Application of extracorporeal membrane oxygenation in the treatment of persistent pulmonary hypertension of the newborn / 中国当代儿科杂志

OBJECTIVES@#To study the clinical value of extracorporeal membrane oxygenation (ECMO) in the treatment of persistent pulmonary hypertension of the newborn (PPHN).@*METHODS@#A retrospective analysis was performed on the medical data of 11 neonates with PPHN who were treated with ECMO in the Neonatal Intensive Care Unit of Zhongshan People's Hospital from January 2015 to December 2021, involving the neonates' general information, clinical diagnosis, laboratory results, duration of ECMO treatment, complications during ECMO treatment, length of hospital stay, and outcome.@*RESULTS@#Of the 11 neonates, 10 (91%) had successful weaning from ECMO, and 8 (73%) survived. For the 11 neonates, the mean duration of ECMO treatment was (81±50) hours (range: 26 to 185 hours), the mean duration of ventilator use was (198±105) hours (range: 57 to 392 hours), and the mean length of hospital stay was (22±15) days (range: 2 to 49 days). The oxygenation index and blood lactate level were significantly improved after 24 hours of ECMO treatment among the 11 neonates (P<0.05). Ten neonates had significantly reduced pulmonary artery pressure after 24 hours of ECMO treatment (P<0.05). One neonate had a progressive increase in the pulmonary artery pressure during EMCO treatment, succumbing to death. This neonate was diagnosed with alveolar capillary dysplasia based on the histopathological findings of the lung tissue and whole-exome sequencing results. Among the 11 children, 5 had intracranial hemorrhage, 1 had disseminated intravascular coagulation, 1 had gastric hemorrhage, 2 had pulmonary hemorrhage, 1 had renal insufficiency, and 3 had bleeding at the puncture site during ECMO treatment.@*CONCLUSIONS@#ECMO is effective for the treatment of PPHN, however, the high incidence of complications of ECMO treatment suggests that it is important to carefully assess the indications and timing of ECMO treatment and improve the management of ECMO, which can improve the weaning rate and survival rate.

The architecture of alveolar capillaries in the lungs of bactrian camels (Camelus bactrianus) / Arquitectura de los capilares alveolares de pulmones de camellos bactrianos (Camelus bactrianus)

SUMMARY: The Bactrian camel, which is native to China and Mongolia, is large in size and is an even-toed ungulate species. The double humps on the Bactrian camel back differentiate it from the dromedary camel, which has a single hump. This species has adapted to unsuitable conditions (lack of food and water) in the Gobi Desert and is advanced in unique anatomical and physiological characteristics during a prolonged evolution period. Several studies have been conducted on the anatomical features of the Bactrian camel, but none have given attention to the alveolar capillaries of the Bactrian camel lung. Therefore, the current study aims to explore the architecture of the alveolar capillary in the Bactrian camel lung and further explain the mechanism of blood flow in its lung. The current study extracted and examined the architecture of the alveolar capillary in the lung of the Bactrian camel (Camelus bactrianus) and further explained the mechanism of blood flow by performing lung casting and replica scanning electron microscopy methods. The reports showed that the resources of the alveolar-capillary originated from the capillaries of the subpleural space or interlobular septulum, sometimes originating from the precapillary arterioles or directly from the terminal arterioles. The alveolar capillaries anastomosed and formed a single layer of dense, basket-like network surrounding the alveolus. The mash diameter of the alveolar-capillary network was larger than that of the capillary, and the appearance of the mash was oval and elliptical. Many of the collapsed alveolar-capillary networks were found in the alveolar microvascular architecture in the lung of the Bactrian camel. The study found that, due to many collapsed alveoli in the Bactrian camel lung, the disproportional pressure between the pulmonary alveoli induced less imbalance of blood flow in the alveolar capillary, which affected the gas exchange efficiency. Therefore, the function of the anastomosing capillary branch was likely to regulate the blood flow between the alveolar-capillary network.

RESUMEN: El camello bactriano, es originario de China y Mongolia, es de gran tamaño y es una especie de ungulado de dedos pares. Las dobles jorobas del lomo del camello bactriano lo diferencian del dromedario, que tiene una sola joroba. Esta especie se ha adaptado a condiciones inadecuadas (falta de alimento y agua) en el desierto de Gobi y ha avanzado en características anatómicas y fisiológicas únicas durante un período de evolución prolongado. Se han realizado varios estudios sobre las características anatómicas del camello bactriano, pero ninguno ha prestado atención a los capilares alveolares del pulmón de este animal. Por lo tanto, el presente estudio tuvo como objetivo principal explorar la arquitectura del capilar alveolar en el pulmón del camello bactriano y explicar el mecanismo del flujo sanguíneo. A partir de nuestro trabajo se examinó la arquitectura del capilar alveolar en el pulmón del camello bactriano (Camelus bactrianus) mediante la realización de métodos de microscopía electrónica de barrido y escaneo pulmonar. Los informes mostraron que los recursos del alvéolo-capilar se originaban en los capilares del espacio subpleural o del tabique interlobulillar y a veces se originaban en las arteriolas precapilares o directamente en las arteriolas terminales. Los capilares alveolares se anastomosaban y formaban una densa red de capa única en forma de cesta que rodeaba el alvéolo. El diámetro del macerado de la red alveolar-capilar era mayor que el del capilar y el aspecto del macerado era ovalado y elíptico. Muchas de las redes alvéolo-capilares colapsadas se encontraron en la arquitectura microvascular alveolar en el pulmón del camello bactriano. El estudio encontró que, muchos alvéolos colapsados en el pulmón del camello bactriano, la presión desproporcionada entre los alvéolos pulmonares inducía un menor desequilibrio del flujo sanguíneo en el capilar alveolar, lo que afectaba la eficiencia del intercambio de gases. Por lo tanto, la función de la rama capilar anastomosante probablemente regularía el flujo sanguíneo entre la red alveolar-capilar.

Research advancement of sphingosine 1-phosphate in alveolar capillary endothelial barrier regulation / 药学学报

The alveolar capillary endothelial barrier is mainly composed of alveolar capillary endothelial cells and alveolar epithelial cells. The destruction of this barrier and the continuous infiltration of inflammatory cells have been considered to play an important role in the development of chronic obstructive pulmonary disease, acute lung injury, and idiopathic pulmonary fibrosis. Therefore, it is of great significance to understand the mechanism of alveolar capillary endothelial barrier regulation. Sphingosine 1-phosphate (S1P) is a bioactive sphingolipid metabolite produced by sphingosine kinase. A large number of studies have shown that S1P not only regulates immune cell transport, but also plays important roles in regulating cell apoptosis, vascular endothelial barrier, and alveolar epithelial barrier. S1P exerts different regulatory effects on alveolar capillary endothelial barrier by activating S1P1 and S1P3. Activation of S1P1 on the alveolar capillary endothelial cells by S1P mediates barrier protection, while the barrier can be broken when S1P3 is stimulated by S1P. S1P can also regulate alveolar epithelial barrier. By activating S1P3 on the alveolar epithelial cells, S1P leads to epithelial barrier damage, which makes interstitial proteins and body fluids infiltrate into alveolar space and causes pulmonary edema. Therefore, it may be a target for the treatment of lots of lung diseases by regulating the homeostasis of alveolar capillary endothelial barrier. This paper reviews the research advancement of S1P in alveolar capillary endothelial barrier regulation.

A Novel De Novo Pathogenic Variant in FOXF1 in a Newborn with Alveolar Capillary Dysplasia with Misalignment of Pulmonary Veins

Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is an autosomal dominant, fatal developmental disorder of the lungs, with a mortality rate of about 100%. ACD/MPV is caused by mutations in FOXF1. Herein, we describe a newborn boy with ACD/MPV carrying a novel pathogenic variant of FOXF1. The patient developed respiratory distress and severe pulmonary hypertension on the first day of life. Despite aggressive cardiorespiratory management, including veno-venous extracorporeal membrane oxygenation, his condition deteriorated rapidly, and he died within the first month of his life. Lung histology showed the characteristic features of ACD/MPV at autopsy. Sequence analysis of FOXF1 from genomic DNA obtained from autopsied lung tissue revealed that the patient was heterozygous for a novel missense variant (c.305T>C; p.Leu102Pro). Further analysis of both parents confirmed the de novo occurrence of the variant. To the best of our knowledge, this is the first report of genetically confirmed ACD/MPV in Korea.

Enfermedad pulmonar difusa: causa de hipertensión pulmonar persistente antes del año de vida / Diffuse lung disease: cause of persistent pulmonary hypertension before one year of age

En pediatría, la enfermedad vascular pulmonar es multifactorial y heterogénea. Si bien comparte algunas características con la hipertensión pulmonar en los adultos, hay diferencias en las comorbilidades y condiciones asociadas, la coexistencia de enfermedades genéticas o del desarrollo. Las enfermedades pulmonares intersticiales pueden ser causantes de esta entidad. Una de ellas es la displasia alvéolo-capilar con mal alineamiento de las venas pulmonares, una patología infrecuente pero con 100% de mortalidad, caracterizada por la falla en la formación del tejido pulmonar que da por resultado final la alteración en la difusión de gases. Se describe un caso clínico de una paciente de 5 meses de edad estudiada a partir de sospechar una cardiopatía congénita con hipoxemia persistente, a cuyo diagnóstico se llega por la biopsia pulmonar.

Pulmonary vascular disease in children is multifactorial and heterogeneous. While it shares some features with pulmonary hypertension in adults, there are differences in the associated comorbidities and conditions, the coexistence of genetic or developmental diseases. Interstitial lung diseases may be responsible for this entity. One is alveolar capillary dysplasia with misalignment of pulmonary veins, a rare pathology but with a mortality rate of 100%, characterized by a failure in the formation of lung tissue that eventually results in impaired gas diffusion. We present a 5-month-old patient studied due to suspected congenital heart disease with persistent hypoxemia; diagnosis was made through lung biopsy.

Alveolar Capillary Dysplasia as a Cause of Persistent Pulmonary Hypertension.

Background: Persistent pulmonary hypertension (PPHN) in a term or late preterm has varied etiology. Case characteristics: A late preterm neonate operated for esophageal atresia with tracheo-esophageal fistula was complicated by severe pulmonary hypertension and unable to be weaned off from respiratory support. Outcome: The neonate expired by 15 weeks of life; diagnosis was made on postmortem lung biopsy. Message: Alveolarcapillary dysplasia should be considered in a neonate with idiopathic refractory PPHN, if associated with anomalies

Alveolar Capillary Dysplasia With Anorectal Anomaly.

Alveolar capillary dysplasia (ACD) is an uncommon cause of irreversible persistent pulmonary hypertension in full-term newborn. In ACD there is a failure of formation of air - blood barrier in addition to misalignment of pulmonary veins. The etiology of the disease is still not understood. We present a case report of a full-term newborn with ACD associated with anorectal anomaly.

Evaluation of Lung Permeability in Patients with Connective Tissue Disease using 99mTc-DTPA Aerosol Scintigraphy / 대한류마티스학회지

OBJECTIVE: The association between the connective tissue diseases and lung diseases is well established. DLCO and 99mTc-DTPA aerosol scintigraphy are used for evaluation of the alvelolar-capillary permeability. This study evaluated the changes in permeability of alveolar-capillary membrane and the utility of the 99mTc-DTPA aerosol clearance to detect lung involvement in patients with connective tissue diseases. METHODS: The patient group consisted of the patients with any proven connective tissue diseases (27 rheumatoid arthritis, 17 systemic lupus erythematosus, 7 other connective tissue diseases) and the control group consisted of healthy 12 persons. The patients and controls were non-smokers and had no concomitant diseases that could affect the result (diabetes, any lung diseases etc). Chest X-ray, spirometric measurements of lung volumes, flow idices, diffusing capacities and 99mTc-DTPA aerosol scintigraphy were performed in the patient group and control group. 99mTc-DTPA aerosol (1110 MBq) was used with the aero-vent jet nebulizer as a lung delivery system. Patients in sitting position inhaled for 5 minutes at normal tidal oral breathing, Scintigraphic data were recorded using the Picker Prism 2000 gamma cammera, 15 frames of the lung were obtained as the area of interest anteriorly and posteriorly (120 msec at each frame, for 30 minutes). 99mTc-DTPA clearance rate was calculated as the time to half clearance (T1/2). RESULTS: The mean clearance rates of 99mTc-DTPA were 64.0+/-24.1 min (RA 70.7+/-26.2 min, SLE 61.6+/-14.0 min, Others 43.9+/-24.7 min), and 47.0+/-10.3 min in the patient group and the control group respectively. Significant correlation was not found between the pulmonary clearance rate of 99mTc-DTPA and other parameters (disease duration, ESR, CRP, DLCO and FEV1/FVC). CONCLUSION: 99mTc-DTPA clearance in the patient group (RA, SLE, others) was significantly decreased than that in control group (p<0.05). In the patient group with normal chest X-ray, 99mTc-DTPA clearance in the connective tissue disorders was significantly decreased than control group (p<0.05). We suggest that 99mTc-DTPA aerosol scintigraphy may be one of useful technique for early detection of the lung involvement in the connective tissue disorders.

Evaluation of Lung Permeability in Patients with Connective Tissue Disease using 99mTc-DTPA Aerosol Scintigraphy / 대한류마티스학회지

OBJECTIVE: The association between the connective tissue diseases and lung diseases is well established. DLCO and 99mTc-DTPA aerosol scintigraphy are used for evaluation of the alvelolar-capillary permeability. This study evaluated the changes in permeability of alveolar-capillary membrane and the utility of the 99mTc-DTPA aerosol clearance to detect lung involvement in patients with connective tissue diseases. METHODS: The patient group consisted of the patients with any proven connective tissue diseases (27 rheumatoid arthritis, 17 systemic lupus erythematosus, 7 other connective tissue diseases) and the control group consisted of healthy 12 persons. The patients and controls were non-smokers and had no concomitant diseases that could affect the result (diabetes, any lung diseases etc). Chest X-ray, spirometric measurements of lung volumes, flow idices, diffusing capacities and 99mTc-DTPA aerosol scintigraphy were performed in the patient group and control group. 99mTc-DTPA aerosol (1110 MBq) was used with the aero-vent jet nebulizer as a lung delivery system. Patients in sitting position inhaled for 5 minutes at normal tidal oral breathing, Scintigraphic data were recorded using the Picker Prism 2000 gamma cammera, 15 frames of the lung were obtained as the area of interest anteriorly and posteriorly (120 msec at each frame, for 30 minutes). 99mTc-DTPA clearance rate was calculated as the time to half clearance (T1/2). RESULTS: The mean clearance rates of 99mTc-DTPA were 64.0+/-24.1 min (RA 70.7+/-26.2 min, SLE 61.6+/-14.0 min, Others 43.9+/-24.7 min), and 47.0+/-10.3 min in the patient group and the control group respectively. Significant correlation was not found between the pulmonary clearance rate of 99mTc-DTPA and other parameters (disease duration, ESR, CRP, DLCO and FEV1/FVC). CONCLUSION: 99mTc-DTPA clearance in the patient group (RA, SLE, others) was significantly decreased than that in control group (p<0.05). In the patient group with normal chest X-ray, 99mTc-DTPA clearance in the connective tissue disorders was significantly decreased than control group (p<0.05). We suggest that 99mTc-DTPA aerosol scintigraphy may be one of useful technique for early detection of the lung involvement in the connective tissue disorders.