scholarly journals Pleuropneumonectomy as Salvage Therapy in Children Suffering from Primary or Metastatic Sarcomas with Pleural Localizations

Cancers ◽  
2021 ◽  
Vol 13 (15) ◽  
pp. 3655
Author(s):  
Frédéric Hameury ◽  
Perrine Marec-Berard ◽  
Mathilde Eymery ◽  
Marc H. W. Wijnen ◽  
Niels van der Kaaij ◽  
...  
Keyword(s):  
Pulmonary Function ◽  
Salvage Therapy ◽  
Therapeutic Option ◽  
Pulmonary Function Tests ◽  
Underlying Disease ◽  
Exercise Intolerance ◽  
Undifferentiated Sarcoma ◽  
Metastatic Recurrence ◽  
Dismal Prognosis ◽  
Metastatic Relapse

Pediatric sarcoma patients with pleuropulmonary lesions have a dismal prognosis because the impossibility to obtain local control. The aim of this study was to determine if pleuropneumonectomy (PP) could be a therapeutic option. We retrospectively reviewed nine patients who underwent salvage PP for pleuropulmonary localization of primary localized sarcoma or metastatic recurrence. Surgery and complications were analyzed, pulmonary function tests were conducted, and quality of life was determined with EORTC-QLQ-C30 questionnaire. At the time of PP age was between 9–17 years. Underlying disease included metastatic osteosarcoma (n = 5), Ewing sarcoma (two metastatic, one primary), and one primary undifferentiated sarcoma. Early complications occurred in three patients. Mean postoperative hospitalization stay was 14.5 days. Pulmonary function test showed 19–66% reduction of total lung capacity which led to mild exercise intolerance but did not affect daily life. Four patients died of multi-metastatic relapse <14 months after PP, one patient had a local recurrence, and four patients are in complete remission between 1.5 and 12 years after PP. In conclusion, in this small patient group treated with a pleuropneumonectomy for primary or metastatic lesions, outcome is variable; however, this extended surgical technique was generally quite well tolerated. Postoperative lung function seems well preserved, and it seems to lead to at least an extension of life with good quality and therefor can be considered as salvage therapy.

Pulmonary Function Tests Fail to Predict Exercise Intolerance in Sheep with Emphysema

2005 ◽  
Vol 37 (4) ◽  
pp. 550-556
Author(s):  
MELISSA R. MAZAN ◽  
EDWARD P. INGENITO ◽  
LARRY TSAI ◽  
ANDREW HOFFMAN
Keyword(s):  
Pulmonary Function ◽  
Pulmonary Function Tests ◽  
Exercise Intolerance ◽  
Function Tests

Role of Hyaline Membrane Disease in Production of Later Childhood Lung Abnormalities

PEDIATRICS ◽  
1982 ◽  
Vol 69 (5) ◽  
pp. 572-576
Author(s):  
Mildred Stahlman ◽  
Gunnel Hedvall ◽  
Dan Lindstrom ◽  
James Snell
Keyword(s):  
Pulmonary Function ◽  
Secondary Infection ◽  
Pulmonary Function Tests ◽  
Hyaline Membrane Disease ◽  
Exercise Intolerance ◽  
Positive Pressure ◽  
Residual Symptoms ◽  
Function Tests ◽  
Hyaline Membrane ◽  
History Of

Between 1961 and 1970, 177 survivors of hyaline membrane disease have been followed clinically and roentgenologically for a minimum of three years to determine the long-term pulmonary outcome. Of these, 153 have been seen for at least six years and 61, for 11 years or more. A total of 175 batteries of pulmonary function tests have been performed on 129 children at age 7 and/or 11 years, Final roentgenograms showed fibrosis in 12 instances, and these changes were positively correlated with the severity of the initial disease, the use of a positive pressure respirator (but not negative pressure respirator), time on the respirator, and length of time in O2, but not high O2 concentrations. Residual symptoms in the first years of life were also correlated with positive pressure respirator use, length of use, and with secondary infection. Pulmonary function tests showed minimal abnormalities in 12 children when last seen. None of these children had a history of exercise intolerance or chronic respiratory symptoms. The ultimate pulmonary history of this population of patients is unknown, but overt childhood lung disease following hyaline membrane disease is not a frequent occurrence.

Study of pulmonary function tests in tobacco factory workers

2018 ◽  
Vol 6 (3) ◽  
pp. 16-19
Author(s):  
Gajanan V Patil ◽  
◽  
Atish Pagar ◽  
U S Patil ◽  
M K Parekh ◽  
...  
Keyword(s):  
Pulmonary Function ◽  
Pulmonary Function Tests ◽  
Factory Workers ◽  
Function Tests

Pulmonary Function Tests in European Birth Cohorts

2013 ◽  
Vol 9 (1) ◽  
pp. 3-10
Author(s):  
Linus Grabenhenrich ◽  
Cynthia Hohmann ◽  
Remy Slama ◽  
Joachim Heinrich ◽  
Magnus Wickman ◽  
...  
Keyword(s):  
Pulmonary Function ◽  
Pulmonary Function Tests ◽  
Birth Cohorts ◽  
Function Tests

scholarly journals Pulmonary Function Testing in Work-Related Asthma: An Overview from Spirometry to Specific Inhalation Challenge

2021 ◽  
Vol 18 (5) ◽  
pp. 2325
Author(s):  
Mathias Poussel ◽  
Isabelle Thaon ◽  
Emmanuelle Penven ◽  
Angelica I. Tiotiu
Keyword(s):  
Pulmonary Function ◽  
Pulmonary Function Tests ◽  
Objective Evaluation ◽  
Pulmonary Function Testing ◽  
Initial Assessment ◽  
Work Related ◽  
Specific Inhalation Challenge ◽  
Occupational Setting ◽  
Inhalation Challenge ◽  
Function Testing

Work-related asthma (WRA) is a very frequent condition in the occupational setting, and refers either to asthma induced (occupational asthma, OA) or worsened (work-exacerbated asthma, WEA) by exposure to allergens (or other sensitizing agents) or to irritant agents at work. Diagnosis of WRA is frequently missed and should take into account clinical features and objective evaluation of lung function. The aim of this overview on pulmonary function testing in the field of WRA is to summarize the different available tests that should be considered in order to accurately diagnose WRA. When WRA is suspected, initial assessment should be carried out with spirometry and bronchodilator responsiveness testing coupled with first-step bronchial provocation testing to assess non-specific bronchial hyper-responsiveness (NSBHR). Further investigations should then refer to specialists with specific functional respiratory tests aiming to consolidate WRA diagnosis and helping to differentiate OA from WEA. Serial peak expiratory flow (PEF) with calculation of the occupation asthma system (OASYS) score as well as serial NSBHR challenge during the working period compared to the off work period are highly informative in the management of WRA. Finally, specific inhalation challenge (SIC) is considered as the reference standard and represents the best way to confirm the specific cause of WRA. Overall, clinicians should be aware that all pulmonary function tests should be standardized in accordance with current guidelines.

ISOLATED ABNORMALITIES OF DIFFUSION CAPACITY (DLCO) IN PULMONARY FUNCTION TESTS AMONG INNER CITY PATIENTS

CHEST Journal ◽  
2008 ◽  
Vol 134 (4) ◽  
pp. 49S
Author(s):  
Ibrahim H. Abou Daya ◽  
Muhammad U. Anwer ◽  
Gilda Diaz-Fuentes ◽  
Steve Blum ◽  
Latha Menon
Keyword(s):  
Pulmonary Function ◽  
Inner City ◽  
Pulmonary Function Tests ◽  
Diffusion Capacity ◽  
Function Tests

scholarly journals Serum IGFBP-2 in systemic sclerosis as a prognostic factor of lung dysfunction

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Julien Guiot ◽  
Makon-Sébastien Njock ◽  
Béatrice André ◽  
Fanny Gester ◽  
Monique Henket ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Prognostic Factor ◽  
Pulmonary Function ◽  
Lung Disease ◽  
Pulmonary Function Tests ◽  
Serum Levels ◽  
Roc Curve Analysis ◽  
Significant Deterioration ◽  
Lung Dysfunction

AbstractSystemic sclerosis (SSc) is a rare connective tissue disease associated with rapid evolving interstitial lung disease (ILD), driving its mortality. Specific biomarkers associated with the progression of this lung disease are highly needed. We aimed to identify specific biomarkers of SSc-ILD to predict the evolution of the disease. For this, we compared prospectively serum levels of several biomarkers associated with lung fibrosis in SSc patients (n = 102), among which SSc-no ILD (n = 63) and SSc-ILD (n = 39), compared to healthy subjects (HS) (n = 39). We also performed a longitudinal study in a subgroup of 28 patients analyzing biomarkers variations and pulmonary function tests over a period of 2 years. Serum level of IGFBP-2 was significantly increased in SSc patients compared to HS, and negatively correlated with pulmonary function (assessed by carbon monoxide transfer coefficient (KCO)) (r = − 0.29, p < 0.01). Two-year longitudinal analysis in a subgroup of 28 SSc patients determined that IGFBP-2 variation was positively correlated with KCO at 2-year follow-up (r = 0.6, p < 0.001). SSc patients with a lower variation of IGFBP-2 (less than 22%) presented significant deterioration of pulmonary function at 2-year follow-up (p < 0.01). ROC curve analysis enabled us to identify that baseline IGFBP-2 > 105 ng/ml was associated with a poor outcome (KCO < 70% predicted) at 2-year follow-up (AUC = 0.75, p < 0.05). We showed for the first time that serum levels of IGFBP-2 might be a prognostic factor of the development of SSc-ILD.

Shrinking lung syndrome in pediatric systemic lupus erythematosus

Lupus ◽  
2021 ◽  
pp. 096120332110103
Author(s):  
Alfonso Ragnar Torres Jimenez ◽  
Nayma Ruiz Vela ◽  
Adriana Ivonne Cespedes Cruz ◽  
Alejandra Velazquez Cruz ◽  
Alma Karina Bernardino Gonzalez
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Pleural Effusion ◽  
Pulmonary Function ◽  
Lupus Erythematosus ◽  
Pulmonary Function Tests ◽  
Age At Diagnosis ◽  
X Rays ◽  
Systemic Lupus ◽  
Function Tests ◽  
Restrictive Pattern

Shrinking Lung Syndrome (SLS) is a rare and little known complication associated with Systemic Lupus Erythematosus (SLE), characterized by progressive and unexplainable dyspnea, pleuritic pain, small pulmonary volumes and elevation of the diaphragm on chest X-rays as well as restrictive pattern on pulmonary function tests. Objective To describe clinical, radiological and treatment characteristics in pediatric patients with SLS. Material and methods This is a descriptive and retrospective study in patients under 16 years old with the diagnosis of SLE complicated by SLS at the General Hospital. National Medical Center La Raza. Clinical, radiological and treatment variables were analyzed. Results are shown in frequencies and percentages. Results Data from 11 patients, 9 females and 2 males were collected. Mean age at diagnosis of SLS was 12.2 years. Age at diagnosis of SLE was 11.1 years. SLEDAI 17.3. Renal desease 72%, hematological 91%, lymphopenia 63%, mucocutaneous 72%, neurological 9%, arthritis 54%, serositis 91%, fever 81%, secondary antiphospholipid syndrome, low C3 72%, low C4 81%, positive ANA 91%, positive anti-DNA 91%. Regarding clinical manifestations of SLE: cough 81%, dyspnea 91%, hipoxemia 81%, pleuritic pain 71%, average oxygen saturation 83%. Chest X-rays findings: right hemidiaphragm affection 18%, left 63%, bilateral 18%. Elevated hemidiaphragm 91%, atelectasis 18%, pleural effusion 91%, over one third of the cardiac silhouette under the diphragm 36%, bulging diaphragm 45%, 5th. anterior rib that crosses over the diaphragm 91%. M-mode ultrasound: diaphragmatic hypomotility 100%, pleural effusion 63%. Pulmonary function tests: restrictive pattern in 45% of the cases. Treatment was with supplementary oxygen 100%, intubation 18%, antibiotics 100%, steroids 100%, intravenous immunoglobulin 54%, plasmapheresis 18%, cyclophosphamide 54% and rituximab 18%. The clinical course was favorable in 81%. Conclusions SLS should be suspected in patients with SLE and active disease who present hipoxemia, pleuritic pain, cough, dyspnea, pleural effusion and signs of restriction on chest X-rays. Therefore, a diaphragmatic M-mode ultrasound should be performed in order to establish the diagnosis.

scholarly journals DIPG-19. TARGETING ATM MUTATION IN METASTATIC DIFFUSE MIDLINE GLIOMA – A CASE OF SUSTAINED RESPONSE USING PARP INHIBITOR

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii290-iii291
Author(s):  
Karen Tsui ◽  
Andrew Law ◽  
Michael K Watson
Keyword(s):  
Parp Inhibitor ◽  
Standard Of Care ◽  
High Grade Glioma ◽  
Diffuse Intrinsic Pontine Glioma ◽  
Radiological Criteria ◽  
Metastatic Recurrence ◽  
Curative Therapy ◽  
First Case ◽  
Metastatic Relapse ◽  
Diffuse Midline Glioma

Abstract Diffuse midline glioma (DMG) with H3.3K27M mutation is associated with an extremely poor prognosis, with a median survival of 10 to 12 months. Radiation remains the standard of care however there is no established curative therapy available. We describe a patient diagnosed with a diffuse intrinsic pontine glioma at 5 years of age by clinical and radiological criteria. He was treated with focal radiation 59Gy which resulted in reduction in size of the tumour, and partial improvement of T2 changes on MRI. At 18 months post diagnosis, the patient developed metastatic recurrence at the anterior fornix. This was biopsied and histopathology demonstrated a high grade glioma. Next generation sequencing revealed a H3F3A K27M mutation, and an ATM R3008H mutation. He received whole ventricular radiation 36Gy and boost to the lesion to 45Gy, followed by Olaparib 135mg/m2/day twice daily. He remains in radiological remission 20 months post metastatic relapse and has no organ toxicity to Olaparib. CONCLUSION: H3.3K27M and ATM co-segregating mutations are described in DMG. This is the first case report of targeting ATM mutation with a PARP inhibitor which resulted in prolonged remission of metastatic DMG. Olaparib was well tolerated.

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