Manifestation of leech infestation as severe gastrointestinal bleeding in a 3-year-old boy: a case report and review of the literature.

BACKGROUND: Leeches are a class of hermaphroditic parasites that can attach to various body parts and start sucking blood. Gastrointestinal (GI) bleeding due to leeches is a rare phenomenon that is more common in less developed countries. Common symptoms include melena, hematemesis, pallor, weakness, and fatigue. Due to the similar symptoms of this issue to the main differential diagnoses of GI bleeding in pediatrics, such as diarrhea, constipation, diverticulitis, esophagitis, and anal fissures, it is challenging to differentiate it from the rest. CASE PRESENTATION: We present a three-year-old boy who was transferred to our center with hematemesis, tarry stool, and a drop in hemoglobin level. He finally was diagnosed with a leech in his stomach. CONCLUSIONS: In less developed counties, the inability to reach safe drinking water, swim in lakes or springs, and inadequate awareness of public health information among individuals can be risk factors for leech infestation.

Different Clinical Features of Celiac Disease in Children, Adolescents, and Adults; a Cross-sectional Study.

BACKGROUND Celiac disease is a common disorder but there are few studies comparing the clinical features of the disease in adults, adolescents and children. METHODS Demographic and clinical characteristics of all patients with celiac disease referred to the Celiac Clinic were evaluated and compared in different age groups. RESULTS Of 3416 participants, 473 patients were included. 302 (63.8%) were women and 171 (36.2%) were men. Overall, 325 (68.7%) and 411 (86.9%) patients had gastrointestinal (GI) and non-GI manifestations, respectively. The most common symptom in adults was psychiatric problems (66.5%), while abdominal discomfort was the most common symptom in adolescents (45.2%) and children (53.8%). According to age groups, GI manifestations were seen in 79 (66.4%), 119 (59.8%), and 127 (81.9%) children, adolescents, and adults, respectively. Adults had significantly more GI manifestations than the other groups (PR 1.167; 95% CI: 1.094- 1.244; p < 0.001). Non-GI manifestations were seen in 90 (75.6%), 174 (87.4%), and 147 (94.8%) children, adolescents, and adults, respectively. Adults had significantly more non-GI manifestations than the other groups (PR 1.112; 95% CI: 1.060-1.168; p < 0.001). CONCLUSION Our study showed that there were significant differences in the clinical features of celiac disease between the different age groups. Considering these results may help plan for future studies.

Evaluation of Validity and Efficiency of Diagnostic Criteria in Autoimmune Hepatitis in Children.

BACKGROUND: Autoimmune hepatitis (AIH) is a progressive inflammatory liver disease with various clinical symptoms, but treatment and prevention of hepatic failure and cirrhosis is possible with early diagnosis. However, no specific test has been approved for the diagnosis of AIH. In 2008, the International Autoimmune Hepatitis Group (IAIHG) developed a simplified diagnostic scoring system that has been widely used in practice. Nevertheless, it cannot distinguish AIH from Primary Sclerosing Cholangitis (PSC) and consensus is lacking with respect to its validity, sensitivity, and applicability for children patients. The newer 2018 version also requires validation. The present study intends to evaluate the validity and efficiency of the IAIHG simplified scoring system and new scoring system in children with AIH. METHODS: The present study is a non-interventional case-control study covering 152 patients with hepatic diseases (83 patients with AIH and 69 patients with Wilson disease (WD)). Titers of autoantibodies, IgG levels, hepatic histology, and absence of viral hepatitis were scored and calculated according to IAIHG diagnostic criteria. Statistics software package (SPSS) and draft receiver operating characteristic (ROC) curves was used to analyze data and determine value of diagnostic criteria. RESULT: In our study, both scoring systems' accuracy was good in AIH diagnosis, although new score displays higher sensitivity and specificity, suggestive of greater accuracy and predictive strength. CONCLUSION: Our study is the first validation study of the new scoring system in diagnosing AIH, and further studies require verifying this scoring system.

The efficacy and duration of treatment with propranolol in children with cyclic vomiting syndrome in southern Iran.

INTRODUCTION: Cyclic vomiting syndrome is a functional disorder characterised by repeated episodes of nausea and vomiting with symptom-free intervals between the attacks. Cyclic vomiting syndrome is a migraine equivalent; therefore, anti-migraine medications are effective for cyclic vomiting syndrome prophylaxis, but duration of treatment is not clear. AIM: To determine the efficacy and duration of drug therapy in cyclic vomiting syndrome. MATERIAL AND METHODS: This study was conducted on 206 cases of cyclic vomiting syndrome, who were treated with propranolol as prophylaxis. After they were symptom-free for at least 9 months, propranolol was tapered and discontinued and then they were followed for at least 6 months after drug withdrawal for evaluation of recurrence. RESULTS: The study subjects included 127 boys and 79 girls. The mean age of symptom onset was 3.4 years (range: 3 months to 14.5 years) and the mean age at the time of diagnosis was 5.7 years (range: 8.4 months to 18 years). Among the 206 patients in whom propranolol was discontinued, only 16 (7.8%) subjects developed recurrence of symptoms in the 6-month follow-up period. CONCLUSIONS: There is no need to continue prophylaxis of cyclic vomiting syndrome for a long time. It is also possible to make the duration of drug therapy shorter, which is useful for the convenience of the patients and prevention of drug side effects.

A Rapid Screening Test on Dried Blood for the Neonatal Diagnosis of Tyrosinemia Type I.

BACKGROUND: Tyrosinemia is an inherited metabolic disorder characterized by elevated levels of tyrosine and its metabolites in plasma. Without treatment, the disease will progress to hepatic and renal failure, so that without liver transplantation will cause death in less than 10 years of age. So, early diagnosis and treatment can be life saving and crucial. It means that with early treatment starting in the neonatal period, the patient can have normal life with very few restrictions in diets containing tyrosine and phenylalanine. OBJECTIVES: In this study we wanted to evaluate an easy to perform, rapid and sensitive qualitative test with low cost, as a part of neonatal screening tests to help early diagnosis and treatment of hereditary tyrosinemia. PATIENTS AND METHODS: In this cross sectional study, during the study period (2013 - 2014), 100 patients were selected. Fifty three (53) of these patients had proven tyrosinemia and the other 47 cases biliary atresia, paucity of intrahepatic bile ducts, cytomegalovirus (CMV) hepatitis, galactosemia and storage diseases. RESULTS: There were 2 false negative and 14 false positive cases of hereditary tyrosinemia (HT-1) in the test. Six cases of biliary atresia, 7 cases of paucity of intrahepatic bile ducts and one patient with cytomegalovirus (CMV) hepatitis were falsely positive with the test. Sensitivity of the test was 96.23%, specificity 71.43%, positive predictive value (PPV) 78.46%, and negative predictive value (NPV) 94.59%. CONCLUSIONS: This rapid qualitative test on dried blood sample is an easy, cheap, and feasible method for the screening of hereditary tyrosinemia in neonatal period.

BOWEL PREPARATION BEFORE COLONOSCOPY FOR CHILDREN: comparison of efficacy of three different methods

Background - Colonoscopy is an important diagnostic and therapeutic procedure. Adequate bowel preparation is mandatory. Several regimens were discussed in the literature. Among the drugs which has recently used, polyethylene glycol is one of the most popular agents. Objectives - The aim of this study was to compare efficacy of three different methods for 1 day preparation before colonoscopy. Methods - This study included children with the range of ages (2-21) who had an indication of colonoscopy. Exclusion criteria were based on the history of previous surgery, parental disagreement, and patients who did not use preparation protocol. Three methods for bowel preparation were studied: 1- Polyethylene glycol only; 2- Polyethylene glycol and bisacodyl suppositories; 3- Polyethylene glycol plus normal saline enema. Boston Bowel Preparation Score was used for evaluation of preparation. SPSS version 16.0 (Chicago, IL, USA) were used for data analysis. Results - In this study 83 cases completed the bowel preparation completely. Acceptable bowel preparation was seen in 24 (85.71%), 36 (94.73%), and 14 (82.35%) of cases in PEG, PEG + bisacodyl, and PEG + normal saline enema groups respectively. PEG + bisacodyl suppositories was more effective than PEG + normal saline for the preparation of the first segment ( P=0.05). For second and third segment of colon, BPPS score was higher in PEG + bisacodyl suppositories compared to other regimens, but this difference was not statistically significant. Conclusion - There was no significant difference between 1 day colonoscopy regimens in terms of bowel preparation score. Lowest score was seen in PEG + enema group compared to other group.

Contexto - A colonoscopia é um procedimento diagnóstico e terapêutico importante. A preparação intestinal adequada é obrigatória. Vários esquemas são discutidos na literatura. Dentre as drogas que se têm usado recentemente, o polietilenoglicol é um dos agentes mais utilizados. Objetivo - O objetivo deste estudo foi comparar a eficácia de três métodos diferentes para a preparação feita 1 dia antes de colonoscopia. Métodos - Este estudo incluiu crianças com a gama de idades entre 2 e 21 anos, que tinham indicação de colonoscopia. Os critérios de exclusão foram baseados em história da cirurgia anterior, não aprovação dos pais e pacientes que não utilizaram o protocolo de preparação. Três métodos para a preparação do intestino foram estudados: 1-polietilenoglicol; 2 - polietilenoglicol e bisacodil supositórios; 3 - polietilenoglicol e enema de solução salina. O escore de Boston para preparação intestinal foi usado para a avaliação e os dados foram analisados pelo SPSS versão 16.0 (Chicago, Il, USA). Resultados - Um total de 83 pacientes concluiu completamente o preparo intestinal. Houve preparo aceitável em 24 (85,71%), 36 (94,73%) e 14 (82,35%) dos casos, nos grupos PEG, PEG + bisacodil e PEG + enema salino, respectivamente. PEG + bisacodil supositórios foi mais eficaz do que a PEG + solução salina para a preparação do primeiro segmento ( P=0,05). Para segundo e terceiro segmento do cólon, a pontuação de BPPS foi maior no grupo PEG + bisacodil supositórios em comparação com outros regimes, mas essa diferença não foi estatisticamente significativa. Conclusão - Não houve nenhuma diferença significativa entre os regimes de preparo para colonoscopia de um dia em termos de pontuação de preparação do intestino. A nota mais baixa foi vista no grupo PEG + enema em comparação com outros grupos.

Clinical Manifestations among Children with Chronic Functional Constipation.

BACKGROUND Constipation is one of the most frequent cause of patient visits to pediatric gastroenterology clinics. Early diagnosis and treatment is important. There are few studies about clinical manifestations of constipation in children. We aimed to find the relative frequency of gastrointestinal manifestations of constipation among constipated children. METHODS This cross-sectional study was carried out on children aged < 18 years old with chronic functional constipation referred to Imam Reza Clinic of Shiraz University of Medical Sciences. Children with organic causes of chronic constipation were excluded from study. Rome III criteria were used for defining constipation. The duration of study was 1 year starting from September 2010. Abdominal pain, fecal mass, rectal bleeding, anorexia, fecal soiling, retentive posture, withholding behavior, anal fissure, and peri-anal erythema were recorded for each case based on history and physical examination. Data were analyzed using SPSS software, version 13.0 (Chicago, IL, USA). RESULTS Of 222 children with functional constipation, 124(55.9%) were girls and 98 (44.1%) were boys with a mean ± SD age of 5±3.12 years. The mean ± SD duration of constipation was 2.2±1.9 years. Large and hard stool was present in 93.7% of the patients. Painful defecation and withholding behavior were seen in 92.3% and 91.9% of the patients, respectively. Fecal impaction was more frequent among boys compared with girls (p<0.01). Fecal soiling was present in 40.8% of the boys and 28.2% of the girls (p=0.04). CONCLUSION Large and hard stool, painful defecation and withholding behavior were the most frequent signs or symptoms among children with chronic functional constipation. Fresh rectal bleeding and anal fissure were the least frequent signs and symptoms in this group.

BOWEL PREPARATION BEFORE COLONOSCOPY FOR CHILDREN: comparison of efficacy of three different methods.

BACKGROUND: Colonoscopy is an important diagnostic and therapeutic procedure. Adequate bowel preparation is mandatory. Several regimens were discussed in the literature. Among the drugs which has recently used, polyethylene glycol is one of the most popular agents. OBJECTIVES: The aim of this study was to compare efficacy of three different methods for 1 day preparation before colonoscopy. METHODS: This study included children with the range of ages (2-21) who had an indication of colonoscopy. Exclusion criteria were based on the history of previous surgery, parental disagreement, and patients who did not use preparation protocol. Three methods for bowel preparation were studied: 1- Polyethylene glycol only; 2- Polyethylene glycol and bisacodyl suppositories; 3- Polyethylene glycol plus normal saline enema. Boston Bowel Preparation Score was used for evaluation of preparation. SPSS version 16.0 (Chicago, IL, USA) were used for data analysis. RESULTS: In this study 83 cases completed the bowel preparation completely. Acceptable bowel preparation was seen in 24 (85.71%), 36 (94.73%), and 14 (82.35%) of cases in PEG, PEG + bisacodyl, and PEG + normal saline enema groups respectively. PEG + bisacodyl suppositories was more effective than PEG + normal saline for the preparation of the first segment ( P=0.05). For second and third segment of colon, BPPS score was higher in PEG + bisacodyl suppositories compared to other regimens, but this difference was not statistically significant. CONCLUSION: There was no significant difference between 1 day colonoscopy regimens in terms of bowel preparation score. Lowest score was seen in PEG + enema group compared to other group.

Remedies for children constipation in medieval Persia.

Constipation in children with bowel movement less than 3 times a week and lasting for more than 3 months is defined as pediatric chronic constipation. According to traditional Persian medicine resources, dryness of food, low nutrition, hotness or dryness of the gastrointestinal tract, intestine sensory loss, excessive urination, increase of evaporation, and sweating because of heavy exercise will together constitute the condition for constipation occurrence. Lifestyle changes considered as premier of medical intervention for constipation. Treatment of constipation in children vastly benefitted from traditional Persian medicine, including simple dietary measures, oil massages, and herbal medicines. This investigation was performed to somewhat help the anxious academics to achieve proper findings in the field of gastroenterology, in pursuit of the traditional Persian medicine advices.

Autoimmune hepatitis in children: experiences in a tertiary center.

OBJECTIVE: Autoimmune hepatitis (AIH) is a necroinflammatory liver disease of unknown etiology that occurs in the children of all ages. The present study aimed to evaluate the clinical and paraclinical presentations, including pattern of autoantibodies, response to treatment, mortality, and liver transplantation outcome in the Iranian children with AIH. METHODS: The medical records of 87 children (56 girls and 31 boy) diagnosed with AIH between 2001 and 2010 were retrospectively analyzed for clinical and paraclinical profiles and also treatment outcome. FINDINGS: The mean age of the patients was 10.1±4.5 years (64.4% females). The most common clinical findings were jaundice (70.1%), splenomegaly (67.8%), and hepatomegaly (51.7%). Antinuclear, anti-smooth muscle, and anti LKM antibodies were positive in 14/62, 22/53 and 6/40 patients, respectively (36 patients had type 1 AIH, 6 patients had type 2 AIH, 26 patients were seronegative, and autoantibodies were not available in 19 cases). The most common histological finding in the liver biopsies was chronic hepatitis with interface activity that was seen in 65 (74.7%) patients. The complete response was seen in 52 (59.8%) patients and 24 (27.6%) patients underwent liver transplantation. One-year and five-year survival rates were 87.5% and 80% in the transplanted patients. CONCLUSION: AIH should be kept in mind in the differential diagnosis of both acute and chronic liver diseases in the children and treatment with combination of corticosteroids and azathioprine is a good treatment option. In the patients with end stage liver cirrhosis that did not respond to medical therapy, liver transplantation is the treatment of choice.

The Role of Cow's Milk Allergy in Pediatric Chronic Constipation: A Randomized Clinical Trial.

OBJECTIVE: Cow's milk allergy has different presentations in children and can cause functional bowel symptoms such as chronic constipation. The aims of this study were to investigate the role of cow's milk allergy as a cause of chronic constipation and effect of cow's milk free diet (CMFD) on its treatment in children. METHODS: We performed a randomized clinical study comparing CMFD with cow's milk diet (CMD) in two groups each consisting of 70 patients (age range, 1-13 years) with chronic functional constipation (defined as Rome III criteria). All subjects had been referred to a pediatric gastroenterology clinic and had previously been treated with laxatives for at least 3 months without success; also all 140 patients performed skin prick test. The case group received CMFD for 4 weeks. After that they received CMD for 2 extra weeks. The control group received CMD for whole 6 weeks. A response was defined as decreased in signs and symptoms that not fulfilled Rome III criteria after 4 weeks of CMFD and came back to Rome III criteria after 2 weeks of CMD challenge. FINDINGS: After 4 weeks 56 (80%) patients of the case group responded in comparison to 33 (47.1%) patients in the control group (P=0.0001). In the case group after 2 weeks challenge 24 out of 56 (42.8%) responders developed constipation according to Rome III criteria. With other words, the frequency of cow's milk allergy among constipated patients was 80%. Only one patient had positive skin prick test. CONCLUSION: In children, chronic constipation can be a manifestation of cow's milk allergy. At present, although several aspects must be further investigated, a therapeutic attempt with elimination diet is advisable in all children with constipation unresponsive to correct laxative treatment.

Post-transplantation lymphoproliferative disorder after liver transplantation: report of 5 cases among more than 550 liver transplants in Iran.

BACKGROUND: Post-transplantation lymphoproliferative disorders (PTLD) are a spectrum of diseases defined as polyclonal or monoclonal proliferations of lymphocytes which occur after solid organ transplants. In this study, we report our first experiences with PTLD following liver transplantation in Iran. METHODS: We retrospectively analyzed five cases of PTLD which followed liver transplantation among more than 550 liver transplants in our center. Of these, three were pediatric cases and two were adults. The underlying causes were tyrosinemia, autoimmune hepatitis, and progressive familial intrahepatic cholestasis (PFIC) in the three pediatric cases. HCV hepatitis was the primary cause for cirrhosis in one of the adults and the other adult was labeled as cryptogenic cirrhosis. All cases, except for one, developed PTLD during the first year following liver transplantation. RESULTS: Patients were diagnosed as PTLD, B-cell, MALT and Hodgkin-like (according to the WHO classification of PTLD). The three pediatric patients died despite discontinuation of immunosuppressive drugs and chemotherapy. Fortunately both adult patients, until now, are still alive. CONCLUSION: The incidence of PTLD in our center is lower than previous reports from other centers (0.9%), with a 60% mortality rate and worse prognosis in the pediatric age group.

Can Campylobacter jejuni play a role in development of celiac disease? A hypothesis.

Celiac disease (CD) is an entropathy with malabsortive condition in which an allergic reaction to the cereal grain-protein (gluten) causes small intestine mucosal injury. CD is a multifactorial disorder in which both genetic and environmental factors contribute to the disease development. Mechanisms have been described to explain the pathology of CD. T cells specific for multiple gluten peptides are found in virtually all patients. Generation of such a broad T cell response may be a prerequisite for disease development. CD is associated with multiple extraintestinal presentations, including neurological deficits. Recent studies have shown a significant correlation between anti-ganglioside antibodies and neurological disorders in patients with underlying CD. Gangliosides are glycosphingolipids which are abundant in nervous system and in other tissues including gastrointestinal tract. It is not known what triggers the release of anti-ganglioside antibodies in people with gluten sensitivity. But, the mechanism is likely to involve the intestinal immune system response to ingested gliadin, a component of wheat gluten. Studies showed that mechanisms different from gluten exposure may be implicated in antibody formation, and other environmental factors may also exist. In addition, considering the fact that genetic predisposition dysregulating mucosal immune responses in the presence of certain environmental triggers like gastrointestinal infections may be strong etiological factors for developing chronic intestinal inflammation including CD, the hypothesis raised in our mind that antiganglioside antibody formation in CD may play a role not only in development of neurological complications in celiac patients, but also in development of CD itself. As presence of Campylobacter jejuni in other diseases with antigangliosides antibody formation has been established, we propose the possible role of Campylobacter jejuni in development of CD in association with other genetic and environmental factors by the mechanism that molecular mimicry of gangliosides-like epitopes common to both lipo-polysacharide coats of certain strains of Campylobacter jejuni and gangliosides in cell structure of gastrointestinal mucosa may cause an autoimmune response and consequently lead to atrophy and degeneration of mucosa possibly by apoptosis.