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Clues to the diagnosis of biliary atresia in neonatal cholestasis.

BACKGROUND/AIMS The purpose of this study was to identify important clues in differentiating biliary atresia (BA) from causes of neonatal cholestasis other than BA (non-BA) and establishing the reliability of current tests. MATERIALS AND METHODS Thirty-four patients with BA and 27 patients with non-BA cholestasis being monitored at the Çukurova University Medical Faculty, the Pediatric Gastroenterology Department and the Pediatric Surgery Department between 2009 and 2015 were retrospectively assessed. RESULTS Cases of early onset jaundice, acholic stool, gamma-glutamyl transferase (GGT) elevation, and absent or small gallbladder on ultrasonography (USG) were greater in the BA group, while the levels of consanguinity and splenomegaly were higher in the non-BA group. The highest positive predictive value and specificity was determined for a GGT level greater than 197 in addition to absent or small gallbladder on USG and acholic stool in the BA group. Moreover, the presence of acholic stool (97%) exhibited the highest sensitivity and accuracy in the diagnosis of BA. CONCLUSION Pale stool, GGT elevation, and absent or small gallbladder on USG are the most reliable tests for diagnosing BA. We recommend that intraoperative cholangiography should be performed without waiting for further test results when a neonate or infant presents with acholic stool, high GGT values, and absent or small gallbladder on abdominal USG.

Gluten-Related Methemoglobinemia

To the Editor: Acquired methemoglobinemia can be caused by drugs, chemicals, and some foodstuff s ( 1,2 ). Dapsone is the most commonly implicated drug, and even topical forms have been shown to cause this condition ( 3 ). Methemoglobinemia may also develop aft er the consumption of some nitrate-rich foods, such as spinach. A 4.5-year-old girl was presented to our clinic with diarrhea, failure to thrive, abdominal distension, and cyanosis. Th e patient’s history revealed that cyanosis had begun at the age of 2 years. An echocardiography examination performed at another center revealed normal results, and a hemoglobin electrophoresis that had been performed before 1 year revealed carboxyhemoglobin levels of 11%, deoxyhemoglobin levels of 11.8%, oxyhemoglobin levels of 40.6%, and methemoglobin (MetHb) levels of 46.8% (0.4–1.5). Th e patient was then diagnosed with methemoglobinemia and was prescribed methylene blue 1 mg/ kg per day. Th e cyanosis had decreased during the drug therapy but recurred aft er the drug therapy ended. Th e patient had no past history of any drug use. On presentation to our hospital, the patient’s body temperature was 36.7 °C, blood pressure was 110/70 mm Hg, heart rate was 98 beats/ min, weight was 12 kg ( P < 3; Z -score: −2.5), and height was 84 cm ( P < 3; Z -score: −5.2). Th e patient had cyanosis, malnourishment, abdominal distension, and a genu valgum deformity of her right leg; however, she was not dehydrated. Other systemic parameters were normal. Oxygen saturation on pulse oximetry was 83% while breathing ambient air; hemoglobin level was 12 g/dl; hematocrit was 38%; mean corpuscular volume was 78 fL; serum iron level was 10 μ g/dl; total iron binding capacity was 401 μ g/dl, and ferritin level was 4.7 ng/ml. Th e serum REFERENCES 1. Ash-Bernal R , Wise R , Wright SM . Acquired methemoglobinemia: a retrospective series of 138 cases at 2 teaching hospitals . Medicine 2004 ; 83 : 265 – 73 . 2. Kudale S , Sethi SK , Dhaliwal M et al. Methemoglobinemia due to quinine causing severe acute kidney injury in a child . Indian J Nephrol 2014 ; 24 : 394 – 6 . 3. Swartzentruber GS , Yanta JH , Pizon AF . Methemoglobinemia as a complication of topical dapsone . N Engl J Med 2015 ; 372 : 491 – 2 . 4. Uspenskaia ID , Shabunina EL , Zhukova EA et al. Endogenous intoxication and biochemical protection in children with celiac disease: state assesment and correlation-regression analysis . Vestn Ross Akad Med Nauk 2014 , 93 – 9 . 5. Pollack ES , Pollack CV . Incidence of subclinical methemoglobinemia in infants with diarrhea . Ann Emerg Med 1994 ; 24 : 652 – 6 .

Endoscopic findings in patients with familial Mediterranean fever and dyspeptic symptoms

Aim The aim of the study was to evaluate the clinical, endoscopic, histopathological, laboratory, and imaging findings of patients with familial Mediterranean fever (FMF) presenting with dyspeptic symptoms. Material and methods Endoscopy was performed in 28 patients with FMF undergoing colchicine therapy, who presented to our hospital between 2012 and 2014 with abdominal pain, diarrhoea, and dyspeptic complaints. Upper gastrointestinal system endoscopy was performed in all patients; 16 of these underwent colonoscopy. Demographic characteristics, clinical and laboratory data, genetic analysis, imaging data, and endoscopy and colonoscopy findings were evaluated in all patients. Results Histopathological analysis of biopsy specimens revealed gastrointestinal pathology in 21 (75%) patients. Upper endoscopy analysis revealed that 14 (50%) patients had antral gastritis (8 Helicobacter pylori (–) antral gastritis and 6 H. pylori (+) chronic active gastritis) and 2 (7%) cases had giardiasis. Four antral gastritis cases had concomitant esophagitis, and 2 patients had widespread ulcers in the terminal ileum. The rest had a normal colonic appearance; however, three patients with normal colonoscopic findings were found to have chronic active colitis on histopathological examination. Conclusions In this study, most patients with FMF presenting with dyspeptic complaints had gastrointestinal pathology. Furthermore, there was higher inflammatory bowel disease incidence in these patients.

Frequency of Celiac Disease in Children with Peptic Ulcers

AbstractAim The aim of the present study is to investigate the frequency of celiac disease in children with peptic ulcers and to compare it with that of non-celiac peptic ulcers in terms of clinical and laboratory values.MethodsUpper gastrointestinal endoscopy was performed in 1769 patients at the Department of Pediatric Gastroenterology, The Faculty of Medicine, Cukurova University, Turkey, between January 2012 and January 2017. These cases consisted of subjects presenting with various GIS symptoms and indicated for endoscopy (with chronic diarrhea, delayed growth and development, abdominal pains, GIS bleeding, etc.). The levels of immunoglobulin A (IgA) serum anti-tissue transglutaminase antibodies, IgA anti-endomysial antibodies (EMA), and IgA serum were estimated in the patients with peptic ulcers.ResultsCeliac disease was diagnosed with serology, endoscopy, and histopathology in 250 (14%) of all cases undergoing endoscopy. Peptic ulcers were diagnosed in 74 patients (4.2%) of all cases undergoing endoscopy. tTGA and EMA (+) levels were determined in 22 (29%) of the 74 patients with peptic ulcers, and then the presence of peptic ulcers was investigated in the upper gastrointestinal system using gastrointestinal endoscopy, followed by histopathological confirmation of celiac disease. HP infection was present in 14 (63%) of the patients with celiac disease and in 23 (44%) of non-celiac peptic ulcers; the difference was not statistically significant (p = 0.12). In the total ulcer group, 10.8% (8/74) of patients with celiac peptic ulcers were negative for HP infection, whereas 21% (8/37) of HP-negative patients with ulcers had celiac disease.ConclusionThere exists a high risk of celiac disease in children with peptic ulcers. We thus recommend celiac disease to be investigated, particularly in HP-negative patients with peptic ulcers but with no history of NSAID use.

A Non-Frequently Considered Diagnosis of Dysphagia; Eosinophilic Esophagitis

Oz Eozinofilik ozofajit, ozofagus mukozasinin eozinofil lokositler ile infiltrasyonudur. Cocuklarda nadir gorulur ve bulgulari gastroozofageal reflu ile benzerdir. Yutma guclugu nedeni ile cocuk cerrahisinde ozofagus balon dilatasyonu uygulanan ve eozinofilik ozofajit oldugu saptanan bu olgu disfajili cocuga yaklasima dikkat cekmek amaci ile sunulmustur. Toplam IgE=834 IU/mL ve spesifik IgE (-), Fx5 (-) negatif saptandi. Ust gastrointestinal sistem endoskopisinde ozofagusun mukozasinin soluk, yapisinin sert, motilitesinin bozuk oldugu gozlendi ve birkac adet milimetrik beyaz lezyonlar goruldu. Ozofagus biyopsi materyallerinde mukozada eozinofil infiltrasyonun %60 oraninda oldugu gozlendi. Olguya eozinofilik ozofajit tanisi ile oral prednizolon 1 mg/kg/gun baslandi. Olgunun bir hafta sonraki poliklinik kontrolunde disfaji yakinmalarinda belirgin duzelme oldugu ve birinci ay kontrolunde hicbir yakinmasinin olmadigi gozlendi. Disfaji ve reflu benzeri semptomlarda ozellikle gastroozofageal reflu tedavisine yanit alinamiyorsa eozinofilik ozofajit tanisi mutlaka dikkate alinmalidir.