Sudanese journal of paediatrics最新文献

Critically ill children frequently encounter the most common and potentially life-threatening electrolyte disturbances, i.e., hyponatremia. It is an independent risk factor for prolonged hospitalization in the intensive care unit and increased in-hospital mortality. Hyponatremia occurs in up to 20%-30% of admissions in the pediatric intensive care unit (PICU). This observational study was conducted in the PICU of a tertiary care hospital in a developing country from September 2018 to September 2019. Admission criteria in our PICU are the need for mechanical ventilation, fulminant hepatic failure, vasopressor support, respiratory failure and poorly controlled seizure. We studied 256 children, aged 1 month to 18 years, with normal serum sodium at admission. In our study, 72 (28.1%) children developed hyponatremia, and about two third (n=48, 66.7%) of them developed within 72 hours of admission in PICU. The majority of children (n = 46, 63.9%) in the hyponatremic group were below 5 years. Wasted children (n = 68, 26.6%) in the hyponatremic and isonatremic groups were 20 (27.8%) and 48 (26%), respectively. The most common etiology of hyponatremia was cerebral salt wasting syndrome (n = 20, 27.8%) followed by drug-induced cases (n = 19, 26.4%). The drugs responsible were diuretics and anti-epileptics. In our study, multiorgan failure (OR = 5.05, 95%CI = 1.90-13.43; p = 0.0001), shock (OR = 7.38, 95%CI = 3.56-12.28; p = 0.0001), vasopressor use (OR = 6.74, 95%CI = 3.45-13.17; p = 0.0001) and coagulopathy (OR = 6.74, 95%CI = 3.45-13.17; p = 0.0001) were the risk factors for the development of hyponatremia. Mortality among the hyponatremic group (44.4%) was significantly higher than in the isonatremic group (21.7%). Hyponatremia is a common electrolyte disturbance found in critically ill patients and is associated with prolonged hospitalization and increased mortality.

Trisomy 22 is a rare autosomal constitutional chromosomal disorder with high survival incompatibility being the third most frequent trisomy in spontaneous abortions and accounting for 10%-15% of all cases. Mosaic trisomy 22 is a less severe variant of the disorder associated with mild phenotype and longer lifespan compared to cases with complete trisomy 22. To date, there have only been 25 reported cases of trisomy 22 worldwide. We report a recent case of a 7-year-old boy brought in by his mother seeking a diagnosis for the condition of her child. The father deceased 6 years earlier, and the obstetrical history of the mother revealed 3 first trimester recurrent abortions. Upon examination, the child was found to have short stature, dysmorphic features, atrial septal defect, cognitive disability and other neurological abnormalities. The mother's karyotype analysis showed a normal female karyotype 46,XX, while the child's analysis revealed mosaic trisomy of chromosome 22 syndrome 47,XY,+22/46,XY. This case marks the first documented case of this syndrome in Sudan.

Enthesitis related arthritis (ERA) is a specific type of juvenile idiopathic arthritis (JIA) and ERA typically begins with enthesitis and peripheral arthritis in the lower extremities, progressing later in the disease to sacroiliitis and spinal involvement. The condition has a strong relationship with the HLA-B27 and primarily affects boys between the ages of 13 and 16 years. We describe an unusual presentation of ERA in a young boy with pubic pain and fever, describing its quintessential magnetic resonance imaging (MRI) findings. A child presented to the paediatric outpatient department with fever, insidious onset pubic pain and tenderness with associated leukocytosis and elevated inflammatory markers. MRI of the pelvis revealed bilateral symmetrical bone marrow edema involving the pubic symphysis and inferior pubic rami with para symphyseal edema involving bilateral obturator externus muscle and bilateral adductor longus muscle insertions suggestive of enthesitis of the pubic symphysis. MRI also showed sacroiliitis and enthesitis at other sites. HLA-B27 was positive. The patient responded well to a short course of steroids and maintained remission with tumour necrosis factor alpha inhibitors.

Due to its hemodynamic stability, ketamine is a commonly used anesthetic agent for sedation during small procedures in the critical care unit. We report a case of an 8-year-old girl, previously diagnosed with tetralogy of Fallot who presented for operative correction of the congenital anomaly. She underwent a successful operation and recovered smoothly. On the second postoperative day, and upon removal of a chest drain with the use of ketamine for sedation, the patient suffered cardiopulmonary arrest. After repeated cycles of resuscitation, the patient returned to spontaneous circulation.

Patients with epilepsy are at significant risk for cognitive impairment and behavioural abnormalities. The aim of this study was to assess the learning abilities and school performance of epileptic children attending Wad Medani Children Teaching Hospital, Gezira State, Sudan. This was a prospective cross-sectional case-control facility-based study. Data were collected from the hospital database and from school records. A total of 70 patients with epilepsies were recruited as index cases and 70 age and sex-matched non-epileptic patients were included as controls. Both groups underwent comprehensive psychometric assessment. Consensus neurobehavioural diagnoses were made with respect to Diagnostic and Statistical Manual. Stanford Binet Test and Battery Scales were used to assess the intelligent quotient (IQ) and learning abilities, respectively. The study population consisted of school aged (5-16 years) children; 48.6% were less than age 10 years. The male: female ratio was 1:1.1. A significant increase in learning difficulties among index cases was recorded compared to controls in all domains. The overall learning difficulties results showed a statistically significant result between index cases and controls, with p value of <0.001. Similarly, a significant reduction in IQ test results was found among the cases compared to controls. In conclusion, significant learning disabilities (LDs), deterioration in school performance and IQs were detected in children with epilepsy. All children with epilepsy should be considered vulnerable to LDs.

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.