The aim of these studies was to characterize the synthesis and secretion of lipoproteins and apolipoprotein B (apo B) and apo A-I by a newborn swine intestinal epithelial cell line (IPEC-1). Differentiated cells exhibited enterocytic features, including microvilli. [3H] oleic acid was taken up and incorporated into cellular lipids and secreted into the basolateral medium in lipoproteins. Total apo B and apo A-I secreted increased with oleic acid incubation. However, cellular apo B and apo A-I content did not change. Whereas undifferentiated cells synthesized and secreted only apo B-100, both apo B-100 and apo B-48 were produced by differentiated cells. The ratio of radiolabeled apo B-48 to apo B-100 in both basolateral medium and cell homogenate increased with oleic acid treatment after 24-h steady-state labeling. However, apo B mRNA editing was unchanged, indicating posttranslational regulation of this ratio. Pulse-chase radiolabeling demonstrated no major changes in cellular or basolateral medium apolipoprotein labeling kinetics with oleic acid or dexamethasone incubation. The dissociation of apo B and apo A-I mass secretion from the secretion of radiolabeled apo B and apo A-I in response to oleic acid absorption suggests the presence of an intracellular pool of apolipoprotein with a slow turnover that is mobilized for secretion in response to fatty acid uptake.
Liver transplantation is an effective and widely accepted therapy for children with end-stage liver disease. Major indications include primary liver disease, resulting in hepatic insufficiency, or severe morbidity secondary to chronic non-progressive liver disease and metabolic diseases of the liver. Liver replacement should not be considered if there is an acceptable alternative therapy. Relative contraindications to transplantation include irreversible impairment of other organ systems, major systemic infection and diseases expected to recur after transplantation. Early referral for pre-transplant evaluation is important to confirm the proper diagnosis and determine priority for transplantation, to identify potential contraindications, and to assist in supportive care of the patient with chronic liver disease. Innovations such as reduced-sized liver grafts and most recently, living related liver transplantation have increased the donor supply of organs for small infants and significantly reduced pre-transplant mortality. In addition, living donor transplantation allows infants to benefit from transplantation before developing severe complications of end-stage liver disease and reduces the incidence of primary graft non-function and rejection. Immunosuppression following transplantation is maintained with methylprednisolone, azathioprine and cyclosporine. Acute rejection is treated with short bursts of high-dose corticosteroids and when necessary OKT3. With this approach, 90% of the episodes of rejection can be successfully controlled. Survival after transplantation has steadily improved and survival rates of 70%-90% are routine. Following transplantation, children experience rapid nutritional restoration, increased muscle strength, marked progress in gross motor development and improved general health.
We report a rare case of 9-month-old girl who presented with a choking episode and was found to have an incidental finding of a lung cyst and iron deficiency anemia leading to the diagnosis of pleuropulmonary blastoma (PPB) and a jejunal hamartoma. Our patient is the eighth that has been reported with the association of PPB with jejunal hamartoma and the first one in the radiological literature. PPB is the pulmonary analog of other dysontogenetic neoplasms in childhood. A biological sequence has been described with the three types of PPB to be interrelated as part of pathologic progression. PPB can be associated with other cysts and/or neoplasms in different organs. PPB is part of a hereditary neoplasia predisposition syndrome in up to 40% of cases. Mutations in DICER gene have been described with PPB. Hence, a pediatric patient diagnosed with PPB should be screened for associated conditions during childhood and adolescence including intestinal polyps. Obtaining family history for other neoplasms or cysts is important information that should raise the possibility of PPB in pediatric patients with cystic lung lesions. The presence of this syndrome should alert the clinician to screen and follow up patients and their relatives.
Pancreatic pseudoaneurysm is a rare but potentially fatal complication in pancreatitis; however, the incidence in children is not well established. Surgery has been the mainstay of treatment; however, we report a case of PSA associated with pancreatitis in a 6-year-old boy, which was successfully managed endovascularly.
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