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Biomedical subjects

J G Chi

Publications and source records attributed to J G Chi.

At least 145 records · Page 8Linked to original sources

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↗

Multicore myopathy--a case report.

Multicore myopathy is a rare congenital myopathy. The multicores consist of numerous small areas of decreased oxidative enzyme activity. The long axis of the lesion is perpendicular or parallel to the long axis of the muscle fiber. These cores are usually smaller than central cores. For this reason they are also called minicores. Although the multicores represent a nonspecific change in that they can be observed in malignant hyperthermia, muscular dystrophy, inflammatory myopathy, etc. Muscular weakness dating from early infancy is combined large proportion of the muscle fibers. In about half of the reported cases the muscular weakness has not been progressive, while in the others a slow progression has occurred. This 9-year-old boy presented with congenital nonprogressive myopathy associated with thoracic scoliosis and bilateral equinovarus deformity. The serum creatine phosphokinase and lactic dehydrogenase levels were normal. Electromyography showed "myopathic" features. The biopsy revealed a marked size variation in myofibers, ranging from 10 microns to 100 microns. A few small angular fibers and slight endomyseal fibrosis were also noted. There was type I fiber predominance. NADH-TR reaction disclosed more well-defined cores with loss of intermyofibrillary mitochondrial activity. These cores were usually located with loss of intermyofibrillary mitochondrial activity. These cores were usually located in the peripheral portions of the myofibers and the core size measured 10-30 microns in diameter. Electron microscopic examination revealed circumscribed areas of disintegrated Z band material and disorganized sarcomeric units near the sarcolemma. A decrease in the number of mitochondria and glycogen particles was noted.

Biopsy↗

Hepatic capillariasis: first case report in the Republic of Korea.

We report a case of massive hepatic infection by Capillaria hepatica in a 14-month-old girl who presented with the symptom triad of persistent fever, hepatomegaly, and leukocytosis with eosinophilia. Twenty-five cases of human infection with this parasite, mostly in children, have been reported in the literature. This is the first case of hepatic capillariasis reported in the Republic of Korea. The diagnosis was made by needle biopsy of the liver. Scanning electron microscopic examination of the biopsy specimen was also performed. Thiabendazole therapy was initiated and the patient developed liver disease-related IgA nephropathy during the therapy. The literature dealing with proven cases of infection with C. hepatica is briefly reviewed.

Animals↗

[Introduction and establishment of pathology in Korea (1910-1960)].

The concept of modern pathology had not been present in Korea until it was introduced through the Japanese pathologists who came to Korea to work as staff pathologists at [Korean] Hospital in 1913. Two prominent Japanese pathologists at that time were Dr. Inamoto and Dr. Tokumitzu who taught pathology at Hospital affiliated Medical Institute. Department of Pathology was established at Keijo Medical College in 1916 and at Keijo Imperial University Faculty of Medicine in 1926. In the private medical school, Severance Union Medical School, the pathology was taught by Japanese and American Physicians until Dr. Il Sun Yun established Department of Pathology in 1929. Papers on histopathological description of disease started to appear in the medical literature, Chosen Medical Journal that was first issued just after Japanese occupation of Korea in 1910. Many diseases of Koreans had been scientifically documented in medical literature since 1911 by the leading role of faculty pathologists of Keijo Medical College and Keijo Imperial University Faculty of Medicine until 1940. ...

Education, Medical↗

MR appearance of central neurocytoma.

To provide a detailed description of the MR appearances of central neurocytoma, MR images of 13 patients with central neurocytoma were retrospectively reviewed and compared with CT examinations. The histology was confirmed by ultrastructural and immunohistochemical studies. In 12 patients the tumors were histologically benign and located in the anterior part of the lateral ventricle, 6 of which extended to the 3rd ventricle. There was one case of a histologically malignant variant involving the thalamus and lateral ventricle. The tumors were primarily solid, but contained cysts (85%, 11/13), calcifications (69%, 9/13), and signal void from tumor vessels (62%, 8/13), frequently producing heterogeneous signal intensity on both T1- and T2-weighted images. Most of the solid portion appeared isointense or slightly hyperintense relative to the cerebral cortex on all MR pulse sequences. Calcifications were iso- or hypointense on MR, making them difficult to characterize with MR alone. Intratumoral hemorrhage was seen in 2 patients on MR but not on CT. Contrast enhancement was variable in degree and pattern. Coronal and sagittal MR images were valuable in evaluating the tumor extent and origin site, and in planning the surgical approach. It is concluded that MR imaging appears to be more useful than CT in the overall evaluation of central neurocytoma, even though calcification is better characterized with CT.

Adult↗

Cerebral sparganosis: analysis of 34 cases with emphasis on CT features.

Cerebral sparganosis is a rare parasitic CNS disease, producing chronic active granulomatous inflammation. We retrospectively reviewed the clinical data, CT scans and histopathologic specimens in 34 patients with cerebral sparganosis. The majority of the patients (89%) were rural inhabitants; 75% had a history of ingestion of frogs and/or snakes. The major presenting symptoms were seizure (84%), hemiparesis (59%) and headache (56%) of chronic course. On CT scans, the disease most frequently involved the cerebral hemispheres, particularly frontoparietal lobes, with occasional extension to the external and internal capsules and basal ganglia. The cerebellum was rarely involved. Bilateral involvement was seen in 26%. The main CT findings consisted of white matter hypodensity with adjacent ventricular dilatation (88%), irregular or nodular enhancing lesion (88%), and small punctate calcifications (76%). In combination, the CT triad above appears to be specific for this disease, and was noted in 62% of cases. Of 16 follow-up CT scans, 5 (38%) showed a change in the location of the enhancing nodule. With a single CT scan, it does not appear to be possible to determine whether the worm is alive or dead, information important for deciding whether to intervene surgically. Change in the location of the enhancing nodule and/or worsening of the other CT findings on sequential CT scans would suggest that the worm is alive and that the patient is a candidate for surgery.

Adult↗

Inflammatory pseudotumor of the lung manifesting as a posterior mediastinal mass.

A case of inflammatory pseudotumor of the lung in a 6-year-old boy is presented. The respiratory illness presented as a mycoplasma pneumonia and there had been a similar episode of mycoplasma pneumonia one and a half years previously. Computed tomography revealed a large, calcified right posterior mediastinal mass and exploratory thoracotomy revealed a large posterior mediastinal mass that had an endobronchial component and grew exophytically from the lung. The present case suggests that inflammatory pseudotumor of the lung can manifest as a mediastinal mass and could be associated with mycoplasma pneumonia infection.

Child↗

HLA-DR expression in human fetal thymocytes.

We analyzed the expression of MHC class I (W6/32) and class II (HLA-DR) antigens on human fetal and postnatal thymocytes by fluorescence-activated cell sorting. Less than 5% of prenatal thymocytes expressed HLA-DR before week 12 of gestation. However, the number of HLA-DR-positive cells significantly increased during the late second and third trimester of gestation, when greater than 50% of prenatal thymocytes expressed HLA-DR. Such high-level expressions of HLA-DR in fetal thymocytes were also demonstrated by Northern-blot analysis and immunohistochemistry. After birth, the percentage of HLA-DR-positive cells in thymocytes decreased gradually. A high-level expression of class I antigen was also observed in thymocytes from the early stages of gestation, but, in contrast to MHC class II, a majority of postnatal thymocytes maintained high levels of class I antigen after birth.

Cell Separation↗

Prenatal growth pattern of the human maxilla.

Regarding maxillofacial morphogenesis there has been a long debate on the growth of the maxillary structure. Using 120 normal fetal maxillae of gestational ages from 16 to 41 weeks, palatal radiograms and frontal histologic sections were made. We have observed two pairs of accentuated growth areas in the fetal maxillae and named them primary growth centers to formulate the maxillary trapezoid (MT) by radiologic image. The MT is formed by four primary growth centers that are best demonstrated by palatal radiograms of the fetal maxilla as well as by frontal histologic sections. The dimensional increase in the MT during the fetal period is documented and statistically analyzed. From this series of results, we have suggested that the growth centers which demarcate the MT are the basic structures of the developing human maxilla. It was also found that the four primary growth centers are the most active sites for maxilla formation until 20 weeks of gestation and thereafter the growth of the maxilla is enhanced by the participation of the intramembranous bone formation along the periphery. This was in contrast to the central primary growth centers that have already finished maturation in the early fetal period and remain only as a peripherally radiating arrangement of thick trabecular bones.

Bone Development↗

Intraventricular neurocytoma: clinicopathological analysis of seven cases.

A retrospective analysis of seven patients with intraventricular neurocytoma is presented. Patient age at diagnosis ranged from 15 to 38 years (mean 24.6 years) and the male:female ratio was 6:1. Raised intracranial pressure due to hydrocephalus was the main cause of the clinical manifestations. An isodense mass with multiple intratumoral cysts and homogeneous contrast enhancement was the characteristic computerized tomography finding. The lesions commonly involved the lateral ventricle with or without extension to the third ventricle. Cerebral angiography showed homogeneous vascular staining in five patients. Magnetic resonance images revealed a mass isointense with the cerebral cortex on both T1- and T2-weighted images. Gadolinium-diethylenetriaminepenta-acetic acid-enhanced images showed homogeneous enhancement. Total removal of the tumor was possible in four patients. Pathologically, six cases were initially diagnosed as oligodendroglioma and the remaining case as ependymoma. However, immunohistochemical studies demonstrated strong positivity for neuron-specific enolase in all seven cases and for synaptophysin in five cases. On electron microscopy, three cases showed well-defined neurosecretory granules and 10-nm microtubules in their cytoplasm and cytoplasmic processes. One patient developed a recurrent tumor 18 months after surgery. The remaining six patients are free of recurrent tumors at 2 to 62 months after surgery. It is suggested that neurocytoma must be included in the differential diagnosis of intraventricular lesions, and that electron microscopic and immunohistochemical studies should be undertaken.

Adolescent↗

Malignant triton tumor of the acoustic nerve. Case report.

The authors present the clinical, radiological, pathological features, and autopsy findings of a patient with malignant triton tumor of the acoustic nerve, which probably arose from a pre-existing acoustic schwannoma. The term "malignant triton tumor" is applied to malignant schwannomas with rhabdomyoblastic differentiation. A cerebellopontine angle tumor with spinal drop metastasis occurred in this patient 10 months after near-total removal of the original tumor.

Female↗

Pancreatoblastoma--histopathological and ultrastructural analysis of two cases.

Pancreatoblastoma has been described in children and characterized by unique histologic features and excellent clinical course. Ultrastructural and immunohistochemical studies of pancreatoblastoma reveal either exocrine alone or both endocrine and exocrine differentiation. We present two cases of pancreatoblastoma in children in which immunohistochemical and ultrastructural examination failed to demonstrate features of either enzyme or hormone production and which became worse in clinical course. We assume that pancreatoblastomas are tumors which differentiate more toward acinar or ductal elements than toward islet cell.

Carcinoma↗

Inflammatory bowel disease in children--clinical, endoscopic, radiologic and histopathologic investigation.

This paper reviews our five years' clinical experience (1987 to 1991) of 22 patients with inflammatory bowel disease (IBD). There were 12 patients with Crohn's disease and 10 patients with ulcerative colitis. The mean age at diagnosis was 8.7 years (2 to 14 years). Clinical impressions before referral were chronic diarrhea in 11, irritable bowel syndrome in 5, colon polyp in 4, lymphoma in 3, intestinal tuberculosis in 2, amoebic colitis in 2, ulcerative colitis in 2 children and other diseases. The mean interval from the onset of symptoms to the diagnosis of IBD was 18 months. Diagnosis of Crohn's disease was delayed for more than 13 months in 8 (67%), whereas that of ulcerative colitis was delayed for more than 13 months in 4 (40%). Diarrhea (50%), abdominal pain (36%) and rectal bleeding (36%) were the three most frequent presenting complaints of IBD. Moderately severe abdominal pain was a more common chief complaint in Crohn's disease (58%) than in ulcerative colitis (10%). Hematochezia (90% vs 17%) and moderately severe diarrhea (90% vs 75%) were more common gastrointestinal manifestations in ulcerative colitis than in Crohn's disease. The associated extraintestinal manifestations were oral ulcer in 7, arthralgia in 11 and arthritis in 4, skin lesions in 2, eye lesions in 2 and growth failure in 9 patients. Of 12 children with Crohn's disease, granuloma was found in 5, aphthous ulcerations in 8, cobble stone appearance in 8, skip area or asymmetric lesions in 6, transmural involvement in 7, and perianal fistula in 3. Among 10 children with ulcerative Colitis, there were crypt abscess in 8, granularity or friability in 10 and rectosigmoid ulcerations with purulent exudate in 8 children. The main sites of involvement in children with Crohn's disease were both the small and large bowels in 7 (58%), small bowel only in 2 (16%), and colon only in 3 (25%). Terminal ileum involvement was seen in 75% of Crohn's disease cases. The main sites of involvement in children with ulcerative colitis were total colon in 4 (40%), up to the splenic flexure in 2 (20%), rectosigmoid in 3 (30%) and rectum only in one (10%). Medical treatment including sulfasalazine, and systemic or topical steroid was administered initially in most patients. Seven of 12 patients with Crohn's disease and 2 of 10 patients with ulcerative colitis were operated on.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗

Papillary muscle necrosis in neonates and infants--analysis of 209 autopsies.

A total of 209 consecutive neonate and infant autopsies were reviewed with special attention to papillary muscle necrosis (PMN) of the heart. Associated major pathological findings were analysed for the evaluation of significant pathological accompaniments of PMN. PMN was found in 52 cases among 171(30.4%) neonates and major pathological accompaniments were bronchopneumonia, hyaline membrane disease, hypoxic neuronal change, sepsis, subarachnoid hemorrhage, disseminated intravascular coagulation (DIC) and acute tubular necrosis, among which hypoxic neuronal change and ATN had a statistically significant higher incidence when compared with the control group. (p < 0.005). PMN was found in 13 cases among 38(34.2%) infants and accompaniments were congenital heart disease, sepsis, bronchopneumonia, DIC and hypoxic neuronal change, all of which showed no difference from the control group in incidence. The results imply that PMN is a kind of organ damage in stressed subjects regardless of age, that it is not a special form of myocardial injury in any specific age group including the newborn period, and is possibly of different pathogenesis and significance.

Humans↗

An unusual combination of trisomy 21 and partial trisomy 5q.

The authors describe a male newborn with multiple congenital anomalies; craniofacial dysmorphism, bilateral cleft palate and lip, ambiguous external genitalia with absence of phallus, ventricular septal defect, agenesis of olfactory bulbs, and presence of small round cells simulating migration defect in the cerebellar white matter. Cytogenetic study demonstrated a chromosomal constitution of 47,XY, +21, +5q. Its pathological significance compared with Down's syndrome and hitherto reported partial trisomy 5q is discussed.

Abnormalities, Multiple↗

[A case of fatal malabsorption syndrome caused by strongyloidiasis complicated with isosporiasis and human cytomegalovirus infection].

This 54-year-old Korean coal miner suffered from continuous watery diarrhea and weight loss after corticosteroid treatment (beta-methasone, 4 mg daily for 1 week) due to hip-bone fracture in January 1991. Except for the short therapy of steroid, no other histories were contributory. The malabsorption syndrome was aggravated while the case was treated under the impression of amebiasis or intestinal tuberculosis. AIDS antibody test by EIA was negative and quantitative analysis of serum immunoglobulins was in normal ranges. Nine months after the onset of symptoms, the case was diagnosed as malabsorption syndrome caused by complexed and aggravated infection by Strongyloides stercoralis, Isospora and cytomegalovirus in the small intestine, which were proved by stool examination and duodenal biopsy. His clinical course became worse even after high-dosed and prolonged albendazole treatment for strongyloidiasis with supportive fluid therapy. The patient was discharged in hopeless status in November, 1991 and died after one week at home.

Animals↗

Idiopathic hypereosinophilic syndrome terminating as disseminated T-cell lymphoma.

The authors describe a case of idiopathic hypereosinophilic syndrome (HES) terminated as a T-cell lymphoma in a 3-year-old girl. The clinical course was chronic and characterized by chronic eczema, persistent peripheral blood eosinophilia, organomegaly, interstitial lung change, and pericarditis. Postmortem examination demonstrated a disseminated T-cell lymphoma involving the inguinal lymph node, liver, lung, and kidney. The findings of the current case suggest a possibility that certain abnormalities in this case of idiopathic HES per se may have triggered the development of malignant lymphoma, and it may represent a transition of idiopathic HES into a T-cell lymphoma. Other possible sequences are discussed. The development of T-cell malignancy in idiopathic HES in a girl is quite an unusual presentation.

Eczema↗