Selective Urokinase Inhibition Plus Chemotherapy: A Novel Approach to Pancreatic Cancer Treatment via Enhanced Antitumor Immunity and Decreased Metastasis.

Background & Aims: Improving outcomes for pancreatic ductal adenocarcinoma (PDAC) is critical, as current therapies offer limited benefits for most patients. The urokinase plasminogen activator (uPA) system is key in PDAC progression, driving invasion and metastasis and mediating interactions between pancreatic stellate cells (PSCs) and cancer cells. We previously developed BB2-30F, a potent uPA-selective inhibitor.

-Related Epilepsy: From Self-Limited Infantile Epilepsy to Atypical Epilepsy Phenotypes.

Background And Objectives: Pathogenic variants in the gene cause self-limited infantile epilepsy (SeLIE). Recently, atypical epilepsy phenotypes have been described. We explore the phenotypic spectrum of -related epilepsy through international collaboration.

Heimler Syndrome: A Report of 2 Indian Children With Review of Literature.

IntroductionHeimler syndrome 1 is a group of peroxisomal biogenesis disorders due to the pathogenic variations in the peroxisomal biogenesis factor 1 () gene resulting in the dysfunction of intracellular peroxisomes. gene encodes proteins that are involved in the import of peroxisomal matrix proteins.PatientsA 6-year-old boy, second born to nonconsanguineous parents, presented with global developmental delay, progressive hearing loss, and night blindness.

Applicability of the International League Against Epilepsy (ILAE) 2022 diagnostic criteria for epilepsy syndromes in children: A retrospective review of 1550 children with epilepsy.

Objective: Recently, the ILAE Nosology and Definitions Task Force defined diagnostic criteria for epilepsy syndromes. There is paucity of data on the use of these new diagnostic criteria in children with epilepsy, and how these criteria may lead to changes from previous practice.

Methods: This was a retrospective chart review of data of children attending the epilepsy clinic in a tertiary care children's hospital from January 2011 to January 2023.

Familial hemiplegic migraine in Indian children-a tertiary center experience.

Familial hemiplegic migraine (FHM), an autosomal dominant subtype of hemiplegic migraine, is a channelopathy presenting with severe headache, visual field defect, paresthesia, unilateral motor deficit, encephalopathy, seizures and aphasia. This cross-sectional study was conducted over 10 months in children aged 1-18 years suspected of hemiplegic migraine at a tertiary care pediatric hospital. Fourteen children were screened and five children with genetically confirmed FHM were included.

Primary Diffuse Leptomeningeal Melanomatosis in an Indian Child With Review of Literature.

Background: Primary diffuse leptomeningeal melanomatosis (PDLM) is an extremely rare, aggressive malignant neoplasia of the central nervous system. We report the first case of pediatric PDLM from India.

Methods: A review of literature was done to describe the 15 pediatric cases reported so far.

Teaching NeuroImage: Neuroimaging Features of Wilson Disease.

A 13-year-old boy of nonconsanguineous parents presented with abnormal body movements, gait difficulty, and slurring of speech for 2 years. On examination, he had rigidity, dystonia, dysarthria, and drooling. Ophthalmologic examination revealed bilateral Kayser-Fleischer rings.

Clinico-Etiologic Profile of Children and Adolescents with Drug-Resistant Epilepsy in a low-Resource Setting: 10 Years' Experience.

Nearly 25% to 30% of children with epilepsy develop drug-resistant epilepsy. Etiology of epilepsy, including drug-resistant epilepsy, varies with geographical region. Identifying paucity of etiologic data on drug-resistant epilepsy from our region and similar low-resource settings, we aimed to describe the clinical and etiologic profile of children and adolescents with drug-resistant epilepsy, to better inform region-specific concerns.

Mucocutaneous Manifestations in Children with Thalassemia: An Observational Study.

Thalassemia syndromes are a group of autosomal, recessively inherited, single-gene hemoglobinopathies with varied mucocutaneous manifestations. There is, however, a scarcity of these findings in the literature. This descriptive observational cross-sectional study was conducted to describe mucocutaneous manifestations in multi-transfused beta-thalassemia major children.

Recent Advances in Targeting the Urokinase Plasminogen Activator with Nanotherapeutics.

The aberrant proteolytic landscape of the tumor microenvironment is a key contributor of cancer progression. Overexpression of urokinase plasminogen activator (uPA) and/or its associated cell-surface receptor (uPAR) in tumor versus normal tissue is significantly associated with worse clinicopathological features and poorer patient survival across multiple cancer types. This is linked to mechanisms that facilitate tumor cell invasion and migration, via direct and downstream activation of various proteolytic processes that degrade the extracellular matrix─ultimately leading to metastasis.

Spectrum of Movement Disorders Among Children With Subacute Sclerosing Panencephalitis: A Cross-Sectional Study.

Subacute sclerosing panencephalitis is a progressive devastating condition due to persistence of mutant measles virus, affecting children and adolescents, characterised by myoclonus, seizures, and neuropsychiatric issues. Movement disorders apart from myoclonus are reportedly uncommon. We aimed to describe frequency and proportion of movement disorders among children with subacute sclerosing panencephalitis, hypothesizing that these occur more frequently than previously reported.

The Urokinase Plasminogen Activation System in Pancreatic Cancer: Prospective Diagnostic and Therapeutic Targets.

Pancreatic cancer is a highly aggressive malignancy that features high recurrence rates and the poorest prognosis of all solid cancers. The urokinase plasminogen activation system (uPAS) is strongly implicated in the pathophysiology and clinical outcomes of patients with pancreatic ductal adenocarcinoma (PDAC), which accounts for more than 90% of all pancreatic cancers. Overexpression of the urokinase-type plasminogen activator (uPA) or its cell surface receptor uPAR is a key step in the acquisition of a metastatic phenotype via multiple mechanisms, including the increased activation of cell surface localised plasminogen which generates the serine protease plasmin.