Crusted Scabies in a Pediatric Liver Transplant Recipient on Immunosuppression.

BACKGROUND Crusted scabies is a severe skin infection resulting from hyper-infestation with the obligate parasite Sarcoptes scabiei var. hominis. In contrast to classic scabies, crusted scabies may involve as many as hundreds to millions of mites. Importantly, this condition is associated with a mortality rate of 60% in 5 years, and is more likely to develop in immunosuppressed hosts, presumably due to an impaired T-cell response against the mite. CASE REPORT We present a case of crusted scabies in a 13-year-old girl during her early post-liver transplant period receiving immunosuppression successfully treated with topical 5% permethrin. She had pruritic erythematous papules, confirmed as scabies through skin scraping. The challenge of misdiagnosis during the initial presentation is noteworthy, given atypical manifestations and more common differentials. To our knowledge, only 2 cases of crusted scabies in pediatric solid-organ transplant recipients have been reported. Despite the absence of guidelines for pediatric solid-organ transplant recipients, urgent treatment is required due to the high associated mortality rates. In our case, early treatment proved successful without any secondary bacterial infections or clinical evidence of relapse during a 6-month follow-up. CONCLUSIONS We report a case of scabies in a pediatric liver transplant recipient that was successfully treated. It is crucial to consider the diagnosis of scabies given the associated morbidity and the risk of secondary bacterial infections among other more common differentials. Notably, the response to standard anti-scabies treatment may lead to resolution without relapse in pediatric solid-organ transplant recipients.

Managing constipation in children with ASD - A challenge worth tackling.

Autism Spectrum disorder (ASD) is well known to be associated with significantly high rates of gastrointestinal problems, constipation being common among them, imposing a significant burden on child and the family. On account of multiple underlying factors, both diagnosis and subsequent management of constipation in children with ASD are much more challenging as compared to managing constipation in 'neurotypical' children. Associated higher rate of presentation to the hospital emergency and subsequent hospital admission rates add to the burden. Hence, there is a need for recognizing constipation as a problem in children with ASD. This review summarizes optimization of its management by adopting a multidisciplinary holistic approach to achieve good outcomes and enhance the quality of life for the child and the family.

Demographics, aetiology and outcome of paediatric acute liver failure in Singapore.

Introduction: The aetiology of paediatric acute liver failure (PALF) varies widely according to age, and geographic and socioeconomic factors. This study aimed to examine the epidemiology, aetiology and outcome of PALF in Singapore at a single centre. Methods: A retrospective review was performed of patients aged 0-18 years who were diagnosed with PALF from 2007 to 2019. PALF was defined by: absence of chronic liver disease; biochemical evidence of acute liver injury; and coagulopathy, non-correctible by vitamin K, defined as prothrombin time (PT) ≥20 seconds or international normalised ratio (INR) ≥2.0 regardless of hepatic encephalopathy (HE) or PT ≥15 seconds or INR ≥1.5 in the presence of HE. Results: 34 patients were included. Median age at diagnosis was 10 months (range 7 days to 156 months). The top three causes of PALF were indeterminate (41.2%), metabolic (26.5%) and infectious (26.5%) aetiologies. A metabolic disorder was the most frequent aetiology in infants <12 months (38.9%), whereas an indeterminate cause was the most common in children >12 months (50%). No cases of viral hepatitis A or B presenting with PALF were detected. Overall spontaneous recovery rate (survival without liver transplantation [LT]) was 38.2%, and overall mortality rate was 47.1%. Six patients underwent living-donor LT, and the post-transplant survival at one year was 83.3%. Conclusion: The aetiologic spectrum of PALF in Singapore is similar to that in developed Western countries, with indeterminate aetiology accounting for the majority. PALF is associated with poor overall survival; hence, timely LT for suitable candidates is critical to improve survival outcomes.

Long-term outcome after liver transplantation in children with type 1 glycogen storage disease.

Patients with GSD type 1 (von Gierke disease) are initially managed medically to maintain normoglycemia. However, if they do not achieve good metabolic control, LT is then considered. We describe the long-term outcome of 6 children with GSD type 1 who underwent LT. Retrospective chart review of the data of 6 children with GSD type 1 who underwent LT at National University Hospital, Singapore, from May 1998 to October 2018, was performed. The median (IQR) age at diagnosis of the GSD was 1 year (0.92-5.50) and at transplant was 13.88 years (11.46-16.38). All of the patients had elevated liver enzymes, hypercholesterolemia, hypertriglyceridemia, and hyperlactatemia prior to transplant. All of the patients are alive at the time of analysis and follow-up. None of them required a re-transplant. For the three patients who had hypoglycemia pretransplant, there was no recurrence post-transplant. All of the patients had normalization of liver enzymes by 1 year post-transplant. Long-term outcome of patients with GSD who underwent LT has been positive with improvement in metabolic control for most patients. We report the unusual finding of two siblings with persistent hyperuricemia post-transplant requiring allopurinol.

Cholestatic Jaundice in Sulphite Oxidase Deficiency - An Unusual Association.

Sulphite oxidase deficiency is an extremely rare inborn error of metabolism of sulphur containing amino acids. There are no reports of liver involvement in this condition. The authors describe a 9-y-old boy with known sulphite oxidase deficiency who presented with worsening cholestatic hepatitis which may be possibly related to underlying metabolic disorder. Although there is no current evidence that treating liver disease and ensuring normal hepatic function in sulphite oxidase deficiency would likely benefit patients, this could potentially contribute to optimising growth and development as well as improving the overall prognosis.

Characteristics and outcome of primary sclerosing cholangitis associated with inflammatory bowel disease in Asian children.

BACKGROUND: Current knowledge on the clinical features and natural history of childhood primary sclerosing cholangitis - inflammatory bowel disease in Asia is limited. We described the presenting features and natural history of primary sclerosing cholangitis-inflammatory bowel disease seen in a cohort of Southeast Asian children. METHODS: We conducted a retrospective review of childhood primary sclerosing cholangitis-inflammatory bowel disease from three tertiary centers in Singapore and Malaysia. RESULTS: Of 24 patients (boys, 58%; median age at diagnosis: 6.3 years) with primary sclerosing cholangitis-inflammatory bowel disease (ulcerative colitis, n = 21; Crohn's disease, n = 1; undifferentiated, n = 2), 63% (n = 15) were diagnosed during follow-up for colitis, and 21% (n = 5) presented with acute or chronic hepatitis, 17% (n = 4) presented simultaneously. Disease phenotype of liver involvement showed 79% had sclerosing cholangitis-autoimmune hepatitis overlap, 54% large duct disease, and 46% small duct disease. All patients received immunosuppression therapy. At final review after a median [±S.D.] duration follow-up of 4.7 [±3.8] years, 12.5% patients had normal liver enzymes, 75% persistent disease, and 12.5% liver failure. The proportion of patients with liver cirrhosis increased from 13% at diagnosis to 29%; 21% had portal hypertension, and 17% had liver dysfunction. One patient required liver transplant. Transplant-free survival was 95%. For colitis, 95% had pancolitis, 27% rectal sparing, and 11% backwash ileitis at initial presentation. At final review, 67% patients had quiescent bowel disease with immunosuppression. One patient who had UC with pancolitis which was diagnosed at 3 years old developed colorectal cancer at 22 years of age. All patients survived. CONCLUSIONS: Liver disease in primary sclerosing cholangitis-inflammatory bowel disease in Asian children has variable severity. With immunosuppression, two-thirds of patients have quiescent bowel disease but the majority have persistent cholangitis and progressive liver disease.

Health-related quality of life, clinical outcomes, and subjective experiences of parent donors of pediatric liver transplant recipients.

PURPOSE: The understanding of the HRQOL issues for parent donors of children who underwent LDLT is lacking. We evaluated the HRQOL of donor and non-donor parents, described their subjective experiences and identified factors associated with lower HRQOL post-donation. METHODS: This is a cross-sectional study of parent donors whose children underwent LDLT, using SF-36v2 Health Survey to measure HRQOL, and a self-developed questionnaire to evaluate their subjective experiences. RESULTS: Of 32 pairs of donor and non-donor parents, 27 donor and 19 non-donor parents responded. The data of respondents were analyzed. Both donor and non-donor parents' SF-36v2 norm-based scores were average or above average as compared to the Singapore population. Donors who made lifestyle changes post-donation (adopting a healthy balanced diet, regular physical activity, quitting smoking, and moderate alcohol intake) were associated with lower GH (P = 0.009) and PF (P = 0.002) scores. Donors who took more than 3 months for full recovery had lower RP (P = 0.022) and BP scores (P = 0.038). On multivariate analysis, recipient complication of Clavien grade 3 or 4 was associated with increased RP score by 8.71 points (95% CI: 1.74-15.68), after adjusting for time taken for full recovery. Majority (88.8%) had self-reported recovery time under 6 months and returned to work within 3 months (74.0%). CONCLUSIONS: Donors with factors potentially associated with lower HRQOL may need more support to ensure better HRQOL outcomes post-donation.

Endoscopic retrograde cholangio-pancreatography in the management of biliary complications after paediatric liver transplantation - a retrospective study.

The published paediatric experience with endoscopic retrograde cholangio-pancreatography (ERCP) in the diagnosis and management of biliary complications following liver transplantation (LT) is limited. We describe our experience with ERCP in the management of children following LT who presented with biliary complications, over a 20-year period (1995-2014). The retrospectively reviewed data are summarized descriptively. Of 94 children (47 boys) who received 102 liver transplants at our centre, seven children (five boys, two girls) underwent ERCP after liver transplantation. In total, 25 ERCP procedures were carried out in these patients. The median age at liver transplantation was 10.7 (3.9-16.2) years. The median interval between LT and the first ERCP was 28 days (12 days-6.8 years). All patients were on standard calcineurin-inhibitor-based immunosuppression regimens. Six of the seven patients underwent ERCP on more than one occasion [median number of ERCP sessions per patient- 4, (1-6)]. Seventeen procedures were carried out under conscious sedation, remaining eight under general anaesthesia. Sedation was achieved employing a standard regimen (Midazolam 5 mg with Pethidine 50 mg) and occasionally Fentanyl. ERCP is an effective and safe intervention from both diagnostic and therapeutic point of view, in the management of post-LT biliary complications in children.

Endoscopic Evaluation in Children With End-Stage Liver Disease-Associated Portal Hypertension Awaiting Liver Transplant.

OBJECTIVES: Routine oesophago-gastro-duodenoscopy (OGD) pre-liver transplantation (LT) for evaluation and management of gastrointestinal (GI) pathology, in particular GI varices secondary to portal hypertension, is common practice in adult LT programmes. There is no universal consensus for this practice in children. We report our endoscopic experience in children with end-stage liver disease (ESLD) pre-LT. METHODS: Retrospective audit of LT database and review of OGD findings of patients who had undergone endoscopy preceding LT. RESULTS: Of 69 patients with ESLD, 50 (72.4%) had pre-LT OGD, 37 of which were done electively, whereas the remaining 13 were event driven. Forty-eight (96%) patients who underwent OGD had abnormalities, in which 38 (76%) patients had varices and 23 (46%) had portal hypertensive gastropathy. Eleven (22%) patients required therapeutic intervention at initial OGD either with endoscopic variceal band ligation or endoscopic sclerotherapy. Compared with the group who underwent elective OGDs, the group who had event-driven OGDs had a significantly higher requirement for endoscopic intervention (P < 0.0001), occurrence of rebleeding (P < 0.029) and requirement for repeat OGDs (P = 0.014). There was no significant difference in terms of patient (P = 0.2746) or graft survival (P = 0.3192) between the 2 groups. CONCLUSIONS: The role of pre-LT OGDs in patients with ESLD associated with portal hypertension is possibly limited to control of bleeding during episodes of GI bleed, where the aim would be to stabilize the patient until eventual LT. Multicentre prospective studies are required to provide more evidence on the use of routine endoscopy for pre-LT assessment in children.

Follicular carcinoma of thyroid following successful liver transplantation--a report.

Follicular carcinoma of the thyroid is a relatively rare malignancy in childhood even in paediatric solid organ transplant recipients. The risk of developing de novo malignancies after liver transplantation is higher compared to the general population. We report an 18-yr-old girl who had successfully undergone liver transplantation five yr earlier for neonatal sclerosing cholangitis complicated by the development of dysplastic nodules. Baseline immunosuppression was with tacrolimus and prednisolone. Mycophenolate mofetil was later added in view of steroid-resistant episodes of graft rejection. She subsequently suffered from marked obesity and essential hypertension needing antihypertensive medication. Five yr after liver transplantation, she presented with a right-sided thyroid swelling that was rapidly progressive with no associated lymphadenopathy and normal systemic examination. Ultrasound of her neck revealed a solid lesion in the right lobe of the thyroid gland with ill-defined margins, and a diagnostic right thyroid lobectomy confirmed the diagnosis of follicular carcinoma with focal capsular and vascular invasion. She underwent total thyroidectomy and currently remains well on thyroxine supplements. Our report highlights the need for high level of suspicion and prompt investigation into any abnormal lesion in the long-term follow-up of solid organ transplant recipients.

Carotenemia in infancy and its association with prevalent feeding practices.

Carotenemia in infancy is a relatively rare but benign condition, invariably of dietary origin, that can be confused with jaundice. It is characterized by an abnormal yellowish orange pigmentation of the skin, most prominently seen in the palms, soles, and naso-labial folds. Infant feeding patterns have shown an increasing trend toward the usage of homogenized and pureed vegetables as well as meat-based commercial preparations. Whether this is reflected in an increased incidence of carotenemia in this age group still remains unclear. We report a series of infants identified by a retrospective review of records, observed over a 3-year period (1999-2002) in a tertiary children's hospital, who developed the condition that resolved spontaneously without intervention, as they grew older, on a changing diet.