Presentation
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]
Entire Body System
- Lymphedema
The course of the patient's recovery from an LT for P-LAM was complicated by lymphedema in the left femoral region that was caused by two E-LAM lesions remaining in the left pelvic cavity and in the retroperitoneal area. [ncbi.nlm.nih.gov]
Lymphedema associated with sirolimus in renal transplant recipients. Transplantation 2004;77:1094-6 [ Links ] 3. Romagnoli J, Citterio F, Nanni G, Tondolo V, Castagneto M. Severe limb lymphedema in sirolimus-treated patients. [scielo.mec.pt]
Aboujaoude W, Milgrom ML, Govani MV "Lymphedema associated with sirolimus in renal transplant recipients1." Transplantation 77 (2004): 1094-6 10. Josef F, Marina K, Alois W "Sirolimus myopathy." Transplantation 76 (2003): 1773-4 11. [drugs.com]
[…] acne, cellulites, non-melanoma skin cancer Other: gingivitis, gum hyperplasia, weight changes, neck pain, fever, abscess, chills, facial edema, flulike symptoms, infection, lymphadenopathy, abnormal healing, lymphocele, fluid accumulation (including lymphedema [medical-dictionary.thefreedictionary.com]
- Sepsis
Sirolimus 5 mg (n=8) 48/M 88 85 Pneumonia 41/M 242 197 Liver failure; recurrent hepatitis B 59/M 24 3 Hemodynamic rejection, sepsis 50/F 21 20 Cardiopulmonary arrest 51/M 75 52 Primary graft failure, MRSA sepsis 51/M 10 9 Primary cardiac graft failure [ahajournals.org]
The most commonly identified cause of death was sepsis/infection (16%). Invited discussant Sandy Srinivas, MD, of Stanford University in Stanford, Calif., noted several limitations of the analysis. [medpagetoday.com]
[…] changes, neck pain, fever, abscess, chills, facial edema, flulike symptoms, infection, lymphadenopathy, abnormal healing, lymphocele, fluid accumulation (including lymphedema, ascites), opportunistic infections (such as tuberculosis, fatal infections, sepsis [medical-dictionary.thefreedictionary.com]
Oversuppression of the immune system can also increase susceptibility to infection, including opportunistic infections such as tuberculosis, fatal infections, and sepsis. [pfizermedicalinformation.com]
10%): Impaired healing [ Ref ] Oncologic Common (1% to 10%): Skin cancer, lymphoma /post-transplant lymphoproliferative disorder Frequency not reported : Hepatocellular adenoma and carcinoma, testicular adenoma [ Ref ] Immunologic Common (1% to 10%): Sepsis [drugs.com]
- Recurrent Infection
Sirolimus is not a curative treatment but can help with some of the issues associated with the condition such as pain, reduced mobility, pain or stiffness, recurrent infections, abnormal clotting and organ dysfunction. [gosh.nhs.uk]
Chronic pain, functional restraint, recurrent infections (defined as > 3 episodes/year), oozing, bleeding and/or coagulation abnormality were the symptoms considered for inclusion in the study.There was no age limitation. [ojrd.biomedcentral.com]
Growth and/or expansion of vascular anomalies can cause clinical problems such as disfigurement, chronic pain, recurrent infections, coagulopathies (thrombotic and hemorrhagic), organ dysfunction, and death. [pediatrics.aappublications.org]
Complications of PROS depend on the anatomical site and extent of overgrowth, but may include functional impairment (e.g., of walking or swallowing), pain, recurrent superficial infections, thromboembolism, and/or hemorrhage, all of which may be debilitating [nature.com]
Respiratoric
- Pneumonia
Sirolimus has side effects on lugs, and may cause cryptogenic organizing pneumonia, diffuse alveolar hemorrhage, lymphocytic pneumonitis, hypersensitivity pneumonitis, desquamative interstitial pneumonia, and pulmonary alveolar proteinosis. [ncbi.nlm.nih.gov]
Therefore, antimicrobial prophylaxis for P. carinii pneumonia should be administered for 1 year following transplantation. [medical-dictionary.thefreedictionary.com]
- Pleural Effusion
CLINICAL RELEVANCE: we present the first clinical cases of EGS with pleural effusion with response to sirolimus treatment that could be an alternative to the current therapy. [ncbi.nlm.nih.gov]
A moderate-large pericardial effusion and bilateral pleural effusions were disclosed. Due to the size of the pericardial effusion and the absence of cardiac tamponade, we presumed that the effusion was slowly progressive. [scielo.mec.pt]
effusion, atelectasis, and lymphoma. [medical-dictionary.thefreedictionary.com]
Cardiovascular
- Thrombosis
KEYWORDS: Drug-eluting stent; animal study; anti-thrombosis; heparinized surface; in-stent restenosis [ncbi.nlm.nih.gov]
Psychiatrical
- Suggestibility
Our structure explains how FKBP-rapamycin and architectural elements of mTORC1 limit access to the recessed active site, and suggests a mechanism for the role of Raptor in substrate recognition and delivery: the conserved amino-terminal CASPase-like domain [bangroup.ethz.ch]
[…] other mTOR inhibitors The risk of cancer is increased after kidney transplantation and is predominantly attributed to oncogenic immunosuppression. 1 It is a leading cause of death for kidney allograft recipients, and analyses of transplant registry data suggest [bmj.com]
However, the proteinuria resolved after discontinuation of sirolimus, which suggests a direct effect of the drug. [cjasn.asnjournals.org]
The second pregnancy on uninterrupted low-dose sirolimus (plasma levels 3-5 mg/L) was uncomplicated both with respect to mother and child suggesting that low-dose sirolimus might be safe in selected pregnant patients with stable LAM. [ncbi.nlm.nih.gov]
Neurologic
- Apathy
[…] risk of abnormal wound healing based on data from medical literature Limit sun exposure because of increased skin cancer risk Progressive multifocal leukoencephalopathy (PML), sometimes fatal, have been reported; commonly presents with hemiparesis, apathy [reference.medscape.com]
PML commonly presents with hemiparesis, apathy, confusion, cognitive deficiencies and ataxia. Risk factors for PML include treatment with immunosuppressant therapies and impairment of immune function. [pfizermedicalinformation.com]
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
- Pericardial Effusion
He underwent surgical drainage of the pericardial effusion and pericardial window and biopsy. [scielo.mec.pt]
effusion (including hemodynamically significant effusions in children and adults), lymphedema Rare (less than 0.1%): Pericardial effusion [ Ref ] Gastrointestinal Very common (10% or more): Constipation (up to 38%), abdominal pain (up to 36%), diarrhea [drugs.com]
[…] depression, anxiety, tremor, insomnia CV: hypertension, hypotension, tachycardia, chest pain, edema, palpitations, vasodilation, peripheral edema, peripheral vascular disorders, thrombophlebitis, thrombosis, heart failure, atrial fibrillation, hemorrhage, pericardial [medical-dictionary.thefreedictionary.com]
ascites, and pericardial effusions (including hemodynamically significant effusions and tamponade requiring intervention in children and adults), in patients receiving therapy Not for liver or lung transplant Impaired or delayed wound healing reported [reference.medscape.com]
There have also been reports of fluid accumulation, including peripheral edema, lymphedema, pleural effusion, ascites, and pericardial effusions (including hemodynamically significant effusions and tamponade requiring intervention in children and adults [pfizermedicalinformation.com]
Microbiology
- Candida
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]
Rapamycin is mainly active against Candida albicans ; minimum inhibitory concentration against ten strains ranged from 0.02 to 0.2 μg/ml. [doi.org]
Pleura
- Pleural Effusion
CLINICAL RELEVANCE: we present the first clinical cases of EGS with pleural effusion with response to sirolimus treatment that could be an alternative to the current therapy. [ncbi.nlm.nih.gov]
A moderate-large pericardial effusion and bilateral pleural effusions were disclosed. Due to the size of the pericardial effusion and the absence of cardiac tamponade, we presumed that the effusion was slowly progressive. [scielo.mec.pt]
effusion, atelectasis, and lymphoma. [medical-dictionary.thefreedictionary.com]
Biopsy
- Hepatocellular Carcinoma
Hepatocellular carcinoma. Lancet. 2003; 362: 1907–1917. 2. Mazzaferro V, Regalia E, Doci R, et al. Liver transplantation for the treatment of small hepatocellular carcinomas in patients with cirrhosis. N Engl J Med. 1996; 334: 693–699. 3. [journals.lww.com]
/ Recurrent Breast Carcinoma / Recurrent Colorectal Carcinoma / Recurrent Esophageal Carcinoma / Recurrent Gastric Carcinoma / Recurrent Hepatocellular Carcinoma / Recurrent Lung Carcinoma / Recurrent Melanoma / Recurrent Ovarian Carcinoma / Recurrent [drugbank.ca]
Other Outcome Measures: Number of Participants Resumed SRL After Minimization [ Time Frame: 12 months ] patient did not have rejection during withdrawal but was withdrawn from the trial due to an unexpected adrenal metastasis of hepatocellular carcinoma [clinicaltrials.gov]
Treatment
There is not sufficient evidence to support the routine use of any treatment in LAM. The only treatment for severe LAM is currently lung transplantation. Activation of mammalian target of rapamycin (mTOR) signalling pathway has been observed in LAM. [archbronconeumol.org]
Despite the severity of potential complications, we lack uniform guidelines for the treatment and response to treatment of children and young adults with these diseases. [novanews.org]
BACKGROUND: Systemic treatment with sirolimus, as used for immunosuppression in transplant patients, results in markedly low rates of in-stent restenosis. [ncbi.nlm.nih.gov]
Effect of treatment discontinuation. Summary of observations in 6 patients who discontinued rapamycin treatment. (A) Status of rapamycin treatment is indicated by [−]/[+]. [doi.org]
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]
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]
Currently, we leverage a variety of techniques to study the etiology and pathogenesis of iMCD. [med.upenn.edu]
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]
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: https://doi.org/10.1681/ASN.2007111202 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. [doi.org]
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]
[…] 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]
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]
The exact pathophysiology is unknown. [revespcardiol.org]
Prevention
antibacterial drug A drug used to treat or prevent bacterial infections. antifungal drug Any antifungal agent used to prevent or treat fungal infections in humans or animals. [ebi.ac.uk]
Also called rapamycin. sirolimus an immunosuppressant. indications It is used after organ transplantation to prevent rejection. [medical-dictionary.thefreedictionary.com]
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]
It is a potent immunosuppressant having a variety of uses both in vitro and in vivo and is used to prevent organ rejection after transplantation. [agscientific.com]