Identification of a single MPV17 nonsense-associated altered splice variant in 24 South African infants with mitochondrial neurohepatopathy.

MPV17-related mitochondrial neurohepatopathy is a rare genetic disorder worldwide. We report on a novel pathogenic variant in the MPV17 gene in 24 unrelated neurohepatopathic infants of non-consanguineous Black South African heritage. Exome sequencing identified homozygosity for a c.106C>T nonsense variant in exon 3 of the human MPV17 gene in 2 unrelated index patients. mRNA analysis revealed transcripts both with and without exon 3, indicating both reduced splice efficiency and premature termination as mechanisms for disease. Carrier frequency in this population was found to be 1 in 68 (95% CI; 1/122-1/38) with an estimated newborn incidence of 1 in 18 496 (95% CI; 1/59 536-1/5776). Affected infants all presented with infantile onset neurohepatopathy with none surviving beyond infancy. This description of a relatively common pathogenic variant underlying a previously uncharacterized severe neurohepatopathy in South Africa will engender increased awareness, earlier diagnosis and possibly improve outcome if preventative or specific therapeutic options can be found.

Maternal and infant factors had a significant impact on birthweight and longitudinal growth in a South African birth cohort.

AIM: This birth cohort study investigated longitudinal infant growth and associated factors in a multiethnic population living in a low-resource district surrounding the town of Paarl in South Africa. METHODS: Between March 2012 and October 2014, all mothers attending their second trimester antenatal visit at Paarl Hospital were approached for enrolment. Mother-infant pairs were followed from birth until 12 months of age. Comprehensive socio-demographic, nutritional and psychosocial data were collected at birth, two, six and 12 months. Infant anthropometry was analysed as z-scores for weight and height. Linear regression was used to investigate predictors of birthweight, and linear mixed-effects models were used to investigate predictors of infant growth. RESULTS: Longitudinal anthropometric data from 792 infants were included: 53% were Black African, 47% were mixed race, and 15% were born preterm. Stunting occurred in 13% of infants at 12 months. Maternal height, antenatal alcohol and tobacco use, ethnicity and socioeconomic status were significant predictors of birthweight. In the adjusted mixed-effects model, birthweight was a significant predictor of growth during the first year of life. CONCLUSION: Birthweight was an important predictor of growth trajectory during infancy. Birthweight and growth were influenced by several important modifiable factors.

Associations between single nucleotide polymorphisms in 33 candidate genes and meat quality traits in commercial pigs.

This study aimed to evaluate the effects of single nucleotide polymorphisms (SNPs) in candidate genes for meat quality using a custom 96-SNP panel (Illumina Vera Code GoldenGate Assay) on 15 traits collected from 400 commercial pigs. Meat quality measurements included muscle pH, color (L*, a* and b*), drip loss, cooking loss, peak shear force and six sensory traits including appearance (outside and inside), tenderness, juiciness, flavor and overall liking as well as carcass weight and probe yield. Thirty-five SNPs with minor allele frequencies > 0.10 remained for the multimarker association using the GLM procedure of sas 9.2. Results showed that 20 SNPs were significantly associated with at least one of the traits with either additive or dominance or both effects (P < 0.05). Among these significant SNPs, five of them in ADIPOQ, FTO, TNF, LEPR and AMPD1 had an effect on more than three traits simultaneously; those in MC4R, CAST, DGAT1 and MYF6 had an effect on two traits, while the others were associated with one trait. The results suggest that these markers could be incorporated into commercial pigs for marker-assisted selection and breeding programs for carcass and meat quality trait improvement.

Effects of expectation on face perception and its association with expertise.

Perceptual decisions are derived from the combination of priors and sensorial input. While priors are broadly understood to reflect experience/expertise developed over one's lifetime, the role of perceptual expertise at the individual level has seldom been directly explored. Here, we manipulate probabilistic information associated with a high and low expertise category (faces and cars respectively), while assessing individual level of expertise with each category. 67 participants learned the probabilistic association between a color cue and each target category (face/car) in a behavioural categorization task. Neural activity (EEG) was then recorded in a similar paradigm in the same participants featuring the previously learned contingencies without the explicit task. Behaviourally, perception of the higher expertise category (faces) was modulated by expectation. Specifically, we observed facilitatory and interference effects when targets were correctly or incorrectly expected, which were also associated with independently measured individual levels of face expertise. Multivariate pattern analysis of the EEG signal revealed clear effects of expectation from 100 ms post stimulus, with significant decoding of the neural response to expected vs. not stimuli, when viewing identical images. Latency of peak decoding when participants saw faces was directly associated with individual level facilitation effects in the behavioural task. The current results not only provide time sensitive evidence of expectation effects on early perception but highlight the role of higher-level expertise on forming priors.

Empirical examination of the interpersonal maintenance model of anorexia nervosa.

OBJECTIVE: A cognitive interpersonal maintenance model of anorexia nervosa (AN) was first proposed in 2006 and updated in 2013 (Schmidt and Treasure, J Br J Clin Psychol, 45, 343-366, 2006; Treasure and Schmidt, J Eat Disorders, in press.). The aim of this study was to test the interpersonal component of this model in people with AN requiring intensive hospital treatment (inpatient/day patient). METHOD: On admission to hospital women with AN or eating disorder not otherwise specified (AN subtype; n = 152; P) and their primary carers (n = 152; C) completed questionnaires on eating symptoms (P), depression and anxiety (P, C), accommodation and enabling (C), and psychological control (C). Structural equation modeling was used to examine relationships among these components. RESULTS: Carers' expressed emotion and level of psychological control were significantly related to carers' distress, which in turn, was related to patients' distress. This pathway significantly predicted eating symptoms in patients. DISCUSSION: The cognitive interpersonal maintenance model of eating disorders (EDs) was confirmed in part and suggests that interventions targeting interpersonal maintaining factors such as carer distress might impact on patient outcomes.

Business analysis of IRT, Visual observation, and Ovsynch as breeding strategies in Alberta dairies.

The dairy industry is searching for new technologies to address low (<50%) estrus detection. However, the lack of information on the potential economic benefits regarding new technology implementation has led some dairy producers to continue using conventional estrus detection methods (e.g. visual observation of standing to be mounted). The objective of this study was to compare the costs of infrared thermography (IRT), visual observation (VO) and ovulation synchronization (Ovsynch: OVS) as breeding strategies at different accuracy levels (Sensitivity [Se], Specificity [Sp]) and pregnancy rates (PR). The costs associated with Breeding, Feeding, Operation Costs, Return to Equity and Culling Risk per estrus detection rate (ER; 30-100%, conception rate for OVS; 30-100%), PR [PR per Parity group; 1-2 (50%), 3-4 (43%), and >4 (41%)], and ER accuracy determined the potential financial benefit of each breeding method for a representative farm. Breeding Cost results (Canadian dollars per cow; CAD/cow) showed a higher cost of OVS (138.99) as compared to VO (115.78) and IRT (127.69). Pregnancy Costs were affected by Breeding Cost; however, ER had a significant effect on PR expense for each method, IRT (ER; 30%: 210.38; 100%: 132.19), VO (ER; 30%: 205.93; 100%: 129.39), and OVS (ER; 30%: 247.21; 100%: 155.33). The minimum Se level with a positive Financial Effect for IRT and VO was 60% with a Sp of 100%, and for the OVS was Se 65% and Sp 100%. However, when the Se was 100% a positive Financial Effect was observed with a minimum Sp of 85% for IRT and 75% for VO. Culling Risk was reduced if ER increases differently depending on the parity group. Implementing of IRT as an estrus detection method yields a competitive breeding cost compared to VO and OVS. Further, breeding methods must accomplish at least ∼60% accuracy to have a positive net return.

Pregnancy following liver transplantation during childhood and adolescence.

More than 80% of pediatric transplant recipients will survive to reach adulthood, and many will consider having children. We report on outcomes and management of five pregnancies in four women undergoing orthotopic liver transplantation during childhood or adolescence and followed up at our Transplant Center. A retrospective clinical folder audit was performed. Mean age at transplantation was 13.3 ± 3.4 yr (range, 10-18 yr). Mean interval between transplantation and pregnancy was 15.4 ± 4.9 yr (range, 10-22 yr). Mean maternal age at conception was 28 ± 3.5 yr (range, 23-32 yr). Mean gestational age was 36.6 ± 1.7 wk. Mean birth weight was 2672 ± 249 g. Immunosuppression was cyclosporin based in three women and tacrolimus based in one woman. Pregnancy complications necessitating the induction of labor included fetal distress and rising maternal liver enzymes in two women, cholestasis of pregnancy and impaired renal graft function in one woman, fetal distress and preeclampsia in one woman. Modes of delivery were normal vaginal delivery in three women and cesarean section in one woman. No maternal or fetal deaths and no congenital malformations occurred. No episodes of rejection occurred during pregnancy. Two women experienced acute cellular rejection requiring an increase in baseline immunosuppression in the first year, following delivery. No graft losses occurred during a mean follow-up of 44 ± 17.9 months post-delivery. With careful management, pregnancy post-liver transplantation can have a successful outcome.

A retrospective description of primary immuno-deficiency diseases at Red Cross War Memorial Children's Hospital, Cape Town, South Africa, 1975 - 2017.

BACKGROUND: The primary immunodeficiency diseases (PIDs) constitute a diverse and ever-expanding group of inborn errors affecting a wide range of immune functions. They are not well documented in sub-Saharan Africa. OBJECTIVES: To describe the spectrum of PIDs at a tertiary paediatric hospital. METHODS: A retrospective descriptive study of PIDs diagnosed at Red Cross War Memorial Children's Hospital, Cape Town, South Africa (SA), between 1975 and 2017 was undertaken. RESULTS: We identified 252 children with PIDs, spanning eight of the nine categories listed in the 2017 classification of the International Union of Immunological Societies. Predominantly antibody deficiencies, combined immunodeficiencies with associated syndromic features, and immunodeficiencies affecting cellular and humoral immunity accounted for most children with PIDs (n=199, 79.0%). The mean age (standard deviation) at diagnosis was 46 (50) months, and the male/female ratio was 1.5:1. There was a history of parental consanguinity in 3 cases (1.2%). Recurrent infection was the most prevalent presenting phenotype, manifesting in 177 patients (70.2%). Genetic or chromosomal confirmation was obtained in 42/252 cases (16.7%). Common interventions used to prevent infection were antimicrobial prophylaxis and immunoglobulin replacement therapy, administered to 95 (37.7%) and 93 (36.9%) of the patients, respectively. Six of 7 children who underwent haematopoietic stem cell transplantation (HSCT) had successful outcomes. The 7th patient died 2 months after HSCT from overwhelming infection. Although we could not account for the children lost to follow-up during the study period, 53 deaths were confirmed (21.0%). CONCLUSIONS: Several challenges exist in the recognition and treatment of children with PIDs in our setting. These include limited access to genetic diagnostics and HSCT. Suboptimal treatment options contribute to the overall mortality of PIDs in SA.

Endoscopy findings in HIV-infected children from sub-Saharan Africa.

BACKGROUND: The causes of persistent gastro-intestinal symptoms in HIV-infected children from sub-Saharan Africa remain poorly documented. METHODS: The clinical, radiological and endoscopic findings of all HIV-infected children who underwent upper GI endoscopy at Red Cross Children's Hospital, Cape Town, South Africa, from February 2003 to October 2005 were documented. RESULTS: Twenty-six HIV-infected children underwent endoscopy; median age 1 year (range: 0.17-10.9 years). The majority had advanced HIV disease; 18 (69%) were WHO Stage 4; median CD4 10.7% (range: 1-39.8%). Presenting symptoms included persistent vomiting (18), dysphagia (4) and GIT bleed (6). Observational and histological findings showed poor correlation. Pathogens were identified in 10 children: cytomegalovirus infection in seven (two with cryptosporidium co-infection), Candida in two, Helicobacter pylori in one. Age and CD4 count were not associated with the pathogens. Endoscopy findings influenced clinical management in 21 (81%) cases. CONCLUSION: Upper-GI endoscopy identified a diverse spectrum of disease and provided information that would be clinically relevant to most HIV-infected children with upper gastro-intestinal symptoms.

Relationship between posttraumatic stress symptoms, caregiving response, and parent mental health in youth exposed to single incident trauma.

BACKGROUND: Family factors may alter the risk of developing posttraumatic stress disorder (PTSD) or depression in young people after a traumatic event, but it is not clear which modifiable family variables can be addressed in psychological therapies. This study examined the relationships between family factors (Expressed Emotion [EE] and family accommodation) and psychopathology (posttraumatic stress symptoms; PTSS) in young people following a single incident trauma. Potential mediators of these relationships were also investigated. METHOD: Sixty-six parent-child dyads (aged 8-17 years) were assessed within one month of attending an Emergency Department. Self-reported PTSS and perceived EE were assessed in young people. Parents' own PTSS, mood symptoms, EE, and accommodating behaviours were also assessed. RESULTS: Cross-sectional analyses revealed that young person-perceived EE, parent-reported emotional over involvement (EOI) and accommodation behaviours significantly predicted higher PTSS in young people. The stress experienced as a consequence of EE mediated the relationship between young person-perceived EOI and PTSS in young people. Parental PTSS and anxiety were positively correlated with EOI and accommodation. Parental PTSS was not significantly associated with symptoms in young people. CONCLUSIONS: The results support the hypothesis that EE and accommodation are positively associated with PTSS in young people in the month following a potentially traumatic event. Understanding the child in the context of their family environment and relationships offers an important framework for making sense of and facilitating adaptive adjustment following a traumatic event.

Liver transplantation for children: Red Cross Children's Hospital experience.

BACKGROUND: The liver transplant program for infants and children at the Red Cross Children's Memorial Hospital is the only established pediatric service in sub-Saharan Africa. Since 1985, 250 infants and children have been assessed and 155 accepted for transplantation. METHODS: Since 1987, 76 children (range 6 months to 14 years) have had 79 liver transplants, with biliary atresia being the most frequent diagnosis. The indications for transplantation include biliary atresia (n = 44), metabolic (n = 7), fulminant hepatic failure (n = 10), redo transplants (n = 3), and other (n = 15). Three combined liver/kidney transplants have been performed. Forty-nine were reduced-size transplants with donor: recipient weight ratios ranging from 2:1 to 11:1, and 29 children weighed < 10 kg. RESULTS: Fifty-six (74%) patients survived 3 months to 12 years posttransplant. Cumulative 1- and 5-year patient survival data are 79% and 70%, respectively. However, with the introduction of prophylactic intravenous gancyclovir and the exclusion of hepatitis B virus (HBV) IgG core Ab-positive donors, the projected 5-year pediatric survival has been >80%. Early (<1 month) post-liver-transplant mortality was low, but included: primary malfunction (n = 1); inferior vena cava thrombosis (n = 1); bleeding esophageal ulcer (n = 1); and sepsis (n = 1). Late morbidity and mortality was mainly due to infections: de novo hepatitis B (5 patients, 2 deaths); Epstein-Barr virus (EBV)-related posttransplantation lymphoproliferative disease (12 patients, 7 deaths); and cytomegalovirus disease (10 patients, 5 deaths). Tuberculosis (TB) treatment in three patients was complicated by chronic rejection (n = 1) and TB drug-induced subfulminant liver failure (n = 1). CONCLUSIONS: Despite limited resources, a successful pediatric program has been established with good patient and graft survival figures and excellent quality of life. Shortage of donors due to HBV and human immunodeficiency virus (HIV) leads to significant waiting list mortality and infrequent transplantation.

Nephrotoxic effects of immunosuppressant therapy in pediatric liver transplant recipients.

Drugs used for immunosuppression have been implicated in causing numerous long-term side effects including nephrotoxicity, glucose intolerance, and hyperlipidemia. In this study, we reviewed our pediatric liver transplant recipients in terms of glomerular filtration rate (GFR) as well as fasting glucose and lipid profiles. To date, 79 pediatric liver transplantations have been performed at our center: 24 transplantations of at least 5 months to a maximum of 7.3 years posttransplant are reviewed herein. The mean time posttransplantation was 2.1 years. Nine boys and 15 girls showed a distribution of 19 mixed race, 3 black, and 2 white patients. The mean age at the time of transplantation was 6.6 years (0.8-13.3 years) with 8 cases under the age of 3 years. All recipients started with Cyclosporine Neoral (CSA) as first line, but, at the time of testing, immunosuppression included 5 children on CSA and 19 on Tacrolimus. Radionuclide 51 Cr-EDTA Glomerular Filtration Rates (GFR) showed a range from 21 to 220 mL/min/1.73 m2 (mean 96.1, median 89.8). Seven cases had a GFR less than 75 mL/min/1.73 m2. Twenty-one children were on antihypertensives agents: 15 children on 1 agent and 6 children on 2 agents. On full fasting lipid profiles, the total cholesterol ranged from 2 to 7.9 mmol/L (mean 4.4). Only 1 child is currently on statin therapy. Fasting glucose ranged from 3.2 to 5.9 mmol/L (mean 4.1) No difference was observed in glucose values between CsA and Tacrolimus. Thus, immunosuppressive therapies, such as the calcineurin inhibitors, are known to cause nephrotoxicity, which is of concern in pediatric liver transplant recipients. Almost all our patients currently require antihypertensive therapy. At present, the renal function is adequate in the majority of the group, but this study needs to be extended to other pediatric liver transplant recipients with particular emphasis on those who are more than 5 years posttransplantation.

South African food allergy consensus document 2014.

The prevalence of food allergy is increasing worldwide and is an important cause of anaphylaxis. There are no local South African food allergy guidelines. This document was devised by the Allergy Society of South Africa (ALLSA), the South African Gastroenterology Society (SAGES) and the Association for Dietetics in South Africa (ADSA). Subjects may have reactions to more than one food, and different types and severity of reactions to different foods may coexist in one individual. A detailed history directed at identifying the type and severity of possible reactions is essential for every food allergen under consideration. Skin-prick tests and specific immunoglobulin E (IgE) (ImmunoCAP) tests prove IgE sensitisation rather than clinical reactivity. The magnitude of sensitisation combined with the history may be sufficient to ascribe causality, but where this is not possible an incremental oral food challenge may be required to assess tolerance or clinical allergy. For milder non-IgE-mediated conditions a diagnostic elimination diet may be followed with food re-introduction at home to assess causality. The primary therapy for food allergy is strict avoidance of the offending food/s, taking into account nutritional status and provision of alternative sources of nutrients. Acute management of severe reactions requires prompt intramuscular administration of adrenaline 0.01 mg/kg and basic resuscitation. Adjunctive therapy includes antihistamines, bronchodilators and corticosteroids. Subjects with food allergy require risk assessment and those at increased risk for future severe reactions require the implementation of risk-reduction strategies, including education of the patient, families and all caregivers (including teachers), the provision of a written emergency action plan, a MedicAlert necklace or bracelet and injectable adrenaline (preferably via auto-injector) where necessary.

Mutations in the human mannose-binding protein gene: frequencies in several population groups.

Mannose-binding protein (MBP; mannan-binding protein, mannan-binding lectin) is a member of the collectin family of proteins and is thought to be important in innate immunity. We have previously shown high frequencies of two distinct mutations in codon 54 and codon 57 of exon 1 of the MBP gene in non-African and African populations, respectively. These result in low levels of the protein and an opsonic deficiency but the frequencies also suggest some selective advantage for low MBP levels. A third mutation in codon 52 occurs at a much lower frequency. We have now extended our earlier studies to other populations. In the south-west Pacific (Papua New Guinea and Vanuatu) neither the codon 52 nor the codon 57 mutation was detected and the codon 54 mutation was significantly less common (gene frequencies of 0.07 and 0.01, respectively) than in other non-African populations (gene frequencies 0.11-0.16). This could be explained by relatively recent admixture. The ancestral Melanesian population probably diverged some 50,000-60,000 years ago and our data suggest that the codon 54 mutation may have occurred after that even but before the divergence of European-Asian groups (40,000 years ago). Two further sub-Saharan populations were also studied: a group of Xhosa from South Africa were similar to Gambians, with a high gene frequency for the codon 57 mutation (0.27) and no evidence of the codon 52 or 54 mutations. In contrast, San Bushmen from Namibia had low frequencies of both the codon 57 mutation (0.07) and the codon 54 mutation (0.03). Again the codon 52 mutation was not found. This pattern is unique amongst sub-Saharan populations studied to date and suggests that this population may have been subjected to different selective pressures.

Immunoglobulin allotypes and genetic susceptibility to invasive Haemophilus influenzae type b and Staphylococcus aureus infections in South African children.

OBJECTIVE: The purpose of this study was to determine whether the G2m(n), G1m(f) and Km(3) immunoglobulin allotypes have any association with susceptibility to invasive Haemophilus influenzae type b (Hib) and Staphylococcus aureus (S. aureus) infections in children. METHODS: Direct enzyme-linked immunosorbent assays with commercially available monoclonal antibodies were established to quantitate G2m(n) and G1m(f) allotypes. A qualitative enzyme-linked immunosorbent assay with polyclonal rabbit anti-Km(3) antibody was established for Km(3) determination. RESULTS: The G2m(n) marker occurred in 34.4% of the mixed ancestry population and 2.9% of the Black population. There was a significantly decreased frequency of the G2m(n) allotype in mixed ancestry children with Hib meningitis (8.5%) and Hib osteomyelitis/septic arthritis and a decreased frequency of Km(3) in black and mixed ancestry children with Hib meningitis. The frequency of G2m(n), G1m(f) and Km(3) allotypes in patients with S. aureus osteomyelitis/septic arthritis were not significantly different from normal population frequency. CONCLUSIONS: This study shows a clear association between the absence of the G2m(n) allotype in mixed ancestry children and susceptibility to invasive infections caused by H. influenzae and an association between the absence of Km(3) and Hib meningitis in both black and mixed ancestry children.

IgG subclass values from normal children in Cape Town.

Sensitive and reproducible enzyme-linked immunoabsorbent assays (ELISA) have been developed to quantitate IgG subclass levels using monoclonal antibodies. Normal values for serum IgG subclass levels were determined in 300 healthy children between 6 months and 14 years of age and in 80 adults. High levels of IgG1 and delayed maturational development of IgG2 in children from Cape Town are different to results reported from developed countries. Genetic differences may account for this.

Do attitudes predict uptake of smoking in teenagers? Case not proven.

A recent paper by Charlton and Blair (Soc. Sci. Med. 29, 813-818, 1989) reported a large prospective study on smoking in 12 and 13 year-old schoolchildren. The main new findings were that attitudes, particularly among girls, were important predictors of the uptake of smoking in never-smokers. The present paper presents a critique of this study and argues that the findings may not reflect true predictive relationships. Data are presented from OPCS-conducted national surveys which indicate that a substantial minority of children who initially classify themselves as never-smokers admit to having tried a cigarette when prompted. These undisclosed triers differ from confirmed never-smokers on relevant predictor variables, and are significantly more likely to agree that they will try a cigarette again before leaving school (12% vs 4%, P less than 0.005). A similar group would be expected in Charlton and Blair's study and might wholly or partially account for the apparent predictive relationships found. This is first because such undisclosed triers might be more accurate in their self-reports when questioned a second time, but also because this group would contribute disproportionately to those trying smoking in the follow-up period. Other issues raised concern the homogeneity of the attitude variables employed, and the difficulty of attributing any specific importance to particular attitude items.

Are children with clefts underweight for age at the time of primary surgery?

Children with clefts, especially those with a cleft palate, have an impaired sucking mechanism and are therefore prone to nutritional problems. This study was undertaken to determine whether children with clefts of the lip and/or palate are underweight for age at the time of primary surgery. Underweight for age was defined as being less than 80 percent of expected weight for age or below the 3rd percentile as plotted on standard percentile charts. The records of all children with clefts seen at the Red Cross Children's Hospital between 1976 and 1996 were reviewed. Of these 740 records, 100 were excluded for inadequate data (47), severe systemic syndrome (27), no operation done (22), or craniofacial cleft (4). The records of 640 children were thus included; 195 (30.5 percent) were underweight for age. By comparison, only 13.7 percent of a similar group of noncleft controls (n = 872) were underweight for age. The difference between these two groups was highly significant (p < 0.01). Factors that influenced weight at the time of primary surgery were type of cleft and age at the time of surgery. Children with cleft palate, whether associated with a cleft lip or not, were found to be more underweight for age than those with an isolated unilateral cleft lip (p = 0.008). Children who had surgery after the age of 1 year were 1.5 times more likely to be underweight for age than children who had surgery under 1 year of age (p < 0.01). Children with isolated cleft palates who were underweight for age had a tendency toward a higher fistula rate (36 percent) than those of normal weight (24 percent) (p = 0.18).

Children's exposure to passive smoking in England since the 1980s: cotinine evidence from population surveys.

OBJECTIVE: To determine whether children's exposure to passive smoking has changed since the late 1980s. DESIGN: Cross sectional surveys of nationally representative samples of secondary school children carried out between 1988 and 1998 by Office for National Statistics. SETTING: England. SUBJECTS: Secondary school children aged 11-15. MAIN OUTCOME MEASURES: Salivary cotinine concentrations in non-smoking children. RESULTS: Cotinine concentrations in all non-smoking children almost halved between 1988 and 1998, from a geometric mean of 0.96 (95% confidence interval 0.83 to 1.11) ng/ml in 1988 to 0.52 (0.43 to 0.62) ng/ml in 1998. This reduction was largely due to reductions in exposure in children from non-smoking households and to decreases in the percentage of parents who smoked. Children living with mothers or fathers who smoked experienced little reduction in exposure. CONCLUSIONS: Exposure to passive smoking among children in England has approximately halved since the late 1980s. This reduction is partly explained by the fall in the percentage of both mothers and fathers who smoke and is also likely to reflect reductions of smoking in public places. However, there is only limited evidence that children from smoking households have experienced a reduction in exposure through parents' avoidance of smoking in their presence.

Liver transplantation in children--the Red Cross War Memorial Children's Hospital experience.

Liver transplantation for infants and children has been available in South Africa for more than a decade. Current concerns have shifted from an initial target of early post-transplant survival to quality of life in the long term. Since 1985, 175 infants and children have been assessed, with 104 accepted for transplantation. Fifty have had orthotopic liver transplants (OLTx), 48 (3 retransplants) in Cape Town and 2 abroad. Biliary atresia was the most frequent diagnosis (52%) followed by acute liver failure (ALF) (16%). Waiting list mortality has remained high (15%), particularly for the ALF group (50%). Thirty-four patients survive 1 month--14 years post-transplant. Early post OLTx mortality was low (7%) but late morbidity and mortality (23%) were mainly due to viral infection: de novo hepatitis B (5 patients, 2 deaths), EBV-related post-transplantation lymphoproliferative disease (PTLPD) (6 patients, 4 deaths) and CMV disease (9 patients, 4 deaths). Tuberculosis prophylaxis, required in 6 cases, resulted in major morbidity in 1. Hypertension requiring medication along with some compromise of renal function has been present in all but 2 patients. However, all those of school-going age (20) attend school normally and remain in good health and only 3 of the survivors have abnormal liver function tests. Successful liver transplantation is possible in a developing country with limited resources. Scarcity of virus-free donors (HBV and HIV) leading to waiting list mortality and infrequent retransplantation along with long-term consequences of immunosuppression (infection, lymphoma and renal toxicity) remain problems.