South African Medical Journal - Volume 102, Issue 11, 2012
Volumes & issues
Volume 102, Issue 11, 2012
Factors influencing the development of early- or late-onset Parkinson's disease in a cohort of South African patients : researchSource: South African Medical Journal 102, pp 848 –851 (2012)More Less
Background. Neurodegenerative disorders such as Parkinson's disease (PD) contribute significantly to global disease burden. PD can be categorised into early-onset PD (EOPD) with an age at onset (AAO) of ≤50 years and late-onset PD (LOPD) with an AAO of >50 years.
Aims. To identify factors influencing EOPD and LOPD development in a group of patients in South Africa (SA).
Methods. A total of 397 unrelated PD patients were recruited from the Movement Disorders Clinic at Tygerberg Hospital and via the Parkinson's Association of SA. Patient demographic and environmental data were recorded and associations with PD onset (EOPD v. LOPD) were analysed with a Pearson's Chi-squared test. The English- and Afrikaans-speaking (Afrikaner) white patients were analysed separately.
Results. Logistic regression analysis showed that ethnicity (p<0.001) and family history (p=0.004) were independently associated with AAO of PD. Average AAO was younger in black, coloured and Afrikaner patients than English-speaking white patients. A positive family history of PD, seen in 31.1% of LOPD patients, was associated with a younger AAO in the study population.
Conclusions. These associations may be attributed to specific genetic and/or environmental risk factors that increase PD susceptibility and influence the clinical course of the disorder. More studies on PD in the unique SA populations are required to provide novel insights into mechanisms underlying this debilitating condition.
Source: South African Medical Journal 102, pp 851 –854 (2012)More Less
Objectives. To study the prevalence of alcohol and substance use in a South African antenatal population and its correlates with sociodemographic factors, depression and perceived stress.
Methods. A prospective self-report study on all women presenting for their first antenatal visit who consented to the study at a midwife obstetric unit (MOU) in the East Metropole district, Cape Town, using the Alcohol Use Disorders Identification Test (AUDIT), Drug Use Disorders Identification Test (DUDIT), Edinburgh Depression Scale (EDS) and Perceived Stress Scale (PSS). Statistical analyses using the chi-square test, separate one-way analyses of variance (ANOVA) and logistic regression analyses were performed as appropriate. Outcome measures were depression, alcohol use and substance use.
Results. The questionnaire was completed by 323 women. During pregnancy 36.8% of women smoked, 20.2% used alcohol and 4% used substances. Using EDS cut-off scores of 12 and 15, respectively, 48.9% and 33.6% of the sample had scores consistent with major depression. An EDS cut-off score of 12 was significantly associated with both alcohol use (25.9% v. 15.2%, p=0.019) and risky drinking (76.9% v. 36.8%, p=0.04), while an EDS cut-off score of 15 was significantly associated with substance use (8.2% v. 1.4%, p=0.004) as well as alcohol dependence (23.1% v. 3.1%).
Conclusions. We found high rates of both alcohol abuse and antenatal depression, and a significant association between depression, substance use and alcohol abuse; EDS scores greater than 12 could be used to identify women at risk of alcohol dependence and/or substance abuse.
Safety of nevirapine in HIV-infected pregnant women initiating antiretroviral therapy at higher CD4 counts : a systematic review and meta-analysis : researchSource: South African Medical Journal 102, pp 855 –859 (2012)More Less
Background. The package insert for nevirapine (NVP) cautions against use in HIV-infected women (including pregnant women) with CD4 counts ≥250 cells/µl. However, recent studies showed that the CD4 count of pregnant women receiving antiretroviral therapy (ART) was not predictive of NVP toxicity.
Objectives. To determine whether ART-naive pregnant women initiating NVP-based ART at higher CD4 counts experience greater toxicity compared with pregnant women at lower CD4 counts.
Methods. We reviewed studies comparing serious adverse NVP-related events among ART-naive pregnant women who commenced therapy at higher v. lower CD4 counts. Relevant studies were extracted from PubMed, SCOPUS and EMBASE, major journals and conference proceedings prior to December 2011. Authors were contacted for additional data. Data were independently extracted and entered into Review Manager.
Results. Fourteen studies (2 663 participants) were included for analysis. The odds ratio (OR) for overall NVP toxicity among pregnant women with CD4 <250 cells/µl was 0.61 (95% confidence interval (CI) 0.43 - 0.85). When analysis was restricted to prospective studies only (7 studies, 1 318 participants), the results were consistent for overall NVP toxicity (OR 0.43; 95% CI 0.25 - 0.73) and severe hepatotoxicity (OR 0.45; 95% CI 0.22 - 0.90), but not for severe cutaneous reaction (OR 0.53; 95% CI 0.26 - 1.10).
Conclusion. Initiating NVP-based ART during pregnancy at CD4 ≥250 cells/µl increases toxicity risk and should be avoided, necessitating urgent revision of current guidelines supporting this practice.
Source: South African Medical Journal 102 (2012)More Less
'Looking at the problem of biliary atresia from the vantage point of 30 years' experience with the lesion, we can say with certainty that the jaundiced baby who has had no extrahepatic bile duct has been the most disappointing patient for the surgeon in the whole realm of lesions theoretically correctable by a surgical procedure.'
Maximising Kasai portoenterostomy in the treatment of biliary atresia : medical and surgical options : paediatric hepatobiliary - forum : reviewSource: South African Medical Journal 102, pp 865 –867 (2012)More Less
Biliary atresia (BA) remains one of the most challenging conditions in paediatric surgery. It has several possible causes, resulting in a range of different clinical scenarios. The current therapeutic approach is almost entirely surgical with an initial attempt to restore bile flow and preserve the native liver using a Kasai-type portoenterostomy. Liver transplantation (cadaveric or living donor) is usually reserved for failure or for infants presenting late with end-stage cirrhosis. The role of adjuvant medical therapy is unclear and evidence of benefit is lacking. Nonetheless, the use of post-operative steroids, prophylactic antibiotics and choleretic agents such as ursodeoxycholic acid is common. Ideally, the entire pathway should be complementary and seamless with few infants dying of end-stage liver disease or uncorrectable associated congenital malformations. Experience from high-volume centres suggests that clearance of jaundice can be achieved in 50 - 60% of infants, with 10-year native liver and real survival rates of 45% and 90%, respectively.
Author N. HadzicSource: South African Medical Journal 102, pp 868 –871 (2012)More Less
The only effective treatment for 'failing' Kasai portoenterostomy is liver transplantation (LT). However, to maximise a patient's chances to achieve the proclaimed >95% survival with sequential surgical management, medical follow-up and treatment must be planned carefully. This includes routine fat-soluble vitamin supplementation with choleretics, aggressive nutritional support, regular ultrasonography, optimal general paediatric care, and psychological support for the family once complications arise. Careful timing of LT is of critical importance, although recent trends include earlier consideration of LT in children with biliary atresia. This management can only be offered through centralised, specialised national services. Due to its ramifications in paediatric surgery, dietetics, metabolic, social, adolescent and transplantation medicine, paediatric hepatology is a fine example of patient care that is genuinely multidisciplinary.
Source: South African Medical Journal 102, pp 872 –875 (2012)More Less
Most difficult hepatobiliary (HPB) problems in infancy and childhood result from pathological anatomical/mechanical derangements; therefore, surgery on the liver and bile ducts depends on a detailed understanding of liver structure, function and repair response to injury or disease. The surgeon must be aware of the very diverse range of anatomical variations. Perhaps key to improving the outcome of paediatric HPB surgery is centralised management and associating this with a paediatric liver transplant programme, which adds expertise and, frequently, the added benefit of adult HPB surgical input to paediatric surgical care. In the United Kingdom, this has resulted in excellent measurable benefit, particularly in the management of biliary atresia, but also of choledochal cysts, portal hypertension and liver tumours. These conditions are briefly discussed here, with focus on the technical aspects of operative management.
Source: South African Medical Journal 102, pp 876 –878 (2012)More Less
The evaluation of the liver transplantation candidate is intended to confirm the indication for transplantation, determine the severity of disease, exclude contra-indications, optimise pre-transplantation care and candidate condition, and educate the patient and family on post-procedure expectations. This article is intended as a guide for the appropriate selection and work-up of patients for liver transplantation.
Author J.F. BothaSource: South African Medical Journal 102, pp 879 –880 (2012)More Less
Paediatric liver transplantation is a highly effective therapy for children with end-stage liver disease; 1-year survival rates currently exceed 90% and long-term survivors enjoy an almost-normal quality of life. Key to the success of paediatric liver transplantation has been the technical refinement to provide children with suitably sized grafts. Adult-to-paediatric living donor liver transplantation highlights this success and has been instrumental in decreasing waiting list mortality to less than 5%.
A review of paediatric liver resections in Johannesburg : experiences and preferred technique : paediatric hepatobiliary - researchSource: South African Medical Journal 102, pp 881 –883 (2012)More Less
Background. Liver resections are widely performed in paediatric surgery. Many techniques exist to achieve vascular control, minimise bleeding and complete the parenchymal division.
Methods. We retrospectively reviewed all liver resections performed in the Department of Paediatric Surgery at our institution between January 2005 and June 2012. Data pertaining to basic demographics, indications for surgery, parenchymal transection techniques, morbidity, mortality and histology were collated.
Results. Twenty-one resections were performed in children aged 6 weeks - 11 years; 18 for malignant liver disease (including 9 hepatoblastomas), and 3 for benign disease. We describe 1 perioperative mortality secondary to torsion of the liver remnant, and no surgical morbidity. Three cases underwent total hepatic vascular exclusion with sharp parenchymal transection. The remaining patients underwent selective vascular inflow and outflow control using the Cavitron Ultra Sonic Aspirator and Harmonic Scalpel to divide the parenchyma.
Conclusion. Care for these patients should be multidisciplinary. High-volume units and access to liver transplantation offer optimal results. No technique is proven superior to the 'clamp crush' technique of parenchymal transection. Knowledge of hepatic anatomy is key to minimising morbidity, and surgeons should be familiar with and have the flexibility to use all techniques of vascular control.
Endoscopic injection sclerotherapy for bleeding varices in children with intrahepatic and extrahepatic portal venous obstruction : benefit of injection tract embolisation : paediatric hepatobiliary - researchSource: South African Medical Journal 102, pp 884 –887 (2012)More Less
Background. The outcome of sclerotherapy for bleeding oesophageal varices may be influenced by injection technique. In a previous study at our institution, sclerotherapy was associated with a high re-bleeding rate and oesophageal ulceration. Embolisation of the injection tract was introduced in an attempt to reduce injection-related complications.
Methods. To determine the outcome and effectiveness of injection tract embolisation in reducing injection-related complications, we retrospectively reviewed a series of 59 children who underwent injection sclerotherapy for oesophageal varices (29 for extrahepatic portal vein obstruction (EHPVO) and 30 for intrahepatic disease) in our centre.
Results. Sclerotherapy resulted in variceal eradication in only 11.8% of the children (mean follow-up duration: 38.4 months). Variceal eradication with sclerotherapy alone was achieved in 20.7% and 3.3% of EHPVO and intrahepatic disease patients, respectively. Injection tract embolisation was successful in reducing the number of complications and re-bleeding rates. Complications that arose included: transient pyrexia (16.7%); deep oesophageal ulcers (6.7%); stricture formation (3.3%); and re-bleeding before variceal sclerosis (23%).
Conclusion. Injection sclerotherapy did not eradicate oesophageal varices in most children. Injection tract embolisation by sclerosant was associated with fewer complications and reduced re-bleeding rates.
Source: South African Medical Journal 102, pp 888 –889 (2012)More Less
Background. Approximately one-third of hepatoblastoma (HB) patients have associated congenital abnormalities, but familial recurrence is rare, except in association with familial adenomatous polyposis (FAP). This correlation may be missed if not actively sought, with implications for long-term outcome and management.
Methods. We retrospectively investigated 3 families with an HB-familial polyposis connection, from a cohort of 113 FAP families (1989 - 2010). Data were analysed to assess clinical problem, treatment, complications and management. Long-term morbidity and functional outcome were analysed to identify management difficulties.
Results. Three FAP families (2.65%) had an HB association. In one case, undiagnosed FAP at the time of HB diagnosis was only detected 5 years later, when the mother presented with advanced colorectal carcinoma. A chromosome 5 APC gene mutation (exon 15 codon 793 C→T) was then identified. In a second case, a non-related boy presented with a stage 4 multifocal HB with lung metastases. Genetic studies identified an APC gene mutation (exon 6 codon 232 C→T). Further family investigation showed >20 related FAP patients. A third HB-FAP association was identified in a known FAP family early in the study, prior to the availability of genetic testing.
Conclusion. Although a rare association, a family history of FAP in HB patients is an important 'hidden connection'. Germline variation may be outside the usual FAP gene site. Identifying families with unknown HB/FAP is important due to long-term management implications and follow-up.
Problems related to cytomegalovirus infection and biliary atresia : paediatric hepatobiliary - researchSource: South African Medical Journal 102, pp 890 –892 (2012)More Less
Background. Human cytomegalovirus (CMV) infection is related to biliary disease, being cholestatic in its own right. It has also been associated with intrahepatic bile duct destruction and duct paucity, indicating a possible role in the pathogenesis and progression of extrahepatic biliary atresia (BA). BA patients who are CMV-IgM-positive appear to have greater liver damage than uninfected patients, consequently affecting outcome.
Methods. We reviewed the medical records of 74 patients diagnosed with hepatobiliary disease between 2000 and 2011, assessing clinical outcome and potential risk factors. Patients, categorised into those with and those without BA, were compared in terms of CMV infection.
Results. The 74 patients included 39 (52%) BA and 35 non-BA patients; following the exclusion of patients due to insufficient data, 27 (69%) BA and 31 non-BA patients were reviewed. Twenty-one (78%) BA patients were CMV-positive (IgM/IgG), including 20 IgM-positive patients versus 8 in the non-BA group (p<0.01). Two (7.5%) CMV-IgM-positive BA infants were HIV-exposed versus 7 (35%) in the non-BA group (p<0.01). Long-term outcomes included 3 deaths in the non-HIV CMV-positive group and a higher rate of severe early liver damage, suggesting a poorer outcome in CMV-affected patients.
Conclusions. Our results suggest a correlation between CMV exposure/infection and BA which affects clinical outcome. HIV-positivity does not preclude BA and should be investigated further.