Can postural changes in spirometry in children with Duchenne muscular dystrophy predict sleep hypoventilation?

AIM: To explore the relationship between postural changes in lung function and polysomnography (PSG) in children with Duchenne muscular dystrophy (DMD). METHODS: In this prospective cross-sectional study, children with DMD performed spirometry in sitting and supine positions. A control group of age and gender matched healthy children also underwent postural lung function testing. PSG was performed within six months of spirometry. RESULTS: Seventeen children with DMD, aged 12.3 ± 3 years performed sitting spirometry. 14 (84%) performed acceptable spirometry in the supine position. Mean FEV1sit and FVCsit were 77% (SD ± 22) and 74% (SD ± 20.4) respectively, with mean% ΔFVC(sit-sup) 9% (SD ± 11) (range 2% to 20%), and was significantly greater than healthy controls 4% (n = 30, SD ± 3, P < 0.001). PSG data on the 14 DMD children with acceptable supine spirometry showed total AHI 6.9 ± 5.9/hour (0.3 to 29), obstructive AHI 5.2 ± 4.0/hour (0.2 to 10), and REM AHI 14.1 ± -5.3/hour (0.1 to 34.7). ΔFVC(sit-sup) had poor correlation with hypoventilation on polysomnography. CONCLUSION: Children with DMD and mild restrictive lung disease showed greater postural changes in spirometry than healthy controls but lower supine spirometry was not predictive of sleep hypoventilation.

Use of non-invasive ventilation in children with congenital tracheal stenosis.

INTRODUCTION: Congenital tracheal stenosis (CTS) is a rare airway condition characterized by complete tracheal rings. Most patients undergo a slide tracheoplasty, which greatly reduces mortality but significant morbidity remains. The assessment of sleep disordered breathing (SDB) and use of non-invasive ventilation (NIV) in these children has not been described. AIM: To describe the presence of SDB and use of NIV in children diagnosed with CTS over a 10-year period (2005-2015). DESIGN: Retrospective case series at a tertiary children's hospital. RESULTS: There were 16 patients identified with CTS with a median [range] age at diagnosis of 2.5 months (0-9 months). One child died in the immediate post-operative period following a slide tracheoplasty, leaving 15 survivors. There were no later deaths during follow-up while using NIV for up to 3 years after surgery. Slide tracheoplasty was undertaken in (12/15) with long-segment tracheal stenosis. 3/15 patients had a short-segment tracheal stenosis and were managed conservatively. The use of NIV occurred in 10/15 (66.67%) patients, all of whom had long-segment CTS. Pre-operative polysomnography (PSG) showed a median (±SD) obstructive apnoea/hypopnoea index (OAHI) of 14.6/hr (±6.2) which reduced to 7.2/hour (±4.2) on NIV prior to slide tracheoplasty. The median oxygen desaturation index (ODI) before NIV use was 15.3 (±19.4) episodes/hour, which reduced to 6.3 (±11) on NIV. The median period of NIV use was 5 [1-24 months] months. CONCLUSION: Patients with CTS have obstructed sleep disordered breathing. Trials of NIV are well-tolerated and improve sleep disordered breathing.

Abnormal preschool Lung Clearance Index (LCI) reflects clinical status and predicts lower spirometry later in childhood in cystic fibrosis.

BACKGROUND: Clinical and prognostic value of preschool Multiple Breath Washout (MBW) remains unclear. METHODS: Initial MBW results (Exhalyzer® D, EcoMedics AG) in preschool Cystic Fibrosis (CF) subjects (age 2-6 years) at a time of clinical stability were compared to (1) concurrent clinical status measures and (2) later spirometry outcomes. Abnormal Lung Clearance Index (LCI) was defined using published reference data (ULN for LCI 8.0). RESULTS: LCI was abnormal in 56% (28/50), with mean (SD) LCI 8.61(1.85) at age 4.71(1.3) years. Abnormal LCI was associated with higher dornase alfa use, previous positive bacterial cultures and pF508.del homozygous genotype. Later spirometry (n = 44; mean (SD) 2.3(0.5) years after MBW) demonstrated that abnormal initial preschool LCI was a strong predictor of lower later spirometry outcomes. CONCLUSION: Abnormal preschool LCI was associated with concurrent measures of clinical status and later spirometry deficits, suggesting early prognostic utility of MBW testing in this age range.

Can daytime measures of lung function predict respiratory failure in children with neuromuscular disease?

Neuromuscular disorders in children are a heterogeneous group of conditions with a variable age of presentation and overlapping clinical manifestations, many of which have progressive respiratory morbidity. Respiratory insufficiency occurs as a consequence of an imbalance between demands on the respiratory system and respiratory muscle capacity. Daytime measures of pulmonary function are used routinely in these children to assess respiratory status and monitor the consequences of the progression of muscle weakness. This review describes the current evidence for daytime pulmonary function tests and their ability to predict imminent respiratory morbidity.

Prevalence of anti-HCV antibodies in central India.

The study group screened for anti-HCV comprised 789 subjects of hepatitis, renal failure, thalassaemia and healthy voluntary blood donors coming from Central India during July 1992 to November 1995. The prevalence of HCV was low (4.85%) among 103 patients of acute viral hepatitis (AVH) while it was higher (25.64%) among 117 patients of chronic liver disease (CLD) with the highest rate of 31.57 percent in 57 patients of cirrhosis. The anti-HCV positivity among 101 patients with hepatic failure was around 10 percent. High risk groups such as chronic renal failure (CRF) patients mainly on haemodialysis and thalassaemics receiving multiple blood transfusions showed the prevalence of anti-HCV in 41.9 and 25.45 percent respectively. Only 1.78 percent of the 280 voluntary blood donors showed positivity for anti-HCV. Comparison of the data on HCV in the present study with data from other parts of India showed a wide variation in the different centers. The higher prevalence of HCV among CRF patients and thalassaemics indicates the need for screening of the blood units for anti-HCV before transfusion to these high risk patients.

Sero-diagnosis of schistosomiasis by ELISA test in an endemic area of Gimvi village, India.

Enzyme-linked immunosorbent assay (ELISA) was carried out in Gimvi village, India, using antigens derived from S. haematobium and S. mansoni adult worms. Patients excreting schistosome ova in urine elicited positive ELISA titres, whereas patients who were previously positive but are no longer passing viable eggs were negative for ELISA.

Micro test for assaying sensitivity of Plasmodium vivax in vitro.

A modified Rieckmann test was developed for assessing chloroquine sensitivity of Plasmodium vivax strains. This test envisages the evaluation of parasite growth with different concentrations of the drug and its comparison with controls, at the end of a 48-hour experimental period. Using this test, 16 strains were assessed for their chloroquine sensitivity. Twelve strains were found to be sensitive, and 4 were resistant to chloroquine.

An extensive community outbreak of enteropathogenic Escherichia coli O86: B7 gastroenteritis.

During the last 20 years numerous reports of hospital-associated epidemics of enteropathogenic Escherichia coli (EEC) gastroenteritis have appeared in the medical literature. Some of the reports suggest that EEC gastroenteritis can also be a community problem. However, community epidemics of EEC gastroenteritis comparable to those of other communicable diseases like cholera or typhoid are rare.An extensive epidemic of acute gastroenteritis occurred among children in Surat, Gujarat State, India, between 29 October and 3 December 1965. A total of 418 patients was hospitalized during this period: 344 of them were under 2 years of age. Faecal specimens from 81 children admitted between 25 November and 30 November were subjected to microbiological examination. Of these samples, 34 yielded pure cultures of E. coli O(86):B(7) and 1 yielded a pure culture of O(126):B(16). The cases occurred throughout the town including a rural ward. The morbidity rate for the total population was 0.16% and the age-specific morbidity rate for the 0-2-years age-group, was 0.98%; the highest morbidity rate (2.4%) was in the 6-11-months age-group in the urban wards. No definite mode of transmission of the disease could be established.