Nutritional and Anthropometric Indices in Children Receiving Haemodiafiltration vs Conventional Haemodialysis - The HDF, Heart and Height (3H) Study.

Background: The "HDF-Heart-Height" study showed that haemodiafiltration (HDF) is associated with improved growth compared to conventional haemodialysis (HD). We report a post-hoc analysis of this study assessing the effect of extracorporeal dialysis therapies on nutritional indices.

Methods: 107 children were included in the baseline cross-sectional analysis, of whom 79 (43 HD, 36 HDF) completed the 12-month follow-up.

Bacteriological Spectrum and Antibiotic Susceptibility on Blood Culture in Newly Diagnosed Pediatric Patients With Acute Lymphoblastic Leukemia During the Induction Phase.

Background Acute lymphoblastic leukemia (ALL) is the most common cancer diagnosed in children worldwide. This study was conducted to find out the trends in the bacteriological spectrum and antibiotic susceptibility on blood culture in newly diagnosed children with acute lymphoblastic leukemia during the induction phase at a pediatric oncology unit in South Punjab, Pakistan. Methodology This cross-sectional study was conducted from January 1, 2020, to June 30, 2021.

Berardinelli Seip Syndrome: A rare case report.

Berardinelli Seip Congenital Lipodystrophy (BSCL) or Congenital Generalized Lipodystrophy (CGL) is one of the four subgroups of lipodystrophy syndrome which is characterized by varying degrees of loss of adipose mass in the body. It is an extremely rare autosomal recessive disorder and commonly reported clinical presentations include muscular hypertrophy, gigantism, hepatomegaly, impaired glucose tolerance, acanthosis nigricans, hypertriglyceridaemia, cardiomyopathy, intellectual impairment, bone cysts and phlebomegaly. We present a case of a 4.

Outcome of paediatric lobectomy by thoracotomy - a single centre, six-year experience.

This short report highlights the patient outcome of lung resections for congenital and acquired lung lesions in children who presented to the Liaquat National Hospital, Karachi, from January 2013 to April 2019. Common indications were congenital pulmonary airway malformation (CPAM), congenital lobar emphysema (CLE), and bronchiectasis. Two patients died whereas, 21 were discharged home.

Naturally occurring stable calcium isotope ratios are a novel biomarker of bone calcium balance in chronic kidney disease.

Dysregulated calcium homeostasis is common in chronic kidney disease and causally associated with disorders of bone mineralization. However, radiological measures and biomarkers do not allow accurate evaluation of bone calcium balance. Non-radioactive calcium isotopes, Ca and Ca, are present in our diet and sequestered into body compartments following principles of kinetic isotope fractionation.

An integrated multiomic approach as an excellent tool for the diagnosis of metabolic diseases: our first 3720 patients.

To present our experience using a multiomic approach, which integrates genetic and biochemical testing as a first-line diagnostic tool for patients with inherited metabolic disorders (IMDs). A cohort of 3720 patients from 62 countries was tested using a panel including 206 genes with single nucleotide and copy number variant (SNV/CNV) detection, followed by semi-automatic variant filtering and reflex biochemical testing (25 assays). In 1389 patients (37%), a genetic diagnosis was achieved.

Risk factors and outcomes of neonates with acute kidney injury needing peritoneal dialysis: Results from the prospective TINKER (The Indian PCRRT-ICONIC Neonatal Kidney Educational Registry) study.

Background: Acute kidney injury (AKI) is common in neonates admitted to neonatal intensive care units (NICUs). There is a need to have prospective data on the risk factors and outcomes of acute peritoneal dialysis (PD) in neonates. The use of kidney replacement therapy in this population compared to older populations has been associated with worse outcomes (mortality rates 17-24%) along with a longer stay in the NICU and/or hospital.

Clinical Characteristics and Etiology of Epilepsy in Children Aged Below Two Years: Perspective From a Tertiary Childcare Hospital in South Punjab, Pakistan.

Background Epilepsy is described as an enduring disposition toward recurrent unprovoked seizures and by the neurobiological, cognitive, psychological, and social consequences of this condition. This study aimed to find the clinical characteristics and etiology of epilepsy in children aged below two years. Methodology This cross-sectional study was conducted at the department of pediatric neurology, the Children's Hospital and Institute of Child Health, Multan, Pakistan, from February 2021 to July 2021.

Comparison of Efficacy and Safety of Low- Versus High-Dose Oral Prednisolone in Infantile Spasm (IS): An Open Label Randomized Controlled Trial at the Children's Hospital & Institute of Child Health, Multan, Pakistan.

Background: Infantile spasm (IS) is an epileptic syndrome characterized by epileptic spasms, hypsarrhythmia on electroencephalography (EEG), and high risk of neurodevelopmental regression. This study was done to compare the efficacy and safety of the high versus the usual dose in children with IS.

Methodology: This open label randomized controlled trial was conducted at Department of Pediatric Neurology, The Children's Hospital & Institute of Child Health, Multan, Pakistan, from January 1, 2020 to December 31, 2020.

Investigation of the relationship between serum sclerostin and dickkopf-1 protein levels with bone turnover in children and adolescents with type-1 diabetes mellitus.

Objectives: Diabetes mellitus (DM) is widely known to have a detrimental effect on bone health and is associated with increased fracture risk. Recently, the Wnt/beta-catenin signaling pathway and its inhibitors sclerostin and dickkopf-1 (Dkk-1) were found to be involved in the control of bone mass. The present study aimed to measure serum sclerostin and Dkk-1 protein levels in children and adolescents with type-1 DM and compare with other bone turnover markers and bone mineral density (BMD).

Mechanistic Impact of Zinc Deficiency in Human Development.

Zinc (Zn) deficiency in humans is an emerging global health issue affecting approximately two billion people across the globe. The situation prevails due to the intake of Zn deficient grains and vegetables worldwide. Clinical identification of Zn deficiency in humans remains problematic because the symptoms do not appear until impair the vital organs, such as the gastrointestinal track, central nervous system, immune system, skeletal, and nervous system.

A Large Hydatid Cyst in the Brain of a 10-year Child.

Hydatid cyst is the larval form of the parasite, echinococcus granulosus. We operated upon a case of a giant hydatid cyst in the left cerebral hemisphere of a 10-year male child. The patient presented to us with a history of headache, vomiting, vertigo and difficulty in walking.

Kleefstra Syndrome with Severe Sensory Neural Deafness and De Novo Novel Mutation.

Kleefstra syndrome is a rare inherited neuro-developmental condition characterised by facial dysmorphism, microcephaly, hypotonia, developmental delay, and intellectual disability. It is a rare syndrome; and less than 100 cases with different genetic mutations are reported so far. We report an eight-month baby boy with Kleefstra syndrome type 2 due to a novel de novo pathogenic mutation in the KMT2C (Lysine methyltransferase 2C) gene.

Implementation research on kangaroo mother care, Bangladesh.

Objective: To describe the implementation, coverage and performance of the national kangaroo mother care programme in Bangladesh.

Methods: Kangaroo mother care services for clinically stable babies with birth weight under 2000 g were set up in government-run health-care facilities in rural and urban areas of Bangladesh. Each facility provided counselling on kangaroo mother care, ensured adequate nutrition, and followed up mothers and babies.

Treatment-related mortality in children with acute lymphoblastic leukaemia in a low-middle income country.

Objective: To determine the proportion of treatment-related mortality among mortalities of paediatric acute lymphoblastic leukaemia and to identify probable causes and risk factors.

Methods: The observational retrospective study was conducted in February-March 2019 at the Department of Paediatric Haematology-Oncology and Bone Marrow Transplant, the Children's Hospital and the Institute of Child Health, Lahore, Pakistan, and comprised data of all paediatric patients of acute lymphoblastic leukaemia who expired during treatment from January 2017 till September 2018. Death due to relapse and deaths before treatment were excluded.

Very early onset inflammatory bowel disease: Spectrum of clinical presentation, diagnostic tools and outcome in children.

Objective: To explore the spectrum of presentation, underlying monogenetic defects and outcome in very early onset inflammatory bowel disease (VEO-IBD).

Method: The prospective, observational study was conducted at the Children's Hospital, Lahore, Pakistan, from January 2017 to December 2018, and comprised children developing features of inflammatory bowel disease aged <6 years. Data included demography, clinical presentation, diagnostic tools and outcome.

GABRA1 and GABRA6 gene mutations in idiopathic generalized epilepsy patients.

The GABA receptor is an important epilepsy-associated candidate gene, and has always been a focus in etiology and in the treatment of epilepsy. This study explores the genetic association between GABA receptor gene polymorphisms and epilepsy in a cohort of the Pakistani population. A case-control study was conducted on 150 patients with idiopathic generalized epilepsy (IGE) and 150 controls.

Hemodiafiltration Is Associated With Reduced Inflammation and Increased Bone Formation Compared With Conventional Hemodialysis in Children: The HDF, Hearts and Heights (3H) Study.

Background: Patients on dialysis have a high burden of bone-related comorbidities, including fractures. We report a analysis of the prospective cohort study HDF, Hearts and Heights (3H) to determine the prevalence and risk factors for chronic kidney disease-related bone disease in children on hemodiafiltration (HDF) and conventional hemodialysis (HD).

Methods: The baseline cross-sectional analysis included 144 children, of which 103 (61 HD, 42 HDF) completed 12-month follow-up.

Knowledge, attitude, and practice of clinicians about antimicrobial stewardship and resistance among hospitals of Pakistan: a multicenter cross-sectional study.

Considering that antimicrobial resistance (AMR) is a global challenge, there is a dire need to assess the knowledge, attitude, and practice (KAP) of clinicians in AMR endemic countries. The current multicenter, cross-sectional study aimed at highlighting gaps in antimicrobial (AM) stewardship and AMR among practicing doctors working in public tertiary care teaching hospitals of Lahore, Pakistan. A KAP survey, based on a self-administered questionnaire containing 45 questions, was distributed among 336 clinicians in 6 randomly selected hospitals.

Kidney involvement in multisystem inflammatory syndrome in children: a pediatric nephrologist's perspective.

The initial report of the multisystem inflammatory syndrome in children (MIS-C) was from the UK in April 2020; since then, cases have been reported worldwide. Renal involvement has been seen commonly, ranging from 10% to 46%. Kidney involvement following severe acute respiratory syndrome coronavirus 2 infection in children with MIS-C is more common than initially thought and is associated with higher morbidity and mortality.

Headache in Children and Adolescents: A Focus on Uncommon Headache Disorders.

Migraine and tension-type headache are common in children and adolescents, but several other headache disorders may pose a great challenge in diagnosis and management to families and attending clinicians. In this review, we highlight several of these disorders, which need appropriate assessment to make the right diagnosis and appropriate investigations where necessary. Timely recognition and implementation of appropriate management strategies can improve the health of children with some disorders, and is vital in achieving improvement in the quality of life.

Developing neck circumference growth reference charts for Pakistani children and adolescents using the lambda-mu-sigma and quantile regression method.

Objective: Neck circumference (NC) is currently used as an embryonic marker of obesity and its associated risks. But its use in clinical evaluations and other epidemiological purposes requires sex and age-specific standardised cut-offs which are still scarce for the Pakistani paediatric population. We therefore developed sex and age-specific growth reference charts for NC for Pakistani children and adolescents aged 2-18 years.