· We suggest offering long-term macrolides to children with noncystic fibrosis bronchiectasis with frequent exacerbations (conditional recommendation, moderate quality of evidence). · We do not recommend the routine use of mucolytic agents, inhaled corticosteroids, or nonsteroidal anti-inflammatory drugs to prevent exacerbation of bronchiectasis in children (inconclusive, very low quality of evidence). · We recommend the use of nebulized hypertonic saline to prevent exacerbations and improve the lung function of children with noncystic fibrosis bronchiectasis (weak recommendation, moderate quality of evidence). |
· Chronic liver diseases in children are heterogenous but converge in the common pathway of fibrosis. · Much of the literature on mechanisms of fibrogenesis focus on adults but pediatric physiology has documented differences. · Understanding of these distinctions are necessary to define, treat, and prevent fibrosis. · Current management of liver fibrosis relies heavily on liver biopsy. Multiple tools have shown high diagnostic performance in pediatric and adult populations. Large, multicenter studies are needed for validation. |
Postinfectious bronchiolitis obliterans (PIBO) is an irreversible obstructive lung disease characterized by subepithelial inflammation and fibrotic narrowing of the bronchioles after lower respiratory tract infection during childhood, especially early childhood. Although diagnosis of PIBO should be confirmed by histopathology, it is generally based on history and clinical findings. Irreversible airway obstruction is demonstrated by decreased forced expiratory volume in 1... |
Cystic fibrosis (CF) is a genetic disease with autosomal recessive inheritance and is common in Caucasian people. The prevalence of this disease is between 1/2,000 and 1/3,500 live births, and the incidence varies between populations. Although the CF transmembrane conductance regulator gene is expressed in the kidneys, renal involvement is rare. With advances in the treatment of CF, life expectancy... |
Because nonalcoholic steatohepatitis can progress towards cirrhosis even in children, early detection of hepatic fibrosis and accurate diagnosis of nonalcoholic fatty liver disease (NAFLD) are important. Although liver biopsy is regarded as the gold standard of diagnosis, its clinical application is somewhat limited in children due to its invasiveness. Noninvasive diagnostic methods, including imaging studies, biomarkers of inflammation, oxidative stress,... |
Childhood obesity is associated with nonalcoholic fatty liver disease (NAFLD), and it has become one of the most common causes of childhood chronic liver diseases which significant as a cause of liver related mortality and morbidity in children in the United States. The development of simpler and easier clinical indices for medical practice is needed to identify advanced hepatic fibrosis... |
Renal fibrosis, characterized by tubulointerstitial fibrosis and glomerulosclerosis, is the final manifestation of chronic kidney disease. Renal fibrosis is characterized by an excessive accumulation and deposition of extracellular matrix components. This pathologic result usually originates from both underlying complicated cellular activities such as epithelial-to-mesenchymal transition, fibroblast activation, monocyte/macrophage infiltration, and cellular apoptosis and the activation of signaling molecules such as... |
Meconium ileus (MI) is the earliest clinical manifestation of cystic fibrosis (CF) in infants. It arises from the intraluminal accumulation of highly viscid, protein-rich meconium, typically present in the terminal ileum as a neonatal intestinal obstruction. Therefore, the clinical symptoms include abdominal distension, bilious vomiting and delayed passage of meconium. CF is caused by mutations in the transmembrane conductance regulator... |
Purpose : We hypothesized dexrazoxane(DXR) and pentoxifylline(PTX) may prevent myocardial damage in adriamycin(ADR)-induced cardiomyopathic rat model. We also investigated their effects on the myocardial apoptosis and fibrosis in ADR induced cardiomyopathy. Methods : The six-week old female Spregue-Dawley rats were divided into control group(CNT, n= 4), ADR group(n=6), ADR+DXR group(DXR, n=5), ADR+PTX group(PTX, n=6), ADR+DXR+PTX group(DXPT, n=5). ADR(5 mg/week, twice) was... |
Congenital hepatic fibrosis is a relatively rare disease, characterized by bile ductular proliferation and prominent fibrosis in the portal area of liver resulting in portal hypertension. It is frequently associated with other abnormalities such as polycystic kidney, Caroli syndrome, cystic dysplasia of pancreas, intestinal lymphangiectasia, pulmonary emphysema, hemangioma, and cleft palate. We report here a case of congenital hepatic fibrosis... |
Purpose : Extrahepatic biliary atresia is the most common indication for liver transplantation in children, but the etiology of this disorders remains unknown. It would be very signficant to identify genes that are specifically expressed in pathologic liver tissue of biliary atresia and analyze the pattern of expression in those genes. Methods : We made dot blot panels consisting of 1,730 different EST(expressed sequence... |
Purpose : This study was aimed to assess the clinical manifestations and courses of autosomal recessive polycystic kidney disease(ARPKD). Methods : The medical records of 10 children diagnosed as infantile or juvenile ARPKD at Seoul National University Children's Hospital between January, 1984 and December, 1996, were reviewed, retrospectively. Results : The average age at diagnosis was 3 8/12 years(4months-7 3/12 years)... |
Chronic iron overload is associated with life-threatening complications, such as cardiomyopathy, liver cirrhosis, diabetes, hypothyroidism, hypoparathyroidism and hypogonadism. We experienced a case of secondary hemosiderosis with hepatic fibrosis in a 8-year-old boy who had been transfused 56 pints of packed red blood cells for a supportive therapy of acute myelogenous leukemia for a 27-month period. Intramuscular injection of Desferroxamine(40mg/kg/day) was... |
Idiopathic myelofibrosis (IMF), which is characterized by marrow fibrosis, leukoerythroblastic anemia, teardrop poikilocytosis and splenomegaly due to extramedullary hematopoiesis, has known to have no form of therapy. On the ground of the possibility of reversing collagen deposition in IMF using 1, 25-dihydroxycholecalciferol [1, 25(OH)2D3], we report here our observations of 5 patients (M:F = 1:4) with IMF before and after treatment... |
Agnogenic myeloid metaplasia (AMM) is a myeloproliferative disorder characterized by leukoerythroblastosis, tear-drop erythrocytes, extramedullary hematopoiesis with hepatosplenomegaly, and varying degrees of myelofibrosis. The mean age at presentation is about 60 years, and pediatric cases are rare. We experienced a case of AMM in 9 months old female who was presented with pallor, huge splenomegaly and intermittent fever. Peripheral blood showed leukoerythroblastosis... |
The congenital hepatic fibrosis is a rare condition defined pathologically by the presence within the line of bands of fibrous tissue which after contain linear or circular spaces lined by bile duct cells, It is commonly associated with intrahepatic portal hypertension but hepatocellular function is almost always preserved. The prinicipal clinical features of this disease are abdominal distension, firm hepatomegaly,... |
Idiopathic pulmonary fibrosis is a heterogeneous group of disorder which predominantly affects the lung parenchyma and spares the airway. We report two cases of idiopathic pulmonary fibrosis in a 2 year 11 month-old female and a 1 year 1 month-old male patients who were treated with steroid. These patients presented with dyspnea and showed fine crepitant rales in both lung... |
Myelofibrrosis is characterized by anemia, leukoerythroblastosis, extramedullary hematopoiesis, hepatosplenomegaly, osteosclerosis and fibrosis of bone marrow. Idiopathc myelofibrosis is usually a disease of the adult and is rare in the pediatric age group. Juvenile xanthogranuloma is characterized by multiple papules on forehead and scalp. Lipid laden histiocytes and Touton giant cell are found in skin biopsy. We experienced a case of myelofibrosis with juvenile xanthogranuloma.... |
Interstitial pulmonary fibrosis is a rare, diffuse lug disease which has a tendency to destroy the lung architectures by consequent healing with progressively severe fibrosis. We report with a brief review of literature, one case of a 7-year old female with the typical pictures of interstitial pulmonary fibrosis, histologically on open lung biopsy, and clinically presenting with chronic respiratory difficulty but without definite symptoms... |
Agnogenic myeloid metaplasia characterized by marrow fibrosis, leukoerythroblastic anemia, extramedullary hematopoiesis with varying degree of hepatosplenomgaly, is very rare disorder in children. We experienced a case of agnogenic myeloid metaplasia 34 months old male patient who was presented with pallor, generalized petechiae, marked hepatosplenomegaly and intermittent fever. Peripheral blood smear showed leukoerythroblastosis, poikilocytosis and tear drop cells. Bone marrow aspiration and biopsy showed fibrosis with no... |
Congenital hepatic fibrosis is a relatively rare disease of liver in children and young adults, manifesting a heredofamilial tendency, that is characterized by hepatosplenomegaly, protal hypertension and frequent renal involvement. A generalized portal and interlobular fibrosis of liver are characteristic finding on microscopy, with relative preservation of hepatocyte and liver function. Recently, we have experienced a case of congenital hepatic fibrosis in a... |
Myelofibrosis is characterized by fibrosis of bone marrow, leukoerythrobla stotic anemia and extramedullary hematopoesis with varying degree of hepatosplenomegaly. Idiopathic myelofibrosis is primarily a disease of the adult and is rare in the pediatric age group. We experienced a case of idiopathic myelofibrosis in a 2 year 5 month old male who showed severe anemia with abnormal forms of red blood cell (teardrops, ovalocytes),... |
The so called “Myelofibrosis” within the category of myeloproliferative disorders consists of various degrees of leukoerythroblatosis, bone marrow fibrosis, and extramedullary hema- topoiesis with hepatosplenomegaly. Primary myelofibrosis occurs predominantly in adults and few in childhood. We experie- nced one case of myelofibrosis, at 17 month old male patient. He was admitted due to intermittent fever, pallor and abdominal distension. On admission, hepatosplenomegaly, anemia and generalized petechia... |
Congenital hepatic fibrosis is a relatively rare liver disease in children and young adults,that is characterized by stony hard hepatomegaly and portal hypertension with relative preservation of liver function and underlying architecture. Since this Condition was first delineated by Kerr et al in 1961, approximately over 150 cases have been reported in the literature. However, congenital hepatic fibrosis was not... |
Caroli*s disease, a rare syndrome characterized by congenital, segmental saccular dilatation of intrahepatic bile ducts, is first described by Caroli & associaties in 1958. It is though by many to be part of spectrum of congenital hepatic fibrosis. The disease usually presents with bile stasis and stone formation with further complication arising from recurrent cholangitis and liver abscess. The preoperative diagnosis... |