Decrease of left ventricular function measured by speckle-tracking echocardiography based on systemic lupus erythematosus severity in children.

INTRODUCTION: Systemic lupus erythematosus in children generally manifests more severely with a more aggressive disease course. Cardiac involvement in systemic lupus erythematosus often does not show specific signs and symptoms, but speckle-tracking echocardiography can detect cardiac dysfunction. This study aimed to determine the differences in left ventricular function as measured by speckle-tracking echocardiography in children with various severity of systemic lupus erythematosus activity. METHODS: A cross-sectional study of 49 children diagnosed with systemic lupus erythematosus are currently undergoing outpatient or inpatient care at Dr Hasan Sadikin General Hospital, Bandung, from May 2023 to June 2023. Disease activity was assessed by Mexican Version of the Systemic Lupus Erythematosus Disease Activity Index (MEX-SLEDAI) with a score of 2-5 classified as mild activity, 6-9 as moderate, and ≥10 as severe. Each subject underwent conventional echocardiography and speckle-tracking echocardiography with a Philips EPIQ machine performed by a Pediatric Cardiologist Consultant 10 days after inclusion. RESULTS: Fifteen (30.6%) subjects had mild disease activity, and 34 (69.4%) subjects had moderate disease activity. Most subjects (81.96%) were female with an average age of 15 years. The mean ejection fraction and fractional shortening as well as the median E/A ratio in the mild and moderate disease activity groups were not significantly different (65.76 versus 67.38%, 35.73 versus 37.11%, 1.6 versus 1.5%, respectively, p > 0.005). The global longitudinal strain in the moderate activity group was reduced more significantly than in the mild activity group (-16.58 versus -19.65, p = 0.008). CONCLUSION: Left ventricular function as measured by speckle-tracking echocardiography was lower in children with moderate systemic lupus erythematosus activity than those with mild disease activity.

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.

Optimizing ultraviolet B radiation exposure to prevent vitamin D deficiency among pregnant women in the tropical zone: report from cohort study on vitamin D status and its impact during pregnancy in Indonesia.

BACKGROUND: Vitamin D deficiency during pregnancy carries potential threat to fetal well being. Natural conversion of vitamin D in the skin can be facilitated by direct ultra violet B (UVB) radiation, but the effect is reduced by wearing umbrellas, clothes, or sunblock cream. Muslim women wear hijab that allows only face and hands to be seen. With increasing proportion of muslim women wearing hijab and the lack of vitamin D fortification and fish consumption in Indonesia, it poses a problem for vitamin D deficiency among pregnant women. This study aimed at finding the best timing of UVB exposure and the duration of exposure which can be suggested to prevent vitamin D deficiency among pregnant women, for those wearing hijab or not. METHODS: This study recruited 304 pregnant women in the first trimester, 75-76 women from 4 cities of the most populated province, West Java, Indonesia which represented 70-80% percent of pregnancy per year. A 3-day notes on duration, time and type of outdoor activity and the clothing wore by the women were collected. UVB intensity radiation were obtained. Calculation on body surface area exposed to direct UVB radiation and UVB radiation intensity were done. Measurement of vitamin D level in sera were done on the same week. RESULTS: The median of maternal sera vitamin D level was 13.6 ng/mL and the mean exposed area was around 0.48 m2 or 18.59% of total body surface area. Radiation intensity reached its peak around 10.00 and 13.00, but the mean duration of exposure to UVB during this window was lower than expected. Significant correlation was found between maternal sera vitamin D level and exposed body surface area (r = 0.36, p < 0.002) or percentage of exposed body surface (r = 0.39, p < 0.001) and radiation intensity (r = 0.15, p = 0.029). Further analysis showed that duration of exposure to UVB should be longer for pregnant women wearing hijab as compared to women without hijab. CONCLUSION: This study suggested that the best timing to get UVB exposure was between 10.00-13.00, with longer duration for women wearing hijab (64.5 vs 37.5 min) of continuous exposure per day.

Association of Colecalciferol, Ferritin, and Anemia among Pregnant Women: Result from Cohort Study on Vitamin D Status and Its Impact during Pregnancy and Childhood in Indonesia.

Studies had shown that iron-cycling was disturbed by inflammatory process through the role of hepcidin. Pregnancy is characterized by shifts of interleukin. Our objective was to determine if 25(OH) vitamin D (colecalciferol) status was associated with ferritin, anemia, and its changes during pregnancy. Method. A cohort study was done in 4 cities in West Java, Indonesia, beginning in July 2016. Subjects were followed up until third trimester. Examinations included were maternal ferritin, colecalciferol, and haemoglobin level. Result. 191 (95.5%) subjects had low colecalciferol, and 151 (75.5%) among them were at deficient state. Anemia is found in 15 (7.5%) subjects, much lower than previous report. Proportion of anemia increased by trimester among women with colecalciferol deficiency. Ferritin status and prepregnancy body mass index in the first trimester were correlated with anemia (r = 0.147, p = 0.038 and r = -0.56, p = 0.03). Anemia in the second trimester was strongly correlated with anemia in the third trimester (r = 0.676, p < 0.01). Conclusion. Our study showed that the state of colecalciferol was not associated with either ferritin state or anemia, but proportion of anemia tends to increase by trimester in the colecalciferol deficient subjects.

Fcγ receptor III expression and morphological maturity on neutrophil are associated with higher iron level of major beta-thalassemia.

Lifetime blood transfusion experienced by major ß-thalassemia patients complicated with iron overload, therefore, may lead to their tissue injury. Ultimately, free toxic iron may alter immune response via dysregulation of immune cell activity producing prolonged effector reaction. Neutrophil as one of the vital innate immune cell despite serves as the first line of defense resulting acute inflammation has a pivotal role in chronic inflammation while releasing the toxic substance that interferes biological processes. This process is initiated by one of them by activation of Fcγ Receptor III (CD16), a neutrophil membrane-bound protein. A cross-sectional laboratory study involving lysed-erythrocyte heparinized whole blood of fifty pediatric major ß-thalassemia patients treated with monoclonal antibodies i.e. CD16, CD14, and HLA-DR, dissected into CD16+ and CD16++ population using flow cytometry. Expression of Fcγ Receptor III was measured as Median Fluorescent Intensity (MFI). Hematology and iron status were measured. A correlation analysis was done. MFI of CD16 neutrophil [509.5 (371 - 796.5)] and ferritin level [(3209 µg/L, 1862 - 4564)] was positively correlated (r = 0.4, P = 0.007). Respectively, ferritin and serum iron were found negatively correlated with segmented neutrophils (r = -0.3, P = 0.02; r = -0.3, P = 0.02). Change in CD16 expression may implicate preliminarily neutrophil activation as a response of iron-overloaded tissue and result in chronic inflammation in ß-thalassemia patients. However, the maturity of this cell may be altered.  Future study in the understanding of neutrophil-mediated inflammation, particularly related to immune complexes and functionality, is imperative to be explored.

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.