Comparison of clinical presentation and outcome of childhood-onset and adulthood-onset of systemic lupus erythematosus among Indonesian patients.

INTRODUCTION: The clinical presentation of childhood-onset systemic lupus erythematosus (SLE) is generally perceived to differ from that of adult-onset SLE. OBJECTIVE: We aimed to compare the demographic and clinical manifestation between childhood-onset vs. adult-onset SLE in a cohort of Indonesian patients at tertiary care centers. METHODS: This retrospective study included patients in the Hasan Sadikin Lupus Registry from 2008 until December 2017. The demographics, clinical presentations, and outcomes were compared between childhood-onset SLE (<18 years old) (Group 1) and adult-onset SLE (≥18 years old) (Group 2). RESULTS: Eight hundred seventy patients were involved into this study. The proportion of childhood-onset SLE was 20% (174 patients). The mean age of group 1 versus group 2 was 13.56 ± 3.04 vs 30.41 ± 8.54 years. The following clinical manifestations at SLE diagnosis were significantly more common in childhood-onset than in adult-onset SLE patients: hematological disorder (p = 0.033) and arthritis (p = 0.006). While discoid rash (p = 0.036) and photosensitivity (p < 0.001) were significantly found higher in adult-onset SLE. Cyclophosphamide therapy was significantly more common to be used in childhood-onset (38.5% vs 21.0%, p = <0.001). However, frequency of mortality on follow-up tended to be higher in childhood-onset group (11.5% vs 7.0%, p = 0.208). CONCLUSION: Arthritis and hematologic involvements at SLE diagnosis were more prominent in childhood-onset compared to adult-onset patients, and mortality in childhood-onset SLE during follow-up relatively higher. This data may suggest the need for more aggressive management approach to childhood-onset patients with SLE.

Stunting as a Risk Factor for Asthma: The Role of Vitamin D, Leptin, IL-4, and CD23.

Stunting, which results from chronic malnutrition, is common in children from low- and middle-income countries. Several studies have reported an association between obesity and asthma. However, only a handful of studies have identified stunting as a significant risk factor for wheezing, a symptom of asthma, although the underlying mechanism remains unclear. This article aimed to review possible mechanisms underlying asthma in stunted children. Overall, changes in diet or nutritional status and deficiencies in certain nutrients, such as vitamin D, can increase the risk of developing asthma. Vitamin D deficiency can cause linear growth disorders such as stunting in children, with lower levels of 25(OH)D found in underweight and stunted children. Stunted children show a decreased lean body mass, which affects lung growth and function. Low leptin levels during undernutrition cause a Th1-Th2 imbalance toward Th2, resulting in increased interleukin (IL)-4 cytokine production and total immunoglobulin E (IgE). Studies in stunted underweight children have also found an increase in the proportion of the total number of B cells with low-affinity IgE receptors (CD23+). CD23+ plays an important role in allergen presentation that is facilitated by IgE to T cells and strongly activates allergen-specific T cells and the secretion of Th2-driving cytokines. Stunted children present with low vitamin D and leptin levels, impaired lung growth, decreased lung function, and increased IL-4 and CD23+ levels. All of these factors may be considered consequential in asthma in stunted children.

The Association between Vitamin D, Interleukin-4, and Interleukin-10 Levels and CD23+ Expression with Bronchial Asthma in Stunted Children.

Children with stunted growth have an increased risk of wheezing, and studies have shown that low levels of vitamin D and interleukin (IL)-10, along with increased IL-4 levels and CD23+ expression, are present in stunted and asthmatic children. To date, it is not known whether these factors are related to the incidence of asthma in stunted children. This case-control study investigated the association between vitamin D, IL-4, and IL-10 levels and CD23+ expression with bronchial asthma in stunted children. The study included 99 children aged 24-59 months, i.e., 37 stunted-sthmatic children (cases), 38 stunted children without asthma, and 24 non-stunted children with asthma. All children were tested for their 25(OH)D levels using chemiluminescent immunoassay (CLIA), IL-4 and IL-10 levels were measured through enzyme-linked immunosorbent assay (ELISA) testing, and CD23+ expression was measured through flow cytometry bead testing. The data were analyzed using chi-squared, Kruskal-Wallis, and Mann-Whitney tests. The results showed that stunted asthmatic children had a higher incidence of atopic family members than those without asthma. Additionally, stunted asthmatic children had a higher prevalence of vitamin D deficiency (48.6%) than the control group (44.7% and 20.8%). Furthermore, stunted asthmatic children had significantly lower levels of 25(OH)D [20.55 (16.18-25.55), p = 0.042] and higher levels of IL-4 [1.41 (0.95-2.40), p = 0.038], although there were no significant differences in IL-10 levels and CD23+ expression. The study concluded that low vitamin D and high IL-4 levels are associated with bronchial asthma in stunted children, while IL-10 and CD23+ do not show a significant association.

Growth Patterns of Indonesian Infants with Cow's Milk Allergy and Fed with Soy-Based Infant Formula.

PURPOSE: The use of soy-based infant formula has increased widely in infants with cow's milk allergy (CMA). This study aimed to provide evidence on the growth pattern of CMA infants fed with soy-based infant formula in an Indonesian setting. METHODS: A multi-site, intervention study was conducted among full-term and normal birth weight CMA infants. Within six months, the subjects were provided with a soy-based infant formula. Weight, height, and head circumference were measured at baseline, weeks 4, 8, 12, 16, 20, and 24. Adverse events were recorded by scoring atopic dermatitis and symptom-based clinical scores. RESULTS: Based on the World Health Organization growth chart, we found that most of subjects had normal nutritional status for weight-for-age, length-for-age, weight-for-length, and head-circumference-for-age. There were statistically significant differences between baseline and end-line for weight-for-age, length-for-age, weight-for-length, and head circumference-for-age nutritional status. No allergic symptoms or intolerance toward soy formula were observed at the end of the intervention period. CONCLUSION: These results show that infants fed with soy-based infant formula have a normal pattern of growth.

Delayed Diagnosis of Tuberculoma in a Child with Nephritis due to Systemic Lupus Erythematosus.

Tuberculoma is one of the manifestations of tuberculosis infection in the central nervous system. Even though its prevalence is only 1%, the mortality rate is high. Clinical presentation in immunocompromised patients with tuberculoma maybe different, thus making the diagnosis difficult. We present the case of a 13-year-old girl who was admitted for routine intravenous administration of cyclophosphamide and steroid therapy for nephritis due to systemic lupus erythematosus. She experienced severe headache and focal seizure on the second day of hospitalization. Neurology examination did not show any abnormalities. The Xpert MTB/RIF from the cerebrospinal fluid and sputum yielded negative results. Computed tomography scan and magnetic resonance imaging showed tuberculoma with caseous necrosisaround the fibrous capsule in the right occipital lobe of the brain. Electroencephalography showed no abnormalities. Clinical improvement was seen after 3weeks of treatment; however, antituberculosis drug-induced hepatotoxicity occurred.

Age of onset as a risk factor of renal involvement in Henoch-Schönlein purpura.

BACKGROUND: Henoch-Schönlein purpura (HSP) is the most common vasculitis in children, characterized by triad of symptoms; palpable purpura without thrombocytopenia, abdominal pain, and arthritis. Renal involvement often occur in children with HSP. No data on the renal involvement of children with HSP in Indonesia, especially West Java. OBJECTIVE: To evaluate renal involvement in children with HSP. METHODS: Retrospective study was conducted in children with HSP in Department of Child Health, Hasan Sadikin Hospital, from 2006 to 2011. Characteristics and clinical manifestations was reviewed from medical record. HSP was diagnosed by American College of Rheumatology 1990 criteria or European League Against Rheumatism/Pediatric Rheumatology International Trials Organization/Pediatric Rheumatology European Society 2008. RESULTS: There were 128 patients, consisting of 82 male (64.9%) and 46 female (35.1%) with ratio 1.8:1. Mean age was 7.9 ± 2.9 years old which range from 6 month to 15 years. Peak morbidity was between 5-10 years old. Prevalence of HSP in Hasan Sadikin Hospital tend to raise from 2.7/100,000 in 2008 to 5.2/100,000 in 2010. In most patients (71%) purpura was the first symptom. Seventy-one patients (44.5%) had arthritis and 89 patients (69.5%) had abdominal pain, while renal involvement was in 28 patients (21.8%). Gastrointestinal manifestations tend to manifest in patients less than 5 years old (p = 0.267), while renal involvement tend to manifest in age group 11-15 years old (p = 0.015) with odds ratio 3.1 (95% confidence interval, 1.2-8.1). CONCLUSION: Renal involvement in children with HSP is more common in age group 11 to 15 years old.

Vogt-Koyanagi-Harada disease in an 8-year-old boy.

Vogt-Koyanagi-Harada (VKH) disease is an autoimmune disease involving pigmented tissue in eyes, auditory system, skin, and central nervous system. The pathogenesis is a result of T cell lymphocyte reaction against melanocyte component, tyrosinase and tyrosinase-related protein. This disease uniquely affected pigmented race in Asia and native America, mostly women aged 20-50. We reported an 8-years-old boy complained for visual disturbance since 6 weeks prior admission. Two years earlier, the parents noted the patient eyes were looked red when photographed (suggesting a dilated pupil) preceded by whitened on the right forehead and nose and whitened hair, eyebrow and eyelashes. The examination showed a vitiligo and skin atrophy on right frontal and right nasal, poliosis on the eyebrows, eyelids and hair. The diameter of right pupil was 8 mm, with a paresis on 3rd, 4th, 6th, and 9th nerves. Fundus examination revealed sunset glow appearance. The visual acuity on the right eye: 2/60, left eye: 1-0. There's positive serology for antitoxoplasma IgG, anti Rubella IgG, anti CMV IgG. The patient was diagnosed with a VKH disease and then prescribed with methylprednisolone 1 mg/kg/day. The patient also had further follow up with dermatovenerologist and ophthalmologist. The eye examination revealed an improvement on right eye panuveitis, with a remaining keratic precipitate in the endothelium, and minimal flare and cell on the anterior chamber. ENT consultation revealed no ear inflammation or hearing disturbance.