ABSTRACT
Congenital neutropenia syndromes encompass a group of genetic disorders characterized by persistent neutropenia and recurrent infections inherited in an autosomal recessive, dominant, or X-linked manner. These syndromes arise from mutations in various genes, and one of the significant genes involved is glucose-6-phosphatase catalytic subunit 3 (G6PC3), giving rise to a condition known as Dursun syndrome. As per existing knowledge, a total of 92 cases of Dursun syndrome have been reported globally, including eight cases from Saudi Arabia. Our study identified two additional cases exhibiting neutropenia since the early postnatal period and recurrent admissions due to infections. Additionally, these patients presented with oral ulcers, chronic diarrhea, and anomalies affecting the cardiac and genitourinary systems. The rising incidence of congenital neutropenia on a global scale necessitates heightened vigilance among clinicians to ensure thorough follow-up of patients with neutropenia. This proactive approach can lead to early detection and appropriate management of associated complications, ultimately improving patient outcomes.
ABSTRACT
We report a 9-year-old female child who presented with fever and focal seizures. The Cerebrospinal Fluid (CSF) analysis was compatible with viral meningoencephalitis and Reverse Transcriptase Polymerase Chain Reaction (RT-PCR) on same sample was positive for dengue virus RNA, serotype 2. The dengue IgM in blood sample was positive on the 8th day of the illness. This case demonstrates the emerging neurological manifestations of dengue infection and the first confirmed pediatric meningoencephalitis reported from Saudi Arabia. In areas where it does exist, dengue should be included in the differential diagnosis of cases of viral meningitis or meningoencephalitis.
