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[A case of traumatic diaphragmatic hernia repaired 40 years after the injury].

A 65-year-old male complaining dyspnea on exertion and epigastric discomfort was diagnosed to have left traumatic diaphragmatic hernia elsewhere and referred to Keio university Hospital for treatment. He had thoracicio-abdominal trauma which was conservatively treated, 40 years ago. Because he was symptomatic and the possibility of future strangulation could not be denied, surgical repair of the hernia was performed. The defect in the diaphragm was too large to be directly sutured and it was repaired with GORE-TEX patch. The gastric fundus partially prolapsed in the paraesophageal region, but the diaphragm was repaired quite satisfactorily otherwise. Pulmonary function and dyspnea on exertion improved.

Aged↗

Thoracoscopic repair for congenital diaphragmatic hernia: lessons from 45 cases.

PURPOSE: To describe the surgical technique, initial results, and overview indications of thoracoscopic repair of congenital diaphragmatic hernia (CDH). MATERIALS AND METHODS: A retrospective review was undertaken of patients with CDH who underwent thoracoscopic repair by the same surgeon from January 2001 to January 2005. Patients underwent surgery under general anesthesia. Reduction of the hernia contents was carried out using 1 optical trocar and 2 operating trocars. Pleural insufflation with carbon dioxide was maintained at a pressure of 2 to 4 mm Hg. The hernia defect was repaired using nonabsorbable interrupted sutures with extracorporeal knots. RESULTS: There were 45 patients, including 29 boys and 16 girls. Among 19 newborn patients, there were 13 patients younger than 7 days. The other 26 patients were infants and elders. The hernia was located in the left side in 37 patients and in the right side in 8 patients. The mean operative time was 54 minutes. Conversion was required in 4 patients. There were no complications. However, there were 2 postoperative deaths. CONCLUSIONS: Thoracoscopic repair is feasible and safe for children with CDH, including selective newborn. The technique causes minimal trauma, results in good respiratory function, and promotes early recovery.

Aged↗

Recovery after unilateral phrenic injury associated with coronary artery revascularization.

Hemidiaphragmatic paralysis occurs in some patients following CAB surgery, possibly related to an intraoperative stretch or cold-induced phrenic injury. To determine the time and extent of recovery of phrenic nerve function, we studied five patients with left phrenic paresis or paralysis after CAB. The FVC, FEV1, Pmax and PEmax pressures, latency of conduction and amplitude of CDAP with phrenic nerve stimulation, and diaphragmatic excursion during fluoroscopy were measured for 12 months after CAB. Left phrenic paralysis was substantiated in four of five patients, and paresis was present in the other patient. Recovery of the left phrenic nerve occurred in all patients, complete in one and partial in four, but was delayed and continued for up to 12 months. We conclude that phrenic nerve recovery is delayed after CAB-associated injury and may be incomplete up to 14 months later, in keeping with rates of regeneration of other peripheral nerves.

Action Potentials↗

Quantitative left ventriculography: methods of assessment of the regional contractility.

To compare different approaches to the quantitative analysis of regional left ventricular (LV) function, six different protocols with various long axis definitions, with or without alignment, with radial or hemiaxial segmental definitions were used. Study group consisted of 20 patients with single vessel coronary artery disease after Q-wave anterior myocardial infarction (MI) and 20 patients after Q-wave diaphragmatic MI. Control group consisted of 100 patients. Analytic protocol with the long axis drawn between the apex of the LV and the center of aortic valve plane, radial coordinate system originating from the midpoint of the long axis and alignment of the long axes in systole and diastole, was found to be most sensitive and specific for detection of both anterior and diaphragmatic contraction abnormalities. Original method to measure both severity and length of the regional contraction abnormality is suggested.

Adult↗

Compensatory growth of porcine right lungs after chronic rejection of transplanted left lungs.

Neonatal lung hypoplasia is frequently a fatal condition often associated with congenital diaphragmatic hernia. Unilateral lung transplantation rarely has been performed for this indication, although it is a potential solution. It is not known whether the transplant needs to function permanently or to act as a bridge until the native lung develops. It is also not known whether the native lung will grow in the face of an immunosuppressed state and chronic rejection of the transplanted lung. We therefore developed a porcine model of left lung rejection to study this. Infant swine underwent left lung transplantation. Chronic rejection occurred in all, resulting in nonfunction of the transplanted lung. The right lungs of these animals were compared with the right lungs of size-matched and age-matched control animals not given immunosuppressive treatment and not undergoing transplantation. There were no differences in terms of the functional residual capacity, airway compliance, and airway resistance among the groups. There was a significant increase in the pulmonary vascular resistance in the animals with transplanted lungs. There was also a significant increase in the lung weight in these animals. Unilateral pneumonectomies were done in 4 infant pigs to serve as controls. Three of the 4 did not survive the operation because of acute pulmonary failure. In conclusion, the study group exhibited evidence of compensatory growth that was not seen in the control animals, as shown by the increase in lung weight. This suggests that contralateral lung growth occurs in a growing animal, despite the effects of immunosuppression therapy and chronic rejection of the transplanted lung.

Animals↗

Influence of the upper airway on breathing pattern and expiratory time constant in unanesthetized dog pups.

Unanesthetized dog pups (2 to 31 days old) respond to sudden opening of a tracheal cannula to atmospheric pressure with a marked increase in breathing frequency. This response is achieved with a 25% decrease in inspiratory and 40% decrease in expiratory times. Expiratory thyroarytenoid muscle activity increased concomitantly, while inspiratory diaphragmatic and posterior cricoarytenoid muscle activities were reduced. These responses are interpreted as a compensatory mechanism for maintenance of an elevated end-expiratory lung volume with functional loss of the upper airway. The changes in expiratory time and thyroarytenoid muscle activity were not observed when positive pressure was applied at the trachea. The expiratory time constant was assessed during spontaneous breathing. The mean value was twice as long during nasal breathing than during tracheal breathing. The nasal value was substantially increased when the thyroarytenoid muscle was active during expiration.

Animals↗

Progressive cardiomyopathy with intercalated disc disorganization in a rat model of Becker dystrophy.

Becker muscular dystrophy (BMD) is an X-linked disorder due to in-frame mutations in the DMD gene, leading to a less abundant and truncated dystrophin. BMD is less common and severe than Duchenne muscular dystrophy (DMD) as well as less investigated. To accelerate the search for innovative treatments, we developed a rat model of BMD by deleting the exons 45-47 of the Dmd gene. Here, we report a functional and histopathological evaluation of these rats during their first year of life, compared to DMD and control littermates. BMD rats exhibit moderate damage to locomotor and diaphragmatic muscles but suffer from a progressive cardiomyopathy. Single nuclei RNA-seq analysis of cardiac samples revealed shared transcriptomic abnormalities in BMD and DMD rats and highlighted an altered end-addressing of TMEM65 and Connexin-43 at the intercalated disc, along with electrocardiographic abnormalities. Our study documents the natural history of a translational preclinical model of BMD and reports a cellular mechanism for the cardiac dysfunction in BMD and DMD offering opportunities to further investigate the organization role of dystrophin in intercellular communication.

Animals↗

Regional intercostal activity during coughing and vomiting in decerebrate cats.

Regional variations in the discharge patterns of the internal and external intercostal muscles of the middle and caudad thorax were studied in decerebrate, spontaneously breathing cats during coughing and vomiting. Coughing, induced by electrical stimulation of the superior laryngeal nerves, consisted of increased and prolonged diaphragmatic activity followed by a burst of abdominal activity. Mid-thoracic external and internal intercostal muscles discharged synchronously with the diaphragm and abdominal muscles, respectively. Caudal external and internal intercostal muscles, however, discharged synchronously with the abdominal muscles. Vomiting, induced by stimulation of the lower thoracic vagi, consisted of a series of synchronous bursts of diaphragmatic and abdominal activity (retching) followed by a prolonged abdominal discharge after the cessation of diaphragmatic activity (expulsion). Caudal external and internal intercostals discharged in phase with diaphragmatic and abdominal activity but both mid-thoracic intercostal muscles discharged out of phase with these muscles. These results indicate major differences in the control and functional roles of intercostal muscles at different thoracic levels during these behaviours.

Abdominal Muscles↗

Evaluation of resolution and sensitometric characteristics of an asymmetric screen-film imaging system.

The authors compared asymmetric and conventional screen-film systems for chest radiography. The new imaging system, with asymmetric construction of the screens and film, has image quality characteristics substantially different from those of available screen-film combinations. This asymmetric screen-film system consists of a thin (high-resolution) front screen and a high-contrast emulsion, a thick (lower-resolution) back screen and a low-contrast emulsion, and technology that reduces the crossover exposure and prevents light from the front screen from exposing the back emulsion and vice versa. With this system, density, contrast, and resolution can be increased in selected regions while maintaining density and contrast in the rest of the image. Also, the resolution of this system varies as a function of density. Preliminary image quality and sensitometric studies indicate the new system is superior for chest radiography because it provides better visualization of mediastinal, retrocardiac, and diaphragmatic regions while yielding better contrast and resolution in lung parenchyma.

Humans↗

Respiratory muscle interaction during NREM sleep in patients with occlusive apnea.

To study respiratory muscle interaction in patients with occlusive apnea, diaphragmatic electromyogram (EMGdi) and gastric, pleural, and transdiaphragmatic pressures (Pga, Ppl, and Pdi, respectively) were studied in seven patients during non-rapid-eye-movement (NREM) sleep. Diaphragmatic force output, as assessed by Pdi, followed the periodic changes in EMGdi but during the occlusive phase the increase in Pdi was more than the increase in EMGdi. This increase in Pdi was essentially due to an increase in Ppl, since Pga and EMGdi had a linear relationship (r = 0.98, P less than 0.001) that did not change during the occlusive and ventilatory phases. Abdominal muscle recruitment evident in Pga and abdominal motion tracings during the occlusive phase when paradoxical rib cage motion was observed suggested that this increase in diaphragmatic efficiency was likely due to a change in diaphragmatic length-tension characteristics. These results demonstrate that, in patients with occlusive apneas, the diaphragm is the predominant respiratory muscle during NREM sleep and that its function is supported by abdominal muscle recruitment.

Abdominal Muscles↗

Effects of theophylline and enprofylline on diaphragmatic contractility.

Experimental data suggest that theophylline (T) enhances diaphragmatic contractility by increasing the influx of calcium at the cell membrane level through an inhibition of adenosine receptors (Aubier et al., J. Appl. Physiol. 54: 460-4, 1983). Enprofylline (E) is a xanthine drug that has poor ability to antagonize physiological actions of adenosine. The aim of this study was to compare the effects on diaphragmatic contractility of T and E in order to determine whether antagonism of adenosine receptors was the underlying mechanism of the inotropic effect of T on diaphragmatic contractility. Ten normal subjects were studied in the sitting position. The contractile properties of the diaphragm were assessed by measuring the transdiaphragmatic pressure (Pdi) generated at functional residual capacity during bilateral electrical stimulation of the phrenic nerves. The subjects were randomized, and after control measurements were performed, they received T or E. This was a double-blind crossover study, the measurements being repeated with the second drug after one week. Both drugs were administered intravenously with a loading dose of 6 and 2 mg/kg administered in 30 min for T and E and a maintenance dose of 0.9 and 0.075 mg.kg-1 X h-1 for T and E, respectively. Measurements were performed before and 60 min after T or E administration. Plasmatic levels of both drugs were also analyzed. In all the subjects, therapeutic levels of T or E were reached (14.8 +/- 0.6 and 3.9 +/- 0.42 mg/l for T and E, respectively, at 30 min).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Effects of diaphragmatic plication on respiratory mechanics in dogs with unilateral and bilateral phrenic nerve paralyses.

To examine the effects of diaphragmatic plication on respiratory mechanics during spontaneous breathing, we grouped 28 dogs into left phrenicotomy and plication (group L, n = 11), bilateral phrenicotomy and plication (group B, n = 9), and sham operations (group C, n = 8). In groups L and B, phrenicotomy caused significant (p < 0.05) decreases in tidal volume (VT), transdiaphragmatic pressure (delta Pdi), the ratio of gastric to esophageal pressure (delta Pga/delta Pes) and dynamic lung compliance (Cdyn), and significant (p < 0.05) increases in esophageal pressure (delta Pes), and the work of breathing (WOB) per liter of ventilation. In group L, plication resulted in significant (p < 0.05) increases in VT, delta Pdi, delta Pga/delta Pes, and Cdyn, and a significant reduction of WOB compared with phrenicotomy condition, indicating an improvement in the respiratory mechanics. However, in group B, no significant changes were observed after plication except for increased VT. In eight left-phrenicotomized open-chest dogs, transdiaphragmatic pressure (Pdi) and fractional shortening (FS) of right hemidiaphragm by right phrenic nerve stimulation increased significantly (p < 0.05) after plication, compared with the phrenicotomy condition, suggesting more effective kinetics of the right hemidiaphragm after plication of the left hemidiaphragm. Our results showed that diaphragmatic plication for unilateral paralysis is more effective than for bilateral paralysis, indicating that an improvement in kinetics of the intact hemidiaphragm plays an important role in functional recovery.

Animals↗

Effect of diaphragmatic respiration on some cardiorespiratory parameters during rehabilitation in bronchopneumopathic patients.

The effect of diaphragmatic respiration in broncopneumopathic patients has been investigated. By using a computerized method of analysis, VO2, VCO2, VE, VA and several other respiratory and metabolic parameters have been investigated. The results obtained in these patients, at rest, have shown that particularly in supine position the abdominal respiration improves the CO2 output by increasing VCO2 and reducing VO2. The reduction in the breathing rate, reduces VD and increases VA, in this way the efficiency of ventilation increases accordingly. The increase of CO2 output at the same metabolic rate is discussed in light of the fact that diaphragmatic respiration seems to improve the VA/Q ratio.

Bronchitis↗

[The association of diffuse pulmonary fibrosis and hiatal hernia: a simple coincidence? (author's transl)].

Gastroesophageal diseases with reflux can cause numerous pulmonary complications (bronchospasm crisis, bronchitis, pneumonias, lung abscesses). These manifestations are very frequent in the hiatal hernias and in some series have reached up to 46 percent of the cases studied. Recently it has been suggested that microaspirations, repeated over a long period of time, may cause an alteration of the pulmonary interstitium and the consecutive development of a clinical and roentgenologic picture similar to pulmonary fibrosis. The aspirations are produced more frequently at night, favoured by the lying down position and because of it the patients present attacks of nocturnal cough. In this paper, two patients who presented the association of pulmonary fibrosis and hiatal hernia with esophageal relux are described. The first case was diagnosed early; he had minimal radiographic anomalies and the disappearance of the respiratory symptomatology was achieved by means of the medical treatment of the esophageal reflux. The second case was in a very advanced stage with severe respiratory insufficiency and gastrointestinal manifestations of many years' evolution. Both patients had been diagnosed as having idiopathic pulmonary fibrosis. Although there is no experimental proof of the "reflux-fibrosis" theory, we think that this possibility should be kept in mind before diagnosing the interstitial pulmonary pattern as idiopathic, especially in the cases of only slight radiographic and functional involvement susceptible to improvement with correction of the esophageal reflux.

Aged↗

[Congenital malformations: care or predict?].

Spectacular scientific and technological advances made in the last decade have had such a profound impact on biological and medical science that they have dramatically modified the citizen's behaviour concerning life events, especially congenital malformation. Prenatal diagnosis (PND) leads to do the diagnosis of almost all fetal internal and external malformations. The matter is, not only to care, but, first to know. The positive efficiency of PND is sometimes preparing the best cares and, of course, to recognize many severe anomalies postnatally diagnosed before PND time, and carrying wellknown 50% rate mortality by neo-natal surgery. Congenital diaphragmatic hernia is pointed out as a good example of it, and of hopes and disappointing in utero foetal surgery. New protocol of assessment of fetal renal function is an appreciated method to do prognosis of some fetal uropathies before late in utero drainage, for a short time waiting for necessary maturation of lungs allowing premature delivery. The possibility to do PND of small and benign malformation leads to ask for the question of utility of to know. In spite of the respect of quality of life, can we really allow this type of human selection to be made? The next knowledges of the human genoma map bring us into the predictive medicine. Using "compulsory" PND is a real risk to practice dangerously, a soft eugenism. PND must be, and remain an outstanding advance to provide better treatment.

Congenital Abnormalities↗

Laparoscopic Nissen fundoplication for hiatus hernia: a case report.

Laparoscopic Nissen fundoplication has rapidly become an established technique in the management of severe or complicated reflux oesophagitis. We describe our initial experience with laparoscopic Nissen fundoplication in a 54-year-old man with intractable severe haemorrhagic oesophagitis and a large sliding hiatus hernia. The Rossetti modification of Nissen fundoplication was used as this is a surgical procedure eminently suited for the laparoscopic approach since minimal dissection is needed. The operative time taken was 260 minutes, there was minimal blood loss, minimal postoperative analgesia requirement and early return of bowel function.

Follow-Up Studies↗

Diaphragmatic dysfunction and dyspnoea in amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a progressive disorder of unknown origin. Respiratory involvement is the principal cause of death, and dyspnoea is a major source of discomfort. In this study, diaphragm function is described and its relationship with dyspnoea examined in 48 ALS patients (32 male, age 26-80 yrs). The detailed neurological and respiratory evaluation (clinical examination, pulmonary function tests, static pressures, mouth twitch pressures (Pm,t), electromyographic responses to phrenic nerve stimulation and cortical magnetic stimulation were analysed after stratification according to dyspnoea. Dyspnoeic (group I) and nondyspnoeic (group II) patients were similar, bulbar signs being more frequent in group I. Vital capacity was lower in group I (mean+/-SD 67.9+/-22.7 versus 87.9+/-15.6% of the predicted value, p=0.0028), as were maximal static inspiratory pressure (41+/-24 versus 60+/-27% pred, p=0.0242) maximal static inspiratory pressure (18+/-11 versus 32+/-14% pred, p=0.0042), and Pm,t (3.71+/-2.5 versus 7.26+/-3.45 cmH2O, p=0.0011). Abdominal (Abd) paradox and respiratory pulse were frequent in group I (15 of 25 and 14 of 25) but absent or rare in group II (0 of 23 and four of 23) (p<0.05). The electromyographic responses to phrenic and cortical stimulation were generally abnormal in group I but subnormal in group II. Multivariate analysis selected only signs of diaphragm dysfunction (namely, Abd paradox and abnormal electromyographic responses) as significant predictors of dyspnoea. It is concluded that dyspnoea in amyotrophic lateral sclerosis patients should prompt diaphragm function tests.

Amyotrophic Lateral Sclerosis↗

Association of deletions of the chromosomal region 15q24-ter and diaphragmatic hernia: a new case and discussion of the literature.

OBJECTIVE: To provide new insights into how chromosomal aberrations affect fetal development, as well as for the counseling of parents in comparable situations, it is important to characterize and report the genotypes of fetuses with clinical anomalies. METHODS: Molecular cytogenetic analyses in a fetus with congenital diaphragmatic hernia (CDH). RESULTS: This report describes the first case of a deletion of the region q26.1-ter on chromosome 15 occurring as a de novo event associated with CDH. A detailed review of the literature provides further evidence of a functional association between deletions within the chromosomal region 15q24-ter and the development of CDH. CONCLUSIONS: The obtained data argue that detection of such a deletion in the region 15q24-ter associated with CDH likely predicts a poor prognosis. This report highlights the importance of giving special diagnostic attention to the chromosomal region 15q24-ter when prenatal ultrasound examination provides evidence of a CDH and warrants further research to identify genetic elements within the chromosomal region 15q24-ter related to the development of diaphragmatic hernia.

Adult↗