Upper airway resistance syndrome: sick, symptomatic but underrecognized.

Upper airway resistance syndrome (UARS) appears to be underrecognized or at least underreported in the medical literature. We have found that these children may suffer the same, if not more, severe consequences of sleep disordered breathing than obstructive sleep apnea patients. Seventy-five infants and children were age and gender matched from a heterogeneous patient population in Southern California. Each patient underwent clinical polysomnography. Twenty-five patients were in each of three groups. Group one patients were clinician referred to the sleep clinic and were found to be free of significant sleep pathology. Group two patients had UARS. Group three patients had frank obstructive sleep apnea. The three groups differed on respiratory, sleep and snoring parameters. A heuristic is presented to help clinicians tailor their treatment approach and sleep scientists to test the model to aid in the understanding of UARS.

Nasal continuous positive airway pressure use in children with obstructive sleep apnea younger than 2 years of age.

STUDY OBJECTIVES: To assess the efficacy of continuous positive airway pressure (CPAP) in obstructive sleep apnea (OSA) patients who are < 2 years of age. DESIGN: A retrospective chart review of 18 patients from 1992 to 1999 who had OSA confirmed by polysomnography. All patients in this study also completed a separate night of CPAP polysomnography to determine the effectiveness of CPAP in the correction of OSA. Nasal CPAP compliance data were gathered via clinical follow-up examination, telephone interview, or mailed questionnaire. SETTING: All patients were studied in the Sleep Disorders Center at Loma Linda University Children's Hospital in Loma Linda, CA. PATIENTS: All patients were < 2 years old. INTERVENTION: After OSA was confirmed by the results of technician-attended nocturnal polysomnography, separate technician-attended nocturnal CPAP polysomnography was completed. On CPAP nights, CPAP pressure was titrated to ameliorate OSA and snoring. CPAP pressure was increased by 2-cm H(2)O or 1-cm H(2)O increments. RESULTS: Data were analyzed by dependent groups t test at p < 0.05 level of significance. CPAP statistically improved respiratory parameters significantly when compared to baseline polysomnography. The following four patient subgroups emerged from the analysis: group 1 consisted of six patients who had tracheostomies prior to the CPAP trial, with two patients using CPAP as an alternative to tracheostomy; group 2 consisted of two patients who had previous unsuccessful adenostonsillectomies and who used CPAP successfully, with both having OSA resolution over time; group 3 consisted of four patients who did not tolerate CPAP on the study night; and group 4 consisted of six patients who used CPAP nightly, had OSA resolution over time, and therefore, no longer needed CPAP therapy. Thus, 10 of 18 patients used CPAP either on an interim basis for corrective therapy or as a primary treatment modality for OSA. CONCLUSIONS: These data show that children < 2 years of age can tolerate and use CPAP effectively. In several cases, CPAP treatment could be discontinued as OSA resolved over time. The reasons for this are discussed in the text.

Respiratory failure complicating rubeola.

We reviewed the charts of 19 patients with the diagnosis of measles who were admitted to the pediatric intensive care unit for respiratory failure requiring intubation and mechanical ventilation. Patients studied were admitted during the period June 1989 to June 1990. The mean age was 19 months (range, 3 to 51 months). The cause for respiratory failure fell into two groups: 47 percent developed pneumonitis and refractory hypoxemia. Patients with pneumonitis and hypoxemic respiratory failure had a 56 percent mortality. An oxygenation index of greater than 40 for 4 h separated survivors from nonsurvivors (oxygenation index = [mean airway pressure x FIo2/PaO2 x 100]). Patients with tracheitis alone all survived. In these patients the organism primarily responsible was Staphylococcus aureus (70 percent). Two of the seven patients with S aureus tracheitis had signs and symptoms of toxic shock syndrome and we subsequently demonstrated toxic shock syndrome toxin 1 in both patients.

Intravenous immunoglobulin as therapy for pediatric Guillain-Barré syndrome.

Seven children with Guillain-Barré syndrome were treated with intravenous immunoglobulin. Median patient age was 5.8 years. A standard dosage of 0.4 g/kg/day for 5 days was administered. Clinical improvement occurred on average within 2.4 +/- 1.3 days of beginning intravenous immunoglobulin. One child required mechanical ventilation for 7 days. Eight comparable children with Guillain-Barré syndrome at our institution in a prior study treated with plasmapheresis alone had similar clinical results. However, the need for admission to the pediatric intensive care unit and duration of pediatric intensive care unit stay were lower in the intravenous immunoglobulin treated group (P < .05). There were no complications with intravenous immunoglobulin therapy except for a brief episode of hypotension in one patient. Review of the literature identified 74 additional children with Guillain-Barré syndrome successfully receiving intravenous immunoglobulin therapy. We suggest intravenous immunoglobulin as initial therapy for pediatric Guillain-Barré syndrome, because it appears equally as effective as plasmapheresis and is associated with fewer complications.

Guillain-Barré syndrome in childhood: natural course and efficacy of plasmapheresis.

Eight children with Guillain-Barré syndrome were treated with plasmapheresis. Retrospective comparisons were made with 11 historic control patients. Eight children required mechanical ventilation, 4 of whom were in the plasmapheresis group. One week after the last plasmapheresis treatment, patients receiving plasmapheresis within 7 days of symptom onset had improved by one Guillain-Barré syndrome score. Discharge Guillain-Barré syndrome scores were significantly lower for those receiving plasmapheresis (P < .05). Patients in the plasmapheresis group had a decrease in the number of days of mechanical ventilation, time until motor recovery, and overall cost. Our results are consistent with published literature and indicate that plasmapheresis for childhood Guillain-Barré syndrome is a safe and effective treatment to shorten the time to recovery.

End of life care in Duchenne muscular dystrophy.

End of life care for patients with Duchenne muscular dystrophy (DMD) has become increasingly complex because of new technologies, changes in medical personnel over periods of time, emergence of home health care systems, and increasing patient and family autonomy in decision-making. In this review, we discuss the medical problems, particularly respiratory and cardiac failure, faced by DMD patients. Current concepts concerning the evaluation and options for treatment of these problems are presented as well as the ethical issues involved in the care of the DMD patient. These issues include the medical indications for treatment, patient preferences, quality-of-life issues, and contextual features related to legal, institutional, religious, geographic, cultural, social, and financial factors. We also present our experience at Loma Linda University Medical Center over the past 10 years in the development of a home mechanical ventilation program for DMD patients and an algorithm for the evaluation of these patients. Many patients with DMD do well on long-term ventilation, but some find that their quality of life is less than desirable and choose to discontinue this method of life-prolongation. Many of these new options are very expensive, making the decision to use them a difficult one. Ultimately, these are societal issues that require clear reflection on matters of resource allocation that should be performed by health care professionals, citizens, and health planners.

CBF and CBF/PCO2 reactivity in childhood strangulation.

Four children with self-inflicted strangulation injuries had cerebral blood flow determined by stable xenon computed tomography (XeCTCBF) within 24 hours of admission. All had suffered a severe hypoxic-ischemic cerebral injury; 3 initially had fixed pupils, all were apneic with varying bradyarrhythmias, and the initial mean arterial pH was 7.26 (+/- 0.18). The initial blood glucose values were greater than 300 mg/dl (334 and 351 mg/dl) in the 2 patients who died compared to the 2 who survived (104 and 295 mg/dl). The cardiac index was depressed during the first several days of hospitalization in the 2 patients who died (less than 2.0 L/min/m2) compared to the 2 who survived. Total CBF was normal (63 +/- 8 ml/min/100 gm) and local variations in CBF were present. PCO2 reactivity was determined by hyperventilating the 4 patients for 20 min from an end tidal PCO2 of 39 +/- 3 torr to 29 +/- 1 torr and then repeating the XeCTCBF study. Marked regional variability in the CBF/PCO2 response was observed, ranging from 0.5-5.5 ml/min/100 gm/torr PCO2. In the 2 patients who died, the CBF/PCO2 was decreased (1.2 ml/min/100 gm/torr PCO2) compared to the 2 patients who survived (2.1 ml/min/100 gm/torr PCO2). Although CBF was normal in these 4 children, the hyperventilation response was depressed, variable, and even paradoxical which may be important in the evolution of further brain injury and is a critical factor in deciding whether hyperventilation may be of clinical benefit.(ABSTRACT TRUNCATED AT 250 WORDS)

Proton magnetic resonance spectroscopy in children with acute central nervous system injury.

Single voxel proton magnetic resonance spectroscopy (1H-MRS) was used in 30 infants and children with acute central nervous system injuries to determine the value of changes in specific metabolite ratios in predicting outcome. The mean age of all patients was 38 +/- 52 months and the mean time of study after insult was 7 +/- 5 days. 1H-MRS was determined in the occipital gray and parietal white matter (8 cm3 volume, STEAM sequence with TE = 20 ms, TR = 3,000 ms). Data were expressed as ratios of different metabolite peak areas including N-acetylaspartate (NA), choline-containing compounds (Ch), creatine and phosphocreatine (Cr), and lactate (Lac). Statistically significant differences were observed when patients with good/moderate (G/M) outcomes (n = 17; mean age: 46 months) were compared to patients with bad outcomes (n = 10; mean age: 26 months). NA/Cr and NA/Ch were significantly lower in the bad outcome group (NA/Cr = 1.15 +/- 0.38; NA/Ch = 1.18 +/- 0.52) compared to the G/M group (NA/Cr = 1.41 +/- 0.28, P < .05; NA/Ch = 1.98 +/- 0.81, P < .01). Lactate was present in 80% of bad outcome patients and in none of the G/M group (P < .0001). Using a linear discriminant analysis and combining 4 clinical variables (Glasgow Coma Scale score, initial pH and glucose, number of days unconscious at time of 1H-MRS) allows classification of 94% of patients into their correct outcome group. Use of spectroscopy variables (NA/Cr, NA/Ch, Ch/Cr, presence of lactate) alone correctly classified 81% of patients. The combination of clinical and 1H-MRS variables correctly classified 100% of patients. Our findings suggest that 1H-MRS adds information which, in combination with clinical examination, may be useful in outcome assessment in children with serious acute central nervous system injury.

Predictive value of proton magnetic resonance spectroscopy in pediatric closed head injury.

We studied 26 infants (1-18 months old) and 27 children (18 months or older) with acute nonaccidental (n = 21) or other forms (n = 32) of traumatic brain injury using clinical rating scales, a 15-point MRI scoring system, and occipital gray matter short-echo proton MRS. We compared the differences between the acutely determined variables (metabolite ratios and the presence of lactate) and 6- to 12-month outcomes. The metabolite ratios were abnormal (lower NAA/Cre or NAA/Cho; higher Cho/Cre) in patients with a poor outcome. Lactate was evident in 91% of infants and 80% of children with poor outcomes; none of the patients with a good outcome had lactate. At best, the clinical variables alone predicted the outcome in 77% of infants and 86% of children, and lactate alone predicted the outcome in 96% of infants and 96% of children. No further improvement in outcome prediction was observed when the lactate variable was combined with MRI ratios or clinical variables. The findings of spectral sampling in areas of brain not directly injured reflected the effects of global metabolic changes. Proton MRS provides objective data early after traumatic brain injury that can improve the ability to predict long-term neurologic outcome.

Common fluid and electrolyte problems in the pediatric intensive care unit.

Administration of intravenous fluid to infants and children requires an understanding of the normal expenditure of water and electrolytes by the healthy child and the effects of specific illnesses on water and electrolyte turnover.

Stress and distress in pediatric nurses: lessons from Baby K.

BACKGROUND: Nurses experience stress and suffering when they care for critically ill and dying patients. Moral distress occurs when nurses are unable to translate their moral choices into moral action. In response to this stress, nurses may experience burnout. OBJECTIVE: To investigate the attitudes and perceptions of the nurses who were ordered by the court to provide long-term care for Baby K, a female child with anencephaly. METHOD: A questionnaire was developed to provide descriptive insight into the stress and distress the nurses experienced while caring for Baby K. RESULTS: Nurses caring for Baby K experienced stress and suffering. Although they felt supported and respected as nurses in their workplace, they felt conflict with the legal system. To compensate for feelings of powerlessness, the nurses simply put on a professional face and continued to provide care. They feared making mistakes, many felt guilty and angry, and all felt that they were not included in decision making. CONCLUSIONS: (1) Suffering among caregivers occurs and must be recognized, (2) measures must be taken to reduce the stress and distress of healthcare professionals as they provide care to patients who cannot recover, and (3) in addition to these measures, society must provide guidance to healthcare professionals, especially concerning the care of patients who are permanently unconscious.

Low-dose continuous intravenous insulin infusion in childhood diabetic ketoacidosis.

We studied 58 children with diabetic ketoacidosis using a random, prospective protocol, with insulin administered either as a low-dose continuous infusion or as high-dose intermittent subcutaneous injections. There were no statistically significant differences between admission pH and glucose determinations or the time to metabolic correction. The incidence of hypoglycemia and hypokalemia was higher in patients receiving subcutaneous insulin. Insulin levels in the low-dose patients were 85--160 microU/ml. The insulin required to achieve metabolic recovery was 1.6 U/kg in the low-dose group and 4.5 U/kg in the high-dose group (p less than 0.01). Glucose administered at a rate of 3 to 4 g er unit of insulin infused in the low-dose group maintained a serum glucose of 150 to 250 mg/dl. Our studies suggest that low-dose intravenous insulin therapy is safe, as effective as high-dose intermittent subcutaneous injections and avoids the risks of hypoglycemia and hypokalemia. Meticulous attention to individual patient care, however, must remain the most important single variable.

Sleep-disordered breathing in infants and children.

Sleep-disordered breathing (SDB) is underdiagnosed in infants and children. In addition to causing physical ailments that range from failure to thrive to cor pulmonale, SDB is often an unrecognized cause of failure in school or of behavioral disorders. Diagnosis of SDB requires a careful and detailed history and physical examination. Polysomnography is required to determine the nature of the problems, the magnitude of the physiologic disturbance, and, ultimately, the significance of the problem for the child. Earlier recognition, accurate diagnosis, and appropriate treatment should alleviate much of the childhood morbidity associated with these conditions.