Celiac Crisis Research Articles

Adult celiac disease presented with celiac crisis: Report of two cases

Two patients (case 1: 29 years old and case 2: 66 years old. female) with no known medical history of chronic diseases, including celiac disease, presented to the hospital with prolonged diarrhea, weight loss, and severe hypocalcemia at different times. They were admitted to the hospital for hemodynamic instability in the setting of severe dehydration and electrolyte disturbances. Physical examination revealed a positive trousseau sign in Case 1. The typical laboratory features of both cases were low magnesium, low potassium, low vitamin D, low ferritin, and prolonged coagulation tests. In addition to those labs, case 2 also has metabolic acidosis. In both cases, the titers of the tissue transglutaminase IgA and IgG and the anti-endomysium antibody were high, and the histopathology of the duodenal biopsy was consistent with villous atrophy, crypt hyperplasia, and an increase in intraepithelial lymphocytes, suggesting celiac disease. Both cases responded quickly to treatment with a gluten-free diet, fluid, electrolyte, vitamin D, and K replacements, and were discharged. Celiac crisis is a rare presentation of celiac disease characterized by acute, severe metabolic imbalances resulting in high mortality and morbidity, with severe diarrhea, hypoproteinemia, and metabolic and electrolyte disturbances. It is typically seen in children under 2 years of age but can also be encountered in adulthood. Most cases respond to gluten cessation, nutritional support, and rarely steroid treatment.

Crisi celiaca: una complicanza rara e insidiosa della celiachia

Coeliac Crisis is an urgent and potentially fatal complication of Coeliac Disease (CeD), which is most frequent in early childhood. The crisis may occur at onset or develop in CeD patients with poor compliance to the gluten-free diet (GFD). Stress, surgery and infections can act as triggers. The diagnosis is based on the acute onset of rapidly progressive diarrhoea associated with at least two conditions among severe dehydration, metabolic acidosis, dyselectrolytemia, renal dysfunction, neurological alterations, hypoproteinemia and weight loss. Haematological manifestations, such as thrombocytopenia, increased INR, aplastic anaemia and leukopenia, are rare. The cornerstones of therapy are intravenous rehydration, correction of hypoalbuminemia, acidosis and dyshionemia along with a strict GFD. Steroids may be also used in refractory cases. The paper describes a recent case of a patient presenting with a severe life-threatening coeliac crisis characterized by inability to walk due to lower limb weakness, severe abdominal distension, diarrhoea and peripheral oedema.

Celiac Disease in Moroccan Children: Diagnostic Characteristics and Determinants of Diagnosis Delay.

Advances in the field of celiac disease have led to a better understanding of the disease, but it remains underdiagnosed and poses a daily challenge to clinicians to make a timely diagnosis. This study aims to analyze and describe diagnosis characteristics, diagnosis delay, and the factors influencing this delay in Moroccan children. Our study included 324 children diagnosed during the study period from January 01, 2010, to December 30, 2019, at the Department of Pediatrics, Hassan II University Hospital inFez, Morocco. Data were collected using a collection grid and then analyzed using SPSS 26 software (IBM Corp., Armonk, NY). The results showed a female predominance (n=197, 60.8%), with a diagnosis age of 73.8±46.8 months. The mean age onset of symptoms was 51.3±41.2 months, and the diagnosis delay was 22.2±22.6 months, with only 32.7% (n=106) diagnosed less than 12 months after symptom onset. The most common consultation reason was diarrhea (n=149, 46%) and growth delay (n=105, 32.4%) and 50.5% (n=98) of parents consulted a pediatrician first. The three clinical, serologic, and histologic criteria made it possible to agree on the diagnosis, with the clinical profile dominated by the digestive form at 84.9% (n=279), serologic with the presence of IgA transglutaminase antibodies (95.7%; n=310), and histologic with villous atrophy at 91.7% (n=297). Unfortunately, 14.8% (n=48) of the children were diagnosed with a celiac crisis. The multivariate logistic regression analysis showed that as symptoms onset age increased, so did the risk of late diagnosis (OR=0.96, 95% CI: 0.94 to 0.97, p<0.001). Age of diagnosis was also associated with delayed diagnosis (OR=19.68, 95% CI: 8.77 to 44.15, p<0.001). The combination of these variables and the diagnosis delay argues in favor of adopting a diagnosis strategy that includes raising awareness among healthcare professionals of the need to identify typical and atypical cases early in order to reduce the adverse effects of late diagnosis and the complications that can result. This methodology for improving diagnoses may also unearth previously unknown aspects of celiac disease in Moroccan children.

Celiac Crisis in a 19-Year-Old Woman with No History of Celiac Disease

Celiac Crisis in a 19-Year-Old Woman with No History of Celiac Disease

English

Celiac disease is also called as non-tropical sprue, celiac sprue, adult celiac disease and gluten sensitive enteropathy. Environmental, immunologic and genetic factors play an important role in the aetiology.1 Symptoms of celiac disease can manifest at any age throughout adulthood. Symptoms range from significant malabsorption of multiple nutrients with diarrhoea, steatorrhea, weight loss and consequences of nutrient depletion (like anaemia and metabolic bone disease). Atypical celiac disease can present with symptoms not related to intestinal malabsorption like neurologic symptoms. Large number of patients with celiac disease can even be asymptomatic. The gold standard in diagnosis of celiac disease is an abnormal small intestinal biopsy and the response of the condition to the elimination of gluten from the diet. Classic changes seen on duodenal /jejunal biopsy 1. An increase in number of intraepithelial lymphocytes 2. Absence or a reduced height of villi 3. A cuboidal appearance and nuclei that are no longer oriented basally in surface epithelial cells 4. Increased numbers of lymphocytes and plasma cells in lamina propria Serology tests for celiac disease: IgA antiendomysial antibody and tissue transglutaminase TG antibodies which when positive is supportive in diagnosis. Acute presentation of celiac disease with severe metabolic derangement in adults is rare and called as “celiac crisis.” 2 In crisis the patient may present with profuse diarrhoea, severe dehydration and metabolic abnormalities, hypokalaemia, hypoalbuminemia, neurologic symptoms and azotaemia. We describe one such case in an elderly female patient.

Celiac crisis with thrombocytopenia and coagulopathy in a child

Celiac disease rarely presents with edema, hypoalbuminemia, acute metabolic deterioration, and electrolyte imbalances. This life-threatening condition is defined as a celiac crisis and may mimic disorders with metabolic derangement and sepsis. The crisis may present at onset or develop in celiac disease patients with poor compliance to a gluten-free diet. The fluid resuscitation and replacement of electrolyte deficits are life-saving modalities. A 14-month-old girl was admitted with fever, lethargy, severe dehydration, edema, hypotension, and commenced sepsis therapy. However, the patient had a growth delay and loss of weight with diarrhea and delayed motor skills. On admission, laboratory evaluation showed anemia, coagulopathy, hypoalbuminemia, electrolyte disturbances, and metabolic acidosis and developed thrombocytopenia during follow-up. The celiac serological tests and upper gastrointestinal endoscopic duodenal mucosa appearance, and duodenum histopathology findings suggested celiac disease. This case highlights that a celiac patient may present with a severe illness like sepsis and may be associated with cytopenia and coagulopathy in the celiac crisis.

Celiac Crisis in an Adult Patient: Case Report and Review of the Literature

We report a case of celiac crisis in a previously healthy 51-year-old female presenting with a month’s history of diarrhea, cachexia and an abnormal metabolic panel.The patient’s diarrhea resolved after initiation of a gluten free diet and she gained 4 kilograms during hospitalization. Celiac crisis is a very rare presentation of celiac disease in adults but nonetheless should be considered in patients with marked metabolic derangements in the setting of osmotic diarrhea.

Una “crisi” celiaca

The authors present the case of a 3-year-old child with severe diarrhoea, hypoalbuminaemia, hypogammaglobulinaemia and anaemia eventually diagnosed as coeliac. The clinical features of the so-called coeliac crisis are discussed.

Celiac Disease.

Celiac Disease.

Generalized edema as the dominant clinical symptom of celiac crisis

ENWEndNote BIBJabRef, Mendeley RISPapers, Reference Manager, RefWorks, Zotero AMA Wilczyńska D, Rekowska M, Lewandowska D, Wyrębska-Ruge J, Krogulska A, Szaflarska-Popławska A. Generalized edema as the dominant clinical symptom of celiac crisis. Pediatria Polska - Polish Journal of Paediatrics. 2021. doi:10.5114/polp.2021.110552. APA Wilczyńska, D., Rekowska, M., Lewandowska, D., Wyrębska-Ruge, J., Krogulska, A., & Szaflarska-Popławska, A. (2021). Generalized edema as the dominant clinical symptom of celiac crisis. Pediatria Polska - Polish Journal of Paediatrics. https://doi.org/10.5114/polp.2021.110552 Chicago Wilczyńska, Dominika, Martyna Rekowska, Dorota Lewandowska, Joanna Wyrębska-Ruge, Aneta Krogulska, and Anna Szaflarska-Popławska. 2021. "Generalized edema as the dominant clinical symptom of celiac crisis". Pediatria Polska - Polish Journal of Paediatrics. doi:10.5114/polp.2021.110552. Harvard Wilczyńska, D., Rekowska, M., Lewandowska, D., Wyrębska-Ruge, J., Krogulska, A., and Szaflarska-Popławska, A. (2021). Generalized edema as the dominant clinical symptom of celiac crisis. Pediatria Polska - Polish Journal of Paediatrics. https://doi.org/10.5114/polp.2021.110552 MLA Wilczyńska, Dominika et al. "Generalized edema as the dominant clinical symptom of celiac crisis." Pediatria Polska - Polish Journal of Paediatrics, 2021. doi:10.5114/polp.2021.110552. Vancouver Wilczyńska D, Rekowska M, Lewandowska D, Wyrębska-Ruge J, Krogulska A, Szaflarska-Popławska A. Generalized edema as the dominant clinical symptom of celiac crisis. Pediatria Polska - Polish Journal of Paediatrics. 2021. doi:10.5114/polp.2021.110552.

Celiac crisis, atypical form of celiac disease onset in teenager – a case report

Celiac crisis, atypical form of celiac disease onset in teenager – a case report

Rotavirus gastroenteritis as a precipitating factor of celiac crisis in infancy: Case reports and review of literature

Introduction. Celiac crisis is a rare and life-threatening complication of celiac disease. Although it occurs in all ages, the most common affects children within the first two years. Outline of cases. We report three infants (two female, one male, age range 9?12) with celiac crises as an initial presentation of celiac disease precipitated with rotavirus gastroenteritis. Celiac crisis was preceded by failure to thrive caused by anorexia, occasional vomiting and frequent abundant stools for 4?8 weeks, and 1?2 days before admission with fever, frequent vomiting and profuse watery diarrhea. They were admitted in a very severe general condition, severely dehydrated, markedly malnourished, with an enormously distended abdomen, edema of the lower legs and feet, and perianal erythema. After correction of dehydration and hypoalbuminemia, they were placed on a gluten- and disaccharide-free diet and within the first two weeks on additional parenteral nutrition. The applied therapeutic measures resulted in stabilization and further rapid improvement of the patient?s condition. In all three patients the latex agglutination test for rotavirus was positive, IgA anti-TTG antibodies elevated (58.6?78 U/ml) and all three were homozygous carriers of the HLA DQ2 gene. Enterobiopsy was performed two weeks after the admission and total villous atrophy (Marsh IIIc) was registered in all three patients. In the further course, the complete recovery of the patient was accomplished by a strict gluten-free diet. Conclusion. Our experience indicates that rotavirus gastroenteritis in timely unrecognized classical celiac disease in infants can lead to celiac crisis.

The Risk of Delayed Diagnosis in COVID-19 Era: Celiac Crisis

COVID-19 lockdown in Italy had a negative impact on common clinical practice showing a substantial decrease in pediatric emergency department visits. Undoubtedly..

Enteric-Release Budesonide May Be Useful in the Management of Non-Responsive Celiac Disease.

Non-responsive celiac disease (NRCD) has many aetiologies, including gluten exposure. Budesonide may be used for refractory celiac disease (RCD) and celiac crisis. We reviewed the effectiveness of budesonide to induce clinical and histologic response in NRCD with villous atrophy (VA). Case series of adult cases with NRCD and VA prescribed budesonide at two celiac centers. Clinical variables and mucosal recovery (i.e., normal villous architecture within 1year of treatment) were evaluated. Forty-two cases [77% female, median age 45.0 (IQR 28.3-60.0) years] were included. Most common symptoms were diarrhea (64%) and abdominal pain (62%). Budesonide was initiated at 9mg (83%) for a median duration of 16.0weeks (IQR 6.8-25.0weeks). In total, 57% exhibited a clinical response, positively associated with diarrhea (adjusted OR 6.08 95% CI 1.04-35.47) and negatively with fatigue (adjusted OR 0.18 95% CI 0.03-0.98). Clinical response was higher among those with dietitian counseling prior to budesonide (n = 29, 70 vs. 23%, p < 0.01). Mucosal recovery was observed in 11/24 with follow-up duodenal biopsies. There was no association between clinical response and mucosal recovery, and 79% of clinical responders had a symptomatic relapse. RCD (48%) and chronic gluten exposure (24%) were the main suspected aetiologies of NRCD. Most individuals without a clinical response subsequently received an IBS-related diagnosis. Budesonide may be effective to induce clinical response in NRCD presenting with diarrhea and VA, but clinical recurrence and lack of mucosal recovery are frequent after tapering. Other diagnoses, including coexisting IBS, may be considered in non-responders to budesonide therapy.

Celiac Disease Presenting as Cardiomyopathy - A Rare Extra Intestinal Manifestation

Cardiac manifestations of celiac disease has been poorly described in literature though some studies have emphasized on correlation of ischemic heart disease, atrial fibrillation and dilated cardiomyopathy with celiac disease. We describe a patient with celiac disease associated cardiomyopathy whose cardiac function improved substantially after treatment with a gluten-free diet. A young lady of 35 years age was admitted with complaints of chronic diarrhea, vomiting, significant weight loss, dizziness and chronic iron deficiency anemia since last 3 months. Physical examination revealed tachycardia, tachypnoea and hypotension requiring multiple high dose inotropic support. She had pallor, skin changes and pedal edema suggestive of malabsorption. Investigations revealed iron deficiency anemia, high bilirubin and transaminases, prothrombin time, low albumin and elevated tissue trasglutaminase antibody. Echocardiography showed global hypokinesia with a left ventricular ejection fraction of 20%. Diagnosis of celiac disease made on clinic features, serology and biopsy finding of subtotal villous atrophy, crypt hyperplasia, and increased intraepithelial lymphocytes. She was kept on gluten free diet and on intravenous steroids for acute celiac crises and celiac related cardiomyopathy as patient had severe diarrhea, hypocalcaemia, weight loss, hypoproteinemia and hypotension. Patient improved within 48 hours of steroid with discontinuation of inotropes. She was discharged with gluten free diet and follow up after 6 months showed near normalization of all biochemical abnormalities and ejection fraction of 55%. Cardiomyopathy associated with celiac disease and celiac crisis is a serious and potentially lethal condition. However, with early diagnosis and treatment with a gluten free diet, and steroids cardiomyopathy in patients with celiac disease may be completely reversible.

Gluten-free diet in the treatment of extra-intestinal forms of gluten intolerance

Following a gluten-free diet is recommended by clinical guidelines in the presence of gluten intolerance. However, due to the variety of clinical picture of various forms of intolerance, the elimination of gluten occurs not always timely. There are also diseases that classic treatment regimen does not include diet therapy, however, studies have confirmed the effectiveness of its use. The aim of the research - to study current data on the effectiveness of a gluten-free diet for extra-intestinal manifestations of gluten intolerance. Material and methods. Literature data concerning the effectiveness of including a gluten-free diet in the treatment of various diseases according to the PubMed and eLIBRARY portal were studied. Results. Modern data on the forms of gluten intolerance and their clinical manifestations are presented. The results of both randomized studies and individual clinical cases of gluten intolerance that occurred under the guise of other diseases are presented. A clinical case of an acute onset of the disease - celiac crisis, accompanied by acute diarrheal syndrome with subsequent malabsorption and progressive loss of body weight, anasarca and electrolyte disorders is considered. Neurological and psycho-neurological manifestations of celiac disease are described, including current data on the results of including a glutenfree diet in the treatment of autism spectrum disorders. The question of using dietary therapy for autism remains controversial to nowadays. The article outlines the arguments of supporters and opponents of excluding gluten in this pathology. Particular attention is paid to the diagnosis of gluten intolerance in patients with hematological disorders. The significance of a complete survey to identify celiac disease and timely diet therapy of the disease under stunted growth in children, after excluding other causes of malabsorption and even in the presence of negative serological markers of celiac disease is shown. The article also contains information on the pathology of kidneys and reproductive system, which were leveled only after the exclusion of gluten from the diet. Conclusion. The presented cases demonstrate a wide variety of clinical forms of gluten intolerance, examples of diagnostic search and dynamics of the clinical picture with the timely appointment of a gluten-free diet are given.

Celiac Disease Presenting as Hypokalemic Quadriparesis

Celiac disease is an autoimmune disorder induced by dietary gluten, characterized by a chronic inflammatory state of the small intestinal mucosa, resulting in villous atrophy that resolves with gluten free diet. A small number have classical symptoms and the majority present as atypical celiac disease with absent or few GI symptoms. Celiac crisis is a rare life threatening complication of celiac disease which needs prompt recognition and intervention. We report a rare case of Celiac disease which presented as Celiac crisis with hypokalemic quadriparesis.

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.

Celiac Crisis: A Rare and Serious Condition Resulting in Death

Celiac disease is an autoimmune disease of the small intestine, resulted from villus damage and malabsorbtion due to ingestion of gluten; and production of antibodies to gliadin. The disease can manifest with various presentations. Celiac crisis was described as an acute, serious, life-threatening form of celiac disease about sixty years ago with a higher incidence among pediatric patients than adults. It is characterized by gastrointestinal symptoms like profuse diarrhea and severe metabolic distubances like hyponatremia and hypoproteinemia. Early suspicion and detection is important. This case report presents a 54-year-old female patient who died from celiac crisis and sepsis.

Tetany Due to Refeeding Syndrome in A Recently Diagnosed Child with Celiac Disease

Celiac disease (CD) occurs in genetically predisposed individuals, and usually presents with gastrointestinal symptoms. Hypocalcemia with associated tetany has rarely been reported as the presenting symptom of CD. A 4-year-old girl presented to us with bilateral painful carpopedal spasm. The patient was diagnosed with CD one month ago, and although adherent with her gluten-free diet, she consumed large amounts of gluten-free foods due to exceptional voracious appetite. Low serum calcium, phosphorus and vitamin D were found on admission and were corrected with appropriate parenteral and oral administration. Symptomatic hypocalcemia can occur in the context of celiac crisis and/or refeeding syndrome, a disorder characterized by the sudden shift in fluids and electrolytes that occurs in malnourished patients, who are refed without restrictions. Newly diagnosed malnourished patients with CD should be advised to increase their oral intake gradually, and to closely monitor their serum electrolytes.