Home ventilation of pediatric patients - description of a program

OBJECTIVE: To describe a German program for home ventilatory support, and to analyze the possibility of applying it in Brazil. MATERIALS AND METHODS: We assessed Dr. von Haunersches Kinderklinikacute;s Pediatric Intensive Care Unity - Ludwig-Maximilians - Universit?t - München (Munich, Germany) home ventilatory support program between April 1997 and June 1998. RESULTS: Patients aged between 1 and 21 years - 11 boys and 15 girls - participated in the study. Fifteen out of 26 children presented neuromuscular pathologies, 8 of them had ventilatory problems of central causes, and 3 children presented obstructive pulmonary diseases. Twelve (46.2%) were receiving noninvasive ventilatory assistance, and 19 (73.1%) only needed intermittent ventilatory support. CONCLUSION: The program relies on a permanent multidisciplinary staff to treat intercurrent diseases. Patients, at predetermined periods, are re-evaluated as to the evolution of respiratory insufficiency. The adequate system organization provides patients and their families with security, and accounts for the success of the home ventilatory support program. A great deal of organizational efforts should be consolidated before implementing similar programs in Brazil.     

Effect of 24-hour intravenous tubing set change on the sterility of repackaged fat emulsion in neonates

BACKGROUND: Duration of intravenous fat emulsion (IVFE) infusions, precise method of administration (manufactured bottle vs repackaged syringe), and interval for administration set change continue to be debated. OBJECTIVE: To determine the contamination rate associated with replacing IVFE administration sets every 24 hours in newborn infants receiving fat emulsion repackaged into unit-of-use syringes. METHODS: This was a prospective, microbiologic study of 90 administration sets used in 19 neonates. IVFE samples were obtained from administration sets at the end of a 19- to 23-hour infusion and prior to daily tubing set change from infants who received repackaged IVFE. Samples of IVFE (1-3 mL) were aseptically removed at the catheter connection site proximal to the patient, transferred into BACTEC PEDSPlus culture media, and continuously monitored for 5 days to detect gram-positive and gram-negative organisms, as well as yeast. RESULTS: Two samples (2.27%) grew coagulase-negative Staphylococcus. Both samples were from the same asymptomatic patient and were obtained on consecutive days. A blood sample obtained through this infant's central catheter grew the same organism and suggested catheter hub colonization as the primary site of microbe origin. CONCLUSIONS: Microbial contamination of IVFE infusion sets changed at 24-hour intervals, using unit-of-use syringes in neonates, was low at 2.2%.     

Surgical revision of an uncommonly dislocated self-expanding Amplatzer septal occluder device

Open heart surgery is the standard procedure for closure of ostium secundum atrial septal defects. Recently, percutaneous transcatheter procedures emerged as therapeutic alternatives for closure of both atrial septal defects and patent foramen ovale. Unfortunately, however, such percutaneous procedures may require surgical intervention for early or late complications. We report a case with emergent surgery for dislocation of the Amplatzer septal occluder into the aortic arch diagnosed 30 days after percutaneous closure of an atrial septal defect.     

Stratification of type 1 diabetes risk on the basis of islet autoantibody characteristics

Family history of type 1 diabetes and autoantibodies to the islet antigens insulin (IAA), glutamate decarboxylase (GADA), and the protein tyrosine phosphatase-like protein IA-2 (IA-2A) are strong predictors of type 1 diabetes, but the rate of progression to diabetes in multiple islet autoantibody-positive relatives varies widely. We asked whether detailed characterization of islet autoantibodies that included determination of titer, epitope specificity, and IgG subclass would improve diabetes prediction in a large cohort of autoantibody-positive relatives. The study shows a strong association between risk and high titer, broad antibody responses to IA-2 and insulin. The highest risks were associated with high-titer IA-2A and IAA, IgG2, IgG3, and/or IgG4 subclass of IA-2A and IAA, and antibodies to the IA-2-related molecule IA-2beta. Using models based on these antibody characteristics, autoantibody-positive relatives can be classified into groups with risks of diabetes ranging from 7 to 89% within 5 years.     

Preoperative oral administration of fast-release morphine sulfate reduces postoperative piritramide consumption

The aim of this prospective randomized placebo-controlled double-blind study was to investigate the effect of premedication with morphine sulfate on postoperative pain. Ninety-eight ASA I-III patients undergoing total replacement of the knee or hip joint were randomly assigned to one of two groups. Group 1 received 20 mg morphine sulfate p.o. approximately one hour before the start of surgery; group 2 received placebo. After surgery, piritramide was administered via patient-controlled analgesia over 24 hours. Piritramide consumption and pain scores (visual analog scale) were recorded. The duration of surgery (mean +/- SD) was comparable in the two groups (group 1: 145 +/- 42 min, group 2: 131 +/- 35 min). In group 1 the cumulative piritramide consumption during 24 hours postoperation was significantly less than in the placebo group (37.5 +/- 12.5 mg versus 46.8 +/- 22.1, t-test, p < 0.05), although similar pain scores were recorded (group 1: 4.8 +/- 1.8 and 3.6 +/- 1.7, group 2: 4.8 +/- 1.6 and 3.4 +/- 2.0, at 1 and 24 hours, respectively). These data show that the preoperative oral administration of morphine sulfate, regardless of its short half-life, can reduce postoperative consumption of opioids at similar pain levels.     

Utility of tonsillectomy in 2 patients with the syndrome of periodic fever,aphthous stomatitis,pharyngitis, and cervical adenitis

OBJECTIVES: To review the various causes of period fever in childhood, including the syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA), and to examine the value of tonsillectomy in the treatment of PFAPA syndrome. DESIGN: Retrospective case series. SETTING: Urban and tertiary care referral children's hospital. PATIENTS: Two patients who underwent tonsillectomy for presumed recurrent adenotonsillitis were later diagnosed as having PFAPA syndrome.Intervention Tonsillectomy. MAIN OUTCOME MEASURE: Frequency of PFAPA symptoms before and after tonsillectomy. RESULTS: No difference was found in the frequency of PFAPA symptoms after tonsillectomy. CONCLUSION: Although a larger series of patients is required, our initial experience suggests that tonsillectomy is not always beneficial for patients with PFAPA syndrome.     

Rosai Dorfman disease presenting as synchronous nasal and intracranial masses

A 56-year-old woman with vertex headaches, new-onset seizure disorder, nasal obstruction, and hyposmia was found to have a dural-based intracranial mass as well as bilateral masses arising from the inferior turbinates and extending into the nasopharynx. The patient underwent endoscopic resection of the involved portions of the inferior turbinates, including the sizable nasopharyngeal component. Craniotomy was performed at a later date. Both pathologic specimens were consistent with extranodal Rosai-Dorfman disease. The patient showed resolution of all symptoms after surgery. Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy, is a rare entity usually presenting as cervical nodal disease. When extranodal disease of the head and neck is present, prompt recognition and conservative surgical management is the treatment of choice for this benign pseudolymphomatous entity.     

Effect of conservative management on the prevalence of surgery in patients with adolescent idiopathic scoliosis

STUDY DESIGN: Retrospective analysis of outcome in terms of prevalence of surgery for adolescent idiopathic scoliosis in patients receiving conservative management. OBJECTIVES: To determine whether a centre with an active policy of conservative management has fewer patients who eventually undergo surgery for adolescent idiopathic scoliosis than a centre where the practice is non-intervention. BACKGROUND DATA: The efficacy of orthoses for the treatment of idiopathic scoliosis was called into question in a recent publication. Because the prevalence of surgery in an untreated group of patients (28.1%) was not significantly different from that in a braced group (22.4%), the authors concluded that bracing appears to make no difference. Based on prior experience, this conclusion is questioned. METHODS: Since 1991, bracing and physical therapy have been recommended for children with adolescent idiopathic scoliosis at a centre in Barcelona, Spain. The scoliosis database was searched for patients with adolescent idiopathic scoliosis who were at least 15 years of age at last review and who had adequate documentation of the Cobb angle. The prevalence of surgery was compared with that of published data from a centre where the practice is non-intervention. RESULTS: From a total of 106 braced cases out of which 97 were followed up, six cases (5.6%) ultimately underwent spinal fusion. A worst case analysis, which assumes that all nine cases that were lost to follow-up had operations, brings the uppermost number of cases that could have undergone spinal fusion to 15 (14.1%). Either percentage is significant statistically when compared to the 28.1% reported surgeries from the centre with the policy of non-intervention. CONCLUSIONS: If conservative management does reduce the proportion of children with adolescent idiopathic scoliosis that require surgery, it can be said to provide a real and meaningful advantage to both the patients and the community. It is contended that conservative methods of treatment should never be ruled out from scoliosis management, because they can and do offer a viable alternative to those patients who cannot or will not opt for surgical treatment.