• Clinical and Experimental Pediatrics
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• v.49 no.4
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• pp.446-450
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• 2006

Congenital central hypoventilation syndrome (CCHS) or Ondine's curse is a very rare sleep disorder that is the result of a congenital failure of the autonomic control of ventilation caused by insensitivity of the chemoreceptor to hypercapnea during sleep. Gastrointestinal motility disorders, particularly a congenital megacolon (Hirschsprung disease) is often combined with CCHS. This combination can be explained by a defect in the migration of neuronal cells from the neural crest (neurocristopathy) during the intrauterine period. A diagnosis of CCHS is made by confirming the failure of adequate ventilation in response to hypercapnea and hypoxia during sleep and the exclusion of other diseases. Young infants frequently show atypical clinical courses, and their conditions are frequently complicated with the long-term sequela of hypoxemic episodes. Therefore, a high index of suspicion and active treatment with mechanical ventilation are important for reducing recurrent hypoxemic episodes in the neonatal period. This paper reports the follow up of a case of CCHS in a neonate who showed frequent intractable apnea and cyanosis and was given artificial mechanical ventilation during sleep.

• Journal of Yeungnam Medical Science
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• v.5 no.2
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• pp.213-219
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• 1988

In the geriatric patient with COPD, incidence of postoperative pulmonary complication is higher than young patient. Therefore, some anesthesiologists preferred spinal anesthesia to general anesthesia for surgery of the perineum, lower extrimities, and pelvic extra peritoneal organs. But, during spinal anesthesia, the same careful observation is requiered as during general anesthesia. We experienced a case of the convulsive seizure at about 1 hour after spinal anesthesia for open prostatectomy in a 76-year-old male patient wit COPD. It was suspected that his convulsive seizure be resulted from hypercapnea combined with hypoxia following upper airway obstruction. This patient was treated successfully by ultrashort acting barbiturate and controlled ventilation.

• Journal of The Korean Society of Clinical Toxicology
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• v.9 no.1
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• pp.30-33
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• 2011

Trichloroethylene (TCE, $C_2HCl_3$), which was introduced as a gas for general anesthesia and analgesia in early 1900's has been widely used in industry as an organic solvent. Occupational exposure to TCE is an important medical problem. Manifestations of acute exposure to TCE include mucocutaneous irritation, hepatotoxicity, cognitive impairment, sleep, headache, respiratory insufficiency and death. We report a 38-year-old man who was admitted to a department of emergency medicine after occupational inhalation exposure to TCE. He rapidly developed semicoma and respiratory depression. After mechanical ventilation, hypercapnea and hypoxemia disappeared and his mental state again became alert. Careful evaluation and proper respiratory support are important for respiratory failure after occupational TCE inhalation.

• Journal of Chest Surgery
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• v.17 no.1
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• pp.101-109
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• 1984

The influences of acute respiratory and metabolic acid-base disturbances on the carotid, renal and coronary blood flow were measured in dogs. Respiratory acidosis was induced by artificial respiration with 8% CO2 -02 gas mixture and respiratory alkalosis was induced by hyperventilation under the control of respirator. Metabolic acidosis and metabolic alkalosis were induced by intravenous infusion of 0.3N hydrochloric acid and 0.6M sodium bicarbonate solution. To observe the effect of hyperkalemia, isotonic potassium chloride solution was infused. CVI electromagnetic flowmeter probes were placed on the left common carotid artery, left renal artery and left circumflex coronary artery. Each flow was recorded on polygraph. 1. The carotid blood flow showed rapid showed rapid and marked increase in acute respiratory acidosis. Even in the cases when arterial blood pressure was lowered during the state of respiratory acidosis, carotid blood flow increased. By the infusion of hydrochloric acid, carotid blood flow increased slowly and returned to the previous label after discontinuation of the infusion. Carotid blood flow also increased by the infusion of large amount of sodium bicarbonate, but it might be the combined effect of expansion of extracellular fluid and compensatory elevation of carbon dioxide tension. 2.The renal blood flow remained unchanged during the acute acid-base disturbances, suggesting effective autoregulation. Renal blood flow, however, increased very slowly when the infusion of potassium chloride continued for a long period. 3.Although less marked than the carotid blood flow, the coronary blood flow increased in the acute respiratory and metabolic acidosis. In asphyxiated condition, coronary blood flow increased most markedly and this might be the combined effect of hypoxia, hypercapnea, and lowering of pH. In summary, the carotid blowflow showed more marked change in the acute respiratory and metabolic acidosis than the renal and coronary blood flow. Respiratory and metabolic components of acid-base disturbances may influence the local blood flow concomitantly, there being more differences in the individual responses, but respiratory component manifested more rapid and marked effect than metabolic component.

• Advances in pediatric surgery
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• v.5 no.2
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• pp.146-151
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• 1999

Neurocristopathy originates from aberrant development of the neural crest by genetic abnormality. Dysgenetic or neoplastic neurocristopathy mayor may not combine at one or more organs. Congenital central hypoventilation syndrome (Ondine's curse) is characterized by the respiratory depression during sleep, although showing normal ventilation while awake, because the baby does not responde to hypercapnea or hypoxia. One newborn girl, full-term, 3,020 g of birth weight with neurocristopathy is reported. It showed poor respiration at birth, and temporary Improvement with oxygen and respiratory stimulations. Abdomen was distended. Abdominal x-ray revealed small bowel obstruction and calcified opacity at the right lower quadrant. Because transitional zone was noticed at the distal jejunum during laparotomy, jejunostomy was performed. Several times trial of extubation have failed becaused of the repeated apneas. Brain sonography and echocardiogram were normal. The patient died of sepsis at 37 days of age. Para-aortic ganglioneuroblastoma was found at autopsy. In this case, congenital central hypoventilation syndrome, Hirschsprung's disease and congenital ganglioneuroblastoma are combined as a neurocristopathy.

• Tuberculosis and Respiratory Diseases
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• v.58 no.2
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• pp.160-166
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• 2005

Background : Although home oxygen therapy in hypoxemic patients with chronic lung disease has been increasing over the decade in Korea, the present state has not been known well. This study was done to know the situation of home oxygen therapy in a Korean university hospital. Methods : Between January 1, 2000 and August 31, 2003, 86 patients prescribed home oxygen therapy by the pulmonary physicians of Asan Medical Center were investigated using their medical record and questionnaire. Results : Patients (52 men, mean age of 61 years) with home oxygen therapy were increasing by every year. Underlying diseases were COPD (n=29), tuberculous destroyed lung (n=18), bronchiectasis (n=15), ILD (n=12), and others. Baseline $FEV_1/FVC$, FVC, and $FEV_1$ of patients were $58.4{\pm}25.2%$, $54.5{\pm}17.1%$ of predicted, and $41.7{\pm}20.6%$ of pred. Mean oxygen flow was 1.5 L/min and mean duration per day was 14.5 hours. During therapy, mean $PaO_2$ values have increased from 51.2 to 77.7 mm Hg and $PaCO_2$ values have increased from 47.5 to 49.6 mm Hg. Only 16.5% of the subjects were monitored by visiting nurses or pulse oximeter. Three year survival rate was 56.6% and hypercapnic patients showed better prognosis. Conclusion : The patients with home oxygen therapy were increasing yearly and a part of them were monitored. The hypercapnea respiratory failure patients would have better prognosis.

• Sleep Medicine and Psychophysiology
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• v.22 no.1
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• pp.30-34
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• 2015

Obesity hypoventilation syndrome (OHS) is characterized by severe obesity, excessive daytime sleepiness, hypoxemia and hypercapnea. Because OHS mimics pulmonary hypertension or cor pulmonale, clinicians should recognize and treat this syndrome appropriately. A 58-year-old female visited the emergency room because of dyspnea. She was obese and had kyphoscoliosis. The patient also experienced snoring, recurrent choking during sleep and daytime hypersomnolence which worsened after gaining weight in the recent year. The arterial blood gas analysis showed she experienced hypoxemia and hypercapnea not only during nighttime but also daytime. We suspected OHS and the patient underwent polysomnography to confirm whether obstructive sleep apnea was present. During the polysomnography test, sleep obstructive apnea was observed and apnea-hypopnea index was 9.2/hr. The patient was treated with bilevel positive airway pressure therapy (BiPAP). After BiPAP for 4 days, hypoxemia and hypercapnia were resolved and she is currently well without BiPAP. We report a case successfully treated with clinical improvement by presuming OHS early in a patient who had typical OHS symptoms, even while having other conditions which could cause hypoventilation.