BRONCHIECTASIS IN CHILDHOOD

PEDIATRICS ◽  
1949 ◽  
Vol 4 (1) ◽  
pp. 21-46
Author(s):  
C. ELAINE FIELD
Keyword(s):  
Main Bronchus ◽  
Age At Onset ◽  
Lower Lobe ◽  
Normal Children ◽  
Parental History ◽  
Abnormal Chest ◽  
Characteristic Features ◽  
Chest Deformities ◽  
Bronchial Dilatation ◽  
First Year Of Life

In a study of 160 cases of irreversible bronchiectasis in childhood the following observations have been made: From the history given by the parents, it was found that the age at onset of symptoms lay in the first year of life in approximately one fifth of the cases, the number thereafter declining with advancing age (except for a slight and possibly insignificant rise at age 5). In 55.6% of cases the parental history associated the onset of symptoms with an attack of pneumonia or pertussis. The characteristic features of the disease included a constant cough with or without sputum (this is often swallowed by children). Haemoptysis was rare, but in 33.1% of cases there were associated asthmatic symptoms. More children were underweight than overweight for age and many had abnormal chest deformities. Clubbing occurred in 43.7% and when present was diagnostic of irreversible bronchiectasis in this series. Physical signs in the chest were variable, but the most useful diagnostic finding was localized rales on deep inspiration over the suspected area of lung. In comparison with nonpulmonary and normal children, there was a marked increased incidence of pneumonia at all ages. There was, however, no suggestion that bronchiectasis increased or decreased susceptibility to tuberculosis. Sinusitis is frequently associated with bronchiectasis although its exact relationship remains obscure. In diagnosis, roentgenographic findings are frequently inconclusive, unless confirmed by bronchogram. Bronchography is by far the most important diagnostic procedure and is an invaluable method of studying the development of the disease. Tubular dilatation was the commonest type of bronchiectasis. In 85.6% of cases the disease was situated in the left lower lobe, and in 65.6%, in the lingula lobe, but in no case was the lingula affected as the only lobe. The disease has shown a predilection for lobes whose bronchi are directed upwards against gravity towards the main bronchus and those which have an anatomic peculiarity impeding drainage. Massive collapse of the lung was associated with bronchiectasis in 74 (46.3%) cases. It is important to recognize in children that bronchial dilatation may be reversible. In the diagnosis of irreversibility, duration of the dilatation and its contour are helpful. Bronchoscopy was not helpful in diagnosis or localization of bronchiectasis. The sedimentation rate was frequently raised, but was of little help in assessing activity of the disease. Alterations in the blood count were few and usually only of significance in severe active cases of the disease. Complications were infrequent, the commonest being pneumonia which occurred in 16.3% of cases.

Epidemiology of Physician-Diagnosed Allergic Rhinitis in Childhood

PEDIATRICS ◽  
1994 ◽  
Vol 94 (6) ◽  
pp. 895-901
Author(s):  
Anne L. Wright ◽  
Catharine J. Holberg ◽  
Marilyn Halonen ◽  
Fernando D. Martinez ◽  
Wayne Morgan ◽  
...  
Keyword(s):  
Risk Factors ◽  
Allergic Rhinitis ◽  
Immunoglobulin E ◽  
Age At Onset ◽  
First Year ◽  
Healthy Children ◽  
Early Introduction ◽  
Maternal Cigarette Smoking ◽  
History Of ◽  
First Year Of Life

Objective. To investigate the natural history of and risk factors for allergic rhinitis in the first 6 years of life. Methods. Parents of 747 healthy children followed from birth completed a questionnaire when the child was 6 years old. Data were obtained regarding physician-diagnosed allergic rhinitis (PDAR), associated symptoms, and age at onset. Risk-factor data were taken from earlier questionnaires, and data regarding immunoglobulin E (IgE) and skin-test reactivity were obtained at age 6. Results. By the age of 6, 42% of children had PDAR. Children whose rhinitis began in the first year of life had more respiratory symptoms at age 6 and were more likely to have a diagnosis of asthma. Early introduction of foods or formula, heavy maternal cigarette smoking in the first year of life, and higher IgE, as well as parental allergic disorders, were associated with early development of rhinitis. Risk factors for PDAR that remained significant in a multivanate model included maternal history of physician-diagnosed allergy (odds ratio: 2.2, 95% confidence interval: 1.35-3.54), asthma in the child (4.06, 2.06-7.99), and IgE greater than 100 IU/mL at age 6 (1.93, 1.18-3.17). The odds for atopic as opposed to nonatopic PDAR were significantly higher only among those with high IgE and those who had dogs. Conclusion. Allergic rhinitis developing in the first years of life is an early manifestation of an atopic predisposition, which may be triggered by early environmental exposures.

A NOMOGRAM FOR THE X-RAY EVALUATION OF SOME MORPHOLOGICAL ANOMALIES OF THE PELVIS IN THE DIAGNOSIS OF MONGOLISM

PEDIATRICS ◽  
1963 ◽  
Vol 32 (6) ◽  
pp. 1074-1077
Author(s):  
F. B. Nicolis ◽  
G. Sacchetti
Keyword(s):  
Quantitative Estimation ◽  
Numerical Calculations ◽  
First Year ◽  
Discriminant Functions ◽  
Normal Children ◽  
X Ray ◽  
Morphological Anomalies ◽  
First Year Of Life

Some morphological anomalies of the pelvis were radiologically evaluated in 25 mongoloid children within the first year of life, in comparison with 45 normal children, through the measurements of the acetabular and iliac angles. The results were statistically analyzed according to the model of discriminant functions, and the combination of the two measurements which gives the best discrimination between mongoloid and normal children was established; on this basis, a nomogram is presented, where-by a quantitative estimation of the morphological anomalies can be made, for diagnostic purposes, without numerical calculations and with a low error.

scholarly journals RPE65-related retinal dystrophy: mutational and phenotypic spectrum in 45 affected patients

2021 ◽  
Author(s):  
R Lopez-Rodriguez ◽  
E Lantero ◽  
F Blanco-Kelly ◽  
A Avila-Fernandez ◽  
I Martin Merida ◽  
...  
Keyword(s):  
Age At Onset ◽  
Disease Onset ◽  
Retinal Dystrophy ◽  
Rank Test ◽  
Ophthalmological Examination ◽  
Symptomatic Disease ◽  
Inherited Retinal Dystrophies ◽  
Kaplan Meier ◽  
Almost All ◽  
First Year Of Life

ABSTRACTBackgroundBiallelic pathogenic RPE65 variants are related to a spectrum of clinically overlapping inherited retinal dystrophies (IRD). Most affected individuals show a severe progression, with 50% of patients legally blind by 20 years of age. A better knowledge of the mutational spectrum and the phenotype-genotype correlation in RPE65-related IRD is needed.MethodsForty-five affected subjects from 27 unrelated families with a clinical diagnosis of RPE65-related IRD were included. Clinical evaluation consisted on self-reported ophthalmological history and objective ophthalmological examination. Patients’ genotype was classified accordingly to variant class (truncating or missense) or to variant location at different protein domains. Main phenotypic outcome was age at onset (AAO) of the symptomatic disease and a Kaplan–Meier analysis of disease symptom event-free survival was performed.ResultsTwenty-nine different RPE65 variants were identified in our cohort, 7 of them novel. Most frequent variants were p.(Ile98Hisfs*26), p.(Pro111Ser) and p.(Gly187Glu) accounting for the 24% of the detected alleles. Patients carrying two missense alleles showed a later disease onset than those with 1 or 2 truncating variants (Log Rank test p<0.05). While the 60% of patients carrying a missense/missense genotype presented symptoms before or at the first year of life, almost all patients with at least 1 truncating allele (91%) had an AAO ≤1 year (p<0.05).ConclusionOur findings suggest an association between the type of the RPE65 carried variant and the AAO. Thus, our results provide useful data on RPE65-associated IRD phenotypes which may help to improve clinical and therapeutic management of these patients.

Upper and Middle Lobe Atelectasis

Chest Imaging ◽  
2019 ◽  
pp. 93-97
Author(s):  
Christopher M. Walker
Keyword(s):  
Lung Cancer ◽  
Main Bronchus ◽  
Lower Lobe ◽  
Outpatient Setting ◽  
Middle Lobe ◽  
Inpatient Setting ◽  
Central Mass ◽  
Contrast Enhanced Ct ◽  
Left Hemidiaphragm ◽  
Contrast Enhanced

Upper and middle lobe atelectasis discusses the radiographic and computed tomography (CT) manifestations of upper and middle lobe atelectasis. The most common radiographic signs of right upper lobe atelectasis include upward and medial displacement of the minor fissure, superior displacement of adjacent structures such as the hilum and main bronchus, and ipsilateral shift of the mediastinal structures. The S sign of Golden results from a centrally obstructing lung cancer as the cause of the atelectasis and manifests as a reverse S configuration of the minor fissure outlined by atelectatic lung and central mass. Left upper lobe atelectasis manifests with a veil-like opacity on frontal radiography with leftward shift of upper mediastinal structures such as the trachea and upward shift of the left main bronchus and left hemidiaphragm. The Luftsichel sign or air crescent sign may be seen and represents the hyperexpanded superior segment of the left lower lobe outlining the transverse aortic arch. Lobar atelectasis in the inpatient setting is most commonly secondary to an obstructing mucus plug. Lobar atelectasis in the outpatient setting is often a heralding sign of a centrally obstructing lung cancer and should be further evaluated with contrast-enhanced CT and/or bronchoscopy.

Parental family history of dementia in relation to subclinical brain disease and dementia risk

Neurology ◽  
2017 ◽  
Vol 88 (17) ◽  
pp. 1642-1649 ◽  
Author(s):  
Frank J. Wolters ◽  
Sven J. van der Lee ◽  
Peter J. Koudstaal ◽  
Cornelia M. van Duijn ◽  
Albert Hofman ◽  
...  
Keyword(s):  
Risk Factors ◽  
Family History ◽  
Genetic Risk ◽  
Brain Mri ◽  
Age At Onset ◽  
Age At Diagnosis ◽  
Parental History ◽  
Dementia Risk ◽  
History Of ◽  
Parental Family

Objective:To determine the association of parental family history with risk of dementia by age at onset and sex of affected parent in a population-based cohort.Methods:From 2000 to 2002, we assessed parental history of dementia in participants without dementia of the Rotterdam Study. We investigated associations of parental history with risk of dementia until 2015, adjusting for demographics, cardiovascular risk factors, and known genetic risk variants. Furthermore, we determined the association between parental history and markers of neurodegeneration and vascular disease on MRI.Results:Of 2,087 participants (mean age 64 years, 55% female), 407 (19.6%) reported a history of dementia in either parent (mean age at diagnosis 79 years). During a mean follow-up of 12.2 years, 142 participants developed dementia. Parental history was associated with risk of dementia independently of known genetic risk factors (hazard ratio [HR] 1.67, 95% confidence interval [CI] 1.12–2.48), in particular when parents were diagnosed at younger age (<80 years: HR 2.58, 95% CI 1.61–4.15; ≥80 years: HR 1.01, 95% CI 0.58–1.77). Accordingly, age at diagnosis in probands was highly correlated with age at diagnosis in their parents <80 years (r = 0.57, p = 0.001) but not thereafter (r = 0.17, p = 0.55). Among 1,161 participants without dementia with brain MRI, parental history was related to lower cerebral perfusion and higher burden of white matter lesions and microbleeds. Dementia risk and MRI markers were similar for paternal and maternal history.Conclusions:Parental history of dementia increases risk of dementia, primarily when age at parental diagnosis is <80 years. Unexplained heredity may be attributed in part to cerebral hypoperfusion and small vessel disease. We found no evidence of preferential maternal compared to paternal transmission.

scholarly journals Sex differences in large conducting airway anatomy

2018 ◽  
Vol 125 (3) ◽  
pp. 960-965 ◽  
Author(s):  
Paolo B. Dominelli ◽  
Juan G. Ripoll ◽  
Troy J. Cross ◽  
Sarah E. Baker ◽  
Chad C. Wiggins ◽  
...  
Keyword(s):  
Sex Differences ◽  
Main Bronchus ◽  
Lower Lobe ◽  
Left Lower Lobe ◽  
Smoking History ◽  
Men And Women ◽  
Previous Evidence ◽  
Healthy Women ◽  
Luminal Area ◽  
Conducting Airways

Airway luminal area is the major determinant of resistance to airflow in the tracheobronchial tree. Women may have smaller central conducting airways than men; however, previous evidence is confounded by an indirect assessment of airway geometry and by subjects with prior smoking history. The purpose of this study was to examine the effect of sex on airway size in healthy nonsmokers. Using low-dose high-resolution computed tomography, we retrospectively assessed airway luminal area in healthy men ( n = 51) and women ( n = 73) of varying ages (19–86 yr). Subjects with a positive smoking history, cardiopulmonary disease, or a body mass index > 40 kg/m2 were excluded. Luminal areas of the trachea, right and left main bronchus, bronchus intermediate, left and right upper lobes, and the left lower lobe were analyzed at three discrete points. The luminal areas of the conducting airways were ~26%–35% smaller in women. The trachea had the largest differences in luminal area between men and women (298 ± 47 vs. 195 ± 28 mm2 or 35% smaller for men and women, respectively), whereas the left lower lobe had the smallest differences (57 ± 15 vs. 42 ± 9 mm2 or 26% smaller for men and women, respectively). When a subset of subjects was matched for height, the sex differences in airway luminal area persisted, with women being ~20%–30% smaller. With all subjects, there were modest relationships between height and airway luminal area ( r = 0.73–0.53, P < 0.05). Although there was considerable overlap between sexes, the luminal areas of the large conducting airways were smaller in healthy women than in men. NEW & NOTEWORTHY Previous evidence for sex differences in airway size has been confounded by indirect measures and/or cohorts with significant smoking histories or pathologies. We found that central airways in healthy women were significantly smaller (~26%–35%) than men. The significant sex-difference in airway size was attenuated (20%–30% smaller) but preserved in a subset of subjects matched for height. Over a range of ages, healthy women have smaller central airways than men.

47, XXX: What Is the Prognosis?

PEDIATRICS ◽  
1988 ◽  
Vol 82 (4) ◽  
pp. 619-630
Author(s):  
Mary G. Linden ◽  
Bruce G. Bender ◽  
Robert J. Harmon ◽  
David A. Mrazek ◽  
Arthur Robinson
Keyword(s):  
Speech Therapy ◽  
Sex Chromosome ◽  
First Year ◽  
Speech And Language ◽  
Prognostic Information ◽  
School Problems ◽  
Normal Children ◽  
Early School ◽  
A Prospective Study ◽  
First Year Of Life

Eleven unselected 47, XXX girls, now 15 to 22 years of age, have been observed from birth in a prospective study of children with sex chromosome anomalies. A description of their growth and development is presented. The 47, XXX infants were not generally distinguishable from chromosomally normal children in the first year of life, even though there was a slight delay in neuromotor development. By 2 years of age, developmental delays in speech and language often became evident, and speech therapy was often necessitated in the preschool years. Early school problems included speech and language deficiencies, lack of coordination, poor academic performance, and immature behavior; these persisted throughout the school years. By high school age, a 47, XXX girl was generally tall and often subject to somatic complaints. Sexual development was generally normal. Seven of the 11 propositae had a diagnosed psychiatric disorder or disturbance at some time during adolescence. Variability within this syndrome is great; one proposita is in college and another is mentally retarded. The frequency of the diagnosis of the 47, XXX karyotype by genetic amniocentesis is estimated to be 1/1000, the same incidence as in the newborn population. Expectant parents must be counseled as to the significance of this karyotype and prognostic information must be given. Suggested guidelines are included.

scholarly journals Implantation of 125I radioactive seeds via c-TBNA combined with chemotherapy in an advanced non-small-cell lung carcinoma patient

2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Ai-Gui Jiang ◽  
Hui-Yu Lu ◽  
Zhong-Qi Ding
Keyword(s):  
Lung Carcinoma ◽  
Main Bronchus ◽  
Small Cell Lung Carcinoma ◽  
Lower Lobe ◽  
Small Cell ◽  
Advanced Lung Cancer ◽  
Small Cell Lung ◽  
Transbronchial Needle Aspiration ◽  
Cell Lung Carcinoma ◽  
Radioactive Seeds

Abstract Background The critical management of advanced non-small-cell lung carcinoma (NSCLC), especially when complicated by severe airway stenosis, is difficult and often leads to high clinical risks and medical costs. Case presentation A 51-year-old previously healthy male was admitted to the Department of Respiratory and Critical Care Medicine, Taizhou People’s Hospital, in November 2018 for haemoptysis and difficulty breathing during a 15-d period. Following admission, chest computed tomography (CT) showed a large mass in the left hilum with atelectasis in the left upper lobe and obstructive pneumonia in the left lower lobe. Bronchoscopy revealed that the lesions occurred in the distal segment of the left main trachea, with occlusion of the left upper bronchus and significant narrowing of the lower bronchus. A basal mucosal biopsy of the lump tissue was performed after haemostasis treatment with sub-plasma coagulation (APC), and squamous lung carcinoma was confirmed. Following the final diagnosis, the patient was successfully treated with implantation of 125I radioactive seeds via transbronchial needle aspiration (c-TBNA) combined with chemotherapy. Conclusion We believe that implantation of 125I radioactive seeds via c-TBNA is an effective treatment for patients with advanced lung cancer and those presenting with severe and mixed main bronchus stenosis.

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