Clinical features and outcomes of patients with diacylglycerol kinase epsilon nephropathy: a nationwide experience.

Background: Thrombotic microangiopathy (TMA) is usually caused due to dysregulation of the alternative complement pathway. Rarely, thrombotic microangiopathy is caused by non-complement mediated mutations in diacylglycerol kinase epsilon (DGKE); information about therapy and outcome of these patients is limited.

Methods: Medical records of patients, younger than 18 years, diagnosed with TMA and variants in DGKE were reviewed to include 12 patients from seven centers.

Sodium intake and urinary losses in children on dialysis: a European multicenter prospective study.

Background: Sodium (Na) balance is unexplored in dialyzed children. We assessed a simplified sodium balance (sNaB) and its correlates in pediatric patients receiving maintenance dialysis.

Methods: Patients < 18 years old on hemodialysis (HD) or peritoneal dialysis (PD) in six European Pediatric Dialysis Working Group centers were recruited.

Surveillance of aetiologies, clinical presentation, and most common types of epilepsy among paediatric patients at a tertiary care hospital in Pakistan.

Epilepsy is the third most common neurological disease in the world associated with a high frequency in the paediatric age group. This study aims to evaluate the prevalence, types and aetiologies of epilepsy within the Pakistani population. A retrospective review of the charts of all patients, below the age of 18 years, presenting with epilepsy to the Department of Neurology at The Children's Hospital and Institute of Child Health, Lahore, from January 2016 to December 2020, was carried out.

Lessons learned during the war in Ukraine: a report from the Renal Disaster Relief Task Force of the ERA.

People living with kidney disease are among the most vulnerable at times of natural or man-made disasters. In addition to their unpredictable course, armed conflicts impose a major threat given the disruption of infrastructure, sanitation and access to food, water and medical care. The ongoing war in Ukraine has once more demonstrated the importance of preparedness, organization, coordination and solidarity during disasters.

Diagnosis and management of mineral and bone disorders in infants with CKD: clinical practice points from the ESPN CKD-MBD and Dialysis working groups and the Pediatric Renal Nutrition Taskforce.

Background: Infants with chronic kidney disease (CKD) form a vulnerable population who are highly prone to mineral and bone disorders (MBD) including biochemical abnormalities, growth retardation, bone deformities, and fractures. We present a position paper on the diagnosis and management of CKD-MBD in infants based on available evidence and the opinion of experts from the European Society for Paediatric Nephrology (ESPN) CKD-MBD and Dialysis working groups and the Pediatric Renal Nutrition Taskforce.

Methods: PICO (Patient, Intervention, Comparator, Outcomes) questions were generated, and relevant literature searches performed covering a population of infants below 2 years of age with CKD stages 2-5 or on dialysis.

The level and distribution of selected organochlorine pesticides in water of River Satluj Pakistan.

The study was aimed to identify different environmental factors (selected organochlorine pesticides) affecting the river water of Satluj. River Sutlej is about 1400 kilometers long and its water is extensively used for irrigation in Punjab, located both in India and Pakistan, which was also a reason of dispute between both countries for its water share. The study area was divided into four zones, Sulemanki Zone, Islam Zone, Mailsi Syphone Zone and Panjnad Zone.

Gutted: constipation in children with chronic kidney disease and on dialysis.

Functional constipation is a common problem in otherwise healthy children. Children with chronic kidney disease (CKD) and on dialysis have additional disease-related risk factors including the uremic milieu, fluid and dietary restrictions, and decreased physical activity, as well as treatment-related risk factors such as dialysis therapy and polypharmacy that contribute to and compound the problem. Constipation causes significant distress for children and their caregivers.

Assessment of the oral health literacy and oral health behaviors among nurses in China: a cross-sectional study.

Purpose: Oral health is important for general health; nurses are expected to possess good oral health awareness and work together for public oral health promotion. The purpose of this study is to investigate oral health literacy (OHL)and oral health behaviors of nurses, and explore the association between oral health literacy with demographic variables and oral health behaviors.

Methods: A cross-sectional study in a tertiary hospital was conducted using a short-form Health Literacy in Dentistry-14 (HeLD-14) and a 16-items oral health behaviors questionnaire.

Intraoperative Needle Tip Tracking with an Integrated Fibre-Optic Ultrasound Sensor.

Ultrasound is an essential tool for guidance of many minimally-invasive surgical and interventional procedures, where accurate placement of the interventional device is critical to avoid adverse events. Needle insertion procedures for anaesthesia, fetal medicine and tumour biopsy are commonly ultrasound-guided, and misplacement of the needle may lead to complications such as nerve damage, organ injury or pregnancy loss. Clear visibility of the needle tip is therefore critical, but visibility is often precluded by tissue heterogeneities or specular reflections from the needle shaft.

Intensive compared with less intensive blood pressure control to prevent adverse cardiac remodelling in children with chronic kidney disease (HOT-KID): a parallel-group, open-label, multicentre, randomised, controlled trial.

Background: Optimal target blood pressure to reduce adverse cardiac remodelling in children with chronic kidney disease is uncertain. We hypothesised that lower blood pressure would reduce adverse cardiac remodelling.

Methods: HOT-KID, a parallel-group, open-label, multicentre, randomised, controlled trial, was done in 14 clinical centres across England and Scotland.

Presence of selected organochlorine pesticides (OCPs) in sediments and biota of River Satluj, Pakistan: first report.

In the developed countries, the use of OCPs (organochlorine pesticides) has been banned. However, in South Asia several of them are still in use. In Pakistan and India a constant addition of OCPs into the atmosphere has been indicated by various researchers.

Burnout Among Postgraduate Residents Using Copenhagen Burnout Inventory.

Background: Generally, the healthcare workers have a stressful job and it includes both physical and mental stress. We conducted this study to determine the burnout among postgraduate residents (PGR's). .

Blood Lactate Levels And Lactate Clearance As Predictors Of Mortality In Neonatal Sepsis.

Background: Sepsis is life-threatening organ dysfunction caused by dysregulated host response to infection. Aim of the study is Neonatal sepsis refers to infection involving the blood stream in neonates. It is major health problem causing neonatal mortality and morbidity in developing countries.

Establishing Height-for-Age Z-Score Growth Reference Curves and Stunting Prevalence in Children and Adolescents in Pakistan.

Height-for-age Z-score (HAZ) curves are widely used for detecting children with stunting. The aim of this study was to provide smoothed HAZ growth reference values and their curves for Pakistani children and adolescents aged 2-18 years. The prevalence of stunting in Pakistani children was determined.

Management of pediatric dialysis and kidney transplant patients after natural or man-made disasters.

Pediatric patients on kidney replacement therapy (KRT) are among the most vulnerable during large-scale disasters, either natural or man-made. Hemodialysis (HD) treatments may be impossible because of structural damage and/or shortage of medical supplies, clean water, electricity, and healthcare professionals. Lack of peritoneal dialysis (PD) solutions and increased risk of infectious/non-infectious complications may make PD therapy challenging.

Risk factors evaluation of cerebral palsy in Hazara division Khyber Pakhtunkhwa Pakistan: A cross-sectional survey.

Objective: To evaluate the prevalence of risk factors of cerebral palsy in the mothers of children with cerebral palsy.

Methods: The cross-sectional study was conducted in 2018 at the Helping Hand Institute of Rehabilitation Sciences, Mansehra, Pakistan, and comprised mothers of cerebral palsy children aged 1-18 years from the Hazara Division. Data was collected by using a modified form of Surveillance of cerebral palsy in Europe questionnaire regarding prenatal, natal and postal natal risk factors.

AP4B1-associated hereditary spastic paraplegia: Expansion of clinico-genetic phenotype and geographic range.

Background: Hereditary spastic paraplegias (HSP) are a group of neurodegenerative diseases that present with weakness and stiffness in the lower limb muscles and lead to progressive neurological decline. Bi-allelic loss-of-function variants in genes that encode subunits of the adaptor protein complex 4 (AP-4) lead to complex HSP. This study aimed to identify causative genetic variants in consanguineous families with HSP from Azerbaijan and Pakistan.

Hemodiafiltration in the pediatric population.

Hemodiafiltration (HDF) is increasingly adopted as a safe and effective treatment compared to conventional hemodialysis (HD) in children. We describe the outcomes of prospective observational studies in children on HDF versus HD showing that HDF was associated with an attenuation of the cardiovascular risk profile, improved blood pressure control, reduced inflammation, improved bone health and growth, and most importantly, an improved health-related quality of life.

Armed conflicts and kidney patients: a consensus statement from the Renal Disaster Relief Task Force of the ERA.

During conflicts, people with kidney disease, either those remaining in the affected zones or those who are displaced, may be exposed to additional threats because of medical and logistical challenges. Acute kidney injury developing on the battlefield, in field hospitals or in higher-level hospital settings is characterized by poor outcomes. People with chronic kidney disease may experience treatment interruptions, contributing to worsening kidney function.

Endoscopic Retrograde Cholangiopancreatography in Bangladeshi Children: Experiences and Challenges in a Developing Country.

Purpose: Although endoscopic retrograde cholangiopancreatography (ERCP) has been used for more than five decades, its applicability in Bangladeshi children has recently become more common. Therefore, this manuscript aims to describe our experience in performing ERCPs in Bangladeshi children with hepatopancreaticobiliary diseases, focusing on presenting diseases, as well as the diagnostic and therapeutic efficacy.

Methods: Between 2018 and 2021, 20 children underwent 30 ERCP procedures at the Bangladesh Specialized Hospital, Dhaka.

Nutritional Calcium Supply Dependent Calcium Balance, Bone Calcification and Calcium Isotope Ratios in Rats.

Serum calcium isotopes (δCa) have been suggested as a non-invasive and sensitive Ca balance marker. Quantitative δCa changes associated with Ca flux across body compartment barriers relative to the dietary Ca and the correlation of δCa with bone histology are unknown. We analyzed Ca and δCa by mass-spectrometry in rats after two weeks of standard-Ca-diet (0.