Patients Received Steroid Therapy Research Articles

Long term response to steroid therapy in Rasmussen encephalitis

Rasmussen encephalitis (RE) is a severe and progressive focal epilepsy of unknown origin that leads to deterioration of motor and cognitive function. In a previous study, we described positive effect of high doses of steroids during the first year after the onset of RE. The objective of this study was to evaluate this therapy at long term. We reviewed 11 patients (7 girls and 4 boys) with RE of the right hemisphere (7) and the left (4) at a follow-up of 9+/-2 years. Age at onset of RE ranged from 2 to 14 years. Six patients had no benefit from steroid therapy and underwent hemispherotomy. Five had significant reduction of seizure frequency with disappearance of epilepsia partialis continua, and improved motor function. Of these, two died of unexpected sudden death 5 and 7 years after seizure control. Two others with initial response experienced progressive recurrence of seizures 1 to 4 years after the end of steroid therapy and required hemispherotomy. Finally, only one patient exhibited total cessation of seizures with steroids for 3 years, but seizures progressively recurred although the frequency was moderate. Our data confirm that although steroid treatment can be useful when given early in the course of RE, long term relapse can occur among the good responders requiring delayed hemispheric disconnection.

Parálisis facial en urgencias de pediatría: actualización de nuestro protocolo y autoevaluación

As result of our aim to improve the quality standard of our emergency system, work has been carried out in relation to the development and monitorization of effective clinical protocols in the department of paediatric practice.An evidence based review approach was taken to design a clinical protocol about Bell's palsy condition for the paediatric emergency department. Previous protocol approved in March 2003 was reviewed accordingly with the new designed protocol's quality standards. The Bell's palsy cases reported since March 2003 until June 2006 to paediatric emergency department were analyzed.A total of 27 patients affected by Bell's palsy were reported to the hospital's emergency department. Facial expression was described in 85.19% of the cases. Cranial nerves normal function was reported in 77.78%. Fundoscopic examination was described in 77.78% and otoscopic findings in 44.44%; the absence of herpes vesicles was analyzed only in 11.11%. All patients received steroid therapy (prednisone) and the treatment resulted in the complete recovery. The mean time to resolution was 58.6 days.In order to improve hospital's quality standards, clinical protocols should be designed and verified regularly to ensure the proper performance. Medical auditing also contributes to improve effectiveness in health attendance.

IRM de diffusion-perfusion dans l’évaluation des lymphomes cérébraux

Because of the increasing incidence of cerebral lymphoma, it is critical for patient management to recognize the MR features of this disease. We present the characteristic morphological and functional MRI features of this tumor. The findings on MRI studies, including morphological, diffusion and perfusion imaging, performed in 9 biopsy-proven cases of cerebral lymphoma with 13 lesions are presented and analyzed, and are discussed in comparison with published literature data. All patients underwent diffusion-weighted imaging with a single shot echo-planar pulse sequence. Dynamic susceptibility-contrast MRI was performed using a T2*-weighted gradient-echo echo-planar sequence after intravenous injection of chelates of gadolinium at the rate of 6 ml/s and a temporal resolution of 1 second. All cases of cerebral lymphoma appeared hypointense or isointense on T1-weighted images and in 75% of cases iso- or hypointense on T2-weighted images. All lesions enhanced except one in a patient receiving steroid therapy. On diffusion-weighted images, tumours were hyperintense with normal or decreased ADC values (0.717+/-0.152.10-3 mm2/sec, range: 0.550-1.014) and an ADC ratio tumour/normal white matter of 0.974+/-0.190 (range: 0.768-1.410). On perfusion, the signal intensity-time curve of each tumour showed a characteristic type of curve with a significant increase of the signal intensity above the baseline and a low maximum relative cerebral blood volume ratio (rCVBmax) of 1.43+/-0.64 (0.55-2.62). Due to their higher cellularity, the lack of neoangiogenesis, and the increased permeability of the blood-brain barrier related to the infiltration of blood vessels wall by lymphomatous cells, cerebral lymphoma presents characteristic diffusion and perfusion MRI features that should be useful for diagnosis and patient follow-up.

Autoimmune Hemolytic Anemia (AIHA) in Children: A French Observational Study of 171 Patients for the French Society of Pediatric Hematology and Immunology (SHIP).

Introduction: AIHA is a rare and potentially severe condition in children. To assess the presentation, treatment response and outcome of childhood AIHA, a national multicentric observational study has been started in 2002 in 32 French pediatric hematologic units.Patients and Methods: A descriptive analysis of 171 children under 18 years old, who are still being followed is presented. Inclusion criteria were hemoglobin <10 g/dl, positive Coombs test, and one of the three following hemolysis criteria: reticulocytes >120 000/mm3, haptoglobin <10 mg/dl, bilirubin >10 mg/dl. Etiological investigations were standardised. Treatment procedures were at the appreciation of each physician, according to the SHIP guidelines. Complete response (CR) was defined as Hb >10g/dl and absence of biological hemolysis for more than 3 months. Variables associated with survival in CR without treatment at the last follow up were assessed using a Cox model of multivariate analysis.Results: AIHA was diagnosed between January 1986 and March 2005 in 94 males and 77 females. The median follow-up is 9 months (range 0.2 to 234). Median age at diagnosis is 3,8 years (range 0.1–17.4). Familial or personal dysimmune history was respectively positive in 21 and 28 patients. A concomitant febrile episode was present in 50% of cases, and for half of the patients, the onset was sudden and severe. Median hemoglobin level at diagnosis was 5,6 g/dl (2 to 12,8) with a reticulocyte count below 100.000/mm3 in 43 patients. The direct Coombs test was positive for IgG in 37%, for IgG+C3b in 37%, for isolated C3b in 17%, for cold agglutinin +IgM in 7% and for IgA in 2% of patients. Thrombopenia and neutropenia were associated in 26% and 18% of patients respectively. Evans syndrome was diagnosed in 66/171 patients, among whom idiopathic thrombopenic purpura and AIHA occurred simultaneously in 31/66. AIHA was a component of a dysimmune clinical or biological disease in 50% of patients, of post infectious origin in 25%, associated with malformations in 5%, and isolated in 20%. All but 8 patients received steroid therapy (1 to 5 mg/kg prednisone daily). Resistance to steroids led 91/163 patients to receive a median of 3 (1 to 8) subsequent modalities of treatment, mainly ciclosporine (n=25), anti-CD20 (n=24), splenectomy (n=22), and azathioprine (n=14). Nine children died, 8 of whom had Evans syndrome. Survival in CR without treatment at 5 and 10 years from diagnosis were respectively 42% and 20%. In multivariate analysis, three variables were associated with the absence of CR without treatment at the last follow up: Coombs test of IgG/IgG+C3b class (RR 0,22, 95% IC [0,09–0,5], p = 0,0004), dysgammaglobulinemia (RR 0,28, 95% IC [0,11–0,71], p = 0,007), and age more than 4 years (RR 0,53, 95% IC [0,27–1,02], p = 0,05).Conclusion: The present French cohort is the largest reported study of AIHA in children. Heterogeneous underlying etiological subsets are confirmed. Two prognostic factors are clearly identified: direct Coombs test of IgG or IgG+C3b class, and presence of a dysgammaglobulinemia. Ongoing hemato-immunologic and therapeutic studies will allow better comprehension and treatment of this rare disease

Clinical significance of fever in the systemic lupus erythematosus patient receiving steroid therapy

Clinical significance of fever in the systemic lupus erythematosus patient receiving steroid therapy. Background Active systemic lupus erythematosus (SLE) can cause fever. Steroids (glucocorticoids) suppress SLE fever; however, the extent to which steroid therapy affects SLE fever not previously been rigorously studied. Methods Study A is a prospective study of recurrently active SLE patients ( N = 92, 60 renal SLE and 32 nonrenal SLE) who recorded daily oral evening temperatures while participating in a longitudinal study of risk factors for SLE flare. Study B is a retrospective study of consecutive febrile SLE patients ( N = 22) who received steroids initially because SLE was suspected. At final analysis 11 had SLE fever and 11 had infection fever. Results In study A during a mean follow-up of 13.2 ± 8.1 months, 51 of the 92 patients experienced 73 SLE flares. In only one patient was SLE fever associated with SLE flare. In the other 50 patients who flared, there was no significant trend to develop fever prior to or at the onset of SLE flare. Prednisone, median dose 10mg, was being received at 82% of the study visits at which an SLE flare was declared. In study B, prednisone 28mg (range 20 to 40mg) completely suppressed SLE fever, usually within 24 hours. In contrast, infection fever persisted despite prednisone 35 to 300mg/day. Of those with infection fever, three developed fatal sepsis when high-dose steroid therapy was continued. Conclusion In SLE patients receiving prednisone at maintenance doses or greater, SLE fever is rare. When fever does develop, it is usually due to infection. Continuing high steroid dose steroid therapy in those with infection fever may increase the risk of severe sepsis.

Leukotriene inhibitors in combination with steroids: potential role in the development of primary bacterial peritonitis

Leukotrienes play a role in inflammation, and their participation in airway inflammation and bronchoconstriction in patients with severe asthma can be ameliorated by a new class of drugs known as leukotriene modulators. The role of leukotrienes in increasing vascular permeability in experimental peritonitis and in inducing chemotaxis of inflammatory cells has recently been documented. Steroids have been incriminated in the development of bacterial translocation in animal models in association with the suppression of mucosal immunity. The development of spontaneous bacterial peritonitis is recognized in cirrhotic patients with ascites and in those with nephrotic syndrome. The onset of bacterial peritonitis in the absence of these predisposing conditions or other underlying cause, such as perforated viscus, is termed 'primary bacterial peritonitis', and has never been described in asthmatic patients. We present an asthmatic patient who developed primary bacterial peritonitis while receiving a leukotriene modulator in combination with prednisolone therapy. The hypothesis that leukotriene receptor blockade might predispose to the development of primary bacterial peritonitis in patients receiving steroid therapy is discussed.

Learning from Incidents and Near-misses Reports

Learning from Incidents and Near-misses Reports

Osteonecrosis of Hip and Knee in Patients with Severe Acute Respiratory Syndrome Treated with Steroids

To evaluate whether there is a relationship between steroid treatment and risk for osteonecrosis of the hip and knee in patients with severe acute respiratory syndrome (SARS). The hospital ethics committee approved the study, and all patients provided written informed consent. A total of 254 patients with confirmed SARS treated with steroids underwent evaluation with magnetic resonance (MR) imaging for osteonecrosis. Clinical profiles, joint symptoms, relevant past medical and drug history, steroid dose, and radiographic and MR imaging evidence of osteonecrosis and other bone abnormalities were evaluated. Mann-Whitney, Kruskal-Wallis, and Pearson exact chi(2) tests were performed, and univariate and multivariate logistic regression analyses were applied. One hundred thirty-four (53%) of 254 patients had recent onset of large joint pain, but 211 (80%) of 264 painful joints were not associated with abnormality on MR images. MR images in 12 (5%) of 254 patients showed evidence of subchondral osteonecrosis in the proximal femur (n = 9), distal femur (n = 2), and proximal and distal femora and proximal tibiae (n = 1). Additional nonspecific subchondral and intramedullary bone marrow abnormalities were present in 77 (30%) of 254 patients. Results of multiple logistic regression analysis confirmed cumulative prednisolone-equivalent dose to be the most important risk factor for osteonecrosis. The risk of osteonecrosis was 0.6% for patients receiving less than 3 g and 13% for patients receiving more than 3 g prednisolone-equivalent dose. No relationship was found between additional nonspecific bone marrow abnormalities and steroid dose. An appreciable dose-related risk was found for osteonecrosis in patients receiving steroid therapy for SARS. Additional nonspecific bone marrow abnormalities were frequent. Joint pain was common after SARS infection and was not a useful clinical indicator of osteonecrosis.

Chronic Steroid and Immunosuppressant Therapy in Children

1. Abraham Gedalia, MD* 2. Avinash K. Shetty, MD† 1. *Professor of Pediatrics and Head, Division of Pediatric Rheumatology, Louisiana State University Health Sciences Center and Children’s Hospital, New Orleans, La 2. †Assistant Professor of Pediatrics, Division of Infectious Diseases, Wake Forest University School of Medicine, Winston-Salem, NC After completing this article, readers should be able to: 1. Describe the mechanism of action, list indications for therapy, and discuss the adverse effects of corticosteroid therapy in children. 2. Delineate the mechanism of action and adverse effects of other immunosuppressive agents frequently used in children. 3. Discuss the immunization recommendations for children receiving immunosuppressive therapy. Corticosteroids are the most potent, naturally occurring anti-inflammatory agents known. Despite more than 50 years of controversy regarding their clinical use, risks, and benefits, corticosteroids still play a significant role in the management of a wide variety of diseases. They have reduced the morbidity and mortality significantly in children afflicted with leukemia, asthma, rheumatic diseases, and other inflammatory disorders. In this article, we review the mechanism of action, clinical indications, and adverse effects of corticosteroids and other commonly used immunosuppressive agents in children. We also review immunization recommendations for children receiving immunosuppressive therapy. Corticosteroids have a diverse role in the human body at the physiologic level. The actions of glucocorticoids include: 1) negative feedback modulation of corticotropin-releasing factor and adrenocorticotropic hormone; 2) regulation of blood glucose and liver glycogen levels; 3) maintenance of water-electrolyte homeostasis; 4) influence on the metabolism of protein, fat, and purine; 5) maintenance of normal cardiovascular, central nervous system (CNS), and renal functions; 6) influence on circadian rhythmicity; 7) maintenance of bone and muscle work capacity; and 8) protection of the body against moderate stress. Glucocorticoids also have pharmacologic properties that are used in the treatment of rheumatic and other inflammatory diseases. Cellular receptors for cortisol are ubiquitous in cell cytoplasm, reflecting the critical role of the hormone in maintaining cell homeostasis. The type 1 glucocorticoid receptor, known as the mineralocorticoid receptor, has high affinity for aldosterone. The type 2 glucocorticoid receptor, known as the glucocorticoid receptor, exhibits strong affinity for dexamethasone. Although …

Cardiac Sarcoidosis

This retrospective study concerned 18 female and 23 male patients with cardiac sarcoidosis (CS). The average age at CS diagnosis was 38 years. CS was observed in white (73% of cases) and in black or Caribbean patients (27% of cases). All patients had extracardiac histologic proof of sarcoid tissue. In 63% of cases, the CS arose during the follow-up of systemic sarcoidosis. Systemic sarcoidosis was not specific except for a high frequency of neurosarcoidosis. Revealing cardiac signs were clinical in 63% of cases and electrical in 22%. In most patients these signs were associated with an abnormal echocardiography (77%) and/or a defect on thallium-201 or sestamibi imaging (75%). Thirty-nine patients received steroid therapy (initial dose mostly equal to 1 mg/kg per day), associated in 13 cases with another immunosuppressive treatment. In 26% of cases the immunosuppressive treatment was associated with a specific cardiac treatment. In the long-term follow-up (average follow-up, 58 mo), 87% of the cases showed an improvement, and 54% were cured from a clinical and laboratory point of view (electrocardiogram, 24-hour monitoring, echocardiography, radionuclide imaging). There was no sudden death. Two patients worsened, which can be explained in 1 case by very late treatment and in the other case by lack of treatment, except for a pacemaker. Our experience leads us to treat CS with corticosteroids as soon as possible and to use another immunosuppressive treatment where there is an insufficient therapeutic response or where there are contraindications to corticosteroids.

Ocular manifestations of dengue fever

Purpose To evaluate ocular manifestations associated with dengue fever. Design Retrospective case series and literature review. Methods Clinical records of patients with dengue fever who subsequently had ocular symptoms and signs develop were reviewed. The clinical presentation and ocular complications were evaluated. Results Six patients, 5 females and 1 male, were seen with a sudden decrease in vision 6 to 7 days after the initial manifestations of dengue fever were identified. The diagnosis was confirmed by detection of dengue-specific IgM antibodies (IgM enzyme-linked immunoassay). The presenting best-corrected visual acuity ranged from 20/30 to counting fingers, and ocular involvement was bilateral but asymmetric in 5 cases and unilateral in 1 case. Fundus findings included small, intraretinal, whitish lesions, with localized retinal and retinal pigment epithelium (RPE) disturbance, small dot hemorrhages, and vascular sheathing around the macula and the papillomacular bundle. Fluorescein angiography showed arteriolar focal knobby hyperfluorescence at the macula with mild staining of the vascular walls and leakage at the level of the RPE. All 5 cases that had indocyanine green angiography done showed early diffuse choroidal hyperfluorescence with late silhouetting of the larger choroidal vessels. Five patients received steroid therapy: 1 topical, 2 periocular, and 2 oral. Over 2 to 4 months, RPE discoloration was observed over the affected areas. After the acute episode, 3 patients showed partial recovery of vision, and in the remaining patients, the visual acuity remained stable. Conclusions Ocular complications associated with dengue fever are rare but may result in permanent visual impairment.

In vitro adherence of lymphocytes to dermal endothelium under shear stress: implications in pathobiology and steroid therapy of acute cutaneous GVHD

The extravasation of leukocytes at sites of inflammation critically depends on initial shear-resistant adhesive interactions between leukocytes in blood flow and target tissue endothelium. Dermal lymphocytic infiltrates are a hallmark feature of acute cutaneous graft-versus-host disease (acGVHD) following allogeneic hematopoietic stem cell (allo-HSC) transplantation. These infiltrates occur commonly during periods of profound lymphopenia, suggesting that the dermal endothelial adhesive mechanism(s) promoting lymphocyte emigration in acGVHD are highly efficient. To examine this issue, we performed Stamper-Woodruff assays on frozen sections of biopsy specimens of cutaneous lesions occurring within 100 days of HSC transplantation in 22 autologous hematopoietic stem cell transplant (auto-HSCT) and 25 allo-HSCT recipients. By using this shear-based assay, we observed lymphocyte adherence to papillary dermal vascular structures in all punch biopsy specimens of allo-HSCT recipients who had clinicohistologic evidence of acGVHD and who were not receiving steroids, whereas no lymphocyte adherence was observed within skin specimens from allo-HSCT recipients who did not develop acGVHD. Within the group of auto-HSCT recipients, 2 of 22 skin biopsies demonstrated lymphocyte binding to dermal vessels. Among allo-HSCT patients receiving steroid therapy for acGVHD, lymphocyte binding to dermal endothelium was abrogated prior to resolution of rash in those who responded, yet binding was persistent in skin from one patient whose rash did not respond to steroid therapy. Collectively, these data indicate that the papillary endothelium of skin in acGVHD displays heightened capacity to support lymphocyte adhesion under shear stress conditions and suggest that down-modulation of this endothelial adhesive capability may be one mechanism by which steroids abrogate acGVHD reactions.

Increased exhaled cysteinyl-leukotrienes and 8-isoprostane in aspirin-induced asthma.

The pathogenesis of aspirin-induced asthma (AIA) has not yet been clearly elucidated, although eicosanoid metabolites appear to play an important role. We hypothesized that levels of eicosanoids in exhaled air condensate are abnormal in patients with AIA and that they change in patients receiving steroid therapy. We measured cysteinyl-leukotrienes (cys-LTs), prostaglandin E(2) (PGE(2)), and leukotriene B(4) (LTB(4)), and also 8-isoprostane as a marker of oxidative stress, by enzyme immunoassay in exhaled breath condensate from patients with AIA (17 steroid naive; mean age, 41 +/- 23 years; FEV(1), 63%pred), 26 patients with aspirin-tolerant asthma (ATA) (11 steroid naive; mean age, 47 +/- 18 years; FEV(1), 69%pred), and 16 healthy subjects (mean age, 45 +/- 17 years; FEV(1), 93%pred). Cys-LTs were significantly higher in steroid-naive patients with AIA compared with steroid-naive patients with ATA and healthy subjects (152.3 +/- 30.4 and 36.6 +/- 7.1 versus 19.4 +/- 2.8 pg/ml; p < 0.05 and p < 0.05, respectively). Steroid-naive patients with AIA also had higher levels of 8-isoprostane than normal subjects (131.8 +/- 31.0 versus 21.9 +/- 4.5 pg/ml; p < 0.05). There were significantly lower levels of both cys-LTs and 8-isoprostanes in steroid-treated patients with AIA. There was no difference in either the PGE(2) or LTB(4) level between the patient groups. This is the first study to show that cys-LTs and 8-isoprostanes are elevated in expired breath condensate of steroid-naive patients with AIA, and that cys-LTs are decreased in steroid-treated patients. Exhaled PGE(2) levels are not reduced, so that it is unlikely that a deficiency of PGE(2) is an important mechanism, whereas exhaled LTB(4) levels are unchanged, indicating an abnormality beyond 5-lipoxygenase.

Sarcoidosis and Opportunistic Infections

Two patients receiving steroid therapy for sarcoidosis had a potentially fatal opportunistic infection that was difficult to differentiate from the underlying illness, but was successfully treated after the diagnosis was made. The effects of sarcoidosis on the immune system and the additional effects of steroid therapy on cell-mediated immunity seem to be of real clinical significance, rather than being of theoretical interest alone, because of the risk of infection with intracellular infecting organisms, even if such infections occur relatively infrequently.

Sarcoidosis and Opportunistic Infections

Two patients receiving steroid therapy for sarcoidosis had a potentially fatal opportunistic infection that was difficult to differentiate from the underlying illness, but was successfully treated after the diagnosis was made. The effects of sarcoidosis on the immune system and the additional effects of steroid therapy on cell-mediated immunity seem to be of real clinical significance, rather than being of theoretical interest alone, because of the risk of infection with intracellular infecting organisms, even if such infections occur relatively infrequently.

Steroid therapy in progressive IgA nephropathy: A 10 year follow‐up study

Summary: This study was undertaken in order to evaluate the effect of steroid therapy on the long‐term clinical course in progressive IgA nephropathy with persistent massive proteinuria of ≤ 1.0 g/day. From 1972 to 1986, 109 patients with persistent massive proteinuria who were continuously followed up during a 10 year period. Seventy‐seven of these patients had moderate proteinuria of 1‐2 g/day and the remaining 32 patients had heavy proteinuria of more than 2 g/day. the 109 patients were divided into three groups according to the grade of renal function and histological severity: group 1 (58 cases), preserved renal function (creatinine clearance rate [Ccr] ≤ 70 mL/min) and moderate to severe histology (total score ≤ 7); group 2 (35 cases), decreased renal function (Ccr &gt;70 mL/min) and moderate to severe histology; and group 3 (16 cases), preserved renal function and mild histology (total score ≥ 6). Fifty‐five patients received steroid therapy for 2 years, followed by an anti‐platelet drug therapy until the completion of the study. Fifty‐four patients were treated with only an anti‐platelet drug therapy for the duration of the study. There were no significant differences in renal function and renal survival rates over the 10 year period between the steroid‐treated and the non‐steroidtreated groups. Among the moderately proteinuric patients of group 1, the 20 steroid‐treated patients showed significantly favourable clinical courses compared to the 26 non‐steroid‐treated patients of the same group. the 12 heavily proteinuric, steroid‐treated patients of the group 1 also showed significantly favourable courses compared to those 26 moderately proteinuric, non‐steroid patients. However, all but one patient in group 2, (with and without steroid therapy) went into end‐stage renal failure within the 10 year follow‐up period. These results indicate that corticosteroid treatment has a long‐term beneficial effect for the stabilization of renal function in progressive IgA nephropathy with massive proteinuria ≤ 1.0 g/day, when patients have preserved renal function of 70 mL/min or more in initial Ccr values.

Avascular necrosis of bone after allogeneic bone marrow transplantation: clinical findings, incidence and risk factors.

In the present study we describe the incidence, clinical course, and management of avascular necrosis of bone following allogeneic bone marrow transplantation, and identify risk factors related to its development. All patients developing avascular necrosis of bone after allogeneic bone marrow transplantation between January 1974 and September 1992 were included in the analysis and were studied using the Hôpital Saint Louis Bone Marrow Transplant Database and hospital records. 27/727 allogeneic transplant recipients developed avascular necrosis leading to an 8.1% incidence at 5 years, by product limit estimate, ranging from 5% to 11.2%. Symptoms developed 119-1747 d (median 398 d) after transplantation. In these 27 patients a total of 52 joints were affected (mean 1.92 per patient, range 1-7). The hip joint was most often affected (69% of patients). All patients had joint pain that led to diagnosis by means of standard radiographs with or without the help of technetium-99 scans and/or magnetic resonance imaging. All but three patients received steroid therapy for acute graft-versus-host disease. Among 10 factors tested, three were shown to be significantly linked to an increased risk for developing avascular necrosis by multivariate analysis: male gender (relative risk (RR) 4.72, P = 0.002), age older than 16 (RR = 3.87, P = 0.004), and acute graft-versus-host disease requiring steroid therapy (RR = 6.30, P = 0.0002). 10 patients (37%) required joint replacement within 19 months (range 2-42) following diagnosis of avascular necrosis. In conclusion, avascular necrosis of bone is a frequent late complication of allogeneic bone marrow transplantation causing significant morbidity and requiring replacement surgery in one-third of affected patients. In this 18-year single-centre survey, older age, male gender and steroid therapy given for acute graft-versus-host disease were shown to independently increase the risk of avascular necrosis of bone.

Steroids, APACHE II Score, and the Outcome of Abdominal Infection

To compare the outcome of abdominal infection in patients with or without previous systemic glucocorticoid therapy and to determine the effect of steroid administration on the relationship between APACHE II (Acute Physiology and Chronic Health Evaluation) scores and mortality. Steroid therapy leads to greater mortality and relatively lower APACHE II scores. Prospective cohort study. University hospital. Two hundred ninety-seven consecutive adult patients with abdominal infection treated by surgical or percutaneous drainage. Treatment was at the clinician's discretion. Seventy-one patients received systemic steroid therapy. APACHE II score, clinical course, and death in hospital; relationship between APACHE II score and mortality in the steroid and no steroid groups. Thirty-three patients receiving steroid therapy (46%) died vs 55 (24%) of 226 patients not receiving steroid therapy. The APACHE II score (P < .0001) and steroid administration (P = .04) were each independently associated with death. Steroid-treated patients had the same probability of dying as "nonsteroid" patients with an APACHE II score a mean of 3.7 points higher (95% confidence limits, 0.03 and 7.7). The confidence that 2, 3, or 4 extra APACHE II points is the appropriate correction for steroid-treated patients is 80%, 60%, or 40%, respectively. Patients receiving steroid therapy appear to be at higher risk of dying of abdominal infection than predicted by APACHE II scores. The number of patients receiving cancer chemotherapy was too small to determine whether this was an additional risk factor. In the design of clinical trials stratified by APACHE II scores, steroid-treated patients should either be excluded or assigned two extra APACHE II points.

Plasma glycosaminoglycans in endocrine ophthalmopathy.

With evidence on the important role of glycosaminoglycans (GAG) in the pathogenesis of endocrine ophthalmopathy (EO) having accumulated, the present study focused on the biochemical assessment of plasma GAG content in 37 EO patients as compared to 20 controls. Glycosaminoglycans were isolated from plasma samples by protein elimination, dialysis, and precipitation with ethanol and cetylpyridinium chloride. Patients (9.71, 5.09, 15.09 mg/100 ml; median, 25th, 75th percentile) exhibited significantly (p = 0.0021) higher plasma GAG levels than controls (4.6, 3.38, 6.8 mg/100 ml). Plasma GAG content was unrelated to age, sex, or antithyroid treatment. However, an even higher level of significance (p = 0.0001) was reached when discriminating between untreated patients with EO of recent onset (14.16, 10.35, 15.51 mg/100 ml) and controls. By contrast, steroid therapy of EO led to values (3.82, 1.85, 6.52 mg/100 ml) indistinguishable from those of the controls. Further statistical analysis of the results, based on a specificity of 95% for the control group, revealed a sensitivity of 91% for patients with untreated EO of recent onset, and a specificity of 100% for patients receiving steroid therapy. In comparison, plasma GAG content was determined in 8 untreated and in 6 treated EO patients by a second method already published. All untreated patients exhibited high GAG levels (median 2.23 mg/100 ml) whereas in treated EO patients normal plasma GAG values (0.17-0.34 mg/100 ml) were found. Follow-up determination of plasma GAG content in 7 patients undergoing steroid treatment unveiled a marked decrease of initially elevated values. These findings correlated well with clinical improvement of thyroid eye disease.(ABSTRACT TRUNCATED AT 250 WORDS)

Steroid-related bilateral osteonecrosis of the patella

We present a case of bilateral osteonecrosis of the patella in a patient during steroid therapy and demonstrate the radiological, arthroscopic, and histological features. Osteonecrosis of the patella should be considered as a possible cause of knee pain in patients receiving steroid therapy.