Elif Sag

Ischemia-Modified Albumin Levels in Children with Chronic Liver Disease

Background/Aims Ischemia-modified albumin (IMA) levels have been shown to correlate with the severity of liver failure in adults. However, the role of IMA levels has not been evaluated in children with chronic liver disease (CLD). We analyzed the clinical significance of IMA levels in children with CLD. Methods Thirty-three children with CLD and 33 healthy children were included in the study. Blood was collected to analyze biochemical parameters, oxidant status, and IMA. Liver biopsies were re-evaluated for liver fibrosis; severe fibrosis (SF) was defined as fibrosis stage ≥4. Results The IMA and and IMA to albumin ratios (IMARs) were significantly higher in children with CLD than in those without (IMA: 0.545±0.095 vs 0.481±0.062, p=0.003; IMAR: 0.152±0.046 vs 0.126±0.018, p=0.04). The IMAR was positively correlated with the pediatric end-stage liver disease score (p=0.03, r=0.503) and fibrosis score (p=0.021, r=0.400). Patients with SF had higher IMARs compared to patients with mild fibrosis (0.181±0.056 vs 0.134±0.025, p=0.003). The area under the receiver operation curve (AUROC) for predicting SF was 0.78 (p=0.006). Using a cutoff ratio value of 0.140, the sensitivity and specificity were 84% and 70%, respectively. The AUROC for predicting the need for liver transplantation and/or death was 0.82 (p=0.013). With a cutoff value of 0.156, the sensitivity and specificity was 83% and 82%, respectively. Kaplan-Meier analysis revealed increased morbidity and/or mortality in the group with an IMAR>0.156 (50% vs 4.3%, p=0.005). Conclusions IMARs have been shown to provide important clues in predicting the fibrosis stage of the disease and determining the outcome in children with CLD.

A Rare Cause of Recurrent Acute Pancreatitis in a Child: Isovaleric Acidemia with Novel Mutation.

Recurrent acute pancreatic attacks is a rare clinical condition (2-5% of all acute pancreatis) in children and is mainly idiopathic in most cases. Sometimes it may be associated with congenital anomalies, metabolic diseases or hereditary conditions. Isovaleric acidemia (IVA) is a rare autosomal recessive amino acid metabolism disorder associated with isovaleryl coenzyme A dehydrogenase deficiency presenting the clinical findings such metabolic acidosis with increased anion gap, hyperammonemia, ketonemia, hypoglycemia, “the odor of sweaty feet,” abdominal pain, vomiting, feeding intolerance, shock and coma. Recurrent acute pancreatitis associated with IVA have been rarely reported. Herein; we report a child who admitted with recurrent acute pancreatic attacks and had the final diagnosis of IVA. Mutation analysis revealed a novel homozygous mutation of (p.E117K [c.349G>A]) in the IVA gene. Organic acidemias must kept in mind in the differential diagnosis of recurrent acute pancreatic attacks in children.

Acute pancreatitis in children: a single center experience over ten years

Sağ E, Kaya G, Bahat-Özdoğan E, Karahan SC, İmamoğlu M, Sarıhan H, Çakır M. Acute pancreatitis in children: A single center experience over ten years. Turk J Pediatr 2018; 60: 153-158. Acute pancreatitis (AP) is an inflammatory disease characterized by sudden onset abdominal pain together with elevation of pancreatic enzymes and radiographic changes. Increased incidence of AP in children have been reported in recent reports. In this study; we aimed to analyze the demographic characteristics, etiology, outcome and incidence of AP among hospitalized children in our center. Medical records of the children with AP since January 2005 were analyzed from hospital files (N=63). Major etiologies were systemic diseases (14.3%), trauma (11.1%), cholelithiasis (9.5%); 54% (N=34) of the patients had mild AP, while 28.6% (N=18) had moderately severe AP and 17.4% (N=11) had severe AP. Organ dysfunction was found in 11 patients (17.4%) at initial examination. During the follow-up period (68.1±24.3 months), 10 patients (15.9%) experienced 24 recurring AP (RAP) attacks. Male gender, presence of local pancreatic or systemic complications at initial attack, metabolic and hereditary diseases were associated with the increased risk of RAP (p < 0.05 for all). The mortality rate associated with AP was 4.84%. There was an increase in the incidence of AP since 2010 (9.57 in 2009-2010 vs. 39.17/10,000 patients in 2015-2016 years; p=0.0002; OR: 4.1) among the hospitalized patients. Our results indicate that AP is a mild disease in children and the incidence is increasing among hospitalized children. Male gender, presence of local pancreatic or systemic complications at initial attack, metabolic diseases and hereditary diseases were associated with the increased risk of RAP.

An infant with cholestasis, acholic stool and high GGT levels

Elif Sağ1 , Güneş Işık2 , Alper Han Çebi3 , Mukaddes Kalyoncu2 , Murat Çakır1 , Sema Aydoğdu4 1Department of Pediatric Gastroenterology, Karadeniz Technical University School of Medicine, Trabzon, Turkey 2Department of Pediatric Nephrology, Karadeniz Technical University School of Medicine, Trabzon, Turkey 3Department of Medical Genetics, Karadeniz Technical University School of Medicine, Trabzon, Turkey 4Department of Pediatric Gastroenterology, Hepatology and Nutrition, Ege University School of Medicine, İzmir, Turkey

Endoscopic Findings of Children with Familial Mediterranean Fever

Purpose Familial Mediterranean fever (FMF) is an auto inflammatory disease characterized by periodic fever, synovitis and serositis. Patients may be admitted to gastroenterology units due to gastrointestinal symptoms. In this study; we aimed to analyze endoscopic findings and diagnostic utility of endoscopic procedure in children with FMF. Methods Patient with FMF that was performed endoscopy for the gastrointestinal symptoms were included to the study (39 of 164 patients, 53 procedure). A control group was randomly designed as age and gender matched four endoscopic procedures per one endoscopic procedure of patients with FMF (n=212). Results No different was found between the patients and control group in esophagogastroscopy findings. However, the diagnosis of gastrointestinal pathology was made by esophagogastroscopy in 46.2% patients. Colonoscopic examination revealed that the frequency of inflammatory bowel disease (IBD) was higher in undiagnosed patients compared to both the control group (50.0% vs. 6.9%, p<0.05, odds ratio [OR]:13.4 and 95% confidence inteval [95% CI]: 2.1–84.3) and the patients under colchicine treatment (50.0% vs. 8.3%, p<0.05, OR: 11 and 95% CI: 0.8–147.8). Colonoscopic procedure that was made after the diagnosis was found to provide contribution by 16.7% in determining the etiology of the additional symptoms. Conclusion Patients with FMF may be admitted to pediatric gastroenterology outpatient clinic prior to diagnosis or during the follow-up period. The frequency of IBD is high in undiagnosed patients with FMF. Endoscopic procedures may be helpful in these patients for the diagnosis accompanying mucosal lesions.

Accessory Hepatic Lobe: A Rare Cause of Prehepatic Portal Hypertension in a Child

Accessory hepatic lobe is noted as and considered a rare disease in children. It can manifest with various symptoms and complications depending on the location, volume, type and position of the disease as presented on a child. The patient presented as a 14-month-old girl who was seen with a notable hepatosplenomegaly and portal hypertension. A diagnosis was made after taking an extensive medical history, observation and radiological examinations. The formal diagnosis was a prehepatic portal hypertension associated with accessory hepatic lobe.

Esophageal eosinophilia in children: a 6-year single-center experience

Çakır M, Sağ E, Mungan S, Akbulut UE, Orhan F. Esophageal eosinophilia in children: A 6-year single-center experience. Turk J Pediatr 2017; 59: 369-378. Esophageal eosinophilia (EE) and eosinophilic esophagitis (EoE) are emerging clinical entities, the prevalence of which has increased during the last 15 years. However, there is a lack of data concerning the etiology and outcomes of EE in children. The aim of this study was therefore to analyze the clinical findings and outcomes of children with EE and EoE in our pediatric gastroenterology unit over a 6-year period. The study included children undergoing esophagogastroduodenoscopy (EGD) during this 6-year period (January 2010 to December 2015) in our pediatric gastroenterology unit. The files of patients with EE were re-evaluated in detail to elicit demographic features, clinical, laboratory and histopathological findings, treatment modalities and outcomes. EE was determined in 33 patients [0.95% (95% CI: 0.63-1.27) among all children, and in 4.66% (95% CI: 3.11-6.21) of children undergoing esophageal biopsy] (8.6±4.2 years and 72.7% male). EoE was the most common cause of EE (n=11, 33.3%), followed by eosinophilic gastroenteropathy (n=6, 18.1%) and proton pump inhibitor responsive esophageal eosinophilia (n=4, 12.1%). Patients with EoE (n=11) were followed up for 21.2±18 (range: 1-60) months, and treatment was discontinued in 2 patients (18.1%). Additionally, 5 patients (45.5%) received diet elimination only and 1 patient (9%) received a combination of low dose steroids and diet. Three patients (27.2%) are still being treated under the initial regimen. The overall incidence of EE increased in 2014-2015 compared to 2010-2011 (0.41% vs. 1.33, p=0.047, OR: 3.22 and 95% CI: 0.94-10.98, p=0.06). EE is an increasingly common clinical entity with a wide spectrum of etiology and clinical presentations in children.

Effects of long-term synbiotic supplementation in addition to lifestyle changes in children with obesity-related non-alcoholic fatty liver disease

BACKGROUND/AIMS We aimed to analyze the efficiency of a novel treatment approach, long-term synbiotic supplementation, in addition to lifestyle changes in children with non-alcoholic fatty liver disease (NAFLD). MATERIALS AND METHODS The study included children with NAFLD (n=28) and a healthy control group (n=30). Children with NAFLD were given 1 capsule/day of synbiotics. Anthropometric parameters; biochemical analysis, including ethanol, tumor necrosis factor-α (TNF-α), total oxidant status (TOS) and anti-oxidant status (TAS), zonulin, and fecal calprotectin; and ultrasonographic examination were performed at baseline and 4 months later. RESULTS The grade of fatty liver was decreased (≥1 grade) in 19 of the 28 patients (67.8%) after synbiotic supplementation. Total cholesterol, low-density lipoprotein (LDL) levels, TNF-α, C-reactive protein (CRP), and ethanol were significantly decreased, and TAS levels were significantly increased at the end of treatment (p<0.05 for all). We found that the median decrease in CRP (-0.16 vs. -0.03 mg/dL, p=0.003) and LDL levels (-17 vs. -3 mg/dL, p=0.019) were higher in patients who responded to the supplementation. CONCLUSION Synbiotic supplementation in addition to lifestyle changes is effective in children with NAFLD.

Interleukin-6 and interleukin-17 gene polymorphism association with celiac disease in children

BACKGROUND/AIMS This study aimed to investigate polymorphisms in the genes responsible for encoding cytokines interleukin-6 (IL-6) (-572G/C) (rs1800796) and IL-17 (-197A/G) (rs2275913) in patients with celiac disease (CD). We further aimed to investigate the relationship between CD symptoms and histopathological findings and the relationship between these polymorphisms. MATERIALS AND METHODS We compared the results with those of healthy control subjects to establish whether any of the polymorphisms are involved in the susceptibility to CD. Eighty-four patients with CD and 83 healthy controls were enrolled in this study. Children with CD were divided into two groups depending on whether their symptoms were typical or atypical. The IL-6 (-572G/C) and IL-17 (-197A/G) polymorphisms were genotyped based on a polymerase chain reaction coupled with restriction fragment length polymorphism. RESULTS Significant differences for the IL-6 (-572G/C) polymorphism were observed between patients with CD and controls (p=0.018, odds ratio (OR): 5.47, 95% confidence interval (CI): 1.161-25.800). No statistically significant association was observed between the IL-17 (-197A/G) polymorphism and CD (p>0.05). In addition, the symptoms and histopathological findings of children with CD were not related to either of the polymorphisms. CONCLUSION The results of our study indicate that the IL-6 (-572G/C) polymorphism may play a role in susceptibility to CD.