• Journal of the korean academy of Pediatric Dentistry
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• v.29 no.2
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• pp.237-242
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• 2002

Lowe syndrome, also known as oculocerebrorenal syndrome, is a rare genetic disorder involving eyes, kidney and nervous system, and occurs predominantly in mostly males. The patients with Lowe syndrome are characterized with prominent forehead, thin and sparse hair, protruding ears, congenital cataracts, glaucoma, mental retardation, stunted growth, hypotonia, decrease in muscle mass and tendon reflexes, renal tubular dysfunction, and metabolic bone disease. A 6-year-old boy with Lowe syndrome was admitted to our clinic, with multiple caries and a chief complaint of intermittent pain on the left mandibular molar area. Because of difficulty in management of behavior and his medical problem, general anesthesia was performed for dental care. No specific complication was noticed during dental treatment procedure under general anesthesia and also during periodic recall-checks. General anesthesia itself, however, could be a potentially life-threatening procedure due to patient's biomedical problems. When a dental procedure under general anesthesia is to be required in patient with Lowe syndrome, it may be advisable being teamed with physicians, and general anesthesia duration should be as short as possible.

• Asian-Australasian Journal of Animal Sciences
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• v.31 no.8
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• pp.1073-1077
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• 2018

Nitrite plays a major role in inhibiting the growth of foodborne pathogens, including Clostridium botulinum (C. botulinum) that causes botulism, a life-threatening disease. Nitrite serves as a color-fixing agent in processed meat products. However, N-nitroso compounds can be produced from nitrite, which are considered as carcinogens. Thus, consumers desire processed meat products that contain lower concentrations (below conventional concentrations of products) of nitrite or no nitrite at all, although the portion of nitrite intake by processed meat consumption in total nitrite intake is very low. However, lower nitrite levels might expose consumers to risk of botulism poisoning due to C. botulinum or illness caused by other foodborne pathogens. Hence, lower nitrite concentrations in combination with other factors such as low pH, high sodium chloride level, and others have been recommended to decrease the risk of food poisoning. In addition, natural compounds that can inhibit bacterial growth and function as color-fixing agents have been developed to replace nitrite in processed meat products. However, their antibotulinal effects have not been fully clarified. Therefore, to have processed meat products with lower nitrite concentrations, low pH, high sodium chloride concentration, and others should also be applied together. Before using natural compounds as replacement of nitrite, their antibotulinal activities should be examined.

• Korean Journal of Bronchoesophagology
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• v.5 no.1
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• pp.78-84
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• 1999

Broncholithiasis is defined as a condition in which calcified material is present within the bronchial lumen. It is a rare but troublesome disease that can cause life-threatening complications such as massive fatal hemoptysis. Therefore, pulmonary resection is frequently required to remove the broncholiths and irreversibly damaged parenchyma. We experienced 3 cases of broncholithiasis. In one case, a 36 year old female patient suffered from coughing, massive hemoptysis with lithoptysis caused by intrinsic obstructive broncholiths in the right middle and lower lobe. In the 2nd case, a 41 year old male patient complained of long-standing blood tinged sputum and frequent pneumonic symptoms for 10 months because of extrinsic broncholithiasis where the calcified peribronchial lymph node eroded into the bronchial lumen of the right lower lobe. The remaining case involved a 30 year old female patient who complained of intermittent blood-tinged sputum induced by intrabronchial broncholith in the orifice of the right middle lobe bronchus. Two patients underwent bilobectomy(right middle and lower lobe) for removal of the broncholiths, damaged bronchi and parenchyma. The other patient was treated with right middle lobectomy and stone removal by bronchotomy of bronchus intermedius. In all patients, the post-operative course was uneventful.

• Tuberculosis and Respiratory Diseases
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• v.62 no.2
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• pp.134-139
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• 2007

Gefitinib is a novel drug used to treat advanced non-small cell lung cancer. However, drug-related interstitial pneumonia is a major life-threatening side effect, which has a worldwide prevalence of 0.3-0.4%. In Japan, the prevalence is high as 3-4% but the actual frequency in Korea has not been officially assessed. We report two cases of gefitinib-induced interstitial lung disease during the treatment of non-small cell lung cancer. High-resolution computerized tomography (HRCT) of one case showed nonspecific ground glass opacity and the chest x-ray of another case showed diffuse bilateral ground glass opacity. The former patient showed a rapid good response to corticosteroid treatment whereas the latter died despite receiving aggressive treatment with high dose corticosteroid and empirical antibiotics.

• Archives of Plastic Surgery
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• v.38 no.4
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• pp.508-511
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• 2011

Purpose: Blue toe syndrome consists of blue or purplish toes in the absence of a history of obvious trauma, serious cold exposure, or disorders producing generalized cyanosis. It is a life-threatening and still underrecognized disease. It can be commonly occurred by vascular surgery, invasive cutaneous procedures or anticoagulant therapy. Our case is presented of blue toe syndrome related to atheromatous embolization that was presumably triggered by angio CT. Methods: A 69-year-old man presented with the suddenly developed pain, cyanosis and livedo reticularis of the toes in right foot. Dorsalis pedis pulses were palpable. He had been performed a diagnostic angio CT 1 month earlier. Angio CT revealed diffuse aortic atheromatous plaque in lower abdominal aorta and both common iliac artery. One month after angio CT, he visited our clinic. There was no visible distal first dorsal metatarsal artery and digital artery of right first toe in lower extremity arteriography. A diagnosis was established of blue toe syndrome. Because his symptom was aggravated, we performed the exploration of the right foot. After exposure of first dorsal metatarsal artery, microsurgical atheroembolectomy was done. Results: There were no postoperative complications. After three months the patient had no clinically demonstrable problems. Conclusion: Patient with blue toe syndrome is at high risk of limb loss and mortality despite treatment. Blue toe syndrome produces painful, cyanosed toes with preserved pedal pulses. It needs to be aware of blue toe syndrome. Careful history should reveal the diagnosis. Treatment is controversial, however, most believe that anticoagulation therapy should be avoided.

• Journal of Chest Surgery
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• v.33 no.2
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• pp.132-138
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• 2000

Background: Inhaled nitric oxide therapy causes selective pulmonary vasodilation in congenital heart diseases with pulmonary hypertension. However discontinuation of inhaled nitric oxide therapy may be complicated by abrupt life-threatening rebound pulmonary hypertension(RPH) The purpose of this study was to prevent by comparing group I(without RPH n=13) and group II(with RPH n=6) to determine the risk factors involved inthe development of the RPH. Material and Method: Between Januarty 6, 1998 and April 14, 1999. we studied 19 consecutive children who were treated with inhaled nitric oxide for clinically significant pulmonary hypertension after an open heart surgery for congenital heart disease. the ratio of males and females was 12:7 ranging in age from 10 days to 6040 days(16 years) To identify the effects of nitric oxide between two groups we measured heart rate mean and systolic pulmonary arterial pressure mean and systolic systemic arterial pressure central venous pressure pH paO2/FiO2 and O2 saturation before and after the initiation and just before the withdrawal of the inhaled nitric oxide. result: In 6 of 19 patients(32%) withdrawal of inhaled nitric oxide caused RPH. In the two groups inhaled nitrix oxide decreased in pulmonary arterial pressure(PAP) without decreasing the systemic arterial pressure(SAP) and increased PaO2/FiO2 Compared with patients who had no RPH(group I) patients who had RPH(group II) were older in age (1204$\pm$1688 versus 546$\pm$1654 days p<0.05) received less nitric oxide therapy(34$\pm$18 versus 67$\pm$46 hours p<0.05) has shorter weaning process(5$\pm$3 versus 15一13 hours p<0.05) and received lowerconcentration of initial nitric oxide supply(11$\pm$8 versus 17$\pm$8 ppm p>0.05) and lower concentration just before the withdrawal nitric oxide(4.2$\pm$2.6 versus 5.6$\pm$2.6 ppm, p>0.05) Conclusion : We speculate that older age shorter of nitric oxide therapy shorter weaning process are the risk factors of RPH.

• Journal of Yeungnam Medical Science
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• v.31 no.2
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• pp.75-81
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• 2014

Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease. Despite of proper treatment and improving treatment regimens, HLH patients still show a fatal prognosis. Therefore the evaluation of prognostic factor is important and there are many studies about hyperbilirubinemia as a prognostic factor in HLH. So we studied the prognostic value of hyperbilirubinemia in HLH children. Methods: A retrospective analysis was performed about 33 patients who were diagnosed with HLH at Pusan National University Hospital and Yangsan Pusan University Hospital between January 2000 to December 2012. We reviewed the clinical characteristics, laboratory findings, and results of treatment to identify hyper-bilirubinemia as a prognostic factor in HLH patients. Results: The median age of patients at diagnosis was 32 months. Most of patients presented with fever, pale appearance, abdominal pain and jaundice. Forty-eight point five percentage of patients showed normal serum bilirubiln level (<2.0 mg/dL) and 51.5% showed hyperbilirubinemia (${\geq}2.0mg/dL$). In normal serum bilirubin group, 1 patient (6.3%) was relapsed and 1 patient (5.9%) was relapsed in hyperbilirubinemia group. In the hyperbilirubinemia group, the mortality was higher than the normal bilirubin group but, there was no statistical significance. Conclusion: As a prognostic factor serum bilirubin at diagnosis in HLH patients, there was no significant correlation between hyperbilirubinemia and poor outcome. But, our study has a limitation that the number of patients is too small and almost showed good prognosis.

• Korean Journal of Bronchoesophagology
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• v.4 no.1
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• pp.52-58
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• 1998

BACKGROUND： Aspiration is defined as the laryngeal penetration of secretions below the level of the true vocal cords. Aspiration can result in life-threatening complications, such as bronchospasm, airway obstruction, pneumonia, pulmonary abscess, sepsis, and death. The patient with high vagal palsy had significant aspiration and dysphagia OBJECTIVE： To formulate a step-by-step management paradign for the patients with high vagal palsy MATERIALS AND METHODS : The medical records of 23 patients who were diagnosed as high vagal palsy from September, 1995 to April, 1998 in Seoul National University Hospital were reviewed retrospectively. Eleven patients were managed conservatively and 12 patients were operated to treat chronic aspiration. RESULTS : The main etiologies of high vagal palsy were mass lesions of the skull base such as neurogenic tumor, pseudotumor, meningioma or nasopharyngeal carcinoma. Aspiration and dysphagia improved in 7 out of 11 patients who were managed conservatively after 2.2 months on the average. The patients who were refractory to the conservative management underwent surgery and showed improvement in 10 out of 12 patients. The employed surgical modalities were vocal cord medialization combined with cricopharyngeal myotomy in 7 patients, laryngotracheal separation in 3 patients and arytenoid adduction only in 2 patients. Two patients still had gastrostomy tube due to the persistent symptoms. Two patients had improved after surgery, but died of underlying disease. CONCLUSION : The patients with high vagal palsy are recommended to be managed conservatively for the first 2 months. If aspiration and dysphagia are persisting after conservative management, vocal cord medialization combined with or without cricopharyngeal myotomy should be considered. If failed, laryngotracheal separation or gastrostomy will be the next option based on the control of the oropharyngeal secretion.

• Clinical and Experimental Pediatrics
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• v.64 no.11
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• pp.559-572
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• 2021

The International Society for the Study of Vascular Anomalies classifies vascular anomalies into vascular tumors and vascular malformations. Vascular tumors are neoplasms of endothelial cells, among which infantile hemangiomas (IHs) are the most common, occurring in 5%-10% of infants. Glucose transporter-1 protein expression in IHs differs from that of other vascular tumors or vascular malformations. IHs are not present at birth but are usually diagnosed at 1 week to 1 month of age, rapidly proliferate between 1 and 3 months of age, mostly complete proliferation by 5 months of age, and then slowly involute to the adipose or fibrous tissue. Approximately 10% of IH cases require early treatment. The 2019 American Academy of Pediatrics clinical practice guideline for the management of IHs recommends that primary care clinicians frequently monitor infants with IHs, educate the parents about the clinical course, and refer infants with high-risk IH to IH specialists ideally at 1 month of age. High-risk IHs include those with life-threatening complications, functional impairment, ulceration, associated structural anomalies, or disfigurement. In Korea, IHs are usually treated by pediatric hematology-oncologists with the cooperation of pediatric cardiologists, radiologists, dermatologists, and plastic surgeons. Oral propranolol, a nonselective beta-adrenergic antagonist, is the first-line treatment for IHs at a dosage of 2-3 mg/kg/day divided into 2 daily doses maintained for at least 6 months and often continuing until 12 months of age. Topical timolol maleate solution, a topical nonselective beta-blocker, may be used for small superficial type IHs at a dosage of 1-2 drops of 0.5% gel-forming ophthalmic solution applied twice daily. Pulse-dye laser therapy or surgery is useful for the treatment of residual skin changes after IH involution.

• Korean Journal of Transplantation
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• v.32 no.4
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• pp.104-107
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• 2018

Mucormycosis is an extremely rare but potentially life-threatening fungal infection. Gastrointestinal (GI) mucormycosis is very rare and occurs primarily in highly malnourished patients, especially in infants and children. A 55-year-old man with end-stage renal disease due to diabetic nephropathy, who had undergone deceased donor kidney transplantation 2 years prior, complained of abdominal pain and distension with a 3-day duration. Computed tomography revealed diffuse gastric wall thickening, and a huge amount of grey colored necrotic debris surrounded by erythematous erosive mucosa was observed at the antrum to upper body by GI endoscopy. The microscopic examination obtained from a GI endoscopic specimen demonstrated peptic detritus with numerous non-septate mucor hyphae in the mucosa and submucosa. Mucormycosis was diagnosed based on the clinical findings and morphological features. A total gastrectomy was performed and an antifungal agent was administered. A microscopic examination of the surgical specimen demonstrated invasive mucormycosis with numerous fungal hyphae with invasion into the mucosa to subserosa. The patient and graft were treated successfully by total gastrectomy and antifungal therapy.