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Sirolimus
RAPA
Domain organization of PKS of rapamycin and biosynthetic intermediates
Presentation
- We present a patient with an intracardiac KHE which presented as a large mass surrounding the atria, pulmonary veins, superior vena cava, and infiltrating the atrial septum with moderate compression of the superior vena cava and mild compression of the[ncbi.nlm.nih.gov]
- A term newborn presented with widespread cutaneous erythematous to bluish lesions since birth. He had extensive lesions in the gastrointestinal tract, brain, retina, heart, and bones.[ncbi.nlm.nih.gov]
- A critical review of the relevant literature on this subject is also presented to determine the best approach.[ncbi.nlm.nih.gov]
- Drug-induced-angioedema is a relatively common presentation and is potentially fatal. It must be aware of potential adverse effects.[ncbi.nlm.nih.gov]
- In the present study, analyzed data are presented to support the use of sirolimus in clinical practise for the treatment of corticosteroid-resistant vascular tumors with KMP in eight infants between June 2015 and April 2017 in a single hospital.[ncbi.nlm.nih.gov]
Respiratoric
Paroxysmal Cough
- However, both patients developed paroxysmal cough and tachypnea shortly after the onset of sirolimus treatment and succumbed to infection thereafter. This report reveals a potential risk of infection in sirolimus-treated infant patients.[ncbi.nlm.nih.gov]
Musculoskeletal
Joint Limitation
- The patient, who had edema on the upper left extremity and experienced joint limitations, was administered complete decongestive therapy (CDT) and manual therapy in combination with sirolimus.[ncbi.nlm.nih.gov]
Skin
Shagreen Patch
- RESULTS: Long-term mTORI use resulted in skin lesion regression (angiofibromas, "confetti" skin lesions, shagreen patch) and disease stabilization in brain, abdominal, and chest magnetic resonance imaging/computed tomography scans.[ncbi.nlm.nih.gov]
Angiokeratoma
- These anomalies consisted of three extratruncular micro- and macrocystic lymphatic malformations and one each verrucous venous malformation, truncular lymphatic malformation with angiokeratomas, and infantile hemangioma.[ncbi.nlm.nih.gov]
Neurologic
Unable to Walk
- We report the case of a 10-year-old boy with a KHE of his right distal thigh who was unable to walk without assistance due to fibrotic change and right knee contracture. He had no laboratory evidence of KMP at the time of representation.[ncbi.nlm.nih.gov]
Workup
- A workup of infectious causes was undertaken and was negative. All the patients had resolution of visceral effusions after sirolimus discontinuation.[scielo.mec.pt]
- An extensive workup was negative for an infectious etiology, including JC polyomavirus or demyelination. Sirolimus was discontinued and the headaches resolved within a few days.[bloodjournal.org]
X-Ray
Alveolar Consolidation
- Thoracic CT scans showed bilateral areas of peripheral alveolar consolidation. Bronchial lavage showed a lymphocytic alveolitis. Open-lung biopsy showed organizing pneumonia associated with diffuse alveolar damage and caseating granulomata.[ncbi.nlm.nih.gov]
Microbiology
Microsporum
- A streptomycete was isolated from an Easter Island soil sample and found to inhibit Candida albicans, Microsporum gypseum and Trichophyton granulosum.[ncbi.nlm.nih.gov]
- Abstract A streptomycete was isolated from an Easter Island soil sample and found to inhibit Candida albicans , Microsporum gypseum and Trichophyton granulosum .[doi.org]
- Abstract A streptomycete was isolated from an Easter Island soil sample and found to inhibit Candida albicans, Microsporum gypseum and Trichophyton granulosum.[joi.jlc.jst.go.jp]
Treatment
- Effect of treatment discontinuation. Summary of observations in 6 patients who discontinued rapamycin treatment. (A) Status of rapamycin treatment is indicated by [ ]/[ ].[haematologica.org]
- P * P *** P Effect of treatment discontinuation. Summary of observations in 6 patients who discontinued rapamycin treatment. (A) Status of rapamycin treatment is indicated by [ ]/[ ].[ncbi.nlm.nih.gov]
- An improvement of the lesions has been shown in 94% of subjects, particularly if the treatment was started at early stages.[ncbi.nlm.nih.gov]
- SLEDAI and BILAG disease activity scores were reduced during 12 months of treatment in 16 (55%) of 29 patients who completed treatment.[ncbi.nlm.nih.gov]
Prognosis
- Its development worsens patient prognosis, with a significant decrease in survival. Currently, there are no unified guidelines regarding its management, and different combinations of immunosuppressants have been used with variable success.[ncbi.nlm.nih.gov]
- However, data are lacking on the clinical spectrum, long-term prognosis, and treatment of MLT.[ncbi.nlm.nih.gov]
- In this report, we will characterize the temsirolimus-related pneumonitis and not the interferon-related pneumonitis that occurred in patients with poor-prognosis ARCC.[doi.org]
- The rapamycin- regulated gene expression signature determines prognosis for breast cancer Molecular Cancer 8(75), (2009) . 3. Zhang, H.H., Huang, J., Düvel, K., et al .[caymanchem.com]
Etiology
- BACKGROUND: Gorham-Stout disease (GSD) is a rare disease of unknown etiology characterized by vascular proliferation that produces destruction of bone matrix.[ncbi.nlm.nih.gov]
- No biopsy was performed to establish etiology and potential association with mTORI. In 2017 creatinine level was 2.2 mg/dL. CONCLUSION: The case of the patient confirms clinical effectiveness and acceptable safety of long-term mTORI treatment.[ncbi.nlm.nih.gov]
- Based on the absence of a previous infectious condition and any signs of rejection in the endocardial biopsy that could be triggers, and given the recent introduction of sirolimus, the latter was considered the etiological factor, with blood levels within[revespcardiol.org]
- Currently, we leverage a variety of techniques to study the etiology and pathogenesis of iMCD.[med.upenn.edu]
- Evidence Rating Level: 2 (Good) Study Rundown: Antiphospholipid syndrome (APLS) is an autoimmune condition with both primary and secondary etiologies that often results in antibody-mediated vascular thrombosis and obstetrical complications.[2minutemedicine.com]
Epidemiology
- Epidemiologic literature is saturated with general discussions of the limitations of retrospective studies. Posttransplantation diabetes is a relatively frequent and unfortunate complication in patients carrying renal allografts.[jasn.asnjournals.org]
- Clinical features, epidemiology, and therapy of lymphangioleiomyomatosis. Clin Epidemiol . 2015;7:249-57. Lee L, Sudentas P, Donohue B, et al. Efficacy of a Rapamycin Analog (CCI-779) and INF-gamma in Tuberous Sclerosis Mouse Models.[specialtypharmacytimes.com]
- An epidemiological study of renal pathology in tuberous sclerosis complex. BJU Int. 2004;94:853–7. 25. Marti HP, Frey FJ. Nephrotoxicity of rapamycin: an emerging problem in clinical medicine. Nephrol Dial Transplant. 2005;20:13–5. 26.[ojrd.biomedcentral.com]
- , Aarhus University Hospital, Aarhus, Denmark (5) Department of Epidemiology, Boston University, Boston, MA, USA References Moses JW, Leon MB, Popma JJ, Fitzgerald PJ, Holmes DR, O’Shaughnessy C, et al: Sirolimus-eluting stents versus standard stents[bmccardiovascdisord.biomedcentral.com]
- CLINICAL EPIDEMIOLOGY JASN July 2008, 19 (7) 1411-1418; DOI: This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased.[doi.org]
Sex distribution
Age distribution
Pathophysiology
- This case highlights the need for early recognition of adverse drug effects and further research into their pathophysiology and management.[ncbi.nlm.nih.gov]
- We review the literature on PSI-induced pneumonitis and discuss the pathophysiology of a potential interaction with pentamidine. We caution against its use in the setting of PSI-induced pneumonitis.[ncbi.nlm.nih.gov]
- Oxygen and BagValveMask Ventilation 217 Assessment and Management of the Pediatric Airway 223 Vascular Access 253 Shock An Overview 274 Acute Respiratory Failure 299 Pediatric Transport Medicine 308 Withdrawal of Life Support 320 Brain Death 324 The Pathophysiology[books.google.ro]
- […] concentration during therapy, particularly in individuals coadministered CYP3A4 substrates, inhibitors, or inducers Adjusting dose to optimize immunosuppression while minimizing toxicity Evaluating patient compliance Clinical Information Discusses physiology, pathophysiology[mayomedicallaboratories.com]
- The exact pathophysiology is unknown.[revespcardiol.org]
Prevention
- CONCLUSION: Our findings show that systemic sirolimus treatment effectively prevents SMC and EC proliferation in vivo without directly affecting these cells.[ncbi.nlm.nih.gov]
- These results suggest that heparinized surface-based sirolimus coating may be a useful approach for the prevention of restenosis and stent thrombosis.[ncbi.nlm.nih.gov]
- Lymphoedema is a rare complication of sirolimus, and the mechanisms are not completely understood; however, early recognition can prevent permanent disfiguration.[ncbi.nlm.nih.gov]
- In the period 1998 to 2003, a number of reviews have appeared evaluating the potential of rapamycin and other immunophilin ligands as therapies for cancer, organ transplantation, restenosis prevention, autoimmune disorders, and neurodegenerative diseases[ncbi.nlm.nih.gov]
- Covering: 2003 to 2008 In the period 1998 to 2003, a number of reviews have appeared evaluating the potential of rapamycin and other immunophilin ligands as therapies for cancer, organ transplantation, restenosis prevention, autoimmune disorders, and[doi.org]