PEDIATRIC PEARLS FOR THE USMLE

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visited=11%2F23%2F2009; visited=11%2F23%2F2009; visited=11%2F23%2F2009; visited=11%2F23%2F2009; visited=11%2F23%2F2009><table cellpadding=0 cellspacing=0 border=0><tr> <td valign="top" colspan=2 height=110 BGCOLOR="#666699" TEXT="#080000" bgproperties="fixed" background="04a00840erlcjm.gif"> <div> <FONT SIZE="5" COLOR="#FFFFFF" FACE="Arial" ><B>pediatryPART I</B></FONT><B>     |     </B><A HREF="index.htm" TARGET="_top" TITLE="pediatryPART I"><U><B>home</B></U></A></div> <div> <B>PEDIATRIC PEARLS FOR THE USMLE</B>   |   <A HREF="id48.htm" TARGET="_top" TITLE="pearls pediatry 2"><U>pearls pediatry 2</U></A>   |   <A HREF="id18.htm" TARGET="_top" TITLE="general"><U>general</U></A>   |   <A HREF="id34.htm" TARGET="_top" TITLE="appendiceal colic"><U>appendiceal colic</U></A>   |   <A HREF="id35.htm" TARGET="_top" TITLE="ATOPIC DERMATITIS"><U>ATOPIC DERMATITIS</U></A>   |   <A HREF="id19.htm" TARGET="_top" TITLE="Stuttering"><U>Stuttering</U></A>   |   <A HREF="id20.htm" TARGET="_top" TITLE="enuresis"><U>enuresis</U></A>   |   <A HREF="id21.htm" TARGET="_top" TITLE="Conduct Disorder in Children and Adolesc"><U>Conduct Disorder in Children and Adolescents </U></A>   |   <A HREF="id22.htm" TARGET="_top" TITLE="Specific Growth Curve Deviations "><U>Specific Growth Curve Deviations </U></A>   |   <A HREF="id23.htm" TARGET="_top" TITLE="Common Medical and Surgical Problems"><U>Common Medical and Surgical Problems</U></A>   |   <A HREF="id24.htm" TARGET="_top" TITLE="Chronic Abdominal Pain in Childhood: Dia"><U>Chronic Abdominal Pain in Childhood: Diagnosis and Management </U></A>   |   <A HREF="id25.htm" TARGET="_top" TITLE="child devlpt"><U>child devlpt</U></A>   |   <A HREF="id26.htm" TARGET="_top" TITLE="ACETAMINOPHEN OVERDOSE"><U>ACETAMINOPHEN OVERDOSE</U></A>   |   <A HREF="id27.htm" TARGET="_top" TITLE="SHAKEN BABY SYNDROME"><U>SHAKEN BABY SYNDROME</U></A>   |   <A HREF="id28.htm" TARGET="_top" TITLE="NEONATAL JAUNDICE (HYPERBILIRUBINEMIA)"><U>NEONATAL JAUNDICE (HYPERBILIRUBINEMIA)</U></A>   |   <A HREF="id29.htm" TARGET="_top" TITLE="Hyaline Membrane Disease (Respiratory Di"><U>Hyaline Membrane Disease (Respiratory Distress Syndrome) (HMD) (RDS</U></A>   |   <A HREF="id30.htm" TARGET="_top" TITLE="infectious disease"><U>infectious disease</U></A>   |   <A HREF="id32.htm" TARGET="_top" TITLE="HEMORRHAGIC DISEASE IN A NEW BORN"><U>HEMORRHAGIC DISEASE IN A NEW BORN</U></A>   |   <A HREF="id33.htm" TARGET="_top" TITLE="OPTIC NERVE GLIOMA"><U>OPTIC NERVE GLIOMA</U></A>   |   <A HREF="id36.htm" TARGET="_top" TITLE="New Born Assessment "><U>New Born Assessment </U></A>   |   <A HREF="id38.htm" TARGET="_top" TITLE="laryngomalacia"><U>laryngomalacia</U></A>   |   <A HREF="id39.htm" TARGET="_top" TITLE="CRANIOSYNOSTOSIS "><U>CRANIOSYNOSTOSIS </U></A>   |   <A HREF="id40.htm" TARGET="_top" TITLE="DANDYWALKER MALFORMATION "><U>DANDY-WALKER MALFORMATION </U></A>   |   <A HREF="id41.htm" TARGET="_top" TITLE="DEJERINESOTTAS DISEASE "><U>DEJERINE-SOTTAS DISEASE </U></A>   |   <A HREF="id42.htm" TARGET="_top" TITLE="DOWN SYNDROME "><U>DOWN SYNDROME </U></A>   |   <A HREF="id43.htm" TARGET="_top" TITLE="Respiratory Syncytial Virus (RSV)"><U>Respiratory Syncytial Virus (RSV)</U></A>   |   <A HREF="id44.htm" TARGET="_top" TITLE="FABRY DISEASE "><U>FABRY DISEASE </U></A>   |   <A HREF="id45.htm" TARGET="_top" TITLE="PRADERWILLI SYNDROME "><U>PRADER-WILLI SYNDROME </U></A>   |   <A HREF="id37.htm" TARGET="_top" TITLE="new born fun"><U>new born fun</U></A>   |   <A HREF="id46.htm" TARGET="_top" TITLE="RETT SYNDROME "><U>RETT SYNDROME </U></A>   |   <A HREF="id47.htm" TARGET="_top" TITLE="Alexander Disease"><U>Alexander Disease</U></A>   |   <A HREF="id49.htm" TARGET="_top" TITLE="leukemia"><U>leukemia</U></A>   |   <A HREF="id17.htm" TARGET="_top" TITLE="Contact Me"><U>Contact Me</U></A></div> <div> </div> </td> </tr><tr> <td valign="top" width=190 BGCOLOR="#CCCCFF" TEXT="#080000" background="04411c00egdysm.gif"> <div> <B> <img src="HTMLobj-187/aniGif.gif" border="0" align="bottom" width="150" height="170">  </B></div> <bR> <div> <B>PEDIATRIC PEARLS FOR THE USMLE</B></div> <div> <A HREF="id48.htm" TARGET="_top" TITLE="pearls pediatry 2"><U><B>pearls pediatry 2</B></U></A></div> <div> <A HREF="id18.htm" TARGET="_top" TITLE="general"><U><B>general</B></U></A></div> <div> <A HREF="id34.htm" TARGET="_top" TITLE="appendiceal colic"><U><B>appendiceal colic</B></U></A></div> <div> <A HREF="id35.htm" TARGET="_top" TITLE="ATOPIC DERMATITIS"><U><B>ATOPIC DERMATITIS</B></U></A></div> <div> <A HREF="id19.htm" TARGET="_top" TITLE="Stuttering"><U><B>Stuttering</B></U></A></div> <div> <A HREF="id20.htm" TARGET="_top" TITLE="enuresis"><U><B>enuresis</B></U></A></div> <div> <A HREF="id21.htm" TARGET="_top" TITLE="Conduct Disorder in Children and Adolesc"><U><B>Conduct Disorder in Children and Adolescents </B></U></A></div> <div> <A HREF="id22.htm" TARGET="_top" TITLE="Specific Growth Curve Deviations "><U><B>Specific Growth Curve Deviations </B></U></A></div> <div> <A HREF="id23.htm" TARGET="_top" TITLE="Common Medical and Surgical Problems"><U><B>Common Medical and Surgical Problems</B></U></A></div> <div> <A HREF="id24.htm" TARGET="_top" TITLE="Chronic Abdominal Pain in Childhood: Dia"><U><B>Chronic Abdominal Pain in Childhood: Diagnosis and Management </B></U></A></div> <div> <A HREF="id25.htm" TARGET="_top" TITLE="child devlpt"><U><B>child devlpt</B></U></A></div> <div> <A HREF="id26.htm" TARGET="_top" TITLE="ACETAMINOPHEN OVERDOSE"><U><B>ACETAMINOPHEN OVERDOSE</B></U></A></div> <div> <A HREF="id27.htm" TARGET="_top" TITLE="SHAKEN BABY SYNDROME"><U><B>SHAKEN BABY SYNDROME</B></U></A></div> <div> <A HREF="id28.htm" TARGET="_top" TITLE="NEONATAL JAUNDICE (HYPERBILIRUBINEMIA)"><U><B>NEONATAL JAUNDICE (HYPERBILIRUBINEMIA)</B></U></A></div> <div> <A HREF="id29.htm" TARGET="_top" TITLE="Hyaline Membrane Disease (Respiratory Di"><U><B>Hyaline Membrane Disease (Respiratory Distress Syndrome) (HMD) (RDS</B></U></A></div> <div> <A HREF="id30.htm" TARGET="_top" TITLE="infectious disease"><U><B>infectious disease</B></U></A></div> <div> <A HREF="id32.htm" TARGET="_top" TITLE="HEMORRHAGIC DISEASE IN A NEW BORN"><U><B>HEMORRHAGIC DISEASE IN A NEW BORN</B></U></A></div> <div> <A HREF="id33.htm" TARGET="_top" TITLE="OPTIC NERVE GLIOMA"><U><B>OPTIC NERVE GLIOMA</B></U></A></div> <div> <A HREF="id36.htm" TARGET="_top" TITLE="New Born Assessment "><U><B>New Born Assessment </B></U></A></div> <div> <A HREF="id38.htm" TARGET="_top" TITLE="laryngomalacia"><U><B>laryngomalacia</B></U></A></div> <div> <A HREF="id39.htm" TARGET="_top" TITLE="CRANIOSYNOSTOSIS "><U><B>CRANIOSYNOSTOSIS </B></U></A></div> <div> <A HREF="id40.htm" TARGET="_top" TITLE="DANDYWALKER MALFORMATION "><U><B>DANDY-WALKER MALFORMATION </B></U></A></div> <div> <A HREF="id41.htm" TARGET="_top" TITLE="DEJERINESOTTAS DISEASE "><U><B>DEJERINE-SOTTAS DISEASE </B></U></A></div> <div> <A HREF="id42.htm" TARGET="_top" TITLE="DOWN SYNDROME "><U><B>DOWN SYNDROME </B></U></A></div> <div> <A HREF="id43.htm" TARGET="_top" TITLE="Respiratory Syncytial Virus (RSV)"><U><B>Respiratory Syncytial Virus (RSV)</B></U></A></div> <div> <A HREF="id44.htm" TARGET="_top" TITLE="FABRY DISEASE "><U><B>FABRY DISEASE </B></U></A></div> <div> <A HREF="id45.htm" TARGET="_top" TITLE="PRADERWILLI SYNDROME "><U><B>PRADER-WILLI SYNDROME </B></U></A></div> <div> <A HREF="id37.htm" TARGET="_top" TITLE="new born fun"><U><B>new born fun</B></U></A></div> <div> <A HREF="id46.htm" TARGET="_top" TITLE="RETT SYNDROME "><U><B>RETT SYNDROME </B></U></A></div> <div> <A HREF="id47.htm" TARGET="_top" TITLE="Alexander Disease"><U><B>Alexander Disease</B></U></A></div> <div> <A HREF="id49.htm" TARGET="_top" TITLE="leukemia"><U><B>leukemia</B></U></A></div> <div> <A HREF="id17.htm" TARGET="_top" TITLE="Contact Me"><U><B>Contact Me</B></U></A></div> </td> <td valign="top" height=370 BGCOLOR="#FFFFFF" TEXT="#080000"> <div> PEDIATRIC PEARLS FOR THE USMLE</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">DETAILS ARE SEE IN DIFFERENT PAGES </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">2-year-old boy presents with recurrent and severe paroxysmal colicky pain accompanied by straining efforts, loud cries, and vomiting. A stool with red blood mixed with mucus is passed. An oblong mass is palpated in the midepigastrum. : <U>THE FIRST DIAG IS AN </U><B><U>INTUSSUCEPTION[SEE MY SURGICAL LECTURES PAGE]</U></B></div> <bR> <bR> <div> <TABLE BORDER="0" CELLPADDING="1" CELLSPACING="1" WIDTH="771" BORDERCOLORLIGHT="#C0C0C0" BORDERCOLORDARK="#808080" FRAME="VOID" RULES="NONE" ALIGN="BOTTOM" HSPACE="0" VSPACE="0" > <tR> <tD VALIGN=CENTER HEIGHT= 58 WIDTH="767"><div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">A 4-month-old infant is brought to the pediatrician by his mother because he has not been feeding well. Physical examination of the mouth reveals curd-like plaques on the tongue and buccal mucosa that do not scrape off easily. Another associated physical finding: <B><U>PERINEUM FOR A DIAG OF CANDIDA</U></B></div> </tD> </tR> </TABLE></div> <bR> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">An infant is brought to the community clinic because the mother states that the baby has a rash, which is extremely pruritic. Further questioning reveals that the mother also has a pruritic rash. The physician determines the diagnosis to be scabies. Which of the following best describes the manner in which infantile scabies differs from adult scabies: <B><U>HAS NO BURROWS</U></B></div> <bR> <bR> <bR> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">A child with known neurofibromatosis presents with unilateral decrease in visual acuity, pallor of the disc, and exophthalmos.  the most likely diagnosis: <B>optic nerve glioma</B></div> <bR> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Milia:</B></div> <div> Appear as tiny white dots on the nose and the face. These are nothing but distended sebaceous glands and they disappear on their own.</div> <bR> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Erythema Toxicum:</B></div> <div> Despite its ominous sounding name these are just a few harmless reddish patches, which may appear on the trunk and face. These also clear on their on.</div> <bR> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Mongolian Spots:</B>Again, don’t worry these are bluish, well-defined spots on the buttocks and trunk and these disappear by the by the 1<FONT SIZE="1" COLOR="#000000" FACE="Arial" >st</FONT> birthday.</div> <bR> <bR> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Factors that Influence the status of child Health:</B></div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">child abuse </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">drugs </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">alcohol </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">adolescent pregnancy </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">violence in school </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">accidents (MVA)</div> <bR> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Stork bites:</B></div> <div> Believed to be due to the mythological stork holding the baby from the nape of neck (for dropping them in the mother’s womb!), these are pinkish gray spots which may also be seen on the upper eyelids, forehead or nose.</div> <bR> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Peeling of the skin:</B></div> <div> It happens in a few babies and is nothing to be worried about. The thing to remember is that the skin underneath the peal is normal and healthy looking, if you find that the skin is raw, contact your doctor.</div> <bR> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Breast engorgement:</B></div> <div> Babies of both sexes may develop a slight engorgement of the breasts on the 3<FONT SIZE="1" COLOR="#000000" FACE="Arial" >rd</FONT> – 4<FONT SIZE="1" COLOR="#000000" FACE="Arial" >th</FONT> day. A creamish white discharge may also ooze from the nipples, this is also known as witch’s milk. No treatment is required but it is important though, not to press, massage or squeeze the breasts</div> <bR> <bR> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Common screening tools used for G&D:</B></div> <div> 1. Growth charts.</div> <div> 2. Denver development screening test: evaluates 4 aspects of child’s development between ages 2 months and 61/2 years. Test given to child before they enter school.</div> <div> A. gross motor skills</div> <div> B. fine motor skills</div> <div> C. language skills</div> <div> D. personal social skills</div> <bR> <bR> <bR> <div> <B>The most common way in which we study children is the </B><B><U>age stage approach</U></B><B>:</B></div> <div> 1. Prenatal: extends from conception to birth.</div> <div> 2. Infancy: birth to 12 months.</div> <div> A. Neonatal: birth to 1 month.</div> <div> B. Infancy: age 29 days to 12 months.</div> <div> 3. Early childhood.</div> <div> A. Toddler: 1 to 3.</div> <div> B. Preschooler: 3 to 5.</div> <div> 4. Middle childhood: 8 to 11, schoolage.</div> <div> 5. Adolescence: 12 to 18.</div> <bR> <div> <B>Maslow</B>: Basic human needs theorist.</div> <bR> <bR> <div> <B>Ericson's Psychosocial Theory:</B></div> <div> Emphasized the development of the individual's identity throughout the lifespan and in the context of parents, family, peers, neighbors and culture. He emphasized the process of socialization - when the child learns the rules of his culture and society.</div> <bR> <div> 1. <I>Trust vs. Mistrust: birth to 12 months.</I></div> <div> Totally dependent on everything. An individual that is getting their needs met on a constant basis is going to learn that the world is a safe place and a predictable place to live and learn to trust. Mistrust is the opposite. Must complete this stage before going to the next. In nursing, we should know to assign the same nurse to care for this infant so trust can be developed.</div> <bR> <div> 2. <I>Autonomy vs. Shame and doubt: 1 year to 3 years.</I></div> <div> Typical toddler. Everything revolves around them. Has learned that parents are going to look out for them and will want to assert their independence. Begins to gain control over personal actions and bodily functions. Will protest if don't get their way. Developmental task is to gain control over bowel and bladder. Conflict is shame and doubt. If not given a chance to explore then they develop a feeling they can't "do" things.</div> <bR> <div> 3. <I>Initiative vs. Guilt: 3 to 5 years.</I></div> <div> Preschool years. 3 year old wants to always please you. Also a time of exploring. Behavior is intrusive. Era of the "whys". Will try to do things that are too difficult and will do things that are unacceptable to people they wish to please. Curious about body parts.</div> <bR> <div> 4. <I>Industry vs. Inferiority: 6 to 12 years.</I></div> <div> Schoolage. Have to be given tasks they can accomplish and finish. Teachers will be very important to them. They will get a lot of self esteem (how you feel about yourself) and self concept (how you see yourself).</div> <bR> <div> 5. <I>Identity vs. Role confusion: 12 to 18 years.</I></div> <div> Adolescent. Searching for who they are. Turns to peers for acceptance. They learn that others have the same views as they do. Start off wanting to be a part of a group. Older adolescents desire to be different than the group.</div> <bR> <bR> <div> <B>Piaget's Theory of Cognitive Development:</B></div> <div> How the individual becomes familiar with the world and the objects in the world thru thinking.</div> <bR> <div> 1. <I>Sensorimotor activity: </I>birth to 2 years.</div> <div> Infants begin to know their world by reflexes such as sucking and grasping. Uses senses to explore. Start off with <U>relfex behavior</U>, then <U>repeatative behavior</U>, and finally <U>imatative behavior</U>. A lot of their learning is cause and effect. <FONT SIZE="3" COLOR="#800000" FACE="Arial" >New</FONT>ly learned knowledge can't be transferred from situation to situation. Ex: cannot distinguish from turning over box of toys vs. box of trash. Problem solving is trial and error. They are going to learn <B>object permanence</B> - even if they cannot see it, it still exists. Jack in the Box is a good toy to teach this and so is peek a boo.</div> <bR> <bR> <div> 2. <I>Pre-operational thought: </I>ages 2 to 7.</div> <bR> <div> A. Preconceptual: ages 2 to 4. </div> <div> See the world in egocentric terms. In terms of "me". Does <U>not</U> mean the child is self-centered. Unable to put themselves in the place of another. Cannot see things from another person's point of view. Conversation revolves around them. They expect you to know what or who they are talking about. Not able to share.</div> <div> B. Intuitive: ages 4 to 7.</div> <div> Egocentric thinking will begin to include other people and see things from another person's point of view. Give simple explanations to their questions. Characteristic of <U>centering</U>. Have the tendency to center their attention on one feature of something. Unable to see that it has other qualities. Ex: give them 4 pennies and will be happier than with a dime. Will begin to share.</div> <div> 3. <I>Concrete operations: </I>ages 7 to 11.</div> <div> Child's thought is logical. Learn how to classify, to sort, organize. Can problem solve. Age of collection. Learn the concept of conservation - realize that physical property like weight can remain the same even though outward appearances changes. Can't deal in abstraction. Solve problems in a systematic fashion. Are able to see other points of view.</div> <bR> <div> 4. <I>Format operations: </I>ages 11 to 15.</div> <div> Learn to reason logically. Are able to thing in abstract terms. Can draw logical conclusions. Adaptable and flexible. When making decisions, needs to look at pros and cons.</div> <bR> <bR> <div> <B>Kohlberg's Theory of Moral Development:</B></div> <div> 1. <I>Preconventional level: ages 0 to 7.</I></div> <div> A. Stage 0: first 2 years.</div> <div> No moral sensitivity. Decisions are made according to what feels good or what does not. Not aware of how behavior hurts others. Responds to pleasure with love and hurt with anger.</div> <div> B. Stage 1: 2 to 3 years.</div> <div> Determines right or wrong by physical consequence of an act.</div> <div> C. Stage 2: 4 to 7 years.</div> <div> View a specific act as right if it satisfies the need. Follow rules to benefit themselves. Attitude of eye for an eye.</div> <bR> <div> 2. <I>Conventional level: ages 7 to 12.</I></div> <div> Make decisions about right and wrong based on expectations of the family or society. Very loyal to their family. Seeks family approval and peer approval. Obey rules, respect authority.</div> <bR> <div> 3. <I>Postconventional level: ages 13 and up.</I></div> <div> Personal standards are defined by culturally accepted values. Rights must not be violated by the group. At top level - do what they thing is right without regard to what it will cost them.</div> <bR> <bR> <div> <B>Spiritual Development</B></div> <div> Infancy: no beliefs to guide behavior.</div> <div> Toddler: behavior is imitated and will imitate religious behaviors without knowing what they mean.</div> <div> Preschooler: will believe what parents believe.</div> <div> School age: strong interest in religion. Conscience begins to bother them when they disobey. Ability to articulate faith.</div> <div> Adolescent: may be exposed to spiritual disappointment. Compares what they believe to what others believe. A time of searching rather than reaching</div> <bR> <bR> <bR> <div> ===================================================================================================================</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">The Arnold-Chiari syndrome consists of </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">hydrocephalus, </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">spina bifida, </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">and meningomyelocele. </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">The hydrocephalus is a communicating one.</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> The fourth ventricle is elongated</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">, there is kinking of the brain stem,</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> and portions of the brain stem and cerebellum are displaced into the cervical spinal canal, causing obstruction of flow of cerebrospinal fluid (CSF). </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Skull films show a small posterior fossa and widened cervical canal (platybasia). </div> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Type I Chiari malformation is not associated with hydrocephalus. </B></div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">In communicating hydrocephalus, the most frequent cause is aqueductal stenosis or the Arnold-Chiari malformation associated with meningomyelocele.</div> <bR> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">A leptomeningeal cyst  is a rare and late complication of a linear skull fracture and appears as an expanding pulsatile mass on the surface of the skull.</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> Chronic subdural hematomas  are characterized by headache, personality change, and sudden loss of consciousness. </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Classically, patients with epidural injury experience a brief period of unconsciousness followed by a variable lucid interval. As the hematoma expands, the patient has progressive loss of consciousness, headache, vomiting, and focal neurologic signs.</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> Retinal hemorrhages would be seen in children who are victims of shaken baby syndrome </div> <bR> <div> =========================================================================================================================</div> <bR> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">A newborn infant is noted to be cyanotic at 24 hours of age. A work-up is done and the child is found to have tetralogy of Fallot. Which of the following characteristics primarily determines the prognosis in patients with tetralogy of Fallot</div> <div> <B>Degree of pulmonic stenosis</B></div> <bR> <bR> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Classic tetralogy of Fallot consists of </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">pulmonic stenosis, </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">overriding aorta, </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">right ventricular hypertrophy,</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> and ventricular septal defect. </div> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Fallot's tetralogy is the most common congenit</B><B>al</B><B> heart disease</B> with cyanosis,</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> although cyanosis may not be present at birth. </div> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Typical chest x-ray shows a boot-shaped heart (coeur en sabot) with diminished pulmonary vascular markings.</B> </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Patients are at higher risk for cerebral thromboses and brain abscess.</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> Treatment is surgical.</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> Prognosis depends on the degree of pulmonic stenosis, which determines the degrees of hypoxemia  and right ventricular hypertrophy </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Overriding aorta  and size of the ventricular septal defect  do not significantly relate to the prognosis</div> <bR> <div> <B>=======================================================================================================================</B></div> <bR> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f00000ff00.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">The most common malignancy in children is acute lymphocytic leukemia.</B><FONT SIZE="3" COLOR="#000000" FACE="Arial" > </FONT></div> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Leukemias in general are the most common form of cancer in children, accounting for about one third of all new cases.</B></div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> Brain tumors, the most common solid tumors, are second. </div> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Cerebellar astrocytomas  are the most common of the posterior fossa tumors.</B> </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Neuroblastomas  comprise the most common tumor of the central nervous system (CNS). </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Bone tumors have an incidence of 5.6/million in white children and 4.8/million in African-American <B>children; osteosarcoma is the most common.</B></div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> Wilms' tumor  accounts for almost all cases of renal cancer in children. </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Ewing's sarcoma  is a type of highly malignant, small round cell, undifferentiated tumor that arises in bone, but can also occur in soft tissues. It is more common in males than in females and rarely occurs in African-American children. It manifests itself most commonly in the 10 to 20 year age-group.</div> <bR> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">leukemia • neuroblastoma • brain cancers • lymphomas • wilms • germ cell tumors • retinoblastoma • signs of childhood cancer </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">The most common type of leukemia in children is acute lymphocytic leukemia or <B>ALL</B>, which is further characterized as pre-B, B, or T-cell ALL. </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Acute myeloid leukemia or <B>AML</B> is less common in children. </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> The chronic forms of these leukemias, <B>CLL</B> and <B>CML</B> respectively, occur less often in children than in adults. </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">The names are derived from the type of white blood cell that is proliferating.</div> <div> Clinical and laboratory features at diagnosis which are associated with outcome include the following: </div> <div> 1. Age at diagnosis: Age at diagnosis has strong prognostic significance, </div> <div> reflecting the different underlying biology of ALL in different age groups. </div> <bR> <div> <FONT SIZE="3" COLOR="#000000" FACE="Arial" >Infants with ALL have a particularly high risk of treatment failure, with the risk of treatment failure being greatest for young infants (< 6 months) compared to older infants (>/= 6-9 months).</FONT><U>5</U><FONT SIZE="1" COLOR="#000000" FACE="Arial" >-</FONT><U>8</U><FONT SIZE="3" COLOR="#000000" FACE="Arial" > Rearrangement of the MLL gene at chromosome band 11q23 can be detected in the leukemia cells of a large percentage of infants with ALL,</FONT><U>9</U><FONT SIZE="3" COLOR="#000000" FACE="Arial" > and the poor outcome for infants with ALL is strongly associated with the presence of the t(4;11) translocation involving the MLL gene.</FONT><U>10</U><FONT SIZE="1" COLOR="#000000" FACE="Arial" >,</FONT><U>11</U><FONT SIZE="3" COLOR="#000000" FACE="Arial" > ALL in infants is also associated with a constellation of other characteristics associated with poor outcome, including elevated white blood cell (WBC) count, central nervous system leukemia, lack of CD10 (cALLa antigen) expression, and poor response to initial treatment.</FONT><U>5</U><FONT SIZE="1" COLOR="#000000" FACE="Arial" >,</FONT><U>7</U><FONT SIZE="3" COLOR="#000000" FACE="Arial" > </FONT></div> <bR> <div> <FONT SIZE="3" COLOR="#000000" FACE="Arial" >Young children (1-9 years) have a favorable outcome in comparison to either older children and adolescents or in comparison to infants.</FONT><U>1</U><FONT SIZE="1" COLOR="#000000" FACE="Arial" >,</FONT><U>12</U><FONT SIZE="1" COLOR="#000000" FACE="Arial" >,</FONT><U>13</U><FONT SIZE="3" COLOR="#000000" FACE="Arial" > </FONT></div> <bR> <div> Older children and adolescents (>/= 10 years) have a less favorable outcome than young children, and more aggressive treatments are generally employed in order to improve outcome for these patients. </div> <bR> <div> 2. WBC count at diagnosis: Patients with higher WBC counts </div> <div> at diagnosis have a higher risk for treatment failure than do patients </div> <div> with lower WBC counts. A WBC count of 50,000/mm3 is generally used as </div> <div> an operational cut point between better and poorer prognosis,<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>1</U></FONT> </div> <div> although the relationship between WBC count and prognosis is a </div> <div> <FONT SIZE="3" COLOR="#000000" FACE="Arial" >continuous rather than a step function.</FONT><U>13</U><FONT SIZE="1" COLOR="#000000" FACE="Arial" >,</FONT><U>14</U><FONT SIZE="3" COLOR="#000000" FACE="Arial" > Elevated WBC count is </FONT></div> <div> associated with other high-risk prognostic factors, including </div> <div> unfavorable chromosomal translocations such as t(4;11) and t(9;22) </div> <div> (see below). </div> <bR> <div> 3. Gender: The prognosis for girls with ALL is slightly better than that for </div> <div> <FONT SIZE="3" COLOR="#000000" FACE="Arial" >boys with ALL.</FONT><U>15</U><FONT SIZE="1" COLOR="#000000" FACE="Arial" >-</FONT><U>17</U><FONT SIZE="3" COLOR="#000000" FACE="Arial" > One reason for the superior prognosis for </FONT></div> <div> girls is the occurrence of testicular relapses among boys, but boys </div> <div> also appear to be at increased risk for bone marrow relapse for </div> <div> reasons that are not well understood.<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>17</U></FONT> </div> <div> 4. Race: Survival rates for black children with ALL are somewhat lower than </div> <div> <FONT SIZE="3" COLOR="#000000" FACE="Arial" >those for white children with ALL.</FONT><U>12</U><FONT SIZE="1" COLOR="#000000" FACE="Arial" >,</FONT><U>18</U><FONT SIZE="1" COLOR="#000000" FACE="Arial" >-</FONT><U>20</U><FONT SIZE="3" COLOR="#000000" FACE="Arial" > The reason for the </FONT></div> <div> better outcome for white children compared to black children is not </div> <div> known, but it can not be completely explained based on known </div> <div> prognostic factors.<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>19</U></FONT> </div> <bR> <div> 5. Cellular Morphology: In the past ALL lymphoblasts were classified using </div> <div> the French-American-British (FAB) criteria as having L1 morphology, L2 </div> <div> morphology, or L3 morphology.<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>21</U></FONT> Due to the lack of independent </div> <div> prognostic significance and the subjective nature of this </div> <div> classification system, it is no longer used in the United States. The </div> <div> FAB L3 morphology is morphologically and cytogenetically identical to </div> <div> that of Burkitt's lymphoma. B-cell ALL (surface immunoglobulin (Ig) </div> <div> expression, generally with FAB L3 morphology and c-myc gene </div> <div> translocation) is a systemic manifestation of Burkitt's and </div> <div> Burkitt's-like non-Hodgkin's lymphoma, and its treatment is completely </div> <div> different from that for other forms of childhood ALL. (NOTE: Rare </div> <div> cases of FAB L3 ALL with c-myc gene translocations lack surface </div> <div> immunoglobulin expression, and these cases are appropriately treated </div> <div> as B-cell ALL).<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>22</U></FONT> Conversely, rare cases of ALL that express </div> <div> surface Ig but that lack L3 morphology and lack c-myc gene </div> <div> translocations are appropriately treated as B-precursor ALL rather </div> <div> than B-cell ALL.<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>23</U></FONT> (Refer to the PDQ summary on Childhood </div> <div> Non-Hodgkin's Lymphoma for more information.) </div> <div> Molecular and biological characteristics of leukemia cells at diagnosis which are associated with outcome include: </div> <div> 1. Immunophenotype: </div> <div> B-precursor ALL: B-cell precursor (or B-lineage) ALL, defined by the </div> <div> expression of CD19, HLA-DR, CD10 (cALLa), and other B-cell associated </div> <div> antigens, represents 80% to 85% of childhood ALL. Approximately 80% </div> <div> of B-precursor ALL express the cALLa, CD10 antigen. The lack of cALLa </div> <div> expression has also been shown in some series to be associated with a </div> <div> worse prognosis. For example, CD10 negativity is observed in a </div> <div> higher proportion of infants with B-precursor ALL and is associated </div> <div> with poor outcome.<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>5</U></FONT> </div> <bR> <div> Stage of B-cell maturation: There are 3 major subtypes of </div> <div> B-lineage ALL: early pre-B (no surface or cytoplasmic </div> <div> immunoglobulin), pre-B (presence of cytoplasmic immunoglobulin), and </div> <div> B-cell (presence of surface immunoglobulin). Approximately three </div> <div> quarters of patients with B-precursor ALL have the early pre-B </div> <div> phenotype and have the best prognosis. The leukemic cells of patients </div> <div> with pre-B ALL contain cytoplasmic immunoglobulin (cIg), an </div> <div> intermediate stage of B-cell differentiation. Twenty-five percent of </div> <div> patients with pre-B ALL have the t(1;19) translocation </div> <div> (see below).<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>24</U></FONT> Approximately 2% of patients present with B-cell ALL </div> <div> (surface Ig expression, generally with FAB L3 morphology and c-myc </div> <div> gene translocation).<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>25</U></FONT> B-cell ALL is a systemic manifestation of </div> <div> Burkitt's and Burkitt's-like non-Hodgkin's lymphoma, and its treatment </div> <div> is completely different from that for other forms of childhood ALL. </div> <div> (NOTE: Rare cases of FAB L3 ALL with c-myc gene translocations lack </div> <div> surface immunoglobulin expression, and these cases are appropriately </div> <div> treated as B-cell ALL).<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>22</U></FONT> Conversely, rare cases of ALL that </div> <div> express surface Ig but that lack L3 morphology and lack c-myc gene </div> <div> translocations are appropriately treated as B-precursor ALL rather </div> <div> than B-cell ALL.<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>23</U></FONT> (Refer to the PDQ summary on Childhood </div> <div> Non-Hodgkin's Lymphoma for more information on the treatment of </div> <div> children with B-cell ALL.) </div> <bR> <div> T-cell ALL: T-cell ALL is defined by the leukemic cell expression of </div> <div> the T-cell-associated antigens CD2, CD7, CD5, or CD3 and is </div> <div> frequently associated with a constellation of clinical features </div> <div> including male sex, older age, leukocytosis, and mediastinal mass.<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>2</U></FONT> </div> <div> Approximately 15% of children with newly diagnosed ALL have the </div> <div> T-cell phenotype. With appropriately intensive therapy, however, </div> <div> children with T-cell ALL have an outcome similar to that for children </div> <div> with B-precursor ALL, when matched for age and WBC count.<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>2</U></FONT> </div> <div> Cytogenetic abnormalities common in B-cell lineage ALL (e.g. </div> <div> hyperdiploidy) are uncommon in T-cell ALL and when present, are not </div> <div> associated with prognostic significance.<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>26</U></FONT> </div> <bR> <div> Myeloid antigen expression: A minority of childhood ALL cases have </div> <div> leukemia cells that express myeloid surface antigens. Myeloid </div> <div> antigen expression appears to be associated with specific ALL </div> <div> subgroups, notably those with MLL gene rearrangements and those with </div> <div> the TEL-AML1 gene rearrangement.<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>27</U></FONT> Early reports suggested a </div> <div> poorer prognosis for these patients,<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>28</U></FONT> but reports from </div> <div> large patient populations indicate no adverse prognostic </div> <div> <FONT SIZE="3" COLOR="#000000" FACE="Arial" >significance for myeloid surface antigen expression.</FONT><U>27</U><FONT SIZE="1" COLOR="#000000" FACE="Arial" >,</FONT><U>29</U><FONT SIZE="1" COLOR="#000000" FACE="Arial" >,</FONT><U>30</U><FONT SIZE="3" COLOR="#000000" FACE="Arial" > </FONT></div> <bR> <div> 2. Chromosome number: </div> <div> Hyperdiploidy: Hyperdiploidy (> 50 chromosomes per cell or DNA index > </div> <div> 1.16) is the presence of additional copies of whole chromosomes and </div> <div> occurs in 20% to 25% of cases of B-precursor ALL but very rarely in </div> <div> cases of T-cell ALL.<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>25</U></FONT> Hyperdiploidy can be evaluated by </div> <div> measuring the DNA content of cells (DNA index) or by karyotyping. </div> <div> Hyperdiploidy generally occurs in cases with favorable prognostic </div> <div> factors (age 1-9 years and low WBC count), and is itself associated </div> <div> <FONT SIZE="3" COLOR="#000000" FACE="Arial" >with favorable prognosis.</FONT><U>31</U><FONT SIZE="1" COLOR="#000000" FACE="Arial" >,</FONT><U>32</U><FONT SIZE="3" COLOR="#000000" FACE="Arial" > Hyperdiploid leukemia cells are </FONT></div> <div> particularly susceptible to undergoing apoptosis, which may explain </div> <div> the favorable outcome commonly observed for these cases.<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>33</U></FONT> </div> <bR> <div> Trisomies: For the treatment approaches utilized by both the </div> <div> Pediatric<FONT SIZE="3" COLOR="#000000" FACE="Arial" > </FONT>Oncology<FONT SIZE="3" COLOR="#000000" FACE="Arial" > Group (POG) and the Children's Cancer Group (CCG), </FONT></div> <div> extra copies of certain chromosomes appear to be specifically </div> <div> associated with favorable prognosis among hyperdiploid ALL cases. In </div> <div> POG studies, patients whose leukemia cells have extra copies of both </div> <div> chromosome 4 and chromosome 10 appear to have particularly favorable </div> <div> outcome.<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>34</U></FONT> In CCG studies, children with trisomies of chromosomes </div> <div> 10 and 17 have an excellent prognosis.<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>35</U></FONT> </div> <bR> <div> Hypodiploidy: Approximately 1% of children with ALL have leukemia </div> <div> cells showing hypodiploidy with less than 45 chromosomes. These </div> <div> <FONT SIZE="3" COLOR="#000000" FACE="Arial" >patients are at high risk for treatment failure.</FONT><U>36</U><FONT SIZE="1" COLOR="#000000" FACE="Arial" >,</FONT><U>37</U><FONT SIZE="3" COLOR="#000000" FACE="Arial" > </FONT></div> <div> 3. Recurring chromosomal translocations can be detected in a substantial </div> <div> number of cases of childhood ALL, and some of these translocations, as described below, have prognostic significance. </div> <bR> <div> TEL-AML1 (t(12;21) cryptic translocation): Fusion of the TEL (ETV6) </div> <div> gene on chromosome 12 to the AML1 (CBFA2) gene on chromosome 21 can be </div> <div> detected in 20% to 25% of cases of B-precursor ALL, but is rarely </div> <div> <FONT SIZE="3" COLOR="#000000" FACE="Arial" >observed in T-cell ALL.</FONT><U>25</U><FONT SIZE="1" COLOR="#000000" FACE="Arial" >,</FONT><U>38</U><FONT SIZE="3" COLOR="#000000" FACE="Arial" > Children with the t(12;21) cryptic </FONT></div> <div> translocation resulting in the TEL-AML1 fusion are generally 2 to 9 </div> <div> years of age.<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>38</U></FONT> Patients with the TEL-AML1 fusion have very good </div> <div> <FONT SIZE="3" COLOR="#000000" FACE="Arial" >outcomes,</FONT><U>38</U><FONT SIZE="1" COLOR="#000000" FACE="Arial" >-</FONT><U>40</U><FONT SIZE="3" COLOR="#000000" FACE="Arial" > although there is controversy about whether the </FONT></div> <div> ultimate cure rate is actually superior to that of other patients with </div> <div> B-precursor ALL or whether the ultimate cure rate is similar but the </div> <div> timing of relapse is significantly later for patients with the </div> <div> TEL-AML1 fusion compared to other patients with B-precursor </div> <div> <FONT SIZE="3" COLOR="#000000" FACE="Arial" >ALL.</FONT><U>39</U><FONT SIZE="1" COLOR="#000000" FACE="Arial" >,</FONT><U>41</U><FONT SIZE="1" COLOR="#000000" FACE="Arial" >-</FONT><U>43</U><FONT SIZE="3" COLOR="#000000" FACE="Arial" > </FONT></div> <bR> <div> The Philadelphia chromosome t(9;22) is present in approximately 4% of </div> <div> pediatric<FONT SIZE="3" COLOR="#000000" FACE="Arial" > ALL patients and confers an unfavorable prognosis, </FONT></div> <div> especially when it is associated with either a high WBC count or slow </div> <div> <FONT SIZE="3" COLOR="#000000" FACE="Arial" >early response to initial therapy.</FONT><U>44</U><FONT SIZE="1" COLOR="#000000" FACE="Arial" >-</FONT><U>46</U><FONT SIZE="3" COLOR="#000000" FACE="Arial" > Philadelphia-positive </FONT></div> <div> ALL is more common in older patients with B-precursor ALL and high WBC </div> <div> count. </div> <bR> <div> Translocations involving the MLL (11q23) gene occur in approximately </div> <div> 6% of childhood ALL cases, and are generally associated with increased </div> <div> risk for treatment failure.<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>25</U></FONT> The t(4;11) is the most common </div> <div> translocation involving the MLL gene in children with ALL and </div> <div> occurs in approximately 4% of cases.<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>47</U></FONT> Patients with t(4;11) </div> <div> generally present in infancy with high WBC count, and they are more </div> <div> likely than other children with ALL to have CNS disease and to have a </div> <div> poor response to initial therapy.<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>48</U></FONT> While both infants and adults </div> <div> with the t(4;11) are at high risk for treatment failure, children with </div> <div> the t(4;11) appear to have a better outcome than either infants or </div> <div> <FONT SIZE="3" COLOR="#000000" FACE="Arial" >adults.</FONT><U>48</U><FONT SIZE="1" COLOR="#000000" FACE="Arial" >-</FONT><U>50</U><FONT SIZE="3" COLOR="#000000" FACE="Arial" > Another translocation involving the MLL gene in </FONT></div> <div> children with ALL is the t(11;19), which occurs in approximately 1% of </div> <div> cases and which occurs in both B-precursor and T-cell ALL.<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>51</U></FONT> </div> <div> Outcome for infants with t(11;19) is poor, but outcome appears </div> <div> relatively favorable for children with T-cell ALL and the t(11;19) </div> <div> translocation.<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>51</U></FONT> </div> <bR> <div> The t(1;19) translocation occurs in 5% to 6% of childhood ALL, and </div> <div> involves fusion of the E2A gene on chromosome 19 to the PBX1 gene on </div> <div> <FONT SIZE="3" COLOR="#000000" FACE="Arial" >chromosome 1.</FONT><U>24</U><FONT SIZE="1" COLOR="#000000" FACE="Arial" >,</FONT><U>25</U><FONT SIZE="1" COLOR="#000000" FACE="Arial" >,</FONT><U>52</U><FONT SIZE="3" COLOR="#000000" FACE="Arial" > The t(1;19) may occur as either </FONT></div> <div> a balanced translocation or as an unbalanced translocation and is </div> <div> primarily associated with pre-B ALL (cytoplasmic immunoglobulin </div> <div> positive). Its presence was initially associated with inferior </div> <div> outcome in the context of antimetabolite based therapy.<FONT SIZE="1" COLOR="#0000FF" FACE="Arial" ><U>24</U></FONT> </div> <div> Studies have shown that the poorer prognosis associated with t(1;19) </div> <div> <FONT SIZE="3" COLOR="#000000" FACE="Arial" >can be largely overcome by more intensive therapy.</FONT><U>53</U><FONT SIZE="1" COLOR="#000000" FACE="Arial" >,</FONT><U>54</U><FONT SIZE="3" COLOR="#000000" FACE="Arial" > </FONT></div> <div> The improved outcome, however, appears to be primarily for patients </div> <div> with the unbalanced t(1;19) (approximately three fourths of all </div> <div> t(1;19) cases), with patients who have the balanced t(1;19) remaining </div> <div> <FONT SIZE="3" COLOR="#000000" FACE="Arial" >at increased risk for treatment failure.</FONT><U>53</U><FONT SIZE="1" COLOR="#000000" FACE="Arial" >,</FONT><U>55</U><FONT SIZE="3" COLOR="#000000" FACE="Arial" > </FONT></div> <bR> <bR> <bR> <bR> <bR> <bR> <div> =====================================================================================================================</div> <div> A 4-year-old child was playing in a field that had been sprayed with insecticides. The parents come to the emergency department carrying the child, who is lethargic, has excessive oral secretions, miosis, tearing, "soiled" trousers from urination and defecation, emesis, and fasciculations. The physician suspects organophosphate poisoning. To treat the nicotinic effects, the physician should use which of the following</div> <div> <B>Pralidoxime (2-PAM)</B></div> <bR> <bR> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">The specific antidote for nicotinic manifestations of organophosphate toxicity is pralidoxime (2-PAM). </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">It <B>restores the activity of acetylcholinesterase.</B></div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> Nicotinic effects for organophosphates include </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">fasciculations, </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">twitching, </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">weakness,</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> areflexia,</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> tachycardia, </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">and hypertension. </div> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Atropine is an antidote for the muscarinic effects of organophosphate toxicity. </B></div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Some muscarinic effects include </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">salivation, </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">lacrimation,</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> urination,</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> defecation, </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">and abdominal cramps.</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> British antilewisite (BAL)  and calcium disodium ethylene-diaminetetraacetate (Ca-EDTA)  are used for the treatment of lead toxicity. </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">These therapies are used in symptomatic patients, regardless of the lead levels, and in patients with a blood concentration of 70 <FONT SIZE="3" COLOR="#000000" FACE="Symbol" >m</FONT>g/dl or more.</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> Naloxone  is used as an antidote for opioid poisoning.</div> <bR> <div> <B>========================================================================================================================</B><FONT SIZE="3" COLOR="#000000" FACE="Arial" >=</FONT></div> <div> A 3-year-old child presents with convulsions followed by coma.</div> <div>  Cerebrospinal fluid (CSF) findings include a <B>total white blood cell (WBC) count of 250 cells/</B><FONT SIZE="3" COLOR="#000000" FACE="Symbol" ><B>m</B></FONT><B>l</B> with predominantly <B>lymphocytes and monocytes</B>, <B>glucose of 20 mg/dl, </B>and a<B> protein concentration of 80 mg/dl. </B></div> <div> A Gram's stain of a spun-down sediment is negative. </div> <div> A computed tomography (CT) scan with contrast shows enhancement at the base of the brain.</div> <div>  Which of the following is the most likely diagnosis</div> <bR> <div> <B>Tuberculosis meningitis</B></div> <bR> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Tuberculosis meningitis usually presents in recently infected individuals and is usually considered a disease of infants and children.</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> The symptoms develop slowly during three stages. </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Stage 1 is a prodromal stage with nonspecific symptomatology.</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> Seizures are common in stage 2; </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">coma occurs in stage 3. </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">A high index of suspicion is necessary for rapid diagnosis. A computed tomography (CT) scan of the head may show periventricular lucencies, edema, infarctions, and hydrocephalus.</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> Cerebrospinal fluid (CSF) findings show a slight elevation in white blood cells (WBCs),<B> usually 250 to 500</B>, p<B>redominantly lymphocytes</B>. </div> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">The CSF glucose is less than 40 mg/dl or less than half the serum glucose performed simultaneously.</B></div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> Protein concentration is normal or slightly high. Gram's stain is negative because the bacilli are acid-fast. CSF chloride is low.</div> <bR> <bR> <bR> <div>  </div> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Cryptococcal meningitis  is associated with</B></div> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> elevated protein, </B></div> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">hypoglycorrhachia,</B></div> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> and mononuclear pleocytosis (rarely > 300 cells/mm</B><FONT SIZE="1" COLOR="#000000" FACE="Arial" ><B>3</B></FONT><B>). </B></div> <div> <FONT SIZE="3" COLOR="#000000" FACE="Arial" ><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Children with subacute sclerosing panencephalitis  have CSF with normal cell content (may show some plasma cells), normal or slightly elevated protein, and</FONT><B><U> greatly increased </U></B><FONT SIZE="3" COLOR="#FF0000" FACE="Symbol" ><B><U>g</U></B></FONT><B><U>-globulin.</U></B></div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> Eastern equine encephalitis  occurs more commonly in <B>young infants</B>, causing serious complications and fatalities.</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> The spectrum and definitive diagnosis of meningococcal meningitis  are made by the isolation of the organism from the CSF.</div> <div> <B>====================================================================================================================</B></div> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Vitamin D-resistant rickets (familial, X-linked hypophosphatemia) is the most common form of rickets in the United States. </B></div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">It is <B>X-linked dominant</B>, so some mothers may have clinical manifestations of the disease. </div> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Defects in the proximal renal tubula</B>r reabsorption of phosphate account for the hypophosphatemia. </div> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Bowing of the legs is a common presentation.</B></div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> In addition, these children may have a <B>waddling gait and short stature</B>. </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Calcium levels in blood are normal.</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> Treatment consists of phosphate supplementation.</div> <div> <B><IMG BORDER="0" SRC="Symb075c00b700f099000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> Tetany , myopathy , rachitic rosary , and Harrison's groove (pectus deformity)  are present in calcium-deficient rickets.</B><FONT SIZE="3" COLOR="#000000" FACE="Arial" > </FONT></div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Vitamin D-deficiency rickets has become rare in developed countries due to food supplementation and styles that bare much of the body to the sun.</div> <div> <IMG SRC="0f526ab0.jpg" border=0 width="550" height="171" ALIGN="BOTTOM" HSPACE="0" VSPACE="0"></div> <div> ========================================================================================================================</div> <bR> </td> </tr></table></body>