High-Resolution Anorectal Manometry-A Prospective Cohort Study in Healthy Infants.

OBJECTIVES: Functional gastrointestinal disorders (FGD) are common during early childhood. In severe defecation disorders, anorectal manometry (AM) is done to exclude aganglionosis. High-resolution anorectal manometry (HRAM) can probably improve diagnostics but normative data in infants are scarce. This study aims to provide HRAM data in healthy infants overall and in subgroups of infants with FGD, defined as functional constipation (FC), infant colic (IC), and infant dyschezia (ID) (ROME 3 criteria). METHODS: This prospective observational cohort study enrolled healthy term infants, having HRAM at 2 and 6 months, using a water-perfused pediatric anorectal silicone catheter. Data were provided for presence of anorectal waves and recto anal inhibitory reflex (RAIR), volume needed to elicit RAIR and anal resting pressure (ARP). Questionnaires at 2 and 6 months identified subjects with FC, IC, and ID. RESULTS: A total of 187 HRAM investigations were done in 107 infants, without any complications. Normal RAIR was found in all, at least on one occasion. Mean ARP increased between 2- and 6-month investigations in infants without FGD. No other differences in HRAM data could be seen between infants with or without an FGD. At 2 months FC, IC, and ID were seen (2%, 4%, and 17%), whereas at 6 months only FC and ID were seen (7% and 1%). CONCLUSION: We can provide data on HRAM in infants at 2 and 6 months of age. When performing repeated HRAM in healthy infants, RAIR was found in all subjects. HRAM is safe, easy tolerable, and can be recommended in infants with or without FGD.

Respiratory polygraphy in children with sleep-disordered breathing.

At-home respiratory polygraphy has been shown to be a reliable substitute for in-laboratory polysomnography in adults for diagnosing obstructive sleep apnea, but this is less well studied in children. One aim of this study was to examine the quality of at-home respiratory polygraphy in children with sleep-disordered breathing and to evaluate the interrater reliability of the results. Another aim was to study whether calibrated respiratory inductance plethysmography (RIP) flow can be used for the scoring of respiratory events when the airflow measurements are unreliable. Children aged 4-10 years, with sleep-disordered breathing, underwent at-home respiratory polygraphy. Of 113 polygraphies, only 46% were of acceptable quality, with missing nasal airflow being the most common problem (40%). The median recorded time with artifact-free signal present in three traces simultaneously was 228 min (0-610 min). Seventeen polygraphy studies were selected for further study. Each study was scored by two independent scorers, with and without the nasal airflow signal present, the latter relying on RIP flow for the scoring of respiratory events. The apnea-hypopnea index (AHI) from the four different measurements was compared using intraclass correlation coefficients (ICC). Comparison of the two scorers showed moderate agreement, with (ICC = 0.66) and without (ICC = 0.53) nasal airflow. One scorer had good agreement between AHI with and without nasal airflow (ICC = 0.81), whereas the other had poor agreement (ICC = 0.12). In conclusion, the scoring of respiratory events based on RIP flow is scorer dependent even for experienced scorers. The nasal airflow signal is frequently missing in paediatric respiratory polygraphies, which limits the usefulness of the method.

Impaired peripheral airway function in adults following repair of esophageal atresia.

BACKGROUND: Esophageal atresia (EA) often leads to persistent symptoms and impaired respiratory function in adulthood. The role of peripheral airways in this impairment has not been previously investigated. Furthermore, asthma-like symptoms are common in these patients. PURPOSE: The purpose of this study was to investigate pulmonary outcome, including possible peripheral airway dysfunction, perhaps missed by conventional pulmonary function tests and to see if the diagnosis asthma was accurate. METHODS: Twenty eight patients operated for EA in Gothenburg 1968-1983 answered a questionnaire regarding respiratory symptoms and underwent pulmonary function tests. Peripheral airway function was measured by multiple breath washout. RESULTS: 22/28 (79%) patients had a history of respiratory symptoms. Abnormal peripheral airway function was found in 17 (61%) patients, while only 6 (21%) patients displayed values indicating central obstruction. Nine patients had restrictive disease. Airway hyperresponsiveness was frequent and associated with atopy and airway inflammation. However, respiratory symptoms or doctor-diagnosed asthma did not correlate with any specific lung function test abnormality. CONCLUSION: Different lung function abnormalities are present in EA survivors, and peripheral airway disease is common. Classical asthma seems to be difficult to diagnose in this patient group. Given the high prevalence of respiratory morbidity, long-term follow-up of pulmonary function, including peripheral airway function, is warranted.

Immediate changes in blood-gas tensions during chest physiotherapy with positive expiratory pressure and oscillating positive expiratory pressure in patients with cystic fibrosis.

OBJECTIVE: To assess and compare immediate effects of chest physiotherapy with positive expiratory pressure (PEP) versus oscillating PEP on transcutaneously measured blood-gas tensions in patients with cystic fibrosis. METHODS: Fifteen patients (mean age 12.5 y, range 6.9-21.5 y) participated. The treatments were randomized and performed on 2 separate occasions, 8 weeks apart. Spirometry was conducted before and after each treatment. We transcutaneously measured oxygen tension (P(tO2). RESULTS: There were no changes in spirometry values. During PEP, different trends in blood-gas tension were seen, and there were no consistent changes. During oscillating PEP, P(tO2) increased and P(tCO2) decreased. During oscillating PEP, P(tCO2) was lower and the intra-individual change in P(tCO2) was more pronounced than during PEP. The results obtained immediately after oscillating PEP showed a higher P(tO2) and a lower P(tCO2) than with PEP. CONCLUSION: PEP and oscillating PEP can both cause transitory effects on blood gases in patients with cystic fibrosis. However, oscillating PEP alters blood-gas tensions more than does PEP, and hyperventilation during oscillating PEP may reduce treatment time.

Cysteinyl leukotrienes are secretagogues in atrophic coeliac and in normal duodenal mucosa of children.

OBJECTIVE: The pathophysiology of intestinal inflammation and diarrhoea is complex and involves the arachidonic acid cascade. Prostaglandins induce chloride secretion in healthy subjects and in patients with coeliac disease. Leukotrienes (LTs) are also known inflammatory mediators which have been shown to stimulate ion secretion in ileum and colon of rats and rabbits. The aim of this study was to determine the effects of leukotrienes C(4) (LTC(4)) and D(4) (LTD(4)) in normal and atrophic intestinal mucosa in children. MATERIAL AND METHODS: Routine paediatric intestinal biopsies were obtained from 109 children. Sixty-seven control biopsies and 42 biopsies from children with different stages of coeliac disease were mounted in a modified Ussing chamber. Potential difference (Pd) was measured continuously and tissue resistance (R(t)) and the generated current (I(m)) were calculated. RESULTS: In morphologically normal mucosa of duodenum, LTC(4) and LTD(4) increased Pd and I(m) in a dose-dependent manner. The increase was more pronounced in the distal than in the proximal part, similar to the response to prostaglandin E(2). The induced current was chloride-mediated, since replacement of Cl(-) with SO(4)(2-) in the bathing solution eliminated the response to the LTs. The LTC(4)-induced secretion was significantly decreased in atrophic mucosa, but the response was partially restored after preincubation with the cyclooxygenase inhibitor indomethacin. CONCLUSIONS: The results showed that LTC(4) and LTD(4) are secretagogues in the duodenal mucosa from healthy children by inducing a net chloride secretion. Restoration of the response in coeliac disease by cyclooxygenase inhibition suggests interactions between the different pathways of the arachidonic cascade in the intestinal mucosa.

Repeated blood gas monitoring in healthy children and adolescents by the transcutaneous route.

The aim of the study was to establish transcutaneous oxygen tension (tcPO(2)), carbon dioxide tension (tcPCO(2)), and oxygen saturation (tcSO(2)) values and to investigate the intra- and interindividual variation in these values in healthy children and adolescents. Forty-one healthy children/adolescents (21 girls; mean age, 12.1 years; SD, 3.5; range, 5.0-19.0 years) participated. Continuous recordings of tcPO(2), tcPCO(2), tcSO(2), and heart rate (HR) were made for 30 min on two occasions, with a mean interval of 10.3 days, with the child/adolescent in a sitting position. The respiratory rate (RR) was counted manually during the last 5 min of each recording. The mean values of tcPO(2), tcPCO(2), tcSO(2), HR, and RR during the last 5 min of the recordings were reported. At the first recording, the mean value for tcPO(2) was 10.8 kPa (SD 1.0), for tcPCO(2) 5.1 kPa (0.4), for tcSO(2) 97.4% (1.1), for HR 82.2 min(-1) (9.9), and for RR 19.0 min(-1) (2.8). The coefficient of variation for tcPO(2) was 9.3%, for tcPCO(2) 7.8%, for tcSO(2) 1.1%, for HR 12.0%, and for RR 14.7%. The mean intraindividual variation in tcPO(2) was 0.3 kPa (SD 1.0), tcPCO(2) 0.0 kPa (0.5), tcSO(2) 0.1% (1.3), HR 0.1 min(-1) (7.7), and RR 0.5 min(-1) (2.7). No significant differences were observed in tcPO(2), tcPCO(2), tcSO(2), HR, or RR between the two recordings. In conclusion, the reproducibility of tcPO(2), tcPCO(2), and tcSO(2) monitoring during 30 min in healthy children and adolescents was good. This indicates that transcutaneous blood gas monitoring may be used to document significant changes in blood gas tensions and oxygen saturation over time.

Syncope--an unusual presentation of ventricle dysfunction in a patient with Fontan circulation.

We report a case of recurrent syncope in association with moderate ventricular dysfunction and mild AV-valve regurgitation in an 18-year-old girl, 4 years after she underwent total cavopulmonary connection surgery. Cardiac catheterization revealed a transpulmonary gradient of 1-2 mmHg. During exercise, a dramatic fall in blood pressure and blood oxygenation was observed, paralleled by an increase in heart rate and central venous pressure. Although a slight increase in pulmonary vascular resistance could not be excluded, the reaction was interpreted in terms of an extremely low transpulmonary gradient in association with ventricular dysfunction. Five months after heart transplantation, the patient has been completely free from syncope. Fontan circulation usually involves a delicate haemodynamic situation which may necessitate haemodynamic re-evaluations, including dynamic measurements of the central venous pressure during exercise. Also a moderate ventricular dysfunction may result in compromised pulmonary circulation which in turn may lead to syncope during exercise as a result of insufficient systemic circulation.