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BACKGROUND: Carbapenem-resistant Enterobacteriaceae (CRE) are an emerging problem in the paediatric population worldwide with high mortality rates in bloodstream infection (BSI). OBJECTIVES: To evaluate predictors of 30 day mortality in CRE BSI in a paediatric cohort. METHODS: A retrospective observational single-centre study (December 2005-August 2018) was conducted. Cases of CRE BSI in children 0 to 16 years were included. Microbiological identification (MALDI Biotyper) and antimicrobial susceptibility testing (Vitek2® and MicroScan panel NBC44) according to EUCAST breakpoints were performed. PCR OXVIKP® was used to confirm carbapenemase genes (OXA-48, VIM, KPC, NDM). Demographic characteristics, underlying diseases, source of bacteraemia, antimicrobial therapy and outcomes were collected from medical records. Survival analysis to establish predictors of 30 day mortality was performed. RESULTS: Thirty-eight cases were included; 76.3% were hospital-acquired infections and 23.7% related to healthcare. All patients had at least one underlying comorbidity and 52.6% were recipients of an organ transplant. VIM carbapenemase was the predominant mechanism (92.1%). Previous CRE colonization or infection rate was 52.6%. Intestinal tract (26.3%) and vascular catheter (21.1%) were the most common sources of infection. Crude mortality within 30 days was 18.4% (7/38); directly related 30 day mortality was 10.5%. Conditions associated with an increment in 30 day mortality were intensive care admission and inadequate empirical therapy (P < 0.05). Combination-antibiotic targeted treatment and a low meropenem MIC were not related to improved survival. CONCLUSIONS: CRE BSI mortality rate is high. The most important factor related to 30 day survival in our CRE BSI cohort in children was empirical treatment that included at least one active antibiotic.
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Enterobacteriaceae Resistentes a los Carbapenémicos , Infecciones por Enterobacteriaceae , Sepsis , Antibacterianos/farmacología , Antibacterianos/uso terapéutico , Proteínas Bacterianas/genética , Carbapenémicos/farmacología , Niño , Infecciones por Enterobacteriaceae/tratamiento farmacológico , Infecciones por Enterobacteriaceae/epidemiología , Humanos , Estudios Retrospectivos , Sepsis/tratamiento farmacológico , beta-Lactamasas/genéticaRESUMEN
Non-Hodgkin lymphoma (NHL) is a hematological tumor caused by abnormal lymphoid proliferation. NHL can arise in any part of the body, including central nervous system (CNS). However, pituitary involvement is a quite rare presentation. The diffuse large B-cell lymphoma (DLBCL) is the most common subtype when pituitary is infiltrated. Here, we report a case of pituitary infiltration of NHL DLBCL type in a woman with hypopituitarism and an infundibulum-hypophysitis-like image on magnetic resonance imaging (MRI). A female aged 64 years, complained of dyspepsia, fatigue, weight loss and urine volume increment with thirst. Endoscopy and gastric biopsy confirmed diffuse large B-cell lymphoma. Treatment with chemotherapy using R-CHOP was initiated. During her hospitalization, hypotension and polyuria were confirmed. Hormonal evaluation was compatible with central diabetes insipidus and hypopituitarism. Simple T1 sequence of MRI showed thickening of the infundibular stalk with homogeneous enhancement. After lumbar puncture analysis, CNS infiltration was confirmed showing positive atypical lymphocytes. Pituitary and infundibular stalk size normalized after R-CHOP chemotherapy treatment. In conclusion, pituitary infiltration of NHL with infundibular-hypophysitis-like image on MRI is a rare finding. Clinical picture included hypopituitarism and central diabetes insipidus. Diagnosis should be suspected after biochemical analysis and MRI results. Treatment consists of chemotherapy against NHL and hormonal replacement for pituitary dysfunction. LEARNING POINTS: Pituitary infiltration by lymphoma can present with signs and symptoms of panhypopituitarism and diabetes insipidus.MRI findings can resemble an autoimmune hypophysitis.Patients can recover pituitary function as well as normalization of MRI after chemotherapy treatment.
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Since 1992, the Spanish and Portuguese-Speaking Working Group of the ISFG (GHEP-ISFG) has been organizing annual Intercomparison Exercises (IEs) coordinated by the Quality Service at the National Institute of Toxicology and Forensic Sciences (INTCF) from Madrid, aiming to provide proficiency tests for forensic DNA laboratories. Each annual exercise comprises a Basic (recently accredited under ISO/IEC 17043: 2010) and an Advanced Level, both including a kinship and a forensic module. Here, we show the results for both autosomal and sex-chromosomal STRs, and for mitochondrial DNA (mtDNA) in two samples included in the forensic modules, namely a mixture 2:1 (v/v) saliva/blood (M4) and a mixture 4:1 (v/v) saliva/semen (M8) out of the five items provided in the 2014 GHEP-ISFG IE. Discrepancies, other than typos or nomenclature errors (over the total allele calls), represented 6.5% (M4) and 4.7% (M8) for autosomal STRs, 15.4% (M4) and 7.8% (M8) for X-STRs, and 1.2% (M4) and 0.0% (M8) for Y-STRs. Drop-out and drop-in alleles were the main cause of errors, with laboratories using different criteria regarding inclusion of minor peaks and stutter bands. Commonly used commercial kits yielded different results for a micro-variant detected at locus D12S391. In addition, the analysis of electropherograms revealed that the proportions of the contributors detected in the mixtures varied among the participants. In regards to mtDNA analysis, besides important discrepancies in reporting heteroplasmies, there was no agreement for the results of sample M4. Thus, while some laboratories documented a single control region haplotype, a few reported unexpected profiles (suggesting contamination problems). For M8, most laboratories detected only the haplotype corresponding to the saliva. Although the GHEP-ISFG has already a large experience in IEs, the present multi-centric study revealed challenges that still exist related to DNA mixtures interpretation. Overall, the results emphasize the need for further research and training actions in order to improve the analysis of mixtures among the forensic practitioners.
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Cromosomas Humanos X , Cromosomas Humanos Y , Dermatoglifia del ADN , ADN Mitocondrial/genética , Laboratorios/normas , Repeticiones de Microsatélite , Amelogenina/genética , Análisis Químico de la Sangre , Femenino , Genética Forense , Marcadores Genéticos , Haplotipos , Humanos , Masculino , Saliva/química , Semen/químicaRESUMEN
UNLABELLED: We examined whether the results in living-related hepatic transplantation (LRLT) are better than those from a cadaveric donor (CDLT). MATERIAL AND METHODS: The last 27 consecutive LRLT, performed from 1998 to 2005, were compared with 27 CDLT matched for age, weight, date, and diagnosis. Grafts in LRLT group were left lateral segment (n = 22), left lobe (n = 3), and right lobe (n = 2). In the CDLT group, the grafts were split in situ (n = 10), hepatic reduction (n = 9) and whole liver (n = 8). We analyzed the actuarial survivals (grafts and children), retransplantation, primary nonfunction, initial graft malfunction (liver enzymes >2000 U/L), surgical complications, rejection, and resource consumption. RESULTS: Patient survivals at 6 months, 1 year, and 5 years were 100%, 96%, and 96% in LRLT and 100%, 100%, and 100% in CDLT (P = NS). Graft survivals were 93%, 89%, and 89% versus 96%, 96%, and 96%, respectively (P = NS). Complications were biliary complications (LRLT, 25% vs CDLT, 3%; P = .021); portal vein thrombosis (LRLT, 7% vs CDLT, 3%; NS), and hepatic artery thrombosis (LRLT, 0% vs CDLT, 3%; NS). The overall incidence of acute rejection was slightly higher (NS) in LRLT (LRLT, 18% vs CDLT, 11%; NS). Liver enzyme levels were higher in the CDLT group, but initial malfunction rate was not statistically different. Regarding resource consumption: blood product needs were higher in LRLT (P < .05) and hospital stay and ICU stay were longer, although not significantly, among LRLT. CONCLUSIONS: The results in LRLT among children are similar to those obtained in CDLT. We found a trend towards less initial graft malfunction in LRLT. Blood product needs were higher in LRLT. Hospital and ICU stay were longer, but not significantly different in LRLT. The benefits of LRLT are saving a scarce resource: a cadaveric donor liver graft.
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Hepatopatías/cirugía , Trasplante de Hígado/fisiología , Donadores Vivos , Donantes de Tejidos , Peso Corporal , Cadáver , Preescolar , Familia , Estudios de Seguimiento , Supervivencia de Injerto , Humanos , Trasplante de Hígado/mortalidad , Reoperación/estadística & datos numéricos , Análisis de Supervivencia , Factores de Tiempo , Resultado del TratamientoRESUMEN
The results of the isolated intestinal grafts were compared with those of composite grafts (intestinal graft + liver) in a series of 18 transplantations performed in 17 children; 5 isolated intestinal grafts, 12 hepatointestinal grafts, and 1 multivisceral graft. Causes of intestinal failure were short bowel syndrome (n = 13), motility disorders (n = 2) and congenital epithelial disorders (n = 2). Transplantation was indicated due to end-stage liver disease (n = 14), loss of venous access (n = 2), untreatable diarrhea (n = 1) and high morbidity associated with a poor quality of life (n = 1). Six children, all with a composite graft, died after transplantation due to lymphoma (n = 2), sepsis (n = 1); intraabdominal bleeding (n = 1); pneumonia (n = 1); and overwhelming adenoviral infection (n = 1). Digestive autonomy was achieved in 16 of 18 grafts, the 11 surviving children are free of parenteral nutrition with a reasonably good quality of life. In conclusion, intestinal transplantation is a viable therapeutic alternative for children with permanent intestinal failure. The results of transplantation with an isolated intestine are clearly better that those with a composite graft.
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Resinas Compuestas/uso terapéutico , Enfermedades Intestinales/cirugía , Intestinos/trasplante , Síndrome del Intestino Corto/cirugía , Adolescente , Adulto , Preescolar , Femenino , Humanos , Lactante , Enfermedades Intestinales/mortalidad , Enfermedades Intestinales/terapia , Masculino , Síndrome del Intestino Corto/mortalidad , Síndrome del Intestino Corto/terapia , Análisis de Supervivencia , Resultado del TratamientoRESUMEN
The aim of this study was to analyze factors affecting mobilization and engraftment in 40 children undergoing autologous peripheral blood progenitor cell transplantation for different malignancies: 19 patients with haematological malignancies and 21 patients with solid tumors. Patients received 4-5 days of rhG-CSF (12 micrograms/kg/day) subcutaneously. Apheresis was performed by continuous flow blood cell separation beginning on the fifth day of rhG-CSF. For patients weighing < or = 25 kg, the extracorporeal line was primed with irradiated red blood cells. After myeloablative conditioning regimens, patients were grafted with 7.21 +/- 7.8 x 10(6)/kg CD34+ cells. Days to achieve an absolute neutrophil count > 0.5 x 10(9)/1 and a platelet count > 20 x 10(9)/1 without platelet support were 9.50 +/- 1.2 (range 7-13) and 18.1 +/- 8.3 (range 9-37), respectively. The number of CD34+ cells infused was highly correlated with engraftment kinetics (P = 0.0001). The patient's body weight and the number of previous chemotherapy courses had a negative influence on CD34+ cells collected.
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Antígenos CD34/sangre , Trasplante de Células Madre Hematopoyéticas , Células Madre Hematopoyéticas/inmunología , Adolescente , Recuento de Células Sanguíneas , Eliminación de Componentes Sanguíneos , Recuento de Células , Niño , Preescolar , Femenino , Supervivencia de Injerto , Factor Estimulante de Colonias de Granulocitos/administración & dosificación , Células Madre Hematopoyéticas/citología , Humanos , Lactante , Cinética , Leucemia/sangre , Leucemia/terapia , Linfoma/sangre , Linfoma/terapia , Masculino , Neoplasias/sangre , Neoplasias/terapia , Proteínas Recombinantes , Trasplante AutólogoRESUMEN
Large volume leukapheresis (LVL) defined as the processing of greater than three volumes of blood in a single session for peripheral blood stem cell (PBSC) collection was performed in 27 children, aged from 1 to 15 years, with various malignancies. Harvesting of PBSC was started after 4 days of cytokine (G-CSF 12 micrograms/kg s.c.) alone. With the exception of two cases the rest (92.5%) needed only a single apheresis to yield the minimum number of cells required for transplantation. No consistent side-effects were observed and the LVL were well tolerated by children. An average of 7.6 x 10(8) MNC/kg, 6.1 x 10(6)/kg CD34+ and 2.1 x 10(4)/kg CFU-GM were harvested. To date, 19 patients have been transplanted after myeloablative treatment and sustained engraftment was achieved in all cases. LVL can be safely and easily performed in children allowing adequate PBSC collection for transplantation with prompt hematological engraftment.
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Separación Celular/métodos , Células Madre Hematopoyéticas , Leucaféresis , Adolescente , Niño , Preescolar , Femenino , Factor Estimulante de Colonias de Granulocitos/uso terapéutico , Trasplante de Células Madre Hematopoyéticas , Humanos , Lactante , MasculinoRESUMEN
Neurotrophic factors modulate synaptic plasticity through mechanisms that include regulation of membrane ion channels and neurotransmitter receptors. Recently, it was shown that insulin-like growth factor I (IGF-I) induces depression of AMPA-mediated currents without affecting NMDA-receptor function in neurons. We now report that IGF-I markedly potentiates the kainate-preferring ionotropic glutamate receptor in young cerebellar granule neurons expressing functional kainate-, but not AMPA-mediated currents. Potentiation of kainate responses by IGF-I is blocked by wortmannin, a phosphatidylinositol 3-kinase (P13K) inhibitor, indicating a role for this kinase in the effect of IGF-I. These results reinforce the notion that modulation of ionotropic glutamate receptors are involved in the regulatory actions of IGF-I on neuronal plasticity.
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Factor I del Crecimiento Similar a la Insulina/farmacología , Plasticidad Neuronal/efectos de los fármacos , Fosfatidilinositol 3-Quinasas/fisiología , Receptores de Ácido Kaínico/fisiología , Transducción de Señal/fisiología , Androstadienos/farmacología , Animales , Inhibidores Enzimáticos/farmacología , Plasticidad Neuronal/fisiología , Ratas , Ratas Wistar , WortmaninaRESUMEN
Fibers coming from the olfactory epithelium and entering the olfactory bulb expressed peripherin. These were not sensory olfactory fibers because: (1) they did not form glomeruli, (2) they did not express olfactory marker protein (OMP), (3) they entered deeply into the olfactory bulb, reaching its white matter. Cells and fibers in the olfactory bulb expressing luteinizing hormone-releasing hormone (LHRH) differed from those expressing peripherin because LHRH and peripherin did not coexist and the distributions of LHRH-positive and peripherin-positive fibers were found to be different.
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Proteínas del Ojo/metabolismo , Hormona Liberadora de Gonadotropina/metabolismo , Proteínas de Filamentos Intermediarios/metabolismo , Glicoproteínas de Membrana , Fibras Nerviosas/fisiología , Proteínas del Tejido Nervioso , Vías Olfatorias/fisiología , Animales , Carbocianinas , Inmunohistoquímica , Fibras Nerviosas/metabolismo , Vías Olfatorias/metabolismo , Vías Olfatorias/ultraestructura , Periferinas , Ratas , Ratas WistarRESUMEN
In mammals LHRH (luteinizing hormone-releasing hormone) is synthesized and released by a set of neurons that have their embryonic origin in the olfactory placode. We have observed that, besides their classical location, LHRH fibers can also be seen in the optic nerve and optic chiasm. Some LHRH fibers could also be traced in the optic tract. The possible course of these projections, and their functional significance are discussed.
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Hormona Liberadora de Gonadotropina/análisis , Quiasma Óptico/química , Nervio Óptico/química , Factores de Edad , Animales , Femenino , Masculino , Ratas , Ratas Wistar , Vías VisualesRESUMEN
AIM: The aim of this study was to analyze the results of living donor in a pediatric liver transplantation program. PATIENTS: Twenty-six living donor liver transplantations were performed in children from 0.5 to 14.8 years of age. The main indication was biliary atresia (72%) followed by tumors (2 hepatoblastomas and 1 hepatocarcinoma). Left lateral segments were used in 23 (1 transformed into a monosegment), 1 left lobe was used in 1, and right lobes were used in 2. Arterial reconstruction employed saphenous venous grafts in the first 3 cases and end-to-end anastomoses with a microsurgical technique in the following 22 cases. RESULTS: There has been no major morbidity in the donors, with a median hospitalization of 6 days. Four grafts have been lost; 2 in the first 3 cases. In only 1 case, the graft loss was related to the procedure saphenous venous graft thrombosis). Early biliary complications were frequent (23%). Six month, 1 year, and 5 year graft and patient survival rates were 91%, 85%, and 85% and 100%, 96%, and 96%, respectively. CONCLUSIONS: Living donor liver transplantation is an excellent option for transplantation in children.
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Hepatopatías/cirugía , Trasplante de Hígado/fisiología , Donadores Vivos/estadística & datos numéricos , Adolescente , Niño , Preescolar , Hepatectomía/métodos , Humanos , Lactante , Hepatopatías/clasificación , Complicaciones Posoperatorias/epidemiología , Recolección de Tejidos y Órganos , Resultado del TratamientoRESUMEN
PURPOSE: The aim of this study was to assess the long-term survival rate in children who have undergone orthotopic liver transplantation (OLT) in the last 13 years. METHODS: The records of 198 consecutive patients under 18 years of age who underwent 249 OLTs between 1986 and 1998 were reviewed. Actuarial patient survival rates were assessed at 1, 3, 5, and 10 years in the whole series, in the last 5 years, and in patients surviving more than 1 year. Age, weight, and indications were analyzed, as well as type and incidence of posttransplant complications. The median follow-up period was 41 months (0 to 154 months). RESULTS: Biliary atresia was the most common indication (41.9%) followed by alpha-1 antitrypsin deficiency (8.1%), Alagille syndrome (7.6%), and fulminant hepatic failure (6.6%). One hundred forty-six patients (58.6%) were below 5 years, and 46 patients were (18.5%) younger than 1 year at operation. Sixty-eight patients (27.3%) weighed less than 10 kg. One hundred seventy whole organs and 70 reduced, 5 living-related donor, and 4 split-liver allografts were used. Hepatic artery thrombosis (n = 18), primary nonfunction (n = 15), and chronic rejection (n = 14) were the most common causes for allograft failure. Fourteen patients (7%) had posttransplant lymphoproliferative disorders (PTLD) at a median time of 28 months (4 to 124 months) postoperation (3 died). The 1-, 3-, 5-, and 10-year actuarial patient survival rates are 80%, 76%, 74%, and 74%, respectively; over the last 5 years it is 88% at 1 year and 82% at 3 and 5 years. For patients surviving more than 1 year, 3-, 5-, and 10-year actuarial survival rates are 95%, 93%, and 93%, respectively. CONCLUSIONS: (1) Overall results of OLT improve with increasing experience. (2) Children who survive more than 1 year after OLT have an excellent prognosis, although long-term complications of immunosuppression can be expected.
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Trasplante de Hígado/mortalidad , Niño , Preescolar , Rechazo de Injerto , Humanos , Lactante , Trasplante de Hígado/efectos adversos , Trastornos Linfoproliferativos/etiología , Complicaciones Posoperatorias , Tasa de SupervivenciaRESUMEN
Familial hypercholesterolemia is the result of mutations in the gene that encodes the synthesis of the cellular receptor for low density lipoprotein (LDL). In the homozygous form of the disease (HFHC), cellular LDL receptors either do not form, or, when present, cannot bond LDL and mediate its cellular uptake LDL, and the cholesterol that it transports accumulate in plasma, producing severe premature atherosclerosis and death from coronary artery disease usually before the age of 20. Currently, the only effective treatment is liver transplantation, which, alone or in association with medications, normalizes plasma cholesterol levels. The authors report the cases of 2 siblings with HFHC who underwent portocaval shunt at the ages of 2.5 and 1.5 years, respectively. Portocaval shunt produced an immediate, but insufficient decrease in cholesterol (by 40% and 35%, respectively), leaving them with cholesterol concentrations of about 500 mg/dL. One year later they each underwent ileal bypass without obtaining any significant response. Liver transplantation at the ages of 18 and 16 years, respectively, reduced plasma cholesterol concentrations to 129 and 225 mg/dL, respectively. The earlier operations seriously increased the technical difficulty of liver transplantation and did not produce a favorable effect on the natural course of the disease, so portocaval shunt and ileal bypass are not indicated in HFHC, not even for the purpose of delaying liver transplantation.
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Hiperlipoproteinemia Tipo II/cirugía , Trasplante de Hígado , Adolescente , LDL-Colesterol/sangre , Femenino , Humanos , Hiperlipoproteinemia Tipo II/sangre , Hiperlipoproteinemia Tipo II/genética , Derivación Yeyunoileal , Trasplante de Hígado/métodos , Masculino , Derivación Portocava QuirúrgicaRESUMEN
PURPOSE: The aim of this study was to review the biliary complications occurring in late follow-up after liver transplantation in children. METHODS: The medical records of 135 children who received orthotopic liver transplantations (OLT) and had graft survival of more than 1 year were reviewed. Technical variants using a reduced-size graft were applied in 32 (23.7%). For biliary reconstruction, 15 patients had choledochocholedochostomy and 120 a Roux-en-Y loop. Biliary reoperation in the early post-OLT period was needed in 24 patients (17.7%). Routine checking of liver function and duplex Doppler ultrasonography (DDS) were performed during the follow-up period, which averaged 58 months. Late biliary complication was defined as that occurring after the first hospital discharge. RESULTS: Late biliary complications occurred in 18 children (13.3%); 16 showed symptoms or analytical disturbances in liver function tests. The Diagnoses included uncomplicated cholangitis (n = 6), anastomotic biliary stricture (n = 7), ischaemic damage of the biliary tree (n = 3) including one late (28 months) hepatic artery thrombosis leading to an intrahepatic biloma. and bile leak after T-tube removal (n = 2). The six children with uncomplicated cholangitis had no repeat episodes in follow-up despite persistent aerobilia. Six patients affected by anastomotic strictures were treated successfully with percutaneous dilatation and, if present, stone removal. Persisting dysfunction and cholangitis occurred in one case affected by ischaemic biliary disease. Biliary leaks after T tube removal settled spontaneously. Risk factors for late biliary complications were determined. There was no relation to the cold ischaemia time, type of graft or biliary reconstruction, or previous early post-OLT biliary reoperation. Aerobilia (affecting 21.5% of OLT patients) was related to cholangitis (P = .001). CONCLUSIONS: Anastomotic strictures, reflux of intestinal contents via the Roux-en-Y loop, and residual ischaemic damage led to late biliary complications in 12% of paediatric OLT patients. Evidence of biliary dilatation on DDS may be delayed in anastomotic strictures; in these cases the results of percutaneous treatment were excellent. Children with aerobilia have and increased risk of cholangitis.
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Enfermedades de las Vías Biliares/epidemiología , Trasplante de Hígado , Complicaciones Posoperatorias/epidemiología , Análisis de Varianza , Enfermedades de las Vías Biliares/diagnóstico por imagen , Enfermedades de las Vías Biliares/cirugía , Niño , Estudios de Seguimiento , Humanos , Pruebas de Función Hepática , Complicaciones Posoperatorias/diagnóstico por imagen , Complicaciones Posoperatorias/cirugía , Modelos de Riesgos Proporcionales , Reoperación , Factores de Riesgo , Resultado del Tratamiento , UltrasonografíaRESUMEN
The authors compared the results of 48 orthotopic liver transplantations (OLT) in which revascularization was achieved with a conduit interposed between the receptor aorta and the graft (vascular graft [VG] group) with those obtained for 56 OLT performed during the same period (1991 to 1994) in which end-to-end anastomosis (EEA) of the hepatic arteries or celiac trunk was used (EEA group). In the VG group, the interposed conduits were the cadaveric iliac artery (37) the living-donor saphenous vein (3), or nonthrombosed conduits from previous transplants (8) (7 iliac arteries, 1 saphenous vein). There were significant differences between the two groups with respect to recipient age, recipient weight, the retransplant:first transplant ratio, the number of emergency transplantations, the use of reduced-size grafts, and intraoperative transfusion requirements. Twenty-nine grafts in the VG group (60.4%) and 43 in the EEA group (76.7%) currently are functioning. The actuarial 3-year graft survival rates are 60% and 71.5% for the VG and EEA groups (P < .05), respectively. The rate of arterial thrombosis did not differ between the two groups. The authors conclude that, although EEA of the hepatic artery is still the preferred revascularization technique for OLT, revascularization of the liver graft by conduit interposition is safe when EEA is not possible. Reutilization of the interposed conduit during retransplantation proved to be safe in the absence of hepatic artery thrombosis.
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Anastomosis Quirúrgica/métodos , Prótesis Vascular , Arteria Celíaca/cirugía , Arteria Hepática/cirugía , Arteria Ilíaca/trasplante , Trasplante de Hígado/métodos , Vena Safena/trasplante , Aorta Abdominal/cirugía , Niño , Preescolar , Femenino , Oclusión de Injerto Vascular/cirugía , Humanos , Lactante , Masculino , Complicaciones Posoperatorias/cirugía , ReoperaciónRESUMEN
Orthotopic liver transplantation (OLT) is nowadays accepted as the best therapy for end-stage liver disease. The difficulties involved are even greater in children than in adults, and it is debatable whether exclusively pediatric programs are warranted. The aim of this paper is to analyze our experience at the Children's Hospital "La Paz", with the first consecutive 100 OLT in children, 61% of whom weighed less than 20 kg. Since 1986, 220 pediatric patients were evaluated as candidates, 100 OLT were performed in 78 patients and 13 died on the waiting list, currently maintained below 15 cases. Indications were: cholestasis (45), metabolic disease (18), fulminant hepatic failure (3), primary liver tumors (2) and cirrhosis (10). Mean age was 66 months (range = 7 to 216) with a mean weight of 21 kg (range = 6 to 60), twenty patients weighed less than 13 kilograms. OLT was performed by standard technique. Reduced or segmental grafts were necessary in 8 instances. Twenty-two patients were retransplanted and 2 received three grafts. Indications for retransplantation were: hepatic artery thrombosis (8), primary nonfunction (4), chronic rejection (7), portal thrombosis (2) and Budd-Chiari recurrence (1). Acute rejection was observed in 52 patients, and eight cases developed a chronic rejection. These episodes were treated with "bolus" of steroids, monoclonal antibodies (OKT-3) and FK-506. Surgical complications included: hepatic artery thrombosis 12%, portal vein thrombosis 3% and biliary fistula or stenosis 13%. The incidence of primary non-function was 7%. Actuarial survival rate at 5 years was 75%.(ABSTRACT TRUNCATED AT 250 WORDS)
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Trasplante de Hígado , Adolescente , Atresia Biliar/cirugía , Niño , Preescolar , Colestasis/cirugía , Rechazo de Injerto , Encefalopatía Hepática/cirugía , Humanos , Lactante , Cirrosis Hepática/cirugía , Neoplasias Hepáticas/cirugía , Trasplante de Hígado/mortalidad , Enfermedades Metabólicas/cirugía , Complicaciones Posoperatorias , Reoperación , Tasa de SupervivenciaRESUMEN
In spite of the increased number of organ donations, the amount of livers available for pediatric patients does not meet requirements. In an attempt to expand the pool of grafts, several techniques of size reduction (RST) have been developed: reduced-liver transplantation (RLT), liver segment transplantation (LST), "split" liver transplantation (SLT) and living-donor liver transplantation (LDLT). The aim of this work is to study the contribution of these techniques to reduction of mortality in the waiting list and the increased risks that we inflict to our patients by using these procedures. We report our experience on RST in the last nine years (23 RLT, 5 LST, 2 SLT and 3 LDLT). More than a half of them were performed in an emergency. Overall survival in children with RST (67%) is similar to that of patients receiving a "whole graft" (74%). We did not find significant differences in the incidence of graft-related complications, only reintervention for abdominal bleeding was needed more often in the RST group (30%) than in whole graft group (24%). Our data confirm the safety of the RST procedures in the pediatric liver transplantation programs and they decrease the waiting-list mortality (2.5%) among the pediatric patients.
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Atresia Biliar/cirugía , Fallo Hepático/cirugía , Trasplante de Hígado/métodos , Adolescente , Adulto , Atresia Biliar/mortalidad , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Hígado/patología , Fallo Hepático/mortalidad , Masculino , Tamaño de los Órganos/fisiología , Complicaciones Posoperatorias/mortalidad , Complicaciones Posoperatorias/cirugía , Reoperación , Tasa de Supervivencia , Resultado del TratamientoRESUMEN
A series of three cases of villous adenoma of the papilla of Vater is reported. In two cases, the pathological diagnosis made before, during and after surgery was of benign villous adenoma. In the third case, the diagnosis made before and during surgery was of adenoma but in the postsurgical examination a macro-invading carcinoma was found. Further endoscopic exploration revealed a recurrent villous adenoma and further surgery was carried out. Total resection, rather than a duodenopancreatectomy, was performed due to the length of time from detection of the recurrence until the second operation as well as to the small size of the recurrent tumor in the surgical examination.
Asunto(s)
Adenoma Velloso/patología , Ampolla Hepatopancreática , Neoplasias del Conducto Colédoco/patología , Adenoma Velloso/cirugía , Anciano , Ampolla Hepatopancreática/cirugía , Neoplasias del Conducto Colédoco/cirugía , Femenino , Humanos , Persona de Mediana Edad , Recurrencia Local de NeoplasiaRESUMEN
OBJECTIVE: To examine the incidence of obstetric complications in fetuses with unrecognized chromosomal anomalies compare with those in which the diagnosis was known before hand. METHODS: All cases followed at a private facility in San Juan, PR during the time from January 1993 through February 1997 were evaluated in terms of gestational age and method of diagnosis and eventual pregnancy outcome. RESULTS: There where 9 cases of chromosomal anomalies documented by karyotype analysis among 1377 (0.65%). Among this group, 5 cases were detected by a combination of maternal serum screening, analysis of risk factors and sonography. Among these, 3 cases elected pregnancy termination, one case of trisomy 21 was delivered stillborn vaginally at 32 weeks and one case of trisomy 18 delivered vaginally at 29 weeks. Among the 4 cases not recognized prenatally, one case of trisomy 21 was delivered at 27 weeks by classical cesarean section due to malpresentation and 3 cases (2 of trisomy 18 and one trisomy 21) where delivered by emergency transverse cesarean section due to suspected fetal hypoxia. CONCLUSIONS: The very high frequency of emergency cesarean section (100%) among fetuses with unrecognized major chromosomal anomalies should make us increase our efforts to obtain at prenatal diagnosis. In all of these cases, a prior diagnosis would have probably avoided a cesarean section and the associated potential maternal morbidity. PRECIS: The high incidence of emergency cesarean section among fetuses with unrecognized chromosomal anomalies should make prenatal diagnosis of these conditions a primary goal.
Asunto(s)
Cesárea , Aberraciones Cromosómicas , Trastornos de los Cromosomas/diagnóstico , Diagnóstico Prenatal , Adulto , Síndrome de Down/diagnóstico , Urgencias Médicas , Femenino , Muerte Fetal/etiología , Edad Gestacional , Humanos , Recién Nacido , Edad Materna , Embarazo , Complicaciones del Embarazo , Factores de RiesgoRESUMEN
OBJECTIVE: A preliminary investigation to test the efficacy of intravenous propranolol in reducing the cesarean section rate in nulliparas in active labor and evaluate its effect on neonatal and maternal outcomes. METHODS: Fifty seven nulliparous patients admitted in active labor were randomly divided into two groups: a control group consisting of 23 patients, and a treatment group consisting of 34 patients given 2 mg of propranolol intravenously every 4 hours until delivery. Total length of labor, time from first administration of medication to delivery, incidence of cesarean section, APGAR scores, maternal and fetal morbidity were assessed. RESULTS: A total of 4 cesarean sections were performed in each group (11.7% in the treatment group and 17.3% in the control group). The rate of cesarean section due to dystocia was 6.25 and 13.6% respectively (P = .367). Statistical significance was not reached due to the small number of subjects (students t test analysis). There was no increase in the incidence of low APGAR scores, intensive care unit admissions, abnormal heart rate patterns during labor, cesarean sections for fetal distress or maternal morbidity in the treated group. CONCLUSIONS: Intravenous administration of 2 mg of propranolol every four hours is safe and not associated to increased neonatal or maternal morbidity. A 50% decrease in the incidence of cesarean sections can be documented among nulliparous patients treated with propranolol although the small numbers and overall low incidence of cesarean section in our population (14%) did not permit these differences to reach statistical significance.