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JPGN Reports May 2024Parents and pediatric patients with ulcerative colitis (UC) who progressed to systemic immunotherapy are concerned about lifelong risks from such treatments. There is...
OBJECTIVE
Parents and pediatric patients with ulcerative colitis (UC) who progressed to systemic immunotherapy are concerned about lifelong risks from such treatments. There is limited knowledge about withdrawal of such agents and step-down (SD) to enteral 5-aminosalicylic acid (mesalamine) before transitioning to adult care.
METHODS
We studied nine pediatric cases with moderate to severe UC who after a median of 2.18 years of clinical remission on systemic immunotherapy stepped down to oral mesalamine treatment.
RESULTS
Average follow-up time from SD was 3.49 years. Five patients (55.5%) had sustained remission (without any flare noted) after SD during follow-up. Sustained clinical remission was 88.9% (8/9) at 1 year, 87.5% (7/8) at 2 years, and 66.7% (4/6) at 3 years after SD. Out of those tested (one patient was not tested), 62.5% (5/8) had fecal calprotectin <50 μg/g. Four out of six patients examined (66.6%) had mucosal healing on post-SD colonoscopy.
CONCLUSION
We propose that SD to mesalamine can be a reasonable therapeutic consideration for pediatric patients with UC before transitioning to adult gastroenterology care. Shared decision-making is important before such treatment changes.
PubMed: 38756120
DOI: 10.1002/jpr3.12048 -
BMJ Open Respiratory Research May 2024Early enteral nutrition (EN) in critically ill adult patients is thought to improve mortality and morbidity; expert guidelines recommend early initiation of EN in...
INTRODUCTION
Early enteral nutrition (EN) in critically ill adult patients is thought to improve mortality and morbidity; expert guidelines recommend early initiation of EN in critically ill adults. However, the ideal schedule and dose of EN remain understudied.
STUDY OBJECTIVE
Our objective was to evaluate the relationship between achieving 70% of recommended EN within 2 days of intubation ('early goal EN') and clinical outcomes in mechanically ventilated medically critically ill adults. We hypothesised that early goal EN would be associated with reduced in-hospital death.
METHODS
We conducted a retrospective cohort study of mechanically ventilated adult patients admitted to our medical intensive care unit during 2013-2019. We assessed the proportion of recommended total EN provided to the patient each day following intubation until extubation, death or 7 days whichever was shortest. Patients who received 70% or more of their recommended total daily EN within 2 days of intubation (ie, 'baseline period') were considered to have achieved 'early goal EN'; these patients were compared with patients who did not ('low EN'). The primary outcome was in-hospital death; secondary outcomes were successful extubation and discharge alive.
RESULTS
938 patients met eligibility criteria and survived the baseline period. During the 7-day postintubation period, 64% of all patients reached 70% of recommended daily calories; 33% of patients achieved early goal EN. In unadjusted and adjusted models, early goal EN versus low EN was associated with a lower incidence of in-hospital death (subdistribution HR (SHR) unadjusted=0.63, p=0.0003, SHR adjusted=0.73, p=0.02). Early goal EN was also associated with a higher incidence of successful extubation (SHR unadjusted=1.41, p<0.00001, SHR adjusted=1.27, p=0.002) and discharge alive (SHR unadjusted=1.54, p<0.00001, SHR adjusted=1.24, p=0.02).
CONCLUSIONS
Early goal EN was associated with significant improvement in clinical metrics of decreased in-hospital death, increased extubation and increased hospital discharge alive.
Topics: Humans; Retrospective Studies; Critical Illness; Male; Enteral Nutrition; Respiration, Artificial; Female; Middle Aged; Hospital Mortality; Aged; Intensive Care Units; Time Factors
PubMed: 38749534
DOI: 10.1136/bmjresp-2023-001962 -
Frontiers in Pediatrics 2024Brown-Vialetto-Van Laere (BVVL) syndrome is an extremely rare autosomal recessive progressive motoneuron disease that is caused by a defect in the riboflavin transporter...
BACKGROUND
Brown-Vialetto-Van Laere (BVVL) syndrome is an extremely rare autosomal recessive progressive motoneuron disease that is caused by a defect in the riboflavin transporter genes SLC52A2 and SLC52A3. BVVL syndrome has a variable age of presentation, and it is characterized by progressive auditory neuropathy, bulbar palsy, stridor, muscle weakness, and respiratory compromise secondary to diaphragmatic and vocal cord paralysis. BVVL syndrome has a poor prognosis in the absence of treatment, including morbidity with quadriparesis and sensorineural hearing loss, with mortality in the younger age group. Early administration of riboflavin is associated with prolonged survival, low morbidity, and reversal of some clinical manifestations.
CASE PRESENTATION
We describe an 18-month-old male infant with progressive pontobulbar palsy, loss of developmental milestones, and a clinical picture suggestive of chronic inflammatory demyelinating neuropathy. A nerve conduction study revealed axonal neuropathy, while molecular analysis revealed a homozygous mutation in one of the riboflavin transporter genes, SLC52A3, confirming BVVL syndrome. The patient needed long-term respiratory support and a gastrostomy tube to support feeding. With high-dose riboflavin supplementation, he experienced moderate recovery of motor function.
CONCLUSION
This report highlights the importance of considering BVVL syndrome in any patient who presents with the clinical phenotype of pontobulbar palsy and peripheral axonal neuropathy, as early riboflavin treatment may improve or halt disease progression, thus reducing the associated mortality and morbidity.
PubMed: 38745833
DOI: 10.3389/fped.2024.1377515 -
Journal of Translational Medicine May 2024Interstitial lung disease (ILD) represents a significant complication of rheumatoid arthritis (RA) that lacks effective treatment options. This study aimed to...
BACKGROUND AND PURPOSE
Interstitial lung disease (ILD) represents a significant complication of rheumatoid arthritis (RA) that lacks effective treatment options. This study aimed to investigate the intrinsic mechanism by which resveratrol attenuates rheumatoid arthritis complicated with interstitial lung disease through the AKT/TMEM175 pathway.
METHODS
We established an arthritis model by combining chicken type II collagen and complete Freund's adjuvant. Resveratrol treatment was administered via tube feeding for 10 days. Pathological changes in both the joints and lungs were evaluated using HE and Masson staining techniques. Protein expression of TGF-β1, AKT, and TMEM175 was examined in lung tissue. MRC-5 cells were stimulated using IL-1β in combination with TGF-β1 as an in vitro model of RA-ILD, and agonists of AKT, metabolic inhibitors, and SiRNA of TMEM175 were used to explore the regulation and mechanism of action of resveratrol RA-ILD.
RESULTS
Resveratrol mitigates fibrosis in rheumatoid arthritis-associated interstitial lung disease and reduces oxidative stress and inflammation in RA-ILD. Furthermore, resveratrol restored cellular autophagy. When combined with the in vitro model, it was further demonstrated that resveratrol could suppress TGF-β1 expression, and reduce AKT metamorphic activation, consequently inhibiting the opening of AKT/MEM175 ion channels. This, in turn, lowers lysosomal pH and enhances the fusion of autophagosomes with lysosomes, ultimately ameliorating the progression of RA-ILD.
CONCLUSION
In this study, we demonstrated that resveratrol restores autophagic flux through the AKT/MEM175 pathway to attenuate inflammation as well as fibrosis in RA-ILD by combining in vivo and in vitro experiments. It further provides a theoretical basis for the selection of therapeutic targets for RA-ILD.
Topics: Resveratrol; Arthritis, Rheumatoid; Proto-Oncogene Proteins c-akt; Animals; Lung Diseases, Interstitial; Humans; Inflammation; Signal Transduction; Fibrosis; Transforming Growth Factor beta1; Membrane Proteins; Autophagy; Oxidative Stress; Cell Line; Lung; Male
PubMed: 38745204
DOI: 10.1186/s12967-024-05228-1 -
Frontiers in Nutrition 2024Emery-Dreifuss muscular dystrophy (EDMD) is a rare, inherited human disease. Similar to other neuromuscular dystrophies, EDMD is clinically characterized by muscle...
Emery-Dreifuss muscular dystrophy (EDMD) is a rare, inherited human disease. Similar to other neuromuscular dystrophies, EDMD is clinically characterized by muscle atrophy and weakness, multi-joint contractures with spine rigidity, and cardiomyopathy. Over time, muscular weakness can lead to dysphagia and a severe lowering of body mass index (BMI), worsening the prognosis. We present the case of a young male patient affected by EDMD, admitted to the hospital for pneumothorax in a severe state of undernourishment. The patient was treated with total parenteral nutrition (TPN) with Smofkabiven®, supplemented with micronutrients (vitamins and trace elements), and with minimal enteral nutrition through food. Within a year, the patient gained 8.5 kg and kept his body weight stable for the 6 years of the follow-up. In this study, we show that TPN ensures the nutritional requirements of EDMD patients in a safe and well-tolerated manner, allowing a considerable and stable improvement in nutritional status, which has a positive impact on the disease itself and the patients' quality of life.
PubMed: 38742022
DOI: 10.3389/fnut.2024.1343548 -
BMJ Open May 2024Gastrointestinal hospitalisations in the USA cause over US$130 billion in expenditures, and acute pancreatitis is a leading cause of these hospitalisations. Adequate...
INTRODUCTION
Gastrointestinal hospitalisations in the USA cause over US$130 billion in expenditures, and acute pancreatitis is a leading cause of these hospitalisations. Adequate pain control is one of the primary treatment goals for acute pancreatitis. Though opioids are commonly used for analgesia in these patients, there have been concerns about short-term and long-term side effects of using opioids. Recently, non-opioid medications have been studied to treat pain in patients with acute pancreatitis. This systematic review and network meta-analysis aims to assess the comparative efficacy of analgesic medication for non-severe, acute pancreatitis.
METHODS AND ANALYSIS
We will search multiple electronic databases for randomised controlled trials that study pain management in patients with non-severe, acute pancreatitis. The intervention will be any analgesic for acute pancreatitis in the hospital setting. The comparison group will be patients who received a placebo or other active interventions for pain management. The primary outcomes of interest include pain scores and the need for supplementary analgesia. The secondary outcomes will be serious adverse events, local complications, progression to severe pancreatitis, transfer to the intensive care unit, length of hospitalisation, time to start enteral feeds, 30-day all-cause mortality and Quality of Life Scale scores. If sufficient homogeneity exists among included studies, the findings will be pooled using a traditional pairwise and network meta-analysis. The risk of bias in randomised control trials will be evaluated using the Cochrane Risk of Bias Tool 2.0. The Grading of Recommendations, Assessment, Development, and Evaluation approach will be used to report the certainty of evidence.
ETHICS AND DISSEMINATION
This systematic review will not involve direct contact with human subjects. The findings of this review will be published in a peer-reviewed journal. They will give healthcare providers a better awareness of the optimal analgesic medication for pain treatment in non-severe, acute pancreatitis.
Topics: Humans; Systematic Reviews as Topic; Pancreatitis; Network Meta-Analysis; Pain Management; Analgesics; Research Design; Acute Disease; Analgesia; Randomized Controlled Trials as Topic; Analgesics, Opioid
PubMed: 38740508
DOI: 10.1136/bmjopen-2023-081971 -
Journal of Thoracic Disease Apr 2024Since the late 1990s, and Henrik Kehlet's hypothesis that a reduction of the body's stress response to major surgeries could decrease postoperative morbidity, "Enhanced... (Review)
Review
Since the late 1990s, and Henrik Kehlet's hypothesis that a reduction of the body's stress response to major surgeries could decrease postoperative morbidity, "Enhanced Recovery After Surgery" (ERAS) care pathways have been streamlined. They are now well accepted and considered standard in many surgical disciplines. Yet, to this day, there is no specific ERAS protocol for chest wall resections (CWRs), the removal of a full-thickness portion of the chest wall, including muscle, bone and possibly skin. This is most unfortunate because these are high-risk surgeries, which carry high morbidity rates. In this review, we propose an overview of the current key elements of the ERAS guidelines for thoracic surgery that might apply to CWRs. A successful ERAS pathway for CWR patients would entail, as is the standard approach, three parts: pre-, peri- and postoperative elements. Preoperative items would include specific information, targeted patient education, involvement of all members of the team, including the plastic surgeons, smoking cessation, dedicated nutrition and carbohydrate loading. Perioperative items would likely be standard for thoracotomy patients, namely carefully selective pre-anesthesia sedative medication only in some rare instances, low-molecular-weight heparin throughout, antibiotic prophylaxis, minimization of postoperative nausea and vomiting, avoidance of fluid overload and of urinary drainage. Postoperative elements would include early mobilization and feeding, swift discontinuation of intravenous fluid supply and chest tube removal as soon as safe. Optimal pain management throughout also appears to be critical to minimize the risk of respiratory complications. Together, all these items are achievable and may hold the key to successful introduction of ERAS pathways to the benefit of CWR patients.
PubMed: 38738262
DOI: 10.21037/jtd-23-911 -
Journal of Pediatric Surgery Apr 2024Gastrostomy insertion is one of the most frequently performed procedures by specialist paediatric surgeons. We aimed to determine practice across the United Kingdom (UK)...
BACKGROUND
Gastrostomy insertion is one of the most frequently performed procedures by specialist paediatric surgeons. We aimed to determine practice across the United Kingdom (UK) and in particular to identify areas where there was consistency or variation in practice between practitioners and centres.
METHODS
A structured survey was distributed to all consultant practitioners who insert gastrostomies in the UK. Practice surrounding a range of aspects of gastrostomy care including insertion technique, device use, post-operative management and subsequent care were determined.
RESULTS
Of total 135 practitioners who insert gastrostomies, responses were received from 103 (76%) with responses received from all UK centres. There was variation between centres in the provision of pre-operative information, and between practitioners in preferred device, insertion techniques, post-operative feeding practice and change/removal procedures. The most frequently preferred device for primary gastrostomy insertion was a Freka® PEG (36%) button device (30%), CorFlo™ PEG (21%), or G-tube (10%). Laparoscopy was always used when inserting either PEG or button device by over 50% of respondents and selectively used by the majority of the remainder. Feeds were started between 1 and 24 h post-insertion, most practitioners (64%) plan a minimum one night hospital stay but a third plan for more than one night.
CONCLUSION
There is considerable variation in practice for most stages of the pathway for children having a gastrostomy. Further work is warranted to understand the relationship between different practices and patient outcomes, resource use and cost and subsequently to develop best practice guidelines.
PubMed: 38735807
DOI: 10.1016/j.jpedsurg.2024.04.013 -
The American Journal of Clinical... May 2024Congenital diarrheas and enteropathies (CODE) are a group of rare, heterogenous, monogenic disorders that lead to chronic diarrhea in infancy. Definitive treatment is... (Review)
Review
Congenital diarrheas and enteropathies (CODE) are a group of rare, heterogenous, monogenic disorders that lead to chronic diarrhea in infancy. Definitive treatment is rarely available, and supportive treatment is the mainstay. Nutritional management in the form of either specialized formulas, restrictive diet, or parenteral nutrition support in CODE with poor enteral tolerance, is the cornerstone of CODE treatment and long-term growth. The evidence to support the use of specific diet regimens and nutritional approaches in most CODE disorders is limited due to the rarity of those diseases and the scant published clinical experience. The goal of this review is to create a comprehensive guide for nutritional management in CODE, based on the currently available literature, disease mechanism and the PediCODE group experience. Enteral diet management in CODE can be divided into 3 distinct conceptual frameworks - nutrient elimination, nutrient supplementation, and generalized nutrient restriction. Response to nutrient elimination or supplementation can lead to resolution or significant improvement in the chronic diarrhea of CODE and resumption of normal growth. This pattern can be seen in CODE due to carbohydrate malabsorption, defects in fat absorption and occasionally in electrolyte transport defects. In contrast, general diet restriction is mainly supportive. However, occasionally it allows parenteral nutrition weaning or reduction overtime mainly in enteroendocrine defects and rarely in epithelial trafficking and polarity defects. Further research is required to better elucidate the role of diet in the treatment of CODE and the appropriate diet management for each disease.
PubMed: 38734141
DOI: 10.1016/j.ajcnut.2024.05.004 -
Nutrients May 2024The purpose of this study was to evaluate the efficacy and safety of intragastric administration of small volumes of sodium enema solution containing phosphorus as...
The purpose of this study was to evaluate the efficacy and safety of intragastric administration of small volumes of sodium enema solution containing phosphorus as phosphorus replacement therapy in critically ill patients with traumatic injuries who required continuous enteral nutrition. Adult patients (>17 years of age) who had a serum phosphorus concentration <3 mg/dL (0.97 mmol/L) were evaluated. Patients with a serum creatinine concentration >1.4 mg/dL (124 µmol/L) were excluded. Patients were given 20 mL of saline enema solution intragastrically, containing 34 mmol of phosphorus and mixed in 240 mL water. A total of 55% and 73% of patients who received one ( = 22) or two doses ( = 11) had an improvement in the serum phosphorus concentration, respectively. The serum phosphorus concentration increased from 2.5 [2.1, 2.8] mg/dL (0.81 [0.69, 0.90] mmol/L) to 2.9 [2.2, 3.0] mg/dL (0.94 [0.71, 0.97 mmol/L) for those who received two doses ( = 0.222). Excluding two patients with a marked decline in serum phosphorus by 1.3 mg/dL (0.32 mmol/L) resulted in an increase in the serum phosphorus concentration from 2.3 [2.0, 2.8] mg/dL (0.74 [0.65, 0.90] mmol/L) to 2.9 [2.5, 3.2] mg/dL (0.94 [0.81, 1.03] mmol/L; = 9; = 0.012). No significant adverse effects were noted. Our data indicated that intragastric phosphate administration using a small volume of saline enema solution improved the serum phosphorus concentrations in most patients.
Topics: Humans; Phosphates; Male; Female; Adult; Phosphorus; Enteral Nutrition; Middle Aged; Critical Illness; Enema; Aged; Treatment Outcome
PubMed: 38732640
DOI: 10.3390/nu16091394