Hemorragia intracraneal como presentación de sindrome hemorragico severo por lonomismo sistemico / Intracranial hemorrhage as presentation of severe hemorragic sindrome by sistemic lonomisms

Los accidentes causados por los estadios larvarios de los lepidópteros se conocen como erucismo. El contacto accidental con las orugas del género Lonomia spp. puede desencadenar síndromes hemorrágicos que en casos severos puede llegar a producir injuria renal aguda y hemorragia intracraneal. Se presenta el caso de un varón de 48 años procedente la selva central del Perú, quien mientras estaba en el campo, incidentalmente entra en contacto con una oruga, cursando luego con un síndrome hemorrágico caracterizado por coagulopatía y hemorragia subaracnoidea. El cuadro se resolvió satisfactoriamente luego de la administración del suero antilonómico. Se discute los aspectos fisiopatológicos, clínicos y laboratoriales que condujeron al diagnóstico, así como la efectividad reportada del tratamiento. (AU)

The accidents caused by the larval stages of the Lepidoptera are known as erucism. Accidental contact with caterpillars of the genus Lonomia spp. can trigger hemorrhagic syndromes that in severe cases can lead to acute renal injury and intracranial hemorrhage. We present the case of a 48 year-old male from Peruvian Central Jungle who while was working in the forest comes in contact with a caterpillar, after which he developed an hemorrhagic syndrome characterized by coagulopathy and subarachnoid hemorrhage. The clinical picture resolved satisfactorily after administration of antilonomic serum. The pathophysiological, clinical and laboratory aspects that led to the diagnosis are discussed, as well as the reported effectiveness of treatment. (AU)

Transaldolase deficiency: a new cause of hydrops fetalis and neonatal multi-organ disease.

Transaldolase (TALDO) deficiency is a newly recognized metabolic disease, which has been reported so far in 2 patients presenting with liver failure and cirrhosis. We report a new sibship of 4 infants born to the same consanguineous parents; all presented at birth or in the antenatal period with dysmorphic features, cutis laxa and hypertrichosis, hepatomegaly, splenomegaly, liver failure, hemolytic anemia, thrombocytopenia, and genitourinary malformations. The clinical courses were variable: the first child died of liver failure at 4 months of age; the second pregnancy was medically terminated at 28 weeks gestation because of hydrops fetalis with oligohydramnios. The third child is doing well at age 7 with liver fibrosis and mild kidney failure. The fourth child is now 21 months old and has hepatosplenomegaly, mild anemia, and thrombocytopenia. Urine assessment of polyols showed elevations of erythritol, arabitol, and ribitol consistent with TALDO deficiency. TALDO activity was undetectable in the patients' tissues, and mutation in the TALDO1 gene was found in the 4 patients.