A case of pediatric HIV myelopathy. / Un caso de mielopatia pediatrica por VIH.

TITLE: Un caso de mielopatia pediatrica por VIH.

The cancer registry as an ally in monitoring treatment effectiveness.

OBJECTIVE: To evaluate if the cancer registry database can be used to monitor treatment effectiveness using nivolumab treatment of non-small cell lung cancer (NSCLC) as an example. METHOD: An observational inception cohort was used, where all registered cases of NSCLC with authorisation to initiate treatment with nivolumab were monitored retrospectively to evaluate disease characteristics and response to prior treatments. Current exposure to nivolumab was prospectively characterised and treatment outcomes classified based on the clinical information registered in the patient medical record. The main outcome measure used to assess treatment effectiveness was overall survival (OS). Secondary outcomes considered were progression free survival (PFS) as a measure of effectiveness and occurrence of Adverse Drug Reaction (ADRs) as a measure of safety. Data were analysed using SPSS, version 24. RESULTS: A total of 115 patients received treatment with nivolumab for NSCLC, between November 1st 2015 and July 31st 2016, and were registered in the database. The majority were non-squamous type (n=107). The median OS was 11.4 months {CI95%: 11.1-11.7}, with a 1-year survival of 44%, in line with clinical trial data. Median PFS was 5.4 months {CI95%: 2.8-7.9}. Treatment was discontinued in 82 cases, most frequently due to disease progression. There were 38 cases of ADRs documented in the patient medical chart, 21 of which led to treatment discontinuation. CONCLUSION: The analysed data suggest that the cancer registry is a powerful tool to monitor treatment effectiveness, although considerable investment is needed to improve the medical culture of recording treatment exposure, particularly documentation of ADRs.

Postinfectious Anti-Myelin Oligodendrocyte Glycoprotein Antibody Positive Optic Neuritis and Myelitis.

We report the case of a 9-year-old girl admitted with fever, headache, and a cerebrospinal fluid lymphocytic pleocytosis. Polymerase chain reaction was positive for human herpes virus 6. She subsequently developed ataxia and bilateral loss of vision. Magnetic resonance imaging (MRI) showed bilateral optic nerve lesions with extension to optic chiasm and a short-segment myelitis. Serologic studies were positive for Borrelia burgdorferi IgM. Anti-aquaporin 4 antibody was negative and anti-myelin oligodendrocyte glycoprotein antibody (MOG) positive. After intravenous methylprednisolone, ceftriaxone, and intravenous immunoglobulin, her vision slowly recovered. The patient was discharged with only mild visual acuity loss, 1 month after admission. Brain MRI was repeated later and was normal and MOG assay became negative. In our view, this patient suffered from a postinfectious, anti-MOG-mediated, spinal cord and optic nerve demyelination.

Cost comparison of pressure ulcer preventive dressings: hydrocolloid dressing versus transparent polyurethane film.

OBJECTIVE: To evaluate and compare the costs of using a transparent polyurethane film (PF) and hydrocolloid dressing (HD) in the prevention of pressure ulcers (PUs). METHOD: This descriptive, observational, longitudinal, comparative study was conducted in the intensive care units, coronary care unit and medical clinic of a charity hospital in Brazil. Data were collected during a 30-day study period, consisting of physical examination, assessment of risk factors for PU development and application of the Braden scale, which were performed at inclusion in the study and once daily during hospitalisation. Either PF or HD was applied bilaterally in the sacral and trochanteric regions for prevention of PUs in patients at a moderate to high risk of PUs according to the Braden scale, and costs of using PU preventive dressings were estimated. RESULTS: The mean total costs per dressing change per patient when using the HD and PF to prevent PUs were 413.60 BRL and 74.04 BRL, respectively. There were significant between-group differences in mean costs for all variables, except for saline solution and nurse-technician services. CONCLUSION: Results showed that the mean cost per dressing change per patient was lower when using the transparent PF than when using the HD.

Impact of surgical wound dehiscence on health-related quality of life and mental health.

OBJECTIVE: To assess the impact of surgical wound dehiscence on health-related quality of life and mental health. Dehiscence of surgical wounds is a serious postoperative complication associated with high morbidity and mortality. METHOD: Sixty-one adult patients, who had undergone neurological, general, colorectal, orthopaedic, gynaecological, plastic, cardiovascular, urological or neurological surgery in a university hospital in Brazil, were assessed between 30 and 180 days after surgery. Twenty participants who achieved complete wound healing were allocated to the control group and 41 participants who developed surgical wound dehiscence were allocated to the dehiscence group. Patients unable to complete the questionnaires because of cognitive impairment and those who declined to participate or died were excluded from the study. Data were collected using a questionnaire assessing sociodemographic and clinical characteristics of participants; the Medical Outcomes Study 36-Item Short Form Health Survey (SF-36); and the Beck Depression Inventory (BDI). RESULTS: Surgical wound dehiscences were 0.5-30 cm in length, 0.5-7 cm in depth, and located in the arms, legs or trunk. There were significant between-group differences in mean scores on the physical functioning (p<0.01), role physical (p<0.01), social functioning (p=0.01), and bodily pain (p=0.01) dimensions of the SF-36. Participants with wound dehiscence reported significantly higher BDI scores (more depressive symptoms) than controls (p=0.01). CONCLUSION: Surgical wound dehiscence had a negative impact on the physical functioning, role physical, social functioning, and bodily pain dimensions of health-related quality of life and on mental health. DECLARATION OF INTEREST: No conflict of interest to declare.

Detection of Dientamoeba fragilis in Portuguese children with acute gastroenteritis between 2011 and 2013.

Dientamoeba fragilis is an inhabitant of human gastrointestinal tract with a worldwide distribution. The first description considered this protozoan a rare and harmless commensal, since then it has struggled to gain recognition as a pathogen. Commercial multiplex real-time PCR was used to detect D. fragilis in fecal samples from hospitalized children (⩽18 years) with acute gastrointestinal disease, admitted to two hospitals of Lisbon area, with different demographic characteristics. A total of 176 children were studied, 103 (58·5%) male, 144 (81·8%) children between 0 and 5 years and 32 (18·2%) above 6 years old. The overall protozoa frequency considering the four tested microorganisms were 8·5% (15/176), and the most frequently found protozoan was D. fragilis, 6·3% (11/176). Dientamoeba fragilis frequency was higher among older children (21·9%), than younger children (2·8%), and greater in boys (6·8%) than in girls (5·5%). All positive children presented with diarrhoea associated with vomiting, fever and abdominal pain. Infection was associated with the age of children (P < 0·001), school attendance (P = 0·002) and consumption of certain foods (P = 0·014), e.g. cakes with crème and ham. The frequency of diantamoebiasis found in a cohort of hospitalized Portuguese children, with acute gastrointestinal disease, could be considered a very high value when compared with the protozoan frequency normally associated with this pathology.

Using transparent polyurethane film and hydrocolloid dressings to prevent pressure ulcers.

OBJECTIVE: To compare the performance and effectiveness of a hydrocolloid dressing (HD) and a transparent polyurethane film (PF) in preventing pressure ulcer (PU) development. METHOD: The study was conducted in the intensive care unit, coronary care unit and medical clinic of the Holy House of Mercy of Passos, Brazil. Data were collected 48 hours after admission and during hospitalisation. The Braden scale was used for PU risk assessment. Consecutive eligible patients without PUs were randomly assigned by lottery to the two groups, either the HD or PF group. RESULTS: Of the 160 eligible patients, significant between-group differences were found in the mean total number of dressing changes (HD, 6.09±1.655 changes; PF, 5.59±2.036 changes; p=0.010), and mean number of dressing changes in the sacral region (HD, 2.50±0.871; PF, 2.05±0.825; p=0.001), with the PF group requiring significantly fewer changes than the HD group. The most common reasons for changing dressings in both groups were moisture (PF 51.1%; HD 47.9%) and shear (HD 43%; PF 38.9%), with a significant difference in shear between groups. The incidence of PUs was significantly lower (p=0.038) in the PF group (8.7%) compared with that in the HD group (15%). CONCLUSION: The results suggest that the transparent polyurethane film had a better performance and was more effective than the hydrocolloid dressing in preventing PU development.

Impact of compression therapy using Unna's boot on the self-esteem of patients with venous leg ulcers.

OBJECTIVE: To assess self-esteem in patients with venous leg ulcers treated with Unna's boot. • METHOD: A descriptive, analytic, clinical study was conducted from June 2010 to May 2011 in an outpatient wound care clinic in São Paulo, Brazil. Patients of both sexes, aged ≥18 years, who had had a venous leg ulcer for more than one year and a Doppler ankle brachial index ranging from 0.8-1.0 were consecutively selected for inclusion. Patients were treated with wound dressings and Unna's boot. Self-esteem was assessed using the Brazilian version of the Rosenberg Self-Esteem Scale (RSE) at inclusion (baseline) and after 4, 8, and 12 months of compression therapy using Unna's boot. The scale is reverse-scored; thus lower scores indicate higher levels of self-esteem. • RESULTS: The patients showed a slight but significant improvement in self-esteem after 4 months of treatment (mean RSE score=17.12) compared with baseline (mean RSE score=24.90). However, a marked and significant improvement in self-esteem was observed after 8 months (mean RSE score=7.40) and 12 months (mean RSE score=2.10) of compression therapy using Unna's boot. • CONCLUSION: Patients with venous leg ulcers treated with Unna's boot for 12 months showed a significant improvement in self-esteem • DECLARATION OF INTEREST: All authors declare that no competing financial interests exist. There was no external funding for this study.

Decreasing incidence and changes in serotype distribution of invasive pneumococcal disease in persons aged under 18 years since introduction of 10-valent and 13-valent conjugate vaccines in Portugal, July 2008 to June 2012.

The 10-valent pneumococcal conjugate vaccine (PCV10) became available in Portugal in mid-2009 and the 13-valent vaccine (PCV13) in early 2010. The incidence of invasive pneumococcal disease (IPD) in patients aged under 18 years decreased from 8.19 cases per 100,000 in 2008­09 to 4.52/100,000 in 2011­12. However, IPD incidence due to the serotypes included in the 7-valent conjugate vaccine (PCV7) in children aged under two years remained constant. This fall resulted from significant decreases in the number of cases due to: (i) the additional serotypes included in PCV10 and PCV13 (1, 5, 7F; from 37.6% to 20.6%), particularly serotype 1 in older children; and (ii) the additional serotypes included in PCV13 (3, 6A, 19A; from 31.6% to 16.2%), particularly serotype 19A in younger children. The decrease in serotype 19A before vaccination indicates that it was not triggered by PCV13 administration. The decrease of serotype 1 in all groups, concomitant with the introduction of PCV10, is also unlikely to have been triggered by vaccination, although PCVs may have intensified and supported these trends. PCV13 serotypes remain major causes of IPD, accounting for 63.2% of isolates recovered in Portugal in 2011­12, highlighting the potential role of enhanced vaccination in reducing paediatric IPD in Portugal.

[Protracted Febrile Myalgia Syndrome with Henoch-Schönlein Purpura: an atypical presentation of Familial Mediterranean Fever]. / Protracted Febrile Myalgia Syndrome com Púrpura de Henoch-Schönlein: forma atípica de apresentação de febre mediterrânica familiar.

Familial Mediterranean Fever (FMF) is an hereditary autosomal recessive disease characterized by recurrent attacks of fever, arthritis and serositis: peritonitis, pleurisy and/or pericarditis. Its main complication is systemic AA amyloidosis. The authors present a case of a 8-years-old female child with african ancestry, who was admitted three times since 5 years-old with abdominal pain, fever and high acute phase reactants. At the first admission appendectomy was made and at the third hospital admission the clinical picture was accompanied by myalgia, purpuric lesions and non nephrotic proteinuria. A renal biopsy was performed and was compatible with Henoch-Schönlein nephritis. Serum Amyloid A protein had high levels - 92 mg/L (> 6.8) and a diagnosis of Familial Mediterranean Fever was confirmed by genetic test (homozygote for M694V in MEFV gene). She started colchicine and is doing well, without any further complaints. FMF must be considered in the differential diagnosis of recurrent attacks of fever and abdominal pain in children, even with an atypical presentation (p.e. Protracted Febrile Myalgia Syndrome). Genetic study allows the confirmation of the diagnosis and has prognostic implications.

High prevalence of ST121 in community-associated methicillin-susceptible Staphylococcus aureus lineages responsible for skin and soft tissue infections in Portuguese children.

In order to evaluate the incidence of community-associated methicillin-resistant Staphylococcus aureus (CA-MRSA) in Portugal, we analyzed a collection of 38 S. aureus isolates recovered from 30 children attending the pediatric emergency department of a central hospital in Lisbon due to skin and soft tissue infections. Molecular characterization identified seven clonal lineages among the 35 methicillin-susceptible S. aureus (MSSA) isolates, of which the major lineage PFGE A/t159/ST121 included 63% of the isolates. The three MRSA isolates belonged to the Pediatric clone PFGE D/t535/ST5-IV (n = 2) and to the European CA-MRSA clone PFGE G/t044/ST80-IVc (n = 1). All isolates harbored several virulence factors, namely, leukocidins. Panton-Valentine leukocidin (PVL) was produced by isolates from five MSSA lineages and by the ST80 MRSA. Of interest, this is the first reported isolation of CA-MRSA ST80 in Portugal.

Shift work at a modern offshore drilling rig.

The oil and gas exploration and production offshore units are classified as hazardous installations. Work in these facilities is complex, confined and associated with a wide range of risks. The continuous operation is secured by various shift work patterns. The objective of this study was to evaluate how offshore drilling workers perceived shift work at high seas and its impacts on their life and working conditions. The main features of the studied offshore shift work schedules are: long time on board (14 to 28 days), extended shifts (12 hours or more per day), slow rotation (7 to 14 days in the same shift), long sequence of days on the night shift (7 to 14 days in a row) and the extra-long extended journey (18 hours) on shift change and landing days. Interviews revealed a wide range of stressors caused by the offshore shift work, as well as difficulties to conciliate work with family life. It was observed that changes of the family model, leading to role conflicts and social isolation, work in a hazardous environment, perceiving poor sleep when working at night shifts and the imbalance between the expected and actual rewards are the major stressors for the offshore drilling workers.

Eleven novel APC mutations identified in Portuguese FAP families.

Germline mutations of the adenomatous polyposis coli (APC) gene are responsible for familial adenomatous polyposis (FAP), an autosomal dominant predisposition to colorectal cancer. In the present study we screened all of the exons of the APC gene in individuals belonging to 85 Portuguese FAP families. We here report eleven novel mutations which are predominantly frameshifts or single base substitutions, resulting in premature stop codons. Hum Mutat 16:178, 2000.

Duodenal-content reflux esophagitis induces the development of glandular metaplasia and adenosquamous carcinoma in rats.

Recent studies have demonstrated that refluxed duodenal contents cause esophageal carcinoma in rats without exposure to carcinogens. The histopathological spectrum of these carcinomas includes squamous-cell carcinoma, adenocarcinoma and adenosquamous carcinoma. Pure adenocarcinomas are thought to arise in areas of columnar metaplasia adjacent to the anastomosis, similar to Barrett's esophagus in humans. In contrast, the histogenesis of adenosquamous carcinomas is unclear. The purpose here was to investigate the pathogenesis of esophageal adenosquamous carcinomas in a time-course experiment of chronic duodenal-content reflux without carcinogen. Forty-two 8-week-old male Sprague-Dawley rats were divided into seven groups and exposed to duodenal-content esophageal reflux during 10, 15, 20, 25, 30, 35 and 40 weeks, respectively. All animals underwent an esophagojejunostomy with gastric preservation in order to produce chronic esophagitis. The rats received a standard diet without addition of carcinogens. An increasing incidence of glandular metaplasia and carcinoma was observed over the time course, starting at 20 weeks. After 40 weeks of reflux, multiple foci of glandular metaplasia and adenosquamous carcinoma were found in 83 and 50% of the animals, respectively. Most of the carcinomas occurred in the middle and proximal esophagus and had a dual pattern of differentiation, glandular and squamous. These findings confirm that duodenal content reflux alone has a carcinogenic effect. We propose that chronic duodenal reflux induces the development of metaplastic cells with glandular differentiation from the stem cells of squamous epithelium, and that glandular metaplastic foci are the morphological element from which tumors with a dual pattern of differentiation arise.

Cytokine levels (IL-6 and IFN-gamma), acute phase response and nutritional status as prognostic factors in lung cancer.

Raised serum levels of interleukin 6 (IL-6) have been described in cancer patients. This cytokine mediates the acute phase response and has been also involved in the pathogenesis of cancer cachexia. The objectives of the present study were: (1) to determine the relationships of IL-6 and other cytokines with neoplasia extension, acute phase response and nutritional status, in lung cancer patients; and (2) to establish the prognostic value of serum cytokine levels. A prospective study in which IL-1, IL-2, IL-6, tumour necrosis factor alpha (TNF-alpha) and interferon gamma (IFN-gamma) have been determined in 66 newly diagnosed lung cancer patients. Nutritional status was assessed objectively. Serum levels of growth hormone (GH), insulin growth factor 1 (IGF-1) and acute phase reactants as C Reactive Protein, alpha1 antitrypsin and ferritine, were determined. Increased IL-6 levels were related to extensive disease, impaired performance status, enhanced acute phase response and malnutrition. Raised serum IL-6 levels, extensive disease, low Karnofsky index, malnutrition, acute phase response and low IFN-gamma were all related to a shorter survival. When assessed by a multivariate analysis, IL-6 kept its independent prognostic value together with age, disease extension, and decreased IFN-gamma serum levels.IL-6 is increased in lung cancer patients, enhances the acute phase response in them, and is correlated with poor nutritional status, impaired performance status and shorter survival.

[Nephrotic syndrome. What is new since the 1988 study?]. / Síndrome nefrótico. O que há de novo desde o levantamento de 1988?

The authors make a retrospective review of 53 new cases of Nephrotic Syndrome followed up in the Nephrology Unit from November 1988 to March 1994, bearing in mind the evaluation of casual changes of the disease standard regarding a previous study of 1988. Epidemiological, clinical, therapeutical and evolutional aspects were studied. Forty-four cases of primary Nephrotic Syndrome (83%) were identified, 61.4% of which behaved as cortico-sensitive, 25% as cortico-dependent, and 13.6% as cortico-resistant; 8 cases (15%) of Nephrotic Syndrome secondary to infection, Systemic Lupus Erythematosus and Amyloidosis, and 1 case of congenital Nephrotic Syndrome (2%). The theory that the high number of cortico-dependent is, probably, related with a higher severity in the relapse diagnosis and/or changes in the children's standard of living is admissible. It was also observed that at present there is a lower number of hospital discharges, related to more careful attitudes adopted regarding the evolution of the disease.

Acute colitis caused by caustic products.

We report two cases of acute proctocolitis caused by rectal application of caustic products of domestic use. One 61-yr-old woman applied an ammonia solution enema; the other patient, a 63-yr-old woman, accidentally applied an enema containing lye. Both patients presented with intense anal pain, but the first patient also had abdominal pain with guarding, hematochezia, and leucocytosis. An acute proctocolitis was found at sigmoidoscopy in both patients. Only conservative and symptomatic measures were prescribed in both cases, and a clinical and endoscopic recovery was seen. In spite of persistent fibrosis in the lamina propria, no signs of stenosis were found.

Charcoal deposits in the esophageal and gastric mucosa.

We report the case of a 75-yr-old woman referred for gastrointestinal endoscopy to investigate severe iron deficiency anemia. Black linear lesions were observed in the distal esophagus and stomach. Biopsies revealed aggregates of coarse black foreign material, which was later identified as charcoal. The patient's previous medical history included an antidepressant overdose 5 yr before the current admission. The patient had a gastric lavage, using a large bore orogastric tube, followed by the administration of activated charcoal. The patient had no other history of charcoal ingestion. We propose that the charcoal became entrapped in mucosal tears caused by the traumatic intubation 5 yr previously, causing the incidental mucosal tattooing seen at endoscopy.

[Is there a relationship between vaccination coverage and pediatric health care?]. / Están la cobertura vacunal y los exámenes de supervisión pediátrica de la salud íntimamente relacionados?

OBJECTIVE: The aims of this study were to evaluate the relationship between pediatric health care visits and immunization coverage. PATIENTS AND METHODS: The study was made in a rural health care center. All of the children between 3 months and 14 years old were included. The data were obtained directly from their clinical histories. The quality of the health care visits was evaluated according to the fulfillment of A.E.P. patterns of health care. RESULTS: We observed that 87% of infants, 74% of preschool children and 74% of school children were correctly vaccinated. We observed a significantly lower (p < 0.05) coverage of the MMR vaccine in respect to the first three doses of DPT and OPV vaccines; and the coverage of OPV and DT at 6 years old was even lower. The quality of health care visits was good in 67% of infants, 10% of preschool children and 12% of school children. There was a relationship between incomplete vaccinations and missed visits (p < 0.001) and also with low quality health care visits (p < 0.001). CONCLUSIONS: We conclude that there is a significant relationship between missed visits and low quality health care visits with delayed immunization.