scholarly journals Clinical and laboratory features of childhood systemic lupus erythematosus and its course in Department of Child Health, Cipto Mangunkusumo Hospital, Jakarta

2006 ◽  
Vol 41 (4) ◽  
pp. 214
Author(s):  
Zakiudin Munasir ◽  
Tuty Mariana ◽  
Rulina Suradi

A descriptive, retrospective study has been performed among 33 children with SLE during the period of January 1986 to December 1999 in Department of Child Health, Cipto Mangunkusumo Hospital. The results of the present study show that SLE in childhood was more commonly found in girls than in boys, with a ratio of 4.5: 1. The mean age of onset was 10.5 years and the mean age of diagnosis was 10.9 years, most frequently observed in the age of > 10 years and rarely found in the age of < 5 years. The time interval between the time of onset and time of diagnosis ranged from 1 to 24 months and the most frequent interval was 1-3 months. The most common initial symptoms were prolonged fever, rash on the skin and face, and athralgia. In its natural history of the disease, kidneys, skin/mucous membrane and joints are the most frequent organs involved. Most of the SLE patients develop anemia. Positive anti ds-DNA, ANA and decreased levels of C3 and C4 respectively in 28 (28/31), in 29 (29/30), in 25 (25/33), and in 19 (19/27) cases. LE cells were encountered only in four (4/17) cases.  Out of 11 cases upon which renal biopsies were done, the most common histological features were mesangeal glomerulonephritis (class II) and diffuse proliferative glomerulonephritis (class IV). The mean time interval between the onset and renal complication manifestations was 6.96 months, cardiac complication was 16.77 months, central nerve system was 22.71 months and lungs were 25.0 months. Duration of illness of patients with SLE ranged from 2 to 175 with the mean of 31.3 months. The causes of death were mostly due to gastrointestinal bleeding and renal failure.

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Nath Pasutharnchat ◽  
Chamaiporn Taychargumpoo ◽  
Yongkasem Vorasettakarnkij ◽  
Jakkrit Amornvit

Abstract Background Ala97Ser transthyretin amyloidosis-associated polyneuropathy (ATTRA97S-PN) is a rare form of inherited polyneuropathy, usually manifesting with late-onset (> 50) progressive polyneuropathy. This mutation is mostly prevalent in Taiwanese and Han-Chinese individuals. The aim of this study was to describe the clinical and comprehensive neurophysiological profiles of ATTRA97S-PN in Thai patients. Methods The clinical profiles and serial neurophysiologic studies (nerve conduction study (NCS), quantitative sensory test (QST), and comprehensive autonomic function test (AFT)) of symptomatic ATTRA97S-PN patients who had been followed-up at King Chulalongkorn Memorial Hospital during 2010–2020 were retrospectively reviewed. Results Nine symptomatic patients (55.6 % were male) from four unrelated families were included. All were Thais of mixed Thai Chinese descent. The mean age of onset was 48.3 (32–60) years. The mean age at diagnosis was 54.8 (33–66) years. Three patients developed early-onset (< 40y) polyneuropathy. The mean Neuropathy Impairment Score was 41.33 (10–92) at diagnosis. Sensory (9/9) and autonomic (9/9) neuropathies were more frequent than motor neuropathy (5/9), which appeared in the late stage of disease. Hypoesthesia in the feet, and gastrointestinal autonomic symptoms were frequently reported as the initial symptoms. The course of neuropathy progressed over years to decades. The worsening of neuropathy tended to progress faster once motor nerves were affected in both clinical and neurophysiological aspects. Concurrent cardiac amyloidosis was found in 6/9 patients. NCS showed length-dependent sensorimotor axonal polyneuropathy in 5/9 patients, and median neuropathy at the wrist (mostly bilateral) in 7/9 patients. QST showed abnormalities in the vibratory detection threshold, the cold detection threshold and the heat pain sensation in 8/9, 8/9 and 7/7 tested patients, respectively. AFT results were abnormal in all. The mean composite autonomic severity score was 5 (3–9). Conclusions This clinical study is the first of ATTRA97S-PN in Thai patients. The mixed polyneuropathy-cardiopathy phenotype was the most common manifestation. In this cohort, the age of onset was lower, and the course of neuropathy was relatively longer, than that in previous studies. Some patients may develop early-onset polyneuropathy. This mutation has not yet been documented in any population other than Han Chinese-related populations, probably suggesting a founder effect. Further studies are warranted.


Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
Kamelia Okka ◽  
M Belghazi ◽  
A Dehimi ◽  
Z Benarab ◽  
S Bouabdallah ◽  
...  

Abstract Background Juvenile systemic lupus erythematosus (J-SLE) is a chronic autoimmune disease characterized by multi-visceral involvement with an unpredictable prognosis. The diagnosis is usually made in young women between the ages of 20 and 40, however, it can set in at any age and will be classified as juvenile (LESj) when it begins before the age of 16.We report the epidemiological, clinical, therapeutic and evolutionary characteristics of a retrospective series carried out at the level of the pediatric center—CHU de Sétif comprising 13 girls and one boy. Methods The mean age of onset is 12 years and 3 months, the mean time to diagnosis is 7 months. The clinical picture is made of e reached articulaire skin and e fever in 86% respectively 57% and 57% of cases, followed by kidney disease in 57% of cases. Cardiac involvement pulmon area ophtalmologiqu e is referred to in low percentages. The blood reached logic of étectée on blood counts in 85% of patient e s i and the syndrome nflammatoire was almost constant. A positive titer of antinuclear antibodies and anti- AD Nn is objectified, as well as a reduction in the level of complement. The anti-GP 2 and anti- cardio lopine antibodies are positive in 57% of cases. Has the present hue kidney in 42% of cases. A single case of overlap syndrome with dermatomyositis has been reported. As for the neurological form, only one adolescent presented it. With a single case of familial lupus and a single case of Rhupus. Results The diagnosis is based on the American College of Rheumatology (ACR) 1982 classification revised in 1997 and the new SLICC “Systemic Lupus International Collaborating Clinics” criteria. The clinical characteristics of our series are consistent with the overall data in the literature with a predominance of cutaneous and joint involvement. with however some specific characteristics which are individualized by a more advanced age of onset, of 13 years on average in our study vs 10 years and 12 years, the rarity of the familial forms (1 case), a lower percentage of renal damage (42% vs 63% and 80%). The therapeutic management was based on corticosteroids and Hydroxychloroquine in most cases, the use of immunosuppressants was reserved for x severe. Conclusion Lupus is an autoimmune disease with protean clinical manifestations, the prognosis of which is dominated by renal, neurological and thrombotic damage. Corticosteroid treatments and immunosuppressants markedly improved the vital prognosis.


2019 ◽  
Vol 5 (2) ◽  
pp. 97-100
Author(s):  
Mohammad Enayet Hussain ◽  
AFM Al Masum Khan ◽  
Md Abdullah Yusuf ◽  
Md Ferdous Mian ◽  
Md Nahidul Islam ◽  
...  

Background: SSPE is a late complication of measles and is fatal in most cases. Objectives: The purpose of the study was to observe the clinico-demographic, investigation and outcome profiles of SSPE patients. Methodology: This prospective cohort study was conducted in the Department of Neurophysiology at National Institute of Neurosciences and Hospital, Dhaka, Bangladesh from September 2014 to August 2017. Patients of SSPE who were diagnosed by Dyken's Criteria were selected as study population. Details socio-demographic, clinical profiles, investigations and outcome were recorded. The outcome was assessed from the time of first onset of the symptoms till closing of the study. Results: The mean age of the study population was 14.3±4.209 years with the male predominance (73.5%). Majority were from rural area (70.6%) and were of low socio-economic condition (52.9%). The mean age of onset of disease was 13.6±4.30 years. History of previous measles infection was reported in 13(38.2%) cases among which 12(92.3%) cases were vaccinated. The mean age of measles infection was 17.0±15.44 months. The time of first symptom of SSPE from primary measles infection was 11.50±4.80 years. Recurrent fall (47.1%) and myoclonic jerks (17.6%) were the most frequent initial symptoms of the patient. Cognitive decline was observed in 28(82.4%) cases. EEG background was slow in 23(67.6%) patients and periodic burst was observed in all (100.0%) cases. Anti-measles antibody in CSF was positive in all cases. MRI was abnormal in 7(25.9%) cases out of 27 cases. Twelve (35.3%) patients died, 18(52.9%) were alive and 4(11.8%) were lost to follow up when the study was closed. Conclusion: Early aged male from lower socioeconomic condition are found to be suffering from SSPE. Recurrent fall is the commonest initial presentation. Spontaneous recovery can occur in very few patients. Journal of National Institute of Neurosciences Bangladesh, 2019;5(2): 97-100


2019 ◽  
Author(s):  
changhao xie ◽  
li li zhang ◽  
yuan yuan wang ◽  
zhi jun li ◽  
lin jie chen ◽  
...  

Abstract Bankground: The objective of this paper was to describe the first symptoms associated with systemic lupus erythematosus (SLE), including clinical manifestations, laboratory findings, prognoses, differences between men and women at the time of first diagnosis of SLE. Methods : We enrolled 223 patients with initial diagnosis of SLE. Their initial symptoms, demographic, clinical and laboratory data,prognoses and causes of death were analyzed retrospectively. Clinical manifestations and laboratory profiles were compared between male and female patients. Results: Compared with female patients, male patients had an earlier age of onset, a higher incidence of neuropsychiatric involvements, a lower incidence of leukocytopenia , and a higher score of SLE Disease Activity Index (SLEDAI)at diagnosis. Fever and malar rash were most frequent presentations at onset of SLE. The most common clinical manifestation at first diagnosis was fever, followed by arthralgia, malar rash, Raynaud ’ s phenomenon, arthritis. The liver function abnormalities included increased ALT,AST,ALP and γ-GGT.ANA were found in 100% of patients, followed by anti-dsDNA(LIA) in 72.1%, anti-Ro60 in 67.8%, anti-Ro52 in 62.3%, anti-nucleosomes in 55.7%. Conclusions: We identified clinical and serological manifestations of Chinese SLE patients at first diagnosis. Male patients showed a distinctive manifestation including younger age of onset,a higher incidence of CNS manifestations, a higher score of SLEDAI compared to females.


Author(s):  
J. Santoantonio ◽  
L. Yazigi ◽  
E. I. Sato

The purpose of this study was to investigate the personality characteristics in adolescents with SLE. The research design is a case-control study by means of the Rorschach Method and the Wechsler Intelligence Scale. Study group: 30 female adolescents with lupus, 12–17 years of age. The SLE Disease Activity Index was administered during the period of psychological evaluation. Control group: 32 nonpatient adolescents were matched for age, sex, and socioeconomic level. In the Wechsler Intelligence Scale the mean IQ of the experimental group was significantly lower than that of the control group (77 and 98, respectively, p < .001). In the Rorschach, the lupus patients showed greater difficulty in interpersonal interactions, although they displayed the resources to process affect and to cope with stressful situations. A positive moderate correlation (p = .069) between the activity index of the disease and the affect constriction proportion of the Rorschach was observed: the higher the SLEDAI score, the lower the capacity to process affect. There is a negative correlation between the activity index of the disease and the IQ (p = .001): with a higher activity index of the disease, less intellectual resources are available.


2013 ◽  
pp. 50-56
Author(s):  
Quoc Tinh Nguyen ◽  
Thi Cu Nguyen

Background: Diarrhea is a leading cause of morbidity and mortality in children in developing countries. Rotavirus is the most important factor cause of severe diarrhea and mortality in children, especially under 2 years. Objective: To study Clinical and paraclinical characteristitics of rotavirus diarrhea in children less than 5 years old in the Pediatric Department of Binh Dinh General Hospital. Methods: Cross observational study, 417 children from 2 months to 5 years old who were hospitalized for acute diarrhea without blood in stool in Pediatric Department of Binh Dinh general hospital, from 15/3/2012 to 14/3/2013. There were 228 patients with rotavirus - positive stool specimens and 189 patients with rotavirus - negative stool specimens. Tested for rotavirus A classification by agglutination techniques. Results: Percentage of Rotavirus diarrhea in children <5 years was 54.7%. The mean age of rotavirus diarrhea was 14.83 ± 9.08 tháng months. Ages with the highest cases of rotavirus diarrhea are 2 - 12 months: 48.2%. Rotavirrus diarrhea is experienced in every month but, the highest prevalence is experienced in the spring - summer) with 71,5%. Clinical characteristitics of rotavirus diarrhea: Vomit presents in 91,7% of cases. 32.9% of the initial symptoms of the disease is vomiting. Mean number of vomiting episodes per day: 7 ± 3.57 times, significantly higher compared with non-rotavirus acute diarrhea (p <0,01). Mean number of bowel movement per day: 10.61±4.18 times, mean duration of diarrhea: 6.66 ± 2.52 days. Younger children have higher frequency and duration of diarrhea compared to older children. Paraclinical characteristitics of rotavirus diarrhea: the mean number leukocyte: 9.74 ±3.61 (x 109/l). 2.2% with low blood sodium status; 8.8% with reduction in serum potassium concentration. 26.3% with low blood calcium and glucose status. Laboratory characteristics of stool: 8.8% with white blood cells in stool, 41.7% with carbohydrate in stool. Conclusion: Rotavirus accounting for 54.7% of the causes of diarrhea in children. Rotavirus diarrhea is experienced throughout the year but the highest prevalence is in the spring-summer. Prominent symptoms of the disease are vomiting and watery stool. Children <24 months experience higher frequency and duration of diarrhea compared to older age groups. Key words: Diarrhea, Rotavirus.


2020 ◽  
Vol 16 (2) ◽  
pp. 125-133
Author(s):  
Zahra Rezaieyazdi ◽  
Sima Sedighi ◽  
Masoumeh Salari ◽  
Mohammadreza H. Fard ◽  
Mahmoud R. Azarpazhooh ◽  
...  

Background: The relationship between SLE and traditional risk factors for cardiovascular events was evaluated. Methods: The data regarding sixty patients with SLE and 30 healthy controls (age and sex matched) were gathered using SLEDAI forms. Venous blood (10mL) from all the participants was examined for hs-CRP, homocysteine, VCAM1, CBC, anti-DNA antibody, C3, C4, low-density lipoprotein (LDL), cholesterol, FBS and triglyceride. : The IMT of carotid arteries was determined bilaterally by ultrasound. Other measurements included insulin levels via Elisa (Linco/Millipore Corp) and the HOMA-IR index for insulin resistance. Results: The mean age (in years) in the test and control groups was 28.8±10.3 (18-52) and 33.8±9.13 (18-48), respectively. Results: The mean age (in years) in the test and control groups was 28.8±10.3 (18-52) and 33.8±9.13 (18-48), respectively. : The average IMT in the test group was directly related to serum levels of VCAM1 (p<0.001), homocysteine (p<0.001), cholesterol (p<0.009), LDL (p<0.001), TG (p<0.001), and FPG (p=0.004). The association between other risk factors, insulin resistance, carotid IMT and SLEDAI, was nonexistent. Mean insulin and insulin resistance levels in all the participants were 0.43±2.06 µU/mL and 0.09±0.44, respectively. There was no significant difference between the test and control groups regarding serum insulin and insulin resistance levels (p=0.42 and p=0.9, respectively). None of the risk factors, such as hsCRP, VCAM1, or homocysteine, were shown to be related to insulin resistance (p=0.6, p=0.6, p=0.09, respectively). Conclusion:: Our findings did not show an increase in the prevalence of atherosclerosis in patients with SLE. There was no association between IMT and insulin resistance. However, the former was associated with FPG, total cholesterol, LDL, TG, homocystein and VCAM1.


2019 ◽  
Vol 15 (02) ◽  
pp. 14-17
Author(s):  
K K Hadiya ◽  
A J Dhami ◽  
D V Chaudhari ◽  
P M Lunagariya

This study was initiated on 24 prepubertal Holstein x Kankrej crossbred heifers of nearly identical age (7-9 months) and body weight (130-140 kg) at University farm to evaluate the effect of high plane of nutrition on blood biochemical and minerals profile and the age at puberty. Twelve heifers were managed under routine farm feeding (control) and the rest 12 under ideal optimum feeding regime (treatment) that included extra 1 kg concentrate, 30 g min mix and ad-lib dry fodder. The body weight and ovarian ultrasonography together with blood sampling was carried out at monthly interval from 10 to 18 months of age to study the ovarian dynamics and blood biochemical changes. High plane of nutrition to growing heifers was beneficial in reducing the age of onset of puberty (by 2-3 months) compared to routine farm fed group. The mean plasma total protein and cholesterol concentrations showed a rising trend with significant variations from 10 to 16 months of age, where it got mostly stabilized indicating adult profile. The activity of enzymes GOT and GPT also rose gradually and significantly from 10 months till 14-15 months of age, and thereafter it remained more or less static till 18 months of age. The levels of both these enzymes were higher, with lower protein and cholesterol, in control than the treatment group from 15-16 months of age onwards. The mean plasma levels of both calcium and phosphorus increased gradually and significantly with advancing age till 16-17 months of age, with little higher values in supplemented than a control group. The plasma levels of zinc, iron, copper, and cobalt also showed rising trend with significant differences between 10th and 12th-14th months of age, and from 15th to 18th months of age the levels were statistically the same in all the groups with slightly higher values in the treatment group.


Lupus ◽  
2021 ◽  
pp. 096120332110345
Author(s):  
Stefan Vordenbäumen ◽  
Alexander Sokolowski ◽  
Anna Rosenbaum ◽  
Claudia Gebhard ◽  
Johanna Raithel ◽  
...  

Objective Hypomethylation of CD40-ligand (CD40L) in T-cells is associated with increased disease activity in systemic lupus erythematosus (SLE). We therefore investigated possible associations of dietary methyl donors and products with CD40L methylation status in SLE. Methods Food frequency questionnaires were employed to calculate methyl donor micronutrients in 61 female SLE patients (age 45.7 ± 12.0 years, disease duration 16.2 ± 8.4 years) and compared to methylation levels of previously identified key DNA methylation sites (CpG17 and CpG22) within CD40L promotor of T-cells using quantitative DNA methylation analysis on the EpiTYPER mass spectrometry platform. Disease activity was assessed by SLE Disease Activity Index (SLEDAI). Linear regression modelling was used. P values were adjusted according to Benjamini & Hochberg. Results Amongst the micronutrients assessed (g per day), methionine and cysteine were associated with methylation of CpG17 (β = 5.0 (95%CI: 0.6-9.4), p = 0.04; and β = 2.4 (0.6-4.1), p = 0.02, respectively). Methionine, choline, and cysteine were additionally associated with the mean methylation of the entire CD40L (β = 9.5 (1.0-18.0), p = 0.04; β = 1.6 (0.4-3.0), p = 0.04; and β = 4.3 (0.9-7.7), p = 0.02, respectively). Associations of the SLEDAI with hypomethylation were confirmed for CpG17 (β=-32.6 (-60.6 to -4.6), p = 0.04) and CpG22 (β=-38.3 (-61.2 to -15.4), p = 0.004), but not the mean methylation of CD40L. Dietary products with the highest impact on methylation included meat, ice cream, white bread, and cooked potatoes. Conclusions Dietary methyl donors may influence DNA methylation levels and thereby disease activity in SLE.


Lupus ◽  
2021 ◽  
pp. 096120332110142
Author(s):  
Tamer A Gheita ◽  
Rasha Abdel Noor ◽  
Esam Abualfadl ◽  
Osama S Abousehly ◽  
Iman I El-Gazzar ◽  
...  

Objective The aim of this study was to present the epidemiology, clinical manifestations and treatment pattern of systemic lupus erythematosus (SLE) in Egyptian patients over the country and compare the findings to large cohorts worldwide. Objectives were extended to focus on the age at onset and gender driven influence on the disease characteristics. Patients and method This population-based, multicenter, cross-sectional study included 3661 adult SLE patients from Egyptian rheumatology departments across the nation. Demographic, clinical, and therapeutic data were assessed for all patients. Results The study included 3661 patients; 3296 females and 365 males (9.03:1) and the median age was 30 years (17–79 years), disease duration 4 years (0–75 years) while the median age at disease onset was 25 years (4–75 years). The overall estimated prevalence of adult SLE in Egypt was 6.1/100,000 population (1.2/100,000 males and 11.3/100,000 females).There were 316 (8.6%) juvenile-onset (Jo-SLE) and 3345 adult-onset (Ao-SLE). Age at onset was highest in South and lowest in Cairo (p < 0.0001). Conclusion SLE in Egypt had a wide variety of clinical and immunological manifestations, with some similarities with that in other nations and differences within the same country. The clinical characteristics, autoantibodies and comorbidities are comparable between Ao-SLE and Jo-SLE. The frequency of various clinical and immunological manifestations varied between gender. Additional studies are needed to determine the underlying factors contributing to gender and age of onset differences.


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