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PubMed1990

[Respiratory aid using a diaphragmatic pacemaker in high paraplegia].

Spastic tetraplegia due to cervical medullary injury above the origin of the phrenic nerve neurons means complete respiratory paralysis. In this case a phrenic pacemaker can be considered as an alternative to long term mechanical ventilation. An implantable 8-channel stimulation device has been developed for functional electrical stimulation of the phrenic nerves. The "Karussellstimulation" provides fatigue free stimulation for 24 hours a day. We report the case of a 12 year old girl with complete ventilatory insufficiency who underwent implantation of a phrenic pacemaker. 18 month after implantation she is independent of a conventional respirator and limits rehabilitation could be achieved. Electrophrenic respiration is of value in the rehabilitation management of tetraplegic patients with ventilatory insufficiency.

Child↗
PubMed1996

Pulmonary surfactant dysfunction in congenital diaphragmatic hernia: experimental and clinical findings.

Experimental and clinical findings indicate immaturity of pulmonary surfactant in congenital diaphragmatic hernia (CDH). Lung histology has shown a decreased amount of lamellar bodies. A low lecithin/sphingomyelin ratio in the amniotic fluid, and decreased concentrations of surfactant protein A and disaturated phosphatidylcholine in the pulmonary tissue and the amniotic fluid have been reported. Furthermore, low compliance and high surface tension have also been found. Evidence of clinical and experimental findings of structural, biochemical and functional pulmonary immaturity in CDH is reviewed. Prenatal administration of corticosteroids to accelerate fetal pulmonary maturation, and the use of early surfactant therapy, should be further evaluated in the clinical management of CDH.

Adrenal Cortex Hormones↗
PubMed1993

Aggressive surgical and medical management of autosomal recessive polycystic kidney disease.

The treatment of autosomal recessive polycystic kidney disease (ARPKD) is evolving. Overall prognosis is difficult to determine, but most neonates without severe pulmonary hypoplasia will survive. It is difficult to predict which neonates with ARPKD requiring immediate ventilatory support have pulmonary hypoplasia incompatible with survival. Massively enlarged kidneys restricting diaphragmatic excursion can cause respiratory distress in addition to pulmonary hypoplasia. Three infants with ARPKD underwent bilateral nephrectomies and institution of peritoneal dialysis in an attempt to improve ventilatory status and assess long-term pulmonary function. Two children are alive at twelve and thirty months postoperatively and awaiting renal transplantation. One infant died of acute cardiorespiratory complications.

Child, Preschool↗
PubMed2000

Intraperitoneal lidocaine for postoperative pain after laparoscopy.

BACKGROUND: A controversy exists over the effectiveness and clinical value of intraperitoneal local anaesthetics for treating pain after laparoscopic cholecystectomy. The use of intraperitoneal lidocaine was evaluated in this study. METHODS: At the end of surgery, 200 ml saline containing 200 mg lidocaine, or the same volume of saline, were randomly splashed under the right diaphragmatic surface in 50 patients in a double-blind manner. Postoperative shoulder and abdominal pain intensity were recorded on a numeric grading scale and a visual analogue scale, respectively. Analgesic consumption was also recorded. Respiratory function tests were compared before and after surgery. Side effects and recovery variables were assessed by the nurses at 2-h intervals. RESULTS: The incidence, severity and duration of shoulder pain were reduced from 40% of patients scoring 3.9+/-0.2 for duration of 17.9+/-0.2 h in the control group to 12% scoring 2.5+/-0.5 for duration of 1.6+/-0.01 h in the lidocaine group. Lidocaine treated patients had significantly less abdominal postoperative pain immediately on return to the ward and during the first postoperative day (P<0.05). "No pain on deep inspiration" was reported by 72% of patients in the lidocaine group immediately on return to the ward compared to 8% of those in the control group. Analgesic consumption for 24 h after surgery was significantly less in the lidocaine group (P<0.05). There were no significant differences in respiratory function tests, recovery variables or incidence of side effects between the two groups. CONCLUSION: Intraperitoneal lidocaine is simple to use and results in a long-lasting reduction of pain after a single administration.

Adult↗
PubMed1985

Gastric tube oesophagoplasty--a long-term assessment.

The first gastric tube oesophagoplasty at the Royal Alexandra Hospital for Children was performed in 1968. Over the next 15 years, until 1982, a total of 46 operations were performed. This represents the largest series of gastric tube oesophagoplasty in children yet reported in the literature. Two techniques have been in use and are compared. The main aim of the paper is to present the long-term results of gastric tube oesophagoplasty. In our 15 years' experience with the gastric tube for oesophageal replacement, we have found it to be a very satisfactory procedure, with a very low mortality and failure rate. The vascularity of the stomach gives rise to less anxiety than with colon. Early postoperative complications are readily identified and treated. There is a higher risk of serious chest complications in bringing the gastric tube through the chest with primary anastomosis in the neck. This also predisposes to diaphragmatic herniation and obstruction, complications not seen when the gastric tube is brought up substernally. The long-term results are very encouraging, with virtually all the children leading active and normal lives. The gastric tube functions satisfactorily with no evidence of the late complications often reported with colonic tubes. There is no significant difference in the long-term results of the two techniques of gastric tube oesophagoplasty used in this hospital.

Adolescent↗
PubMed2006

Rocuronium exacerbates mechanical ventilation-induced diaphragm dysfunction in rats.

OBJECTIVE: Nondepolarizing neuromuscular blocking agents are commonly used in the intensive care setting, but they have occasionally been associated with development of myopathy. In addition, diaphragmatic atrophy and a reduction in diaphragmatic force were reported after short-term controlled mechanical ventilation in animal models. We hypothesized that infusion of rocuronium, an aminosteroidal neuromuscular blocking agent, during 24 hrs of controlled mechanical ventilation would further alter diaphragm function and would enhance activation of the ubiquitin- proteasome pathway. DESIGN: Randomized, controlled experiment. SETTING: Basic animal science laboratory. SUBJECTS: Male Wistar rats, 14 wks old. INTERVENTIONS: Rats were divided into four groups: a control group, a group of anesthetized rats breathing spontaneously for 24 hrs, and two groups submitted to mechanical ventilation for 24 hrs, receiving a continuous infusion of either 0.9% NaCl or rocuronium. MEASUREMENTS AND MAIN RESULTS: In vitro diaphragm force was decreased more significantly after 24 hrs of mechanical ventilation combined with rocuronium infusion than after mechanical ventilation alone (e.g., tetanic force, -27%; p < .001 vs. mechanical ventilation). Similarly, the decrease in diaphragm type IIx/b fiber dimensions was more pronounced after mechanical ventilation with rocuronium treatment than with saline treatment (-38% and -29%, respectively; p < .001 vs. control). Diaphragm hydroperoxide levels increased similarly in both mechanically ventilated groups. Diaphragm muscle RING-finger protein-1 (MURF-1) messenger RNA expression, an E3 ligase of the ubiquitin-proteasome pathway, increased after mechanical ventilation (+212%, p < .001 vs. control) and increased further with combination of rocuronium (+320%, p < .001 vs. control). Significant correlations were found between expression of MURF-1 messenger RNA, diaphragm force, and type IIx/b fiber dimensions. CONCLUSIONS: Infusion of rocuronium during controlled mechanical ventilation leads to further deterioration of diaphragm function, additional atrophy of type IIx/b fibers, and an increase in MURF-1 messenger RNA in the diaphragm, which suggests an activation of the ubiquitin-proteasome pathway. These findings could be important with regard to weaning failure in patients receiving this drug for prolonged periods in the intensive care unit setting.

Androstanols↗
PubMed1986

Upper and lower rib cage deformation during breathing in quadriplegics.

In quadriplegia, the anteroposterior diameter of the rib cage (RC) decreases during inspiration. Our aim was to characterize this paradoxical motion further and to determine to what extent it was due to a diminished inspiratory effect of the diaphragm on the RC because of abdominal muscle paralysis. In six quadriplegics, upper and lower RC (URC, LRC) displacements were studied in various postures with and without extrinsic abdominal support. LRC was in its relaxed configuration at all lung volumes studied in three subjects before and four subjects during abdominal compression. URC distorted most and, despite improvement during abdominal compression, was never in the relaxed configuration during tidal inspiration. Thoracic distortability was further studied by noting the effect on URC and LRC of a cephalad force applied to the RC at the costal margin. This produced distortion similar to that observed during spontaneous inspiration. It was concluded that during spontaneous inspiration in quadriplegics lack of abdominal support does not entirely explain the RC distortion which occurs, and high abdominal pressure results in diminished RC distortion, an effect which we attribute to an increase in the area of diaphragmatic apposition to the RC.

Abdomen↗
PubMed2025

Mitochondrial Lon Peptidase 1 Controls Diaphragm and Lung Development in a Context-Dependent Manner.

Congenital Diaphragmatic Hernia (CDH) is a rare neonatal disorder causing diaphragmatic defects and cardiopulmonary hypoplasia, traditionally attributed to mechanical compression from organ herniation. However, emerging evidence suggests genetic mutations may independently impair lung development, prompting debate over CDH etiology. Here, we investigated the requirement of mitochondrial function guarded by LON peptidase 1 (Lonp1), a CDH risk gene, in either diaphragm or lung development. Lonp1 loss in skeletal muscles of the diaphragm led to its thinning and membranization, recapitulating the pathology of sac-type CDH. On the other hand, lung-specific inactivation caused severe hypoplasia with defective branching morphogenesis, independent of diaphragm anomalies. Molecularly, Lonp1 disruption dysregulated key transcription factors and signaling pathways known to be critical for early lung development. Our findings here revealed that mitochondrial defects contribute to the pathogenesis of CDH in an organ and cell type specific manner, opening new avenues for drug and therapeutic development.

CDH↗
PubMed1994

Resuscitation from cardiogenic shock by direct angioplasty and 23-hour balloon inflation using a coronary perfusion balloon.

Direct angioplasty is an accepted treatment for acute myocardial infarction and has resulted in stabilization and improvement in the clinical, electrocardiographic, and hemodynamic consequences of acute myocardial infarction. This case demonstrates the effectiveness of coronary perfusion as a method of resuscitation during cardiogenic shock and asystole in a patient with massive acute diaphragmatic and right ventricular infarction. Utilization of prolonged balloon inflation in this case obviated the need for emergency coronary bypass surgery and provided the patient with remarkable and almost complete recovery of left and right ventricular function.

Aged↗
PubMedDate not supplied

Breathing pattern assessment in normocapnic and hypercapnic patients in chronic obstructive pulmonary disease.

Thirty-six patients, 24 normocapnic (mean age +/- SD: 60 +/- 10) and 12 hypercapnic (mean age +/- SD: 64 +/- 9) were compared with a control group (10 volunteers, mean age +/- SD: 46 +/- 8) for the following patterns of respiratory drive and respiratory timing: (1) mean inspiratory flow (Vt/Ti); (2) mouth occlusion pressure 100 ms after the onset of inspiration (P 0.1) and minute ventilation (Ve); (3) inspiratory duty cycle Ti/Tt). The data suggest COPD "respiratory patterns" which may be characterized by the following features: (1) increased mean inspiratory flow (Vt/Ti); (2) increased P 0.1 (in absolute values), P 0.1/Ve and P 0.1/Vt/Ti; (3) reduction in inspiratory duty cycle (Ti/Tt). Changes are more evident in inspiratory duty cycle among hypercapnic pts. When respiratory obstruction becomes worse and hypercapnia appears, the Ti/Tt decrease could be explained by a reduction in diaphragm muscle work, that can prevent the failure of diaphragmatic contractility.

Adult↗
PubMed1981

Splenosis on the right kidney and the diaphragmatic surface following traumatic rupture of the spleen.

A case of an unusual location of splenosis is reported in a 30-year-old man who underwent autopsy after committing suicide. Twelve years before he was splenectomized after traumatic rupture of the spleen. At the postmortem examination splenosis was found on the right kidney and the diaphragmatic surface resembling hemangiomas. It is noteworthy that the patient had never experienced any severe infection after splenectomy, and the possible protective influence of splenosis is discussed in the light of our present knowledge of spleen function.

Adult↗
PubMed1997

Neurological outcome is diminished in survivors of congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation.

In a series of 61 infants who had congenital diaphragmatic hernia (CDH) treated at our center from 1978 through 1996, 37 of 59 (61%) survived the perioperative period with two infants lost to follow-up. Nine (47%) of 19 infants survived before the introduction of extracorporeal membrane oxygenation (ECMO) into our region in 1986. Since 1986, 28 (70%) of 40 infants survived. Eighteen infants required ECMO, and 12 (75%) survived. A chart review was performed to determine whether infants surviving CDH are suffering from delays in neurological development, and, if so, whether this is attributable to ECMO. Of 12 ECMO survivors, 8 (67%) exhibited functional or anatomic evidence for neurological delay. Of 21 non-ECMO survivors, where adequate follow-up was available to make an assessment of neurological development, five (24%) exhibited evidence for delay. This difference was significant (P < .05, Fisher's Exact test). Of these five infants, three were premature, and one had DiGeorge syndrome. More ECMO survivors required diaphragmatic (67%) and abdominal (67%) patches at the time of diaphragmatic repair than non-ECMO survivors (4% and 12%, respectively; P < .05, Fisher's Exact test). In addition, more ECMO survivors required gastrostomy tube placement for feeding (50%) than non-ECMO survivors (16%; P < .05, Fisher's Exact test). A greater need for Nissen fundoplication in ECMO survivors (42%) than in non-ECMO survivors (12%) approached significance (P = .05, Fisher's Exact test). There were trends toward higher 1 and 5 minute APGAR scores and initial and best preoperative P(O2) in the non-ECMO survivors. A comparison between ECMO survivors who exhibited evidence of neurological delay with those who did not showed no differences in duration of ECMO, incidence of intracranial complications during ECMO, need for gastrostomy tube feeding or Nissen fundoplication, or incidence of carotid artery repair between the two groups. Infants surviving CDH who require ECMO have a greater incidence of neurological delay than those who do not. This is likely because of severity of the presenting illness as reflected by a greater need for diaphragmatic and abdominal patches during diaphragmatic repair, the need for Nissen fundoplication and gastrostomy tube feeding, and a trend toward poor APGAR scores and best preoperative P(O2) levels in these patients. However, there may be characteristics of ECMO, as yet unidentified, that may contribute to this outcome.

Apgar Score↗
PubMed1996

Tracheal ligation does not correct the surfactant deficiency associated with congenital diaphragmatic hernia.

INTRODUCTION: Experimental tracheal ligation (CDH + TL) has been shown to reverse the profound lung hypoplasia associated with congenital diaphragmatic hernia (CDH) and to normalize gas exchange. The aim of this study was to determine whether this experimental therapy would correct the surfactant deficiency present in the fetal lamb model of CDH. METHODS: The CDH lamb model was created at 80 days' gestation, and tracheal ligation was performed at 110 days. At term, the lambs were delivered and were ventilated for 30 minutes. The lambs were killed, a pressure-volume curve performed, and the lungs lavaged to measure total phospholipid content. Finally, type II pneumocytes were isolated, and surfactant synthesis was assessed by the incorporation of tritiated choline into phosphatidylcholine. RESULTS: CDH + TL resulted in a lung significantly larger than that of CDH alone. The lungs of the former also had better oxygenation and ventilation. However, lung compliance was reduced compared with controls. Total alveolar phospholipid was dramatically lower, with a decrease in the proportion of phosphatidylcholine present. Surfactant synthesis by the isolated type II pneumocyte was significantly impaired. CONCLUSION: Occlusion of the fetal trachea produces a lung comparable in size to a normal control lung. However, broncheoalveolar lavage analysis shows a marked reduction in total phospholipid, with a decrease in surfactant synthesis by the type II pneumocyte. The normalization of gas exchange reported for this animal model may be only a transient phenomenon. Further studies are required to assess the impact of this surfactant deficiency on long-term lung function.

Animals↗
PubMed1996

Effects of fenoterol on inspiratory effort sensation and fatigue during inspiratory threshold loading.

We studied the effects of a single dose of fenoterol on the relationship between inspiratory effort sensation (IES) and inspiratory muscle fatigue induced by inspiratory threshold loading in healthy subjects. The magnitude of the threshold was 60% of maximal static inspiratory mouth pressure (PI,mmax) at functional residual capacity, and the duty cycle was 0.5. Subjects continued the threshold loaded breathing until the target mouth pressure could no longer be maintained (endurance time). The intensity of the IES was scored with a modified Borg scale. Either fenoterol (5 mg) or a placebo was given orally 2 h before loading in a randomized double-blind crossover protocol. The endurance time with fenoterol (34.4 +/- 8.6 min) was longer than that with the placebo (22.2 +/- 7.1 min; P < 0.05). The ratio of high- to low-frequency power of the diaphragmatic electromyogram (EMGdi) decreased during loading; the decrease was less with fenoterol (P < 0.05). The EMGdi also decreased with loading; the decrease was greater on fenoterol treatment (P < 0.01). The PI,mmax and maximal transdiaphragmatic pressure (Pdi) were similarly decreased after loading on either treatment. The intensity of the IES rose with time during loading in both groups but was lower with fenoterol than with the placebo (P < 0.05). The ratio of Pdi to integrated activity of the EMGdi increased with fenoterol (P < 0.05). Fenoterol treatment increased both superimposed Pdi twitch and Pdi twitch of relaxed diaphragm and decreased the value of (1-superimposed Pdi twitch/Pdi twitch of relaxed diaphragm). Thus we conclude that in normal subjects fenoterol reduces diaphragmatic fatigue and decreases the motor command to the diaphragm, resulting in a decrease in IES during inspiratory threshold loading and a prolongation of endurance.

Adult↗
PubMed1997

Hepatic transplantation in children under 3 months of age: a single centre's experience.

Liver transplantation in neonates represents a major medical and technical challenge particularly in babies weighing less than 5 kg. The authors report the experience of 10 liver transplants in 9 babies (6 boys and 3 girls), mean age, 6 weeks (range, 2 to 11); median weight, 3.7 kg (range, 2.4 to 5). All had fulminant hepatic failure caused by neonatal haemochromatosis (n = 3), non-A non-B hepatitis (n = 3), total parenteral nutrition induced (n = 1), hepatitis B (n = 1), and hepatic haemangio-endothelioma (n = 1). One child underwent retransplantation for hepatic artery thrombosis. Immunosuppression was by Cyclosporine A-based triple therapy in all cases. All received a reduced size graft consisting of left lobe (n = 1), left lateral segment (n = 6) and monosegment III (n = 3). In nine cases the donor hepatic artery was anastomosed to an iliac artery conduit from the infrarenal aorta, and a Roux loop was used for bile duct reconstruction. Primary abdominal wound closure was possible in six patients, skin closure alone in one, and a silastic patch was used in three. Complications included infection (n = 5), bowel perforation (n = 2), diaphragmatic perforation (n = 2), bile leak (n = 1), hepatic artery thrombosis (n = 1), and portal vein thrombosis (n = 1). None of the babies experienced acute rejection. Currently five of the nine recipients are alive with good graft function at a mean follow-up of 22 months (range, 5 to 58). Of the four deaths, two were related to infection (one in combination with portal vein thrombosis), one to multiorgan failure and fluid overload, and one to early graft dysfunction and sepsis after undergoing retransplantation for hepatic artery thrombosis. From our experience liver transplantation offers a promising option for the treatment of severe liver disease in children less than 3 months old.

Age Factors↗
PubMed1993

No effect of naloxone on ventilatory response to progressive hypercapnia in IDDM patients.

The ventilatory response to hyperoxic progressive hypercapnia was examined by comparing 3 test groups: 7 diabetic patients with AN, 8 diabetic patients without AN, and 8 normal control subjects. In each group, a significant linear correlation was found between PaCO2 and VE. The slopes of the regression curves relating PaCO2 to VE were significantly steeper in the healthy control subjects and diabetic patients without AN than in those with AN (P < 0.01). We conclude that the ventilatory response to progressive hypercapnia is reduced in diabetic patients with AN. By analyzing the power spectrum and the amplitude behavior of the diaphragmatic EMG (calculated from the fc and RMS, respectively), we could exclude a disturbance of neural descending pathways and respiratory muscle dysfunction as possible causal mechanisms for the impaired ventilatory response to increasing CO2. By using lung function analysis, causal factors such as alterations in respiratory system mechanics also could be excluded. As diabetes is known to affect the endogenous opioid system, which, in turn, affects the ventilatory response to CO2, naloxone, as a specific opioid antagonist, was administered in all 3 test groups. Naloxone produced a significant increase of ventilatory response to hypercapnia in the healthy control subjects (P < 0.01), but produced no effect in either of the diabetic groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1984

Pulmonary and hematologic changes in swine with Mycoplasma hyopneumoniae pneumonia.

Studies were made of the physiologic changes in the lung of young growing pigs intratracheally inoculated at 5.5 to 6 weeks of age with Mycoplasma hyopneumoniae. Hematologic changes also were studied. Studies were performed at the 4th, 5th, and 6th weeks after inoculations were done. The inoculated pigs had a marked decrease in body weight gain. There were no significant changes in the heart rate, respiratory rate, and rectal temperature, even though at necropsy the apical, cardiac, and diaphragmatic lobes of the lungs of inoculated pigs had scattered, but well-demarcated, pneumonic lesions. The lungs were edematous and increased in weight, compared with those of control (noninoculated) pigs. There were several changes in respiratory functions in the inoculated animals, including significant increases in esophageal pressure negativity, expiratory flow rate, tidal and minute volumes, and work of breathing and significantly less oxygen consumption. Inspiratory flow rate and airway resistance were markedly increased. The elasticity of infected lungs was reduced, as shown by notable decreases of dynamic lung compliance. According to blood gas analyses, acid-base balance had changed in the inoculated pigs. Arterial blood pH, arterial partial pressures of O2 and CO2, and arterial concentration of HCO3- decreased. To some degree respiratory acidosis indicated by a significant decrease of base excess was evident in the blood of inoculated pigs. As a result, inoculated animals required hyperventilation (compensatory respiratory alkalosis) to maintain the pH of arterial blood near a physiologically normal value. The pigs with mycoplasmal pneumonia showed no drastic hematologic changes.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗