Budd-chiari syndrome causing acute liver failure: A multicenter case series.

Budd-Chiari syndrome (BCS) is a rare disease resulting from obstruction of the hepatic venous outflow tract that typically presents with abdominal pain, jaundice, and ascites without frank liver failure. However, BCS may also evolve more rapidly to acute liver failure (ALF). In this study, we describe the clinical features, treatment, and outcomes of ALF due to BCS and compare our results with those in the published literature. Twenty of the 2344 patients enrolled in the Acute Liver Failure Study Group (ALFSG) registry since 1998 presented with a clinical diagnosis of BCS. An additional 19 patients of ALF-BCS in the English language literature were reviewed and compared with the ALFSG cases. Most ALF-BCS patients were white (84%) and female (84%) in their fourth decade. A hypercoagulable state was noted in 63% of patients. BCS was diagnosed by Doppler ultrasonography or abdominal computed tomography in all patients. Liver biopsies (n = 6) all had evidence of severe pericentral necrosis. Treatments used included most commonly anticoagulation (71%), but also transjugular intrahepatic portosystemic shunt (TIPS; 37%) and orthotopic liver transplantation (37%). In-hospital mortality was approximately 60%. In conclusion, BCS is a rare cause of ALF and mandates prompt diagnosis and management for successful outcomes. Once the diagnosis is confirmed, prompt anticoagulation is recommended in conjunction with evaluation for malignancy or thrombophilic disorder. Mortality may have improved in recent years with use of TIPS and/or orthotopic liver transplantation compared with prior published reports. Liver Transplantation 23 135-142 2017 AASLD.

Hepatotoxicity due to hydroxycut: a case series.

OBJECTIVES: Muscletech Hydroxycut (Iovate Health Sciences Research, Oakville, Ontario, Canada) was a popular weight-loss supplement that was recalled by the manufacturer in May 2009 on the basis of reports of hepatotoxicity associated with this supplement. We sought to characterize the clinical presentation of Hydroxycut-associated liver injury and to adjudicate these cases for causal association with Hydroxycut. METHODS: We assessed the causality and grading of severity of liver injury using methodology developed by the Drug-Induced Liver Injury Network (DILIN) study. RESULTS: Eight patients who developed liver injury after taking Hydroxycut treated at different medical centers were identified. All were hospitalized, and three of eight patients required liver transplantation. Nine other cases with adequate clinical information were obtained from the FDA MedWatch database, including one fatal case of acute liver failure. Usual symptoms were jaundice, fatigue, nausea, vomiting, and abdominal pain. Most patients exhibited a hepatocellular pattern of injury. Adjudication for causality revealed eight cases as definite, five highly likely, two probable, and two were considered to be possible. CONCLUSIONS: Hydroxycut has been clearly implicated as a cause for severe liver injury that may lead to acute liver failure and death. Weight-loss supplements represent a class of dietary supplements that should be regarded as capable of causing severe hepatic toxicity when the usual causes of identified liver injury cannot be otherwise elucidated.

Portadores asintomáticos que serán sometidos a inmunosupresión / Asymptomatic carriers that will be subjected to immunosuppression

La reactivación del virus B en portadores crónicos asintomáticos, evidenciado como aumento en la alanino aminotransferasa (ALT) o aumento del ADN viral, se ha descrito en 20 a 50% de los pacientes que son sometidos a tratamiento inmunosupresor o quimioterapia por neoplasias. La mayoría de las ocasiones, las descompensaciones son asintomáticas, aunque pueden tener periodos de ictericia y descompensación severa. La reactivación ha demostrado ser mayor cuando esteroides son parte del régimen de tratamiento. Estudios no controlados han demostrado que el uso de lamivudina reduce la tasa de reactivación y las posibles complicaciones de la misma. Se recomienda que todo paciente que va a ser sometido a quimioterapia por neoplasia o tratamiento inmunosupresor sea tamizado por hepatitis B. En pacientes trasplantados, no hepático, hay estudios pequeños que demuestran que hay mayor mortalidad al reactivarse el virus. La mayoría de estos estudios se han realizado en pacientes con trasplante renal. No hay una recomendación normada sobre el manejo de estos pacientes.

The reactivation of HBV in asymptomatic carriers, evidenced as an increase in the alanine aminotransferasa (ALT) or increase in the viral DNA has been described in 20-50% of patients who are subjected to immunosuppressive or neoplasm chemotherapy. Most of the time, decompensations are asymptomatic, but they can show periods of jaundice and severe decompensation. Reactivation has shown to be higher when steroids are part of the treatment. Non-controlled studies have demonstrated that the use of lamivudine reduces reactivation rate and possible complications. It is recommended that any patient who will be subjected to neoplasm chemotherapy or immunosuppressive treatment must be sieved for hepatitis B. There are brief studies that demonstrate that there is higher morbidity when there is virus reactivation in those patients who have had non-hepatic transplants. Most research has been done in patients with renal transplants. There is not normative recommendation about the management of these patients.

Seroprevalence of dengue virus antibodies in asymptomatic Costa Rican children, 2002-2003: a pilot study.

OBJECTIVES: Since 1993 dengue has become more frequent in Costa Rica. Adults have been the most affected population, while children have remained virtually unharmed. So far no studies have investigated how many asymptomatic children have been affected by this virus. This pilot study documents the seroprevalence, measured as the presence of IgG antibodies, of dengue virus in asymptomatic children from two different geographical areas. METHODS: This descriptive, prospective epidemiologic study compared the presence of antibodies in children who live in a coastal region of a tropical country where dengue is endemic, and an inland area where dengue is not endemic. An enzyme-linked immunosorbent assay was used to test the serum for dengue virus IgG antibodies. None of the children had a prior history of dengue, fever, immunosuppressive therapy or underlying disease. RESULTS: During the period from July 2002 to July 2003, 103 children were recruited from each area. In the costal region we found a seroprevalence of 36.9%. In the inland area seroprevalence was 2.9%. CONCLUSIONS: We found a substantial number of asymptomatic infections in Costa Rican children. This greatly increases the risk of dengue hemorrhagic fever or dengue shock syndrome in these children, in whom previous dengue infection had gone undetected. Preventive efforts should be targeted at the costal region due to the higher prevalence in this area.

Seroprevalence of dengue virus antibodies in asymptomatic Costa Rican children, 2002-2003: a pilot study / La seroprevalencia de anticuerpos contra el virus del dengue en niños costarricenses asintomáticos, 2002-2003: estudio piloto

OBJECTIVES: Since 1993 dengue has become more frequent in Costa Rica. Adults have been the most affected population, while children have remained virtually unharmed. So far no studies have investigated how many asymptomatic children have been affected by this virus. This pilot study documents the seroprevalence, measured as the presence of IgG antibodies, of dengue virus in asymptomatic children from two different geographical areas. METHODS: This descriptive, prospective epidemiologic study compared the presence of antibodies in children who live in a coastal region of a tropical country where dengue is endemic, and an inland area where dengue is not endemic. An enzyme-linked immunosorbent assay was used to test the serum for dengue virus IgG antibodies. None of the children had a prior history of dengue, fever, immunosuppressive therapy or underlying disease. RESULTS: During the period from July 2002 to July 2003, 103 children were recruited from each area. In the costal region we found a seroprevalence of 36.9 percent. In the inland area seroprevalence was 2.9 percent CONCLUSIONS: We found a substantial number of asymptomatic infections in Costa Rican children. This greatly increases the risk of dengue hemorrhagic fever or dengue shock syndrome in these children, in whom previous dengue infection had gone undetected. Preventive efforts should be targeted at the costal region due to the higher prevalence in this area.

OBJETIVOS: Desde 1993, la frecuencia de dengue en Costa Rica ha venido aumentando. La población de adultos ha sido la más afectada, mientras que en los niños apenas se han presentado casos. Hasta el momento no se han realizado estudios para determinar cuántos niños asintomáticos se han visto afectados por el virus de la enfermedad. Este estudio piloto documenta la seroprevalencia de anticuerpos de tipo IgG contra el virus del dengue en niños asintomáticos procedentes de dos zonas geográficas distintas. MÉTODOS: En este estudio epidemiológico descriptivo y prospectivo se comparó la presencia de anticuerpos en niños que vivían en la zona costera de un país tropical donde el dengue es endémico, y en una zona del interior donde no lo es. Se usó inmunoadsorción enzimática para detectar IgG en el suero. Ninguno de los niños tenía antecedentes de dengue, enfermedad febril, tratamiento inmunosupresor o enfermedad subyacente. RESULTADOS: Durante el período transcurrido desde julio de 2003 hasta julio de 2003, se reunió a 103 niños de cada área. En la zona costera encontramos una seroprevalencia de IgG de 36,9 por ciento; en el interior, de 2,9 por ciento. CONCLUSIONES: Encontramos muchos casos de infección asintomática por el virus del dengue en niños costarricenses. Esto conlleva un riesgo elevado de fiebre hemorrágica del dengue o de síndrome de choque por dengue en estos niños en quienes la infección había pasado inadvertida. Es necesario tomar medidas preventivas en la región del litoral debido a la mayor prevalencia de la enfermedad en ella.

[Kikuchi-Fujimoto disease in pediatric population: a case report and a review of literature]. / Enfermedad de kikuchi-fujimoto en pediatría. Reporte de un caso y revisión de la literatura.

A 10 year-old child, with a history of a right cervical mass, is admitted to the Costa Rican National Children's Hospital for workup. The mass appeared approximately 4 weeks before admission. Laboratory tests were performed and malignity, infection and immunologic causes were ruled out. A biopsy was performed revealing granulomas characterized by central necrosis with abundant karyorrhexis, surrounded by histiocytes, lymphocytes and giant multinucleated cells, without neutrophils. Special stains showed no microorganisms. Once infectious and immunologic causes were excluded, and based on the biopsy's result, treatment was ruled out. Twelve months later, the patient is still asymptomatic; therefore, the diagnosis of a Kikuchi-Fujimoto syndrome was proposed. This report constitutes the first pediatric case diagnosed in our country.

Risk factors for Candida infections in a neonatal intensive care unit in Costa Rica.

OBJECTIVE: To identify potential risk factors associated with Candida infections and compare these risk factors between patients who both died and survived. STUDY DESIGN: A group of patients with positive Candida spp. blood cultures admitted to a neonatal intensive care unit (NICU) in Costa Rica between January 1994 and December 1998. Cases were identified through a computerized search of the microbiology laboratory's database on blood cultures. RESULTS: One hundred and ten newborns were identified. Sixty-six patients (60%) were male; 46 (62%) were preterm infants. Thirty-seven (34%) patients died. Twenty (54%) of them died within three days of the candidemia diagnosis and 17 had disseminated Candida infection on autopsy. Candida albicans and Candida tropicalis were isolated in 90% and 10% of blood cultures, respectively. Mean +/- SD (range) number of days from admission to NICU to the initial positive blood culture were 13.5 +/- 8.5 (1-30) days. Most patients had at least two positive blood cultures (range 1-8). Median (range) days for the sterilization of blood culture were four (1-25) days. Significant differences in survival were identified in patients with axillary-inguinal lesions, apnea and seizures. CONCLUSIONS: Invasive fungal infections are frequent in NICU. Future case-control prospective studies should be carried out to confirm the findings from this report.

Enfermedad de Kikuchi-Fujimoto en pediatría: Reporte de un caso y revisión de la literatura / Kikuchi-Fijimoto disease in pediatric population: a case report and a review of literature

Un niño de 10 años de edad fue ingresado al Hospital Nacional de Niños "Dr. Carlos Luis Sáenz Herrera" con una masa cervical derecha de aproximadamente cuatro semanas de evolución. Se realizaron pruebas de gabinete y laboratorio que descartaron malignidad, infecciones o procesos inmunológicos. Una biopsia a cielo abierto reportó la presencia de granulomas con necrosis central, abundante cariorrexis, histiocitos, linfocitos y células gigantes multinucleadas, sin neutrófilos. Las tinciones especiales no mostraron ningún microorganismo. En valoraciones posteriores al mes, a los seis y 12 meses el niño continuaba asintomático. Por no tener etiología precisa, no se administró ningún medicamento. Con base en la evolución clínica, los hallazgos de la biopsia y al excluirse causas infecciosas, tumorales e inmunológicas, se concluyó que el paciente presentó una enfermedad de Kikuchi Fujimoto, siendo el primer caso pediátrico reportado en nuestro país.

A 10 year-old child, with a history of a right cervical mass, is admitted to the Costa Rican National Children's Hospital for workup. The mass appeared approximately 4 weeks before admission. Laboratory tests were performed and malignity, infection and immunologic causes were ruled out. A biopsy was performed revealing granulomas characterized by central necrosis with abundant karyorrhexis, surrounded by histiocytes, lymphocytes and giant multi-nucleated cells, without neutrophils. Special stains showed no microorganisms. Once infectious and immunologic causes were excluded, and based on the biopsy's result, treatment was ruled out. Twelve months later, the patient is still asymptomatic; therefore, the diagnosis of a Kikuchi-Fujimoto syndrome was proposed. This report constitutes the first pediatric case diagnosed in our country.

Albendazole therapy for Microsporidium diarrhea in immunocompetent Costa Rican children.

BACKGROUND: Microsporidia comprise a large group of obligate intracellular parasites. Although several species have emerged as opportunistic agents in immunocompromised patients, cases have also been reported in immunocompetent patients. METHODS: During 21 months, we conducted a randomized, open label study in 200 children hospitalized with Microsporidium subacute diarrhea. Patients had prolonged, nonbloody, nonmucoid diarrhea, with > or =10 bowel movements/day for >10 days. Patients had negative rotavirus tests, bacterial stool cultures and sugar reductive tests in feces. Stool examinations to rule out Giardia intestinalis and intestinal nematodes were performed. Microsporidium was identified by light microscopy in stool specimens stained with Giemsa and Weber techniques. One hundred patients received oral albendazole (15 mg/kg/day twice a day for 7 days) and 100 patients received only supportive therapy. RESULTS: Both groups were comparable regarding gender, age, clinical evolution and weight. Median (range) age was 24 (6-36) months. All children had abdominal pain, nausea, vomiting and anorexia. The primary endpoint, defined as clinical improvement within 48 h of initial therapy, occurred in 95 and 30% of the albendazole-treated and untreated patients, respectively (P < 0.05). There was a significant decrease in stool frequency, reduction of clinical findings and decrease in Microsporidium parasites in stool specimens of children treated with albendazole compared with the untreated group. Median (range) duration of diarrhea was 5 (3-7) days in albendazole-treated patients versus 10 (8-15) days in untreated patients (P < 0.05). CONCLUSION: Albendazole therapy was effective in improving the clinical manifestations and decreasing the duration of the illness of children with diarrhea caused by Microsporidium.

Systemic host inflammatory and coagulation response in the Dengue virus primo-infection.

BACKGROUND: Dengue virus infection has been rising in tropical countries. Clinical manifestations range from fever and general malaise to hemorrhagic manifestations and death. The role of endothelial damage and cytokines has not been well established for dengue infection. OBJECTIVE: Determine the profile of the pro-inflammatory cytokines and several markers of coagulopathy of dengue infection. METHODS: Patients admitted between September 2000 and April 2001, who met the WHO dengue diagnosis criteria, were enrolled. Blood samples were collected at 0, 6, 12, 24, 48, 72 h and 5 and 7 days after hospitalization. Profile of pro-inflammatory cytokines, markers of coagulopathy, protein C, protein S, d-dimer, prothrombin time, activated partial thromboplastin time, fibrinogen and activated protein C levels were determined. RESULTS: Thirty-three patients were enrolled. Median (range) age was 31 (13-70) years; 51.5% (17/33) were female. Ten of 33 (30%) presented with hemorrhagic manifestations. Patients were classified: Grade 1: 23/33 (70%), Grade II: 10/33 (30%). At study entry IL-6 was the most elevated, followed by IL-8 and TNF alpha. IL-10 was not elevated. No significant differences (P < 0.05) were demonstrated in the levels of any of the haemostatic or cytokine markers by disease severity (Grade I versus Grade II patients). CONCLUSION: The systemic host inflammatory and coagulation activation response occurs early in patients with dengue viral infection in the absence of severe hemorrhagic manifestations, and provides the basis for considering future clinical study in the use of recombinant human activated protein C to treat patients with severe sepsis from dengue infection.