Celiac Crisis Research Articles

A Case of Celiac Crisis in an Elderly Male

Celiac disease (CD) has many faces and can present in many ways. Celiac crisis is one such rare manifestation of the disease. Celiac crisis is characterized by watery diarrhea and life-threatening electrolyte abnormalities requiring hospitalization. We present a case of the diagnostic dilemma in an elderly male, who presented with anemia and was diagnosed with celiac disease only after developing so-called celiac crisis. A 76-year-old male with a history of alcoholism, chronic pancreatitis and rheumatoid arthritis was admitted to our hospital with anorexia and significant weight loss. Initial work-up revealed marked microcytic anemia with a hemoglobin of 6g/dl. History did not suggest gastrointestinal bleeding, however patient reported lifetime constipation. Other laboratory work-up showed a low iron level, ferritin >2000, and an elevated ESR and CRP. A subsequent upper endoscopy and colonoscopy was unremarkable with no identifiable bleeding source. There were no mucosal changes noted and no biopsies were performed. The patient underwent extensive testing in the hospital looking for the etiology of the anemia and weight loss. At the end of two weeks, he developed profuse watery diarrhea with severe electrolyte derangements. Infectious work-up including a stool Clostridium difficile toxin was negative. Stool studies were consistent with secretory diarrhea. Patient underwent repeat upper endoscopy and colonoscopy, which showed cobblestoning of duodenal mucosa and mucosal edema throughout the colon. TTG of the serum was positive for celiac disease. Histopathology of the biopsy showed villous blunting of the duodenum consistent with celiac disease. The patient received parenteral fluid and nutrition replacement and his diarrhea resolved with a gluten free diet. CD is known for its atypical presentations and it still remains under-diagnosed. Celiac crisis is a rare life-threatening syndrome where celiac disease causes acute diarrhea and severe metabolic derangements. General immune stimuli such as infection, surgery or pregnancy have been known to precipitate celiac crisis. It is unknown why a small subset of patients with CD develops celiac crisis.2581_A Figure 1. Normal appearing duodenal mucosa on Endoscopy #12581_B Figure 2. Cobblestoning apperance of Duodenal mucosa on endoscopy #2, 2 weeks later.2581_C Figure 3. Histopathology of Duodenal Biopsy showing Villous blunting and lymphocytesOur case is an example of the heterogeneous clinical course of CD and emphasizes the need to consider it in the differential diagnosis in elderly presenting with anemia and weight loss. Delay in diagnosis can lead to complication such as celiac crisis. Treatment for Celiac crisis is gluten free diet and steroids.

P124 Celiac crisis should still be borne in mind

P124 Celiac crisis should still be borne in mind

Celiac crisis, a rare and profound presentation of celiac disease: a case report

BackgroundCeliac crisis is a life-threatening manifestation of celiac disease and is rare in adults, with only a handful of cases documented worldwide and mostly in children.Case presentationA profoundly emaciated 43-year-old female presented with profuse diarrhoea, shortness of breath, left leg swelling with ulceration and immobility (Body Mass Index (BMI) = 14.7 kg/m2). The patient had normal anion-gap metabolic acidosis (pH = 7.16) with persisting hypokalemia, hyponatremia, hypomagnesemia and hypophosphatemia. In addition, severe vitamin deficiencies and coagulopathy were present. A computed tomography pulmonary angiogram (CT-PA) revealed bilateral massive pulmonary embolism causing infarction, arising from a left lower limb extensive deep vein thrombosis (DVT). Bone marrow suppression was seen on aspirate. The patient developed severe urosepsis in her immunocompromised state. Prolonged lower limb weakness despite supportive therapy, rehabilitation and strict adherence to a gluten-free diet prompted the magnetic resonance imaging (MRI) confirmed diagnosis of subacute combined degeneration of the spinal cord due to Vitamin-B12 deficiency.ConclusionsCeliac crisis is a rare and potentially life-threatening presentation of celiac disease, often a diagnosis of exclusion. Subacute combined degeneration of the spinal cord should be considered in patients with chronic Vitamin B12 deficiency presenting with neurological symptoms.

Refeeding syndrome in adults with celiac crisis: a case report

BackgroundRefeeding syndrome is a rare and life-threatening pathology with polyvisceral manifestations occurring in severely malnourished patients. It is rarely described in adults with celiac disease.Case presentationWe report the case of a 28-year-old Tunisian woman followed up for celiac disease, who did not adhere to the gluten-free diet. She presented to our hospital with celiac crisis manifested by severe diarrhea, and metabolic and electrolyte disturbances. The treatment of electrolyte abnormalities, hydration, and nutritional support was marked by the occurrence on the fifth day of refeeding syndrome with psychomotor agitation followed by respiratory distress and a state of cardiogenic shock.ConclusionsRefeeding syndrome is still under-recognized. It should be systematically prevented for high-risk patients. Nutritional support in patients with celiac crisis should be monitored carefully since the risk of refeeding syndrome is very high with a poor prognosis.

Non-responsive coeliac disease: Coeliac crisis vs. refractory coeliac disease with response to corticosteroids

Non-responsive coeliac disease: Coeliac crisis vs. refractory coeliac disease with response to corticosteroids

Celiac Crisis: A Rare Presentation of Celiac Disease in Adults

Background: Celiac Disease is an immune-mediated enteropathy resulting from an inappropriate and exaggerated response to dietary gluten [1, 2]. Celiac Crisis is an un-common, life-threatening condition resulting from acute severe diarrheal illness, severe metabolic disturbances, and malabsorption that is usually found in children, with only 22 cases reported in adults [3]. Here we report a case of celiac crisis in a middle aged male with characteristic endoscopic and pathological findings. Case Report: A 58-year-old male with previous history of left facial crush injury necessitating several reconstructive surgeries in 2012 presented with profuse, watery, foul-smelling diarrhea of 2 months duration with episodic fatigue, melena, and 20 kg weight loss. Since his accident in 2012 he had intermittent episodes of loose stools that got significantly worse within 2 months. He had also been diagnosed with Clotridium difficle colitis and received 14 days of oral antibiotics one month prior to his presentation. Physical exam revealed pallor and cachexia. Laboratory findings showed hemoglobin of 4.7 g/dL, INR greater than 10 (without anticoagulation), albumin 1.7 g/dL, potassium 2.9 mmol/L, undetectable levels of fat soluble vitamins, and negative stool studies. Esophagogastroduodenoscopy revealed diffuse granular mucosa in the duodenum with fissuring and scalloping appearance [Fig 1]. Pathologic examination showed severe villous atrophy and hypoplasia (March IV) with increased intra-epithelial lymphocytes [Fig 2]. IgA anti-tissue transglutaminase antibodies titers were >200 U/mL (normal, 0-19 U/mL). He was diagnosed with celiac disease presenting with celiac crisis and was started on a gluten-free diet with vitamin supplementations, following which he made a full recovery.Figure 1Figure 2Discussion: Celiac crisis has been described in association with or following surgery, pregnancy, immunosuppressive therapy, and infection. The probable triggering factor in our patient was clostridium difficle infection. However, another association can also be made between the patient's head trauma and triggering of celiac disease. This link was investigated by a Swedish study, which found a very small excess risk for future celiac disease in individuals with an earlier head trauma [4].Figure 3

Celiac crisis in an adult type 1 diabetes mellitus patient presented with diarrhea, weight loss and hypoglycemic attacks

Type 1 diabetes mellitus (T1DM) is an autoimmune disease, characterized by loss of the insulin-producing β cells of the islets of Langerhans in the pancreas, causing insulin deficiency. Celiac disease has been seen in 3 to 8 % of T1DM patients. Celiac crisis, an acute severe onset of celiac disease, is a rare and life-threatening manifestation. We report a 50-year-old man with type 1 diabetes mellitus who arrived at our service with a 2-month history of watery diarrhea associated with hypoglycemic attacks, abdominal pain, and weight loss of 13 kg. The diagnosis of celiac crisis was made based on diarrhea leading to dehydration, severe metabolic and electrolyte abnormalities, and subsequent improvement after introduction of a gluten-free diet.

Celiac crisis in children in Serbia.

BackgroundTo assess the prevalence and risk factors of celiac crisis (CC) in children with classical celiac disease (CD).MethodsThis retrospective study comprised 367 children with classical CD diagnosed from 1994 to 2015. The diagnosis of CD was based on the revised ESPGHAN criteria and CC on acute worsening and rapid progression of chronic diarrhea and vomiting followed by severe dehydration, multiple metabolic derangements and a marked decrease of body weight.ResultsCeliac crisis was confirmed in six (1.63 %) children, five in the first and one in the second year of life. In three patients CC was precipitated by rotavirus and in one by Salmonella enteritidis infection, while in the remaining two, except for a too long-standing disease and severe malnutrition, no additional causes of CC were found.ConclusionCeliac crisis in Serbia is still-present in children exclusively below the second year of life as a spontaneous or intestinal infection precipitated complication of previously unrecognized CD.

A Celiac Crisis in an Adult: Raising Awareness of a Life-threatening Condition.

ObjectivesThe Authors report the case of a 56-year-old man with celiac disease, who after ingesting a food containing gluten and experiencing a flu-like syndrome, developed severe diarrhea, vomiting, weight loss (15 kg), hypotension, renal dysfunction, hypokalemia and metabolic acidosis.Materials and MethodsAdmission to the Intensive Care Unit and exclusion of an infectious cause was determined.ResultsAfter receiving noradrenaline, methylprednisolone and correction of ionic disturbances, the patient recovered rapidly and had no further complication.ConclusionThe authors intend to increase awareness of celiac crisis, because despite being extremely rare in adults, it is potentially fatal and an quick diagnosis and treatment are crucial.LEARNING POINTSCeliac crisis is an acute, life-threatening presentation of celiac disease.Its clinical presentation consists of severe diarrhea, metabolic disturbances (namely hypokalemia, hyponatremia, hypomagnesemia, hypocalcemia and metabolic acidosis), hypoproteinemia and dehydration.Hospitalization and correction of metabolic imbalance with intravenous fluids are required, with corticotherapy being necessary in some cases.

A Case with Acute Celiac Crisis

Colyak hastaliginin belirti ve bulgulari genis bir yelpazeye yayilir. Bazi hastalar asemptomatik olabildigi gibi bazi hastalarda ise hastaligin cok nadir ve ciddi bir komplikasyonu olan colyak krizi gorulebilmektedir. Burada genel durum bozuklugu hipokalemi, hipoalbuminemi klinigi ile basvuran; glutensiz diyet, steroid tedavisi ve elektrolit replasmani ile klinik bulgulari duzelen bir olgu sunulmustur. Ozellikle kronik ishali olan ve hiponatremi, hipokalemi, hipoalbuminemi, metabolik asidoz tablosunda gelen hastalarin ayirici tanisinda colyak hastaligina ait nadir gorulen ve mortalitesi yuksek bir komplikasyon olan akut colyak krizi goz onunde bulundurulmali ve en kisa surede tedaviye baslanmalidir

Celiac Crisis: A Rare Manifestation of a Common Disease

Introduction: Celiac crisis is a rare and life threatening manifestation of celiac disease. Case presentation: We present a 46 year old male with a history of DM1 complicated by ESRD on HD and a recent diagnosis of Bell's Palsy who was admitted from a dialysis center with hypotension and voluminous diarrhea. Reported up to 20 watery, nonbloody bowel movements per day x 7 days. The diarrhea was associated with LLQ crampy pain. Denied fevers. +Nocturnal diarrhea. The diarrhea did not improve with fasting. Denied recent weight loss.Figure 1Figure 2Figure 3Vital Signs and PE: Afebrile BP 55/42 Pulse 102. Dry mucous membranes. Mild LLQ tenderness with hyperactive bowel sounds. A rectal tube was in place with voluminous light brown watery stool identified. Inital laboratory results and imaging. 6.8/11\173. 130 | 98 | 98. 4.6 | 19 | 13.4. pH 7.27 Alb 2.9 Ca 7.2 C. Diff PCR negative CT abdomen and pelvis with IV contrast revealed mild thickening of proximal small bowel loops, suspicious for enteritis. Further pertinent labs: Stool 0 + P and culture negative. Lactoferrin: positive. ESR and CRP elevated. TTG IgA 80 (0-19). Gliadin IgA 39 (0-19). Stool Osmolar gap 36 Gastrin and VIP normal 5HIAA excretion normal. Over a 24 hour period, the patient required 13 L's of IVF's to keep up with GI losses and maintain blood pressure. An esophagogastroduodenoscopy revealed scalloping in the duodenal bulb and 2nd part of the duodenum. Biopsies were obtained and revealed villous atrophy, increased intraepithelial lymphocytes, and crypt hyperplasia (Marsh 3C). The diagnosis of celiac crisis was made based on the endoscopic and serologic results in conjuction with the dramatic presentation. After 3 days of gluten withdrawal our patients bowel movement frequency and caliber improved dramatically. Discussion: In this rare manifestation of celiac disease marked metabolic derangements are encountered. It is unclear what triggers this more aggressive disease course, however it has been suggested to be a combination of severe mucosal inflammation and immune activation.1 In our case, we propose that his recent diagnosis of Bell's Palsy precipitated an immune activation leading to the crisis. Celiac disease should be considered in adults presenting with acute voluminous diarrhea and profound electrolyte abnormalities.

Hypokalemic paraparesis: Presenting feature of previously undiagnosed celiac disease in celiac crisis.

Sir, Celiac crisis is a life-threatening cause of acute diarrhea and multiple metabolic emergencies.[1] Clinically, it is characterized by severe diarrhea, dehydration and metabolic disturbances such as hypokalemia, hypomagnesemia, hypocalcemia, hypoproteinemia, etc. Various precipitating factors identified for the crisis are severe malnutrition, infections, hypoproteinemia, and poor compliance with gluten-free diet, bacterial overgrowth in setting of altered motility in celiac disease and anticholinergic drugs. Hypokalemia, a prominent feature in celiac crisis can lead to muscle weakness. Potassium is needed to repolarize the cell membrane to a resting state after an action potential has passed. Lower potassium levels in the extracellular space cause hyperpolarization of the resting membrane potential. This hyperpolarization is caused by the effect of the altered potassium gradient on resting membrane potential. As a result, a greater than normal stimulus is required for depolarization of the membrane to initiate an action potential leading to muscle paralysis. Paralysis usually starts in the legs and progresses to involve the upper limbs. There are only a few reports of celiac crisis presenting as quadriparesis and that too in adults.[2,3,4] We present a 3 years old thriving boy who was admitted with complaints of watery diarrhea and vomiting with dehydration. He was managed with 75 ml/kg RL correction with which dehydration improved, but loose stools persisted though the frequency decreased. On day 2 of admission, the child developed flaccid paraparesis with abdominal distension attributable to hypokalemia. Laboratory reports of electrolytes revealed severe hypokalemia (1.4 meq/L), which was confirmed by an electrocardiogram. Hence, the child was given rapid potassium correction at the rate of 0.3 meq/kg/h for 3 h with subsequently maintenance therapy up to 4 meq/kg/day. Despite potassium replacement paraparesis and hypokalemia did not show improvement. Even with repeated rapid corrections and maintenance dose increased to 6 meq/kg/day hypokalemia, as well as paraparesis persisted (serial potassium values of 1.4, 1.7, 2.3, 2.9, 2.1, 3.2 meq/L) and was accompanied by hyponatremia (serum sodium 124, 131, 134 meq/L) and glycosuria. Review of history elicited intermittent loose stools. At this point of time, the possibility of the celiac crisis was raised. Plasma tissue transglutaminase IgA was returned positive (22.14 U/ml). With a diagnosis of the celiac crisis, the child was started on steroids and gluten-free diet after which he showed significant improvement. A duodenal biopsy in a referral hospital confirmed the diagnosis of celiac disease. The child is currently doing well on a gluten-free diet. Timely diagnosis of such a case is the key to prompt management. Though corticosteroids are the mainstay of management, supportive care, particularly management of electrolyte imbalance is very important.

Unresponsive and refractory hypokalemia in celiac crisis- A predictor of high mortality

Celiac disease is an immune mediated disorder elicited by the ingestion of gluten in genetically susceptible person and associated with some HLA subtypes. It is characterized by chronic inflammation of small intestine associated with villous atrophy. Symptoms usually appear when weaning from breast milk is done and gluten is introduced in diet. Failure to thrive, chronic diarrhea, vomiting abdominal distension are usual symptoms. Celiac crisis is rare but well known complication of celiac disease presenting with various systemic and metabolic manifestations like hypokalemia, hyponatremia, shock and bleeding diathesis. Celiac crisis usually has some underlying precipitating factor and is more common in patient with noncompliance with gluten free diet. We report a case of 14 year old female child, a known case of celiac disease having the poor compliancewith gluten free diet, presenting in celiac crisis with shock, refractory hypokalemia and bleeding diathesis.

Unusual Case of Celiac Disease

Introduction: Celiac crisis is an uncommon presentation in adults. While serology is sensitive and specific, it can be negative in some patients, which make intestinal biopsy indispensable in diagnosis. An 80-year-old woman presented to the hospital with loose stools; 15 episodes /day for 1 week, and increasing weakness. She had 10-year history of 3-4 loose stools/day. She has no significant past medical or family history, nor recent travel history. Labs showed low sodium, potassium, chloride, metabolic acidosis, and acute renal failure. She was initially treated with intravenous normal saline and bicarbonate; however, diarrhea continued. Investigations showed elevated stool osmotic gap and negative stool cultures for bacteria, ova, and parasites. Anti-endomysial (EMA) and anti-tissue transglutaminase (TTG) antibodies and 5HIAA were normal. Colonoscopy demonstrated normal colonic mucosa; however, random biopsies demonstrated lymphocytic colitis and increased intraepithelial lymphocytes. Small bowel biopsy showed partial villous atrophy, crypt hyperplasia, and increased inflammatory infiltrate consistent with celiac disease. She was started on a gluten-free diet, and over the subsequent weeks, she had resolution of symptoms. Two interesting aspects of this case are that she presented with celiac crisis, which is rare in adults, and diagnosis depended solely on biopsy because she did not have antibodies to EMA and TTG. Celiac crisis is a life-threatening syndrome of unclear etiology. Manifestations include diarrhea and electrolyte disturbances. It was thought to occur exclusively in patients younger than 2 years of age. The association between microscopic colitis and celiac disease may be 1 factor that contributes to the development celiac crisis due to defective active and passive absorption of sodium and chloride, and reduced chloride-bicarbonate exchange. Diagnosis of celiac disease is based on serologic tests of specific antibodies, including EMA and TTG antibodies, which are highly specific and sensitive (95% and 96%). It was found that 7% of patients were negative for both auto-antibodies despite having normal IgA levels. Intestinal biopsy is the gold standard for diagnosis because EMA and TTG antibodies decrease in patients with partial villous atrophy. Histological criteria are increased intraepithelial lymphocyte infiltrate, crypt hyperplasia, and villous atrophy. Gluten-free diet is the mainstay of treatment with dramatic response and improvement in symptoms. Although celiac crisis is an uncommon presentation in adults, it should be considered in patients presenting with unexplained severe diarrhea and metabolic acidosis, even if EMA and TTG antibodies are negative.

Celiac Crisis as a First Presentation of Celiac Disease: A Life-Threatening Syndrome

Celiac Crisis as a First Presentation of Celiac Disease: A Life-Threatening Syndrome

PO-0145 Celiac Disease – The Experience Of A Single Centre In 29 Years

Aims The study aimed to evaluate the epidemiological and clinical profile of the patients diagnosed with celiac disease in ‘Grigore Alexandrescu’ Children’s Hospital over a 29 years period. Methods We performed a retrospective study including 224 patients diagnosed with celiac disease, from January 1985 until December 2013. Three groups resulted. The first group included patients diagnosed from 1985 until 1995, before celiac serology was available, the second group patients diagnosed from 1996 until 2006 using qualitative antitissue-transglutaminase antibodies and the third group patients diagnosed from 2007 until 2013 using quantitative antitissue-transglutaminase antibodies and HLA DQ2/DQ8 typing. From the medical records we extracted age and clinical characteristics of patients at presentation. Results The groups included 1.8, 13.8 and 7.7 patients/year. The mean age at diagnosis was 5.9 ± 4.7, 4.4 ± 3.6 and 4.4 ± 3.5 years respectively. Gastrointestinal symptoms were described in 100%, 86.8% and 59.2%, particularly chronic diarrhoea in 90%, 51.9% and 48.1%. Twenty percent of patients in the first group presented with celiac crisis, only 0.6% in the second group and none in the third. Non-gastrointestinal symptoms were not observed in the first group and were reported in the second and third groups in 13.1% and 61.1%. Conclusion Since 2007 a decreasing tendency in the number of patients diagnosed/year was observed. Gastrointestinal symptoms were initially the sole clinical finding, particularly chronic diarrhoea, but in time patients presented with non-gastrointestinal symptoms or oligosymptomatic/asymptomatic celiac disease with an increasing frequency.

Celiac crisis in children: A case report and review of the literature

Celiac crisis is a complication associatedwith celiac disease that could cause the death of the patient if left untreated. A 7-year-old boy of Pakistani origin had an acute onset of severe diarrhoea, vomiting started 2 weeks before, with a refusal to feed, dehydration and pedal edema. Hewasunderweight. Hehad coarse scaly dry skin anddistended soft abdomen. His investigations revealed hypocalcemia, normal anion-gab metabolic acidosis, hypoproteinemia and elevated transaminasis. The serum tissue transglutaminase antibodies titres came elevated. An endoscopy biopsy of duodenum revealed a Marsh score 3b, confirming the diagnosis of celiac crisis. Oral prednisone (2mg/kg/die) was started and was tapered after 15 days. A glutenfree diet was also started. He had a marked clinical improvement. Celiac crisis is a rare condition in the Western countries, as the majority of the case reports come from the developing countries where other causes of chronic diarrhoea are more frequent. Celiac crisis presented with severe diarrhoea, vomiting, dehydration and shock. It is associated to metabolic disturbances and it may also occur with neurologic abnormalities. The treatment of celiac crisis is still a matter of debate. It can be treated only with a gluten-free diet. Steroid therapy can be added or started simultaneously to the gluten-freediet. Steroids controlmucosal inflammationand restore the brush border enzymes. Our case report highlights physician to consider celiac disease in children with no typical symptoms to avoid the life-treating crisis. Rapid serologic test support the diagnosis. Steroid therapy may be life saving.

Crise celíaca e diátese hemorrágica em um adulto

AIMS: Case-report of a patient with a rare presentation of celiac disease. CASE DESCRIPTION: A 49 year-old woman, with a long-standing course of diarrhea and weight loss of 35 kg in a three-year period, presented to the emergency department complaining of an increase in diarrheal depositions, hematemesis, melena, lethargy, anasarca and syncope episodes. After clinical evaluation, upper gastrointestinal endoscopy and laboratorial exams, a rare illness known as celiac crisis was diagnosed. This is a rare presentation of celiac disease, associated with profuse diarrhea and severe metabolic/electrolyte disturbances, more frequent in children under two years, and rarely described in adults. Conclusions: Celiac disease usually manifests itself through chronic diarrhea, abdominal pain and weight loss, but celiac crisis and hemorrhagic diathesis, although rarely, may also occur.

Çölyak Kriziyle Başlangıç Gösteren Bir Gluten Enteropatisi

Gluten duyarlı enteropati, insanlarda en sık görülen otoimmün karakterli ve gıda kökenli ince barsak mukoza hastalığıdır. Çölyak krizi ise yaşamı tehdit eden ve çölyak hastalığının nadir görülen bir komplikasyonudur. Bu komplikasyon klinik olarak; hipokalemi, hipomagnezemi, hipokalsemi, hipoproteinemi, demir ve folik asit eksikliğine yol açan ağır derecede ishal, dehidrasyon ve metabolik değişikliklerle karakterize bir tablodur. Çölyak krizi hastalığın nadir görülen ciddi bir komplikasyonudur. Otuz yedi yaşındaki kadın olgu, ishal ve kilo kaybı ile acil servise başvurdu. Laboratuvar tetkikleri ve ileri değerlendirme ile hastaya çölyak hastalığı ve akut krizi tanısı kondu. Bu olgu sunumunda, çölyak hastalığının tipik özellikleri ve nadir bir komplikasyonu olan çölyak krizi literatür bilgileri ışığı altında sunulmuştur.

Celiac crisis in an adult type 1 diabetes mellitus patient: a rare manifestation of celiac disease

Celiac crisis, an acute severe onset of celiac disease, is a rare and life-threatening manifestation. We report a 30-year-old woman with type 1 diabetes mellitus who arrived at our service with one-month history of severe acute watery diarrhea associated with nausea, vomiting, abdominal pain, and weight loss of 9 kg. The diagnostic hypothesis of celiac crisis was reached based on profuse diarrhea leading to dehydration, severe metabolic and electrolyte abnormalities, and subsequent improvement after introduction of a gluten-free diet.