We report a rare case of overlapping immunoglobulin G4-sclerosing cholangitis (IgG4-SC) and primary biliary cholangitis (PBC) in a 78-year-old woman presenting with sudden-onset jaundice, cholestatic liver enzyme elevation, and biliary strictures. Elevated alkaline phosphatase levels, positive antimitochondrial antibodies, increased serum IgG4 levels, and a high IgG4/IgG ratio, combined with endobiliary biopsy via endoscopic retrograde cholangiopancreatography biopsy showing lymphoplasmacytic infiltration and IgG4-positive cells, confirmed the diagnosis. Initial treatment with biliary drainage, ursodeoxycholic acid (UDCA), and corticosteroids provided temporary relief; however, the patient experienced recurrent episodes of cholangitis and biliary obstruction, eventually progressing to decompensated cirrhosis over 2 years. This case highlights the importance of considering IgG4-SC in patients with PBC who fail to respond adequately to UDCA and underscores the significant challenges in diagnosing and managing such overlapping syndromes. Further research is crucial to better understand the underlying pathophysiology, refine therapeutic strategies, and improve clinical outcomes of these rare, complex autoimmune conditions.

We report a rare case of overlapping immunoglobulin G4-sclerosing cholangitis (IgG4-SC) and primary biliary cholangitis (PBC) in a 78-year-old woman presenting with sudden-onset jaundice, cholestatic liver enzyme elevation, and biliary strictures. Elevated alkaline phosphatase levels, positive antimitochondrial antibodies, increased serum IgG4 levels, and a high IgG4/IgG ratio, combined with endobiliary biopsy via endoscopic retrograde cholangiopancreatography biopsy showing lymphoplasmacytic infiltration and IgG4-positive cells, confirmed the diagnosis. Initial treatment with biliary drainage, ursodeoxycholic acid (UDCA), and corticosteroids provided temporary relief; however, the patient experienced recurrent episodes of cholangitis and biliary obstruction, eventually progressing to decompensated cirrhosis over 2 years. This case highlights the importance of considering IgG4-SC in patients with PBC who fail to respond adequately to UDCA and underscores the significant challenges in diagnosing and managing such overlapping syndromes. Further research is crucial to better understand the underlying pathophysiology, refine therapeutic strategies, and improve clinical outcomes of these rare, complex autoimmune conditions.

We report a rare case of overlapping immunoglobulin G4-sclerosing cholangitis (IgG4-SC) and primary biliary cholangitis (PBC) in a 78-year-old woman presenting with sudden-onset jaundice, cholestatic liver enzyme elevation, and biliary strictures. Elevated alkaline phosphatase levels, positive antimitochondrial antibodies, increased serum IgG4 levels, and a high IgG4/IgG ratio, combined with endobiliary biopsy via endoscopic retrograde cholangiopancreatography biopsy showing lymphoplasmacytic infiltration and IgG4-positive cells, confirmed the diagnosis. Initial treatment with biliary drainage, ursodeoxycholic acid (UDCA), and corticosteroids provided temporary relief; however, the patient experienced recurrent episodes of cholangitis and biliary obstruction, eventually progressing to decompensated cirrhosis over 2 years. This case highlights the importance of considering IgG4-SC in patients with PBC who fail to respond adequately to UDCA and underscores the significant challenges in diagnosing and managing such overlapping syndromes. Further research is crucial to better understand the underlying pathophysiology, refine therapeutic strategies, and improve clinical outcomes of these rare, complex autoimmune conditions.

We report a rare case of overlapping immunoglobulin G4-sclerosing cholangitis (IgG4-SC) and primary biliary cholangitis (PBC) in a 78-year-old woman presenting with sudden-onset jaundice, cholestatic liver enzyme elevation, and biliary strictures. Elevated alkaline phosphatase levels, positive antimitochondrial antibodies, increased serum IgG4 levels, and a high IgG4/IgG ratio, combined with endobiliary biopsy via endoscopic retrograde cholangiopancreatography biopsy showing lymphoplasmacytic infiltration and IgG4-positive cells, confirmed the diagnosis. Initial treatment with biliary drainage, ursodeoxycholic acid (UDCA), and corticosteroids provided temporary relief; however, the patient experienced recurrent episodes of cholangitis and biliary obstruction, eventually progressing to decompensated cirrhosis over 2 years. This case highlights the importance of considering IgG4-SC in patients with PBC who fail to respond adequately to UDCA and underscores the significant challenges in diagnosing and managing such overlapping syndromes. Further research is crucial to better understand the underlying pathophysiology, refine therapeutic strategies, and improve clinical outcomes of these rare, complex autoimmune conditions.

We report a rare case of overlapping immunoglobulin G4-sclerosing cholangitis (IgG4-SC) and primary biliary cholangitis (PBC) in a 78-year-old woman presenting with sudden-onset jaundice, cholestatic liver enzyme elevation, and biliary strictures. Elevated alkaline phosphatase levels, positive antimitochondrial antibodies, increased serum IgG4 levels, and a high IgG4/IgG ratio, combined with endobiliary biopsy via endoscopic retrograde cholangiopancreatography biopsy showing lymphoplasmacytic infiltration and IgG4-positive cells, confirmed the diagnosis. Initial treatment with biliary drainage, ursodeoxycholic acid (UDCA), and corticosteroids provided temporary relief; however, the patient experienced recurrent episodes of cholangitis and biliary obstruction, eventually progressing to decompensated cirrhosis over 2 years. This case highlights the importance of considering IgG4-SC in patients with PBC who fail to respond adequately to UDCA and underscores the significant challenges in diagnosing and managing such overlapping syndromes. Further research is crucial to better understand the underlying pathophysiology, refine therapeutic strategies, and improve clinical outcomes of these rare, complex autoimmune conditions.

No abstract available.
Breast Neoplasms* ; Breast* ; Estrogens*

Transesophageal echocardiogram (TEE) was performed in the 86 normal persons using a UM9 of ATL with a 3.5 MHZ transducer in the Heart Center of Dong-A University Hospital during March-September 1990. 1) The transesophageal basal short axis views in the normal were seen in the Fig. 2~6. The Fig. 2 showed 3 aortic valve cusps, Fig. 3 the left coronary artery, Fig. 4 the right pulmonary artery bifurcated from the main pulmonary artery, Fig. 5 3 major vessels of superior vena cava, aorta and pulmonary artery and Fig. 6 the Left atrial appendage. 2) The transesophageal 4-chamber views in the normal were seen in Fig. 7~10. The Fig. 7 showed the left ventricular outflow tract, Fig. 8 right and left atrium and ventricle, Fig. 9 the atrial septum containing the membrane of fossa ovalis and Fig. 10 right atrium and ventricle. 3) The transesophageal transgastric short axis view in the normal was seen in Fig. 11. Fig. 11 showed the transverse image of LV and RV. 4) The transesophageal ascending aorta image was observed in Fig. 3. descending aorta image in Fig. 12 and the transesophageal aortic arch image in Fig. 14. 5) From the transesophageal 4 chamber view the septum-lateral wall dimension of the left ventricle was 5.0cm and the dimension between the apex and the closed mitral valve 6.3cm. The medial-lateral dimension of the left atrial appendage was 3.0cm and the superior-inferior dimension 4.1cm. The dimension of the descending aorta was 2.7cm and the ascending aorta 3.0cm.
Aorta ; Aorta, Thoracic ; Aortic Valve ; Atrial Appendage ; Atrial Septum ; Axis, Cervical Vertebra ; Coronary Vessels ; Echocardiography, Transesophageal ; Heart ; Heart Atria ; Heart Ventricles ; Humans ; Membranes ; Mitral Valve ; Pulmonary Artery ; Transducers ; Vena Cava, Superior

A 64-year-old man was treated with sunitinib as a first-line therapy for metastatic renal cell carcinoma. He was given oral sunitinib in cycles of 50 mg once daily for 2 weeks followed by a week off. During the 5th week of treatment right upper quadrant pain developed, but this resolved spontaneously during the 6th week (off treatment). However, on the 8th week of treatment, he was admitted to hospital because the acute right upper quadrant pain recurred with nausea, vomiting, and fever. Acute acalculous cholecystitis was then diagnosed by ultrasonography and CT. In addition, his laboratory findings indicated disseminated intravascular coagulation. Accordingly, sunitinib therapy was discontinued and broad-spectrum antibiotics initiated. He subsequently recovered after emergent percutaneous cholecystostomy. His Naranjo Adverse Drug Reaction Probability Scale score was 7, indicaing a probable association of the event with sunitinib. Suspicion of sunitinib-related acute cholecystitis is required, because, although uncommon, it can be life-threatening.
Acalculous Cholecystitis ; Anti-Bacterial Agents ; Carcinoma, Renal Cell ; Cholecystitis, Acute ; Cholecystostomy ; Disseminated Intravascular Coagulation ; Drug-Related Side Effects and Adverse Reactions ; Fever ; Humans ; Middle Aged ; Nausea ; Ultrasonography ; Vomiting

Pancreatic arteriovenous malformation (PAVM) is very rare anomaly. It occurs most commonly in the pancreatic head portion and gastrointestinal bleeding is the most common symptom. The management of PAVM is rather complex, with complete treatment usually accomplished by a total extirpation of the affected organ or at least its involved portion. We report the clinical presentation and management of 64 year-old male patient with PAVM, which was developed in pancreatic tail portion with sudden abdominal pain. Pancreatic computed tomography and magnetic resonance cholangiopancreatography were consistent with a vascular formation on pancreatic tail portion and simultaneously revealed with pseudocysts beside it. A subsequent superior mesenteric artery angiographic findings depicted PAVM on pancreatic tail portion. The patient underwent laparoscopic distal pancreatectomy and splenectomy and had a favorable outcome.
Abdominal Pain ; Arteriovenous Malformations* ; Cholangiopancreatography, Magnetic Resonance ; Head ; Hemorrhage ; Humans ; Male ; Mesenteric Artery, Superior ; Pancreas ; Pancreatectomy ; Splenectomy ; Tail

Congenital cysts of the gallbladder are extremely rare, hence only a few ciliated foregut cysts of gallbladder have been reported. We report a case of a 20-year-old woman presenting with mild right upper quadrant abdominal discomfort, with normal levels of serum bilirubin and liver function tests. Abdominal ultrasonography revealed a well-defined cystic mass measured about 2 cm attached to the neck of gallbladder, with internal echogenic debris suggesting a complicated cyst, such as a hemorrhagic cyst. Abdominal computed tomography and magnetic resonance cholangiopancreatography revealed similar findings. Laparoscopic cholecystectomy showed a slightly distended gallbladder. The size of cyst on the neck was 1.6x1.2 cm, and it contained mucosa lined by ciliated pseudostratified columnar epithelium and underlying smooth muscle layers. Histopathology identified a ciliated foregut cyst of gallbladder.
Abdomen/diagnostic imaging ; Cholangiopancreatography, Magnetic Resonance ; Cholecystectomy, Laparoscopic ; Cysts/diagnostic imaging/pathology/surgery ; Female ; Gallbladder/pathology/surgery ; Gallbladder Diseases/*diagnosis ; Humans ; Tomography, X-Ray Computed ; Ultrasonography ; Young Adult