Improving Survival in Lymphangioleio­myomatosis: A 16-Year Observational Study in a Large Cohort of Patients

Respiration ◽  
2021 ◽  
pp. 1-11
Author(s):  
Sergio Harari ◽  
Olga Torre ◽  
Davide Elia ◽  
Antonella Caminati ◽  
Giuseppe Pelosi ◽  
...  
Keyword(s):  
Natural History ◽  
Cumulative Incidence ◽  
Pulmonary Function Tests ◽  
Diagnostic Techniques ◽  
Term Treatment ◽  
Large Cohort ◽  
Clinical Aspects ◽  
Long Term Treatment ◽  
History Of

<b><i>Background:</i></b> Over the last 2 decades, great progress has been made in the understanding of the clinical aspects and pathogenesis of lymphangioleiomyomatosis (LAM), leading to publication of guidelines and approval of an effective therapy. <b><i>Objectives:</i></b> Aim of our study was to describe how the management and the natural history of this rare disease have changed after the publication of the ERS and American Thoracic Society/Japanese Respiratory Society guidelines and the introduction of sirolimus. <b><i>Methods:</i></b> We examined 162 LAM patients followed at our center between 2001 and 2017, reporting clinical characteristics and diagnostic approach. Response to sirolimus in patients undergoing long-term treatment and mortality risk, estimated in terms of cumulative incidence taking into account organ transplantation as a competing cause of the event, were evaluated. The difference in the cumulative incidence between the patients admitted to the observation before 2011 and after 2011, year of the publication of the MILES trial for the efficacy of sirolimus, has also been estimated. <b><i>Results:</i></b> Sixty-one patients had a histological diagnosis (22 from 2010 onward). 101 patients received a radiological diagnosis according to the guidelines criteria. Pulmonary function tests remained stable over a 3-year treatment period in patients who received sirolimus for over 12 months. The cumulative incidence of mortality after 10 years in the whole population was 25.5%. The cumulative incidence of mortality after 5 years was significantly lower in patients who entered the study since 2011 (after publication of the MILES trial) than in patients who entered the study before. <b><i>Conclusions:</i></b> We provide the data supporting the long-term efficacy of sirolimus therapy in a large cohort of patients with functional impairment and other manifestations of the disease. Our results also suggest that the advent of sirolimus and the publication of international guidelines changed the natural history of the disease lowering the mortality and reducing the need of invasive diagnostic techniques.

scholarly journals Homocysteine (Hcy) Follow-Up Study

2007 ◽  
Vol 30 (1) ◽  
pp. 21 ◽  
Author(s):  
Arnon Blum ◽  
Ihsan Hijazi ◽  
Michal Mashiach Eizenberg ◽  
Nava Blum
Keyword(s):  
Diabetes Mellitus ◽  
Risk Factors ◽  
Natural History ◽  
Beta Blockers ◽  
Term Treatment ◽  
Channel Blockers ◽  
Long Term Treatment ◽  
History Of

Background: Hyperhomocysteinemia confers an increased risk of coronary artery disease, stroke, and deep vein thrombosis, and is a strong predictor of mortality among patients with ischemic heart disease. Purpose: To determne the long term clinical outcome of patients with risk factors to atherosclerosis with high concentrations of homocysteine (Hcy). Methods: 89 patients with one or more risk factors for atherosclerosis, whose plasma total Hcy concentrations were measured, were followed for 5 years. Patients were interviewed and underwent a clinical examination in the outpatient clinic. Their medical records were reviewed in the last 5 years including smoking habits, medications, other diseases (hypertension, diabetes mellitus, hyperlipidemia) and their management. SPSS was used to describe and explore possible relationships between Hcy concentration, other diseases, medications and the clinical long term outcome. Results: All men with normal Hcy concentrations (10.76±1.71µmol/L) survived during the 5 years’ follow up, while 5 of the men with high Hcy concentrations (21.27±5.37µmol/L), died (17%) (P< 0.05). In women Hcy concentration did not affect survival. No association was found between diabetes mellitus, hypercholesterolemia, hypertension and Hcy. Long term treatment with Beta Blockers, ACE inhibitors, Calcium Channel blockers, and especially with Aspirin prevented death and changed the natural history of patients with high Hcy concentrations (P < 0.05). Conclusions – Hyperhomocysteinemia may have an effect on survival in men. Long term treatment with Beta Blockers, ACE inhibitors, Calcium Channel Blockers, and especially with Aspirin – prevented death and changed the natural history of patients with high Hcy concentrations.

The Role of Arthroscopy in the Treatment of Gouty Arthritis of the Knee Case presentation

2018 ◽  
Vol 69 (8) ◽  
pp. 2236-2239
Author(s):  
Marius Moga ◽  
Mark Edward Pogarasteanu ◽  
Dumitru Ferechide ◽  
Antoine Edu ◽  
Chen Feng Ifrim
Keyword(s):  
Gouty Arthritis ◽  
Surgical Techniques ◽  
Term Treatment ◽  
Clinical Aspects ◽  
Associated Lesions ◽  
Number Of Patients ◽  
Long Term Treatment ◽  
Functional Scores ◽  
Urate Crystals

Gout is a metabolic disease involving the impregnation of joints and other tissues with urate crystals. The onset is often brutal, and it manifests itself with pain and inflammation in the affected joint. The treatment usually involves rest, ice, NSAIDs and anti-gout medication. The long-term treatment involves medication and dietary changes. In the joint, urate crystals are deposited in the synovial, in the cartilage and in the menisci. In the arthroscopic practice, the gouty knee is a rare occurrence. We present a relevant case, that of a 57 years old patient without a prior gout diagnosis where we found urate crystal deposits covering the synovium, cartilage and meniscus, and we discuss the current and recent year Pub Med indexed literature in order to evaluate the possibilities for arthroscopic treatment of this pathology. We looked at the number of patients involved, their characteristics, and the surgical techniques used. We also looked at the temporal relation of the arthroscopic intervention to the recent gout attacks, and at the described lesions that were found. Also, we evaluated the papers for joint liquid analysis, gout drug treatment, and description of clinical aspects involved and associated lesions. Finally, we looked at the follow-up, at the functional scores used to monitor the patient�s evolution, at the associated medication and at the long-term outcomes, if described. We have found opinions to vary. In the end, we draw conclusions pertaining to the practical short-term and long-term use of knee arthroscopy in the treatment of gout.

scholarly journals Long term dual antiplatelet therapy after myocardial infarction: retrospective analysis in an outpatient population

2021 ◽  
Author(s):  
Gennaro Ratti ◽  
Antonio Maglione ◽  
Emilia Biglietto ◽  
Cinzia Monda ◽  
Ciro Elettrico ◽  
...  
Keyword(s):  
Myocardial Infarction ◽  
Term Treatment ◽  
Multivessel Disease ◽  
Coronary Syndrome ◽  
Anticoagulant Drugs ◽  
Long Term Treatment ◽  
Risk Condition ◽  
History Of

Long term treatment with ticagrelor 60 mg and low-dose aspirin are indicated after acute coronary syndrome (ACS). We retrospectively reviewed aggregate data of 187 patients (155 M and 38 F) (mean age 63.8±9 years) in follow up after ACS with at least one high risk condition (Multivessel disease, diabetes, GFR<60 mL/min, history of prior myocardial infarction, age >65 years) treated with ticagrelor 60 mg twice daily (after 90 mg twice daily for 12 months). The results were compared with findings (characteristics of the patients at baseline, outcomes, bleeding) of PEGASUS-TIMI 54 trial and Eu Label. The highrisk groups were represented as follows: multivessel disease 105 pts (82%), diabetes 63 pts (33%), GFR< 60 mL/min 27 pts (14%), history of prior MI 33 pts (17%), >65 year aged 85 pts (45%). Treatment was withdrawn in 7 patients: 3 cases showed atrial fibrillation and were placed on oral anticoagulant drugs, one developed intracranial bleeding, in three patients a temporary withdrawal was due to surgery (1 colon polyposis and 2 cases of bladder papilloma). Chest pain without myocardial infarction occurred in 16 patients (revascularization was required in 9 patients). Dyspnea was present in 15 patients, but was not a cause for discontinuation of therapy. Long term treatment with ticagrelor 60 mg twice daily plus aspirin 100 mg/day showed a favourable benefit/risk profile after ACS.  In this study all patients had been given ticagrelor 90 mg twice daily for 12 months and the 60 mg twice daily dosage was started immediately thereafter, unlike PEGASUS-TIMI 54 trial in which it was prescribed within a period ranging from 1 day to 1 year after discontinuation of the 90 mg dose. This makes our results more consistent with current clinical practice. However, a careful outpatient follow-up and constant counseling are mandatory to check out compliance to therapy and adverse side effects.

Long-term treatment with leuprorelin for spinal and bulbar muscular atrophy: natural history-controlled study

2017 ◽  
Vol 88 (12) ◽  
pp. 1026-1032 ◽  
Author(s):  
Atsushi Hashizume ◽  
Masahisa Katsuno ◽  
Keisuke Suzuki ◽  
Akihiro Hirakawa ◽  
Yasuhiro Hijikata ◽  
...  
Keyword(s):  
Natural History ◽  
Muscular Atrophy ◽  
Term Treatment ◽  
Controlled Study ◽  
Long Term Treatment

scholarly journals Acne Conglobata Associated with Hidradenitis Suppurativa, Disorders of Follicular Occlusion (Case Report)

2015 ◽  
Vol 15 (2) ◽  
pp. 30-34
Author(s):  
K. Pecova
Keyword(s):  
Hidradenitis Suppurativa ◽  
Severe Form ◽  
Term Treatment ◽  
Acne Conglobata ◽  
Long Term Treatment ◽  
Associated Symptoms ◽  
History Of ◽  
Inflammatory Bowel ◽  
Prepubertal Age

Abstract The author is presenting the case of a 23-year-old female patient with a severe form of acne conglobata, with the first symptoms of the disease occurring as far back as the prepubertal age. In the past year the disease has combined with hidradenitis suppurativa (to be referred to henceforth as “HS”), Hurley stage I, in the axillae and both sides of the inguinal region, with a family history of acne conglobata (both her mother and brother were affected). Further examinations ruled out inflammatory bowel disease because of a lack of further associated symptoms, except for sideropenic anaemia (lesser form) and lower serum values of vitamin D. Up until now the disease has been resistant to treatment, including the long-term treatment of methylprednisolone in combination with isotretinoid as well as dapsone and antibiotics.

scholarly journals Prolonged remission after long-term treatment with steroidogenesis inhibitors in Cushing's syndrome caused by ectopic ACTH secretion

2012 ◽  
Vol 166 (3) ◽  
pp. 531-536 ◽  
Author(s):  
S T Sharma ◽  
L K Nieman
Keyword(s):  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Term Treatment ◽  
Sustained Remission ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
Free Cortisol ◽  
Long Term Treatment ◽  
History Of

Spontaneous remission is rare in ectopic ACTH syndrome (EAS). We describe four patients with presumed EAS in whom long-term treatment with steroidogenesis inhibitors was followed by prolonged remission of hypercortisolemia. Biochemical testing was consistent with EAS, but imaging failed to identify a tumor. Patients were treated with ketoconazole alone or with mitotane and/or metyrapone to control hypercortisolemia. Dexamethasone was added when a block and replace strategy was used. Treatment with steroidogenesis inhibitors for 3–10 years in these patients was followed by a prolonged period of remission (15–60 months). During remission, the first patient had an elevated ACTH, low cortisol and 24-h urinary free cortisol (UFC), and adrenal atrophy on computerized tomography scan during remission, suggesting a direct toxic effect on the adrenal glands. Cases 2 and 3 had normal to low ACTH levels and low-normal UFC, consistent with an effect at the level of the ectopic tumor. They did not have a history of cyclicity and case 3 has been in remission for ∼5 years, making cyclic Cushing's syndrome less likely. Case 4, with a history of cyclic hypercortisolism, had normal to slightly elevated ACTH levels and low-normal UFC during remission. The most likely etiology of remission is cyclic production of ACTH by the ectopic tumor. Spontaneous and sustained remission of hypercortisolemia is possible in EAS after long-term treatment with steroidogenesis inhibitors; a drug holiday may be warranted during chronic therapy to evaluate this. The pathophysiology remains unclear but may involve several different mechanisms.

scholarly journals Diagnostics of medullary thyroid cancer and the strategy of its management in the absence of biochemical remission

2011 ◽  
Vol 57 (6) ◽  
pp. 45-51
Author(s):  
D O Gazizova ◽  
D G Bel'tsevich ◽  
A N Tiul'pakov ◽  
O V Simakina ◽  
T V Soldatova
Keyword(s):  
Thyroid Cancer ◽  
Early Diagnosis ◽  
Successful Treatment ◽  
Medullary Thyroid Cancer ◽  
Diagnostic Techniques ◽  
Term Treatment ◽  
Medullary Thyroid ◽  
Long Term Treatment ◽  
Biochemical Remission

Early diagnosis greatly facilitates successful treatment of medullary thyroid cancer. The present paper is designed to report the results of analysis of the studies carried out with the use of various diagnostic techniques and the data obtained during the long-term treatment of the patients with this pathology in the absence of biochemical remission.

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