• Journal of the Korean Society of Radiology
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• v.85 no.1
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• pp.109-123
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• 2024

Xanthogranulomatous (XG) inflammatory disease is a rare benign disease involving various organs, including the gallbladder, bile duct, pancreas, spleen, stomach, small bowel, colon, appendix, kidney, adrenal gland, urachus, urinary bladder, retroperitoneum, and female genital organs. The imaging features of XG inflammatory disease are nonspecific, usually presenting as a heterogeneous solid or cystic mass. The disease may also extend to adjacent structures. Due to its aggressive nature, it is occasionally misdiagnosed as a malignant neoplasm. Herein, we review the radiological features and clinical manifestations of XG inflammatory diseases in various organs of the abdomen and pelvis.

• Neonatal Medicine
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• v.17 no.2
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• pp.254-261
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• 2010

Little is known about neonatal mitochondrial disease, though mitochondrial metabolic disorders may often present in the neonatal period because of the high energy requirement of neonate. In newborn period, common presentations are not specific and the disease course may be rapid and fatal. In this study, we report three cases of neonatal mitochondrial disease. The first case was strongly suspected because of sudden seizure and mental change with severe lactic acidosis, and multiorgan failure. Plasma lactate/pyruvate (L/P) ratio was increased to 55.6 with marked lactic aciduria and increased plasma alanin up to 2,237 nmol/mL. In the second patient, a peritoneal dialysis was performed for acute adrenal and renal failure, but metabolic acidosis persisted. Plasma L/P ratio was increased to 23.9, and MRC I (mitochondrial respiratory chain defect) was diagnosed through the enzymatic analysis of the muscles. The third case showed repetitive episode of lactic acidosis during the first two months of life, hypotonia, failure to thrive and feeding difficulties. We found markedly increased cerebrospinal fluid L/P ratio up to 57 though plasma L/P ratio(19.4) was borderline with increased plasma lactate. The lactate peak was prominent in brain magnetic resonance spectroscopy (MRS). MRC II was confirmed through muscle biopsy. Plasma lactate level and lactate peak of brain MRS were normalized after conservative treatment.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.2
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• pp.35-42
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• 2018

Purpose: To follow up Korean patients with metabolic and endocrine disorders ascertained by Korea Genetics Research Center, and assess the long-term effectiveness of extended newborn screening program in Korea. Methods: From January 2000 to December 2017, tandem mass spectrometry and fluoroimmunoassay were employed in extended newborn screening (NBS). The NBS program obtained dried blood spots from 283,626 babies, 48 hours after birth, and screened for galactosemia, congenital hypothyroidism (CH), congenital adrenal hyperplasia (CAH), and 50 preventable inborn errors of amino acid, fatty acid, and organic acid metabolism. Results: 28 cases of amino acid disorders, 75 cases of organic acid disorders, 27 cases of fatty acid disorders, 51 cases of urea cycle disorders, 127 cases of CH, 14 cases of CAH, and 15 cases of galactosemia were ascertained through NBS and subsequent confirmatory laboratory tests. Patients with amino acid metabolic disorders, galactosemia, CH, or CAH were more likely to have a better long-term outcome if detected early. Early management of MSUD led to much better outcome in over 90%. Despite early intervention, 32% of other organic acidemia cases still resulted in developmental delay and neurological problems. Fatty acid disorders showed varied results; those with EMA and MCAD had a good outcome, but those with VLCAD had serious neurological problems and considerably higher mortality. 75% with UCD experienced serious neurological complications and higher mortality. Conclusion: The nation-wide NBS program must be accompanied by comprehensive long-term management and physician and family education of inborn errors of metabolism for a better outcome.

• Journal of Life Science
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• v.32 no.1
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• pp.70-77
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• 2022

Endocrine disrupting chemicals (EDCs) have been attracting significant attention in modern society, owing to the increased incidence rate of various diseases along with population growth. EDCs are found in many commercial products, including some plastic bottles and containers, detergents, liners of metal food cans, flame retardants, food, toys, cosmetics, and pesticides. EDCs have a hormonal effect on the human body, which disrupts the endocrine system, notably affecting sexual differentiation and normal reproduction, and can trigger cancer as well. Recently, the association between neurological diseases and EDCs has become a hot topic of research in the field of neuroscience. Considering that EDCs negatively affect not only neuronal proliferation and neurotransmission but also the formation of the neuronal networks, EDCs may induce neurodevelopmental disorders, such as autism spectrum disorders and attention-deficit/hyperactivity disorder as well as neurodegenerative diseases, including Parkinson's disease and Alzheimer's disease. In light of these potentially deleterious outcomes, important efforts have been underway to minimize the exposure to EDCs through appropriate regulations and policies around the world, but chemicals that have not yet been associated with endocrine disrupting properties are still in wide use. Therefore, more epidemiological investigations and research are needed to fully understand the effects of EDCs on the nervous system.

• Journal of Chest Surgery
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• v.37 no.3
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• pp.228-234
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• 2004

Primary cardiac tumors are rare disease and they present nonspecific symptom. They are divided in benign and malignant tumors and require surgical therapy and/or additional therapy. From March 1995 to March 2003, twenty one patients were diagnosed as having primary cardiac tumors. We analysed them retrospectively in terms of various perioperative factors and early and late results. 6 men and 15 women and their average age was 45.44$\pm$18.76. Pathology revealed eighteen benign (fourteen myxoma, two fibroelastoma, one hemangioma and one paraganglioma) disease and three malignant (one angiosarcoma, one mesothelioma and one myxofibrosarcoma) disease. There was one (myxoma) operative mortality and three late death (hemangioma, angiosarcoma and mesothelioma) during additional therapy and follow up. Surgical treatment of primary cardiac tumor is important and sometimes additional therapy is required but the prognosis of malignant cardiac tumor is still very poor.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.248-251
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• 2009

The urachus is a normal embryonic remnant of the primitive dome. It generally exists as a fibrous cord extending from the dome of the bladder to the umbilicus. Disorders of the urachus are developed as a result of its incomplete regression. The urachal cyst is the most common urachal anomaly, and is usually asymptomatic in infancy and childhood. However, when the cysts are large or accompanied with secondary infection, they may be detected in its early stage. A sonography or CT scan may be helpful to confirm the diagnosis of urachal cyst. The managements of infected urachal cyst are varied from simple drainage to radical excision. Here, we report an unusual case of urachal cyst infection that occurred during corticosteroids therapy in a girl with IgA nephropathy.

• Pediatric Infection and Vaccine
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• v.12 no.1
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• pp.95-99
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• 2005

Pneumocystis carinii pneumonia is an infectious disease which is highly prevalent in the group of immunosuppressed patients, particularly with hematologic tumors as lymphomas and acquired immune deficiency syndrome(AIDS), severe malnutrition, organ transplantations, high dose corticosteroid therapy. Some cases of Pneumocystis carinii pneumonia in infants with primary immune deficiency were already reported. The authors present a case of Pneumocystis carinii pneumonia developed in an infant who suffered from 10 days of poor feeding and failure to thrive and not included in the risk groups listed above. He had bilateral interstitial infiltrations on the chest radiography, diagnosed as Pneumocystis carinii pneumonia after Gomori-methenamine silver staining of his sputum that was taken through tracheal intubation. He improved after administering Trimethoprim-sulfamethoxazole for 14 days.

• Journal of the Korean Society of Radiology
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• v.8 no.1
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• pp.35-42
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• 2014

The purpose of this study is to know the differences of metabolism in abnormal brain disease using a single-voxel proton MR spectroscopy(1H MRS) Together with five normal volunteers and each five patients with brain diseases, pathologically proved, underwent MRI and 1H MRS. The quantitative results of 1H MRS in adrenoleukodystrophy(ALD), hepatic encephalopathy(HE), and infarction gave unique information on the metabolite changes related with the white matter: the concentration of NAA decreased in all diseases; Cho, mI and Lac increased in ALD; Cho decreased in HE; and ${\beta}{\cdot}{\gamma}$-Glx and Lac increased in infarction. It is concluded that 1H MRS is capable of diagnosing brain diseases by monitoring metabolite changes in vivo that subsequently develope into abnormalities. 1H MRS may be a useful clinical tool for in both diagnosis and prognosis of brain diseases.

• Korean Journal of Psychosomatic Medicine
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• v.9 no.1
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• pp.81-92
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• 2001

Stress has been linked to the pathophysiology and pathogenesis of various psychiatric illnesses. Over the past few years, our understanding of the brain and neuroendocrine systems that are linked to stress responses has increased enormously. This article reviews a series of animal and human studies to understand what are the central pathways by which stress is perceived, processed, and transduced into a neuroendocrine response. We focus on the limbic-hypothalamic-pituitary-adrenal(LHPA) axis and several neurotransmitter systems such as norepinephrine, CRF, serotonin, acetylcholine, and dopamine. LHPA stress circuit is a complex system with multiple control mechanisms which are altered in pathological states. CRF and related peptides in the central nervous system appear to enhance behavioral responses to stressors. Norepinephrine systems are also activated by stressors and cause the release of catecholamines from the autonomic nervous system. CRF-norepinephrine interaction makes a feed-forward system which may be important for an organism to mobilize not only the pituitary system but also the central nervous system, in response to environmental challenges. The interactions among several neurotransmitters and endocrine systems appear to play key roles in mediating various behavioral and psychological stress responses involving abnormal responses to stressors such as anxiety and affective disorders.

• Journal of Oral Medicine and Pain
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• v.35 no.4
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• pp.259-264
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• 2010

The majority of chronic gingival ulcerative lesions are known to be due to autoimmune disease such as oral lichen planus, benign mucous membrane pemphigoid or pemphigus vulgaris. Topical or systemic corticosteroids are mainly used and adjuvant drugs like immunosuppressant, anti-inflammatory drugs, antimalarials or antimetabolites can also be prescribed. Because systemic corticosteroids causes various side effects, such as gastrointestinal disturbance, osteoporosis, diabetes or adrenal suppression. So, topical steroid therapy is main treatment for chronic gingival ulcerative lesion confined to small area. However, there's also limitation of topical corticosteroids. The effect of the corticosteroids decreases due to salivary flow and the movement of the tongue, lips, or buccal mucosa. When the lesions are widely distributed or positioned deeply in oral cavity, it is hard to apply the medication on patients' own. Moreover, it can be applied to unaffected mucosa. Although occlusive steroid therapy using stent was reported to minimize taking steroid and overcome the faults of applying topical steroids, it has been used less frequently in the clinical field. Therefore, this report is going to find out the usefulness and the way to utilize clinically through the case which acted satisfactorily by performing topical steroid therapy using stent on chronic ulcerative gingival lesions.