Sudan's rheumatic fever and rheumatic heart disease guidelines: a simplified approach in an endemic country.

Background: Rheumatic heart disease (RHD) is a preventable sequelae of group A beta hemolytic streptococcal infection leading to an immune reaction: acute rheumatic fever (ARF) and progressive heart valve dysfunction. RHD is the leading cause of acquired heart disease in children and young adults in Sudan and many low/middle-income countries. In 2018, the World Health Organization (WHO) issued a resolution for RHD mandating that each country adopt updated guidelines for ARF and RHD management. These current guidelines are mainly directed to primary healthcare workers. Methods: Sudan's Federal Ministry of Health (FMOH) in collaboration with the WHO East Mediterranean Regional Office (EMRO) assembled a committee for updating RHD guidelines. We conducted a systematic literature search from 2000 to 2022 in National Institute of Health Database (PubMed) under the following titles: streptococcal pharyngitis, acute rheumatic fever, rheumatic heart disease, benzathine penicillin. Best available, evidence-based practices for diagnosis and management of ARF/RHD were selected and adapted to Sudan's situation. The guidelines were critically appraised by the committee then endorsed to the FMOH and WHO EMRO Noncommunicable Disease Departments in January 2023. This paper describes the updated guidelines. Results: Simplified algorithms are provided for diagnosis of bacterial pharyngitis including two clinical criteria: sore throat and the absence of viral symptoms in the target age group. A simplified algorithm for diagnosis and management of ARF is adopted using two levels of diagnosis: suspected case at primary level where penicillin prophylaxis is started and secondary/tertiary care where echocardiography is performed and diagnosis confirmed or excluded. Echocardiography screening is recognized as the standard method for early diagnosis of RHD; however, due to the anticipated limitations, its implementation was not adopted at this time. Streptococcal skin infection is included as a precursor of ARF and a detailed protocol for benzathine penicillin administration is described. Conclusion: The Sudan guidelines for ARF/RHD management were updated. Endorsement of these guidelines to FMOH and WHO EMRO is expected to improve control of RHD in the region.

Clinical presentation of coeliac disease and the effect of sorghum-based diet on anthropometric measurements among Sudanese children.

Coeliac disease (CD) is a chronic enteropathy. Sorghum (Sorghum vulgaris) is a common staple in Sudan. The literature on the growth of children with CD following sorghum diet is scanty. The aim of this study was to identify the demographic and clinical characteristics of CD in children and to determine the anthropometric response to a diet based on sorghum. This prospective study included children with probable CD in Ibn Sina Hospital, Khartoum, Sudan, from January 2002 to February 2012. The demographic, anthropometric, clinical and laboratory data were recorded. The CD serology was done, and the diagnosis of CD was based on Marsh criteria. Children who consumed sorghum and their anthropometry were recorded at 3 and 6 months after diagnosis. Children enrolled were 218. The mean ± SD age was 8.2 ± 4.5, median was 7 years and female/male ratio was 1.12/1. CD was common among Nubians and Arabs. The majority (145, 66.5%) presented with gastrointestinal symptoms. Feeding on sorghum diet resulted in a significant increase in weight after 3 and 6 months (21.1 ± 9.8 and 25.1 ± 14.2 kg, respectively) of the initial visit (18.5 ± 9.4 kg), p < 0.001 and p = 0.001, respectively. The mean weight for height had significantly increased at the second compared to the initial visit (0.17 ± 0.05 vs. 0.15 ± 0.5), p < 0.001. There was no association between gaining weight and age, gender, or a family history of CD. In conclusion, Sudanese children with CD presented over 8 years of age. The common presentation was gastrointestinal symptoms. The initial weight and weight-for-height increment were significant on sorghum diet.

Prevalence of epilepsy in 74,949 school children in Khartoum State, Sudan.

BACKGROUND: Data on childhood epilepsy in Sudan are scarce and the only published study on its prevalence was published in 1983. This study aimed to determine the current prevalence of epilepsy in school children in Khartoum State. METHODS: This is an analytical population-based, cross-sectional study conducted in Khartoum State, Sudan. The study included students in the basic (primary) schools aged 6-14 years. Simple random sampling was used to draw a cluster of four of the seven localities comprising Khartoum State. The sample frame consisted of 1609 public schools (808,624 pupils) and 787 private schools (194,613 pupils), a total of 2396 schools (1,003,237 pupils). A sample size of 75 940 pupils was estimated and 250 schools were drawn from a sample frame of 2396 schools using a stratified random sampling technique. Consent was obtained from the headmaster/head teacher of the selected schools who arranged a meeting with the tutor/teacher responsible for each class. The study team asked whether any of the pupils was ever noticed or known to have had any kind of seizures, and a confidential letter was sent to the parents of each identified pupil. The letter included an explanation of the aims of the study, information on the research group and the kind of help the research group could offer; contact numbers and email addresses were made available if they wished to participate in this study. Those who consented to participate were then given an appointment at the Epilepsy Outpatient Department, Gaafar Ibnauf Children's Hospital, Khartoum where they were evaluated by the paediatric neurologist. RESULTS: Altogether, 74,949 pupils were enrolled for the study, 398 of whom were identified initially as having seizures and 332 of whom (83.4%) were identified by a paediatric neurologist. Of the 332, 303 (91.3%) proved to have epileptic seizures, 250 (82.5%) were known to have epilepsy, and 53 (17.5%) were newly diagnosed during the survey. The male to female ratio was 1.5:1. The total prevalence of epilepsy in Khartoum State was estimated to be 4/1000. The highest prevalence was in Jabal Awliya Locality (4.87/1000) and the lowest was in Khartoum Locality (3.35/1000). Twenty-nine (8.7%) patients proved to have non-epileptic seizures. The majority (15, 51.6%) had psychogenic non-epileptic seizures, and four (13.6%) had syncope. The majority (171, 56.43%) of patients had generalised epilepsy, 109 (35.97%) had focal epilepsy, and 23 (7.6%) had unclassified epilepsy. CONCLUSION: The prevalence of epilepsy in school children in Khartoum State (4/1000) is higher than that reported previously from Khartoum Province in 1983 (0.9/1000).

Pattern and outcome of renal diseases in hospitalized children in Khartoum State, Sudan.

In developing countries, renal diseases in children constitute important causes of morbidity and mortality. In Sudan, data about patterns and outcome of these disorders is generally scanty. We conducted this study to provide basic renal data that may be utilized by researchers and health planners in a resource poor setting. A retrospective record review of all pediatric patients, followed in four teaching hospitals in Khartoum State over a five-year period (January 2000-June 2004), was achieved. In 150 hospitalized children a total of 200 renal diagnoses were recorded. Urinary tract infection (UTI), occurring with other underlying renal morbidities or isolated, was the commonest renal diagnosis (20%). The second common renal disorders were nephrotic syndrome (NS) and urolithiasis/stones accounting for 16% and 15.5% of cases, respectively. Acute glomerulonephritis (AGN) and congenital anomalies were relatively less common (12% and 10.5%, respectively). Other less frequently detected diseases were acute renal failure (ARF) in 6%, chronic renal failure (CRF) in 4%, hereditary nephropathies in 3.5% and renal tumors in 2.5%. There was a significant correlation between the pattern of renal diseases and age of patients (P =0.001) but not their gender or social class (P = 0.211 and 0.34, respectively). On follow up, 99 out of 150 patients (66%) recovered their normal renal function, 6/150 (4%) remained with persistent proteinuria, 30/150 (20%) progressed to CRF, 10/150 (6.7%) died, and 5/150 (3.3%) were referred to radiotherapy department for further management. Our data reflects geographical variations of patterns of renal diseases in Sudanese children as in other countries. Many of these diseases are preventable or potentially curable. Therefore, improvement of pediatric renal services and training of health workers would help in early detection and treatment of these conditions leading to reduction in their morbidity and mortality.

Variable disease severity in Saudi Arabian and Sudanese families with c.3924 + 2 T > C mutation of LAMA2.

BACKGROUND: Congenital muscular dystrophy type 1A is caused by mutations in the LAMA2 gene that encodes the laminin α2 chain, a component of the skeletal muscle extracellular matrix protein laminin-211. The clinical spectrum of the disease is more heterogeneous than previously thought, particularly in terms of motor achievement and disease progression. We investigated clinical findings and performed molecular genetic analysis in 3 families from Saudi Arabia and 1 from Sudan in whom congenital muscular dystrophy 1A was suspected based on homozygosity mapping and laminin α2 chain deficiency. METHODS: We investigated 9 affected individuals from 1 Sudanese and 3 Saudi families in whom MDC1A was suggested by clinical, neuroimaging and/or pathological findings and by homozygosity mapping at the LAMA2 locus. Morphological and immunohistochemical analysis were performed in 3 patients from the 3 Saudi families. SSCP analysis, DNA sequencing and microsatellite analysis were carried out in the 4 index cases. RESULTS: A previously described mutation in the LAMA2 gene, a homozygous T > C substitution at position +2 of the consensus donor splice site of exon 26, was found in the 4 index patients. Clinical evaluation of 9 patients from the 4 families revealed variable disease severity particularly as regards motor achievement and disease progression. Microsatellite analysis showed an identical mutation-associated haplotype in the 4 index cases indicating a founder effect of the mutation in all 4 families. CONCLUSIONS: Our data provide further evidence that the clinical spectrum of MDC1A due to a single mutation is heterogeneous, particularly in terms of motor achievement and disease progression, making it difficult to give a reliable prognosis even in patients with identical LAMA2-associated haplotype. The c.3924 + 2 T > C mutation to date has been found only in patients originating from the Middle East or Sudan; therefore laminin 2 chain deficiency in patients from those regions should initially prompt a search for this mutation.

Acute phase reactants in Sudanese children with severe protein-energy malnutrition.

The pre-dietary rehabilitation levels of acute phase proteins (APP) namely, alpha-1-antitrypsin (AAT), orosomucoid (ORO), haptoglobin (HAP), fibrinogen (FIB) and C-reactive protein (CRP) in the plasma of Sudanese children with severe protein energy malnutrition (PEM) were compared with those of normal controls, and with the levels after dietary rehabilitation. Eighty one children were included in the study; 49 with severe PEM (23 with marasmus, 17 with marasmic-kwashiorkor and 9 with kwashiorkor), 13 with tuberculosis (TB) and 19 healthy children as controls. The study showed a high incidence of infections, especially acute respiratory infection (ARI), diarrhoeal diseases and intestinal parasites in the malnourished children. The mean plasma level of albumin was significantly lower in the malnourished children compared to controls (P<0.001), with kwashiorkor children showing the lowest mean level. This hypoalbuminaemia was significantly associated with the presence of ARI and intestinal parasites. The mean plasma levels of the APP, except FIB, were significantly higher in malnourished children than in controls, with higher levels associated with ARI and the presence of fever. Malnourished children with TB had significantly higher mean levels of the APP (AAT, HAP, FIB, CRP) compared to those without TB. The mean levels of HAP and AAT were significantly lower in the presence of diarrhoea, suggesting their loss in the stool. The mean levels of the APP after two weeks dietary rehabilitation and antimicrobial treatment showed a significant drop in only two of the APP, namely CRP, ORO, while FIB showed a significant rise.

Infection and immunoglobulin levels in Sudanese children with severe protein-energy malnutrition.

A hospital-based case control study was carried out to determine the pattern of infections and immunoblobulin levels in Sudanese children with severe protein energy malnutrition (PEM). The pre-dietary rehabilitation levels of the three major immunoglobulins (IgG, IgA and IgM) were compared with those of normal controls, and with the levels after dietary rehabilitation. Eighty one children were included in the study: 49 with severe PEM (23 with marasmus, 17 with marasmic - kwashiorkor and 9 with kwashiorkor), 13 with tuberculosis and 19 healthy children as controls. The study showed high incidence of infections, especially pneumonia and gastrointestinal infections in the malnourished children. Of special concern was the high incidence of urinary tract infection: 13 (26.5%) had significant pyuria and 9 of them had positive urine cultures, mainly Escherichia coli. Eight of the malnourished children also had pulmonary TB, and the ESR and Mantoux tests were not helpful in the diagnosis. The Mantoux test was negative in 88.8% of the malnourished group compared to 62.5% in those malnourished with TB. The malnourished groups had significantly higher plasma levels of the 3 immunoglobulins. While the maramic group attained significantly higher levels of IgG and IgA compared to the marasmic -kwashiorkor and kwashiorkor groups, the 3 groups of PEM showed a uniformly higher level of the IgM. After 2 weeks of rehabilitation, the levels of the 3 immunoglobulins showed no significant changes, except for the IgA which significantly decreased in all malnourished and the oedematous groups, and the IgM which increased significantly in the oedematous group.