Background: Primary sclerosing cholangitis (PSC) is a rare chronic immune-mediated liver disease affecting intra and extrahepatic bile ducts, in which inflammation and fibrosis are the main factors that lead to biliary stricture. The pathogenesis of this disease is still unclear. It happens approximately 0.5 to 1.3 cases per 100,000 person-years in Northern Europe. PSC is difficult to diagnose until complications arise. PSC can also be a manifestation of mixed connective tissue disease (MCTD) in the liver. A chronic immune disease that has occurred since childhood may result in delayed development and short stature. Case Presentation: A 18-year-old female presented with icteric, anemia, short stature, and absence of menstruation. Laboratory and radiological findings suggested obstruction of intra and extrahepatic biliary duct (conjugated bilirubin 11,80 mg/dL), severe anemia, normal sex hormone levels, increased RNP/SM antibodies. Histopathology showed liver cirrhosis suggestive for primary sclerosing cholangitis. The patient then was given biliary stenting, transfusion of packed red cell, immunosuppresive, corticosteroid, ursodeoxycholic acid. Within a year of treatment, there was a significant decrease of direct bilirubin and improvement in symptoms.Conclusion: Patient with PSC, MCTD, and short stature is a very rare case. This is the first case that has been found in dr. Kariadi Hospital, Semarang. It has high mortality rate and difficult to diagnose and treat. According to multiple guidelines, this patient should have liver transplantation , but in this case, biliary stenting and oral treatments have resulted in an excellent outcome.
Diabetic ulcer patients can be hampered their ulcer healing process. The condition is caused by hyperglycemia and accumulation of advanced glycation end-products (AGEs) that can cause interference with VEGF and its receptors and signaling pathways. The VEGF core region is formed by a cystine bond motif with 8 invariant cystine residues in inter and intramolecular disulfide bound to the end of the central 4-stranded at each monomer with a side-by-side antiparallel orientation. VEGF stimulates angiogenesis in three dimensions, causing the encounter of microvascular endothelial cells, penetration into collagen gels and forming capillary-like structures. Regulation of VEGF gene expression through: (1) hypoxia; (2) cytokines and (3) differentiation and transformation. VEGF stimulates wound healing through several mechanisms such as collagen deposition, angiogenesis and epithelialisation.
Monitoring of glucose levels is essential in preventing the complications of diabetes mellitus, including short, medium and long-term monitoring. Short-term monitoring includes random plasma glucose, fasting plasma glucose, 2-hour post prandial plasma glucose and Oral glucose tolerance tests (OGTT). The medium-term monitoring includes fructosamine and Glycated albumin (GA) while the long-term monitoring is glycated hemoglobin (HbA1c). Currently, the most recommended examination for glucose level monitoring in patients with diabetes mellitus is the glycated hemoglobin (HbA1c). However, there seem to be some conditions where the HbA1c value is doubtful or unreliable. Some of these conditions include anemia, thalassemia, dialysis and pregnancy. The best choice at this time is GA.
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