Aydan Kansu - Academia.edu (original) (raw)

Papers by Aydan Kansu

Research paper thumbnail of Role of Liver Biopsy in the Diagnosis of Liver Diseases in Children

Ankara Üniversitesi tıp fakültesi mecmuası, Mar 25, 2022

Merkezimizde yapılmış olan karaciğer biyopsileri endikasyon, klinik ve laboratuvar veriler, ön ta... more Merkezimizde yapılmış olan karaciğer biyopsileri endikasyon, klinik ve laboratuvar veriler, ön tanı ve histopatolojik tanılar açısından analiz edilerek elde edilen klinik ve laboratuvar verileriyle, karaciğer biyopsisinin tanıdaki etkinliğinin belirlenmesi amaçlanmıştır. Gereç ve Yöntem: Çalışma, 2008-2017 yılları arasında yenidoğan kolestazı, kronik karaciğer hastalığı, izole hipertransaminazemi, izole hepatomegali ve ultrasonografik parankimal bulgularla karaciğer biyopsisi yapılan 0-18 yaş grubundaki hastaların demografik, fizik inceleme, laboratuvar ve ultrasonografi bulguları, biyopsi endikasyonları ve histopatolojik tanı bilgilerinin geriye dönük incelenmesiyle yürütülmüştür. Hastalar, ön tanısı olan, ön tanının doğrulanması amacıyla karaciğer biyopsi yapılanlar (Grup 1) ve klinik ve laboratuvar olarak ön tanısı olmayan, tanı amacıyla biyopsi yapılanlar (Grup 2) olarak ikiye ayrılarak değerlendirilmiştir. Bulgular: Yaş ortalaması 8,8±5,7 olan, 139'u (%53) erkek olan 262 hasta çalışmaya alınmıştır. Hastaların 183'ü (%69,8) Grup 1'de, 79'u (%30,2) Grup 2'de bulunmaktadır. Grup 1'de hastaların %87,4'ünde, Grup 2'de hastaların %26,6'sında kesin tanıya ulaşılmıştır. Biyopsiyle tanı elde edilebilmesinde ön tanı varlığı (p=0,001), fiziksel incelemede hepatomegali saptanması (p=0,001), splenomegali saptanması (p=0,001) ve ultrasonografide karaciğer parankiminde patoloji saptanması (p=0,008) istatistiksel olarak anlamlı bulunmuştur. Sonuç: Tüm hasta grubumuzda, hastaların %69,1'i kesin tanı almıştır. Ön tanı varlığının histopatolojik olarak kesin tanı elde edebilmede istatistiksel olarak anlamlı olduğu görülmüştür. Ancak verilerimiz sonucunda klinik, laboratuvar ve görüntüleme yöntemleriyle ön tanıya gidilemeyen hastalarda da karaciğer biyopsisinin riskleri ve spesifik bir tanı elde etmedeki yararları göz önünde bulundurularak, hasta özelinde biyopsi kararı verilmesinin uygun olacağı düşünülmüştür.

Research paper thumbnail of Açıklanamayan Transaminaz Yüksekliği ve Atipik Çölyak Hastalığı: Olgu Sunumu

Türkiye çocuk hastalıkları dergisi, Jun 1, 2008

Research paper thumbnail of The frequency of Duchenne muscular dystrophy/Becker muscular dystrophy and Pompe disease in children with isolated transaminase elevation: results from the observational VICTORIA study

Frontiers in Pediatrics

IntroductionElevated transaminases and/or creatine phosphokinase can indicate underlying muscle d... more IntroductionElevated transaminases and/or creatine phosphokinase can indicate underlying muscle disease. Therefore, this study aims to determine the frequency of Duchenne muscular dystrophy/Becker muscular dystrophy (DMD/BMD) in male children and Pompe disease (PD) in male and female children with isolated hypertransaminasemia.MethodsThis multi-center, prospective study enrolled patients aged 3–216 months with serum alanine transaminase (ALT) and/or aspartate transaminase (AST) levels >2× the upper limit of normal (ULN) for ≥3 months. Patients with a known history of liver or muscle disease or physical examination findings suggestive of liver disease were excluded. Patients were screened for creatinine phosphokinase (CPK) levels, and molecular genetic tests for DMD/BMD in male patients and enzyme analysis for PD in male and female patients with elevated CPK levels were performed. Genetic analyses confirmed PD. Demographic, clinical, and laboratory characteristics of the patients ...

Research paper thumbnail of The significance of MUAC z-scores in diagnosing pediatric malnutrition: A scoping review with special emphasis on neurologically disabled children

Frontiers in Pediatrics

This review by a panel of pediatric gastroenterology-hepatology-nutrition and pediatric neurology... more This review by a panel of pediatric gastroenterology-hepatology-nutrition and pediatric neurology experts aimed to address the significance of mid-upper arm circumference (MUAC) assessment in diagnosis of pediatric malnutrition. Specifically, the potential utility of recently developed MUAC z-score tape in clinical practice for larger patient populations was addressed including the neurologically disabled children. In accordance with the evidence-based data, four statements were identified by the participating experts on the utility of MUAC z-score tape, including (1) MUAC z-scores correlate with body mass index (BMI) and weight for height/length (WFH/l) z-scores in diagnosing malnutrition; (2) MUAC z-score tape offers a higher sensitivity to diagnose the mild and moderate malnutrition and better ability to track the changes in nutritional status over time than the other single datapoint measurements; (3) Using single-step MUAC z-score tape in children with cerebral palsy (CP) seems...

Research paper thumbnail of A Rare Contiguous Gene Deletion Leading to Trichothiodystrophy Type 4 and Glutaric Aciduria Type 3

Molecular Syndromology

Introduction: Trichothiodystrophy type 4 and glutaric aciduria type 3 are rare autosomal recessiv... more Introduction: Trichothiodystrophy type 4 and glutaric aciduria type 3 are rare autosomal recessive disorders caused by biallelic variants in the MPLKIP and SUGCT genes on chromosome 7p14, respectively. Trichothiodystrophy type 4 is characterized by neurologic and cutaneous abnormalities. Glutaric aciduria type 3 is a rare metabolic disorder with inconsistent phenotype and elevated urinary excretion of glutaric acid. Case Presentation: Here, we report on an infant presenting with hypotonia, failure to thrive, microcephaly, dysmorphic features, brittle hair, hypertransaminasemia, and recurrent lower respiratory tract infections. Microarray analysis revealed a homozygous microdeletion involving the MPLKIP and SUGCT genes, which are located close to each other. Conclusion: Copy number variations should be considered in patients with coexisting clinical expression of different genetic alterations. To the best of our knowledge, our patient is the second case with co-occurrence of trichoth...

Research paper thumbnail of Celiac Disease? Should We Screen Children with Functional Constipation for

DergiPark (Istanbul University), Dec 1, 2016

Kabızlık yakınması ile getirilen çocuklarda organik nedenlerin dışlanması için tanısal testlere i... more Kabızlık yakınması ile getirilen çocuklarda organik nedenlerin dışlanması için tanısal testlere ihtiyaç duyulabilir. Kabızlık çölyak hastalığının ilk bulgusu olabilir. Çalışmada fonksiyonel kabızlık tanısı alan çocuklarda dışkılama özelliklerinin ve çölyak hastalığı sıklığının belirlenmesi amaçlandı. Gereç ve Yöntemler: Roma III ölçütlerine göre fonksiyonel kabızlığı olan 150 çocuk dosya taraması ile geriye dönük olarak değerlendirildi. Bulgular: Ortalama 6,5 ± 4,2 (1-17) yaşında olan, 150 çocuğun [74 kız (% 49.3)], ortanca 24 (1-198) aydır kabızlığı vardı. Ortalama kabızlık başlangıç yaşı 3,6 ± 3,7 yıldı. Ağrılı dışkılaması olanlar (ortalama 3.1 ± 3.4 yıl), diğerlerinden (ortalama 5 ± 4.3 yıl) daha küçüktü (p= 0.018). Büyük ve sert dışkılaması olanlar (ortalama 7.7 ± 4.3 yıl), çakıl taşı benzeri küçük ve sert yapanlara (ortalama 5.2 ± 4.2 yıl) ve her iki tipte de dışkılaması olanlara (ortalama 5 ± 3.5 yıl) göre daha büyüktü (sırasıyla p = 0.002, p = 0.003). Dışkı tutma davranışı olanlarda, ağrılı dışkılama, kanama ve dışkı kaçırma sıklığı daha yüksekti (sırasıyla p = 0.034, p = 0.001, p = 0.001). İdrar yolu enfeksiyonu öyküsü büyük çocuklarda ve kızlarda (36 kız, %67.9) daha sıktı (sırasıyla p = 0.008, p = 0.001). Olguların 1'inde (% 0.65) doku transglutaminaz IgA (+) olup ince bağırsak biyopsisi ile çölyak hastalığı tanısı aldı. Sonuç: Fonksiyonel kabızlığı olan çocuklarda çölyak hastalığı sıklığı yüksek bulunmamıştır.

Research paper thumbnail of Role of Liver Biopsy in the Diagnosis of Liver Diseases in Children

Journal of Ankara University Faculty of Medicine, 2022

Merkezimizde yapılmış olan karaciğer biyopsileri endikasyon, klinik ve laboratuvar veriler, ön ta... more Merkezimizde yapılmış olan karaciğer biyopsileri endikasyon, klinik ve laboratuvar veriler, ön tanı ve histopatolojik tanılar açısından analiz edilerek elde edilen klinik ve laboratuvar verileriyle, karaciğer biyopsisinin tanıdaki etkinliğinin belirlenmesi amaçlanmıştır. Gereç ve Yöntem: Çalışma, 2008-2017 yılları arasında yenidoğan kolestazı, kronik karaciğer hastalığı, izole hipertransaminazemi, izole hepatomegali ve ultrasonografik parankimal bulgularla karaciğer biyopsisi yapılan 0-18 yaş grubundaki hastaların demografik, fizik inceleme, laboratuvar ve ultrasonografi bulguları, biyopsi endikasyonları ve histopatolojik tanı bilgilerinin geriye dönük incelenmesiyle yürütülmüştür. Hastalar, ön tanısı olan, ön tanının doğrulanması amacıyla karaciğer biyopsi yapılanlar (Grup 1) ve klinik ve laboratuvar olarak ön tanısı olmayan, tanı amacıyla biyopsi yapılanlar (Grup 2) olarak ikiye ayrılarak değerlendirilmiştir. Bulgular: Yaş ortalaması 8,8±5,7 olan, 139'u (%53) erkek olan 262 hasta çalışmaya alınmıştır. Hastaların 183'ü (%69,8) Grup 1'de, 79'u (%30,2) Grup 2'de bulunmaktadır. Grup 1'de hastaların %87,4'ünde, Grup 2'de hastaların %26,6'sında kesin tanıya ulaşılmıştır. Biyopsiyle tanı elde edilebilmesinde ön tanı varlığı (p=0,001), fiziksel incelemede hepatomegali saptanması (p=0,001), splenomegali saptanması (p=0,001) ve ultrasonografide karaciğer parankiminde patoloji saptanması (p=0,008) istatistiksel olarak anlamlı bulunmuştur. Sonuç: Tüm hasta grubumuzda, hastaların %69,1'i kesin tanı almıştır. Ön tanı varlığının histopatolojik olarak kesin tanı elde edebilmede istatistiksel olarak anlamlı olduğu görülmüştür. Ancak verilerimiz sonucunda klinik, laboratuvar ve görüntüleme yöntemleriyle ön tanıya gidilemeyen hastalarda da karaciğer biyopsisinin riskleri ve spesifik bir tanı elde etmedeki yararları göz önünde bulundurularak, hasta özelinde biyopsi kararı verilmesinin uygun olacağı düşünülmüştür.

Research paper thumbnail of Çocukluk Çağında Fonksiyonel Kabızlık Tedavisi: Senna, Trimebutin ve Laktülozun Karşılaştırılması

Turkish Journal of Pediatric Disease, 2020

Objective: Data for the drugs used in maintenance therapy of functional constipation is limited i... more Objective: Data for the drugs used in maintenance therapy of functional constipation is limited in childhood. We aimed to evaluate the efficacy of senna, trimebutine, and lactulose in children with functional constipation (FC) or functional fecal incontinence (FFI). Material and Methods: Patients (2-18 years old) diagnosed as FC or FFI according to Rome III criteria who were treated with lactulose (n=36), senna (n=29), and trimebutine (n=33)were included. Patients completed a 0-5 Likert scale questionnaire about their symptoms. Stool consistency was evaluated by the Bristol Stool Chart. Compliance and tolerance to treatment and side effects were recorded. Clinical improvement and pharmacoeconomic evaluation were performed. Results: Clinical improvement in the senna group was significantly higher (p=0.036). Senna showed the highest improvement in stool consistency (p<0.001) and decreased fecal incontinence (p=0.039). Abdominal distention, abdominal pain, nausea/vomiting, and anal fissure were improved by trimebutine (p<0.05). Both senna and trimebutine decreased painful defecation and stool withholding. Lactulose improved anorexia and anal fissure. Senna was the most cost-effective drug. Treatment options for constipation should be tailored according to the predominant symptom. Lactulose may be recommended as the initial treatment for children with anorexia or anal fissure, trimebutine for abdominal distention, abdominal pain, nausea/vomiting, or anal fissure, respectively. Senna may be prescribed for the cost-effectiveness with high clinical improvement and less unpleasant taste.

Research paper thumbnail of High-fibre enteral feeding results in improved anthropometrics and favourable gastrointestinal tolerance in malnourished children with growth failure

Acta Paediatrica, 2018

Aim: The practical value of using fibre-enriched enteral feeding regimens to rehabilitate malnour... more Aim: The practical value of using fibre-enriched enteral feeding regimens to rehabilitate malnourished children remains inconclusive. This study determined the usage patterns, gastrointestinal tolerance, anthropometrics and safety of high-fibre enteral feeding in malnourished children with growth failure. Methods: This Turkish observational study between February 2013 and June 2015 comprised 345 paediatric patients from 17 centres with malnutrition-related growth failure, with a weight and height of <2 SD percentiles for their age. Changes in anthropometrics, gastrointestinal symptoms, defecation habits and safety data relating to adverse events were analysed during the six-month follow-up period. Results: Most subjects (99.7%) were supplemented with enteral feeding. The absolute difference and 95% confidence interval values for the Z scores of height for age, weight for age, weight for height and body mass index for height increased significantly in four months to six months to 0.21 (0.09-0.32), 0.61 (0.51-0.70), 0.81 (0.56-1.06) and 0.70 (0.53-0.86), respectively (p < 0.001 for each). The percentage of patients with normal defecation frequency significantly increased from 70.3% to 92.8% at the four months to six months visit (p = 0.004). Adverse events occurred in 15 (4.3%) of patients. Using a six-month high-fibre enteral feeding was associated with favourable outcomes in anthropometrics, appetite, gastrointestinal tolerance and safety in malnourished children.

Research paper thumbnail of Change of Helicobacter pylori prevalence in a decade among children undergoing endoscopy

The Turkish journal of pediatrics, 2016

Our aim was to investigate the trend of H. pylori infection among children during the last decade... more Our aim was to investigate the trend of H. pylori infection among children during the last decade by a retrospective analysis. Reports of children in whom esophago-gastroduodenoscopy was performed at our institution during two periods 2002-2003 and 2012-2013 were seperated into Group I and Group II, respectively. Pathology reports were investigated for gastritis, atrophy and H. pylori presence. A total of 380 children, 131 in Group I and 249 in Goup II were recruited in the study. H. pylori postivity was found to be higher in Group I (% 48.1 and % 23.1, respectively, p < 0.001). Gastritis and atrophy were associated with H. pylori and both were more prevalent in Group I (p < 0.001). Our study demonstrates that H. pylori prevalence is decreasing in a pediatric population undergoing EGD in Ankara. This is the most recent study regarding pediatric H. pylori prevalence change in Turkey that we know of.

Research paper thumbnail of Variability of anti-human transglutaminase testing in celiac disease across Mediterranean countries

World journal of gastroenterology, Jan 28, 2017

To verify the precision and accuracy of transglutaminase antibodies (TGA) assays across Mediterra... more To verify the precision and accuracy of transglutaminase antibodies (TGA) assays across Mediterranean countries. This study involved 8 referral centres for celiac disease (CD) in 7 Mediterranean countries. A central laboratory prepared 8 kits of 7 blinded and randomized serum samples, with a titrated amount of Human TGA IgA. Each sample was analysed three times on three different days, with each centre running a total of 21 tests. The results were included in a blindly coded report form, which was sent to the coordinator centre. The coordinator estimated the mean coefficient of Variation (CoVar = σ/μ), the mean accuracy (Accur = Vobserved - Vreal) and the mean percent variation (Var% = [(Vobserved - Vreal)/Vreal] × 100). The analysis showed that 79.17% of the mean variation fell between -25% and +25% of the expected value, with the accuracy and precision progressively increasing with higher titres of TGA. From values 1.25 times greater than the normal cut-off, the measurements were ...

Research paper thumbnail of Diagnosis of celiac disease and applicability of ESPGHAN guidelines in Mediterranean countries: a real life prospective study

BMC gastroenterology, Jan 21, 2017

We assessed how the diagnosis of Celiac Disease (CD) is made and how the new ESPGHAN guidelines c... more We assessed how the diagnosis of Celiac Disease (CD) is made and how the new ESPGHAN guidelines can be applied in children from countries with different resources. A real life prospective study was performed in 14 centres of 13 different Mediterranean countries. Participants were asked to apply the usual diagnostic work-up for CD according to their diagnostic facilities. There were 1974 patients enrolled in the study, mean age 4 years, 10 months; 865 male, 1109 female. CD was confirmed in 511 (25.9%) and was unconfirmed in 1391 (70.5%) patients; 14 patients were diagnosed as having CD according to the new ESPGHAN guidelines, 43 patients were classified as having potential CD. In all participating countries the diagnosis of CD relied on histology of duodenal biopsy; in 5 countries, HLA, and in one country endomysial antibodies (EMA) were not available. Symptoms did not add a significant increase to the pre-test probability of serological tests. The positive predictive value of tissue...

Research paper thumbnail of Can partially hydrolyzed guar gum be an alternative to lactulose in treatment of childhood constipation?

The Turkish Journal of Gastroenterology, 2010

Bu çal›flmada, çocuklarda kab›zl›¤›n tedavisinde, lif kayna¤› olarak k›smi hidrolize guar gumun k... more Bu çal›flmada, çocuklarda kab›zl›¤›n tedavisinde, lif kayna¤› olarak k›smi hidrolize guar gumun kullan›m›n›n gü-venli¤i ve baflar›s›n›n, en s›k kullan›lan ozmotik laksatif olan laktuloz ile karfl›laflt›r›lmas› amaçlanm›flt›r. Yöntem: 61 hastada (31 k›smi hidrolize guar gum, 30 laktuloz) randomize prospektif kontrollü çal›flma yap›ld›. Hastalara 4 hafta boyunca laktuloz veya k›smi hidrolize guar gum verildi. Standardize edilmifl bir form verilerek d›flk›lama s›kl›¤›, d›flk› k›vam›, afl›r› gaz ç›karma ve kar›n a¤r›s›n›n kay›t edilmesi istendi. Her iki tedavi grubunda baflar›, güvenilirlik ve yan etki profili aile anketi ile elde olundu. Bulgular: Günlük bazal lif (meyve ve sebze) tüketiminde her 2 grup aras›nda fark saptanmad›. Kar›n a¤r›s› olan ve d›flk› tutan çocuklar›n yüzdesi her 2 tedavi grubunda belirgin olarak azald› (p<0.05). Laktuloz grubunda haf-tal›k d›flk›lama s›kl›¤›nda 4+0.7'den 6+1.06'ya, k›smi hidrolize guar gum alan hasta grubunda ise 4+0.7'den 5+1.7'ye art›fl oldu (p<0.05). Aile anketinde, laktuloz tedavi grubunda ebeveynler kötü tad, afl›r› gaz ç›karma ve fazla miktarda ilaç tüketim zorunlu¤undan yak›nd›lar. K›smi hidrolize guar gum tedavi grubunda ise ebeveynler çocuklar›n›n d›flk›lama s›kl›¤›ndan memnundular. Sonuç: D›flk› tutma ve kar›n a¤r›s› ile beraber olan kab›zl›¤›n giderilmesinde k›smi hidrolize guar gum tedavisi, laktuloz tedavisi kadar etkin bulunmufltur. Laktulozun afl›r› gaz hissi ve kötü tad gibi yan etkileri daha çok bulunmak-tad›r.

Research paper thumbnail of Açıklanamayan Transaminaz Yüksekliği ve Atipik Çölyak Hastalığı: Olgu Sunumu

Turkiye Cocuk Hastalıkları Dergisi, Jun 1, 2008

Research paper thumbnail of Ciddi kanamayla tanı alan alfa 1 antitripsin eksikliği olan bir olgu Olgu Sunumu

Turk Pediatri Arsivi-turkish Archives of Pediatrics, Dec 1, 2006

Alfa-1 antitripsin eksikliği (A1ATE) karaciğer hücresi endoplazmik retikulumunda (ER) sıra dışı A... more Alfa-1 antitripsin eksikliği (A1ATE) karaciğer hücresi endoplazmik retikulumunda (ER) sıra dışı A1AT birikimine neden olarak karaciğer hasarına yol açan otozomal çekinik (resesif) geçen kalıtsal bir hastalıktır. Alfa-1 antitripsin eksikliği bebeklik dönemi hemorajik hastalığına, kolestaza ya da kronik karaciğer hastalığına neden olabilir. Kolestaz ve bebeklik dönemi hemorajik hastalığı gelişen bir A1ATE (PiZZ fenotipi) olan olgu sunulmuştur. Bu olgu ile yenidoğanın geç hemorajik hastalığının nedeninin tanı almamış kolestaz olabileceğinin unutulmaması, 15 günden uzun süren sarılığı olan bebeklerin yenidoğan kolestaz nedenleri açısından mutlaka değerlendirilmeleri gerektiği, erken tanı almayan yenidoğan kolestazlarının kafa içi kanama gibi ciddi sonuçlar doğurabileceği ve erken tanı ve tedavinin önemi vurgulanmak istendi. Alpha-1-antitrypsin deficiency is an autosomal recessive disease in which liver disease results from retention of abnormal alpha-1-antitrypsin in the endoplasmic reticulum of hepatocytes. In childhood, alpha-1-antitrypsin deficiency may present as cholestasis, late hemorrhagic disease of infancy or chronic liver disease. Here, we present a case with alpha-1-antitrypsin deficiency who developed cholestasis and late hemorrahagic disease of infancy. We emphasize that the cause of late hemorrhagic disease of infancy may be unrecognized neonatal cholestasis and, that all infants who have jaundice for more than 15 days should be evaluated for neonatal cholestasis. Unrecognized neonatal cholestasis can result in severe conditions such as intracranial bleeding therefore, early diagnosis and treatment are essential.

Research paper thumbnail of Crohn's disease of the vulva in a 10-year-old girl

The Turkish journal of pediatrics

Crohn's disease may involve all parts of the gastrointestinal tract and may often involve oth... more Crohn's disease may involve all parts of the gastrointestinal tract and may often involve other organs as well. These non-intestinal affections are termed extraintestinal manifestations. Vulval involvement is an uncommon extraintestinal manifestation of Crohn's disease, and it is very rare in children. Patients with vulval CD typically present with erythema and edema of the labia majora, which progresses to extensive ulcer formation. Vulval Crohn's disease can appear before or after intestinal problems or it may occur simultaneously. We present a 10-year-old girl with intestinal Crohn's disease complicated with perianal skin tags and asymptomatic unilateral labial hypertrophy. The course of her lesion was independent of the intestinal disease and responded significantly to medical treatment including azathioprine and topical steroid. We emphasize that although vulval involvement in childhood is uncommon, Crohn's disease must be considered in the differential diag...

Research paper thumbnail of 976 Cyclosporine-Associated Thrombotic Microangiopathy and Thrombocytopenia-Associated Multiple Organ Failure: a Case Successfully Treated with Therapeutic Plasma Exchange

Archives of Disease in Childhood, 2012

The signal-to-noise ratio, i.e., ΔrStO 2 /S w, was 17.6, 14.5, and 12.5 for FORE-SIGHT, INVOS and... more The signal-to-noise ratio, i.e., ΔrStO 2 /S w, was 17.6, 14.5, and 12.5 for FORE-SIGHT, INVOS and NONIN, respectively. Conclusion The different absolute values and dynamic ranges will make comparison of data collected with different devices difficult.

Research paper thumbnail of Burden of celiac disease in the Mediterranean area

World Journal of Gastroenterology, 2011

To estimate the burden of undiagnosed celiac disease (CD) in the Mediterranean area in terms of m... more To estimate the burden of undiagnosed celiac disease (CD) in the Mediterranean area in terms of morbidity, mortality and health cost. For statistics regarding the population of each country in the Mediterranean area, we accessed authoritative international sources (World Bank, World Health Organization and United Nations). The prevalence of CD was obtained for most countries from published reports. An overall prevalence rate of 1% cases/total population was finally estimated to represent the frequency of the disease in the area, since none of the available confidence intervals of the reported rates significantly excluded this rate. The distribution of symptoms and complications was obtained from reliable reports in the same cohort. A standardized mortality rate of 1.8 was obtained from recent reports. Crude health cost was estimated for the years between symptoms and diagnosis for adults and children, and was standardized for purchasing power parity to account for the different economic profiles amongst Mediterranean countries.

Research paper thumbnail of Celiac disease in the Mediterranean area

BMC Gastroenterology, 2014

Background The World Gastroenterology Organization recommends developing national guidelines for ... more Background The World Gastroenterology Organization recommends developing national guidelines for the diagnosis of Celiac Disease (CD): hence a profile of the diagnosis of CD in each country is required. We aim to describe a cross-sectional picture of the clinical features and diagnostic facilities in 16 countries of the Mediterranean basin. Since a new ESPGHAN diagnostic protocol was recently published, our secondary aim is to estimate how many cases in the same area could be identified without a small intestinal biopsy. Methods By a stratified cross-sectional retrospective study design, we examined clinical, histological and laboratory data from 749 consecutive unselected CD children diagnosed by national referral centers. Results The vast majority of cases were diagnosed before the age of 10 (median: 5 years), affected by diarrhea, weight loss and food refusal, as expected. Only 59 cases (7.8%) did not suffer of major complaints. Tissue transglutaminase (tTG) assay was available, ...

Research paper thumbnail of Effects of cisapride on ventricular repolarization in children

Research paper thumbnail of Role of Liver Biopsy in the Diagnosis of Liver Diseases in Children

Ankara Üniversitesi tıp fakültesi mecmuası, Mar 25, 2022

Merkezimizde yapılmış olan karaciğer biyopsileri endikasyon, klinik ve laboratuvar veriler, ön ta... more Merkezimizde yapılmış olan karaciğer biyopsileri endikasyon, klinik ve laboratuvar veriler, ön tanı ve histopatolojik tanılar açısından analiz edilerek elde edilen klinik ve laboratuvar verileriyle, karaciğer biyopsisinin tanıdaki etkinliğinin belirlenmesi amaçlanmıştır. Gereç ve Yöntem: Çalışma, 2008-2017 yılları arasında yenidoğan kolestazı, kronik karaciğer hastalığı, izole hipertransaminazemi, izole hepatomegali ve ultrasonografik parankimal bulgularla karaciğer biyopsisi yapılan 0-18 yaş grubundaki hastaların demografik, fizik inceleme, laboratuvar ve ultrasonografi bulguları, biyopsi endikasyonları ve histopatolojik tanı bilgilerinin geriye dönük incelenmesiyle yürütülmüştür. Hastalar, ön tanısı olan, ön tanının doğrulanması amacıyla karaciğer biyopsi yapılanlar (Grup 1) ve klinik ve laboratuvar olarak ön tanısı olmayan, tanı amacıyla biyopsi yapılanlar (Grup 2) olarak ikiye ayrılarak değerlendirilmiştir. Bulgular: Yaş ortalaması 8,8±5,7 olan, 139'u (%53) erkek olan 262 hasta çalışmaya alınmıştır. Hastaların 183'ü (%69,8) Grup 1'de, 79'u (%30,2) Grup 2'de bulunmaktadır. Grup 1'de hastaların %87,4'ünde, Grup 2'de hastaların %26,6'sında kesin tanıya ulaşılmıştır. Biyopsiyle tanı elde edilebilmesinde ön tanı varlığı (p=0,001), fiziksel incelemede hepatomegali saptanması (p=0,001), splenomegali saptanması (p=0,001) ve ultrasonografide karaciğer parankiminde patoloji saptanması (p=0,008) istatistiksel olarak anlamlı bulunmuştur. Sonuç: Tüm hasta grubumuzda, hastaların %69,1'i kesin tanı almıştır. Ön tanı varlığının histopatolojik olarak kesin tanı elde edebilmede istatistiksel olarak anlamlı olduğu görülmüştür. Ancak verilerimiz sonucunda klinik, laboratuvar ve görüntüleme yöntemleriyle ön tanıya gidilemeyen hastalarda da karaciğer biyopsisinin riskleri ve spesifik bir tanı elde etmedeki yararları göz önünde bulundurularak, hasta özelinde biyopsi kararı verilmesinin uygun olacağı düşünülmüştür.

Research paper thumbnail of Açıklanamayan Transaminaz Yüksekliği ve Atipik Çölyak Hastalığı: Olgu Sunumu

Türkiye çocuk hastalıkları dergisi, Jun 1, 2008

Research paper thumbnail of The frequency of Duchenne muscular dystrophy/Becker muscular dystrophy and Pompe disease in children with isolated transaminase elevation: results from the observational VICTORIA study

Frontiers in Pediatrics

IntroductionElevated transaminases and/or creatine phosphokinase can indicate underlying muscle d... more IntroductionElevated transaminases and/or creatine phosphokinase can indicate underlying muscle disease. Therefore, this study aims to determine the frequency of Duchenne muscular dystrophy/Becker muscular dystrophy (DMD/BMD) in male children and Pompe disease (PD) in male and female children with isolated hypertransaminasemia.MethodsThis multi-center, prospective study enrolled patients aged 3–216 months with serum alanine transaminase (ALT) and/or aspartate transaminase (AST) levels >2× the upper limit of normal (ULN) for ≥3 months. Patients with a known history of liver or muscle disease or physical examination findings suggestive of liver disease were excluded. Patients were screened for creatinine phosphokinase (CPK) levels, and molecular genetic tests for DMD/BMD in male patients and enzyme analysis for PD in male and female patients with elevated CPK levels were performed. Genetic analyses confirmed PD. Demographic, clinical, and laboratory characteristics of the patients ...

Research paper thumbnail of The significance of MUAC z-scores in diagnosing pediatric malnutrition: A scoping review with special emphasis on neurologically disabled children

Frontiers in Pediatrics

This review by a panel of pediatric gastroenterology-hepatology-nutrition and pediatric neurology... more This review by a panel of pediatric gastroenterology-hepatology-nutrition and pediatric neurology experts aimed to address the significance of mid-upper arm circumference (MUAC) assessment in diagnosis of pediatric malnutrition. Specifically, the potential utility of recently developed MUAC z-score tape in clinical practice for larger patient populations was addressed including the neurologically disabled children. In accordance with the evidence-based data, four statements were identified by the participating experts on the utility of MUAC z-score tape, including (1) MUAC z-scores correlate with body mass index (BMI) and weight for height/length (WFH/l) z-scores in diagnosing malnutrition; (2) MUAC z-score tape offers a higher sensitivity to diagnose the mild and moderate malnutrition and better ability to track the changes in nutritional status over time than the other single datapoint measurements; (3) Using single-step MUAC z-score tape in children with cerebral palsy (CP) seems...

Research paper thumbnail of A Rare Contiguous Gene Deletion Leading to Trichothiodystrophy Type 4 and Glutaric Aciduria Type 3

Molecular Syndromology

Introduction: Trichothiodystrophy type 4 and glutaric aciduria type 3 are rare autosomal recessiv... more Introduction: Trichothiodystrophy type 4 and glutaric aciduria type 3 are rare autosomal recessive disorders caused by biallelic variants in the MPLKIP and SUGCT genes on chromosome 7p14, respectively. Trichothiodystrophy type 4 is characterized by neurologic and cutaneous abnormalities. Glutaric aciduria type 3 is a rare metabolic disorder with inconsistent phenotype and elevated urinary excretion of glutaric acid. Case Presentation: Here, we report on an infant presenting with hypotonia, failure to thrive, microcephaly, dysmorphic features, brittle hair, hypertransaminasemia, and recurrent lower respiratory tract infections. Microarray analysis revealed a homozygous microdeletion involving the MPLKIP and SUGCT genes, which are located close to each other. Conclusion: Copy number variations should be considered in patients with coexisting clinical expression of different genetic alterations. To the best of our knowledge, our patient is the second case with co-occurrence of trichoth...

Research paper thumbnail of Celiac Disease? Should We Screen Children with Functional Constipation for

DergiPark (Istanbul University), Dec 1, 2016

Kabızlık yakınması ile getirilen çocuklarda organik nedenlerin dışlanması için tanısal testlere i... more Kabızlık yakınması ile getirilen çocuklarda organik nedenlerin dışlanması için tanısal testlere ihtiyaç duyulabilir. Kabızlık çölyak hastalığının ilk bulgusu olabilir. Çalışmada fonksiyonel kabızlık tanısı alan çocuklarda dışkılama özelliklerinin ve çölyak hastalığı sıklığının belirlenmesi amaçlandı. Gereç ve Yöntemler: Roma III ölçütlerine göre fonksiyonel kabızlığı olan 150 çocuk dosya taraması ile geriye dönük olarak değerlendirildi. Bulgular: Ortalama 6,5 ± 4,2 (1-17) yaşında olan, 150 çocuğun [74 kız (% 49.3)], ortanca 24 (1-198) aydır kabızlığı vardı. Ortalama kabızlık başlangıç yaşı 3,6 ± 3,7 yıldı. Ağrılı dışkılaması olanlar (ortalama 3.1 ± 3.4 yıl), diğerlerinden (ortalama 5 ± 4.3 yıl) daha küçüktü (p= 0.018). Büyük ve sert dışkılaması olanlar (ortalama 7.7 ± 4.3 yıl), çakıl taşı benzeri küçük ve sert yapanlara (ortalama 5.2 ± 4.2 yıl) ve her iki tipte de dışkılaması olanlara (ortalama 5 ± 3.5 yıl) göre daha büyüktü (sırasıyla p = 0.002, p = 0.003). Dışkı tutma davranışı olanlarda, ağrılı dışkılama, kanama ve dışkı kaçırma sıklığı daha yüksekti (sırasıyla p = 0.034, p = 0.001, p = 0.001). İdrar yolu enfeksiyonu öyküsü büyük çocuklarda ve kızlarda (36 kız, %67.9) daha sıktı (sırasıyla p = 0.008, p = 0.001). Olguların 1'inde (% 0.65) doku transglutaminaz IgA (+) olup ince bağırsak biyopsisi ile çölyak hastalığı tanısı aldı. Sonuç: Fonksiyonel kabızlığı olan çocuklarda çölyak hastalığı sıklığı yüksek bulunmamıştır.

Research paper thumbnail of Role of Liver Biopsy in the Diagnosis of Liver Diseases in Children

Journal of Ankara University Faculty of Medicine, 2022

Merkezimizde yapılmış olan karaciğer biyopsileri endikasyon, klinik ve laboratuvar veriler, ön ta... more Merkezimizde yapılmış olan karaciğer biyopsileri endikasyon, klinik ve laboratuvar veriler, ön tanı ve histopatolojik tanılar açısından analiz edilerek elde edilen klinik ve laboratuvar verileriyle, karaciğer biyopsisinin tanıdaki etkinliğinin belirlenmesi amaçlanmıştır. Gereç ve Yöntem: Çalışma, 2008-2017 yılları arasında yenidoğan kolestazı, kronik karaciğer hastalığı, izole hipertransaminazemi, izole hepatomegali ve ultrasonografik parankimal bulgularla karaciğer biyopsisi yapılan 0-18 yaş grubundaki hastaların demografik, fizik inceleme, laboratuvar ve ultrasonografi bulguları, biyopsi endikasyonları ve histopatolojik tanı bilgilerinin geriye dönük incelenmesiyle yürütülmüştür. Hastalar, ön tanısı olan, ön tanının doğrulanması amacıyla karaciğer biyopsi yapılanlar (Grup 1) ve klinik ve laboratuvar olarak ön tanısı olmayan, tanı amacıyla biyopsi yapılanlar (Grup 2) olarak ikiye ayrılarak değerlendirilmiştir. Bulgular: Yaş ortalaması 8,8±5,7 olan, 139'u (%53) erkek olan 262 hasta çalışmaya alınmıştır. Hastaların 183'ü (%69,8) Grup 1'de, 79'u (%30,2) Grup 2'de bulunmaktadır. Grup 1'de hastaların %87,4'ünde, Grup 2'de hastaların %26,6'sında kesin tanıya ulaşılmıştır. Biyopsiyle tanı elde edilebilmesinde ön tanı varlığı (p=0,001), fiziksel incelemede hepatomegali saptanması (p=0,001), splenomegali saptanması (p=0,001) ve ultrasonografide karaciğer parankiminde patoloji saptanması (p=0,008) istatistiksel olarak anlamlı bulunmuştur. Sonuç: Tüm hasta grubumuzda, hastaların %69,1'i kesin tanı almıştır. Ön tanı varlığının histopatolojik olarak kesin tanı elde edebilmede istatistiksel olarak anlamlı olduğu görülmüştür. Ancak verilerimiz sonucunda klinik, laboratuvar ve görüntüleme yöntemleriyle ön tanıya gidilemeyen hastalarda da karaciğer biyopsisinin riskleri ve spesifik bir tanı elde etmedeki yararları göz önünde bulundurularak, hasta özelinde biyopsi kararı verilmesinin uygun olacağı düşünülmüştür.

Research paper thumbnail of Çocukluk Çağında Fonksiyonel Kabızlık Tedavisi: Senna, Trimebutin ve Laktülozun Karşılaştırılması

Turkish Journal of Pediatric Disease, 2020

Objective: Data for the drugs used in maintenance therapy of functional constipation is limited i... more Objective: Data for the drugs used in maintenance therapy of functional constipation is limited in childhood. We aimed to evaluate the efficacy of senna, trimebutine, and lactulose in children with functional constipation (FC) or functional fecal incontinence (FFI). Material and Methods: Patients (2-18 years old) diagnosed as FC or FFI according to Rome III criteria who were treated with lactulose (n=36), senna (n=29), and trimebutine (n=33)were included. Patients completed a 0-5 Likert scale questionnaire about their symptoms. Stool consistency was evaluated by the Bristol Stool Chart. Compliance and tolerance to treatment and side effects were recorded. Clinical improvement and pharmacoeconomic evaluation were performed. Results: Clinical improvement in the senna group was significantly higher (p=0.036). Senna showed the highest improvement in stool consistency (p<0.001) and decreased fecal incontinence (p=0.039). Abdominal distention, abdominal pain, nausea/vomiting, and anal fissure were improved by trimebutine (p<0.05). Both senna and trimebutine decreased painful defecation and stool withholding. Lactulose improved anorexia and anal fissure. Senna was the most cost-effective drug. Treatment options for constipation should be tailored according to the predominant symptom. Lactulose may be recommended as the initial treatment for children with anorexia or anal fissure, trimebutine for abdominal distention, abdominal pain, nausea/vomiting, or anal fissure, respectively. Senna may be prescribed for the cost-effectiveness with high clinical improvement and less unpleasant taste.

Research paper thumbnail of High-fibre enteral feeding results in improved anthropometrics and favourable gastrointestinal tolerance in malnourished children with growth failure

Acta Paediatrica, 2018

Aim: The practical value of using fibre-enriched enteral feeding regimens to rehabilitate malnour... more Aim: The practical value of using fibre-enriched enteral feeding regimens to rehabilitate malnourished children remains inconclusive. This study determined the usage patterns, gastrointestinal tolerance, anthropometrics and safety of high-fibre enteral feeding in malnourished children with growth failure. Methods: This Turkish observational study between February 2013 and June 2015 comprised 345 paediatric patients from 17 centres with malnutrition-related growth failure, with a weight and height of <2 SD percentiles for their age. Changes in anthropometrics, gastrointestinal symptoms, defecation habits and safety data relating to adverse events were analysed during the six-month follow-up period. Results: Most subjects (99.7%) were supplemented with enteral feeding. The absolute difference and 95% confidence interval values for the Z scores of height for age, weight for age, weight for height and body mass index for height increased significantly in four months to six months to 0.21 (0.09-0.32), 0.61 (0.51-0.70), 0.81 (0.56-1.06) and 0.70 (0.53-0.86), respectively (p < 0.001 for each). The percentage of patients with normal defecation frequency significantly increased from 70.3% to 92.8% at the four months to six months visit (p = 0.004). Adverse events occurred in 15 (4.3%) of patients. Using a six-month high-fibre enteral feeding was associated with favourable outcomes in anthropometrics, appetite, gastrointestinal tolerance and safety in malnourished children.

Research paper thumbnail of Change of Helicobacter pylori prevalence in a decade among children undergoing endoscopy

The Turkish journal of pediatrics, 2016

Our aim was to investigate the trend of H. pylori infection among children during the last decade... more Our aim was to investigate the trend of H. pylori infection among children during the last decade by a retrospective analysis. Reports of children in whom esophago-gastroduodenoscopy was performed at our institution during two periods 2002-2003 and 2012-2013 were seperated into Group I and Group II, respectively. Pathology reports were investigated for gastritis, atrophy and H. pylori presence. A total of 380 children, 131 in Group I and 249 in Goup II were recruited in the study. H. pylori postivity was found to be higher in Group I (% 48.1 and % 23.1, respectively, p < 0.001). Gastritis and atrophy were associated with H. pylori and both were more prevalent in Group I (p < 0.001). Our study demonstrates that H. pylori prevalence is decreasing in a pediatric population undergoing EGD in Ankara. This is the most recent study regarding pediatric H. pylori prevalence change in Turkey that we know of.

Research paper thumbnail of Variability of anti-human transglutaminase testing in celiac disease across Mediterranean countries

World journal of gastroenterology, Jan 28, 2017

To verify the precision and accuracy of transglutaminase antibodies (TGA) assays across Mediterra... more To verify the precision and accuracy of transglutaminase antibodies (TGA) assays across Mediterranean countries. This study involved 8 referral centres for celiac disease (CD) in 7 Mediterranean countries. A central laboratory prepared 8 kits of 7 blinded and randomized serum samples, with a titrated amount of Human TGA IgA. Each sample was analysed three times on three different days, with each centre running a total of 21 tests. The results were included in a blindly coded report form, which was sent to the coordinator centre. The coordinator estimated the mean coefficient of Variation (CoVar = σ/μ), the mean accuracy (Accur = Vobserved - Vreal) and the mean percent variation (Var% = [(Vobserved - Vreal)/Vreal] × 100). The analysis showed that 79.17% of the mean variation fell between -25% and +25% of the expected value, with the accuracy and precision progressively increasing with higher titres of TGA. From values 1.25 times greater than the normal cut-off, the measurements were ...

Research paper thumbnail of Diagnosis of celiac disease and applicability of ESPGHAN guidelines in Mediterranean countries: a real life prospective study

BMC gastroenterology, Jan 21, 2017

We assessed how the diagnosis of Celiac Disease (CD) is made and how the new ESPGHAN guidelines c... more We assessed how the diagnosis of Celiac Disease (CD) is made and how the new ESPGHAN guidelines can be applied in children from countries with different resources. A real life prospective study was performed in 14 centres of 13 different Mediterranean countries. Participants were asked to apply the usual diagnostic work-up for CD according to their diagnostic facilities. There were 1974 patients enrolled in the study, mean age 4 years, 10 months; 865 male, 1109 female. CD was confirmed in 511 (25.9%) and was unconfirmed in 1391 (70.5%) patients; 14 patients were diagnosed as having CD according to the new ESPGHAN guidelines, 43 patients were classified as having potential CD. In all participating countries the diagnosis of CD relied on histology of duodenal biopsy; in 5 countries, HLA, and in one country endomysial antibodies (EMA) were not available. Symptoms did not add a significant increase to the pre-test probability of serological tests. The positive predictive value of tissue...

Research paper thumbnail of Can partially hydrolyzed guar gum be an alternative to lactulose in treatment of childhood constipation?

The Turkish Journal of Gastroenterology, 2010

Bu çal›flmada, çocuklarda kab›zl›¤›n tedavisinde, lif kayna¤› olarak k›smi hidrolize guar gumun k... more Bu çal›flmada, çocuklarda kab›zl›¤›n tedavisinde, lif kayna¤› olarak k›smi hidrolize guar gumun kullan›m›n›n gü-venli¤i ve baflar›s›n›n, en s›k kullan›lan ozmotik laksatif olan laktuloz ile karfl›laflt›r›lmas› amaçlanm›flt›r. Yöntem: 61 hastada (31 k›smi hidrolize guar gum, 30 laktuloz) randomize prospektif kontrollü çal›flma yap›ld›. Hastalara 4 hafta boyunca laktuloz veya k›smi hidrolize guar gum verildi. Standardize edilmifl bir form verilerek d›flk›lama s›kl›¤›, d›flk› k›vam›, afl›r› gaz ç›karma ve kar›n a¤r›s›n›n kay›t edilmesi istendi. Her iki tedavi grubunda baflar›, güvenilirlik ve yan etki profili aile anketi ile elde olundu. Bulgular: Günlük bazal lif (meyve ve sebze) tüketiminde her 2 grup aras›nda fark saptanmad›. Kar›n a¤r›s› olan ve d›flk› tutan çocuklar›n yüzdesi her 2 tedavi grubunda belirgin olarak azald› (p<0.05). Laktuloz grubunda haf-tal›k d›flk›lama s›kl›¤›nda 4+0.7'den 6+1.06'ya, k›smi hidrolize guar gum alan hasta grubunda ise 4+0.7'den 5+1.7'ye art›fl oldu (p<0.05). Aile anketinde, laktuloz tedavi grubunda ebeveynler kötü tad, afl›r› gaz ç›karma ve fazla miktarda ilaç tüketim zorunlu¤undan yak›nd›lar. K›smi hidrolize guar gum tedavi grubunda ise ebeveynler çocuklar›n›n d›flk›lama s›kl›¤›ndan memnundular. Sonuç: D›flk› tutma ve kar›n a¤r›s› ile beraber olan kab›zl›¤›n giderilmesinde k›smi hidrolize guar gum tedavisi, laktuloz tedavisi kadar etkin bulunmufltur. Laktulozun afl›r› gaz hissi ve kötü tad gibi yan etkileri daha çok bulunmak-tad›r.

Research paper thumbnail of Açıklanamayan Transaminaz Yüksekliği ve Atipik Çölyak Hastalığı: Olgu Sunumu

Turkiye Cocuk Hastalıkları Dergisi, Jun 1, 2008

Research paper thumbnail of Ciddi kanamayla tanı alan alfa 1 antitripsin eksikliği olan bir olgu Olgu Sunumu

Turk Pediatri Arsivi-turkish Archives of Pediatrics, Dec 1, 2006

Alfa-1 antitripsin eksikliği (A1ATE) karaciğer hücresi endoplazmik retikulumunda (ER) sıra dışı A... more Alfa-1 antitripsin eksikliği (A1ATE) karaciğer hücresi endoplazmik retikulumunda (ER) sıra dışı A1AT birikimine neden olarak karaciğer hasarına yol açan otozomal çekinik (resesif) geçen kalıtsal bir hastalıktır. Alfa-1 antitripsin eksikliği bebeklik dönemi hemorajik hastalığına, kolestaza ya da kronik karaciğer hastalığına neden olabilir. Kolestaz ve bebeklik dönemi hemorajik hastalığı gelişen bir A1ATE (PiZZ fenotipi) olan olgu sunulmuştur. Bu olgu ile yenidoğanın geç hemorajik hastalığının nedeninin tanı almamış kolestaz olabileceğinin unutulmaması, 15 günden uzun süren sarılığı olan bebeklerin yenidoğan kolestaz nedenleri açısından mutlaka değerlendirilmeleri gerektiği, erken tanı almayan yenidoğan kolestazlarının kafa içi kanama gibi ciddi sonuçlar doğurabileceği ve erken tanı ve tedavinin önemi vurgulanmak istendi. Alpha-1-antitrypsin deficiency is an autosomal recessive disease in which liver disease results from retention of abnormal alpha-1-antitrypsin in the endoplasmic reticulum of hepatocytes. In childhood, alpha-1-antitrypsin deficiency may present as cholestasis, late hemorrhagic disease of infancy or chronic liver disease. Here, we present a case with alpha-1-antitrypsin deficiency who developed cholestasis and late hemorrahagic disease of infancy. We emphasize that the cause of late hemorrhagic disease of infancy may be unrecognized neonatal cholestasis and, that all infants who have jaundice for more than 15 days should be evaluated for neonatal cholestasis. Unrecognized neonatal cholestasis can result in severe conditions such as intracranial bleeding therefore, early diagnosis and treatment are essential.

Research paper thumbnail of Crohn's disease of the vulva in a 10-year-old girl

The Turkish journal of pediatrics

Crohn's disease may involve all parts of the gastrointestinal tract and may often involve oth... more Crohn's disease may involve all parts of the gastrointestinal tract and may often involve other organs as well. These non-intestinal affections are termed extraintestinal manifestations. Vulval involvement is an uncommon extraintestinal manifestation of Crohn's disease, and it is very rare in children. Patients with vulval CD typically present with erythema and edema of the labia majora, which progresses to extensive ulcer formation. Vulval Crohn's disease can appear before or after intestinal problems or it may occur simultaneously. We present a 10-year-old girl with intestinal Crohn's disease complicated with perianal skin tags and asymptomatic unilateral labial hypertrophy. The course of her lesion was independent of the intestinal disease and responded significantly to medical treatment including azathioprine and topical steroid. We emphasize that although vulval involvement in childhood is uncommon, Crohn's disease must be considered in the differential diag...

Research paper thumbnail of 976 Cyclosporine-Associated Thrombotic Microangiopathy and Thrombocytopenia-Associated Multiple Organ Failure: a Case Successfully Treated with Therapeutic Plasma Exchange

Archives of Disease in Childhood, 2012

The signal-to-noise ratio, i.e., ΔrStO 2 /S w, was 17.6, 14.5, and 12.5 for FORE-SIGHT, INVOS and... more The signal-to-noise ratio, i.e., ΔrStO 2 /S w, was 17.6, 14.5, and 12.5 for FORE-SIGHT, INVOS and NONIN, respectively. Conclusion The different absolute values and dynamic ranges will make comparison of data collected with different devices difficult.

Research paper thumbnail of Burden of celiac disease in the Mediterranean area

World Journal of Gastroenterology, 2011

To estimate the burden of undiagnosed celiac disease (CD) in the Mediterranean area in terms of m... more To estimate the burden of undiagnosed celiac disease (CD) in the Mediterranean area in terms of morbidity, mortality and health cost. For statistics regarding the population of each country in the Mediterranean area, we accessed authoritative international sources (World Bank, World Health Organization and United Nations). The prevalence of CD was obtained for most countries from published reports. An overall prevalence rate of 1% cases/total population was finally estimated to represent the frequency of the disease in the area, since none of the available confidence intervals of the reported rates significantly excluded this rate. The distribution of symptoms and complications was obtained from reliable reports in the same cohort. A standardized mortality rate of 1.8 was obtained from recent reports. Crude health cost was estimated for the years between symptoms and diagnosis for adults and children, and was standardized for purchasing power parity to account for the different economic profiles amongst Mediterranean countries.

Research paper thumbnail of Celiac disease in the Mediterranean area

BMC Gastroenterology, 2014

Background The World Gastroenterology Organization recommends developing national guidelines for ... more Background The World Gastroenterology Organization recommends developing national guidelines for the diagnosis of Celiac Disease (CD): hence a profile of the diagnosis of CD in each country is required. We aim to describe a cross-sectional picture of the clinical features and diagnostic facilities in 16 countries of the Mediterranean basin. Since a new ESPGHAN diagnostic protocol was recently published, our secondary aim is to estimate how many cases in the same area could be identified without a small intestinal biopsy. Methods By a stratified cross-sectional retrospective study design, we examined clinical, histological and laboratory data from 749 consecutive unselected CD children diagnosed by national referral centers. Results The vast majority of cases were diagnosed before the age of 10 (median: 5 years), affected by diarrhea, weight loss and food refusal, as expected. Only 59 cases (7.8%) did not suffer of major complaints. Tissue transglutaminase (tTG) assay was available, ...

Research paper thumbnail of Effects of cisapride on ventricular repolarization in children