Clinical and genetic analysis of a pedigree of a thirty-six-year-old familial Alzheimer's disease patient.
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Adult↗
Biomedical subjects
Y L Suh
Publications and source records attributed to Y L Suh.
Pseudopapillary neurocytoma of temporal lobe with glial differentiation.
We present a case of cerebral neurocytoma with unusual pseudopapillary pattern, which was a predominant feature in the tumor and was characterized histologically by hyalinized vascular cores surrounded by a single or multilayered small round cells. Vascular hyalinization was also evident in the linear arborizing capillary networks in the cellular mass of the tumor. Immunohistochemically, the tumor cells were strongly positive for synaptophysin and neuron-specific enolase except some cells lining the pseudopapillae, which showed immunoreactivity for glial fibrillary acidic protein, vimentin and S-100 protein. Ultrastructural examination revealed neuritic process of the tumor cells with occasional synaptic structures and neurosecretory granules. This report suggests that neurocytoma should be included in the differential diagnosis of papillary tumors in the central nervous system.
Adult↗
Upper esophageal stenosis: two case reports.
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Child, Preschool↗
Congenital cystic adenomatoid malformation of the lung: CT-pathologic correlation.
OBJECTIVE: The purpose of this study was to correlate CT findings of congenital cystic adenomatoid malformation (CCAM) of the lung with pathologic findings. MATERIALS AND METHODS: CT scans of CCAM from 21 consecutive patients were analyzed retrospectively by two chest radiologists who achieved consensus. Pathologic findings were assessed by an experienced pulmonary pathologist. Preoperative CT findings were correlated with pathologic findings. RESULTS: Areas with small cysts (< 2 cm in diameter) were seen on CT scans in 19 (90%) of 21 patients, whereas areas with a large cyst (> 2 cm in diameter) were observed in 18 patients (86%). Areas of consolidation (n = 9; 43%) with heterogeneous attenuation on enhanced scans and areas of low attenuation (lower than normal lung) around cystic lesions (n = 6; 29%) were also seen on CT scans. The diameter of the largest cyst seen on CT scans in each patient ranged from 1.0 to 8.0 cm (median, 4.5 cm). Cysts that CT showed to be filled with air, fluid, or both correlated completely with the pathologic findings. Areas of consolidation corresponded histologically to areas of glandular or bronchiolar structures with or without areas of endogenous lipoid or organizing pneumonia or mucus plugs. Areas of low attenuation corresponded to areas of microcysts blended with normal lung parenchyma. CONCLUSION: CT scans show the variable internal characteristics of CCAM and can suggest the underlying pathology of such lesions.
Adolescent↗
Microvillus inclusion disease in two Korean infants.
We report two cases of microvillus inclusion disease and these are the first cases in Korea. The two babies (one baby had a sibling who died of diarrhea in the neonatal period) had excreted their stools up to 200 ml/kg per day since several days after birth. Workup's included extensive infectious, immunologic, hormonal and rheumatologic studies, all of which were negative or normal. Diagnosis rested on the ultrastructural finding of intracytoplasmic inclusions that contained intact microvilli on electron microscopy. We tried somatostatin analogue (octreotide, 4 micrograms/kg/day), cholestyramine (up to 4g t.i.d.), steroid (prednisone, 2 mg/kg/day) and intravenous epidermal growth factor (100 ng/kg/hr for 2 weeks), but there was mild improvement with cholestyramine (decrease stool volume) and epidermal growth factor (increase the number of microvilli per cell) but no improvement was noted with the other treatments. Although it is a rare disorder and the prognosis of microvillus inclusion disease is poor, it must be considered if an infant has chronic secretory diarrhea.
Diarrhea, Infantile↗
Prenatal and postnatal changes of the human tonsillar crypt epithelium.
The tonsillar crypt shows infiltration of lymphoid cells in the epithelium and the highly porous or discontinuous basement membrane. However, the relationship between developmental period and the significance of the disrupted epithelial basement membrane and infiltration of lymphoid cells within the human tonsillar crypt epithelium is still obscure. The present study was designed to determine the prenatal and postnatal changes of the human tonsillar crypt epithelium in 4 fetuses, 4 neonates, 1 infant, 5 children and 4 adults using immunohistochemical stains with polyclonal anti-laminin and monoclonal anti-type IV collagen antibody, and transmission electron microscope. The tonsillar crypt epithelium from all fetuses and neonates showed infiltration of many lymphoid cells and an intact basement membrane by immunohistochemistry. Transmission electron microscope revealed pores in the basement membrane through which lymphocytes passed. Tonsils from a 4-month-old infant showed focal disruption of the basement membrane by immunohistochemical staining. The tonsils from children and adults showed that there was massive disruption of the basement membrane with lymphoid cell infiltration in the tonsillar crypt epithelium by immunohistochemical staining and transmission electron microscopy. In conclusion, lymphoid cell infiltration and holes of the basement membrane of the tonsillar crypt epithelium are normal developmental findings, whereas disruption of the basement membrane of the tonsillar crypt epithelium is pathological.
Adolescent↗
Nonspecific interstitial pneumonia with fibrosis: radiographic and CT findings in seven patients.
PURPOSE: To describe the radiographic and computed tomographic (CT) findings in seven patients with nonspecific interstitial pneumonia with fibrosis (NIP). MATERIALS AND METHODS: NIP was proved pathologically in all patients. Findings at radiography and initial and 1-15-month follow-up CT were reviewed. RESULTS: The predominant radiographic abnormalities were areas of patchy parenchymal opacification present bilaterally in the middle and lower lung zones in six patients; in one patient, the chest radiograph depicted no abnormality. The most common finding observed on initial thin-section CT scans was bilateral, patchy areas of ground-glass attenuation present alone or with areas of consolidation in five patients (71%) or irregular lines in two (29%). At follow-up CT, the initial parenchymal abnormalities had resolved completely in three patients, improved in another three, and persisted in one. CONCLUSION: Bilateral patchy areas of opacity depicted radiographically and good clinical response to treatment may differentiate NIP from other interstitial pneumonias.
Adult↗
Mansonian schistosomiasis in rectum--report of a case.
Schistosomiasis is a snail-transmitted trematodiasis acquired by immersion in water which contains the cercariae. In Korea, six imported cases of urinary schistosomiasis by Schistosoma haematobium and one case of imported cerebral schistosomiasis by S. mansoni were reported. Herein we report a case of S. mansoni infecting rectum of a 46 year-old Korean male, who had been to Saudi Arabia for two years. On colonoscopy for routine physical check up, a 0.4 cm polyp in the rectum was detected and biopsy was done. Microscopically, rectal mucosa showed several granulomas which were composed of macrophages, lymphocytes, neutrophils and eosinophils. The center of each granuloma showed an ovoid egg often containing miracidium. The eggs measured 130 x 60 microns in average size. They had yellowish-brown transparent shell with the characteristic lateral spine. This is the 8th imported case of schistosomiasis in Korea and the second one infected by S. mansoni.
Animals↗
Effect of tibial lengthening on the gastrocnemius muscle. A histopathologic and morphometric study in rabbits.
We observed the changes of the gastrocnemius muscle in relation to the percentage of lengthening of the rabbit's tibia by callotasis. 75 rabbits were separated into 3 lengthening groups, 10, 20, and 30 percent lengthening, respectively. Histopathologic observations, based on the fiber size variation, internalization of the nuclei, degeneration, regeneration, and endomysial fibrosis of muscle fibers, revealed that substantial changes occurred in the latter groups. Histomorphometrically, the decrease in the mean size of Types I and II muscle fibers was observed in all lengthening groups, but there was no significant change in the proportion of the muscle fiber types in any of the lengthening groups.
Animals↗
Central core disease--a case report.
Central core disease is a rare congenital myopathy characterized by the formation of "cores" that consist of abnormal arrangement of myofibrils inside the myofibers. We report a 5-year-old Korean girl who showed a fairly typical clinical course of non-progressive muscle weakness. Electrodiagnostic studies showed low-amplitude polyphasic electromyograph and normal nerve conduction velocity. Gastrocnemius muscle biopsy showed central cores in over 80% of the fibers on H&E section. Histochemistry revealed deficient or absent mitochondrial enzyme in the cores and type I predominance. Ultrastructurally both structured and non-structured cores were found separately or simultaneously in one fiber. This case is the first report in the Korean literature.
Child, Preschool↗
Nerve sheath myxoma (neurothekeoma)--a case report.
A case of nerve sheath myxoma also called as neurothekeoma in a 33-year-old woman is described. The lesion appeared as a painful, elevated nodule on the scalp for several months, without an appreciable increase in size. Microscopically, it showed typical histologic characteristics of nerve sheath myxoma, and tumor cells revealed strong, positive reaction for S-100 protein and negativity for epithelial membrane antigen (EMA) on immunohistochemical staining. These immunohistochemical findings of this case support the view that the origin cells of this tumor may be schwann cells rather than perineurial cells. The histogenesis and differential diagnosis of this tumor are discussed.
Adult↗
Autoimmune oophoritis--a case report.
A case is described of an autoimmune oophoritis that was diagnosed unexpectedly after a hysterectomy and bilateral salpingo-oophorectomy had been performed on the suspicion of ovarian cysts. The patient was a 43-year-old multiparous woman who presented with vaginal bleeding and lower abdominal pain which she had had for one month. Grossly, the ovaries were enlarged and multicystic. The cysts measured up to 3.0 cm. The major histological change was a lymphoplasmacytic infiltrate in close relation to the theca interna of developing, cystic and atretic follicles, but sparing the primordial follicles. The infiltrate increased in density with the follicular maturation and culminated against the corpus luteum. With involution of the developing follicles, the inflammatory infiltrate subsided to some extent. The proportion of the plasma cells increased with the density of the infiltrates. Immunohistochemical study of the ovarian mononuclear cell infiltrate revealed a mixture of B- and T-lymphocytes. The plasma cells were polyclonal. These histological features of the present case are typical of autoimmune oophoritis although the presence of autoantibodies and hormonal level in the patient's serum were unknown. This case may be identified as in the early active stage of autoimmune oophoritis.
Adult↗
Neonatal hemochromatosis--report of an autopsy case.
A case of neonatal hemochromatosis in a 3-hour-old male is described. He presented with hypotonia, mild jaundice, and respiratory difficulty immediately after birth. He had no evidence of congenital infection, immune-related hemolysis or exogenous iron uptake. Postmortem examination revealed abnormal facial features. The organs were of normal weight for his age except a small liver and lungs, and a large spleen. The most prominent changes were in the liver and pancreas. The liver was coarsely nodular and fibrotic. The lobular architecture was totally distorted by innumerable multinucleated giant cells, loss or collapse of the hepatocytes, and diffuse fibrosis. A large amount of hemosiderin was seen in the liver, pancreatic acini and thyroid follicular cells. Scanty amount of hemosiderin was also found in the myocardial fibers and renal tubular cells. The pancreas showed hyperplasia and hypertrophy of the islets. The spleen showed severe congestion and a moderate extramedullary hemopoiesis but no deposits of hemosiderin. This patient had three siblings died in neonatal period, one of which had clinical features of neonatal hemochromatosis.
Face↗
Benign cystic mesothelioma of the peritoneum--a case report.
A case of benign cystic mesothelioma in a 53-year-old woman is presented. The patient had abdominal pain and a palpable mass for 4 days. This mass was noticed incidentally by an obstetrician. A computed tomographic scan of the abdomen and pelvic sonography showed a cystic mass, 6 x 4 cm, in the right lower quadrant close to the cecum and suggested cystadenocarcinoma of the ovary. Operation showed that the mass involved peritoneum in region of the appendix. Complete removal of the mass was done. Grossly it consisted of multilocular cyst containing clear, serous, gelatinous fluid. The light microscopic examination revealed that this lesion consisted of cystic spaces of various size and intervening connective tissue stroma. The cells lined the cysts varied from flattened to cuboidal with occasionally a picket-fence or hobnailed appearance in areas. Brush borders were seen on the luminal surface of some cells. Electron microscopic examination confirmed that the cells were mesothelial origin. This lesion mimics cystic lymphangioma of the abdomen grossly and light microscopically, from which differential features are discussed.
Female↗
Telangiectatic osteosarcoma--a case report.
Telangiectatic osteosarcoma is a rare and special variant of osteogenic sarcoma with distinct radiologic, gross and microscopic features. This tumor is predominantly lytic, destructive tumor without sclerosis on roentgenogram, and is soft and cystic on gross examination. Histologically aneurysmally dilated spaces lined or traversed by stromal cells producing osteoid are noted. This report concerns a case of telangiectatic osteosarcoma occurring in a 7 years old boy. He presented with pathologic fracture of the right distal tibia, followed by a purely lytic lesion on X-ray examination. This lesion recurred five times during a span of one year. Microscopic features of the biopsy specimen was difficult to differentiate from aneurysmal bone cyst because of prominant blood-filled cyst formation. It was finally identified as osteosarcoma from the below-knee amputation specimen through the close examination for anaplastic osteoid-producing stromal cells in the septa that separate the blood cysts.
Aneurysm↗
Primary intimal fibroplasia of the renal artery.
Two cases of primary intimal fibroplasia of the renal artery with renovascular hypertension are described. Case 1 was 27 year old female who was incidentally found to have blood pressure of 210/130 mmHg on routine physical examination. Renal arteriogram revealed tubular narrowing of the mid protion of the left renal artery. Both patients showed lateralization in renin activity at the involved side renal vein. Case 2 was a 10 year old girl who was first noted to have a hypertension of 180/120mmHg after a sudden attack of seizure, vomiting and altered consciousness. Renal arteriogram showed concentric narrowing of the proximal half of the right renal artery. Histopathologic examination of the affected arterial segments from both cases showed essentially same findings, i,e., diffuse fibrous thickening of the intima occluding the lumen, focal fragmentation, duplication and disappearance of the internal elastic membranes. There were no deposit of lipid and inflammatory cells. The media and adventitia remained intact. The blood pressure of both patients became normal, after the surgery and the patients are in good health up to this time.
Adult↗
Congenital absence of ductus arteriosus--an autopsy case.
Isolated absence of the ductus arteriosus is extremely rare condition although the ductus arteriosus may be hypoplastic or aplastic in association with other aortic arch anomalies. Authors described a case of isolated agenesis of the ductus arteriosus documented by postmortem examination of a newborn infant who died of pneumonia following operation for a large omphalocele. The heart showed ventricular septal defect. However, no other cardiovascular anomalies were associated in this case. There were three vessels that were taking off from the aorta consisted of the right brachiocephalic artery, left common carotid-artery and left subclavian artery. The anteriorly located pulmonary artery was divided into the right and left pulmonary arteries. There was no connection of vessel between the pulmonary artery and the aorta.
Abnormalities, Multiple↗
A case of progressive hypertrophic neuropathy in childhood with facial diplegia (Dejerine-Sottas disease).
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Axons↗