This monitor will watch your baby: the home apnea monitoring controversy.
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Apnea↗
Biomedical subjects
R C Beckerman
Publications and source records attributed to R C Beckerman.
A comparison of tracheal breath sounds, airflow, and impedance pneumography in the detection of childhood apnea.
Impedance respiratory monitoring is not capable of detecting obstructive apneas. We compared a microphone breath sound detector, coupled to the chest wall, with a standard impedance device in 10 sleeping infants and children in order to determine the ability of the breath sound detector to detect normal respirations and central and obstructive apneas. Airflow was used as a standard for all measurements. No difference was found between the breath sound detector and impedance device techniques in the detection rate of either normal respirations or central apneic intervals. There was no statistically significant difference between breath sounds and airflow in the ability of either technique to detect obstructive apnea. The use of a breath sound detector avoids unnecessary stimulation of a sleeping child, whose monitoring would otherwise require that two or three airflow sensing devices be taped on the face. Breath sound monitoring may represent an alternative to impedance and airflow techniques for evaluation of apnea in closely observed infants and children.
Adolescent↗
Cystic fibrosis mistaken for idiopathic biliary atresia.
Previous reports of prolonged jaundice in cystic fibrosis have not described operative and histopathological findings in the liver and biliary tree. In the two cases reported here, obstructive jaundice in the neonatal period was associated with anatomical evidence of intra- or extrahepatic biliary obstruction. Hepatoportoenterostomy, a surgical procedure that is not without complications, was performed on one of the patients for biliary atresia before the diagnosis of cystic fibrosis was suspected. Prolonged obstructive jaundice may be an early manifestation of cystic fibrosis and may resolve without operative management. A sweat test should be performed on all patients with prolonged obstructive neonatal jaundice to rule out cystic fibrosis.
Bile Ducts↗
Benign hemangioendothelioma--a rare type of posterior mediastinal mass in children.
Two infants with benign hemangioendotheliomas of the posterior mediastinum are reported here. The neoplasms did not produce symptoms and were fortuitously discovered by chest roentgenograms. Computed tomography (CT) was useful in delineating the extent of the lesions preoperatively.
Diagnosis, Differential↗
Tracheal breath sounds for detection of apnea in infants and children.
Impedance respiratory monitoring is the standard technique for detecting apnea in infants. A miniature microphone/amplifier system has been used to sense breath sounds over the trachea in order to detect both central and obstructive apnea episodes in infants and children. The tracheal microphone has several advantages over other devices that sense airflow (thermistors, CO2 sampling catheters) because it is not easily dislodged during restless sleep. This simple device may be used in monitoring of neonates and infants; it may eventually replace impedance monitoring.
Amplifiers, Electronic↗
Circulating immune complexes in cystic fibrosis.
The presence or absence of circulating immune complexes (CIC) was studied longitudinally over a period of 15 months in 17 patients with cystic fibrosis. (CF). Using the Raji cell assay, CIC were found in 47% of patients. Presence of immune complexes did not correlate with age at midpoint of the study severity of disease, acute pulmonary exacerbations, progression of lung disease, or nonspecific, humoral and cellular immune functions. In addition, the presence of CIC did not correlate with colonization with Pseudomonas aeruginosa, Staphylococcus aureus, Hemophilus influenzae, or streptococcus species (viridans or pneumoniae). Because CIC were present in patients without concurrent sputum colonization with Pseudomonas aeruginosa, antigens other than components of this organism may be capable of forming CIC in CF. The clinical and pathologic significant of CIC in CF remains to be determined.
Adolescent↗
Fibromuscular hamartoma of the esophagus in an infant.
An 11-month-old infant had a history of dysphagia and continuous drooling without evidence for airway obstruction. A biopsy specimen of a neck mass on the left side adherent to the larynx and esophagus demonstrated pathologic features characteristic of a fibromuscular hamartoma. Upper airway obstruction subsequently ensued and the patient was managed for 16 months with a tracheostomy and feeding gastrostomy. Although posing an extremely difficult management problem, this benign tumor was surgically removed without damage to the larynx when the patient was 28 months old. The differential diagnosis of this unusual lesion must consider congenital fibromatosis and fetal rhabdomyoma.
Biopsy↗
Familial idiopathic pulmonary hemosiderosis.
Two brothers, aged 3 and 6 years, respectively, had their pulmonary conditions diagnosed as idiopathic pulmonary hemosiderosis (IPH). Both boys had severe iron-deficiency anemia, chronic cough, hemoptysis, and exertional dyspnea, and one had recurrent epistaxis. The results of light microscopic lung histopathologic studies in both patients showed numerous hemosiderinladen macrophages and chronic interstitial pneumonitis. No specific patterns of immunofluorescence of the alveolar capillary basement membranes were found. The results of electron microscopic examinations showed intact alveolar and capillary basement membranes and no evidence of electron-dense deposits. The lack of clinical or biochemical evidence for renal disease as well as the absence of serum antinuclear and antibasement membrane antibodies excluded associated autoimmune disorders. Evaluation for milk-protein allergy was negative and neither child demonstrated a clinical response to a milk-free diet. Sequential pulmonary function studies performed over four years showed episodes of acute obstructive airway disease that correlated with pulmonary hemorrhage and mild persistent restrictive lung disease. The results of this family study suggested that some cases of IPH may have a genetic basis.
Anemia, Hypochromic↗
Altered polyamine metabolism in cystic fibrosis.
Children with cystic fibrosis excreted elevated urinary levels of all three polyamines--putrescine, spermidine, and spermine. Heterozygote parents excreted intermediate concentrations of the polyamines, but not levels significantly different from levels in normal controls. Patients with cystic fibrosis who were administered a tracer amount of [14C]spermidine excreted 11--13% of the radiolabel within 72 hr whereas normal controls excreted 60--76% of the radiolabel within 72 hr. Spermine excretion was positively correlated with increased pathology as assessed by the National Institutes of Health (NIH) clinical score, whereas urinary putrescine and spermidine levels were negatively correlated with increased pathology.
Adult↗
Hypoelectrolytemia and metabolic alkalosis in infants with cystic fibrosis.
The records of all children in the Tucson area diagnosed as having cystic fibrosis (CF) before the age of 12 months were reviewed to ascertain the prevalence of metabolic alkalosis as a major presenting manifestation of CF. Five of eleven infants (46%) in whom CF had been diagnosed between 1 and 12 months of age initially were seen with hypokalemia, hypochloremia, and metabolic alkalosis unassociated with marked dehydration, hyperpyrexia, or major pulmonary and/or gastrointestinal symptoms. Two infants had repeated episodes of metabolic alkalosis; for one of these infants, both episodes of metabolic alkalosis occurred before the diagnosis of CF. It is postulated that chronic loss of sweat electrolytes together with mild gastrointestinal or respiratory illness may predispose the infant with cystic fibrosis to a severe electrolyte and acid-base disturbance. The lack of shock and hyperpyrexia together with the apparent chronicity of electrolyte losses differentiates metabolic alkalosis from the heat prostration syndrome, a more acute complication of cystic fibrosis. Quantitative sweat testing should be part of the evaluation of any infant with unexplained metabolic alkalosis. Serum electrolytes should be assessed regularly in infants with cystic fibrosis during hot weather months.
Alkalosis↗
Metabolic alkalosis in infants with cystic fibrosis.
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Alkalosis↗
Cystic fibrosis: decreased conjugation and excretion of [14C]spermidine.
Free and conjugated [14C]spermidine were measured in plasma samples from normal individuals and cystic fibrosis patients. Within 4 minutes, the 14C-labeled material in the plasma from normal individuals was 70 percent conjugated compared to no detectable conjugation by cystic fibrosis patients. Further, the patients excreted only 11 to 13 percent of the [14C]spermidine in their urine within 72 hours whereas normal excretion was 60 to 76 percent. In both cases, the labeled material was in a conjugated form.
Cystic Fibrosis↗
Arthrogryposis multiplex congenita as part of an inherited symptom complex: two case reports and a review of the literature.
Two first cousins appear to have an autosomal recessive disorder consisting of arthrogryposis multiplex congenita (AMC), pterygium coli, congenital cervical spine fusions, and some features of the Eagle-Barrett syndrome. A review of the literature is summarized including the broad concepts of AMC as well as some syndromes in which AMC is but one manifestation.
Arthrogryposis↗
The spectrum of cystic fibrosis in children and adults.
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Adolescent↗