RESUMO
Due to the possibility of underlying hepatobiliaryor bone diseases, the diagnostic work up of a child with elevated alkaline phosphatase (AP) levels can be quite costly. In a significant proportion of these patients, elevated AP is benign, requiring no intervention: hence, known as transient hyperphosphatasemia (THP) of infants and children. A 27-month old previously healthy Caucasian female was found to have isolated elevation of AP four weeks after the initial symptoms of acute gastroenteritis. One month later, when seen in hepatobiliary clinic, signs and symptoms of gastrointestinal, hepatobiliary, or bone disease were absent and physical examination was normal. The diagnosis of THP was made, and, as anticipated, AP levels normalized after four months. Using this case as an example, we suggest an algorithm that can be utilized as a guide in a primary care setting to arrive at the diagnosis of THP and avoid further tests or referrals.
RESUMO
A 6-day-old, known to have transposition of the great vessels, received care in the neonatal intensive care unit at a tertiary care center. A computed tomography scan was performed for abdominal distention and upper gastrointestinal bleeding, which revealed a "mass lesion" in the left liver lobe. Analysis of antecedent events and the clinical and laboratory course uncovered an iatrogenic etiology and pathogenesis of the lesion. As the nature of the lesion was clarified, no specific therapy was required. This case is presented to show a serious yet preventable complication of a commonly performed procedure.
RESUMO
PURPOSE: Biliary atresia (BA) is the most common indication of liver transplantation in children. Pathogenesis of hepatic fibrosis, which is a prominent feature of BA, remains obscure. The purpose of this work was to determine the cellular sources of transforming growth factor beta-1 (TGFß1) and establish the relationship between TGFß1-producing cells and extracellular matrix producing myofibroblasts (MFBs) in advanced BA. METHODS: Trichrome staining and immunohistochemistry were carried out to determine the expression pattern of collagen and TGFß1 protein in explant liver specimens from patients with BA. The intensities of portal and lobular TGFß1 expressions were compared. Immunofluorescence technique was carried out to determine the relationship between α-smooth muscle actin (α-SMA)-positive-MFB and TGFß1-positve cells. RESULTS: Lobular TGFß1 protein expression was significantly higher than portal (89 ± 6 versus 10 ± 1 arbitrary units, P ≤ 0.05), whereas no difference was noted in livers used as control (10 ± 1.6 versus 19 ± 5 arbitrary units, P = 0.11). TGFß1 expression was more in the center of nodules versus MFB in surrounding fibrous septa. Contrary to TGFß1 expression, α1-SMA was mostly expressed in the portal structures and the adjacent fibrous septa enacting lobulation of the parenchyma. The results obtained by coimmunofluorescence staining showed no colocalization of α-SMA and TGFß1. CONCLUSIONS: TGFß1 protein expression is mostly localized to hepatocytes in advanced BA. These findings suggest a paracrine mechanisms of TGFß1-driven fibrogenesis in advanced BA.
RESUMO
Amino acid transport System A (SysA) plays an important role in mediating the transplacental transfer of neutral amino acids from mother to fetus. Given that prior work has demonstrated that SysA activity is regulated, both over gestation and in response to dietary restriction during pregnancy, we examined the response of SysA activity and sodium-dependent neutral amino acid transporter (SNAT; responsible for SysA activity) expression to cAMP analogues and amino acid deprivation in BeWo cells, an accepted model of placental syncytia. SysA activity was unaffected by forskolin, a cAMP agonist, at 48 and 72 h. Amino acid depletion was associated with an up-regulation of SysA activity, largely mediated through an enhancement of SNAT2 (Slc38a2) expression at both the protein and mRNA level. SNAT1 (Slc38a1) expression did not change in response to amino acid depletion, while SNAT4 (Slc38a4) could not be detected. In summary, SysA activity in BeWo cells responds to amino acid depletion through the differential regulation of SNAT subtypes.