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1.
Acta Gastroenterol Belg ; 84(3): 477-486, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34599573

RESUMO

Benign recurrent intrahepatic cholestasis (BRIC) is a rare genetic disorder that is characterized by episodes of cholestasis followed by complete resolution. The episodic nature of BRIC raises concerns about its possible trigger factors. Indeed, case reports of this orphan disease have associated BRIC to some triggers. In the absence of any reviews, we reviewed BRIC trigger factors and its pathophysiology. The study consisted of a systematic search for case reports using PubMed. Articles describing a clear case of BRIC associated with a trigger were included resulting in 22 articles that describe 35 patients. Infection was responsible for 54.3% of triggered episodes, followed by hormonal, drugs, and miscellaneous causes reporting as 30%, 10%, and 5.7% respectively. Females predominated with 62.9%. The longest episode ranged between 3 months to 2 years with a mean of 32.37 weeks. The mean age of the first episode was 14.28 ranging between 3 months to 48 years. Winter and autumn were the major seasons during which episodes happened. Hence, BRIC is potentially triggered by infection, which is most commonly a viral infection, hormonal disturbances as seen in oral contraceptive pills and pregnancy state, and less commonly by certain drugs and other causes. The appearance of cholestasis during the first two trimesters of pregnancy compared to intrahepatic cholestasis of pregnancy could help to differentiate between the two conditions. The possible mechanism of BRIC induction implicates a role of BSEP and ATP8B1. While estrogen, drugs, and cytokines are known to affect BSEP, less is known about their action on ATP8B1.


Assuntos
Colestase Intra-Hepática , Colestase , Colestase Intra-Hepática/diagnóstico , Feminino , Humanos , Lactente , Gravidez
2.
Mymensingh Med J ; 30(4): 1079-1085, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34605480

RESUMO

Complete assessment of obstructive jaundice requires the use of various imaging modalities that are required to detect the cause and level of obstruction thus helping in treatment planning. Magnetic Resonance Cholangio Pancreatography (MRCP) is a current available technology which is a non-invasive technique that visualizes the gallbladder, biliary tree and pancreatic duct and also provides information about surrounding structures. This study was done to correlate the MRCP findings with post-operative result and thereby demonstrate the specificity, sensitivity and efficacy of MRCP as an accurate investigatory tool for biliary obstruction. Total of fifty (50) patients of clinically diagnosed obstructive jaundice were studied from March 2017 to August 2017 in the Department of Radiology and Imaging, Combined Military Hospital, Dhaka, Bangladesh. In all these cases, USG was the initial screening investigation followed by MRCP. Cause and level of obstruction were evaluated using MRCP findings. MRCP results were correlated with surgical findings and few cases also with direct ERCP findings. Statistical analysis was done to see the sensitivity, specificity, accuracy, positive and negative predictive values of MRCP in diagnosis of biliary obstruction. In this study, USG detected level of obstruction in 56% (28 out of 50) cases. USG could detect causes of obstruction in 100% (02 out of 02) cases of choledocal cyst and 66.67% (02 out of 03) benign stricture, 60% (03 out of 05) cases of periampullary carcinoma, 57.70% (06 out of 14) cases of choledocholithiasis, and 42.86% (15 out of 26) cases of cholangiocarcinoma. On the other hand, MRCP detected level of obstruction in 98% (49 out of 50) cases. MRCP could detect causes of obstruction in 100% cases of cholangiocarcinoma, choledocholithiasis, benign stricture and choledocal cyst and 80% (04 out of 05) cases of periampullary carcinoma. In this study, ERCP could detect causes of obstruction in 32 cases of choledocholithiasis and benign stricture, but in case of cholangiocarcinoma ERCP was failed in 3 cases. In this study, for detection of cause of obstruction, ERCP had the highest sensitivity (97.79%); followed by MRCP (96.65%) and USG (60.25%). The overall diagnostic accuracy for detection of cause of obstruction was the highest for ERCP (95.50%); followed by MRCP (94.50%) and USG (64.50%). MRCP can be done in a short duration and is a noninvasive diagnostic modality compared to ERCP. MRCP needs to be advocated as a viable and non-invasive alternative with compararable sensitivity and specificity to ERCP.


Assuntos
Neoplasias dos Ductos Biliares , Colangiocarcinoma , Colestase , Bangladesh , Ductos Biliares Intra-Hepáticos , Colangiopancreatografia por Ressonância Magnética , Colestase/diagnóstico por imagem , Colestase/etiologia , Humanos
3.
Z Gastroenterol ; 59(9): 933-943, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34507372

RESUMO

BACKGROUND AND STUDY AIM: Endoscopic retrograde cholangiopancreatography (ERCP) is the gold standard for the treatment of biliary obstruction of any etiology. However, cannulation failure of the common bile duct (CBD) by ERCP occurs in 5-10%. Alternatives after a failed ERCP are re-ERCP by an expert endoscopist, percutaneous transhepatic cholangio drainage (PTCD), (balloon) enteroscopy-assisted ERCP, or surgery. Endoscopic ultrasonography-guided drainage of the bile ducts (EUS-BD) is becoming the standard of care in tertiary referral centers for cases of failed ERCP in patients with malignant obstruction of the CBD. In expert hands, EUS-guided biliary drainage has excellent technical/clinical success rates and lower complication rates compared to PTCD. Despite the successful performance of EUS-BD in malignant cases, its use in benign cases is limited. The aim of this study (design, systematic prospective clinical observational study on quality assurance in daily clinical practice) was to evaluate the efficacy and safety of EUS-BD in benign indications. PATIENTS AND METHODS: Patients with cholestasis and failed ERCP were recruited from a prospective EUS-BD registry (2004-2020). One hundred and three patients with EUS-BD and benign cholestasis were extracted from the registry (nTotal = 474). Indications of EUS-BDs included surgically altered anatomy (n = 65), atypical bile duct percutaneous transhepatic cholangio orifice at the duodenal junction from the longitudinal to the horizontal segment (n = 1), papilla of Vater not reached due to the gastric outlet/duodenal stenoses (n = 6), papilla that cannot be catheterized (n = 24), and proximal bile duct stenosis (n = 7). The primary endpoint was technical and clinical success. Secondary endpoints were procedure-related complications during the hospital stay. RESULTS: 103 patients with EUS-BD and benign cholestasis were extracted from the registry (nTotal=474). Different transluminal access routes were used to reach the bile ducts: transgastric (n = 72/103); -duodenal (n = 16/103); -jejunal (n = 14/103); combined -duodenal and -gastric (n = 1/103). The technical success rate was 96 % (n = 99) for cholangiography. Drainage was not required in 2 patients; balloon dilatation including stone extraction was sufficient in 17 cases (16.5 %; no additional or prophylactic insertion of a drain). Transluminal drainage was achieved in n = 68/103 (66 %; even higher in patients with drain indication only) by placement of a plastic stent (n = 29), conventional biliary metal stents (n = 24), HotAXIOS stents (n = 5; Boston Scientific, Ratingen, Germany), Hanaro stents (n = 6; Olympus, Hamburg, Germany), HotAXIOS stents and plastic stents (n = 1), HotAXIOS stents and metal stents (n = 1) and metal stents and plastic stents (n = 2). Techniques for stone extraction alone (nSuccessful=17) or stent insertion (nTotal = 85; nSuccessful=85 - rate, 100 %) and final EUS-BD access pathway included: Rendezvous technique (n = 14/85; 16.5 %), antegrade internal drainage (n = 20/85; 23.5 %), choledochointestinostomy (n = 7/85; 8.2 %), antegrade internal and hepaticointestinostomy (n = 22/85; 25.9 %), hepaticointestinostomy (n = 21/85; 24.7 %), choledochointestinostomy and hepaticointestinostomy (n = 1/85; 1.2 %).The complication rate was 25 % (n = 26) - the spectrum comprised stent dislocation (n = 11), perforation (n = 1), pain (n = 2), hemorrhage (n = 6), biliary ascites/leakage (n = 3) and bilioma/liver abscess (n = 3; major complication rate, n = 12/68 - 17.6 %). Re-interventions were required in 19 patients (24 interventions in total). DISCUSSION: EUS-BD can be considered an elegant and safe alternative to PTCD or reoperation for failed ERCP to achieve the necessary drainage of the biliary system even in underlying benign diseases. An interventional EUS-based internal procedure can resolve cholestasis, avoid PTCD or reoperation, and thus improve quality of life. Due to the often complex (pathological and/or postoperative) anatomy, EUS-BD should only be performed in centers with interventional endoscopy/EUS experience including adequate abdominal surgery and interventional radiology expertise in the background. This enables adequately adapted therapeutic management in the event of challenging complications. It seems appropriate to conduct further studies with larger numbers of cases to systematize the approach and peri-interventional management and to successively develop specific equipment.


Assuntos
Colangiopancreatografia Retrógrada Endoscópica , Colestase , Colestase/diagnóstico por imagem , Colestase/cirurgia , Ducto Colédoco , Drenagem , Endossonografia , Estudos de Viabilidade , Humanos , Estudos Prospectivos , Qualidade de Vida , Stents , Resultado do Tratamento , Ultrassonografia de Intervenção
4.
Ann Palliat Med ; 10(8): 9267-9275, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-34488412

RESUMO

In recent years, immune checkpoint inhibitors (ICIs) have become a standard treatment for patients with advanced lung cancers. With the widespread use of immunotherapy in clinical practice, immune-related adverse events (irAEs) have become increasingly common. This case report details a 72-year-old man with small-cell lung cancer (SCLC) who developed pneumonitis, appendicitis, and biliary obstruction during treatment with toripalimab. The patient was initially diagnosed with limited-stage SCLC in January 2019 and completed 5 sequential cycles of etoposide/cisplatin (EP) and radiotherapy (60 Gy/30 F). The overall response was complete response (CR) after first line treatment. He developed radiation pneumonitis after completion of radiotherapy, which responded well to symptomatic treatment. Due to newly diagnosed bone metastasis, he was administered toripalimab intravenously every 3 weeks and 12 mg anlotinib orally once a day from January 2020. By the third cycle, the patient presented with electrocardiographic abnormalities, gingival swelling and pain, and hoarseness, and consequently, the anlotinib was suspended. After 4 cycles, he developed suppurative appendicitis, which was managed successfully with anti-inflammatory agents. He then presented with shortness of breath on exertion and after a comprehensive examination, he was diagnosed with ICI-related-pneumonitis. After 6 weeks of treatment with methylprednisolone, the shortness of breath was mostly relieved and treatment continued. In June 2020, the patient developed severe vomiting. Computed tomography (CT) indicated biliary obstruction, and at endoscopic retrograde cholangiopancreatography (ERCP) there was edema of the major papilla of the duodenum. The patient's symptoms were relieved after treatment with gastric acid suppression and antiemetics. Re-examination by magnetic resonance imaging (MRI) showed that the biliary obstruction had been resolved. Although the disease progressed after immunotherapy, no tumor tissue related to the biliary obstruction was detected, and this was therefore classified as an irAE.


Assuntos
Apendicite , Colestase , Neoplasias Pulmonares , Pneumonia , Idoso , Anticorpos Monoclonais Humanizados , Humanos , Neoplasias Pulmonares/tratamento farmacológico , Masculino , Pneumonia/induzido quimicamente , Pneumonia/tratamento farmacológico
5.
Am J Case Rep ; 22: e932769, 2021 Sep 17.
Artigo em Inglês | MEDLINE | ID: mdl-34531360

RESUMO

BACKGROUND ARCS1 is an acronym for arthrogryposis, renal dysfunction, and cholestasis. It is a congenital malfunction with autosomal recessive inheritance, and, unfortunately, its prognosis is still poor. It is believed that VPS33B is altered in 75% of cases and that the VIPAR gene is altered in approximately 25% of them. CASE REPORT An affected child was born from the first pregnancy of 26-year-old mother and a 30-year-old father with no previous medical history and no genetic conditions. The first clinical symptoms were observed at the end of the child's second week of life. The mother reported the child has decreasing body weight and loss of appetite. After admission to the ward, the child was apathetic and sleepy. Symptoms of conjunctivitis, pale and dry skin, and mild face and mild body dysmorphia were observed. CONCLUSIONS Laboratory tests revealed proteinuria of up to 1.36 g/l and glycosuria of up to 28 mmol/l, as well as fluctuating metabolic acidosis. The bilirubin level reached 6.62 mg/dl, along with alkaline phosphatase at 470 U/l. Moreover, hypothyroidism with TSH at 16.71 uU/ml was observed. Because of the co-occurrence of cholestasis and renal dysfunction, molecular testing was done. The 17th exon of VPS33B was sequenced by Sanger DNA sequencing method. To the best of our knowledge, this is the first report of homozygotic mutation c.1235_1236delinsG (p.Pro412ArgfsTer7) in the VPS33B gene. The risk of transfer of the mutation to future descendants was calculated as 25%. Due to the wide landscape of molecular alternation in the 17th exon of the VPS33B gene, we propose using Sanger whole-exon sequencing as a first-choice diagnostic test.


Assuntos
Artrogripose , Colestase , Insuficiência Renal , Adulto , Criança , Humanos , Mutação , Proteínas de Transporte Vesicular/genética
6.
BMJ Case Rep ; 14(9)2021 Sep 07.
Artigo em Inglês | MEDLINE | ID: mdl-34493554

RESUMO

We report the case of a 29-year-old man who presented with progressive weight loss, night sweats, abdominal pain and pruritus who was found to have obstructive jaundice and cholestatic pattern of liver injury on laboratory workup. Though findings on magnetic resonance cholangiopancreatography were initially concerning primary sclerosing cholangitis, he was ultimately diagnosed with biliary sarcoidosis after a liver biopsy. This case brings attention to the rare phenomenon of hepatic sarcoidosis causing hyperbilirubinemia and highlights the importance of reaching the correct diagnosis early, as the patient's symptoms improved after initiation of steroids.


Assuntos
Colangite Esclerosante , Colestase , Icterícia Obstrutiva , Sarcoidose , Adulto , Colangiopancreatografia por Ressonância Magnética , Colangite Esclerosante/diagnóstico , Colangite Esclerosante/tratamento farmacológico , Humanos , Masculino , Sarcoidose/diagnóstico , Sarcoidose/tratamento farmacológico
7.
BMJ Case Rep ; 14(9)2021 Sep 14.
Artigo em Inglês | MEDLINE | ID: mdl-34521738

RESUMO

Neonatal hyperbilirubinaemia is a very common entity witnessed in most of the newborns. Rarely are there events where the bilirubin levels reach extreme values mandating invasive therapy. Unconjugated hyperbilirubinaemia when solely present is easy to manage and diagnose the common aetiological factors associated with it. The issue arises when we come across a mixed picture of conjugated with unconjugated hyperbilirubinaemia and puts us in a dilemma as to what are we treating. Our case highlights a similar picture where we witnessed the highest documented levels of total bilirubin but to our surprise the major component of which was direct bilirubin. This report takes us through the differentials which were ruled out and our management strategies for solving this rare mystery.


Assuntos
Colestase , Hiperbilirrubinemia Neonatal , Icterícia Neonatal , Bilirrubina , Colestase/diagnóstico , Colestase/etiologia , Hemólise , Humanos , Hiperbilirrubinemia Neonatal/diagnóstico , Hiperbilirrubinemia Neonatal/etiologia , Recém-Nascido
8.
BMC Pediatr ; 21(1): 345, 2021 08 16.
Artigo em Inglês | MEDLINE | ID: mdl-34399711

RESUMO

BACKGROUND: Drug-induced aseptic meningitis is a rare, but challenging diagnosis, most commonly reported with nonsteoroidal anti-inflammatory drugs (NSAIDs) and antibiotics. Trimethoprim/sulfamethoxazole (TMP/SMX) is a sulfonamide that is widely used in clinical practice for the treatment and prophylaxis of various infections. The most common side effects associated with TMP/SMX are generally mild and self-limited, but serious side effects have been reported, including liver injury and aseptic meningitis. CASE PRESENTATION: We report a 2,5 year old Dutch girl with both drug-induced aseptic meningitis and drug-induced liver injury while using TMP/SMX prophylaxis. Ursodeoxycholic acid was started because of cholestatic injury. After cessation of TMP/SMX, full convalescence was reached within weeks. CONCLUSIONS: This is the first report of a young patient with both aseptic meningitis and drug-induced liver injury caused by TMP/SMX. Drug-induced aseptic meningitis and cholestatic hepatitis constitute a considerable diagnostic challenge to clinicians. In addition to a thorough evaluation for infectious causes, clinicians should be aware of drug-induced aseptic meningitis and cholestatic hepatitis.


Assuntos
Anti-Infecciosos , Colestase , Hepatite , Meningite Asséptica , Pré-Escolar , Colestase/induzido quimicamente , Colestase/diagnóstico , Feminino , Hepatite/diagnóstico , Hepatite/etiologia , Humanos , Meningite Asséptica/induzido quimicamente , Meningite Asséptica/diagnóstico , Combinação Trimetoprima e Sulfametoxazol/efeitos adversos
10.
Biomed Res Int ; 2021: 9945149, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34368363

RESUMO

Picroside II is an important ingredient agent in Traditional Chinese medicine and hoped to reduce hepatocellular injury caused by severe acute pancreatitis (SAP). An SAP-induced hepatocellular injury model was established in rats by using pentobarbital sodium. 27 rats were divided into 3 groups: the sham group (SG), model group (MG), and Picroside groups (PG). SAP-induced hepatocellular injury was assessed using hematoxylin and eosin staining. We measured hepatocellular enzymes (amylase (AMY), alanine aminotransferase (ALT), and aspartate aminotransferase (AST)), oxidative stress factors (superoxidase dismutase (SOD) and malondialdehyde (MDA)), and inflammatory factors (tumor necrosis factor α (TNF-α), interleukin- (IL-) 6, and IL-10), apoptotic factors (BAX and cleaved caspase 3), and inflammatory signaling (Janus kinase 2 (JAK2)/signal transducer and activator of transcription 3 (STAT3), p-JAK2, and p-STAT3) in hepatocellular tissues. The SAP-induced hepatocellular injury model was successfully established. Picroside II treatment repaired hepatocellular injury by reducing the activities of AMY, ALT, and AST; reducing the levels of MDA, TNF-α, IL-1, IL-6, p-JAK2, p-STAT3, BAX, and cleaved caspase 3; and increasing the levels of SOD and IL-10. Picroside II exerted protective function for the SAP-induced hepatocellular injury model. Picroside II improved SAP-induced hepatocellular injury and antioxidant and anti-inflammatory properties by affecting JAK2/STAT3 phosphorylation signaling.


Assuntos
Cinamatos/farmacologia , Hepatócitos/patologia , Glucosídeos Iridoides/farmacologia , Janus Quinase 2/metabolismo , Pancreatite/patologia , Fator de Transcrição STAT3/metabolismo , Transdução de Sinais , Doença Aguda , Animais , Anti-Inflamatórios/farmacologia , Antioxidantes/metabolismo , Apoptose/efeitos dos fármacos , Ácidos e Sais Biliares/sangue , Colestase/patologia , Cinamatos/química , Citocinas/metabolismo , Hepatócitos/efeitos dos fármacos , Glucosídeos Iridoides/química , Fígado/enzimologia , Fígado/patologia , Masculino , Modelos Biológicos , Pancreatite/sangue , Fosforilação/efeitos dos fármacos , RNA Mensageiro/genética , RNA Mensageiro/metabolismo , Ratos Sprague-Dawley
12.
Medicina (Kaunas) ; 57(8)2021 Jul 28.
Artigo em Inglês | MEDLINE | ID: mdl-34440971

RESUMO

Background: Malignant gastric outlet obstruction (MGOD) is an extremely rare expression of advanced extra-gastrointestinal cancer, such as squamous cell carcinoma (SCC) of the cervix, and only sixcases are described in the literature.Because of the short life expectancyand the high surgical risk involving these patients, less invasive approaches have been developed over time, such asthe use of an enteral stent or less invasive surgical techniques (i.e., laparoscopic gastrojejunostomy). However, MGOD could make it difficult to perform an endoscopic retrograde cholangio-pancreatography (ERCP) for standard endoscopic drainage, so in this case a combined endoscopic-percutaneous technique may be performed. This article, therefore, aims to highlight the presence in the doctor's armamentarium of the "rendezvous technique", few case reports of whichare described in the literature, and, moreover, this article aims to underline the technique'sfeasibility. Case Presentation: The case is that of a 38-year-old woman who presented with MGOD three years after the diagnosis of SCC of the cervix, who successfully underwent the rendezvous technique with the resolution of duodenal obstruction. Endoscopic enteral stenting treatment with the placement of a metal stent (SEMSs) represents the mainstay of MGOD treatment compared withsurgery due to its lower morbidity, mortality, shorter hospitalization and earlier symptom relief. However, in patients with both duodenal and biliary obstruction, a combined endoscopic-percutaneous approach may be necessary because of the difficulty in passing the duodenal stricture or in accessing the papilla through the mesh of the duodenal SEMS. Conclusion: The rendezvous procedure is a technicallyfeasible and minimally invasive approach to the double stenting of biliary and duodenal strictures. It achieves the desired therapeutic result while avoiding the need to perform more invasive procedures that could have a negative impact on the patient'sprognosis.


Assuntos
Colestase , Obstrução Duodenal , Obstrução da Saída Gástrica , Adulto , Colo do Útero , Obstrução Duodenal/etiologia , Obstrução Duodenal/cirurgia , Feminino , Obstrução da Saída Gástrica/etiologia , Obstrução da Saída Gástrica/cirurgia , Humanos , Estudos Retrospectivos , Stents , Resultado do Tratamento
13.
Ned Tijdschr Geneeskd ; 1652021 07 26.
Artigo em Holandês | MEDLINE | ID: mdl-34346618

RESUMO

BACKGROUND: Infants who are born in The Netherlands receive oral vitamin K to prevent bleeding due to a vitamin K deficiency. However the incidence of such bleedings are higher compared to other European countries. Therefore, the Dutch Health Council advised in 2017 to change this guideline from oral to intramuscular administration. CASE DESCRIPTION: A 2 months old girl presented with a fatal intracranial hemorrhage. A day before she developed a hematoma on her foot and orbit. Despite daily oral vitamin K, blood results revealed a severe vitamin K deficiency-related bleeding. Postmortem liver biopsy and genetic studies showed cholestasis as the most likely cause of malabsorption of fat soluble vitamins due to a heterozygous pathogenic variant in the ABCB11 gene, which could possibly be transient. CONCLUSION: Our case illustrates the importance of revising the national guideline for vitamin K prophylaxis to intramuscular administration, according to the recommendation of the Dutch Health Council.


Assuntos
Colestase , Sangramento por Deficiência de Vitamina K , Feminino , Hemorragia , Humanos , Lactente , Recém-Nascido , Hemorragias Intracranianas , Vitamina K , Sangramento por Deficiência de Vitamina K/tratamento farmacológico , Sangramento por Deficiência de Vitamina K/prevenção & controle
14.
World J Gastroenterol ; 27(28): 4697-4709, 2021 Jul 28.
Artigo em Inglês | MEDLINE | ID: mdl-34366630

RESUMO

BACKGROUND: There is little data available on the role of new anti-reflux plastic stents (ARPSs). AIM: To compare the use of ARPSs with that of traditional plastic stents (TPSs) for patients with biliary strictures. METHODS: Consecutive patients with biliary strictures who underwent first endoscopic biliary stenting between February 2016 and May 2019 were included. The onset of stent-related cholangitis, stent patency, clinical success, and other adverse events were evaluated. RESULTS: Sixty-seven patients in the ARPS group and 66 patients in the TPS group were included in the final analyses. Fewer patients experienced stent-related cholangitis in the ARPS group than that in the TPS group (8 patients vs 18 patients; P = 0.030). The median time till the onset of first stent-related cholangitis was later in the ARPS group than that in the TPS group (128.5 d vs 76 d; P = 0.039). The cumulative median stent patency in the ARPS group was 185 d, which was significantly longer than that in the TPS group (133 d; P = 0.001). The clinical success rates and other adverse events did not significantly differ between both groups. CONCLUSION: Placement of new ARPS might be a safe and effective optional therapeutic strategy to reduce the risk of stent-related cholangitis and prolong stent patency.


Assuntos
Colangite , Colestase , Colangiopancreatografia Retrógrada Endoscópica/efeitos adversos , Colangite/etiologia , Colangite/prevenção & controle , Colestase/etiologia , Constrição Patológica , Humanos , Plásticos , Estudos Retrospectivos , Stents , Resultado do Tratamento
15.
Nutrients ; 13(8)2021 Aug 13.
Artigo em Inglês | MEDLINE | ID: mdl-34444944

RESUMO

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.


Assuntos
Colestase/epidemiologia , Hepatopatias/epidemiologia , Desnutrição/epidemiologia , Estado Nutricional , Adolescente , Criança , Pré-Escolar , Colestase/complicações , Doença Crônica , Dieta/métodos , Ingestão de Energia , Feminino , Humanos , Lactente , Fórmulas Infantis , Hepatopatias/complicações , Falência Hepática/epidemiologia , Falência Hepática/etiologia , Transplante de Fígado , Masculino , Desnutrição/etiologia , Necessidades Nutricionais , Qualidade de Vida , Vitaminas/administração & dosagem
16.
Afr J Paediatr Surg ; 18(3): 168-170, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34341203

RESUMO

Context: Choledochal cyst is a rare malformation that mainly affects girls. Aims: The aim of this work is to report the diagnostic, therapeutic and evolutionary aspects of bile duct cyst in children in Dakar. Subjects and Methods: we conducted a prospective monocentre descriptive study from 1 July, 2016, to 30 June, 2019, in the Pediatric Surgery Department of Albert Royer Children's Hospital in Dakar. The studied parameters featured clinical, biological, radiological, therapeutic and evolutionary data. Overall, ten patients, including eight girls and two boys, were selected. Results: Two patients presented the classic triple-syndrome complex featuring an abdominal pain, an abdominal mass and jaundice. Most of the patients presented a symptomatology associating abdominal pain and vomiting. Biological cholestasis syndrome was present in six cases and biological cytolysis syndrome in five cases. The abdominal ultrasound-computed tomography (CT) scan confirmed the diagnosis and helped to set the Todani classification with a predominance of Types 1 and 4. Surgical treatment by laparotomy consisted of total excision of the cyst followed by a Y-en-Roux hepatico-jejunal anastomosis in nine cases, whereas we performed a drainage of the cyst in one case. Two patients presented a morbidity of anastomotic suture release and evisceration and had a good outcome after surgical repair. There was no mortality. Conclusions: The diagnosis of choledochal cyst dilatation (CCD) can be suspected in the postnatal period on the basis of a gastro-intestinal symptomatology with or without associated to jaundice. The combination ultrasound-abdominal CT-scan helps set the diagnosis. The laparatomic approach always has its place for the management of CCD, especially in our context.


Assuntos
Cisto do Colédoco , Colestase , Criança , Cisto do Colédoco/diagnóstico , Cisto do Colédoco/cirurgia , Feminino , Humanos , Masculino , Estudos Prospectivos , Senegal , Ultrassonografia
17.
Nutrients ; 13(8)2021 Jul 29.
Artigo em Inglês | MEDLINE | ID: mdl-34444777

RESUMO

Ursodeoxycholic acid (UDCA) is the first line therapy for the treatment of cholestatic and autoimmune liver diseases. Its clinical use is currently limited by a significant proportion of non-responder patients. Polyunsaturated fatty acids (n-3 PUFAs) possess important anti-inflammatory properties and protect liver cells against bile acid (BA)-induced toxicity. The present study was designed to rapidly evaluate whether combining n-3 PUFAs (i.e., eicosapentaenoic [EPA] and docosahexaenoic [DHA] acids) to UDCA would provide additional benefits when compared to the drug alone. The parameters evaluated were (i) the expression of genes governing BA synthesis, transport, and metabolism; (ii) the prevention of BA-induced apoptosis and endoplasmic reticulum (ER)-stress; and (iii) the control of BA- and LPS-dependent inflammation. In the absence of n-3 PUFAs, most of the parameters investigated were unaffected by UDCA or were only altered by the higher dose (500 µM) of the drug. By contrast, in the presence of EPA/DHA (50/50 µM), all parameters showed a strongly improved response and the lowest UDCA dosage (50 µM) provided equal or better benefits than the highest dose used alone. For example, the combination EPA/DHA + UDCA 50 µM caused comparable down-regulation of the CYP7A1 gene expression and of the BA-induced caspase 3 activity as observed with UDCA 500 µM. In conclusion, these results suggest that the addition of n-3 PUFAs to UDCA may improve the response to the drug, and that such a pharmaco-nutraceutical approach could be used in clinic to open the narrow therapeutic dose of UDCA in cholestatic liver diseases.


Assuntos
Suplementos Nutricionais , Ácidos Graxos Ômega-3/metabolismo , Ácidos Graxos Ômega-3/farmacologia , Ácido Ursodesoxicólico/farmacologia , Apoptose/efeitos dos fármacos , Doenças Autoimunes , Ácidos e Sais Biliares/metabolismo , Ácidos e Sais Biliares/toxicidade , Carcinoma Hepatocelular , Caspase 3 , Colangite Esclerosante , Colestanotriol 26-Mono-Oxigenase/genética , Colestase , Colesterol 7-alfa-Hidroxilase/genética , Regulação para Baixo/efeitos dos fármacos , Quimioterapia Combinada , Estresse do Retículo Endoplasmático/efeitos dos fármacos , Expressão Gênica/efeitos dos fármacos , Células Hep G2 , Humanos , Inflamação , Fígado/metabolismo , Cirrose Hepática Biliar , Hepatopatias , Células THP-1
18.
Phytomedicine ; 91: 153683, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34364160

RESUMO

BACKGROUND: Rabdosia Serra, the dried aerial parts of Rabdosia serra (Maxim.) Hara (RS) from the Labiatae family, is a traditional Chinese herbal medicine called Xihuangcao. Although RS has been found to exert a therapeutic effect on cholestasis, the underlying molecular mechanism remains unclear. PURPOSE: This study was designed to investigate the pharmacological effect and mechanism of RS on cholestatic rats using metabolomics platform. METHODS: Histopathology and biochemical evaluations were performed to determine the therapeutic effect of RS and developed a rapid metabolite detection technology method based on UPLC-MS/MS to perform metabolomics research. Further, quantitative real-time polymerase chain reaction (RT-qPCR) was used to study the effect of RS on the bile acid metabolism pathway at the transcriptional level. RESULTS: RS significantly reduced the bile flow rates in cholestatic rats and decreased the levels of ALT, AST, TBA, T-BIL, and LDH, which were increased in the model group. Histological analysis showed that RS alleviated the liver injury induced by ANIT. Serum metabolomics results revealed 33 of the 37 biomarkers were found to be significantly altered by ANIT, and 26 were considerably changed following treatment with RS. Metabolic pathway analysis revealed four pathways such as primary bile acid biosynthesis, biosynthesis of unsaturated fatty acids, and arachidonic acid and tryptophan metabolism. The bile acid secretion process and the inflammation and oxidative stress processes are the major biochemical reactions following treatment with ANIT and RS. Bile acid-targeted metabolomics study showed that TCA, GCA, GCDCA, and GDCA might be sensitive biomarkers that induced liver injury. we found that treatment with RS regulated the levels of bile acid in the serum and liver and restored the proportion of bile acids, especially CA and conjugated bile acids, such as TCA and GCA, in the bile duct. RS increased the mRNA expression levels of FXR, SHP, BSEP, and MRP2 in livers, and IBABP, OST-α, and OST-ß in the ileum. CONCLUSION: In this study, RS was found to protect the liver by regulating multiple metabolic pathways and promoting the excretion of bile acids. Simultaneously, RS played an essential role in reversing the imbalance of bile acids and protected against cholestasis by regulating the expression of transporters associated with bile acids. We demonstrated the correlation between molecular mechanisms and metabolites, provide a reference for the fabrication of extracts that can be used to treat cholestasis.


Assuntos
Colestase , Medicamentos de Ervas Chinesas/farmacologia , Isodon , Metabolômica , 1-Naftilisotiocianato , Animais , Ácidos e Sais Biliares , Colestase/induzido quimicamente , Colestase/tratamento farmacológico , Cromatografia Líquida de Alta Pressão , Isodon/química , Fígado/efeitos dos fármacos , Ratos , Espectrometria de Massas em Tandem
19.
Clin J Gastroenterol ; 14(5): 1503-1510, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34228348

RESUMO

Doxycycline-induced liver injury is a rare phenomenon, with an unclear clinical course and etiopathogenesis. The onset of injury may be acute-to-subacute, with a pattern ranging from hepatocellular or cholestatic to mixed, and it often lasts up to several weeks. We present a case of cholestatic liver injury secondary to doxycycline use in a middle-aged woman. In patients with a history of doxycycline exposure and subsequent hepatic injury, an adverse drug reaction due to doxycycline should remain on the differential, and immediate removal of the offending agent with close monitoring of the clinical condition should be pursued.


Assuntos
Doença Hepática Induzida por Substâncias e Drogas , Colestase , Doença Hepática Induzida por Substâncias e Drogas/diagnóstico , Doença Hepática Induzida por Substâncias e Drogas/etiologia , Colestase/induzido quimicamente , Doxiciclina/efeitos adversos , Feminino , Humanos , Fígado , Pessoa de Meia-Idade
20.
ANZ J Surg ; 91(9): 1841-1846, 2021 09.
Artigo em Inglês | MEDLINE | ID: mdl-34309143

RESUMO

BACKGROUNDS: To compare the complication rates and overall costs of self-expandable metal stents (SEMS) and plastic stents (PS) in clinically indicated preoperative biliary drainage (PBD) prior to a pancreatoduodenectomy (PD). METHODS: We conducted an Australian multicentre retrospective cohort study using the databases of four tertiary hospitals. Adult patients who underwent clinically indicated endoscopic PBD prior to PD from 2010 to 2019 were included. Rates of complications attributable to PBD, surgical complications and pre-operative endoscopic re-intervention were calculated. Costing data were retrieved from our Financial department. RESULTS: Among the 157 included patients (mean age 66.6 ± 9.8 years, 45.2% male), 49 (31.2%) received SEMS and 108 received PS (68.8%). Baseline bilirubin was 187.5 ± 122.6 µmol/L. Resection histopathology showed mainly adenocarcinoma (93.0%). Overall SEMS was associated less complications (12.2% vs. 28.7%, p = 0.02) and a lower pre-operative endoscopic re-intervention rate (4.3 vs. 20.8%, p = 0.03) compared with PS. There was no difference in post-PD complication rates. On multivariate logistic regression analysis, stent type was an independent risk factor of PBD complication (OR of SEMS compared to PS 0.24, 95% CI 0.07-0.79, p = 0.02) but not for any secondary outcome measures. Upfront material costs were $56USD for PS and $1991USD for SEMS. Accounting for rates of complications, average costs were similar ($3110USD for PS and $3026USD for SEMS). CONCLUSION: In resectable pancreaticobiliary tumours, SEMS for PBD was associated with reduced risk of overall PBD-related complications and pre-surgical endoscopic reintervention rates and was comparable to PS in terms of overall cost.


Assuntos
Adenocarcinoma , Colestase , Neoplasias Pancreáticas , Adulto , Idoso , Austrália/epidemiologia , Colangiopancreatografia Retrógrada Endoscópica , Colestase/etiologia , Colestase/cirurgia , Análise Custo-Benefício , Drenagem , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Pancreáticas/complicações , Neoplasias Pancreáticas/cirurgia , Plásticos , Estudos Retrospectivos , Stents , Resultado do Tratamento
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