Malabsorptive States Research Articles

The Use of Pancreatic Exocrine Insufficiency-Questionnaire (PEI-Q)

Chronic pancreatitis is a non-reversible, progressive inflammatory disease of the pancreas that is caused by repeated inflammation. It is characterized by morphological changes in the parenchyma and main pancreatic duct as pancreatic tissue is damaged and fibrosis progresses. These changes can lead to exocrine and endocrine dysfunction in patients with chronic pancreatitis, with major complications including pain, endocrine or exocrine insufficiency, and increased risk of pancreatic cancer. Of these, pancreatic exocrine insufficiency (PEI) is a state of malabsorption in which more than 15 g of fat is excreted per day. It can be diagnosed and assessed using several methods, but these methods are currently difficult to use in Korea and have low compliance, making them low utility. Recently, the PEI-questionnaire (PEI-Q) was developed to diagnose and assess patients’ exocrine insufficiency. It has been validated in foreign countries and has the potential to be used as a tool for diagnosing exocrine insufficiency with simple questions. This paper will review the diagnostic methods for existing exocrine insufficiency and the usefulness and utilization of the PEI-Q.

An Overview of Short-Bowel Syndrome in Pediatric Patients: Focus on Clinical Management and Prevention of Complications.

Short-bowel syndrome (SBS) in pediatric age is defined as a malabsorptive state, resulting from congenital malformations, significant small intestine surgical resection or disease-associated loss of absorption. SBS is the leading cause of intestinal failure in children and the underlying cause in 50% of patients on home parental nutrition. It is a life-altering and life-threatening disease due to the inability of the residual intestinal function to maintain nutritional homeostasis of protein, fluid, electrolyte or micronutrient without parenteral or enteral supplementation. The use of parenteral nutrition (PN) has improved medical care in SBS, decreasing mortality and improving the overall prognosis. However, the long-term use of PN is associated with the incidence of many complications, including liver disease and catheter-associated malfunction and bloodstream infections (CRBSIs). This manuscript is a narrative review of the current available evidence on the management of SBS in the pediatric population, focusing on prognostic factors and outcome. The literature review showed that in recent years, the standardization of management has demonstrated to improve the quality of life in these complex patients. Moreover, the development of knowledge in clinical practice has led to a reduction in mortality and morbidity. Diagnostic and therapeutic decisions should be made by a multidisciplinary team that includes neonatologists, pediatric surgeons, gastroenterologists, pediatricians, nutritionists and nurses. A significant improvement in prognosis can occur through the careful monitoring of nutritional status, avoiding dependence on PN and favoring an early introduction of enteral nutrition, and through the prevention, diagnosis and aggressive treatment of CRSBIs and SIBO. Multicenter initiatives, such as research consortium or data registries, are mandatory in order to personalize the management of these patients, improve their quality of life and reduce the cost of care.

Levothyroxine Absorption Test - Results and Follow Up of Patients from a Single Center.

Treating primary hypothyroidism with appropriate weight-based dosing is usually straightforward. When supraphysiologic doses of thyroxine are required, it is important to know the reason behind the increased requirement. When compliance with medication is assured and when there are no drug interactions resulting in increased thyroxine requirements, a levothyroxine absorption test (LT4AT) could be performed to diagnose the coexistence of malabsorption. This was a single-center, retrospective chart review. Patients who had undergone LT4AT between 2018 and 2020 with a complete evaluation and follow-up records were reviewed. LT4AT and further evaluation (if necessary) were standardized, and details until the last review were obtained. In all, 11 patients had complete records and follow-up details. The median age (range) of the cohort was 24 years (19 to 46), and the duration of hypothyroidism was 8 years (4 to 24). Seven had low absorption (<60%) in LT4AT of which two each had celiac disease and Helicobacter pylori infection; one had autoimmune gastritis, and in two the reasons of malabsorption was not known. The median follow-up duration was 13 months. Nine of 11 (81%) achieved euthyroid state with/without thyroxine dose reduction on follow-up. A high proportion of patients requiring supraphysiological doses of levothyroxine had occult malabsorptive states, some of which were curable. Even in those with pseudo-malabsorption, LT4AT helped in reinforcing compliance and thereby thyroxine dose reduction.

Undernutrition was a prevalent clinical problem among older adult patients with heart failure in a hospital setting in Northwest Ethiopia.

Undernutrition is a frequently noticed medical problem in patients with heart failure. It is caused by poor nutrient intake, malabsorption, systemic inflammation, neurohumoral activation, oxidative stress, and hypermetabolic state. Undernutrition results in a decrease in the quality of life and the survival rate in patients with heart failure. There is a paucity of documentation on undernutrition among patients with heart failure in sub-Saharan African countries. The study aimed to determine the magnitude and associated factors of undernutrition among older adult patients with heart failure in the hospital setting in Northwest Ethiopia. An institutional-based cross-sectional study was conducted at the University of Gondar Hospital, Northwest Ethiopia, between 1 June 2021 and 31 October 2021. A consecutive sampling method was used to recruit 262 study subjects. A Mini-nutritional assessment-full form (MNA-FL) Questionnaire was used to extract nutritional information among patients with heart failure. Patients with heart failure, who scored MNA-FL score <17, were declared to have undernutrition. The data were entered into EPI Info version 4.6.0.0 and then exported to SPSS version 26 for analysis. Explanatory variables associated with undernutrition in patients with heart failure were analyzed by applying a logistic regression model. A P-value of <0.05 was used to declare a significant association. A total of 262 patients with heart failure were included in the study. The mean age (± SD) of the study subjects was 64.6 (± 9.2) years. Hypertensive heart disease (111/262, 42%) was the most common cause of heart failure. Hypertension was the frequently observed comorbid disease. Based on the MNA-FL score for nutritional status, 75 out of 262 (28.6%, 95% CI: 22.9-34.4%) were undernourished (MNA-FL < 17), while 124 out of 262 (47.3%, CI: 41.5-53.1%) were at risk of undernutrition (MNA-FL = 17-23.5). The remaining 63 out of 262 (24.1%, 95% CI: 18.2-29.8%) study subjects were well nourished (MNA-FL > 24). On a multivariate analysis, patients with severe heart failure (New York Heart Association (NYHA) functional class III/IV) (AOR = 4.287, CI: 2.012-9.134, P-value < 0.001), with a duration of illness of 3-5 years (AOR = 3.225, CI: 1.138-9.137, P-value = 0.028), with a duration of illness of >5 years (AOR = 4.349, CI: 1.592-11.879, P-value = 0.001), presence of comorbidities (AOR = 2.29, CI: 1.06-4.96, P-value = 0.036), who underwent treatment with loop diuretics (AOR = 2.983, CI: 1.407-6.326, P-value = 0.040), and who reside in a rural area (AOR = 5.119, CI: 2.481-10.560, P-value < 0.001) were at risk of developing undernutrition. Undernutrition was a significant clinical problem in older patients with heart failure. Nutritional interventions should be prioritized for patients with chronic and severe heart failure.

S125 Predictors and Causes of 30-Day Readmissions in Primary Biliary Cholangitis: Analysis of the Nationwide Readmission Database

Introduction: Primary biliary cholangitis (PBC) is an autoimmune disorder that most commonly affects middle-aged women. Cirrhosis, hepatocellular carcinoma, metabolic bone disease, and malabsorption can complicate PBC. In this study we aimed to describe the causes and predictors of 30-day readmissions in patients with PBC. Methods: This was an observational retrospective study involving adult patients hospitalized with PBC in the US. We utilized the Nationwide Readmission Databases 2016 to 2019. The first hospitalization for PBC within the year was marked as the index admission. We identified one subsequent hospitalization within 30 days, this was marked as a readmission. We analyzed the rates, causes, and outcomes of the readmitted cohort. We used multivariable cox regression analysis to identify independent predictors of readmissions. Results: We included a total of 3954 hospitalizations with PBC as the primary diagnosis for admission. 30.5% of the discharged cohort were readmitted within 30 days. Excluding PBC, the most common reasons for readmission were hepatic failure (9.6%) and sepsis (6.7%). Fluid and electrolyte disorders were the most common comorbidities in both the index hospitalization and readmission cohorts. Mortality was higher in the readmitted patients compared to index hospitalizations (10.1% and 4.3%, respectively, p < 0.001). Predictors of 30-day readmissions were peptic ulcer disease (aHR 1.64, p = 0.040), renal failure (aHR 1.29, p = 0.038), weight loss (aHR 1.28, p = 0.024), fluid and electrolyte disorders (aHR 1.28, p = 0.011), and coagulopathy (aHR 1.22, p = 0.029). Conclusion: Patients with PBC have high readmission rates, these readmissions are associated with high inpatient mortality. PBC patients are at risk of liver cirrhosis and malabsorption which explains the high rates of sepsis and electrolyte abnormalities observed in our study. Coagulopathy and weight loss might be reflective of the severity of liver dysfunction and the malabsorptive state, hence, there were associated higher readmission risk. Excellent short and long-term survival have been described following liver transplantation for PBC patients. However, recent data demonstrated that they have higher wait-list mortality among patients listed for liver transplantation.

Severe Coagulopathy in a Patient With Short Bowel Syndrome

BackgroundShort bowel syndrome (SBS) refers to a malabsorptive state caused by extensive resection of the intestinal tract that leads to chronic diarrhea, electrolyte disturbances, and malnutrition. Although relatively uncommon, patients with SBS can present to the emergency department with more serious complications that are potentially life-threatening. Among these complications, coagulopathy secondary to SBS is an underrecognized condition. Case ReportWe present a case of severe coagulopathy secondary to vitamin K deficiency in SBS. The patient presented with unexplained coagulopathy and spontaneous bleeding in multiple organs. With a review of surgical history and detailed clinical evaluation, SBS complicated with vitamin K deficiency was diagnosed, and the patient was treated successfully. Why Should an Emergency Physician Be Aware of This?When a patient with a history of repeated intestinal surgery presents with diarrhea, malnutrition, or electrolyte abnormalities, emergency physicians should suspect SBS. Among complications of SBS, vitamin K deficiency is a rare but serious cause of unexplained coagulopathy presenting to the emergency department. Understanding the pathophysiology of SBS facilitates early identification of complications and improves patient outcomes.

Cashew-Induced Oxalate Nephropathy: A Rare Cause of Acute Renal Failure.

We present a rare case of cashew-induced oxalate nephropathy in a 69 year old veteran male with history of type 2 diabetes mellitus, nephrolithiasis, and undiagnosed chronic kidney disease (CKD). Oxalate nephropathy is a rare cause of acute renal failure with poor prognosis. The various causes of oxalate nephropathy are categorized as primary or secondary hyperoxaluria. Primary hyperoxaluria is caused by genetic mutation in genes involved in the metabolism of glyoxylate. Secondary hyperoxaluria is caused by mal-absorptive state, excessive intake of oxalate-rich diet, inflammatory diseases, and medications such as orlistat and antibiotics. Diet-induced oxalate nephropathy is often identified after unexplained acute kidney injury in patients with underlying CKD. Definitive diagnosis requires renal biopsy as laboratory tests are non-specific. A simple dietary history in CKD patients during routine primary care visit may lead to early diagnosis and lead to prevention of acute renal failure and progression of renal disease.

Prevalence and management of vitamin D deficiency in children with newly diagnosed coeliac disease: cohort study

ABSTRACT Background Coeliac disease (CD) causes deficiency of various micronutrients including vitamin D, and there are no specific guidelines for treatment. Aims To determine the prevalence of vitamin D deficiency in children newly diagnosed with CD and the role of oral high-dose vitamin D in its treatment. Methods Calcium intake, sun exposure and biochemical and radiological parameters related to vitamin D deficiency were compared between 60 children aged 0–18 years diagnosed with CD and 60 healthy age- and sex-matched controls. The cases with serum 25(OH)D (<20 ng/ml) were given oral vitamin D (60,000 IU/week) and calcium (500 mg/day) for 12 weeks, along with a gluten-free diet (GFD); they were re-evaluated within a week of completion. The primary outcome measure was the serum 25(OH)D level, and secondary measures included serum calcium, phosphorus, alkaline phosphatase, parathormone and clinical and/or radiological rickets. Results The prevalence of vitamin D deficiency (25(OH)D <20 ng/ml) was significantly greater in the cases (n=38, 63.3%) than in the controls (n=27, 45.0%). Upon treatment, all 38 cases with vitamin D deficiency showed a significant rise in 25(OH)D levels along with normalisation of other biochemical abnormalities. Two children had 25(OH)D levels >100 ng/ml with no other feature suggestive of vitamin D toxicity. Conclusions Vitamin D deficiency is more prevalent in children with CD. Administration of oral high-dose vitamin D for 12 weeks along with a GFD leads to a robust response, indicating rapid mucosal recovery. The vitamin D dosage recommended for malabsorption states may be excessive in CD. Abbreviations: ALP: alkaline phosphatase; CaBP: calcium-binding proteins; CD: coeliac disease; GFD: gluten-free diet; PTH: parathormone; RU: reproducibility units; 25(OH)D: 25 hydroxy vitamin D

Celiac Disease Diagnosed after Gastrectomy for Gastric Cancer

Celiac disease is a systemic autoimmune disorder leading to manifestations of malabsorption syndrome. A 47-year-old Japanese man developed severe diarrhea after surgery for gastric cancer. The diarrhea persisted for seven months, leading to a state of malabsorption. Celiac disease was suspected based on small bowel capsule endoscopy findings. The duodenal findings observed during gastric cancer surgery were reassessed, and Marsh-Oberhuber classification type 3c celiac disease was diagnosed. The anti-tissue glutaminase antibody test results were positive. The patient was started on a gluten-free diet, following which the diarrhea resolved, and the nutritional status improved. Adjuvant therapy after gastric cancer surgery was initiated.

Butyrate and the Intestinal Epithelium: Modulation of Proliferation and Inflammation in Homeostasis and Disease.

The microbial metabolite butyrate serves as a link between the intestinal microbiome and epithelium. The monocarboxylate transporters MCT1 and SMCT1 are the predominant means of butyrate transport from the intestinal lumen to epithelial cytoplasm, where the molecule undergoes rapid β-oxidation to generate cellular fuel. However, not all epithelial cells metabolize butyrate equally. Undifferentiated colonocytes, including neoplastic cells and intestinal stem cells at the epithelial crypt base preferentially utilize glucose over butyrate for cellular fuel. This divergent metabolic conditioning is central to the phenomenon known as “butyrate paradox”, in which butyrate induces contradictory effects on epithelial proliferation in undifferentiated and differentiated colonocytes. There is evidence that accumulation of butyrate in epithelial cells results in histone modification and altered transcriptional activation that halts cell cycle progression. This manifests in the apparent protective effect of butyrate against colonic neoplasia. A corollary to this process is butyrate-induced inhibition of intestinal stem cells. Yet, emerging research has illustrated that the evolution of the crypt, along with butyrate-producing bacteria in the intestine, serve to protect crypt base stem cells from butyrate’s anti-proliferative effects. Butyrate also regulates epithelial inflammation and tolerance to antigens, through production of anti-inflammatory cytokines and induction of tolerogenic dendritic cells. The role of butyrate in the pathogenesis and treatment of intestinal neoplasia, inflammatory bowel disease and malabsorptive states is evolving, and holds promise for the potential translation of butyrate’s cellular function into clinical therapies.

The Stability of TSH, and Thyroid Hormones, in Patients Treated With Tablet, or Liquid Levo-Thyroxine.

Approximately, 5% of the population is affected by hypothyroidism, mainly women and persons aged more than 60 years. After the diagnosis of hypothyroidism the usual therapy is tablet levothyroxine (L-T4), with a monitoring of the thyroid-stimulating hormone (TSH) level in primary hypothyroidism every 6–8 weeks and L-T4 is adjusted as necessary to reach an euthyroid state. Once TSH is stabilized in the normal range, it is recommended to conduct annual testing in the treated subjects to warrant suitable replacement. More recently advances regarding L-T4 treatment are the introduction of new oral formulations: the liquid solution, and soft gel capsule. The soft gel capsule permits a quick dissolution in the acid gastric pH. The liquid preparation does not require an acid gastric environment. Many pharmacokinetic studies demonstrated a more rapid absorption for the liquid L-T4, or capsule, than with tablet. Many studies have shown that the liquid, or capsule, formulations can overcome the interaction with foods, drugs or malabsorptive conditions, that are able to impair the tablet L-T4 absorption. Lately studies have suggested that liquid L-T4 can permit to maintain more efficiently normal TSH levels in hypothyroid patients in the long-term follow-up, than tablet L-T4, both in patients with malabsorptive states, and in those without malabsorption. Further large, prospective, longitudinal studies are needed to evaluate the stability of TSH, in hypothyroid patients treated with different L-T4 formulations.

An overview of the current management of short-bowel syndrome in pediatric patients.

Short-bowel syndrome (SBS) is defined as a state of malabsorption after resection or loss of a major portion of the bowel due to congenital or acquired factors. This article presents an overview on the recent management of pediatric SBS. The pediatric SBS population is very heterogeneous. The incidence of SBS is estimated to be 24.5 per 100,000 live births. The nutritional, medical, and surgical therapies available require a comprehensive evaluation. Thus, multidisciplinary intestinal rehabilitation programs (IRPs) are necessary for the management of these complex patients. The key points of focus in IRP management are hepato-protective strategies to minimize intestinal failure-associated liver disease; the aggressive prevention of catheter-related bloodstream infections; strategic nutritional supply to optimize the absorption of enteral calories; and the management and prevention of small bowel bacterial overgrowth, nephrocalcinosis, and metabolic bone disease. As the survival rate of children with SBS currently exceeds 90%, the application of small bowel transplantation has been evolving. The introduction of innovative treatments, such as combined therapy of intestinotrophic hormones, including glucagon-like peptide-2, may lead to further improvements in patients' quality of life.

Antiseizure Medication use in Gastric Bypass Patients and Other Post-Surgical Malabsorptive States.

Healthcare professionals are encountering an increasing number of patients who have undergone bariatric surgeries. Antiseizure medications (ASM) have a narrow therapeutic window, and patients with malabsorptive states receiving ASM present a complex situation as the pharmacokinetics of these drugs have only been studied in patients with a normal functioning gastrointestinal tract. Patients with malabsorptive states may have altered pharmacokinetics, and there is limited literature to guide drug selection and dosage adjustment in patients with malabsorptive states. This review highlights pharmacokinetic parameters of common ASM, and considerations when managing patients on them. The effect of pH, lipophilicity, absorption, and metabolism should be taken into account when selecting and managing ASMs in this patient population. Based on these parameters, levetiracetam, and topiramate have fewer issues referable to absorption related to bariatric surgery while oral formulations of phenytoin, carbamazepine, oxcarbamazepine and valproic acid have reduced absorption due to effects of bariatric surgery based on the pharmacokinetic properties of these medications. Extended formulations should be avoided and ASM serum concentrations should be checked before and after surgery. The care of patients with epilepsy who are scheduled to undergo bariatric surgery should be guided by a multidisciplinary team including a pharmacist and a neurologist who should be involved in the adjustment of the ASMs throughout the pre-surgical and post-surgical periods.

Senescence-accelerated mouse prone 8 mice exhibit specific morphological changes in the small intestine during senescence and after pectin supplemented diet.

Impairment of gastrointestinal function and reduction of nutrient absorption associated with aging contribute to increased risk of malnutrition in the elderly population, resulting in physical weakness and vulnerability to disease. The present study was performed to examine the relationships between aging-associated morphological changes of the small intestine and nutrient malabsorption using senescence-accelerated mouse prone 8 (SAMP8) mice. Comparison of the morphology of the small intestine of young (22-week-old) and senescent (43-week-old) SAMP8 mice showed no significant changes in villus length, while the mRNA expression levels of secretory cell marker genes were significantly reduced in senescent mice. In addition, crypts recovered from the small intestine of senescent mice showed a good capacity to form intestinal organoids ex vivo, suggesting that the regenerative capacity of intestinal stem cells (ISCs) was unaffected by accelerated senescence. These results indicated that changes induced by accelerated senescence in the small intestine of SAMP8 mice are different from changes reported previously in normal aging mouse models. Biochemical analyses of serum before and during senescence also indicated that senescent SAMP8 mice are not in a malabsorption state. Furthermore, a diet supplemented with persimmon pectin had a mild effect on the small intestine of senescent SAMP8 mice. Intestinal villus length was slightly increased in the medial part of the small intestine of pectin-fed mice. In contrast, intestinal crypt formation capacity was enhanced by the pectin diet. Organoid culture derived from the small intestine of mice fed pectin exhibited a greater number of lobes per organoid compared with those from mice fed a control diet, and Lyz1 and Olfm4 mRNA levels were significantly increased. In conclusion, accelerated senescence induced exclusive changes in the small intestine, which were not related to nutrient malabsorption. Therefore, the SAMP8 strain may not be a suitable model to evaluate the effects of aging on intestinal homeostasis and nutrient absorption impairment.

Short Bowel Syndrome in an Infant

Short bowel syndrome (SBS) is a malabsorptive state that may occur either after surgical bowel resection or as the result of congenital bowel anomalies. SBS can incur significant morbidity and mortality including intestinal failure, cholestasis, sepsis, and death. For patients with SBS, management involves a multidisciplinary approach that begins with neonatology, pediatric surgery, nutritionists, pharmacists, and nurses in the NICU and also includes the transition to an intestinal rehabilitation program. The aim of this review is to provide the neonatologist with an overview of the common causes of neonatal SBS, anticipated nutritional deficiencies, complications associated with SBS, and the surgical and medical management of SBS to assist in counseling affected families.

SAT-503 Levothyroxine Absorption Testing in Three Patients with Severe Hypothyroidism on Adequate Replacement

Background: Levothyroxine (L-T4) absorption is a concern in patients who report appropriate self-administration but remain clinically and biochemically hypothyroid. However, there are no established guidelines for L-T4 absorption testing. Clinical Cases: Two women (A and B) with a history of Graves’ disease and radioactive iodine ablation (RAI) on weight-based L-T4 replacement presented to the hospital with severe fatigue and bilateral leg swelling. A third woman (C) with a history of papillary thyroid carcinoma, total thyroidectomy and RAI, on suppressive doses of L-T4 presented to the clinic with fatigue, constipation and weight gain. All patients reported compliance with taking L-T4 with appropriate technique. They had no known medical problems contributing to a malabsorptive state, and were not taking any medications known to interfere with L-T4 absorption.Initial testing of thyroid function revealed TSH (0.57-3.5 μIU/mL)/Free T4 (0.61-1.18 ng/dL) levels of 10.12/0.249, 45.95/<0.2 and 142/<0.2 in patients A, B and C respectively. Patients A and B tested negative for Helicobacter pylori infection, celiac disease and lactose intolerance. Patient C had been successfully treated for H. pylori infection in the past. Celiac testing was negative, but the patient tested positive for anti-parietal cell antibodies and vitamin B12 deficiency. She was referred to Hematology & GI specialists. None of the patients had clinical evidence of heart failure and had normal echocardiograms.In order to evaluate for poor absorption, all patients underwent L-T4 absorption testing. Based on published data, baseline TSH and Free T4 levels were obtained followed by administration of 1000 mcg of L-T4. Next, free T4 levels were obtained at 2 hours for all patients and at 4, 8 and 24 hours for patient A & B. Patients A and B underwent testing in the inpatient setting, while Patient C was tested in the outpatient clinic.In all cases, a > 50% rise in free T4 levels was observed at 2 hours, suggesting non-adherence or “pseudo-malabsorption”. Patients were counseled extensively regarding proper technique and compliance with L-T4. Patients A and B demonstrated rapid clinical improvement in the hospital, and Patient C reported to her next clinic visit with clinical and biochemical improvement. Conclusions: Though guidelines have not been established for L-T4 absorption testing, case reports exist exhibiting the safety and efficacy of L-T4 absorption challenge with a weekly weight-based dose or 1000 mcg of LT4. Our case series show the success of the latter with meaningful results in as little as 2 hours. Case C demonstrates that this test can be done safely and cost-effectively as an outpatient. Standardization of L-T4 absorption test will be of great utility in the management of this common problem encountered by endocrinologists.

A man in his fifties with chronic diarrhoea and weight loss.

Watery diarrhoea coupled with weight loss is a serious condition with many potential causes. We present a possibly underappreciated cause which usually responds well to treatment; left untreated it may have a severe course. A man in his fifties with known coronary and cerebrovascular disease was admitted for watery diarrhoea. Prerenal kidney failure occurred on the same day as the initial colonoscopy. The next day he suffered a stroke. He was anticoagulated and recovered within days. In the following months his state of malabsorption continued, with ultimately 50% weight loss (BMI 14.7) and severe electrolyte disturbances. Intravenous electrolyte solutions and nutrition were administered. Oedema and aphthous duodenal lesions were the only endoscopic findings. Microscopic findings of total villus atrophy in all sampled sites in the small intestine, including the ileum, were striking. There were inflammatory cells in lamina propria, apoptotic cells and disappearance of goblet cells. Coeliac disease was ruled out by serology and HLA typing. A final diagnosis of autoimmune enteropathy was made, based on exclusion of other intestinal and systemic diseases. Treatment with infliximab intravenously and budesonide in an open capsule regime was successful.

Diagnosing celiac disease: A critical overview.

The diagnosis of celiac disease (CD) no longer rests on a malabsorptive state or severe mucosal lesions. For the present, diagnosis will always require the gold-standard of a biopsy, interpreted through its progressive phases (Marsh classification). Marsh classification articulated the immunopathological spectrum of gluten-induced mucosal changes in association with the recognition of innate (Marsh I infiltration) and T cell-based adaptive (Marsh II, and the surface re-organisation typifying Marsh III lesions) responses. Through the Marsh classification the diagnostic goalposts were considerably widened thus, over its time-course, permitting countless patients to begin a gluten-free diet but who, on previous criteria, would have been denied such vital treatment. The revisions of this classification failed to provide additional insight in the interpretation of mucosal pathology. Morever, the subclassification of Marsh 3 imposed an enormous amount of extra work on pathologists with no aid in diagnosis, treatment, or prognosis. Therefore, it should now be apparent that if gastroenterologists ignore these sub-classifications in clinical decision-making, then on that basis alone, there is no need whatsoever for pathologists to persist in reporting them. Since new treatments are under critical assessment, we might have to consider use of some other higher level histological techniques sensitive enough to detect the changes sought. A promising alternative would be to hear more voices from imaginative histopathologists or morphologists together with some more insightful approaches, involving molecular-based techniques and stem cell research may be to evaluate mucosal pathology in CD.

Celiac crisis, a rare occurrence in adult celiac disease: A systematic review.

BACKGROUNDCeliac crisis (CC), a potentially life-threatening condition, is one of the rare clinical presentations of celiac disease (CD). Several cases have been documented in the literature, mostly in children.AIMTo perform a review of CC cases reported in adult CD patients.METHODSA systematic search of the literature was conducted in two databases, PubMed/MEDLINE and EMBASE, using the term “celiac crisis" and its variant "coeliac crisis", from January 1970 onwards. Altogether, 29 articles reporting 42 biopsy-proven cases were found in the search. Here, we summarized the demographic, clinical characteristics, laboratory and diagnostic work-ups, and therapeutic management in these patients.RESULTSAmong the 42 CD cases, the median age was 50 years (range 23-83), with a 2:1 female to male ratio. The majority of patients (88.1%) developed CC prior to CD diagnosis, while the remaining were previously diagnosed CD cases reporting low adherence to a gluten-free diet (GFD). Clinically, patients presented with severe diarrhea (all cases), weight loss (about two thirds) and, in particular situations, with neurologic (6 cases) or cardiovascular (1 case) manifestations or bleeding diathesis (4 cases). One in four patients had a precipitating factor that could have triggered the CC (e.g. trauma, surgery, infections). Laboratory workup of patients revealed a severe malabsorptive state with metabolic acidosis, dehydration, hypoalbuminemia and anemia. The evolution of GFD was favorable in all cases except one, in whom death was reported due to refeeding syndrome.CONCLUSIONCeliac crisis is a rare but severe and potentially fatal clinical feature of CD. A high index of suspicion is needed to recognize this clinical entity and to deliver proper therapy consisting of supportive care and, subsequently, GFD.

Remnant Small Bowel Length in Pediatric Short Bowel Syndrome and the Correlation with Intestinal Dysbiosis and Linear Growth

Pediatric short bowel syndrome (SBS) is a malabsorptive state placing patients at risk for malnutrition, dehydration, and bacterial overgrowth. These patients are often dependent on parenteral nutrition (PN) while intestinal adaptation is underway. The aim of this study was to characterize the effect of remnant small bowel length on the gut microbiome. Further, we sought to examine the contribution of clinical and nutritional variables to the gut microbiota and anthropometric growth. Clinical data, anthropometrics, and fecal samples were collected from 14 SBS patients and 10 age- and sex-matched controls. Fecal bacterial DNA composition was analyzed using 16s ribosomal RNA gene sequencing. Statistical analysis was completed using the Mann-Whitney or Fisher's exact tests when applicable and linear mixed effect modeling. Distinct microbiota changes were found among those with the least remaining small bowel (<35 cm) compared with those with longer remaining bowel and controls. Those with <35 cm small bowel displayed an increased relative abundance of Proteobacteria, while those with longer remaining small bowel had a higher proportion of Firmicutes. Further, patients with less remaining bowel required more PN (p < 0.01), with a tendency to be shorter in height (p= 0.05) and with a higher BMI (p= 0.05). Remnant small bowel length appears to be a predictor of stunting with diminished linear growth, parenteral nutrition dependency, and a greater relative abundance of Proteobacteria in the gut. These findings suggest an integrated adaptive response predicted by remnant intestinal length. Further research is necessary to examine the effects of intestinal dysbiosis on clinical outcomes.