Sudanese journal of paediatrics最新文献

Scrub typhus is an acute febrile illness caused by Orientia Tsutsugamushi. It usually presents with high fever, lymphadenopathy, rash, organomegaly and an eschar formation at the site of the bite. Doxycycline is the drug of choice usually showing rapid defervescence, but rarely some cases does not respond. Here, we present 2 such cases that did not respond to Doxycycline and had atypical presentation. Our first case presented with a fever for 15 days and then went into shock and multiorgan dysfunction despite having no fever after admission. Our second case presented in status epilepticus and septic shock. He developed purpura fulminans even after starting treatment. Both the cases did not respond to Doxycycline. The diagnosis was confirmed by Scrub Typhus IgM Antibody positivity. They were successfully treated with second-line drugs i.e. Azithromycin and Chloramphenicol.

Caring for critically ill children presents unique challenges due to their rapid deterioration and the need for immediate, complex interventions. The assessment, diagnosis and treatment of deteriorating paediatric patients require a comprehensive and holistic, systematic approach. However, the dynamic nature of critical illness and the need for stabilisation can often lead to missed opportunities for assessment and intervention. To improve the quality and timeliness of care for critically ill children, we developed standardised checklist proformas to guide focused and efficient assessments. These proformas, originally adapted from adult intensive care settings, have been refined over two decades through extensive clinical experience and collaboration with experts worldwide. The proformas provide a framework for diagnostic reasoning and ensure through predictive modelling that essential components of the assessment are not overlooked. By using these tools, healthcare professionals can effectively identify and address the needs of critically ill children, improving patient outcomes and enhancing the efficiency of care.

Spinal dural arteriovenous fistula (AVF) is an abnormal shunting between the segmental artery and radicular vein adjacent to the dural root sleeve in the spine. This is the most common vascular malformation of the spinal cord and is a rare but treatable cause of para or quadriplegia. It most commonly occurs in elderly men and often affects the thoracolumbar region. These patients clinically present with progressive myelopathies, and other autonomic symptoms (e.g., bladder and bowel dysfunction) subsequently in the later course of the disease. Computed tomography angiography and magnetic resonance imaging remain the modality for initial evaluation. Herein, we present a rare case of spinal dural AVF in a child along with a review of imaging modalities. To the best of our knowledge, there are few case reports of this condition in a paediatric age group.

Orofacial cleft (OC) is a group of heterogeneous congenital abnormalities affecting the orofacial region. All over the world, several studies have been conducted on OC. This study aims to analyze OC research outputs in Nigeria. A SCOPUS-based bibliometric analysis of OC research outputs in Nigeria was done. A total of 130 outputs, of which 90% were articles, published within a 5-2-year (1970-2022) interval were identified. These publications had a total of 1,145 citations with a h-index of 19. There was skewedness in the geopolitical distribution and ownership of Nigerian institutions engaged in OC research; the majority of which were owned by the federal government and situated in the southern parts of Nigeria. The University of Lagos was the Nigerian institution with the highest frequency (n = 52) of outputs on OC research. Adeyemo WL was the Nigerian researcher with the highest: number of publications on OC (n = 46), h-index (n = 11) and citations on OC publications (n = 474). Half of the top ten overseas institutions having researchers collaborating with OC researchers in Nigerian institutions were based in the USA. All the institutions funding OC research in Nigeria were foreign and predominantly in the USA. The majority of the 10 most-cited OC outputs from Nigerian institutions were first authored by foreign researchers. There is a need to locally strengthen OC research capacity in Nigeria, especially in the area of funding, and institutional strengthening.

Alagille syndrome (ALGS) is a multisystem autosomal dominant disorder in which patients may have characteristic facial features and involvement of the liver, heart, vessels, bones, eyes, kidneys and central nervous system. As there is little published data on ALGS in Africa, our aim was to describe the presentation and outcomes of ALGS in South Africa. The study constitutes a retrospective analysis of 25 patient medical records diagnosed as ALGS at Chris Hani Baragwanath Academic Hospital Pediatric Gastroenterology clinic between January 1992 and January 2020. Twenty-five patients met the diagnostic criteria for ALGS over the period investigated. Eighteen (72%) patients were less than 1 year old at first presentation. Seven patients (28%) had all five main clinical manifestations of ALGS, and the rest had an equal proportion of four and three main clinical manifestations. Cholestasis, one of the main clinical manifestations, was present in 72%; 80.0% had the typical Alagille facial features; 64% had cardiovascular disease, 36% had ocular abnormalities and 40% had skeletal abnormalities. Of the 16 patients, (64%) who presented with cardiovascular disease, seven patients presented with more than one cardiac lesion. As of January 2020, 8 (32%) patients are still being followed up at the pediatric GIT clinic, 13 (52%) patients were lost to follow-up and four patients (16%) were demised. Low- to middle-income countries, with no readily available access to genetic testing, need to rely on diagnostic criteria to make a diagnosis of Alagille syndrome in infants who present with cholestasis.