Sudanese journal of paediatrics最新文献

Russell-Silver syndrome, also called asymmetric dwarf dysgenesis syndrome is an uncommon genetic disorder presenting with low birth weight, failure to thrive and growth retardation (short stature), developmental delay, facial dysmorphism and hemihypertrophy. The estimated incidence is between 1 case in 3,000 to 1 case in 100,000. We are hereby reporting one such case of postnatal growth retardation with facial dysmorphism and several other features of Russell-Silver syndrome and confirmed by genetic analysis.

Paediatric haemophagocytic lymphohistiocytosis (pHLH) is a potentially life-threatening condition with significant diagnostic and therapeutic difficulties. The purpose of this study was to describe the clinical presentation, the diagnostic challenges, and the outcomes of haemophagocytic lymphohistiocytosis (HLH) in children assessed at Mukalla Hospital, Yemen. Data from 20 medical records of HLH patients admitted between January 2010 and May 2022 were retrospectively analysed. The median age at presentation was 3.5 ± 5.1 years. Male: female ratio was 1:1. The median time for referral to the hospital was 30 ± 64 days. The most common clinical manifestations were fever and pallor in 95% of cases, and splenomegaly (85%). Hepatomegaly, chest, renal and neurological manifestations were detected in 80%, 45%, 15% and 20% of cases, respectively. Bone marrow haemophagocytosis was detected in 60% of cases. Sixteen patients fulfilled the HLH diagnostic criteria, and 11 patients (55%) received the HLH 2004 protocol. Out of the 20 patients, three (15%) patients are alive. Fourteen patients died, with overall mortality of 82.35%. All mortalities were due to HLH disease with multi-organ failure. Relapse was noticed in five patients either during treatment or after full recovery. pHLH is a challenging emergency with a high mortality rate. High clinical suspicion is essential for early detection and intervention to improve the prognosis.

Noma is an orofacial gangrenous infection commonly affecting malnourished children in the tropical region of the World, particularly the sub-Saharan Africa. Nigeria is a sub-Saharan African country which is among the countries seriously affected by noma. In Nigeria, noma has been classified as a priority disease. However, only very little attention has been focused on noma research in Nigeria, unlike many other priority diseases. This study conducted an informetric analysis of research outputs on noma in Nigeria, using the PubMed database - a world-leading and authoritative database of medical literature. This study revealed that only 26 PubMed-indexed publications on noma (PONs), published between January 1990 and September 2021, were available. A trend analysis of these PONs showed that the average output (outputs from 1999 to 2020 = 23) rate per year (from 1990 to 2020 [31 years]) was 0.74 (23/31). Sokoto (n = 11), Oyo (n = 4) and Kebbi (n = 3) were the top three Nigerian states surveyed on noma in the included PONs. Only 12 publications had international co-authors, of which only few publications had co-authors affiliated with institutions in other African countries: South Africa - 5 publications; and Rwanda - 2 publications. Only two publications were funded. Also, the top five prolific Nigerian authors on noma in Nigeria were affiliated with Noma Children Hospital, Sokoto State, Nigeria. In conclusion, PONs by Nigerian authors is so meagre in quantity. This low output is an issue of medical concern. More research focused on noma in Nigeria is needed through funding and other research capacity-strengthening measures.

In addition the nature of nurses working environment, duties and responsibilities, nurses are facing numerous health hazards while working at hospitals. This study aimed to identify the health-related risks of nursing practice at Ahmed Gasim Hospital and Gafaar Ibnauf Children's Hospital, Khartoum State, Sudan. A descriptive cross-sectional study was conducted at two governmental paediatric hospitals in Khartoum, from August to December 2017, in which 74 nurses were selected by convenience sampling to participate. The data was collected by a validated questionnaire and analysed by Statistical Package for Social Sciences, version 25. About 65% of nurses reported that they had high level of awareness of health risks they were exposed to on their job while 75.7% indicated that the major factors contributing to health risks in the hospitals were working pressure and poor working environment. Low back pain was found to be the most common health-related risks (77%) that might result from the extreme pressure at work (75.7%). Gender and years of experience had statistically significant association with health hazards among nurses (0.042 and 0.047, respectively). The study concluded that the majority of nurses have high awareness of health-related risks. The reported problems were low back pain which was found to be the most common health-related risk that might result from the extreme pressure at work.

Neonatal intensive care unit (NICU) admission increases parents' stress levels and it might be even higher in the crisis of coronavirus disease 2019 (COVID-19) pandemic and lockdown. This study was done to identify the stress levels of parents of admitted neonates and the difficulties encountered in neonatal care and follow-up during the COVID-19 pandemic and lockdown. The Parental Stressor Scale (PSS:NICU) and Perceived Stress Scale (PeSS) were used to identify the stress levels of parents of admitted neonates. Online survey form with a structured questionnaire comprising PeSS and NICU:PSS was sent through messaging app (Google form) after informed consent. PSS score of <14 was considered low stress, 14-26 moderate and >26 as high. A total of 118 parental responses (mother /father in 26, both in 46) for 72 admitted neonates, were obtained. The mean (SD) PeSS score was 19.7 (5.8%) and 92 (78%) had moderate stress while 11 (9%) had high stress. In NICU:PSS, sights-sounds and parental role had more median scores: 2.25 (1-3.75) and 2.21 (1-3.57), respectively. Maternal and paternal NICU:PSS (p-0.67) and PeSS (p-0.056) scores were not statistically different. Keeping nil per oral, invasive ventilation, culture-positive sepsis, fathers' transport difficulty and longer duration of mothers' and neonates' hospital stay was associated with increased NICU: PSS scores. Twenty (29%) parents could not bring their child for follow-up and there was a delay in immunisation in 21 (30%). The pandemic and the lockdown might have disrupted antenatal and postnatal follow-ups further adding to the parental stress.

Central precocious puberty (CPP) is frequently seen among cases presenting to our endocrine clinics. The purpose of this study was to have base line data on this condition with an attempt to point out any possible unique features of Sudan and to explore challenges faced in management and how that cultural and traditional practices may hamper care. Here, we are reporting the first data on this aspect from Sudanese subjects. Patients labelled as having CPP in Gafaar Ibnauf Children's Hospital and Soba University Hospital Endocrinology Clinics from January 2006 to 2016 are included in a descriptive hospital-based study which was conducted over 10 years in these two main paediatric endocrinology centres. Records of all patients with CPP were reviewed and challenges in diagnosis and management were identified. Most of the children with CPP presented late. Organic causes were more frequent among girls than what has been reported in the literature; in most boys, it was idiopathic. Almost half of the patients with underlying pathology were older than 6 years of age. Most cases including girls have an organic cause, thus magnetic resonance image should be done in all patients. Management of precocious puberty in a resource-limited country is faced with various challenges (e.g., expensive investigations and medications). We suggest establishing a unified protocol for managing these cases and creating collaboration between governmental, nongovernmental organisations and health services.

Dengue fever is an important cause of acute febrile illness in the postmonsoon season in India. This study was done to record the incidence of dengue in admitted patients with acute febrile illness in a hospital setting. The study also intends to record the clinical, biochemical and outcome profile of paediatric dengue cases admitted in tertiary centres in Eastern Uttar Pradesh, India. It was a prospective case record analysis at a tertiary care research hospital in Eastern Uttar Pradesh. The study recruited fifty-53 children (<18 years) with serology-proven diagnosis of dengue disease. Disease was confirmed by doing Ns1Ag, IgM antibody test by ELISA method. Six hundred children were screened and 53 met the inclusion criteria. The incidence of dengue disease in hospitalised acute febrile illness was 8.8%. There were thirty-one males. The mean age of presentation of the study population was 9.32 ± 5 years with a range of 0.25 - 17 years. Fever (94%), nausea and vomiting (59 %), abdominal pain (55%), persistent vomiting (49%), thrombocytopenia (<100,000 [66%]), and petechiae and purpura (43%) were the important clinical manifestations. Six required intensive care monitoring. There was only one death. Dengue fever is an important cause of acute febrile illness in children. Case fatality rate can be minimised with proper World Health Organisation classification and protocol-based management of cases.

In this article, I will try to shine a light on the life and contribution to child health of the Late Professor Hafiz Elshazali, who passed away in December 2020, through my eyes. He is my father but more than that he was my mentor and role model.