Low-dose Steroid Therapy Research Articles

A limitation and efficacy of low dose steroid therapy in IgA nephropathy: Prospective randomized control trial in the two study groups according to the amount of urinary protein excretion

A limitation and efficacy of low dose steroid therapy in IgA nephropathy: Prospective randomized control trial in the two study groups according to the amount of urinary protein excretion

Hashimoto's Encephalopathy Presenting as an Acute Medical Emergency

Three cases are described of a reversible encephalopathy, all presenting with marked neurological disturbance. In all three, the diagnosis was not clear at the time of presentation but eventually it was felt all of the cases were consistent with Hashimoto's encephalopathy. The diagnosis of Hashimoto's encephalopathy should be considered in any case of unexplained encephalopathy. Common features are high anti-thyroid peroxidase antibody titres, an abnormal EEG and an elevated CSF protein concentration. The encephalopathy is independent of thyroid hormonal status. Treatment with corticosteroids leads to a prompt resolution of symptoms and long-term low dose steroid therapy prevents further neurological recurrence.

IS THERE AN ASSOCIATION BETWEEN ULCERATIVE COLITIS AND FAMILIAL NONKETOTIC HYPERGLYCINEMIA

Introduction: Familial Nonketotic Hyperglycinemia (FNHG) is an autosomal recessive disorder of amino acid metabolism, a defect in glycine cleavage, which leads to rapidly progressing neurologic symptoms. These symptoms include failure to thrive, hypotonia, seizures, drowsiness and lethargy which can progress to a comatose state. There is currently no known association between FNHG and Ulcerative Colitis (UC); we here present our experience with a brother and sister who have both. Case Reports: Our patients are a 20-year-old female and a 15-year-old male, both of whom were diagnosed with FNHG shortly after birth, and despite lifelong treatment with Sodium Benzoate, have experienced the continued CNS deterioration associated with this disease. They have severe mental retardation, cortical blindness and seizures. The 20-year-old female presented at 7 years of age with bloody stools and severe abdominal pain. She was subsequently diagnosed with UC based on the microscopic pathology of biopsies obtained endoscopically from her upper and lower GI tract. Since diagnosis, her UC has been relatively well controlled with Sulfasalazine and steroid bursts for infrequent exacerbations. Her 15-year-old brother presented with failure to thrive and bloody stools at 6 years of age, and was also diagnosed with UC based on microscopic pathology. Initially his colitis was also manageable with Sulfasalazine and Prednisone. However, he soon became refractory to treatment. More aggressive treatment, which included Cyclosporine and Methotrexate, was unable to control his symptoms, and he required multiple hospitalizations for repeated lower GI bleeding. At 7 years of age, his UC could no longer be medically managed, and he required a colectomy. He did well for approximately 2 1 years after the colectomy, requiring only low dose steroid therapy. At 9 years of age, he had a sudden and life threatening lower GI bleed of the retained rectal segment that could not be controlled. This uncontrollable bleeding necessitated a complete proctectomy and anal closure. Family history is negative for Inflammatory Bowel Disease. Conclusion: We note these siblings with FNHG both developed UC at an early age. Even with the differing severities of their UC, it raises the question as to whether there is an association between Familial Nonketotic Hyperglycinemia and Ulcerative Colitis.

Low Dose Steroid Therapy at an Early Phase of Acute Respiratory Distress Syndrome After Thoracic Surgery

Low Dose Steroid Therapy at an Early Phase of Acute Respiratory Distress Syndrome After Thoracic Surgery

Campath-1H in renal transplantation: The University of Wisconsin experience

Immune cell depletion is known to prevent renal allograft rejection and injury. We evaluated the humanized monoclonal antibody Campath-1H (alemtuzumab; ILEX Oncology, San Antonio, Texas) in renal transplant recipients for its safety and efficacy in preventing rejection when used in combination with a calcineurin inhibitor, mycophenolate mofetil, and low-dose steroid therapy. One hundred twenty-six consecutive renal allograft recipients received 2 doses of Campath-1H antibody on days 0 and 1. Outcomes were compared to patients who received an anti-CD25 antibody (n=799), Thymoglobulin (n=160), or other antibody treatment (n=156) in combination with a calcineurin inhibitor, mycophenolate mofetil, and higher dose steroids. The Campath-1H group overall experienced less rejection than the other 3 groups (P=.037). Patients with delayed graft function experienced less rejection with Campath-1H than control groups (P=.0096) and improved graft survival (P=.0119). There was no difference in infection or malignancies between the 4 groups. Campath-1H was well tolerated in renal transplant patients and led to significant reductions in incidence of rejection. Patients with delayed graft function experienced significantly improved graft survival.

Intensity modulated radiation therapy for optic nerve sheath meningioma

Optic nerve sheath meningiomas (ONSM) account for approximately 2% of all orbital tumors. They often have an indolent course, and consequently observation is usually indicated. However, when visual acuity worsens or the lesion progresses on imaging, intervention is typically warranted to preserve vision or prevent further neurological damage. Surgical intervention is often associated with visual morbidity. Radiation therapy has been proven to halt further progression and often improves visual function. Conventional radiation therapy has been effective in halting growth and preserving vision, however, it is associated with high rates of complication. Here we update previously reported data in 1999 by investigating the outcome and toxicities of intensity modulated radiation therapy (IMRT) of ONSM. The mean follow-up was 20 months with a range of 2 to 71 months. A retrospective analysis of 22 patients with ONSM that presented to our institution between 1994 and 2003 was performed. All patients were treated using IMRT with dynamic arcs delivered via the NOMOS Peacock/Corvus system. Patients presented with substantial variety in initial visual function, extent of tumor, and prior procedure. Our definition of surgical intervention did not include biopsy for the purpose of diagnosis. The range of prescribed dose was 4930 cGy to 5040 cGy with daily fraction sizes of 160 cGy to 200 cGy. Results were assessed based on subjective patient report, visual field testing, optic examination, and magnetic resonance imaging. Visual evaluation was precluded in one patient who received enucleation after numerous years of uniocular blindness. Subjective visual improvement was present in 71% of patients (83% radiation alone / 40% surgerysradiation). Subjectively stable vision was present in 24% of patients (17% radiation alone / 40% surgerysradiation). Objective visual field improvement was present in 63% of patients (73% radiation alone / 40% surgerysradiation). Objectively stable vision was present in 31% of patients (27% radiation alone / 40% surgerysradiation). Worsening of objectivessubjective vision was present in one patient that received radiation immediately following subtotal surgical excision. Magnetic resonance imaging showed stable disease in 83% of patients and regression in 17% of patients. Most patients that had improvement in vision showed some improvement on examination during radiotherapy. The majority of patients received low dose steroid therapy as prophylaxis, as well as oral antibiotics to prevent infection of surgical-fixed immobilization screws. Consequently, most patients were given grade 2 acute toxicity by RTOG standards. Most patients had minimal side effects during treatment. Three major acute toxicities were identified: hospitalization for a persistent CSF leak at the site of surgical screw placement (for immobilization and localization) and contralateral retinal detachment (likely idiopathic). Both of these patients had full uncomplicated recovery with improvement of vision. The third patient was the only patient to have worsening vision (as mention above). Rapid visual loss to complete monocular blindness occurred during radiation therapy, and represents the only late toxicity (RTOG Grade 4). Radiation therapy provides excellent long term control of progressive ONSM and often improves subjective and objective vision. This is often identified during the radiation treatments. Conformal therapy techniques such as IMRT offer the same benefit with a very low rate of late toxicity. Surgical management has been associated with higher rates of visual morbidity. Consequently, cases of progressive ONSM should utilize radiation therapy with advanced conformal techniques when vision preservation is a therapeutic goal

Pharmacological management to reduce exacerbations in adults with asthma: a systematic review and meta-analysis.

Over the last 2 decades, many new pharmacological agents have been introduced to reduce the growing morbidity associated with asthma, but the long-term effects of these agents on exacerbations are unclear. To systematically review and quantitatively synthesize the long-term effects of inhaled corticosteroids, long-acting beta2 agonists, leukotriene pathway modifiers/receptor antagonists, and anti-IgE therapies on clinical outcomes and particular clinically relevant exacerbations in adult patients with chronic asthma. MEDLINE, EMBASE, and Cochrane databases were searched to identify relevant randomized controlled trials and systematic reviews published from January 1, 1980, to April 30, 2004. We identified additional studies by searching bibliographies of retrieved articles and contacting experts in the field. Included trials were double-blind, had follow-up periods of at least 3 months, and contained data on exacerbations and/or forced expiratory volume in 1 second. The effects of interventions were compared with placebo, short-acting beta2 agonists, or each other. Inhaled corticosteroids were most effective, reducing exacerbations by nearly 55% compared with placebo or short-acting beta2 agonists (relative risk [RR], 0.46; 95% confidence interval [CI], 0.34-0.62; P<.001 for heterogeneity). Compared with placebo, the use of long-acting beta2 agonists was associated with 25% fewer exacerbations (RR, 0.75; 95% CI, 0.64-0.88; P =.43 for heterogeneity); when added to inhaled corticosteroids, there was a 26% reduction above that achieved by steroid monotherapy (RR, 0.74; 95% CI, 0.61-0.91; P =.07 for heterogeneity). Combination therapy was associated with fewer exacerbations than was increasing the dose of inhaled corticosteroids (RR, 0.86; 95% CI, 0.76-0.96; P =.65 for heterogeneity). Compared with placebo, leukotriene modifiers/receptor antagonists reduced exacerbations by 41% (RR, 0.59; 95% CI, 0.49-0.71; P =.44 for heterogeneity) but were less effective than inhaled corticosteroids (RR, 1.72; 95% CI, 1.28-2.31; P =.91 for heterogeneity). Use of monoclonal anti-IgE antibodies with concomitant inhaled corticosteroid therapy was associated with 45% fewer exacerbations (RR, 0.55; 95% CI, 0.45-0.66; P =.15 for heterogeneity). Inhaled corticosteroids are the single most effective therapy for adult patients with asthma. However, for those unable or unwilling to take corticosteroids, the use of leukotriene modifiers/receptor agonists appears reasonable. Long-acting beta2 agonists may be added to corticosteroids for those who remain symptomatic despite low-dose steroid therapy. Anti-IgE therapy may be considered as adjunctive therapy for young adults with asthma who have clear evidence of allergies and elevated serum IgE levels.

Evaluation and management of alveolitis and interstitial lung disease in scleroderma.

In the fibrosing alveolitis of systemic sclerosis, treatment decisions depend on prognostic evaluation, which continues to excite considerable interest and debate. Advances in the staging of fibrosing alveolitis of systemic sclerosis and recent therapeutic studies are discussed in this review. The decision about whether to start treatment is often the most difficult clinical challenge, because many patients have limited pulmonary fibrosis that will not necessarily progress. The estimation of disease extent (using high-resolution CT) and disease severity (using pulmonary function tests) is pivotal. Factors reducing the threshold for treatment, in addition to severe disease, include evidence of recent deterioration, a short duration of systemic disease, antitopoisomerase antibody positivity, and, in some cases, bronchoalveolar lavage findings (although the role of bronchoalveolar lavage remains contentious). Histologic appearances at surgical biopsy have little prognostic value, with the great majority of patients having nonspecific interstitial pneumonia. Best current initial treatment consists of either oral or intravenous cyclophosphamide, usually administered with low-dose corticosteroid therapy, although the risk of scleroderma renal crisis with low-dose steroid therapy requires further evaluation. Careful prognostic evaluation, including the staging of disease severity and the definition of longitudinal disease behavior (by serial imaging and pulmonary function tests), is central to the formulation of a logical management plan in fibrosing alveolitis of systemic sclerosis. Cyclophosphamide, the best initial treatment currently, is associated with significant toxicity, justifying therapeutic studies of other immunosuppressive agents and a wide range of anticytokine and antifibrotic agents.

Preoperative steroid administration: effect on morbidity among patients undergoing intestinal bowel resection for Crohńs disease.

Long-term steroid therapy may predispose to increased perioperative morbidity in patients undergoing surgery with bowel anastomoses. The aim of our study was to review our data to determine if the steroid dosage is associated with the incidence of early complications after bowel resection in patients with prolonged steroid therapy for Crohńs disease (CD). Altogether, 397 patients underwent bowel resection with primary intestinal anastomoses for CD between 1982 and 2000 in our institution. The mortality and morbidity rates, anastomotic leakage, wound infections, intraabdominal abscesses, reoperation rate, and length of postoperative hospitalization in patients who were having high-dose (>/= 20 mg of prednisolone per day, n = 73) and low-dose (< 20 mg prednisolone per day, n = 146) steroid therapy for more than 1 month before surgery were compared with those of patients ( n = 177) who were not receiving steroids. Statistical analysis was performed using Fisher's exact test and Student's t-test, with p < 0.05 considered significant. The three groups were similar in terms of gender, duration since first diagnosis, American Society of Anesthesiologists classification, and obesity. Mortality, morbidity, anastomotic leakage, wound infections, intraabdominal abscesses, reoperation rate, and average postoperative stay were not statistically different in patients with high-dose, low-dose, or no steroid therapy. The only factor associated with increased morbidity was a low preoperative hemoglobin level. Our results demonstrate that, in patients who are undergoing bowel resection for CD, even high-dose prolonged preoperative systemic steroid therapy is not associated with increased postoperative complications.

Renal function improves in liver transplant recipients when switched from a calcineurin inhibitor to sirolimus

Sirolimus (Rapamune; Wyeth-Ayerst, Philadelphia, PA) is a newer immunosuppressive drug with no known acute or chronic nephrotoxic effects; however, limited data are available in liver transplant recipients. We prospectively evaluated changes in renal function in liver transplant recipients after conversion from a calcineurin inhibitor to sirolimus monotherapy. We measured serial serum creatinine levels in liver transplant recipients with chronic nephrotoxicity caused by calcineurin inhibitors before and after conversion to sirolimus therapy. Estimated glomerular filtration rate (eGFR) was calculated from the Modification of Diet in Renal Disease formula. Change in eGFR over time, incidence of acute hepatocellular rejection, and adverse events while being administered sirolimus monotherapy were recorded. Mean interval between liver transplantation and initiation of sirolimus therapy was 310 weeks (range, 9 to 780 weeks). Of 21 patients included in our study, 18 patients were converted to sirolimus monotherapy and 3 patients were switched to sirolimus and low-dose steroid therapy. Patients were followed up for a mean of 66.8 ± 38.9 (SD) weeks after conversion. Renal function improved in 71% of patients (15 of 21 patients). Median eGFR improved significantly from 34 mL/min/1.73 m2 at the time of conversion to 43 mL/min/1.73 m2 at the last follow-up (27% increase in eGFR; P = 001). Median monthly change in eGFR was from −0.25 mL/min/1.73 m2 pre-sirolimus therapy to +1.28 mL/min/1.73 m2 post-sirolimus therapy (P = .09). Adverse events were mostly mild and self-limited. Only 1 patient developed biopsy-proven acute cellular rejection, which was treated with sirolimus and mycophenolate mofetil. Two patients discontinued sirolimus therapy because of toxicity (oral ulceration, 1 patient; interstitial pneumonitis, 1 patient). Renal function improved significantly in the majority of liver transplant recipients with renal insufficiency caused by calcineurin inhibitors when converted to sirolimus therapy. Sirolimus monotherapy provided adequate immunosuppression with a low incidence of acute cellular rejection and minimal adverse events. (Liver Transpl 2003;9:1079-1085.)

Genetic and molecular genetic studies in the diagnosis of atypical lymphoid hyperplasias versus lymphoma

The major emphasis in this section of the Workshop was the use of molecular diagnostic techniques in the differential diagnosis of benign versus malignant lymphoid proliferations at nodal and extranodal sites. In 1 case, these techniques were used to help make a very specific benign diagnosis. In the remaining cases, their main intention was to help prove that a lymphoid proliferation was benign or malignant. Review of the cases described herein highlighted both their utility in this regard as well as how they can be problematic and misleading. They also highlighted how important it is for the diagnostic pathologist to know the precise studies performed and to correlate the results with the morphological, phenotypical, and other findings before reaching a conclusion. Some of these issues are discussed in greater detail herein. J. Melnick, S. Shenoy, and M. Kraus of Washington University in St. Louis discussed a classic case (90) of autoimmune lymphoproliferative syndrome (ALPS) (or Canale-Smith syndrome).1 A 16-month-old male presented with anemia, adenopathy, and splenomegaly and a lymph node biopsy showed a very worrisome and very typical interfollicular lymphoid proliferation (Fig 1). Flow cytometric analysis of the “large cell” gate and immunohistochemical evaluation of the interfollicular cells demonstrated the following classic “double-negative” T-cell phenotype: CD2 , CD3 , CD5 , and CD43 with diminished CD7 but absent CD45RO, CD4, CD8, gamma-delta, CD20, CD30, CD56, myeloperoxidase, cytomegalovirus, and Epstein-Barr virus.1 Southern blot analysis did not demonstrate evidence of either a clonal B-cell or T-cell population. Fas (CD95) gene sequencing showed a heterozygous mutation in the death domain. These findings supported the benign nature of the lymph node biopsy and confirmed the diagnosis of ALPS. It is of interest that the patient’s mother is also heterozygous for the mutation so that, as is well recognized, other factors must play a role in the phenotypic expression of this disorder.2,3 The child was reported to be doing well at 6.5 years on low-dose steroid therapy. Criteria for ALPS include (1) chronic nonmalignant lymphoproliferation, (2) defective lymphocyte apoptosis in vitro and (3) 1% T-cell receptor (TCR) alpha/beta , CD4 , CD8 (double-negative) T cells in peripheral blood and/or the presence of doublenegative cells in lymphoid tissue.2 In addition to the marked paracortical expansion as described earlier, the lymphoproliferation includes florid follicular hyperplasia often with progressively transformed follicular centers and a polyclonal plasmacytosis.1 The double-negative T-cell population also expresses cytotoxic proteins, such as TIA-1, and many are CD57 .1 A CD5 polyclonal B-lymphocytosis is also characteristic.1 Supporting criteria include autoimmunity/autoantibodies and mutations in TNFRSF6 (Fas gene), FasL (Fas ligand), or caspase 10 gene. Fas is a cell surface receptor that binds Fas ligand and then initiates apoptosis initially by binding FADD, which in turn leads to activation of caspase 8 or 10. Patients with ALPS usually present as infants or young children with lymphadenopathy, splenomegaly, and variable autoimmune phenomena, particularly hemolytic anemia and thrombocytopenia. Hepatomegaly is seen in about 50% of patients. There are also many other less common clinical manifestations.2 Although the lymphoproliferative component is said to regress over time, the autoimmune phenomena are more likely to occur. Molecular studies have been used to identify 4 different types of ALPS.4,5 These include cases with generally heterozygous mutations in TNFRSF6 (type Ia), in the Fas ligand gene (Ib) or in the gene for the “downstream” caspase 10 (II), and those without any recognized molecular abnormalities (III). The Fas mutations are found throughout the gene, but most are in the exon that contains the death domain. Mutations in the extracellular portion of Fas are less likely to have ALPS and, if they do, to have less severe ALPS.4 It should be recognized that Fas gene mutations are not restricted to patients with ALPS and their relatives. Fas mutations occur normally in germinal centers,6 have been reported in a patient fulfilling the criteria for systemic lupus erythematosus,7 and occur in mucosaassociated lymphoid tissue and other lymphomas8 as well as in other malignancies.2 Molecular studies are also important in these cases, because they can be used (as in the case presented here) to support a benign diagnosis in the face of worrisome morphology by failing to demonstrate a From the Division of Hematopathology, University of Pittsburgh Health System, Pittsburgh, PA. Accepted for publication December 1, 2002. Address correspondence and reprint requests to Steven H. Swerdlow, MD, UPMC-Presbyterian, UPMC Health System, Division of Hematopathology, 200 Lothrop St., Room PUH C606, Pittsburgh, PA 15213-2582. © 2003 Elsevier Inc. All rights reserved. 0046-8177/03/3404-0008$30.00/0 doi:10.1053/hupa.2003.95

Low-Dose Steroid Therapy Reduces Mortality from Septic Shock

Because septic shock may be associated with relative adrenal insufficiency, corticosteroid replacement has been advocated. Trials of short-course,

Alterations of glucocorticoid receptor expression during glucocorticoid hormone therapy in renal transplant patients.

Previous studies have demonstrated that glucocorticoid resistance develops in some patients during glucocorticoid therapy due to the down-regulation of glucocorticoid receptor (GCR) expression. A new flow cytometric method has been used for monitoring the intracellular GCR level in peripheral blood mononuclear cells (PBMCs). GCR expression by different lymphocyte subpopulations (bearing surface CD4+ CD8+ or CD19+) in steroid-treated (ST) and non-treated (NT) renal transplant patients was also compared. High, decreasing-dose steroid treatment caused a reduced GCR expression in 50% of the renal transplant patients. Long-lasting, low-dose steroid therapy caused a fall in GCR level in only 11% of patients. CD4+ T lymphocytes showed both the lowest GCR level and its alteration due to steroid therapy, while CD8+ T cells and CD19+ B lymphocytes expressed higher GCR levels and were more sensitive to steroid treatment. Detection of GCR expression in lymphocytes may provide useful information about the changes of glucocorticoid sensitivity during the therapy.

Short-Term Outcome and Prognosis of Acute Low-Tone Sensorineural Hearing Loss by Administration of Steroid

Acute low-tone hearing loss (ALHL) is a typical type of hearing loss in Ménière’s disease and thought to be caused by endolymphatic hydrops in the inner ear. We treated 40 patients with ALHL by administration of the steroid and the early outcome and prognosis of the hearing level was retrospectively evaluated. The prognosis was generally determined within 7–10 days after administration of steroid. High-dose steroid cured some patients who failed to recover with low-dose steroid therapy. Our results showed that steroid is one of the effective therapies for ALHL and supported that etiology of ALHL involves an immune response.

Transcervical Thymectomy

Removal of the thymus gland is an accepted component of the overall therapy for myasthenia gravis. As yet there is no clearly delineated pathophysiology to explain the role of the thymus in the generation of myasthenia gravis, but over the past several decades it has become increasingly clear that a complete thymectomy improves the clinical course of patients with myasthenia. This article outlines the techniques and results from transcervical thymectomy for myasthenia gravis. Patients with thymomas are generally treated with sternotomy, with the transcervical approach reserved for patients with thymic hyperplasia or normal thymus seen on computed tomography (CT) scan. A recent report suggests that the transcervical approach may be appropriate for small tumors as well, but such extended indications should be used only by those familiar with the technique in typical patients.1Deeb ME Brinster CJ Kucharzuk J et al.Expanded indications for transcervical thymectomy in the management of anterior mediastinal masses.Ann Thorac Surg. 2001; 72: 208-211Abstract Full Text Full Text PDF PubMed Scopus (38) Google Scholar Patients with previous median sternotomy for other reasons are controversial candidates, and careful consideration should be given to full or partial sternotomy. The short- and long-term outcomes after transcervical thymectomy have been previously reported. Our report of 100 consecutive patients noted a low morbidity rate and no perioperative deaths.2Calhoun RF Ritter JH Guthrie TJ et al.Results of transcervical thymectomy for myasthenia gravis in 100 consecutive patients.Ann Surg. 1999; 230: 555-561Crossref PubMed Scopus (115) Google Scholar The complication rate was 8%, including one seizure, one deep vein thrombosis, one myasthenic crisis, and five episodes of postoperative pneumothorax. No phrenic nerve or recurrent laryngeal nerve injuries occurred. The mean operative time was 104 minutes and postoperative length of stay was just longer than 1 day. In this report, 78 patients had more than 12 months of follow-up and thus were available for long-term analysis. Overall, the median Osserman grade improved from 3 before surgery to 1 after surgery. Eighty-five percent (66 of 78) of the patients improved by 1 or more Osserman grades; 63% (49 of 78) improved by 2 or more Osserman grades. Thirty-five percent achieved complete remission, experiencing no symptoms and requiring no medications. This complete remission rate is as high as 50% if patients who are free of symptoms and off Mestinon therapy but maintained on low-dose steroid therapy at the preference of their neurologist are included. Seventy-one percent (55 of 78) were free of any generalized symptoms of weakness. Only one patient deteriorated by a single Osserman grade, and 14% of the patients (11 of 78) experienced no change in Osserman grade after thymectomy. The transcervical approach for thymectomy allows safe, complete removal of the thymus and provides clinical benefit equal to that from more extensive resections.3Bril V Kojic J Ilse WK et al.Long-term clinical outcome after transcervical thymectomy for myasthenia gravis.Ann Thorac Surg. 1998; 65: 1520-1522Abstract Full Text Full Text PDF PubMed Scopus (90) Google Scholar The low morbidity and short hospitalization after transcervical thymectomy present minimal barriers and should improve the neurologists' willingness to refer suitable patients and increase the patients' acceptance of surgical therapy. In addition, the transcervical approach is attractive for older patients with less likelihood for improvement and for patients at higher risk for complications such as those on high-dose prednisone therapy. Early, safe, and complete thymectomy offers all of the benefits of surgical removal of the thymus to a patient with myasthenia with a minimal risk of morbidity and postoperative pain. The report by Gronseth suggested that multiple confounding variables prevent the comparison of results of uncontrolled trials and, as a result, there is no conclusive evidence of the superiority of one technique over another.4Gronseth GS Barohn RJ Practice parameter: Thymectomy for autoimmune myasthenia gravis (an evidence-based review). Report of the Quality Standards Subcommittee of the American Academy of Neurology.Neurology. 2000; 55: 7-15Crossref PubMed Scopus (459) Google Scholar

Impact of cyclosporine and low-dose steroid therapy on insulin sensitivity and beta-cell function in patients with long-term liver grafts.

To examine whether factors controlling glucose tolerance, i.e., insulin sensitivity (SI) and first-(phi1) and second-phase insulin secretion (phi2), are impaired in after orthotopic liver transplantation (OLT), they were assesssed in patients that had undergone OLT for cirrhosis (n = 10) with cyclosporin A and low-dose steroid therapy (5 mg prednisone per day) and were compared with those of healthy matched control subjects (n = 10). These factors were determined by means of computer-based analysis of frequently sampled intravenous glucose tolerance tests (FSIGTT). Glucose and insulin profiles (posthepatic insulin) did not differ between both groups, whereas C-peptide levels (prehepatic insulin) were elevated in the transplant group after the FSIGTT, indicating an increased hepatic insulin degradation. SI and (phi1 did not differ between both groups. phi2, however, was significantly enhanced (23.94 +/- 2.63 vs 13.88 +/- 1.25 min(-1), P < 0.05). These results indicate that cyclosporine and low-dose steroid therapy do not impair SI and phi1. However, enhanced phi2 compensates the increased hepatic insulin clearance.

Impact of cyclosporine and low-dose steroid therapy on insulin sensitivity and beta-cell function in patients with longterm liver grafts

Impact of cyclosporine and low-dose steroid therapy on insulin sensitivity and beta-cell function in patients with longterm liver grafts

Response to steroid therapy in autism secondary to autoimmune lymphoproliferative syndrome

We report a child who developed autoimmune lymphoproliferative syndrome (ALPS) secondary to a heterozygous dominant negative mutation in the death domain of the Fas receptor. Previously developmentally normal, he had symptoms of autism with rapid regression in developmental milestones coincident with the onset of lymphoproliferation and autoimmune hemolytic anemia. Low-dose steroid therapy induced early and complete remission in the ALPS phenotype. There was subjective improvement, followed by objective improvement in speech and developmental milestones. We propose that autism may be part of the autoimmune disease spectrum of ALPS in this child, and this case represents a novel manifestation and target organ involvement in this disease.

Churg-Strauss Syndrome Associated with Pregnancy.

Churg-Strauss syndrome, an uncommon condition, occurs even more rarely in association with pregnancy. One month postpartum, a 25-year-old Japanese woman was hospitalized for dyspnea, fever, diarrhea, and complaints of dysesthesia in the right lower limb and right lumbar and abdominal region. Marked eosinophilia was present. Symptoms of bronchial asthma had developed early in the pregnancy, which concluded with delivery of a healthy baby at 39 weeks of gestation. Churg-Strauss syndrome was diagnosed and prednisolone was administered with a good response leading to remission. Three years after the first pregnancy, low-dose steroid therapy was continued through another pregnancy. The patient delivered another healthy baby at 39 weeks of gestation, this time with no exacerbation of symptoms.

Safety of prolonged low-dose alternate-day adrenocortical steroid therapy in Crohn's disease

Safety of prolonged low-dose alternate-day adrenocortical steroid therapy in Crohn's disease