Displaying publications 61 - 80 of 90 in total

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  1. Ab Rahman N, Lim MT, Lee FY, Lee SC, Ramli A, Saharudin SN, et al.
    Vaccine, 2022 Jul 30;40(32):4394-4402.
    PMID: 35667917 DOI: 10.1016/j.vaccine.2022.05.075
    BACKGROUND: Rapid deployment of COVID-19 vaccines is challenging for safety surveillance, especially on adverse events of special interest (AESIs) that were not identified during the pre-licensure studies. This study evaluated the risk of hospitalisations for predefined diagnoses among the vaccinated population in Malaysia.

    METHODS: Hospital admissions for selected diagnoses between 1 February 2021 and 30 September 2021 were linked to the national COVID-19 immunisation register. We conducted self-controlled case-series study by identifying individuals who received COVID-19 vaccine and diagnosis of thrombocytopenia, venous thromboembolism, myocardial infarction, myocarditis/pericarditis, arrhythmia, stroke, Bell's Palsy, and convulsion/seizure. The incidence of events was assessed in risk period of 21 days postvaccination relative to the control period. We used conditional Poisson regression to calculate the incidence rate ratio (IRR) and 95% confidence interval (CI) with adjustment for calendar period.

    RESULTS: There was no increase in the risk for myocarditis/pericarditis, Bell's Palsy, stroke, and myocardial infarction in the 21 days following either dose of BNT162b2, CoronaVac, and ChAdOx1 vaccines. A small increased risk of venous thromboembolism (IRR 1.24; 95% CI 1.02, 1.49), arrhythmia (IRR 1.16, 95% CI 1.07, 1.26), and convulsion/seizure (IRR 1.26; 95% CI 1.07, 1.48) was observed among BNT162b2 recipients. No association between CoronaVac vaccine was found with all events except arrhythmia (IRR 1.15; 95% CI 1.01, 1.30). ChAdOx1 vaccine was associated with an increased risk of thrombocytopenia (IRR 2.67; 95% CI 1.21, 5.89) and venous thromboembolism (IRR 2.22; 95% CI 1.17, 4.21).

    CONCLUSION: This study shows acceptable safety profiles of COVID-19 vaccines among recipients of BNT162b2, CoronaVac, and ChAdOx1 vaccines. This information can be used together with effectiveness data for risk-benefit analysis of the vaccination program. Further surveillance with more data is required to assess AESIs following COVID-19 vaccination in short- and long-term.

    Matched MeSH terms: Thrombocytopenia/chemically induced; Thrombocytopenia/epidemiology
  2. Saheb Sharif-Askari F, Syed Sulaiman SA, Saheb Sharif-Askari N
    Adv Exp Med Biol, 2017;906:101-114.
    PMID: 27628006
    Patients with chronic kidney disease (CKD) are at increased risk for both thrombotic events and bleeding. The early stages of CKD are mainly associated with prothrombotic tendency, whereas in its more advanced stages, beside the prothrombotic state, platelets can become dysfunctional due to uremic-related toxin exposure leading to an increased bleeding tendency. Patients with CKD usually require anticoagulation therapy for treatment or prevention of thromboembolic diseases. However, this benefit could easily be offset by the risk of anticoagulant-induced bleeding. Treatment of patients with CKD should be based on evidence from randomized clinical trials, but usually CKD patients are excluded from these trials. In the past, unfractionated heparins were the anticoagulant of choice for patients with CKD because of its independence of kidney elimination. However, currently low-molecular-weight heparins have largely replaced the use of unfractionated heparins owing to fewer incidences of heparin-induced thrombocytopenia and bleeding. We undertook this review in order to explain the practical considerations for the management of anticoagulation in these high risk population.
    Matched MeSH terms: Thrombocytopenia/complications; Thrombocytopenia/pathology; Thrombocytopenia/prevention & control
  3. Hasliza AH, Tohid H, Loh KY, Santhi P
    Malays Fam Physician, 2015;10(2):49-51.
    PMID: 27099661 MyJurnal
    Dengue infection is highly endemic in many tropical countries including Malaysia. However, neurological complications arising from dengue infection is not common; Gullain-Barre syndrome (GBS) is one of these infrequent complications. In this paper, we have reported a case in which a 39-year-old woman presented with a neurological complication of dengue infection without typical symptoms and signs of dengue fever. She had a history of acute gastroenteritis (AGE) followed by an upper respiratory tract infection (URTI) weeks prior to her presentation rendering GBS secondary to the post viral URTI and AGE as the most likely diagnosis. Presence of thrombocytopenia was the only clue for dengue in this case.
    Matched MeSH terms: Thrombocytopenia
  4. Mohamad AS, Hamzah R, Selvaratnam V, Yegapan S, Sathar J
    Hematol Rep, 2018 Sep 05;10(3):7210.
    PMID: 30344984 DOI: 10.4081/hr.2018.7210
    Human hemoglobin of G-Makassar variant has been reported very rarely with Beta Thalassemia. In year 1969 Hb GMakassar was first identified in Makassar, Sulawesi (Celebes), Republic of Indonesia. The disease was first published in 1969 and 33 years later it has been reported at a family of Thailand origin. We report a 45-yearold Malay man who was investigated for anemia and thrombocytopenia then diagnosed with Hb G-Makassar. This finding describes as a new Hemoglobin GMakassar discovered in Malaysia after 14 years diagnosed in Thailand.
    Matched MeSH terms: Thrombocytopenia
  5. White JC, Chan LK, Lau KS, Sen DK
    J Trop Med Hyg, 1976 Jun;79(6):132-6.
    PMID: 1084931
    Five patients illustrate various aspects of obstetrical defibrination in West Malaysia, resulting from exaggeration of changes in fibrinolytic-coagulation equilibrium that occur at delivery. Hypofibrinogenaemia and fibrinolysis may occur in association or either feature predominate. These patients are from a population in which a variety of genetic and environmental factors may interact, e.g. abnormal haemoglobins, cold agglutinins, viral and other infections, introducing additional complications.
    Matched MeSH terms: Thrombocytopenia/blood
  6. Çetin HS, Ekici O, Küçükyildiz F, Senlik B
    Trop Biomed, 2021 Jun 01;38(2):149-158.
    PMID: 34172704 DOI: 10.47665/tb.38.2.050
    This study was planned to determine the efficacy of doxycycline and oxytetracycline in the treatment of feline Mycoplasma spp. infections and to analyze risk factors as well as clinical and haematological findings. Cats accepted to the clinic with complaints such as weakness, loss of appetite and fever were routinely examined. Clinical history of animal was demanded from the owner. An interview and a short questionnaire were conducted to owner to evaluate the risk factors associated with possible Mycoplasma status of animal. During the interview, the age, sex, breed, housing, flea infestation, worm control, external parasite control, other accompanying infections and vaccination status of cats were asked and the data recorded. Peripheral blood smears were stained with the DIFF-QUICK. Fifty positive cats were included in the study, 16 of which were treated with doxycycline (orally, 5 mg/kg twice a day) while other 34 with oxytetracycline (intramuscular injection, 10 mg/kg once a day). It was determined that all (100%) cats with Mycoplasma spp. infection had a flea infestation. Majority of cats (94%) did not receive the regular antiparasitic application and only 6 (12%) of them had complete vaccinations. Mycoplasma spp. infection was associated with younger age and outdoor access. Among the feline breed, striped cat (74%) was more prone to infection. The most common clinical signs encountered in cats were weakness (94%) and anorexia (84%). Haematological parameters revealed thrombocytopenia (86%), and anaemia (40%) in infected cats. At the end of the treatment, recovery rates for doxycycline and oxytetracycline groups were 87.5% and 67.6%, respectively. General status of these cats indicated significant improvement on the 4th day of treatment, and clinical symptoms are restored on the th day. Mycoplasmosis is an important infection for cats having anaemia, thrombocytopenia and leukocytosis. Flea infestations are risk factor for contamination of the disease. Based on our results, we recommend doxycycline treatment (5 mg/kg) for up to 21 days for elimination of Mycoplasma spp. in the blood of infected cats.
    Matched MeSH terms: Thrombocytopenia
  7. Hanafiah M, Shahizon AM, Low SF, Shahrina MH
    BMJ Case Rep, 2013 Jul 05;2013.
    PMID: 23833091 DOI: 10.1136/bcr-2013-010163
    A 35-year-old woman with background of liver cirrhosis and portal hypertension secondary to chronic hepatitis C presented with complication of hypersplenism and thrombocytopenia. She developed severe menorrhagia requiring multiple blood transfusions. In addition, her interferon therapy was withheld owing to the underlying thrombocytopenia. Partial splenic embolisation was performed, which improved her platelet counts. Subsequently, the menorrhagia was resolved and her interferon therapy was restarted.
    Matched MeSH terms: Thrombocytopenia/etiology*
  8. Thapa B, Pandey A, Gautum S, Kc S, Chhetri PD, Pokhrel E, et al.
    J Nepal Health Res Counc, 2023 Jul 20;20(4):859-867.
    PMID: 37489668 DOI: 10.33314/jnhrc.v20i4.4172
    BACKGROUND: Dengue is a mosquito-borne viral disease with a wide spectrum of presentations ranging from subclinical disease to severe dengue. Dengue is endemic to the Terai of Nepal. Interestingly, an increasing incidence has been reported from hilly areas like Kathmandu valley. This study explored the clinicopathological profile of dengue infection.

    METHODS: A total of 84 serologically confirmed dengue cases from September to November 2019 at KIST Medical College were recruited in a cross-sectional study after obtaining ethical approval. Dengue was categorized as dengue without warning signs, dengue with warning signs, and severe dengue. Clinicopathological information was recorded in the proforma by reviewing patients' records. A descriptive statistical tool and chi-square test were carried out.

    RESULTS: Out of 84 patients, 76% (64) were dengue without warning signs, 21.4% (18) were dengue with warning signs and 2.4% (2) were severe dengue. About 97.6% (82) presented with fever. During the course of illness, anemia was identified in 38.1% (32), thrombocytopenia in 65.5% (55), hemoconcentration in 6% (5), and leucopenia in 82.1% (69). Similarly, elevated aspartate transaminase and alanine transaminase (ALT) was observed in 67.7% (42) and 53.2% (33) respectively. The severity of dengue on presentation to hospital was significantly associated with thrombocytopenia, leucopenia, and elevated ALT. Similarly, the severity during course of illness in hospital was significantly associated with hemoconcentration, thrombocytopenia, leucopenia, and elevated ALT.

    CONCLUSIONS: Most common presentation of dengue infection was fever. The most common laboratory abnormalities were leucopenia, thrombocytopenia, hemoconcentration, anemia, and elevated liver enzymes. Awareness of these clinical and laboratory parameters is important for the prompt diagnosis, severity estimation, and overall management of dengue infection.

    Matched MeSH terms: Thrombocytopenia*
  9. Lau, E.F., Mazlan, M., Shanmugam, H.
    JUMMEC, 2018;21(2):31-34.
    MyJurnal
    Phenytoin is commonly prescribed for the prophylaxis of seizures in neurosurgical patients. A phenytoininduced
    serious adverse effect of thrombocytopenia has been reported in the literature. The concurrent
    use of dexamethasone, another commonly prescribed drug in neurosurgical patients, has been reported to
    aggravate this adverse haematological effect. We present a report of phenytoin-induced thrombocytopenia
    in a patient concurrently prescribed with dexamethasone, after an intracerebral haemorrhage secondary to
    a rupture of an arteriovenous malformation. The thrombocytopenia was noted after two weeks of phenytoin
    medication. Phenytoin was immediately withheld, and seven units of random donor platelets were transfused.
    A gradual resolution of thrombocytopenia was observed within a week.
    Matched MeSH terms: Thrombocytopenia
  10. Daneshvar C, Davis TM, Cox-Singh J, Rafa'ee MZ, Zakaria SK, Divis PC, et al.
    Clin Infect Dis, 2009 Sep 15;49(6):852-60.
    PMID: 19635025 DOI: 10.1086/605439
    BACKGROUND: Plasmodium knowlesi is increasingly recognized as a cause of human malaria in Southeast Asia but there are no detailed prospective clinical studies of naturally acquired infections.

    METHODS: In a systematic study of the presentation and course of patients with acute P. knowlesi infection, clinical and laboratory data were collected from previously untreated, nonpregnant adults admitted to the hospital with polymerase chain reaction-confirmed acute malaria at Kapit Hospital (Sarawak, Malaysia) from July 2006 through February 2008.

    RESULTS: Of 152 patients recruited, 107 (70%) had P. knowlesi infection, 24 (16%) had Plasmodium falciparum infection, and 21 (14%) had Plasmodium vivax. Patients with P. knowlesi infection presented with a nonspecific febrile illness, had a baseline median parasitemia value at hospital admission of 1387 parasites/microL (interquartile range, 6-222,570 parasites/microL), and all were thrombocytopenic at hospital admission or on the following day. Most (93.5%) of the patients with P. knowlesi infection had uncomplicated malaria that responded to chloroquine and primaquine treatment. Based on World Health Organization criteria for falciparum malaria, 7 patients with P. knowlesi infection (6.5%) had severe infections at hospital admission. The most frequent complication was respiratory distress, which was present at hospital admission in 4 patients and developed after admission in an additional 3 patients. P. knowlesi parasitemia at hospital admission was an independent determinant of respiratory distress, as were serum creatinine level, serum bilirubin, and platelet count at admission (p < .002 for each). Two patients with knowlesi malaria died, representing a case fatality rate of 1.8% (95% confidence interval, 0.2%-6.6%).

    CONCLUSIONS: Knowlesi malaria causes a wide spectrum of disease. Most cases are uncomplicated and respond promptly to treatment, but approximately 1 in 10 patients develop potentially fatal complications.

    Matched MeSH terms: Thrombocytopenia/etiology; Thrombocytopenia/parasitology
  11. Wahid FS, Cheong SK, Sivagengei K
    Acta Haematol., 2002;107(4):237-8.
    PMID: 12053154
    Matched MeSH terms: Thrombocytopenia/drug therapy; Thrombocytopenia/etiology*; Thrombocytopenia/immunology
  12. Chooi, Fun Leong, Soon, Keng Cheong
    Medicine & Health, 2006;1(1):81-84.
    MyJurnal
     Myelofibrosis is a chronic myeloproliferative disorder characterised by splenomegaly, leukoerythroblastic anaemia, tear-drop poikilocytosis and marrow fibrosis. Splenectomy is indicated for patients requiring frequent transfusions or massive splenomegaly causing distressing symptoms and severe thrombocytopenia secondary to hypersplenism.A 56 year-old lady diagnosed to have primary myelofibrosis in year 2000 was referred to HUKM for further management of her increasing requirement of blood transfusion and massive splenomegaly. She has had two episodes of acute cholecytitis. Investigations done in our hospital showed that her hemoglobin level was 6.4g/dl, white cell count of 23.4x109/l, platelet count 163x109/l and peripheral blood showing leukoerythroblastic picture and tear-drop poikilocytosis. Leukocyte alkaline phosphatase score (NAP) was 184/100 neutrophils. Ultrasound of the abdomen showed massive splenomegaly with multiple gall stones. In view of the frequent transfusion requirements and symptomatic massive splenomegaly, splenectomy and cholecystectomy was performed. A review of her peripheral blood picture, post-splenectomy, showed marked reduction of the tear-drop poikilocytosis and leukoerythroblastosis. We believe that the spleen plays an important role in their formation, but the exact mechanism remains unclear.
    Matched MeSH terms: Thrombocytopenia
  13. Rabbolini DJ, Morel-Kopp MC, Chen Q, Gabrielli S, Dunlop LC, Chew LP, et al.
    J Thromb Haemost, 2017 Nov;15(11):2245-2258.
    PMID: 28880435 DOI: 10.1111/jth.13843
    Essentials The phenotypes of different growth factor-independent 1B (GFI1B) variants are not established. GFI1B variants produce heterogeneous clinical phenotypes dependent on the site of mutation. Mutation of the first non-DNA-binding zinc-finger causes a mild platelet and clinical phenotype. GFI1B regulates the CD34 promoter; platelet CD34 expression is an indicator of GFI1B mutation.

    SUMMARY: Background Mutation of the growth factor-independent 1B (GFI1B) fifth DNA-binding zinc-finger domain causes macrothrombocytopenia and α-granule deficiency leading to clinical bleeding. The phenotypes associated with GFI1B variants disrupting non-DNA-binding zinc-fingers remain uncharacterized. Objectives To determine the functional and phenotypic consequences of GFI1B variants disrupting non-DNA-binding zinc-finger domains. Methods The GFI1B C168F variant and a novel GFI1B c.2520 + 1_2520 + 8delGTGGGCAC splice variant were identified in four unrelated families. Phenotypic features, DNA-binding properties and transcriptional effects were determined and compared with those in individuals with a GFI1B H294 fs mutation of the fifth DNA-binding zinc-finger. Patient-specific induced pluripotent stem cell (iPSC)-derived megakaryocytes were generated to facilitate disease modeling. Results The DNA-binding GFI1B variant C168F, which is predicted to disrupt the first non-DNA-binding zinc-finger domain, is associated with macrothrombocytopenia without α-granule deficiency or bleeding symptoms. A GFI1B splice variant, c.2520 + 1_2520 + 8delGTGGGCAC, which generates a short GFI1B isoform that lacks non-DNA-binding zinc-fingers 1 and 2, is associated with increased platelet CD34 expression only, without quantitative or morphologic platelet abnormalities. GFI1B represses the CD34 promoter, and this repression is attenuated by different GFI1B zinc-finger mutations, suggesting that deregulation of CD34 expression occurs at a direct transcriptional level. Patient-specific iPSC-derived megakaryocytes phenocopy these observations. Conclusions Disruption of GFI1B non-DNA-binding zinc-finger 1 is associated with mild to moderate thrombocytopenia without α-granule deficiency or bleeding symptomatology, indicating that the site of GFI1B mutation has important phenotypic implications. Platelet CD34 expression appears to be a common feature of perturbed GFI1B function, and may have diagnostic utility.

    Matched MeSH terms: Thrombocytopenia/blood*; Thrombocytopenia/diagnosis; Thrombocytopenia/genetics*
  14. Lim CC, Patel DK, Bakhtiari A, Subrayan V
    Platelets, 2013;24(6):498-9.
    PMID: 22994680 DOI: 10.3109/09537104.2012.724484
    Thrombocytopenia is classically defined as a platelet count of less than 150 000/µl. Counts from 100 000 to 150 000/µl are considered mildly depressed, 50 000 to 100 000/µl moderately depressed, and less than 50 000/µl severely depressed. Thrombocytopenia occurs in about 10% of pregnant women. Gestational thrombocytopenia (GT) is a diagnosis of exclusion and considered the most prevalent cause of thrombocytopenia in pregnancy. GT accounts for almost 75% of cases of thrombocytopenia in pregnancy. The cause of GT is unclear, although existing studies denote the possibility of accelerated platelet consumption and the increased plasma volume during pregnancy. The presence of antiplatelet antibodies is not specific to GT. The degree of thrombocytopenia in GT is usually mild to moderate, usually remaining greater than 70 000/µl. Patients are asymptomatic with no evidence of bleeding and rarely preconception history of thrombocytopenia. The platelet count returns to normal within 2-12 weeks post partum. We wish to report a unique case of GT presenting as blurred vision due to retinal hemorrhages.
    Matched MeSH terms: Thrombocytopenia/diagnosis*
  15. Norasyikin AW, Rozita M, Mohd Johan MJ, Suehazlyn Z
    Med Princ Pract, 2014;23(4):387-9.
    PMID: 24401542 DOI: 10.1159/000357645
    OBJECTIVE: To report an uncommon presentation of a rare case of autoimmune polyglandular syndrome type IIIb in an elderly woman.
    CLINICAL PRESENTATION AND INTERVENTION: A 62-year-old woman presented with anaemic symptoms and jaundice. Blood tests showed macrocytic anaemia due to vitamin B12 deficiency with Coombs negative haemolysis. A thyroid function test was consistent with hypothyroidism. Autoimmune antibody assays were positive for anti-parietal cell, anti-intrinsic factor and anti-thyroid peroxidase antibodies. A final diagnosis of autoimmune thyroiditis with pernicious anaemia, which constituted autoimmune polyglandular syndrome type IIIb, was made and the patient was treated with L-thyroxine, vitamin B12 injection and a blood transfusion. She was discharged uneventfully after a week of hospitalization.
    CONCLUSION: This case showed that the presence of one autoimmune endocrine disease should prompt clinicians to look for other coexisting autoimmune diseases which may be asymptomatic despite positive autoantibodies.
    Matched MeSH terms: Thrombocytopenia/physiopathology
  16. Tang ELH, Tan NH, Fung SY, Tan CH
    Toxicon, 2019 Aug 22;169:91-102.
    PMID: 31445943 DOI: 10.1016/j.toxicon.2019.08.004
    The intraspecific geographical venom variations of Calloselasma rhodostoma from Malaysia (CR-M), Indonesia (CR-I), Thailand (CR-T) and Vietnam (CR-V) were investigated through 1D SDS-PAGE and nano-ESI-LCMS/MS. The venom antigenicity, procoagulant activities and neutralization using Thai C. rhodostoma Monovalent Antivenom (CRMAV) were also investigated. SDS-PAGE patterns of the venoms were relatively similar with minor variations. Proteomic analysis revealed that snake venom metalloproteinases (SVMPs, particularly P-I class), serine proteases (SVSPs) and snaclecs dominated the venom protein composition (68.96-81.80%), followed by L-amino acid oxidase (LAAO) and phospholipase A2 (PLA2) (7.37-11.08% and 5.18-13.81%, respectively), corroborating C. rhodostoma envenoming effects (hemorrhage, consumptive coagulopathy, thrombocytopenia and local tissue necrosis). Other proteins of lower abundances (2.82-9.13%) identified include cysteine-rich secretory proteins (CRISP), phospholipase B, phosphodiesterase, nerve growth factor, 5'-nucleotidase, aminopeptidase and hyaluronidase. All four venoms exhibited strong procoagulant effects which were neutralized by CRMAV to different extents. CRMAV immunoreactivity was high toward venoms of CR-M, CR-I and CR-T but relatively low for CR-V venom. Among the venom samples from different locales, CR-V venom proteome has the smallest SVMP composition while SVSP, PLA2 and phosphodiesterase were more abundant in the venom. These variations in C. rhodostoma venom protein composition could partly explain the differences seen in immunoreactivity. (198 words).
    Matched MeSH terms: Thrombocytopenia
  17. Chan YQ, Lee ZM, Tan SL
    Med J Malaysia, 2020 07;75(4):433-435.
    PMID: 32724010
    Intracranial haemorrhage (ICH) in a patient with relapse of idiopathic thrombocytopaenic purpura (ITP) can be lethal. The site of haemorrhage, compounded by low platelets in this disease, makes its management extremely challenging, especially when a neurosurgical procedure is warranted. We report a case report of an unconventional way of increasing platelet counts in ITP rapidly in an emergency setting.
    Matched MeSH terms: Thrombocytopenia/physiopathology*
  18. Tan, H.P.J.
    MyJurnal
    This article highlights the case of a 44-year old Malay man who is diagnosed as having treatment resistant schizophrenia on Clozapine, which then developed Polycythemia Rubra Vera (PRV). It is known that a major side effect for Clozapine is of agranulocytosis, that is a potentially fatal side effect. However, there have been reported disturbances of other hematological parameters, which result in other abnormalities including leucopenia, leucocytosis, thrombocytopenia, thrombocytosis and eosinophilia. Could this case be a pure medical condition of PRV or is there a relation to the effects of Clozapine? In this paper, the aim is to report a case of blood dyscrasia in a 44-year old male who developed Polycythemia Rubra Vera a year after he was observed to have abnormal full blood count results.
    Matched MeSH terms: Thrombocytopenia
  19. Lim SC, Chan EWL, Tang SP
    Lupus, 2020 Aug;29(9):1106-1114.
    PMID: 32631203 DOI: 10.1177/0961203320939185
    BACKGROUND: Paediatric systemic lupus erythematosus is a rare autoimmune disease with a wide spectrum of clinical presentation in different populations. We present a cohort of paediatric systemic lupus erythematosus in Malaysia where the disease features and outcomes are still largely unknown.

    METHODS: A retrospective review of all paediatric systemic lupus erythematosus patients with at least 6 months follow-up at Selayang Hospital from 2004 to 2016. Epidemiological, clinical and outcome data were collected and analysed.

    RESULTS: A total of 141 paediatric systemic lupus erythematosus patients, 87.9% females, were followed up for a median 6.3 years (interquartile range 3.6-9.0). The median age at diagnosis was 10.8 years (interquartile range 9.0-12.0 years), positive family history of systemic lupus erythematosus was present in 12.1% and the majority (61.7%) were of Malay ethnicity. Common presentations included fever (87.2%), vasculitic rash (72.3%) and lethargy (69.5%). At diagnosis, leukopenia (51.1%), thrombocytopenia (41.8%) and cutaneous lupus (56%) predominate with significant renal involvement (39.7%). Renal (45.4%), liver (26%) and the central nervous system (17%) were important major organs involved during the course of the disease. At diagnosis, almost all (99.3%) patients had high disease activity (mean Systemic Lupus Erythematosus Disease Activity Index score 20.1 ± 9.6). The majority (62.4%) achieved remission or low disease activity after 6 months, maintained over the next 10 years. Damage occurred early (39.1% at 1 year) and increased with time. Ocular damage was the most common side effect (29%) and was predominantly corticosteroid related (93%). Growth retardation was significant (38.2%) with no gonadal failure or secondary malignancies. End-stage renal disease occurred in 3.1% patients whereas 53.1% had sustained renal remission. Overall mortality was 1.4%.

    CONCLUSION: Despite high disease activity at diagnosis, the majority had good sustained response to treatment with low overall mortality. However, there was progressive accrual of organ damage, highlighting the need for further research and refinements into therapies for paediatric systemic lupus erythematosus.

    Matched MeSH terms: Thrombocytopenia/epidemiology
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