Rhabdomyolysis due to rosuvastatin in a patient with ROHHAD syndrome.

We report a 13-year-old female with rapid-onset obesity, hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome, panhypopituitarism, dyslipidemia, type 2 diabetes mellitus, and nonalcoholic fatty liver disease, who developed rhabdomyolysis and acute kidney injury, two weeks after switching from lovastatin to rosuvastatin. She had been on lovastatin for eight years without any adverse effects.

Improved Behavior and Neuropsychological Function in Children With ROHHAD After High-Dose Cyclophosphamide.

Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) is a rare, generally progressive, and potentially fatal syndrome of unclear etiology. The syndrome is characterized by normal development followed by a sudden, rapid hyperphagic weight gain beginning during the preschool period, hypothalamic dysfunction, and central hypoventilation, and is often accompanied by personality changes and developmental regression, leading to substantial morbidity and mortality. We describe 2 children who had symptomatic and neuropsychological improvement after high-dose cyclophosphamide treatment. Our experience supports an autoimmune pathogenesis and provides the first neuropsychological profile of patients with rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation.

Pharmacologic treatment of sleep-disordered breathing.

Available literature on the use of pharmacologic agents for the treatment of sleep-disordered breathing was reviewed by evidenced-based methodology. Evidence tables were created and studies were graded according to study design and the number of subjects included. Scores for each group of studies evaluating each pharmacologic agent were established so that the quality of research for different drugs could be compared. The use of various ventilatory stimulants, psychotropic drugs, and antihypertensive agents were reviewed. The most objective data are available on theophylline and opioid antagonist/nicotine groups. Although more controlled studies would be helpful, relatively clear-cut indications for the use of ventilatory stimulants exist for hypercapnic obesity-hypoventilation patients (medroxyprogesterone), myxedema (thyroid replacement), central apnea (acetazolamide), and periodic breathing in congestive heart failure (theophylline). Few randomized, well-controlled trials have been published that evaluate pharmacologic agents in the treatment of classic OSA. To date, no one agent stands out as being useful for OSA. Future research will need to characterize subjects so that various subsets of patients can be tried on one or on a combination of various pharmacologic agents.

Hipoventilación alveolar primaria: ¿Enfermedad de monge en Bogotá?

Se presentan dos casos de hipoventilación alveolar sin enfermedad pulmonar ni apneas de sueño, secundarias a trastornos no anatómicos del sistema de control de la respiración. Se discuten los diagnósticos diferenciales y se plantea la hipótesis que su etiología es la hipoventilación crónica de la altura o enfermedad de Monge

[Proteolytic enzymes in the prevention and treatment of early ventilatory complications following lung resection in patients with tuberculosis]. / Proteoliticheskie fermenty v profilaktike i lechenii rannikh ventiliatsionnykh oslozhnenii posle rezektsii legkikh u bol'nykh tuberkulezom.

The authors examined 259 patients with tuberculosis of the lungs before and after partial resection of the lungs. Proteolytic enzymes were administered endotracheally in the pre- and postoperative periods to 132 patients (main group), 127 patients (control group) received a placebo. Endotracheal administration of proteolytic enzymes in the main group reduced the number of early pleuropulmonary complications of the type of atelectasis and hypoventilation by half to one third and accelerated their resolution by 3-4 times as compared to these values in the control group. Fibrous changes and pleural adhesions on the side of the operation formed from the early pleuropulmonary complications more often in the control than in the main group. The function of external respiration was restored under the effect of the proteolytic enzymes in the control group 1-2 months after segmental and combined resection, in the control group it was restored 3-4 months and later after the operation.

Right ventricular failure in a patient with diabetic neuropathy (myopathy) and central alveolar hypoventilation.

An unusual patient with hypoxemia, hypercapnia, and right ventricular failure is presented. Minimal skeletal muscle weakness, although present, cannot explain hypercapnia. Muscle biopsy revealed diabetic microangiopathy. Carbon dioxide stimulation demonstrated a diminished hypercapnic ventilatory response. The patient benefited from progesterone therapy. In this unusual patient, mild muscular weakness, caused by diabetes, and central alveolar hypoventilation have acted in synergism to cause abnormal ventilation and right ventricular failure.

Effect of hemorrhagic hypotension and hypoventilation on lower esophageal sphincter pressure.

Dysfunction of the lower esophageal sphincter has been demonstrated in the cat shock model. Analogies have been drawn to reflux and aspiration in the critically ill patient. In a cat shock model, lower esophageal sphincter pressure is reduced and response to bethanechol is impaired. Upon resuscitation to basal state with heparinized shed blood, the lower esophageal basal pressure returns to normal values, and response to bethanechol is restored. This model may explain reflux and aspiration in the critically ill patient.

Progesterone in obese patients with respiratory failure.

Two obese patients with acute respiratory failure are described. Both required mechanical ventilation to reverse the respiratory acidosis. On recovery, the major abnormality was failure of ventilatory response to inhaled carbon dioxide. After oral progesterone (100 mg/day and 20 mg/day, respectively) the slope of the carbon dioxide response line increased from 0,02 to 1,14 I min-1 mmHg-1 and from 0,04 to 1,14 I min-1 mmHg-1, respectively. This was associated with sustained clinical remission in 1 patient.

[Antagonistic effects of naloxone against morphinomimetic agents. Apropos of 10 cases in gynecologic surgery]. / Etude des effets antagonistes de la naloxone à l'égard des analgésiques morphinomimétiques. A propos de 10 cas en chirurgie gynécologique.

The object of this study was the use of naloxone to correct hypoventilation related to the use of morphinomimetics without suppressing the analgesic effect of these agents. The study involved ten patients undergoing gynaecological surgery under neuroleptanalgesia and who at the end of surgery had hypoventilation due to the use of fentanyl (average dose : 4.87 microgram/kg/h) or phenoperidine (average dose : 48.7 microgram/kg/h). Naloxone was administered intravenously in an average dose of 1.37 microgram/kg (in one or two injections) followed by an intramuscular injection of an average of 0.73 microgram/kg. Under these conditions, respiratory depression was completely corrected in all cases, the effect being durable. Good analgesia was retained and there was a normal return to consciousness without undesirable effects.

Pulmonary microembolism: attenuated pulmonary vasoconstriction with prostaglandin inhibitors and antihistamines.

The mechanism(s) involved in the pulmonary vascular and airway responses to pulmonary microembolism have not been clearly defined. Therefore, we determined the effects of specific prostaglandin and histamine blockade on the hemodynamic and arterial blood gas tension responses to particulate microembolism (200 mu glass beads) in intact anesthetized dogs. The marked increases in pulmonary arterial pressure and pulmonary vascular resistance observed in the untreated dogs were attenuated, but not abolished, following both prostaglandin blockade (with either meclofenamate or polyphloretin phosphate) and histamine blockade (with chlorpheniramine and metiamide) at 5 minutes, and were still attenuated 30 minutes post embolization. Combined prostaglandin and histamine blockade further attenuated, but again did not abolish, the pulmonary vascular responses. Cardiac outputs and systemic arterial pressures were unchanged from control by embolism. The alveolar hypoventilation (decreased arterial oxygen tension and increased carbon dioxide tension) observed in the untreated embolized dogs was prevented only with the prostaglandin inhibitors. Pulmonary microembolism in intact dogs, therefore, appears to induce vasoconstriction mediated partially by prostaglandin and histamine action, and alveolar hypoventilation mediated by prostaglandin, but not histamine, action.