3. Electronic Theses and Dissertations (ETDs) - All submissions
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Item Clinical and laboratory presentation of classical galactosaemia in infants and children at Chris Hani Baragwanath Academic hospital(2017) Monareng, MohamedBackground. Classical Galactosaemia is an autosomal recessive disorder of galactose metabolism due to a deficiency of galactose-1-phosphate uridyltransferase (GALT) enzyme. In the neonatal period it can be a life threatening disease with multi organ involvement and non-specific symptoms and signs. In infancy and childhood, it can present with cataracts and cirrhosis. Long-term complications include verbal dyspraxia, cognitive disability and hypergonadotrophic hypogonadism. Galactosaemia responds to a galactose free diet and early diagnosis reduces morbidity and mortality, especially in early infancy. Objectives. To determine the number of cases with classical galactosaemia seen at Chris Hani Baragwanath Academic Hospital (CHBAH) Paediatric Gastroenterology, Hepatology and Nutrition Unit (PGHNU) Soweto Gauteng, between January 1994 and December 2013 and to document the clinical, laboratory characteristics and outcome of these patients. Methods. All children diagnosed at PGHNU with classical galactosaemia between January 1994 and December 2013 were included in the study. Clinical and laboratory parameters were determined and analysed. Outcome and long-term complications were documented. Results. Twenty-three children with classical galactosaemia were diagnosed during the study period. All patients had severely low or absent GALT enzyme activity. Mean age at diagnosis was 4.6 months (age range 3 -24 months). The commonest presenting symptoms were yellow discolouration of the eyes (74%), abdominal distension (65%) and failure to gain weight (57%). Most frequent clinical features at diagnosis were hepatomegaly (100%), pallor (78%), jaundice (78%), ascites (61%) failure to thrive (52%) and cataracts (55%). Laboratory findings included metabolic acidosis (78%), coagulopathy (65%) and liver derangements (61%). Anaemia was noted in 78% of the patients, which was primarily macrocytic. Eight out of the 23 patients presented with evidence of infection. Long-term complications in those assessed, included visual problems (12/18patients), developmental delay(9/16patients) and speech impairment (4/9patients). Conclusion. The study provides information on the clinical, haematological and biochemical presentation of galactosaemia thus increasing the awareness of the condition and encouraging strategies aimed at early recognition. This will prevent on- going damage to target organs in postnatal life. The study also documents the presence of long-term complications in the South African black child with classical galactosaemia, encouraging a structured follow up programme to prevent, identify and manage complications such as neurodevelopmental delay and speech impairement.Item A randomised trial of the safety and immunogenicity of low dose haemophilus conjugate vaccine in healthy infants at 6,10, and 14 weeks of age(2002-09-01) Nicol, Mark. Patrick.Background Despite their proven efficacy, Haemophilus influenzae type b (Hib) conjugate vaccines are not given to most children in the developing world in the face of an estimated global Hib disease burden of nearly 2 million cases per annum. A major barrier to the introduction of the vaccine would be overcome by diluting the vaccine tenfold in DTP. We report a randomised trial comparing the use of Hib conjugate vaccine diluted tenfold in a multidose vial of DTP, with that of the full Hib dose.Item The extension of the infant gross motor screening test to include infants from birth to five months in infants with HIV(2018) Otten, Kirsty MaeThe aim of this study was to extend the age range of the Infant Gross Motor Screening Test (IGMST) to make it appropriate for use in infants infected with HIV from birth to five months. Previously completed Bayley Scales on Infant and Toddler Development (3rd Version) (BSID III) assessments, from two 2013 physiotherapy masters studies completed through the University of the Witwatersrand, were used to compile neurodevelopmental scores achieved by infants infected with HIV. These scores were used to select developmentally appropriate items for inclusion in the new section of the IGMST. These items were statistically tested against the original BSID III scores using the Pearson correlation coefficient. Following statistical testing, content validity of the new section of the IGMST was established with an expert panel using a modified nominal group technique (NGT). The new section of the IGMST included two age bands; one to three months and four to five months. Each age band consisted of five items. Age band one to three months had a Pearson correlation coefficient of r =0.68 (p = 0.003) and 84% of the infants performed the same on both tests. Age band four to five achieved a Pearson correlation coefficient r = 0.71 (p = 0.0009) and 83% of infants performed the same on both tests. When presented to the expert panel using a modified NGT, all items and questions raised were accepted by 100% after appropriate adjustments were made. In conclusion, the new section of the IGMST has been developed for infants from one to five months and the content validity has been established during the course of this study. The new section must still undergo further validity and reliability testing before it can be used clinically.Item Infants of diabetic mothers: maternal and infant characteristics and incidence of hypoglcemia(2016) Magadla, YoliswaBackground: Diabetes mellitus is the most common metabolic disease affecting women during pregnancy and is associated with adverse outcomes during the neonatal period, common one being hypoglycemia. The characteristics and incidence of hypoglycemia in infants of diabetic mothers (IDM) are not well reported in South Africa. Objectives: To describe the characteristics of IDM with or without hypoglycemia and to determine prevalence of hypoglycemia in IDM. Methods: Medical records of mothers, and their infants admitted with a diagnosis of IDM at gestational age ≥34 weeks and/ or birth weight ≥2000 grams and admitted at CHBAH from January 2012 to December 2013, were retrieved. Maternal characteristics, type and treatment of diabetes, infant characteristics and glucose measurements were captured for analysis. Results: A total of 234 IDM were admitted over this 2-year period and 207 met inclusion criteria. Median maternal age was 33 years. Seven percent of mothers had stillbirths and 14% had miscarriages in previous pregnancies. A total of 56% of mothers had gestational diabetes. Among infants, 54% were born preterm, 19% were large for gestational age (LGA) and 10% were macrosomic. Pre-gestational diabetic mothers had higher preterm births than gestational diabetic mothers (64% vs 48%, p=0.037). Hypoglycemia occurred in 39% of IDM, occurring within the first 3 hours of life in 85% of infants. There were no statistically significant differences in types of maternal diabetes and its treatment between hypoglycaemic and normoglycaemic infants, but hypoglycaemic infants were more likely to be LGA (28.2% vs 12.8%, p=0.009). Conclusion: Hypoglycemia is a common finding in IDM, presenting early in postnatal age. Only just over a quarter of hypoglycaemic infants are LGA. All IDM should be monitored for hypoglycemia, especially within the first 3 hours of life.Item Epidemiology of acinetobacter sepsis in infants admitted to a neonatal unit(2016) Thomas, ReenuBackground: Acinetobacter baumannii (A. baumannii) is emerging as one of the pathogens causing sepsis in neonates. Prevalence, antibiotic susceptibilities and case-fatality rate (CFR) of A. baumannii in the neonatal units are not well known. Objective: To determine the prevalence, antibiotic susceptibility patterns and CFR of A. baumannii infection in neonates. Methods: Medical records of neonates admitted to Chris Hani Baragwanath Academic Hospital from 1st October 2007 to 31st October 2011 with a positive blood or cerebrospinal fluid culture due to A. baumannii were reviewed for demographic characteristics, clinical presentation, laboratory findings, antibiotic susceptibility and outcome. Results: There were 399 isolates of A. baumannii, with a prevalence of 4.3/1000 live births or 2/ 1000 patient-days, and accounting for 13% of all bacterial and fungal isolates. Antimicrobial susceptibility results were available for 379 isolates and only 155 medical records could be retrieved for analysis. The mean gestational age and birth weight of infected neonates was 30 weeks and 1400 grams respectively. Thirty seven (24%) were isolated from neonates with early onset sepsis and 118 (76%) from those with late onset sepsis. Sixty four percent of isolates were susceptible to Cephalosporins, 21% to Aminoglycosides and 17% were multi-drug resistant (MDR) isolates. The CFR was 32%. Factors associated with mortality were presence of a central venous catheter prior to onset of sepsis (49% vs 31%, p=0.03); need for ventilatory support (62% vs 36%, p=0.005) and inotropic support (57% vs 17%, p<0.001). Conclusions: A. baumannii is a common pathogen causing sepsis in neonates, with 17% of them being MDR. It is associated with high CFR. These findings highlight the need for strict enforcement of infection control and antibiotic stewardship practices.Item Characteristics of infants exposed to maternal tuberculosis and chemoprophylaxis using three months of isoniazid and rifampicin(2016) Mathivha, Khakhu TshilidziBackground: Though features of infants with congenital tubercuiosis (TB) are known including being low-birth-weight (LBw), features of in-utero TB-exposed infants including non-infected are not well reported. Infants bom to TB-infected women are at risk of contracting TB post-delivery, therefore chemoprophylaxis is recommended, and this includes use of isoniazid and rifampicin combination, but littie is known about its effectiveness. Objective: To determine features of in-utero TB-exposed infants and proportion with TB after chemoprophylaxis with isoniazid and rifampicin. Methods: Retrospective review of records of TB-infected women and their infants, from ZA07-20rc. Clinical features of mothers and infants at time of delivery; and follow-up of infants after completion of isoniazid and rifampicin are described. Results: Eighty-eight infants bom to 86 women with a diagnosis of TB were studied. TB diagnosis was made peripartum in24.4Yoof women, 23.3%had exka-pulmonary TB. Among those diagnosed antepa$um 46.2o/owere on treatment for >2 months. Human immunodeficiency virus (HIV) was positive in 97.7Yo;wi& CD4 count <200 cells/mm3 in 74'6yo' Eight mothers (9.3%) died before discharge. There were 56 {63.6%)LBW and 45 (51'2W preterun infants. Culture for acid-fast-bacilli was positive in 4 (4.5%)infants. At 3- months follow-up, 17 (20.2%)defaulted, and among 67 who returned, 7 OAoA)did not return for Mantoux test reading, 1160 (1.7%)had positive Manroux. Conclusion: Majority of TB-exposed infants are born to mothers with TB/ HIV co-infection. A high proportion of TB-exposed infants are born preterm and LBW. The high attrition rate made it difficult to assess effectiveness of chemoprophylaxis with isoniazid and rifampicinItem Neurodevelopmental delays in children with perinatally acquired human immunodeficiency virus infection, with respect to antiretroviral therapy initiation and virological suppression(2013) Strehlau, RenateHuman Immunodeficiency Virus (HIV) infection in infancy may influence the developing brain and lead to adverse neurodevelopmental consequences. We aim to describe the neurodevelopmental characteristics of a cohort of young children infected with HIV prior to antiretroviral therapy (ART) initiation and after achieving viral suppression. A retrospective analysis of data collected as part of a randomised equivalence trial between April 2005 and May 2009, at a hospital in Johannesburg, South Africa. 195 HIV-infected children under 2 years of age were assessed. A simple, inexpensive screening questionnaire (Ages and Stages Questionnaire - ASQ) was used to identify neurodevelopmental delays. The ASQ was administered prior to ART initiation, and again after viral suppression on a protease inhibitor-based regimen had been achieved. Median age pre-ART was 8.8 months (range 2.2 - 24.9), 53.9% were male. Mean time to viral suppression was 9.4 months (range 5.9 - 14.5) and the ASQ was administered to 108 caregivers at this time. Compared to pre-ART, at viral suppression, there was significant reduction in the proportion of children failing the gross motor (31.5% vs. 13%, p<0.01), fine motor (21.3% vs. 10.2%, p=0.02), problem solving (26.9% vs. 9.3%, p<0.001) and personal social (17.6% vs. 7.4%, p=0.02) domains. The proportion of children failing the communication domain was similar at each time point (14.8% vs. 12%, p=0.61). At time of viral suppression 10.2% failed at least one of the five domains. Achieving viral suppression on ART resulted in significant improvements in the neurodevelopmental function of young HIV-infected children, however, neurodevelopmental problems still persisted in a large proportion. Appropriate screening for neurodevelopmental delay and timely referral could help improve outcomes.Item An evaluation of the health related quality of life of children with HIV/AIDS(2011-11-10) Goldberg, Linda HazelIn 2008, 1.8 million children under the age of 15 were living with HIV/AIDS in sub-Saharan Africa. The same report estimates that in 2008, there were 390 000 new infections in children below the age of 15 in Sub- Saharan Africa. Children appear to be the generation most affected by the HIV/AIDS epidemic. With the introduction of Highly Active Antiretroviral therapy (HAART) more perinatally infected children are living into adolescence and beyond. They will have to learn to live with a stigmatising, potentially fatal chronic illness. Health care workers can no longer rely solely on traditionally used outcome measures, such as viral loads and CD4+ percentages, to monitor effectiveness of interventions and treatments. Quality of Life (QoL) has been suggested as an additional essential outcome measure in clinical practice and research involving children living with a chronic illness. In this research the concept of Health Related Quality of Life (HRQoL) is evaluated in HIV-infected children using the PedsQL 4.0 Generic Core Scale (child self-reports, ages five-seven). The PedsQL 4.0 Generic Core Scales has been found to be a valid and reliable HRQoL measurement tool in children with chronic diseases, school-going children and children infected with HIV. Domains of Physical, Emotional, Social and School Functioning were evaluated. The children in the comparison group scored significantly higher (p<0.01) indicating a better quality of life. The HIV-infected children scored significantly lower in all four domains, with Physical Functioning being most affected (p<0.01). The children with HIV were found to be shorter (p<0.01) and lighter (p<0.01) than those in the comparison group. This could be a contributing factor to the physical difficulties experienced by the HIV-infected children. No relationship could be established between total scores of HRQoL and CD4+ percentages, viral load and duration of HAART treatment. Demographic data collected indicate that the HIV-infected children were more likely to have a primary caregiver with a lower level of education (p=0.01) and more likely to be receiving a Dependency Care Grant (p=0.05). The HRQoL results of this study are similar to those conducted in other parts of the world. The results stress the need for a multi-disciplinary approach when treating HIV-infected children. It has become essential to focus on the medical, physical and psychosocial functioning of the HIV-infected child thereby promoting participation in the family, school and the broader community.