Complex Presentation of Congenital Heart Block and Coexisting Congenital Heart Disease in a One-Year-Old Girl: A Case Report.

Congenital complete heart block (CCHB) is a rare and potentially life-threatening condition, often associated with maternal autoantibodies. We present the case of a one-year-old girl with recurrent respiratory symptoms, ultimately diagnosed with CCHB and congenital heart disease. She exhibited bradycardia and signs of congestive heart failure. A diagnostic workup revealed significant cardiac abnormalities, including dilated chambers, ventricular septal defect, and patent ductus arteriosus. Serological tests for maternal autoantibodies were negative. The child's parents opted for discharge without surgical intervention. This case underscores the importance of comprehensive evaluation and management strategies in patients with congenital heart block, particularly in resource-limited settings.

Sprengel's Deformity: A Paediatric Case Report.

Sprengel's deformity is a conspicuous anomaly, affecting one or both scapulas. The congenital elevation of the scapula is frequently accompanied by additional anomalies, such as rib, vertebral, or muscular deformities, among which are rib fusion or vertebral deformity. Defects in the cervical vertebrae are most likely to result in Klippel-Feil syndrome, which is characterised by a short neck, restrictions on head mobility, and low-growing neck hair. Fewer than half of the instances had scoliosis, which is compensatory due to efforts to straighten the spine. The present case report was the case of Sprengel's deformity reported to our department.

Unlocking Vitality: A Comprehensive Review of Vitamin D's Impact on Clinical Outcomes in Critically Ill Children.

This comprehensive review explores the multifaceted role of vitamin D (VD) in critically ill children, examining its implications for clinical outcomes. Although this substance has long been known for its function in maintaining bone health, it is now becoming more widely known for its extensive physiological effects, which include immune system and inflammation regulation. Observational research consistently associates VD levels with outcomes like duration of hospitalization, mortality, and illness severity in critically ill pediatric patients. Mechanistically, it exerts anti-inflammatory and endothelial protective effects while modulating the renin-angiotensin system. Increasing VD levels through supplementation presents promise as a therapeutic strategy; however, further research is necessary to elucidate optimal dosage regimens and safety profiles. This review emphasizes the significance of comprehending the intricate relationship between VD and critical illnesses among pediatric populations.

An Atypical Presentation of Cerebellar Abscess: A Case Report.

An infratentorial abscess is a medical emergency. Common sources of abscesses are otogenic foci, sinusitis, or dental abscess, rarely congenital defects like dermoid cysts with sinus along with cerebrospinal axis can lead to infratentorial abscess. This case report describes a four-year-old girl with pus discharging from the occipital area. Radiological imaging revealed a cerebellar abscess with the sinus tract open exteriorly through an occipital cortical defect with obstructive hydrocephalus. The patient underwent neurosurgical intervention followed by antibiotic therapy. Histopathology of the tissue sample was suggestive of a dermoid cyst. Congenital defects should not be ignored. All newborns should have a thorough physical examination to identify birth defects. As these defects can cause life-threatening complications, early recognition with early surgical intervention is the treatment of choice.

Beyond Skin Deep: A Case Report of Infantile Systemic Hyalinosis in a Six-Month-Old Infant.

A rare autosomal recessive condition called infantile systemic hyalinosis (ISH) is characterized by early-onset skin lesions that progress to the formation of numerous contractures. The underlying disease is the progressive accumulation of hyaline substances in many tissues. We are presenting the case of a male infant who was referred for evaluation and management at the age of six months. The infant had a history of recurrent episodes of diarrhea and showed limited movement in all four limbs. Upon physical examination, hyperpigmented papulonodular lesions on bony prominences and perianal regions were found, coupled with contractures in the elbow and knee joints. Hyaline deposition in the mid-dermal region was confirmed by histopathological analysis of a skin biopsy sample. The baby also had acute otitis media, which needed to be treated with antibiotics. Parents were counseled regarding the disease's diagnosis, complications, prognosis, and inheritance pattern. This case highlights the clinical presentation, diagnostic process, and management strategies employed in the care of ISH, emphasizing the importance of early recognition and multidisciplinary management in mitigating its devastating effects.

Estimation of Prevalence of Thrombocytopenia in Cyanotic Congenital Heart Disease: A Cross-Sectional Study Among the Pediatric Population.

Background Congenital heart disease (CHD) is one of the leading causes of mortality in India, with the majority being attributed to cyanotic conditions. Hence, it is crucial to assess the factors that play a significant role in patient prognosis in heart defects of a child. The present cross-sectional study assessed the prevalence of thrombocytopenia in patients with cyanotic congenital heart defects (CCHD). The objectives of our study were to assess the levels of platelets in various cyanotic congenital heart defects and then infer the prevalence of thrombocytopenia in these patients as a whole.  Methodology The study population comprised children aged fifteen days to twelve years with two-dimensional (2D) echocardiography confirmation of CHD; those who were critically ill, had proven sepsis, and were not willing to participate in the study were excluded. Blood samples of enrolled patients were obtained and collected in ethylenediamine tetraacetic acid (EDTA) tubes for assessment. The prevalence was then calculated. Results were obtained and interpreted based on these observations. Result Out of 268 children with CHD, 52 reported thrombocytopenia, and the prevalence rate was found to be 19.4. The comparative analysis of thrombocytopenia showed a significant p-value only in cases with total anomalous pulmonary venous connection (TAPVC). Conclusion Patients with cyanotic congenital heart defects are often diagnosed with various hematological derangements, and while hemoglobin levels are usually seen to rise, significant thrombocytopenia is reported in these patients. The low platelet counts often pose a risk peri-surgically and can also affect the surgical outcomes of the patient. Therefore, it is imperative to study further the relationship between thrombocytopenia and an independent risk factor for patient prognosis in patients of CCHD.

Illuminating Progress: A Comprehensive Review of the Evolution of Phototherapy for Neonatal Hyperbilirubinemia.

This comprehensive review thoroughly examines the historical evolution, physiological foundations, and contemporary advancements in the application of phototherapy for neonatal hyperbilirubinemia. Neonatal hyperbilirubinemia, a common condition resulting from the immature hepatic processes in newborns, poses potential risks, including neurotoxicity, if left untreated. The review traces the historical progression from early recognition of neonatal jaundice to the development of various phototherapy modalities, showcasing the dynamic landscape of neonatal care. Emphasizing the physiological intricacies of bilirubin metabolism in neonates, the study underscores the vulnerability of newborns to hyperbilirubinemia due to delayed hepatic maturation. Phototherapy is a cornerstone in managing hyperbilirubinemia, demonstrating consistent efficacy in reducing unconjugated bilirubin levels. The implications for clinical practice are significant, offering healthcare professionals insights into tailoring treatment strategies based on individual neonatal characteristics and the severity of jaundice. Integrating advanced monitoring and control systems enhances the precision and safety of phototherapy. Recommendations for future research emphasize the need to investigate long-term outcomes, explore adjunctive therapies, and address resource limitations to ensure global access to effective neonatal care. Overall, this review contributes to the ongoing refinement of neonatal care practices, offering a comprehensive understanding of neonatal hyperbilirubinemia and its evolving treatment landscape.

A Comprehensive Review on Unveiling the Journey of Digoxin: Past, Present, and Future Perspectives.

Digoxin, a cardiac glycoside derived from the foxglove plant (Digitalis spp.), has been utilized for centuries in managing various cardiac conditions due to its ability to increase myocardial contractility and regulate heart rate. This comprehensive review explores the historical context, pharmacological properties, clinical applications, efficacy, safety profile, challenges, and future perspectives of digoxin. Tracing its journey from traditional medicine to modern cardiovascular therapeutics, we delve into its mechanism of action, therapeutic indications, and clinical guidelines. While digoxin remains a cornerstone therapy for heart failure and atrial fibrillation, its narrow therapeutic index and individual variability in response pose challenges in clinical practice. Nevertheless, ongoing research efforts aim to elucidate its role in emerging therapeutic areas and technological advancements in drug delivery. Despite the advent of newer pharmacological agents, digoxin's enduring relevance lies in its established efficacy, affordability, and global accessibility. This review underscores the symbiotic relationship between tradition and progress in cardiovascular medicine, highlighting the timeless pursuit of medical innovation to optimize patient care.

Immersive Innovations: Exploring the Diverse Applications of Virtual Reality (VR) in Healthcare.

Virtual reality (VR) has experienced a remarkable evolution over recent decades, evolving from its initial applications in specific military domains to becoming a ubiquitous and easily accessible technology. This thorough review delves into the intricate domain of VR within healthcare, seeking to offer a comprehensive understanding of its historical evolution, theoretical foundations, and current adoption status. The examination explores the advantages of VR in enhancing the educational experience for medical students, with a particular focus on skill acquisition and retention. Within this exploration, the review dissects the applications of VR across diverse medical disciplines, highlighting its role in surgical training and anatomy/physiology education. While navigating the expansive landscape of VR, the review addresses challenges related to technology and pedagogy, providing insights into overcoming technical hurdles and seamlessly integrating VR into healthcare practices. Additionally, the review looks ahead to future directions and emerging trends, examining the potential impact of technological advancements and innovative applications in healthcare. This review illuminates the transformative potential of VR as a tool poised to revolutionize healthcare practices.

Atypical Presentation of Wilson Disease: Unravelling a Clinical and Radiological Complexity in a Rare Case.

Wilson disease (WD) is an autosomal recessive disorder marked by aberrations in copper metabolism, leading to its accumulation in vital organs such as the liver, brain, cornea, kidneys, and heart. While WD typically presents with hepatic symptoms in early childhood, neuropsychiatric manifestations are more prevalent during adolescence. This case report highlights an extraordinary instance of WD in an eight-year-old girl, characterized by intricate clinical and radiological features. The patient exhibited atypical symptoms, emphasizing the importance of recognizing diverse presentations of WD. Delayed diagnosis and treatment initiation can prove fatal in WD cases, underscoring the significance of awareness regarding these unusual clinical and radiological features to facilitate prompt intervention and prevent adverse outcomes.

A Case of Ectopic Ureter With Right Atrophied Kidney in a Pediatric Patient.

An ectopic ureter (EU) is a ureter that does not connect appropriately to the bladder and drains somewhere other than the urinary bladder. Ectopic ureter is not so common in kidney anomalies. In men, the EU usually opens near the prostate into the urethra; however, in females, it mainly opens into organs of reproduction or into the urethra. Differential diagnosis of urinary incontinence from other causes, such as EU has a potential cure through surgery. Most women with ectopic ureters have duplex kidneys. An EU emptying a single-system ectopic dysplastic but functioning kidney is uncommon, especially in females. Computed tomography and magnetic resonance imaging provide a clearer image of the ectopic kidney. The surgical techniques used to correct this type of EU are determined based on the functioning of the kidney and anomalies related to the EU site. This is a case of a 9-year-old female who presented with complaints of dribbling urine, which was discovered to be caused by an ectopic ureter with an atrophic kidney.

Spinal Muscular Atrophy With Severe Hyperlordosis: A Case Report.

Spinal muscular atrophy (SMA) indicates a set of inherited autosomal recessive genetic disorders, where, specifically, the anterior horn cell motor neurons in the brain and spinal cord are affected, leading to a severe form of hypotonia and muscle weakness. The incidence is exceptionally rare, commonly manifesting as slowly progressive muscular weakness and atrophy of lower limbs. As per our existing knowledge, this is the first case of SMA associated with hyperlordosis in a patient. Hyperlordosis is a deformity in spinal curvature characterized by an excessive forward spinal curve in the region of the lower back, forming the characteristic C-shape curvature in the lumbar region, just above the buttocks. Parents brought an 11-year-old male child with complaints of inability to get up from a sitting position along with difficulty in walking for the past six months. Upon physical examination, deep tendon reflexes were absent; there was severe hyperlordosis, proximal limb weakness, and notable hypotonia. In our study, we aim to understand the clinical presentation, impact, and association of hyperlordosis in a child diagnosed with SMA. This case report describes the complaints and successful diagnosis of a patient of survivor motor neuron (SMN) gene-related SMA along with severe hyperlordosis backed by evidences of electrophysiology and neuropathology. However, a complete cure and normal lifestyle are not possible due to the lack of affordable and easily accessible therapies.

A Comprehensive Review of Inhaled Nitric Oxide Therapy: Current Trends, Challenges, and Future Directions.

This comprehensive review explores the multifaceted landscape of inhaled nitric oxide (iNO) therapy, tracing its historical evolution, mechanisms of action, clinical applications, challenges, and future directions. The nitric oxide signaling pathway, characterized by vasodilatory effects and anti-inflammatory properties, forms the foundation of iNO's therapeutic efficacy. Clinical applications are found in neonatal respiratory distress syndrome, pulmonary hypertension, and acute respiratory distress syndrome, showcasing its versatility. However, challenges, including cost considerations, technical intricacies, safety concerns, and resistance, highlight the nuanced landscape surrounding iNO therapy. Implications for clinical practice underscore the need for a tailored and evidence-based approach, considering individual patient characteristics and indications. Recommendations for future research emphasize ongoing exploration, novel indications, and the development of targeted therapies. In conclusion, this review positions iNO as a dynamic and adaptable intervention, poised to reshape therapeutic strategies and enhance patient outcomes in critical care.

A phase III randomized-controlled study of safety and immunogenicity of DTwP-HepB-IPV-Hib vaccine (HEXASIIL®) in infants.

A fully liquid hexavalent containing Diphtheria (D), Tetanus (T) toxoids, whole cell Pertussis (wP), Hepatitis B (Hep B), type 1, 2, 3 of inactivated poliovirus (IPV) and Haemophilus influenzae type b (Hib) conjugate vaccine (DTwP-HepB-IPV-Hib vaccine, HEXASIIL®) was tested for lot-to-lot consistency and non-inferiority against licensed DTwP-HepB-Hib + IPV in an open label, randomized Phase II/III study. In Phase III part, healthy infants received DTwP-HepB-IPV-Hib or DTwP-HepB-Hib + IPV vaccines at 6, 10 and 14 weeks of age. Blood samples were collected prior to the first dose and 28 days, post dose 3. Non inferiority versus DTwP-HepB-Hib + IPV was demonstrated with 95% CIs for the treatment difference for seroprotection/seroconversion rates. For DTwP-HepB-IPV-Hib lots, limits of 95% CI for post-vaccination geometric mean concentration ratios were within equivalence limits (0.5 and 2). Vaccine was well-tolerated and no safety concerns observed.Clinical Trial Registration - CTRI/2019/11/022052.

Navigating Idiopathic Masseter Muscle Hypertrophy in a 14-Year-Old Female Child: A Report of a Unique Case.

This case report documents the clinical journey of a 14-year-old female child experiencing bilateral swelling and pain in the mandibular region, suggestive of idiopathic masseter muscle hypertrophy. This condition, although relatively uncommon, can present itself either unilaterally or bilaterally. While cosmetic concerns, often denoted as a "square face," are commonly expressed by patients, additional symptoms like protrusion, bruxism, or trismus may also be present. The patient reported a gradual and asymptomatic bilateral bulging in the mandible angle region, with an explicit complaint of pain. The physical examination revealed bilateral masseter hypertrophy without accompanying local inflammatory changes. Diagnosing this condition necessitates discerning its characteristics, evaluating clinical and radiographic findings, and excluding more severe pathologies like parotid diseases, lymphangioma, and rhabdomyoma. In cases of diagnostic uncertainty, complementary tests are deemed appropriate. Treatment strategies range from conservative measures to surgical interventions. This investigation aims to fulfill its primary objectives by presenting a case study elucidating the intricacies of idiopathic masseter hypertrophy, detailing associated symptoms, and exploring the spectrum of potential treatment options. Through this exploration, we contribute to the evolving understanding and management of this unique condition, especially within the pediatric age group.

A Rare Pediatric Neurological Case: Dyke-Davidoff-Masson Syndrome Unprecedented at 10 Months.

This case report presents a rare occurrence of Dyke-Davidoff-Masson Syndrome (DDMS) in a 10-month-old male child, highlighting the atypical presentation of this neurological disorder in early infancy. The child initially presented with irritability, loss of appetite, and right-sided weakness following episodes of fever. A comprehensive medical history revealed the sudden onset of generalized tonic-clonic seizures, prompting further investigation. Diagnostic imaging, including CT and MRI, confirmed features consistent with DDMS, including cerebral hemiatrophy, ventricular enlargement, and calvarial thickening. Notably, the child's seizures were successfully managed with antiepileptic medication, leading to stabilized vital signs. This case emphasizes the importance of considering rare neurological disorders in pediatric patients with unusual presentations and underscores the challenges in diagnosing and managing DDMS in infancy. Further research is warranted to elucidate the underlying mechanisms, contributing factors, and optimal management strategies for DDMS in this age group.

A Rare Case of a Subgaleal Hematoma With Global Developmental Delay, Scurvy, and Congenital Toxoplasmosis, Rubella, Cytomegalovirus, Herpes Simplex, and HIV (TORCH) Infection.

This case report details the complex presentation of a six-year-old female child with global developmental delay (GDD), scurvy, congenital toxoplasmosis, rubella, cytomegalovirus, herpes simplex, and HIV (TORCH) infection and a subgaleal hematoma. The patient's medical history included delayed developmental milestones, bilateral congenital cataract, and a previous generalized tonic-clonic seizure. Thorough investigations revealed cerebral atrophy, bilateral ventricular dilatation, and periosteal thinning consistent with scurvy. The interdisciplinary approach involving neurology, ophthalmology, and orthopedics resolved the subgaleal hematoma. This case underscores the intricate interplay of neurological, nutritional, and infectious factors in pediatric conditions and highlights the importance of a collaborative, multidisciplinary approach for accurate diagnosis and effective management.

A Rare Presentation of Edwards Syndrome in a Three-Month-Old Infant: A Case Report.

Edwards syndrome, also known as trisomy 18, is a rare chromosomal disorder associated with multiple congenital anomalies and high morbidity. This report presents the case of a three-month-old female infant diagnosed with Edwards syndrome, presenting classic phenotypic features, including low-set ears, micrognathia, and a rocker bottom foot. The infant's condition was further complicated by cardiac abnormalities and respiratory distress, necessitating a comprehensive, multidisciplinary approach involving pediatricians, cardiologists, and orthopedic specialists. The diagnostic journey involved addressing challenges related to respiratory distress syndrome, bronchiolitis, and cardiac complications. The management approach underscored the significance of individualized care tailored to the patient's unique needs. Genetic counseling played a pivotal role in providing essential support to the family facing the complexities associated with Edwards syndrome. This case report highlights the intricacies of Edwards syndrome and contributes to the ongoing discourse on refining clinical strategies for enhanced care and compassionate support. Additionally, it emphasizes the need for further research to advance our understanding of this condition and guide future interventions.

A Review of the Occurrence of Intraventricular Hemorrhage in Preterm Newborns and its Future Neurodevelopmental Consequences.

Intraventricular hemorrhage (IVH) is a type of bleeding that occurs through the germinal matrix and comes through the ependymal cells into the ventricular cavity. It is mostly seen in preterm neonates but can also be seen sometimes in term neonates. Various factors predispose to preterm delivery; it can be spontaneous or medically induced. Spontaneous IVH occurs in cases of intrauterine infections in the mother, and it can be induced in cases of medical emergencies such as preeclampsia and eclampsia. The brain of a preterm newborn is not fully developed as it does not have pericytes and proteins, so it can bleed very quickly, which can cause IVH. Also, the vessels supplying the germinal matrix are immature and highly vascularized. IVH has four grades based on findings detected on cranial ultrasound and MRI. Management includes medical and surgical management; medical management includes phenobarbitone used for seizures and prophylaxis. Surgical management includes drainage, irrigation, and fibrinolytic therapy (DRIFT), and neuro-endoscopic lavage. IVH causes various short-term and long-term neurodevelopmental consequences. Long-term complications include cerebral palsy and intellectual disability, which hamper the life of the child. It mainly presents with seizures, flaccidity, decerebrate posture, etc. Various preventive measures can be taken to tackle IVH in newborns. First of all, preterm delivery should be avoided, and intrauterine infections in mothers should be treated. The administration of corticosteroids should be done for all preterm deliveries as it helps in the maturation of organs. The administration of magnesium sulfate should be done as it is neuroprotective and reduces cerebral palsy in the future. Delayed cord clamping is to be done to reduce recurrent blood transfusions and decrease the risk of IVH. This article explains the pathogenesis, management, prevention, and future outcomes of IVH.

Phase III randomized clinical studies to evaluate the immunogenicity, lot-to-lot consistency, and safety of ROTAVAC® liquid formulations (ROTAVAC 5C & 5D) and non-inferiority comparisons with licensed ROTAVAC® (frozen formulation) in healthy infants.

The WHO pre-qualified rotavirus vaccine, ROTAVAC®, is derived naturally from the neonatal 116E rotavirus strain, and stored at -20°C. As refrigerator storage is preferable, immunogenicity and safety of liquid formulations kept at 2-8°C, having excipients to stabilize the rotavirus, with or without buffers, were compared with ROTAVAC® in different clinical studies. Study-1, the pivotal trial for this entire product development work, was a randomized, single-blind trial with two operationally seamless phases: (i) an exploratory phase involving 675 infants in which two formulations, ROTAVAC 5C (LnHRV-1.5 mL and LnHRV-2.0 mL) containing buffer and excipients to stabilize the virus against gastric acidity and temperature, were compared with ROTAVAC®. As the immune response of ROTAVAC 5C (LnHRV-2.0 mL) was non-inferior to ROTAVAC®, it was selected for (ii) confirmatory phase, involving 1,302 infants randomized 1:1:1:1 to receive three lots of LnHRV-2.0 mL, or ROTAVAC®. Primary objectives were the evaluation of non-inferiority and lot-to-lot consistency. The secondary objectives were to assess the safety and interference with the concomitant pentavalent vaccine. As it was separately established that buffers are not required for ROTAVAC®, in Study-2, the safety and immunogenicity of ROTAVAC 5D® (with excipients) were compared with ROTAVAC® and lot-to-lot consistency was assessed in another study. All lots elicited consistent immune responses, did not interfere with UIP vaccines, and had reactogenicity similar to ROTAVAC®. ROTAVAC 5C and ROTAVAC 5D® were immunogenic and well tolerated as ROTAVAC®. ROTAVAC 5D® had comparable immunogenicity and safety profiles with ROTAVAC® and can be stored at 2-8°C, leading to WHO pre-qualification.Clinical Trials Registration: Clinical Trials Registry of India (CTRI): CTRI/2015/02/005577CTRI/2016/11/007481 and CTRI/2019/03/017934.