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Hypercapnia in the obstructive sleep apnea syndrome. A reevaluation of the "Pickwickian syndrome".

The mechanisms of hypercapnia in eight patients with the "Pickwickian" syndrome and obstructive sleep apnea (OSAS) were evaluated pretherapy and posttherapy (tracheostomy in seven patients and chronic nocturnal use of nasal CPAP in one). Four patients (correctors) became eucapnic within two weeks of therapy. Four others (noncorrectors) remained hypercapnic. Neither residual apneas, changes in pulmonary function, change in anatomic dead space, nor changes in ventilatory chemoresponsiveness differentiated the two groups, nor did the last three factors account for return to eucapnia in the correctors. The results indicated two separate mechanisms exist for chronic hypercapnia in OSAS: a critical balance between the ventilation during the time spent awake and hypoventilation due to apneas, a mechanism removed by treatment for obstructive apnea; and sustained hypoventilation independent of the apnea phenomenon and therefore not correctible. The subset of patients with the second mechanism appears to represent the true "Pickwickian" syndrome and can be identified before therapy by measuring a low level of ventilation in the sustained awake state.

Aged↗

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.

Acidosis, Respiratory↗

Progesterone for outpatient treatment of Pickwickian syndrome.

Ten patients with the Pickwickian syndrome, characterized by obesity, hypoxemia, hypercapnia, polycythemia, and cor pulmonale, underwent long-term treatment as outpatients with medroxyprogesterone acetate. Although there was no significant weight change in the group, PaO2 rose 12.6 +/- 2.7 mm Hg (SEM) from 49 +/- 2.6 mm Hg to 62 +/- 2.3 mm Hg (P less than 0.001), while PaCO2 fell 13 +/- 2.6 mm Hg from 51 +/- 1.9 mm Hg to 38 +/- 1.2 mm Hg (P less than 0.001). Hematocrit fell from 56 +/- 2.5% to 50 +/- 1.2%, a mean fall of 6% (P less than 0.01), during medroxyprogesterone acetate therapy. In the 2 patients who had cardiac catheterization before and during medroxyprogesterone acetate therapy, mean pulmonary arterial pressure fell 13 and 19 mm Hg. There were no recurrences of cor pulmonale during treatment. These effects on arterial blood gas values and clinical state were sustained during therapy. On withdrawal of medroxyprogesterone acetate during 1-month period, arterial oxygen and carbon dioxide tensions deteriorated to their previous pretreatment values. Reinstitution of medroxyprogesterone acetate caused improvement in both the oxygen and carbon dioxide tensions. We conclude that sublingual medroxyprogesterone acetate therapy is useful in the management of the Pickwickian syndrome.

Adult↗

[Pickwickian syndrome and hypersomnia with periodic respiration].

A classical definition of Pickwickian syndrome associates alveolar hypoventilation, obesity and hypersomnia with periodic breathing. Obesity in itself is enough to explain the alveolar hypoventilation and some of the sleep disorders, but in fact all three elements of this syndrome are intricated. Obesity, whether associated with a Pickwickian syndrome or not, affects ventilatory mechanics similarly. With the cause of sleep disorders are associated central ventilatory pauses, appearing periodically followed by phases of apnoea secondary to buccopharyngial hypotony. The alveolar hypoventilation is therefore the consequence of obesity and periodic apnoea. It also results from a low respiratory frequency considering that the tidal volume is also decreased. These different elements suggest some disorder of the centers controling ventilation. One can describe different nosological forms, all having as a common factor hypersomnia with periodic respiration, the latter being a determining factor in the diagnosis. Therapy, including weight reduction and symptomatic treatment of alveolar hypoventilation, is now augmented by new drugs acting on the central nervous system.

Circadian Rhythm↗

Pulmonary function in a typical case of Pickwickian syndrome.

Pulmonary function was studied in a patient with Pickwickian syndrome, before and after a weight reduction of 160 kg which was brought about by jejunoileal bypass surgery. After weight reduction, there was an increase in alveolar ventilation, lung volumes and respiratory flow rates. Arterial blood oxygen tension, arterial oxygen saturation, arterial carbon dioxide tension and pH also showed a significant improvement towards normal. These findings indicate that reduction of body weight is beneficial for Pickwickian syndrome patients. The improvement of pulmonary function was probably due to the improvement of the patient's respiratory mechanics.

Adult↗

[Treatment of patients with the Pickwickian syndrome by reduction of body weight].

The characteristic signs of Pickwickian syndrome are the high degree of obesity and cardiovascular and respiratory insufficiency, to various degrees. Forty five patients with Pickwickian syndrome were treated at the Clinic of Metabolic Diseases. They represent 3.4 per cent of the total number of patients with obesity of various degrees, observed and treated. The majority of the affected were established to be males (34), mainly III and IV degree of obesity. The average age of the patients was 51 years. Somnolence, lassitude, enhanced appetite, cyanosis and dyspnea with unrestful sleep, with sloring were observed in all patients. The moderate motive regiment applied and the hypoenergy diet under clinical condicions with an average stay of 26 days at the clinic led to considerable reduction of body mass (with 11,7 kg on the average), resulting into an improvement of the functional respiratory parameters and ECG. The elevated values of blood pressure were normalized in the majority of the patients after the treatment. In all patients, the unpleasant subjective complaints, somnolence including, disappeared and respiratory indices were improved. The significant disorders in lipid, carbohydrate and purine metabolism were favourably influenced by the therapeutic regiment applied.

Body Weight↗

[A case of Pickwickian syndrome treated by implantation of a cardiac permanent pacemaker].

A 41-year-old male was admitted to our hospital with congestive heart failure and bronchopneumonia. During hospitalization extreme sinus bradycardia, sinus arrest up to 6.2 seconds and high grade AV block were observed to occur simultaneously with apneic episodes of ECG monitoring. After that, a diagnosis of Pickwickian syndrome was made in this obese patient. In spite of weight reduction, change of sleep position and acetazolamide administration, obstructive sleep apnea and severe bradyarrhythmias were not improve. These severe bradyarrhythmias and ventricular tachyarrhythmias may be one cause of the not infrequent sudden deaths in patients with this Pickwickian syndrome. In addition to the tracheostomy, we propose that implantation of a cardiac permanent pacemaker should be selected for the bradyarrhythmias in association with the Pickwickian syndrome.

Adult↗

Pickwickian syndrome: the challenge of severe sleep apnea.

Pickwickian syndrome is a severe form of sleep apnea in obese persons which involves mechanical impairment of ventilation resulting in greatly compromised gas exchange. Manifestations of the syndrome are associated with deposits of adipose tissue around the abdomen and diaphragm and are completely reversible with weight loss. Since sleep apnea is now recognized as a significant chronic health problem, nurses in intensive care, medical-surgical, and home care settings are increasingly challenged to provide competent assessment, care, and rehabilitation of affected individuals.

Adult↗

[Neurophysiologic studies in Pickwickian syndrome].

In 11 patients clinically diagnosed as Pickwickian syndrome the results of daytime and night polysomnogramm were compared. The daytime recording was very efficient in identifying apnoea and correlating various types of apnoea to different sleep stages. In polysomnogramms of night sleep we found, as did Lugaresi, a reduction of sleep stages III and IV, and a slight increase of sleep stages I and II. Patients without apnea in daytime sleep also showed a normal apnea index in night sleep. The BAEP's especially the pontomesencephalic components were pathological in six patients (55%). This might be due to a primary lesion or to hypoxic damage of the brainstem.

Adult↗

[A case of Pickwickian syndrome with improvement of obesity-related sleep apnea following vertical banded gastroplasty].

The patient was 22-year-old male who had suffered from hypermotility during sleep and excessive daytime sleepiness, and 270% in excess body weight. Values of arterial blood gas analysis during sleep were 65mmHg in PaCO2 and 19mmHg in PaO2. He was diagnosed as morbid obesity with Pickwickian syndrome and underwent vertical banded gastroplasty. His body weight decreased from 170kg to 113kg four months after surgery and 107kg one year after surgery. The whole-night polyhypnographic sleep study was performed before and after operation and showed dramatic improvement of apnea index which was calculated by dividing the number of apneas by the total sleep time. We analyzed 199 case reports which had noted symptoms of Pickwickian syndrome between 1963 and 1989 in Japan. In many reported cases, body weight decreased transiently but returned to the level before medical treatment. Under these considerations, gastroplasty is a beneficial surgical treatment for the morbid obesity with Pickwickian syndrome.

Adult↗

[Differential diagnostic problems and polygraphic studies in a case of Pickwickian syndrome].

The case report of a male patient with the Pickwickian syndrome is presented. The results of the relevant neurophysiological examinations (polygraphic registration of EEG, electrocardiogram, respiration, thorax excursions, submental and intercostal electromyogram, electrooculogram) give an insight into the pathomechanisms and enable the differential diagnosis to be made between this condition and narcolepsy and also the Kleine-Levin syndrome; respiratory investigations (spirometry, hypercapnic ventilatory response: mouth occlusion pressure) make it possible to differentiate between the Pickwickian and Ondine's curse syndrome. The therapeutic management is discussed.

Adult↗

[A 13-year-old-girl with Pickwickian syndrome and Asperger syndrome].

We reported a 13-year-old girl with Pickwickian syndrome and Asperger syndrome. The chief complaint on admission was apnea attacks during sleep. She had severe obesity. Whole night polysomnography showed that the apnea attacks occurred during light and REM sleep, and that slow wave sleep and REM sleep volumes were decreased. These findings were the same as those on adult cases. Weight control was very difficult because of Asperger syndrome.

Adolescent↗

[Clinical characteristics and morphology of Pickwickian syndrome].

Clinical and morphological evidence obtained for 23 patients with Pickwickian syndrome has been analysed. 18 patients had no other symptoms, 12 patients died of the disease. Standard signs of the syndrome (drowsiness, respiratory distress, diffuse cyanosis, etc.) were not universal, whereas mixed-type respiratory disorders occurred in all the patients as well as right heart hypertrophy, sclerosis and hyalinosis of the lesser circulatory bed found morphologically and histologically.

Age Factors↗

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.

Apnea↗

Obesity-sleep apnea (Pickwickian) syndrome: autopsy findings and a medicolegal review.

A 24-year-old obese woman was found dead in her boyfriend's apartment in his absence. She had been admitted to the hospital six times previously because of diminished consciousness, respiratory failure, and pneumonia. A diagnosis of obesity-sleep apnea (Pickwickian) syndrome was made. An autopsy showed that she had an extremely small larynx, intra-alveolar hemorrhage, edema, pulmonary lymphocyte infiltration, and severe focal myocardial fibrosis. No fresh myocardial lesion, coronary arterial lesion, or findings of heart failure were seen. The woman's elder sister had also died of the same disease at the age of 23. The cause of death was diagnosed as respiratory failure and pneumonia with the sleep-apnea syndrome as the underlying cause of death. Although no autopsy reports of the sleep-apnea syndrome have been published in the field of forensic pathology, this syndrome is a predominant cause of sudden death in obese persons and could be a hidden cause of accidental death in such persons.

Journal Article↗

[Contribution of nocturnal polygraphy to the diagnosis of Pickwickian syndrome. 10 cases].

The authors studied a series of 10 obese patients with respiratory failure referred for treatment because of sleep disorders and diurnal, sometimes uncontrollable, episodes of somnolence. 8 parameters were recorded in the polygraphic study performed during a night of hospitalization: electroencephalogram, electrocardiogram, electro-oculogram, chin electromyogram, thoracic movements, and nasal and buccal air flows. SaO2 and transcutaneous PO2 were recorded simultaneously. A sleep apnea syndrome was diagnosed in 6 of the 10 patients, whose apnea index was markedly above the limit of 5 apneas per hour. The apnea index was below 5 in the other 4 patients. Most patients with sleep apnea syndrome suffer from obstructive apneas of varying duration taking up as much as 48% of total sleep time. The cardiorespiratory effects of these events are apparent, with a drop in PO2 and SaO2 and a decrease in heart rate at the end of apnea. Polygraphic studies seem useful in the diagnosis of the pickwickian syndrome. They allow the type of apnea and its effects to be specified and thus guide treatment.

Aged↗