A Child as a Donor for Hematopoietic Stem Cell Transplantation: Bioethical Justification-A Case Study on Sickle Cell Disease.

Hematopoietic stem cell transplantation (HSCT) is an important treatment option for children with severe and refractory sickle cell disease (SCD) with debilitating clinical complications. HSCT with cells from the bone marrow of a HLA-identical sibling used in SCD has a low mortality risk, high cure rate, and high event-free survival rate after a median follow-up of 5-6 years. However, matched donors are found in only about 20% of the patients. A boy aged 8 years with SCD had a sister, <2 years old, a fully compatible donor. The boy met all eligibility criteria to undergo HSCT, and he was suffering from cognitive and neurologic impairment due to ischemic events. A Bioethical Committee jointly discussed the ethical issues on this case after a pediatric evaluation released the very young sister for donation. The justification was that the sister would benefit from the donation too because of the greater likelihood of survival and cure and less suffering of her brother. The parents were informed about the risks and benefits for both children, and the family was psychologically evaluated. After their consent, HSCT was performed and the patient is cured from SCD. The complication for the donor was the need for blood transfusion.

Evaluation of airway obstruction by nasopharyngoscopy: comparison of the Müller maneuver versus induced sleep.

UNLABELLED: The use of nasopharyngoscopy during the application of intrathoracic pressure (Müller maneuver) is frequently employed to establish the site of upper airway obstruction. The Müller maneuver, however, is used when the patient is awake and therefore may not correlate with obstruction occurring during sleep. AIM: To compare the degree of pharyngeal obstruction in the retropalatal and retroglossal regions during the Müller maneuver versus induced sleep using nasopharyngoscopy. STUDY DESIGN: A prospective, case series study. MATERIAL AND METHODS: Eight patients (three males, five females), with a mean age of 48.6 +/- 9.2 year, underwent nasopharyngoscopy to assess airway anatomy and function during the Müller maneuver while awake and during sleep induced by drip infusion of Midazolam. RESULTS: Retropalatal obstruction was similar during the Müller maneuver and sleep (mean + standard deviation = 3.13 +/- 0.99 and 2.75 +/- 0.46, p= 0.234). Retroglossal obstruction was significantly lower during Müller maneuver compared to sleep (mean + standard deviation 0.63 +/- 1.06 and 2.63 +/- 1.30, respectively, p= 0.005). CONCLUSIONS: The sleep inducing method was safe under the monitored conditions of this study, and detected more retroglossal obstruction than the Müller maneuver.

Functional implications of BAL in the presence of restrictive or obstructive lung disease.

Most of the complications associated to bronchoscopy are related to changes of the respiratory function during or after its performance. Prevention of complications should be achieved by understanding the effects of bronchoscopic procedures and their relation to the pulmonary function deterioration. Previous studies regarding the functional impairment caused by bronchoalveolar lavage (BAL) were mostly limited by the presence of interferent factors such as sedative drugs. Furthermore, it is not clear whether or not patients with different ventilatory disturbances present the same functional response to bronchoscopy and BAL. The aim of this study was to determine the additional effects of BAL over the respiratory function deterioration related to bronchoscopy in patients with different respiratory function profiles (normal, restrictive and obstructive). Forty patients submitted to bronchoscopy without premedication were divided into four groups: group I-normal pulmonary function submitted to basic bronchoscopy; group II-bronchoscopy in combination with BAL, subdivided according to pulmonary function: group IIa (normal function), group IIb (restrictive ventilatory disturbances) and group IIc (obstructive ventilatory disturbances). Spirometry was made before and after the bronchoscopic procedure. Baseline hemoglobin saturation was compared to the lowest level during the procedure. Functional worsening caused by the procedure was observed with a decrease in forced vital capacity (FVC), forced expiratory volume in the first second (FEV(1)) and Hemoglobin saturation in all groups. Comparison between groups showed no significant difference regarding the changes in FVC (P=0.8324), FEV(1) (P=0.6952) and hemoglobin saturation (P=0.5044). We conclude that standardized BAL, like the one used in our study, does not result in an increased risk for ventilatory impairment compared to bronchoscopy itself, independently of the presence of previous respiratory disease.

High dose-rate brachytherapy as a treatment option in primary tracheal tumors.

PURPOSE: To present experience with high dose-rate endobronchial brachytherapy in the treatment of primary tracheal tumors. PATIENTS AND METHODS: Four patients with nonresected primary tracheal tumors are presented: 2 cases of squamous cell carcinoma of the trachea, 1 of recurrent adenoid cystic carcinoma, and 1 with recurrent plasmacytoma. All received brachytherapy, alone or as a boost for primary irradiation, in 3 or 4 fractions of 7.5 Gy, calculated at a depth of 1 cm. Follow-up was considered to start from the end of brachytherapy. RESULTS: Local control was achieved in all cases at the time of first bronchoscopic evaluation. Two patients with squamous cell carcinoma died at 6th and 33rd months after brachytherapy, respectively. The first had no evidence of disease, and the latter had local recurrence. The other 2 patients were alive after 64 and 110 months of follow-up, respectively, both with no evidence of disease. Tracheal stenosis developed in these 2 cases, 22 and 69 months after brachytherapy. Tracheal stent placement was needed only for the patient with an adenoid cystic carcinoma. CONCLUSIONS: Endobronchial high dose-rate brachytherapy may be used for tracheal tumors, even as a boost for external beam irradiation, or in recurrences. Local control in 3 out of 4 patients indicates that individual cases may benefit from the treatment. Long-term survival may also be expected, mainly for tumors with adenoid cystic histology.