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Pulmonary Hypoplasia (pulmonary + hypoplasia)
Selected AbstractsThe outcome in newborns with congenital diaphragmatic hernia in a Norwegian regionACTA PAEDIATRICA, Issue 1 2009S Fredly Abstract Aim: To evaluate the therapeutic strategies used in neonates with congenital diaphragmatic hernia (CDH) during the last 15 years in our department. Method: A retrospective study of 27 neonates with CDH treated at the Neonatal Intensive Care Unit at Ullevaal University Hospital between 1992 and 2006. Since 1992 we have used delayed operative repair and high-frequency ventilation (HFV). Because surfactant replacement and inhaled nitric oxide (iNO) therapy have been used since 1997, we divided the patients into two groups; group 1 from 1992 to 1996 (9 patients) and group 2 from 1997 to 2006 (18 patients). Results: The overall survival was 70%. Group 1 had an exceptionally good outcome, 100% survival versus 56% in the last group. Conclusion: Pulmonary hypoplasia and pulmonary hypertension are still the most challenging factors in treatment of neonates with CDH, despite novel therapeutic modalities, such as HFV, surfactant and iNO. Delayed surgery in CDH allows pre-operative stabilization. Extracorporeal membrane oxygenation must be considered in the most severe cases. [source] Contractile activity of skeletal musculature involved in breathing is essential for normal lung cell differentiation, as revealed in Myf5,/,:MyoD,/, embryosDEVELOPMENTAL DYNAMICS, Issue 3 2005Mohammad Reza Inanlou Abstract In the current study, the role of contractile activity of respiratory muscles in fetal lung growth and cell differentiation was examined using Myf5,/,:MyoD,/, mouse embryos. As previously found, Myf5,/,:MyoD,/, mouse embryos had no respiratory musculature. Consequently, they suffered from pulmonary hypoplasia and died shortly after birth. The hypoplastic lung had decreased proliferation and increased apoptotic index as early as embryonic day 14.5. By contrast, only at the last gestational day, the number of lung cells expressing platelet derived growth factor B and insulin growth factor I was decreased, while the gradient of the thyroid transcription factor 1 was not maintained. Type II pneumocytes had a failure in glycogen utilization and surfactant storage and secretion but were able to synthesize the surfactant-associated proteins. Type I pneumocytes were readily detectable using an early differentiation marker (i.e., Gp38). However, the late differentiation of type I pneumocytes never occurred, as revealed by transmission electron microscopy. Together, our findings suggest that pulmonary distension due to fetal breathing-like movements plays an important role not only in lung growth but also in lung cell differentiation. Developmental Dynamics 233:772,782, 2005. © 2005 Wiley-Liss, Inc. [source] Congenital lung malformations in the second trimester: Prenatal ultrasound diagnosis and pathologic findingsJOURNAL OF CLINICAL ULTRASOUND, Issue 5 2007Ágnes Harmath MD Abstract Purpose. To correlate prenatal sonographic diagnosis of cystic lung malformations with fetopathologic findings after termination of pregnancy. Methods. We retrospectively analyzed the data of 16 terminated cases in which a cystic lung lesion was diagnosed pre- or postnatally. Results. On average, prenatal diagnosis was established on the 21st gestational week (range, 19,26 weeks). The cause of termination was severe polyhydramnios in 4 cases, nonimmune fetal hydrops in 4 cases, other congenital malformation in 5 cases (renal malformation, 2 cases; congenital diaphragmatic hernia, 3 cases), and obstetrical conditions (intrauterine death, placental abruption, spontaneous abortion) in 3 cases. In 11 cases, congenital cystic adenomatoid malformation (CCAM) was the presumptive prenatal diagnosis. Autopsy confirmed the prenatal diagnosis in 6 of them, while in the other 5 cases, an enteric cyst, a laryngeal atresia, an unidentified tumor, a pulmonary hypoplasia, and an extralobar pulmonary sequestration were found on histologic examination. On the other hand, the autopsy revealed CCAM in those 5 cases in which other malformations were suggested prenatally. Conclusion. The prenatal sonographic diagnosis of CCAM is difficult. Our cases emphasize the important role of fetopathology even today in the verification of prenatal diagnosis based on sonographic examinations. © 2007 Wiley Periodicals, Inc. J Clin Ultrasound, 2007 [source] A novel epidermal nevus syndrome with congenital cylindromatous turban tumorJOURNAL OF CUTANEOUS PATHOLOGY, Issue 9 2003Jacinto J. Regalado Background:, Epidermal nevi (in the broad sense of epithelial nevi) may give rise to benign or malignant skin tumors. They may also be associated with anomalies of other organ systems in an epidermal nevus syndrome. Results:, This article describes a preterm infant with nevus sebaceus of the scalp and face, a large turban tumor with features of malignant cylindroma and multiple non-cutaneous defects. These included skeletal, hematopoietic, hepatobiliary, and urinary anomalies. Severe secondary lesions were present (pulmonary hypoplasia due to oligohydramnios; cerebral infarcts probably related to the turban tumor). Karyotype was normal, and family history was negative. Conclusions:, This unique case is unlike any reported epidermal nevus syndrome. Similarly, there is no prior report of a congenital cylindroma, certainly not as a turban tumor, which implies very rapid growth. The presence of both overgrowth and undergrowth phenomena (e.g. hypoplastic urinary tract and biliary atresia) may reflect dysregulation of paracrine growth factors, presumably due to genetic mutation. [source] Exomphalos , a major or minor problem?PEDIATRIC ANESTHESIA, Issue 9 2002T. O'Neill Introduction The mortality and morbidity statistics associated with exomphalos major remain discouraging despite advances in management techniques (1). Congenital lung pathology, in particular pulmonary hypoplasia, and thoracic maldevelopment, have been strongly allied to this condition, accounting for the high incidence of pulmonary insufficiency necessitating prolonged ventilatory support in these infants (2). We discuss the respiratory issues in an infant with a particularly severe form of exomphalos major, and the impact of a comprehensive parental website devoted to the infants' management and progress. Case Report A female infant, born at 38 weeks' gestation, was referred for management of exomphalos major. Due to the extensive nature of the abdominal wall defect, primary surgical closure was impossible and initial management consisted of staged reduction by external compression of the exomphalos. This resulted in escalating cardiovascular and respiratory embarrassment, and was abandoned in favour of conservative treatment, whereby the sac was dressed and allowed to epithelialise. Thereafter the clinical course was characterised by chronic pulmonary insufficiency requiring prolonged ventilatory support. Ventilator dependence did not significantly decrease until lung growth occurred and the sitting position was adopted, enabling weaning from conventional ventilation to genuine BIPAP at 6 months. Currently, after 11 months, we are preparing her for entry into a home ventilation programme. Throughout this period, progress and realistic goals were discussed at multidisciplinary case conferences involving the parents. The interpreted medical management has subsequently been displayed by the parents on an elaborate, up-to-date website, which is part of a larger ,Mother Of Omphalocele' network. Although innovative, this highlights the fact that we the medical profession, should be vigilant with regard to potential public exposure of patient management. Whilst the Internet has become an integral part of our own continuing education, this case highlights a new aspect of how it may be used by our patients and their relatives to compare and contrast management policies in various institutions. [source] Expression and function of phosphodiesterases in nitrofen-induced congenital diaphragmatic hernia in ratsPEDIATRIC PULMONOLOGY, Issue 4 2010Irene W.J.M. van der Horst MD Abstract Background Congenital diaphragmatic hernia (CDH) is an anomaly associated with pulmonary hypoplasia and pulmonary hypertension (PH). The limited efficacy of current approaches to treat PH in CDH, including inhaled nitric oxide (NO), drives the search for other therapies. Phosphodiesterases (PDEs) degrade cyclic nucleotide second messenger cAMP and cGMP downstream of NO thereby limiting the vasodilatory response to NO. Objective To identify therapeutic targets by cataloguing the expression and function of PDE isoforms in the pulmonary vasculature in nitrofen-induced CDH in fetal rats. Methods/Results Quantitative RT-PCR revealed PDE1,5 and PDE9 mRNA expression in pulmonary arteries (PAs) of control and nitrofen-induced CDH term fetal rats. In this order of potency, the PDE inhibitors Sildenafil (PDE5),>,EHNA (PDE2),>,Rolipram (PDE4),>,Cilostamide (PDE3) all dilated isolated third generation PA after pre-constriction with the thromboxane analog U46619. Hyperoxic pre-incubation of PAs significantly attenuated vasodilatation induced by the PDE5 inhibitor Sildenafil (65% vs. 33%, P,<,0.004). CDH PAs dilated significantly less to PDE2 inhibitor EHNA compared to control (51% vs. 72%, P,<,0.05). Subsequently PDE2 protein expression was higher in PAs of CDH animals. Conclusion Most PDE isoforms exist in the PAs of fetal rats and their inhibition causes pulmonary vasodilatation. PDE5 inhibition was the most potent vasodilator, however, there were no differences between groups. PDE5-induced vasodilatation was attenuated by hyperoxic pre-incubation. PDE inhibitors might be considered therapeutic targets in combination with iNO in neonates with CDH. Pediatr Pulmonol. 2010; 45:320,325. © 2010 Wiley-Liss, Inc. [source] Rodriguez lethal acrofacial dysostosis syndrome with pulmonary hypoplasiaPEDIATRICS INTERNATIONAL, Issue 4 2009Masakazu Miyawaki No abstract is available for this article. [source] Nitric oxide inhalation therapy in very low-birthweight infants with hypoplastic lung due to oligohydramniosPEDIATRICS INTERNATIONAL, Issue 1 2004Naoki Uga AbstractBackground: Although nitric oxide inhalation (iNO) therapy improves arterial oxygenation and reduces the rate of extracorporeal membrane oxygenation in term neonates, the efficacy of this therapy in premature infants is controversial. The objective of the present study was to determine whether iNO therapy improves the survival of very low-birthweight infants with pulmonary hypoplasia due to prolonged rupture of membrane. Methods: A retrospective comparative study of very low-birthweight infants with pulmonary hypoplasia due to oligohydramnios who had or had not been treated with iNO therapy, was performed (iNO-treated group, eight infants; control group, 10 infants). A neonate was considered to have pulmonary hypoplasia due to oligohydramnios if the following conditions were satisfied: (i) artificial surfactant treatment did not improve the respiratory distress; (ii) prolonged rupture of membrane (PROM) continued for more than 5 days with oligohydramnios; and (iii) sufficient arterial oxygenation did not occur even after giving 100% oxygen, and more than 8 cm H2O of mean airway pressure was needed to maintain arterial oxygenation. Results: Nitric oxide inhalation improved arterial oxygenation rapidly and consistently in all eight infants with pulmonary hypoplasia. All eight iNO-treated infants survived longer than 28 days, while five of the 10 control infants died within 24 h of birth (P < 0.05). Before starting iNO, seven of the eight treated infants had shown persistent pulmonary hypertension, which was confirmed by echocardiography. No iNO-treated infant had IVH greater than grade 1, while one control infant had grade 2 IVH. All six long-term survivors in the iNO-treated group are developing normally, while only two of the control infants are developing normally as of February 2002. Conclusions: The majority of the infants with pulmonary hypoplasia due to oligohydramnios had persistent pulmonary hypertension. iNO improved the arterial oxygenation and significantly improved the survival rate. A controlled study to determine whether iNO therapy improves the survival rate of preterm infants with pulmonary hypoplasia due to oligohydramnios is necessary. [source] Fetal enterolithiasis: prenatal sonographic and MRI diagnosis in two cases of urorectal septum malformation (URSM) sequencePRENATAL DIAGNOSIS, Issue 4 2006Marek Lubusky Abstract Objectives Enterolithiasis (multiple calcifications of intraluminal meconium) is a rare, prenatal ultrasonographic finding. In this study, our aim was to evaluate the prenatal diagnostic features and discuss the management of the patients. Methods The data of two cases of prenatally diagnosed fetal enterolithiasis were collected from ultrasound scan, magnetic resonance imaging (MRI) and neonatal or postnatal autopsy records. The findings were evaluated in both prenatal and postnatal periods. Chromosomal analysis was performed in one case. An evaluation of primary and secondary malformations was done. Coexisting anomalies were searched for via radiology, neonatal surgery and histopathology. Results Malformations in two cases (both males) with partial and complete urorectal septum malformation (URSM) sequence were described. The absence of an anal opening and presence of a fistula between the urinary and gastrointestinal tract were common findings. These features were considered as primary malformations contributing to the formation of enterolithiasis. Secondary anomalies (urinary and gastrointestinal system malformations, pulmonary hypoplasia, genital and other coexisting anomalies) were evaluated. Conclusions The prenatal detection of enterolithiasis carries a poor prognosis. Most of the previously reported cases were invariably associated with major fetal malformations of the urinary and gastrointestinal tract. It is a warning sign for large bowel obstruction with or without enterourinary fistula. Therefore, adequate gastrointestinal and urologic studies must be undertaken after birth for the final diagnosis. There is a high mortality rate in the reported cases, mostly attributed to associated anomalies, and all survivors required neonatal surgery. It is important to differentiate the partial from the full URSM sequence because the prognosis in the partial URSM sequence is generally good, with long-term survival being common. Copyright © 2006 John Wiley & Sons, Ltd. [source] Congenital diaphragmatic hernia and pulmonary hypoplasia: New insights from developmental biology and genetics,AMERICAN JOURNAL OF MEDICAL GENETICS, Issue 2 2007Kate G. Ackerman First page of article [source] Laryngeal hamartoma causing polyhydramnios and lethal pulmonary hypoplasiaAUSTRALIAN AND NEW ZEALAND JOURNAL OF OBSTETRICS AND GYNAECOLOGY, Issue 3 2010S. WEBB No abstract is available for this article. [source] Linking animal models to human congenital diaphragmatic herniaBIRTH DEFECTS RESEARCH, Issue 8 2007Niels Beurskens Abstract BACKGROUND:Congenital diaphragmatic hernia (CDH) is a major life-threatening malformation, occurring in approximately 1 in 3,000 live births. Over the years, different animal models have been used to gain insight into the etiology of this complex congenital anomaly and to develop treatment strategies. However, to date the pathogenic mechanism is still not understood, and treatment remains difficult because of the associated pulmonary hypoplasia and pulmonary hypertension. METHODS: In this review, data available from several animal models will be discussed. The retinoic acid signaling pathway (RA pathway, retinoid pathway) will be addressed as a developmental pathway that is potentially disrupted in the pathogenesis of CDH. Furthermore, genetic factors involved in diaphragm and lung development will be discussed. CONCLUSIONS: With this review article, we aim to provide a concise overview of the current most important experimental genetic data available in the field of CDH. Birth Defects Research (Part A), 2007. © 2007 Wiley-Liss, Inc. [source] Congenital diaphragmatic hernia: an overview of the etiology and current managementACTA PAEDIATRICA, Issue 4 2009Alejandra Gaxiola Abstract Aim: To review provide an overview of the etiology and current strategies in the management of congenital diaphragmatic hernia (CDH). Methods: We did a comprehensive review of research trends, evidence based studies and epidemiologic studies. Results: CDH is a life-threatening pathology in infants, and a major cause of death due to the pulmonary hypoplasia and pulmonary hypertension. There is much research related to elucidating the etiology of CDH and developing management strategies to improve the outcomes in these infants. Conclusion: An early diagnosis with increased understanding of this disease is a crucial factor for a timely approach to managing the critically ill infant, and to offer the potential for improved outcomes and substantial reductions in morbidity. [source] | |||||||||||||