Clinical Profile and Outcome of Pediatric Demyelinating Disorders in Calabar, Nigeria: A Case Series.

BACKGROUND: Demyelinating disorders of the central nervous system (CNS) are rare disorders characterized by inflammation and the selective destruction of CNS myelin. The incidence of this disorder is increasing in developed countries. Nigerian studies on the pediatric population on the subject are very scarce. AIMS: The aim of the study was to document the epidemiology, clinical profile, and impact of late presentation on the treatment outcome of demyelinating diseases of the CNS in pediatric patients. METHODS: The retrospective review of patients aged 1-15 years admitted in a tertiary hospital from January 2018 to December 2022 with various symptoms suggestive of demyelinating CNS disorders. The diagnosis was clinically and radiologically confirmed. Information retrieved from the case notes included patients' demographics, clinical symptoms and signs, number of days with symptoms to presentation in the hospital, results of the magnetic resonance imaging (MRI), treatment, and treatment outcomes. Data were entered in Excel sheet and results were presented in tables and percentages. RESULTS: The incidence of demyelinating disorders over the period was 0.013% (10 out of 769 patients admitted over the period). Acute demyelinating encephalomyelitis (ADEM) was the most common disorder seen in the study population (60%, n = 6), followed by transverse myelitis and two (20%) had optic neuritis (ON). Most of the patients with ADEM were in the 1-5-year age group. The female-to-male ratio was 2.3:1. Paraplegia, visual impairment, and ataxia were the most common clinical presentations in the study population. One of the patients met the criteria for the diagnosis of multiple sclerosis during follow-up. Human immunodeficiency virus (HIV) was identified as the cause of demyelination in one case. Most of the patients improved with steroids. CONCLUSION: ADEM was the most common clinical phenotype seen in this study. Patients with ADEM and ON had a better prognosis than transverse myelitis. Late presentation was also identified as a poor prognostic factor. Follow-up of cases is very important to monitor disease progression to multiple sclerosis.

Seizure-related death in children with epilepsy

Children with epilepsy have a significantly higher rate of death than the general population. The cause of premature death among epileptics is contributed by the underlying cause, coexisting neurologic compromise and the epilepsy itself. Mortality directly related to seizures in paediatric epilepsy is the subject of this review. Seizure-related deaths in epileptics arise from status epilepticus, aspiration pneumonia, as well as sudden and unexpected death in epilepsy (SUDEP). Epilepsy per se enhances the risks of accidents and suicide. Children with epilepsy who are otherwise neurologically intact and with normal neuroimaging findings have an exceedingly low risk of seizure-related death. Poor compliance to antiepileptic drugs (AED) , poor ly co nt rol led (intractable and refractory) seizures, impaired cognition, structural/ metabolic aetiology and antiepileptic drug polypharmacy tend to carry poor prognosis. Therefore, parents need to be appropriately advised about the risk of seizurerelated premature death. Early identification, compliance with AED prescription, and treatment of comorbid conditions can reduce mortality risk and improve health outcomes in children with epilepsy. Children with intractable types of epilepsy may benefit from medical marijuana and neurosurgery

Stray dog trade fuelled by dog meat consumption as a risk factor for rabies infection in Calabar, southern Nigeria.

BACKGROUND: Rabies is a preventable zoonosis with the highest case fatality of any disease in the world. In the developing world, it is transmitted mainly by dog bites. In parts of southern Nigeria, dog meat is a delicacy. OBJECTIVE: To highlight trade in stray dogs as a major risk factor for rabies in animals and humans in south-south Nigeria. METHOD: Patients admitted into the University of Calabar Teaching Hospital (UCTH) with a diagnosis of rabies between July and October 2012 were analysed for risk factors, post exposure prophylaxis (PEP), health seeking behaviour and outcome. Focused group interview were also conducted among traders/handlers of stray dogs. RESULTS: Ten cases of rabies in subjects aged 3 to 52 years were recorded in these five months period. Eight of the cases were male and apparently got infected directly or indirectly through the trade in stray dogs for human consumption. None had proper PEP and all patients died. CONCLUSION: Stray dog trade, fuelled by eating of dog meat, is a risk factor for human and animal rabies in Calabar, southern Nigeria. Culling of stray dogs, control of stray dogs' trade and public enlightenment on PEP is recommended.

Recurrent pericardial effusion associated with hypothyroidism in Down Syndrome: a case report.

BACKGROUND: The complex of Down Syndrome-hypothyroidism-pericardial effusion is largely unreported in sub-Sahara. OBJECTIVE: To present and highlight an unusual manifestation of hypothyroidism. METHODS: A 16-year-old girl with confirmed Down Syndrome presented with complaints of generalised body swelling of eight months' duration. Her work-up consisted of full clinical and laboratory evaluation including ECG and echocardiography. RESULTS: The patient was diagnosed of Down's Syndrome at birth but was lost to follow-up after at eight years of age. Body swelling was associated with clinical features of heart failure. Echocardiography showed massive pericardial effusion. Serum l-thyroxine was less than 0.5mg/dl and TSH >40iu/l. The heart failure and menstrual irregularities responded to l-thyroxine therapy. CONCLUSION: This appears to be the first report of the complex of Down syndrome, hypothyroidism and pericardial effusion in a Nigerian child. Thyroid function test is therefore recommended as a part of baseline investigation for Nigerian children with Down syndrome as is the practice.

Junctional epidermolysis bullosa in a female Nigerian child: a case report.

AIM: To report the case of Junctional Epidermolysis bullosa seen in the University of Calabar Teaching Hospital, Calabar and to draw attention to the existence of this condition among Nigerians. SUBJECT AND METHODS: A Nigerian female infant, normal at birth, developed extensive blistering skin eruptions from the age of ten days. The lesions involved the face, neck, shoulders, upper trunk and the buccal mucosa of the lower lip sparing the limbs. The cornea were cloudy. The history, type and distribution of the lesions were consistent with Junctional Epidermolysis bullosa (EB). This was confirmed by skin biopsy and histology. Various medications including systemic and topical steroids, antibiotics and other topical creams only produce transient relief with frequent relapses. The Junctional EB type is known to be autosomal recessive in inheritance though there was no positive family history. Avoidance of heat, warm bath and trauma and genetic counseling can be helpful in reducing frequent relapses of the condition that has no specific treatment. CONCLUSION: This appears to be the first case of Epidermolysis bullosa to be reported from Calabar, south eastern Nigeria. This case report shows that this rare condition does affect people in the tropics. Efforts should always be made to reach a definitive diagnosis whenever unusual conditions are encountered in clinical practice.

Antenatal intrauterine fetal death: a prospective study in a tertiary hospital in south-western Saudi Arabia.

This study was conducted to determine the frequency of antenatal intrauterine fetal death (IUFD) and the associated maternal and fetal risk factors in women who presented at a regional tertiary hospital in Saudi Arabia. Over a 5-year period, 191 consecutive cases of IUFD presented and were studied prospectively. Relevant maternal and fetal data were obtained. The stillbirth rate during the period was 10 per 1000 deliveries. In 41% of the cases, the associated causal factors could not be determined. There was a significant (P = 0.00122) linear trend showing an increased risk of IUFD above the age of 20 years and, similarly, a significant (P = 0.00047) linear trend after the first pregnancy. Lack of antenatal care (88.5%), hypertensive diseases in pregnancy (12%) and diabetes mellitus (8%) were associated risk factors while 'major congenital malformation' (14%) was an outstanding fetal factor. The risk factors of antenatal stillbirth in our community seem to be avoidable. Health education to encourage the utilisation of the available antenatal care services, family planning and genetic counselling are being advocated strongly as possible preventive measures.

Screening for asthma and associated risk factors among urban school boys in Abha city.

OBJECTIVE: The objective of the present study was to measure the prevalence of asthma and asthma-related symptoms among male school children in Abha City and to determine some of the possible risk factors influencing its occurrence. METHODS: A randomly selected sample of 4300 male school children aged 7 to 15 years in Abha were subjected to a previously validated questionnaire for asthma to be completed by parents. Asthma was identified based on the Rush Medical College and International Study of Asthma and Allergies in Children questionnaire. Information of asthma family history, asthma related symptoms, and other atopic conditions, smokers in the family, pets ownership and monthly family income were collected. RESULTS: The overall prevalence of asthma was 9% (95% Confidence Interval: 7.73%-9.67%). Doctor-diagnosed asthma was reported by 4%, exercise-induced asthma by 4% and wheeze in the past year by 8%. Multiple logistic regression analysis showed that positive family history of atopic condition (Odds Ratio=437.11, P<0.001), pets ownership (Odds Ratio=2.91, p<0.001), and lower monthly family income (Odds Ratio=2.00, P<0.02) were significant factors influencing the development of asthma. CONCLUSION: In conclusion, the screening methodology adopted in this study could be applied for all children at the beginning of the school year, being simple and non-invasive measure. The prevalence of asthma in school children in Abha is greater than that reported from most developing countries and closer to the rates reported in developed countries. Avoidance of pets ownership at home, improving social class and premarital counselling for atopic persons are all recommended.

Neonatal meningitis.

OBJECTIVE: To determine the prevalent bacterial agents of neonatal meningitis and their antibiotic susceptibility in a referral intensive care unit in Assir Central Hospital, Saudi Arabia, during the years 1993-1998. METHODS: Records of newborn infants with positive cerebrospinal fluid culture during the period were retrospectively studied. RESULTS: There were 1473 nursery admissions, of which 32 episodes of meningitis occurred amongst 31 neonates. Klebsiella pneumoniae (31%) and Serratia marcescens (21%) were the main pathogens. The incidence of concurrent septicemia among these infants was 58%. Klebsiella pneumoniae appears to dominate in both early and late onset infections. The sex incidence was equal and the mortality rate was 48%. CONCLUSION: The survey identifies Klebsiella pneumoniae and Serratia sp. as the leading bacterial agents of neonatal meningitis in our environment. The relatively high frequency of Serratia infection in the present survey appears unique as this organism is comparatively rare in other reports across the globe. No Group B Streptococcus was isolated, which is in contrast to reports obtained in Europe, America and Australia where it is the predominant organism of neonatal sepsis or meningitis. Antibiogram identified imipenem and cefotaxime as the empirical antibiotics in infants with a clinical diagnosis of neonatal sepsis in our hospital; no more conventional use of ampicillin. In view of the changing bacterial pattern of infant infection with time even in the same environment, a periodic review of this subject is advocated.

Neonatal meningitis.

OBJECTIVE: To determine the prevalent bacterial agents of neonatal meningitis and their antibiotic susceptibility in a referral intensive care unit in Assir Central Hospital, Saudi Arabia, during the years 1993-1998. METHODS: Records of newborn infants with positive cerebrospinal fluid culture during the period were retrospectively studied. RESULTS: There were 1473 nursery admissions, of which 32 episodes of meningitis occurred amongst 31 neonates. Klebsiella pneumoniae (31%) and Serratia marcescens (21%) were the main pathogens. The incidence of concurrent septicemia among these infants was 58%. Klebsiella pneumoniae appears to dominate in both early and late onset infections. The sex incidence was equal and the mortality rate was 48%. CONCLUSION: The survey identifies Klebsiella pneumoniae and Serratia sp. as the leading bacterial agents of neonatal meningitis in our environment. The relatively high frequency of Serratia infection in the present survey appears unique as this organism is comparatively rare in other reports across the globe. No Group B Streptococcus was isolated, which is in contrast to reports obtained in Europe, America and Australia where it is the predominant organism of neonatal sepsis or meningitis. Antibiogram identified imipenem and cefotaxime as the empirical antibiotics in infants with a clinical diagnosis of neonatal sepsis in our hospital; no more conventional use of ampicillin. In view of the changing bacterial pattern of infant infection with time even in the same environment, a periodic review of this subject is advocated.

Faecal excretion of Vibrio cholerae during convalescence of cholera patients in Calabar, Nigeria.

The pattern of faecal excretion of Vibrio cholerae was studied over a duration of eight months among 13 cholera convalescents by two-weekly surveillance cultures. Stools and rectal swabs were cultured on Thiosulphate citrate bile salts sucrose (TCBS) agar for the recovery of vibrio pathogens. Clinical phase and convalescent phase V. cholerae strains were compared for antibiogram profiles. The population of vibrios recovered from faecal inocula was usually scanty (<10(3) vibrios/g). All clinical isolates except three were concordant with convalescent phase strains. Sensitivity to tetracycline was uniform for concordant V. cholerae strains, with minimum inhibitory concentration (MIC) ranging from 0.54-4.0 microg/ml. Nine (69.2%) of the convalescents had positive faecal cultures for periods ranging from two weeks to more than seven months. Two adults whose excretions lasted several months also tested positive for human immuno-deficiency virus (HIV) infections. The significance of stool surveillance cultures for identifying asymptomatic infections among convalescents who may need chemotherapy to abolish excretion is emphasised. However, it could not be established with certainty if vibrios excreted during convalescence were from enteric colonization by the causative strains, or re-infections with the common strains in circulation.

Congenital diaphragmatic hernia.

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Neonatal septicemia.

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Congenital hydrocephalus due to aqueductal stenosis of probable autosomal recessive inheritance.

Full text is available as a scanned copy of the original print version.

The pattern of neurological sequelae of childhood cerebral malaria among survivors in Calabar, Nigeria.

OBJECTIVE: To determine the pattern and long term outcome of neurological complications following cerebral malaria (CM) in a group of Nigerian children treated in Calabar. DESIGN: Prospective, follow up study. SETTING: Children's emergency room (CHER) of the University of Calabar Teaching Hospital (UCTH) located in a malaria-holoendemic rainforest belt of south eastern Nigeria. SUBJECTS: Survivors among 45 children with CM treated between February and December, 1991. All received intravenous quinine infusion and supportive care. Survivors (39) were followed up until detected neurological sequelae had resolved. RESULTS: Case fatality rate was 13.3%, 95% CI. Eleven (28.2%) of the survivors developed neurological sequelae. Prolonged coma, focal seizures and abnormal posturing (decorticate/decerebrate) were associated with increased risk of sequelae. Commonest neurological sequelae were cortical blindness (3/11), speech disorders (3/11: aphasia or echolalia) and motor abnormalities (5/11: dyskinesia/hemiplegia). Eight cases recovered completely from the neurological deficits within a mean period of three (1.3) weeks. One persisted with hyperactivity and attention deficit, had a remarkable improvement at the sixth month of follow up but developed secondary dyslexia and other learning disabilities by the third year of follow up. CONCLUSION: Although short lived, neurological sequelae of CM appear common among these Nigerian children. This problem could significantly add to the burden of childhood disability in Nigeria. Early diagnosis, use of appropriate drugs and large scale malaria control programmes can prevent malady.

The influence of breast feeding on the occurrence of dysentery, persistent diarrhoea and malnutrition among Nigerian children with diarrhoea.

A case-control study of children attending a diarrhoea treatment unit (DTU) in Calabar, Nigeria was undertaken to evaluate the association between failure to breastfeed on one hand, and dysentery, persistent diarrhoea and malnutrition on the other. The prevalence of dysentery, persistent diarrhoea and underweight were 6.3%, 1.2% and 36.4% respectively among a total of 1133 children with diarrhoea. The proportion of the children with persistent diarrhoea or underweight was significantly lower among currently breastfeeding children than the age-matched, non-breastfeeding counterparts (p < 0.05). Dysentery was also less frequent among breastfeeding children (5.8%) than the non-breastfeeding ones (7.4%), but this did not reach statistical significance. The findings lend support to the enormous benefit of breastfeeding as a child survival strategy. Global efforts geared towards the support of breastfeeding should be intensified especially the less developed countries where diarrhoea-related morbidity and mortality constitute a major public health problem.

PIP: The influence of breast feeding on dysentery, persistent diarrhea, and malnutrition was examined in a case-control study of 1133 children 0-3 years of age attending a diarrhea treatment unit at a university teaching hospital in Calabar, Nigeria. 485 children were currently breast feeding. 6.3% of children had dysentery (defined as blood in stool), 1.2% were afflicted with chronic diarrhea (lasting 14 or more days), and 36.4% were underweight ( -2 SD scores below the reference standard). The proportion of children with persistent diarrhea was significantly lower (p 0.05) among breast-fed than age-matched non-breast-fed children (0.4% vs. 1.9%). The proportion of children underweight was also significantly lower (p 0.01) among breast-fed than non-breast-fed children (35.9% vs. 49.6%). Although dysentery was less frequent in breast-fed than non-breast-fed children (5.8 vs. 7.4%), this difference was not statistically significant. These findings underscore the importance of breast feeding promotion as a child survival strategy in countries where diarrhea-related morbidity and mortality is a major public health problem.

Hemoglobin and hematocrit values of Saudi newborns in the high altitude of Abha, Saudi Arabia.

A study was designed to determine the red cell values (hemoglobin and hematocrit) of neonates born in the high altitude of Abha and to compare these values with known values of other lowland areas of Saudi Arabia. From the cord blood of 587 normal, appropriate for gestational age and term infants born in 1993 in Abha Maternity Hospital, the ranges of Hb and Hct were 130 to 240 g/L and 0.24 to 0.79 L/L respectively. The mean Hb was 187 g/L. There was no significant difference between the male and female values. Also, 17% of the infants in this study were polycythemic, while no polycythemia was recorded in these lowland areas, and only 2% to 4% in the general global newborn population. It was therefore revealed that Abha newborns had higher red cell values at birth when compared to other newborns in the low altitude areas of Riyadh and Jeddah (P <0.001). We postulate that the high altitude (2700 meters above sea level) of Abha, and therefore its relative hypoxia, has induced high red cell values in infants born in the city. This phenomenon therefore warrants the adoption of higher red cell reference values and not necessarily those already documented in other Saudi newborn populations.

Neonatal seizures in Nigerian infants.

Nigerian newborns presenting with convulsion in University of Calabar Teaching Hospital, Calabar during the period January 1989 to December 1990 were prospectively studied to determine the aetiology and pattern of their seizures. There were 60 patients representing 4% of admissions into the Newborn Unit during the period. Birth asphyxia, infections and hypoglycaemia were the important identifiable aetiological factors which operated either singly (48% of cases) or in concert (in another 48%) of the infants. Detectable infections included meningitis and septicaemia caused predominantly by coliforms and Staphylococcus aureus. Hypocalcaemia and electrolyte imbalance did not feature. There was an unusually high prevalence (63% of cases) of the generalised type of seizures probably due to the high frequency of mixed aetiology. The mortality rate of 50% encountered appears to be related to the underlying aetiology and prematurity. Detectable caused of neonatal seizures in our environment appear to be potentially preventable by improved obstetric and neonatal care. There is dire need also to provide modern facilities for investigating newborn seizures in order to improve upon the diagnostic yield.

PIP: During 1989-1990 in Nigeria, 60 newborns were admitted to the Special Care Baby Unit of the University of Calabar Teaching Hospital for seizures, representing 4.1% of admissions. 11.7% of the newborns with seizures were preterm. 48.3% had more than 1 detectable cause of the seizures. The leading cause of seizures was asphyxia (36 newborns) followed by infections (16), especially meningitis (8). Coliforms and Staphylococcus aureus were the primary etiologic agents for the infections. Among the 36 newborns with asphyxia, 13 also suffered from hypoglycemia, 13 also suffered from infection, and 5 also suffered from infection and hypoglycemia. 63% of the newborns had generalized seizures, which may be associated with the mixed etiology in 48% of the cases. 50% of the 60 newborns presenting with seizures died. Newborns whose seizures were associated with infection had the highest fatality rate (50%). The mortality rate was lowest in infants whose seizures occurred during the first day of life (36.7% vs. 86% 4-7 days after birth). The causes of neonatal seizures in these newborns can be prevented by improving obstetric and neonatal care. Pediatricians need access to modern diagnostic and therapeutic facilities to improve the accuracy of diagnoses in cases of newborn seizures. Health workers need to increase health education activities geared to prenatal patients.

Urinary symptoms and blood pressure of children with Schistosoma haematobium infection in south-eastern Nigeria.

The urinary symptoms and blood pressures of 510 children (aged 5-15 years) with Schistosoma haematobium infection in Ijiman community, Cross River State of Nigeria, were studied in 1992. The prevalence rate of infection was 44%, a majority of the children presenting with light infection. Significant symptoms were visible haematuria, dysuria, suprapubic pain and strangury. The sensitivity and specificity of these symptoms were too low for them to be recommended as distinct clinical diagnostic criteria. The impact of the disease could not be demonstrated on blood pressures of the children. Nevertheless, urgent control measures with emphasis on the provision of ventilated improved pit toilets and potable water are strongly recommended for the control of the disease as persistent infection is known to mask manifestation of severe complications till an older age.