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PEDIATRIC DEVELOPMENTAL AND BEHAVIORAL DISORDERS EO 018.13 REFERENCES • • • • • • C290- Nelson’s Essentials of Pediatrics C277 - Toronto Notes C 306 Toronto sick Kids Manual Class handout C20 Merck Manual- current edition C291 Primary Care for the PA OUTLINE • • • • • • • • Failure To Thrive (FTT) Fetal Alcohol Syndrome ( FAS) Attention Deficit hyperactivity Disorder(ADHD) Enuresis Encopresis Autism ( briefly) Learning Disorders ( briefly) Dyslexia FAILURE TO THRIVE (FTT) • Definition: - Weight <3rd percentile, or falls below two major percentile curves, or <80% of expected weight for height and age - 50% organic, 50% non-organic - Inadequate caloric intake most important factor in poor weight gain FAILURE TO THRIVE (FTT) • Normal energy requirements - Wt: 0-10 kg: 100 cal/kg/day - Wt: 10-20 kg: 1,000 cal + 50 cal/kg/day for each kg >10 - Wt: 20 kg+: 1,500 cal + 20 cal/dg/day for each kg >20 - May have other nutritional deficiencies, e.g. protein, iron, vitamin D as well NON-ORGANIC (FTT) • Presents by 6-12 months • Often due to malnutrition • These children are often picky, poor eaters with poor emotional support at home • Psychomotor, language and personal/social development delays possible CAUSES NON- ORGANIC (FTT) • • • • • • • Inadequate intake Inappropriate feeding practices Emotional deprivation Poor parent-child interaction Dysfunctional home situation Parental psychosocial stress Child abuse and/or neglect must be considered CAUSES OF ORGANIC FTT • Insufficient breast milk production • CNS, neuromuscular, mechanical problems with swallowing, sucking • Anorexia (associated with chronic disease) • Inadequate absorption- malabsorption: - Celiac disease - Cystic fibrosis (CF) - Pancreatic insufficiency CAUSES OF ORGANIC FTT • Inappropriate utilization of nutrients - Renal loss: e.g. tubular disorders - Losses from the GI tract: chronic diarrhea, vomiting - Inborn errors of metabolism - Endocrine disorders: type 1 diabetes, diabetes insipidus (DI), hypopituitarism CAUSES OF ORGANIC FTT • Increased energy requirements - Pulmonary disease: CF Cardiac disease Endocrine: hyperthyroidism, DI, hypopituitarism Malignancies Chronic infections Inflammatory: systemic lupus erythematosus (SLE) CAUSES OF ORGANIC FTT • Decreased growth potential - Specific congenital syndromes - Chromosomal abnormalities - Intrauterine insults e.g fetal alcohol syndrome (FAS) FAILURE TO THRIVE PATTERNS • Growth Parameters - Decreased Wt. Normal Ht. • Possible problem - Caloric insufficiency Decreased intake Hypermetabolic state Increased losses Normal HC FAILURE TO THRIVE PATTERNS • Growth Parameters - Decreased Wt. Decreased Ht. • Possible problem - Structural dystrophies Endocrine disorder Constitutional growth delay Genetic short stature Normal HC FAILURE TO THRIVE PATTERNS • Growth Parameters - Decreased Wt. Decreased Ht. Dec. HC • Possible problem - Intrauterine insult - Genetic abnormality FAILURE TO THRIVE (FTT) • History - Duration of problem - Detailed dietary and feeding history, appetite, behaviour during feeds - Pregnancy, birth and postpartum history; - Developmental and medical history, including medications; - Social and family history (parental height and weight) - Assess child's temperament, child-parent interaction, feeding behaviour and parental psychosocial stressors FAILURE TO THRIVE (FTT) • Physical Examination - Height (Ht), weight (Wt), head circumference (HC), arm span, upper/ lower body segment ratio - Assessment of nutritional status - Dysmorphic features - Evidence of chronic disease - Observation of a feeding session and parent-child interaction - Any signs of abuse or neglect FAILURE TO THRIVE (FTT) • Laboratory investigations: as indicated by clinical presentation - CBC, blood smear, electrolytes, urea, ESR, T4, TSH, urinalysis - Bone age x-ray - Genetic karyotype in all short girls and in short boys where appropriate - Any other tests indicated from history and physical exam: e.g. renal or liver function tests, venous blood gases, ferritin, immunoglobulins, sweat chloride, fecal fat FAILURE TO THRIVE (FTT) • Treatment: Non-organic FTT - Most are managed as outpatients with multidisciplinary approach - Short hospitalization to monitor food/feeding to see if child gains weight - Primary care physician, dietitian, psychologist, social work, child protection services • Treatment: Non-organic FTT - Cause specific FETAL ALCOHOL SYNDROME (FAS): • Medical diagnosis referring to a set of alcoholrelated disabilities associated with maternal use of alcohol during pregnancy • Recognized in Canada as one of the leading causes of preventable birth defects and developmental delay in children FETAL ALCOHOL EFFECTS (FAE): • Birth defects or developmental abnormalities for which alcohol is being considered one of the possible causes • Used to describe children with prenatal exposure to alcohol, but only some of the characteristics of FAS, including reduced or delayed growth, single birth defects, or developmental learning and behavioral disorders that may not be noticed until months or years after the child's birth FETAL ALCOHOL SYNDROME (FAS): • High-risk Populations - Women who drink and have the following characteristics: Low socioeconomic status Poverty Lack of education Smoker Use of other illicit drugs Poor health FETAL ALCOHOL SYNDROME (FAS): • Diagnostic Criteria For FAS: - History of maternal alcohol consumption during pregnancy - Prenatal or postnatal growth retardation - Involvement of CNS, such as: neurologic abnormalities developmental delay behavioral dysfunction learning disabilities or other intellectual impairments, skull and brain malformations FETAL ALCOHOL SYNDROME (FAS • Diagnostic Criteria For FAS: • Characteristic facial features: - Short eye slits (palpebral fissures) - Thin upper lip - Flattened cheek bones - Indistinct groove between the upper lip and the nose FETAL ALCOHOL SYNDROME (FAS • Prevention Strategies - Screen all pregnant women about alcohol use - Advise stopping all use in pregnancy - Healthcare professionals working with members and leaders of communities must be consistent in advising women and their partners that the prudent choice is not to drink alcohol during pregnancy FETAL ALCOHOL SYNDROME (FAS • Tertiary Prevention - Strategies should include early diagnosis of the condition and programs designed specifically for children with FAS or FAE and their parents or caregivers - Refer women who are at high risk to appropriate treatment resources for alcohol abuse - Identify and treat women and their partners who already have one FAS/FAE child and who plan to have more children ATTENTION DEFICIT HYPERACTIVE DISORDER(ADHD) • A cluster of behavioral symptoms: - Poor attention span - Impulsiveness - Hyperactivity • Not all children with the disorder will exhibit all three behaviors CAUSES ATTENTION DEFICIT HYPERACTIVE DISORDER(ADHD) • Genetic Syndromes - Fragile X syndrome - Phenylketonuria (PKU) - Gilles de la Tourette syndrome • Intrauterine or prenatal /postnatal damage - Fetal alcohol exposure Intrauterine anoxia Prematurity Meningitis Significant head injuries ATTENTION DEFICIT HYPERACTIVE DISORDER(ADHD) • History - Prenatal: pregnancy, exposure to drugs or alcohol Perinatal: delivery, asphyxia, illnesses Family history: ADHD, related behavioral disorders Past medical history: illnesses such as meningitis, injuries, hospital admissions - History of school progress and behavior (talk with teacher) - Symptoms usually present before child enters school ATTENTION DEFICIT HYPERACTIVE DISORDER(ADHD) • Physical Examination - Complete general examination: look for dysmorphic features of genetic conditions, FAS - Examine ears and check hearing - Examine eyes and check vision - "Soft neurologic signs" often present (e.g., increased reflexes, poor coordination, poor balance) - Educational evaluation done through the school system ATTENTION DEFICIT HYPERACTIVE DISORDER(ADHD) • Appropriate management includes the involvement of a multidisciplinary team, of which educational specialists are the mainstay • Many specific methods can be used to overcome the child's weaknesses and take advantage of his or her strengths • The medical role involves advocacy and sometimes the administration of medication ATTENTION DEFICIT HYPERACTIVE DISORDER(ADHD) • Counsel parents or caregiver about behavioral strategies: - Decrease environmental stimuli Focus on the child's positive traits to increase self-esteem Give simple directions Make eye contact with the child Use "time out" as a prime disciplinary tactic ATTENTION DEFICIT HYPERACTIVE DISORDER(ADHD) • Pharmacologic Interventions- Drug of choice: • Methylphenidate (Ritalin) daily in two doses, morning and noon • Dextroamphetamine another approved drug for use ENCOPRESIS • Fecal incontinence in a child at least 4 years old • Prevalence: 1-1.5% of school-aged children (rare in adolescence) • M:F = 6:1 • Must exclude medical causes (e.g. Hirschsprung disease, hypothyroidism, hypercalcemia, spinal cord lesions, anorectal malformations) RETENTIVE ENCOPRESIS • Psychogenic Megacolon • Causes - Physical: anal fissure (painful stooling) - Emotional: disturbed parent-child relationship, coercive toilet training RETENTIVE ENCOPRESIS • History: - Child withholds bowel movement - Develops constipation, leading to fecal impaction and seepage of soft or liquid stool - Crosses legs or stands on toes to resist urge to defecate - Psychologically distressed by symptoms, soiling of clothes - Toilet training hx: coercive or lackadaisical RETENTIVE ENCOPRESIS • Physical exam: - Abdominal exam Urinary - urinalysis Rectal exam: large fecal mass in rectal vault Anal fissures (result from passage of hard stools Abdominal x-ray RETENTIVE ENCOPRESIS • Treatment - Diet modification fiber, roughage Enemas and suppositories completely clean-out bowel Stool softeners (e.g. Senokot, Lansoyl at bedtime) Toileting schedule Positive reinforcement CASE HISTORY • Three year-old John presents with his mother to the office today because he "Just won't talk me, and won't play with his brother." - With further questioning, she states that he does not engage in any verbal play, dress-up play, or appropriate play with toys - The only words he says are words he hears on the television, or he repeats words back that he has heard. - He does not come to her for hugs and kisses - This has been going on for about a year, but she thought he would just "grow out of it". CASE HISTORY • Upon interacting with John, he will not make eye contact, and will not respond to questions posed to him • His right hand "flaps" in an intermittent pattern • In observing John, he has taken one stuffed animal from the toy area, and is repeatedly hitting it against the floor • When this behavior is redirected, John dropped to the floor, crying AUTISM • Autism, as defined in the DSM-IV (APA, 1994, p. 66-71) is a Pervasive Developmental Disorder (PDD) • The DSM-IV notes three areas from which diagnosis must be made, social interaction, communication, and behavior/motor activity. Additionally, cognitive impairments are commonly seen • The diagnostic criteria from the DSM-IV are outlined specifically AUTISM • An overall prevalence rate of approximately 5/10,000 • Autism is four times as likely to strike a male as a female • There is strong evidence that autism has a genetic component • Intrauterine insult or birth insult cause is seen in many cases • Brain defects have long been considered as a culprit in autism AUTISM • Symptoms may manifest in early infancy, with the infant shying away from the parents touch • Not responding to a parent who returns after an absence • No eye contact • The child may fail to meet early language and other developmental milestones • This is the time when most parents begin to become aware that there is something "different AUTISM • There is no "cure" for autism • However, as autism occurs on a spectrum from mild to severe, there are varying degrees of functioning, which may be reached as an adult • This ranges from holding a job to requiring complete 24-hour care • Intense therapy before age 6 can have significant effects on overall outcomes LEARNING DISABILITY • Inability to process language and its symbols or lack of arithmetic-related skills at a level equal to peer group • Affected children usually suffer from learning disability in a specific area and are normal in all other areas of development LEARNING DISABILITY • CAUSES - Specific learning disabilities are generally thought to be biologic in origin, although the exact mechanisms and biology have not yet been determined - Major psychiatric disturbances, social deprivation, or loss of vision or hearing can also produce poor learning skills and must be differentiated from specific disabilities LEARNING DISABILITY • History - Current and past behavior and school performance - Look for specific patterns and for hyperactivity, which is often associated with a learning disability - Perinatal history (perinatal asphyxia or intrauterine injury may play a role in some cases), prematurity - Family history (such disorders often run in families) LEARNING DISABILITY • History - Early development: recognition of risk factors such as delayed language development - Social, environmental, family and social factors, which may aggravate the problem (e.g., constant derision may lead to low self-esteem) - History of meningitis, head trauma LEARNING DISABILITY • Perform a physical examination to rule out the following conditions: - Hearing and vision problems Medical problems Fetal alcohol syndrome (FAS) Abuse Iron deficiency anemia Neurologic abnormality LEARNING DISABILITY • Refer the child to a Pediatrician for evaluation as soon as possible (elective) • Management of this problem should be done through the education system - Advocate for the child in the education system - Support the child's self-esteem - Support child and parents or caregiver with behavioral strategies in conjunction with psychological counseling and education DYSLEXIA • Dyslexia is often referred to as a 'specific learning difficulty' • Usually with symptoms such as difficulty with writing and spelling, and sometimes with reading and working with numbers. • A dyslexic person may have problems putting things in order, following instructions, and may confuse left and right DYSLEXIA • In the school situation, a dyslexic child may find he or she is experiencing failure, but is not able to understand why • This frequently results in low self-esteem and a severe loss of confidence, which can lead to the child being reluctant to go to school • The first step is for an accurate diagnosis to be made. This usually undertaken by the school educational psychologist DYSLEXIA • The causes of dyslexia are not fully understood, but it is thought to be inherited as it usually runs in families • There are well-developed courses of learning which can be used to bring child up to the average level for their age in the areas they find difficulty with • Given the proper help, in most cases a dyslexic child can succeed at school at a level roughly equal to his or her classmates