Enlarged coronary-bronchial artery anastomosis in cystic bronchiectasis: A case report / 대한내과학회지

Anastomoses between a coronary artery and bronchial or mediastinal arteries have been described since the 19th century. Although coronary-to-bronchial artery communication has been well described, it remains an unusual finding. We report one adult case of abnormally enlarged two coronary-to-bronchial artery communications in a severe cystic bronchiectasis patient. In this case, the bronchial arteries were also supplied from the multiple systemic arteries (the internal mammary, subclavian, inferior phrenic and intercostal arteries) because of severe cystic bronchiectasis. We treated the patient by embolization of the bronchial artery with the abnormal anastomoses and then by surgical resection of the cystic bronchiectatic lung lesion. To date, the patient remains free of symptoms.

Cardiac Dysrhythmias, Cardiomyopathy and Muscular Dystrophy in Patients with Emery-Dreifuss Muscular Dystrophy and Limb-Girdle Muscular Dystrophy Type 1B

Emery-Dreifuss muscular dystrophy (EDMD) and limb-girdle muscular dystrophy type 1B (LGMD1B) are characterized by cardiac dysrhythmias, late-onset cardiomyopathy, slowly progressive skeletal myopathy and contractures of the neck, elbows and ankles. The causative mutation is either in the emerin gene (X-linked recessive EDMD) or lamin A/C gene (autosomal dominant EDMD2 or LGMD1B). We report three cases of EDMD, EDMD2 and LGMD1B. A 14-yr-old boy showed limitation of cervical flexion and contractures of both elbows and ankles. Sinus arrest with junctional escape beats was noted. He was diagnosed as X-linked recessive EDMD (MIM 310300). A 28-yr-old female showed severe wasting and weakness of humeroperoneal muscles. Marked limitation of cervical flexion and contractures of both elbows and ankles were noted. Varying degrees of AV block were noted. She was diagnosed as autosomal dominant EDMD2 (MIM 181350). A 41-yr-old female had contractures of both ankles and limb-girdle type muscular dystrophy. ECG revealed atrial tachycardia with high grade AV block. She was diagnosed as autosomal dominant LGMD1B (MIM 159001). Cardiac dysrhythmias in EDMD and LGMD1B include AV block, bradycardia, atrial tachycardia, atrial fibrillation, and atrial standstill, causing sudden death necessitating pacemaker implantation. Cardiologists should know about these unusual genetic diseases with conduction defects, especially in young adults.

A Case of Emery-Dreifuss Muscular Dystrophy by Emerin Gene Mutation

Emery-Dreifuss muscular dystrophy (EDMD) is a degenerative myopathy characterized by mild, slowly progressing weakness, muscle atrophy, and early contracture of the neck, ankle and elbow. Heart involvement becomes apparent during the teenage years and is characterized by cardiac conduction defects and the infiltration of the myocardium by fibrous and adipose tissues. Heart block can eventually lead to sudden death, and therefore, early treatment with a cardiac pacemaker may improve symptoms and be lifesaving in patients with heart block. We describe our experience of pacemaker implantation in a 14-year old boy with X-linked recessive EDMD and emerin gene mutation. His electrocardiogram findings showed junctional escape beats, and his clinical features, i.e., ECG, nerve conduction test, electromyography and muscle biopsy findings were compatible with EDMD. He was implanted with a VVI type permanent pacemaker following an electrophysiologic study.

Clinical Aspects of Coronary Artery Perforation during Percutaneous Coronary Intervention

BACKGROUND AND OBJECTIVES: Coronary artery perforation is a rare, but potentially deadly, complication of percutaneous coronary intervention (PCI). The purpose of this study was to analyze the clinical characteristics, and outcome, of coronary artery perforation. SUBJECTS AND METHODS: We retrospectively reviewed 3,782 consecutive PCIs, performed between January, 1994 and May, 2002 at the Samsung Medical Center, from the database records. The medical records and angiograms of the patients were also reviewed. The coronary artery perforations were classified according to Ellis' classification. RESULTS: A coronary artery perforation was noted in 24 patients (0.6%). It was most commonly observed during PCI of the right coronary artery (46%) and a chronic total occlusion intervention (42%). The number of the patients with Ellis'classes I, II and III were 11, 8 and 5, respectively. The most frequent causes of the perforation were guidewire, followed by balloon (11 and 8 cases, respectively). The interventional modality with the highest risk of perforation in this study was rotational atherectomy, (4 out of 157, 2.6%). Five patients had cardiac tamponade, of which four occurred during a rotablator procedure. Pericardiocentesis was performed in 5 patients, while 3 patients with class III perforations received emergent coronary artery bypass surgery. There were no in-hospital mortalities, although the duration of the hospital stay for the class III patients was longer than those with classes I or II perforations. CONCLUSION: A coronary artery perforation during percutaneous coronary intervention is a potentially serious complication. However, the immediate and adequate management results in a fairly good prognosis.

A case of chemical pneumonitis induced by hydrocarbon ingestion / 대한내과학회지

Chemical pneumonitis induced by hydrocarbon ingestion is rare in Korea. Cresol and xylenol, commonly used hydrocarbon disinfectants, can cause chemical burn on skin, gastrointestinal corrosive injury, central nervous system disturbance, and multiorgan failure including acute respiratory failure and chemical pneumonitis following intoxication1, 2, 8). We will report a case of chemical pneumonitis induced by ingestion of a hydrocarbon disinfectant as suicidal attempt.A 39-years-old female was found unconscious after ingestion of 100-200mL of solution containing cresol (5.5 g/100 mL), xylenol (7.5 g/100 mL), and benzene (37.5 g/100 mL).Upon arriving at emergency room the patient was in coma, had undectable blood pressure (0/0) and had no self respiration. Cardiopulmonary resuscitation were given immediately, resulting in reversed her consciousness and elevated blood pressure.The patient had dermal burn on face, erosion of oral and gastric mucosa, impairment of liver function, leukocytosis, metabolic acidosis with hypoxemia, chemical pneumonitis, and spontaneous pneumothorax. The patient survived after artificial ventilation, intensive general supportive treatment and wound care. She was discharged in relatively good clinical condition with minimal sequele.

A Case of Renal Oncocytomatosis with Chronic Renal Failure / 대한신장학회잡지

Renal oncocytomas account for approximately 5% of renal parenchymal tumors. Usually it has unilateral solitary oncocytic nodule, but bilateral multifocal renal oncocytomasis is rare. The term renal oncocytoma should be used to characterize a well-differentiated renal epithelial tumor with eosinophilic granular cytoplasm that has benign behavior and favourable progress. Also, multiple oncocytoma distributed diffusely in both kidneys is termed renal oncocytomatosis Because of the benign nature, multicentricity, possible bilaterality and absence of pathognomonic radiographic features, renal oncocytomas should be considered in differential diagnosis of solid masses, especially renal cell carcinoma. We report a patient with bilaleral multifocal renal oncocytomatosis, who had progressive renal failure. Renal oncocytomatosis was diagnosed pathologically after bilateral nephrectomy.

A Case of High Degree AV Block Treated by Implantation of Permanent Pacemaker in Emery-Dreifuss Muscular Dystrophy

Emery-Dreifuss muscular dystrophy is characterized by 1) early contractures of the elbows, Achilles tendons, and postcervical muscles, 2) slowly progressive muscle wasting and weakness with a humeroperoneal distribution in the early stages, and 3) cardiomyopathy with conduction defects and risk of sudden death. The inheritance is usually X-linked recessive but can be autosomal dominant and recessive. We report a case of 28-year old woman who presented with dizziness, palpitation, and progressive muscular weakness. Her ECG revealed high degree AV block and muscle biopsy demonstrated diffuse degenerative change consistent with Emery-Dreifuss muscular dystrophy. She was diagnosed as autosomal dominant Emery-Dreifuss muscular dystrophy by characteristic clinical features, and findings of ECG, nerve conduction test, electromyography and muscle biopsy findings. A VVI-type permanent pacemaker was implanted.