The 5 cases of human fetal liver aged from 11 to 23 weeks of gestation were investigated for the ultrastructure of sinusoidal wall by electron microscopy. The endothelial cells deficient in basement membrane formed almost all the part of sinusoidal wall. The cells were continued with neighboring cells by intercellular junction, and overlapped them and exhibited to maintain unfenestrated capillary wall, which was different from those with fenestra in adults. The cells were found to have coated pits on luminal side and several various vesicles in the cytoplasms. The cells were related with transcellular migration of reticulocytes and acidophilic erythroblastes, which penetrated into the endothelial cytoplasm to form transient migrating pore closing after the migration into sinusoidal lumen. The perivascular cells were present at perivascular space and surrounding the sinusoid discontinuously. The Kupffer cells were easily identified with filipodia and lamellipodia and with phagosome of nuclei enucleated from acidophilic erythroblasts.
Adult ; Basement Membrane ; Capillaries ; Cytoplasm ; Endothelial Cells* ; Erythroblasts ; Erythrocytes ; Humans* ; Intercellular Junctions ; Kupffer Cells* ; Liver* ; Microscopy, Electron ; Phagosomes ; Phenobarbital ; Pregnancy ; Pseudopodia ; Reticulocytes

Holoprosencephaly is a rare malformation complex or development defect including different degrees of incomplete cleavages of the embryonic prosencephalon and varying degrees of the midface defects, resulting from the defect of prechordal mesoderm, migrating forward into the area anterior to the notochord during the third week of fetal development. Early antenatal diagnosis of holoprosencephaly is important to find out its severity, to predict its prognosis, and to determine proper management according to its prognosis and severity. The possibility of early antenatal diagnosis of holoprosencephaly by ultrasound has been suggested, but occasionally missed and rarely confirmed. We present one case of lobar holoprosencephaly, diagnosed postnatally and one case of alobar holoprosencephaly, diagnosed antenatally in our hospitals.
Fetal Development ; Holoprosencephaly* ; Mesoderm ; Notochord ; Prenatal Diagnosis ; Prognosis ; Prosencephalon ; Ultrasonography

A 24-year-old man was admitted due to an incidentally detected mass in his left testis, which showed radiopaque calcification on plain X-ray film. Left orchiectomy was performed, and the resected testis contained a well-demarcated, hard mass measuring 1.1 cm. Histological analysis revealed that the tumor was composed of neoplastic cells, fibrotic stroma, and laminated or irregularly shaped calcific bodies. The individual cells had abundant eosinophilic or clear cytoplasm with round nuclei, each of which contained one or two conspicuous nucleoli. They were arranged in cords, trabeculae, clusters, and diffuse sheets. There were several foci of intra-tubular growth patterns, with thickening of the basal lamina. Immunohistochemically, the neoplastic cells were positive for S-100 protein and vimentin, focally positive for inhibin alpha, and negative for cytokeratin, CD10, and Melan-A. In addition to reporting this rare case, we also review the relevant literature regarding large cell calcifying Sertoli cell tumors.
Basement Membrane ; Cytoplasm ; Eosinophils ; Humans ; Immunohistochemistry ; Inhibins ; Keratins ; MART-1 Antigen ; Orchiectomy ; S100 Proteins ; Sertoli Cell Tumor* ; Testis* ; Vimentin ; X-Ray Film ; Young Adult

BACKGROUND/AIMS: Proton pump inhibitor-responsive esophageal eosinophilia (PPI-REE) is a newly recognized form of eosinophilic esophagitis (EoE) that responds to PPI therapy. It remains unclear whether PPI-REE represents a subphenotype of gastroesophageal reflux disease, a subphenotype of EoE, or its own distinct entity. The aim was to evaluate the clinicopathologic features of PPI-REE. METHODS: Six patients were diagnosed with PPI-REE based on symptoms, endoscopic abnormalities, esophageal eosinophilia with > or =15 eosinophils/high-power field, and a response to PPI treatment. Symptoms and endoscopic and pathological findings were evaluated. RESULTS: The median follow-up duration was 12 months. Presenting symptoms included dysphagia, heartburn, chest pain, foreign body sensation, acid reflux, and sore throat. All patients had typical endoscopic findings of EoE such as esophageal rings, linear furrows, nodularity, and whitish plaques. Three patients had a concomitant allergic disorder, and one had reflux esophagitis. Four patients exhibited elevated serum IgE, and five had positive skin prick tests. All patients experienced symptomatic resolution within 4 weeks and histologic resolution within 8 weeks after starting PPI therapy. There was no symptomatic recurrence. CONCLUSIONS: PPI therapy induced rapid resolution of symptoms and eosinophil counts in patients with PPI-REE. Large-scale studies with long-term follow-up are warranted.
Adult ; Asian Continental Ancestry Group ; Chest Pain/etiology ; Deglutition Disorders/etiology ; Diagnosis, Differential ; Eosinophilic Esophagitis/complications/*drug therapy/*pathology ; Esophagus/pathology ; Female ; Follow-Up Studies ; Gastroesophageal Reflux/etiology ; Heartburn/etiology ; Humans ; Male ; Middle Aged ; Pharyngitis/etiology ; Phenotype ; Proton Pump Inhibitors/*therapeutic use ; Republic of Korea ; Retrospective Studies ; Sensation Disorders/etiology ; Treatment Outcome ; Young Adult

A 75-year-old woman with hypertension presented with acute suppurative cholangitis. Chest radiography revealed severe kyphosis. Abdominal computed tomography revealed a large stone impacted in the common bile duct (CBD). The patient underwent emergent endoscopic retrograde cholangiopancreatography, and cholangiography revealed a large stone (7×3 cm) in the CBD that could not be captured using a large basket. We could not use the percutaneous approach for stone fragmentation by using a cholangioscope because of severe degenerative kyphosis. Finally, we performed holmium laser lithotripsy under peroral cholangioscopy by using an ultraslim endoscope, and the large stone in the CBD was successfully fragmented and removed without complications.
Aged ; Cholangiography ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis ; Common Bile Duct* ; Endoscopes ; Female ; Gallstones ; Humans ; Hypertension ; Kyphosis* ; Lasers, Solid-State ; Lithotripsy ; Lithotripsy, Laser* ; Radiography ; Thorax

Biopsy ; Drug Therapy ; Female ; Humans ; Male ; Middle Aged ; Mouth Neoplasms ; Neck Dissection ; Prognosis ; Radiotherapy ; Retrospective Studies ; Survival Rate

PURPOSE: To investigate the relationship between changes in the posterior and anterior circulation, as seen at angiography, and the frequency and extent of cerebral infarction revealed by MR imaging in moyamoya disease. MATERIALS AND METHODS: This study involved 34 patients (22 females and 12 males, aged 2-52years) in whom cerebral angiography revealed the presence of moyamoya disease (bilateral: unilateral=24:10; total hemispheres=58) and who also underwent brain MR imaging. To evaluate the angiographic findings, we applied each angiographic staging system to the anterior and posterior circulation. Leptomeningeal collateral circulation from the cortical branches of the posterior cerebral artery (PCA) was also assigned one of four grades. At MR imaging, areas of cerebral cortical or subcortical infarction in the hemisphere were divided into six zones. White matter and basal ganglionic infarction, ventricular dilatation, cortical atrophy, and hemorrhagic lesions were also evaluated. To demonstrate the statistical significance of the relationship between the angiographic and the MR findings, both the Mantel-Haenszel chi-square test for trend and the chi-square test were used. RESULTS: The degree of steno-occlusive PCA change correlated significantly with the internal carotid artery (ICA) stage (p<0.0001). As PCA stages advanced, the degree of leptomeningeal collaterals from the PCA decreased significantly (p<0.0001), but ICA stages were not significant (p>0.05). The prevalence of infarction showed significant correlation with the degree of steno-occlusive change in both the ICA and PCA. The degree of cerebral ischemia in moyamoya patients increased proportionally with the severity of PCA stenosis rather than with that of steno-occlusive lesions of the anterior circulation. Infarctions tended to be distributed in the anterior part of the hemisphere at PCA stage I or II , while in more advanced PCA lesions, they were also found posteriorly, especially in the territories of the posterior middle cerebral artery (MCA), the posterior border zone, and the PCA (p<0.0001). The frequency of infarctions in the territories of the anterior cerebral artery (ACA) and the anterior MCA was unrelated to the degree of steno-occlusive ICA and PCA lesions (p>0.05). CONCLUSION: The degree of steno-occlusive lesions of the PCA correlated with the ICA stage. Progressive changes in steno-occlusive lesions of the ICA and PCA are associated with the extent and distribution of cerebral infarction. The degree of cerebral ischemia in moyamoya patients increased proportionally with the severity of PCA stenosis rather than with that of steno-occlusive lesions of the anterior circulation. In these patients, the presence of stenotic or occlusive PCA lesions appears to be significantly related to the occurrence of cerebral infarction.
Angiography ; Anterior Cerebral Artery ; Atrophy ; Brain ; Brain Ischemia ; Carotid Artery, Internal ; Cerebral Angiography ; Cerebral Infarction* ; Collateral Circulation ; Constriction, Pathologic ; Dilatation ; Female ; Ganglion Cysts ; Humans ; Infarction ; Magnetic Resonance Imaging ; Male ; Middle Cerebral Artery ; Moyamoya Disease* ; Passive Cutaneous Anaphylaxis ; Posterior Cerebral Artery ; Prevalence

Multiple solitary plasmacytoma is a very rare disease entity, which occurs in up to 5% of patients with solitary plasmacytomas. We report an atypical case of multiple solitary plasmacytoma that recurred in multiple visceral organs without any evidence of bone marrow involvement. A 68-year-old male presented with voiding difficulty. Twenty months earlier, he had been placed on local radiotherapy for solitary plasmacytomas in the right 6th rib and right iliac bone. Recurrences were noted 14 and 12 months later in several ribs and the 5th cervical vertebra, respectively. These were well controlled with local radiotherapy and conventional systemic chemotherapy. He had multiple soft tissue masses in the stomach, pancreas, pelvic cavity, and right buttock. An endoscopic biopsy of the gastric mass confirmed the diagnosis of plasmacytoma. Local radiotherapy to the pelvic mass and systemic therapy consisting of bortezomib and dexamethasone were given, and he has been well for 8 months.
Aged ; Biopsy ; Bone Marrow ; Boronic Acids ; Buttocks ; Dexamethasone ; Humans ; Male ; Pancreas ; Plasmacytoma ; Pyrazines ; Rare Diseases ; Recurrence ; Ribs ; Spine ; Stomach ; Viscera ; Bortezomib

BACKGROUND/AIMS: Endoscopic biliary sphincterotomy (EST) has a very important role in the treatment of pancreatico-biliary diseases, but it has complications. We retrospectively investigated whether the types of electrosurgical currents affect occurence of complications. MEHTODS: In pancreatico-biliary diseases, consecutive 150 patients undergoing EST with pure cutting current (cutting group) and then consecutive 150 patient with blend current (blended group) were studied in respect to post-EST complications and the presence of periampullary diverticulum and papillitis in major papilla. Major bleeding was defined as a decrease in hemoglobin of at least 2 g/dL. Clinical pancreatitis was defined as abdominal pain with elevated serum amylase above three times about the upper normal limit after 48 hour. RESULTS: Major bleeding has not occurred. Minor bleeding occurred in 25/300 patients (8.3%), but was not different in cutting group (n=150) and blended group (n=150). Except 54 patients with elevated amylase before procedure, pancreatitis occurred in 13/246 patients (5.3%), but was not different in cutting group (n=122) and blended group (n=124). Two cases in blended group had moderate pancreatitis. There was no differences of bleeding, pancreatitis, in patients with diverticulum (n=112) and without diverticulum (n=188). Minor bleeding was more common in patients with papillitis (n=24) than without papillitis (n=276) (p=0.000), but the incidence of post EST pancreatitis was similar. In 235 patients with choledocholithiasis, there was no difference in terms of bleeding, pancreatitis in cutting group (n=120) and blended group (n=115). CONCLUSIONS: Post- EST bleeding and pancreatitis were not affected by the types of electrosurgical current used. When EST was performed in patient with papillitis, minor bleeding was observed commonly.
Abdominal Pain ; Amylases ; Choledocholithiasis ; Diverticulum ; Hemorrhage ; Humans ; Incidence ; Pancreatitis ; Papilledema ; Retrospective Studies ; Sphincterotomy, Endoscopic*

Central nervous system (CNS) myelomatosis, which is the presence of monoclonal plasma cells in the cerebrospinal fluid (CSF), is extremely rare. We report a case of CNS myelomatosis developed in a 45-year-old woman with multiple myeloma in complete response, which was achieved by allogeneic peripheral blood stem cell transplantation using a reduced-intensity conditioning regimen consisting of melphalan, fludarabine, and antithymocyte globulin. Two months after the transplant, she developed a moderate motor and sensory weakness in both lower extremities. Atypical plasma cells were found in the CSF, and immunofixation revealed monoclonal light chain in the CSF. She was given three courses of weekly intra-thecal chemotherapy consisting of methotrexate, cytarabine, and dexamethasone, which cleared the CSF. This case indicates that the allogeneic transplantation could not control CNS myelomatosis, despite successfully treating the bone marrow myeloma.
Antilymphocyte Serum ; Bone Marrow ; Central Nervous System ; Cytarabine ; Dexamethasone ; Female ; Hematopoietic Stem Cell Transplantation ; Hematopoietic Stem Cells ; Humans ; Light ; Lower Extremity ; Melphalan ; Methotrexate ; Middle Aged ; Multiple Myeloma ; Nervous System ; Peripheral Blood Stem Cell Transplantation ; Plasma Cells ; Transplantation, Homologous ; Transplants ; Vidarabine