RESUMEN
Progressive rubella panencephalitis (PRP) is a rare late complication of rubella that affects mainly teenagers. It is characterized by progressive white matter destruction, gliosis and cerebral atrophy, similar to subacute sclerosing panencephalitis (SSPE) and congenital rubella syndrome. This paper examines the complexity of PRP, its clinical manifestations, pathogenesis, diagnostic criteria, treatment and prophylaxis. PRP should be considered in adolescents with progressive dementia with pyramidal and cerebral dysfunction. With respect to the most affected children, survival and recovery is expected to be poor, with high mortality rates, especially in the first six months of life. The development of progressive spasticity, ataxia, mental deterioration and convulsions in late childhood and early childhood with mothers' rubella or stigmata histories is the subject of research on PRP. Therefore, continued efforts to understand and address PRP are important to improve the quality of diagnosis, treatment and ultimately the quality of life of affected persons.
RESUMEN
Even if life expectancy has increased, sickle cell disease (SCD) still presents difficulties, especially because of the painful episodes that occur frequently and without warning, known as Vaso-occlusive crises (VOCs). These crises are brought on by different cells adhering to one another and obstructing tiny blood veins, which can cause excruciating agony and eventually harm organs and tissues. While the majority of current treatments concentrate on symptom management and pain relief with the use of medications, hydration, and other general approaches, new discoveries about the fundamental mechanisms of VOCs provide intriguing new therapeutic options. With the goal of precisely addressing the pathways causing inflammation and cell adhesion, these cutting-edge treatments may lessen the frequency of VOCs and shield vital organs from harm. Though these treatments provide hope for improved SCD management, careful assessment and analysis of their efficacy and accessibility are necessary to guarantee their general benefit.
RESUMEN
There are many different clinical manifestations of celiac disease (CD), including the classical form, in which intestinal symptomatology predominates on the contrary there is atypical forms, in which extra-intestinal clinical features predominate, and the silent form, in which there are no clinical symptoms. Few or no gastrointestinal symptoms and a predominance of extra-intestinal features, including liver, kidney, skeletal, psychiatric, neurologic, dermatologic, hematologic, endocrinological, and reproductive involvements, define the atypical forms of the disease. Through screening high-risk groups, silent presentations of CD may be found. It is crucial for healthcare professionals to have a high level of suspicion for the atypical presentations of CD because it is now well known that CD may account for a number of chronic health issues.
RESUMEN
This systematic review critically investigates the administration of the Bacillus Calmette-Guérin (BCG) vaccine in neonates with severe combined immunodeficiency (SCID). The BCG vaccine, derived from Mycobacterium bovis, is a live attenuated vaccine recognized for its significant role in mitigating the impacts of tuberculosis (TB) in endemic areas. Despite its beneficial effects in controlling TB, safety and efficacy concerns have been raised when the vaccine is administered to SCID patients, who have a severe dysfunction or absence of the immune system. The potential for the vaccine to lead to severe complications due to the immunocompromised state of SCID patients necessitates a comprehensive investigation. To better understand these issues, a thorough literature review was carried out, integrating data from clinical trials and observational studies available on the PubMed database. An extensive review and analysis of 32 relevant articles revealed substantial evidence of complications from BCG vaccination in SCID patients. These findings emphasize the urgency for a more effective pre-vaccination screening process to circumvent potential adverse effects. Given the crucial role of the BCG vaccine in controlling TB, its potential to induce severe complications in SCID patients warrants careful consideration. Therefore, this review proposes an in-depth screening algorithm for newborns before BCG vaccination administration. The goal is to prevent these adverse events, offering critical insights to health policymakers, researchers, and clinicians in the field.
RESUMEN
Mesoamerican nephropathy (MeN) also known as chronic kidney disease of unknown etiology (CKDu) is prevalent in agriculturally rich areas. The most widely accepted pathophysiological explanation for MeN is chronic dehydration caused by prolonged exposure to the sun. Other theories include oxidative stress, chronic inflammation, infection and tubulointerstitial fibrosis. The clinical presentation is quite vague and is diagnosed similar to CKD from any cause using blood, urine analysis and ultrasound. The study highlights the need for interdisciplinary cooperation among physicians, epidemiologists, toxicologists, and geneticists while identifying significant research gaps and future objectives. Occupational health related to agriculture is not emphasised enough especially in third world countries where a large chunk of population heavily depend on farming. To safeguard the population at risk, the significance of community-based initiatives, occupational health measures, and regulatory changes is emphasised.
RESUMEN
Renal artery stenosis (RAS) is a major contributor to the prevalence of secondary hypertension. Fibromuscular dysplasia and atherosclerosis are commonly responsible for the occurrence of the disease. Medical therapy is the primary means of treatment for RAS. However, surgical interventions for revascularization are also considered, in selected group of patients, which can effectively cure hypertension and chronic kidney disease. An older man presented at Venus hospital, Surat, Gujarat with the complaints of severe dyspnea, edema, uncontrolled hypertension and renal insufficiency. He was diagnosed RAS and was operatively managed with percutaneous transluminal renal angioplasty. Written consent was taken from the patient mentioned in the study. During the procedure, the renal artery got ruptured, which was managed by placing a covered stent. The patient was successfully treated for RAS, in spite of comorbidities and intraoperative complication. In the subsequent clinical follow-up, the patient was asymptomatic. There was marked reduction in serum creatinine levels and even the blood pressure improved significantly. Absence of post-operative complications and positive recovery of the patient signifies the fact that management of renal artery rupture with a covered stent is a convenient approach in acquiring effective haemostasis. This approach can be useful in managing any sort of vessel rupture, related to revascularization procedures.
RESUMEN
Renal artery stenosis (RAS) is a major contributor to the prevalence of secondary hypertension. Fibromuscular dysplasia and atherosclerosis are commonly responsible for the occurrence of the disease. Medical therapy is the primary means of treatment for RAS. However, surgical interventions for revascularization are also considered, in selected group of patients, which can effectively cure hypertension and chronic kidney disease. An older man presented at Venus hospital, Surat, Gujarat with the complaints of severe dyspnea, edema, uncontrolled hypertension and renal insufficiency. He was diagnosed RAS and was operatively managed with percutaneous transluminal renal angioplasty. Written consent was taken from the patient mentioned in the study. During the procedure, the renal artery got ruptured, which was managed by placing a covered stent. The patient was successfully treated for RAS, in spite of comorbidities and intraoperative complication. In the subsequent clinical follow-up, the patient was asymptomatic. There was marked reduction in serum creatinine levels and even the blood pressure improved significantly. Absence of post-operative complications and positive recovery of the patient signifies the fact that management of renal artery rupture with a covered stent is a convenient approach in acquiring effective haemostasis. This approach can be useful in managing any sort of vessel rupture, related to revascularization procedures.