We report a 14 year old Indian-Muslim girl who developed a fulminant, disseminated and fatal varicella infection while receiving steroids for nephrotic syndrome. The terminal phase of her illness was complicated by a bleeding dyscrasia and circulatory collapse. Varicella infection in healthy children is a benign disease. However in neonates and immunosuppressed patients it may be severe and often fatal. There are many reports of fatalities occurring in cancer patients receiving chemotherapy, patients on immunosuppressives for asthma, haemolytic anaemia, rheumatic fever, and renal and bone marrow transplantation. Patients with nephrotic syndrome receiving cyclophosphamide treatment are at particular risk of developing severe chickenpox infection. To our knowledge, there has been only one report of fatal chickenpox infection in a child who received steroids for nephrotic syndrome. We report here a case of fatal haemorrhagic chickenpox complicating nephrotic syndrome.
Two children with Juvenile Rheumatoid Arthritis (JRA) and severe growth suppression from corticosteroid therapy are described. Prolonged 'tailing-off' of steroids occurred during outpatients follow-up and this may be related to the high turnover of doctors involved. Suggestions for improving such follow-ups and caution against the continuous use of steroids are made.
Study site: Queen Elizabeth Hospital, Kota Kinabalu, Sabah, Malaysia
Strongyloides stercoralis is a widespread, soil-transmitted helminth affecting humans. Autoinfection occurs in S. stercoralis infection and this leads to a continuous build-up of worm burden in human host. This may lead to hyperinfection syndrome which has the potential to cause serious life-threatening disease especially in immunocompromised and immunosuppressed patients. Thus, patient with underlying risk factors should be suspicious of having this infection as severe strongyloidiasis carries a high mortality rate if the diagnosis is delayed. Here, we report a case of S. stercoralis hyperinfection in a diabetic patient.
The characteristics of three primary bullous diseases, pemphigus, pemphigoid, and dermatitis herpetiformis, seen in this country, probably reflecting this region, are discussed and compared to those reported in the literature in the West. One hundred and forty-eight patients with bullous diseases were seen over a period of 15 years. The criteria for confirmation of clinical diagnosis were the findings of the direct immunofluorescent test. Pemphigus vulgaris was the commonest bullous disease. The incidence of bullous pemphigoid was highest in the Indians of Malaysian origin, and they are also more likely to develop pemphigus vulgaris than any other ethnic group. Linear IgA type formed the entire group of dermatitis herpetiformis. The granular type was not seen at all. The patients were treated with prednisolone alone or together with methotrexate or azathioprine. Dapsone alone controlled the dermatitis herpetiformis. The known association between pemphigus and other diseases with immunologic disturbances was not found in this study. The natural history of the bullous disease as seen in the follow-up patients is described. Deaths in pemphigus and pemphigoid were more due to either complications of steroid therapy or unassociated diseases than the primary disease itself. Introduction of immunofluorescence as a diagnostic tool in pemphigus and extension of this facility to other bullous diseases has led to detailed characterization of these diseases as seen in the West; however, publications in English dealing with epidemiologic aspects of bullous diseases in this region are rare.(ABSTRACT TRUNCATED AT 250 WORDS)
AIMS: The aim was to examine relationships between total and unbound mycophenolic acid (MPA) and prednisolone exposure and clinical outcomes in patients with lupus nephritis.
METHODS: Six blood samples were drawn pre- and at 1, 2, 4, 6 and 8 h post-dose and total and unbound MPA and prednisolone pre-dose (C0 ), maximum concentration (Cmax ) and area under the concentration-time curve (AUC) were determined using non-compartmental analysis in 25 patients. The analyses evaluated drug exposures in relation to treatment response since starting MPA and drug-related adverse events.
RESULTS: Dose-normalized AUC varied 10-, 8-, 7- and 19-fold for total MPA, unbound MPA, total prednisolone and unbound prednisolone, respectively. Median values (95% CI) of total MPA AUC(0,8 h) (21.5 [15.0, 42.0] vs. 11.2 [4.8, 30.0] mg l(-1) h, P= 0.048) and Cmax (11.9 [6.7, 26.3] vs. 6.1 [1.6, 9.2] mg l(-1) , P = 0.016) were significantly higher in responders than non-responders. Anaemia was significantly associated with higher total (37.8 [14.1, 77.5] vs. 18.5 [11.7, 32.7] mg l(-1) h, P = 0.038) and unbound MPA AUC(0,12 h) (751 [214, 830] vs. 227 [151, 389] mg l(-1) h, P = 0.004). Unbound prednisolone AUC(0,24 h) was significantly higher in patients with Cushingoid appearance (unbound: 1372 [1242, 1774] vs. 846 [528, 1049] nmol l(-1) h, P = 0.019) than in those without. Poorer treatment response was observed in patients with lowest tertile exposure to both total MPA and prednisolone as compared with patients with middle and higher tertile exposure (17% vs. 74%, P = 0.023).
CONCLUSIONS: This study suggests a potential role for therapeutic drug monitoring in individualizing immunosuppressant therapy in patients with lupus nephritis.
KEYWORDS: lupus nephritis; mycophenolic acid; pharmacodynamics; pharmacokinetics; prednisolone; treatment outcome
Study site: Nephrology and SLE Clinics, Pusat Perubatan Universiti Kebangsaan Malaysia (PPUKM), Kuala Lumpur, Malaysia
OBJECTIVE:
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease and glucocorticoid is the mainstay of treatment in SLE. The reported incidence of steroid-induced diabetes mellitus (SDM) ranged between 1-53%. We sought to investigate the prevalence and associated factors of SDM in patients with SLE.
METHODOLOGY:
A total of 100 SLE patients attending the Nephrology/SLE and Rheumatology Clinic, Universiti Kebangsaan Malaysia Medical Centre (UKMMC) who received corticosteroid treatment were recruited. The diagnosis of diabetes mellitus was based on the 2010 American Diabetes Association's criteria. Prevalent cases of SDM were also included. Statistical analysis was performed to determine the factors associated with SDM.
RESULTS:
Thirteen of them (13%) developed SDM, with the median onset of diagnosis from commencement of glucocorticoid treatment being 8 years (range 0.5-21 years). Although only seven Indians were recruited into the study, three of them (42.9%) had SDM compared to Malays (9.3%) and Chinese (12.8%) (P ≤ 0.05). Univariate and multivariate analysis showed that higher numbers of system or organ involvement in SLE, abdominal obesity, hypertriglyceridemia and daily prednisolone of ≥ 1 mg/kg/day were the important associated factors of SDM (P ≤ 0.05). Meanwhile, hydroxychloroquine (HCQ) use was associated with reduced SDM prevalence (P < 0.05).
CONCLUSION:
The prevalence of SDM among SLE patients was 13% and Indians were more prone to develop SDM compared to other races. Higher numbers of system involvement, abdominal obesity, hypertriglyceridemia and the use of oral prednisolone of ≥ 1 mg/kg/day were associated with SDM, while HCQ use potentially protects against SDM.