D-lactoacidosis como complicación del síndrome de intestino corto / D-lactoacidosis as short bowel syndrome complication

Short bowel syndrome is defined as the loss, congenital or acquired, anatomical or functional, of a large part of the small intestine that generates inadequate absorption of nutrients and the frequent need of prolonged parenteral nutrition. The etiology of short bowel is diverse and varies with age. The necrotizing enterocolitisis and the midgut volvulus are among the most frequent causes. The bacterial overgrowth is frequently observed in children with short bowel, due to the secondary dilation of the remaining small bowel and to the associated intestinal dysmotility. It is more frequent in absence of the ileocecal valve. We present a 6-year-old boy with short bowel syndrome secondary to extensive intestinal resection after a volvulous of the medium small intestine, 9 months before admission to the hospital, and who was on cyclical parenteral nutrition at home. The child developed ataxia, disarthria, dizziness and conscience compromise been admitted to de intensive care unit in deep sopor. An extensive work up including metabolic, infectious, toxicology and SNC imaging was negative except for metabolic acidosis. He was discharged on good conditions. Even though the child was on supportive therapy, the patient was readmitted few hours later with similar symptoms. D-lacto acidosis was suspected and confirmed with a value of 6.69 mmol/1 (normal range: 0,0-0,25). Literature about this uncommon complication and its mechanism is reviewed. D-lacto acidosis should be suspected in every patient with short bowel syndrome and unexplained metabolic acidosis associated with neurological symptoms.

El síndrome de intestino corto se define como la pérdida, congénita o adquirida, anatómica o funcional, de una extensa área del intestino delgado que genera inadecuada absorción de nutrientes y la frecuente necesidad de nutrición parenteral prolongada. Su etiología varía con la edad. Entre las causas más frecuentes están la enterocolitis necrotizante y el vólvulo de intestino medio. El sobrecrecimiento bacteriano es frecuente en estos pacientes, debido a la dilatación secundaria del remanente de intestino delgado y a la dismotilidad intestinal asociada. Objetivo: Comunicar un caso de acidosis D-láctica en un niño con Intestino corto. Caso clínico: Niño de 6 años con antecedente de intestino corto secundario a resección intestinal por vólvulo de intestino medio 9 meses previos al ingreso y que estaba recibiendo nutrición parenteral domiciliaria ciclada. El niño desarrolló ataxia, disartria, mareos y compromiso de conciencia, ingresando a la unidad de cuidados intensivos en sopor profundo. Un extenso estudio metabólico, infectológico, toxicológico y de neuroimágenes fue negativo, excepto por acidosis metabólica. Fue dado de alta en buenas condiciones. A pesar del tratamiento de soporte vuelve a ingresar horas después con similar cuadro clínico. Se sospecha y confirma elevación de D-lactato en sangre (6,69 mmol/1 con rango normal de 0,0-0,25). Se revisa la literatura acerca de esta infrecuente complicación y su mecanismo de producción. La D-lactoacidosis debiera ser sospechada en todos los pacientes portadores de intestino corto que presenten un cuadro de acidosis metabólica no explicada, especialmente si se acompaña de compromiso de conciencia y síntomas cerebelosos.

D-Lactic Acidosis in Humans: Review of Update

D-Lactic acidosis has been well documented in ruminants. In humans, D-lactic acidosis is very rare, but D-lactic acidosis may be more common than generally believed and should be looked for in a case of metabolic acidosis in which the cause of acidosis is not apparent. The clinical presentation of D-lactic acidosis is characterized by episodes of encephalopathy and metabolic acidosis. The entity should be considered as a diagnosis in a patient who presents with metabolic acidosis accompanied by high anion gap, normal lactate level, negative Acetest, history of short bowel syndrome or malabsorption, and characteristic neurologic manifestations. Low carbohydrate diet, bicarbonate treatment, rehydration, and oral antibiotics would be helpful in controlling symptoms.