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This repository contains the published and unpublished research of the Faculty of Medicine by the staff members of the faculty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 Internal biliary diversion for medically refractory pruritus in a child with Alagille syndrome: a novel alternative for liver transplantation(Sri Lanka College of Paediatricians, 2024-12) Tillakaratne, S.; Fernando, M.; Batepola, T.; Gunetilleke, B.; Appuhamy, C.; Weerasuriya, A.; Siriwardana, R.No abstract availableItem 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 Prediction equation for physical activity energy expenditure in 11-13-year-old Sri Lankan children(MDPI Publishing, 2023) Dabare, P.; Wickramasinghe, P.; Waidyatilaka, I.; Devi, S.; Kurpad, A.V.; Samaranayake, D.; de Lanerolle-Dias, M.; Wickremasinghe, R.; Hills, A.P.; Lanerolle, P.This study aimed to develop a regression equation to predict physical activity energy expenditure (PAEE) using accelerometry. Children aged 11-13 years were recruited and randomly assigned to validation (n = 54) and cross-validation (n = 25) groups. The doubly labelled water (DLW) technique was used to assess energy expenditure and accelerometers were worn by participants across the same period. A preliminary equation was developed using stepwise multiple regression analysis with sex, height, weight, body mass index, fat-free mass, fat mass and counts per minute (CPM) as independent variables. Goodness-of-fit statistics were used to select the best prediction variables. The PRESS (predicted residual error sum of squares) statistical method was used to validate the final prediction equation. The preliminary equation was cross-validated on an independent group and no significant (p > 0.05) difference was observed in the PAEE estimated from the two methods. Independent variables of the final prediction equation (PAEE = [0.001CPM] - 0.112) accounted for 70.6% of the variance. The new equation developed to predict PAEE from accelerometry was found to be valid for use in Sri Lankan children.Item Wilson disease presenting as acute haemolytic anaemia in a Sri Lankan child(Sri Lanka College of Paediatricians, 2022) Chandrakumara, J.; Rathnayake, C.; Muthukumarana, O.; Amarakoon, G.; Fernando, M.No abstract availableItem Acute appendicitis during the recovery phase of dengue hemorrhagic fever: two case reports(BioMed Central, 2022) Thadchanamoorthy, V.; Ganeshrajah, A.; Dayasiri, K.; Jayasekara, N.P.Background: Dengue fever is one of the most common tropical diseases, with high prevalence in many tropical countries including Sri Lanka. Dengue infection can present from subclinical infection to dengue shock syndrome. Further, the disease also shows a variety of atypical presentations and has been reported to mimic a number of causes of acute abdomen. Case presentation: The authors report two children (a 6-year-old Tamil girl and an 8-year-old Muslim girl) who were diagnosed to have acute appendicitis during the early recovery phase of dengue hemorrhagic fever (DHF) and late recovery period of dengue hemorrhagic fever with platelet count of 92 × 103/cumm and 102 × 103/cumm, respectively. Both children were investigated with abdomen ultrasound as they developed severe abdominal pain and tenderness on palpation during the recovery phase, which was felt to be very unusual. Acute appendicitis was diagnosed in one child, while the other child had a ruptured appendicular abscess. Both children were treated with laparoscopic appendectomy and a 7-day course of intravenous antibiotics. Both children were reviewed in 1 month following treatment and had complete recovery. Conclusion: Although precise pathophysiology and associations of the surgical abdomen with dengue fever remain to be elucidated, there are known factors in dengue fever that can potentially lead to secondary bacterial infections and surgical abdomen. Awareness and increased suspicion by the clinician are paramount to detect such complications early, especially in children who demonstrate unusual clinical features during various stages of dengue infection.Item Expanded dengue syndrome presenting with acute liver failure, acute kidney injury, pancreatic involvement, coagulopathy, and multiple intracranial hemorrhages in a young child: a case report(BioMed Central, 2022) Thadchanamoorthy, V.; Dayasiri, K.Background: Dengue is a mosquito-borne viral infection that typically occurs in tropical and subtropical countries. The clinical manifestations of dengue infection range from an asymptomatic subclinical course to severe dengue shock syndrome. Besides, dengue can affect any organ in the body and can present with atypical manifestations. Case presentation: We report a 6-year-old previously healthy Tamil child who had dengue complicated with multiorgan involvement. She initially presented with high fever, headache, body aches for 5 days, blood and mucus diarrhea, hematuria, and right knee joint swelling for 2 days. Dengue NS1 antigen was positive on day 2 of febrile illness. She was managed symptomatically in the local hospital for 3 days and transferred to the tertiary care hospital for further management. She was eventually diagnosed as having dengue hemorrhagic fever complicated with multiorgan involvement including acute liver failure, pancreatic involvement, coagulopathy, arthritis, acute kidney injury, and multiple intracranial hemorrhages. The constellation of disease manifestations was identified as expanded dengue syndrome. She was managed with fresh blood, platelet, and cryoprecipitate transfusions and intravenous antibiotics in addition to renal and liver support in the intensive care unit. On day 14 of illness, she deteriorated while on the ventilator and died due to multiple intracranial hemorrhages. Conclusion: The reported child with dengue hemorrhagic fever developed several unusual presentations such as acute liver and renal failure, disseminated intravascular coagulopathy, pancreatic involvement, and multiple intracranial hemorrhages, which form part of expanded dengue syndrome. In the seriously unwell child, it is important to look for unusual complications actively to improve outcomes.Item Acute disseminated encephalomyelitis presenting as bilateral ptosis in a Sri Lankan Child(Hindawi Pub. Corp., 2022) Kumarasiri, I.; Samararathna, R.; Sandakelum, U.; Muthukumarana, O.; Balasubramaniam, R.; Mettananda, S.Introduction: Acute disseminated encephalomyelitis is a rare inflammatory demyelinating disease characterized by acute onset polyfocal neurological deficits associated with encephalopathy. It commonly presents with fever, meningism, seizures, ataxia, motor deficits, and bladder dysfunction. Although cranial neuropathies, including optic neuritis and facial nerve palsies, have previously been reported, children presenting with bilateral ptosis is extremely rare. Here, we report a 3-year-old child with acute disseminated encephalomyelitis presenting with acute onset bilateral ptosis due to involvement of the single central levator subnucleus of the oculomotor nerve. Case Presentation. A 3-year-old Sri Lankan boy presented with drooping of the upper eyelids for three days and unsteady gait for two days. He did not have seizures, blurring of vision, limb weakness, swallowing or breathing difficulties, or bladder dysfunction. On examination, he had bilateral ptosis, gait ataxia, and dysmetria. His vision, eye movements, and examination of other cranial nerves were normal. MRI brain revealed high signal intensities involving the subcortical white matter of parietal and occipital lobes, midbrain in the area of single central levator subnucleus of the oculomotor nerve, cerebellar vermis, and right cerebellar hemisphere. Based on the clinical features suggesting polyfocal neurological involvement of the midbrain and cerebellum and characteristic MRI findings, the diagnosis of acute disseminated encephalomyelitis was made. He responded well and rapidly to high-dose intravenous methylprednisolone and showed a complete clinical and radiological recovery. Conclusion: This case report describes a rare presentation of acute disseminated encephalomyelitis, bilateral ptosis due to involvement of the single central levator subnucleus of the oculomotor nerve. It highlights that the presenting manifestations of acute disseminated encephalomyelitis can be subtle and vary; however, timely diagnosis and treatment result in complete recovery.Item The Children of Afghanistan need urgent mental health support.(Elsevier, 2022) Shoib, S.; Essar, M.Y.; Saleem, S.M.; Legris, Z.; Chandradasa, M.No abstract availableItem First paediatric live donor liver transplant in Sri Lanka with 1 year outcome : challenges for the future(The College of Surgeons of Sri Lanka, 2021) Siriwardana, R.; Thilakarathne, S.; Fernando, M.; Gunetilleke, M.B.; Weerasooriya, A.; Appuhamy, C.INTRODUCTION: Liver transplantation in the paediatric age group is demanding due to smaller body proportions and physiology. This paper describes the first successful paediatric liver transplant in Sri Lanka along with its one-year outcome. Describing the challenges faced during the process, we highlight the factors that need to be considered for a sustainable programme in the future. METHODOLOGY: A 9-year-old girl who had progressive familial intra hepatic cholestasis type 3 was referred to us with features of end stage liver disease. She was identified as a suitable candidate for liver transplantation. Her 38-year-old mother was selected as the donor, who was evaluated for suitability of a left lateral segment donation. RESULTS: The first paediatric liver transplantation was performed in July 2020. The child's mother donated the left lateral segment weighing 325g. During the postoperative period the child developed outflow tract obstruction at the hepatic venous anastomosis. This was managed with a percutaneously placed stent. Six months after transplant, she developed an acute rejection that required steroids. Treatment of rejection was complicated with multiple liver abscesses caused byAspergillus. The infection was treated with systemic antifungals and drainage. At one-year post transplant, the recipient had recovered from the trauma of surgery and had normal liver biochemistry, a patent hepatic venous stented anastomosis and complete resolution of the abscesses. We faced dual challenges in dealing with a live liver transplant donor and a young child who was the recipient. Our success, on this occasion, was underscored by the multidisciplinary contribution from specialists scattered across the island combined with state-public partnership. CONCLUSION: To offer a sustainable live donor liver transplant service for the future, many other aspects, beyond surgery itself, need to be addressed.Item Parental perceptions towards childhood stuttering in Sri Lanka.(Elsevier Scientific Publishing, 2022) Nonis, D.; Unicomb, R.; Hewat, S.Introduction: Parental perceptions towards stuttering is an important consideration as parents play a crucial role in the initial identification and management of stuttering in young children. Although several studies have been conducted on parental perceptions towards childhood stuttering in other countries, little is known about how stuttering is perceived and managed by parents in Sri Lanka. Aims: This study explored Sri Lankan parents' perceptions towards childhood stuttering and their experiences regarding attending speech and language therapy for stuttering. Methodology: Using a qualitative approach, 15 parents of children who stutter were recruited from a stuttering clinic at a state university in Sri Lanka. Parents participated in semi-structured interviews with the first author. The interviews were conducted via telephone in Sinhala language, recorded, transcribed verbatim in Sinhala and then translated into English. The data were analysed using thematic analysis. Results: Five themes emerged from the data: (1) limited knowledge about stuttering and management (2) influence of religion and culture on stuttering (3) variable responses to stuttering (4) impact of stuttering on the parent and child (5) impact and engagement in speech therapy. Conclusion: The findings highlighted the need to educate the Sri Lankan public about stuttering as a communication disorder and raise awareness about the profession of speech language therapy in the country. Specifically, it is important for other health professionals and teaching professionals to learn more about stuttering, so that appropriate early referrals can be made for speech and language therapy, lessening the impact on children and their families.