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[Functional abnormality of the diaphragm in the "shrinking lung" in systemic lupus erythematosus].

Functional respiratory disorders in patients with systemic lupus erythematosus (SLE) have been previously reviewed. We report a patient with SLE who developed progressive dyspnea with a restrictive pattern in the functional respiratory tests, and diaphragmatic disfunction. Symptoms and functional tests improved after a treatment with steroids. We also review the pathophysiologic mechanisms of this disorder.

Diaphragm↗

Ventilation and exercise performance after phrenic nerve and multiple intercostal nerve transfers for avulsed brachial plexus injury.

BACKGROUND: Diaphragmatic excursion, lung function, exercise performance, and clinical symptoms have not been previously described in patients after phrenic nerve transfer (PNT) and/or multiple intercostal nerve transfer (MIT) for the repair of avulsed brachial plexus injury (ABPI) to prevent functional musculoskeletal impairment in the shoulder. SETTING: A university-based hospital. METHODS: Dyspnea scores, chest ultrasonography to assess diaphragmatic excursion, and pulmonary function testing were performed to assess ventilation in patients sustaining trauma to their brachial plexus. In addition, cardiopulmonary exercise testing was also performed. These studies were obtained prior to surgical intervention, and were repeated postoperatively at 6, 12, 18, 24, and 36 months. The results obtained preoperatively were compared to those obtained throughout the postoperative monitoring period. RESULTS: This study demonstrates that the PNT-MIT procedure results in permanent ipsilateral diaphragmatic paralysis accompanied by an approximately 8% decrease in inspiratory capacity, FVC, and total lung capacity. There was also an 11% increase in diffusing capacity noted during the period between 6 months and 3 years after PNT-MIT procedure. Despite these measurable changes in lung function, the patients reported amelioration of their dyspnea complaint within 6 months of undergoing this procedure, which was due mainly to an improvement in their cardiovascular exercise performance related to increased daily activity. CONCLUSIONS: This study demonstrates that the PNT-MIT procedure is a safe method for the restoration of drop shoulder incurred by ABPI. This surgery has an impact on measurable diaphragmatic and lung function but with minimal impact in terms of postoperative clinical symptoms and exercise performance.

Adult↗

[Respiratory function after traumatic rupture of the diaphragm].

In eight patients who underwent surgical treatment for diaphragmatic rupture, pulmonary function and differential ventilation of the lungs were evaluated four months-one year after surgery. The results of the study show that diaphragmatic rupture produces only a small reduction in total ventilatory function. Moreover our data indicate that the ventilation estimated to be contributed by the side injured, is decreased in a larger extent in abdominal than in thoracic trauma.

Adolescent↗

The effect of diaphragmatic peritoneal lymphatics on peritoneal adhesions: an experimental study.

In this study, we examined the effect of diaphragmatic peritoneal lymphatic function on the formation of peritoneal adhesions. A two phased design was used in fifty-two Wistar albino female rats. In the first phase (n = 12), the effects of diaphragmatic peritoneum damage model on the cecum and the terminal ileum were evaluated. In the second phase, the rats (n = 40) were divided into two equal groups. The adhesion model was created only in the cecum and the terminal ileum in the first group, whereas the diaphragmatic peritoneal damage model was created in the second group together with the same adhesion model. The rats were sacrificed on day 10 postoperatively and the adhesions formed were graded. In the first group, adhesions were formed at grade 1 in 5 rats (25%), grade 2 in 11 rats (55%) and grade 3 in 4 rats (20%), whereas adhesions were formed at grade 1 in 2 rats (10%), grade 2 in 8 rats (40%) and grade 3 in 10 rats (50%) in the second group (X2: 21.65, p < 0.0001). Diaphragmatic peritoneal lymphatic function reduced the number of adhesions and severity of the adhesions which occurred among intraabdominal organs after laparotomy. These findings suggest that special care should be undertaken to avoid damaging the diaphragmatic peritoneum during abdominal surgery so as to reduce the incidence of peritoneal adhesions.

Abdomen↗

Improved pulmonary function after surgical reduction of congenital diaphragmatic hernia in lambs.

BACKGROUND/PURPOSE: Modern trends are toward delayed surgical reduction of congenital diaphragmatic hernia. This study was conducted to verify the hypothesis that the "ease" of ventilation found in the authors' postoperative experience in infants with congenital diaphragmatic hernia (CDH) is associated with postsurgical improvement in pulmonary mechanics. METHODS: Very severe CDH was surgically induced in utero at 90 days' gestation in 31 lambs. At birth pulmonary mechanics (PeDS-Lab) was measured in these preterm lambs with (n = 24) and without (n = 7) early surgical reduction, and in eight non-CDH controls over the same period; functional residual capacity (FRC) also was obtained from 14 of the 31 CDH lambs (seven reduced animals and seven unreduced ones). Management excluded aspiration from the thorax and insertion of chest drains. RESULTS: After 30 minutes of life CDH animals with early surgical reduction demonstrated significantly greater improvement in lung volume, pulmonary mechanics, and oxygenation than those without reduction: FRC, 235% versus 19%; compliance, 57% versus 14%; minute ventilation, 71% versus 30%; and PO2, 143% versus -15%. Over the same period, in preterm controls without CDH, only the compliance varied significantly, demonstrating a 32% increase. CONCLUSION: Based on the mechanics of breathing in these lambs, the authors speculate that neonates with CDH could benefit from early surgical repair because of improvement in pulmonary function, provided extra care is taken to prevent pulmonary overdistension.

Animals↗

Late return of diaphragm function in a ventilator-dependent patient with a high cervical tetraplegia: case report, and interactive review.

Return of diaphragm function in an individual with ventilator-dependent high cervical tetraplegia is presented. The patient was maintained on a ventilator for five years after sustaining a C3-C4 vertebral fracture with a complete high cervical tetraplegia. Diaphragmatic fluoroscopic evaluation at the initial injury time revealed a nonfunctioning diaphragm, and the patient was subsequently discharged home on a portable ventilator. Respiratory evaluation 5 years post injury, including, diaphragmatic fluoroscopy, transcutaneous phrenic nerve conduction studies and pulmonary function tests, revealed intact phrenic nerve function bilaterally with spontaneous diaphragmatic motion. Subsequently, successful ventilatory weaning and diaphragmatic muscle strengthening was achieved. This case report emphasises the importance of serial evaluation of phrenic nerve viability, diaphragmatic function and ventilator dependency in patients with a high cervical tetraplegia in light of potential neurological recovery.

Adult↗

Prognosis with preoperative pulmonary function and lung volume assessment in infants with congenital diaphragmatic hernia.

OBJECTIVES: To determine whether fatal pulmonary hypoplasia, as assessed by functional residual capacity (FRC), can be distinguished from other reversible causes of respiratory failure in infants with congenital diaphragmatic hernia (CDH). METHODS: In the present study, 25 term neonates having CDH without other anomalies (mean birth weight +/- SD, 3.25 +/- 0.50 kg) were enrolled prospectively into a protocol evaluating pulmonary function. Lung compliance (CL) and FRC were measured before diaphragmatic repair and compared with the highest oxygenation index (OI) and lowest PaCO2, also obtained preoperatively. Pulmonary function assessment was repeated after diaphragm repair on postoperative days 3 and 7. CL was determined by esophageal manometry and pneumotachography, and FRC was determined by helium dilution. RESULTS: Fifteen infants (60%) survived to hospital discharge. Eighteen (72%) required extracorporeal membrane oxygenation (ECMO) for support, and of these, 8 (44%) survived. PaCO2 was similar preoperatively in infants grouped as survivors without ECMO, survivors with ECMO, and nonsurvivors. In nonsurvivors (all of whom received ECMO), the preoperative OI was significantly higher (51 +/- 21), CL was less (0.11 +/- 0.04 mL/cm of water per kg), and FRC was smaller (4.5 +/- 1.0 mL/kg) than in the survivors who required ECMO (26 +/- 18, 0.18 +/- 0.08 mL/cm of water per kg, and 12 +/- 5 mL/kg, respectively), as well as in the survivors without ECMO, (6 +/- 2, 0.32 +/- 0.16 mL/cm of water per kg, and 15.8 +/- 4 mL/kg, respectively). The group surviving with ECMO had a higher OI than the infants surviving without ECMO. All nonsurviving infants had FRCs of less than 9.0 mL/kg preoperatively. In contrast, only 2 of the 15 survivors had preoperative FRCs less than 9 mL/kg. CONCLUSIONS: The results of this study suggest that preoperative assessment of FRC may predict fatal pulmonary hypoplasia in most infants with CDH.

Analysis of Variance↗

[Postoperative follow-up of cardia function in newborn infants after esophageal, diaphragmatic and abdominal wall abnormalities].

From 1986 to 1989 46 newborn were examined after correction of oesophageal atresia, defects of diaphragm or abdominal wall, to record postoperative cardial insufficiency. In 15 of 40 long-time pH metry analysis (37.5%) and in 11 of 22 x-ray studies (50%) a pathological result was found; in 8 times these findings were followed by operation and in 8 times by conservative therapy. pH metry is suitable even in very small newborn for postoperative screening of cardial function; if the results are positive, x-ray examination is supplementary to diagnosis and therapeutical planning.

Abdominal Muscles↗

ECMO in newborn infants: the Melbourne experience.

At the Royal Children's Hospital, Melbourne, extracorporeal membrane oxygenation (ECMO) has been used in the treatment of newborn infants with life-threatening respiratory or cardiac failure since May 1989. The main indications for the use of ECMO are that the disease is reversible, the surviving infant is likely to be normal and there is an 80% likelihood of death without ECMO. Sixteen of 22 (73%) newborn infants have survived at least 6 months after ECMO. Fourteen of 16 (87.5%) infants receiving ECMO (who did not have a congenital diaphragmatic hernia) were functionally normal survivors; the other two infants died. Two of six infants with congenital diaphragmatic hernia who received ECMO were discharged and survived to have normal neurological and respiratory function at 6 month follow up. These results are similar to results from other centres internationally. It would appear that ECMO is a useful therapy for near-term newborn infants with otherwise fatal cardiorespiratory failure.

Australia↗

Lung function in infants after repair of congenital diaphragmatic hernia.

BACKGROUND/PURPOSE: Congenital diaphragmatic hernia (CDH) is initially characterized by severe restrictive lung defect and low lung compliance, but survivors have relatively few abnormalities later in life. We studied the changes in lung growth and function in infants after the repair of CDH. METHODS: Retrospective analysis of pulmonary function tests was performed during the first 24 months of life in 56 infants (33 male and 23 female) after repair of CDH. Lung function (functional residual capacity [FRC], respiratory system compliance [C(rs)] and resistance [R(rs)], and maximum expiratory flow rate at FRC [V'(maxFRC)]) were compared among 4 different ages (0-3, 4-6, 7-12, and 13-24 months). RESULTS: All indices of lung function (mean +/- SD of z scores) were abnormal during the first 6 months of life but were almost normalized by 24 months (P < .0001): FRC, from -0.84 +/- 0.5 to 3.26 +/- 2.07; C(rs), from -0.87 +/- 0.4 to 1.84 +/- 1.75; R(rs), from 2.85 +/- 2.71 to -0.23 +/- 2.03, and V'(maxFRC), from -1.63 +/- 0.4 to -0.09 +/- 0.94. There was significant correlation (P < .001) between lung function and increase in age, height, and especially weight. CONCLUSIONS: Lung growth and function gradually normalize between 6 and 24 months of life after repair of CDH.

Airway Resistance↗

Predictive capabilities of preoperative and postoperative pulmonary function tests in delayed repair of congenital diaphragmatic hernia.

To improve the survival of newborns with congenital diaphragmatic hernia (CHD), preoperative stabilization with conventional ventilatory therapy and extracorporeal membrane oxygenation (ECMO) have been used. Measurements that quantify pulmonary function may allow an accurate assessment of lethal pulmonary hypoplasia and predict outcome. Pulmonary function tests (PFTs) were obtained in 20 infants preoperatively and postoperatively; these included measurements of compliance, dynamic compliance, and tidal volume. Overall survival was 75%. Six surviving infants were initially managed with ventilator therapy alone, followed by repair (group 1). The remaining 14 patients, who were moribund at presentation or whose initial ventilator therapy failed, were placed on ECMO and received repair during bypass; nine survived (group 2), and five died (group 3). Compliance, dynamic compliance, and tidal volume obtained at initial presentation and immediately preoperatively were significantly higher for group 1 as compared with groups 2 and 3. Infants whose initial compliance was greater than 0.25 mL/cm H2O/kg and initial tidal volume was greater than 3.5 mL/kg did not require ECMO. Ultimate improvement in compliance was noted in 5 of 6 patients in group 1, 8 of 8 patients in group 2, and 5 of 5 in group 3. This improvement followed an initial decline in compliance in 9 of 14 survivors, from 15% to 76%. All six patients in group 1 had tidal volumes of more than 4 mL/kg, as did 7 of 9 patients in group 2. Only one patient among the ECMO nonsurvivors (group 3) had a postoperative tidal volume of this magnitude. These data suggest that initial PFTs may predict which infants will require ECMO.(ABSTRACT TRUNCATED AT 250 WORDS)

Extracorporeal Membrane Oxygenation↗

Lung function in children after repair of congenital diaphragmatic hernia.

Sixteen children who had had congenital diaphragmatic herniae repaired either in the neonatal period (11), or in early childhood (5), were reviewed at between 7 and 19 years of age. Only minor abnormalities were shown in their chest x-rays and in a variety of lung function tests. This suggests that the long-term prognosis in this condition is good.

Adolescent↗