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Background: Dysphagia impacts infants' health and well-being and may result in aspiration pneumonia., Objective: This study aimed to evaluate feeding and swallowing functions in infants with dysphagia to determine the possible causes of dysphagia and their relationship with the medical diagnoses. Clinical and videofluoroscopic findings were compared to determine the diagnostic accuracy of clinical evaluation and identify clinical predictors of laryngeal penetration and aspiration., Methods: This study was conducted on 60 infants in the age range 2-19 months (median seven months) with suspected aspiration and/or feeding problems. All cases were subjected to both clinical and videofluoroscopic evaluation of swallowing., Results: The two most frequently observed videofluoroscopic findings were aspiration and suck-swallow-breathing incoordination. Infants with structural laryngeal abnormalities were significantly more likely to experience aspiration. A significant association was found between the presence of aspiration and the presence of both recurrent chest infection and gurgly voice combined, which increased the risk of aspiration by 3.57 times. However, the presence of gagging alone and gagging combined with a recurrent chest infection significantly reduced the risk of aspiration. The clinical assessment accuracy presented 56.70%, with 55.60% sensitivity, 58.30% specificity, and a positive predictive value higher than the negative., Conclusion: The study highlights the importance of complementing clinical evaluation, in infants with dysphagia, with an objective evaluation of swallowing such as videofluoroscopy due to the high proportion of false positives noticed in clinical evaluation and the high prevalence of silent aspiration in infants., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Purpose: This study aimed to evaluate the role of diffusion tensor imaging of microstructural changes in gray and white matter in Crigler-Najjar syndrome type I., Patient and Methods: A prospective study was conducted on 10 patients with Crigler-Najjar syndrome type I and 10 age- and sex-matched children who underwent diffusion tensor imaging of the brain. Mean diffusivity (MD) and fractional anisotropy (FA) of gray and white matter were measured., Results: There was a significantly higher MD of the gray matter regions including the globus pallidus, thalamus, caudate head, substantia nigra, and dentate nucleus in patients versus controls (P = 0.007, 0.001, 0.014, 0.003, and 0.002), respectively. The areas under the curve (AUC) of MD of the globus pallidus and thalamus used to differentiate patients from controls were 0.93 and 0.925, respectively. There was a significant difference in MD of the frontal white matter and posterior limb of the internal capsule in patients versus controls (P = 0.001 and 0.02), respectively. The AUCs of MD of these regions used to differentiate patients from controls were 0.82 and 0.8. There was a significant difference in FA of the frontal white matter and posterior limb of the internal capsule in patients versus controls (P = 0.006 and 0.006), respectively. The AUCs of FA of these regions were 0.83 and 0.85, respectively. The MD of the globus pallidus correlated with serum bilirubin (r = 0.87 and P = 0.001)., Conclusion: Diffusion tensor imaging can detect microstructural changes of deep gray matter and some regions of white matter in Crigler-Najjar syndrome type I.

Nonalcoholic fatty liver disease (NAFLD) is quickly becoming one of the most prominent causes of liver disease worldwide. The increasing incidence of NAFLD is tied to the obesity epidemic and the subsequent metabolic derangements brought along with it. Current efforts to elucidate the mechanism and causes of the disease have answered some questions, but much remains unknown about NAFLD. The aim of this article is to discuss the current knowledge regarding the pathogenesis of the disease, as well as the current and future diagnostic, preventative, and therapeutic options available to clinicians for the management of NAFLD.

Background: Neonatal cholestasis syndrome is considered as a major challenge in pediatric practice. This study was undertaken to investigate the value of morphometric assessment of hepatic fibrosis in early diagnosis of biliary atresia., Methods: We studied liver biopsy specimens from 53 patients with neonatal cholestasis. The patients were assigned to two groups: group 1 (25 patients with biliary atresia) and group 2 (28 patients with non-obstructive cholestasis). Morphometric assessment of fibrosis was performed for all biopsies; in addition, another twelve histological parameters were estimated and scored on a scale of 0 to 4. Biopsies of infants aged 60 days or younger were characterized and analyzed separately., Results: Morphometric value of fibrosis was significantly higher in group 1 than in group 2 (16.8 ± 8.4% vs. 5.9 ± 2.3%, respectively; P<0.001). By multiple regression analysis, bile ductular plugs, morphometric assessment of fibrosis, rosetting, portal tract inflammation and pattern of cholestasis were found to be significant in discriminating the two groups. In infants aged 60 days or younger, a cutoff value for morphometric assessment of fibrosis of 7.5% was the discriminating point between the two groups with a sensitivity of 80% and a specificity of 84%., Conclusion: Morphometric assessment of hepatic fibrosis could enhance the value of liver biopsy in early diagnosis of biliary atresia.

Tumor necrosis factor (TNF) alpha-308 and interleukin (IL)-10(-1082) have potent inflammatory responses in the process of airway inflammation in asthma. The purpose of this study was to check for association of polymorphisms related to cytokine genes with susceptibility and severity of bronchial asthma in Egyptian children. Blood samples of 69 asthmatic children receiving treatment and follow-up at the Allergy and Respiratory Medicine Unit, Mansoura University Children Hospital, Mansoura, Egypt, were subjected to DNA extraction and amplification using polymerase chain reaction with sequence-specific primers for detection of single nucleotide polymorphisms in the promoter regions of cytokine genes TNF-alpha(-308(G-->A)), IL-10(-1082(G-->A)). Compared with normal controls, Egyptian asthmatic children showed a significant higher frequency of IL-10(-1082) G/G homozygosity genotype (p < 0.001; odds ratio [OR] = 7) with lower frequency of G/A heterozygosity genotype among cases. This finding also was detected in cases with persistent asthma and eczema. These cases showed significant lower frequency of TNF-alpha-308 G/A heterozygosity (p < 0.05; OR = 0.44). Also, male cases, cases with positive family history, and those patients with persistent types of asthma showed a higher frequency of TNF-alpha-308 G/G homozygosity. IL-10(-1082(G-->A)) G/G and TNF-alpha-308(G-->A) G/G may be a contributing factor in susceptibility as well as severity of asthma among Egyptian children. Separate studies should be specified relating these cytokine genotypes to response to various modalities in asthma therapy. This study reports that IL-10(-1082(G-->A)) G/G and TNF-alpha-308(G-->A) G/G genotypes may be contributing factors in susceptibility as well as in severity of asthma among Egyptian children. Separate studies may be specified relating these cytokine genotypes to response to various modalities in asthma therapy.

Background: Plasma endothelin-1 (ET-1) is a potent vasoconstrictor peptide involved in the pathogenesis of several disorders. Endothelin-1 concentrations are increased in adult patients with cirrhosis. However, little is known about ET-1 concentrations in children with cirrhosis., Methods: Radioimmune assay was used to measure plasma ET-1 concentrations in 19 children with cirrhosis (8 patients with ascites, and 11 without ascites), and 11 age- and sex-matched healthy children. The plasma ET-1 concentrations were correlated with the mean blood pressure, creatinine clearance, and severity of portal hypertension, as measured by portal flow volume and portal flow velocity., Results: Patients with cirrhosis and ascites had increased plasma ET-1 concentrations compared with patients who did not have ascites (6.8 pg/mL +/- 0.62 pg/mL vs. 4.6 pg/mL +/- 0.35 pg/mL; mean +/- SEM; < 0.01) and controls (3.6 pg/mL +/- 0.27 pg/mL; mean +/- SEM; < 0.0005). Plasma ET-1 concentrations were higher in patients with cirrhosis who did not have ascites compared with controls ( < 0.005). No significant differences were observed between concentrations of the patients with cholestasis and those without cholestasis (5.4 pg/mL +/- 0.52 pg/mL vs. 5.2 +/- 0.32 pg/mL; mean +/- SEM; = 0.1). Plasma ET-1 concentrations correlated positively with the mean blood pressure ( = 0.58; < 0.05) and negatively with renal function, as measured by creatinine clearance ( = -0.7; <0.005). However, no correlation was detected between ET-1 concentrations and portal flow volume ( = -0.02; = 0.4) or portal flow velocity ( = -0.16; = 0.4)., Conclusions: Plasma ET-1 concentrations are increased in children with cirrhosis, with or without ascites, compared with controls. Patients with cirrhosis and ascites have increased ET-1 concentrations compared with those without ascites. The degree of increase does not relate to the severity of portal hypertension. This increase tends to maintain systemic blood pressure but is associated with a decrease in renal function.

Intercellular adhesion molecule-I (ICAM-I) is a member of the immunoglobulin supergene family. It is expressed on the surface membrane of cells of multiple lineages at sites of inflammation. A soluble form of ICAM (sICAM-I) comprising the five extracellular Ig-like domains of ICAM-I has been detected in human serum and has been found to be increased in a variety of acute and chronic liver disorders. However, little is known about sICAM-I levels in children with chronic liver disease. Therefore, we measured sICAM-I in 23 children with chronic hepatitis, 14 children with cirrhosis, and 10 age- and sex-matched normal children by commercially available ELISA. We also correlated the sICAM-I levels with the histological activity index (HAI) score as determined from liver biopsies. Patients with chronic hepatitis had higher sICAM-I levels compared to controls (723 +/- 272 ng/ml vs 282 +/- 43 ng/ml, mean +/- SD; P < 0.05). sICAM-I levels were also higher in patients with cirrhosis compared to controls (630 +/- 218 ng, mean +/- SD; P < 0.05). However, there was no significant difference between sICAM levels in patients with chronic hepatitis and cirrhosis. A significant correlation was found between the ICAM-I level and the histological activity index score in patients with chronic hepatitis (r = 0.58; P < 0.001) and in patients with cirrhosis (r = 0.7; P < 0.001). We also found that by using the cutoff level of 346 ng/ml, sICAM-I can be used as a screening test with high specificity (100%) and sensitivity (94%) to differentiate children with chronic liver disease from normal children. We conclude that sICAM is increased in children with chronic hepatitis and cirrhosis compared to controls. The degree of elevation correlates with the HAI score. sICAM may be used as a marker of the disease activity and may provide diagnostic and prognostic information in children with chronic liver disease. However, this needs to be further studied.

Background: Infantile cholestasis continues to represent a diagnostic challenge. It is very important to diagnose surgically correctable disorders, such as biliary atresia, in a timely manner to prevent progressive damage to the liver. It has been recently suggested that the triangular cord (TC) sign is a simple and useful tool in the diagnosis of biliary atresia., Methods: We prospectively studied 65 infants presenting with conjugated hyperbilirubinemia (age range: 32-161 days). All patients underwent ultrasonographic examination with a 7.0-MHz transducer (Acuson, Mountain View, CA). The TC was defined as a triangular, or tubular, echogenic density seen immediately cranial to the portal vein bifurcation., Results: The TC sign was identified in 25 infants, and all of them had histologic features suggestive of biliary atresia; the diagnosis was confirmed at surgery by gross morphology of hepatobiliary system, and liver biopsy, with or without intraoperative cholangiogram. Among the 40 patients who did not have the TC sign, 6 had paucity of the intrahepatic bile ducts. Three had alph-1-antitrypsin deficiency, and 31 had neonatal hepatitis. None of the 40 patients who did not have the TC sign developed acholic stools. Seven patients with biliary atresia were followed by ultrasonographic examination for 6 months after the Kasai procedure. The TC sign disappeared in all patients after the surgery; however, the TC sign reappeared in 3 patients who developed progressive cholestasis after the procedure., Conclusion: The TC sign is a simple, timesaving, and reliable diagnostic tool in the evaluation of infants with infantile cholestasis. The TC sign may also prove to be helpful in following patients after hepatoportoenterostomy. We suggest a new diagnostic strategy for patients suspected to have biliary atresia. When the TC sign is visualized, the patient should undergo intraoperative cholangiogram to confirm the diagnosis of biliary atresia, reserving percutaneous liver biopsy for those patients in whom the TC sign could not be detected.

Bupivacaine wound infiltration together with efficient esophagogastric decompression and immediate elemental feeding in 43 consecutive, well-instructed patients who had undergone cholecystectomy reduced their postoperative analgesia requirements to an average of 17 (+/- 46, SD) mg of meperidine during the initial 24 hours. No analgesics were requested by 35/43 patients (81%) and 40/43 were discharged within 24 hours of surgery. All 86 conventionally treated control patients required narcotics, averaging 4.4 (+/- 0.3, SD) doses and amounting to 290 (+/- 25, SD) mg of meperidine or its equivalent during their first postoperative day and were discharged in 6.5 (+/- 0.7, SD) days. None were discharged in less than 3 days. Home interview by a visiting nurse was carried out for a block of 19 treated patients and their families. Six patients were interviewed prospectively within 33 to 64 hours of surgery, with a repeat visit 1 to 2 months later. Retrospectively studied patients were only seen 1 to 4 months after surgery. These interviews confirmed the patients' and their families' satisfaction with their bodily functions, their limited discomfort, and their independence from the hospital. Wound infiltration with a long-acting local anesthetic, efficient removal of swallowed air, and immediate, full enteral nutrition contribute to reduced postoperative pain and narcotic use in well-instructed patients, thus leading to safe and satisfactory early discharge.