• The Korean Journal of Nuclear Medicine
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• v.24 no.2
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• pp.332-336
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• 1990

Pheochromocytoma occurs at all ages but is most common in young to midadult life. In adults 10 percent of pheochronocytoma are bilateral. And bilateral lesions are common in the familial syndromes; within Multiple Endocrine Neoplasia (MEN) kindreds over half with pheochromocytomas have bilateral lesions. We report a case of bilateral pheochromocytoma firstly detected by ultrasonogram. This 24-year-old male was normotensive and had symptoms of dilated cardiomyopathy and elevated VMA level. CT and MRI were performed to evaluate the tissue nature and associated abnormalities. $^{131}I-MIBG$ scan seems to be the imaging of choice in patients with suspected multicentric/bilateral or recurrent or metastatic pheochromocytomas.

• Journal of Chest Surgery
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• v.53 no.2
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• pp.79-81
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• 2020

Treatment options for children with end-stage heart failure are limited. We report the first case of a successful pediatric heart transplantation bridged with a durable left ventricular assist device in Korea. A 10-month-old female infant with dilated cardiomyopathy and left ventricular non-compaction was listed for heart transplantation. During the waiting period, the patient's status deteriorated. Therefore, we decided to provide support with a durable left ventricular assist device as a bridge to transplantation. The patient was successfully bridged to heart transplantation with effective support and without any major adverse events.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.5 no.1
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• pp.18-22
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• 2005

Pompe disease is a genetic disorder caused by a deficiency of acid ${\alpha}$-glucosidase (GAA). This enzyme defect results in lysosomal glycogen accumulation in multiple tissues and cell types, with cardiac, skeletal, and smooth muscle cells the most seriously affected. Infantile-onset Pompe disease is uniformly lethal. Affected infants present in the first few months of life with hypotonia, generalized muscle weakness, and a hypertrophic cardiomyopathy, followed by death from cardiorespiratory failure or respiratory infection, usually by 1 year of age. Late-onset forms is characterized by a lack of severe cardiac involvement and a less severe short-term prognosis. Enzyme replacement therapy for Pompe disease is intended to address directly the underlying metabolic defect via intravenous infusions of recombinant human GAA to provide the missing enzyme. We experienced one case of Pompe disease in 3-years old boy that has improved his exercise ability and cardiac function after GAA enzyme replacement therapy.

• Proceedings of the Plant Resources Society of Korea Conference
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• 2000.10a
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• pp.96-113
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• 2000

Coleus forskohlii Briq. of the family Lamiaceae yields a valuable secondary metabolite known as forskolin which is a labdane diterpenoid. Coleus forskohlii is the only known source of this compound. Forskolin is used in medicine for the treatment of glaucoma, congestive cardiomyopathy and asthma. Morphogenic callus was induced from young leaves on MS medium augmented with NAA and BA. These calli, when subcultured on MS with KN alone gave rise to shoots. The regenerated shoot developed good root system on MS medium fortified with NAA. The fully grown plantlets were transferred to soil for acclimatization. Coleus plant is mainly infected by a fungi Lasiodiplodia theobromae which causes root of disease. The fungal culture filterate (FCF) of Lasiodiplodia theobromae, has been used in regeneration media to find the MIC and further to select resistant plants to the pathogen. In the present study 40% FCF in the medium showed maximum inhibition and is there fore considered as the MIC level of Coleus forskohlii. This data could prove to be useful for the future for selecting a resistant C,forskohlii plant against the root disease caused by L.theobromae.

• Journal of Microbiology and Biotechnology
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• v.16 no.4
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• pp.550-555
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• 2006

CVB3 is a virulent human pathogen that induces myocarditis and ultimately dilated cardiomyopathy. Although several apoptotic factors are involved in the cell death induced by CVB3, the upstream signal transduction factors of CVB3-induced apoptosis are still unclear. We explored and characterized the role of PKC $\delta$ in CVB3-infected cells. PKC $\delta$ was cleaved after CVB3 infection and was activated at 6 h postinfection. PKC $\delta$ was also translocated into the nucleus via mitochondria after CVB3 infection, and overexpression of wild-type PKC $\delta$ reduced the apoptotic cell death caused by CVB3. These results indicate that PKC $\delta$ has an antiapoptotic role in CVB3 infection.

• Journal of Chest Surgery
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• v.44 no.1
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• pp.61-63
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• 2011

Implantable cardioverter defibrillator (ICD) can be a crucial therapeutic modality for pediatric patients with congenital heart disease, Brugada syndrome, long QT syndrome and cardiomyopathy. Because transvenous implantation of ICD is mostly unfeasible for pediatric patients due to anatomical and technical limitations, epicardial patch type or subcutaneous type ICD have been used. Implantation of these alternative ICDs, however, was reported to be frequently associated with significant complications. We report a case of successful intrapericardial implantation of a single coil-type ICD through the transverse sinus in a 27 month-old child weighing lesser than 10 kg, and it was inferred from this experience that this alternative technique may decrease complications and morbidities after ICD implantation in children.

• Journal of Veterinary Clinics
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• v.37 no.4
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• pp.227-230
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• 2020

A 8-year-old spayed female Korean short-haired cat was presented with respiratory distress. CBC, serum chemistry analysis, plain radiography, and abdominal ultrasonography were performed. Besides hypertrophic cardiomyopathy (HCM) suspected by the thoracic radiograph, a tubular, tortuous soft tissue structure was detected at the region of the left retroperitoneal cavity on the abdominal radiograph. On the abdominal ultrasonography, a shunt vessel is identified caudo-lateral to the left kidney region. These findings are consistent with spleno-systemic shunts in cats. Furthermore, portal hypertension and diffuse hepatic lesion were also identified. Although the cause of a shunt vessel is not easy to diagnose, it is important to include spleno-systemic shunt into differential diagnosis list, when convoluted, tubular soft tissue opacity is seen on the digital radiography (DR). This report will allow clinicians to raise awareness of complications of portosystemic shunt (PSS) and better treat PSS suspected feline patients when the advanced modalities such as computed tomography and magnetic resonance imaging are not available.

• The Journal of Internal Korean Medicine
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• v.24 no.2
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• pp.387-394
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• 2003

Amiodarone is an effective antiarrhythmic agent because of its vasodilator actions. Nowadays it is mostly used to treat patients with severe cardiomyopathy or coronary artery disease complicated by disturbances in the supraventricular or ventricular rhythm. But, some doctors are reluctant to prescribe it because of its many side effects. These include impairment of liver and thyroid fuction and, rarely, damage to the lungs. Most of all, its most serious side effect is amiodarone-induced pulmonary toxicity, which can occur in up to 10% of patients, with mortality rates as high as 50%. We recently experienced one case of the patient with the Amiodarone-induced pulmonary toxicity. The clinical manifestations of the patient was cough, painful breathing, fever, presence of rales, decreased breath sounds, and sputum. We report the change of the patient's symptoms through both western medical treatment and oriental medical treatment.

• Annals of Pediatric Endocrinology and Metabolism
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• v.23 no.4
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• pp.176-181
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• 2018

Noonan syndrome (NS) is an autosomal dominant disorder that involves multiple organ systems, with short stature as the most common presentation (>70%). Possible mechanisms of short stature in NS include growth hormone (GH) deficiency, neurosecretory dysfunction, and GH resistance. Accordingly, GH therapy has been carried out for NS patients over the last three decades, and multiple studies have reported acceleration of growth velocity (GV) and increase of height standard deviation score (SDS) in both prepubertal and pubertal NS patients upon GH therapy. One year of GH therapy resulted in almost doubling of GV compared with baseline; afterwards, the increase in GV gradually decreased in the following years, showing that the effect of GH therapy wanes over time. After four years of GH therapy, ~70% of NS patients reached normal height considering their age and sex. Early initiation, long duration of GH therapy, and higher height SDS at the onset of puberty were associated with improved final height, whereas gender, dosage of GH, and the clinical severity did not show significant association with final height. Studies have reported no significant adverse events of GH therapy regarding progression of hypertrophic cardiomyopathy, alteration of metabolism, and tumor development. Therefore, GH therapy is effective for improving height and GV of NS patients; nevertheless, concerns on possible malignancy remains, which necessitates continuous monitoring of NS patients receiving GH therapy.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.25 no.4
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• pp.321-331
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• 2022

Purpose: This study aimed to assess the quality of life (QoL) of children with glycogen storage disease (GSD) and their parents and to determine the impact of myopathies. Methods: A prospective case-control study was conducted at the Cairo University Children's Hospital and National Liver Institute, Menoufia University. A promising new style of questionnaire called the Stark Quality of Life Questionnaire was used to assess the quality of life. Results: Fifty-two children diagnosed with GSD (cases) and 55 age- and sex-matched healthy children (controls) were included. A statistically significant difference was found between cases and controls regarding food intake; mental behavior parameters such as mood, energy, and social contact; and physical behavior parameters such as running and tying shoelaces. Children with myopathies had significantly lower QoL scores in most of the parameters. Conclusion: GSDs alter children and their parents' mental and physical abilities. Lower QoL scores were detected in children with both skeletal myopathy and cardiomyopathy, but the difference was not statistically significant when compared with the children without myopathies.