Ultrasonic study of pancreas in cystic fibrosis.
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Biomedical subjects
Publications and source records attributed to I Dab.
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In 76 children, 102 unilateral bronchograms were compared with the results of chest radiography and Tc-99m perfusion scintigraphy to assess the presence of bronchiectasis. Additional Kr-81m ventilation lung scintigraphy was obtained in 16 patients. For the chest radiograph the sensitivity was 73% and the specificity was 76%. For lung scintigraphy the sensitivity was 92% and the specificity 60%. When both chest radiography and lung scintigraphy were considered, sensitivity reached 96% and specificity fell to 53%. Forty per cent of the bronchrograms which were performed might have been avoided on the basis of the normal chest radiograph and lung scintigraphy. The combination of lung scintigraphy and chest radiography is an excellent screening method when a decision on the advisability of bronchography is being taken in children.
The absolute and the percentage plasma NEFA pattern of healthy children, Cystic Fibrosis (C.F.) patients and their parents have been determined (table I, III, V) and compared with those of age and sex matched control subjects (table II, Iv, V). There is a striking difference in the percentage plasma NEFA pattern of C.F. patients with pancreatic insufficiency: palmitic, palmitoleic and oleic acid are significantly increased while linoleic, linolenic and stearic acid are decreased (table IV). Four C.F. children without pancreatic involvement have the same abnormal NEFA pattern (fig. 3). The overlapping areas of the distributions of both palmitoleic and linoleic acid (as shown in fig. 2a and B) are small for healthy and C.F. subjects. About 90% of the C.F. subjects examined show an abnormal palmitoleic and/or linoleic acid concentration. Tested individually, recognition of this typical C.F. pattern involves a possible error of 15% (fig. 3). A correlation exists between the abnormality of the plasma NEFA pattern in C.F. patients and the severity of pulmonary disease. The percentage plasma NEFA pattern of C.F. parents is modified by an increase of linoleic acid and a decrease of saturated fatty acids (table V, fig. 1a, B). This is possibly due to an higher dietary intake of polyunsaturated fatty acids.
A long-term study has been performed on 285 lung perfusion scintigrams obtained from 113 patients with cystic fibrosis. Transverse and longitudinal comparisons with clinical and radiological scores, as well as retrospective analysis of the deceased patients, were the methods used in order to evaluate the importance of the scintigraphic images. It appears that lung scintigraphy is the best index of the regional lung impairment, and contributes, as does a chest radiograph, to the early detection of lung lesions, the two methods being complementary. The survival rate of CF patients reached 0.80 at 9 yr when initial scintigraphy was normal or only moderately impaired, but fell to 0.18 when severe lesions were seen on the first scintigrams.
The authors draw the attention on the existence of an "Immotile-cilia Syndrome" in patients with chronic respiratory infections of unknown origin. The study of the ultra structure of the ciliae (TEM, SEM) in a case of Kartagener Syndrome supports the existence of an "Immotile-cilia Syndrome".
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The specific airway resistance (SRaw) is a relatively precise parameter of bronchial quality and in normal children its relation with body length, although very significant (P less than 0.001), is so poor that it can safely be disregarded (Fig. 4). Also the dispersion around the mean value remains stable at any body size (Fig. 4). Contrasting with the inverse relation between airway resistance (Raw) and lung volume, SRaw does not show any systematic variation with lung filling (Fig. 5). These data are consistent with the new formulas: SRaw = tg beta (PB - PH2O) and Raw = SRaw/TGV in which tg beta stands for relation between the plethysmographic box volume and breathing flow fluctuations (Fig. 2); PB - PH2O represents the ambient pressure corrected for water vapor pressure at body temperature; TGV is the thoracic gas volume at mean expiratory level. In disease SRaw varies more rapidly and markedly with any alteration of the airways than does Raw (Fig. 6). Consequently, a normal value for SRaw would indicate that both Raw and TGV must be in a normal range.
Thirty-one cases of persistent stridor during infancy, which on study proved to be of congenital origin, were analyzed. The breakdown of these cases is as follows: 4 laryngotracheomalacia, 3 vascular anomalies, 4 angiomas, 1 mucous membrane, 1 laryngeal cyst. The remaining cases (18) belong to the so-called "benign" stridor group in that no specific etiology could be demonstrated and in that evolution was spontaneously favorable. In every case of stridor, the precise underlying cause should be looked for. In addition to clinical assessment the investigation of an infant with stridor calls for the following methods of examination: chest x-ray; larynx x-ray (anterior and lateral view) during iopneumography should be confined to specific cases.
A simple algebraic manipulation of known formulas leads to a direct expression for the specific airway resistance (SRaw) which precludes separate measurements of the airway resistance (Raw) and the thoracic gas volume (TGV). The equation is: SRaw = tg beta (PBar - PH2O) e2, in which tg beta stands for the relation between the plethysmographic box volume and pneumotachograph flow fluctuations; PBar - PH2O is the barometric por graphical units. Thanks to this new formula SRaw can now be easily measured with great precision and quickly calculated, even in the case of those children (Table 1) with whom this was not previously possible. No further cooperation is needed than breathing at a normal rate through mouthpiece into a whole-body plethysmograph. The intraindividual coefficient of variation (CV) for SRaw is about 8% and never exceeds 15% (Table 1).
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The radionuclide right ventricular ejection fraction (RVEF) determined by means of Krypton-81m represents a simple, noninvasive, and accurate procedure to quantify the right ventricular contractility. This procedure was applied to 25 young patients with cystic fibrosis. The RVEF tended to decrease with the progression of the lung disease, as assessed by the clinical S-K score, the degree of the defects on lung scintigraphy, the PaO2, and the lung function tests. However, the decrease of RVEF in patients with marked lung function tests. However, the decrease of RVEF in patients with marked lung involvement was moderate, and terminal lung disease was sometimes associated with normal right heart contractility.
Thirty-eight children, aged from a few weeks to 7 years, with severe chronic pulmonary disease and without gastrointestinal symptoms, were investigated for gastroesophageal reflux (GER), using prolonged pH probe monitoring and gastroesophageal scintiscanning. All treatments were discontinued before testing. GER was found in 24 patients (63%) (group I) and it was not observed in 14 patients (group II). All patients of group I received antireflux treatment, consisting of cisapride; in 22 of 24 patients, GER was controlled, as indicated by improvement of either pH monitoring or scintiscanning, or both. Eighteen of these 22 (82%) had remission of their pulmonary disease, and only two patients of group II (14%) had spontaneous remission of the respiratory symptoms. We concluded that GER was probably the cause of the respiratory disease in 63% of our patients, since treatment of GER was followed by disappearance of the respiratory complaints in most of them. The combination of gastroesophageal scintiscanning and pH probe study improved the diagnostic accuracy.
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The purpose of this work was to evaluate bone marrow abnormalities in the lower limbs of patients with cystic fibrosis by means of MR imaging. Eight patients with cystic fibrosis ranging in age from 16 to 35 years (average age 25.1 years) were evaluated with MR imaging of the lower extremities. T1 weighted spin echo sequences were obtained in all patients. Pelvis, femora and tibia were imaged in the coronal plane whereas the feet were imaged in the sagittal plane. The studies were independently evaluated by two musculoskeletal radiologists. The images were not mixed with disease-free images, but the readers were completely unaware of clinical data. Distribution of haematopoietic and fatty marrow was graded on a 5-point scale. In five patients, both observers considered marrow distribution as probably or definitely abnormal relative to their age. Although our findings are very preliminary, our investigation suggests that cystic fibrosis may be added to the list of disorders in which bone marrow abnormalities may be observed.
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Residual volume (RV), functional residual capacity (TGV) and total lung capacity (TLC) have been measured in 42 adolescents aged 16-22 years (22 young men and 20 young women) and in 60 children aged 12-15 years (29 boys and 31 girls) who were selected as having the same ranges of heights. TLC and TGV were related to height in each age and sex group; the slope of the regression curves did not differ between subgroups. RV was related to height in the boys and girls and not in the adolescents; however the number of subjects in each group was small. Relative to height the residual volumes of boys and girls were similar and in both sexes were larger than those for adolescents. Amongst the latter the values for young women were smaller than those for young men. The possible mechanisms are discussed.