Spirometric changes in myelomeningocele patients after surgical correction of scoliosis.
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Biomedical subjects
Publications and source records attributed to K Paul.
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Prospective evaluation of cardiac function was undertaken in 21 patients with Kaposi's sarcoma (KS) and the acquired immune deficiency syndrome (AIDS) as part of a phase II clinical trial of 4'epirubicin. All patients were homosexual or bisexual males of median age 34 years (range 23-65). All patients had disseminated cutaneous involvement by KS and seven had systemic organ involvement. No patient had a history of cardiac symptoms, and physical examination of the cardiovascular system was within normal limits for all patients. The baseline ECG was normal in only nine patients (45%). Five had non-specific S-T segment and T-wave changes. Two had poor R-wave progression, and one patient each had incomplete right bundle branch block, left axis deviation, and voltage criteria suggestive of left ventricular hypertrophy. Radionuclide angiography was performed on all patients and compared to 12 age-matched, low-likelihood male controls. The mean ejection fraction (EF) at rest was 61.0 +/- 8.4%, and was not significantly different from the normal control value of 62.4 +/- 7.0%. Similarly the EF during exercise was 66.7 +/- 5.0%, again not significantly different from the control value of 67.8 +/- 5.8%. A drop in EF during exercise was seen in five of 15 KS patients compared to three of 12 controls. Chamber size was normal for both ventricles in only 14 patients (67%) with right ventricular dilatation seen in seven patients (33%). Wall motion abnormalities were also seen in nine patients (42.9%), with the right ventricle affected in eight patients (38.1%), chiefly involving the apex. No chamber size or wall motion abnormalities were seen in the control patients.(ABSTRACT TRUNCATED AT 250 WORDS)
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The clinical course and pulmonary function tests of individuals with severe Alpha-1-Antitrypsin (Alpha-1-AT) deficiency reveal a marked interindividual variability. 4 patients with PI type ZZ and 2 patients with PI type SZ had been identified by neonatal cholestasis. None had pulmonary symptoms at a mean age of 13 (range 9-16) years. Lung function tests disclosed signs of incipient pulmonary emphysema in one girl. Bronchial hyperreactivity had led to the diagnosis of Alpha-1 AT-deficiency (PI type ZZ) in another girl at the age of 7 years. The biochemical analysis of the serum of three children (two PI ZZ and one PI SZ) revealed additional protease inhibitors that may be effective as compensatory mechanisms. The lack of those protective factors as well as the presence of environmental hazards may contribute to the increased risk of emphysema in individual patients.
Both the clinical course of the homozygous alpha 1-PI deficiency and also the pulmonary function and measured clinical parameters in these children revealed very marked inter-individual fluctuations. In one child, the lung function revealed certain signs of incipient pulmonary emphysema. The biochemical parameters of two children revealed TIC/alpha 1-PI- and PEIC/alpha 1-PI ratios greater than 1. This indicates that, in addition to alpha 1-PI, further inhibitors may develop a compensatory effect.
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20 healthy full-term infants have been studied in their 2nd, 10th, 18th and 26th weeks of life in three standard situations: before feeding, in the course of milk drinking and after feeding. A polygraphic record of sucking, breathing, swallowing, the oculomotor activity, the EEG and vocalization has been taken simultaneously with a video-recording of the infant's behaviour. Physiological patterns and developmental changes of sucking, breathing and swallowing are described. These activities are considered as fine motor activity of the earliest age. The possibility of using polygraphic recordings of these variables for the diagnosis of the neurological syndrome called feeding problems is discussed. Further, behavioural activities in above mentioned three situations and their developmental changes, are described. None of the infants, not even the smallest ones, were asleep in the beginning of feeding. On the contrary, their eyes were open and there were more eye movements during feeding than in other situations. In the authors' opinions, the situation of feeding has, especially in the first days and weeks of life, a special significance for the development of social interactions between the infant and the person taking care of him. The behaviour of a regularly fed infant in the second quarter of his first year seems to be no longer influenced in the periods proceeding and following the feeding act by the prandial cycle but rather by cognitive needs. The influences involved in the regulation of the behaviour of the youngest infants are discussed.
In ten healthy neonates the influence of blue light was analyzed, using polygraphy. In each child the polygrams were assessed under two conditions--without irradiation and without irradiation. The length of the sleep, cycle, the length of quit and paradoxical sleep, the respiration rate during both behavioural states, the frequency of rapid eye movements during paradoxical sleep, the incidence of EEG activity corresponding to age and the EMG activity during both behavioural states did not change significantly under the influence of light. Only the number of apnoeic intervals during paradoxical sleep under blue light increased significantly. The authors assessed, using polygraphy, also a control group of ten healthy mature neonates. The two groups of children did not differ significantly in the investigated parameters. The results revealed a great inter- and intraindividual variability which suggests the low stability of the child's behaviour during the first days of life. Among the numerous stimuli of the external and internal environment which modify the behaviour during the period of postpartum adaptation, the influence of blue light did not play a significant role under the above conditions.
A library containing more than 80% of the Vibrio cholerae genome was constructed by cloning BamH1 restriction fragments into pBR322. Using interspecific complementation of an Escherichia coli recA mutant with plasmids containing the gene bank of V. cholerae, a recA-like gene was identified. The recombinant plasmid, designated as pDP145, contained a 1.45 kb segment of V. cholerae DNA which codes for a protein of molecular weight 39,000. The product of this gene confers methyl methane sulphonate resistance on the E. coli recA mutant, suppresses its ultraviolet (UV) light sensitive phenotype and has proteolytic activity on the phage lambda repressor. Induction of a 39,000 dalton protein in UV-irradiated V. cholerae cells was demonstrated.
Fifty-three patients with inoperable adenocarcinoma of the lung were treated with 5-fluorouracil, Adriamycin, and mitomycin-C (FAM) in two dose schedules: schedule 1--5-FU 600 mg/m2 days 1, 8, 29, and 36 and Adriamycin 30 mg/m2 days 1 and 29 and mitomycin-C 10 mg/m2 day 1 repeated every 8 weeks. Schedule 2--5-FU 600 mg/m2 and Adriamycin 30 mg/m2 days 1 and 22 and mitomycin-C 10 mg/m2 day 1, repeated every 6 weeks. There were 28 males and 25 females, median age 57 years. There were 11 patients with limited disease and 42 patients with extensive disease. Ten patients had ECOG performance status 0; 34, 1; 6, 2; and 3, 3. Ten patients (18.8%) achieved a partial response. Response was seen in four limited disease patients (36.3%) and only six extensive disease patients (11.9%). The median duration of response was 33 weeks (range 17-50 weeks), and the median survival of these patients was 89.7 weeks. Sixteen patients achieved stable disease with a median response duration of 22 weeks (range 8-91 weeks). The median survival of the entire group was 32.6 weeks. The FAM regimen was tolerated well, with only mild gastrointestinal symptoms and moderate myelosuppression. The granulocyte nadir was less than 1,000 in 8% of patients and 1,000-1,500 in 20%. Only 5% of patients had a platelet count under 100,000 and three patients required red cell transfusions during treatment. These results indicate that FAM chemotherapy may be administered as an outpatient with minimal toxicity and can cause objective tumor regression in patients with adenocarcinoma of the lung.
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Cartilaginous tumours of the larynx are uncommon. For the first time, a case report of a congenital chondroma of the larynx in a two-day-old girl is presented. Only a few hours after birth the tumour caused inspiratory stridor and dyspnoea and secondarily pneumothorax and atelectasis of the lung. After intubation and tumour resection the atelectasis and the pneumothorax disappeared spontaneously. Chondromas of the larynx should be considered in different diagnosis of congenital anomalies of the larynx.
Multiple arteriovenous fistulae of the entire left pulmonary lobe were found to be the cause of increasing cyanosis on exertion and hour-glass nails in a 19-year-old patient. The right-to-left shunt amounted to 16%. The 2 possible forms of treatment were embolization of the arteriovenous fistulae or pneumonectomy. The first procedure was unsuitable because of the diffuse spread of the fistulae. In order to avoid all the early and late complications associated with a pneumonectomy, an unusual method was adopted: The left pulmonary artery was transected and the lung was left in place as a "physiological filling". Both a subjective and objective improvement could already be seen in the immediate postoperative period. At the last follow-up examination, 16 months after the operation, this was still the case.
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