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.

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.

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.

Genotyping and subtyping are important to understand epidemiology of the hepatitis E virus so as to improve control measures to prevent transmission of virus in the community. Hence, the aim of the current study was to identify the prevalent HEV genotypes in Eastern India in acute sporadic hepatitis E cases with varying degree of liver failure. The study was carried out in Calcutta Medical College, Kolkata, a tertiary care centre in West Bengal from Aug, 2012 to Nov, 2014. The study was approved by the institutional ethics committee. On the basis of disease severity the study comprised two HEV-induced groups: acute viral hepatitis (AVH) and acute liver failure (ALF), respectively. Samples were tested for hepatitis B virus (HBV) surface antigen, anti-hepatitis C virus antibodies, anti-hepatitis A virus IgM, anti-HEV antibodies (IgM and IgG) by ELISA. Of the 285 patients with acute hepatitis, 117 (41.05%) were positive for HEV either alone (n = 98) or in combination with another hepatotropic virus by serology. Among these 117 patients, 70 had AVH and 47 developed acute liver failure during the course of infection. There was no mortality among the 70 AVH patients, while seven patients (14.9%) with acute liver failure died. Serum samples from 42 acute sporadic hepatitis cases (within 7 days of illness and negative for all serological markers) were investigated for the presence of HEV RNA by nested polymerase chain reaction (RTnPCR) using primers designed with in RdRp (RNA dependent RNA polymerase) region of open reading frame-1 (ORF-1). Seventeen patients (10 AVH, 7 ALF) found to be positive for HEV RNA were sequenced by direct sequencing on ABI prism 310. The sequences (343 nucleotides) were compared with each other and were aligned with previously reported HEV sequences obtained from GeneBank, using Clustal W software. A Phylogenetic tree was constructed by using PHYLIP version 3.67 software. The sequences of these 17 HEV isolates shared 91.3–98.5% sequence homology among themselves. All AVH and only two ALF isolates belonged to genotype 1a and the remaining 5 ALF isolates were clustered with genotype 1c. HEV isolates which belonged to genotype 1a had 91.5–94.2% homology with subtype 1a and HEV isolates which belonged to genotype 1c had 90.1–93.5% homology with subtype 1c. There was a high degree of variability between genotypes 3 and 4, with a divergence of 31.2% and 30.1%, respectively. Patients with genotype 1c had significantly higher alanine aminotransferase levels (median 2930 IU/L, 1837–3763 versus 1324 IU/L, 945–2317; p = 0.01) than genotype 1a and prothrombin time was lower in the genotype 1c patients (median 61% versus 84%; p = 0.05). This is the first study from Eastern India reported genotype 1 in humans with subgroup ‘a’ and ‘c.’ Interestingly, we observed that the patients with genotype 1c tend to have more clinical manifestation than those with genotype 1a infection.

The primary therapy for food allergy is strict avoidance of the offending food or foods. Dietary restriction should be tailored to meet the nutritional requirements of each patient.Patients should be educated on how to avoid allergens safely by understanding terminology for common ingredients and how to read food labels. Information regarding safe, cost-effective and freely available substitutes for the avoided foods should be provided. Patients should be re-evaluated at regular intervals to see if they have developed tolerance. Mothers of infants with cow's milk protein allergy (CMPA) who are breastfeeding should be supported and encouraged to continue breastfeeding. Partially hydrolysed infant formulas are not hypoallergenic (tolerated by 90% of subjects with proven CMPA) and are therefore not recommended for the treatment of CMPA, but may have a role in prevention of eczema or CMPA in high-risk individuals. Some extensively hydrolysed and amino-acid-based formulas are truly hypoallergenic. The recommended feed of choice for the dietary management of mild-to-moderate CMPA in infants not breastfed is an extensively hydrolysed cow's milk formula. The recommended formula for the dietary management of non-breastfed infants and children with known severe CMPA is an amino-acid-based formula. Soya-based formulas may be useful in infants with immunoglobulin E (IgE)-mediated CMPA with proven tolerance to soya, and some cases of mild-to-moderate non-IgE-mediated CMPA, bearing in mind the increased risk of co-reactivity between CMPA and soya allergy in non-IgE-mediated conditions. Other mammalian milks and plant-based milks, including rice and oat milks, are not suitable as sole nutrition for cow's milk protein allergic individuals.

One cannot depend on one single test to diagnose food allergy. A detailed history is an essential initial step in cases of suspected food allergy. Aspects of the history should be gathered separately for each food being considered, as a patient may experience different types of reactions with various foods, each of which requires individual diagnostic and management strategies. History alone is not diagnostic and additional measures of sensitisation or food challenges are often required. In suspected immunoglobulin E (IgE)-mediated allergy, skin-prick tests (SPTs) and/or measurement of serum specific IgE antibodies (ImmunoCAP) to suspected foods is used to prove sensitisation. Sensitisation does not, however, confirm clinical food allergy as these tests indicate an immunological response to the specific allergen, but the diagnosis requires a clear correlation between the test result and clinical reaction (by positive history or food challenge). The magnitude of the test result (SPT mean wheal size or ImmunoCAP level in kU/L) correlates with the likelihood of clinical allergy, but not the severity of a reaction. Choice of the allergens tested should be guided by the history, but limited to the lowest necessary number to avoid false-positive results. Tests for sensitisation to foods should not be performed when the history indicates that such foods are tolerated. Ninety-five per cent positive predictive values (where a clinical reaction can be predicted in 95% of cases) have been described for immediate reactions, but may be population specific. There are no validated tests to confirm non-IgE- or mixed IgE- and non-IgE-mediated food allergies. Diagnosis of this group of allergies depends on elimination of the suspected food, clearance of symptoms, and recurrence of symptoms on re-introduction of the food.

Despite the large number of foods that may cause immunoglobulin E (IgE)-mediated reactions, most prevalence studies have focused on the most common allergenic foods, i.e. cow's milk, hen's egg, peanut, tree nut, wheat, soya, fish and shellfish. Food allergy peaks during the first two years of life, and then diminishes towards late childhood as tolerance to several foods develops. Based on meta-analyses and large population-based studies, the true prevalence of food allergy varies from 1% to >10%, depending on the geographical area and age of the patient. The prevalence of food allergy in South Africa (SA) is currently being studied. The prevalence of IgE-mediated food allergy in SA children with moderate-to-severe atopic dermatitis is 40%; however, this represents a high-risk population for food allergy. Preliminary data from the South African Food Sensitisation and Food Allergy (SAFFA) study, which is investigating food allergy in an unselected cohort of 1 - 3-year olds, show a prevalence of 11.6% sensitisation to common foods. Food allergy was most common to egg (1.4%) and peanut (1.1%). Food allergy appears to be the most common trigger of anaphylactic reactions in the community, especially in children, in whom food is responsible for ≥85% of such reactions. In adults, shellfish and nut, and in children, peanut, tree nut, milk and egg, are the most common triggers of food-induced anaphylaxis.

Instituting an exclusion diet for 2 - 6 weeks, and following it up with a planned and intentional re-introduction of the diet, is important for the diagnosis of a food allergy when a cause-and-effect relationship between ingestion of food and symptoms is unclear. Food may be re-introduced after (short-term) exclusion diets for mild-to-moderate non-immunoglobulin E (IgE)-mediated conditions in a safe clinical environment or cautiously at home. However, patients who have had an IgE-mediated immediate reaction to food, a previous severe non-IgE-mediated reaction or a long period of food exclusion should not have a home challenge, but rather a formal incremental food challenge protocol in a controlled setting. An incremental oral food challenge (OFC) test is the gold standard to diagnose clinical food allergy or demonstrate tolerance. It consists of gradual feeding of the suspected food under close observation. It should be done by trained practitioners in centres that have experience in performing the procedure in an appropriate setting. An OFC must be performed in a setting where resuscitation equipment is available in the event of a severe anaphylactic reaction. OFCs are terminated when a reaction becomes apparent. Standardised and pre-set criteria are available on when to discontinue challenges. Patients who tolerate the full dose ‘pass’ the challenge and are advised to eat a full portion of the food at least twice a week to maintain tolerance. Those who have reactions have ‘failed’ the challenge, should avoid the food, receive education and implement risk-reduction strategies where appropriate. Patients should be observed for a minimum of 2 hours following a negative challenge and for 4 hours after a positive one.

Important potential food allergens in vaccines include egg and gelatin. Rare cases of reactions to yeast, lactose and casein have been reported. It is strongly recommended that when vaccines are being administered resuscitation equipment must be available to manage potential anaphylactic reactions, and that all patients receiving a vaccine are observed for a sufficient period. Children who are allergic to egg may safely receive the measles-mumps-rubella (MMR) vaccine; it may also be given routinely in primary healthcare settings. People with egg allergy may receive influenza vaccination routinely; however, some authorities still perform prior skin- prick testing and give two-stage dosing. The purified chick embryo cell culture rabies vaccine contains egg protein, and therefore the human diploid cell and purified verocell rabies vaccines are preferred in cases of egg allergy. Yellow fever vaccine has the greatest likelihood of containing amounts of egg protein sufficient to cause an allergic reaction in allergic individuals. This vaccine should not be routinely administered in egg allergic patients and referral to an allergy specialist is recommended, as vaccination might be possible after careful evaluation, skin-testing and graded challenge or desensitisation.

South African Food Allergy Working Group (SAFAWG) authors of the South African food allergy consensus document 2014

The process of oral immunotherapy (OIT) consists of a series of dose escalations with the immediate goal of inducing desensitisation and ultimately achieving a state of tolerance. Owing to the limitations of OIT, including side-effects and lack of proven efficacy in long-term tolerance induction, it is not yet recommended in routine clinical practice and should be restricted to the research setting. Studies using anti-immunoglobulin E (IgE) antibody in food allergy management are limited, but show promising results. The possible applications are for increasing the sensitivity threshold to certain foods such as peanut, and also for use in combination with OIT to enhance safety and rapidity of the OIT process; however, anti-IgE is not yet licensed for use in food allergy.

An anaphylactic reaction may be fatal if not recognised and managed appropriately with rapid treatment. Key steps in the management of anaphylaxis include eliminating additional exposure to the allergen, basic life-support measures and prompt intramuscular administration of adrenaline 0.01 mg/kg (maximum 0.5 mL). Adjunctive measures include nebulised bronchodilators for lower-airway obstruction, nebulised adrenaline for stridor, antihistamines and corticosteroids. Patients with an anaphylactic reaction should be admitted to a medical facility so that possible biphasic reactions may be observed and risk-reduction strategies initiated or reviewed after recovery from the acute episode. Factors associated with increased risk of severe reactions include co-existing asthma (and poor asthma control), previous severe reactions, delayed administration of adrenaline, adolescents and young adults, reaction to trace amounts of foods, use of non-selective β-blockers and patients who live far from medical care. Risk-reduction measures include providing education with regard to food allergy and a written emergency treatment plan on allergen avoidance, early symptom recognition and appropriate emergency treatment. Risk assessment allows stratification with provision of injectable adrenaline (preferably via an auto-injector) if necessary. Patients with ambulatory adrenaline should be provided with written instructions regarding the indications for and method of administration of this drug and trained in its administration. Patients and their caregivers should be instructed about how to avoid foods to which the former are allergic and provided with alternatives. Permission must be given to inform all relevant caregivers of the diagnosis of food allergy. The patient must always wear a MedicAlert necklace or bracelet and be encouraged to join an appropriate patient support organisation.

Introduction: Combined liver and kidney transplantation is a life saving procedure for patients with combined liver and kidney failure or metabolic liver diseases leading to renal failure. Methods: To document the management and outcomes of combined liver- kidney transplantation at our Transplant Centre. Methods: Retrospective clinical folder audit. Results: 7 children and 3 adults underwent combined liver and kidney transplantation. The indications included Type 1 primary hyperoxaluria (6), polycystic kidney disease with congenital hepatic fibrosis (1), toxin-induced acute liver and renal failure (1), re-do liver transplantation due to chronic ductopaenic rejection and associated renal failure (2). 9 patients were on dialysis prior to transplantation. 2 patients died within 1 week of transplantation: 1 adult who received blood group incompatible liver and kidney grafts for toxin-induced acute liver and renal failure and 1 child with Type1 primary hyperoxaluria as a result of hepatic artery thrombosis and primary non-function of the renal graft. The surviving 8 patients have normal liver graft function and stable renal graft function (mean GFR 55ml/min/1.73m2, range 46-102) with a mean follow-up of 10.4 years (range 2-17.6 years). Conclusion: The longterm outcomes of combined liver-kidney transplantation at our Transplant centre have been excellent with a 80% 5 year patient and graft survival.

Spearman CWN, Goddard E, McCulloch MI, Hairwadzi HN, Sonderup MW, Kahn D, Millar AJW. Pregnancy following liver transplantation during childhood and adolescence. Pediatr Transplantation 2011: 15: 712–717. © 2011 John Wiley & Sons A/S.Abstract: 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.

Goddard, E.1; Spearman, W.2; NUMANOGLU, A.3; McCulloch, M.1; Millar, A.3; Kahn, D.4 Author Information

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.

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 m(2) (mean 96.1, median 89.8). Seven cases had a GFR less than 75 mL/min/1.73 m(2). 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.

UNLABELLED:Liver transplantation for infants and children has been available in South Africa at a single centre, the only established service in Sub-Saharan 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. MATERIALS AND METHODS:Since 1985, 225 infants and children have been assessed, with 146 accepted for transplantation. Sixty-nine have had 71 orthotopic liver transplants (OLTx). Biliary atresia was the most frequent diagnosis (54%) followed by acute liver failure (ALF) (15%). Waiting list mortality has remained high (23%), particularly for the ALF group (50%). Forty-three were reduced size transplants with donor: recipient weight ratios ranging from 2:1 to 11:1. Twenty-seven were <10 kg. RESULTS:Fifty (74%) survive 1 month-12 years post-transplant. Actuarial survival after 1996 since HBV core antibody positive donor livers were refused and prophylactic IV ganciclovir used has been >82%. Early post-OLTx mortality was low (5%), one primary non-function, one IVC thrombosis, one PV thrombosis, but late morbidity and mortality (20%) was mainly due to viral infection: de novo hepatitis B (five patients, three deaths), EBV-related post-transplantation lymphoproliferative disease (PTLPD) (eight patients, six deaths) and CMV disease (11 patients, five deaths). Tuberculosis prophylaxis, required in six cases, resulted in major morbidity in two and mortality in one. Poor compliance played a significant role in seven deaths. Hypertension requiring medication along with some compromise of renal function has been present in all but two patients. However, all those of school-going age (25) attend school normally and remain in good health and only three of the survivors have abnormal liver function tests. CONCLUSIONS: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 re-transplantation along with long-term consequences of immunosuppression (infection, lymphoma and renal toxicity) remain problems. Intense education of the caregiver and close follow-up, particularly of those living at long distances has partly addressed the compliance problem.

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.