The article presents a mathematical model for pressure growth in the line supplying the engine KAMAZ with cryogenic fuel under specific insulation conditions. By using the heat balance equation the ...model in a simple linear form is obtained. This makes the model suitable for use in solving practical tasks related to the design of a liquefied natural gas engine fuel system. According to the above-mentioned method, the geometric parameters of the section of the main elements are determined under different external conditions and the boundary parameters of the gas fuel (maximum and minimum values of pressure, temperature, and flow). These data are necessary to determine the consumption, hydraulic and project features of the project. Fuel pipes are optimized (for hydraulic resistance, pressure and flow impulses, structural strength) to improve the accuracy of fuel dosing. The adequacy of the model was tested on a special modified test stand.
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.
INTRODUCTIONThe aim of this study was to present our patients with corrosive ingestion retrospectively, to analyze the validity of clinical signs as predictors of outcome, and to emphasize the ...necessity of esophagogastroduodenoscopy.
MATERIALS AND METHODSData were evaluated from the medical records of patients admitted at the Mother and Child Health Care Institute, Serbia over a 10-year period.
RESULTSA total of 176 children, mean age 36.2±18.1 months (range 9 months to 18 years), with corrosive ingestion were evaluated. The ingested substances were alkali in 96 cases (54.5%), acid in 41 (23.3%), and others in 39 cases (22.1%). In all, 116 patients (65.9%) were symptom free on admission. Positive clinical findings were observed in 60 (34.1%) patients. Upper endoscopy was performed in all children within the first 48 h. Ninety-five patients (54%) had normal endoscopic evaluation, 54 (30.6%) had mild lesions, and 27 (15.3%) had severe corrosive injuries. The validity of clinical findings in predicting the severity of esophageal and gastric injury was as followssensitivity – 74 and 75% and specificity – 73 and 68%, retrospectively. Eighteen patients (10.2%) developed esophageal stricture.
CONCLUSIONEndoscopy is a mandatory technique in children with gastroesophageal caustic injuries, and should be performed to prevent unnecessary hospitalization and to plan future treatment. This study emphasizes that clinical signs and symptoms are not predictors of esophageal and gastric injury and that the absence of any clinical findings does not rule out a severe esophageal or gastric injury.
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.
The involvement of the peripheral nervous system in children with celiac disease is rare.
A 15- year- old girl affected by celiac disease, who presented with an acute polyneuropathy after accidental ...reintroduction of gluten in her diet.
Neurological examination suggested asymmetric weakness of both legs distally. Anti-tissue transglutaminase antibodies were positive. Nerve conduction studies were consistent with a sensory-motor demyelinating peripheral neuropathy.
Symptoms improved spontaneously on a gluten-free diet.
Polyneuropathy may occur as a complication of celiac disease in childhood.
Abstract Background Current predictive severity scores for pediatric acute pancreatitis are either extrapolated from adult studies or difficult to use in practice. The aim of this study was to assess ...the value of the plasma D-dimer level as a marker of severity and outcome in pediatric AP. Methods 36 patients (aged 1–17 yrs) with AP were included in the study. Levels of D-dimer and other routine laboratory parameters for AP were determined on admission. The Pediatric Acute Pancreatitis Severity Score was used to assess disease severity. The development of systemic and local complications was also recorded. Results D-dimer level was significantly higher in a group of patients with complications, median 1189.5 (271–4800) vs 172.5 (105–1086) in a group of patients without complications ( p < 0.001). D-dimer showed high precision in the prediction of acute necrotic collection, with the optimal cut-off values of 442.5 μg/L, Sensitivity (Sn) 100%, Specificity (Sp) 62.1% and in the prediction of multiple organ failure with optimal cut-off value 1189 μg/L, Sn 100% and Sp 87.5%. According to the areas under the curve (AUCs) of each parameter, D-dimer seemed to have predictive power similar to PAPS score and higher than C-reactive protein and Lactate dehydrogenase level. Conclusion D-dimer level may be a simple clinical predictor of severity in pediatric acute pancreatitis.
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.
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.
Celotno besedilo
Dostopno za:
DOBA, IJS, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
PDF
