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Säo Paulo med. j ; 139(4): 351-363, Jul.-Aug. 2021. tab, graf
Article in English | LILACS | ID: biblio-1290251


ABSTRACT BACKGROUND: The role of transient obstructive cholestasis on liver histology remains undetermined. OBJECTIVE: To investigate whether transient cholestasis impairs liver histology. DESIGN AND SETTING: Cross-sectional study at a public university hospital (UNICAMP), Brazil. METHODS: 169 individuals undergoing cholecystectomy, with or without cholestasis. were enrolled. Histopathological findings were correlated with clinical and biochemical characteristics. RESULTS: Biliary hepatopathy was more frequent in individuals with resolved cholestasis than in those with active obstruction or no jaundice (P < 0.01), as also were fibrosis and ductular proliferation (P = 0.02). Cholestasis was commoner in individuals with resolved obstruction than in those with no history (P < 0.01) or active cholestasis (P < 0.05). Biliary hepatopathy was associated with longer duration of cholestasis (P < 0.001) and higher bilirubin levels (P = 0.02) in individuals with active obstruction; with lower body mass index (P = 0.02) and longer cholestasis (P < 0.001) in individuals with resolved obstruction; and with longer cholestasis (P < 0.001) and longer interval between endoscopic retrograde cholangiopancreatography and surgery (P = 0.03) overall. In individuals with active obstruction, duration of cholestasis (R = 0.7; P < 0.001) and bilirubin levels (R = 0.6; P = 0.004) were independently correlated with cholestasis severity. Duration of cholestasis (R = 0.7; P < 0.001) was independently correlated with ductular proliferation severity. CONCLUSIONS: Transient cholestasis was associated with significant histopathological changes, even after its resolution. Longer duration of obstruction correlated with greater severity of histopathological changes, especially cholestasis and ductular proliferation. This emphasizes the need for early treatment of obstructive cholestasis.

Humans , Cholestasis/etiology , Liver , Brazil/epidemiology , Cross-Sectional Studies , Cholangiopancreatography, Endoscopic Retrograde
Article in English | WPRIM | ID: wpr-880621


OBJECTIVES@#A variety of causes can lead to cholestasis, however, cholestasis caused by Graves' disease is usually overlooked clinically. Here we analyze the clinical characteristics of Graves' disease associated cholestasis so as to have a better understanding for the disease.@*METHODS@#We retrospectively collected 13 inpatients' data who suffered from the Graves' disease associated cholestasis in the Department of Infectious Disease of Xiangya Hospital from January 2000 to December 2018. The characteristics of the patients' age, gender, liver function, thyroid function, coagulation function, the special cardiac examination, treatment, and follow-up data were analyzed.@*RESULTS@#Thirteen patients, including 10 males and 3 females with the age range from 33 to 55 (median 43) years old presented cholestasis, pruritus, and hypermetabolic symptoms. The levels of total bilirubin (TBIL), direct bilirubin (DBIL), glutamic-pyruvic transferase, glutamic-oxaloacetic transferase, alkaline phosphosphatase, and gamma glutamyl transpeptidase were 170.4-976.7 (median 388.8) µmol/L, 93.2-418.1 (median 199.2) µmol/L, 25.1-182.1 (median 106.4) U/L, 38.2-265.7 (median 59.7) U/L, 105.3-332.0 (median 184.5) U/L, and 20.7-345.1 (median 47.6) U/L, respectively. The levels of free triiodothyronine (FT@*CONCLUSIONS@#Graves' disease can cause cholestasis, with the low incidence. The symptoms of cholestasis can be improved or even eradicated with the cure of the Graves' disease. The cholestasis may be idiopathic. For patients with cholestasis and hyperthyroidism, Graves' disease should be considered for differential diagnosis.

Adult , Cholestasis/etiology , Female , Graves Disease/complications , Humans , Male , Middle Aged , Retrospective Studies , Thyroid Function Tests , Thyroxine , Triiodothyronine
Rev. méd. Chile ; 148(5): 697-701, mayo 2020. tab, graf
Article in English | LILACS | ID: biblio-1139355


ABSTRACT Hyperthyroidism can induce elevation in several liver function tests including aminotransferases, alkaline phosphatases and, less frequently, serum bilirubin. These alterations are usually mild and asymptomatic. We report a 26 year-old male presenting with palpitations, progressive jaundice, choluria and generalized itching. Laboratory tests were compatible with hyperthyroidism and a mild elevation of bilirubin, alkaline phosphatases and gamma glutamyl transpeptidase. A liver biopsy showed portal hepatitis with canalicular cholestasis. The patient was treated temporarily with glucocorticoids, cholestyramine and betablockade. Thereafter, he was treated with radioactive iodine, after which serum bilirubin decreased steadily until normalization in ten weeks.

El hipertiroidismo puede producir elevación de aminotransferasas, fosfatasas alcalinas y, menos frecuentemente, de bilirrubina sérica. Habitualmente, estas alteraciones son leves y asintomáticas. Reportamos un hombre de 26 años con hipertiroidismo secundario a enfermedad de Basedow-Graves, que debutó con un cuadro colestásico, inicialmente estudiado por sospecha de patología hepática autoinmune que incluyó biopsia hepática. Posteriormente, se diagnosticó hipertiroidismo que fue tratado con glucocorticoides, colestiramina y beta bloqueo como puente a terapia definitiva con radioyodo. La evolución mostró disminución progresiva hasta la normalización de bilirrubina sérica.

Humans , Male , Adult , Graves Disease/complications , Cholestasis/diagnosis , Cholestasis/etiology , Hyperthyroidism/complications
Arch. endocrinol. metab. (Online) ; 64(2): 179-184, Mar.-Apr. 2020. tab
Article in English | LILACS | ID: biblio-1131073


SUMMARY Most papillary thyroid carcinomas (PTC) harbor excellent prognosis. Although rare, distant metastases normally occur in lungs and/or bones. Here we describe a rare case of pancreatic metastasis presenting with rapid onset cholestatic syndrome. A literature review was also performed. A 73-year-old man with a high risk PTC was submitted to total thyroidectomy (TT) followed by radioiodine therapy. After initial therapy, he persisted with progressive rising serum thyroglobulin levels but with no evidence of structural disease. Recently, the patient presented with a rapid onset and progressive cholestatic syndrome. A 4 cm lesion in pancreas was identified, with echoendoscopy fine-needle aspiration biopsy (FNAB) confirming a pancreatic metastasis from PTC. The patient was submitted to a successful pancreaticoduodenectomy. Pancreatic metastases of PTC are rare and few long-term follow-up data are available to guide management. Fourteen cases were former reported, mean age was 65.7 years-old with mean time between PTC and pancreatic metastasis diagnosis of 7.9 years. Nine of them had another distant metastasis, nine were diagnosed by FNAB and just two received sorafenib.

Humans , Male , Aged , Pancreatic Neoplasms/complications , Thyroid Neoplasms/pathology , Cholestasis/etiology , Thyroid Cancer, Papillary/pathology , Pancreatic Neoplasms/surgery , Pancreatic Neoplasms/secondary , Syndrome , Thyroidectomy , Thyroid Neoplasms/surgery , Cholestasis/diagnosis , Biopsy, Fine-Needle , Thyroid Cancer, Papillary/surgery
Arch. argent. pediatr ; 118(1): S12-S49, 2020-02-00. tab, ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1096510


La presencia de ictericia en la etapa neonatal puede responder a diversas causas, desde situaciones fisiológicas hasta enfermedades graves. En los neonatos de término que persisten ictéricos más allá de los 14 días de vida, debe determinarse si la hiperbilirrubinemia es no conjugada o conjugada para establecer, a la brevedad, el plan de estudios etiológicos y la terapéutica correspondiente. La hiperbilirrubinemia conjugada (colestasis) refleja una disfunción hepática en la mayoría de los casos, cuyas consecuencias son alteraciones del flujo biliar secundarias a anormalidades estructurales o moleculares del hígado y/o del tracto biliar.Durante la última década, los nuevos estudios moleculares revolucionaron el abordaje de los pacientes colestáticos, lo que permitió el diagnóstico de diversas entidades genéticas. La etiología de la hiperbilirrubinemia del primer trimestre debe determinarse con urgencia, ya que, en muchos casos, el tratamiento instituido de modo precoz puede modificar sustancialmente la evolución de la enfermedad o salvar la vida del paciente.

Neonatal jaundice may be due to different causes, ranging from physiological conditions to severe diseases. In term neonates with persistent jaundice beyond 14 days of life, it should be determined whether hyperbilirubinemia is unconjugated or conjugated, in order to study the etiology and start early treatment. In the majority of cases, conjugated hyperbilirubinemia (cholestasis) is a sign of liver dysfunction possibly associated with alterations in the bile flow secondary to structural or molecular abnormalities of the liver and/or the biliary tract. Over the past decade, new molecular studies have revolutionized the approach of cholestatic patients, leading to the identification of different genetic entities. It is important to determine the etilogy of neonatal hyperbilirubinemia since in many cases early treatment will substantially improve morbidity and mortality.

Humans , Male , Female , Infant, Newborn , Cholestasis/diagnosis , Cholestasis/genetics , Cholestasis/immunology , Cholestasis, Intrahepatic/genetics , Hyperbilirubinemia, Neonatal/diagnosis , Hyperbilirubinemia, Neonatal/etiology , Cholestasis/etiology , Cholestasis/drug therapy , Cholestasis, Intrahepatic/diagnosis , Cholestasis, Intrahepatic/drug therapy
Rev. pediatr. electrón ; 16(1): 18-24, abr. 2019. ilus, tab
Article in Spanish | LILACS | ID: biblio-998476


Resumen Introducción: El síndrome de Alagille es una enfermedad con múltiples afectaciones, es autosómica dominante, con expresividad variable. Se identifica por manifestaciones hepáticas, vertebrales, cardiacas, oculares y dismorfia facial. Objetivo: Reportar un caso de S. de Alagille con afectación hepática, que debuta con hemorragia de vías digestivas altas. Materiales y métodos: Reporte de caso clínico confrontando con artículos de revisiones de temas en búsqueda electrónica en bases de datos de RIMA, MEDLINE, PUBMED, MEDSCAPE, de 1993-2018. Resultado: Paciente de 2 años, con diagnóstico tardío de enfermedad hepática, con progresión a cirrosis y hallazgos al examen físico que confirman Síndrome de Alagille. Se confirma el diagnóstico molecular coincidiendo con el principal hallazgo genético con anomalías asociadas al gen Jagged 1 (JAG1) localizado en el cromosoma 20 y el NOTCH2 del cromosoma 1. Conclusiones: Es de gran importancia resaltar esta patología infrecuente la cual representa un reto diagnóstico, debe tenerse en cuenta la múltiple afectación orgánica por lo cual es fundamental un manejo interdisciplinario

Abstract Introduction: Alagille syndrome is a disease with multiple impairments, is autosomal dominant with variable expressivity. It is identified by manifestations of vertebral, liver, heart, eye and facial dysmorphia. Objective: Report a case of Alagille S. with hepatic involvement, debuting with hemorrhage of upper digestive tract. Materials and methods: Clinical case report confronting articles reviewing subjects in electronic search in RIMA databases, MEDLINE, PUBMED, MEDSCAPE, from 1993-2018. Result: 2 year old patient, with late diagnosis of liver disease, with progression to cirrhosis and physical exam findings that confirm Alagille Syndrome. Confirmed the diagnostic molecular coinciding with the main genetic finding which are anomalies associated with the gene Jagged 1 (JAG1) located on chromosome 20 and the NOTCH2 of chromosome 1. Conclusions: It is important to highlight this uncommon disease which poses a diagnostic challenge, multiple organic involvement must be taken into account by which an interdisciplinary management is essential.

Humans , Male , Child, Preschool , Alagille Syndrome/complications , Alagille Syndrome/diagnosis , Gastrointestinal Hemorrhage/etiology , Cholestasis/diagnosis , Cholestasis/etiology , Alagille Syndrome/genetics , Alagille Syndrome/therapy , Receptor, Notch2 , Face/abnormalities , Jagged-1 Protein , Liver Cirrhosis/diagnosis , Liver Cirrhosis/etiology
Rev. argent. endocrinol. metab ; 54(4): 151-159, dic. 2017. graf, tab
Article in Spanish | LILACS | ID: biblio-957982


El síndrome de interrupción del tallo pituitario (PSIS) se caracteriza por la demostración neurorradiológica de un tallo pituitario ausente, interrumpido o hipoplásico, adenohipófisis aplásica/hipoplásica o neurohipófisis ectópica. Este síndrome se ha relacionado con formas severas de hipopituitarismo congénito (HPC), asociado a múltiples deficiencias de hormonas pituitarias (MPHD). Evaluamos a pacientes con HPC y PSIS, analizando los signos y los síntomas neonatales al diagnóstico, relacionándolos con las deficiencias hormonales pituitarias y signos neurorradiológicos presentes. Estudiamos retrospectivamente a 80 pacientes asistidos en el Hospital de Niños de Córdoba, con diagnóstico de HPC, de los cuales 42 (52%) presentaron PSIS; 22 mujeres y 20 varones, EC: 5 días-9,5 años. El 62% presentó MPHD y el 38% insuficiencia somatotrófica aislada (IGHD). El análisis de las variables perinatales demostró antecedentes de parto natural en el 52% (11/21) de las MPHD vs. 13% (2/15) de las IGHD. Cuatro pacientes, 2 con MPHD y 2 con IGHD presentaban antecedentes obstétricos consistentes en presentación podálica y transversa respectivamente, todos ellos resueltos mediante operación cesárea. Los signos y los síntomas perinatales fueron hipo- glucemia: 61% en MPHD vs. 19% en IGHD, p: 0,0105; ictericia: 38% en MPHD vs. 25% en IGHD; micropene: 77% en MPHD y colestasis: 19% en MPHD. Convulsiones neonatales se presentaron en el 75% de los niños con MPHD e hipoglucemia. EC media de consulta: 2,1 años en MPHD (30% en el período neonatal, 70% antes de 2 años) y 3,6 años en IGHD (44% en menores de 2 años). Los pacientes con MPHD presentaban: tallo no visible 81% (n: 21/26) vs. tallo hipoplásico: 19% (n: 5/26), p: 0,0001; en IGHD 56% (n: 9/16) vs. 44% (n: 7/16), p: 0,5067, respectivamente. El 100% de los neonatos con HPC tenían tallo pituitario ausente. Concluimos que la demostración de PSIS en niños con HPC proporciona información valiosa como predictor de la severidad fenotípica, la presencia de MPHD y de la respuesta al tratamiento. La baja frecuencia de antecedentes obstétricos posicionales potencialmente distócicos, como parte de los mecanismos fisiopatogénicos responsables de PSIS, indicaría la necesidad de analizar la importancia de posibles factores genéticos y epigenéticos involucrados. El diagnóstico precoz de HPC debe sospecharse en presencia de signos y síntomas clínicos, tales como hipoglucemia, colestasis, micropene y defectos asociados en la línea media facial. La resonancia magnética cerebral debe formar parte de los estudios complementarios en pacientes con esta presunción diagnóstica, especialmente a edades tempranas. El reconocimiento tardío de esta entidad puede aumentar la morbilidad y la mortalidad con efectos potenciales deletéreos y permanentes.

Pituitary stalk interruption syndrome (PSIS) is characterised by the combination of an interrupted or thin pituitary stalk, absent or ectopic posterior pituitary, and anterior pituitary hypoplasia. It is manifested as isolated (IGHD) or combined pituitary hormone deficiencies (CPHD) of variable degrees and timing of onset, with a wide spectrum of clinical phenotypes. PSIS may be an isolated morphological abnormality or be part of a syndrome. A retrospective evaluation is presented of clinical signs and symptoms present at early life stages, as well as an analysis of their relationship with hormone laboratory tests and diagnostic imaging in children with congenital hypopituitarism (CHP), and PSIS. This study was performed in a single centre on a sample of 42 children out of a total of 80 CHP patients, with a chronological age range between 5 days and 9.5 years from a database analysed over a period of 26 years. The study included 26/42 (62%) with CPHD and 16/42 (38%) with IGHD. The analysis of perinatal variables showed a natural delivery in 52% (11/21) of CPHD vs 13% (2/15) of IGHD. Four patients, two with CPHD and two IGHD had breech and transverse presentation respectively. All of them were resolved by caesarean section. The perinatal histories showed hypoglycaemia (61% CPHD vs 19% IGHD, P=.0105), jaundice (38% CPHDvs25% IGHD), micropenis (75%CPHD), hypoglycaemic seizures (75% CPHD), and cholestasis (19% CPHD). The mean CA of consulting for CPHD patients was 2.1 years, 30% in neonatal period and 70% before 2 years. The mean chronical age (CA) was 3.6 years in IGHD patients, with 44% of them less than 2 years. MRI showed that 81% of CPHD patients had absence of pituitary stalk vs 19% with thin pituitary stalk (P=.0001); Patients with IGHD presented 56% absence of pituitary stalk vs 44% with thin pituitary stalk (P=.5067). All (100%) of the patients diagnosed in the neonatal stage had absent pituitary stalk. The characterisation of GH deficient patients by presence and type of hypothalamic-pituitary imaging abnormality provides valuable information as a predictor of phenotypic severity, treatment response, and the potential to develop additional hormonal deficiencies. We conclude that demonstrating PSIS in children with HPC provides valuable information as a predictor of phenotypic severity, presence of MPHD, and response to treatment. The low frequency of potentially dysfunctional positional obstetric history, as part of the pathophysiological mechanisms responsible for PSIS, would indicate the need to analyse the importance of possible genetic and epigenetic factors involved. Early diagnosis of HPC should be suspected in the presence of clinical signs and symptoms, such as hypoglycaemia, cholestasis, micropenis, and associated facial midline defects. MRI should be part of complementary studies in patients with this diagnostic suspicion, especially at an early age. Late recognition of this entity may increase morbidity and mortality with potential permanent deleterious effects.

Humans , Male , Female , Infant, Newborn , Infant , Child, Preschool , Child , Pituitary Gland/abnormalities , Pituitary Gland/physiopathology , Hypopituitarism/congenital , Growth Hormone/deficiency , Cholestasis/etiology , Hypoglycemia/etiology , Hypopituitarism/diagnosis
Rev. Col. Bras. Cir ; 44(1): 107-108, Jan.-Feb. 2017. graf
Article in English | LILACS | ID: biblio-842642


ABSTRACT Percutaneous drainage of the bile ducts is an established procedure for malignant obstructions, in which a histological diagnosis is often not obtained. We describe the biopsy technique of obstructive lesions through biliary drainage access, using a 7F endoscopic biopsy forceps, widely available; some are even reusable. This technique applies to lesions of the hepatic ducts, of the common hepatic duct and of all extension of the common bile duct.

RESUMO A drenagem percutânea das vias biliares é um procedimento estabelecido para obstruções malignas, nos quais, muitas vezes, não se consegue um diagnóstico histológico. Descrevemos a técnica de biópsia da lesão obstrutiva através do acesso de drenagem biliar, utilizando um fórcipe de biópsia endoscópica 7F, amplamente disponível e alguns reutilizáveis. Esta técnica aplica-se a lesões dos ductos hepáticos, do hepático comum e de toda extensão do colédoco.

Humans , Bile Duct Neoplasms/pathology , Bile Duct Neoplasms/complications , Biopsy/methods , Cholestasis/etiology , Cholestasis/pathology
Arch. argent. pediatr ; 114(1): e9-e12, feb. 2016. ilus
Article in English, Spanish | LILACS, BINACIS | ID: biblio-838167


El síndrome de artrogriposis, disfunción tubular renal y colestasis es un trastorno fatal infrecuente que compromete múltiples aparatos y sistemas de órganos. Es un trastorno autosómico recesivo hereditario, causado por defectos en los genes VPS33B y VIPAR. Los tres signos primordiales de este síndrome son la artrogriposis, la disfunción tubular renal y la colestasis. Otros compromisos orgánicos a veces asociados con este síndrome son ictiosis, malformación del sistema nervioso central, anomalías trombocíticas, defectos cardíacos congénitos y grave retraso del crecimiento. Las manifestaciones clínicas, la biopsia de un órgano y los análisis de mutaciones pueden ayudar con el diagnóstico, pero no existe un tratamiento curativo; solamente puede instaurarse un tratamiento sintomático. Varios síntomas de esta afección usualmente se manifiestan en el período neonatal: artrogriposis, colestasis neonatal, lesiones cutáneas, entre otros. En general, la supervivencia se prolonga hasta el primer año de vida. Presentamos el caso de una recién nacida con una rápida evolución y desenlace fatal.

Arthrogryposis-renal dysfunction-cholestasis syndrome is a rare lethal disorder that involves multipl organ system. It is inherited autosomal recessive and caused by defects in the VPS33B and VIPAR genes. Three cardinal findings of this syndrome are arthrogryposis, renal tubular dysfunction and cholestasis.The other organ involvements including ichthyosis, central nervous system malformation, platelet anomalies, congenital heart defects and severe failure to thrive are sometimes associated with this syndrome. Clinical findings, organ biopsy and mutational analysis can help for diagnosing but there is no curative treatment except supportive care. Several symptoms of this condition are already usually present in the neonatal period: arthrogryposis, neonatal cholestasis, skin lesions, among others. Usually survival is until the first year of life. We present a newborn whose evolution was rapidly fatal.

Humans , Female , Infant, Newborn , Arthrogryposis/complications , Arthrogryposis/diagnosis , Cholestasis/diagnosis , Cholestasis/etiology , Fatal Outcome , Renal Insufficiency/complications , Renal Insufficiency/diagnosis , Infant, Newborn, Diseases/diagnosis , Infant, Newborn, Diseases/etiology
SQUMJ-Sultan Qaboos University Medical Journal. 2015; 15 (2): 218-224
in English | IMEMR | ID: emr-171464


Parenteral nutrition-associated cholestasis [PNAC] is one of the most challenging complications of prolonged parenteral nutrition [PN] in neonates. There is a lack of research investigating its incidence in newborn infants in Oman and the Arab region. Therefore, this study aimed to assess the incidence of PNAC and its risk factors in Omani neonates. This retrospective study took place between January and April 2014. All neonates who received PN for >/=14 days during a four-year period [June 2009 to May 2013] at the neonatal intensive care unit [NICU] in Sultan Qaboos University Hospital, Muscat, Oman, were enrolled. A total of 1,857 neonates were admitted to the NICU over the study period and 135 neonates [7.3%] received PN for >/=14 days. Determining the incidence of PNAC was only possible in 97 neonates; of these, 38 [39%] had PNAC. The main risk factors associated with PNAC were duration of PN, duration of enteral starvation, gastrointestinal surgeries, blood transfusions and sepsis. Neonates with PNAC had a slightly higher incidence of necrotising enterocolitis in comparison to those without PNAC. This study found a PNAC incidence of 39% in Omani neonates. There were several significant risk factors for PNAC in Omani neonates; however, after logistic regression analysis, only total PN duration remained statistically significant. Preventive strategies should be implemented in NICUs so as to avoid future chronic liver disease in this population

Female , Humans , Infant, Newborn , Male , Cholestasis/etiology , Cholestasis/diagnosis , Parenteral Nutrition , Risk Factors
Gut and Liver ; : 669-673, 2014.
Article in English | WPRIM | ID: wpr-37647


BACKGROUND/AIMS: There are several methods for obtaining tissue samples to diagnose malignant biliary strictures during endoscopic retrograde cholangiopancreatography (ERCP). However, each method has only limited sensitivity. This study aimed to evaluate the diagnostic accuracy of a combined triple-tissue sampling (TTS) method (on-site bile aspiration cytology, brush cytology, and forceps biopsy). METHODS: We retrospectively reviewed 168 patients with suspicious malignant biliary strictures who underwent double-tissue sampling (DTS; n=121) or TTS (n=47) via ERCP at our institution from 2004 to 2011. RESULTS: Among the 168 patients reviewed, 117 patients (69.6%) were eventually diagnosed with malignancies. The diagnostic sensitivity for cancer was significantly higher in the TTS group than the DTS group (85.0% vs 64.9%, respectively; p=0.022). Furthermore, the combination of brush cytology and forceps biopsy was superior to the other method combinations in the DTS group. With respect to cancer type (cholangiocarcinoma vs noncholangiocarcinoma), interestingly, the diagnostic sensitivity was higher for cholangiocarcinoma in the TTS group than the DTS group (100% vs 69.4%, respectively; p<0.001) but not for the non-cholangiocarcinoma patients (57.1% vs 57.1%, respectively). CONCLUSIONS: TTS can provide an improved diagnostic accuracy in suspicious malignant biliary strictures, particularly for cholangiocarcinoma.

Aged , Ampulla of Vater/pathology , Bile Duct Neoplasms/complications , Bile Ducts, Intrahepatic/pathology , Biopsy/methods , Biopsy, Needle , Carcinoma/complications , Cholangiocarcinoma/complications , Cholangiopancreatography, Endoscopic Retrograde/methods , Cholestasis/etiology , Common Bile Duct Neoplasms/complications , Female , Humans , Male , Middle Aged , Pancreatic Neoplasms/complications , Retrospective Studies , Sensitivity and Specificity
Saudi Journal of Gastroenterology [The]. 2013; 19 (6): 286-289
in English | IMEMR | ID: emr-143010


Vanishing bile duct syndrome [VBDS] is a condition resulting from severe bile duct injury, progressive destruction, and disappearance of intrahepatic bile ducts [ductopenia] leading to cholestasis, biliary cirrhosis, and liver failure. VBDS can be associated with a variety of disorders, including Hodgkin's lymphoma [HL]. We describe a 33-year-old male patient who presented with lymphadenopathy and jaundice, and was diagnosed to have HL. Serum bilirubin worsened progressively despite chemotherapy, with a cholestatic pattern of liver enzymes. Diagnosis of VBDS was established on liver biopsy. Although remission from HL was achieved, the patient died of liver failure. Presence of jaundice in HL patients should raise the possibility of VBDS. This report discusses the difficulties of delivering chemotherapy in patients with liver dysfunction. HL-associated VBDS carries a high mortality but lymphoma remission can be achieved in some patients. Therefore, liver transplantation should be considered early in these patients.

Humans , Male , Hodgkin Disease/complications , Cholestasis/etiology , Fatal Outcome , Bile Duct Diseases/diagnosis , Bile Duct Diseases/etiology , Bile Duct Diseases/mortality , Hyperbilirubinemia/etiology , Antineoplastic Combined Chemotherapy Protocols
Acta gastroenterol. latinoam ; 43(2): 146-8, 2013 Jun.
Article in Spanish | LILACS, BINACIS | ID: biblio-1157362


Biliary obstructions are infrequently caused by foreign bodies. We present an unusual case of angiographically placed metallic coils into the intrahepatic arteries to provide hemostasis, that subsequently eroded into the common bile duct leading to obstructive jaundice and cholangitis a year later. In patients with history of invasive procedures, the possibility of foreign body migration into the common bile duct should always be considered in the differential diagnosis of obstructive jaundice and cholangitis.

Cholangitis/etiology , Cholestasis/etiology , Foreign-Body Migration/complications , Hepatic Artery , Acute Disease , Embolization, Therapeutic/adverse effects , Embolization, Therapeutic/instrumentation , Humans , Male , Middle Aged
Gut and Liver ; : 725-730, 2013.
Article in English | WPRIM | ID: wpr-209552


BACKGROUND/AIMS: Stent migration occurs frequently, but the prevention of complications resulting from covered self-expandable metal stents (C-SEMSs) remains unresolved. We prospectively assessed a newly developed C-SEMS, a modified covered Zeo stent (m-CZS), in terms of its antimigration effect. METHODS: Between February 2010 and January 2011, an m-CZS was inserted into 42 patients (31 initial drainage cases and 11 reintervention cases) at a tertiary referral center and three affiliated hospitals. The laser-cut stent was flared for 1.5 cm at both ends, with a 1 cm raised bank located 1 cm in from each flared end. The main outcome of this study was the rate of stent migration, and secondary outcomes were the rate of recurrent biliary obstruction (RBO), the time to RBO, the frequencies of complications, and overall survival. RESULTS: Of the 31 patients with initial drainage, stent migration occurred in four (12.9%, 95% confidence interval, 5.1% to 29.0%), with a mean time of 131 days. RBO occurred in 18 (58%), with a median time to RBO of 107 days. Following previous C-SEMS migration, seven of 10 patients (70%) did not experience m-CZS migration until death. CONCLUSIONS: m-CZSs with antimigration properties effectively, although not completely, prevented stent migration after stent insertion.

Aged , Aged, 80 and over , Alloys , Carcinoma/complications , Cholestasis/etiology , Digestive System Neoplasms/complications , Drainage , Equipment Design , Feasibility Studies , Female , Humans , Kaplan-Meier Estimate , Lymphatic Metastasis , Male , Middle Aged , Prosthesis Failure , Recurrence , Reoperation , Stents/adverse effects , Time Factors
Article in English | WPRIM | ID: wpr-60496


Risk factors for rickets of prematurity have not been re-examined since introduction of high mineral formula, particularly in ELBW infants. We analyzed the incidence and the risk factors of rickets in extremely low birth weight (ELBW) infants. As a retrospective case-control study from 2004 to 2008, risk factors were analyzed in 24 patients with rickets versus 31 patients without. The frequency of rickets in ELBW infants was 24/55 (44%). Infants with rickets were diagnosed at 48.2 +/- 16.1 days of age, and improved by 85.3 +/- 25.3 days. By radiologic evaluation, 29% were grade 1 rickets, 58% grade 2 and 13% grade 3. In univariate analysis, infants with rickets had significantly higher incidence of patent ductus arteriosus, parenteral nutrition associated cholestasis (PNAC), severe PNAC and moderate/severe bronchopulmonary dysplasia (BPD). In multiple regression analysis, after adjustment for gestation and birth weight, rickets significantly correlated with severe PNAC and with moderate/severe BPD. Serum peak alkaline phosphatase levels were significantly elevated in rickets (P < 0.001). In ELBW infants, the incidence of rickets of prematurity remains high and the incidence of severe PNAC and moderate/severe BPD was significantly increased 18 and 3 times, respectively.

Alkaline Phosphatase/blood , Birth Weight , Bronchopulmonary Dysplasia/etiology , Case-Control Studies , Cholestasis/etiology , Female , Gestational Age , Humans , Incidence , Infant , Infant, Extremely Low Birth Weight , Infant, Newborn , Infant, Premature , Male , Parenteral Nutrition/adverse effects , Regression Analysis , Retrospective Studies , Rickets/enzymology , Risk Factors , Severity of Illness Index
Arq. bras. endocrinol. metab ; 55(8): 622-627, nov. 2011. graf
Article in English | LILACS | ID: lil-610464


INTRODUCTION: Neonatal cholestasis due to endocrine diseases is infrequent and poorly reco-gnized. Referral to the pediatric endocrinologist is delayed. OBJECTIVE: We characterized cholestasis in infants with congenital pituitary hormone deficiencies (CPHD), and its resolution after hormone replacement therapy (HRT). SUBJECTS AND METHODS: Sixteen patients (12 males) were included; eleven with CPHD, and five with isolated central hypocortisolism. RESULTS: Onset of cholestasis occurred at a median age of 18 days of life (range 2-120). Ten and nine patients had elevated transaminases and γGT, respectively. Referral to the endocrinologist occurred at 32 days (range 1 - 72). Remission of cholestasis occurred at a median age of 65 days, whereas liver enzymes occurred at 90 days. In our cohort isolated, hypocortisolism was a transient disorder. CONCLUSION: Cholestasis due to hormonal deficiencies completely resolved upon introduction of HRT. Isolated hypocortisolism may be a transient cause of cholestasis that needs to be re-evaluated after remission of cholestasis.

INTRODUÇÃO: A colestase neonatal causada por doenças endócrinas é pouco frequente e reconhecida. Existe um atraso no encaminhamento dos pacientes a um endocrinologista pediátrico. OBJETIVO: Caracterizamos a colestase em recém-nascidos com deficiências congênitas de hormônio hipofisário (DCHH) e sua resolução após a terapia de reposição hormonal (TRH). SUJEITOS E MÉTODOS: Dezesseis pacientes (12 do sexo masculino) foram incluídos; sete com DCHH, e cinco com hipocortisolismo central isolado. RESULTADOS: O início da colestase ocorreu aos 18 dias de vida (variação 2-120). Dez e nove pacientes apresentaram elevação das transaminases e γGT, respectivamente. A consulta com um endocrinologista aconteceu aos 32 dias (variação 1-72). A remissão da colestase ocorreu em uma idade mediana de 65 dias, enquanto a remissão das enzimas hepáticas aconteceu aos 90 dias. Na coorte isolada, o hipocortisolismo foi uma desordem transitória. CONCLUSÃO: A colestase causada por deficiências hormonais foi completamente resolvida após a introdução da TRH. O hipocortisolismo pode ser uma causa transitória da colestase e precisa ser reavaliado após a remissão da colestase.

Female , Humans , Infant , Male , Adrenal Insufficiency/etiology , Cholestasis/etiology , Hydrocortisone/therapeutic use , Hypopituitarism/congenital , Liver Diseases/etiology , Thyroxine/therapeutic use , Age of Onset , Adrenal Insufficiency/physiopathology , Cholestasis/physiopathology , Follow-Up Studies , Hormone Replacement Therapy/methods , Hydrocortisone/deficiency , Hypopituitarism/drug therapy , Liver Diseases/physiopathology , Pituitary Hormones, Anterior/deficiency , Remission Induction , Retrospective Studies , Treatment Outcome