Leprosy in a Patient With Lymphoma: A Challenge in the Twenty-First Century.

Leprosy, or Hansen's disease, mistakenly considered a disease from the past by some, is still common nowadays, especially in tropical and subtropical regions. In the absence of appropriate medical treatment, it may progress and cause permanent damage to multiple organs. This case report illustrates the diagnostic challenge of a south-american adult man who had been living in Europe for over 14 years. He was referred to the Hematology department due to persistent lymphocytosis and a CD5+ B-cell lymphoproliferative disorder was identified. During clinical surveillance, the patient developed skin lesions in his limbs with associated hypoesthesia. A histological diagnosis of lepromatous leprosy was made, and he underwent a long-term three-drug therapeutic regimen (dapsone, rifampicin, and clofazimine). Adding to the complexity of the case, the patient progressed with splenomegaly and constitutional symptoms, more than 7 years after development of lymphocytosis. Through a comprehensive evaluation, a definitive diagnosis of mantle cell lymphoma was established and received 6-cycle R-CHOP induction, followed by maintenance rituximab. Importantly, prophylaxis for leprosy reactivation was not administered as there were no recommendations in available guidelines. Eventually, the patient experienced a leprosy relapse while on maintenance therapy, 58 months after completing the initial anti-leprous treatment. Clinical response was attained with a new treatment regimen consisting of rifampicin, clofazimine, and minocycline.  Although leprosy is primarily observed in tropical and subtropical regions, the long incubation period of this disease combined with the global flow of migrants, made us consider it. Despite being rare, leprosy relapses can occur even after a few decades. The contribution of rituximab or previously administered chemotherapeutic agents is still unknown. The question remains whether antibiotic prophylaxis should be performed in patients undergoing immunochemotherapy for malignant diseases.

Adolescent Pregnancy: A Case-Series Study of 112 Adolescent Mothers and Their Newborns.

Background Although declining, adolescent pregnancy remains an important health concern and is associated with adverse maternal and fetal outcomes. We aimed to characterize adolescent pregnancy in a level II hospital and the observed maternal, fetal, and perinatal complications. Methods We conducted a case-series study of five-years duration with adolescent mothers and their newborns. We collected sociodemographic, obstetric, and neonatal data through chart review and conducted a comparison analysis between newborns who needed hospitalization and those who did not. Results We identified 112 newborns with adolescent mothers. Most pregnancies were unplanned (89.3%) and the start of pregnancy surveillance was late. The most frequent complications were intrauterine growth restriction, oligohydramnios, and threatened preterm labor. Prematurity was found in 9.8% of the newborns and 0.9% had less than 32 weeks at the time of birth. Thirteen newborns (11.6%) needed hospitalization in the neonatal intensive care unit, with three (23%) needing invasive ventilation. The main diagnoses of admission were prematurity, social risk, low birth weight, feeding difficulties, and newborn respiratory distress syndrome. Hospitalization seemed associated with less frequent breastfeeding. Among the hospitalized newborns, there was a high rate of discharge at the care of a relative or an institution (30.8%). Surprisingly, surveillance during pregnancy did not seem to differ between newborns that needed hospitalization and those that did not. Discussion Adolescent pregnancy is associated with poor surveillance as well as obstetric and neonatal complications. Newborns of adolescent mothers have a high hospitalization rate, but further investigations are needed to fully understand the contributing factors. The creation of multidisciplinary teams is fundamental for reducing complications, and appropriate reproductive health programs should focus on reducing adolescent pregnancy through better access to education and contraception.

Caloric intake and weight gain in a neonatal intensive care unit.

The aim of this paper was to study the weight gain in very-low-birthweight (VLBW) infants by adopting earlier and higher intake of proteins and earlier intake of lipids. We studied 28 VLBW infants admitted to Neonatal Intensive Care Unit during the year 2004 (group 1) and 18 during the first semester of 2006 (group 2). Dietary intakes for group 1 were: 1 g kg(-1) day(-1) of proteins started at postnatal day 2 (P2) and 0.5-1 g kg(-1) day(-1) of lipids at P3; for group 2, 1-1.5 g kg(-1) day(-1) of proteins and 0.5-1 g kg(-1) day(-1) of lipids, both started at P1. Caloric intake was significantly higher in group 2 (p < 0.05), whereas cumulative nutritional deficit was higher in group 1 (p < or = 0.01). Weight z scores were significantly lower at discharge comparing with z scores at birth for each group (p < or = 0.01), with no differences between the two groups. Despite a higher protein intake which resulted in a lower nutritional deficit, the weight z score did not improve significantly at discharge.

[Immunocytoma IgA. Case report]. / Imunocitoma IgA. A propósito de um caso clínico.

Immunocytoma is a non-Hodgkin's indolent evolution B cell lymphoma. It accounts for approximately 1-3% of non-Hodgkin's limphomas and usually onsets in adults aged over 50 years old. It manifests as lymphadenopathy, splenomegaly, hepatomegaly and lymphcytosis in 15 -30% of cases and is rarely seen with pulmonary involvement. Monocloncal peaks of serum immunoglobulin often occur. These are IgM and rarely IgA. We present as an example a male patient aged 52 years old, with recurrent respiratory infections. Clinical work -up identified an immunocytoma IgA stage IV. Diagnosing an indolent lymphoma, we prophylactic polyspecific human immunoglobulin to treat the respiratory infection. Evidence of lymphoma progression leads us to prescribe combined cyclo- phosphamide (C), vincristine (V), prednisone (P) e rituximab (R) (CVP-R), which has obtained a partial response over two years.

Late-onset neonatal sepsis: A cinetobacter ursingii as an infectious agent.

The authors report a case of a premature male newborn admitted to the neonatal intensive care unit after an emergent caesarean due to maternal pre-eclampsia and foetal bradycardia at 32 weeks of gestational age and birth weight of 1440 g. There were no infection risk factors reported. On day 3 his clinical condition deteriorated, with tachycardia and subfebrile temperature and C-reactive protein at 1.25 mg/dL. Empirical antibiotics (flucloxacillin and gentamicin) were started, with no clinical improvement and C-reactive protein increasing to a maximum of 19 mg/dL (upper normal level of 1 mg/dL) after 3 days. Blood cultures from the third to the eighth day of life were positive for Acinetobacter ursingii Targeted therapy was administered for 14 days with clinical and laboratorial improvement and he was discharged on the 28th day of life without any known sequelae. A. ursingii is emerging as an infectious agent of late-onset sepsis in immunosuppressed neonates.

Internal jugular vein tumor thrombus due to parapharyngeal extraosseous plasmacytoma.

Tumor thrombosis of the internal jugular vein (IJV) represents an uncommon event, usually in the setting of underlying thyroid neoplasms. Extraosseous plasmacytoma (EMP) with tumor thrombosis of the IJV has not yet been reported in the literature. We present a unique case of a plasmacytoma in the left parapharyngeal space with direct extension to the left IJV, documented with contrast enhanced computed tomography and US Doppler. Presence of avid thrombus enhancement allowed differentiation between tumoral extension and thrombothic changes.

Boosting bioethanol production from Eucalyptus wood by whey incorporation.

The mixture of Eucalyptus globulus wood (EGW) and cheese whey powder (CWP) was proposed for intensification of simultaneous saccharification and fermentation (SSF) at high temperature and solid loadings using the industrial Saccharomyces cerevisiae Ethanol Red® strain. High ethanol concentration (93 g/L), corresponding to 94% ethanol yield, was obtained at 35 °C from 37% of solid mixture using cellulase and ß-galactosidase enzymes (24.2 FPU/g and 20.0 U/g, respectively). The use of CWP mixed with pretreated EGW increased the ethanol concentration in 1.5-fold, in comparison with SSF experiments without CWP for both Ethanol Red® and CEN.PK113-7D strains. Moreover, 1.4-fold higher ethanol concentration was obtained with Ethanol Red®, in comparison with CEN.PK113-7D strain. Ethanol Red® strain was genetically engineered for ß-galactosidase production in order to advance towards a fully integrated process. This work shows the feasibility of attaining high ethanol concentrations in second generation bioprocesses by a multi-waste valorization approach.

Neonatal morbidity and outcome of live born premature babies after attempted illegal abortion with misoprostol.

UNLABELLED: Misoprostol is a synthetic prostaglandin currently employed to induce labor. Association with illegal abortion has been reported; however, neonatal outcome and morbidity after a failed attempt of abortion has not been described. OBJECTIVES: To report the association between misoprostol self-medication and preterm labor and to assess perinatal risk factors, morbidity and early outcomes. METHODS: We conducted a prospective study of all very low birth weight (VLBW) infants delivered in Hospital Fernando Fonseca, during a 5-year period. VLBW infants were assigned to misoprostol group (MG) when preterm delivery was attributed to misoprostol and matched with newborns with similar gestational age, birth- weight, and gender. RESULTS: During the study period 311 VLBW infants were born. Nineteen belonged to misoprostol group (MG) and 58 were selected for controls. Mothers from MG were significantly younger (21.5 vs 27.5, p = 0.001) and from African origin (74 vs 31%, p = 0.006), had significantly less prenatal care (21 vs 67%, p = 0.000), less antenatal steroids (5 vs 50%, p = 0.001), and were delivered less often by C-section (11 vs 60%, p = 0.000). MG infants had significantly higher rates of patent ductus arteriosus (58 vs 29%, p = 0.031) and chronic lung disease (47 vs 14%, p = 0.026). Mortality rate at 3 months was similar in both groups, but the incidence of abnormal neurodevelopment at 1 year of age was significantly higher in the MG (50 vs 16%, p= 0.02). COMMENTS: The worse outcome of MG infants seems to be determined by prenatal, natal, and postnatal factors and we believe that misoprostol is probably a significant adjuvant cofactor.

Aggressive mature natural killer cell neoplasms: report on a series of 12 European patients with emphasis on flow cytometry based immunophenotype and DNA content of neoplastic natural killer cells.

We report 12 cases of aggressive natural killer (NK) cell neoplasms diagnosed in Portugal, with emphasis on flow cytometry. Ten patients had extranodal NK/T cell lymphoma, nasal type and two had aggressive NK cell leukemia, and seven were men and five were women, with a median age of 50 years. NK cells brightly expressed the CD56 adhesion molecule and CD94 lectin type killer receptor and had an activation-related HLA-DR+ CD45RA+ CD45RO+ immunophenotype, in most cases. In contrast, dim CD16 expression was found in a minor proportion of cases, whereas CD57 and the CD158a and CD158e1 killer immunoglobulin-like receptors were negative. One-third of cases showed a hyperploid DNA content and nearly all had a very high S-phase proliferative rate. The phenotypic features of the neoplastic NK cells would suggest that they represent the transformed counterpart of the CD56 + bright NK cells that circulate in normal blood.

Hematological remission and long term hematological control of acute myeloblastic leukemia induced and maintained by granulocyte-colony stimulating factor (G-CSF) therapy.

We describe a case of a patient with CD34+, TdT+, CD13-, CD33-, MPO- undifferentiated acute leukemia who refused chemotherapy and who achieved complete hematological remission 14 months after the diagnosis, during a short course of granulocyte-colony stimulating factor (G-CSF) for neutropenia and life threatening infection. Relapse occurred approximately one year later and G-CSF was reintroduced, being maintained for 4 months, at a dose and frequency adapted to maintain normal blood counts, a complete hematological remission being achieved again. Five months after withdrawing the G-CSF therapy a second relapse was observed; G-CSF was tried again with success, resulting in a very good hematological response that was sustained by G-CSF maintenance therapy. One year latter there was the need of increasing the doses of G-CSF in order to obtain the same hematological effect, at same time blast cells acquired a more mature CD34+, TdT-, CD13+, CD33-, MPO+ myeloid phenotype. Finally, the patient developed progressive neutropenia, anemia, thrombocytopenia and acute leukemia in spite of G-CSF therapy, dying 64 months after initial diagnosis (50 months after starting G-CSF therapy) with overt G-CSF resistant acute myeloblastic leukemia (AML), after failure of conventional induction chemotherapy.

Atopic dermatitis-like non-erythrodermic leukemic variant of CD3(-/+dim) CD4(+) cutaneous T-cell lymphoma preceded by cutaneous papular xanthomatosis.

We report a patient with cutaneous papular xanthomatosis who 4 years later developed a CD3(-/+dim)/CD4(+) T-cell lymphoma. Pruritic xerotic non-erythrodermic skin, eosinophilia and hyper-IgE were present and erroneously classified as atopic dermatitis. Flow cytometry and DNA ploidy analysis of both blood and skin lymphocytes, skin histology and blood T-cell receptor gene rearrangement studies confirmed diagnosis of T-cell lymphoma. Monoclonal CD3(-/+dim)/CD4(+) T-cells were especially prone to the synthesis of IL-13, a cytokine that is involved in IgE-secretion, and comprised both a medium (diploid) and large (hyperploid) sized T-cell populations with a similar immunophenotype. The majority of the normal residual T-cells were large granular lymphocytes, expressed activation-related and natural-killer-associated markers and secreted high levels of interferon gamma, suggesting that they might correspond to active cytotoxic cells directed against the neoplastic T-lymphocytes.

Serviço de Apoio Domiciliário como substituição ou complemento do cuidado familiar / Home Care as substitution or complementary of familiarly care / Servicio de Apoyo Domicilio como sustitución o complemento del cuidado familiar

Este estudo exploratório aborda o papel do Serviço de Apoio Domiciliário no quotidiano dos idosos e suas famílias, em função do contexto urbano e rural, e o percurso de vida dos utentes de dois SAD do norte de Portugal. Recorreu-se a uma metodologia qualitativa, com a aplicação de 11 entrevistas semiestruturadas. O SAD mantém uma função assistencialista/tradicional, tendo um papel de substituição em meio urbano e complementar em meio rural. Este estudo aponta para a necessidade de apostar numa lógica de prevenção, educação e reabilitação, em torno da criação de um plano de cuidados personalizado, baseado num sistema de classificação dos utentes.

This exploratory study deals with the role of the Domiciliary Support Service in the daily life of the elderly and their families, depending on the urban and rural context and the life course of the users of two SADs in the north of Portugal. A qualitative methodology was used, with the application of 11 semi-structured interviews. The SAD maintains a welfare / traditional function, having primarily a substitution role in an urban and complementary environment in rural areas. This study points to the need of betting on a logic of prevention, education and rehabilitation, around the creation of a personalized care plan, based on a classification system of the users.

Este estudio exploratorio aborda el papel de la atención domiciliaria de la vida cotidiana de las personas mayores y sus familias, de acuerdo con el contexto urbano y rural y el modo de vida de los usuarios del SAD dos norte de Portugal. Se recurrió a una metodología cualitativa, con la aplicación de 11 entrevistas semiestructuradas. El SAD mantiene una función asistencialista / tradicional, teniendo un papel de sustitución en medio urbano y complementario en medio rural. Este estudio apunta a la necesidad de apostar en una lógica de prevención, educación y rehabilitación, en torno a la creación de un plan de cuidados personalizado, basado en un sistema de clasificación de los usuarios.

Incontinentia pigmenti in the neonatal period.

Incontinentia pigmenti (IP) is a rare multisystem disease, X linked dominant disorder. As all X linked dominant diseases, it is usually male-lethal. Female newborn admitted to the neonatal intensive care unit on the first day of life was diagnosed as having probable herpetic infection with vesicular skin lesions distributed on upper right limb and inferior limbs. Family history showed that her 22-year-old mother had hypopigmented lesions on the lower limbs and her 13-month-old sister had hyperpigmented lesions on the trunk and limbs. In newborns, herpes infection emerges as the principal diagnosis of vesicular rash, due to the importance of precocious diagnosis and treatment. Other hypothesis must be considered in a newborn with vesicobullous rash, such as IP.

Nutritive sucking pattern--from very low birth weight preterm to term newborn.

UNLABELLED: The contribution of maturation and stimulation to the development of oral feeding was investigated, with two main objectives: (1) to analyze the nutritive sucking pattern of very-low-birth-weight newborns from their first oral feeding to the acquisition of independent oral feeding, and (2) to compare the nutritive sucking patterns of these babies, after feeding autonomy, with healthy term newborns. METHODS: Two groups were considered for analysis. Group 1: N=15 Very-Low-Birth-Weight (VLBW), gestacional age (GA)=28.15+/-1.5, birth weight (BW)=1178.3+/-174.4. The intervention program began at 30.19+/-1.52 weeks GA. Group 2: N=25 term newborns, healthy, GA=39.04+/-1.2, BW=3370.42+/-310.76. Repeated measures of the following variables were taken (weekly for group 1): suction efficacy (SEF), rhythm of milk transfer (RMT), suctions, bursts and pauses. Group 2 was analysed only once between the 2nd and 5th day of life. RESULTS: Group 1 has revealed a minimal suction number at 32 GA weeks (82+/-77.6) and maximal suction number at 36-37 GA weeks (162.7+/-60.7). The number of sucks seemed to be dependent of weight (p=0.005), duration of intervention (p=0.001) and chronological age (p=0.000). Significant statistical effects of gestational age were not observed (p=0.904). Sucks in bursts represented 77% at the beginning of oral feeding (32 weeks GA), and 96% at 33 weeks GA, remaining constant thereafter. The number of sucks and bursts increased with GA and weeks of feeding. The mean duration of the pauses decreased from first to fourth week of feeding (week1=14.1+/-9.1 and week4=6.4+/-1.4 s). The sucking efficacy (SEF) was better explained by weight (p=0.000), number of sucks in 5 min (p=0.025) and chronological age (p=0.044). Gestational age (p=0.051) and nutritive intervention duration (NDI) (p=0.110) did not contribute to explain SEF. Despite the observation of significant statistical differences between groups regarding GA (35.9/39.08; p=0.00), chronological age (53.3/2.5; p=0.00) and weight (1875/3360; p=0.00), the nutritive suction pattern was not statistically different between groups after feeding autonomy. CONCLUSION: in VLBW oral feeding before 32 weeks GA allows the attainment of a mature nutritive suction pattern before term (37-40 weeks). Experience seems to be one of the influencing factors in the change of the nutritive suction pattern.

Congenital laryngomucocoele: a rare cause for CHAOS.

Congenital high airway obstruction syndrome (CHAOS) is a rare but life-threatening condition that results from the obstruction of the upper airways.We describe a female newborn, from a Grávida II, Para 0, 36-year-old woman, with a routine ultrasound at 30 weeks' gestation that showed polyhydramnios. She delivered a live-born female baby at 36 weeks without any dismorphic features but with respiratory distress. Attempts at endotracheal intubation were unsuccessful due to the presence of a mass obstructing the larynx. The reanimation process was stopped after 20 minutes. Post-mortem examination demonstrated the presence of a total occlusion of the larynx by a laryngomucocoele. Laryngocele, a congenital cyst of the larynx, occurs rarely and hardly ever as a cause of CHAOS. What is more, laryngomucocoele has not been previously reported as a cause of CHAOS. These conditions represent a neonatal emergency with reserved prognosis unless diagnosed antenatally allowing for a programmed ex utero intrapartum treatment (EXIT) by performing tracheostomy while maintaining the placental circulation.