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1/9. lung volume reduction surgery in bronchopulmonary dysplasia.

    We report on a female preterm infant of 29 wk gestational age, who developed acquired lobar emphysema after prolonged artificial ventilation secondary to respiratory disease syndrome and bronchopulmonary dysplasia. The infant underwent atypical segmentectomy at the age of 12 mo because of life-threatening hypoxaemia with pulmonary hypertension and failure of conservative treatment. Conclusion: lung volume reduction surgery (LVRS) dramatically improved the respiratory function and resulted in adequate weight gain and psychomotor development. In selected cases LVRS can be an option for lobar emphysema in premature infants with severe bronchopulmonary dysplasia.
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ranking = 1
keywords = pulmonary hypertension, hypertension
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2/9. Successful treatment of ARDS and severe pulmonary hypertension in a child with bordetella pertussis infection.

    infection with bordetella pertussis can cause severe illness with neurological and pulmonary complications in children. Pulmonary hypertension is an early sign of potentially fatal disease and can cause failure of conventional respiratory therapy in severe acute respiratory distress syndrome (ARDS). We report a 4 1/2-year-old boy with B. pertussis infection who developed severe ARDS and pulmonary hypertension. Because of severe neurological signs the patient did not qualify for extracorporal membrane oxygenation (ECMO). After conventional ventilation, surfactant and high frequency oscillation ventilation (HFOV) failed, treatment with nitric oxide (NO) improved oxygenation, allowing recovery without the need for ECMO. The patient survived with few sequelae. Thus, this treatment may be an option in high-risk children who meet the criteria for ECMO but are excluded because of poor neurological status, as in our patient.
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ranking = 5.1416367479495
keywords = pulmonary hypertension, hypertension
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3/9. epoprostenol and home mechanical ventilation for pulmonary hypertension associated with chronic lung disease.

    Pulmonary hypertension (PH) can be associated with bronchopulmonary dysplasia (BPD) of infancy, and mortality in these pediatric patients is high without aggressive medical treatment. Continuous intravenous epoprostenol (prostacyclin) was shown to lower pulmonary artery pressures (PAP) in children with idiopathic pulmonary arterial hypertension (PAH), formerly referred to as primary pulmonary hypertension. We report on the first case of long-term home ventilation in combination with chronic intravenous epoprostenol in a child with severe pulmonary hypertension associated with chronic lung disease. This aggressive combination resulted in significant improvement in pulmonary artery pressures, substantial improvement in quality of life, and eventual discontinuation of home ventilation.
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ranking = 5.4249102438486
keywords = pulmonary hypertension, hypertension
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4/9. Oral sildenafil for treatment of severe pulmonary hypertension in an infant.

    We report the use of oral sildenafil in a 5-month-old preterm infant with severe bronchopulmonary dysplasia and pulmonary arterial hypertension refractory to inhaled nitric oxide treatment, maximal ventilatory support and conventional vasodilator therapy. Sildenafil was prepared as a liquid suspension by the method of trituration and administered via an orogastric tube to the patient. Forty-eight hours after sildenafil treatment, echocardiography revealed that the tricuspid incompetence was substantially diminished and the contractility of both ventricles improved, indicating a marked reduction in pulmonary arterial pressure. Oral sildenafil treatment was continued for 6 months until complete resolution of pulmonary arterial hypertension, and oxygen supplement was weaned off. There was no adverse effect during the treatment period. Oral sildenafil may be useful in reducing pulmonary vascular resistance and can be considered for treatment of severe pulmonary arterial hypertension secondary to bronchopulmonary dysplasia.
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ranking = 4.4249102438486
keywords = pulmonary hypertension, hypertension
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5/9. Effects of supplemental oxygen administration in an infant with pulmonary artery hypertension.

    In patients with pulmonary disease, pulmonary artery hypertension often occurs as a result of pulmonary artery vasoconstriction, primarily from hypoxia and alveolar hypotension. In this report we describe the hemodynamic effects of breathing supplemental oxygen in a child with bronchopulmonary dysplasia and pulmonary artery hypertension. These hemodynamic effects include an improvement in oxygenation, an increase in systemic vascular resistance, and a decrease in the pulmonary vascular resistance. As a direct result of these changes in vascular resistances, alterations of heart rate, cardiac index, stroke volume, aortic pressure, oxygen consumption, and pulmonary artery pressure have been shown to occur. Oxygen is widely used to treat many physiologic conditions. However, during the administration of supplemental oxygen, rarely do we recognize the hemodynamic changes associated with its use. These hemodynamic effects must be clearly understood and appreciated before oxygen administration in any clinical situation.
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ranking = 0.84982048769719
keywords = hypertension
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6/9. In utero resolution of hydrops fetalis following the death of one twin in twin-twin transfusion.

    A case of massive hydramnios involving a twin-twin transfusion syndrome is presented with death of the larger twin and the development of hydrops fetalis in the surviving smaller twin. The amniotic fluid volume of the surviving twin became normal and the hydrops fetalis resolved spontaneously in utero. Concomitant with the resolution of the hydrops fetalis, the umbilical cord systolic to diastolic ratio improved. At the time of delivery, the hydrops had resolved. The liveborn infant died 11 days later secondary to pulmonary hypertension and cardiorespiratory failure. Although the fetal status improved in utero, the pulmonary hypertension postdelivery resulted in neonatal death.
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ranking = 2
keywords = pulmonary hypertension, hypertension
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7/9. Treatment of pulmonary hypertension with diltiazem in a child with bronchopulmonary dysplasia.

    A two-year-old child dying of pulmonary hypertension and cor pulmonale secondary to bronchopulmonary dysplasia, was demonstrated to have reactive pulmonary hypertension in response to 100% oxygen and isoproterenol infusion. In an attempt to find an oral medication to maintain pulmonary vasodilatation, experimental trials were done using hydralazine, salbutamol, nifedipine and diltiazem. Cardiac index, pulmonary and systemic vascular resistances and intrapulmonary shunts were monitored during the trials. hydralazine, salbutamol and nifedipine were ineffective. diltiazem 2.0 mg given every 6 h resulted in a profound and sustained decrease in pulmonary pressures and resistance, and a reversal of the cor pulmonale.
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ranking = 6
keywords = pulmonary hypertension, hypertension
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8/9. bronchopulmonary dysplasia and corticosteroid therapy: a case review.

    The use of corticosteroids as a treatment for chronic lung disease is increasing in newborn intensive care units. The anti-inflammatory mechanism of steroids suppresses the pulmonary inflammatory response, leading to improved pulmonary compliance and often a successful extubation. However, side effects, including sepsis, hypertension, glucosuria, and heme positive stools, have been reported. caregivers must appreciate the multisystem biochemical effects of corticosteroids and anticipate the potential side effects when administering this medication. The article reviews the biochemical processes of dexamethasone, a synthetic corticosteroid, and presents a case study of an infant receiving dexamethasone. A question-and-answer format is interpolated throughout the case study and is intended to challenge the reader's knowledge of the pathophysiology of chronic lung disease and the biochemical actions of corticosteroids. Implications for nursing assessment and management of infants receiving this medication are reviewed.
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ranking = 0.14163674794953
keywords = hypertension
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9/9. Congenital alveolar capillary dysplasia: rare cause of persistent pulmonary hypertension.

    We report on a rare case of fatal congenital alveolar capillary dysplasia. The newborn boy of a 37 weeks' normal gestation suffered from persistent pulmonary hypertension without any cardiovascular malformation and died at the age of 4 weeks despite intensive treatment. The autopsy tissue was examined histologically, immunohistochemically, and ultrastructurally. Moreover, a three-dimensional tissue reconstruction based on serial sections was performed comparing the affected lung with normal lung tissue. We observed a unique pattern of pulmonary dysplasia: An extreme decrease of capillaries was localized centrally within thickened intra-acinar septa instead of capillaries intensely neighboring pneumocytes; ectatic veins normally running in the interlobular septa were found to accompany intralobular bronchovascular bundles, denying a clear distinction between pulmonary and bronchial veins; small muscular pulmonary arteries extended to the precapillary level and type 2 pneumocytes exceeded by far the type 1 pneumocytes, inverting the normal ratio. In summary, alveolar capillary dysplasia is assumed to be a primary capillary disorder of unknown origin, which possibly involves the regular differentiation of pneumocytes, according to the close alveolocapillary relationship during pulmonary ontogenesis. We consider the venous alterations as being part of the dysplasia, whereas the arterial phenomena might occur secondarily. Recent reports on affected siblings suggest a genetic component of pathogenesis.
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ranking = 5
keywords = pulmonary hypertension, hypertension
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