Endoscopy 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.
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.
Several reports have documented various forms of glomerular diseases in adults with myelodysplastic syndromes (MDS), but similar reports in children are lacking. We describe two children with MDS-associated steroid-responsive nephrotic syndrome (NS). Patient 1, who had MDS with myelofibrosis, presented with hepatosplenomegaly, pancytopenia, chronic hepatitis, moderate proteinuria, hypocomplementemia and elevated ANA titer. During initial prednisone treatment proteinuria markedly diminished and partial but transient hematological improvement occurred. Relapse subsequently occurred that manifested by overt NS and pancytopenia. High doses of prednisolone led to remission of the renal disease, but hematological remission did not occur. Persisting pancytopenia and repeated infections terminated in sepsis, 2 years after the onset of the MDS. Patient 2, who had refractory anemia with clonal monosomy 19, presented with bowel disease, hepatosplenomegaly, anemia and non-organ-specific autoantibodies. Prednisone led to both clinical and hematological remission. The hematologic disease relapsed 12 months later, when nephrotic-range proteinuria, hematuria and mild azotemia were also found. Corticosteroid treatment led to long-lasting renal and hematologic remission, maintained by a small dosage of prednisone. In both patients, renal biopsy findings were consistent with those seen in idiopathic NS. A Medline search disclosed 16 cases of glomerulopathy in the course of MDS in adult patients. Clinical features included NS, usually accompanied by renal insufficiency with acute, chronic, or rapidly progressive glomerulonephritis. On biopsy, membranous nephropathy, crescentic or mesangial proliferative glomerulonephritis, and AL amyloidosis were found. We conclude: (1) that glomerular disease may be present and should be searched for in patients with MDS and (2) that MDS can be added to the list of rare conditions associated with corticosteroid-responsive NS in children.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.