[Kawasaki disease: a clinical serie]. / Enfermedad de Kawasaki: una serie clínica.

INTRODUCTION: Kawasaki disease (KD) is a serious disease in children due to its potential complications and sequelae if not promptly and adequately managed. OBJECTIVES: To describe clinical and epidemiological characteristics of children hospitalized due to KD at a tertiary care center and identify risk factors for poor outcome. PATIENTS AND METHODS: Retrospective and descriptive study of 32 medical records of patients hospitalized with diagnosis of KD at a tertiary care center of Santiago, Chile between February 1999 and May 2007. RESULTS: The annual frequency was of 5 cases, mainly boys and during spring. The median age at diagnosis was 1.5 years and 87.5% of the children were younger than 5 years. Typical presentation prevailed in all ages (68,7%). Coronary artery affection, including dilatation or aneurisms, occurred in 21.9% of the cases, with aneurysms in 3 cases. All patients were treated with aspirin and intravenous immunoglobulin (IVIG); 4 patients required a second dose. No deaths were reported. The identified risk factors for poor outcome were age older than 5 years and hypoalbuminemia. CONCLUSIONS: KD is an infrequent disease that mainly occurs in children younger than 5 years and with a typical presentation. There are risk factors associated with poor outcome.

[Nursemaid's elbow: diagnosis and treatment in emergency department]. / Pronación dolorosa: diagnóstico y manejo de urgencia.

INTRODUCTION: Painful pronation (PD), also known as "nursemaid's elbow", is a common injury caused by abrupt longitudinal traction of the hand while the forearm is pronated and the elbow extended. OBJECTIVE: to describe a sample of patients diagnosed with painful pronation and the different reduction tech niques used for this pathology. PATIENTS AND METHOD: retrospective study of patients who visited the emergency department of a referral hospital, from January 2018 to September 2019. Patients under 7 years of age consulting due to a condition compatible with PD were included. We recorded demogra phic data, sex, and age, number of previous episodes (defining recurrent as three or more episodes), affected extremity, mechanism of injury, diagnostic images, reduction maneuver used, and success obtained measured through the presence of "click" and decrease in pain. Patients who presented any sign of trauma or fractures were excluded from the study. RESULTS: 172 patients were included, 57.6% were female, and a median age of 25.5 months. The most affected side was the left one and the main mechanism was traction of the extremity, followed by a same-level fall. The hyperpronation method was effective in 66% of the cases, and the remaining 34% required a supination maneuver. CONCLUSIONS: PD is a frequent reason for consultation in pediatric patients around the age of 2 years. The hyperpronation method was the most commonly used for its management. It is important to be aware of this pathology in the context of emergency care.

SWAN-Venule: An Optimized MRI Technique to Detect the Central Vein Sign in MS Plaques.

BACKGROUND AND PURPOSE: Multiple sclerosis lesions develop around small veins that are radiologically described as the so-called central vein sign. With 7T MR imaging and magnetic susceptibility-based sequences, the central vein sign has been observed in 80%-100% of MS lesions in patients' brains. However, a lower proportion ∼50% has been reported at 3T using susceptibility-weighted angiography (SWAN). Our aim was to assess a modified version of SWAN optimized at 3T for sensitive detection of the central vein sign. MATERIALS AND METHODS: Thirty subjects with MS were scanned on a 3T clinical MR imaging system. 3D T2-weighted FLAIR and optimized 3D SWAN called SWAN-venule, were acquired after injection of a gadolinium-based contrast agent. Patients showing >3 focal white matter lesions were included. The central vein sign was recorded by 2 trained raters on SWAN-venule images in the supratentorial brain. RESULTS: Twenty patients showing >3 white matter lesions were included. A total of 380 white matter lesions (135 periventricular, 144 deep white matter, and 101 juxtacortical) seen on both FLAIR and SWAN-venule images were analyzed. Overall, the central vein sign was detected in 86% of the white matter lesions (periventricular, 89%; deep white matter, 95%; and juxtacortical, 78%). CONCLUSIONS: The SWAN-venule technique is an optimized MR imaging sequence for highly sensitive detection of the central vein sign in MS brain lesions. This work will facilitate the validation and integration of the central vein sign to increase the diagnostic certainty of MS and further prevent misdiagnosis in clinical practice.

Brain MRI findings in children and adolescents with Fabry disease.

OBJECTIVE: To evaluate the presence of white matter and hemorrhagic lesions in brain MRI of children and adolescents with Fabry disease (FD). METHODS: Brain MRI studies in 44 consecutive children and teenagers (20 boys, mean age 14.6 years, range 7-21 years) were evaluated using classic sequences as well as, GRE-weighted images, for white matter lesions (WML) and chronic microbleed detection. All patients lacked history of stroke or TIA. Brain MRI findings in 46 consecutive children and adolescents without FD, referred for the evaluation of headaches (36 females, mean age 14.1 years, range 7-21 years) were evaluated as a control group. Additionally, we assessed the clinical manifestations of FD. RESULTS: Seven children (15.9%) with FD had brain MRI evidence of asymptomatic WML (5 girls, mean age 14.8 years, range: 13-20 years) compared with 3 children (6.5%) in the control group (p = 0.01). Brain abnormalities in patients with FD revealed WML, deep gray matter and infratentorial involvement. Three patients presented two lesions each. None of the children showed microbleeds. Regarding clinical manifestations, 90.9% of the patients had signs or symptoms of FD. CONCLUSION: We identified asymptomatic white matter brain lesions in 15.9% of children with FD without clinical history of stroke. FD is a treatable disorder that should be routinely included in the differential diagnosis of both symptomatic and asymptomatic brain lesions in children and adolescents. The detection of brain lesions may foster earlier treatment.

Tension pneumocranium, a rare complication of transsphenoidal pituitary surgery: Mayo Clinic experience 1976-1998.

We describe four cases of symptomatic pneumocranium, a rare, potentially life-threatening complication of transsphenoidal pituitary surgery. Symptomatic pneumocranium manifested as impaired mental status, headaches, and grand mal seizures, early in the postoperative course after transsphenoidal pituitary surgery. Furthermore, a Cushing response, including systemic hypertension and bradycardia (secondary to intracranial hypertension) was seen, which has not been previously described in association with symptomatic pneumocranium. We describe a previously unreported risk factor for tension pneumocranium, untreated obstructive sleep apnea. Other factors predisposing to tension pneumocranium in our patients included: cerebrospinal fluid leaks, postoperative positive-pressure mask ventilation, large pituitary tumors, and intraoperative lumbar drainage catheters. Surgical drainage of the pneumocranium and repair of any coexistent cerebrospinal fluid leak markedly improved neurologic status. Symptomatic pneumocranium occurring early in the postoperative course after transsphenoidal pituitary surgery is rare, but prompt recognition and treatment of this condition can be life-saving.

Nitric oxide modulates light-evoked compound action potentials in the intact rabbit retina.

This study examines the effects of alterations in nitric oxide (NO) production on rabbit retinal function. NG-nitro-L-arginine (L-NA) and L-arginine (L-Arg) inhibited the light-evoked compound action potentials (CAPs) from the optic nerve, but not the electroretinogram (ERG), in a concentration-dependent manner, whereas D-arginine had no effect. L-Arg partially reversed the L-NA-induced inhibitory effect, and L-NA prevented the L-Arg-induced attenuation of the CAPs. Sodium nitroprusside reduced both the CAPs and the ERG, whilst potassium ferricyanide did not affect the CAPs, and potentiated the ERG. We conclude that, independent of the vasculature, endogenous NO modulates neurotransmission (i.e. light-evoked CAPs), but probably not phototransduction (i.e. light-evoked ERG) in this intact in vitro central nervous system preparation.

Sodium valproate reduces immobility in the behavioral 'despair' test in rats.

The present study was conducted to investigate whether sodium valproate could affect immobility in the 'behavioral despair' test in rats. Acute (one injection), subacute (three injections) and chronic treatment with sodium valproate reduced the immobility time in this test, whereas a stimulation of motor activity in the open-field test was not observed with the same drug treatments. The anti-immobility activity of valproate was partially counteracted by the administration of bicuculline (2 mg/kg) or picrotoxin (1.4 mg/kg) before the immobility test. The data agree with previous findings from several animal models of depression of an antidepressant-like activity of GABA mimetics or agents which stimulate GABAergic function.

Anterior communicating artery collateral flow protection against ischemic change during carotid endarterectomy.

The purpose of this study was to determine whether preoperative angiographic patterns of collateral cerebral blood flow correlate with protection against intraoperative electroencephalographic (EEG) evidence of cerebral ischemia caused by carotid artery cross-clamping during carotid endarterectomy. Previous studies have shown that contralateral carotid artery occlusion and intracranial stenoses are associated with cerebral ischemia during carotid endarterectomy; however, the angiographic collateral flow patterns associated with cerebral ischemia have not been identified. This paper reports a retrospective study of 67 patients who underwent two- to four-vessel cerebral angiography followed by carotid endarterectomy with 16-channel EEG monitoring. The angiograms were reviewed for extracranial occlusive disease and collateral flow patterns, and the EEG recordings were analyzed for ischemic changes during carotid artery cross-clamping. Statistical analysis was by Fisher's exact test. Cross-filling of the anterior and middle cerebral arteries from the contralateral carotid artery through the anterior communicating artery correlated with a decreased incidence of EEG ischemic changes. Only 21% of patients with this collateral flow pattern showed ischemic changes compared to 50% of patients without this pattern (p < 0.03). Three angiographic findings occurring in combination on the side contralateral to surgery correlated with EEG ischemia: 1) occlusion of the contralateral internal carotid artery (five of seven or 71%, p < 0.03); 2) collateral flow from the external carotid circulation to the internal carotid circulation via the ophthalmic artery; and 3) collateral flow from the posterior circulation to the contralateral anterior circulation via the posterior communicating artery. The data presented here corroborate the correlation between contralateral carotid artery occlusion and cerebral ischemia during carotid endarterectomy. They also demonstrate that cross-filling of the anterior and middle cerebral arteries by the contralateral carotid artery protects against such ischemia. This collateral flow may serve as an indicator of tolerance to carotid artery cross-clamping.

Storage histiocytes and hemophagocytosis: a common finding in the bone marrow of patients with active systemic lupus erythematosus.

We report the frequency of the finding of storage and hemophagocytic histiocytes in the bone marrow of patients with systemic lupus erythematosus with one or more hemocytopenias. The study was performed on bone marrows of ten patients with systemic lupus erythematosus during an episode of hemocytopenia. Four patients were not receiving any treatment and six had been taking oral prednisone (3.5 +/- 1.5 mg/day) for the previous 6 months. Hemocytopenias found were lymphocytopenia in five, thrombocytopenia in three, and neutropenia and anemia in two each. The bone marrow had variable cell content; megakaryocytes, the myeloid:erythroid ratio, as well as lymphocyte, plasma cell, and reticular cell contents were usually increased. Seven bone marrow samples showed storage histiocytes (sea-blue histiocytes and other histiocytes that contained unidentified intracytoplasmic material). These same samples revealed histiocytes phagocytosing erythoblasts, erythrocytes, polymorphonuclear neutrophils, lymphocytes or platelets. The presence of these histiocytes in all seven patients was related to a decrease in serum complement and with lupus disease activity as measured with the SLEDAI index (mean +/- SD = 2.1 +/- 1.5). The SLEDAI score of the remaining three patients, who had no histiocytes in their bone marrow, was 0, 0, and 1, respectively. The present study reveals that the bone marrow in patients with systemic lupus erythematosus and peripheral hemocytopenia contains storage and hemophagocytic histiocytes and the significance of these cells is discussed.

Comparison of invasive and noninvasive methods for the diagnosis and evaluation of eradication of Helicobacter pylori infection in children.

BACKGROUND: Acquisition of Helicobacter pylori infection occurs mainly during childhood. To study the events associated with H. pylori colonization in children it is important to have reliable diagnostic methods. Our objective was to validate invasive and noninvasive tests for diagnosis of H. pylori infection in children before and after antimicrobial treatment. METHODS: Before treatment, invasive rapid urease test (RUT) culture and histology, as well as the noninvasive carbon-13 urea breath test (13C-UBT) and serology were validated in 59 children. The gold standard for H. pylori infection was any of three positives of the five tests. After antimicrobial treatment culture, histology, and 13C-UBT were validated in 43 children to determine eradication. The gold standard for eradication was negative in all three tests. RESULTS: For primary diagnosis, RUT was the most sensitive and specific test, followed by 13C-UBT, which performed better than serology, culture, and histology. Concordance tests also showed that RUT and 13C-UBT performed better. For determination of eradication, 13C-UBT and histology were better than culture, which showed poor sensitivity. CONCLUSIONS: RUT performed better for primary diagnosis. However, as endoscopy might not be indicated in most children, 13C-UBT could be the test of choice for diagnosis of H. pylori infection both before and after eradication treatment.

Two-dimensional echo Doppler findings in juvenile and adult onset ankylosing spondylitis with long-term disease.

The authors' objective was to determine by 2-dimensional echo Doppler (2DECHO) the cardiac abnormalities in juvenile onset ankylosing spondylitis (JOAS) and adult onset ankylosing spondylitis (AOAS) in male patients with long-term disease. Twenty patients with JOAS, 31 with AOAS, and 20 healthy controls of the same age and gender without cardiopulmonary symptoms were studied. Using 2DECHO, the heart dimensions were determined according to American Society of Echocardiography guidelines. The left ventricle ejection fraction (LVEF) was calculated by Teichholz's formula. Cardiomyopathy was established when 2DECHO had diminished LVEF. Statistics used were the Student t and Fisher test, chi2, and ANOVA. Ninety percent of JOAS and 51% of AOAS patients were B27+ (p=0.005). The disease duration was 19.3 +/- 8.8 years in JOAS and 14.8 +/- 12.8 years in AOAS (p=NS). Age at the time of the study was 30.7 +/- 9.9 years in JOAS vs 40.3 +/- 12.7 in AOAS (p=0.003), and vs 40.2 +/- 17 years in controls (p=NS). There was a higher frequency of cardiomyopathy in AOAS (32.2%) than in JOAS (25%) and the controls (0%) (p=0.01). Patients with JOAS had a higher mitral valve gradient (25%) than AOAS patients (19%, p=NS) and controls (0%, p=0.04). Abnormal aortic ring reflectance was shown in 19% of AOAS vs 0% abnormalities in JOAS and controls (p=0.01). The aortic root diameter was increased in 58% of AOAS, 30% of JOAS, and 0% of controls (p=0.001). The frequency of 2DECHO abnormalities was increased in cardiopulmonary asymptomatic spondylitis patients. Despite the high frequency of B27+, JOAS had a lower frequency of aortic abnormalities than AOAS. Mitral valve gradient was found in JOAS and in AOAS that could contribute to a decreased ejection fraction and to left ventricular dysfunction.

Screening program for retinopathy of prematurity in Spain.

OBJECTIVE: To prepare a retinopathy of prematurity (ROP) screening program as agreed by most of Spanish ophthalmologists dedicated to this topic. MATERIALS AND METHODS: A draft of the protocol was produced taking into account the experience of the participants and current publications. This draft was corrected by all the ophthalmologists participating in the project and the final document produced was agreed by all of them. RESULTS: We present general guidelines to help in the screening of ROP, including treatment criteria, treatment methods, and a calendar of action. CONCLUSIONS: It is important to have a common working protocol in the screening of ROP to improve the action and to avoid mistakes. Although individual Hospitals may adapt the protocol to their daily activity, it is recommended that there is a minimal working protocol agreed by most of professionals dedicated to pediatric ophthalmology in Spain.

[A protocol for the treatment of retinopathy of prematurity in Spain]. / Protocolo de tratamiento de la retinopatía del prematuro en España.

OBJECTIVE: To prepare a protocol for the treatment of retinopathy of prematurity (ROP) agreed by the majority of Spanish ophthalmologists dedicated to this topic. MATERIAL AND METHOD: A draft of the protocol was produced taking into account the experience of the participants and up to date publications. This draft was corrected by all the ophthalmologists participating in the project, and the final document was agreed by all of them. RESULTS: We present general guidelines as an aid for the treatment of ROP, including treatment criteria, treatment methods, a calendar of action, and follow-up. CONCLUSIONS: It is important to have a common working protocol for the treatment of ROP to improve care and to avoid mistakes. Although individual Hospitals may adapt the protocol to their daily activity, it is recommended that there is a minimal working protocol agreed by most of professionals dedicated to pediatric ophthalmology in Spain.

Ventilación no invasiva en el paciente con falla respiratoria aguda / Noninvasive ventilation in acute pediatric respiratory failure

Although most cases of respiratory failure resolve with medical treatment and are self-limited, some of them may require a higher level of ventilatory support. Noninvasive ventilation (NIV) is an alternative to improve gas exchange in selected patients and may prevent intubation, the use of invasive ventilation and its associated risks. This article describes the use of noninvasive ventilation in acute respiratory pathology.

Aunque la mayoría de los casos de falla respiratoria son de evolución favorable y autolimitados algunos pueden requerir soporte ventilatorio de mayor nivel. La ventilación no invasiva (VNI) es una alternativa que mejora el intercambio gaseoso en pacientes seleccionados y puede prevenir la intubación y uso de ventilación invasiva con sus riesgos asociados. Este artículo describe el uso de la ventilación no invasiva en la patología respiratoria aguda.

[Design of computerised database for clinical and basic management of uveal melanoma]. / Diseño de una base de datos informatizada para la gestión clínica y básica del melanoma uveal.

PURPOSE: The uveal melanoma is the most common primary intraocular tumour in adults. The objective of this work is to show how a computerised database has been formed with specific applications, for clinical and research use, to an extensive group of patients diagnosed with uveal melanoma. METHOD: For the design of the database a selection of categories, attributes and values was created based on the classifications and parameters given by various authors of articles which have had great relevance in the field of uveal melanoma in recent years. RESULTS: The database has over 250 patient entries with specific information on their clinical history, diagnosis, treatment and progress. It enables us to search any parameter of the entry and make quick and simple statistical studies of them. CONCLUSION: The database models have been transformed into a basic tool for clinical practice, as they are an efficient way of storing, compiling and selective searching of information. When creating a database it is very important to define a common strategy and the use of a standard language.

Vía aérea fisiológicamente difícil: nuevo concepto a considerar: Caso clínico vía aérea difícil fisiológica / Physiologically difficult airway: new concept to consider: case report

Airway management in the ICU scenario is a real challenge due to instability imposed by illness severity. Some of these patients may also suffer from other diseases associated to difficult airway. Rheumatoid arthritis represents a special condition because not only can limit access to the hypopharynx, but also can hamper laryngoscopy. Among many options for difficult airway management, only a few can be safely applied to intubate the trachea while preserving physiologic stability in an emergency setting. We chose awake fiberoptic intubation as the first and safest approach for this combined difficult patient. This approach requires not only individual skills but also a plan in advance and a well coordinated team to be successful. A case is presented as an example of difficulties imposed by physiologic instability added to anatomic difficult.

El manejo de la vía aérea en la UCI representa un desafío debido a la inestabilidad derivada de la severidad de la patología. Además, algunos de estos pacientes pueden presentar condiciones preexistentes asociadas a vía aérea difícil. La artritis reumatoide representa una condición especial debido a que no sólo limita el acceso a la hipofaringe, sino que también puede dificultar la laringoscopía. Entre las diferentes opciones para manejar la vía aérea, sólo algunas pueden ser aplicadas para intubar la tráquea de manera segura y al mismo tiempo preservar la estabilidad fisiológica en un contexto de urgencia. Este abordaje requiere no sólo habilidades individuales, sino que también trabajo en equipo coordinado y planificación. Se presenta un caso como ejemplo de dificultades impuestas por la inestabilidad fisiológica agregada a dificultad anatómica.

Displasia cortical focal: hallazgos en imágenes de tensor de difusión

Objetivo: La displasia cortical focal (DCF) es una anomalía del desarrollo cortical. Representa una de las causas más frecuentes de epilepsia refractaria, siendo fundamental la resonancia magnética (RM) para su diagnóstico. Dada la importancia que ha cobrado la secuencia de tensor de difusión (DTI), el objetivo de este trabajo fue evaluar retrospectivamente los hallazgos en el mapa de fracción de anisotropía del DTI en pacientes con DCF. Observaciones: Se buscó retrospectivamente a pacientes con diagnóstico anatomopatológico de DCF en la base de datos de nuestro hospital. De un total de 74, se seleccionaron 8 casos con diagnóstico aislado de DCF y estudio de RM prequirúrgico con DTI. El foco de displasia se identificó en las imágenes anatómicas convencionales de la RM. Se evaluó el mapa de fracción de anisotropía (FA) y se definieron las alteraciones en la región de la DCF. Se observó una disminución de la FA en la sustancia blanca adyacente a la DCF en 7 de los 8 pacientes (87,5%). Discusión: Los hallazgos con el DTI brindan información complementaria en relación con la RM. En la mayoría de nuestros pacientes no hubo cambios de señal en la sustancia blanca en las imágenes convencionales, pero sí se identificó una disminución de la FAen el DTI. Se desconoce si esto refleja cambios estructurales o únicamente funcionales, secundarios a la lesión primaria. Creemos que el DTI puede agregar información complementaria de valor para el diagnóstico y valoración de la extensión de esta patología.

Objective: Focal cortical dysplasia (DCF) in an anomaly of cortical development. It represents one of the most frequent causes of drug resistant epilepsy and the magnetic resonance imaging (MRI) is trivial for its diagnosis. In the last years the use of diffusion tensor imaging (DTI) has increased in this kind of pathology. The purpose of this work was to evalúate retrospectively DTI findings on fractional anisotropy maps in patients with FCD. Observations: We retrospectively searched patients with confirmed anatomo-pathological diagnosis of FCD in our hospital datábase. From a total of 74, 8 patients, with isolated diagnosis of FCD and preoperative MRI with DTI, were selected. The FCD was identified in conventional anatomical MRI in all patients. Fractional anisotropy (FA) maps were evaluated and changes in the región of FCD were defined. Decreased FA was observed in white matter adjacent to the FCD, in 7 of 8 patients (87.5%). Discussion: Findings of DTI gives us complementary information to those of conventional MRI. Most of our patients showed no signal changes of white matter in conventional sequences and they presented decreased FA in DTI. We don't actually know if these DTI findings represent structural changes in white matter or just functional changes secondary to the adjacent FCD. We think DTI can give valuable complementary information for the diagnosis and determination of the extensión of this pathology.

Cirugía laparoscópica de quistes adrenales: resultados de una serie de 18 pacientes / Laparoscopic surgery of adrenal cysts: results of a series of 18 patients

Introduction: Adrenal cysts have an incidence of 0.064 percent to 0.18 percent at autopsy. Aim: To show our experience in 18 patients with an adrenal cyst operated by laparoscopy. Material and Methods: The series consisted of 18 patients operated, in a series of 344 laparoscopic adrenalectomy performed between 1993 and 2011. The age range was 20 to 61 years with an average of 38 years, 11 women and 7 men. Results: Thirteen cysts were left and 5 were right sided. The lesion size varied between 4.5 and 12.5 cm, with an average of 9.2 cm. Eight patients complained of abdominal discomfort and in the remaining 12 patients there were no signs or symptoms of clinical suspicion. The surgery performed was a laparoscopic decortication-marsupialization in 11 patients, laparoscopic adrenalectomy in 8 cases and partial adrenalectomy in 1 case. Mean operative time was 83.1 minutes (30-180 minutes), and mean hospital stay was 36 hours. Intraoperative complications occurred in 2 patients: 1 diaphragm injury and one renal vein injury, repaired in the same surgery. Postoperative complications occurred in 2 patients, both Clavien 3a, treated by conservative management. Conclusion: Small and non-functional lesions require observation. Partial or total laparoscopic adrenalectomy is indicated in large lesions or in functional and suspected malignant lesions.

Introducción: Los quistes suprarrenales tienen una incidencia de 0,064 por ciento a 0,18 por ciento en autopsias. El objetivo de este trabajo es mostrar nuestra experiencia en 18 pacientes portadores de un quiste adrenal operados por vía laparoscópica. Material y Métodos: La serie está constituida por 18 pacientes, de una serie de 344 adrenalectomías laparoscópicas realizadas entre los años 1993 y 2011. El rango de edad fue de 20 a 61 años, con un promedio de 38 años, 11 mujeres y 7 hombres. Resultados: Trece quistes eran del lado izquierdo y 5 del derecho. El tamaño de la lesión varió entre 4,5 y 12,5 cm, con un promedio de 9,2 cm. En 8 pacientes hubo molestias abdominales vagas y en los restantes 12 pacientes no hubo signos ni síntomas de sospecha clínica. La cirugía realizada fue una decorticación-marsupialización laparoscópica en 11 pacientes, adrenalectomía laparoscópica en bloque en 8 casos y adrenalectomía parcial en 1 caso. El tiempo operatorio promedio fue de 83,1 min (30-180 min), y el tiempo medio de hospitalización fue de 36 h. Complicaciones intraoperatorias ocurrieron en 2 pacientes: 1 lesión de diafragma y 1 lesión de vena renal, reparadas en el mismo acto quirúrgico. Complicaciones postoperatorias ocurrieron en 2 pacientes, ambas Clavien 3a y de manejo conservador. Conclusión: Las lesiones pequeñas y no funcionantes requieren observación. La adrenalectomía laparoscópica parcial o total está indicada en lesiones de gran tamaño, con importante compromiso de la glándula o ante la sospecha de lesión funcionante o malignidad.

[Hemolytic-uremic syndrome in Chile: clinical features, evolution and prognostic factors]. / Síndrome hemolítico urémico en Chile: presentación clínica, evolución y factores pronósticos.

BACKGROUND: Hemolytic-uremic syndrome (HUS) is characterized by acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. AIM: To describe the characteristics of patients with the diagnosis of HUS in Chile, and to identify the most reliable early predictors of morbidity and mortality. MATERIAL AND METHODS: The clinical records of patients with HUS aged less than 15 years, attended between January 1990 and December 2003 in 15 hospitals, were reviewed. Demographic, clinical, biochemical, hematological parameters, morbidity and mortality were analyzed. RESULTS: A cohort of 587 patients aged 2 to 8 years, 48% males, was analyzed. Ninety two percent had diarrhea. At the moment of diagnosis, anuria was observed in 39% of the patients, hypertension in 45% and seizures in 17%. Forty two percent required renal replacement therapy (RRT) and peritoneal dialysis was used in the majority of cases (78%). The most frequently isolated etiological agent was Escherichia coli. Mortality rate was 2.9% in the acute phase of the disease and there was a positive correlation between mortality and anuria, seizures, white blood cell count (WCC)>20.000/mm3 and requirements of renal replacement therapy (p<0.05). Twelve percent of patients evolved to chronic renal failure and the risk factors during the acute phase were the need for renal replacement therapy, anuria, WCC>20.000/mm3, seizures and hypertension. CONCLUSIONS: The present study emphasizes important clinical and epidemiological aspects of HUS in a Chilean pediatric population.