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.
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.
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%.
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.
Introduction/Objective. The objective of the paper is to assess the applicability of serum anti-tissue transglutaminase (tTG) antibodies IgA and IgG concentration and HLA-DQ2/DQ8 typing as a ...non-invasive alternative to duodenal biopsy in diagnosing celiac disease (CD) in pediatric population. Methods. A prospective cohort study included a total of 179 pediatric patients aged 1?18 years. Determination of tTG IgA and tTG IgG antibodies and human leukocyte antigen (HLA) DQ2/DQ8 typing was performed for all patients. Histology of duodenal biopsies was interpreted by the modified Marsh scoring system. Results. The diagnosis of CD was confirmed in 101 (56%) patients of the studied population. In cases of CD, HLA-DQ2/DQ8 was positive in 100 patients (99%). The tTG IgA antibodies in concentration higher than 100 U/ml were detected in 77 (76.2%) of the CD patients and in significantly smaller number for tTG IgG 29 (28.7%) (p < 0.001). Statistically highly significant association of duodenal lesions Marsh grade 3 with concentration of tTG IgA 10-fold higher than the upper level of normal (ULN) was established (p < 0.001) Conclusion. Concentration of tTG IgA 10-fold higher than ULN is significantly positively correlated with Marsh grade 3 histopathology findings. Specific antibodies determination in combination with HLA-DQ2/ DQ8 typing proves to be sufficient for a diagnosis of CD, supporting the fact that duodenal biopsy may be avoided in a significant majority of patients ? 75%.
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.
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.
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.
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.
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).
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.
We retrospectively reviewed medical records of 156 neonates and infants with chronic cholestatic syndrome, hospitalized at Mother and Child Health Care Institute, Serbia.
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.
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.
In this paper, we propose a novel metamaterial-based microfluidic sensor that permits the monitoring of properties of the fluid flowing in the microfluidic reservoir embedded between the composite ...left–right handed (CLRH) microstrip line and the ground plane. The sensor’s working principle is based on the phase shift measurement of the two signals, the referent one that is guided through conventional microstrip line and measurement signal guided through the CLRH line. At the operating frequency of 1.275 GHz, the CLRH line supports electromagnetic waves with group and phase velocities that are antiparallel, and therefore the phase “advance” occurs in the case of CLRH line, while phase delay arises in the right-handed (RH) frequency band. The change of the fluid’s properties that flow in the microfluidic reservoir causes the change of effective permittivity of the microstrip substrate, and subsequently the phase velocity changes, as well as the phase shift. This effect was used in the design of the microfluidic sensor for the measurement of characteristics of the fluid that flows in the microfluidic reservoir placed under the CLRH line. The complete measurement system was developed including the Wilkinson power divider that splits the signal between conventional RH and CLRH section, transmission lines with the microfluidic reservoirs, and a detection circuit for phase shift measurement. Measurement results for different fluids confirm that the proposed sensor is characterized by relatively high sensitivity and good linearity (R2 = 0.94). In this study, the practical application of the proposed sensor is demonstrated for the biomass estimation inside the microfluidic bioreactors, which are used for the cultivation of MRC-5 fibroblasts.
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