• Neonatal Medicine
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• v.15 no.2
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• pp.200-206
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• 2008

Human chromosome 9 is characterized by a high degree of morphologic heteromorphisms, including variation in the size of the heterochromatin. We present a case of a de novo short arm addition of chromosome 9, [46, XY, add(9)(p13)], associated with multiple anomalies, including trigonocephaly, upward slant of the palpebral fissures, patent ductus arteriosus, pulmonary hypertension, hypertrophic cardiomyopathy, umbilical hernia, ambiguous genitalia, and sensorineural hearing and visual loss. This mutation affects the pericentric region of the heterochromatin. This patient exhibited a clinically important breakpoint of the heterochromatic region of chromosome 9 short arm and the associated anomalies.

• The Korean Journal of Nuclear Medicine
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• v.38 no.5
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• pp.331-337
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• 2004

Cardiac neurotransmission imaging allows in vivo assessment of presynaptic reuptake, neurotransmitter storage and postsynaptic receptors. Among the various neurotransmitter, I-123 MIBG is most available and relatively well-established. Metaiodobenzylguanidine (MIBG) is an analogue of the false neurotransmitter guanethidine. It is taken up to adrenergic neurons by uptake-1 mechanism as same as norepinephrine. As tagged with I-123, it can be used to image sympathetic function in various organs including heart with planar or SPECT techniques. I-123 MIBG imaging has a unique advantage to evaluate myocardial neuronal activity in which the heart has no significant structural abnormality or even no functional derangement measured with other conventional examination. In patients with cardiomyopathy and heart failure, this imaging has most sensitive technique to predict prognosis and treatment response of betablocker or ACE inhibitor. In diabetic patients, it allow very early detection of autonomic neuropathy. In patients with dangerous arrhythmia such as ventricular tachycardia or fibrillation, MIBG imaging may be only an abnormal result among various exams. In patients with ischemic heart disease, sympathetic derangement may be used as the method of risk stratification. In heart transplanted patients, sympathetic reinnervation is well evaluated. Adriamycin-induced cardiotoxicity is detected earlier than ventricular dysfunction with sympathetic dysfunction. Neurodegenerative disorder such as Parkinson's disease or dementia with Lewy bodies has also cardiac sympathetic dysfunction. Noninvasive assessment of cardiac sympathetic nerve activity with I-123 MIBG imaging nay be improve understanding of the pathophysiology of cardiac disease and make a contribution to predict survival and therapy efficacy.

• Plant Resources
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• v.4 no.2
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• pp.65-74
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• 2001

Coleus forskohlii Briq, of the family Lamiaceae yields a valuable secondary metabolity 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 rot disease. The fungal culture filterate (ECE) 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% ECF in the medium showed maximum inhibition and is there fore considered as the MIC level of Coleus forshohlii. 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.

• Archives of Reconstructive Microsurgery
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• v.23 no.2
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• pp.89-92
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• 2014

An 8-year-old girl diagnosed with dilated cardiomyopathy and Russell-Silver syndrome was admitted to our pediatric intensive care unit due to low cardiac output and multiple-organ dysfunction. The patient was placed on the heart transplant waiting list and extracorporeal membrane oxygenation was performed as a bridge to transplantation. After 17 days, heart transplantation was performed. The donor was a 46-year-old female (weight, 50 kg; height, 150 cm). The donor:recipient weight ratio was 3.37:1. Because the dimension and volume of the recipient's thoracic cage were insufficient, the sternum could not be closed. Nine days after transplantation, the patient underwent delayed sternal closure. To obtain adequate space, we left the sternum 4.5 cm apart from each margin using four transverse titanium plates. A transverse rectus abdominis musculocutaneous flap was chosen to cover the wound. Due to the shortage of donors, a size-mismatched pediatric heart transplantation is sometimes unavoidable. Closure of the opened sternum of a transplant recipient can be challenging. Sternal reconstruction after an extremely oversized heart transplantation with transverse titanium plate fixation and a musculocutaneous flap can effectively achieve sternal closure and stability.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.23 no.1
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• pp.31-35
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• 2012

Noonan syndrome is characterized by short stature, typical facial dysmorphology, and congenital heart defects. The main facial features of Noonan syndrome are hypertelorism with down-slanting palpebral fissures, ptosis, and low-set posteriorly-rotated ears with a thickened helix. The cardiovascular defects most commonly associated with this condition are pulmonary stenosis and hypertrophic cardiomyopathy. Other associated features are webbed neck, chest deformity, mild intellectual deficit, cryptorchidism, poor feeding in infancy, bleeding tendency, and lymphatic dysplasias. The patient is a 10-year-old boy. He had experienced repeated febrile convulsions. He had typical facial features, a short stature, chest deformity, cryptorchidism, vesicoureteral reflux, and mental retardation. His language and motor development were delayed. When he went to school, it was difficult for him to pay attention, follow directions, and organize tasks. He also displayed behavior such as squirming, leaving his seat in class, and running around inappropriately. Clinical observation is important for the diagnosis, so we report a patient who was diagnosed with Noonan syndrome, mental retardation, and attention-deficit hyperactivity disorder.

• BMB Reports
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• v.39 no.6
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• pp.759-765
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• 2006

In this study, we investigate Nitric oxide synthase (NOS) expressions in adriamycin (ADR)-induced cadiomyopathy in rats. Sixty male Wistar rats were randomly divided into two main groups: control and ADR groups. Myocardial histopathological observation was performed; Expressions of 3 isoforms of NOS genes were examined by RT-PCR analysis; Expressions of 3 isoforms of NOS protein was assessed by Western blot analysis. Myocardium exhibited intensive morphological changes after 8 weeks of ADR treatment. The expression levels of inducible NOS (iNOS) gene and protein were significantly increased in ADR-treated rats after 8 weeks of treatment and then slightly increased at weeks 9 and 10. No significantly difference of neuronal NOS (nNOS) or endothelial NOS (eNOS) gene and protein were observed in the myocardium obtained from the control rats and ADR-injected rats at any time point. iNOS gene expression is selectively induced by ADR in heart. The upregulation of iNOS gene and protein may be somehow correlated with morphological changes seen in heart of rat treated with ADR.

• Reproductive and Developmental Biology
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• v.32 no.1
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• pp.21-25
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• 2008

Bone marrow mesenchymal stem cells (BMMSCs) have the capacity for self-renewal and differentiation into a variety of cell types. They represent an attractive source of cells for gene and cell therapy. The purpose of this study is to direct the specific expression of the DsRed reporter gene in $Sca-1^+$ BMMSCs differentiated into a cardiomyogenic lineage. We constructed the prMLC-2v-DsRed vector expressing DsRed under the control of the 309 tp fragment of the rat MLC-2v 5'-flanking region. The specific expression of the DsRed reporter gene under the transcriptional control of the 309 bp fragment of the rat MLC-2v promoter was tested in 5-azacytidine healed-$Sca-1^+$ BMMSCs over 2 weeks after the prMLC-2v-DsRed transfection. The prMLC-2v-DsRed was specifically expressed in the $Sca-1^+$ BMMSCs with cardiomyogenic lineage differentiation and it demonstrates that the 309 bp sequences of the rat MLC-2v 5'-flanking region is sufficient to confer cardiac specific expression on a DsRed reporter gene. The cardiac-specific promoter-driven reporter vector provides an important tool for the study of stem cell differentiation and cell replacement therapy in ischemic cardiomyopathy.

• Pediatric Infection and Vaccine
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• v.2 no.2
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• pp.200-206
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• 1995

Myocarditis refers to inflammation, necrosis, or myocytolysis that may be due to many infectious, connective tissue and many other causes affecting the myocardium or involvement of the endocardium or pericardium. The most common manifestation is congestive heart failure, although arrhythmias and sudden death may be the first sign of myocarditis. Viral myocarditis is typically a sporadic but occasionally epidemic illness, noted as an acute potentially fulminant disease of 1-to 4-wk-old infants, as an acute but more benign myopericarditis of toddlers and young children. The most common casuative agent in viral myocarditis is Coxsackievirus and the outcome of the biopsy-proven chronic dilated cardiomyopathy associated with Coxsackievirus is poor without therapy. Myocarditis may be confirmed by percutaneous endomyocardial biopsy and the viral myocarditis may be diagnosed by the serological viral study with the clinical manifestations. He was admitted for the management of tachyarrhythmias occurred suddenly without prodromal symptoms and signs and diagnosed as viral pancarditis by serological Coxsackievirus study, echocardiogram, chest x-ray, EKG and other clinical manifestations.

• Journal of Chest Surgery
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• v.45 no.2
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• pp.116-119
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• 2012

A 61-year-old female patient was diagnosed with dilated cardiomyopathy with severe left ventricle dysfunction. Two days after admission, continuous renal replacement therapy was performed due to oliguria and lactic acidosis. On the fifth day, an intra-aortic balloon pump was inserted due to low cardiac output syndrome. Beginning 4 days after admission, she was supported for 15 days thereafter with an extracorporeal left ventricular assist device (LVAD) because of heart failure with multi-organ failure. A heart transplant was performed while the patient was stabilized with the LVAD. She developed several complications after the surgery, such as cytomegalovirus pneumonia, pulmonary tuberculosis, wound dehiscence, and H1N1 infection. On postoperative day 19, she was discharged from the hospital with close follow-up and treatment for infection. She received follow-up care for 10 months without any immune rejection reaction.

• Journal of Chest Surgery
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• v.16 no.2
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• pp.175-183
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• 1983

Ventricular aneurysm which was first described by John Hunter on 18th century, has been experienced by many surgeons after successful using of cardiopulmonary bypass by Cooley on 1958. According to Gorlin, the definition of ventricular aneyrysm is portion of the ventricle which is not motile at systole (akinesis) or which has paradoxical dilatation at systole(dyskinesis). The ventricular aneurysm is classified to anatomical and functional. The anatomical ventricular aneurysm is devided into true or false again. Average age incidence is ranged from 49 to 60 and male predominance is reported. The cause is ischemic coronary artery disease in almost cases but hypertropoc cardiomyopathy, congenital abscence of myocardium, complication after mitral valvular replacement and trauma may also cause the ventricular aneurysm. Angina pectoris and congestive heart failure are most common clinical manifestations Ventricular tachycardia and systemic embolization are also complained. Using cardiopulmonary bypass, aneurysmectomy alone or combination with coronary artery revasculization are currently done for surgical treatment with steady improvenment of mortality. The first patient was 33 years old man who had true type of ventricular aneurysm on inferior wall the left ventricle near apex with protruded huge organized thrombus. The thromboembolic phenomenon was noted on both lower extremities. Under cardiopulmonary bypass, aneurysmectomy and thrombectomy were done. The aneurysmal orifice was repaired with Teflon buttless suture. The second patient was 30 years old female who had large true type of ventricular aneurysm on inferior wall of the left ventricle. Under cardiopulmonary bypass, aneurysmectomy with repair of aneurysmmal orifice defect by means of double layered Dacron patch was done with reinforce by outer silastic sheet covering. She was discharged from hospoital at post op. 15th day uneventfully.