The Spetzler-Martin grading system and management of patients with intracranial Arteriovenous malformation in a tertiary referral hospital

Introduction: An arteriovenous malformation (AVM) is an abnormal collection of blood vessels in which arterial blood flows directly into the draining vein without the normal interposed capillaries. The Spetzler-Martin grading system has been widely accepted worldwide to estimate the pretreatment risks and predict the outcome of patients with intracranial AVM. In Malaysia, we still do not have the baseline data of this grading system. Methods: A total of 33 patients from a tertiary referral hospital diagnosed with intracranial AVM based on neuroimaging findings over a 4-year period were studied. Medical records were traced and neuroimaging findings were analysed. The AVMs were graded according to the Spetzler-Martin grading system and Fisher’s exact test was used to assess statistical difference between the grades of the AVM and management plan for the patients. Results: Four patients were graded as Grade 1, 9 patients as Grade II, 10 patients as Grade III, 6 patients as Grade IV and 4 patients as Grade V. Ten patients were treated conservatively; six patients underwent surgery and embolisation respectively. Four patients underwent radiosurgery and a combination of embolisation while surgery and radiosurgery were given to 5 patients and 2 patients respectively. Statistically significant difference (p=0.016) was found between the Spetzler-Martin grading system and the management of intracranial AVMs. Conclusion: The management decision was not made based on the grading of the AVMs. It is recommended that all AVM patients be routinely graded according to this system prior to treatment. Keywords: Intracranial arteriovenous malformation

Familial hypercholesterolaemia in children and adolescents from 48 countries: a cross-sectional study

Background: Approximately 450 000 children are born with familial hypercholesterolaemia worldwide every year, yet only 2·1% of adults with familial hypercholesterolaemia were diagnosed before age 18 years via current diagnostic approaches, which are derived from observations in adults. We aimed to characterise children and adolescents with heterozygous familial hypercholesterolaemia (HeFH) and understand current approaches to the identification and management of familial hypercholesterolaemia to inform future public health strategies. Methods: For this cross-sectional study, we assessed children and adolescents younger than 18 years with a clinical or genetic diagnosis of HeFH at the time of entry into the Familial Hypercholesterolaemia Studies Collaboration (FHSC) registry between Oct 1, 2015, and Jan 31, 2021. Data in the registry were collected from 55 regional or national registries in 48 countries. Diagnoses relying on self-reported history of familial hypercholesterolaemia and suspected secondary hypercholesterolaemia were excluded from the registry; people with untreated LDL cholesterol (LDL-C) of at least 13·0 mmol/L were excluded from this study. Data were assessed overall and by WHO region, World Bank country income status, age, diagnostic criteria, and index-case status. The main outcome of this study was to assess current identification and management of children and adolescents with familial hypercholesterolaemia. Findings: Of 63 093 individuals in the FHSC registry, 11 848 (18·8%) were children or adolescents younger than 18 years with HeFH and were included in this study; 5756 (50·2%) of 11 476 included individuals were female and 5720 (49·8%) were male. Sex data were missing for 372 (3·1%) of 11 848 individuals. Median age at registry entry was 9·6 years (IQR 5·8-13·2). 10 099 (89·9%) of 11 235 included individuals had a final genetically confirmed diagnosis of familial hypercholesterolaemia and 1136 (10·1%) had a clinical diagnosis. Genetically confirmed diagnosis data or clinical diagnosis data were missing for 613 (5·2%) of 11 848 individuals. Genetic diagnosis was more common in children and adolescents from high-income countries (9427 [92·4%] of 10 202) than in children and adolescents from non-high-income countries (199 [48·0%] of 415). 3414 (31·6%) of 10 804 children or adolescents were index cases. Familial-hypercholesterolaemia-related physical signs, cardiovascular risk factors, and cardiovascular disease were uncommon, but were more common in non-high-income countries. 7557 (72·4%) of 10 428 included children or adolescents were not taking lipid-lowering medication (LLM) and had a median LDL-C of 5·00 mmol/L (IQR 4·05-6·08). Compared with genetic diagnosis, the use of unadapted clinical criteria intended for use in adults and reliant on more extreme phenotypes could result in 50-75% of children and adolescents with familial hypercholesterolaemia not being identified. Interpretation: Clinical characteristics observed in adults with familial hypercholesterolaemia are uncommon in children and adolescents with familial hypercholesterolaemia, hence detection in this age group relies on measurement of LDL-C and genetic confirmation. Where genetic testing is unavailable, increased availability and use of LDL-C measurements in the first few years of life could help reduce the current gap between prevalence and detection, enabling increased use of combination LLM to reach recommended LDL-C targets early in life

Head Injury from Fan Blades Among Children

Head injury caused by fan blades is rare among children. We analysed 14 cases of such injury and discuss the causes, type of injury and preventive measures. Methods: A retrospective analysis of 14 cases of children who were admitted to the Pediatric Neurosurgical Unit of Hospital Kuala Lumpur after sustaining head injuries caused by fan blades between January 2000 and December 2002 was performed. Results: The causes of fan-blade head injury included jumping on the upper bunk of a bunk-bed, climbing on a ladder, climbing up onto a table, and being lifted by an adult. Thirteen patients were injured by ceiling fans and one by falling onto an uncovered table fan. School-aged boys were the predominant victims. Mean patient age was 7.9 years (range, 1.0-12.2 years). There was a twin peak incidence of when the accidents occurred: just before lunch in the afternoon and bedtime at night. The types of injury were scalp lacerations, compound depressed fractures and multiple intracranial haemorrhages. Two patients had the complication of wound infection, and one of these patients developed cerebral spinal fluid leak. One patient died from severe head injuries. Conclusion: Safety awareness among parents and caretakers are important as fan-blade head injury among children is preventable

Screening Of Bismillah Leaf (Vernonia Amygdalina) Extraction For Antiproliferative Activies In Human Glioblastoma Brain Cancer Cell Lines

ABSTRACT Vernonia amygdalina is a member of the Asteraceae family. It is a shrub or small tree that can reach up to 10 m in height and with a trunk diameter reaching up to 40 cm. The plant is a vegetable in its native land and is put through a detoxification process before it is cooked as food. The process involves boiling the leaves several times while changing the water after each cycle until the bitterness disappears. This study was carried out to screen the ethanol, methanol and ethyl acetate extracts of Vernonia Amygdalina as an anti-proliferative agent that inhibits the growth of human glioblastoma brain cancer cell lines (U-87). The anti-proliferative study indicated that only ethyl acetate extracts from Vernonia amygdalina were found to be cytotoxic towards U-87 cancer cell lines with an IC 50 value of 18.80 ± 1.11 µg/ml. There was no IC 50 value for ethanol and methanol extracts from Vernonia amygdalina. Thus, ethyl acetate extracts from Vernonia amygdalina could be envisaged for further exploration in efforts to develop newer and safer cytotoxic compounds