Medicine
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This repository contains the published and unpublished research of the Faculty of Medicine by the staff members of the faculty
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Item Acute hemolytic crisis complicated with ischemic cardiac injury and methemoglobinaemia following ingestion of naphthalene: a case report(BioMed Central, 2024-12) Dayasiri, K.; Pathiraja, H.; Thadchanamoorthy, V.BACKGROUND Naphthalene is an aromatic hydrocarbon that potentially produces methemoglobinaemia but rarely causes hemolysis, especially in children with underlying glucose-6-phosphate dehydrogenase deficiency. Although ingestion of a single moth ball by an older child may not be life threatening, it can be fatal if ingested by a toddler. CASE PRESENTATION A 2-year-old Singhalese boy developed acute severe hemolysis and methemoglobinaemia following ingestion of a mothball. On admission, the patient was ill and pale. The child was tachycardic and tachypnoiec with oxygen saturation of 76% on air. Blood investigations showed significant anemia, elevated reticulocytes, and evidence of hemolysis in a blood picture, along with elevated lactate dehydrogenase and indirect bilirubin. Child also had ST depressions on electrocardiogram examination with negative troponin-I. He was given four packed red blood cell (PRBC) transfusions and was successfully discharged in 3 days time following optimal supportive treatment. A glucose-6-phosphate dehydrogenase assay confirmed the diagnosis of glucose-6-phosphate dehydrogenase deficiency in this child: 0.9 U/gHb (4.0-13.0 U/gHb).CONCLUSION This case report highlights a rare life-threatening presentation of naphthalene ingestion in a child with previously undiagnosed glucose-6-phosphate dehydrogenase deficiency. Ingestion of even a single moth ball can be fatal in vulnerable children given the altered toxicokinetics of naphthalene in children.Item Methotrexate-induced leukoencephalopathy presenting as acute-onset limb weakness in a child: A case report(BioMed Central, 2024) Pathiraja, H.; Abrew, G.D.; De Silva, L.; Fernando, S.; Randeny, S.; Mettananda, S.BACKGROUND Methotrexate is an essential medicine used to treat childhood malignancies including acute lymphoblastic leukemia. Neurotoxicity manifesting as leukoencephalopathy is an important adverse effect of methotrexate. Methotrexate-induced leukoencephalopathy classically demonstrates sub-acute-onset neurological manifestations that include learning disability, progressive dementia, drowsiness, seizures, ataxia, and hemiparesis. These are rare in children and are generally reported following intrathecal or intravenous use of methotrexate. In contrast, acute onset neurotoxicity with oral use of methotrexate is very rare. We report a 10-year-old boy presenting with acute onset limb weakness and neurological signs due to methotrexate-induced leukoencephalopathy following oral methotrexate.CASE PRESENTATION A 10-year-old Sri Lankan boy presented with fever and headache for 5 days and difficulty in walking for 2 days. He was unable to stand unaided on admission, and his parents complained of repetitive, involuntary extension movements involving the right upper limb. He is a child diagnosed with acute lymphoblastic leukemia who was on treatment for a relapse with daily oral dexamethasone and mercaptopurine, weekly oral methotrexate and folinic acid, and once every two weeks intrathecal vincristine. On examination, he had dystonic movements of the right upper limb and hypotonia and reduced muscle power (grade 3/5) of the left upper and lower limbs proximally and distally. The muscle power of the right side was grade 4 (out of 5). Tendon reflexes were diminished in all four limbs, and the plantar response was flexor bilaterally. The child had dysmetria and intension tremors on both sides. T2-weighted magnetic resonance imaging of the brain revealed symmetrical high signal intensities with diffusion restriction involving bilateral putamen, subcortical areas, and deep white matter, suggesting treatment-related neurotoxicity due to methotrexate-induced leukoencephalopathy. Oral methotrexate was discontinued. He showed gradual improvement in limb weakness and other neurological signs following treatment with intravenous folinic acid, aminophylline, dexamethasone, and oral dextromethorphan.CONCLUSION This case report describes a patient with rapidly progressing methotrexate-induced leukoencephalopathy following oral methotrexate. It highlights that the risk of neurotoxicity persists even with the oral use of methotrexate; therefore, the prescribers should be vigilant of this uncommon side effect.Item Parental knowledge, attitudes and practices regarding safe handling and disposal of alcohol-based hand sanitizers and surface disinfectants in urban Sri Lanka(Ethiopian Pediatric Society, 2024) Dayasiri, K.; Pathiraja, H.; De Soyza, E.K.N.; Thadchanamoorthy, V.; Dassanayaka, S.J.S.INTRODUCTION Hand sanitizers were increasingly used in most households during the COVID-19 pandemic. This study aimed to assess knowledge and practices regarding the safe handling and disposal of alcohol-based hand sanitizers and surface disinfectants in urban Sri Lanka.METHODS A cross-sectional study was performed including parents of children admitted to North Colombo Teaching Hospital for a period of one year in August 2021. Data were collected regarding parental knowledge about the safe handling and disposal of hand sanitizers, and their health hazards by paediatric post-graduate trainees. All data were analyzed using SPSS 17.0. Chi-square test was used to find the association of overall knowledge and attitude scores with potential sociodemographic determining factors.RESULTS A total of 153 parents were recruited. The majority of mothers (126, 82.4%) and fathers (133, 86.9%) had attended up to secondary school. Approximately 113(73.9%) parents believed that pre-school children were the most vulnerable for accidental ingestion of sanitizers and 40 parents (26.1%) did not believe that sanitizer solutions can be accidentally inhaled by toddlers. Only 132 parents (86.2%) knew how to disinfect their home premises safely and 29 parents (18.9%) didn’t know how to store cleaning products safely. Overall, lower knowledge scores correlated with lower maternal education (p<0.05) and lower socio-economic status (p<0.05). The gender (p = 0.06) and age of the parent (>35 years versus <35 years) (p = 0.21) did not show a significant association. Attitude scores positively correlated with parental education (p<0.002) and socio-economic status (p<0.03). The gender (p = 0.12) and age of the parent (>35 years versus <35 years) (p = 0.07) did not show a significant association.CONCLUSION Overall knowledge and attitude scores in parents were associated with education level of parents and the level of socio- economic status. Gender and parental age did not show a significant association.Item Primary mediastinal diffuse large B-Cell lymphoma presenting with pyrexia of unknown origin in a 13-year-old boy – A case report(Batticaloa Medical Association, 2023) Pathiraja, H.; Dayasiri, K.Item Hepatic and renal status of paediatric patients with thalassaemia(Sri Lanka Medical Association, 2023) Wijenayake, W.; Pathiraja, H.; Thennakoon, R.; Fernando, M.; Bandara, D.; Mettananda, S.INTRODUCTION: Thalassaemia is a chronic disorder affecting many organ systems. Although cirrhosis is a well-recognised complication, sub-clinical hepatic and renal dysfunction in thalassaemia are poorly studied. OBJECTIVES: We aimed to describe the hepatic and renal status and dysfunction of paediatric patients with thalassaemia. METHODS: A cross-sectional descriptive study was conducted at Kurunegala and Ragama Thalassaemia Centres during February and March 2023. All patients aged less than 16 years attending the thalassaemia centres were recruited. Data were collected using a data collection form by interviewing parents and perusal of clinical records and analysed using SPSS 27.0. Ethical approval was obtained from the Sri Lanka College of Paediatricians. RESULTS: Sixty-five patients (mean age-7.7; males-46%) were recruited. Of them 48(73%) had homozygous beta-thalassaemia and 17(26%) had HbE thalassaemia; 52(80%) were transfusion-dependent and 13(20%) were non-transfusion-dependent. Hepatomegaly and splenomegaly were found in 45(69%) and 30(46%), respectively. Regarding hepatic status, 34(52%) had high (>40IU/L) alanine transaminases, of which 8(12%) had >3-fold elevation of alanine transaminases. A higher proportion of children with HbE thalassaemia (71%) had elevated alanine transaminases compared to homozygous beta-thalassaemia (46%, χ2=3.0, p=0.07). Also, a higher proportion of children on deferasirox (57%) had elevated alanine transaminases compared to those who were not on the drug (27%, χ2=3.3, p=0.06). Four (5%) had <2+ proteinuria in urinalysis however, the urine protein: creatine ratio was normal in all. CONCLUSION: High alanine transaminases were noted in over 50% of paediatric patients with thalassaemia. HbE thalassaemia type and use of deferasirox were associated with high alanine transaminase levels.Item Lysinuric protein intolerance presenting as pancytopenia and splenomegaly mimicking acute leukaemia: a case report(BioMed Central, 2023) Lokuhewage, C.; Pathiraja, H.; Madawala, P.; Bandara, S.; Mettananda, S.BACKGROUND: Lysinuric protein intolerance is a rare inherited metabolic disease due to autosomal recessive mutations of the SLC7A7 gene. The affected patients commonly present with protein-rich food intolerance, failure to thrive, hepatosplenomegaly, muscle hypotonia and lung involvement due to impaired intestinal absorption and excessive urinary excretion of dibasic amino acids. Presentation with splenomegaly and cytopenia without other features has not been reported. Here we report a Sri Lankan girl with lysinuric protein intolerance presenting with pancytopenia and splenomegaly mimicking acute leukaemia. CASE PRESENTATION: Two years and six months old Sri Lankan girl presented with persistent pancytopenia following a viral illness. She was asymptomatic without vomiting, diarrhoea, abdominal pain or irritability. Physical examination revealed pallor and isolated firm splenomegaly of 2 cm. Growth parameters and other system examinations were normal. Full blood count revealed anaemia, leukopenia and thrombocytopenia. The blood picture showed a mixture of hypochromic microcytic and normochromic normocytic red cells with occasional pencil cells and macrocytes. Bone marrow examination was normal except for occasional megaloblasts; however, serum vitamin B12 and red blood cell folate were normal. The metabolic screen showed a high anion gap compensated metabolic acidosis, high lactate and ketosis. Genetic mutation analysis using whole exome sequencing revealed compound heterozygous variants of the SLC7A7 gene, confirming the diagnosis of lysinuric protein intolerance. CONCLUSION: We report a child with lysinuric protein intolerance presenting with pancytopenia and splenomegaly without other disease features. This case report adds to the heterogenic presentations of lysinuric protein intolerance, which is considered a multifaceted disease.Item Hemitruncus arteriosus: A rare congenital cardiac anomaly diagnosed and treated during the neonatal period(Sri Lanka College of Paediatricians, 2023) Pathiraja, H.; de Livera, R.; Wickramaratne, S.; Perera, S.; Mugunthan, M.S.; Mettananda, S.No abstract availableItem A large pericardial cystic lymphangioma presenting as acute-onset respiratory distress in a child: a case report(BioMed Central, London, 2022) Pathiraja, H.; Rasnayake, D.; Muthukumarana, T.; de Silva, C.; Sathkorala, W.; Gunaratne, S.; Rajindrajith, S.; Mettananda, S.Background: Lymphangiomas are rare benign malformations of the lymphatics that occur due to blockage of the lymphatic system during fetal development. They commonly occur in the neck and axilla, while involvement of the pericardium is rare. We report herein the case of a 16-month-old Sri Lankan child with a large pericardial cystic lymphangioma presenting with sudden-onset shortness of breath. Case presentation: A 16-month-old Sri Lankan boy presented with sudden-onset dyspnea for 1-day duration following a febrile illness that lasted 2 days. On examination, he was afebrile and had subcostal, intercostal, and suprasternal recessions, with a respiratory rate of 50 breaths per minute. He had a loud expiratory grunt. The chest expansion was reduced on the right side, which was dull to percussion. Auscultation revealed a marked reduction of air entry over the right lower and mid zones. Chest X-ray showed a well-demarcated opacity involving the lower and mid zones of the right hemithorax associated with a tracheal shift to the opposite side. Ultrasound scan of the chest revealed fluid-filled right hemithorax suggesting a septate pleural effusion. A contrast-enhanced computed tomography scan of the thorax showed a large multiloculated extrapulmonary cystic lesion involving the right hemithorax with a mediastinal shift towards the left side associated with displacement of the right-side mediastinal structures. He underwent mini-thoracotomy and surgical excision of the cyst. A large cyst originating from the pericardium was observed and excised during surgery. Histological examination revealed a lesion composed of cysts devoid of a lining epithelium but separated by connective tissue, mature adipose tissue, and lymphoid aggregates. The child showed complete recovery postoperatively with full expansion of the ipsilateral lung. Conclusion: We report the case of a patient with cystic lymphangioma who was perfectly well and asymptomatic until 16 months of age. This case report presents the very rare occurrence of a large cystic lymphangioma originating from the pericardium. It highlights the importance of considering rare possibilities and performing prompt imaging in situations of diagnostic uncertainty to arrive at an accurate diagnosis that can be lifesaving.Item Maternal knowledge on curative therapies and its impact on medical care and psychological health among children with thalassaemia in Sri Lanka(Sri Lanka College of Paediatricians, 2022) Mettananda, S.; Pathiraja, H.; Peiris, R.; Bandara, D.; de Silva, U.; Mettananda, C.; Premawardhena, A.Background: b-thalassaemia is an inherited disorder of haemoglobin synthesis which results in severe transfusion-dependent anaemia from infancy. Although considered a life-limiting disease, it can be cured by allogeneic haematopoietic stem cell transplantation and gene therapy. However, many patients and their families in developing countries are unaware of these treatment options. Objectives: To assess the maternal knowledge on curative therapies and to determine its association with the adequacy of current medical treatment and psychological health among children with b-thalassaemia. Method: We conducted a cross-sectional study at the three largest thalassaemia centres of Sri Lanka. All patients with transfusion-dependent b-thalassaemia aged 2-18 years were eligible for the study. Data were collected using an interviewer-administered questionnaire by interviewing mothers and from medical records. The questionnaire contained questions to gather information on socio-demographic background, clinical details and maternal knowledge on curative therapies for thalassaemia. The psychological morbidity of children was assessed using the previously validated ‘strengths and difficulties questionnaire’. Binary logistic regression was used in the analysis. Results: A total of 304 patients (mean age 9.8years; females 54%) were recruited. A majority (86%) of mothers knew that b-thalassaemia can be cured by haematopoietic stem cell transplantation; however, only 1% were aware of gene therapy. Detailed knowledge on curative therapies was lacking in most mothers; only 22% could identify suitable donors for transplantation. Maternal knowledge on curative therapies was associated with higher educational level and income of parents. Accurate maternal knowledge on haematopoietic stem cell transplantation was significantly associated with lower rates of hepatomegaly, splenomegaly, emotional symptoms, conduct symptoms, hyperactive symptoms and abnormal peer relationships in patients. Conclusions: This study demonstrated that maternal knowledge on curative therapies among patients with b-thalassaemia is sub-optimal. It further demonstrated that having an accurate maternal knowledge is associated with improved medical care and a lower prevalence of psychological symptoms among patients.Item Clinical management of β-thalassemia(New York; Nova Science Publishers, 2020) Mettananda, S.; Pathiraja, H.No abstract available