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BACKGROUND AND AIMS: A new definition of dominant stricture (NDS) has recently been defined for patients with primary sclerosing cholangitis (PSC). Prevalence and clinical features of this, compared to traditional dominant stricture (TDS), have not been reported. METHODS: In this single-centre longitudinal prospective cohort study, all PSC patients who underwent endoscopic retrograde cholangiopancreatography (ERCP) between October 2021 and 2022 were recruited. Symptoms of cholestasis, laboratory values (P-alkaline phosphatase, P-Bilirubin), Helsinki PSC-score, brush cytology findings and need for endoscopic therapy (i.e. dilation, stenting) were prospectively collected. RESULTS: Overall, 228 patients with PSC underwent 248 ERCPs. NDS was detected in 43 (17%; 36 patients) and TDS without NDS (TDS group) was detected in 62 (25%; 58 patients) ERCPs, respectively; in the remaining 143 ERCPs, neither TDS nor NDS was seen (no dominant stricture [NoDS] group). PSC duration (median 8 years) and patient's age did not differ between the three groups; males presented more often with NDS. Patients with NDS were more often symptomatic, had higher cholestatic liver enzymes, advanced bile duct disease and markers of biliary inflammation (p < .001). Patients with NDS needed dilation (81%) and stenting (21%) more often than the TDS group (60% and 5%, respectively). Dysplasia in brush cytology was more common in TDS (5%) and NDS (9%) than in NoDS (3%) groups (p = .04), but did not differ between TDS and NDS groups. CONCLUSIONS: Dominant stricture according to the new definition developed in 17% of PSC patients in our cohort and identifies patients with more advanced disease, biliary inflammation and need of endo-therapy.
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Colangiopancreatografia Retrógrada Endoscópica , Colangitis Esclerosante , Humanos , Colangitis Esclerosante/epidemiología , Colangitis Esclerosante/complicaciones , Colangitis Esclerosante/patología , Masculino , Femenino , Persona de Mediana Edad , Estudios Prospectivos , Adulto , Prevalencia , Estudios Longitudinales , Constricción Patológica , Colestasis/etiología , Colestasis/epidemiología , Anciano , Stents , Relevancia ClínicaRESUMEN
BACKGROUND: Reference guidelines for neonatal conjugated hyperbilirubinemia (cholestasis) management use a uniform approach regardless of gestational age (GA). We hypothesize that the clinical pattern of neonatal cholestasis is tightly related to GA. The aim of this study was to describe the effects of GA on neonatal cholestasis. METHODS: A retrospective 4-year cohort study in a 70-bed neonatal care unit. Neonates with conjugated bilirubin≥34.2µmol/L (2âmg/dL) were identified. The incidence, clinical characteristics, etiology, treatment, and prognosis were compared between infantsâ<32 and≥32 weeks GA. RESULTS: Overall incidence of cholestasis was 4% (125/3402). It wasâ>5 times higher and the mean duration wasâ>1.5 times longer in neonatesâ<32 weeks GA (10% versus 1.8%, pâ<0.01 and 49 versus 31 days, pâ<0.01, respectively). The onset of cholestasis was later in neonatesâ<32 weeks (22 versus 10 days of life, pâ<0.001). This later onset of cholestasis was associated with parenteral nutrition, whereas the earlier onset was associated with other causes. Treatment using fish oil lipids was more frequently administrated to infantsâ<32 weeks GA, whereas Ursodeoxycholic acid was administrated more frequently in≥32 weeks GA. Cholestasis resolved during hospitalization in 73% ofâ<32 versus 38% in≥32 weeks GA infants (pâ<0.01). CONCLUSIONS: The incidence, clinical presentation, etiology, treatment, and clinical evolution of neonatal cholestasis were all significantly affected by GA. Our results support the use of a GA-oriented approach for the management of neonatal cholestasis.
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Colestasis , Enfermedades del Recién Nacido , Lactante , Recién Nacido , Humanos , Edad Gestacional , Recien Nacido Prematuro , Estudios Retrospectivos , Estudios de Cohortes , Colestasis/epidemiología , Colestasis/etiologíaRESUMEN
BACKGROUND: It is proposed that the development of parenteral nutrition-associated cholestasis (PNAC) was significantly associated with preterm birth, low birth weight, infection, etc.; however, the etiology and pathogenesis of PNAC are not fully understood. Most of the studies examining PNAC-associated risk factors were single-center studies with relatively small sample sizes. OBJECTIVE: To analyze the risk factors associated with PNAC in preterm infants in China. METHODS: This is a retrospective multicenter observational study. Clinical data on the effect of multiple oil-fat emulsions (soybean oil-medium chain triglycerides-olive oil-fish oil, SMOF) in preterm infants were collected from a prospective multicenter randomized controlled study. A secondary analysis was performed in which preterm infants were divided into the PNAC group and the non-PNAC group based on the PNAC status. RESULTS: A total of 465 cases very preterm infants or very low birth weight infants were included in the study in which 81 cases were assigned to the PNAC group and 384 cases were assigned to the non-PNAC group. The PNAC group had a lower mean gestational age, lower mean birth weight, longer duration of invasive and non-invasive mechanical ventilation, a longer duration oxygen support, and longer hospital stay (P < 0.001 for all). The PNAC group had higher respiratory distress syndrome, hemodynamically significant patent ductus arteriosus, necrotizing enterocolitis (NEC) with stage II or higher, surgically treated NEC, late-onset sepsis, metabolic bone disease, and extrauterine growth retardation (EUGR) compared to the non-PNAC group (P < 0.05 for all). In contrast with the non-PNAC group, the PNAC group received a higher maximum dose of amino acids and fat emulsion, more medium/long-chain fatty emulsion, less SMOF, had a longer duration of parenteral nutrition, lower rates of breastfeeding, higher incidence of feeding intolerance (FI), more accumulated days to achieve total enteral nutrition, less accumulated days of total calories up to standard 110 kcal/kg/day and slower velocity of weight growth (P < 0.05 for all). Logistic regression analysis indicated that the maximum dose of amino acids (OR, 5.352; 95% CI, 2.355 to 12.161), EUGR (OR, 2.396; 95% CI, 1.255 to 4.572), FI (OR, 2.581; 95% CI, 1.395 to 4.775), surgically treated NEC (OR, 11.300; 95% CI, 2.127 ~ 60.035), and longer total hospital stay (OR, 1.030; 95% CI, 1.014 to 1.046) were independent risk factors for the development of PNAC. SMOF (OR, 0.358; 95% CI, 0.193 to 0.663) and breastfeeding (OR, 0.297; 95% CI, 0.157 to 0.559) were protective factors for PNAC. CONCLUSIONS: PNAC can be reduced by optimizing the management of enteral and parenteral nutrition and reducing gastrointestinal comorbidities in preterm infants.
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Colestasis , Nacimiento Prematuro , Femenino , Recién Nacido , Humanos , Recien Nacido Prematuro , Emulsiones/química , Peso al Nacer , Estudios Prospectivos , Nacimiento Prematuro/etiología , Colestasis/etiología , Colestasis/epidemiología , Nutrición Parenteral/efectos adversos , Recién Nacido de muy Bajo Peso , Aminoácidos , Factores de RiesgoRESUMEN
INTRODUCTION: The development of non-anastomotic biliary strictures (NAS) following orthotopic adult liver transplantation (OLT) is associated with significant morbidity. We performed a systematic review and meta-analysis to identify all prognostic factors for the development of NAS. METHODS: A systematic review was conducted following preferred reporting items for systematic reviews and meta-analyses (PRISMA) and the meta-analysis of observational studies in epidemiology (MOOSE) guidelines. We used the Newcastle-Ottawa scale to assess the quality of the included studies. Using the random-effects model, we calculated the weighted pooled odds ratios (OR), mean differences (MD), hazard ratios (HR), and 95% confidence intervals (CI) of the risk factors. RESULTS: Based on 19 international studies that included a total of 8269 adult LT patients, we calculated an 8% overall incidence of NAS. In this study, 7 potential prognostic factors were associated with a statistically significant hazard ratio for NAS in pooled analyses including (1) DCD donors compared to DBD donors (2) PSC as an indication for a liver transplant (3) Roux-en-Y bile duct reconstruction compared to duct-to-duct reconstruction (4) hepatic artery thrombosis (5) longer cold ischemia time (6) longer warm ischemia time (7) and total operative times. CONCLUSION: In this systematic review and meta-analysis, we identified 7 prognostic factors for the development of NAS following OLT. These findings might lay the groundwork for development of diagnostic algorithms to better risk stratify patients at risk for development of NAS.
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Colangitis Esclerosante , Colestasis , Trasplante de Hígado , Humanos , Trasplante de Hígado/efectos adversos , Colangitis Esclerosante/cirugía , Constricción Patológica/etiología , Pronóstico , Colestasis/epidemiología , Colestasis/etiología , Estudios Retrospectivos , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiologíaRESUMEN
INTRODUCTION: The occurrence of anastomotic biliary stricture (BS) remains an essential issue following liver transplantation (LT). The present study aimed to compare our findings regarding the incidence of anastomotic BS to what is known. METHODS: The present study is a single-center, retrospective cohort study of a total number of 717 consecutive patients (426 men and 291 women) who had undergone LT from January 2001 to March 2016. Multivariable Cox regression analysis was conducted to evaluate the risk factors associated with anastomotic BS development. RESULTS: Post-transplant anastomotic BS developed in 70 patients (9.8%). In the Cox multivariate analysis (a stepwise forward conditional method), factors including biliary leak (hazard ratio [HR]: 6.61, 95% confidence interval [CI]: 3.08-17.58, p < 0.001), hepatic artery thrombosis (HR: 2.29, 95% CI: 1.03-5.88; p = 0.003), and acute rejection (HR: 2.18, 95% CI: 1.16-3.37; p = 0.006) were identified as independent risk factors for the development of anastomotic BS. Surgery in 6 cases (66.7%), followed by endoscopic retrograde cholangiopancreatography (ECRP) with a metal stent in 18 cases (62.1%), percutaneous transhepatic biliary drainage in 9 (20.9%), and ERCP with a single plastic stent in 8 (18.2%), had the highest effectiveness rates in the management of BS, respectively. CONCLUSIONS: Risk factors including biliary leak, hepatic artery thrombosis, and acute rejection were independently associated with an anastomotic BS. ERCP with a metal stent may be considered as an effective treatment procedure with a relatively low complication rate in the management of benign post-LT anastomotic BS.
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Colestasis , Trasplante de Hígado , Colangiopancreatografia Retrógrada Endoscópica/efectos adversos , Colestasis/epidemiología , Colestasis/etiología , Colestasis/cirugía , Constricción Patológica/etiología , Femenino , Humanos , Trasplante de Hígado/efectos adversos , Trasplante de Hígado/métodos , Masculino , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
INTRODUCTION: The aims of the study were to describe the neurodevelopmental outcome of extremely low birth weight (ELBW) infants with parenteral nutrition-associated cholestasis (PNAC) and to assess whether PNAC is associated with adverse neurodevelopmental outcome. METHODS: The study is a secondary analysis of controlled trial (June 2012-October 2017) on PNAC incidence in ELBW infants receiving two different parenteral lipid emulsions (mixed lipid emulsion containing fish oil vs. soybean oil-based). Neurodevelopmental follow-up at 12- and 24-month corrected age was compared in infants with and without PNAC. A machine learning-based regression analysis was used to assess whether PNAC was associated with adverse neurodevelopmental outcome. RESULTS: For assessment of neurodevelopmental outcome (Bayley-III), 174 infants were available at 12-month (PNAC: n = 21; no PNAC: n = 153) and 164 infants at 24-month (PNAC: n = 20; no PNAC: n = 144) corrected age. The neurodevelopment of ELBW infants with PNAC was globally delayed, with significantly lower cognitive, language, and motor scores at both 12- and 24-month corrected age. Regression analyses revealed that PNAC was associated with an adverse motor outcome. CONCLUSION: ELBW infants with PNAC are at increased risk for adverse neurodevelopmental outcome.
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Colestasis , Recien Nacido con Peso al Nacer Extremadamente Bajo , Peso al Nacer , Colestasis/epidemiología , Colestasis/etiología , Colestasis/terapia , Aceites de Pescado , Humanos , Recién Nacido , Nutrición Parenteral/efectos adversos , Aceite de SojaRESUMEN
OBJECTIVE: Persistent cholestasis may follow acute liver failure (ALF), but its course remains unknown. We aimed to describe the prevalence, onset, severity, duration and resolution of post-ALF cholestasis. DESIGN: Cohort of 127 adult patients with ALF at a liver transplantation centre identified using electronic databases. We obtained laboratory data every 6 hours for the first week, daily until day 30 and weekly, when documented, until day 180. RESULTS: Median age was 40.7 (IQR 31.0-52.4) years, median peak alanine aminotransferase level was 5494 (2521-8819) U/L and 87 (68.5%) cases had paracetamol toxicity. Overall, 12.6% underwent transplantation (3.4% for paracetamol vs 32.5% for non-paracetamol; p<0.001). Ninety-day mortality was 20.7% for paracetamol versus 30.0% for non-paracetamol patients. All non-transplanted survivors reached a bilirubin level>50 µmol/L, which peaked 3.5 (1.0-10.1) days after admission at 169.0 (80.0-302.0) µmol/L. At hospital discharge, 18.8% of patients had normal bilirubin levels and, at a median follow-up time from admission to last measurement of 16 (10-30) days, 46.9% had normal levels. Similarly, there was an increase in alkaline phosphatase (ALP) (207.0 (148.0-292.5) U/L) and gamma-glutamyl transferase (GGT) (336.0 (209.5-554.5) U/L) peaking at 4.5 days, with normalised values in 40.3% and 8.3% at hospital discharge. CONCLUSION: Post-ALF cholestasis is ubiquitous. Bilirubin, ALP and GGT peak at 3 to 5 days and, return to baseline in the minority of patients at median follow-up of 16 days. These data inform clinical expectations of the natural course of this condition.
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Colestasis , Fallo Hepático Agudo , Acetaminofén/efectos adversos , Adulto , Fosfatasa Alcalina , Bilirrubina , Colestasis/epidemiología , Humanos , Fallo Hepático Agudo/epidemiología , Prevalencia , gamma-GlutamiltransferasaRESUMEN
OBJECTIVE: To estimate the incidence of cholestasis in neonates with hemolytic disease of the fetus and newborn (HDFN) and investigate risk factors and long-term liver disease. STUDY DESIGN: A population-based cohort study of all infants born with HDFN within the Stockholm region between 2006 and 2015. The study period was the first 90 days of life, and presence of any chronic liver disease was evaluated at two years of age. RESULTS: Cholestasis occurred in 7% (11/149). Median age at detection was 1.1 days. Intrauterine blood transfusions and maternal alloimmunization with multiple red blood cell antibodies including D-, c- or K-antibodies were independent risk factors for cholestasis. No infant had chronic liver disease at two years of age. CONCLUSIONS: Infants with severe HDFN have increased risk for cholestasis, particularly those requiring multiple intrauterine transfusions. Early and repeated screening for conjugated hyperbilirubinemia in the first week of life is needed to ensure adequate management.
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Colestasis , Eritroblastosis Fetal , Colestasis/epidemiología , Colestasis/etiología , Estudios de Cohortes , Eritroblastosis Fetal/epidemiología , Eritroblastosis Fetal/etiología , Femenino , Feto , Humanos , Incidencia , Lactante , Recién Nacido , Factores de RiesgoRESUMEN
BACKGROUND: Pancreatic cancer (PCa) and biliary tract carcinomas (BTCa) have high morbidity and mortality rates. Bile duct obstruction (BDO) develops in approximately 65-75% of PCa at diagnosis, delaying the administration of optimal treatment. In patients not candidates for surgery, BDO is usually treated through the endoscopy-guided placement of self-expanding stents in the bile duct. METHODS: In this retrospective study, we sought to describe clinical characteristics and outcomes of a cohort of patients with BDO of malignant origin who underwent biliary stent placement (BSP), with a special focus regarding complications developed after the procedure. Patients with PCa, BTCa, colon cancer, lung cancer, gastric cancer, and ovarian cancer who underwent BSP from 2014 to April 2019 at our institution were included in this cohort. Demographic and clinicopathologic characteristics were collected. Statistical analysis stratified according to ECOG performance status. Specific information regarding stent material (metallic vs. plastic), as well as incidence and type of complications derived from BSP, was also recorded. RESULTS: One hundred fifteen patients were included. The median age was 72 years. Sixty-six patients (57%) had PCa. All patients presented hyperbilirubinemia, which decreased after the procedure in 111 (96%) patients. Complications were observed in 44 (38%) patients, most of which 33 (75%) were infections. The median time to the complication was 1 month. The mean overall survival (OS) in our study was 20.3 weeks. CONCLUSION: BSP effectively decreased hyperbilirubinemia in patients with BDO; however, the procedure associated a significant rate of infectious complications, which can further compromise an effective anti-cancer therapy as well as optimal palliative strategies.
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Colestasis , Neoplasias Pancreáticas , Anciano , Conductos Biliares , Colestasis/epidemiología , Colestasis/etiología , Colestasis/cirugía , Humanos , Incidencia , Cuidados Paliativos/métodos , Neoplasias Pancreáticas/complicaciones , Estudios Retrospectivos , Stents/efectos adversos , Resultado del TratamientoRESUMEN
Trisomy 13 and 18 are common chromosomal abnormalities that affect multiple organ systems. There is a paucity of published data, however, on the hepatic complications seen in these patient populations. One of the most common pathologic hepatobiliary issues seen in the newborn period is direct hyperbilirubinemia (DH). Thus, this study sought to estimate the incidence and evaluate possible etiologies of DH in neonates with trisomy 13 or 18. This retrospective cohort study included all infants admitted to our two neonatal intensive care units between 2012 and 2020 with the diagnosis of trisomy 13 or 18. DH is most commonly diagnosed as a direct bilirubin >1 mg/dl but a cutoff of >2 mg/dl is more specific for cholestasis, so both cutoffs were evaluated. Continuous data were compared using Fisher's exact test and categorical variables by the Mann-Whitney U test. Thirty-five patients met inclusion: 13 with trisomy 13 and 22 with trisomy 18. DH of >2 mg/dl was seen in seven (53.8%) patients with trisomy 13 and five (22.7%) with trisomy 18. Using a cutoff of >1 mg/dl, the rate of trisomy 13 was unchanged, but the rate in trisomy 18 increased to 9/22 (40.9%). There was a trend toward more DH in trisomy 13 patients (p = 0.079) versus trisomy 18 and higher rates in infants who received total parenteral nutrition (TPN) (50.0 vs. 13.3%, p = 0.026). The presence of cardiac or ultrasound-defined hepatobiliary abnormalities was not correlated with DH. Due to the high rates of DH in hospitalized neonates with trisomy 13 and 18, we recommend screening newborns with trisomy 13 or 18 for DH starting in the first week of life and continuing at least weekly until 4 weeks of life or until completion of TPN, whichever comes later. Future studies should further evaluate possible etiologies of DH in this population.
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Colestasis , Hiperbilirrubinemia , Colestasis/complicaciones , Colestasis/diagnóstico , Colestasis/epidemiología , Humanos , Hiperbilirrubinemia/complicaciones , Hiperbilirrubinemia/epidemiología , Lactante , Recién Nacido , Nutrición Parenteral Total/efectos adversos , Estudios Retrospectivos , Síndrome de la Trisomía 13/complicaciones , Síndrome de la Trisomía 13/diagnóstico , Síndrome de la Trisomía 13/epidemiologíaRESUMEN
BACKGROUND/AIMS: Biliary complications including biliary strictures and bile leaks are the most common complications that occur after liver transplantation (LT). Endoscopic treatment with endoscopic retrograde cholangiography (ERC) is considered the first-line treatment, and percutaneous transhepatic cholangiography (PTC) can serve as an alternative or rescue therapy. However, nationwide clinical data on the frequency of ERC and PTC following LT have not yet been investigated. METHODS: Using the nationwide claims database, we investigated patients who underwent LT between 2012 and 2014 in Korea and followed them until 2015. We analyzed the prevalence and characteristics of patients and biliary procedures, including ERC and PTC implemented after LT. RESULTS: A total of 3,481 patients underwent LT during the 3-year study period. Among them, 3.0% of patients underwent biliary intervention postoperatively during the same hospitalization period, and 21.4% of patients received biliary intervention later on after initially being discharged from the hospital following LT. A total of 16.9% and 12.1% of patients underwent ERC and PTC after LT, respectively. The median period from LT to the first biliary intervention was 7.8 months (interquartile range, 3.5 to 14.6 months), and these patients underwent an average of 3.2±2.8 biliary procedures during the follow-up period. Patients undergoing living donor LT were more than twice as likely to undergo biliary procedures as those undergoing deceased donor LT (25.5% vs 12.1%). CONCLUSIONS: Approximately one-fourth of patients in Korea who underwent LT subsequently underwent ERC or PTC. Compared with deceased donor LT patients, those undergoing living donor LT underwent more biliary interventions and were more difficult to treat.
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Colestasis , Trasplante de Hígado , Colangiopancreatografia Retrógrada Endoscópica/métodos , Colestasis/epidemiología , Colestasis/etiología , Colestasis/cirugía , Humanos , Trasplante de Hígado/efectos adversos , Donadores Vivos , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/cirugía , Estudios RetrospectivosRESUMEN
Despite recent advances, the causes of and effective therapies for pediatric chronic cholestatic diseases remain elusive, and many patients progress to liver failure and need liver transplantation. Malnutrition is a common complication in these patients and is a well-recognized, tremendous challenge for the clinician. We undertook a narrative review of both recent and relevant older literature, published during the last 20 years, for studies linking nutrition to pediatric chronic cholestasis. The collected data confirm that malnutrition and failure to thrive are associated with increased risks of morbidity and mortality, and they also affect the outcomes of liver transplantation, including long-term survival. Malnutrition in children with chronic liver disease is multifactorial and with multiple potential nutritional deficiencies. To improve life expectancy and the quality of life, patients require careful assessments and appropriate management of their nutritional statuses by multidisciplinary teams, which can identify and/or prevent specific deficiencies and initiate appropriate interventions. Solutions available for the clinical management of these children in general, as well as those directed to specific etiologies, are summarized. We particularly focus on fat-soluble vitamin deficiency and malnutrition due to fat malabsorption. Supplemental feeding, including medium-chain triglycerides, essential fatty acids, branched-chain amino acids, and the extra calories needed to overcome the consequences of anorexia and high energy requirements, is reviewed. Future studies should address the need for further improving commercially available and nutritionally complete infant milk formulae for the dietary management of this fragile category of patients. The aid of a specialist dietitian, educational training regarding nutritional guidelines for stakeholders, and improving family nutritional health literacy appear essential.
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Colestasis/epidemiología , Hepatopatías/epidemiología , Desnutrición/epidemiología , Estado Nutricional , Adolescente , Niño , Preescolar , Colestasis/complicaciones , Enfermedad Crónica , Dieta/métodos , Ingestión de Energía , Femenino , Humanos , Lactante , Fórmulas Infantiles , Hepatopatías/complicaciones , Fallo Hepático/epidemiología , Fallo Hepático/etiología , Trasplante de Hígado , Masculino , Desnutrición/etiología , Necesidades Nutricionales , Calidad de Vida , Vitaminas/administración & dosificaciónRESUMEN
OBJECTIVES: The aim of the study was to determine the frequency and natural history of infantile idiopathic cholestasis (IC) in a large, prospective, multicenter cohort of infants. METHODS: We studied 94 cholestatic infants enrolled up to 6âmonths of age in the NIDDK ChiLDReN (Childhood Liver Disease Research Network) "PROBE" protocol with a final diagnosis of IC; they were followed up to 30âmonths of age. RESULTS: Male sex (66/94; 70%), preterm birth (22/90 with data; 24% born at < 37 weeks' gestational age), and low birth weight (25/89; 28% born at <2500âg) were frequent, with no significant differences between outcomes. Clinical outcomes included death (nâ=â1), liver transplant (nâ=â1), biochemical resolution (total bilirubin [TB] ≤1âmg/dL and ALTâ<â35âU/L; nâ=â51), partial resolution (TBâ>â1âmg/dL and/or ALTâ>â35âU/L; nâ=â7), and exited healthy (resolved disease per study site report but without documented biochemical resolution; nâ=â34). Biochemical resolution occurred at median of 9âmonths of age. GGT was <100âU/L at baseline in 34 of 83 participants (41%). CONCLUSIONS: Frequency of IC and of death or liver transplant was less common in this cohort than in previously published cohorts, likely because of recent discovery and diagnosis of genetic etiologies of severe/persistent cholestasis that previously were labeled as idiopathic. Preterm birth and other factors associated with increased vulnerability in neonates are relatively frequent and may contribute to IC. Overall outcome in IC is excellent. Low/normal GGT was common, possibly indicating a role for variants in genes associated with low-GGT cholestasis-this warrants further study.
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Colestasis , Nacimiento Prematuro , Bilirrubina , Niño , Preescolar , Colestasis/diagnóstico , Colestasis/epidemiología , Colestasis/etiología , Femenino , Edad Gestacional , Humanos , Lactante , Recién Nacido , Masculino , Embarazo , Estudios ProspectivosRESUMEN
BACKGROUND: Neonatal cholestasis (NC) is one of the most serious diseases in newborns and infants and results from metabolic disorders, such as Niemann-Pick type C (NPC), among other causes. OBJECTIVE: We evaluated the incidence of NPC in our NC plus lysosomal storage disease (LSD) suspicious neonates and infants series. METHODS: The study included children (≤3 years old) with a history of NC together with a suspicion of LSD, referred from Spanish Hospitals during the period 2011-2020. Screening for NPC was done by plasma biomarker assay (chitotriosidase activity and 7-ketocholesterol), and Sanger sequencing for NPC1 and NPC2 genes. RESULTS: We screened NPC disease in 17 patients with NC plus organomegaly and that were LSD suspicious, finding 5 NPC patients (29.4%) and 2 carriers. CONCLUSIONS: Our results emphasize the need to study NPC when NC and visceral enlargement arise in a newborn or infant.
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Colestasis , Enfermedad de Niemann-Pick Tipo C , Biomarcadores/sangre , Preescolar , Colestasis/diagnóstico , Colestasis/epidemiología , Humanos , Lactante , Recién Nacido , Tamizaje Masivo , Enfermedad de Niemann-Pick Tipo C/diagnóstico , Enfermedad de Niemann-Pick Tipo C/epidemiologíaRESUMEN
PURPOSE: To investigate late complications after surgery for congenital biliary dilatation (CBD). METHODS: We retrospectively reviewed the patients treated for late postoperative complications of extrahepatic bile duct resection with bilioenteric anastomosis for CBD at our hospital between 1999 and 2019. RESULTS: Twenty-seven complications, including bile duct stenosis with (n = 19) or without (n = 3) hepatolithiasis, remnant intrapancreatic bile duct (n = 2), intestinal obstruction (n = 2), and refractory cholangitis (n = 1) were treated in 26 patients. The median age at radical surgery and the initial treatment of complications was 3 years, 2 months and 14 years, 5 months, respectively. The median period from radical surgery to initial treatment of complications was 7 years, 1 month. Before 2013, bile duct stenosis was initially treated with bile duct plasty (n = 11) or hepatectomy (n = 3), and 71.4% (n = 10) of patients needed further treatment; after 2013, double-balloon endoscopic retrograde cholangiography (DBERC) was used (n = 8), and 25% (n = 2) of patients needed further treatment. Patients with remnant intrapancreatic bile duct, intestinal obstruction, and refractory cholangitis required surgery. CONCLUSION: Long-term follow-up is necessary after surgery for congenital biliary dilatation. DBERC is thus considered to be useful for bile duct stenosis management.
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Anastomosis Quirúrgica/métodos , Conductos Biliares Extrahepáticos/cirugía , Procedimientos Quirúrgicos del Sistema Biliar/métodos , Colangitis/terapia , Quiste del Colédoco/cirugía , Colestasis/terapia , Obstrucción Intestinal/terapia , Complicaciones Posoperatorias/terapia , Factores de Edad , Colangiopancreatografia Retrógrada Endoscópica/métodos , Colangitis/epidemiología , Colestasis/epidemiología , Femenino , Estudios de Seguimiento , Humanos , Obstrucción Intestinal/epidemiología , Masculino , Complicaciones Posoperatorias/epidemiología , Estudios Retrospectivos , Factores de TiempoRESUMEN
PURPOSE: The etiology of cholestasis in neonates is associated with several factors including gastrointestinal disease and surgery. We aimed to identify the potential risk factors for perioperative cholestasis in patients with duodenal atresia and determine specific cutoff values for the risk factors. METHODS: This retrospective cohort study included 76 neonates diagnosed with duodenal atresia surgically treated during the neonatal period at our institution between January 1990 and March 2017. The neonates were categorized into two groups: those with and without cholestasis. Univariate and multivariate analyses were performed to identify the possible risk factors for cholestasis. RESULTS: Among the 76 neonates with duodenal atresia, 21 (27%) developed cholestasis. The duration of total parenteral nutrition was identified as a risk factor in univariate analysis; however, it was not an independent risk factor for cholestasis. Gestational age and highest C-reactive protein (CRP) values were independent risk factors, with adjusted odds ratios of 0.53 and 1.25, respectively. To predict the occurrence of cholestasis, the cutoff value for gestational age was 35.0 weeks, and highest CRP value was 2.4 mg/dL. CONCLUSIONS: The occurrence of cholestasis in patients with duodenal atresia was associated with preterm delivery and severity of the inflammatory response during the perioperative period.
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Colestasis/etiología , Obstrucción Duodenal/complicaciones , Atresia Intestinal/complicaciones , Medición de Riesgo/métodos , Colestasis/epidemiología , Femenino , Edad Gestacional , Humanos , Incidencia , Recién Nacido , Japón/epidemiología , Masculino , Nacimiento Prematuro , Estudios Retrospectivos , Factores de RiesgoRESUMEN
Nimesulide is a non-steroidal anti-inflammatory drug still marketed in many countries. We aim to analyze the clinical phenotype, outcome, and histological features of nimesulide-induced liver injury (nimesulide-DILI). We analyzed 57 cases recruited from the Spanish and Latin American DILI registries. Causality was assessed by the RUCAM scale. Mean age of the whole case series was 59 years (86% women) with a median time to onset of 40 days. A total of 46 patients (81%) were jaundiced. Nimesulide-DILI pattern was hepatocellular in 38 (67%), mixed in 12 (21%), and cholestatic in 7 (12%) cases. Transaminases were elevated with a mean of nearly 20-fold the upper limit of normality (ULN), while alkaline phosphatase showed a twofold mean elevation above ULN. Total bilirubin showed a mean elevation of 13-fold the ULN. Liver histology was obtained in 14 cases (25%), most of them with a hepatocellular pattern. Median time to recovery was 60 days. Overall, 12 patients (21%) developed acute liver failure (ALF), five (8.8%) died, three underwent liver transplantation (5.3%), and the remaining four resolved. Latency was ≤ 15 days in 12 patients (21%) and one patient developed ALF within 7 days from treatment initiation. Increased total bilirubin and aspartate transaminase levels were independently associated with the development of ALF. In summary, nimesulide-DILI affects mainly women and presents typically with a hepatocellular pattern. It is associated with ALF and death in a high proportion of patients. Shorter (≤ 15 days) duration of therapy does not prevent serious nimesulide hepatotoxicity, making its risk/benefit ratio clearly unfavorable.
Asunto(s)
Antiinflamatorios no Esteroideos/efectos adversos , Enfermedad Hepática Inducida por Sustancias y Drogas/etiología , Fallo Hepático Agudo/inducido químicamente , Sulfonamidas/efectos adversos , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Antiinflamatorios no Esteroideos/administración & dosificación , Bilirrubina/metabolismo , Enfermedad Hepática Inducida por Sustancias y Drogas/epidemiología , Enfermedad Hepática Inducida por Sustancias y Drogas/fisiopatología , Niño , Colestasis/inducido químicamente , Colestasis/epidemiología , Estudios de Cohortes , Femenino , Humanos , Ictericia/inducido químicamente , Ictericia/epidemiología , América Latina/epidemiología , Fallo Hepático Agudo/epidemiología , Masculino , Persona de Mediana Edad , Sistema de Registros , Factores de Riesgo , España/epidemiología , Sulfonamidas/administración & dosificación , Factores de Tiempo , Adulto JovenRESUMEN
BACKGROUND: Malignant biliary obstruction (MBO) is usually caused by biliary tract cancer or pancreatic cancer. This study was performed to summarize the current situation regarding palliative endoscopic retrograde biliary drainage (ERBD) for MBO in Korea and to determine its clinical significance by analyzing representative nationwide data. METHODS: Patients that underwent palliative ERBD for MBO between 2012 and 2015 were identified using the Health Insurance Review and Assessment database, which covers the entire Korean population. We assessed clinical characteristics and complications and compared the clinical impacts of initial metal and plastic stenting in these patients. RESULTS: A total of 9,728 subjects (mean age, 65 ± 11.4 years; male, 61.4%) were identified and analyzed. The most common diagnosis was malignant neoplasm of liver and intrahepatic bile ducts (32.1%) and this was followed by extrahepatic or Ampulla of Vater cancer and pancreatic cancer. Initial plastic stent(s) placement was performed in 52.9% of the study subjects, and metal stent(s) placement was performed in 23.3%. The number of sessions of endoscopic retrograde cholangiopancreatography (ERCP) or percutaneous transhepatic biliary drainage (PTBD) was significantly higher in patients that underwent initial plastic stenting than in patients that underwent metal stenting (2.2 ± 1.7 vs 1.8 ± 1.4, P < 0.0001), but rates of post-ERCP pancreatitis, hospital days, and time to second ERCP or PTBD were not significantly different. CONCLUSION: This nationwide assessment study suggests that initial metal stenting is associated with fewer sessions of total ERCP or PTBD following the initial procedure, despite the preference for initial plastic stenting in Korea.
Asunto(s)
Colestasis , Neoplasias Pancreáticas , Anciano , Colangiopancreatografia Retrógrada Endoscópica , Colestasis/epidemiología , Colestasis/etiología , Colestasis/cirugía , Drenaje , Humanos , Masculino , Persona de Mediana Edad , Neoplasias Pancreáticas/complicaciones , Neoplasias Pancreáticas/epidemiología , República de Corea/epidemiología , Estudios Retrospectivos , Stents , Resultado del TratamientoRESUMEN
Intralipid (Fresenius Kabi) was the most commonly used lipid emulsion in parenteral nutrition (PN), with a 100% soybean oil composition, a low vitamin E content, and a ω-6: ω-3 ratio of 7:1. A recent alternative formulation is SMOFlipid (Fresenius Kabi), with a ω-6: ω-3 ratio of 5:2 and higher vitamin E content. A retrospective observational study was conducted to determine neonatal morbidity in very low birth weight (VLBW) premature infants during two periods: P1, when PN was based exclusively on Intralipid, and P2, when only SMOFlipid was supplied. In total, 170 VLBW neonates were analyzed, of whom 103 received PN for more than 6 days, 56 during P1, and 47 during P2. In both periods, the antenatal and neonatal characteristics of the cohort were comparable. In this analysis, the prevalence of associated comorbidities was determined. During P2, there were fewer cases of moderate to severe bronchopulmonary dysplasia (BPD) and of cholestasis, but more cases of late sepsis, mainly Staphylococcus epidermidis. No changes in the prevalence of other neonatal comorbidities were observed. We believe that the SMOFlipid used in PN could discreetly improve the prevalence of cholestasis or BPD.
Asunto(s)
Emulsiones Grasas Intravenosas , Aceites de Pescado , Recién Nacido de muy Bajo Peso , Aceite de Oliva , Nutrición Parenteral , Fosfolípidos , Aceite de Soja , Triglicéridos , Displasia Broncopulmonar/epidemiología , Displasia Broncopulmonar/prevención & control , Colestasis/epidemiología , Colestasis/prevención & control , Emulsiones , Humanos , Incidencia , Recién Nacido , Recien Nacido Prematuro , Estudios Retrospectivos , Sepsis/epidemiología , Sepsis/microbiología , Staphylococcus epidermidisRESUMEN
BACKGROUND: Parenteral nutrition (PN) is crucial for the improvement of long-term outcomes in very low birth weight (VLBW) neonates. Lipid injectable emulsions are a key component of PN, as they contain essential fatty acids and provide energy critical for brain growth. Prolonged administration increases risk of intestinal failure-associated liver disease, including cholestasis, and other complications. METHODS: This is a retrospective, quasi-experimental cohort study of 215 VLBW neonates. The primary outcome was a change in direct bilirubin concentration. Secondary outcomes included change in total bilirubin concentration and incidences of cholestasis and other disease states associated with PN and prematurity. Cholestasis was defined as direct bilirubin ≥ 1.0 mg/dL with total bilirubin < 5.0 mg/dL or direct bilirubin > 20% of total bilirubin with total bilirubin > 5.0 mg/dL. RESULTS: Change in direct bilirubin concentration was not different between groups. Incidence of cholestasis was not different between groups per charted diagnosis or per study definition. Non-stage-0 retinopathy of prematurity, bronchopulmonary dysplasia, sepsis, and necrotizing enterocolitis were all lower in the mixed oil lipid emulsion group, which remained significant after adjustment for differences in gestational age, birth weight, and PN duration. CONCLUSIONS: Although mixed oil lipid emulsion was not found to be associated with a lower risk of cholestasis, it may decrease risks of other disease states associated with PN therapy.