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1/7. Pickwickian syndrome, 20 years later.

    The Pickwickian Syndrome stimulated new pathophysiological concepts in regard to control of ventilation. With the advent of sleep laboratories, the peculiar sleep apnea occurring in some of these patients has been explained on the basis of intermittent upper airway obstruction. Two patients with different manifestations of the Pickwickian Syndrome are presented. The suggestion is made that these two subsyndromes should have unique designations. The Auchincloss Syndrome is manifested by right heart failure and respiratory acidosis in obese patients who are alert and have no major abnormality of breathing pattern. The fundamental cause of this abnormality is the increased work of breathing caused by the obesity. The cost of breathing is so high that the ventilatory regulation is compromised and respiratory acidosis results. The Gastaut Syndrome is characterized principally by hypersomnia and sleep apnea. The fundamental defect is upper airway obstruction during sleep, resulting in increased work of breathing, which together with the increased work caused by obesity leads to respiratory acidosis and right ventricular failure. Hypersomnia, rather than heart failure or respiratory acidosis, is the major manifestation of this syndrome, and is the result of sleep loss.
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ranking = 1
keywords = obesity
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2/7. Narcotic antagonist therapy of the obesity hypoventilation syndrome.

    A child with respiratory failure and cor pulmonale secondary to the obesity hypoventilation syndrome (OHS) was found to have abnormal beta-endorphin levels in cerebrospinal fluid (CSF) and serum. A single iv dose of 10 microgram/kg of naloxone early in the course of respiratory failure resulted in dramatic improvement which lasted approximately 3 to 4 h. The patient failed to response to progesterone, and because of deteriorating respiratory status a low-dose continuous infusion of naloxone, 2 microgram/kg.h, was begun and gradually increased to 10 microgram/kg.h, during which time there was a dramatic improvement in respiratory status and clinical condition. After 5 days, naloxone infusion was discontinued and progressive respiratory deterioration recurred. The child died of over-whelming sepsis and disseminated intravascular coagulation.
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ranking = 2.5
keywords = obesity
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3/7. Remission of severe obesity-hypoventilation syndrome after short-term treatment during sleep with nasal continuous positive airway pressure.

    Two patients with the Pickwickian syndrome and with life-threatening sleep hypoxemia were treated with continuous positive airway pressure (CPAP) applied through the nares only during sleep periods. Each patient presented with severe daytime somnolence, disturbed sleep, nocturnal confusion, and daytime awake cardiorespiratory failure (PaCO2, 63 and 55 mmHg). Both patients demonstrated grossly abnormal breathing during sleep with severe sleep hypoxemia, the arterial oxyhemoglobin saturation (SaO2%) falling repetitively to levels below 50%. One patient had a hypoxemic convulsion during the initial sleep evaluation. Low levels (3.5 and 8.0 cm H2O) of continuous positive airway pressure, when applied via a comfortable nose mask, prevented occlusive apnea and obstructive hypopnea during sleep in both patients and maintained steady levels of arterial oxyhemoglobin saturation. There was rapid recovery of mental function and loss of cardiorespiratory failure within 3 days of treatment. After short-term treatment with nocturnal CPAP therapy (23 days and 35 days) both patients were able to sleep, unaided, without sleep-induced upper airway occlusion with arterial oxyhemoglobin levels sustained above 80%. We conclude that nasal CPAP therapy during sleep is an effective noninvasive therapy for patients with the Pickwickian syndrome, and may lead to a stable remission of the underlying severe disordered breathing in sleep.
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ranking = 2
keywords = obesity
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4/7. diagnosis of clinically unsuspected gallbladder perforation in an obese patient, by Tc-99m IDA cholescintigraphy.

    A male Pickwickian syndrome patient was admitted to the hospital with sudden onset of abdominal pain. physical examination was equivocal. Due to patient's ileus and morbid obesity (weight 450 lb), neither TCT scan nor ultrasound was possible. A Tc-99m PIPIDA hepatobiliary imaging study revealed intraperitoneal leakage of radioactive bile with collection of the activity in both abdominal gutters, indicating gallbladder rupture. Prompt surgery confirmed the diagnosis.
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ranking = 0.5
keywords = obesity
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5/7. Obesity as a cause of mechanical entropion.

    PURPOSE: Although lower eyelid entropion can result from many conditions, obesity is not a generally recognized factor. We treated a case of recurrent severe entropion that was a result of morbid obesity in a patient with de Morsier's syndrome. methods: The patient underwent surgery on both lower eyelids. RESULTS: The entropion was corrected by advancing the lower eyelid retractors and debulking the subcutaneous tissue in the lower eyelids. CONCLUSIONS: Mechanical entropion can occur as a rare complication of morbid obesity and may respond to surgical procedures that address its cause.
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ranking = 1.5
keywords = obesity
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6/7. Sleep apnea in a child with the pickwickian syndrome.

    A 3-year-old girl with a history of excessive weight gain from birth presented with obesity, somnolence, and cyanosis, characteristic of the Pickwickian syndrome. Obesity was familial and exogenous without endocrine or neurologic anomaly. respiratory center sensitivity to carbon dioxide was normal. Excessive somnolence was due to the obesity, which during sleep caused airway obstruction, apnea, and awakening, finally resulting in sleep deprivation. The sleep apneas and the daytime somnolence disappeared with weight reduction, showing that obesity alone had been responsible for the disorder.
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ranking = 1.5
keywords = obesity
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7/7. Complications of obesity-hypoventilation syndrome in childhood.

    The obesity-hypoventilation syndrome is rare in children, but it leads to serious complications and is associated with a high mortality. We report a child with this syndrome whose condition improved after intestinal bypass surgery. review of the literature indicates that vigorous treatment of this disorder is necessary to prevent fatalities.
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ranking = 2.5
keywords = obesity
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