Age-specific causes of upper gastrointestinal bleeding in children.

The etiology of upper gastrointestinal bleeding (UGIB) varies by age, from newborns to adolescents, with some of the causes overlapping between age groups. While particular causes such as vitamin K deficiency and cow's milk protein allergy are limited to specific age groups, occurring only in neonates and infants, others such as erosive esophagitis and gastritis may be identified at all ages. Furthermore, the incidence of UGIB is variable throughout the world and in different hospital settings. In North America and Europe, most UGIBs are non-variceal, associated with erosive esophagitis, gastritis, and gastric and duodenal ulcers. In recent years, the most common causes in some Middle Eastern and Far Eastern countries are becoming similar to those in Western countries. However, variceal bleeding still predominates in certain parts of the world, especially in South Asia. The most severe hemorrhage arises from variceal bleeding, peptic ulceration, and disseminated intravascular coagulation. Hematemesis is a credible indicator of a UGI source of bleeding in the majority of patients. Being familiar with the most likely UGIB causes in specific ages and geographic areas is especially important for adequate orientation in clinical settings, the use of proper diagnostic tests, and rapid initiation of the therapy. The fundamental approach to the management of UGIB includes an immediate assessment of severity, detecting possible causes, and providing hemodynamic stability, followed by early endoscopy. Unusual UGIB causes must always be considered when establishing a diagnosis in the pediatric population because some of them are unique to children. Endoscopic techniques are of significant diagnostic value, and combined with medicaments, may be used for the management of acute bleeding. Finally, surgical treatment is reserved for the most severe bleeding.

Assessment of Quality of Life, Anxiety and Depressive Symptoms in Serbian Children with Celiac Disease and their Parents.

OBJECTIVES: To evaluate the level of health-related quality of life (QoL) and presence of anxiety and depressive symptoms in Serbian children with celiac disease from the perspective of patients and their parents. METHODS: This cross-sectional study investigated the group of children and adolescents with celiac disease aged 5-18 y, and at least one parent of each patient with celiac disease. The patients and their parents were recruited at the Institute of Mother and Child Health of Serbia and the University Children's Hospital in Belgrade. The instruments used in this study were child-self and parent-proxy versions of the Pediatric Quality of Life Inventory (PedsQL), Screen for Child Anxiety Related Emotional Disorder (SCARED) and Short Mood and Feelings Questionnaire (MFQ). Additional information was collected from the medical records of each patient. RESULTS: According to the PedsQL questionnaire, the quality of life was similarly assessed by both parents and their children (p > 0.05), as well as the presence of depressive symptoms according to MFQ questionnaire. However, a statistically significant difference was observed in the total score of the SCARED questionnaire for children and parents [total score (p < 0.05), panic-somatic disturbance (p < 0.01) and social anxiety (p < 0.01)]. CONCLUSIONS: The patients and their parents in Serbia have similarly assessed the quality of life of children with celiac disease, but the differences in the scores of SCARED questionnaire indicate that it is necessary to include both children and parents in the assessment of QOL.

Predictive value of hepatic ultrasound, liver biopsy, and duodenal tube test in the diagnosis of extrahepatic biliary atresia in Serbian infants.

BACKGROUND/AIMS: Extrahepatic biliary atresia (EHBA) is the most important cause of neonatal cholestasis. The validity of different diagnostic methods in the diagnosis of EHBA in developed countries has been presented elsewhere, but data from developing countries with low national incomes are scarce. The aim of this study was to investigate the relative accuracy and roles of abdominal ultrasonography, duodenal tube test (DTT), and liver biopsy in the diagnosis of EHBA in Serbia. MATERIALS AND METHODS: The study included 156 infants with cholestasis admitted at the Mother and Child Health Care Institute. Data were collected according to the medical records observation technique. RESULTS: Extrahepatic biliary atresia was diagnosed in 72 of 156 infants with cholestasis. The frequency was insignificantly higher in females than in males (1.25:1). Most patients were diagnosed prior to 60 days of life (median 58, range 30-67). In a group of 156 infants with cholestasis, 109 had ultrasound, liver biopsy, duodenal tube test, and intraoperative cholangiography done. Liver biopsy confirmed surgical disease in 71/109 patients and denied it in 38/109 patients (sensitivity- Sn 98%, specificity- Sp 100%, diagnostic efficiency of test- DgEf 99.08%). Duodenal tube test had Sn 97%, Sp 72%, and DgEf 88.99%, and the ultrasound findings showed Sn 78%, Sp 81%, and DgEf 77.92%. Five-year survival rate after Kasai operation was 76%. CONCLUSION: A well-coordinated multidisciplinary approach is required in the assessment of suspected cases of biliary atresia. Histology examination of biopsy specimens is an integral part of the diagnostic algorithm and, therefore, plays a pivotal role in the diagnostic evaluation of this disease.

Primary Budd-Chiari syndrome in a 3-year-old boy with homozygous factor V Leiden G1691A mutation.

UNLABELLED: Budd-Chiari syndrome (BCS) is an uncommon disorder characterized by obstruction of hepatic venous outflow. The primary BCS is a rare disease with an incidence about 0.2 per million inhabitants per year. We present a 3-year-old boy with intrahepatic inferior vena cava clot. Because of decreased levels of protein C (38.7 %), F II (69.1 %), and activated protein C resistance (1.43), a mutational gene analysis was performed. The patient was found to be homozygous for the FV G1691A mutation. CONCLUSION: The primary BCS is a rare disease especially in childhood. Activated protein C resistance caused by the factor V Leiden mutation may be responsible for primary BCS. Prompt recognition of underlying prothrombotic disease and early initiation of their specific therapy might translate into rapid improvement of liver disease.

[Sample representativeness and incidence of liver biopsy complications caused by needles of bigger diameter (1.6 mm) and smaller diameter (1.2 mm) in children with cholestatic syndrome].

INTRODUCTION: Percutaneous liver biopsy and histomorphological analysis of liver tissue is an important diagnostic procedure in the investigation of neonates and infants with cholestatic syndrome. This study has been aimed at determining whether there is a difference in the incidence of complications after liver biopsy performed by Menghini technique using a needle of 1.6 mm as compared to 1.2 mm diameter and if there is a difference in the sample representativeness of liver tissue after liver biopsy with those two different needle diameters. MATERIAL AND METHODS: We retrospectively reviewed medical records of 156 neonates and infants with chronic cholestatic syndrome, hospitalized at Mother and Child Health Care Institute, Serbia. RESULTS: One hundred and fifty six children underwent liver biopsy. There was no difference in frequency of liver biopsy complications performed by Menghini technique using a larger diameter needle (1.6 mm) as compared to 1.2 mm diameter. The mortality after liver biopsy was 0% while the frequency of complications with a needle of 1.6 mm in diameter was 3.8% the percentage of serious complications being 0.6%. Among the samples of liver biopsy taken by a larger diameter needle (1.6 mm), 108/109 were representative samples (> 5 portal areas), and among those taken by a smaller diameter needle (1.2 mm), 34/47 were representative samples. Of 109 liver biopsy specimens obtained by Menghini technique using a needle of larger diameter (1.6 mm), 109/109 were representative samples (> 3 portal areas), and when a smaller diameter needle (1.2 mm) was used, 42/47 were representative samples. CONCLUSION: Our results indicate that the sample representativeness was significantly higher when a larger diameter needle was used for liver biopsy by Menghini technique; however, no difference in the incidence of complications was observed.

Profile of typical and atypical celiac disease in Serbian children.

We compared the clinical, biopsy and serology profile in typical vs atypical celiac disease. Mean TTG value for Marsh 3b/c in typical group was (140.53+/-88.77) and in atypical (140.66+/-73.53) (P=0.622). Seventy seven percent of patients had Marsh 3b/c in typical and 67.5% in atypical group (P=0.400).

Twelve-year-old girl with primary biliary cirrhosis.

Primary biliary cirrhosis (PBC) is a slowly progressive cholestatic liver disease of autoimmune etiology. The initial presentation of PBC is varies from asymptomatic, abnormal liver biochemical tests to overt cirrhosis. Unlike other autoimmune liver diseases, PBC has rarely been reported in childhood. We report a case of primary biliary cirrhosis in a 12-year-old girl. In addition to characteristic histology features, strongly positive antimitochondrial antibodies, increased liver enzyme levels, increased serum quantitative immunoglobulin M levels, and cholestasis were discovered. She had been treated with ursodeoxycholic acid. In the world literature, we found only few pediatric patients of primary biliary cirrhosis. Aetiology, pathogenesis, the long-term natural history, and prognosis remain obscure. Due to increased awareness of early-onset PBC, rather than typical older ones, further pediatric cases may be discovered.

Cardiomyopathy associated with celiac disease in childhood.

Celiac disease is predominantly a disease of the small intestine characterized by chronic malabsorption in genetically susceptible individuals who ingest grains containing gluten, such as wheat, barley, and rye. Although previously believed to be uncommon, celiac disease may be present in up to 1% of the adult and children population. Celiac disease is associated frequently with iron-deficiency anemia, dermatitis herpetiformis, selective IgA deficiency, thyroid disorders, diabetes mellitus, and various connective tissue disorders but is rarely associated with cardiomyopathy.

Alpha-1-antitrypsin deficiency in early childhood.

Alpha-1-antitrypsin deficiency (AATD), which predisposes liver disease in children, is often undiagnosed. Isoelectric focusing in 161 infants with liver dysfunction revealed 14.7% severe and 12.2% moderate AATD. Positive PAS-D and immunohistochemical staining was found in 60% of severe AATD, but in moderate AATD, only immunohistochemistry was positive in 100%. Bilirubinostasis, hepatomegaly, splenomegaly, cholestasis, hepatomegaly associated with cholestasis, acholia, high transaminases, and low birthweight were significantly more frequent in severe than in moderate AATD. Both AATDs showed significant portal inflammation, hepatic fibrosis, and viral infection. Early screening in children with liver dysfunction can contribute to the successful detection of AATD.

Pancreatitis and atypical Kawasaki disease.

We report on pediatric patient with clinical and laboratory evidence of pancreatitis at onset of atypical Kawasaki disease (KD). In KD pancreatic inflammation was described previously, but clinical pancreatitis was rarely reported and its true incidence is unknown.In febrile pediatric patients suspected to have KD, but not fulfilling classical diagnostic criteria, signs of pancreatic inflammation may help in making correct diagnosis. Further analysis of cases with atypical KD developing pancreatitis may reveal if signs of pancreatic inflammation can be used as supportive diagnostic finding.

[A histologic variant of autoimmune hepatitis with zonal necrosis]. / Histoloska varijanta autoimunskog hepatitisa sa zonskom nekrozom.

Autoimmune hepatitis type 1 in a 8-year old girl is described. The diagnosis was established using International Autoimmune hepatitis group scoring system. In addition to characteristic histologic features of autoimmune hepatitis (periportal hepatitis, piecemeal necrosis and rozettes) prominent centrilobular necrosis was discovered. As an isolate finding in autoimmune hepatitis, this type was described only in five cases. In our unique case centrilobular necrosis is a very important parallel finding previously not detected in autoimmune hepatitis. Some experimental studies suggest that cytocins present in inflammatory cell infiltrate in the liver play a pathologic role in autoimmune liver cell damage.