better tolerability of a specialised elimination diet, the Crohn's Disease Exclusion Diet (CDED), provide the basis for a change in recommendations. This article discusses the mechanism of action, principles of use, and scientific evidence evaluating the efficacy of CDED in the treatment of children with Crohn's disease. [ABSTRACT FROM AUTHOR]

Background: Diarrhea is common among kidney transplant recipients (KTR). Exhaled hydrogen (H2) is a surrogate marker of small bowel dysbiosis, which may drive diarrhea. We studied the relationship between exhaled H2 and diarrhea in KTR, and explored potential clinical and dietary determinants. Methods: Clinical, laboratory, and dietary data were analyzed from 424 KTR participating in the TransplantLines Biobank and Cohort Study (NCT03272841). Fasting exhaled H2 concentration was measured using a model DP Quintron Gas Chromatograph. Diarrhea was defined as fast transit time (types 6 and 7 according to the Bristol Stool Form Scale, BSFS) of 3 or more episodes per day. We studied the association between exhaled H2 and diarrhea with multivariable logistic regression analysis, and explored potential determinants using linear regression. Results: KTR (55.4 ± 13.2 years, 60.8% male, mean eGFR 49.8 ± 19.1 mL/min/1.73 m2) had a median exhaled H2 of 11 (5.0–25.0) ppm. Signs of small intestinal bacterial overgrowth (exhaled H2 ≥ 20 ppm) were present in 31.6% of the KTR, and 33.0% had diarrhea. Exhaled H2 was associated with an increased risk of diarrhea (odds ratio 1.51, 95% confidence interval 1.07–2.14 per log2 ppm, p = 0.02). Polysaccharide intake was independently associated with higher H2 (std. β 0.24, p = 0.01), and a trend for an association with proton-pump inhibitor use was observed (std. β 0.16 p = 0.05). Conclusion: Higher exhaled H2 is associated with an increased risk of diarrhea in KTR. Our findings set the stage for further studies investigating the relationship between dietary factors, small bowel dysbiosis, and diarrhea after kidney transplantation. [ABSTRACT FROM AUTHOR]

Neuroendocrine neoplasms (NENs) are rare and indolent tumors characterized by the ability to synthesize, store, and secrete a variety of neuro-amines and peptides which can result in a secretory syndrome. 21306238 12 Camus B., Cottereau A.-S., Palmieri L.-J., Dermine S., Tenenbaum F., Brezault C., Coriat R. Indications of Peptide Receptor Radionuclide Therapy (PRRT) in Gastroenteropancreatic and Pulmonary Neuroendocrine Tumors: An Updated Review. 33803817 13 Palmieri L.-J., Dermine S., Barré A., Dhooge M., Brezault C., Cottereau A.-S., Coriat R. Medical Treatment of Advanced Pancreatic Neuroendocrine Neoplasms. NEN grouped Neuroendocrine tumors (NET) and Neuroendocrine Carcinoma (NEC). [Extracted from the article]

Myosin Vb (MYO5B) is a motor protein that facilitates protein trafficking and recycling in polarized cells by RAB11- and RAB8-dependent mechanisms. Biallelic MYO5B mutations are identified in the majority of patients with microvillus inclusion disease (MVID). MVID is an intractable diarrhea of infantile onset with characteristic histopathologic findings that requires life-long parenteral nutrition or intestinal transplantation. A large number of such patients eventually develop cholestatic liver disease. Bi-allelic MYO5B mutations are also identified in a subset of patients with predominant early-onset cholestatic liver disease. We present here the compilation of 114 patients with disease-causing MYO5B genotypes, including 44 novel patients as well as 35 novel MYO5B mutations, and an analysis of MYO5B mutations with regard to functional consequences. Our data support the concept that (1) a complete lack of MYO5B protein or early MYO5B truncation causes predominant intestinal disease (MYO5B-MVID), (2) the expression of full-length mutant MYO5B proteins with residual function causes predominant cholestatic liver disease (MYO5B-PFIC), and (3) the expression of mutant MYO5B proteins without residual function causes both intestinal and hepatic disease (MYO5B-MIXED). Genotype-phenotype data are deposited in the existing open MYO5B database in order to improve disease diagnosis, prognosis, and genetic counseling. [ABSTRACT FROM AUTHOR]

Diarrhea is a recurrent symptom in patients with neuroendocrine tumors (NETs) and can represent different etiologies; thus, differential diagnosis is challenging. This paper distinguishes the different causes of chronic diarrhea in patients with gastroenteropancreatic NETs, with the aim to identify the most appropriate therapeutic approach. Underlying causes of diarrhea can be multifactorial, including not only diarrhea that is related to specific hormonal hypersecretory syndromes, but also diarrhea that is secondary to the following: extensive surgery which can cause pancreatic exocrine insufficiency or short bowel syndrome, treatment with somatostatin analogs or other antineoplastic agents, and bile acid malabsorption. After initial management of diarrhea with general treatments (dietary modification, use of antidiarrheals), a proper differential diagnosis is necessary to treat patients with specific etiology-driven therapeutic approaches, such as somatostatin analogs, pancreatic enzyme replacement therapy, and tryptophan hydroxylase inhibitors. In conclusion, NETs should be considered in the differential diagnosis of patients suffering from chronic diarrhea, after the exclusion of more common etiologies. Furthermore, physicians should keep in mind that several different etiologies might be responsible for diarrhea occurrence in NET patients. A prompt diagnosis of the actual cause of diarrhea is necessary to guide the treatment and a multidisciplinary approach is mandatory. [ABSTRACT FROM AUTHOR]

Small-intestinal neuroendocrine tumors (SI-NETs) are the most prevalent small bowel neoplasms with an increasing frequency. In the multimodal management of SI-NETs, surgery plays a key role, either in curative intent, even if R0 resection is feasible in only 20% of patients due to advanced stage at diagnosis, or palliative intent. Surgeons must be informed about the specific surgical management of SI-NETs according to their hormonal secretion, their usual dissemination at the time of diagnosis and the need for bowel-preserving surgery to avoid short bowel syndrome. The aim of this paper is to review the surgical indications and techniques, and perioperative and postoperative management of SI-NETs. [ABSTRACT FROM AUTHOR]

Swallowing difficulties, also called dysphagia, can have various causes and may occur at many points in the swallowing process. The treatment and rehabilitation of dysphagia represent a major interdisciplinary and multiprofessional challenge. In dysphagic patients, dehydration is frequent and often accelerated as a result of limited fluid intake. This condition results from loss of water from the intracellular space, disturbing the normal levels of electrolytes and fluid interfering with metabolic processes and body functions. Dehydration is associated with increased morbidity and mortality rates. Dysphagic patients at risk of dehydration thus require close monitoring of their hydration state, and existing imbalances should be addressed quickly. This review gives an overview on dehydration, as well as its pathophysiology, risk factors, and clinical signs/symptoms in general. Available management strategies of dehydration are presented for oral, enteral, and parenteral fluid replacement. [ABSTRACT FROM AUTHOR]

Malnutrition is widespread in older people and represents a major geriatric syndrome with multifactorial etiology and severe consequences for health outcomes and quality of life. The aim of the present paper is to describe current approaches and evidence regarding malnutrition treatment and to highlight relevant knowledge gaps that need to be addressed. Recently published guidelines of the European Society for Clinical Nutrition and Metabolism (ESPEN) provide a summary of the available evidence and highlight the wide range of different measures that can be taken—from the identification and elimination of potential causes to enteral and parenteral nutrition—depending on the patient's abilities and needs. However, more than half of the recommendations therein are based on expert consensus because of a lack of evidence, and only three are concern patient-centred outcomes. Future research should further clarify the etiology of malnutrition and identify the most relevant causes in order to prevent malnutrition. Based on limited and partly conflicting evidence and the limitations of existing studies, it remains unclear which interventions are most effective in which patient groups, and if specific situations, diseases or etiologies of malnutrition require specific approaches. Patient-relevant outcomes such as functionality and quality of life need more attention, and research methodology should be harmonised to allow for the comparability of studies. [ABSTRACT FROM AUTHOR]

In critically ill children admitted to pediatric intensive care units (PICUs), enteral nutrition (EN) is often delayed due to gastrointestinal dysfunction or interrupted. Since a macronutrient deficit in these patients has been associated with adverse outcomes in observational studies, supplemental parenteral nutrition (PN) in PICUs has long been widely advised to meeting nutritional requirements. However, uncertainty of timing of initiation, optimal dose and composition of PN has led to a wide variation in previous guidelines and current clinical practices. The PEPaNIC (Early versus Late Parenteral Nutrition in the Pediatric ICU) randomized controlled trial recently showed that withholding PN in the first week in PICUs reduced incidence of new infections and accelerated recovery as compared with providing supplemental PN early (within 24 hours after PICU admission), irrespective of diagnosis, severity of illness, risk of malnutrition or age. The early withholding of amino acids in particular, which are powerful suppressors of intracellular quality control by autophagy, statistically explained this outcome benefit. Importantly, two years after PICU admission, not providing supplemental PN early in PICUs did not negatively affect mortality, growth or health status, and significantly improved neurocognitive development. These findings have an important impact on the recently issued guidelines for PN administration to critically ill children. In this review, we summarize the most recent literature that provides evidence on the implications for clinical practice with regard to the use of early supplemental PN in critically ill children. [ABSTRACT FROM AUTHOR]