Epidemiological patterns and trends of paediatric snakebites in Sri Lanka.

Objectives: This study aimed to analyse the epidemiological patterns of paediatric snake bites in Sri Lanka over a 4-year period (2020-2024).

Methods: A multi-centre, retrospective observational study was conducted from June 2020 to June 2024 across nine governmental hospitals in seven provinces of Sri Lanka. Data were collected based on 757 children presenting with snake bites.

Management strategies for common viral infections in pediatric renal transplant recipients.

Viral infections have been considered as a major cause of morbidity and mortality after kidney transplantation in pediatric cohort. Children are at high risk of acquiring virus-related complications due to immunological immaturity and the enhanced alloreactivity risk that led to maintenance of high immunosuppressive regimes. Hence, prevention, early detection, and prompt treatment of such infe ctions are of paramount importance.

Expanding the phenotype of DYNC1H1-associated diseases with a rare variant resulting in spinal muscular atrophy with lower extremity predominance (SMA-LED) and upper motor neuron signs.

Background: Spinal muscular atrophy with lower extremity predominance (SMA-LED) is an autosomal dominant disorder. Since SMA-LED affects lower motor neurons, the disease is characterized by weakness and atrophy of lower limb muscles. We present a familial case series of SMA-LED with upper motor neuron signs associated with a rare variant in DYNC1H1.

Atypical Kawasaki disease presenting with macroscopic hematuria in an infant: a case report.

Background: Kawasaki disease is an acute febrile condition in children. It affects mainly children under 5 years old, and is known to cause coronary artery abnormalities if treatment is delayed. The diagnosis rests mainly on clinical criteria.

Arthrogryposis multiplex congenita in a child with congenital fractures: a case report.

Background: Bruck syndrome is an exceedingly rare form of osteogenesis imperfecta, inherited autosomal recessively and presenting with the concurrence of bone fragility and congenital contractures of large joints. The disease usually progresses relentlessly to result in recurrent fractures, short stature, severe kyphoscoliosis, and susceptibility to recurrent respiratory tract infections.

Case Presentation: The index child was a male newborn to healthy, nonconsanguineous, Sinhalese parents.

Acute appendicitis during the recovery phase of dengue hemorrhagic fever: two case reports.

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.

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.

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.

Autoimmune hepatitis and acquired partial lipodystrophy.

The lipodystrophies are an extremely rare group of metabolic conditions which are categorised based on their pathogenesis and phenotype. While primarily known for the striking loss of subcutaneous adipose tissue which they induce, they may also be associated with significant liver injury. In most cases, this results from the secondary deposition of lipid within hepatic parenchyma and is seen predominantly in generalised lipodystrophy.

Unexplained Tachypneoa and Severe Metabolic Acidosis in a Three-Month-Old Child: A Rare Presentation of Beta-Ketothiolose Deficiency.

Beta-ketothiolase (mitochondrial acetoacetyl-CoA thiolase, T2) deficiency is a rare inborn error of metabolism that is characterized by impaired metabolism of ketones and isoleucine. The condition is inherited as an autosomal recessive disorder. Herein, we present a child with T2 deficiency from Mahaoya, Eastern Province, Sri Lanka.

Fifteen-minute update: International normalised ratio as the treatment end point in children with acute paracetamol poisoning.

Paracetamol is one of the most frequent reasons for poisonings across the UK with an estimated 90,000 patients and 150 deaths annually. International normalised ratio (INR) may be elevated due to hepatocellular damage and is frequently used to monitor progress on N-acetyl cysteine. N-acetyl cysteine is associated with reduced activity of vitamin K dependent clotting factors leading to a benign elevation of INR.

Fifteen-minute consultation: Evaluation of paediatric torticollis.

Torticollis refers to a state in which the neck is twisted due to excessive contraction or shortening of the muscles on one side. Congenital muscular torticollis, which is more common than acquired torticollis, has an incidence of 0.3%-1.

Approach to the Adolescent With Substance Use in the Acute Setting.

Psychoactive substance use during adolescence is an emerging challenge to the public health system due to the potential negative impact on the emotional, cognitive, social, physical and academic outcomes of adolescents. An increase in autonomy during adolescence, peer influence and willingness to experiment, lead to taking high-risk decisions subsequently prompting towards substance abuse and alcohol use. Substance use is heterogeneous among adolescents, which differs with availability, perceptions of use and specific drug usage.

Review on Pneumococcal Infection in Children.

Childhood pneumococcal infection is a growing concern among paediatricians especially, in countries where there is no routine vaccination program against . The disease is associated with significant morbidity and mortality in young children particularly those who are under the age of two years. Its main virulent factors include polysaccharide capsule, autolysin, pneumolysin, choline-binding Protein A, the higher chance for genetic transformation, and the presence of pilli that facilitate enhanced binding of bacteria to host cellular surfaces.

Effect of Prior Symptomatic Dengue Infection on Dengue Haemorrhagic Fever (DHF) in Children.

Pathogenesis of dengue haemorrhagic fever is not fully understood, but it is thought that there is antibody enhancement during the secondary infection, which causes severe dengue haemorrhagic fever (DHF). Therefore, patients who have DHF should have a documented history of symptomatic dengue infection in the past. A retrospective descriptive-analytical study was conducted at the University Paediatric Unit at Lady Ridgeway Hospital for Children, Colombo, Sri Lanka.

Retrospective Study of Clinico-Aetiological Factors of Chronic Urticaria Among Children Attending a Tertiary Care Paediatric Centre in Eastern Province of Sri Lanka.

Introduction Chronic urticaria is one of the growing problems worldwide and the prevalence is increasing. Around 2% of children have been shown to be affected with chronic urticaria. Objectives To evaluate demographic, clinical and aetiological factors related to chronic urticaria in children and to assess investigations and treatment.

Postdengue chronic fatigue syndrome in an adolescent boy.

Chronic fatigue syndrome (CFS) is often preceded by a viral illness and has recurrent 'flulike' symptoms which include a wide spectrum of musculoskeletal and neurological clinical features. The condition is also known as myalgic encephalomyelitis and systemic exertional intolerance syndrome. CFS has been reported following dengue among adult patients.

Clinical Profile, Risk Factors and Outcomes of Children With Cutaneous Larva Migrans Infection: A Hospital-Based Study.

Background Cutaneous larva migrans (CLM) is a neglected parasitic skin disease caused by hookworm larvae such as and . It is more common in tropical and subtropical countries. Evidence regarding clinical profiles, risk factors and outcomes of children with CLM in the Eastern province of Sri Lanka is scarce.

Early-Onset Multiple Sclerosis With Frequent Relapses: A Challenging Diagnosis With a Less Favorable Prognosis.

Pediatric multiple sclerosis (MS) is a rare demyelinating disease of the brain, spinal cord, and optic nerve caused by immune modulators mediating against the neuronal axons of the central nervous system. MS is usually characterized by a series of neurological events, without any features of encephalopathy, separated in time and space. The complications arise from the permanent degeneration of the nerves.

A Study on Digit Sucking Among Children Presented to a Tertiary Care Paediatric Clinic in Sri Lanka.

Background Sucking habits are common among children, especially in early childhood. There is, however, controversy about its prevalence probably due to under-reporting. The objective of this study is to analyze the demographic factors, reasons for clinic attendance, and treatment options chosen by parents and health care providers to control digit sucking.

Child with dengue haemorrhagic fever complicated by ileocaecal intussusception.

Dengue fever is one of the most common neglected tropical diseases with an increasing trend seen in Sri Lanka and many other tropical countries. A number of unusual presentations and complications of dengue fever have been reported, and acute abdomen is one such presentation. However, intussusception as the cause of acute abdomen in a patient with dengue haemorrhagic fever has not been previously reported.

Multiple aplasia cutis congenita type V and fetus papyraceous: a case report and review of the literature.

Background: Aplasia cutis congenita is regarded as congenital focal absence of skin in the newborn, and occurrence of more than three similar skin defects is rare. The etiology is thought to be multifactorial, and precise etiopathogenesis is unknown.

Case Presentation: A 13-day-old newborn Sri Lankan Tamil girl was referred to the dermatologic clinic with multiple skin defects at birth.

PHACE Syndrome Presenting With Retinal Degeneration, Cortical Dysplasia, Microphthalmia, and Atrial Septal Defect in a South Asian Boy.

PHACE syndrome is a rare disorder of vasculogenesis that occurs during the first trimester of pregnancy. The disorder commonly presents with posterior fossa brain anomalies and coarctation of aorta/arterial anomalies and is predominantly seen in female patients. Herein, we report a male child with PHACE syndrome who had several less common features such as cortical dysplasia, retinal degeneration, and microphthalmia.

Exome sequencing detected an extremely rare case of foetal onset familial haemophagocytic lymphohistiocytosis type 5 presenting with hydrops foetalis.

Background: Familial hemophagocytic lymphohistiocytosis (FHL) is a genetically heterogeneous autosomal recessive hyper-inflammatory syndrome which needs early accurate diagnosis and appropriate treatment to prevent complications and early mortality. Recently, it was reported that mutations in STXBP2 gene are linked to FHL type 5 (FHL-5).

Case Presentation: We report a Sri Lankan neonate who presented with low Apgar scores at birth, abdominal distension, and hepatosplenomegaly, followed by lethargy, poor sucking and rapid decompensation with wide spread activation of inflammation within 48 h of birth.

Primary Hypertension as the Presenting Feature of Laurence-Moon-Bardet-Biedl Syndrome: A Report of Two Children.

Laurence-Moon-Bardet-Biedl syndrome (LMBBS) is a rare ciliopathic genetic multisystem disorder. It has several primary and secondary clinical features, which include central obesity, retinitis pigmentosa, mental subnormalities, polydactyly, and renal dysfunction. The majority of children may not show all clinical features at birth, and clinical features can develop progressively over the years.

Subgaleal Abscess Following Staphylococcal Cellulitis in a 10-Month-Old Child: A Diagnostic Challenge for the Clinician.

Subgaleal abscess is an extremely rare and unusual presentation of head and neck infections and has been reported to occur following scalp infections, head trauma, sinusitis, septicemia, scalp monitoring, and surgical interventions. We report a 10-month-old child who presented with cellulitis around a pustule on the right forehead of three days duration. It was initially treated with oral cloxacillin for five-days.