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La piel y sus anexos tienen amplia relación con todos los órganos y sistemas. Los cambios presentes en estos pueden ser el primer hallazgo en un paciente con enfermedad hepática, encontrándose hasta en el 20 % de los casos, por lo que las manifestaciones extrahepáticas adquieren importancia, y aunque muchas de estas no son específicas, algunos marcadores dermatológicos pueden ayudar al diagnóstico de la enfermedad y se pueden correlacionar con su severidad. El desarrollo de las lesiones cutáneas en los pacientes con cirrosis hepática se genera principalmente por hipertensión portal y exceso de estrógenos circulantes, por lo tanto, su tratamiento se basa en el manejo de la patología hepática subyacente. En el presente artículo se hace una revisión de la literatura y se describe un amplio espectro de manifestaciones dermatológicas asociadas a cirrosis hepática, con sus características y etiopatogénesis, siendo las más frecuentes la ictericia, el prurito, los nevus en araña, el eritema palmar, las venas en cabeza de Medusa, y los cambios de uñas y del vello, entre otras.
The skin and its annexes have a broad relationship with all organs and systems. Changes present in these can be the initial finding in a patient with liver disease, occurring in up to 20% of cases, therefore the extrahepatic manifestations become important, and although many of these are not specific, some dermatological markers can help in the diagnosis of the disease and may correlate with its severity. The development of cutaneous lesions in patients with liver cirrhosis is mainly generated by portal hypertension and excess of circulating estrogens, therefore their treatment is based on managing the underlying liver pathology. This article reviews the literature and describes a wide range of dermatological manifestations associated with liver cirrhosis, with their characteristics and etiopathogenesis, being the most frequent jaundice, pruritus, spider nevus, palmar erythema, caput Medusae veins, nail and hair changes, among others.
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HumanosRESUMO
Las enfermedades hepáticas presentan múltiples manifestaciones sistémicas, entre las cuales se destacan los hallazgos en piel, siendo los más comunes el prurito y la ictericia; así mismo, se pueden encontrar angiomas en araña, eritema palmar, xantomas, vasculitis y cambios en anexos. Este artículo tiene como objetivo describir los principales signos y síntomas cutáneos en las enfermedades hepáticas para brindar herramientas semiológicas al clínico en su práctica diaria
Liver disease present multiple systemic manifestations, among which skin findings stand out, being the most common pruritus and jaundice. Other findings can also be manifested like spider angiomas, palmar erythema, xanthomas, vasculitis and changes in skin appendages. The objective of this article is to describe the main skin signs and symptoms of liver diseases to provide semiological tools to the physician in his daily practice.
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HumanosRESUMO
RESUMO Objetivo Analisar a hiperbilirrubinemia como indicador para a realização do protocolo de risco na triagem auditiva neonatal (TAN) e no monitoramento auditivo em neonatos a termo e prematuros. Método Trata-se de um estudo observacional, transversal e retrospectivo. Foram incluídas 554 crianças nascidas em uma maternidade pública, subdivididas em dois grupos: (G1) com 373 recém-nascidos a termo; (G2) com 181 neonatos prematuros. Os dados foram coletados nos prontuários dos participantes, a fim de se obter informações referentes ao resultado da TAN realizada por meio do registro do Potencial Evocado Auditivo de Tronco Encefálico, às condições de nascimento, características clínicas, intervenções realizadas, resultados do primeiro exame de bilirrubina total (BT) e bilirrubina indireta (BI) e do pico de BT e BI. Realizou-se análise estatística descritiva e inferencial dos dados, com adoção do nível de significância de 5%. Resultados No teste da TAN, foram observadas taxas de encaminhamento para reteste inferiores no G1 em relação ao G2. Não houve diferença entre os grupos quanto à ocorrência do tipo de parto, sexo, presença de incompatibilidade sanguínea Rh e ABO, deficiência de enzima G6PD e realização de fototerapia. Em relação aos níveis de BT e BI no primeiro exame e no momento do pico, não houve diferenças entre os neonatos com resultado "passa" e "falha" na TAN-teste nos dois grupos. Conclusão Os níveis de bilirrubina no período neonatal abaixo dos valores recomendados para indicação de exsanguineotransfusão não estão diretamente relacionados ao resultado "falha" na TAN em neonatos a termo e prematuros.
ABSTRACT Purpose To analyze hyperbilirubinemia as an indicator for the definition of risk protocol in newborn hearing screening (NHS) and in auditory monitoring in full-term and preterm neonates. Methods This is an observational, cross-sectional and retrospective study. A total of 554 children born in a public maternity hospital were included and divided into two groups: (G1) with 373 full-terms neonates; (G2) with 181 preterm neonates. Data were collected from the participant's medical records to obtain information regarding the result of the NHS, performed by recording the automated auditory brainstem response (AABR), birth conditions, clinical characteristics, interventions performed, and results of the first test of total bilirubin (TB) and indirect bilirubin (IB) as well as the peak of TB and IB. A descriptive statistical analysis of the results was performed, and the level of significance adopted was 5%. Results On the NHS test, quotes of retest referral rates were smaller in G1 when compared to G2. There was no significant difference between the groups regarding type of delivery, gender, presence of Rh and ABO incompatibility, G6PD enzyme deficiency, and performance of phototherapy. TB and IB levels at the first exam and at peak time did not differ between neonates with "pass" and "fail" results on the NHS test in both groups. Conclusion Bilirubin levels in the neonatal period below the recommended values for indication of exchange transfusion are not directly related to the "fail" result on the NHS tests in term and preterm neonates.
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Contexte et objectifs. L'ictère néonatal est un symptôme fréquent. L'objectif de la présente étude était d'actualiser le profil épidémiologique et d'identifier les facteurs associés à l'ictère néonatal chez les nouveau-nés malades. Méthodes. Une étude transversale descriptive a été menée de juin 2022 à avril 2023 aux Cliniques Universitaires de Kinshasa. L'étude a concerné les nouveau-nés malades ayant présenté un ictère cutanéomuqueux. Les variables sociodémographiques, périnatales, cliniques et paracliniques ont été recherchées. Résultats. Sur 152 nouveau-nés malades, 102 (67,1%) cas d'ictère ont été identifiés. Les nouveau-nés à terme (72,5%), nés par voie basse (67,6%) et dont les mères avaient présenté des infections uro-génitales (98%) et de groupe sanguin O (53%) rhésus positif (97,1%) étaient les plus représentés. L'ictère s'est manifesté dans la première semaine de vie (85,3 %). La bilirubine sérique totale initiale se situait entre 10 et 15 mmol/L (57,8 %). L'origine infectieuse était notée dans 85 % des cas (Klebsiella pneumoniae dans 50 % des cas). La photothérapie conventionnelle a été utilisée chez 74,5 %. L'accouchement par voie basse était le seul facteur associé (p=0,001). Conclusion : L'ictère néonatal est fréquent chez les nouveau-nés malades. L'étiologie infectieuse doit être recherchée systématiquement. Une prise en charge appropriée permet de réduire la survenue de séquelles neurosensorielles.
Context and objective. Neonatal jaundice is a common symptom. The objective of the present study was to update the epidemiological profile and identify the factors associated with neonatal jaundice in sick newborns. Methods. A descriptive cross-sectional study was conducted from June 2022 to April 2023 at the Kinshasa University Hospital. The study included sick newborns who presented with mucocutaneous jaundice. Sociodemographic, perinatal, clinical and paraclinical variables were sought. Results. Out of 152 sick newborns, 102 (67.1 %) cases of jaundice were identified. Fullterm newborns (72.5 %), born vaginally (67.6 %) and whose mothers had presented with urogenital infections (98 %) and blood group O (53 %) rhesus positive (97.1 %) were the most represented. Jaundice appeared in the first week of life (85.3 %). Baseline total serum bilirubin was between 10 and 15 mmol/L (57.8 %). The infectious origin was noted in 85 % of cases (Klebsiella pneumoniae in 50 % of cases). Conventional phototherapy was used in 74.5 %. Vaginal delivery was the only associated factor (p=0.001). Conclusion. Neonatal jaundice is common in sick newborns. The infectious etiology must be systematically sought. Appropriate management helps reduce the occurrence of neurosensory aftereffects.
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Humanos , Masculino , Feminino , Icterícia NeonatalRESUMO
La colangiopatía portal hace referencia a anomalías colangiográficas que se producen en pacientes con cavernomatosis portal, siendo progresiva, cursando con enfermedad biliar sintomática y anomalías graves de las vías biliares. Y, representa una complicación infrecuente de la hipertensión portal. Se describe el caso de un hombre de 53 años, con historia de larga data de hipertensión portal nocirrótica y cavernomatosis portal, quien presentó un episodio de enfermedad biliar obstructiva sintomática, y en estudios se documentó tejido fibrótico de extensión periportal ascendente con compresión extrínseca del colédoco distal y dilatación de la vía biliar extra e intrahepática. Por lo que se procedió a colangiopancreatografía retrógrada endoscópica, realizándose tratamiento paliativo, con papilotomía pequeña y colocación de endoprótesis biliar plástica, siendo exitoso por ausencia de complicaciones procedimentales, y mejoría clínica y parámetros bioquímicos. Finalmente, recibiendo de alta con indicación de seguimiento prioritario para recambios periódicos de endoprótesis biliares, y valoración por hepatología. La colangiopatía portal es una entidad rara que debe sospecharse en sujetos con hipertensión portal de origen no-cirrótico, con hallazgos imagenológicos de estenosis, angulaciones o dilataciones segmentarias, su tratamiento debe ser individualizado, y la terapia endoscópica es de elección en enfermedad biliar sintomática.
Portal cholangiopathy refers to cholangiographic abnormalities occurring in patients with portal cavernomatosis, being progressive, presenting with symptomatic biliary disease and severe biliary tract abnormalities. And, it represents an infrequent complication of portal hypertension. We describe the case of a 53-year-old man with a long history of non-cirrhotic portal hypertension and portal cavernomatosis, who presented an episode of symptomatic obstructive biliary disease, and studies documented fibrotic tissue of ascending periportal extension with extrinsic compression of the distal common bile duct and dilatation of the extra and intrahepatic biliary tract. Therefore, endoscopic retrograde cholangiopancreatography was performed, and palliative treatment with small papillotomy and placement of a plastic biliary endoprosthesis was successful due to the absence of procedural complications, and clinical improvement and biochemical parameters. Finally, the patient was discharged with indication of priority follow-up for periodic replacement of biliary stents, and evaluation by hepatology. Portal cholangiopathy is a rare entity that should be suspected in subjects with portal hypertension of non-cirrhotic origin, with imaging findings of stenosis, angulations or segmental dilatations, its treatment should be individualized, and endoscopic therapy is of choice in symptomatic biliary disease.
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In Colombia, coccidioidomycosis is a rare entity, and the intestinal manifestation is infrequent, with around a dozen cases reported in the world literature. This article reports the case of a 29-year-old male Venezuelan immigrant with a 4-month history of abdominal pain, jaundice, nausea, and vomiting. The tomography and the endoscopic study revealed a circumferential exophytic mass in the second portion of the duodenum. The biopsy revealed multiple spherules filled with round fungal endospores with a final diagnosis of disseminated coccidioidomycosis. The patient was discharged before the final pathology report with fluconazole doses of 200 mg every other day and an order for outpatient magnetic resonance cholangiography for outpatient follow-up, which he has not attended.
La coccidioidomicosis en Colombia es una entidad infrecuente y la presentación intestinal es extremadamente rara, con alrededor de una docena de casos reportados en la literatura mundial. En el presente artículo se reporta el caso de un paciente inmigrante venezolano masculino de 29 años con historia de dolor abdominal, ictericia, náuseas y vómito de 4 meses de evolución. La tomografía y el estudio endoscópico evidenciaron una masa exofítica circunferencial en la segunda porción del duodeno. La biopsia reveló múltiples esférulas llenas de endosporas fúngicas redondas con diagnóstico final de coccidioidomicosis diseminada. El paciente fue dado de alta antes del reporte final anatomopatológico con dosis de fluconazol de 200 mg interdiario y orden de colangiorresonancia ambulatoria para control ambulatorio, mismo al cual no ha asistido.
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La ictericia colestásica se debe a la alteración de la secreción de bilirrubina conjugada; es una de las posibles causas la alteración del flujo biliar por obstrucción de la vía biliar extrahepática. El linfoma es la tercera neoplasia más frecuente en pediatría, mientras que los tumores pancreáticos son poco frecuentes y, en su mayoría, lesiones benignas. Las manifestaciones clínicas de los tumores de localización retroperitoneal son poco específicas y suelen ser tardías, por lo que la sospecha clínica debe ser alta. El objetivo del siguiente trabajo es presentar el caso de un niño de 7 años con síndrome colestásico en el que se halló un tumor en la cabeza del páncreas que comprimía la vía biliar extrahepática. El diagnóstico del tumor fue linfoma no Hodgkin (LNH). Se destaca la infrecuencia de este tumor en esta localización en la edad pediátrica
Cholestatic jaundice is due to an alteration in conjugated bilirubin secretion; a possible cause is an altered bile flow resulting from an obstruction of the extrahepatic bile duct. A lymphoma is the third most common neoplasm in pediatrics, while pancreatic tumors are rare and mostly benign. The clinical manifestations of retroperitoneal tumors are not very specific and are usually late, so a high level of clinical suspicion is required. The objective of this study is to describe the case of a 7-year-old boy with cholestatic syndrome with a tumor in the head of the pancreas compressing the extrahepatic bile duct. The tumor diagnosis was non-Hodgkin lymphoma (NHL). It is worth noting that the presence of a tumor in this location in pediatric age is uncommon
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Humanos , Masculino , Criança , Linfoma não Hodgkin/complicações , Linfoma não Hodgkin/diagnóstico , Linfoma não Hodgkin/patologia , Colestase/etiologia , Icterícia Obstrutiva/diagnóstico , Icterícia Obstrutiva/etiologia , Icterícia Obstrutiva/patologia , Pâncreas , Síndrome , Colestase/diagnósticoRESUMO
Abstract Objectives To evaluate the therapeutic effect of Saccharomyces boulardii supplementation on jaundice in premature infants undergoing phototherapy. Methods In this article, the authors reviewed 100 hospitalized jaundiced premature infants under 35 weeks of gestational age. All infants were assigned to a control group (n= 45) and a treatment group (n= 55) randomly. The infants in the treatment group received S. boulardii supplementation by undergoing phototherapy and the infants in the control group were only treated by phototherapy. The total serum bilirubin levels were detected before and at the end of phototherapy, and transcutaneous bilirubin levels were measured on the 1st, 4th, 8th and 15th day of treatment. The duration of jaundice resolution and phototherapy, stool frequency, and characteristics were compared after phototherapy. Results The duration of jaundice resolution and phototherapy were shortened. Total serum bilirubin level was lower than the control group at the end of phototherapy (p < 0.05). Transcutaneous bilirubin levels decreased more significantly on the 8th and 15th day of treatment (p < 0.05), while there were no significant differences on the post-treatment 1st and 4th day (p > 0.05). In addition, bowel movements including stool frequency and Bristol Stool Form Scale ratings of stools also improved after treatment. Conclusions S. boulardii in combination with phototherapy is effective and safe in reducing bilirubin levels and duration of phototherapy, accelerating jaundice resolution in premature infants with jaundice. The procedure also provided an ideal therapeutic effect of diarrhea induced by phototherapy to promote compliance and maternal-infant bonding.
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Fibrolamellar hepatocellular carcinoma is a rare primary hepatic tumor that usually occurs in youth. The common presenting features are vague abdominal pain, nausea, vomiting and weight loss. We present a case report of a young male who presented with cholestatic jaundice and on evaluation was diagnosed to have fibrolamellar hepatocellular carcinoma. He underwent successful surgical resection of the tumor. In young individuals presenting with unexplained cholestasis, fibrolamellar hepatocellular carcinoma should be considered.
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Background: Pregnancy with jaundice is considered a high-risk pregnancy. It is the most usual health complaint in pregnant women found more often in developing countries than in developed ones. Jaundice can be concurrent with 1st trimester due to pathological infection for instance viral hepatitis or gall stones or due to the drug administered during pregnancy. These patients may feel better if initial diagnosis and suitable management are provided. This present study intended to evaluate the clinical profile & complications of jaundice during pregnancy. Material & Methods: This descriptive cross-sectional study was conducted in the Department of Obstetrics and Gynecology, Dhaka Medical College Hospital, Dhaka, Bangladesh from January 2012 to June 2012. A total (N=50) of pregnant women with symptoms of jaundice were enrolled in the study. Completed data forms were reviewed, edited, and processed for computer data entry. The data analysis was performed using Statistical Package for the Social Sciences (SPSS) Version 16.0. Descriptive inferential statistics were performed to determine the results of this study. Results: Among the study population (N=50), the majority of patients (34, 68.0%) age were between 20 to 24 years. The mean gestational age of patients was 35.3±3.2 weeks and twelve patients (12, 24.0%) underwent caesarean delivery. Based on clinical features, twenty-seven respondents (27, 54.0%) had mild jaundice, fourteen respondents (14, 28.0%) had moderate jaundice & about one-fifth of the patients (9, 18.0%) had severe jaundice. Fifteen pregnant (15, 30.0%) women had a fever, around two-fifth of the patients (19, 38.0%) had vomiting, pruritus in one patient (1, 2.0%), vaginal bleeding in twelve patients (12, 24.05), & more than half of the patients (27, 54.0%) had ruptured membrane. The causes of jaundice during pregnancy were viral hepatitis in forty-three patients (43, 86.0%). The majority of the patients (27,54.0%) had serum bilirubin less than 10mg, fourteen patients (14,28.0%) had 10 to 15mg of serum bilirubin & only one patient (1,2.0%) had serum bilirubin more than 20mg. SGOT & SGPT was found 100-500 IU/ml in twenty-three patients (23,46.0%) & 500-1000 IU/ml in ten patients (10,20.0%). Alkaline was raised in eighteen cases (18, 36.0%) Based on maternal complications, the majority of patients (31, 62.0%) had a postpartum haemorrhage, and ten patients (10, 20.0%) experienced encephalopathy. Thirty-eight babies (38, 76.0%) were born alive, intrauterine death was in seven cases (7, 14.0%), and stillborn in five cases (5, 10.0%). Conclusion: Hepatitis E was the most common cause of viral hepatitis in our study. Major complications were postpartum haemorrhage and encephalopathy. This study also prevailed, jaundice in pregnancy is related to an increase in maternal mortality and morbidity, obstetric complications, and perinatal complications.
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The liver is a crucial organ in metabolism, and some substances can induce toxic hepatitis with high morbidity and mortality. Chemical and drug-induced liver disease is a diagnostic and therapeutic challenge since it requires extension studies to rule out other entities. We present the case of a 51-year-old female patient without underlying comorbidities, admitted due to symptoms of two-day evolution consisting of progressive jaundice, diarrheal episodes without acholia, or any other additional manifestation. Her condition was caused by the intake of nimesulide, two tablets a day for two days, for pain secondary to a mandibular cyst diagnosed in previous days. During her admission to the emergency room, the patient described chronic consumption of Herbalife® products daily for four years. She presented with elevated transaminases, prolonged prothrombin time (PT), and direct hyperbilirubinemia. Infectious and immunological diseases were ruled out. We decided to start antibiotic and vitamin K coverage. Finally, and by exclusion, a liver biopsy suggested an inflammatory process compatible with drug-induced hepatitis. The woman evolved favorably when the medication and dietary supplement were discontinued. In conclusion, this case constitutes an initial point in advancing research into hepatotoxicity by shared mechanisms of various substances simultaneously, such as what happened to the patient with the parallel use of Herbalife® and nimesulide.
El hígado es un órgano crucial en el metabolismo y algunas sustancias pueden inducir hepatitis toxica con alta morbimortalidad. La enfermedad hepática inducida por sustancias químicas y medicamentos es un desafío tanto diagnostico como terapéutico, puesto que requiere la realización de estudios de extensión para descartar otras entidades. A continuación se presenta el caso de una paciente femenina de 51 años sin comorbilidades de base, ingresada por clínica de 2 días de evolución consistente en ictericia progresiva, episodios diarreicos sin acolia ni otra manifestación adicional. Aparentemente, su cuadro fue provocado por la administración de nimesulida, 2 tabletas al día por 2 días, contra el dolor secundario a un quiste mandibular diagnosticado en días anteriores. Durante su ingreso a urgencias la paciente describió consumo crónico, a diario desde hace 4 años, de productos de Herbalife®. Cursa con elevación de transaminasas, prolongación del tiempo de protrombina (TP) e hiperbilirrubinemia directa. Se descartan enfermedades infecciosas e inmunológicas. Se decidió iniciar el cubrimiento antibiótico y vitamina K. Finalmente y por exclusión, se realizó una biopsia hepática que sugirió un proceso inflamatorio compatible con hepatitis inducida por fármacos. La mujer evolucionó favorablemente al suspender la medicación y el suplemento dietético referido. En conclusión, el caso expuesto constituye un punto inicial en el avance hacia la investigación en hepatotoxicidad por mecanismos compartidos de diversas sustancias simultáneamente, como lo sucedido a la paciente con el uso paralelo de Herbalife® y de nimesulida.
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Introducción: La deshidratación hipernatrémica neonatal es un problema creciente, en la que la alimentación y los conocimientos sobre cuidados del recién nacido juegan un papel primordial. Objetivo: Determinar los factores de riesgo asociados a la deshidratación hipernatrémica en neonatos. Métodos: Estudio observacional descriptivo retrospectivo en una población de 20 neonatos con deshidratación hipernatrémica ingresados en el servicio de cuidados intermedios de un hospital de Lima, Perú entre junio 2017- septiembre 2018. Todos tenían concentraciones de sodio sérico≥150 mmol/L. La recolección de la información se extrajo de las historias clínicas. Resultados: De los 20 neonatos con deshidratación hipernatrémica; los nacidos por cesárea equivalen a 30 %, mientras que por parto vaginal, a 70% de los casos. Se demostró que hay una relación inversa entre número de gestación y concentraciones de sodio al ingreso, sin embargo, no hubo significación estadística. Se logró una disminución entre sodio de entrada y su primer control, con una media de disminución de 0,57 meq/L/h. Clínicamente los neonatos afectados desarrollaron en mayor porcentaje ictericia y fiebre. No se encontró relación significativa entre el resto de los factores asociados. Conclusiones: Entre los factores de riesgo asociados a la deshidratación hipernatrémica, destaca que el grupo nacidos por cesárea fue menor que los nacidos por parto vaginal, lo que influye en el número de altas precoces al nacimiento. Ictericia y fiebre continúan siendo las características clínicas que debe identificarse tempranamente. Se trata de un cuadro prevenible si se brinda adecuada información a la madre sobre cuidado neonatal.
Introduction: Neonatal hypernatremic dehydration is a growing problem, in which feeding and knowledge about newborn care play a key role. Objective: To determine the risk factors associated with hypernatremic dehydration in neonates. Methods: Retrospective descriptive observational study in a population of 20 neonates with hypernatremic dehydration admitted to the intermediate care service of a hospital in Lima, Peru between June 2017 and September 2018. All of them had serum sodium concentrations≥150 mmol/L. The collection of information was extracted from medical records. Results: Of the 20 neonates with hypernatremic dehydration, those born by cesarean section are equivalent to 30%, while by vaginal delivery, 70% of cases. It was shown that there is an inverse relationship between gestation number and sodium concentrations at admission, however, there was no statistical significance. A decrease was achieved between input sodium and its first control, with a mean decrease of 0.57 meq/L/h. Clinically, the affected neonates developed a higher percentage of jaundice and fever. No significant relationship was found between the rest of the associated factors. Conclusions: Among the risk factors associated with hypernatremic dehydration, it stands out that the group born by cesarean section was lower than those born by vaginal delivery, which influences the number of early discharges at birth. Jaundice and fever continue to be the clinical features that should be identified early. This is a preventable condition if adequate information is provided to the mother about neonatal care.
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La tirotoxicosis es la manifestación clínica de una liberación excesiva de hormonas tiroideas, asociada o no a una función glandular autónoma; en este primer escenario, se denomina específicamente hipertiroidismo. Las principales etiologías son la enfermedad de Graves (EG), el adenoma tóxico, el bocio multinodular tóxico y el grupo de tiroiditis, predominando sus formas aguda y subaguda. La EG es la forma más común de hipertiroidismo, representando entre el 60 % y el 80 % de los casos, con una mayor incidencia en personas entre 40 y 60 años. Se ha descrito un compromiso hepático entre 45 % y el 90 % de pacientes con hipertiroidismo. Presentamos el caso de un hombre de 47 años con tirotoxicosis secundaria a enfermedad de Graves con compromiso bioquímico hepático manifestado como colestasis intrahepática refractaria al tratamiento médico en el corto plazo, tratado exitosamente con plasmaféresis como terapia puente a tiroidectomía total, proporcionando un análisis de la respuesta a la terapia a través de un cambio en los niveles de tiroxina libre (T4) y bilirrubina total a lo largo de su evolución.
Thyrotoxicosis is the clinical manifestation of excessive thyroid hormone release, whether or not asso-ciated with autonomous glandular function; in this first scenario, it is specifically termed hyperthyroi-dism. The main etiologies are Graves' disease (GD), toxic adenoma, toxic multinodular goiter ant the group of thyroiditis, predominantly acute and subacute forms. GD is the most common form of hyperthyroidism, accounting for 60% to 80% of cases, with a higher incidence among people aged 40 to 60 years. Liver involvement has been reported in 45% to 90% of patients with hyperthyroi-dism. We present the case of a 47-year-old man with thyrotoxicosis secondary to Graves' disease with hepatic biochemical involvement manifested as intrahepatic cholestasis refractory to medical management in the short term, successfully treated with plasmapheresis as bridge therapy to total thyroidectomy, providing an analysis of the response to therapy through a change in free thyroxine (T4) and total bilirubin levels throughout his evolutio
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HumanosRESUMO
ABSTRACT A male infant presented with progressive jaundice immediately after birth. Fecal acholia and choluria associated with extensive bullous skin lesions in his trunk, abdomen, and upper and lower limbs developed during phototherapy. Several diagnostic hypotheses were presented, including neonatal porphyria, hemochromatosis, Alagille syndrome, and neonatal lupus. A 24-hour urine sample for the dosage of urinary porphyrins was collected, showing high results (1823.6µg in 100mL). At 50 days of life, fluorescence spectroscopy using a Wood's lamp revealed simultaneous bright red fluorescence of urine-stained diapers and sample blood. A definitive diagnosis of congenital erythropoietic porphyria was made following identification of a mutation of the uroporphyrinogen synthetases III gene on genetic testing. The patient was subsequently maintained in a low light environment since then, resulting in improvement of the lesions. Congenital erythropoietic porphyria is a disease of the group of porphyrias that presents shortly after birth with blistering occurring in regions exposed to the sun or other ultraviolet light. Atrophic scars, mutilated fingers, and bright red fluorescence of the urine and teeth may also be observed. There is no specific treatment, and prophylaxis comprising a total avoidance of sunlight is generally recommended. A high degree of suspicion is required for diagnosis. An early diagnosis can lead to less damage. Here, we present the case of a newborn with congenital erythropoietic porphyria diagnosed after presenting with bullous lesions secondary to phototherapy.
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Objective To investigate the effect of Yinchenhao decoction on renal oxidative stress injury in rats with obstructive jaundice and its association with the regulation of the expression of nuclear factor erythroid 2-related factor 2 (Nrf2) and nuclear translocation. Methods A total of 32 male Sprague-Dawley rats were randomly divided into sham-operation group (S group), model group (O group), low-dose Yinchenhao decoction group (LY group), and high-dose Yinchenhao decoction group (HY group), with 8 rats in each group. For the rats in the S group, the upper common bile duct was isolated without ligation, and for those in the other groups, double ligation of the middle and upper 1/3 of the common bile duct was performed to establish a model of obstructive jaundice. After 7 days, the rats in the LY group and the HY group were given Yinchenhao decoction by gavage at a dose of 6.3 and 18.9 mL/kg, respectively, while those in the S and O groups were given an equal volume of distilled water by gavage every day for 7 consecutive days, and the rats were treated on day 14. ELISA was used to measure the serum levels of total bilirubin (TBil), direct bilirubin (DBil), alanine aminotransferase (ALT), gamma-glutamyl transpeptidase (GGT), blood urea nitrogen (BUN), and creatinine (Cr); spectrophotometry was used to measure the activity of the oxidative stress factors superoxide dismutase (SOD) and malondialdehyde (MDA) in renal tissue; quantitative real- time PCR and Western blotting were used to measure the mRNA and protein expression levels of Nrf2, Kelch-like ECH-associated protein 1 (Keap1), and NAD(P)H quinone dehydrogenase 1 (NQO1) in renal tissue; immunohistochemistry was used to measure observe the nuclear translocation of Nrf2 protein in renal tissue. A one-way analysis of variance was used for comparison of continuous data between multiple groups, and the least significant difference t -test was used for further pairwise comparison within groups. Results Compared with the S group, the O group had significant increases in the levels of TBil, DBil, ALT、GGT, BUN, and Cr, a significant reduction in the activity of SOD, and a significant increase in the level of MDA (all P 0.05). Compared with the S group, the O group had a significant reduction in the positive rate of Nrf2 in cell nucleus in renal tissue ( P < 0.05), and compared with the O group, the LY group and the HY group had a significant increase in the positive rate of Nrf2 in cell nucleus ( P < 0.05). Conclusion Yinchenhao decoction can effectively alleviate renal injury caused by obstructive jaundice, possibly by upregulating the protein expression of Nrf2 in renal tissue and regulating the nuclear translocation of Nrf2 protein, so as to mediate the protein expression of downstream NQO1, regulate oxidative stress response caused by obstructive jaundice, and thereby alleviate renal injury in rats.
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Objective To investigate the etiological and clinical features of patients with unexplained liver disease manifesting as isolated jaundice and the value of whole-exome sequencing in the diagnosis of such diseases. Methods A retrospective analysis was performed for the clinical data of the patients who attended Nanjing Second Hospital due to unexplained liver disease and underwent whole-exome sequencing from February 2017 to December 2021, and according to liver function parameters and imaging data, all cases were classified based on clinical phenotype and were diagnosed based on the whole-exome sequencing report. The Mann-Whitney U test was used for comparison of non-normally distributed continuous data between two groups, and the Kruskal-Wallis H test was used for comparison between multiple groups. Results A total of 519 patients underwent whole-exome sequencing, among whom 102 patients with missing or incomplete clinical data were excluded, and finally 417 patients were included in analysis, among whom 91(91/417, 21.82%) had the manifestation of isolated jaundice. The etiology of jaundice was not determined by whole-exome sequencing in 8 patients (8/91, 8.79%). With reference to genetic testing results, 83 patients (83/91, 91.21%) had a confirmed diagnosis, among whom there were 68 patients with hereditary hyperbilirubinemia (68/91, 74.72%), 3 patients with hereditary spherocytosis (3/91, 3.30%), 2 patients with pyruvate kinase deficiency (2/91, 2.20%), and 10 patients with UGT1A1 gene disease combined with other diseases (10/91, 10.99%). Hereditary hyperbilirubinemia was the main etiology, and there were 61 patients with UGT1A1 gene disease (61/91, 67.03%), 5 patients with Dubin-Johnson syndrome (5/91, 5.49%) and 2 patients with Rotor syndrome (2/91, 2.20%). There was a significant difference in indirect bilirubin/total bilirubin ratio between the patients with the different diagnoses above ( H =22.835, P < 0.05), and the patients with UGT1A1 gene disease and other diseases had a significantly higher level of total bilirubin than those with UGT1A1 gene disease alone [95.8 (37.5-187.1) μmol/L vs 51.4 (34.8-267.1) μmol/L, Z =-2.372, P =0.018]. Conclusion Whole-exome sequencing can help with the diagnosis of most cases of unexplained liver disease manifesting as isolated jaundice. Hereditary hyperbilirubinemia is the main etiology, and UGT1A1 gene disease is the most common disease. Whole-exome sequencing can assist the clinical diagnosis of unexplained liver disease manifesting as isolated jaundice.
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Objective To investigate the safety and efficacy of photodynamic therapy (PDT) for malignant obstruction of the biliary tract. Methods We retrospectively analyzed the clinical data of patients with malignant biliary obstruction treated by PDT in our medical center. On the basis of different treatment plans, the patients were categorized into the photodynamic only group and the combined treatment group, in which additional interventional operations, targeted therapy, or immunotherapy were arranged. The alterations in liver function, duration of biliary patency, and postoperative complications that occurred within one month were closely monitored in both groups. Results A total number of 19 patients were enrolled in this study. The technical success rate of PDT was 100%. The deterioration of liver function was not observed in any patients within one month after PDT. Within a maximum of 17.7 months follow-up, the patency rates of the biliary tract were 100.0%, 89.5%, 72%, and 64% at 1, 3, 6, and 12 months after the procedure, respectively. The mean biliary patency time was 6.9±0.8 months (95%CI: 5.2-8.7 months). Specifically, the biliary patency times for Bismuth type Ⅲ and Ⅳ were 7.5±1.1 and 6.1±1.3 months, respectively. The biliary patency time was around 3.3±0.7 months in the photodynamic only group and 7.9±0.9 months in the combined treatment group (P=0.017). Conclusion PDT for Bismuth Ⅲ-Ⅳ malignant biliary obstruction is safe and effective. Moreover, the period of biliary patency is greatly extended when PDT is combined with systemic therapy.
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OBJECTIVES@#To examine the global, regional, and national disease burden of neonatal jaundice.@*METHODS@#The 2019 Global Burden of Disease database was searched to collect incident cases/incidence and deaths/mortality of neonatal jaundice, as well as global socio-demographic index (SDI) and universal health coverage index (UHCI). The epidemiological trend of neonatal jaundice from 1990 to 2019 was analyzed. The correlations between incidence/mortality of neonatal jaundice and SDI and UHCI were evaluated.@*RESULTS@#From 601 681 in 1990 to 626 005 in 2019, with a 4.04% increase in global incident cases of neonatal jaundice. The overall age-standardized incidence rate exhibited an increase [estimated annual percent change=0.13 (95%CI: 0.03 to 0.23)] during this period. Additionally, deaths due to neonatal jaundice decreased by 58.83%, from 128 119 in 1990 to 52 742 in 2019. The overall age-standardized mortality rate showed a decrease [estimated annual percent change=-2.78 (95%CI: -3.00 to -2.57)] over the same period. Countries with lower SDI, such as India, Pakistan, and Nigeria, reported a higher proportion of neonatal morbidity and mortality. In 2019, a negative correlation was observed between estimated annual percent change in age-standardized mortality rate and SDI (ρ=-0.320, P<0.05) or UHCI (ρ=-0.252, P<0.05).@*CONCLUSIONS@#The global incidence of neonatal jaundice is on the rise, while the mortality rate is declining. The burden of neonatal jaundice is influenced by social development, economic factors, and the level of medical care.
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Recém-Nascido , Humanos , Carga Global da Doença , Icterícia Neonatal/epidemiologia , IncidênciaRESUMO
Hepatocelluar carcinoma presenting as a biliary duct tumor thrombus is a relatively rare entity, with poor prognosis. The primary clinical manifestation of this disease is obstructive jaundice, which can often be misdiagnosed. A 59-year-old female patient was admitted with sudden onset of abdominal pain. Laboratory tests suggested obstructive jaundice, and enhanced magnetic resonance imaging of the upper abdomen did not show obvious biliary dilatation. Endoscopic ultrasound and endoscopic retrograde cholangiopancreatography suggested an occupying lesion in the upper bile duct. SpyGlass and biopsy finally confirmed hepatocellular carcinoma with right hepatic duct tumor thrombus hemorrhage. The SpyGlass Direct Visualization System, as an advanced biliary cholangioscopy device, showed the advantages of single-person operation as well as easy access to and visualization of the lesion.
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Feminino , Humanos , Pessoa de Meia-Idade , Carcinoma Hepatocelular/diagnóstico por imagem , Icterícia Obstrutiva/etiologia , Neoplasias Hepáticas/diagnóstico por imagem , Ducto Hepático Comum/patologia , Trombose/complicações , Hemorragia/complicaçõesRESUMO
The growth environment of medicinal plants plays an important role in the formation of their medicinal quality. However, there is a lack of combined analysis studying the close relationship between the growth environment, chemical components, and related biological activities of medicinal plants. Therefore, this study investigated the effect of different soil moisture treatments on the efficacy to eliminate dampness and relieve jaundice and the flavonoid content of Sedum sarmentosum, and explored their correlation. The flavonoid content in the decoction of S. sarmentosum growing under field conditions with soil moisture levels of 35%-40%(T1), 55%-60%(T2), 75%-80%(T3), and 95%-100%(T4) was compared. The effects of these treatments on liver function parameters, liver inflammation, and oxidative damage in mice with dampness-heat jaundice were evaluated, and the correlation between pharmacological indicators and flavonoid content was analyzed. The results showed that the total flavonoid and total phenolic acid content in the decoction of S. sarmentosum were highest in the T1 treatment, followed by the T3 treatment. The content of quercetin, kaempferol, and isorhamnetin was highest in the T2, T1, and T3 treatments, respectively. Among the different moisture treatments, the T3 group of S. sarmentosum effectively reduced the levels of serum ALT, AKP, TBIL, DBIL, TBA, as well as hepatic TNF-α and IL-6 in mice with jaundice, followed by T2 treatment, especially in reducing AST level. The T4 treatment had the poorest effect. Correlation analysis showed a significant negative correlation between AST, ALT, AKP levels in mice and the total content of quercetin and the three flavonoids. MDA showed a significant negative correlation with the total flavonoid content and kaempferol. TNF-α exhibited a significant negative correlation with the content of isorhamnetin. In conclusion, S. sarmentosum growing under field conditions with a soil moisture level of 75%-80% exhibited the best efficacy to eliminate dampness and relieve jaundice. This study provides insights for optimizing the cultivation mode of medicinal plants guided by pharmacological experiments.