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1 B LETÍN ASOCIACIÓN MÉDICA DE PUERTO RICO PERMIT No SAN JUAN, PR Vol Núm 2 - Abril-Junio 2008 PRESORT PAID U.S. POSTAGE STANDARD

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3 B LETÍN ASOCIACIÓN MÉDICA DE PUERTO RICO CONTENIDO 2 JUNTA DE DIRECTORES / JUNTA EDITORA 3 MENSAJE DEL PRESIDENTE Y PORTADA Por: Eduardo Rodríguez Vázquez, MD 7 From the Editorial Desk... Por Humberto Lugo-Vicente, MD ARTÍCULOS ORIGINALES - ORIGINAL ARTICLES 8 A COMPETENCY-BASED COMMUNICATION SKILLS WORKSHOP SERIES FOR PEDIATRIC RESIDENTS By Débora H. Silva, MD 14 HEMOLYTIC UREMIC SYNDROME IN CHILDREN IN PUERTO RICO: A RARE DISEASE WITH ATYPICAL FEATURES By: Yasmín Pedrogo-Rodríguez, MD, Juan O. Pérez-Rodríguez, MD, Melvin Bonilla-Felix, MD 18 THROMBOCYTOSIS IN ILLICIT DRUGS-EXPOSED NEWBORNS By: Thea Calderón MD, Sonia Medina MD, Inés García MD, Lourdes García MD, Marta Varcárcel MD 21 MANEJO DE LACTANCIA Y AMAMANTAMIENTO: ROL DEL MEDICO RESIDENTE Por: Nerian Ortiz Matos MD, Lourdes García Fragoso MD 24 GENITOANAL FINDINGS IN PUERTO RICAN CHIL- DREN WITH SUSPECTED SEXUAL ABUSE By: Amaris Rivas Carlo MD, Brenda Mirabal MD, MPH 28 LUPUS NEPHRITIS IN PUERTO RICAN CHILDREN AND ADOLESCENTS By: Tami O. Tiamfook MD, Ivonne Arroyo MD, Enid Del Valle MD, Juan O. Pérez-Rodríguez MD, Anarda González MD, and Melvin Bonilla-Félix MD PORTADA 33 PERCEPTION OF PARENTS REGARDING THEIR CHILDREN S WEIGHT By: Ilsa J. Nazario Rodriguez MD, Wanda I. Figueroa MD, Jaime Rosado MD, Iris del C. Parrilla MS 39 DO PARENTS KNOW ABOUT THE ADVERSE EF- FECTS OF PASSIVE SMOKING AND THE RELA- TIONSHIP WITH RESPIRATORY ILLNESS ON THEIR CHILDREN? By:Cristina Jiménez-González MD, Vanessa Santini MD, Wanda I. Figueroa Cosme MD, Iris del C. Parilla MS ARTÍCULOS DE REPASO - REVIEW ARTICLES 47 DEVELOPMENTAL SEX DISORDERS: BRIEF RE VIEW ON CURRENT ETHICAL ASPECTS. By: Francisco Nieves-Rivera, MD and Lilliam González-Pijem, MD 52 DIABETIC KETOACIDOSIS IN PEDIATRICS: MANAGEMENT UPDATE By: Ricardo García-De Jesús MD REPORTES DE CASOS - CASE REPORTS 57 EVIDENCED BASED MANAGEMENT OF NEONA- TAL HEMANGIOLYMPHANGIOMA: A Case Report. By: Maribel Campos MD, Víctor Ortiz MD, Maria S. Correa MD, Pedro J.Santiago Borrero MD, Ines Garcia MD, Lourdes Garcia MD, Marta Valcárcel 60 PORTADA 62 CME Credits for Vol. 100 Núm 2. BOLETIN - Asociación Médica de Puerto Rico Ave. Fernández Juncos Núm P.O.Box SANTURCE, Puerto Rico Tel.: (787) Fax: (787) Web site: Catalogado en Cumulative Index e Index Medicus Listed in Cumulative Index and Index Medicus No. ISSN Registrado en Latindex -Sistema Regional de Información en Línea para Revistas Científicas de América Latina, el Caribe, España y Portugal Dr. Manuel Quevedo Báez Óleo de Miguel Pou, 1955 Diseño Gráfico y Emplanaje realizado por el Departamento de Prensa y Publicidad de la AMPR Vol Núm 2 - Abril - Junio 2008

4 2 BOLETÍN - ASOCIACIÓN MÉDICA DE PUERTO RICO ASOCIACIÓN MÉDICA DE PUERTO RICO PRESIDENTES SECCIONES DE ESPECIALIDAD JUNTA DE DIRECTORES Eduardo Rodríguez Vázquez, MD Presidente Ricardo Marrero Santiago, MD Presidente Saliente Verónica Rodríguez, MD Secretaria Raúl Casstellanos Bran, MD Tesorero Hilda Ocasio Maldonado, MD Vicepresidente AMPR Rolance Chavier Roper, MD Vicepresidente AMPR Raúl A. Jordán Rivera, MD Vicepresidente AMPR Arturo Arché Matta, MD Presidente Cámara de Delegados José I. Iglesias, MD Vicepresidente Cámara de Delegados Rafael Fernández Feliberti Delegado Alterno AMA Eladio Santos Aponte, MD Delegado Alterno AMA Wanda Vélez Andujar, MD Delegado Alterno AMA José Gerena Díaz Presidente Distrito Este Gustavo Cedeño Quintero, MD Presidente Distrito Noreste Wanda Vélez Andujar, MD Presidente Distrito Sur Mildred Arché Matta Presidente Distrito Central Verónica Rodríguez, MD Presidente Consejo de Educación Médica Continuada Ismael Toro Grajales, MD Presidente Consejo Ético-Judicial Alejandro Medina Vilar Presidente Consejo Relaciones Públicas y Servicios Públicos Jorge Vélez Soto, MD Presidente Consejo Servicios Médicos Eladio Santos Aponte, MD Presidente Consejo Salud Pública y Bienestar Social Natalio Debs Elías, MD Presidente Consejo Política Pública y Legislación Emilio Arce Ortiz, MD Presidente Comité Asesor Presidente Ilia E. Zayas Ortiz, MD Presidente Instituto Educación Médica ANESTESIOLOGÍA Carlos Estrada Gutiérrez, MD CIRUGÍA GENERAL José García Troncoso, MD CIRUGÍA ORTOPÉDICA Kenneth Cintrón, MD CIRUGÍA ESTÉTICA Y RECONSTRUCTIVA Natalio Debs Elías, MD CIRUGÍA TORÁCICA Y CARDIOVASCULAR José O Neill Rivera, MD CIRUGÍA DE MANO José Santiago Figueroa. MD DERMATOLOGÍA Luis J. Ortiz Espinosa, MD ENDOCRINOLOGÍA Eladio Santos Aponte, MD MEDICINA DE FAMILIA Marina Almenas, MD MEDICINA FÍSICA Y REHABILITACIÓN Miguel Berríos, MD MEDICINA INTERNA Ramón A. Suárez Villamil MEDICINA PREVENTIVA Y SALUD PÚBLICA Roberto Rosso Quevedo, MD OFTALMOLOGÍA Emilio Arce López, MD OTORRINONARINGOLOGÍA Charles Juarbe PSIQUIATRÍA Pedro Colberg, MD NEUROLOGÍA Edwin Lugo Piazza, MD UROLOGÍA Pedro Piquer Merino, MD MEDICINA DE EMERGENCIA Pablo Laureano Marti, MD JUNTA EDITORA Humberto Lugo Vicente, MD Presidente Luis Izquierdo Mora, MD Melvin Bonilla Félix, MD Carlos González Oppenheimer, MD Eduardo Santiago Delpin, MD Francisco Joglar Pesquera, MD Yocasta Brugal, MD Juan Aranda Ramírez, MD Francisco J. Muñiz Vázquez, MD Walter Frontera, MD Mario. R. García Palmieri, MD Raúl Armstrong Mayoral, MD José Ginel Rodríguez, MD

5 BOLETÍN - ASOCIACIÓN MÉDICA DE PUERTO RICO 3 Mensaje del Presidente Message from the President Por: Eduardo Rodríguez Vázquez, MD Presidente, Asociación Médica de Puerto Rico La Asociación Médica de Puerto Rico fue fundada en 1902 por el Dr. Manuel Quevedo Báez, en unión a otros distinguidos médicos. Como todos los grupos en que median los seres humanos, la Asociación Médica ha atravesado y sobrepasado varias crisis en su historia. En el último año de su presidencia en la Asociación Médica de Puerto Rico, el sabio científico Dr. Agustín Sthal ( ) publicó en el periódico La Correspondencia de Puerto Rico el anuncio de su renuncia, obedeciendo a la indiferencia completa de los asociados observada desde hace un año y su retraimiento de este centro profesional y científico y conceptuó extinguida la Asociación. Una de las razones que alegaba para su acción era que nuestro Boletín Científico no se había publicado durante más de un año. Gracias a la iniciativa de varios médicos, entre ellos el Dr. Pedro Gutiérrez Igaravidez y el Dr. Rafael Vélez López, se llevó a cabo una reunión en Ponce, pudiéndose, entonces, reorganizar nuestra Asociación Médica. De esto resultó el que nos uniéramos, al año siguiente, a la Asociación Médica Americana, por primera vez. La carta que reproducimos a continuación, escrita por el Dr. Manuel Quevedo Báez y dirigida al Dr. Manuel Pavía Fernández, obedece a otra de las crisis institucionales por las que atravesó la Asociación Médica en la década de Me he tomado la libertad de reproducir todo el contenido de la misma para que sirva de mensaje aleccionador a nuestros colegas que aspiran a ser los futuros líderes de esta venerable institución. Las palabras de nuestro fundador y primer presidente tienen, todavía, gran vigencia en nuestros días.

6 4 BOLETÍN - ASOCIACIÓN MÉDICA DE PUERTO RICO Carta del Dr. Manuel Quevedo Báez al Dr. M. Pavía Fernández 16 de diciembre de 1935 Dr. M. Pavia Fernández Santurce, P.R. Mi querido y buen amigo: no puedo silenciar la satisfacción con que he visto su nombramiento, surgido con unanimidad de la Asamblea, para la presidencia de la Asociación Médica, durante el año próximo. Es motivo justificado para mi la felicitación que debo darte, bien sentida y franca, por el honor que recibes. Espero que seas, en ese puesto, un continuador feliz del pensamiento original y básico que trajo a la vida esa Asociación y de los empeños puestos en práctica, por cuantos te hemos precedido en esa posición de tanta responsabilidad. Sé que arribas a ella en días y momentos difíciles, porque llega, hasta nosotros, a través de todas las distancias, esa ola agria y funesta de los ciegos egoísmos, de irreverencias y de maldades que ha echado a correr, loca, por el mundo. Ojalá no fueran motivos míseros y pobres los que han sembrado la cizaña en nuestro campo! Ojalá que fueran motivos políticos de honda significación y trascendencia; que obedecieran a esa política de verdad, de alto sentido moral y constructivo; la que enaltece a hombres y pue-blos y deja honda huella a su paso por la historia, y no a esa menguada y mezquina que más bien sirve de bochorno y de descrédito a los que la sirven y, de ella, regocijados se nutren! Fuera una fortuna y no pequeña para ti y para cuantos amamos la cordialidad y la unión sincera y fraternal de todas las fuerzas médicas y para cuantos, primero y principalmente, por encima de todo credo u opinión sectarios, nos senti mos puertorriqueños; fortuna grande fuera que, bajo tu presidencia, lograras conjurar esas bochornosas diferencias que tanto lastiman al honor y prestigio de nuestra clase médica. Yo así lo espero: primero, por la virtud médica y el patriotismo de nuestros alejados compañeros, quienes tienen, siempre, muy hondas sus raíces vinculadas en la Asociación Médica y segundo, porque me abona, para esperarlo así, la confianza que tengo en tu probada discreción y buen juicio y tu buena disposición a mantener incólume el espíritu de cordialidad entre la clase. Si ello no se consiguiese, tendríamos dolorosamente, que acusarnos todos, de una grave responsabilidad, porque estaríamos haciendo traición a uno de los más firmes exponentes de la cultura puertorriqueña y, así, estaríamos culpables, maculando el manto de bien ganado prestigio, que, durante un cuarto y más de siglo, cubrió, con grandes honores, a esta Institución Médica. Las páginas que llenan su historia durante treinta y tres laboriosos y fecundos años, son más que luminosas y brillantes. En ellas se copia todo ese proceso maravilloso de avance, que ha seguido el pensamiento médico, desenvolviéndose a tono con los progresos médicos, que se realizan en el mundo científico. La medicina que hemos cultivado, nos ha permitido ver como, en fases sucesivas de progreso, hemos ido pasando de las viejas normas de pura intuición, a través de lo objetivo y sintomático, a las formas que, por mediación del microscopio arrancábamos a la Anatomía Patológica. Y ya esta en nsus vastos campos explorada, comenzamos a entrar vislumbrando un campo nuevo prometedor de grandes conquistas para la Clínica: el de la Biología. Con estos créditos nuestra gloriosa Asociación tiene un buen crédito conquistado y nuestro deber médico y cívico, como verdaderos puertorriqueños, es mantenerlo y defenderlo, a toda costa. Está todo eso, de gloria y prestigio, en tus manos y yo me prometo grandes éxitos para tu honor y el de la clase médica. Sinceramente, compañero y amigo P.S.: Aparte esto, buscar quiero un refugio de honor en esta carta para poner en él una flor, que prendieras en el noble pecho de Mimi tu inspiradora y colaboradora de tus exitos. Ahí va la flor!

7 BOLETÍN - ASOCIACIÓN MÉDICA DE PUERTO RICO 5 Dr. Manuel Quevedo Báez Versión en tamaño real de esta carta puede obtenerse en nuestro web site en la sección President Desk

8 Copyright 2008 Daiichi Sankyo, Inc. and Eli Lilly and Company. All Rights Reserved. PG51957 Printed in USA. June 2008.

9 BOLETÍN - ASOCIACIÓN MÉDICA DE PUERTO RICO 7 Since the opportunity is there I will take advantage of it. From the Editorial Desk... By: Humberto Lugo-Vicente, MD FACS FAAP Editor-in-chief Boletín Asociación Médica de PR While we move the golden pages of this journal a little further we have dedicated this second issue of the Boletin to pediatrics. To the effort of those local scientist who care for the health of newborns, infants and children around our island. Our guest editor for this issue is Dr. Melvin Bonilla, a pediatric nephrologists and member of the editorial board. He has compiled a fruitful group of interesting original articles, reviews and case report. We have managed to continue with providing 4.0 CME credits awards after reviewing and studying several articles. Not so long ago, reckon was the 2004, that we had the sad news of losing one of our beloved pediatrician in our community. He was the heart and soul of academics pediatrics within the private practice organizing for eighteen years the Annual Ashford Presbyterian Hospital Pediatric Course. Of course I m referring to our beloved and friend Dr. Simon Piovanetti. Two weeks after the idles of March Dr. Piovanetti was born in the rural areas of Sabana Grande. Finishing high school in San German, he did a bachelor degree in science at the University of Puerto Rico. The Second World War caused a personal and academic parenthesis in his life while serving with the 65th battalion of infantry. After serving in the army, he completed his general medicine doctorate and pediatric residency at the Jefferson Medical College in Philadelphia. Marries Provi Keelan Capo and has a daughter, Yvette. Yvette is a renowned pediatrician with a keen interest in breast feeding. After finishing the residency years, Simon returns to Puerto Rico where he serves as pediatrician for more than 50 years. Simón Piovanetti, MD FAAP Dr. Piovanetti directed the Department of Pediatrics of the Ashford Presbyterian Hospital for more than fifteen years and participated as member of the directive board of the Puerto Rico Medical Association for more than ten years. Since then he wrote four didactic books in Pediatrics such as: Manual de Pediatría, Simón Dice y las Perlas de Pediatría I and II. Patients and family members describe his best assets as the ability to listen and the ability to forgive without resentments. He could transform pain and suffering into happiness and humor in the blink of an eye. In memorial, this pediatric issue is dedicated to the loving character of Dr. Simon Piovanetti, a great community pediatrician. From a friend to a friend,

10 8 BOLETÍN - ASOCIACIÓN MÉDICA DE PUERTO RICO Artículos Originales - Original Articles A COMPETENCY-BASED COMMUNICATION SKILLS WORKSHOP SERIES FOR PEDIATRIC RESIDENTS By: Débora H. Silva, MD ABSTRACT Background: The use of advanced communication skills to deal with difficult situations is essential to deliver adequate medical care. Description: A four-unit competency-based workshop series was developed for Pediatric Residents. The units are: Communicating Bad News, Communicating in Difficult Physician-Patient Situations, Communicating with Adolescents and Providing Telephone Consultation. The Communicating Bad News unit was fully developed, implemented and pilot tested. Evaluation: The intervention group performed significantly better (p = 0.001) than the non-intervention group in the Pilot Test. Residents found the instructional sessions to be excellent and effective. Conclusions: A competency-based curriculum is likely to be an effective way to teach the use of advanced communication skills needed in complex situations. In addition to learning the skills, residents are also likely to feel more prepared to deal with the situations they encounter. Both skill and confidence are necessary for the adequate delivery of medical care. Key words: Communication Skills, Clinical Skills, Giving Bad News * From the Department of Pediatrics, University of Puerto Rico, School of Medicine Michigan State University Primary Care and Faculty Development Fellowship. Address reprints to: Débora H. Silva MD, FAAP, Curriculum Office, U.P.R. School of Medicine P.O. Box San Juan PR rcm.upr.edu> Good doctor-patient communication is essential for delivery of adequate medical care (1-9, 20). Effective communication is a core clinical skill and should be taught and evaluated at different levels in medical education (1, 10-13). Although most medical schools have established a communication skills curriculum giving emphasis to the medical interview, less attention has been given to teaching advanced communication skills at the residency level (4-5, 16-17). The Accreditation Council on Graduate Medical Education (ACGME) is requiring evidence of residents competence in communication skills since 2003 (13). In addition, by 2010, pediatricians who want to re-certify will need to provide evidence of competency in interpersonal communication skills to the American Board of Pediatrics (14). Although residency programs are starting to develop communication skills curriculums, descriptions are usually narrow in scope, do not address assessment of the curriculum, or do not address the use of skills in a more complex context (4, 15, 17-21, 24). Two developers describe their curriculum and include the use of advanced communications skills. Morgan et al. found that residents thought the curriculum was valuable and effective, but there was no significant change in communication skills. They argued that residents were already evaluated positively in the pre-test, so no change was observed (17). On the other hand, Smith and his colleagues observed that residents trained in communication were superior to untrained residents in knowledge, attitudes and interviewing skills (4). The Pediatric Residency Program at the UPR School of Medicine needs an advanced communication skills curriculum. The residency program is mostly located at a tertiary care center, which serves most of the population in Puerto Rico. In this context, dealing with complex and difficult situations is a common occurrence for residents and they should be adequately prepared. We conducted a literature review and needs assessment at the residency program and developed a competency-based, advanced communication skills curriculum. The curriculum contains four units: Communicating Bad News, Communi cating in Difficult Physician-Patient Situations,

11 BOLETÍN - ASOCIACIÓN MÉDICA DE PUERTO RICO 9 Figure 1: Curriculum Structure Residents will use adequate communication skills to deal complex situations Communicating Bad News Giving Dx/Prognosis Taking DNR Communicating in Difficult Physician-Patient Situations Anxious Angry Depressed Denial Communicating with Adolescents Health Maintenance Education Providing Telephone Consultations Communicating with Adolescents and Answering Telephone Consultations (See Figure1). To begin the assessment of the curriculum, we fully developed, implemented and tested the unit on Communicating Bad News. METHODS Unit Development Process In able to develop the Communicating Bad News Unit, an extensive literature search was conducted. Also the goals and objectives on communication skills of the Residency Program (23) and of the ACGME Competencies were revised (13). Taking all this into consideration, unit objectives, unit content, instructional and evaluation strategies were developed (See Table 1). Unit Implementation The unit on Communicating Bad News was implemented in March Two social workers and five standardized patients (SP) were trained to teach, give feedback and evaluate residents performance in giving bad news following the Giving Bad News Checklist based on the SPIKES Protocol for Giving Bad News (26). The first year pediatric residents were divided into an intervention and a non-intervention group by following the residency programs master program in which some residents are required to attend the teaching activities each month and some are exempt. Those residents required to attend the activities during the month of March 2004 comprised the intervention group; those exempt were the controls. Those residents required to attend the activities during the month of March 2004 comprised the intervention group; those exempt were the controls. They all signed consent forms to participate in the pilot Objective Structured Clinical Exam (OSCE). Five residents were used as controls in the nonintervention group. Seven residents comprised the intervention group; six completed the training. The intervention group underwent four hours of training. In session one, residents listened to a lecture on how to give bad news according to the Giving Bad News Checklist, viewed a live demonstration given by an SP and a faculty member and received the article on the SPIKES Protocol to read (26). In session two, they viewed two videotapes of scenarios where bad news was given in an appropriate and an inappropriate manner. Then the residents analyzed the performances shown in the videotapes according to the checklist. In session three, residents role-played various scenarios in which the physician had to give bad news. Each resident received feedback from a standardized patient (SP). Only two residents also received feedback from one social worker. They had 15 minutes to complete each role-play and 5 minutes for feedback. In the last session residents were divided in groups of two and assigned to an SP. Each student had 15 minutes to give bad news to an SP, following the instructions given to them at the beginning of the interaction. At the completion of the interaction they received five minutes of feedback from the SP. While one student was giving the bad news to the SP, the other was evaluating the interaction following the checklist.

12 10 BOLETÍN - ASOCIACIÓN MÉDICA DE PUERTO RICO Table 1: Unit Development Table Unit Objectives Unit Content Instructional Strategies Learner Evaluation Given a scenario where they have to communicate bad news to a patient/family (Giving Prognosis, Diagnosis and taking DNR) residents will follow the Giving Bad News Checklist adapted from the SPIKES Protocol for Giving Bad News 1. Article on the SPIKES Protocol for Giving Bad News (26) 2. The Giving Bad News Checklist Based on SPIKES Protocol 3. Examples and criterion for the Giving Bad News Checklist 1. Explanation: Pre-lecture reading. Enhanced Skills Lecture 2. Demonstration: Live and video demonstration following the Giving Bad News Checklist 3. Practice: Role- Playing between residents and practice with simulated patients giving bad news following the Giving Bad News Checklist Following OSCE format residents will communicate bad news to 2 simulated patients/families following the Giving Bad News Checklist adapted from the SPIKES Protocol for Giving Bad News. They will be expected to complete 100% of the major checklist criteria and 80% of the sub-criteria on each encounter Unit Pilot Test- Evaluation of Learners Three weeks after the four training sessions were completed and after receiving IRB approval, eleven residents, six of the intervention group and five of the non-intervention group, participated in a two station OSCE. During the OSCE they had to give bad news in two different scenarios: announce to the parents the sudden death of their child due to trauma; and give the diagnosis of Down s syndrome to the grandparent of a newborn child. Both encounters were evaluated by SPs following the Giving Bad News Checklist. Prior to entering the first station, the intervention group also completed a quiz on knowledge about the SPIKES Protocol. Evaluation of the Teaching Strategies To evaluate the teaching strategies used in this unit, the residents in the intervention group filled out an evaluation at the end of the four sessions. In this questionnaire residents were asked to use a Likert Scale to evaluate the overall effectiveness of the sessions in their learning process, and how each of the teaching strategies helped them learn. They were also asked about their confidence level in the skill of giving bad news. Both groups of residents also completed another questionnaire at the end of the OSCE. By means of open-ended questions, they were as ked their opinion about the OSCE, their perceived preparedness and their confidence level in giving bad news. RESULTS Results of Pilot Test-Learner Evaluation The intervention s group average result on the SPIKES Protocol quiz was 93%. The results for the intervention and non-intervention group on the OSCE were compared using the Mann-Whitney Test (Table 2). The intervention group performed significantly better than the non-intervention group on the OSCE (p = 0.001). When evaluating each case separately, the intervention group performed significantly better than the non-intervention group on the sudden death case (p = 0.01), while this difference was marginally significant for the Down s syndrome case (p = 0.05). Based on an item analysis we noted that the intervention group had one checkpoint in each scenario consistently missing: for the sudden death case they did not ask how the caretaker was feeling; and for the Down s syndrome case they did not ask about expectations or hopes. On the other hand, the non-intervention group missed a wide variety of checkpoints. The only consistent checkpoints they followed well were giving empathic and validating statements and offering to call for support. Table 2: Results of Pilot Test N=11 p =.010 p =.052 p =.001 Intervention N=6 Intervention N=5 Sudden Death Case Down s Syndrome Case (Mean Checklist Score) (Mean Checklist Score) 96 % 68 % 91 % 78 % Overall Score 93 % 73 %

13 BOLETÍN - ASOCIACIÓN MÉDICA DE PUERTO RICO 11 Results of Teaching Strategies Evaluation The responses of the intervention group in the evaluation of the teaching strategies indicated that they were very satisfied with the curriculum. All of them rated the overall effectiveness of the sessions as excellent. They all strongly agreed or agreed that the article on the SPIKES Protocol, the demonstrations, the role-playing, and the practice with SPs helped them learn. They also strongly agreed or agreed that they received adequate feedback about their performance and they felt more confident in giving bad news after the sessions. They consistently gave two valuable recommendations: to assign more time for each session, and to involve more faculty members. The questionnaire that the intervention and non-intervention group completed at the end of the OSCE revealed that all the residents in the intervention group felt prepared to give the bad news while none in the non-intervention group did. On the other hand, at the time of the OSCE, only two of the six residents in the intervention group felt comfortable giving the bad news; the other four and all the residents in the non-intervention group felt uncomfortable and thought they needed considerable practice to improve. All residents agreed a curriculum is necessary to learn these techniques. DISCUSSION As evidenced by the results, in the pilot test residents in the intervention group performed significantly better than those in the non-intervention group when asked to give bad news. The item analysis found that residents in the intervention group did not ask about feelings and hopes. Both gaps are consistent with the usual fears of giving bad news. When someone dies it is hard to ask how the family is feeling because the expected answer is that they are devastated. Further, when we give a diagnosis of a chronic illness, such as Down s syndrome, we do not want to hear the parents hopes because we do not want to destroy them. More meaningful is the fact that the residents in the intervention group stated they felt prepared to give bad news even when they still felt uncomfortable with the situations. At the same time, those in the non-intervention group felt uncomfortable and ill prepared. A reasonable question would be if physicians can ever feel comfortable giving bad news, irrespective of training. Therefore, feeling prepared could arguably be good enough. The didactic component of the curriculum is being implemented in a stepwise manner. At this time all the residents have attended the Communicating Bad News workshops, which have been repeated very two years since The Unit on Adolescent Communication was offered in The Unit on Communicating in Difficult Physician Patient Situations was offered to all second year residents as part of the Residents-as-Teachers Curriculum of the Medical School in The next unit to be developed and implemented will be the Telephone Interview Unit. For this curriculum to work, faculty must be trained to teach and assess learners in order to achieve consistency in the residents performance. Faculty must give reliable feedback throughout the years and among the clinical settings. For this to be achieved, the Faculty Development Program at the University of Puerto Rico School of Medicine has developed a Clinical Educators Curriculum which includes training in Giving Bad News, Dealing with Difficult Physician-Patient Situations and Giving Feedback. All of these topics have been offered in a yearly basis since The next step will be the development of an OSCE that tests all the clinical skills expected of a pediatrician, since it would be too costly to do one just for communication skills. Meanwhile, evaluation of learners is being done in a monthly manner as part of the usual resident evaluation. The faculty member in charge evaluates and gives feedback on these skills as observed during that month s rotation. With the new era of competency assessments and the increasing requirements by the general public and the accreditation agencies, we must teach residents the use of communication skills needed to deal with complex situations. We developed and tested a curriculum to teach advanced communication skills. The results of the pilot test suggest that residents are likely to learn these skills when properly taught in a competency-based manner. REFERENCES 1. Association of American Medical Colleges, Report III Contemporary Issues in Medicine: Communication in Medicine. Medical School Objectives Project, October P U.S. Department of Health and Human Services, Healthy People 2010 Conference Edition. January Volume 1: p NYU Macy Initiative on Health Communication, Curriculum. Retrieved August 17, 2003 from the World Wide Web: 4. Smith, R., et al., The Effectiveness of Intensive Training for Residents in Interviewing A Randomized, Controlled Study. Annals of Internal Medicine, (2): p Kurtz, S., J. Silverman, and J. Draper (1998). Teaching and Learning Communication Skills in Medicine. Radcliffe Medical Press, UK. 6. Meryn, S., Improving doctor-patient communication. BJM, : p

14 12 BOLETÍN - ASOCIACIÓN MÉDICA DE PUERTO RICO 7. Pfeiffer, C., H. Mandray, A. Ardolino, and F. Willms, The rise and fall of students skills in obtaining a medical history. Medical Education, : p Suzuki Laidlaw, T. et. al.,implementing a communication skills programme in medical school: needs assessment and programme change. Medical Education, : p Van Dalen, J., et. al., Teaching and assessing communication skills in Maastricht: the first twenty years. Medical Teacher, (3): p Kurtz, S. and J. Silverman, The Calgary-Cambridge Referenced Observation Guides: an aid to defining the curriculum and organizing the teaching in communication training programmes. Medical Education, : p Grant, V.J. and S. J. Hawken, What do they think of it now? Medical graduates views of earlier training in communication skills. Medical Teacher, (3): p Spencer, J. and J. Silverman, Education for communication: much already known, so much more to understand. Medical Education, (3): p ACGME Outcome Project, UPDATE/VERIFY GENERAL COMPETENCIES ASSESSMENT. Retrieved August 17, 2003 from the World Wide Web: acgme.org 14. Stockman, J. A., P. Miles, and H. P. Ham, The Program for Maintenance of Certification in Pediatrics. Pediatric Diplomats, Fall p Dalhousie University, Communication Skills Program. Retrieved August 17, 2003 from the World Wide Web: 16. Hulsman, R.L., et.al., Teaching clinically experienced physicians communication skills. A review of evaluation studies. Medical Education, : p Morgan E. and R. Winter, Teaching Communication Skills: An Essential Part of Residency Training. Archives of Pediatric and Adolescent Medicine, : p Langewitz, W.A., et. al., Improving communication Skills-A Randomized Controlled Behaviorally Oriented Intervention Study for Residents in Internal Medicine. American Psychosomatic Society, (3): p Chou, C. and L. Kewchang, Improving Residents Interviewing Skills by Group Videotape Review. Academic Medicine, : p Oh, J., et. al., Retention and Use of Patient-centered Interviewing Skills after Intensive Training. Academic Medicine, (6): p Ruiz Morales, R., et. al., Effectiveness of a Clinical Interviewing Training Program for Family Practice Residents: A Randomized Controlled Trial. Family Medicine, (7):p Participants in the Bayer-Fetzer Conference on Physician-Patient Communication in Medical Education, Essential Elements of Communication in Medical Encounters: The Kalamazoo Consensus Statement. Academic Medicine, (4): p General Pediatrics Residency Program, University of Puerto Rico, School of Medicine, Goals and Objectives Manual, p Roth, C.S., et. al., A communication Assessment and Skill-building Exercise (CASE) for First-year Residents. Academic Medicine, : p Coulehan, J. L. and M. R. Block (2001). Difficult Feelings in the Medical Interview. The medical Interview: Mastering Skills for Clinical Practice Fourth Edition. F.A. Davis. p Baile, W. F., et.al. SPIKES-A Six Step Protocol for Delivering Bad News: Application to the Patient with Cancer. The Oncologist, : p Vanderkieft, G. K. Breaking Bad News. American Family Physician, (12): p Acknowledgements The author would like to thank Dr. Ken Sheets, Dr. Suzanne Kurtz, Dr. Karen Kent and Dr Barbara Korsch for their critical review of the overall curriculum manuscript, submitted as part of the Michigan State University Primary Care and Faculty Development Fellowship at Michigan State University. Special thanks to Dr. Steve Yelon and John Williamson at Michigan State University Primary Care and Faculty Development Fellowship for all the advice and mentorship through out all the steps of the curriculum development and the pilot test implementation. Resumen Introducción: Saber utilizar destrezas de comunicación avanzadas en situaciones difíciles es un componente esencial de la relación médico-paciente. Descripción: Con este propósito, se desarrolló un currículo basado en competencias de comunicación avanzada para residentes de Pediatría. El currículo consta de cuatro unidades. La primera unidad, Como Comunicar Malas Noticias, se desarrolló en su totalidad como proyecto piloto. La unidad se implementó utilizando el protocolo de SPIKES para dar malas noticias como base. Los residentes que participaron del grupo de intervención recibieron práctica y retro-insumo en la destreza de ofrecer malas noticias. Al finalizar el piloto, los residentes del grupo de intervención y un grupo control fueron examinados a través de un examen práctico, objetivo y estandarizado utilizando la metodología de Pacientes Estandarizados. Evaluación: Se observó que el grupo de intervención ejecutó significativamente mejor que el grupo control en este examen (p<0.001). Conclusión: En conclusión, un currículo basado en competencias parece ser una manera efectiva de enseñar las destrezas de comunicación avanzadas que se necesitan en situaciones difíciles. Web Site de la Asociación Médica de Puerto Rico Calendario de actividades Publicaciones on-line Créditos Educación Médica Continuada Servicios con PayPal y tarjetas de crédito Suscripciones on-line y mucho más..

15

16 14 BOLETÍN - ASOCIACIÓN MÉDICA DE PUERTO RICO HEMOLYTIC UREMIC SYNDROME IN CHILDREN IN PUERTO RICO: A RARE DISEASE WITH ATYPICAL FEATURES By:Yasmín Pedrogo-Rodríguez, MD *, Juan O. Pérez-Rodríguez, MD **, Melvin Bonilla-Felix, MD ** ABSTRACT Hemolytic Uremic Syndrome (HUS) consists of a triad of acquired hemolytic anemia, thrombocytopenia, and renal failure that occurs acutely in otherwise healthy individuals. HUS may be divided into two broad categories, typical, preceded by a diarrheal prodrome, and atypical. The clinical symptoms of HUS as well as its course, prognosis, and response to treatment appear to be significantly influenced by a number of factors, including age at onset, type and severity of underlying infections, and/or systemic diseases. A retrospective case series review of five patients diagnosed with Hemolytic Uremic Syndrome at the Pediatric University Hospital in Puerto Rico between was performed. The study showed that the incidence of HUS in children in Puerto Rico is lower than other countries. However, the majority of cases have an atypical presentation, which places our patients at higher risk for life-threatening complications. Index words: hemolytic, uremic, syndrome, pediatrics Hemolytic Uremic Syndrome (HUS) consists of a triad of acquired hemolytic anemia, thrombocytopenia, and renal failure that occurs acutely in otherwise healthy individuals. Since its description in 1955 (1), HUS has been recognized predominantly in children and in this age group is a common cause of acute renal failure. Initially believed to be a renal disorder with secondary hematologic manifestations, the syndrome should be regarded as a systemic disease. It occurs most commonly in young children with a mean age of four years old. HUS may be divided into two broad categories, typical, preceded by a diarrheal prodrome, and atypical. The disease most frequently follows an episode of gastroenteritis caused by an enterohemorrhagic strain of Eschericia coli 0157:H7, (2). Atypical HUS is often insidious in onset, it does not follow a diarrheal illness; and has a high incidence of extrarenal involvement, especially neurologic abnormalities such as focal or generalized seizures, transient hemiparesis, or even coma (2, 4). Secondary forms of HUS have been reported following use of oral contraceptives, cyclosporine, mitomycin and pyran copolymer. Several reports describe occurrence in more than one member of a family, but the role of genetics factors in predisposition to the disease is unkown. The severity of the renal involvement and the complications, vary from mild renal insufficiency to acute renal failure requiring dialysis and/or plasmapheresis (5). The incidence of HUS is apparently lower in Puerto Rico than in the United States and other countries. The clinical symptoms of HUS as well as its course, prognosis, and response to treatment appear to be significantly influenced by a number of factors, including age at onset, type and severity of underlying infections, and/or systemic diseases. We herein report the clinical features of the pediatric patients diagnosed with HUS at the Pediatric University Hospital between 1997 and METHODS A retrospective review of the medical records of five patients diagnosed with Hemolytic Uremic Syndrome at the Pediatric University Hospital in Puerto Rico between 1997 and 2007 was performed. All children presented with the classic triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. A small group of older children diagnosed during the same period with thrombotic thrombocytopenic purpura due to presence of prominent neurological symptoms with mild or absent renal disease, were not included in this analysis. The data collected included the age at presentation of the disease, gender, the presence of sings and symptoms such as diarrhea, bloody stools, anuria, hypertension, neurological features, the treatment modalities used (dialysis, plasmapheresis, platelet and pack red blood cell transfusions). In addition, important laboratory data such as the urinalysis, estimated glomerular filtration rate (GFR) at presentation and on last evaluation were collected. The GFR was calculated using Schwartz formula (6). * Fom the Department of Pediatrics and ** Section of Pediatric Nephrology, Department of Pediatrics, University of Puerto Rico Medical Sciences Campus. Address reprints to: Melvin Bonilla-Félix, MD, Department of Pediatrics, University of Puerto Rico Medical Sciences Campus. PO Box , San Juan, Puerto Rico Tel x7300. Fax

17 BOLETÍN - ASOCIACIÓN MÉDICA DE PUERTO RICO 15 These results were compared with the course of illness and survival of patients. RESULTS The demographic and clinical features are shown on Table 1. There was no significant gender predominance. Ages ranged between 6 to 204 months, with a mean age of 82 months. Three out of the five patients presented with diarrhea, two of them with bloody stools (patients 2 and 3). There was no family history of HUS, and no neurological changes associated with the diagnosis. Also four out of 5 patients had hypertension at presentation. One of them had history of high blood pressure since infancy (patient 1) and presented with a hypertensive crisis before developing the classic features of HUS (anemia, thrombocytopenia and acute renal failure symptoms). All the patients presented with hematuria and proteinuria at the time of diagnosis of HUS (Table 2). At the time of last follow up (2 14 months after diagnosis), two of the patients showed no evidence of proteinuria (patients 1 and 2). All the patients had decreased GFR at presentation. Only one patient showed complete resolution of renal disease demonstrated by a normal GFR and urinary sediment. Four out of five patients required acute dialysis (Table 3). Three out of five patients required transfusions with red blood cells and platelets. Two of the patients died. Both of them had recurrent episodes of hemolysis and thrombocytopenia associated with decreased GFR, requiring treatment with plasmapheresis. DISCUSSION The incidence of HUS in children in Puerto Rico is lower than other countries. The mean age for HUS in the island (82 months) is similar to other countries (48 months) with higher incidence of the disease. Our report most likely represents most, if not all the cases of HUS in Puerto Rico in the last 10 years. The majority of these cases did not show the typical features of HUS. Only 2 out of 5 had a prodrome of bloody diarrhea. Two out of five patients presented recurrent episodes of hemolysis, thrombocytopenia and renal failure. Both of these patients died during the course of the disease. One of them died from Staphylococcal sepsis, since he had reached end stage renal disease and was receiving peritoneal dialysis. In addition, these two patients received dialysis, plasmapheresis, platelet and pack red blood cell transfusions due to multiple relapses of the illness, ending in death. Hemolytic Uremic Syndrome is a rare disease in Puerto Rico. However, the absence of the typical diarrheal prodrome in the majority of our patients, place them in a higher risk category. This may delay the diagnosis and treatment since it can be confused with other febrile illnesses that are common in the island, such as leptospirosis and dengue fever. Since most of our patients required acute dialysis, early recognition and prompt referral to a Pediatric Tertiary Hospital with dialysis facilities available is important in order to prevent life-threatening complications. The lower incidence of the disease in Puerto Rico as compared with other countries in the world could result from the cultural preference of most Puerto Ricans to eat well-cooked meat, which decreases the risk of contamination with Eschericia coli. Although we were not able to obtain data on the association of E. coli 0157:H7 and HUS in our population, the absence of diarrheal prodrome in three out 5 patients suggests that most of our cases are not associated with E. coli, which might explain the severity of the disease and high mortality rate observed. In summary, the incidence of HUS in the pediatric population in Puerto Rico is low. However, because of the absence of a diarrheal prodrome, the disease usually follows an atypical course, resulting in significant morbidity and mortality. We believe that all children with the triad of anemia, thrombocytopenia and renal symptoms should be referred immediately to a pediatric tertiary care facility to begin aggressive therapy, including dialysis, if necessary. REFERENCES 1. Tarr PI, and, Hickman, Robert: Hemolytic Uremic Syndrome epidemiology: A population-based study in King County, Washington, 1971 to 1980, Pediatrics July 1987; 80 (1): Walters M, Levin M, Smith C, Nokes T, Hardisty R, Dillon M, Martin Barrat T: Intravascular platelet activation in the hemolytic uremic syndrome, Advances in Pediatrics Infectious Diseases 1989; 4: Neill MA, Tarr PI, Clausen CR, Christie DL, Hickman RO: Eschericia coli 0157:H7 as the predominant pathogen associated with the Hemolytic Uremic Syndrome: A prospective study in the Pacific Northwest, Pediatrics July 1987; 80(1) : Remuzzi G, Garella S: HUS and TTP: Variable expression of a single entity, Kidney International 1987; 32: Rizzoni G, Claris A, Edefonti A, Facchin P, Franchini F, Gusmano R, Imbasciati E, Pavanello L, Perfumo F, Remuzzi G. Plasma infusion for hemolytic-uremic syndrome in children: Results of a multicenter controlled trial, J Pediatrics 1988; 112: Robertson J, Shilkofski N: The Harriet Lane Handbook, 17th Ed., Pennsylvania, Elsevier Mosby, 2005:

18 16 BOLETÍN - ASOCIACIÓN MÉDICA DE PUERTO RICO Table 1. Demographic and Clinical Data TABLES Patient Age (months) Gender Bloddy diarrhea HTN Anuria 1 32 M F F M M HTN = Hypertension Table 2. Laboratory Data and Prognosis Patient Urine sediment (Last F/U) GFR (presentation) GFR (Last F/U) Outcome (Death) unknoww U/A = Urinalysis F/U = Follow up GFR : Ml/min/1.73m2 Table 3. Treatment Data Patient Dialysis Plasmapheresis PRBC transfusion Platelet transfusion PRBC = Pack Red Blood Cell RESUMEN El síndrome hemolítico urémico (HUS) consiste de la triada de anemia hemolítica, trombocitopenia y fallo renal que ocurre de forma aguda en individuos saludables. Éste síndrome se puede clasificar en dos categorías amplias; típico, precedido por diarreas, usualmente sanguinolentas y atípico, o no asociado a diarreas. Los síntomas clínicos de HUS, al igual que su curso, pronóstico y respuesta a tratamiento parecen estar influenciados por un grupo de factores, incluyendo la edad del paciente y presencia de otr os factores o infecciones. Se realizó una revisión retrospectiva de los pacientes diagnosticados con HUS en el Hospital Pediátrico Universitario de Puerto Rico entre 1997 y El estudio demostró una incidencia baja de HUS en Puerto Rico. La mayoría de los casos observados tuvieron una presentación atípica, lo cual coloca nuestros pacientes a un riesgo mayor de manifestaciones serias y pobre pronóstico a largo plazo.

19

20 18 BOLETÍN - ASOCIACIÓN MÉDICA DE PUERTO RICO THROMBOCYTOSIS IN ILLICIT DRUGS-EXPOSED NEWBORNS By: Thea Calderón MD *, Sonia Medina MD *, Inés García MD **, Lourdes García MD **, Marta Varcárcel MD ** ABSTRACT Thrombocytosis in infants exposed in-utero to illicit drugs has been associated to methadone exposure. Although is reported to present in the first two weeks, few studies address its duration and timing of resolution. This study evaluated the presence, duration, and complications of thrombocytosis in newborns exposed to illicit drugs. Methods: A retrospective review of medical records of newborns with intrauterine drug exposure admitted to the San Juan City Hospital from 1999 to 2001 was performed. Results: Thirty-one newborns were included. Eighty-seven percent (87%) presented abstinence syndrome. Of these, 96% presented thrombocytosis. All infants exposed to methadone presented thrombocytosis and 75% of those exposed to heroin and cocaine. Thrombocytosis presented at ten days of life with a median resolution at 26 days. Conclusions: In this group of newborns, thrombocytosis was associated to intrauterine exposure to methadone, heroin, and cocaine. Thrombocytosis presented at ten days of life and resolution was seen in three to 4 weeks without complications observed. Index words: thrombocytosis, illicit-drugs, newborns The use of drugs during pregnancy presents consequences to the fetus and neonate. Short and long- term neurobehavioral problems have been documented in infants born to substance-abusing mothers (1). Frequently, these infants are also exposed to lack of prenatal care, poor nutrition, and infectious agents. Thrombocytosis in infants with intrauterine exposure to illicit drugs has been reported in the literature (2). Most reports associate thrombocytosis to methadone exposure. Thrombocytosis is reported to present in the first two weeks of life, but few reports address its duration and timing of resolution. The purpose of this study was to obtain further information on the effects of illicit drugs on newborns along with the natural history of thrombocytosis in this group of patients. MATERIALS AND METHODS Medical records of newborns with intrauterine exposure to illicit drugs, admitted to the San Juan City Hospital during the period of 1999 to 2001 were reviewed. Data gathered included sex, birth weight, and gestational age. Serum platelet counts throughout hospitalization were recorded. Presence of thrombocytosis, duration, and complications were recorded. Thrombocytosis was defined as platelet levels higher than or equal to 450,000 x 103/ul for term infants and higher than or equal to 600,000 x 103/ul for preterm infants3. Exclusion criteria included medical conditions associated to thrombocytosis such as infections or inflammation, Down syndrome, congenital adrenal hyperplasia, and exposure to cephalosporins (3, 4). The study was approved by the Institutional Review Board. RESULTS Thirty-one newborns met inclusion criteria. Mean gestational age was 36 weeks and mean birth weight 2600 grams. Forty-eight percent (48%) of the newborns were males (17 males, 14 females) and 87% presented positive urine toxicology. Eighty-seven percent (87%) of all infants presented drug withdrawal syndrome and all of these infants were treated with paregoric, phenobarbital or both. Twelve percent (12%) of the term infants were products of mothers that used more than one illicit drug during pregnancy, in comparison with 54% of the preterm infants (p= ). The most frequent illicit drug used by mothers was cocaine (40%) followed by multiple drugs (30%), methadone (13.3%), heroin (13.3%), and marihuana (3.3%). Ninety-six percent (96%) of patients admitted due to drug withdrawal syndrome presented thrombocytosis. Median platelet count in infants with thrombocytosis was 693,000 x 103/ul (range 456,000 x 103 /ul to 1,343,000 x 103/ul). * From the Department of Pediatrics, San Juan City Hospital, and ** Section of Neonatology, Department of Pediatrics, U.P.R. School of Medicine, San Juan, Puerto Rico. Address reprints to: Lourdes García MD, UPR School of Medicine, Department of Pediatrics, Neonatology Section, GPO Box , San Juan, PR Tel , fax ,

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