ABSTRACT
Ammonium is an important source of nitrogen for amino acid synthesis and is necessary for normal acid base balance. When ammonium concentrations are high it becomes a toxic compound. Hyperammonemia is a metabolic emergency. When underdiagnosed and not treated appropriately, it produces severe neurological sequelae and/or death. The clinical presentation of hyperammonemic encephalopathy varies, and includes from personality disorders, psychiatric disorders, confusion, irritability, lethargy, seizures to coma. Hyperammonemia occurs with an increase in ammonium production, as in intestinal hemorrhage, or with a decrease in the elimination of ammonium, such as in congenital metabolic errors, hepatic insufficiency or drug intoxication. As we can see, it may have multiple origins, but congenital errors of metabolism are always suspected as one of the causes. However, there are less frequent causes, such as urinary tract infection, especially in predisposing conditions. We describe the case of a 2-year-old boy with a history of horseshoe kidney and right ureterohydronephrosis, surgical correction of imperforate anus and rectal bladder fistula. This patient presented hyperammonemia with encephalopathy (Glasgow 7/15) while undergoing a urinary infection with Corynebacterium riegelii. Hyperammonemia is the result of the production in the dilated urinary tract of large amounts of ammonium due to bacterial urease and its subsequent reabsorption in the systemic circulation. The patient improved clinically (Glasgow 15/15) after parenteral antibiotic therapy and urinary tract clearance
El amonio es una fuente importante de nitrógeno para la síntesis de aminoácidos y necesario para el balance ácido base; si se encuentra elevado, se convierte en un compuesto tóxico. La hiperamoniemia es una urgencia metabólica; cuando no es diagnosticada y tratada de manera oportuna, produce graves secuelas de tipo neurológico o la muerte. La presentación clínica de la encefalopatía hiperamoniémica es variable, pudiéndose observar trastornos en la personalidad, trastornos psiquiátricos, confusión e irritabilidad, letargia, convulsión y coma. La hiperamoniemia se presenta por aumento en la producción de amonio, como en la hemorragia intestinal, o por disminución de la eliminación del mismo, como ocurre en los errores congénitos del metabolismo, en la insuficiencia hepática o en la intoxicación por fármacos. Puede tener múltiples orígenes, pero los errores congénitos del metabolismo son una de las causas que siempre se sospechan. Sin embargo, existen causas menos frecuentes, como la infección del tracto urinario (sobre todo en condiciones que predispongan a las mismas). Describimos aquí el caso de un niño de 2 años, con antecedentes de riñón en herradura y ureterohidronefrosis derecha, corrección quirúrgica de ano imperforado y fistula recto vesical. Este paciente presentó hiperamoniemia con encefalopatía (Glasgow 7/15) mientras cursaba una infección urinaria por Corynebacterium riegelii. La hiperamoniemia es el resultado de la producción en el tracto urinario dilatado de grandes cantidades de amonio, debido a la ureasa bacteriana y su posterior reabsorción en la circulación sistémica. El paciente mejoró clínicamente (Glasgow 15/15) después de la terapia antibiótica parenteral y desobstrucción de tracto urinario
Subject(s)
Humans , Male , Child, Preschool , Urinary Tract Infections , Brain Diseases , Corynebacterium , HyperammonemiaABSTRACT
Resumen El compromiso gastrointestinal en pacientes con lupus eritematoso sistémico (LES) es muy diverso. Su frecuencia y ubicación a lo largo del tracto digestivo varían ampliamente. Los procesos inflamatorios secundarios a los depósitos de complejos inmunes o eventos vasculares pueden ser los causantes de este compromiso. Una de las manifestaciones gastrointestinales características en los pacientes con LES es la pseudoobstrucción intestinal, que se define como la propulsión intestinal ineficaz que se produce en ausencia de factores mecánicos u obstructivos. Esta es, sin embargo, una complicación rara y poco entendida del LES. En este artículo, reportamos el caso de un paciente masculino con diagnóstico de LES y pseudoobstrucción intestinal, que fue tratado exitosamente con esteroides e inmunoglobulinas intravenosas. Se presenta una revisión completa de la literatura y una propuesta de la fisiopatología de la manifestación.
Abstract Gastrointestinal involvement in patients with systemic lupus erythematosus (SLE) is very diverse, and the frequency of occurrence and location along the digestive tract varies widely. Inflammatory processes secondary to immune complex deposits or vascular events may cause this involvement. One of the most characteristic gastrointestinal manifestations in these patients is the intestinal pseudo-obstruction, which is defined as the ineffective intestinal propulsion that occurs in the absence of mechanical or obstructive factors. This is, however, a rare and poorly understood complication of SLE. The case is presented of a male SLE patient presenting with intestinal pseudo-obstruction, and was successfully treated with steroids and intravenous immunoglobulin. A complete review of the literature and a proposal for the pathophysiology of intestinal pseudo-obstruction are presented.
Subject(s)
Humans , Male , Adult , Therapeutics , Intestinal Pseudo-Obstruction , Immunoglobulins, Intravenous , Lupus Erythematosus, Systemic , SteroidsABSTRACT
Objective To improve the ability of rheumatologist to diagnose systemic lupus erythematosus (SLE) complicated with ureterohydronephrosis by analyzing the characteristics of clinical manifestations.Methods Patients with ureterohydronephrosis hospitalized in Peking Union Medical College Hospital between 2000 to 2008 were analyzed retrospectively. The clinical characteristics, serological findings, treatment and prognosis of these patients were reviewed. Comparisons between the groups were performed with X2 test and t-test. Results SLE patient with ureterohydronephrosis accounted for 1.26% of the SLE patients hospitalized in the same period. Twenty-eight patients presented with gastrointestinal symptoms, 14 patients suffered from bladder irritative symptoms. Nineteen patients were with bilateral ureterohydronephrosis, and 8 patients were with unilateral ureterohydronephrosis. Fourteen patients had stype positive ANA, 14 patients had positive antiSSA antibodies. All patients were treated with steroid and immune suppressive therapy, 11 patients were cured, while 3 patients had no improvement. Conclusion Ureterohydronephrosis isn't a very rare complication of SLE. SLE patients with ureterohydronephrosis often present with gastrointestinal symptoms and have high incidence of chronic intestinal pseudo obstruction. High ratio of stype ANA antibody and high positive rate of anti-SSA are most important characteristics in this subtype of SLE patients. The complications can be reversed if the patients are treated early and appropritaely.
ABSTRACT
Intestinal pseudo-obstruction is an uncommon and poorly understood complication of systemic lupus erythematosus. It is characterized by acute or chronic symptoms suggestive of the obstruction of small or large intestine without any radiologic, surgical or endoscopic evidence of mechanical obstruction. Although the exact pathogenesis of intestinal pseudo-obstruction in SLE remains unknown, it can be caused by derangement of the visceral smooth muscle, enteric nerves or visceral autonomic nervous system. Concomitant involvement with dilatation of gastrointestinal and genitourinary tract highly suggests of intestinal pseudo-obstruction in systemic lupus erythematosus. There is an apparent association between lupus-related intestinal pseudo-obstruction and ureterohydronephrosis. The management of intestinal pseudo-obstruction consists of high dose corticosteroid, adequate nutrition, hydration and prokinetics. Early recognition of intestinal pseudo-obstruction in systemic lupus erythematosus and the use of adequate dose of corticosteroid are important. We report a case of systemic lupus erythematosus presenting as intestinal pseudo-obstruction.
Subject(s)
Humans , Autonomic Nervous System , Dilatation , Intestinal Pseudo-Obstruction , Intestine, Large , Lupus Erythematosus, Systemic , Muscle, SmoothABSTRACT
@#Bricker operation was used to treat advanced paraplegia with severe upper uninary tract deterioration, bilateral ureterohydronephrosis, recurrence urinary tract infection, and chronic renal failure.Three cases have good results. The operation is an extensive and complex procedure,indication should be strict.