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Severe rhabdomyolysis and celiac disease
e272
Abstracts / Digestive and Liver Disease 47S (2015) e237–e276
P095 GLUTEN PSYCHOSIS: A CASE REPORT AND REVIEW OF LITERATURE E. Lionetti 1,∗ , S. Leonardi 1 , C. Franzonello 1 , M. Mancardi 2 , M. Ruggieri 1 , C. Catassi 3 1
Dip. Pediatria, Università di Catania, Catania, Italy Unità di Neuro-pschiatria Infantile, Istituto G. Gaslini, Genova, Italy 3 Dip. Pediatria, Università Politecnica delle Marche, Ancona, Italy 2
Objectives: A singular report of non-celiac gluten sensitivity (NCGS) presenting with hallucinations has been described in an adult patient. We report the first pediatric case of a psychotic disorder clearly related to NCGS and investigate the causes by a review of literature. Case report: A 12 year-old girl reported daily headache, and behavior alterations. One month after, she began to have complex hallucinations. Screening for infectious, metabolic, autoimmune, and neurologic diseases resulted negative. A psychotic disorder was diagnosed. Treatment with a second-generation anti-psychotic was started but psychotic symptoms persisted. After 2 years, due to gastro-intestinal complaints, a nutritionist was consulted, and a gluten-free diet (GFD) was recommended; within a week both gastro-intestinal and psychiatric symptoms dramatically improved. After 5 months she was admitted to our outpatient. We performed a double-blind challenge test with wheat and rice flour. During the administration of rice flour, symptoms were absent. During the second day of wheat flour intake, the girl presented headache, halitosis, abdominal distension, mood disorders, and three episodes of severe hallucinations. After the challenge she tested negative for: CD serology, food specific-IgE, skin prick test to wheat, atopy patch test to wheat, duodenal biopsy. A GFD was started with a complete regression of all symptoms. Nine months after she is still symptoms-free. Conclusions: The present case-report confirms that psychosis may be a manifestation of NCGS, and may involve also children. Well-designed prospective studies are needed to establish the real role of gluten as a triggering factor in neuro-psychiatric disorders. http://dx.doi.org/10.1016/j.dld.2015.07.143 P096 SEVERE RHABDOMYOLYSIS AND CELIAC DISEASE C. Mandato 1,∗ , A. Rossi 1 , M. Lamba 1 , M. Caldore 1 , P. Siani 1 , P. Vajro 2 1 Pediatrics, AORN Santobono-Pausilipon-Naples, Italy 2 Pediatrics, Department of Medicine and Surgery, University of Salerno, Baronissi (Sa), Italy
Introduction: Rhabdomyolysis is a rare, potentially lifethreatening condition, caused by infections, trauma, extreme exertion, drugs, toxins, venoms, hyper/hypothermia, genetic/metabolic, endocrine and electrolyte disorders. Celiac disease (CD) has been rarely reported, and in these cases muscolar damage was imputed to hypokalemia. We describe a case of normokalemic rhabdomyolisis associated with the presence of anti Transglutaminase Ig A Ab (AntiTGase), and review previous reports. Case report: A previously clinically well 3 y.o. boy was referred for brown urine, muscular pain/weakness, and no history of muscular trauma. At entry lab tests showed elevated levels of Creatine
Kinase (CK) 37,891 U/L, Aspartate Aminotransferase (AST) 525 U/L; electrolytes were within normal range. 24 h after admission serum CPK peaked 115,000 U/L and transaminases increased (AST 1,507 U/L, ALT 523 U/L). Hyperhydration treatment was started and renal function monitored. Urine output decreased little, while serum creatinine and urea nitrogen remained near normal. The patient completely recovered and was discharged on day 16. Main causes of rhabdomyolysis were ruled out by appropriate tests. Because of the reported cases of CD/rhabdomyolysis, anti TGase Abs were measured and found positive [IgA 34 U/ml]. HLA was DQA1 02 05, DQB1 02 03. Digiunal biopsy is in progress. Discussion and conclusion: The 1st case of rhabdomyolysis/CD was reported in 1982; since then, only less than a dozen (mostly adult) cases followed. Our pediatric pt suggests to check CD in children with severe rhabdomyolysis, also in absence of classical CD symptoms. As rhabdomyolysis itself may elevate the serum [K], hypokalemia might go unrecognized as the cause of muscular damage. http://dx.doi.org/10.1016/j.dld.2015.07.144 P097 TISSUE TRANSGLUTAMINASE ANTIBODY CUT-OFF AND DIAGNOSIS OF CELIAC DISEASE G. Raimondi 1,∗ , I. Mezzina 1 , M.E. Tripaldi 1 , S. Castellaneta 3 , F. Cristofori 2 , C. Ciullo 1 , R. Francavilla 1 1
Dip. Interdisciplinare di Medicina, Sezione di Pediatria. Università di Bari, Italy 2 UOC Pediatria. P.O. Centrale Santissima Annunziata Taranto, Italy 3 Dip. di Pediatria P.O. San Paolo, Bari, Italy Background: It has been reported a correlation between tissue transglutaminase antibodies titers and degrees of duodenal biopsy, and based on this, ESPGHAN guidelines have proposed that duodenal biopsy can be omitted in same patients with high levels of tTg antibodies (N10 x upper normal limit). Objective: To evaluate the relationship between tTg antibody levels and histologic Marsh grading and to identify a lower value of tTG for which intestinal biopsy can be avoided. Methods: A retrospective analysis of 524 celiac pediatric patients with positive tTG and small bowel biopsy were included. The positive predictive values (PPV) for different cut-off points of transglutaminase levels for the diagnosis of celiac disease was assessed. Results: We studied 524 patients (322 women – 61%) diagnosed in our Centre in the last ten years. The mean ± SD age of the patients was 11.6 years (range 3.8–18 years). 515 out of 524 patients had villous atrophy (Marsh III). Abdominal pain, diarrhea and bloating were the first three common symptoms and anemia was the most common sign in this patients. Mean tTG titers was significantly higher in patients graded as Marsh III as compared to Marsh II and I (11.2 UNL vs. 5.1 UNL; p < 0.001). Our results showed that tTg titer more than 6 folds higher then the kit’s cut-off value have a specificity of 96% and a positive predictive value of 99.7% for Marsh III lesion. Conclusion: There is a clear correlation between tTg titers and degrees of duodenal damage in patients with celiac disease. We propose that, in case of high clinical suspicion, when tTg level is more than 6 folds higher than the manufacture’s recommended cut-off value, duodenal biopsy can be avoided. http://dx.doi.org/10.1016/j.dld.2015.07.145
Abstracts / Digestive and Liver Disease 47S (2015) e237–e276
P095 GLUTEN PSYCHOSIS: A CASE REPORT AND REVIEW OF LITERATURE E. Lionetti 1,∗ , S. Leonardi 1 , C. Franzonello 1 , M. Mancardi 2 , M. Ruggieri 1 , C. Catassi 3 1
Dip. Pediatria, Università di Catania, Catania, Italy Unità di Neuro-pschiatria Infantile, Istituto G. Gaslini, Genova, Italy 3 Dip. Pediatria, Università Politecnica delle Marche, Ancona, Italy 2
Objectives: A singular report of non-celiac gluten sensitivity (NCGS) presenting with hallucinations has been described in an adult patient. We report the first pediatric case of a psychotic disorder clearly related to NCGS and investigate the causes by a review of literature. Case report: A 12 year-old girl reported daily headache, and behavior alterations. One month after, she began to have complex hallucinations. Screening for infectious, metabolic, autoimmune, and neurologic diseases resulted negative. A psychotic disorder was diagnosed. Treatment with a second-generation anti-psychotic was started but psychotic symptoms persisted. After 2 years, due to gastro-intestinal complaints, a nutritionist was consulted, and a gluten-free diet (GFD) was recommended; within a week both gastro-intestinal and psychiatric symptoms dramatically improved. After 5 months she was admitted to our outpatient. We performed a double-blind challenge test with wheat and rice flour. During the administration of rice flour, symptoms were absent. During the second day of wheat flour intake, the girl presented headache, halitosis, abdominal distension, mood disorders, and three episodes of severe hallucinations. After the challenge she tested negative for: CD serology, food specific-IgE, skin prick test to wheat, atopy patch test to wheat, duodenal biopsy. A GFD was started with a complete regression of all symptoms. Nine months after she is still symptoms-free. Conclusions: The present case-report confirms that psychosis may be a manifestation of NCGS, and may involve also children. Well-designed prospective studies are needed to establish the real role of gluten as a triggering factor in neuro-psychiatric disorders. http://dx.doi.org/10.1016/j.dld.2015.07.143 P096 SEVERE RHABDOMYOLYSIS AND CELIAC DISEASE C. Mandato 1,∗ , A. Rossi 1 , M. Lamba 1 , M. Caldore 1 , P. Siani 1 , P. Vajro 2 1 Pediatrics, AORN Santobono-Pausilipon-Naples, Italy 2 Pediatrics, Department of Medicine and Surgery, University of Salerno, Baronissi (Sa), Italy
Introduction: Rhabdomyolysis is a rare, potentially lifethreatening condition, caused by infections, trauma, extreme exertion, drugs, toxins, venoms, hyper/hypothermia, genetic/metabolic, endocrine and electrolyte disorders. Celiac disease (CD) has been rarely reported, and in these cases muscolar damage was imputed to hypokalemia. We describe a case of normokalemic rhabdomyolisis associated with the presence of anti Transglutaminase Ig A Ab (AntiTGase), and review previous reports. Case report: A previously clinically well 3 y.o. boy was referred for brown urine, muscular pain/weakness, and no history of muscular trauma. At entry lab tests showed elevated levels of Creatine
Kinase (CK) 37,891 U/L, Aspartate Aminotransferase (AST) 525 U/L; electrolytes were within normal range. 24 h after admission serum CPK peaked 115,000 U/L and transaminases increased (AST 1,507 U/L, ALT 523 U/L). Hyperhydration treatment was started and renal function monitored. Urine output decreased little, while serum creatinine and urea nitrogen remained near normal. The patient completely recovered and was discharged on day 16. Main causes of rhabdomyolysis were ruled out by appropriate tests. Because of the reported cases of CD/rhabdomyolysis, anti TGase Abs were measured and found positive [IgA 34 U/ml]. HLA was DQA1 02 05, DQB1 02 03. Digiunal biopsy is in progress. Discussion and conclusion: The 1st case of rhabdomyolysis/CD was reported in 1982; since then, only less than a dozen (mostly adult) cases followed. Our pediatric pt suggests to check CD in children with severe rhabdomyolysis, also in absence of classical CD symptoms. As rhabdomyolysis itself may elevate the serum [K], hypokalemia might go unrecognized as the cause of muscular damage. http://dx.doi.org/10.1016/j.dld.2015.07.144 P097 TISSUE TRANSGLUTAMINASE ANTIBODY CUT-OFF AND DIAGNOSIS OF CELIAC DISEASE G. Raimondi 1,∗ , I. Mezzina 1 , M.E. Tripaldi 1 , S. Castellaneta 3 , F. Cristofori 2 , C. Ciullo 1 , R. Francavilla 1 1
Dip. Interdisciplinare di Medicina, Sezione di Pediatria. Università di Bari, Italy 2 UOC Pediatria. P.O. Centrale Santissima Annunziata Taranto, Italy 3 Dip. di Pediatria P.O. San Paolo, Bari, Italy Background: It has been reported a correlation between tissue transglutaminase antibodies titers and degrees of duodenal biopsy, and based on this, ESPGHAN guidelines have proposed that duodenal biopsy can be omitted in same patients with high levels of tTg antibodies (N10 x upper normal limit). Objective: To evaluate the relationship between tTg antibody levels and histologic Marsh grading and to identify a lower value of tTG for which intestinal biopsy can be avoided. Methods: A retrospective analysis of 524 celiac pediatric patients with positive tTG and small bowel biopsy were included. The positive predictive values (PPV) for different cut-off points of transglutaminase levels for the diagnosis of celiac disease was assessed. Results: We studied 524 patients (322 women – 61%) diagnosed in our Centre in the last ten years. The mean ± SD age of the patients was 11.6 years (range 3.8–18 years). 515 out of 524 patients had villous atrophy (Marsh III). Abdominal pain, diarrhea and bloating were the first three common symptoms and anemia was the most common sign in this patients. Mean tTG titers was significantly higher in patients graded as Marsh III as compared to Marsh II and I (11.2 UNL vs. 5.1 UNL; p < 0.001). Our results showed that tTg titer more than 6 folds higher then the kit’s cut-off value have a specificity of 96% and a positive predictive value of 99.7% for Marsh III lesion. Conclusion: There is a clear correlation between tTg titers and degrees of duodenal damage in patients with celiac disease. We propose that, in case of high clinical suspicion, when tTg level is more than 6 folds higher than the manufacture’s recommended cut-off value, duodenal biopsy can be avoided. http://dx.doi.org/10.1016/j.dld.2015.07.145