Noninfectious Pulmonary Manifestations of Renal Disease In Children
Lourdes R. Laraya-Cuasay, Walter T. Hughes in Interstitial Lung Diseases in Children, 2019
Approximately 90% of the patients have the anti-GBM antibodies, but such antibodies by themselves are not absolutely diagnostic of Goodpasture’s syndrome as they have been found in cases of lupus, polyarteritis nodosa, diabetic nephropathy, penicillamine sensitivity, focal glomerular sclerosis, and Henoch-Schonlein purpura. These diseases, however, are not characterized by the linear ribbon-like glomerular deposits that are seen with Goodpasture’s syndrome. This syndrome is more prevalent among young adult males, the usual age of onset being between 20 and 30 years. There is a male to female ratio of 3:1. The classic presentation is with pulmonary hemorrhage, although pulmonary symptoms may precede or may present coincident with the renal lesion. Clinically one will see hemoptysis, anemia, and nephritis with symptoms of dyspnea, cough, malaise, and fever. Sputum samples may have hemosiderin-laden macrophages. The radiograph of the lungs may show patchy lung infiltrates in a “butterfly” pattern or an acinar, reticular, or reticulonodular pattern. These radiographic findings may be transitory. Pulmonary insufficiency with recurrent hemoptysis also occurs.
Pediatric And Adult Congenital Cardiac Interventions
Debabrata Mukherjee, Eric R. Bates, Marco Roffi, Richard A. Lange, David J. Moliterno, Nadia M. Whitehead in Cardiovascular Catheterization and Intervention, 2017
The presence of severe pulmonary insufficiency may lead to RV enlargement and dysfunction. Surgical placement of a competent valve between the RV and pulmonary arteries to treat this condition requires cardiopulmonary bypass, which may aggravate an already compromised RV. Bonhoeffer was the first to place a valve in this position percutaneously40 and since then, thousands of patients have benefited from his technique.41 Currently, two valves are being evaluated for the percutaneous management of patients with severe conduit failure due to regurgitation and/or stenosis. The Medtronic Melody valve developed by Bonhoeffer (Figure 47.6) consists of a bovine jugular vein with a valve inside sewn into a Platinum Cheatham stent. The Edwards SAPIEN THV42 is made of bovine pericardial leaflets sewn inside a stainless steel stent. Both valves are balloon expandable and require a large delivery system for deployment (up to 22- to 24-Fr).
Successful Pulmonary Rescue of Adult Onset Granulomatosis with Polyangiitis Using Extracorporeal Membrane Oxygenation and Window Thoracostomy
Wickii T. Vigneswaran in Thoracic Surgery, 2019
Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s disease, is an autoimmune disease with features of medium-to-small vessel vasculitis and granulomatous inflammation most frequently affecting the pulmonary-renal axis. While the prevalence in the United States is only three cases per 100,000 people, there is a strong predominance to male individuals of northern European descent (>90% of cases in the US). The symptoms manifest with varying degrees of severity, and the development of pulmonary insufficiency necessitating extracorporeal oxygenation is exceedingly rare and infrequently reported in this patient population [1]. Our patient represents one of only thirteen reported cases of successful hospital discharge following severe pulmonary GPA treated with aggressive immunosuppressive therapies vvECMO. As well, our patient is the only reported case requiring adjunctive window thoracostomy for Stenotrophomonal/Pseudomonal cavitary superinfection and aspergillosis. All medical and surgical treatment was delivered at a major academic quaternary care hospital.
CO2 narcosis as a notable cause of premature death in Nakajo-Nishimura syndrome
Published in Modern Rheumatology Case Reports, 2019
Nobuo Kanazawa, Mariko Hara, Tomoyuki Hara, Kayo Kunimoto, Naoya Mikita, Fukumi Furukawa
Our case looks quite similar to the cases reported as JMP syndrome, who showed severe lipodystrophy in the whole body and remarkable contracture of finger joints. While most cases diagnosed as CANDLE syndrome were infants or children, JMP and NNS patients were mostly reported in adults. Collectively, cases with these diseases usually onset in infancy with inflammatory attacks and, after grown-up, rather clinically resemble progeria with marked emaciation especially in the upper body. Besides some CANDLE cases died during infancy, a number of NNS cases have been reported to be dead suddenly or prematurely in their 20th–40th years of age [1]. Regarding the cause of death, cardiac failure is considered to be a major one as revealed in an autopsy case with NNS [5]. Left cardiac insufficiency would be developed primarily from the defective proteasome function, suggested by ST and R changes and transmission problems in ECG. Right cardiac insufficiency would follow pulmonary insufficiency, which develops acutely in case of pneumonitis and chronically in association with the chest wall atrophy and decreased respiratory muscle power, suggested by restrictive changes in the respiratory examination.
Intermediate outcomes of transcatheter pulmonary valve replacement with the Edwards Sapien 3 valve – German experience
Published in Expert Review of Medical Devices, 2019
Anja Lehner, Tsvetina Dashkalova, Sarah Ulrich, Silvia Fernandez Rodriguez, Guido Mandilaras, Andre Jakob, Robert Dalla-Pozza, Marcus Fischer, Heike Schneider, Gleb Tarusinov, Christoph Kampmann, Michael Hofbeck, Ingo Dähnert, Majed Kanaan, Nikolaus A. Haas
Of the 26 patients with predominant pulmonary stenosis or both, stenosis and regurgitation, mean peak systolic gradient across the RVOT before prestenting was estimated with 36.9 mmHg (SD 17.6). The mean peak gradient of the whole cohort was 22.3 mmHg (SD 18.6). Pulmonary regurgitation was graded as moderate and severe in 40 patients with predominant pulmonary insufficiency or both. After TPVR residual RVOT gradient of the whole cohort was 6.7 mmHg (SD 4.4) with 52 patients with no/trivial pulmonary regurgitation (Figures 1 and 2). Echo-data during the first 10 days after the intervention documented a mean peak flow velocity of 2.1 m/s across the S3 valve (SD 0.5). In two patients, a mild pulmonary insufficiency was noted.
Current challenges in managing comorbid heart failure and COPD
Published in Expert Review of Cardiovascular Therapy, 2018
J. Alberto Neder, Alcides Rocha, Maria Clara N. Alencar, Flavio Arbex, Danilo C. Berton, Mayron F. Oliveira, Priscila A. Sperandio, Luiz E. Nery, Denis E. O’Donnell
‘Functionally, it is obvious that the pulmonary and circulatory apparatus are one unit’Baldwin ED, Cournand A, Richards DW. Pulmonary insufficiency; a study of 122 cases of chronic pulmonary emphysema. Medicine (Baltimore). 1949;28:201–37.
Related Knowledge Centers
- Echocardiography
- Pulmonary Artery
- Pulmonary Hypertension
- Pulmonary Valve
- Diastole
- Ventricle
- Heart
- Regurgitation
- Physical Examination
- Signs & Symptoms