Neonatal Purpura Fulminans is a rare and fatal disorder associated with perivascular haemorrhage and disseminated intravascular coagulation. Early clinical recognition, timely investigation and treatment is utmost important. A 6 days old baby boy was brought to emergency with blackish ulcers all over the body. Initially these were over the feet and scalp but later appeared on the abdomen. On examination, child was vitally stable, mildly icteric and had multiple erythematous large bullous blackish lesions on scalp, lower abdomen, perineum, back and soles. Neonatal reflexes and systemic examination was normal. Laboratory investigations showed normal CBC, PT/APTT and Protein S level while Protein C and Antithrombin III levels were low. Neonatal Purpura Fulminans is a life threatening condition and family screening is also mandatory for early recognition of disease in the siblings.
Download full-text PDF |
Source |
|---|
Publication Analysis
Top Keywords
Similar Publications
Onset and Outcome of Ocular Lesions in Neonatal Severe Protein C Deficiency: Case Report and Literature Review.
Pediatr Blood Cancer
January 2025
Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Article Synopsis
- Neonatal protein C deficiency can lead to severe conditions like purpura fulminans and has a generally poor prognosis, especially regarding vision.
- In a study of 38 survivors with biallelic PROC variants, a significant number suffered from severe visual impairments: 23 were totally blind, and only one had normal vision.
- Prenatal intervention is crucial to enhance visual outcomes, as ocular lesions often present early in these cases, affecting the eyes before other organs.
Association of HPA Antigens with Immune Thrombocytopenia: A Case-Control Study by PCR-SSP Method.
Int J Hematol Oncol Stem Cell Res
October 2024
Department of Immunology, Faculty of Medicine, Lorestan University of Medical Sciences, Khorramabad, Iran.
Human platelet antigens (HPAs) play a clinically significant role in alloimmunization and the development of immune-mediated disorders such as immune thrombocytopenia (ITP), fetal and neonatal alloimmune thrombocytopenia (FNAIT), and post-transfusion purpura (PTP). Understanding the genetic profiles of HPAs is critical for preventing and treating these conditions. Given the limitations of serological methods in determining HPA genotypes, this study aims to investigate the association between the genotypes of HPA1, HPA2, HPA3, HPA4, and HPA15 antigens and autoimmune thrombocytopenia in Lorestan Province, utilizing the PCR-SSP method.
View Article and Find Full Text PDFITP in pregnancy: diagnostics and therapeutics in 2024.
Hematology Am Soc Hematol Educ Program
December 2024
Division of Hematology, Massachusetts General Hospital, Boston, MA.
Thrombocytopenia will occur in 10% of pregnancies-ranging from the clinically benign to processes that can threaten both mother and fetus. Accurately identifying the specific etiology and appropriate clinical management is challenging due to the breadth of possible diagnoses and the potential of shared features among them. Further complicating diagnostic certainty is the lack of confirmatory testing for most possible pathophysiologies.
View Article and Find Full Text PDFILAE neonatal seizure framework to aide in determining etiology.
Epileptic Disord
November 2024
Clinical Neuroscience, UCL GOS Institute of Child Health and Department of Clinical Neurophysiology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
Objective: To employ the neonatal seizure framework developed by the International League Against Epilepsy (ILAE) Neonatal Task force to assess its usefulness in determining the etiology of neonatal seizures.
Methods: The members of the ILAE Neonatal Task Force evaluated 157 seizures from 146 neonates to determine internal validity and associations between semiology and a specific etiology.
Results: Provoked neonatal electrographic and electroclinical seizures were due to multiple etiologies.
WONOEP appraisal: Targeted therapy development for early onset epilepsies.
Epilepsia
November 2024
Saul R. Korey Department of Neurology, Isabelle Rapin Division of Child Neurology, Laboratory of Developmental Epilepsy, Dominick P. Purpura Department of Neuroscience, Albert Einstein College of Medicine, Bronx, New York, USA.
The early onset epilepsies encompass a heterogeneous group of disorders, some of which result in drug-resistant seizures, developmental delay, psychiatric comorbidities, and sudden death. Advancement in the widespread use of targeted gene panels as well as genome and exome sequencing has facilitated the identification of different causative genes in a subset of these patients. The ability to recognize the genetic basis of early onset epilepsies continues to improve, with de novo coding variants accounting for most of the genetic etiologies identified.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!