Gastrointestinal Conditions: Malabsorption Syndromes.

Malabsorption syndromes are a heterogenous group of conditions that can cause distressing gastrointestinal symptoms. Celiac disease is most common and is triggered by exposure to gluten. Tissue transglutaminase immunoglobulin A is the diagnostic test of choice; management is gluten avoidance. Lactose intolerance is caused by absence or declining levels of the enzyme lactase. Diagnosis typically is clinical, but breath tests can be helpful if diagnosis is uncertain. Management is lactose avoidance. Bile acid malabsorption results in unabsorbed bile acids in the colon, leading to diarrhea. The 75selenium homotaurocholic acid test is most accurate but is not widely available. Therefore, a trial of bile acid sequestrants (typically cholestyramine) is a reasonable alternative when the diagnosis is suspected. Exocrine pancreatic insufficiency is caused by decreased production of pancreatic enzymes, typically occurring in patients with preexisting pancreatic damage from alcohol, surgery, radiation, diabetes, or cystic fibrosis. Diagnosis involves fecal fat or fecal elastase-1 tests. Management is pancreatic enzyme replacement. Small intestinal bacterial overgrowth is caused by pathologic overgrowth of the small bowel microbiome. Diagnosis is by jejunal biopsy or, more commonly, breath tests. Antibiotics (typically rifaximin) are the initial management. Other options include dietary changes, probiotics, and prokinetic drugs.

Incidence, Diagnosis and Management of Malabsorption Following Oesophagectomy: a Systematic Review.

BACKGROUND: Survival following oesophagectomy for cancer is improving, resulting in increased focus on quality of life and survivorship. Malabsorption syndrome is multifactorial and includes exocrine pancreatic insufficiency (EPI), small intestinal bacterial overgrowth (SIBO) and bile acid malabsorption (BAM). The aim of this study was to evaluate the reported incidence and management of malabsorption syndromes post-oesophagectomy. METHODS: A systematic search of PubMed, EMBASE, MEDLINE, Scopus and the Cochrane Library evaluating incidence, diagnosis and management of malabsorption was performed for studies published until December 2021. RESULTS: Of 464 identified studies, eight studies (n = 7 non-randomised longitudinal studies) were included where patients were identified with malnutrition following oesophagectomy. Studies included a combined sample of 328 (range 7-63) patients. Malabsorption syndromes including EPI, SIBO and BAM occurred in 15.9-100%, 37.8-100% and 3.33-100% over 21 days-60 months, 1-24 months and 1-24 months respectively. There was no consensus definition for EPI, SIBO or BAM, and there was variation in diagnostic methods. Diagnostic criteria varied from clinical (gastrointestinal symptoms or weight loss), or biochemical (faecal elastase, hydrogen breath test and Selenium-75-labelled synthetic bile acid measurements). Treatment modalities using pancreatic enzyme replacement, rifaximin or colesevelam showed improvement in symptoms and weight in all studies, where investigated. CONCLUSIONS: Malabsorption syndromes following oesophagectomy are under-recognised, and thus under-reported. The resultant gastrointestinal symptoms may have a negative effect on post-operative quality of life. Current literature suggests benefit with outlined therapies; however, greater understanding of these conditions, their diagnosis and management is required to further understand which patients will benefit from treatment.

Fructose malabsorption: causes, diagnosis and treatment.

This review intends to act as an overview of fructose malabsorption (FM) and its role in the aetiology of diseases including, but not limited to, irritable bowel syndrome (IBS) and infantile colic and the relationship between fructose absorption and the propagation of some cancers. IBS results in a variety of symptoms including stomach pains, cramps and bloating. Patients can be categorised into two groups, depending on whether the patients' experiences either constipation (IBS-C) or diarrhoea (IBS-D). FM has been proposed as a potential cause of IBS-D and other diseases, such as infantile colic. However, our knowledge of FM is limited by our understanding of the biochemistry related to the absorption of fructose in the small intestine and FM's relationship with small intestinal bacterial overgrowth. It is important to consider the dietary effects on FM and most importantly, the quantity of excess free fructose consumed. The diagnosis of FM is difficult and often requires indirect means that may result in false positives. Current treatments of FM include dietary intervention, such as low fermentable oligo-, di-, monosaccharides and polyols diets and enzymatic treatments, such as the use of xylose isomerase. More research is needed to accurately diagnose and effectively treat FM. This review is designed with the goal of providing a detailed outline of the issues regarding the causes, diagnosis and treatment of FM.

Urinary Diversion: Core Curriculum 2021.

Urinary diversion after cystectomy has been a historical standard for the treatment of numerous benign and malignant diseases of the bladder. Since the first published description in the early 1900s, improvements in surgical technique and a better understanding of the metabolic sequelae postoperatively have greatly enhanced patient outcomes. Both continent and incontinent diversions are available to patients after cystectomy. In appropriately selected patients, orthotopic neobladder reconstruction can offer preservation of body image and continence, and continent cutaneous diversions represent a reasonable alternative. Conduit diversion, which remains the most commonly performed diversion technique, is ideal for patients who would benefit from a less morbid surgical procedure that negates the need for self-catheterization. This installment of the Core Curriculum in Nephrology outlines numerous aspects of urinary diversion, in which a multidisciplinary approach to postoperative management at the intersection of nephrology and urology is required to effectively optimize patient outcomes. This article includes a discussion of the various reconstructive options after cystectomy as well as a comprehensive review of frequently encountered short-term and long-term metabolic abnormalities associated with altered electrolyte and acid-base homeostasis.

The role of histopathology in the diagnosis and management of coeliac disease and other malabsorptive conditions.

Most absorption of nutrients takes place in the proximal small intestine, and the most common disorders leading to malabsorption are associated with a morphological abnormality in the duodenal mucosa that is appreciable in histological sections of biopsy specimens. Coeliac disease is the most well-known example, causing intraepithelial lymphocytosis, inflammation and villous atrophy in the duodenum. Remarkably similar inflammatory changes can be induced by other processes, including medications, e.g. angiotensin II receptor blockers and immune checkpoint inhibitors, immune dysregulation disorders, e.g. common variable immunodeficiency and autoimmune enteropathy, infections, collagenous sprue, and tropical sprue. However, there are often subtle histological differences from coeliac disease in the type of inflammatory infiltrate, the presence of crypt apoptosis, and the extent and type of inflammation beyond the duodenum. The clinical setting and serological investigation usually allow diagnostic separation, but some cases remain challenging. Histopathology is also important in assessing the response to treatment, such as the change in villous architecture caused by a gluten-free diet, or the response to cessation of a potentially causative medication. This review examines the practical role that histopathology of duodenal biopsy specimens plays in the assessment and management of inflammatory malabsorptive processes of the proximal small intestine, with a particular emphasis on coeliac disease.

Challenges of Microvillus Inclusion Disease in the NICU.

Mutations in the myosin 5ß, syntaxin-binding protein 2, and syntaxin 3 genes lead to microvillus inclusion disease (MVID), an autosomal recessive congenital enteropathy. This rare disease is characterized by lack of microvilli on the surface of enterocytes in the small intestine, the presence of pathognomonic intracellular microvillus inclusions, and vesicular bodies within these enterocytes. This pathology leads to the characteristic intractable, life-threatening, watery diarrhea. In the more common early-onset form, affected patients present in the first few days after birth, whereas in the late-onset form, clinical manifestations appear at approximately 2 to 3 months of age. Genetic testing can confirm the diagnosis, but the infant's medical history, clinical presentation, and small intestinal biopsy results are strongly suggestive of the diagnosis. The prevalence of MVID is thought to be higher in countries with a high degree of consanguinity. Patients with MVID cannot tolerate feedings and require continuous total parenteral nutrition. Mortality is extremely high in the early-onset type with reports of survival in patients treated with small intestinal transplantation. Medical counseling for parents of infants with MVID needs to reflect our current understanding of the various genetic forms of this disease, the feasible management, and anticipated outcomes.

Improved residual fat malabsorption and growth in children with cystic fibrosis treated with a novel oral structured lipid supplement: A randomized controlled trial.

BACKGROUND: In the primary analysis of a 12-month double-blind randomized active placebo-controlled trial, treatment of children with cystic fibrosis (CF) and pancreatic insufficiency (PI) with a readily absorbable structured lipid (Encala™, Envara Health, Wayne, PA) was safe, well-tolerated and improved dietary fat absorption (stool coefficient of fat absorption [CFA]), growth, and plasma fatty acids (FA). OBJECTIVE: To determine if the Encala™ treatment effect varied by severity of baseline fat malabsorption. METHODS: Subjects (n = 66, 10.5±3.0 yrs, 39% female) with baseline CFA who completed a three-month treatment with Encala™ or a calorie and macronutrient-matched placebo were included in this subgroup analysis. Subjects were categorized by median baseline CFA: low CFA (<88%) and high CFA (≥88%). At baseline and 3-month evaluations, CFA (72-hour stool, weighed food record) and height (HAZ), weight (WAZ) and BMI (BMIZ) Z-scores were calculated. Fasting plasma fatty acid (FA) concentrations were also measured. RESULTS: Subjects in the low CFA subgroup had significantly improved CFA (+7.5±7.2%, mean 86.3±6.7, p = 0.002), and reduced stool fat loss (-5.7±7.2 g/24 hours) following three months of EncalaTM treatment. These subjects also had increased plasma linoleic acid (+20%), α-linolenic acid (+56%), and total FA (+20%) (p≤0.005 for all) concentrations and improvements in HAZ (0.06±0.08), WAZ (0.17±0.16), and BMIZ (0.20±0.25) (p≤0.002 for all). CFA and FA were unchanged with placebo in the low CFA group, with some WAZ increases (0.14±0.24, p = 0.02). High CFA subjects (both placebo and Encala™ groups) had improvements in WAZ and some FA. CONCLUSIONS: Subjects with CF, PI and more severe fat malabsorption experienced greater improvements in CFA, FA and growth after three months of Encala™ treatment. Encala™ was safe, well-tolerated and efficacious in patients with CF and PI with residual fat malabsorption and improved dietary energy absorption, weight gain and FA status in this at-risk group.

Use of a mobile application for self-management of pancreatic enzyme replacement therapy is associated with improved gastro-intestinal related quality of life in children with Cystic Fibrosis.

BACKGROUND: Most patients with cystic fibrosis (CF) suffer from pancreatic insufficiency (PI), leading to fat malabsorption, malnutrition, abdominal discomfort and impaired growth. Pancreatic enzyme replacement therapy (PERT) is effective, but evidence based guidelines for dose adjustment are lacking. A mobile app for self-management of PERT was developed in the context of the HORIZON 2020 project MyCyFAPP. It contains an algorithm to calculate individual PERT-doses for optimal fat digestion, based on in vitro and in vivo studies carried out in the same project. In addition, the app includes a symptoms diary, educational material, and it is linked to a web tool allowing health care professionals to evaluate patient's data and provide feedback. METHODS: A 6-month open label prospective multicenter interventional clinical trial was performed to assess effects of using the app on gastro-intestinal related quality of life (GI QOL), measured by the CF-PedsQL-GI (shortened, CF specific version of the Pediatric Quality of Life Inventory, Gastrointestinal Symptoms Module). RESULTS: One hundred and seventy-one patients with CF and PI between 2 and 18 years were recruited at 6 European CF centers. Self-reported CF-PedsQL-GI improved significantly from month 0 (M0) (84.3, 76.4-90.3) to month 6 (M6) (89.4, 80.35-93.5) (p< 0.0001). Similar improvements were reported by parents. Lower baseline CF-PedsQL-GI was associated with a greater improvement at M6 (p < 0.001). CONCLUSIONS: The results suggest that the MyCyFAPP may improve GI QOL for children with CF. This tool may help patients to improve self-management of PERT, especially those with considerable GI symptoms.

Bariatric surgery and type 2 diabetes.

Type 2 diabetes is primarily managed with lifestyle modifications, self-monitoring of blood glucose, and medication. The goal is to maintain A1C less than 7% in most patients and prevent damage to other organs such as the kidneys and heart. Patients who are obese and cannot achieve normal blood glucose levels despite diet, exercise, and multiple medications may be considered for bariatric surgery. The Roux-en-Y gastric bypass and laparoscopic sleeve gastrectomy have been shown to improve A1C, reduce weight, and reduce the number of medications patients need for diabetes management. Comorbidities such as hyperlipidemia and hypertension also may improve. This article describes types of bariatric surgery, proper selection of surgical candidates, patient education, and the postoperative patient management necessary for long-term success in improving blood glucose control.

Management of the Patient with Chronic Intestinal Pseudo-Obstruction and Intestinal Failure.

Chronic intestinal pseudo-obstruction (CIPO) is a severe form of intestinal dysmotility disorder, characterized by the impairment of gastrointestinal propulsion of the gut content in the absence of fixed occluding lesions. CIPO is a rare disease that can develop in both children and adults. CIPO is classified as primary/idiopathic, when no underlying disorder is demonstrated, or secondary, when related to systemic diseases. Diagnosis relies on the finding of chronic/recurrent obstructive type symptoms with radiological features of dilated intestine with air/fluid levels without any lumen occluding lesion. Therapy is based on nutrition, pharmacologic and surgical intervention and requires a multidisciplinary approach.

A Spanish Society joint SECO and SEEDO approach to the Post-operative management of the patients undergoing surgery for obesity.

PURPOSE: Bariatric surgery is the method of choice for the management or treatment of obesity. Bariatric surgery brings about several physiological changes in the body and is associated with set of complications. The aim of this study is to provide guidelines on post bariatric surgery management based on consensus by the Spanish society for Obesity Surgery (Sociedad Española de Cirugía de la Obesidad) (SECO) and the Spanish Society for the Study of Obesity (Sociedad Española para el Estudio de la Obesidad) (SEEDO). METHOD: The boards proposed seven experts from each society. The experts provided the evidence and a grade of recommendation on the selected topics based on systematic reviews/meta-analysis. A list of clinical practical recommendations levels of evidence and grades of these recommendations was derived from the consensus statements from the members of these societies. RESULTS: Seventeen topics related to post-operative management were reviewed after bariatric surgery. The experts came with 47 recommendations and statements. The mean number of persons voting at each statement was 54 (range 36-76). CONCLUSION: In this consensus, we have designed a set of guidelines to be followed while managing patients after bariatric surgery. Expertise and knowledge of the clinicians are required to convey suitable considerations to the post-bariatric patients. There should also be extensive follow-up plans for the bariatric surgery patients.

Development of venous thrombi in a pediatric population of intestinal failure.

BACKGROUND/PURPOSE: Although pediatric intestinal failure (IF) is now a survivable diagnosis, children are still at risk for complications. Loss of venous access persists as a leading indication for intestinal transplantation. The goal of this study was to identify risk factors for loss of venous access in a pediatric intestinal failure population on long-term PN. METHODS: We identified all patients who were PN dependent. RESULTS: Patients that developed venous thrombosis had significantly more lines placed in the first 2 years of life compared to those who did not develop thrombosis. Multivariate regression analysis revealed that diagnosis (NEC and gastroschisis) and parental education were significant predictors of venous thrombosis. CONCLUSION: By identifying potential risk factors for thrombus development, interventions can be developed to improve the overall outcome in pediatric IF patients. TYPE OF STUDY: Diagnostic LEVEL OF EVIDENCE: III.

Short article: Frequency, pathophysiology, and clinical classification of intestinal failure type II and III at a tertiary referral center.

OBJECTIVE: Intestinal failure (IF) is a complex disease that is gaining significance and attention throughout the world. In Mexico, there are no available data on this condition. The aim of this study was to determine the frequency and characteristics of patients with IF type II and III hospitalized at a tertiary referral center in our country. PATIENTS AND METHODS: A cross-sectional study was carried out from August 2016 to July 2017. Adult patients hospitalized in noncritical areas with a recent diagnosis of IF type II or III according to the European Society for Clinical Nutrition and Metabolism classification were included. Demographic, anthropometric, nutritional therapy, biochemical, and clinical characteristics were registered. Nutritional risk was determined by the Nutritional Risk Score 2002. RESULTS: During the study, 4144 patients were admitted to noncritical areas; 21 (5/1000) of these patients were included. The mean age of the patients was 51±18.8 years, and the mean BMI was 17.6±5.5 kg/m. Fifteen (71.5%) patients were diagnosed with IF type II and six (28.5%) with IF type III. The primary diagnosis was surgical complications in seven (33.3%) of the cases and the principal pathophysiological mechanism was short bowel syndrome in nine (42.8%) patients. The most frequent (37%) clinical classification was D2: more than 20 kcal/kg/day and 1001-2000 ml/day and parenteral nutrition and PN2: 1001-2000 ml/day (52.3%) based on the modified European Society for Clinical Nutrition and Metabolism clinical classification. CONCLUSION: In this study, a high frequency of IF was found; surgical complications and short bowel syndrome were the main mechanisms involved, and D2 was the most frequent clinical category.

Diagnosis and treatment of pancreatic exocrine insufficiency.

PURPOSE OF REVIEW: Pancreatic exocrine insufficiency (PEI), defined as a secretion of pancreatic enzymes and bicarbonate insufficient to maintain a normal digestion, is a frequent but frequently underdiagnosed and undertreated condition. PEI may be secondary to different pancreatic diseases and extrapancreatic conditions. Recent data support the high clinical relevance of PEI and its treatment. RECENT FINDINGS: Together with symptoms of maldigestion, PEI is associated with nutritional deficiencies leading to osteoporosis, low-trauma fractures, sarcopenia and increased mortality. No single widely available test allows to diagnose PEI accurately. Diagnosis of PEI requires the evaluation of symptoms, nutritional markers and a noninvasive pancreatic function test in the appropriate clinical context. Pancreatic enzyme replacement therapy (PERT) improves digestion, symptoms, nutritional status and quality of life of patients with PEI. In addition, PERT is associated with a longer survival in patients with unresectable pancreatic cancer and after surgery for pancreatic cancer or chronic pancreatitis. SUMMARY: Awareness of PEI in different clinical conditions is required. Nutritional advice and appropriate PERT are mandatory to reduce the morbidity and mortality associated with PEI. Further studies on the clinical impact of PEI and its treatment are needed, especially in diseases other than chronic pancreatitis and cystic fibrosis.

Is Parenteral Levothyroxine Therapy Safe in Intractable Hypothyroidism?

CASE: A 32-year old woman was admitted to the hospital due to intractable hypothyroidism refractory to high dose of oral l-thyroxine therapy. She underwent total thyroidectomy and radioactive iodine therapy due to papillary thyroid cancer. After excluding poor adherence to therapy and malabsorption, levothyroxine absorption test was performed. No response was detected. Transient neurologic symptoms developed during the test. She developed 3 attacks consisting of neurologic symptoms during high dose administration. The patient was considered a case of isolated l-thyroxine malabsorption. She became euthyroid after intramuscular twice weekly l-thyroxine therapy. DISCUSSION: There are a few case reports regarding isolated l-thyroxine. We report successful long term results of twice weekly administered intramuscular l-thyroxine therapy. We also draw attention to neurologic side effects of high dose l-thyroxine therapy.

Severe neurological complication following adjustable gastric banding.

AIM: In the last years with the increase of bariatric surgery, first of all as a result of new indications, a rise in the incidence of nutrient-related complications has been observed. Currently little is known about the impact of post-bariatric malnutrition and neurological complications. Wernicke's encephalopathy is a severe neurological syndrome which occurs as a result of thiamine deficiency. Wernicke-Korsakoff syndrome must be considered a serious neurological complication of bariatric surgery with significant morbidity and mortality, with rapidly progressing neurological symptoms, and must be treated immediately. CASE REPORT: We report the case of a 35 years-old male patient, affected by morbid obesity, anxious-depressive syndrome and alcohol use disorder, who after adjustable gastric banding implanted in another hospital developed a severe malnutrition and neurological syndrome. The patient showed poor adherence to the follow-up and to the dietary indications and after all, we needed to place a PEG for enteral nutrition in order to resolve the malnutrition condition and the neurological syndrome. Our experience emphasizes that preoperative selection and assessment of a patient's nutritional status according to guidelines, is required to identify potential problems, and that bariatric surgeons or physicians caring for patient who have undergone bariatric surgery should be familiar with the constellation of nutritional and neurological disorder that may occur after surgery. CONCLUSION: We want to remark the importance of preoperative selection of the patients, the follow-up and the cooperation between patient and physician in order to obtain the best result and avoid severe complications.

Hidden morbidity of ethanol lock therapy.

AIMS: Long-term central venous catheters are essential in sustaining growth and development in patients with intestinal failure (IF). Several strategies have been developed to prevent and treat catheter-related blood stream infections (CRBSIs), including ethanol lock therapy. We sought to evaluate the efficacy of ethanol lock therapy in our IF population. METHOD: This is a retrospective review of IF patients treated with ethanol lock therapy at a single institution from 2006 to 2013. We evaluated the number of catheter days, rate of CRBSI per 1000 catheter days, rate of central venous catheter (CVC) thrombosis, rate of CVC breakage, total number of CVC replacements, total number of hospital admissions, and total number days in the hospital. RESULT: We identified 19 patients who underwent ethanol lock therapy for CRBSI. There was no difference in CRBSI rate prior to (5.6 per 1000 catheter days) and after (7 per 1000 catheter days) initiation of ethanol lock therapy. The mean rate of thrombosis increased from 0 to 3 per 1000 catheter days with ethanol lock therapy (p < 0.05). In addition, the CVC breakage rate increased from 0 to 13.7 per 1000 catheter days with ethanol lock therapy (p < 0.001). Hospital admissions and catheter-related ER visits increased following the initiation of ethanol lock therapy. CONCLUSIONS: Contrary to other studies, there was no difference in CRBSI rate prior to and after initiation of ethanol lock therapy. Factors in the methodology of ethanol lock therapy may influence the effectiveness of infection prevention associated with ethanol lock therapy, as well as the rate of line breakage, line thrombosis, and the need for line replacement.

Effect of Measuring γ-Glutamyl Transpeptidase and Using Smoflipid in a Parenteral Nutrition Infusion in a Patient With Severe Malabsorption and Overlapping Primary Sclerosing Cholangitis and Ulcerative Colitis.

Studies discussing inflammation and oxidative stress state that these conditions are known contributors in the pathogenesis of cholestatic diseases and ulcerative colitis, and studies examining patients with liver disease have found decreased antioxidant status and significant elevation of lipid peroxides as compared with healthy subjects. One hypothesis in liver disease is that deficient antioxidant defense mechanisms may lead to excess oxygen free radical formation, which promotes deleterious processes in the liver. The role of oxidant agents in cells is complex and depends on the balance between oxidant and antioxidant particles, but there is 1 potential marker of oxidative stress that can be readily utilized for our patients who are receiving nutrition support: γ-glutamyl transpeptidase (GGT). GGT is thought to induce oxidative stress in the artery wall in the presence of free iron and is likely an indicator of a depleted supply of glutathione, especially in the liver, which can lead to a cascade of problems related to increased oxidative stress. One could consider giving these patients liposomal glutathione or the components that make up glutathione, such as glycine, glutamine, and N-acetyl-cysteine, but unfortunately total parenteral nutrition (TPN) in the United States contains no cysteine or glutamine. Another possible way would be to give additional antioxidants, such as selenium and zinc, as well as vitamins A, C, and E. In this case report, I demonstrate the potential effect that switching from a straight ω-6 fatty acid solution to a blended fatty acid solution had on liver function tests, specifically GGT, for a 66-year-old patient dependent on TPN for the prior 16 months.