Polymyxin B Hemoperfusion in Pediatric Septic Shock: Single-Center Observational Case Series.

Objectives: To evaluate the use of direct hemoperfusion with polymyxin B-immobilized fiber (PMX-DHP) as adjunctive therapy during pediatric patients with septic shock.

Design: Prospective observational study.

Setting: Nine-bed PICUs at university referral hospital.

Late sequelae of drug reaction with eosinophilia and systemic symptoms (DRESS) cause thyroid dysfunction and thyroiditis: review of literature.

Drug reaction with eosinophilia and systemic symptoms (DRESS) is one of the severe cutaneous adverse drug reactions (SCARs) with high mortality rate and variable long term sequelae, especially in thyroid dysfunction and thyroiditis. In this article, we review clinical course, culprit drugs, onset of diagnosis, and type of thyroid dysfunction in DRESS patients. There were a total of 51 cases including 12 children (aged less than 18 years old) and 39 adults from our review.

Low bone mineral density in Thai children with systemic lupus erythematosus: prevalence and risk factors.

Background: Improvement of disease recognition and management has increased the survival of children with systemic lupus erythematosus (SLE), but has shifted the morbidity focus toward long-term complications, such as low bone mass and osteoporosis. Studies in adults with SLE show older age, chronic inflammation, and corticosteroid therapy are risk factors for low bone mineral density (BMD) and osteoporosis.

Objectives: To determine the prevalence of and identify risk factors associated with low BMD in Thai children with SLE.

The clinical significance of antinuclear antibodies and specific autoantibodies in juvenile and adult systemic lupus erythematosus patients.

Background: Juvenile systemic lupus erythematosus (JSLE) and adult SLE (ASLE) patients present with different clinical manifestations, but it is unknown if there are differences in their antinuclear autoantibody (ANA) profiles or if staining patterns are associated with specific autoantibodies and clinical manifestations.

Objective: To determine whether distinct types and numbers of ANA-staining patterns are associated with specific autoantibodies and clinical manifestations in JSLE and ASLE patients.

Methods: A retrospective study was performed in Thai children (n = 146) and adults (n = 180) diagnosed with SLE using the Systemic Lupus International Collaborating Clinics classification criteria.

Skin signs in juvenile- and adult-onset systemic lupus erythematosus: clues to different systemic involvement.

Objective: We aim to explore the differences of skin signs between juvenile- and adult-onset systemic lupus erythematosus and to identify their associations to the development of systemic involvement.

Methods: A retrospective chart review of 377 systemic lupus erythematosus patients was performed.

Results: In total, 171 patients with juvenile systemic lupus erythematosus and 206 with adult systemic lupus erythematosus were studied.

Anti-neutrophil cytoplasmic antibodies and their clinical significance.

Anti-neutrophil cytoplasmic antibodies (ANCA) are a group of autoantibodies that cause systemic vascular inflammation by binding to target antigens of neutrophils. These autoantibodies can be found in serum from patients with systemic small-vessel vasculitis and they are considered as a biomarker for ANCA-associated vasculitis (AAV). A conventional screening test to detect ANCA in the serum is indirect immunofluorescence study, and subsequently confirmed by enzyme-linked immunosorbent assay.

Oral Ulcers in Juvenile-Onset Systemic Lupus Erythematosus: A Review of the Literature.

Oral ulcers are the most common mucosal sign in juvenile-onset systemic lupus erythematosus (JSLE). The ulcers are one of the key clinical features; however, the terminology of oral ulcers, especially in JSLE patients, is often vague and ill-defined. In fact, there are several clinical manifestations of oral ulcers in JSLE, and some lesions occur when the disease is active, indicating that early management of the disease should be started.

Early stage of vascular disease and diabetic kidney disease: an under-recognized entity.

Early stage of vascular disease and diabetic kidney disease (DKD stages 1 and 2) has been under-recognized, under common practice worldwide. The lack of sensitive diagnostic marker leads to late diagnosis and a progression of underlying vascular disease associated with chronic renal ischemia, which eventually intensifies the magnitude of DKD damage. Treatment at this late stage fails to correct the renal ischemia, or restore renal function, due to the altered vascular homeostasis associated with an impaired nitric oxide production.

The protective effect of GM-CSF on serum-induced neutrophil apoptosis in juvenile systemic lupus erythematosus patients.

Juvenile systemic lupus erythematosus (JSLE) is one of the most common autoimmune diseases in children and can affect multiple organs and systems. The etiology remains unclear, and current management only suppresses rather than eliminates the disease. The pathogenesis is triggered by autoantigens that induce autoantibody production.

Novel AQP2 mutation causing congenital nephrogenic diabetes insipidus: challenges in management during infancy.

Congenital nephrogenic diabetes insipidus (NDI) is a rare inherited disorder, mostly caused by AVPR2 mutations. Less than 10% of cases are due to mutations in the aquaporin-2 (AQP2) gene. Diagnosis and management of this condition remain challenging especially during infancy.

DcR3 mutations in patients with juvenile-onset systemic lupus erythematosus lead to enhanced lymphocyte proliferation.

Objective: Previous studies suggested a role for the death decoy receptor 3 (DcR3) in the pathogenesis of adult systemic lupus erythematosus (SLE). We investigated the role of DcR3 in juvenile-onset SLE, to identify polymorphisms that might alter the function of this protein.

Methods: DcR3 was measured in the serum of 61 patients with juvenile SLE.

Interleukin-10 promoter polymorphisms and expression in Thai children with juvenile systemic lupus erythematosus.

Interleukin (IL)-10 expression is regulated by its promoter and correlated with the activity of adult-onset lupus (systemic lupus erythematosus (SLE)). As the pathogenesis of adult-onset SLE may differ from SLE with the age at onset <18 years old (juvenile SLE or JSLE), we evaluated polymorphisms at positions -1082A/G, -819T/C and -592A/C of the IL-10 promoter and serum IL-10 levels in 71 patients with JSLE. Disease activity was determined by the SLE disease activity index (SLEDAI).

Prevention of bone loss in children receiving long-term glucocorticoids with calcium and alfacalcidol or menatetrenone.

Background: Long-term treatment with glucocorticoids can induce bone loss and increase fracture risks.

Aim: To compare the efficacy of a 12-month treatment between alfacalcidol and menatetrenone in preventing bone loss in children treated with long-term glucocorticoids.

Patients And Methods: Twenty children on a stable dosage of glucocorticoids were randomly divided into two groups (alfacalcidol or menatetrenone).

Two novel CTNS mutations in cystinosis patients in Thailand.

Cystinosis is an autosomal recessive disorder characterized by defective transport of cystine across the lysosomal membrane and resulting in renal, ophthalmic, and other organ abnormalities. Mutations in the CTNS gene cause a deficiency of the transport protein, cystinosin. We performed mutation analysis of CTNS in six cystinosis patients from four families in Thailand.

Enhanced peritubular capillary flow and renal function can be accomplished in normoalbuminuric type 2 diabetic nephropathy.

Under common practice, treatment of diabetic nephropathy (DN) is usually initiated at late stage of CKD due to the insensitiveness of the available diagnostic markers. Such treatment fails to restore renal perfusion and function. This is due to the defective mechanism of vascular homeostasis and impaired nitric oxide production observed in late stage of DN.

A biomarker for detecting early tubulointerstitial disease and ischemia in glomerulonephropathy.

Present diagnostic tests such as serum creatinine determination and creatinine clearance are unable to reflect early tubulointerstitial disease. Because a kidney biopsy cannot be performed in every single patient, tubular epithelial function (namely, the fractional excretion of magnesium; FE Mg) that correlates directly with the degree of tubulointerstitial fibrosis would serve this purpose. FE Mg is normal in acute post-streptococcal glomerulonephritis and mesangial proliferative nephrosis with intact tubulointerstitial structure, and is abnormally elevated in nephrosis with focal segmental glomerulosclerosis (FSGS) and in chronic kidney diseases in which kidney biopsies have been obtained.

Fractional excretion magnesium (FE Mg) in systemic lupus erythematosus.

Background: Tubulointerstitial fibrosis is an index of clinical severity. FE Mg has been delineated to correlate directly with the magnitude of tubulointerstitial fibrosis in clinical setting of glomerulonephropathy. A correlation between FE Mg tubulointerstitial fibrosis has never been assessed in nephritis associated with systemic lupus erythematosus.

Acute renal failure in a child with jellyfish contact dermatitis.

A 7-year-old boy suffered from jellyfish contact dermatitis and acute renal failure following a jellyfish sting. Three days before being admitted, he accidentally contacted a jellyfish on the left forearm, left thigh and trunk while wading at Pattaya beach, Eastern Thailand. Investigation revealed hemoglobinuria.

Mucocutaneous findings in pediatric AIDS related to degree of immunosuppression.

The normal value of the absolute CD4-positive T-lymphocyte count is relatively high in normal infants and declines steadily until 6 years of age, whereas the CD4 percentage of the total lymphocyte count is constant. The immunologic categories according to the 1994 revised pediatric human immunodeficiency virus (HIV) classification, based on CD4-positive percentage of the total lymphocyte count, is classified into three categories: no evidence of suppression (> or =25%), moderate suppression (15-24%), and severe suppression (1-14%). Our objective was to determine the prevalence of mucocutaneous findings in pediatric acquired immunodeficiency syndrome (AIDS) related to the degree of immunosuppression.