Scielo RSS <![CDATA[South African Journal of Child Health]]> vol. 8 num. 1 lang. pt <![CDATA[SciELO Logo]]> <![CDATA[<b><i>SAJCH</i></b><b> online: Heading for a brighter future</b>]]> <![CDATA[<b>Waardenburg syndrome in childhood deafness in Cameroon</b>]]> BACKGROUND: Waardenburg syndrome (WS) is a rare hereditary disorder essentially characterised by deafness and pigment disorders of the eyes, hair and skin. METHODS: Between October 2010 and December 2011, we identified six patients with WS during an aetiological survey of 582 deaf participants recruited in schools for the deaf and ear, nose and throat outpatient clinics in seven of the ten regions of Cameroon. Two classic characteristics of WS were used as diagnostic criteria: deafness and pigmentation abnormalities (heterochromia iridis, white forelock and depigmented skin patches). In addition, to identify dystopia canthorum, a sign of WS type I, we calculated the W-index. RESULTS: WS comprised 1% of the whole sample, 7% of the genetic cases, and 50% of the genetic syndromic cases. All patients with WS had severe to profound congenital sensorineural and symmetrical hearing loss with flat audiograms. They also had pigment disorders of the eyes and the skin. In the absence of dystopia canthorum, they were all classified as having WS type II. The pedigree was suggestive of autosomal dominant inheritance in two cases, and the four others seemed to be de novo cases. CONCLUSION: The results suggest that WS type II is the most common syndromic form of hearing loss among Cameroonians. This has implications for retrospective genetic counselling and hearing tests for earlier management in affected families. <![CDATA[<b>A study investigating sound sources and noise levels in neonatal intensive care units</b>]]> BACKGROUND: Exposure to noise in the neonatal intensive care unit (NICU) has the potential to affect neonatal auditory development, sleep patterns and physiological stability, thus impacting on developmental progress. OBJECTIVES: This study aimed to identify noise sources in three NICUs in Johannesburg, South Africa, and to determine the sound levels to which neonates in incubators are exposed at various positions in the NICU. These findings were then compared with the standards recommended by the American Academy of Pediatrics (AAP). METHODS: A prospective, repeated designs measure was adopted. Sound levels were recorded in five different areas of the NICUs at four different times of the day over the course of 3 days. Data were analysed using descriptive statistics. RESULTS: Sound levels recorded in all the NICUs were considerably higher than those recommended by the AAP, placing vulnerable neonates at risk for developmental disorders. Significantly, most of the noise was human-generated, highlighting the need to develop awareness of the negative effects of noise in the NICU and to implement programmes to reduce noise. CONCLUSION: The findings have important implications for neonatal care, and highlight the importance of noise reduction and monitoring strategies in the NICU. <![CDATA[<b>Predictors of prolonged hospitalisation in childhood pneumonia in a rural health centre</b>]]> BACKGROUND: Pneumonia is a major cause of morbidity and mortality in under-5 children. Caring for children with pneumonia, especially in the case of prolonged hospitalisation, is a daunting task in resource-poor countries where caregivers are required to pay for treatment 'out of pocket' at the point of care. These children are often discharged against medical advice with incomplete treatment, with a consequent high rate of re-admission and complications. OBJECTIVE: To determine factors that predict prolonged hospitalisation among under-5 children with severe pneumonia admitted to a comprehensive health centre in rural Gambia. METHODS: We prospectively assessed 420 consecutive under-5 admissions with severe pneumonia, diagnosed using World Health Organization criteria, for factors in patient history, examination or investigations that could predict a hospital stay longer than 5 full days. RESULTS: Over the 6-month study period, pneumonia accounted for 27.6% of the total number of under-5 admissions. The mean age of children with pneumonia was 18.0 months (standard deviation (SD) ±13.7), there was a male to female ratio of 1.2:1, and 40.0% of the children were infants. Their mean hospital stay was 4.5 (SD ±3.0) days and 105 (25.0%) of the children had a prolonged hospital stay. Head nodding (odds ratio (OR) 1.929; 95% confidence interval (CI) 1.124 - 3.414; p=0.018), the presence of oedematous protein-energy malnutrition (OR 5.813; CI 1.060 - 3.616; p=0.006), severe wasting (OR 2.071; CI 1.047 - 3.241; p=0.034) and hypoxaemia at presentation (OR 2.220; CI 1.097 - 3.405; p=0.023) were independent predictors of prolonged hospital stay. CONCLUSION: Caregivers of children hospitalised with pneumonia and severe wasting, head nodding, oedema or hypoxaemia should be counselled at admission about the possibility of prolonged hospitalisation. <![CDATA[<b>Whose child is dying? Household characteristics and under-5 mortality in Nigeria</b>]]> BACKGROUND: Over 2 000 under-5-year-olds die daily in Nigeria from vaccine-preventable diseases, placing the country as the third largest contributor to the global under-5 mortality rate. Nigeria is at serious risk of not meeting the Millennium Development Goal (MDG) of reducing child mortality by two-thirds (i.e. from an under-5 mortality rate of 93/1 000 in 1990 to 31/1 000 in 2015). OBJECTIVE: To examine the association between household-level variables and under-5 mortality in Nigeria. METHODS: Data were drawn from the 2008 Nigeria Demographic and Health Survey, which elicited information on demographic and health indicators at the national and state levels. A nationally representative sample of 36 800 households was selected. Data were collected from 33 385 women of reproductive age (15 - 49 years) and who had given birth to at least one live infant in the 5 years preceding the survey. Data were analysed using a multilevel-model approach. RESULTS: In total, there were 104 808 live births; 18 121 (17.29%) children died as under-5s and 86 687 (82.71%) survived. Poverty, number of children ever born in a household, number of under-5s in the household, place and region of residence, maternal and paternal age, and maternal and paternal education level were critical determinants of under-5 mortality. CONCLUSION: The rate of under-5 mortality remains high in Nigeria. This will not be resolved until household-focused interventions are implemented using a tailored framework, and the need to improve maternal education in the country is addressed. <![CDATA[<b>Reticulocyte haemoglobin content as a diagnostic tool for iron deficiency and iron-deficiency anaemia in ill infants and children</b>]]> BACKGROUND: The diagnosis of iron deficiency (ID) and iron-deficiency anaemia (IDA) in ill children is complicated by the unreliability of serum ferritin (S-ferritin). The presence of a microcytic, hypochromic anaemia suggests IDA but is not specific. There is a need for a diagnostic test that will be accessible, cost-effective and accurate for the diagnosis of ID in ill children. Studies done in healthy children have reported that reticulocyte haemoglobin content (CHr) is a reliable diagnostic test for ID, eliminating the need for S-ferritin determination. OBJECTIVE: To evaluate the accuracy of CHr to diagnose ID and IDA in ill infants and children. METHODS: A prospective, descriptive study was conducted. One hundred children, aged 6 months to 6 years, who were admitted to Pelonomi Regional Hospital, Bloemfontein, South Africa, during July 2012 and August 2012 were included. RESULTS: The study group was divided into an iron-deficient group and an iron-sufficient group based on transferrin saturation (TfS). A statistically significant difference was found between mean corpuscular haemoglobin, serum transferrin and CHr in these two groups (p=0.0001). The sensitivity of a CHr level <29 pg to detect ID was 86%, and the specificity was 50%. CONCLUSION: CHr is an accurate diagnostic test for ID, and for IDA in combination with a low haemoglobin level, in ill infants and children. <![CDATA[<b>Rubinstein-Taybi syndrome: Dental manifestations and management</b>]]> Rubinstein-Taybi syndrome (RSTS) is an uncommon genetic disorder characterised by a typical facies, small stature, broad angulated thumbs and intellectual impairment. Dental changes are a minor, yet significant component of the condition. Craniofacial growth retardation in RSTS is frequently complicated by unerupted teeth, while dental caries is related to the inherent intellectual deficit. Dental problems necessitate interdisciplinary management in terms of oral surgery, conservative dentistry, periodontics and orthodontics. When affected individuals are unco-operative, certain dental procedures may warrant general anaesthesia. In these instances, dental and medical staff will combine their expertise to enhance the well-being of the patient. In addition, specific dental changes may alert the medical practitioner to the possible diagnosis of RSTS. In this article we document the oro-dental manifestations and review the oro-dental approach in the management of three patients with RSTS. Our experience in South Africa may be relevant to other countries at a similar stage of development. <![CDATA[<b>Salivary gland anlage tumour - a rare cause of congenital nasal obstruction</b>]]> Salivary gland anlage tumour, also known as congenital pleomorphic adenoma, is a rare salivary hamartoma that presents in the nasopharynx of neonates. It is important to distinguish this lesion from other causes of neonatal respiratory distress. Treatment usually involves simple excision, and recurrence is rare. No cases of malignant progression have been reported in the literature. We report an interesting case referred to our unit a day after birth with respiratory distress. <![CDATA[<b>The mystery of the disappearing eye: A neonatal case report</b>]]> Neonatal endophthalmitis is a rare but life-threatening condition that can have both endogenous and exogenous origins. Early diagnosis and aggressive treatment are vitally important to prevent death and disability, but mortality rates remain high. This case report demonstrates the rapid progression of the condition, which resulted in severe morbidity. <![CDATA[<b>A case of incomplete and refractory Kawasaki disease</b>: <b>Diagnostic and therapeutic challenges</b>]]> The diagnosis of incomplete Kawasaki disease (KD) - i.e. cases that do not fulfil all the diagnostic criteria - requires a high index of suspicion. We report a case of incomplete KD that was refractory to treatment with intravenous immunoglobulin and subsequently responded to treatment with intravenous methylprednisolone. <![CDATA[<b>Plasmablastic lymphoma in childhood: A report of two cases</b>]]> Plasmablastic lymphoma is an aggressive non-Hodgkin lymphoma predominantly seen in adult patients. Only eight cases of plasmablastic lymphoma in children have been published to date. In this report, we present an additional two cases. The first patient was a 9-year-old girl presenting with a nasal mass, while the second was a 15-year-old girl with swelling of the right side of the face and proptosis. Both patients were HIV-positive.