Dapsone hypersensitivity syndrome in an adolescent during treatment during of leprosy Síndrome de hipersensibilidade à dapsona em uma adolescente durante tratamento de hanseníase

A 12 y old girl was admitted 24 days after start a WHO multidrug therapy scheme for multibacillary leprosy (dapsone, clofazimine and rifampicin) with intense jaundice, generalized lymphadenopathy, hepatoesplenomegaly, oral erosions, conjunctivitis, morbiliform rash and edema of face, ankles and hands. The main laboratory data on admission included: hemoglobin, 8.4 g/dL; WBC, 15,710 cells/mm³; platelet count, 100,000 cells/mm³; INR = 1.49; increased serum levels of aspartate and alanine aminotransferases, gamma-glutamyl transpeptidase, alkaline phosphatase, direct and indirect bilirubin. Following, the clinical conditions had deteriorated, developing exfoliative dermatitis, shock, generalized edema, acute renal and hepatic failure, pancytopenia, intestinal bleeding, pneumonia, urinary tract infection and bacteremia, needing adrenergic drugs, replacement of fluids and blood product components, and antibiotics. Ten days after admission she started to improve, and was discharged to home at day 39th, after start new supervised treatment for leprosy with clofazimine and rifampicin, without adverse effects. This presentation fulfils the criteria for the diagnosis of dapsone hypersensitivity syndrome (fever, generalized lymphadenopathy, exfoliative rash, anemia and liver involvement with mixed hepatocellular and cholestatic features). Physicians, mainly in geographical areas with high prevalence rates of leprosy, should be aware to this severe, and probably not so rare, hypersensitivity reaction to dapsone.<br>Menina, 12 anos, foi admitida referindo o uso de esquema de poliquimioterapia preconizado pela OMS para tratamento de hanseníase forma multicibacilar (dapsona, rifampicina e clofazimina) há 24 dias, apresentando icterícia, linfadenomegalia generalizada, hepatoesplenomegalia, conjuntivite, úlceras orais, exantema morbiliforme e edema de face, mãos e tornozelo. Os principais achados laboratoriais à admissão incluíam: hemoglobina, 8,4 g/dl; leucograma, 15.710 céls/mm³; contagem de plaquetas, 100.000 céls/mm³; RNI = 1,49; aumento dos níveis séricos da alanino e aspartato aminotransferases, gama-glutamil transpeptidase, fosfatase alcalina e bilirrubinas. Em seqüência, ocorreu piora do quadro, desenvolvendo dermatite esfoliativa, choque, edema generalizado, insuficiências renal e hepática, pancitopenia, sangramento intestinal, pneumonia, infecção urinária e bacteremia, necessitando de drogas adrenérgicas, antibióticos, infusão de líquidos e hemoderivados. Iniciou melhora no 10º dia de internação, recebendo alta hospitalar no 39º dia, tendo iniciado novo tratamento supervisionado para hanseníase com rifampicina e clofazimina, sem efeitos adversos. O caso relatado preenche os critérios para o diagnóstico de síndrome de hipersensibilidade à dapsona (febre, dermatite esfoliativa, linfadenopatia, anemia e acometimento hepático com necrose hepatocítica e colestase). Os médicos, principalmente em regiões com alta prevalência de hanseníase, devem estar atentos para esta grave, e provavelmente não tão rara, reação de hipersensibilidade à dapsona

Proprioceptive Opsin Functions in Drosophila Larval Locomotion

Animals rely on mechanosensory feedback from proprioceptors to control locomotory body movements. Unexpectedly, we found that this movement control requires visual opsins. Disrupting the Drosophila opsins NINAE or Rh6 impaired larval locomotion and body contractions, independently of light and vision. Opsins were detected in chordotonal proprioceptors along the larval body, localizing to their ciliated dendrites. Loss of opsins impaired mechanically evoked proprioceptor spiking and cilium ultrastructure. Without NINAE or Rh6, NOMPC mechanotransduction channels leaked from proprioceptor cilia and ciliary Inactive (Iav) channels partly disappeared. Locomotion is shown to require opsins in proprioceptors, and the receptors are found to express the opsin gene Rh7, in addition to ninaE and Rh6. Besides implicating opsins in movement control, this documents roles of non-ciliary, rhabdomeric opsins in cilium organization, providing a model for a key transition in opsin evolution and suggesting that structural roles of rhabdomeric opsins preceded their use for light detection