Among asthma medications reported during the periconceptional period among controls, albuterol accounted for 85.1% of all bronchodilator use, and fluticasone, prednisone, and montelukast accounted for 46.1%, 15.6%, and 14.9% of anti-inflammatory use, respectively. Of the women who reported bronchodilators during the periconceptional period, 71.1% reported use throughout pregnancy and only 29.4% reported concurrent use of an anti-inflammatory. We observed one statistically significant association between maternal bronchodilator use only and anomalous pulmonary venous return (odds ratio 2.3, 95% confidence interval 1.1-4.8) among numerous comparisons.
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COPD was associated with increased risk of bipolar disorder independent of a number of potential confounding factors in this study.
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The medical charts of 59 patients with total loss of hearing, defined as pure tone thresholds in the profound range (> 90 dB) with an unobtainable speech reception threshold (SRT) that were treated with OP (n = 20), ITD (n = 13), or OP followed by salvage ITD (n = 26) were analyzed. Response to treatment was evaluated by means of pure tone thresholds, SRT, and speech discrimination score (SDS), immediately after treatment and on a follow-up visit.
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Italian Agency of Drug Evaluation, Istituto Giannina Gaslini (Genoa, Italy), Myositis Association (USA).
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We evaluated 42 patients with lupus nephritis that performed 109 different blood collections; their mean age was 37.7 ± 13.1 years old, and 88% were female; the mean estimated glomerular filtration rate was of 61.9 ± 20.0 mL/min. Patients were divided according to their glucocorticoid dose in two groups: A - high (pulse therapy with methylprednisolone and prednisone > 0.5 mg/kg/d, n = 14) versus B - low doses (prednisone ≤ 0.5 mg/kg/d, n = 28). Serum creatinine levels were used as parameters for renal function comparison. Cystatin C was determined by an in-house methodology, using Luminex system flow citometry.
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Coexistence of idiopathic orbital inflammatory pseudotumor (IOIP) and thyroid-associated ophthalmopathy (TAO) is extremely rare. The purpose of this article is to analyze the clinical features, image findings, and therapeutic outcomes of concomitant IOIP and TAO in China.
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The study objective was to report treatment with an interleukin (IL)-1 receptor antagonist, anakinra, in patients with multiorgan Behcet's disease (BD). Comparison of clinical manifestations, previous treatments, markers of inflammation, concomitant medications, treatment regimen modifications, relapses, and adverse events before and during anakinra administration among patients with BD were evaluated. Nine BD patients (mean age 34.55 ± 16.30 years) refractory to tumor necrosis factor blockers and standardized therapies are reported in our survey. Their mean age at disease onset was 25 ± 13.88 years and their overall disease duration was 9.55 ± 5.33 years. All patients were positive for the HLA-B51 allele. Within 1 or 2 weeks following the initiation of anakinra, eight out of nine patients promptly responded, and most of them were maintained on 100 mg of daily anakinra with low doses of prednisone. However, most patients experienced a relapse in one or more clinical manifestations over time (mean time to relapse 29 ± 21.65 weeks), and only one patient remained completely under control on anakinra monotherapy. Despite a relapse in one or more disease manifestations, treatment was continued in most patients for a mean period of 13.75 ± 6.49 months. No serious adverse events occurred. Eight out of nine refractory BD patients showed a prompt improvement after starting anakinra, supporting the concept that IL-1 plays a pathological role in this disease. Nevertheless, after several months, most patients experienced a relapse. It remains unclear whether increasing the dose of anakinra would have prevented the reoccurrence of disease activity.
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Charts of non-Hodgkin lymphoma (NHL) and prostate cancer (PC) patients treated at Moffitt Cancer Center between January 1999 and September 2006 were reviewed, and patients who received cyclophosphamide, doxoruicin, vincristine, prednisone, rituximab or Docetaxel plus steroids were eligible. Demographics, vitals, comorbidity, laboratory parameters including baseline and average glucose level during chemotherapy, G4 hematological toxicity (HemT), and G3-G4 non-hematological toxicity (NHemT), progression, and death dates were recorded.
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To evaluate bone mineral density (BMD) and levels of bone turnover markers in Egyptian children with classic congenital adrenal hyperplasia (CAH) caused by 21-hydroxylase deficiency and its relationship with disease-related variables.
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Dexamethasone is more efficacious than prednisone in the treatment of acute lymphoblastic leukemia (ALL), but has also been associated with greater toxicity. We compared neuropsychological outcomes for patients treated on DFCI ALL Consortium Protocol 00-01, which included a randomized comparison of the two steroid preparations during post-induction therapy in children and adolescents with ALL.
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An effective treatment for children with refractory nephrotic syndrome (NS), especially in those with cyclosporine (CsA)-resistant or CsA-intolerant NS, has yet to be established. Recently, the efficacy of multidrug therapy consisting of tacrolimus (Tac), mycophenolate mofetil (MMF) in combination with prednisolone (PDN) in adult patients with refractory NS has been reported. We successfully treated 14 consecutive children with refractory CsA-resistant or CsA-intolerant NS using combination therapy consisting of relatively low-dose Tac, mizoribine (MZR), which has a mechanism of action very similar to that of MMF, and PDN. There were no serious clinical toxicities. Of the 14 children, 9 with a mean age of 13.0 years had steroid-dependent NS (SDNS) and 5 with a mean age of 9.6 years had steroid-resistant NS (SRNS). All SDNS patients had minimal change disease (MCD), 4 with SRNS had focal segmental glomerulosclerosis (FSGS), and the remaining child had MCD on renal biopsy. All patients were in a prospective cohort, but were evaluated retrospectively. The mean follow-up from the initiation of multidrug therapy was 18.4 months in SDNS and 18.6 months in SRNS patients. At the last observation point, the calculated relapse rate and minimum dose of PDN required for maintenance of clinical remission after the start of multidrug therapy were significantly decreased compared with those prior to this therapy, while on CsA, in SDNS patients (0.4 ± 0.5 times/year vs 2.9 ± 1.5 times/year, P = 0.0077, and 0.3 ± 0.2 mg/kg on alternate days vs 0.5 ± 0.2 mg/kg on alternate days, P = 0.0184 respectively). All SDNS and two SRNS patients (40%) achieved complete remission, allowing further decreases in the minimal doses of PDN required for maintenance of clinical remission in most our patients. However, one patient with FSGS remained refractory to multidrug therapy and subsequently developed end-stage renal disease. These clinical observations, although preliminary and involving a small number of patients, suggest that multidrug therapy consisting of relatively low-dose Tac, MZR, and PDN might be effective and safe for treating children with refractory CsA-resistant or CsA-intolerant NS. However, further studies involving larger numbers of patients are needed.
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The heterogeneous keratinocyte cytokine gene expression reflects the complex immunological and inflammatory background in SLE. Patients with severe/extensive skin lesions showed a higher frequency of cytokine gene overexpression. Increased IFN-γ and IL-5 expression suggests that Th1 and Th2 cells are involved in SLE skin inflammation. The possibility that prednisone and antimalarial drugs may have contributed to low cytokine gene expression in some samples cannot be ruled out.
The treatment of adult refractory idiopathic membranous nephropathy with steroid and other immunosuppressant-resistant nephrotic syndrome can be a significant challenge. The authors investigated the efficacy and safety of tacrolimus (TAC) as a promising regimen.
Fifty-four ALHL patients were randomly divided into no-prednisone group (n = ten), low-dose prednisone group (n = 22) and high-dose prednisone group (n = 22). All patients were treated for ten days and followed up for six months from the initial examination.
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The median age was 83 years (range, 80-96 years). Fifty-four percent of the patients had intermediate- to high-risk or high-risk International Prognostic Index scores. Fifteen percent had scores of 4 or higher on the Charlson Comorbidity Index (CCI). The initial therapies included rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP; 70%); rituximab, etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin (R-EPOCH; 6%); and non-anthracycline-based therapies, including rituximab, cyclophosphamide, etoposide, vincristine, and prednisone (R-CEOP) and rituximab, cyclophosphamide, vincristine, and prednisone (R-CVP; 10%). With a median follow-up of 38.1 months, the 3-year failure-free survival (FFS) and overall survival (OS) rates were 55% and 54%, respectively. Eighty-eight patients experienced relapse during the follow-up, but only 3 patients (3.4%) experienced relapse beyond 3 years. Patients who received R-CHOP or R-EPOCH had significantly longer FFS than those who received R-CEOP or R-CVP, with 3-year FFS rates of 63% for R-CHOP, 74% for R-EPOCH, and 23% for R-CEOP and R-CVP. Male sex, a monocyte count ≥ 500 × 10(7) /L, and a CCI score ≥ 4 were significantly associated with inferior OS. Extranodal disease (≥2) and a higher CCI score were associated with a high risk of treatment-related mortality.
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We describe a breast cancer patient who developed heavy proteinuria (protein/creatinine ratio 9.1) and nephrotic syndrome following treatment with oral ibandronate for 29 months. CFSGS was proven by biopsy. There was no improvement 1 month after ibandronate was discontinued. Prednisone and tacrolimus were started and she experienced a decreased in proteinuria.
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Results support Rd continuous treatment as a new standard of care for stem-cell transplantation-ineligible patients with newly diagnosed MM of all ages.
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Pediatric hepatic angiosarcoma is a very rare malignant vascular tumor. A few cases have shown pediatric hepatic angiosarcoma occurring on a background of preexisting vascular lesions. We report the case of a newborn girl who presented extensive limbs and upper trunk cutaneous mixed vascular malformations at birth. These malformations were associated with thrombocytopenia. Cutaneous biopsies revealed complex vascular malformations with a significant lymphatic component. Compressive body suit therapy led to regression of the limbs' cutaneous vascular malformations. At the age of 9 months, the patient presented multiple heterogeneous hepatosplenic nodules. Aggressive treatment with prednisone, vincristine, and hepatosplenic embolizations resulted in initial improvement of the hepatosplenic lesions for few months, followed by an increase of the lesions with failure of response to treatment despite adding alpha-interferon-2b to treatment. The patient died at the age of 19 months. The autopsy's pathological examination revealed a hepatic-based angiosarcoma with plurimetastatic dissemination to the spleen, lungs, peritoneum, pleura, mesenteric linings as well as the serosa of the stomach and small intestine. Multiple cutaneous and visceral complex capillaro-lymphatico-venous malformations were also identified. We hypothesize that these multiple extensive mixed vascular malformations were associated with chronic lymphedema which probably predisposed to the development of the angiosarcoma in our patient.
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Acquired hemophilia A (AHA) is a rare, potentially life-threatening hemorrhagic disorder that presents a complex clinical challenge. The immune-mediated production of autoantibodies, known as factor VIII inhibitors, often results in clinically significant soft tissue or post-procedural bleeding episodes in patients without a previous diagnosis of a bleeding disorder. Acquired antibodies against factor VIII are associated with an extensive list of conditions, including pregnancy, autoimmune disease, and malignancy. There is great potential for morbidity and mortality resulting from autoantibody development. Death is more frequent within the first few weeks after symptomatic manifestation, making prompt recognition and treatment vitally important. Treatment focuses on stabilization of initial bleeding and long-term eradication of the acquired inhibitor. As no randomized clinical trials have been conducted regarding treatment in this patient population, clinical expertise and experience continue to guide treatment recommendations. This report provides an algorithm for the diagnosis of AHA and outlines potential treatment recommendations, most notably concomitant use of recombinant factor VIIa (rF7a) and factor VIII inhibitor bypassing agent to control bleeding in patients not responsive to single-agent therapy, and use of rituximab and prednisone for inhibitor eradication therapy.
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We describe in this first series of lupus documented in Niger, the epidemiological, diagnostic, therapeutic and prognostic aspects.
Thirty-three patients (mean (SD) age 59 (12) years, 27% males) were included. Mean (SD) FVC predicted and DLCO predicted at baseline were 108% (18%) and 88% (18%), respectively. In contrast to FVC, DLCO showed a progressive decline during follow-up with a maximum reduction of 6.1% (95%CI 2.5%, 9.7%; p = 0.001) at 26 weeks compared with baseline. After 26 weeks, 22% of the patients had a ≥15% DLCO decline. None of the patients reported increased dyspnea during follow-up. Risk factors for pulmonary function changes after treatment with RTX were cigarette smoking, repeated administration of the drug, and co-medication with Prednisone.
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Baseline nDNA level was significantly higher in all lymphoma subtypes compared with 61 healthy controls and was prognostic for progression-free survival in diffuse large B-cell lymphoma (DLBCL). Decreases in nDNA levels were observed in the first week after chemotherapy; in FL, early falls in nDNA predicted for long remission following therapy. In DLBCL, elevations in nDNA occurred in cases with progressive disease. Circulating CK18 increased within 48 h of chemotherapy and was significantly higher in patients experiencing epithelial toxicity graded >3 by Common Terminology for Classification of Adverse Events criteria. FLT3 ligand was elevated within 3-8 days of chemotherapy initiation and predicted those patients who subsequently developed neutropenic sepsis.
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We present a case of infantile bullous pemphigoid (BP) triggered by primary infection with varicella zoster virus and we analyze the correlation between autoantibody levels and disease activity. With this report we suggest that serum autoantibody titers may not necessarily mirror the clinical course of the disease or represent a helpful tool in guiding therapeutic decisions in infantile BP.
Evans syndrome (ES) in childhood-onset systemic lupus erythematosus (cSLE) patients has been rarely reported and limited to small populations.
Favorable phase I results justified this pilot phase II study to assess the efficacy of docetaxel/curcumin in patients with chemotherapy-naive metastatic castration-resistant prostate cancer (CRPC).
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Eight (15.1%) of the 53 children receiving prednisone and 2 (8%) of the 25 who did not were readmitted within 2 weeks (P=0.33), usually for treatment of pain. Patients with moderate-to-severe ACS were more likely to receive prednisone. There was no difference in the duration of stay or the need for blood transfusion between the 2 groups; 21.8% of all the patients received blood.
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Graham-Little syndrome, also know as Graham-Little-Piccardi-Lassueur syndrome, is an unusual form of lichen planopilaris, characterized by the presence of cicatricial alopecia on the scalp, keratosis pilaris of the trunk and extremities, and non-cicatricial hair loss of the pubis and axillae. We present the case of a 47-year-old woman whose condition was unusual in that there was a prominence of scalp findings. Her treatment included a topical steroid plus systemic prednisone beginning at 30 mg every morning, which rendered her skin smooth, but did not alter her scalp lopecia.
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Dermatology departments from 13 university hospitals in France. Patients The study population comprised 26 patients with typical clinical, histologic, and immunofluorescence findings of pemphigus, who were followed up over a 17-month period.