Pulmonary hypertension with diffuse lung lesions in cobalamin C defect

2019 ◽  
Vol 61 (10) ◽  
pp. 1062-1063
Author(s):  
Hiroyuki Yoshizawa ◽  
Keiji Nogami ◽  
Hiroaki Yaoi ◽  
Midori Shima
Keyword(s):  
Pulmonary Hypertension ◽  
Lung Lesions ◽  
Diffuse Lung

scholarly journals Adult-onset hypoxaemia, diffuse lung lesions, and pulmonary hypertension in cobalamin C defect: a case report

2021 ◽  
Vol 5 (6) ◽  
Author(s):  
Qin-Hua Zhao ◽  
Wen-Hui Wu ◽  
Li-Jun Fu ◽  
Lan Wang
Keyword(s):  
Pulmonary Hypertension ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
Gene Mutations ◽  
Autosomal Recessive Disorder ◽  
Serum Levels ◽  
Lung Lesions ◽  
The Central Nervous System ◽  
Metabolic Screening ◽  
Diffuse Lung

Abstract Background Cobalamin C (cbl-C) defect is an inherited autosomal recessive disorder that commonly affects the central nervous system of infants. Severe pulmonary hypertension (PH) and diffuse lung lesions are unusual clinical manifestations, especially among adults. Case summary A 25-year-old man with hypoxaemia, diffuse lung lesions, and PH, suddenly developed nausea, vomiting, headache, and worsening of dyspnoea. Metabolic screening showed elevated serum levels of methylmalonic acid and homocysteine, and genetic testing revealed MMACHC gene mutations. He was eventually diagnosed with severe PH secondary to cbl-C defect and was successfully managed with vitamin B12, betaine, L-carnitine, folate, as well as ambrisentan and sildenafil. Discussion cbl-C is a rare cause of PH and can present with severe PH and diffuse lung lesions in adults. Given that the condition is treatable, a careful metabolic screening should be considered when a diagnosis of PH is made.

scholarly journals Cavitating Lung Lesions in Chronic Thromboembolic Pulmonary Hypertension

2008 ◽  
Vol 2 (3) ◽  
Author(s):  
Heather Harris ◽  
Richard Barraclough ◽  
Christine Davies ◽  
Iain Armstrong ◽  
David Kiely ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Lung Lesions ◽  
Thromboembolic Pulmonary Hypertension

scholarly journals Intracardiac hemodynamic abnormalities in patients with sarcoidosis

2018 ◽  
Vol 28 (5) ◽  
pp. 567-575
Author(s):  
E. I. Leonova ◽  
E. I. Shmelev ◽  
R. B. Amansakhedov
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Fibrosis ◽  
Right Ventricular ◽  
Diastolic Dysfunction ◽  
Systolic Function ◽  
Cor Pulmonale ◽  
Pulmonary Sarcoidosis ◽  
Right Heart ◽  
Lung Lesions ◽  
The Right

The aim of this study was to evaluate intracardiac hemodynamic abnormalities in patients with pulmonary sarcoidosis and to investigate relevant factors. Methods. The study involved 42 patients with chronic pulmonary sarcoidosis. Chest computed tomography (CT), echocardiography, body plethysmography, spirometry, 6-minute walking test, and measurements of lung diffusing capacity (DLCO), blood gases and blood C-reactive protein (CRP) were performed in all patients. Results. Chronic cor pulmonale was detected in 26.2% of patients. The right heart diastolic dysfunction was found in 14.3% of patients, the left heart diastolic dysfunction was found in 23.8% of patients, the mean pulmonary artery pressure (mPAP) was increased in 19% of patients. The right ventricular diameter was found to be related to the inspiratory bronchial resistance (Rin) (R = 0.480; p = 0.02), the intrathoracic gas volume (ITGV) (R = –0.670; p = 0.001), DLCO (R = 0.438; p = 0.013), and pulmonary fibrosis. The right heart systolic function (TAPSE) was related to DLCO (R = 0.518; p = 0.006), the total lung capacity (TLC) (R = 0.639; p = 0.001) and pulmonary fibrosis. The increased mPAP was related to the extension of disseminated lung lesions (R = 0.716; p = 0.018), blood oxygen partial pressure (R = 0.486; p = 0.017) and CRP level. The 6-min distance was related to Rin, the right ventricular diameter, parameters of the right heart diastolic function (tricuspid E / A ratio) (R = 0.486; p = 0.01), and ITGV (R = 0.494; p = 0.006). Conclusion. The development of cor pulmonale in patients with pulmonary sarcoidosis was related to DLCO, Rin and pulmonary fibrosis. The extension of disseminated lung lesions, hypoxemia and increased CRP were related to pulmonary hypertension. Different factors associated with cor pulmonale and pulmonary hypertension in sarcoidosis could prompt further investigation of different phenotypes of this disease with the intrinsic central hemodynamic abnormalities. 

scholarly journals Trephine biopsy for diffuse lung lesions.

BMJ ◽  
1967 ◽  
Vol 3 (5556) ◽  
pp. 30-32 ◽  
Author(s):  
S. J. Steel ◽  
D. P. Winstanley
Keyword(s):  
Trephine Biopsy ◽  
Lung Lesions ◽  
Diffuse Lung

Acute Response to Inhaled Nitric Oxide in Newborns With Respiratory Failure and Pulmonary Hypertension

PEDIATRICS ◽  
1996 ◽  
Vol 98 (4) ◽  
pp. 698-705 ◽  
Author(s):  
Ronald W. Day ◽  
Joanna M. Lynch ◽  
Keith S. White ◽  
Robert M. Ward
Keyword(s):  
Nitric Oxide ◽  
Pulmonary Hypertension ◽  
Respiratory Failure ◽  
Lung Disease ◽  
Blood Gases ◽  
Inhaled Nitric Oxide ◽  
Diffuse Lung Disease ◽  
Diffuse Disease ◽  
Oxygenation Indices ◽  
Diffuse Lung

Objective. Systemic oxygenation is improved by inhaled nitric oxide therapy in some newborns with respiratory failure and pulmonary hypertension. Our results with inhaled nitric oxide were reviewed to determine factors associated with an acute improvement in systemic oxygenation. Methods. Newborns with oxygenation indices of 25 to 40 were prospectively randomized to receive conventional therapy with or without 20 ppm inhaled nitric oxide. All newborns with oxygenation indices greater than 40 were treated with inhaled nitric oxide. Hemodynamic, blood gas, and Doppler ultrasound measurements were performed before and after 30 to 60 minutes of observation or therapy. The severity of lung disease was classified by the chest radiograph as: (1) normal or focal disease; (2) moderate diffuse disease—diffuse lung disease with well-defined heart borders; or (3) severe diffuse disease—diffuse lung opacification with indistinct heart borders. Results. Heart rate, blood pressure, and ductal diameters did not change. Blood gases and ductal shunting acutely improved only in patients treated with inhaled nitric oxide. Patients with normal lung fields or focal disease had the greatest degree of improvement in systemic oxygenation. Changes in oxygenation were not influenced by gestational age, baseline blood gases, the proportion of right-to-left ductal shunting, prior treatment with a surfactant, or the use of conventional or high-frequency jet ventilation. Collectively, blood gases and ductal shunting did not improve with inhaled nitric oxide in patients with lung hypoplasia or severe diffuse lung disease. Sustained improvement in oxygenation occurred in 87% of patients with oxygenation indices greater than 40 in whom oxygenation indices less than 40 acutely developed after exposure to nitric oxide, whereas 90% of patients in whom oxygenation indices less than 40 did not acutely develop were treated with extracorporeal membrane oxygenation or ultimately died. Conclusions. Inhaled nitric oxide acutely improves systemic oxygenation in many newborns with respiratory failure and pulmonary hypertension. The diagnosis and chest radiograph are helpful in identifying patients who will have favorable acute responses to therapy. In patients with severe hypoxemia, the need for invasive support with extracorporeal membrane oxygenation may be determined by an acute trial of inhaled nitric oxide.

scholarly journals DIFFERENTIATION BETWEEN SOME TOXIC SUBSTANCES IN ANAEROBICALLY PRODUCED AUTOLYSATES OF PNEUMOCOCCI (TYPES I AND II)

1929 ◽  
Vol 49 (4) ◽  
pp. 695-700 ◽  
Author(s):  
Julia T. Parker
Keyword(s):  
Guinea Pigs ◽  
Red Cells ◽  
Toxic Substances ◽  
Lung Lesions ◽  
Toxic Principle ◽  
Diffuse Lung ◽  
Intratracheal Injection

The necrotizing and lung-toxic principles present in certain anaerobically prepared autolysates of Pneumococcus Types I and II are similar in respect to extreme sensitiveness to heat and to oxidation, and to their ability to be neutralized by the same anti-autolysate serums. These two poisons differ, however, in their ability to be adsorbed or inactivated by red cells; the lung-toxic principle being adsorbed or inactivated by such procedure while the necrotizing principle is not. Since pneumococcus hemotoxin is present in the anaerobic autolysates and is also adsorbed by red cells, it seemed possible that it was this substance in the autolysates which caused the diffuse lung lesions and death of guinea pigs. However, it was found that the intratracheal injection of pneumococcus hemotoxin prepared by the method of Avery and Neill only occasionally produced the characteristic reaction caused by the intratracheal injection of the anaerobic autolysates.

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