Stevens-Johnson syndrome and toxic epidermal necrolysis in childhood-onset systemic lupus erythematosus patients: a multicenter study.

Objective: To assess Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in a large population of childhood-onset systemic lupus erythematosus (cSLE) patients.

Methods: Multicenter study including 852 cSLE patients followed in Pediatric Rheumatology centers in São Paulo, Brazil. SJS was defined as epidermal detachment below 10% of body surface area (BSA), overlap SJS-TEN 10-30% and TEN greater than 30% of BSA.

Outcomes of 847 childhood-onset systemic lupus erythematosus patients in three age groups.

Objective The objective of this study was to assess outcomes of childhood systemic lupus erythematosus (cSLE) in three different age groups evaluated at last visit: group A early-onset disease (<6 years), group B school age (≥6 and <12 years) and group C adolescent (≥12 and <18 years). Methods An observational cohort study was performed in ten pediatric rheumatology centers, including 847 cSLE patients. Results Group A had 39 (4%), B 395 (47%) and C 413 (49%).

Phenotypes of asthma in low-income children and adolescents: cluster analysis.

Objective: Studies characterizing asthma phenotypes have predominantly included adults or have involved children and adolescents in developed countries. Therefore, their applicability in other populations, such as those of developing countries, remains indeterminate. Our objective was to determine how low-income children and adolescents with asthma in Brazil are distributed across a cluster analysis.

Anti-RO/SSA and anti-La/SSB antibodies: Association with mild lupus manifestations in 645 childhood-onset systemic lupus erythematosus.

Background: To our knowledge there are no studies assessing anti-Ro/SSA and anti-La/SSB autoantibodies in a large population of childhood-systemic lupus erythematosus (cSLE) patients.

Methods: This was a retrospective multicenter cohort study performed in 10 Pediatric Rheumatology services, São Paulo state, Brazil. Anti-Ro/SSA and anti-La/SSB antibodies were measured by enzyme linked immunosorbent assay (ELISA) in 645 cSLE patients.

Features of 847 Childhood-Onset Systemic Lupus Erythematosus Patients in Three Age Groups at Diagnosis: A Brazilian Multicenter Study.

Objective: To evaluate demographic data and clinical and laboratory features at disease diagnosis in 3 different age groups of childhood-onset systemic lupus erythematosus (SLE): group A, early-onset (<6 years); group B, school age (≥6 to <12 years); and group C, adolescent (≥12 to <18 years).

Methods: This was a Brazilian multicenter cohort retrospective study in 10 pediatric rheumatology centers, including 847 childhood-onset SLE patients.

Results: Patients were divided into 3 groups: group A with 39 patients (4%), group B with 395 patients (47%), and group C with 413 patients (49%).

Nursing process documentation: rationale and methods of analytical study.

Objective: to describe the methods used to analyze the associations between variables of service, nursing and the nursing process documentation in institutions of the Department of Health of the State of São Paulo.

Method: multilevel analytical study with data obtained in the domains of institution, units of the institution and nursing professionals who work there, using standardized instruments. The analyses had as axis the degree of completeness of the nursing process documentation in units or institutions and their association with variables of nursing personnel, of units and of institutions.

Herpes zoster infection in childhood-onset systemic lupus erythematosus patients: a large multicenter study.

Objective: The aim of this multicenter study in a large childhood-onset systemic lupus erythematosus (cSLE) population was to assess the herpes zoster infection (HZI) prevalence, demographic data, clinical manifestations, laboratory findings, treatment, and outcome.

Methods: A retrospective multicenter cohort study (Brazilian cSLE group) was performed in ten Pediatric Rheumatology services in São Paulo State, Brazil, and included 852 cSLE patients. HZI was defined according to the presence of acute vesicular-bullous lesions on erythematous/edematous base, in a dermatomal distribution.

Lowered Cisplatin Dose and No Bleomycin in the Treatment of Pediatric Germ Cell Tumors: Results of the GCT-99 Protocol From the Brazilian Germ Cell Pediatric Oncology Cooperative Group.

Purpose: We describe the results of a risk-adapted, response-based therapeutic approach from the Brazilian GCT-99 study on germ cell tumors.

Patients And Methods: From May 1999 to October 2009, 579 participants were enrolled in the Brazilian GCT-99 study. Treatment, defined as specific chemotherapy regimen and number of cycles, was allocated by means of risk-group assignment at diagnosis with consideration for stage and primary tumor site.

A Multicenter Study of Invasive Fungal Infections in Patients with Childhood-onset Systemic Lupus Erythematosus.

Objective: To study the prevalence, risk factors, and mortality of invasive fungal infections (IFI) in patients with childhood-onset systemic lupus erythematosus (cSLE).

Methods: A retrospective multicenter cohort study was performed in 852 patients with cSLE from 10 pediatric rheumatology services. An investigator meeting was held and all participants received database training.

Pancreatitis Subtypes Survey in 852 Childhood-Onset Systemic Lupus Erythematosus Patients.

Objective: Pancreatitis is a rare and a life-threatening systemic lupus erythematosus (SLE) manifestation in childhood-onset SLE (cSLE). The objective of this study was to systematically classify pancreatitis in cSLE according to the International Study Group of Pediatric Pancreatitis and determine the overall prevalence, clinical features, laboratory, and first episode outcomes.

Methods: A multicenter cohort study in 10 pediatric rheumatology centers, including 852 patients with cSLE.

Rituximab use in young adults diagnosed with juvenile idiopathic arthritis unresponsive to conventional treatment: report of 6 cases.

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood. Without an effective therapy, patients may progress quickly to functional disability. Recently, depletion of B cells emerged as a new approach for the treatment of autoimmune diseases, including JIA.

Urethral duplication--a wide spectrum of anomalies.

Objective: Urethral duplications are rare lower urinary tract anomalies, with multiple anatomical variants described. This paper aims to separate this complex anomaly into different diseases, each with distinct clinical forms according to the disturbance during embryogenesis, yet noting a few similarities that may be helpful in their management. The classification system of urethral duplication is also discussed.

Acute respiratory failure during pediatric anesthesia: atelectasis and hypertensive pneumothorax: case report.

Background And Objectives: The main anesthesiologist's task is to ensure appropriate oxygenation of patient. The objective of this report is to describe both diagnosis and behavior in case of acute respiratory failure during anesthesia, with educational purposes.

Case Report: Three-year-old child underwent anesthesia for urologic surgery presented respiratory failure by bronchial obstruction by secretion, evolving to atelectasis and hypertensive pneumothorax.

Pelvic fracture urethral distraction defects in children managed by anterior sagittal trans anorectal approach: a facilitating and safe access.

Purpose: Trauma injuries of the posterior urethra resulting from pelvic fracture in children tend to be complete ruptures, with upper dislocation of the prostate. This paper aims to show our experience in using an anterior sagittal transanorectal approach (ASTRA) in the treatment of such injuries.

Materials And Methods: The medical records of 11 patients with pelvic fracture urethral distraction defects who had undergone anastomotic urethroplasty through ASTRA between 1997 and 2009 were reviewed.

Lowe syndrome: report of five cases.

Introduction: Lowe Syndrome, or Oculocerebrorenal Dystrophy (OCRL), has a recessive inheritance linked to X chromosome. It presents cataracts and glaucoma, delay in neuropsychomotor development, cognitive deficits, and renal Fanconi syndrome.

Objective: Describe five patients with OCRL, attended at Tubulopathy outpatient clinic.

Extreme subcutaneous, intramuscular and inhaled insulin resistance treated by pancreas transplantation alone.

Diabetes mellitus with resistance to insulin administered subcutaneously or intramuscularly (DRIASM) is a rare syndrome and is usually treated with continuous intravenous insulin infusion. We present here two cases of DRIASM in 16 and 18 years female patients that were submitted to pancreas transplantation alone (PTA). Both were diagnosed with type 1 diabetes as young children and had labile glycemic control with recurrent episodes of diabetic ketoacidosis.

Ectopic opening of the vas deferens into a Müllerian duct cyst.

Two consecutive cases of ectopic opening of the vas deferens into Müllerian duct cysts are presented, with descriptions of the presentation, physical examination, radiographic and ultrasonographic images, surgical findings and treatment undertaken. In both cases, ablation of the cyst and ligature of the vas deferens was the technique employed. Both patients are currently well, with normal urinary flow and no urinary tract infections, 3 years after surgery.

Sacral ratio and fecal continence in children with anorectal malformations.

Objective: To evaluate the sacral ratio (SR) in patients with an anorectal malformation (ARM) and verify whether it has predictive value for fecal continence.

Patient And Methods: From January 1990 to April 2002, 42 patients (aged 3-14 years) with an ARM and having already been operated on were reassessed and enrolled in the study. Patients with solid or paste-like stools but no soiling were deemed continent, those with similar stool and episodes of soiling partially continent, and those with no sphincter control, incontinent.

The exstrophy-epispadias complex: is aesthetic appearance important?

Objective: To show the relevance of cosmetic appearance in the adequate treatment of patients with exstrophy-epispadias complex (EEC), and to indicate that surgery by experienced teams can improve the long-term treatment forecast and the patient's body image.

Patients And Methods: From 1978 to 2002, 71 patients diagnosed with EEC were treated in the authors' institution; 24 (aged 2-23 years) were selected to undergo different surgical procedures. The criterion for surgery considered interviews conducted by the psychology team with the parents and children.

[Clinical assessment of sexual maturation in adolescents].

OBJECTIVE: To present the methods for clinical evaluation of sexual maturation in adolescents. METHODS: Bibliographic review concerning the practice of pubertal staging. RESULTS: The assessment of sexual maturation is an essential step in the comprehensive health care of adolescents, allowing for the evaluation of their developmental stage.

Posterior urethral injuries and the Mitrofanoff principle in children.

Objective: To report our experience of children with trauma causing posterior urethral injury who at some stage underwent a Mitrofanoff intervention, as post-traumatic urethral injuries can demand long-term treatment which (regardless of the surgical intervention) requires a period of dilatation of the reconstructed urethra.

Patients And Methods: From 1992 to 2001, 14 patients with urethral injuries underwent a Mitrofanoff procedure. Thirteen had been run over by a motor vehicle and had severe hip injuries, and one had a direct non-penetrating perineal impact lesion (13 boys and one girl, aged 2-13 years at the time of the accident).

A posterior-based omega-shaped flap vaginoplasty in girls with congenital adrenal hyperplasia caused by 21-hydroxylase deficiency.

Objective: To describe a technical modification for constructing a vagina in girls with congenital adrenal hyperplasia caused by 21-hydroxylase deficiency, using a narrower skin base but rich subcutaneous tissue, aiming to obtain both longer and larger vaginal segments with better cosmesis of the external genitalia.

Patients And Methods: From August 1997 to February 2001, 28 girls (aged 5 months to 17 years) had a neovagina constructed using a posterior-based omega-shaped flap. Twenty-six patients had a low vagina entering into the urogenital sinus and two had a high vagina that entered the urogenital sinus.

Sigmoid reconfigured vaginal construction in children.

Purpose: We present a modified technique of sigmoid neovaginal construction in children that protects the sigmoid pedicle from traction, allows easy adjustment of caliber and reorients the mucosal fold in a longitudinal direction.

Materials And Methods: From 1997 to 2000, 10 genetically male (46 XY) children 1 to 13 years old underwent construction of a neovagina with sigmoid, incorporating the Yang-Monti concept of intestinal reconfiguration. The diagnosis was androgen insensitivity in 7 patients, congenital adrenal hyperplasia in 2 due to 17 alpha-hydroxylase deficiency and 3 beta-hydroxysteroid dehydrogenase deficiency, respectively, and bladder exstrophy in 1 who required sex reassignment.

[Active immunization in adolescence].

Objective: The purpose of this review is to offer updating on recent changes in the schedule of active immunization routine in adolescence.

Methods: This review covers specialized medical literature between 1985 and 1996, and follows the recommendations of American Academy of Pediatrics and Advisory Committee on Immunization Practices.

Results: Active immunization in adolescents has been changed in the few last years.

Use of videoscopy as an aid in the placement of tissue expanders.

In this preliminary note the authors describe a new technique consisting in the use of videoscopy as an aid in surgery for the placement of tissue expanders in a patient with sequelae due to burn scarring on the posterior thorax. The surgical steps are described, together with the technical details of the use of the videolaparoscopy equipment. The authors discuss the advantages of this new technique which permits the use of small routes of approach, perpendicular to the expansion area, thus, providing an excellent view with a minimal amount of trauma.