The introduction of dornase alfa improved the HRQL of the patients with CF during the first year of treatment.
OBJECTIVE: To describe the clinical impact of the first year treatment with dornase alfa, according to age groups, in a cohort of Brazilian Cystic Fibrosis (CF) patients. METHODS: The data on 152 eligible patients, from 16 CF reference centers, that answered the medical questionnaires and performed laboratory tests at baseline (T0), and at six (T2) and 12 (T4) months after dornase alfa initiation, were analyzed. Three age groups were assessed: six to 11, 12 to 13, and >14 years. Pulmonary tests, airway microbiology, emergency room visits, hospitalizations, emergency and routine treatments were evaluated. Student's t-test, chi-square test and analysis of variance were used when appropriated. RESULTS: Routine treatments were based on respiratory physical therapy, regular exercises, pancreatic enzymes, vitamins, bronchodilators, corticosteroids, and antibiotics. In the six months prior the study (T0 phase), hospitalizations for pulmonary exacerbations occurred in 38.0, 10.0 and 61.4% in the three age groups, respectively. After one year of intervention, there was a significant reduction in the number of emergency room visits in the six to 11 years group. There were no significant changes in forced expiratory volume in one second (VEF1), in forced vital capacity (FVC), in oxygen saturation (SpO2), and in Tiffenau index for all age groups. A significant improvement in Shwachman-Kulczychi score was observed in the older group. In the last six months of therapy, chronic or intermittent colonization by P. aeruginosa was detected in 75.0, 71.4 and 62.5% of the studied groups, respectively, while S. aureus colonization was identified in 68.6, 66.6 and 41.9% of the cases. CONCLUSIONS: The treatment with dornase alfa promoted the maintenance of pulmonary function parameters and was associated with a significant reduction of emergency room visits due to pulmonary exacerbations in the six to 11 years age group, with better clinical scores in the >14 age group, one year after the intervention.
Patients who survive a traumatic brain injury (TBI) can sometimes experience symptoms of excessive sympathetic discharge. Despite being known about for more than 60 years, the diagnostic criteria for this condition have only recently been defined under the name "paroxysmal sympathetic hyperactivity". Failure to recognize this syndrome leads to excessive costs, prolonged hospital stays and delayed recovery for TBI patients. This case report describes a patient whose specific rehabilitation program was affected by a failure to identify this entity, even though he presented with many of the characteristics of this condition. LEARNING POINTS• Fever is a common sign in hospitalized patients and can result in expensive investigations and prolong length of stay.• It is crucial to consider paroxysmal sympathetic hyperactivity (PSH) as a cause of fever in traumatic brain injury patients (TBI).• A delay in diagnosing and treating PSH can have devastating consequences for these patients' recovery programs. KEYWORDS Paroxysmal sympathetic hyperactivity (PSH), traumatic brain injury (TBI), fever, beta-blocker CASE PRESENTATION The authors report the case of 27-year-old male who was aphasic and tetraplegic following a traumatic brain injury (TBI) with severe right hemisphere lesions sustained in a road traffic collision. Five months after the accident, the patient was readmitted to hospital because of nosocomial pneumonia and was started on a 10-day course of the antibiotic meropenem. No pathogens were isolated from blood or sputum cultures and the patient improved clinically to be stable on air, with no respiratory symptoms and negative inflammatory markers. Despite the apparently treated infection, the patient was still febrile with his temperature rising to 39.5°C once or twice a day with very little response to antipyretic drugs. During these episodes, the patient became tachycardic, hypertensive, tachypnoeic and sweated profusely. The patient´s mother reported that these episodes were also frequent at home and that because of them, the patient had been refused care in a specialized rehabilitation hospital as he was thought to be infectious. A complete septic screen was performed including blood, sputum (obtained from bronchoscopy), urine and liquor samples but no bacteria, yeast or fungus were found, and no changes were seen on imaging tests. The inflammatory markers were also negative: white cell count 7,000×10 9 /l (normal value <11,000×109/l), C-reactive protein <0.5 mg/dl (normal value <0.5 mg/dl) and erythrocyte sedimentation rate 23 mm/h (normal value <20 mm/h). Both renal and liver function were normal. Paroxysmal sympathetic hyperactivity (PSH) was then considered as the cause of the patient's febrile episodes. PSH is a common but unrecognized complication after TBI and is characterized by hypertension, tachycardia, sweating, tachypnoea and hyperthermia, the last
Syndrome of Inappropriate Antidiuretic Hormone Secretion (SIADH) is a common cause of euvolemic hyponatremia which has many possible etiologies such as tumors (mainly lung cancers). We present a case of a 79-year old women hospitalized due to microcytic anemia with transfusion need and hyponatremia due to SIADH. Three primary tumors without known metastasis were discovered. The case shows the importance of studying causes of hyponatremia. LEARNING POINTS• Hyponatremia is a common electrolytic disorder during hospitalization and is associated with greater mortality.• Correction of hyponatremia, cause can be devastating unless the underlying causes are investigated as it can be caused by severe diseases .• Cancer must be excluded in the absence of an obvious etiology of SIADH (drugs, pulmonary and nervous system disorders).
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