• Clinical and Experimental Pediatrics
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• v.49 no.2
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• pp.203-207
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• 2006

Neurofibromatosis type 1 (von Recklinghausen disease, NF1) involves the central and peripheral nervous systems as well as the skin, bone, endocrine, gastrointestinal and vascular systems. The gastrointestinal neurofibroma associated with NF1 has been infrequently reported. We report our experience with a 15-year-old boy who had a gastric plexiform neurofibroma with upper gastrointestinal bleeding and underwent a tumorectomy because of massive upper gastrointestinal bleeding. We conclude that gastrointestinal bleeding and anemia in the setting of NF1 mandates complete endoscopic examination of the digestive tract to rule out neurofibromas. Surgical resection is the standard treatment.

• Journal of Gastric Cancer
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• v.18 no.1
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• pp.99-107
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• 2018

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibro-inflammatory disorder characterized by specific pathological findings and elevated serum IgG4 level. IgG4-RD in the stomach is rare, and occasionally diagnosed as gastric subepithelial tumor (SET) by endoscopy or computed tomography scan. Two female patients in the age group of 40-50 years were diagnosed with 4 cm sized gastric SET. One underwent laparoscopic gastric wedge resection. Another one had a history of subtotal gastrectomy for early gastric cancer and idiopathic thrombocytopenic purpura with oral steroids administration. She underwent a completion total gastrectomy with splenectomy for the gastric SET and ITP. The pathology showed storiform fibrosis, and IgG4 was positive in immunohistochemistry (IHC) stain. IgG4-RD is known as a medical disease that could be treated with oral steroids. The difficulty in preoperative diagnosis of the disease occasionally causes unnecessary gastric resection. Thus, preoperative diagnostic methods for IgG4-RD such as deep biopsy with IHC stain or magnetic resonance imaging are needed.

• Journal of Gastric Cancer
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• v.15 no.2
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• pp.113-120
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• 2015

Purpose: Although several clinical trials have proven the efficacy of adjuvant S-1 treatment in gastric cancers, it is still unclear which patients receive the most benefit. In this study, we prospectively recruited patients with locally advanced gastric cancer who had undergone curative resection followed by adjuvant S-1 administration to investigate which factors affect the outcomes. Materials and Methods: Between July 2010 and October 2011, we enrolled 49 patients who underwent curative resection for stage II or III gastric cancer and who subsequently received adjuvant S-1 treatment for 1 year. Results: Twenty-nine patients (59.2%) continued S-1 treatment for 1 year, and 12 patients (24.5%) experienced recurrent disease during the follow-up period. Patients with continuation of S-1 for 1 year had significantly increased rates of disease-free survival (P<0.001) and overall survival (P=0.001) relative to the patients who discontinued S-1 during year 1. Multivariate analysis indicated poor outcomes for patients with stage III disease and those who discontinued S-1 treatment. Excluding patients who discontinued S-1 due to cancer progression (n=7), adjuvant treatment with S-1 still demonstrated a significant difference in the disease-free survival rate between the patients who continued treatment and those who discontinued it (P=0.020). Conclusions: S-1 is tolerated as adjuvant treatment in gastric cancer patients. However, discontinuing S-1 treatment may be an unfavorable factor in the prevention of recurrence. S-1 adjuvant treatment should be continued for 1 year if possible through the proper management of toxicities.

• Tuberculosis and Respiratory Diseases
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• v.79 no.4
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• pp.267-273
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• 2016

Background: Cystic fibrosis (CF) is an autosomal recessive disorder with several clinical presentations. This study was undertaken in the Azeri Turkish population in Iran, to investigate gender differences in the age at onset and diagnosis, age of death, and duration of illness of CF. Methods: The data of 331 CF patients from 2001 to 2015 was surveyed. Parameters including age, sex, ${\Delta}F508$ mutation, age at onset, age at diagnosis, age of death and clinical presentations were evaluated for both sexes, using descriptive analysis. The association of gender with these variables was studied using logistic regression, chi-square test and Mann-Whitney U test by SPSS version 18. Odds ratio with a confidence interval of 95% and $p{\leq}0.05$ was considered statistically significant. Results: The study included 191 males (57.7%) and 140 females (42.3%), all showing statistically significant difference (p<0.001). Age duration differed between genders. Male and female patients were further under 9 and 4 years, respectively. The occurrence of ${\Delta}F508$ mutation was 0.51 times more in females than in males. Age, diagnosis and sex were closely associated: males were diagnosed at a significantly later age than females (p=0.05). While this compression performed based on clinical presentations, males with respiratory disease had a later median age at diagnosis than females at lifespan (p=0.001). The risk of infertility in males was approximately two times greater than in females (p=0.02). Conclusion: These findings indicate gender differences in CF patients. Future studies are needed to establish other differences and evaluate the causes for the gender variations.

• Clinical and Experimental Pediatrics
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• v.62 no.8
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• pp.287-291
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• 2019

Fecal calprotectin (FC) is a calcium- and zinc-binding protein of the S100 family, mainly expressed by neutrophils and released during inflammation. FC became an increasingly useful tool both for gastroenterologists and for general practitioners for distinguishing inflammatory bowel disease (IBD) from irritable bowel syndrome. Increasing evidences support the use of this biomarker for diagnosis, follow-up and evaluation of response to therapy of several pediatric gastrointestinal diseases, ranging from IBD to nonspecific colitis and necrotizing enterocolitis. This article summarizes the current literature on the use of FC in clinical practice.

• Clinical and Experimental Pediatrics
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• v.65 no.1
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• pp.21-28
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• 2022

Crohn disease (CD) is a multifactorial inflammatory disorder that can affect all segments of the gastrointestinal (GI) tract but typically involves the ileum and/or colon. To assess patient prognosis and choose appropriate treatment, it is necessary to accurately evaluate the factors influencing poor outcomes, including disease phenotype. Pediatric CD involving the upper GI (UGI) tract has become increasingly recognized with the introduction of routine upper endoscopy with biopsies for all patients and the increased availability of accurate small bowel evaluations. Most clinical manifestations are mild and nonspecific; however, UGI involvement should not be overlooked since it can cause serious complications. Although controversy persists about the definition of upper GI involvement, aphthoid ulcers, longitudinal ulcers, a bamboo joint-like appearance, stenosis, and fistula are endoscopic findings suggestive of CD. In addition, the primary histological findings, such as focally enhanced gastritis and noncaseating granulomas, are highly suggestive of CD. The association between UGI involvement and poor prognosis of CD remains controversial. However, the unstandardized definition and absence of a validated tool for evaluating disease severity complicate the objective assessment of UGI involvement in CD. Therefore, more prospective studies are needed to provide further insight into the standardized assessment of UGI involvement and long-term prognosis of CD. Our review summarizes the findings to date in the literature as well as UGI involvement in CD and its clinical implications.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.11 no.2
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• pp.122-129
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• 2008

Purpose: Patients with chronic gastrointestinal disease are at risk for trace element deficiency due to impaired absorption and gastrointestinal loss. The aim of this study was to evaluate the trace element status of patients with gastrointestinal disease by blood and hair analysis, and to determine the usefulness of hair mineral analysis for diagnosing trace element deficiency not detected by a blood test. Methods: An analysis of hair minerals was performed and compared with blood mineral analysis in 13 patients with chronic gastrointestinal disease. The concentration of each element in the hair and blood was compared in the subgroups based on parenteral nutritional support or clinical symptoms. Results: Almost all patients had trace element deficiency. The trace elements deficient in the blood or hair analysis included zinc, selenium and copper. The hair zinc concentration was significantly lower in the group receiving parenteral nutritional support. The hair selenium concentration was statistically associated with the clinical symptoms of hair loss, brittle hair and loss of hair pigmentation. Conclusion: The results of this study suggest that patients with chronic gastrointestinal disease should receive adequate zinc and selenium replacement to avoid trace element deficiency especially when treated with long-term parenteral nutrition. Hair mineral analysis is useful as a complementary tool for the detection of a trace element deficiency.

• Journal of Gastric Cancer
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• v.15 no.2
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• pp.75-76
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• 2015

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.17 no.1
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• pp.52-56
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• 2014

We present a case of a 13-year-old boy with Gorham's disease involving the thoracic and lumbar spine, femur, and gastrointestinal (GI) tract, which was complicated by recurrent chylothorax and GI bleeding. The presenting symptoms were intermittent abdominal pain, back pain, and melena. Esophagogastroduodenoscopy and colonoscopy showed no abnormal lesions, but duodenal biopsy showed marked dilation of the lymphatics in the mucosa and submucosa, which revealed positive staining with a D2-40 immunohistochemical marker. In cases of GI bleeding with osteolysis, the expression of a D2-40 marker in the lymphatic endothelium of the GI tract may help to diagnose GI involvement in Gorham's disease. To the best of our knowledge, this is the first case report to pathologically demonstrate intestinal lymphatic malformation as a cause of GI bleeding in Gorham's disease.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.11 no.1
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• pp.80-83
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• 2008

Behcet's disease (BD) is a multisystem inflammatory disorder dominated clinically by recurrent oral and genital ulceration, uveitis and erythema nodosum. BD is very rare in children, especially those less than 10 years of age, who account for only an estimated 5% of all cases. Gastrointestinal ulcers, in patients with Behcet's disease with intestinal involvement are rare and have been reported in only 1-2% of all cases. The intestinal ulcers of Behcet's disease are usually multiple and scattered and tend to cause perforations associated with significant morbidity. Patients with BD and abdominal symptoms must be evaluated thoroughly for potential perforation of the gastrointestinal tract. Here we report the case of a 4 year 9 month old child with multiple perforations of the gastrointestinal tract associated with BD.