scholarly journals A Scoring Tool to Predict Pulmonary Complications in Severe Leptospirosis with Kidney Failure

2022 ◽  
Vol 7 (1) ◽  
pp. 7
Author(s):  
Rizza Antoinette Yap So ◽  
Romina A. Danguilan ◽  
Eric Chua ◽  
Mel-Hatra I. Arakama ◽  
Joann Kathleen B. Ginete-Garcia ◽  
...  
Keyword(s):  
Kidney Failure ◽  
Multivariate Analyses ◽  
Survival Rates ◽  
Pulmonary Hemorrhage ◽  
Rapid Identification ◽  
Pulmonary Complications ◽  
Aggressive Management ◽  
Severe Leptospirosis ◽  
Scoring Tool ◽  
Overall Mortality

Rapid identification of patients likely to develop pulmonary complications in severe leptospirosis is crucial to prompt aggressive management and improve survival. The following article is a cohort study of leptospirosis patients admitted at the National Kidney and Transplant Institute (NKTI). Logistic regression was used to predict pulmonary complications and obtain a scoring tool. The Kaplan–Meir method was used to describe survival rates. Among 380 patients with severe leptospirosis and kidney failure, the overall mortality was 14%, with pulmonary hemorrhage as the most common cause. In total, there were 85 (22.4%) individuals who developed pulmonary complications, the majority (95.3%) were observed within three days of admission. Among the patients with pulmonary complications, 56.5% died. Patients placed on mechanical ventilation had an 82.1% mortality rate. Multivariate analyses showed that dyspnea (OR = 28.76, p < 0.0001), hemoptysis (OR = 20.73, p < 0.0001), diabetes (OR = 10.21, p < 0.0001), renal replacement therapy (RRT) requirement (OR = 6.25, p < 0.0001), thrombocytopenia (OR = 3.54, p < 0.0029), and oliguria/anuria (OR = 3.15, p < 0.0108) were significantly associated with pulmonary complications. A scoring index was developed termed THe-RADS score (Thrombocytopenia, Hemoptysis, RRT, Anuria, Diabetes, Shortness of breath). The odds of developing pulmonary complications were 13.90 times higher among patients with a score >2 (63% sensitivity, 88% specificity). Pulmonary complications in severe leptospirosis with kidney failure have high mortality and warrant timely and aggressive management.

A Scoring Tool to Predict Pulmonary Complications in Severe Leptospirosis with Kidney Failure

2021 ◽  
Author(s):  
Rizza Antoinette Yap So ◽  
Romina A. Danguilan ◽  
Eric Chua ◽  
Mel-Hatra I. Arakama ◽  
Joselito R. Chavez ◽  
...  
Keyword(s):  
Kidney Failure ◽  
Pulmonary Complications ◽  
Severe Leptospirosis ◽  
Scoring Tool

Superselective Radioembolization of Hepatocellular Carcinoma: 5-Year Results of a Prospective Study

1994 ◽  
Vol 33 (05) ◽  
pp. 206-214 ◽  
Author(s):  
J. Triller ◽  
H. U. Baer ◽  
Livia Geiger ◽  
H. F. Beer ◽  
C. Becker ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Survival Rates ◽  
Absorbed Dose ◽  
Pulmonary Complications ◽  
Pulmonary Shunt ◽  
Recurrent Tumour ◽  
Activity Distribution ◽  
A Prospective Study ◽  
Overall Survival Rates

SummaryTwenty patients with unresectable hepatocellular carcinoma (HCC) were followed up to 5 years after transarterial radiotherapy with 90Y-resin particles. Diagnostic radioembolizations of 99mTc-macroaggregates facilitated scintigraphic assessment of activity distribution, dose evaluation and final procedural verification. The overall survival rates were 56, 38 and 14% (after 1, 2 and 3 years, resp.). Patients with unifocal HCC and a single feeding artery (n = 7) even presented 83, 67 and 40% (2 alive after 2.75 and 4 years). With multiple arteries (n = 7), the longest survival was 26 months. Patients with multifocal HCC survived up to 33 months after selective radioembolization. Quality of life was improved in all. Survival was positively correlated with absorbed dose but residual/recurrent tumour occurred even after ≥300 Gy. Post-treatment symptoms were minimal (35 applications), pulmonary shunt rates were correctly predicted and pulmonary complications avoided.

scholarly journals Centralisation of Oesophagogastric Cancer Services: Can Specialist Units Deliver?

2006 ◽  
Vol 88 (6) ◽  
pp. 566-570 ◽  
Author(s):  
MJ Forshaw ◽  
JA Gossage ◽  
J Stephens ◽  
D Strauss ◽  
AJ Botha ◽  
...  
Keyword(s):  
Postoperative Morbidity ◽  
Survival Rates ◽  
Length Of Hospital Stay ◽  
High Volume ◽  
Cardiovascular Complications ◽  
Pulmonary Complications ◽  
Minimal Impact ◽  
Cancer Services ◽  
Clinically Significant ◽  
Oesophagogastric Cancer

INTRODUCTION Oesophagogastric cancer surgery is increasingly being performed in only centralised units. The aim of the study was to examine surgical outcomes and service delivery within a specialist unit. PATIENTS AND METHODS The case notes of all patients undergoing attempted oesophagogastrectomy between January 2000 and May 2003 were identified from a prospective consultant database. RESULTS A total of 187 patients (median age, 63 years; range, 29–83 years; M:F ratio, 3.9:1) underwent attempted oesophagogastrectomy. Of these, 91% were seen within 2 weeks of referral and treatment was instituted after a mean of 31 days (range, 1–109 days). More patients underwent surgery (63%) than neoadjuvant therapy (56%) within 1 month of referral. The main indication for surgery was invasive malignancy in 166 patients (89%). The 30-day mortality was 0.5% (1 death) and in-hospital mortality was 1.1% (2 deaths). The median length of hospital stay was 14 days (range, 7–69 days). Significant postoperative morbidity included: pulmonary complications (36%), cardiovascular complications (16%), wound infection (13%) and clinically significant anastomotic leaks (7%). Of the study group, 28 patients (15%) were admitted to ICU with a median stay of 10 days (range, 1–44 days); this accounted for 0.9% of ICU bed availability. Twelve patients (6.4%) were returned to theatre, most commonly for bleeding. The 1-year survival rates were 78%. During 2002–2003, national waiting list targets for both hernia repair and cholecystectomy were achieved. CONCLUSIONS Despite recent increases in workload, high volume specialist units can deliver an efficient and timely service with both good treatment outcomes and minimal impact upon elective surgical waiting lists and ICU provision.

scholarly journals Treatment and Survival Differences in Older Medicare Patients With Lung Cancer as Compared With Those Who Are Dually Eligible for Medicare and Medicaid

2008 ◽  
Vol 26 (31) ◽  
pp. 5067-5073 ◽  
Author(s):  
Cathy J. Bradley ◽  
Bassam Dahman ◽  
Charles W. Given
Keyword(s):  
Lung Cancer ◽  
Low Socioeconomic Status ◽  
Older Patients ◽  
Multivariate Analyses ◽  
Insurance Coverage ◽  
Survival Rates ◽  
Financial Barriers ◽  
Medicare Patients ◽  
Dual Eligibility ◽  
Survival Differences

Purpose This study compares non–small-cell lung cancer (NSCLC) treatments provided to older patients (age ≥ 66 years) who are dually eligible for Medicare and Medicaid with treatments provided to similar patients who are insured by Medicare. We extend the analysis to include a comparison of survival rates between Medicare and dually eligible patients. Dual eligibility is associated with low socioeconomic status. However, Medicaid coverage in addition to Medicare removes many financial barriers to care. Patients and Methods The sample included 2,626 older patients with local and regional stage NSCLC diagnosed between 1997 and 2000. Four outcomes were studied: the likelihood of receiving resection, chemotherapy, radiation therapy, and survival (perioperative and longer-term). Logistic regression was used to predict the likelihood of treatment, and stratified and multivariate analyses were used to evaluate differences in survival. Results Dually eligible patients were half as likely to undergo resection as Medicare patients (P < .001) and were more likely to receive radiation than Medicare patients. Stratified and multivariate analyses showed that surgically treated dually eligible patients had slightly inferior survival as compared with that of Medicare patients. Survival was equivalent among patients who did not undergo resection, regardless of insurance coverage. Conclusion Older dually eligible patients with NSCLC had a lower likelihood of undergoing resection despite controls for socioeconomic factors and comorbidities. However, if such patients were surgically treated, survival improved substantially, but it remained inferior to the survival of Medicare patients. Additional research is needed to understand why resection rates were substantially lower among dually eligible patients.

Stereotactic hypofractionated high-dose irradiation (STI) for stage I non-small cell lung cancer (NSCLC): Mature results in 300 cases of a Japanese multi-institutional study

2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 7045-7045 ◽  
Author(s):  
H. Onishi ◽  
Y. Nagata ◽  
H. Shirato ◽  
K. Karasawa ◽  
K. Gomi ◽  
...  
Keyword(s):  
Total Dose ◽  
Local Control ◽  
Survival Rates ◽  
Progression Free Survival ◽  
Pulmonary Complications ◽  
Stage I ◽  
High Dose ◽  
Stage Ib ◽  
Local Progression ◽  
Stage Ia

7045 Background: With the increasing accuracy of localization for tumor-bearing areas using various new techniques, hypofractionated or single high-dose stereotactic irradiation (STI) has been actively investigated for stage I NSCLC in Japan. The current study retrospectively evaluated Japanese multi-institutional results for high-dose STI for stage I NSCLC. Methods: From 1993 to 2003, stereotactic three-dimensional treatment was performed using 3–10 non-coplanar dynamic arcs or 6–20 static ports for a total of 300 stage I (median age, 75 years; T1N0M0, n = 193; T2N0M0, n = 107) patients with primary NSCLC (adenocarcinoma, n = 138; squamous cell carcinoma, n = 129; and others, n = 33) in 14 institutions. Totally 190 patients were medically inoperable, and other 110 were medically operable but selected STI. A total dose of 18–75 Gy at the isocenter was administered in 1–22 fractions. Median calculated biological effective dose (BED) was 108 Gy (range, 57–180 Gy). Results: Median follow-up period of survivors was 38 months (range; 2–128 months). Pulmonary complications of NCI-CTC criteria (version 2.0) grade ≥ 3 were noted in 9 patients (3.0%). Local progression occurred in 44 patients (14.7%), and 5-year local control rate was high (86%) for BED ≥100 Gy (n = 227) compared to 67% for <100 Gy (n = 73) (P < 0.001). Overall 5-year survival rates of operable and inoperable patients were 65% and 37%, respectively. Overall 5-year survival rates in operable cases was high (74%) for BED ≥100 Gy (n = 85) compared to 37% for <100 Gy (n = 24) (P < 0.01). In a subset of operable patients irradiated with BED ≥100, 3-year locally progression-free survival rates was high (81%) for stage IA (n = 60) compared to 67% for stage IB (n = 23) (P < 0.05) Conclusions: Local control and survival rates of STI for stage I NSCLC are better with BED ≥100 Gy compared to <100 Gy. Survival rates in selected patients (medically operable, BED ≥100 Gy) were excellent, and potentially comparable to those of surgery. Stage IB patients displayed higher rate of local progression than stage IA. We have started multi-institutional prospective study for stage IA NSCLC with a schedule of total dose of 48 Gy in 4 fractions during 4–8 days. No significant financial relationships to disclose.

Impact of relapse site on oncological outcomes after radical nephroureterectomy for upper urinary tract urothelial carcinoma.

2016 ◽  
Vol 34 (2_suppl) ◽  
pp. 426-426
Author(s):  
Shinichi Yamashita ◽  
Akihiro Ito ◽  
Koji Mitsuzuka ◽  
Yoshihide Kawasaki ◽  
Ichiro Shintaku ◽  
...  
Keyword(s):  
Urinary Tract ◽  
Local Recurrence ◽  
Lymph Nodes ◽  
Multivariate Analyses ◽  
Survival Rates ◽  
Upper Urinary Tract ◽  
Radical Nephroureterectomy ◽  
Cancer Specific Survival ◽  
Oncological Outcomes ◽  
The Impact

426 Background: Most upper urinary tract urothelial carcinomas (UTUC) are invasive and advanced with a poor prognosis. Patients often relapse after treatment with radical nephroureterectomy (RNU). However, little is known about an association between sites of recurrence after RNU and oncological outcomes. The present study retrospectively evaluated the impact of a first recurrence site on survival. Methods: A total of 650 patients with UTUC treated by RNU at 12 institutions that participated in the Tohoku Urological Evidence-Based Medicine Study Group between 2000 and 2011 were initially enrolled. Patients who experienced relapse other than intravesical recurrence after RNU were included in this study. Those who had metastasis at the time of UTUC diagnosis or missing data regarding the time and location of relapse were excluded. Finally, 173 patients were eligible to participate in this study. Survival rates were analyzed using Kaplan-Meier curves and the log-rank test, and factors predicting survival were assessed using multivariate analyses. Results: The median follow-up after relapse was nine months. The cancer-specific survival rates at one and two years after relapse were 49% and 22%, respectively. Cancer in 59 (34%), 32 (18%), 23 (13%), and 94 (53%) patients recurred in the lung, liver, bone and lymph nodes, and 23 (13%) had local recurrence. The one-year cancer-specific survival rates were 44%, 27%, 22% and 52% among patients with metastasis of the lungs, liver, bone and lymph nodes respectively, and 41% among those with local recurrence. The survival rate was worse among 48 patients with metastasis at multiple sites, than in those with metastasis at a single site. Fourteen (8%) patients with a single lymph, lung or local recurrence survived for over three years after a first relapse. Multivariate analyses selected the liver, bone and local recurrence as critical factors for cancer-specific survival. Conclusions: Liver or bone metastasis was a negative prognostic factor for UTUC with relapse after RNU. Information about recurrence sites might be helpful for patient counseling.

14-3-3Zeta Positive Expression is Associated With a Poor Prognosis in Patients With Glioblastoma

Neurosurgery ◽  
2011 ◽  
Vol 68 (4) ◽  
pp. 932-9385 ◽  
Author(s):  
Xiaoliang Yang ◽  
Weidong Cao ◽  
Jie Zhou ◽  
Wei Zhang ◽  
Xiang Zhang ◽  
...  
Keyword(s):  
Survival Time ◽  
Median Survival ◽  
Survival Data ◽  
Median Survival Time ◽  
Multivariate Analyses ◽  
Survival Rates ◽  
Negative Group ◽  
Actuarial Survival ◽  
Positive Group ◽  
Positive Expression

Abstract BACKGROUND: When identifying clinical markers predicting clinical outcome, disease recurrence and resistance to therapies often determine the diagnosis and therapy of some cancer types. OBJECTIVE: To investigate whether 14-3-3zeta positive expression is an indicator of prognosis in patients with glioblastoma. METHODS: Forty-seven patients treated with surgery, radiotherapy, and adjuvant chemotherapy between 2005 and 2007 were divided into 2 groups according to 14-3-3zeta expression in an immunohistochemical study: the 14-3-3zeta negative group (n = 12 patients) and the 14-3-3zeta positive group (n = 35 patients). The clinicopathologic features and survival data for patients in the 14-3-3zeta positive group were compared with data from the patients in the 14-3-3zeta negative group. Kaplan-Meier survival analysis and univariate and multivariate analyses were performed to determine the prognostic factors that influenced patient survival. RESULTS: 14-3-3zeta positive expression was observed in approximately 74.5% of patients with glioblastoma. Patients in the 14-3-3zeta positive group had lower overall survival rates and median survival time than those in the 14-3-3zeta negative group (overall 2-year actuarial survival rates, 8.6% for the 14-3-3zeta positive group vs 16.7% for the 14-3-3zeta negative group; overall 2-year median survival time, 12.9 months for the 14-3-3zeta positive group vs 17.9 months for the 14-3-3zeta negative group, P = .019). 14-3-3zeta positive expression in tumor cells also was correlated with a shorter interval to tumor recurrence (median interval to recurrence, 5.9 months in the 14-3-3zeta positive group vs 8.3 months in the 14-3-3zeta negative group, P = .002). Univariate and multivariate analyses showed that 14-3-3zeta positive expression was an independent prognostic factor. CONCLUSION: 14-3-3zeta positive expression can be used as a potential molecular risk factor in patients with glioblastoma.

scholarly journals SP4.2.15 An international multi-centre appraisal of the management and outcomes of acute CHOLEcystitis during the COVID-19 pandemic: The CHOLECOVID Study

2021 ◽  
Vol 108 (Supplement_7) ◽  
Author(s):  
Harry VM Spiers ◽  
Keyword(s):  
Acute Cholecystitis ◽  
Primary Outcome ◽  
Pulmonary Complications ◽  
National Guidance ◽  
Significant Difference ◽  
All Cause Mortality ◽  
The Face ◽  
Diagnostic Modalities ◽  
Definitive Management ◽  
Overall Mortality

Abstract Introduction National guidance issued in response to COVID-19 resulted in adoption of non-surgical modes of treatment in emergency surgery, including acute cholecystitis (AC). The CHOLECOVID Study is the definitive global audit of the management and outcomes of AC during COVID19. Methods Patients &gt;18 years admitted to hospital with radiologically confirmed AC, during two predefined 8-week time periods: P1 (pre-pandemic) 12/09/19- 12/11/19; P2 (during the pandemic) 12/03/20-12/05/20, were included. The primary outcome was 30-day all-cause mortality. Secondary outcomes included severity of AC, radiological diagnostic modalities implemented, definitive management and pulmonary complications. Results 9,615 patients were included from 39 countries (P1:5,381; P2:4,234). 30-day mortality was higher in P2 (1.7%vs2.4%; p &lt; 0.015). Higher rates of moderate and severe AC were seen in P2 (30.1%vs35.1%;3.7%vs4.1%). First-line CT imaging was more common in P2 (36.3%vs46.3%; p &lt; 0.001). There were higher failure rates of conservative management in P2 (37.4% vs 44.4%; P &lt; 0.001). Cholecystostomy rates were higher in P2 (5.8%vs8.8%; P &lt; 0.001). Overall 4.6% (n = 193) of P2 patients were COVID-19 positive, with overall mortality of 0.7% (n = 30). There was no significant difference in pulmonary complications between COVID-19 positive or negative patients. Conclusion During the COVID-19 pandemic, a small increase in mortality among AC patients was noted, when compared to the pre-pandemic cohort. Patients during the COVID-19 pandemic presented with more severe AC, resulting in altered trends in diagnosis and management. New guidance and clear pathways are required to safely manage AC moving forward, in the face of further waves of COVID-19.

scholarly journals Impact of Graft-Versus-Host Disease and Graft-Versus-Leukemia Effect Based on Minimal Residual Disease in Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4522-4522
Author(s):  
Yu Akahoshi ◽  
Aiko Igarashi ◽  
Takahiro Fukuda ◽  
Naoyuki Uchida ◽  
Masatsugu Tanaka ◽  
...  
Keyword(s):  
Research Funding ◽  
Acute Gvhd ◽  
Multivariate Analyses ◽  
Residual Disease ◽  
Lymphoblastic Leukemia ◽  
Philadelphia Chromosome ◽  
Graft Versus Host ◽  
Graft Versus Leukemia ◽  
Grade 3 ◽  
Overall Mortality

[Background] Previous studies have shown that a graft-versus-leukemia (GVL) effect was augmented in patients who developed graft-versus-host disease (GVHD). The benefit of the GVL effect is counter-balanced by treatment-related mortality (TRM) due to GVHD. In addition, the development of the ability to detect minimal residual disease (MRD) has changed the landscape of risk stratification. Therefore, patients with positive-MRD require a more intensive GVL effect to reduce relapse, whereas a "mild" GVL effect may be sufficient in patients with negative-MRD. However, it is uncertain whether the influence of a GVL effect would differ depending on the MRD status at HSCT. Here, we conducted a nationwide retrospective study to evaluate the impact of GVHD and a GVL effect according to the MRD status for Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph-positive ALL) in the TKI era. [Patients & Methods] Clinical data were obtained from the Transplant Registry Unified Management Program (TRUMP), which is the registry database of the Japan Society for Hematopoietic Cell Transplantation (JSHCT). We examined 1022 recipients who underwent their first allogeneic hematopoietic stem cell transplantation (HSCT) for Ph-positive ALL in first complete remission between 2005 and 2017. We excluded patients who lacked MRD status at HSCT or who received in vivo T-cell depletion or high-dose post-transplantation cyclophosphamide. The impacts of acute GVHD (aGVHD) and chronic GVHD (cGVHD) as time-dependent covariates on transplant outcomes were analyzed while adjusting for other significant variables in multivariate analyses. In the analysis of cGVHD, only patients who survived at least 100 days without hematological relapse were included. This retrospective study was approved by the data management committee of TRUMP and by the Institutional Review Board of Jichi Medical University Saitama Medical Center. [Results] The median age at HSCT was 45 years (range, 16 to 71 years). MRD status at HSCT was negative in 791 (77.4%) and positive in 231 (22.6%). The median observation period of the survivors was 1505 days (range, 18 to 4944 days). To graphically illustrate the impacts of aGVHD and cGVHD, a Simon-Makuch plot were drawn in the whole cohort and in the groups limited to negative-MRD and positive-MRD at HSCT (Figure 1 and 2). The impacts of acute GVHD on overall survival, hematological relapse, and non-relapse mortality (NRM) were summarized in Table. In multivariate analyses, the HRs for hematological relapse with positive-MRD at HSCT (0.80 for grade 1-2 aGVHD and 0.31 for grade 3-4 aGVHD) were smaller than those with negative-MRD at HSCT (1.07 for grade 1-2 aGVHD and 0.45 for grade 3-4 aGVHD), respectively. In addition, the risk of hematological relapse gradually decreased proportionally to the severity of aGVHD. Because the risks of NRM for grade 1-2 aGVHD were not significant regardless of MRD-positivity, grade 1-2 aGVHD was not significantly associated with superior overall mortality. Grade 3-4 aGVHD was significantly associated with inferior overall survival in the whole cohort and in the group limited to negative-MRD at HSCT because of high NRM. Meanwhile, grade 3-4 aGVHD was not significantly associated with overall mortality due to the potent GVL effect in the analysis limited to positive-MRD at HSCT. In the analysis of cGVHD, although cGVHD reduced the risk of hematological relapse, the survival advantage of cGVHD was not significant regardless of MRD-positivity. Because the NIH severity score was not available in our database, further evaluations of cGVHD considering the severity score are needed. [Conclusion] Our study showed that both MRD status at HSCT and the severity of aGVHD might be associated with the intensity of a GVHD-associated GVL effect for Ph-positive ALL. However, because GVHD had no apparent survival benefit regardless of MRD-positivity at HSCT or the severity of GVHD, less intensive GVHD prophylaxis to obtain the GVL effect is not recommended for Ph-positive ALL. Disclosures Kanda: Nippon-Shinyaku: Research Funding; Kyowa-Hakko Kirin: Consultancy, Honoraria, Research Funding; MSD: Research Funding; Shionogi: Consultancy, Honoraria, Research Funding; Chugai: Consultancy, Honoraria, Research Funding; Takeda: Consultancy, Honoraria, Research Funding; Chugai: Consultancy, Honoraria, Research Funding; Alexion: Consultancy, Honoraria; Pfizer: Research Funding; Novartis: Research Funding; Dainippon Sumitomo: Consultancy, Honoraria, Research Funding; CSL Behring: Research Funding; Bristol-Myers Squibb: Consultancy, Honoraria; Pfizer: Research Funding; Tanabe Mitsubishi: Research Funding; Sanofi: Research Funding; Celgene: Consultancy, Research Funding; Taiho: Research Funding; Astellas: Consultancy, Honoraria, Research Funding; Bristol-Myers Squibb: Consultancy, Honoraria; Eisai: Consultancy, Honoraria, Research Funding; Otsuka: Research Funding; CSL Behring: Research Funding; Dainippon Sumitomo: Consultancy, Honoraria, Research Funding; Kyowa-Hakko Kirin: Consultancy, Honoraria, Research Funding; Sanofi: Research Funding; Taisho-Toyama: Research Funding; Asahi-Kasei: Research Funding; Taiho: Research Funding; Tanabe Mitsubishi: Research Funding; Takeda: Consultancy, Honoraria, Research Funding; Novartis: Research Funding; Celgene: Consultancy, Research Funding; Mochida: Consultancy, Honoraria; Astellas: Consultancy, Honoraria, Research Funding; Alexion: Consultancy, Honoraria; Eisai: Consultancy, Honoraria, Research Funding; Takara-bio: Consultancy, Honoraria; Otsuka: Research Funding; Asahi-Kasei: Research Funding; Mochida: Consultancy, Honoraria; Taisho-Toyama: Research Funding; Ono: Consultancy, Honoraria, Research Funding; MSD: Research Funding; Ono: Consultancy, Honoraria, Research Funding; Nippon-Shinyaku: Research Funding; Shionogi: Consultancy, Honoraria, Research Funding; Takara-bio: Consultancy, Honoraria. Ichinohe:Astellas Pharma: Research Funding; Chugai Pharmaceutical Co.: Research Funding; CSL Behring: Research Funding; Eisai Co.: Research Funding; Kyowa Hakko Kirin Co.: Research Funding; Ono Pharmaceutical Co.: Research Funding; Pfizer: Research Funding; Nippon Shinyaku Co.: Research Funding; MSD: Research Funding; Otsuka Pharmaceutical Co.: Research Funding; Repertoire Genesis Inc.: Research Funding; Sumitomo Dainippon Pharma Co.: Research Funding; Taiho Pharmaceutical Co.: Research Funding; Takeda Pharmaceutical Co.: Research Funding; Zenyaku Kogyo Co.: Research Funding; Alexion Pharmaceuticals: Honoraria; Bristol-Myers Squibb: Honoraria; Celgene: Honoraria; JCR Pharmaceuticals: Honoraria; Janssen Pharmaceutical K.K.: Honoraria; Mundipharma: Honoraria; Novartis: Honoraria. Tanaka:Bristol-Myers Squibb: Research Funding. Atsuta:CHUGAI PHARMACEUTICAL CO., LTD.: Honoraria; Kyowa Kirin Co., Ltd: Honoraria. Kako:Bristol-Myers Squibb: Honoraria; Pfizer Japan Inc.: Honoraria.

scholarly journals Intra-Alveolar Hemorrhage in Certain Orphan Lung Diseases in Children (Lecture)

2020 ◽  
pp. 72-80
Author(s):  
O.L. Tsymbalista ◽  
Keyword(s):  
Kidney Failure ◽  
Clinical Course ◽  
Pulmonary Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Rapid Progression ◽  
Goodpasture Syndrome ◽  
Pulmonary Hemosiderosis ◽  
Short Period ◽  
Pulmonary Pathology ◽  
Idiopathic Pulmonary Hemosiderosis

The theme is relevant due to the diagnostic difficulties, severe clinical course and prognosis of idiopathic pulmonary hemosiderosis and Goodpasture syndrome. Idiopathic pulmonary hemosiderosis and Goodpasture syndrome are severe, life-threatening immunopathologic diseases due to alveolar hemorrhage and a hundred percent mortality within a short period of time after the onset of clinical manifestations. Idiopathic pulmonary hemosiderosis generally occurs in children at the age of 3–8 years as a separate condition, or as a stage of Goodpasture syndrome. It manifests itself as shortness of breath, pneumonia, prune juice sputum, hemoptysis, hemorrhage. During exacerbation, the patients' condition is determined by the degree of pulmonary hemorrhage, pulmonary heart disease, acute posthemorrhagic anemia. The exacerbation lasts from a few hours to 1–2 weeks. The duration of each episode and remission varies among patients being unpredictable. Each new exacerbation is more severe. In Goodpasture syndrome, predominant pulmonary and renal vascular lesions of autoimmune nature are observed. It affects young males more frequently; is rare in children. Hemorrhagic alveolitis as a form of lung damage develops first; then, the kidneys are involved, and anemia occurs. Glomerulonephritis (GN) manifests itself as nephrotic syndrome with rapid progression of kidney failure. In case of the predominant pulmonary pathology, recurrent hemoptysis and pulmonary hemorrhage are observed; in end-stage disease with cardiopulmonary failure manifestations, rapidly progressive GN and kidney failure develop. The second variant of Goodpasture syndrome is characterized by relatively slow progression of pulmonary changes and renal lesions. Goodpasture syndrome is rarely accompanied by GN from the onset to the end of the disease and pulmonary pathology manifests itself at the terminal phase of the disease. The treatment of both diseases includes lifetime therapy with glucocorticoids, cytostatics. Pulse therapy using these preparations, discrete plasma exchange and intravenous immunoglobulin administration, syndromic treatment are carried out. No conflict of interest was declared by the authors. Keywords: children, idiopathic pulmonary hemosiderosis, Goodpasture syndrome, clinical course, therapy.

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