Efficacy and safety of oral cyclophosphamide versus mycophenolate mofetil in childhood nephrotic syndrome: an open-label comparative study.

Introduction: There is a scarcity of research comparing the efficacy of cyclophosphamide and mycophenolate mofetil in childhood nephrotic syndrome. The aim was to evaluate the efficacy and safety of oral cyclophosphamide (CYC) and mycophenolate mofetil (MMF) in children with steroid-sensitive nephrotic syndrome in terms of the proportion of children who have been off steroids for at least 6 months without proteinuria (responders).

Methods: This open-label retrospective-prospective comparative study was conducted in a pediatric nephrology clinic of a referral center for children between 1 and 18 years of age with FR/SD nephrotic syndrome.

Prevalence, Severity, and Determinants of Pain in Thalassemia.

As the life expectancy in thalassemia is improving, pain is being recognized as an emerging problem. To document the pain prevalence and severity in patients with transfusion-dependent thalassemia all transfusion-dependent thalassemia patients >10 years of age ( = 165) attending the Thalassemia Day Care Center were assessed for pain prevalence, severity, and its effect on various life activities using the Brief Pain Inventory. Their medical records were reviewed for the presence of various co-morbidities.

Adverse Effects of COVID-19 Vaccination in Patients with Transfusion-Dependent Thalassemia: An Observational Study from a Tertiary Care Center in Punjab, India.

Vaccination against SARS-CoV-2 is the most important weapon in the arsenal in the battle against COVID-19. There is concern about an increased risk of adverse effects in patients with transfusion-dependent thalassemia (TDT), which affects vaccine acceptance. A predesigned questionnaire was used to evaluate adverse effects (local/systemic within 90 days after vaccination) in participants >18 years of age with TDT.

Spectrum of magnetic resonance abnormalities in leigh syndrome with emphasis on correlation of diffusion-weighted imaging findings with clinical presentation.

Background: Leigh syndrome (LS) is a progressive neurodegenerative disorder of infancy/early childhood secondary to mitochondrial dysfunction. Imaging plays a pivotal role in the diagnosis of LS with certain typical magnetic resonance imaging (MRI) findings considered as a part of diagnostic criteria. We appraised various MRI findings on conventional MRI sequences and also assessed potential correlation between diffusion abnormalities and patient's clinical presentation.

Osteomalacic Myopathy in Children and Adolescents with Vitamin-D Deficiency.

Background: Osteomalacic myopathy secondary to vitamin-D deficiency is an under-recognized cause of muscle weakness in children and adolescents.

Aim: To describe a cohort of children and adolescents with osteomalacic myopathy.

Settings And Design: Pediatric neurology unit of a tertiary care hospital.

Considerations for Peripheral Blood Stem Cell Apheresis in a Low Body Weight Infant.

Introduction: Peripheral blood stem cell (PBSC) apheresis in infants (<10 kg body weight) requires specific precautions to prevent periprocedural complications.

Case Report: A 9 month old child was diagnosed with high-risk neuroblastoma and planned for autologous stem cell transplantation after induction chemotherapy. We illustrate the precautions and technical details observed while performing PBSC collection in this patient.

Comparison of Noninvasive Oscillometric and Intra-Arterial Blood Pressure Measurements in Children Admitted to the Pediatric Intensive Care Unit.

Intra-arterial blood pressure (IABP) measurement, although considered the gold standard in critically ill children, is associated with certain risks and lacks widespread availability. This study was conducted to determine the differences and agreements between oscillometric non-invasive blood pressure (NIBP) and invasive IABP measurements in children. Inclusion criteria consisted of children (from 1 month to 18 years) admitted to the pediatric intensive care unit (PICU) of a teaching hospital who required arterial catheter insertion for blood pressure (BP) monitoring.

Comparison of Clinical Features and Outcome of Dengue Fever and Multisystem Inflammatory Syndrome in Children Associated With COVID-19 (MIS-C).

Objective: To identify clinical and laboratory features that differentiate dengue fever patients from MIS-C patients and determine their outcomes.

Methods: This comparative cross-sectional study was done at a tertiary care teaching institute. We enrolled all hospitalized children aged 1 month - 18 years and diagnosed with either MIS-C and/or dengue fever according to WHO criteria between June and December, 2020.

Oral cyclophosphamide therapy in 100 children with steroid-sensitive nephrotic syndrome: experience from a developing country.

Background: Oral cyclophosphamide (CYP) is an important therapeutic agent in treatment of steroid-sensitive nephrotic syndrome having a steroid-dependent (SD) or frequent relapsing (FR) course. This retrospective observational study aimed to determine response to oral CYP and factors associated with positive response in these patients.

Methods: We studied 100 children (male; 75) with FR (19%) and SD (81%) NS treated with CYP in the Pediatric Nephrology clinic.

Body image issues and self-concept dilemmas in adolescents living with thalassemia.

Thalassemia, a genetic blood disorder, involves an inability to produce sufficient hemoglobin and comprises two types: alpha thalassemia and beta thalassemia. Beta thalassemia's immediate treatment measures include frequent blood transmissions, stem cell and bone marrow transplants; all capable of altering an individual's idea of body image, self-concept, growth, and socialization, resulting in several emotional, psychological, and behavioral concerns. This study aimed at comprehending the dilemmas of body image and self-concept encountered by adolescents with thalassemia, particularly the resulting influence on physical development and socialization.

Adherence to Iron Chelation Therapy and Its Determinants.

: Thalassemia is a chronic disease requiring lifelong treatment. The adherence to regular iron chelation therapy is important to ensure complication-free survival and good quality of life. The study aim to assess the adherence to iron chelation therapy (ICT) in patients with transfusion-dependent thalassemia (TDT), evaluate various causes of non-adherence and study the impact of non-adherence on the prevalence of complications secondary to iron overload.

A Concise Review on the Frequency, Major Risk Factors and Surveillance of Hepatocellular Carcinoma (HCC) in β-Thalassemias: Past, Present and Future Perspectives and the ICET-A Experience.

Due to the recent alarming increase in the incidence of hepatocellular carcinoma (HCC) in thalassemias, the present report reviews briefly the frequency, the major risk factors, and the surveillance of HCC in β-thalassemias. Over the past 33 years, 153 cases of HCC were reported in patients with thalassemia, mainly in Italy and Greece. Among HCV-infected patients, additional factors promoting the development of HCC included: advanced age, male sex, chronic hepatitis B (CHB) co-infection, and iron overload.

Marital status and paternity in patients with Transfusion- Dependent Thalassemia (TDT) and Non Transfusion-Dependent Thalassemia (NTDT): an ICET - A survey in different countries.

Background: More than five decades ago, thalassemia major (TDT) was fatal in the first decade of life. Survival and quality of life have improved progressively thanks to the implementation of a significant advance in diagnostic and therapeutic methods, consisting mainly of a frequent transfusion program combined with intensive chelation therapy. Improvement also includes imaging methods used to measure liver and cardiac iron overload.

An ICET-A survey on occult and emerging endocrine complications in patients with β-thalassemia major: Conclusions and recommendations.

In adult thalassemia major (TM) patients, a number of occult and emerging endocrine complications, such as: central hypothyroidism (CH), thyroid cancer, latent hypocortisolism, and growth hormone deficiency (GHD) have emerged and been reported. As the early detection of these complications is essential for appropriate treatment and follow-up, the International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescent Medicine (ICET-A) promoted a survey on these complications in adult TM patients, among physicians (pediatricians, hematologists and endocrinologists) caring for TM patients in different countries. The data reported by 15 countries are presented.

Utilization of the renal angina index in PICU of a developing country for prediction of subsequent severe acute kidney injury.

Background: Acute kidney injury (AKI) is independently associated with worsened morbidity and increased mortality in the pediatric intensive care unit (PICU). AKI risk score, termed renal angina index (RAI) is used in developed countries to predict subsequent severe AKI. Very few studies have investigated application of RAI in the PICU of a developing country.

Cost Effectiveness of Hematopoietic Stem Cell Transplantation Compared with Transfusion Chelation for Treatment of Thalassemia Major.

Hematopoietic stem cell transplantation (HSCT) is the only cure for thalassemia major (TM), which inflicts a significant 1-time cost. Hence, it is important to explore the cost effectiveness of HSCT versus lifelong regular transfusion-chelation (TC) therapy. This study was undertaken to estimate incremental cost per quality-adjusted life-year (QALY) gained with the intervention group HSCT, and the comparator group TC, in TM patients.

An ICET- A survey on Hypoparathyroidism in Patients with Thalassaemia Major and Intermedia: A preliminary report.

Hypoparathyroidism (HPT) is a rare disease with leading symptoms of hypocalcemia, associated with high serum phosphorus levels and absent or inappropriately low levels of parathyroid hormone (PTH). In patients with thalassemias it is mainly attributed to transfusional iron overload, and suboptimal iron chelation therapy. The main objectives of this survey were to provide data on the prevalence, demographic and clinical features of HPT in thalassemia major (TM) and intermedia (TI) patients living in different countries, and to assess its impact in clinical medical practice.

Paediatric brainstem: A comprehensive review of pathologies on MR imaging.

The brainstem is a midline structure formed by the midbrain, pons and medulla and is a home for various vital neurological centres of the human body. A diverse spectrum of disease entities can involve the brainstem, which includes infections, metabolic disorders, demyelination, vascular conditions, neurodegenerative disorders and tumours. Brainstem involvement can be primary or secondary, i.

Hemiconvulsion-hemiplegia-epilepsy syndrome: clinical course and neuroradiological features in a 20-month-old girl.

Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome involves initial sudden and prolonged unilateral convulsive seizures, followed by transient or permanent hemiplegia and epilepsy during infancy or early childhood. Seizures are prolonged, difficult to control and sometimes may require surgery. Hemiplegia varies in intensity, differs from Todd paralysis and disappears in about 20% of cases.

Combating inadequate anesthesia in periapical infections, with sodium bicarbonate: a clinical double blind study.

Introduction: Local anesthetics are generally much less effective when administered in inflamed tissues.

Purpose: This study was conducted to validate the addition of sodium bicarbonate in local anesthetics to increase its effectiveness as local infiltrations in teeth associated with periapical infections.

Methods: Two hundred subjects requiring extraction of maxillary teeth with periapical infections were enrolled.

Complications of exodontia: a retrospective study.

Purpose: The purpose of this study was to analyze the incidence of various complications following routine exodontia performed using fixed protocols.

Materials And Methods: A total of 22,330 extractions carried out in 14,975 patients, aged between 14 and 82 years, who reported to the Department of Oral and Maxillofacial Surgery at Padmashree Dr. D.