Doctors' awareness concerning primary immunodeficiencies in Brazil.

BACKGROUND: PIDs are a heterogeneous group of genetic illnesses, and delay in their diagnosis is thought to be caused by a lack of awareness among physicians concerning PIDs. The latter is what we aimed to evaluate in Brazil. METHODS: Physicians working at general hospitals all over the country were asked to complete a 14-item questionnaire. One of the questions described 25 clinical situations that could be associated with PIDs and a score was created based on percentages of appropriate answers. RESULTS: A total of 4026 physicians participated in the study: 1628 paediatricians (40.4%), 1436 clinicians (35.7%), and 962 surgeons (23.9%). About 67% of the physicians had learned about PIDs in medical school or residency training, 84.6% evaluated patients who frequently took antibiotics, but only 40.3% of them participated in the immunological evaluation of these patients. Seventy-seven percent of the participating physicians were not familiar with the warning signs for PIDs. The mean score of correct answers for the 25 clinical situations was 48.08% (±16.06). Only 18.3% of the paediatricians, 7.4% of the clinicians, and 5.8% of the surgeons answered at least 2/3 of these situations appropriately. CONCLUSIONS: There is a lack of medical awareness concerning PIDs, even among paediatricians, who have been targeted with PID educational programmes in recent years in Brazil. An increase in awareness with regard to these disorders within the medical community is an important step towards improving recognition and treatment of PIDs.

Severe combined immunodeficiency in Brazil: management, prognosis, and BCG-associated complications.

BACKGROUND: Severe combined immunodeficiency (SCID) is one of the most severe forms of primary immunodeficiency. The objectives of this study were to analyze the diagnosis, treatment, and prognosis of SCID in Brazil and to document the impact of BCG vaccine. METHODS: We actively searched for cases by contacting all Brazilian referral centers. RESULTS: We contacted 23 centers and 70 patients from 65 families. Patients were born between 1996 and 2011, and 49 (70%) were male. More than half (39) of the diagnoses were made after 2006. Mean age at diagnosis declined from 9.7 to 6.1 months (P = .058) before and after 2000, respectively, and mean delay in diagnosis decreased from 7.9 to 4.2 months (P = .009). Most patients (60/70) were vaccinated with BCG before the diagnosis, 39 of 60 (65%) had complications related to BCG vaccine, and the complication was disseminated in 29 of 39 (74.3%). Less than half of the patients (30, 42.9%) underwent hematopoietic stem cell transplantation (HSCT). Half of the patients died (35, 50%), and 23 of these patients had not undergone HSCT. Disseminated BCG was the cause of death, either alone or in association with other causes, in 9 of 31 cases (29%, no data for 4 cases). CONCLUSIONS: In Brazil, diagnosis of SCID has improved over the last decade, both in terms of the number of cases and age at diagnosis, although a much higher number of cases had been expected. Mortality is higher than in developed countries. Complications of BCG vaccine are an important warning sign for the presence of SCID and account for significant morbidity during disease progression.

Methotrexate in the treatment of corticodependent asthmatic children.

Methotrexate has been used as an anti-inflammatory agent in chronic asthma. We evaluated the action of methotrexate in eight corticodependent severely asthmatic children (more than 10 mg of prednisone per day for at least one year). The patients (3 males and 5 females; aged 8 to 14 years) received a single weekly dose of 0.6 mg/kg methotrexate (maximum 25 mg) and folic acid (15 mg/day for 5 days in the week). The children were examined and had their pulmonary function test evaluated weekly. As the study progressed, the dose of prednisone was reduced and maintained till the next evaluation if the patient's symptoms were under control. After the 3rd month of treatment, we observed a significant reduction in the dose of prednisone and maintenance of the spirometric parameters. At the end of the trial, in 4 patients it was possible to reduce the basal prednisone dose 56% or more. In the remaining 4, one did not show any benefit and in the other 3, it was possible to obtain an average reduction of 40% of the basal prednisone dose. The total mean reduction was 55.9%. This oral corticoid reduction was not associated with clinical or pulmonary function deterioration, except in one patient. The patients were submitted to white blood cell count, hepatic transaminases, urine tests and other determinations at least once a month. There were no changes in biochemical tests. The side-effects were nausea, vomiting and abdominal pain. In conclusion, methotrexate given to severely corticodependent asthmatic children permitted a reduction in the daily intake of prednisone, reducing the severe side-effects of chronic corticotherapy.

Efficacy and tolerability of fitostimoline (vaginal cream, ovules, and vaginal washing) and of benzydamine hydrochloride (tantum rosa vaginal cream and vaginal washing) in the topical treatment of symptoms of bacterial vaginosis.

Two hundred and 91 patients showing signs and symptoms of bacterial vaginosis (BV) were randomized to receive topical treatment with Fitostimoline (vaginal cream and vaginal ovules + vaginal washing) or benzydamine hydrochloride (vaginal cream + vaginal washing) for 7 days. Signs (leucorrhoea, erythema, oedema, and erosion) and symptoms (burning, pain, itching, vaginal dryness, dyspareunia, and dysuria) (scored 0-3) were evaluated at baseline and at the end of treatment; the total symptoms score (TSS) was also calculated. In 125 patients, a bacterial vaginosis was confirmed by vaginal swab test. The primary efficacy variable analysis, that is, the percentage of patients with therapeutic success (almost complete disappearance of signs and symptoms), demonstrated that Fitostimoline ovules and vaginal cream were therapeutically equivalent and that pooled Fitostimoline treatment was not inferior to benzydamine hydrochloride. All the treatments were well tolerated, with only minor local adverse events infrequently reported. The results of this study confirmed that gynaecological Fitostimoline is a safe and effective topical treatment for BV.

Severity scoring of atopic dermatitis: a comparison of two scoring systems.

BACKGROUND: Atopic dermatitis (AD) is a chronic inflammatory pruritic skin heterogeneous disease, which does not have an objective tool able to permit comparison between patients and to monitor changes due to a specific treatment. Several assessment methods have been developed to evaluate the AD. OBJECTIVES: The aim of this study was to investigate the agreement between two observers in the assessment of the severity of AD applying SCORAD index and EASI in infants and young children with AD. The variations of parameters that compose each score, as well as the inter-observer variation for both scores, were also studied. METHODS: We evaluated 42 infants and young children admitted and followed in a paediatric allergy centre (UNIFESP-EPM). All children met the diagnostic criteria for clinical AD established by Hanifin and Rajka. Two investigators graded the severity of AD, applying the SCORAD index and EASI. The two scoring systems assessed the variation between baseline evaluation and one month after treatment. RESULTS: Significant correlations were observed comparing both scores for each physician and each evaluation. There were no differences between the mean SCORAD and EASI scores for each physician, in the two evaluations. There was a significant decrease in the mean total SCORAD and EASI score and its components, except for the item Upper Extremities EASI CONCLUSION: Both scoring systems for AD are valid, reproducible and responsive in monitoring AD patients. Further studies will be necessary to investigate the development of AD and the best evaluation. SCORAD showed itself to be an excellent score to detect the development of AD, whereas the EASI are suitable to follow up in drug-effect studies of AD for research purposes.

[Purification of waste water from olive-oil pressing plants using hydrophytes. Preliminary study on the use of Eichornia crassipes]. / Depurazione delle acque reflue di frantoio mediante idrofite. Indagini preliminari sull'impiego dell' Eichornia crassipes.

The use of Eichornia crassipes has been tested in order to depurate the waste water from olive oil factories. The control of some parameter variations during the treatment, such as COD, total azote, dissolved oxygen and pH, has given encouraging results about the depurative efficacy of employed hydrophytae. Particularly indicative is, in this regard, COD rejection of about 60%.

[Identification of the root of Punica granatum in galenic preparations using TLC]. / Riconoscimento della radice di "Punica granatum" nelle preparazioni galeniche mediante TLC.

The alkaloids present in the root of "Punica granatum" have been extracted by two different methods: extraction by Soxlet and extraction by steam distillation. Then the extracts have been compared by TLC chromatography using different solvents and specific chromogen reagents. The presence of pseudo-pelletierine has been confirmed in both the extractive solution by reaction with conc. K2Cr2O7. The above results explains the toxic activity of the unsuitable galenic preparations.