RESUMEN
Patients post liver transplant (LT) with progressive familial intrahepatic cholestasis type 1 (PFIC-1) often develop progressive graft steatohepatitis, intractable diarrhea, and growth failure. A total internal biliary diversion (TIBD) during an LT may prevent or reverse these adverse events. Children with PFIC-1 who underwent an LT at our institute were divided into 2 groups, A and B based on the timeline where we started offering a TIBD in association with LT. Pre-LT parameters, intraoperative details, and posttransplant complications like graft steatosis and diarrhea were also analyzed between the 2 groups, and their growth velocity was measured in the follow-up period. Of 550 pediatric LT performed between 2011 and 2022, 13 children underwent LT for PFIC-1. Group A had 7 patients (A1-A7) and group B had 6 (B1-B6). Patients A1, A4, B4, and B5 had a failed partial internal biliary diversion before offering them an LT. Patients A1, A2, and A6 in group A died in the post-LT period (2 early allograft dysfunction and 1 posttransplant lymphoproliferative disorder) whereas A3, A4, and A5 had graft steatosis in the follow-up period. A4 was offered a TIBD 4 years after LT following which the graft steatosis fully resolved. In group B, B1, B2, B5, and B6 underwent TIBD during LT, and B3 and B4 had it 24 and 5 months subsequently for intractable diarrhea and graft steatosis. None of the patients in group B demonstrated graft steatosis or diarrhea and had good growth catch-up during follow-up. We demonstrate that simultaneous TIBD in patients undergoing LT should be a standard practice as it helps dramatically improve outcomes in PFIC-1 as it prevents graft steatosis and/or fibrosis, diarrhea, and improves growth catch-up.
Asunto(s)
Procedimientos Quirúrgicos del Sistema Biliar , Colestasis Intrahepática , Trasplante de Hígado , Complicaciones Posoperatorias , Humanos , Trasplante de Hígado/efectos adversos , Trasplante de Hígado/normas , Trasplante de Hígado/métodos , Colestasis Intrahepática/cirugía , Colestasis Intrahepática/etiología , Colestasis Intrahepática/diagnóstico , Masculino , Femenino , Lactante , Preescolar , Resultado del Tratamiento , Procedimientos Quirúrgicos del Sistema Biliar/efectos adversos , Procedimientos Quirúrgicos del Sistema Biliar/métodos , Estudios Retrospectivos , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/prevención & control , Niño , Diarrea/etiología , Hígado Graso/etiología , Hígado Graso/cirugía , Hígado Graso/diagnóstico , Estudios de Seguimiento , Supervivencia de InjertoRESUMEN
INTRODUCTION: Maple syrup urine disease (MSUD) is caused by the deficiency of branched-chain keto acid dehydrogenase (BCKAD) and, it is well described that BCKAD contributed by an allograft following liver transplantation (LT) phenotypically normalizes this inborn error of metabolism (IEM). There is, however, a paucity of data especially with regards to the neurodevelopmental aspects and catch-up growth profiles after LT in a resource-challenged setting. We present our series of children under 6 years of age who underwent LT for MSUD particularly focusing on their amino acid homeostasis, neurodevelopmental and somatic growth profiles. METHODS: Of 580 consecutive pediatric LT (PLT) performed between January 2011 and December 2022, all children who underwent LT for MSUD were included for analysis. Data accrued included peri-LT details, pre- and post-LT metabolic profile, neurodevelopmental assessment, somatic growth evaluation, and long-term outcomes. RESULTS: Six children underwent LT for MSUD with a median age and weight at LT of 20.5 (IQR: 8-60) months and 10.1 (IQR: 6.7-15.8) kg, respectively. One explanted liver was used as a domino graft for Arginase deficiency. Median follow-up period was 52.5 (IQR: 27-94) months. None had vascular or biliary complications. Following LT, all children were started on an unrestricted protein diet and had normalization of BCAA levels. Post-LT height and weight improved by 1 SD but did not achieve the normal profile. None of the children had neuro-deterioration and have achieved new milestones. CONCLUSION: This is the first-report presenting the growth aspects, amino acid and neurodevelopmental profiles of children who underwent LT for MSUD within the socio-economic-cultural idiosyncrasies and constraints prevalent in our part of the world.
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Aminoácidos , Homeostasis , Trasplante de Hígado , Enfermedad de la Orina de Jarabe de Arce , Humanos , Enfermedad de la Orina de Jarabe de Arce/cirugía , Masculino , Femenino , Lactante , Preescolar , Aminoácidos/metabolismo , Estudios Retrospectivos , Estudios de Seguimiento , Desarrollo InfantilRESUMEN
BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare but important cause of end-stage liver disease in children. Conventional chemotherapeutic agents that are otherwise the standard-of-care in LCH may be counterproductive in patients with hepatic decompensation. Furthermore, the precise role of liver transplantation (LT) in the management of LCH remains unclear. METHODS: Review of a prospectively collected database (January 2014 to December 2020) of children with liver disease was performed. All clinical details of patients with LCH managed at our center were collected and data analyzed. Based on the outcomes, a management algorithm was proposed. RESULTS: Of the eight (five male) patients referred to our unit, six (75%) underwent LT (four and two for compensated and decompensated cirrhosis, respectively). Median age at diagnosis of LCH was 25 (range: 9-48) months. Two patients, who had previously completed LCH-specific chemotherapy, underwent upfront LT for compensated cirrhosis. Other two patients with compensated cirrhosis showed evidence of active disease. They underwent LT following completion of chemotherapy. Two children with decompensated cirrhosis also had evidence of active disease and were started on modified chemotherapy Both of them had progression of liver disease while on chemotherapy. Hence, an urgent LT was performed which was followed by completion of chemotherapy in these patients. On a median follow-up of 30.5 (10.5-50) months, all post-LT patients were alive with stable graft function and showed no disease recurrence. CONCLUSION: We demonstrate that an algorithmic approach, along with newer chemotherapeutic agents, results in excellent outcomes in LCH patients with liver involvement. Larger multicentric studies on this rare disease are, however, needed to validate our findings.
Asunto(s)
Histiocitosis de Células de Langerhans , Trasplante de Hígado , Niño , Humanos , Masculino , Lactante , Preescolar , Trasplante de Hígado/efectos adversos , Histiocitosis de Células de Langerhans/tratamiento farmacológico , Cirrosis Hepática/cirugía , Cirrosis Hepática/etiología , Recurrencia , Estudios RetrospectivosRESUMEN
OBJECTIVE: Pediatric irritable bowel syndrome (IBS) is a common functional gastrointestinal disorder with variable response to various therapeutic agents. Psyllium has been proven to be effective in adults; however, there is no study in children. The objective of this study is to evaluate the efficacy of psyllium husk as compared to placebo in pediatric IBS patients. METHODS: In this double-blind randomized controlled trial, 43 children were assigned to psyllium arm (Group A) and 38 into placebo arm (Group B). Severity is assessed at baseline and after 4 weeks of treatment using IBS severity scoring scale (IBS-SSS) and classified into mild, moderate, and severe categories. Categorical data was compared with chi-square test and paired categorical variable was compared with McNemer test. RESULTS: Mean ages (±SD; in years) of Groups A and B were 9.87 (2.7) and 9.82 (3.17), respectively, with median duration of illness of 12 months. At baseline, type, severity, and parameters (IBS-SSS) of IBS were equally distributed in 2 groups. There was a significant reduction in median interquartile range (IQR) of total IBS-SSS in psyllium versus placebo [75 (42.5-140) vs 225 (185-270); P < 0.001] at 4 weeks. Similarly 43.9% in Group A versus 9.7% in Group B attained remission [IBS-SSS < 75 ( P < 0.0001)]. The mean difference in IBS-SSS between Group A and Group B was -122.85 with risk ratio of 0.64 (95% CI; 0.42-0.83; P = 0.001) and absolute risk reduction of 32% (NNT = 3). CONCLUSIONS: Psyllium husk is effective for the therapy of pediatric IBS when compared with placebo in short term.
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Síndrome del Colon Irritable , Psyllium , Adulto , Humanos , Niño , Síndrome del Colon Irritable/complicaciones , Síndrome del Colon Irritable/tratamiento farmacológico , Psyllium/uso terapéutico , Método Doble Ciego , Índice de Severidad de la Enfermedad , Resultado del Tratamiento , Calidad de VidaRESUMEN
BACKGROUND: Progressive familial intrahepatic cholestasis (PFIC) is a heterogenous group of inherited hepatocellular disorders and the clinical aspects, role of liver transplantation (LT), and its outcomes remain largely unelucidated. We present our data of LT for each type of PFIC and compare their early, and long-term outcomes, highlighting their individual differences and management strategies. METHODS: Prospectively collected data over a decade (2011-2022) of children with PFIC who underwent LT was analyzed. The groups (PFIC 1-4) were compared with regard to early and long-term outcomes including attainment of catch-up growth. RESULTS: Of 60 children with PFIC who underwent LT, 13, 11, 31 & 5 were of PFIC 1, 2, 3 & 4, respectively. There were no significant differences in gender, PELD scores, BMI, type of grafts, cold and warm ischemia times, intraoperative blood loss, and morbidity among the groups. Post-LT chronic diarrhea was observed in 6 (46.1%) children with PFIC-I, and of them, 3 (23%) developed graft steatohepatitis. Three of these children underwent total internal biliary diversion (TIBD) and on 1-year follow-up, their graft steatosis resolved and they attained catch-up growth. Catch-up growth was significantly poorer in the PFIC1 group (44.4% vs. 88%, 90%, 100% p < .001). Overall 1- and 5-year patient survival of the four PFIC groups (1-4) were 69.2%, 81.8%, 96.8%, 100% & 69.2%, 81.8%, 96.8%, 100%, respectively. CONCLUSION: Ours is the largest to-date series of LT for PFIC illustrating their short- and long-term outcomes. While the results for the whole cohort were excellent, those after LT for PFIC1 was relatively poorer as reflected by catch-up growth, graft steatosis, and post-LT diarrhea, which can be optimized by the addition of TIBD during LT.
Asunto(s)
Colestasis Intrahepática , Hígado Graso , Trasplante de Hígado , Niño , Humanos , Progresión de la Enfermedad , Colestasis Intrahepática/cirugía , DiarreaRESUMEN
INTRODUCTION: Hepatocellular carcinoma (HCC) in pediatrics has a uniformly poor prognosis. Complete surgical resection or liver transplantation remain the only curative options. In contrast to adult HCC, literature on pediatric HCC is sparse and a majority of the distinct subtypes are undefined with regards to their histology, immunohistochemistry and prognosis. CASE REPORT: Two infants, one with biliary atresia and another with transaldolase deficiency, underwent living donor liver transplants. Explant-liver histopathology revealed tumor with diffuse neoplastic syncytial giant cell pattern. Immunophenotypic characterization highlighted expression of epithelial cell adhesion molecule, alpha fetoprotein and metallothionein. CONCLUSION: HCC with syncytial giant cells variant can occur in infants with underlying liver disease, specifically in our experience, with biliary atresia and another with transaldolase deficiency.
Asunto(s)
Atresia Biliar , Carcinoma Hepatocelular , Neoplasias Hepáticas , Trasplante de Hígado , Adulto , Lactante , Humanos , Niño , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/patología , Carcinoma Hepatocelular/diagnóstico , Carcinoma Hepatocelular/patología , Donadores Vivos , Pronóstico , Células Gigantes/patologíaRESUMEN
INTRODUCTION: There is paucity of data on neurological complications (NCs) and its predisposing factors, in pediatric liver transplant (PLT) recipients. METHODS: Records of seventy-one children who underwent LT between October 2018 and November 2019 were reviewed. Patients were categorized into group A: with NC and group B: without NC in the post-LT period. Various risk factors contributing to NC were studied. RESULTS: In total, 15 (21.1%) had NC (group A) and 56 (78.9%) had no NC in the post-LT period. NC included cerebrovascular accident (n = 1), seizures (n = 5; 4 generalized, 1 focal), central pontine myelolysis (CPM) (n = 1), diaphragmatic palsy (n = 2), peripheral neuropathy (n = 1), extrapyramidal movements (n = 3), and encephalopathy beyond 96 h (n = 2). The median onset of NC was at 8.5 days post-LT (1-58 days). Ten (66.7%) patients in group A had grades 2-4 hepatic encephalopathy (HE) prior to LT. Eight (14.3%) patients in group B also had pre-LT neurological issues including HE in six, epilepsy and spastic diplegia in one each. On univariate analysis, pre-existing HE, high PELD/MELD score, pre-LT ventilation, pre-LT infection, higher day 1 post-operative bilirubin (all p < .05), and higher tacrolimus were found to predict post-operative NC whereas on multivariate analysis, pre-LT HE was the only predictive factor. Median follow-up was 15.5 months. Four patients died in each group (survival log-rank p = .369). All the surviving patients in group A (n = 11) fully recovered from the NC. CONCLUSION: Pre-transplant HE was the single most significant predisposing factor for post-LT neurological complications.
Asunto(s)
Encefalopatía Hepática , Trasplante de Hígado , Humanos , Niño , Trasplante de Hígado/efectos adversos , Estudios Retrospectivos , Tacrolimus , Encefalopatía Hepática/etiología , Factores de Riesgo , Convulsiones/etiología , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiologíaRESUMEN
Primary tuberculosis (TB) of the graft presenting as multiple liver abscesses is previously unreported. A 14-month-old male child in the early post liver transplant (LT) period presented with high-grade fever spikes and on evaluation was found to have multiple pyogenic liver abscesses (PLA) in the CT abdomen. His fever was not responding to intravenous antibiotics and liver biopsy was done which showed numerous acid fast bacilli. Genetic analysis confirmed the bacilli as Mycobacterium tuberculosis (MTB). Timely diagnosis and prompt introduction of antituberculosis therapy were lifesaving.
Asunto(s)
Absceso Piógeno Hepático , Mycobacterium tuberculosis , Tuberculosis , Antituberculosos/uso terapéutico , Humanos , Lactante , Absceso Piógeno Hepático/tratamiento farmacológico , Masculino , Tuberculosis/tratamiento farmacológicoRESUMEN
OBJECTIVES: Pediatric transplant recipients may be at increased risk of developing serious infections due to COVID-19. We undertook a web-based survey among parents of post-liver transplant pediatric patients to assess knowledge and concerns regarding COVID-19 pandemic and impact of social media on them. METHODS: This cross-sectional online survey was conducted between March 21 and March 26, 2020. A 19-item questionnaire was sent to 172 parents of post-liver transplant children. RESULTS: 106 (62%) of parents responded. Median time since transplant was 31 (12-52) months. The majority of parents had good understanding regarding symptomatology and routes of transmission. Only 27% were aware of feco-oral transmission, and 34% knew about gastrointestinal symptoms of COVID-19. 100% of parents understood concept of social distancing, and 70% knew that asymptomatic individuals can transmit the virus. Television followed by newspapers was the main source of their information, though over 40% claim to regularly receive information through social media. 87% would consult their doctor if the child had flu-like symptoms rather than modify immunosuppression or try alternative medications. Parental concerns mainly revolved around early recognition of symptoms, queries on unconventional treatments circulating over social media, and supply of medications during the lockdown period. CONCLUSIONS: The majority of parents had basic understanding of COVID-19 pandemic. Social media appeared to be an important source of information. Results from this survey helped us in modifying patient care protocols to ensure continuity of care while maintaining social distancing.
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Concienciación , COVID-19/prevención & control , Trasplante de Hígado , Padres/psicología , Niño , Estudios Transversales , Femenino , Humanos , Masculino , SARS-CoV-2 , Medios de Comunicación Sociales , Encuestas y CuestionariosRESUMEN
DH is a rare but well-recognized complication of PLT. However, a recurrent DH in the setting of PLT has not been reported. We report the case of a child who had previously undergone a DH repair early after PLT and presented more than two years later with atypical findings of severe sepsis and a tender abdominal swelling.
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Hernia Diafragmática/diagnóstico , Hernia Diafragmática/cirugía , Trasplante de Hígado , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/cirugía , Diagnóstico Diferencial , Femenino , Humanos , Lactante , RecurrenciaRESUMEN
BACKGROUND AND OBJECTIVES: Nearly three months after its first recorded case, the progression of the coronavirus disease (COVID-19) pandemic has been slow in India so far, with relatively low number of cases and deaths. The behavior of the general public will probably have the most important bearing on the course of the disease over the next few months in India. We aim to study the awareness, attitudes and perceptions of COVID-19 among the adult Indian population. METHODS: A cross-sectional online survey was conducted using the 'Google Survey Forms' between 29th March and 14th April 2020 and distributed through email and various social media groups. RESULTS: There were 1502 respondents, majority were male (56.7%), between 30-49 years (47.7%). 90% of the respondents had either an undergraduate or postgraduate degree, with a third of them being in the healthcare sector (34.6%). Most of the respondents were aware of the common symptoms of COVID-19, but worryingly only a third (31%) were aware of the risk of spread from infected asymptomatic individuals, which is a major concern in India. Majority were aware of the modes of virus transmission, but only two-thirds (68.6%) were aware of the safe physical distance (6 feet) for maintain social distancing. A majority of respondents were appreciative of the government interventions in containing the virus spread and would support further extension of lockdown if necessary. CONCLUSION: Despite limitations of generalizability, this survey has identified areas which the public health authorities need to target in future information campaigns.
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Infecciones por Coronavirus , Coronavirus , Conocimientos, Actitudes y Práctica en Salud , Pandemias , Neumonía Viral , Adulto , Betacoronavirus , COVID-19 , Estudios Transversales , Humanos , India/epidemiología , Masculino , SARS-CoV-2 , Encuestas y CuestionariosAsunto(s)
Neoplasias Ováricas , Teratoma , Humanos , Femenino , Vesícula Biliar/diagnóstico por imagen , Teratoma/diagnóstico , Teratoma/cirugía , HígadoRESUMEN
Bertiella is a common parasite seen in non-human primates. It is rarely seen in humans. We present the case of a 2-year-old child with bertiellosis. He had recurrent abdominal pain, and worm fragments were found in stool, which were refractory to albendazole therapy.
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Dolor Abdominal/etiología , Cestodos/aislamiento & purificación , Infecciones por Cestodos/diagnóstico , Heces/parasitología , Animales , Antihelmínticos/uso terapéutico , Infecciones por Cestodos/tratamiento farmacológico , Preescolar , Humanos , Masculino , Praziquantel/uso terapéutico , Resultado del TratamientoRESUMEN
Kawasaki disease (KD) is an acute febrile illness of childhood associated with vasculitis of medium-sized arteries especially the coronary arteries. Typical clinical features involving the skin, mucous surfaces, etc., occur sequentially over a few days. We report a rare presentation of KD as a surgical abdomen in a 2-year-old boy. Awareness of this presentation is important as it can otherwise lead to a delay in starting potentially life-saving intervention like intravenous immunoglobulins for cardiac complications kept cryptic by the manifest acute abdomen.