Polineuropatía por amiloidosis por transtiretina de inicio tardío. Caso clínico / Late-onset hereditary transthyretin amyloidosis with polyneuropathy. Report of one case

Hereditary transthyretin amyloidosis is a multisystemic autosomal dominant genetic disorder characterized by progressive distal sensory-motor polyneuropathy or restrictive cardiomyopathy, secondary to amyloid deposits. Its pathogenesis lies in the TTR gene mutation, and the Val50Met mutation is the most frequent. Patients have significant differences in the onset and severity of clinical presentation according to their country of origin. The diagnosis of this pathology is complex, even more in countries where it is not considered endemic. However, early suspicion and management are essential to improve survival and avoid unnecessary diagnostic and therapeutic strategies. We report a 69-year-old woman who presented a sensory-motor polyneuropathy, predominantly sensory, associated with distal neuropathic pain and bilateral vitritis. The history of her Italian father with polyneuropathy of unspecified etiology stood out. A vitreous biopsy identified amyloid substance deposits (congo red positive). These were also confirmed on a superficial peroneal nerve biopsy. During the etiological study of her polyneuropathy, an increased Kappa/Lambda index of 2.55 mg/L stood out. Therefore, light chain amyloidosis was suspected, and chemotherapy treatment was indicated without favorable response. After 10 years of progressive neurological and ophthalmological involvement, a genetic study confirmed the first case of late-onset hereditary transthyretin amyloidosis Val50Met with polyneuropathy in Chile.

Effects of the economic recession on suicide mortality in Brazil: interrupted time series analysis / Efectos de la recesión económica en la mortalidad por suicidio en Brasil: análisis de series de tiempo interrumpido / Efeitos da recessão econômica na mortalidade por suicídio no Brasil: análise com séries temporais interrompidas

ABSTRACT Objectives: to analyze trends in suicide rates in Brazil in the period before and after the start of the economic recession. Methods: interrupted time series research using national suicide data recorded in the period between 2012 and 2017 with socioeconomic subgroups analyses. Quasi-Poisson regression model was employed to analyze trends in seasonally adjusted data. Results: there was an abrupt increase in the risk of suicide after economic recession in the population with less education (12.5%; RR = 1.125; 95%CI: 1.027; 1.232) and in the South Region (17.7%; 1.044; 1.328). After an abrupt reduction, there was a progressive increase in risk for the black and brown population and for those with higher education. In most other population strata, there was a progressive increase in the risk of suicide. Conclusions: the Brazilian economic recession caused different effects on suicide rates, considering social strata, which requires health strategies and policies that are sensitive to the most vulnerable populations.

RESUMEN Objetivos: analizar tendencias de tasas de suicidio en Brasil, antes y después del inicio de la recesión económica. Métodos: estudio de series de tiempo interrumpido utilizando datos nacionales de suicidio registrados entre 2012 y 2017 con análisis por subgrupos socioeconómicos. Modelo de regresión quasi-Poisson empleado para analizar tendencias de datos ajustados estacionalmente. Resultados: observado aumento abrupto en el riesgo de suicidio pos recesión económica en la población con menor escolaridad (12,5%; RR = 1,125; IC95%:1,027; 1,232) y en la Región Sur (17,7%; 1,044; 1,328). Pos reducción abrupta, ocurrió aumento progresivo en el riesgo para la población de negros y pardos y de mayor escolaridad. En la mayoría de los demás estratos poblacionales, verificado aumento progresivo en el riesgo de suicidio. Conclusiones: la recesión económica brasileña produzco efectos diferentes en las tasas de suicidio, considerando los estratos sociales, lo que demanda estrategias de salud y políticas sensibles a poblaciones más vulnerables.

RESUMO Objetivos: analisar as tendências nas taxas de suicídio no Brasil, no período antes e depois do início da recessão econômica. Métodos: estudo de séries temporais interrompidas utilizando dados nacionais de suicídio registrados no período entre 2012 e 2017 com análises por subgrupos socioeconômicos. Modelo de regressão quasi-Poisson foi empregado para analisar as tendências dos dados ajustados sazonalmente. Resultados: observou-se aumento abrupto no risco de suicídio após recessão econômica na população com menor escolaridade (12,5%; RR = 1,125; IC95%:1,027; 1,232) e na Região Sul (17,7%; 1,044; 1,328). Após redução abrupta, ocorreu aumento progressivo no risco para a população de pretos e pardos e na de maior escolaridade. Na maioria dos demais estratos populacionais, verificou-se aumento progressivo no risco de suicídio. Conclusões: a recessão econômica brasileira produziu efeitos diferentes nas taxas de suicídio, considerando os estratos sociais, o que demanda estratégias de saúde e políticas sensíveis às populações mais vulneráveis.

Necrolisis epidérmica tóxica en el curso de un síndrome hemofagocítico secundario a linfoma de Hodgkin: caso clínico / Toxic epidermal necrolysis during a hemophagocytic syndrome secondary to Hodgkin lymphoma: report of one case

Toxic epidermal necrolysis (TEN) is a lethal entity, characterized by extensive epidermal necrosis and multiorgan failure. Hemophagocytic syndrome (HFS) is also a rare and lethal syndrome characterized by hyperinflammation that leads to the appearance of fever, pancytopenia, organomegaly and hemophagocytosis. The concomitance of these diseases is extremely uncommon. We report a 38 years old female, who during the course of a HFS secondary to Hodgkin Lymphoma (HL), presented a TEN secondary to antibiotics. She was admitted due to a consumptive syndrome, lymphadenopathy, visceromegaly and severe pancytopenia. Laboratory and bone marrow tests confirmed HFS. Due to constant fever, imipenem was indicated. On the third day she started with pain and skin rash. She evolved with positive Nikolsky sign. Cutaneous biopsy was concordant with extensive TEN, which was managed with intravenous immunoglobulin and dexamethasone. A complete response and normalization of the blood count were achieved. Finally, the lymph node biopsy showed HL of mixed cellularity type, which was managed with 8 cycles of ABVD chemotherapy, achieving complete remission.

Hipercalcemia, peak monoclonal y leucemización como presentación de un linfoma de células B de alto grado, con reordenamiento de MYC, BCL-2 y BCL-6 (Triple Hit). Caso clínico / Hypercalcemia, monoclonal peak and leukemic presentation of a High-grade B-cell lymphoma with MYC, BCL-2 and BCL-6 translocations (triple hit). Report of one case

High-grade B-cell lymphomas with rearrangement of MYC, BCL-2 and/or BCL-6 were introduced by the update of the WHO classification of lymphoid neoplasms. They usually present unique morphological and molecular characteristics, with an aggressive clinical outcome and worse prognosis. We report a 48 year-old female patient presenting with B symptoms and enlarged lymph nodes. Blood count showed pancytopenia and peripheral blood smears showed large lymphoid cells, some with nuclei and vacuoles. LDH was 3524 g/L and serum calcium was 11.5 mg/dL. Flow cytometry immunophenotyping showed pathological mature B lymphocytes. Protein electrophoresis showed a slight monoclonal peak. The biopsy disclosed a triple expressor diffuse large B-cell lymphoma, arising from germinal center. FISH was positive for MYC, BCL-2 and BCL-6 (triple hit) with a clonal evolution. Conventional cytogenetics showed a complex karyotype. Chemotherapy was started with R-CHOP (Rituximab/cyclophosphamide/doxorubicin/vincristine/prednisone). She developed impaired consciousness; the brain CT scan showed a large brain mass. The patient died within 3 weeks.

Granulomas pulmonares y en médula ósea: más allá de la tuberculosis. A propósito de un caso / Differential diagnosis of bone marrow and lung granulomas: Report of one case

The differential diagnosis of pulmonary tuberculosis and lymphoma with pulmonary infiltration is very difficult, given their similar clinical characteristics. We report a 59 year old female with weight loss, fever, dyspnea and cough of several months of duration. She had a cavitated mass in lung imaging. A positive conventional PCR lead to the diagnosis of tuberculosis, but she had negative smears and cultures for Mycobacterium tuberculosis. The patient did not respond to treatment and her clinical condition worsened. A peripheral lymph node biopsy confirmed the presence of a diffuse large B cell non-Hodgkin lymphoma. Bone marrow pathology showed non caseating granulomas, again with negative microbiological tests for M. tuberculosis. Findings in the bone marrow were interpreted as a secondary sarcoid reaction to cancer and PCR results as a false positive. The lymphoma was treated, achieving complete remission. This case highlights the importance of the differential diagnosis between these two entities.

Phenolic composition, antioxidant and anti-proliferative activities of edible and medicinal plants from the Peruvian Amazon

ABSTRACT Among 23 extracts of medicinal and edible plants tested, Mauritia flexuosa L.f., Arecaceae, showed significant antioxidant ability (DPPH and ORAC = 1062.9 and 645.9 ± 51.4 µg TE/mg extract, respectively), while Annona montana Macfad., Annonaceae, demonstrated the most promising anti-proliferative effect (IC50 for Hep-G2 and HT-29 = 2.7 and 9.0 µg/ml, respectively). However, combinatory antioxidant/anti-proliferative effect was only detected in Oenocarpus bataua Mart., Arecaceae (DPPH = 903.8 and ORAC = 1024 µg TE/mg extract; IC50 for Hep-G2 and HT-29 at 102.6 and 38.8 µg/ml, respectively) and Inga edulis Mart., Fabaceae (DPPH = 337.0 and ORAC = 795.7 µg TE/mg extract; IC50 for Hep-G2 and HT-29 at 36.3 and 57.9 µg/ml, respectively). Phenolic content was positively correlated with antioxidant potential, however not with anti-proliferative effect. None of these extracts possessed toxicity towards normal foetal lung cells, suggesting their possible use in development of novel plant-based agents with preventive and/or therapeutic action against oxidative stress-related diseases.

Análisis de la distribución geográfica de médicos especialistas en la República Argentina / Analysis of the geographical distribution of medical specialists in the Argentine Republic

A lo largo de las tres últimas décadas, muchos países de Latinoamérica y el Caribe (LAC) han reconocido la salud como un derecho humano y han actuado de acuerdo a ese reconocimiento. En consecuencia las exigencias a los sistemas de salud han aumentado de forma sostenida con el objetivo de responder mejor a la prestación de servicios. De ahí que se hayan promovido y puesto en marcha políticas y programas encaminados a lograr la cobertura universal de salud(1). En este contexto las políticas de Recursos Humanos han adquirido, si cabe, una mayor relevancia.