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.

HIGH BODY MASS INDEX AMONG PATIENTS UNDERGOING HEMATOPOIETIC STEM CELL TRANSPLANTATION: RESULTS OF A CROSS-SECTIONAL EVALUATION OF NUTRITIONAL STATUS IN A PRIVATE HOSPITAL.

BACKGROUND: nutritional status before hematopoietic stem cell transplantation (HSCT) affects prognosis: better nourished patients have shorter time to engraftment, while malnutrition is associated with increase of mortality rates, complications, medical costs, poor quality of life and hospitalization stay. Furthermore, underweight patients have increased risk of death in the early post- HSCT period, and non-relapse mortality is greater for those who are extremely underweight, overweight and obese. Obesity is associated with treatment-related toxicity, higher incidence of grade II-IV acute graft-versus- host disease (GVHD), infections and mortality. The objective of this study was to investigate the nutritional status of patients undergoing HSCT between 2007-2013 in a private hospital, by calculating the body mass index (BMI), to verify the prevalence of any nutritional imbalances, especially obesity. METHODS: in this retrospective study, based on medical records, we analyzed data from all patients with malignant and nonmalignant diseases who underwent HSCT from January 2007 to February 2014 in the Hematology- Oncology and Bone Marrow Transplantation Center at a large, tertiary referral center in Brazil. RESULTS: a total of 257 cases were treated in the period and analyzed, of which 79% were aged up to 65 years old. Among these, 56% were overweight or obese. We observed a higher prevalence of obesity in elderly patients (P < 0.001). The mean BMI of the total sample was 26.4 kg/m2. BMI was significantly different between genders, with higher prevalence of overweight among men (P < 0.001). CONCLUSION: differently from other studies, our investigation has shown low rates of underweight and more overweight and obesity rates in men and elderly patients undergoing HSCT.

Introducción: el estado nutricional previo al trasplante de células madre hematopoyéticas (HSCT) afecta al pronóstico: los pacientes con una mejor nutrición necesitan un menor tiempo de injerto, mientras que una nutrición pobre estaría asociada a un aumento de la tasa de mortalidad, complicaciones, costes médicos, mala calidad de vida y hospitalización. Además, los pacientes con bajo peso presentan un mayor riesgo de muerte al principio del periodo post-HSCT, y la tasa de mortalidad sin recaída es mayor en aquellos con bajo peso extremo, sobrepeso y obesidad. La obesidad está relacionada con una toxicidad asociada al tratamiento, una incidencia más alta de enfermedad de injerto-huésped de grado II-IV (GVHD), infecciones y mortalidad. El objetivo de este estudio fue investigar el estado nutricional de pacientes que se sometieron a HSCT entre 2007 y 2013 en un hospital privado, calculando el índice de masa corporal (IMC), para comprobar la prevalencia de posibles desajustes nutricionales, especialmente obesidad. Métodos: en este estudio retrospectivo, basado en informes médicos, analizamos datos de todos los pacientes con enfermedades tumorales y no tumorales que se sometieron a HSCT desde enero de 2007 hasta febrero de 2014 en el Centro de hematología-oncología y trasplante de médula ósea en un gran centro de especialidades médicas en Brasil. Resultados: en el periodo se trató y analizó un total de 257 casos, de los cuales el 79% tenían edades de hasta 65 años. Entre estos, el 56% tenían sobrepeso u obesidad. Observamos una mayor prevalencia de obesidad en pacientes ancianos (P < 0,001). El IMC medio de la muestra total fue 26,4 kg/m2. El IMC fue significativamente diferente entre géneros, con una prevalencia mayor de sobrepeso entre los hombres (P < 0,001). Conclusión: a diferencia de otros estudios, nuestra investigación ha mostrado bajas tasas de tasas de bajo peso y altas tasas de sobrepeso y de obesidad en los hombres y pacientes ancianos sometidos a HSCT.

Fetal bronchoscopy: its successful use in a case of extralobar pulmonary sequestration.

OBJECTIVE: To report the performance of fetal bronchoscopy in a case of pulmonary sequestration. MATERIALS AND METHODS: A 24 year-old female, Gravida 2, Para 1, was referred at 27.5 weeks with a large fetal left lung mass with marked right mediastinal shift and no visible normal left lung. Differential diagnosis included possible bronchial atresia. RESULTS: The patient underwent fetal laryngoscopy and fetal bronchoscopy at 31.5 weeks. The right lung and a portion of the left lung expanded during surgery as a result of bronchial lavage. Bronchial atresia or bronchogenic cyst were not found. Pregnancy continued uneventfully, with continuous growth of the right lung and a small amount of left lung. The patient delivered vaginally at term. The baby underwent thoracoscopic resection of a pulmonary sequestration at 10.5 months of age and did well. CONCLUSION: Fetal bronchoscopy is feasible. The procedure may prove useful in the differential diagnosis and in the potential treatment of different fetal lung lesions, as well as aid in the understanding of the role of bronchial obstruction as a common pathophysiologic mechanism for different fetal lung masses. Risks and benefits of fetal bronchoscopy warrant further experience.

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.

Irradiated volume and the risk of fatal hemoptysis in patients submitted to high dose-rate endobronchial brachytherapy.

To determine risk factors associated with fatal hemoptysis (FH) in endobronchial high dose-rate brachytherapy (EHDRB) 84 patients treated with EHDRB from January 1991 to June 2002 were studied. Clinical and technical parameters (including treatment volumes) were analyzed. Eight (9.5%) patients died of FH, all but one with recurrent or persistent local disease. Median interval until death due to FH was 4 months versus 6 months for the whole group. The only factor with significant correlation with FH was the 100% isodose volume (V100) (p=0.04). Larger irradiated volumes were related to FH. Analysis of volume parameters is suggested, together with the dose and number of fractions prescribed for each patient.

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.