Gastrointestinal Symptoms in Lysosmal Disease

 How to Cite this Article: Imanzadeh F. Gastrointestinal Symptoms in Lysosmal Disease. Iran J Child Neurol Autumn 2012; 6:4 (suppl. 1):17-18.pls see PDF. References: 1. Semenza  GL,  Pyeritz  RE.  Respiratory complications of mucopolysaccharide storage disorders. Medicine (Baltimore) 1988; 67:209. 2.   Wraith   JE,   Scarpa   M,   Beck   M,   et   al. Mucopolysaccharidosis type II (Hunter syndrome): a clinical review and recommendations for treatment in the era of enzyme replacement therapy. Eur J Pediatr 2008; 167:267. 3. Stevens JM, Kendall BE, Crockard HA, Ransford   A.   The   odontoid   process   in Morquio-Brailsford’s disease. The effects of occipitocervical fusion. J Bone Joint Surg Br 1991; 73:851. 4.   Jones AE, Croley TF. Morquio syndrome and anesthesia. Anesthesiology 1979; 51:261. 5.   Ashraf J, Crockard HA, Ransford AO, Stevens JM. Transoral decompression and posterior stabilisation in Morquio’s disease. Arch Dis Child 1991; 66:1318. 6.  Neufeld EF, Muenzer J. The metabolic and molecular bases of inherited disease, Scriver C, Beaudet AL, Valle D, Sly W (Eds), McGraw- Hill, New York 2001. p.3421. 7.   Wraith   JE.   The   mucopolysaccharidoses:   a clinical review and guide to management. Arch Dis Child 1995; 72:263. 8.   Cleary MA, Wraith JE. The presenting features of mucopolysaccharidosis type IH (Hurler syndrome). Acta Paediatr 1995; 84:337. 9.   Elsner B. Ultrastructure of the rectal wall in Hunter’s   syndrome.   Gastroenterology   1970; 58:856. 10. Cleary   MA,   Wraith   JE.   Management   of mucopolysaccharidosis type III. Arch Dis Child 1993; 69:403.

Hepatic Encephalopathy: Early Diagnosis in Pediatric Patients With Cirrhosis

How to Cite This Article: Dara N, Sayyari AA, Imanzadeh F. Hepatic Encephalopathy: Early Diagnosis in Pediatric Patients With Cirrhosis. Iran J Child Neurol. 2014 Winter; 8(1):1-11.ObjectiveAs acute liver failure (ALF) and chronic liver disease (cirrhosis) continue to increase in prevalence, we will see more cases of hepatic encephalopathy.Primary care physician are often the first to suspect it, since they are familiar with the patient’s usual physical and mental status. This serious complication typically occurs in patients with severe comorbidities and needs multidisciplinary evaluation and care. Hepatic encephalopathy should be considered in any patient with acute liver failure and cirrhosis who presents with neuropsychiatric manifestations, decrease level of consciousness (coma), change of personality, intellectualand behavioral deterioration, speech and motor dysfunction.Every cirrhotic patient may be at risk; potential precipitating factors should be addressed in regular clinic visits. The encephalopathy of liver disease may be prominent, or can be present in subtle forms, such as decline of school performance, emotional outbursts, or depression.“Subtle form” of hepatic encephalopathy may not be obvious on clinical examination, but can be detected by neurophysiologic and neuropsychiatric testing.References:Ferenci P, Lockwood A, Mullen K, Tarter R, Weissenborn K, Blei AT. 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Gut 2008;57:1156-65.McClain CJ, Zieve L, Doizaki WM, Gilberstadt S, Onstad GR. Blood methanethiol in alcoholic liver disease with and without hepatic encephalopathy. Gut 1980;21:318–23.Guevara M, Baccaro ME, Torre A, Gómez-Ansón B, Ríos J, Torres F, et al. Hyponatremia is a risk factor of hepatic encephalopathy in patients with cirrhosis: a prospective study with time-dependent analysis. Am J Gastroenterol 2009;104:1382–9.Poveda MJ, Bernabeu A, Concepción L, Roa E, de Madaria E, Zapater P, et al. Brain edema dynamics in patients with overt hepatic encephalopathy A magnetic resonance imaging study. Neuroimage 2010;52:481-7.Bernthal P, Hays A, Tarter RE, Van Thiel D, Lecky J, Hegedus A. Cerebral CT scan abnormalities in cholestatic and hepatocellular disease and their relationship to neuropsychologic test performance. Hepatology 1987;7:107-14.Sugimoto R, Iwasa M, Maeda M, Urawa N, Tanaka H, Fujita N, et al. Value of the apparent diffusion coefficient for quantification of low-grade hepatic encephalopathy. Am J Gastroenterol 2008;103:1413–20.Häussinger D. Low grade cerebral edema and the pathogenesis of hepatic encephalopathy in cirrhosis. Hepatology 2006;43:1187–90.Masson S, Mardini HA, Rose JD, Record CO. Hepatic encephalopathy after transjugular intrahepatic portosystemic shunt insertion: a decade of experience. QJM 2008;101:493–501.Boyer TD, Haskal ZJ. American Association for the Study of Liver Diseases. The role of transjugular intrahepatic portosystemic shunt (TIPS) in the management of portal hypertension: update 2009. Hepatology 2010;51:306.Kircheis G, Knoche A, Hilger N, Manhart F, Schnitzler A, Schulze H, et al. Hepatic encephalopathy and fitness to drive. Gastroenterology 2009; 137:1706–1715.e1–9.Bajaj JS, Saeian K, Schubert CM, Hafeezullah M, Franco J, Varma RR, et al. Minimal hepatic encephalopathy is associated with motor vehicle crashes: the reality beyond the driving test. Hepatology 2009;50:1175–83.Hartmann IJ, Groeneweg M, Quero JC, Beijeman SJ, de Man RA, Hop WC, et al. The prognostic significance of subclinical hepatic encephalopathy. AmJ Gastroenterol 2000;95:2029–2034.Bustamante J, Rimola A, Ventura PJ, Navasa M, Cirera I, Reggiardo V, et al. Prognostic significance of hepatic encephalopathy in patients with cirrhosis. J Hepatol 1999;30:890–895.Murray KF, Carithers RL Jr; AASLD. AASLD practice guidelines: evaluation of the patient for liver transplantation. Hepatology 2005;41:1407–32.Ferenci P, Lockwood A, Mullen K, Tarter R, Weissenborn K, Blei A. Hepatic encephalopathy– definition, nomenclature, diagnosis, and quantification: final report of theworking party at the 11thWorldCongresses of Gastroenterology, Vienna, 1998. Hepatology 2002;35:716–21.Kugler CF, Taghavy A, Fleig WE,Hahn EG. 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A double blind controlled trial. Gastroenterology 1977;72(4 pt 1):573–83.Prasad S, Dhiman RK, Duseja A, Chawla YK, Sharma A, Agarwal R. Lactulose improves cognitive functions and health-related quality of life in patients with cirrhosis who have minimal hepatic encephalopathy. Hepatology 2007;45:549–59.Weber FL Jr. Effects of lactulose on nitrogen metabolism. Scand J Gastroenterol Suppl 1997;222:83–7.Morgan MY, Hawley KE. Lactitol vs. lactulose in the treatment of acute hepatic encephalopathy in cirrhotic patients: a doubleblind, randomized trial. Hepatology 1987;7:1278–84.Dawson AM, McLaren J, Sherlock S.Neomycin in the treatment of hepatic coma. Lancet 1957;273:1262–8.248.Atterbury CE, Maddrey WC, Conn HO. Neomycinsorbitol and lactulose in the treatment of acute portal systemic encephalopathy. A controlled, double-blind clinical trial. Am J Dig Dis 1978;23:398–406.44. Berk DP, Chalmers T. Deafness complicating antibiotic therapy of hepatic encephalopathy. Ann Intern Med 1970;73:393–6.Cabrera J, Arroyo V, Ballesta AM, Rimola A, Gual J, Elena M, et al. Aminoglycoside nephrotoxicityin cirrhosis. Value of urinary beta 2-microglobulin to discriminate functional renal failure from acute tubular damage. Gastroenterology 1982;82:97–105.Blei AT, Córdoba J; Practice Parameters Committee of the American College of Gastroenterology. Hepatic encephalopathy. Am J Gastroenterol 2001;96:1968–76.Rothenberg ME, Keeffe EB. Antibiotics in the management of hepatic encephalopathy: an evidence based review. Rev Gastroenterol Disord 2005;5(suppl 3):26–35.Murphy N, Auzinger G, Bernel W, Wendon J. The effect of hypertonic sodium chloride on intracranial pressure in patients with acute liver failure. Hepatology 2004;39:464-70.49. Charlton M. Branched-chain amino acid enriched supplements as therapy for liver disease. J Nutr 2006;136(suppl 1):295S–298S.Egberts EH, Schomerus H, Hamster W, Jürgens P. [Branched-chain amino acids in the treatment of latent porto-systemic encephalopathy. A placebo-controlled double-blind cross-over study] [in German]. Z Ernahrung swiss 1986; 25:9–28.Plauth M, Egberts EH, Hamster W, Török M, Müller PH, Brand O, et al. Long-term treatment of latent portosystemic encephalopathy with branched-chain amino acids. A double-blind placebo-controlled crossover study. J Hepatol 1993;17:308–14.Les I, Doval E, García-Martínez R, Planas M, Cárdenas G, Gómez P, et al. Effects of branched-chain amino acids supplementation in patients with cirrhosis and a previous episode of hepatic encephalopathy: a randomized study. Am J Gastroenterol 2011;106:1081-8.Schliess F, Görg B, Häussinger D. RNA oxidation and zinc in hepatic encephalopathy and hyperammonemia. Metab Brain Dis 2009;24:119–34.Efrati C, Masini A, Merli M, Valeriano V, Riggio O. Effect of sodium benzoate on blood ammonia response to oral glutamine challenge in cirrhotic patients: a note of caution. 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Considerations on the impact of hepatic encephalopathy treatments in the pretransplant setting. Transplantation 2010; 89:771–8.61. Senzolo M, Pizzolato G, Ferronato C, Chierichetti F, Boccagni P, Dam M, et al. Long-term evaluation of cognitive function and cerebral metabolism in liver transplanted patients. Transplant Proc 2009; 41:1295–1296.Butterworth RF. Neuronal cell death in hepatic encephalo- pathy. Metab Brain Dis 2007;22:309–320.DiMartini A, Chopra K. The importance of hepatic encephalo -pathy:pre-transplant and post-transplant. Liver Transpl 2009;15:121–3.Saner FH, Nadalin S, Radtke A, Sotiropoulos GC, Kaiser GM, Paul A. Liver transplantation and neurological side effects. Metab Brain Dis 2009;24:183-7.Sotil EU, Gottstein J, Ayala E, Randolph C, Blei AT. Impact of preoperative overt hepatic encephalopathy on neurocognitive function after liver transplantation. Liver Transpl 2009;15:184-92

Relationship between Celiac Disease and Refractory Idiopathic Epilepsy in Children

ObjectiveEpilepsy occurs with a yearly incidence of 40 per 100,000 children, of which more than 25% are resistant to drug therapy. Epilepsy may occur in autoimmunediseases like lupus, celiac disease and myasthenia gravis. In this study, therelationship between celiac disease and refractory epilepsy was evaluated inchildren with idiopathic epilepsy.Material & MethodsHundred-fifty-five children (mean age, 6.7±3.3 years) with idiopathic andcryptogenic epilepsy referred to the neurology clinic were studied in two groups;drug controlled epilepsy (control, 82 patients) and refractory epilepsy groups(case, 73 patients). Both groups underwent serological tissue transglutaminaseantibody measurement by ELISA. In seropositive cases, small intestine biopsywas conducted. Data analysis was performed using student's t test and 2 test.ResultsSeven (0.04%) patients had celiac disease based on a positive tissuetransglutaminase antibody and three patients (0.01%) based on a positive biopsy.Three patients (2.4%) with drug controlled epilepsy (control group) and fivewith refractory epilepsy (case group) had seropositive celiac disease (p=0.255).In the biopsy survey of six seropositive patients, one patient (1.2%) in the drugcontrolled epilepsy and two patients (2.7%) in the refractory epilepsy group hadpositive biopsy for celiac disease (p = 0.604). One seropositive patient did notcooperate for biopsy.ConclusionIf the relationship between celiac disease and epilepsy, especially in casesof symptomatic or oligosymptomatic celiac is proved, using gluten freediet increases the ability to control epilepsy particularly in refractory cases.We suggest celiac disease survey is not required in patients with idiopathicepilepsy

The Late presenting Bochdalek hernia; A Case report and review of literature

We report a 22-month-old boy who referred due to nausea, vomiting, abdominal pain and watery non-bloody diarrhea and after thorough evaluation, a large defect in the left postero-lateral side of diaphragm and presence of bowel loops, spleen, stomach and left lobe of liver in the left hemi-thorax were detected. So, he was operated and managed with the impression of Bochdalek hernia. We have also reviewed the similar case reports in the past 10 years, briefly, in order to map the presentations and clinical course of cases with Bochdalek hernia which were diagnosed late, for giving physicians a better insight on this issue

Assessment of Serum Amino Acid Chromatography in Children with Inflammatory Bowel Diseases

Background: Given the important role of amino acids in regulating many metabolic pathways of the body and considering the scarcity of markers for the diagnosis of inflammatory bowel disease (IBD) and its differentiation, we aimed to investigate the status of serum amino acids chromatography in children with IBD. Materials and Methods This case-control study was conducted among children with primary diagnosis of IBD who referred to Mofid Children's hospital in Tehran, Iran. Children with a definite diagnosis of chronic IBD on the basis of endoscopy and biopsy were enrolled. In addition, 100 children without any history of predisposing, chronic, or inflammatory disease who referred to the same hospital during the period of the study were also selected. All samples underwent serum amino acids chromatography via HPLC method. Results Of all the patients in the IBD group, 18 patients (18%) suffered from Crohn's disease and 82 patients (82%) had ulcerative colitis; the disease was active in 54 patients (54%). The results of serum amino acids chromatography showed that several amino acids were significantly higher in patients with IBD. Considering the normal serum levels of amino acids, only the levels of two amino acids of histidine and tryptophan were significantly different in the IBD group compared the control group. In total, of all the subjects, 30 children (15%) had abnormal amino acid serum chromatography; hence, its prevalence was significantly higher in the IBD group (P=0.048). Conclusion The current study showed that serum amino acid chromatography in children with IBD were different from that in healthy children. More specifically, the decrease in tryptophan level was more observed in patients with active disease

Creatinine Phosphokinase (CPK) Elevation in the Coexistence of Wilson's Disease and Autoimmune Hepatitis with Atypical Presentation: A Diagnostic Dilemma

Background: Wilson's disease (WD) is a genetic disorder with various clinical presentations due to excessive accumulation of copper in the liver and other organs. It can present as acute/chronic hepatitis, liver failure, extrahepatic and neuromuscular manifestations. Autoimmune hepatitis (AIH) is a necroinflammatory disease of the liver, which affects a lot of people particularly the children population. AIH has a broad clinical presentation that is similar to WD. Coexistence of WD with elevated creatinine phosphokinase (CPK) and AIH, may be a diagnostic dilemma. Case Report: We presented a 6 years old boy with dysarthria, aggressive behavior, weak attention, concentration and weight loss with abnormal physical examination. Laboratory, histochemical, genomic studies, muscle/liver biopsy and atomic absorption test confirmed the diagnosis of both WD and AIH in the boy. Conclusion Although CPK and liver enzyme elevation is a rare presentation of chronic hepatitis with dominant feature of WD and AIH; however, simultaneous therapy with immunosuppressive drugs and Penicillamine may have superior benefit with a significant response

Prevalence of Malnutrition among Iranian Pediatric Patients before and After Hospitalization (2015 To 2017): A Multicenter Study

Background: Malnutrition undermines the beneficial outcomes of clinical interventions and also increases hospital costs. Therefore, this study aimed to estimate the prevalence of malnutrition through a multicenter observational study at the time of admission and discharge in Iranian hospitalized children and adolescents. Methods: The present cross-sectional study was performed on children and adolescents aged one month to 18 years from three Iranian public tertiary pediatric hospitals located in different cities of Iran. To determine the participants’ nutritional status, Z-score of the weight for height (for those with 1month to 5years of age) and Z-score of BMI (for ≥5 to 18-year-old patients) were calculated using the WHO growth standards. Data were analyzed using SPSS version 23. Results: Information about 1499 patients was collected. At the time of admission, 64% of the participants had a good nutritional status, 15.5% were at high risk of wasting, 8.4% were wasted, and 12.1% were severely wasted. Among 295 malnourished patients, the nutritional status of 182 patients (63%) had been improved at the time of discharge. Also, 23% of all subjects with normal nutritional status at the admission time (85 participants), were at risk of malnutrition at discharge. The prevalence of moderate and severe malnutrition at the discharge time was about 20%. Conclusion: More than one-third of the hospitalized children had moderate or severe malnutrition or were at high risk. Although the prevalence of malnutrition decreased somewhat during hospitalization, some children were not malnourished at the time of admission and were malnourished at discharge

Evaluation of Clarithromycin and Metronidazole Resistance of Helicobacter Pylori Infection in Symptomatic Iranian Children

Helicobacter Pylori (H. pylori) as a gram-negative bacterium is the most common infection of the gastrointestinal tract, and worldwide it affects the children over three years of age. H. pylori could cause gastrointestinal and extra-intestinal manifestations. Antibiotic resistance can happen primarily and occurs during treatment. We aimed to evaluate the resistance gene of H. pylori obtained from gastric biopsy by polymerase chain reaction (PCR) method in Iranian children over 3 years old. Materials and Methods This study was a cross-sectional to evaluate the resistance gene of H. pylori obtained from gastric biopsy by polymerase chain reaction method for metronidazole and clarithromycin in children over three years old referring to the Mofid Children's Medical Center in Tehran, Iran. Results: Finally, data from seventy-nine samples included (mean age=10.7 years and male gender = 60.8%). Beta Globulin (BG) gene were detectable in 75 (94.93%) specimens of 79 (100%). Seventeen out of 75 specimens showed positive results for molecular detection of H. pylori. The results of RFLP-PCR technique showed that mutation of RdxA gene in seven of 17 (41.1%) for Metronidazole resistance and one case of 17 (5.8%) mutation of 23Y RNA gene that leads to clarithromycin resistance. Conclusion: Regarding the results of our study, it is better to check microbial resistance by culture and antibiogram for the antibiotic regimen of the first and second line of H. pylori treatment in children

Solitary polypoid lesion in gastric cardia; a case report and review of literature

AbstractIntroduction: Hyperplastic polyps of the esophago-gastric junction are seen during endoscopic evaluation of upper gastrointestinal tract. They are usually asymptomatic and discovered accidentally during endoscopic evaluation for other problems. These protrusions are mucosal regenerative response to surrounding mucosal injury. Case: Here we present a 9-year-old boy presenting with abdominal pain and vomiting since about one year ago. Recently, he was suffering from retrosternal chest pain. So after thorough clinical and paraclinical evaluations, upper endoscopy was done and, a small polypoid lesion in esophago-gastric junction was seen. Its surface seems normal and the polyp has no true stalk. Polypectomy was performed without any complication.Discussion: These lesions usually are regenerative response to surrounding mucosal injury. So, a thorough clinical evaluation and obtaining the sufficient biopsy specimen of the nonpolypoid mucosa are necessary for determining the clinicopathologic context in which the polyps have developed

Immunoproliferative Small Intestine Disease (IPSID): A Case Report

Immunoproliferative small intestinal disease (IPSID) is the syndrome associated with Mediterranean lymphoma (a rare form of non-Hodgkin’s lymphoma). Many of the patients diagnosed with secretory IPSID have variable level of abnormal immunoglobulins in serum or other bodily fluids, identified as truncated alpha heavy chain globulins. Most cases are characterized by a loss of ability to synthesize light chains. As such, IPSID has been classified as a heavy chain disorder B-cell lymphoma. We present here the case of a 12-year-old boy admitted in our department for edema, abdominal pain and FTT, in whom we suspected the diagnosis of IPSID