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At least 703 records · Page 39Linked to original sources
PubMed1976

Persistent hypoplasia of the lung after repair of congenital diaphragmatic hernia.

Quantitative analysis has been used to assess growth in a lung from an infant aged 21/2 months in whom a diaphragmatic hernia was repaired at birth. The lungs had been abnormally small at birth but at 21/2 months were of normal volume. Alveoli had multiplied at the normal rate after birth but had not reached the number normal of age. The number per acinus was normal but the alveoli were increased in size, particularly in the left lung. The airway number, and thus alveolar, acinar, and arterial number, were all reduced in both lungs, the ipsilateral being most affected. The pulmonary blood vessels in both lungs showed an increased muscularity that did not correlate with lung volume or alveolar number, a feature that may have been present at birth. The degrees of hypoplasia in the two lungs were different at birth and this difference had been maintained. The effect of the disturbance to lung growth on the functioning of the lung is discussed.

Female↗
PubMed2005

Ectopic thoracic kidney in a child with congenital diaphragmatic hernia.

Thoracic ectopic kidney with partial or complete renal protrusion above the level of the diaphragma into the posterior mediastinum is the rarest form of all ectopic kidneys with an incidence of less than 1 per 10,000 cases. We present a newborn with right congenital diaphragmatic hernia associated with thoracic ectopic kidney. The diagnosis of ectopia was made prior to surgery. Gerota's fascia of kidney was used to close the diaphragmatic defect. Since this renal anomaly is usually asymptomatic, it does not require any specific treatment. However, a close examination of function and anatomy of the kidney prior to surgery of hernia is important and beneficial. We discuss the embryological context and the importance of renal scintigraphy in patients with ectopic kidney.

Choristoma↗
PubMed1997

Partial liquid ventilation in critically ill infants receiving extracorporeal life support. Philadelphia Liquid Ventilation Consortium.

OBJECTIVES: To demonstrate that a period of partial liquid ventilation (PLV) with perflubron improves pulmonary function, without adverse events, in a select group of critically ill infants receiving extracorporeal life support (ECLS) with a high likelihood of mortality. METHODS: This was an open-label, noncontrolled, phase I and II trial of PLV in two infants with congenital diaphragmatic hernia and four infants with acute respiratory distress syndrome (ARDS) who were failing to improve while receiving ECLS. PLV was performed by instilling and maintaining a functional residual capacity of sterile perflubron for 4 to 96 hours. RESULTS: Four infants were successfully weaned off ECLS for at least 3 days, and two infants (both with ARDS) are long-term survivors after PLV. All infants demonstrated lung recruitment and improved lung compliance, and there were no adverse events related to PLV. CONCLUSIONS: The study suggests that perflubron PLV is safe, improves lung function, and recruits lung volume in critically ill infants receiving ECLS. PLV therapy for infants with ARDS seems to have a great deal of promise. Based on this and other phase I and II trials, studies of PLV on selected full-term infants before ECLS have been initiated.

Emulsions↗
PubMed2001

An unusual combination of diaphragmatic hernias in a patient presenting with the clinical features of restrictive pulmonary disease: report of a case.

The combination of a Morgagni hernia and a paraesophageal hernia in adults is very rarely encountered in clinical practice. In fact, to our knowledge, only three cases of this condition, which is probably a coincidental occurrence, have been reported in the medical literature. We discuss the management of a 74-year-old man found to have combined Morgagni and paraesophageal hernia who presented with clinical features of a restrictive pulmonary disease.

Aged↗
PubMedDate not supplied

Dysfunctional metabolism induced by the systemic effects of burn trauma: the role of rates of polyinositol and glycerophosphate formation in diaphragm.

Common complications among patients with burns are respiratory problems. Inspiration for breathing normally is facilitated by diaphragmatic contractility. However, in burn trauma numerous factors impose on skeletal muscle activity and chest wall function. These present limitations to surgical procedures requiring anesthesia. The hypothesis of this study was that the systemic effects of increasing burn size may result in metabolic changes in the polyinositol part of the phosphatidyl inositol signal transduction system in diaphragm. In this report, rate changes in polyinositol phosphates (i.e., inositol triphosphate (IP3), inositol 1,4 biphosphate (I1,4P2), inositol 1 phosphate (I1P), and inositol) that occurred in diaphragm subjected to the systemic effects of burn trauma were examined. Third-degree burn injury was applied by scalding predefined areas (i.e., 0%, 20%, and 50% body surface area) on the dorsal and ventral surfaces of mice. At postburn day 21 tissues were harvested and the levels of the polyinositol phosphates were measured by incorporation of myo-[2-3H]-inositol with separation of the phosphates by anion-exchange chromatography. All actual levels and rates of formation were analyzed with standard statistical tests and curve-fitting routines. Rate changes of the labelled [3H]-polyinositol phosphates were determined for the control, 20%, and 50% groups. These data show that rate changes occur in the polyinositol part of the phosphatidyl signal transduction system and are caused by the systemic effects of large percent body surface area burns. The changes that occur in the polyinositol phosphates provide insight into dysfunctional processes that can affect skeletal muscle physiology. The long-term goal of these studies is that they will lead to therapeutic advancements in the treatment of respiratory dysfunction in patients with burns.

Animals↗
PubMed1991

Measurement of twitch transdiaphragmatic pressure: surface versus needle electrode stimulation.

The transdiaphragmatic pressure (Pdi) generated during bilateral supramaximal phrenic nerve stimulation at 1 Hz from surface stimulating electrodes was compared with pressures obtained from needle electrodes inserted under local anaesthesia. Surface electrodes were used to obtain diaphragmatic electromyograms and magnetometers to monitor rib cage and abdominal configuration. Twitch Pdi was recorded at functional residual capacity in three normal subjects. Mean (SD) twitch Pdi in the three subjects during stimulation with surface electrodes was 19.4 (1.8), 22.5 (1.1), and 29.3 (2.2) cm H2O compared with 12.9 (1.5), 17:4 (1.3), and 22.6 (3.0) cm H2O with needle stimulating electrodes. Thus phrenic nerve stimulation with needle electrodes was more complicated and more invasive than stimulation with surface electrodes and resulted in lower transdiaphragmatic pressures.

Adult↗
PubMed1987

Response to resistive loading in the newborn piglet.

The diaphragmatic force generation and electromyographic response to long-term (1 h) inspiratory resistive loading was examined in the newborn piglet during the 3rd postnatal wk of life. Minute ventilation decreased to approximately 50% of baseline level within 5 min of imposition of a severe resistive load and remained at this level for the duration of loading. The decrease in ventilation was secondary to a fall in tidal volume at a constant frequency. There was a significant increase in central nervous system output to the diaphragm as manifested by integrated diaphragmatic electromyogram. Progressive augmentation of this index of central drive continued throughout the period of loading. Functional residual capacity fell significantly by 60 min of inspiratory resistive loading. This strategy should allow greater force generation by placing the diaphragm at a more optimal length-tension relationship. However, the force generating capability of the diaphragm was compromised as assessed by force-frequency curve analysis. These results suggest that the diaphragm of the neonatal piglet fatigues during prolonged inspiratory resistive loading.

Airway Resistance↗
PubMed2001

A novel autosomal dominant distal myopathy with early respiratory failure: clinico-pathologic characteristics and exclusion of linkage to candidate genetic loci.

We describe a novel autosomal dominant myopathy presenting in mid-adult life with tibialis anterior weakness. We carried out a detailed clinical assessment of 24 individuals spanning three generations, documenting pathologic features of the muscles in 7 of the 11 affected individuals, including an autopsy study on one case. The second generation of affected individuals presented at an earlier age, and the disease progressed more rapidly than in the first generation. Lung function tests revealed progressive global respiratory muscle weakness detectable from the time of presentation, with preferential diaphragmatic involvement in some cases. Hip girdle and shoulder girdle weakness appeared later in the disease course. We observed a striking correlation between the clinical and pathological features. Clinically unaffected muscles had minimal pathologic change. Fiber splitting, eosinophilic inclusions, and vacuoles with basophilic rims were seen in moderately affected muscles, and fat and fibrous connective tissue replaced muscle fibers in the severely involved muscles. The inclusions were Congophilic and reacted with antibodies to desmin, beta-amyloid, and phosphorylated tau protein. The disease was not linked to any of the known loci associated with distal myopathies, confirming that the disorder in this family is both genetically and phenotypically distinct.

Adult↗
PubMed2005

[A case of bilateral diaphragmatic paralysis clinically suggestive of brachial neuritis].

A 55-year-old man complained of acute onset of shoulder pain and dyspnea in the supine position. A diagnosis of bilateral diaphragmatic paralysis was made based on clinical inspection of his breathing pattern, radiographic appearance, and pulmonary function tests. He had had no traumatic or thoracic surgery or inflammatory episode. He did not suffer from diabetes, other central neural diseases, or any neoplastic disease. From the clinical feature and electromyographic findings, phrenic nerve involvement of brachial neuritis without any other muscle involvement was considered as a causative disease. When he received non-invasive intermittent positive-pressure ventilation by nasal mask in a supine position, his dyspnea was substantially attenuated and Carbon dioxide retention was lessened. After 1 year, his shoulder pain is still persisting and radiographic findings are not remarkably improved.

Brachial Plexus Neuritis↗
PubMed1993

[The mechanism of apnea during difficult breathing].

The experiments on anesthetized cats demonstrated that only a combination of hypoxia and inspiratory resistive loading induced respiratory arrest. The findings suggest that both central and peripheral mechanisms of apnea exist due to functional insufficiency of respiratory muscles as a result of synaptic disorder and excitatory contractility disorder in diaphragmatic muscle fibers.

Animals↗
PubMed1998

Acquired intrathoracic kidney.

A 63 year old female with neurofibromatosis was referred to the Pulmonary Clinic of the University Hospital for evaluation of a right thoracic mass. Physical examination revealed subcutaneous nodules over the trunk area, café au lait spots, and dullness to percussion with decreased breath sounds at the right lung base. The chest X ray showed a 4 cm mass at the right lung base. Computerized tomography of the chest demonstrated a right posterior diaphragmatic hernia with displacement of the liver and kidney into the thoracic cavity. Renal scan confirmed the location of the kidney in the right hemithorax showing normal renal cortical function. Intrathoracic kidney is a very rare finding, most are found in males and are asymptomatic. They are usually found in the left hemithorax and associated to a normal diaphragm. Only 0.25% are associated with a diaphragmatic hernia. In our patient, there was a history of mild trauma five years earlier, but this did not clearly explain how the kidney became ectopic. Acquired intrathoracic kidney should be considered in the differential diagnosis of a lung mass to avoid an unnecessary thoracotomy and nephrectomy.

Female↗
PubMed2006

[Amyotrophic lateral sclerosis (ALS): evaluation of respiratory function].

Respiratory involvement is an almost constant feature of als, with a usually rapid progression leading to respiratory failure. These characteristics justify a close follow up, usually at three-month intervals. A systematic, careful clinical evaluation is essential to detect the subtle respiratory symptoms and signs related to respiratory muscle failure. Dyspnea and orthopnea are often late findings in patients with a usually severe functional impairment due to peripheral muscle weakness. Nocturnal respiratory events (obstructive sleep apnea syndrome and hypoventilation) are strongly suggested by daytime hypersomnolence and frequent morning headaches. Physical evaluation is essential to detect accessory muscle recruitment, supine abdominal paradox, and encumbrance of upper or lower airways. Vital capacity (VC) is the most classical lung function test. The major limitation of spirometry is its poor sensitivity to detect a moderate inspiratory muscle weakness. Supine VC may improve the detection of diaphragmatic involvement. Peak expiratory flow during cough (cough PEF) gives an overall evaluation of cough efficiency, values below 160 to 270 L/min suggesting poor airway clearance. Arterial blood gases are performed at first evaluation and subsequently in case of clinical signs, significant deterioration of lung function tests, or sleep desaturations. Hypercapnia is weakly related to lung function results in bulbar patients. A specific evaluation of respiratory muscle strength is mandatory, as these tests are both sensitive and highly prognostic. Possible discrepancies (particularly in bulbar patients) between Maximal inspiratory pressure (PImax) and sniff nasal inspiratory pressure (SNIP) justify to perform both measurements and to select the highest pressure. A maximal expiratory pressure (PEmax) below 45 cm H2O may indicate a compromised cough efficiency but the correlation with cough PEF may be poor. Screening nocturnal oxymetry is useful to detect sleep apneas and hypoventilation. Criteria defining significant desaturations remain however controversial. Suspicion of obstructive sleep apnea syndrome on clinical grounds or oxymetry findings should be confirmed by a conventional polysomnography.

Amyotrophic Lateral Sclerosis↗
PubMed1989

Diaphragmatic weakness and paralysis.

Diaphragmatic weakness implies a decrease in the strength of the diaphragm. Diaphragmatic paralysis is an extreme form of diaphragmatic weakness. Diaphragmatic paralysis is an uncommon clinical problem while diaphragmatic weakness, although uncommon, is probably frequently unrecognized because appropriate tests to detect its presence are not performed. Weakness of the diaphragm can result from abnormalities at any site along its neuromuscular axis, although it most frequently arises from diseases in the phrenic nerves or from myopathies affecting the diaphragm itself. Presence of diaphragmatic weakness may be suspected from the complaint of dyspnea (particularly on exertion) or orthopnea; the presence of rapid, shallow breathing or, more importantly, paradoxical inward motion of the abdomen during inspiration on physical examination; a restrictive pattern on lung function testing; an elevated hemidiaphragm on chest radiograph; paradoxical upward movement of 1 hemidiaphragm during fluoroscopic imaging; or reductions in maximal static inspiratory pressure. The diagnosis of diaphragmatic weakness is confirmed, however, by a reduction in maximal static transdiaphragmatic pressure (Pdimax). The diagnosis of diaphragmatic paralysis is confirmed by the absence of a compound diaphragm action potential on phrenic nerve stimulation. There are many causes of diaphragmatic weakness and paralysis. In this review we outline an approach we have found useful in attempting to determine a specific cause. Most frequently the cause is either a phrenic neuropathy or diaphragmatic myopathy. Often the neuropathy or myopathy affects other nerves or muscles that can be more easily investigated to determine the specific pathologic basis, and, by association, it is presumed that the diaphragmatic weakness or paralysis is secondary to the same disease process.

Diaphragm↗
PubMed2007

(Paediatric) magnetic resonance urography: just fancy images or a new important diagnostic tool?

PURPOSE OF REVIEW: Magnetic resonance urography has become an established imaging tool in uroradiology. Its potential to assess anatomy and function makes it an ideal tool for evaluation of urinary tract malformations, renal cysts, genito-urinary tract tumours, infections and renal transplants. This review tries to highlight the potential of magnetic resonance urography in the light of new advances, particularly focusing on paediatric applications. RECENT FINDINGS: Technical innovations such as diaphragmatic tracking, parallel or propeller imaging, faster gradients and higher field strength improve applicability in infants and children. Dynamic studies enable assessment of renal functional parameters such as split renal function, glomerular filtration rate or urinary drainage. Recent advances in magnetic resonance spectroscopy, diffusion imaging and perfusion imaging and new contrast agents promise to widen the potential of magnetic resonance urography as a functional imaging tool, not only in paediatrics but also for other magnetic resonance applications in the genito-urinary tract, such as prostate imaging or in the staging of ovarian and endometrial cancer. SUMMARY: Besides ultrasound being used as the initial imaging method, particularly in children (and as computed tomography in adults), magnetic resonance urography can be envisioned as the major imaging modality for almost all (paediatric) uroradiological queries, consequently creating a growing demand for available equipment and procedural expertise.

Child↗
PubMed2006

Liver transplant in a four-month-old child with biliary atresia, unilateral pulmonary agenesis, and diaphragmatic hernia: first case report.

Bilateral pulmonary agenesis (PA) is a rare embryological defect incompatible with life. Unilateral PA has a wide range of clinical presentations: its prognosis depends on the presence and severity of other associated anomalies. Fetal biliary atresia has been associated with a number of congenital anomalies, but the etiology is still not understood. An unusual case of a child with right PA, right diaphragmatic hernia, and delayed diagnosed biliary atresia leading to liver failure is presented herein. At the age of 4 months the patient was referred to the Transplant Department at Children Healthcare of Atlanta at Egleston with cholestasis and failure to thrive. With a rapidly progressive liver insufficiency, this child was evaluated for liver transplantation. In the absence of any respiratory symptom, the patient received a deceased donor size-matched left lateral segment liver transplant, which covered the diaphragmatic defect, with no further repair required. Twenty-seven months post-transplant, the patient has good graft function, a normal Z-score and is thriving. In spite of the increased physiological and surgical challenges (absence of right lung tissue, hemi-diaphragm, and ectopic position of the liver in the right chest), liver transplantation was performed with positive outcome in this high-risk child. Whether PA, may have developmentally contributed to expression of biliary atresia will need further investigation.

Biliary Atresia↗
PubMed1986

[Functional significance of the left adrenal vein and the gonadal veins].

Owing to the investigation of changeability variants of the left adrenal, anastomosis of the inferior diaphragmatic and ovarian (testicular) veins--tributaries of the human left renal vein, three variants in inflow of the left adrenal vein in comparison to the left ovarian (testicular) vein have been revealed. In the first variant, that occurs in 70% of cases, the left adrenal vein flows into the left renal vein by 15 cm more medially from the ostium of the ovarial (testicular) vein. The second variant occurs in 11%. The left adrenal and ovarian (testicular) ostia are situated at the same level. The distance from the hilus renalis to the place, where the ostia mentioned get into the renal vein, is about 39 mm. In the third variant (19%) the left adrenal vein gets into the left renal vein nearer to the hilus renalis than the ovarian (testicular) veins. An average distance from the hilus renalis to the ostium of the left adrenal vein is 33 mm. The second variant, when the left adrenal vein gets into the left renal vein, contributes to appearance of the primary varicocele at the left. When the operation for nephrectomy is made at the left, our data make it possible to recommend a purposeful ligation of the left renal vein more laterally to the ostium of the ovarian (testicular) vein. In 80% this ligation will not disturb the function of the left adrenal vein.

Adolescent↗