Browsing by Author "Pathiraja, H."

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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.
Blood transfusion therapy for β-thalassemia major and hemoglobin E β-thalassemia: adequacy, trends, and determinants in Sri Lanka.
(John Wiley, 2019) Mettananda, S.; Pathiraja, H.; Peiris, R.; Wickramarathne, N.; Bandara, D.; de Silva, U.; Mettananda, C.; Premawardhena, A.
BACKGROUND: Regular blood transfusion therapy still remains the cornerstone in the management of β-thalassemia. Although recommendations are clear for patients with β-thalassemia major, uniform transfusion guidelines are lacking for patients with hemoglobin E β-thalassemia. In this study, we aim to describe the adequacy, trends, and determinants of blood transfusion therapy in a large cohort of pediatric patients with β-thalassemia major and hemoglobin E β-thalassemia. METHODS/PROCEDURE: This cross-sectional study was performed among all regularly transfused patents with β-thalassemia aged 2 to 18 years attending three large thalassemia centers in Sri Lanka. Data were collected using an interviewer-administered questionnaire, perusal of clinical records, and physical examination of patients by trained doctors. RESULTS: A total of 328 patients (male 47%) were recruited; 83% had β-thalassemia major, whereas 16% had hemoglobin E β-thalassemia. Sixty-one percent of patients had low pretransfusion hemoglobin levels (< 9.0 g/dL) despite receiving high transfusion volumes (> 200 mL/kg/year) by a majority (56%). Median pretransfusion hemoglobin was significantly lower in patients with hemoglobin E β-thalassemia compared with β-thalassemia major (P < 0.001); however, there was no difference in requirement for high transfusion volumes over 200 mL/kg/year in both groups (P = 0.14). Hepatomegaly and splenomegaly were more common in hemoglobin E β-thalassemia and were associated with lower pretransfusion hemoglobin. Transfusion requirements were higher among patients with hepatitis C and in those who are underweight. CONCLUSIONS: Over 60% of regularly transfused patients with β-thalassemia have low pretransfusion hemoglobin levels despite receiving large transfusion volumes. Patients with hemoglobin E β-thalassemia are undertransfused and specific recommendations should be developed to guide transfusions in these patients.
Clinical management of β-thalassemia
(New York; Nova Science Publishers, 2020) Mettananda, S.; Pathiraja, H.
No abstract available
Health related quality of life among children with transfusion dependent β-thalassaemia major and haemoglobin E β-thalassaemia in Sri Lanka: a case control study.
(BioMed Central, 2019) Mettananda, S.; Pathiraja, H.; Peiris, R.; Bandara, D.; de Silva, U.; Mettananda, C.; Premawardhena, A.
BACKGROUND:Thalassaemia is a chronic disease without an effective cure in a majority. The clinical management has improved considerably during recent years; however, minimal attempts are made to up lift the quality of life among patients, especially in developing countries. Here we aim to describe and compare and to determine factors associated with health related quality of life among patients with transfusion dependent β-thalassaemia major and haemoglobin E β-thalassemia in Sri Lanka.METHODS:A case control study was conducted in the three largest thalassaemia centres of Sri Lanka. All patients with transfusion dependent β-thalassaemia (β-thalassaemia major and haemoglobin E β-thalassaemia) aged 5-18 years were recruited as cases whilst a randomly selected group of children without chronic diseases were recruited as controls. Socio-demographic and clinical data were collected using an interviewer-administered questionnaire and health related quality of life was measured using the validated Paediatric Quality of Life Inventory Version 4.0.RESULTS:Two hundred and seventy one patients with transfusion dependent β-thalassaemia (male-49.1%; mean age- 10.9 ± 3.6 years) and 254 controls (male-47.2%; mean age- 10.4 ± 3.5 years) were recruited. Mean health-related quality of life scores were significantly lower in patients compared to controls (72.9 vs. 91.5, p < 0.001). Of the patients, 224 (84%) had β-thalassaemia major and 43 (16%) had haemoglobin E β-thalassaemia. Quality of life scores in psychological health (p < 0.05), emotional functioning (p < 0.05) and social functioning (p < 0.05) were significantly lower in patients with haemoglobin E β-thalassaemia compared to β-thalassaemia major. Splenectomy (p < 0.05), short stature (p < 0.05), under nutrition (p < 0.05) and longer hospital stays (p < 0.05) were significantly associated with lower quality of life scores.CONCLUSIONS:Despite improvements in management, the quality of life among patients with β-thalassaemia still remains low. This is more pronounced in the subset of patients with haemoglobin E β-thalassaemia. Splenectomy, short stature, undernutrition and longer hospital stays were significantly associated with poor quality of life. It is timely, even in developing countries, to direct emphasis and to take appropriate steps to improve standards of living and quality of life of patients with β-thalassaemia.
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 available
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.
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.
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.
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.
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.
Multisystem inflammatory syndrome in children: A Sri Lankan case series
(Sri Lanka College of Paediatricians, 2021) Sandakelum, U.; Samararathna, R.; Pathiraja, H.; de Silva, L.; Balasubramaniam, R.; de Abrew, G.; Adihetty, D.; Fernando, M.; Randeny, S.; Mettananda, S.
No Abstract Available
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.
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.
Psychological morbidity among children with transfusion dependent β-thalassaemia and their parents in Sri Lanka
(Public Library of Science, 2020) Mettananda, S.; Peiris, R.; Pathiraja, H.; Chandradasa, M.; Bandara, D.; de Silva, U.; Mettananda, C.; Premawardhena, A.
BACKGROUND: Thalassaemia is a chronic disease which requires lifelong treatment in a majority. Despite recent advances in the medical care, minimal attempts are made to improve psychological health in these patients. In this study, we aim to describe the psychological morbidity in patients with transfusion dependent β-thalassaemia and their mothers in Sri Lanka. METHODS: This case control study was conducted in the three largest thalassaemia centres of Sri Lanka. All patients with transfusion dependent β-thalassaemia aged 4-18 years were recruited as cases whilst a randomly selected group of children without chronic diseases were recruited as controls. Psychological morbidity of children was assessed using the Strengths and Difficulties Questionnaire and depressive symptoms of mothers was assessed by the Centre for Epidemiological Studies Depression Scale. RESULTS: 288 transfusion dependent β-thalassaemia patients and equal number of controls were recruited. Abnormal emotional, conduct, hyperactivity and peer relationship symptom scores were reported by 18%, 17%, 9% and 14% of patients with thalassaemia respectively. Prevalences of abnormal psychological symptom scores in all domains were significantly higher among patients compared to controls. Abnormal conduct symptoms were significantly more prevalent among patients with HbE β-thalassaemia and those with suboptimal pretransfusion haemoglobin levels, lower transfusion volumes, hypothyroidism and undernutrition. Short stature was associated with abnormal emotional and hyperactivity scores. Depressive symptoms were significantly higher among mothers of patients with thalassaemia. Higher depressive symptom scores in mothers were significantly associated with abnormal emotional, conduct and peer relationship symptom scores in children. CONCLUSIONS: A higher proportion of patients with transfusion dependent β-thalassaemia had abnormal psychological symptom scores. Abnormal conduct symptoms were more prevalent among patients with HbE β-thalassaemia, those who were inadequately transfused and having hypothyroidism and undernutrition. Mothers of the children with transfusion dependent β-thalassaemia had significantly higher depressive symptoms which were significantly associated with psychological symptoms among children.
Quality of life and psychological morbidity among children with transfusion dependent thalassaemia and their parents
(Sri Lanka Medical Association, 2018) Mettananda, S.; Pathiraja, H.; Peiris, R.; Bandara, D.; de Silva, T.U.N.; Mettananda, K.C.D.; Premawardhena, A.
INTRODUCTION AND OBJECTIVES: Thalassaemia is a chronic illness which requires life-long supportive care. We aim to describe quality of life and psychological morbidity among children with transfusion-dependent thalassaemia and their parents.METHODS: This case-control study was conducted from September to December 2017. All patients with transfusion-dependent thalassaemia aged 2-18 years attending three largest thalassemia centers of Sri Lanka (Kurunegala, Anuradhapura and Ragama) were recruited as cases Children without chronic diseases admitting to same hospitals for acute non-life threatening illnesses were recruited as controls. Data were collected using an interviewer-administered questionnaire with validated questionnaires to measure quality of life (PedsQL 4.0 Generic core scales) and psychological morbidity (Strengths and difficulties questionnaire) of children and depressive symptoms of parents (Centre for epidemiological studies depression scale). Ethical approval was obtained from Ethics Committee of University of Kelaniya. RESULTS: 321 (male-46.4%; mean age-9 9±4 2years) cases and 194 (male-47.4% mean age- 8.9±3 7years) controls were recruited Mean quality of life score was significantly lower in cases (72.6±12.0%) compared to controls (88.6±11.6%), (p<0 001) Prevalence of psychological issues were significantly higher among patients with thalassaemia compared to controls in emotional (17 6% vs 0%; 2=32.8, p<0 001), conduct (18.6% vs 0 6%;2=35.4, p<0 001), hyperactive (9 5% vs 0%; 2=18 9 p<0.001) and peer relationship (14.4% vs 2 5%; 2=15 7 p<0 001) domains Significantly higher proportion of mothers of cases reported depressive symptoms compared to controls (40 1% vs 8.9%; 2=57 0 p<0.001).CONCLUSION: Patients with transfusion-dependent thalassaemia experience a poor quality of life and are victims of psychological and behavioural problems compared to their peers. Similarly, mothers of children with thalassaemia have higher prevalence of depression Psychological support should be an essential component in management of thalassaemia