Ghai Essential Pediatrics8th

While drawing anyconclusionsaboutdevelopment, one should remember the wide variations in normality. For example, let us consider the milestone of standing alone. The average agefor attainment of this milestone in a WHO survey was 10.8 months (Fig. 3.42). However, the 3rd and 97thcentilesfor normalchildrenwere 7.7 and15.2months, respectively. The same is true for many other milestones as is shown in Fig. 3.42. The bars illustrate the age range for normal children toattain thatparticularmilestone.This range of normalcy should always be kept in mind while assessing development.

Retardation should not be diagnosed or suggested on a singlefeature. Repeatexaminationis desirable in any child who does not have a gross delay. Factors such as recent illness, significant malnutrition, emotional deprivation, slow maturation, sensory deficits and neuromuscular disorders should always be taken into account.

One should keep in mind the opportunities provided to the child to achieve that milestone. For example, a child who has not been allowed to move around on the ground sufficiently by the apprehensive parents may have delay in gross motor skills.

At times, there can be significant variations in attainment of milestones in individual fields, this is called dissociation. For example, a 1-yr-old child who speaks 2-3 words with meaning andhas finger thumb opposition (10-12 months), may not be able to stand with support (less than 10 months). Such children require evaluation for physical disorder affecting a particular domain of development. A child having normal development in all domains except language may have hearing deficit.

Table 3.5 gives the upper limits by which a milestone mustbe attained. Achild whodoes notattainthe milestone

o_e_v_e_io_p_m_e_n_t

Table 3.5: Upper limit of age for attainment of milestone

Loss of comprehension, single words or phrases at any age

Adapted from WHO; MGRS group, WHO motor development study. Acta Pediatrics 2006;450:86-95

by the recommended limit should be evaluated for cause of developmental delay.

Thepredictivevalueofdifferentdomains ofdevelopment for subsequent intelligence is not the same. Fine motor, personal-social andlinguisticmilestonespredictintelligence far better than grossmotor skills. Inparticular, an advanced language predicts high intelligence in a child.

Development Screening Tests

Screening is a brief assessment procedure designed to identify children who should receive more intensive diag­ nosis or assessment. Such an assessment aids early intervention services, making a positive impact on development, behavior and subsequent school perfor­ mance. It also provides an opportunity for early identi­ fication of comorbid developmental disabilities. Ideally,

all children should be periodically screened but short of this, at least those with perinatal risk factors should be screened.

Developmental SuNeil/ance

Child development is a dynamic process and difficult to quantitate by one time assessment. During surveillance repeated observations on development are made by a skilled physician over time to see the rate and pattern of development. Periodic screeninghelpsto detect emerging disabilities as the child grows. However, using clinical judgment alone has a potential for bias and it has been suggested to use periodic screening tools for ongoing developmental surveillance. The physicianshouldchoose a standardized developmental screening tool that is practical and easy to use in office setting. Once skilled with thetool,it canbe used asscreeningmethodto identify at risk children. Screening tests popular in the west include Parents' Evaluations of Development Status (PEDS) and Ages and Stages Questionnaires (ASQ). Some of the common screening tools used in India are described below.

Phatak's Baroda screening test This is India's best knowndevelopmenttestingsystemthatwas developed by Dr Promila Phatak. It is meant to be used by child psychologists rather than physicians. It is the Indian adaptation of Bayley's development scale and is applied tochildrenup to 30 months. It requiresseveraltestingtools and objects that are arranged according to age. The kit is available commercially.

Denver development screening test The revised Denver development screening test (DOST) or Denver II assesses child development in four domains, i.e. gross motor, fine motor adaptive, language and personal social behavior, which are presented as age norms, just like physical growth curves.

Trivandrum development screening chart This simplified adaptation of the Baroda development screen­ ing system is applicable to children up to 2 yr of age. It consists of 17 items selected from Bayley Scale of infant development (BSID) and Baroda tests. It is a simple test that can be administered in 5 min by a health worker, and

is useful as a mass screening test.

Clinical adaptive test and clinical linguistic and auditory milestone scale (CAT/CLAMS) This easy to

learn scale can be used to assess the child's cognitive and language skills. It uses parental report and direct testing of the child's skills.It is usedat ages of 0-36months and takes 10-20 min to apply. It is useful in discriminating children withmentalretardation(i.e.bothlanguageandvisualmotor delay) and those with communication disorders (low

language scores).

Goodenough-Harrisdrawingtest Thissimplenonverbal intelligence test requires only a pencil or pen and white unlinedpaper. Here the child is asked to draw a man in the best possible manner and points are given for each detail that the child draws. One can determine the mental age by comparing scores obtained with normative sample. This test allows a quick but rough estimate of a child's intelli­ gence, and is useful as a group screening tool.

Definitive Tests

These tests are required once screening tests or clinical assessment is abnormal. They are primarily aimed to accurately define the impairments in both degree and sphere. For example, by giving scores for verbal, perfor­ mance abilities and personal and social skills, these can be differentially quantified. Some of the common scales used are detailed in Table 3.6.

Early Stimulation

Infantswho show suspected orearly signs of development delay need to be provided opportunities that promote

body control, acquisition of motor skills, language development and psychosocial maturity. These inputs, termed early stimulation, include measures such as making additional efforts to make the child sit or walk, givingtoys to manipulate,playingwith thechild, showing objects, speaking to the child and encouraging him to speak andpromptingthe child to interact withothers, etc.

There is a general lack of evidence for effectiveness of these early interventions in improving neurodevelop­ mentaloutcome and motor abilities. However, studies in premature babies, cerebral palsy, institutionalized child­ ren and other children at high risk for adverse neuro­ developmental outcomes suggest that these interventions are effective if started early. Compliance to interventions is important for favorable results on neurodevelopment. Systematic reviews suggest that the effect of these interventions is sustained in later childhood. For example, play and reading were effective in earlychildhood in low­ and middle-income countries, and kangaroo mother care was effective for low birth weight babies in resource poor settings.

Promoting Development by Effective Parenting

Comprehensive care to children requires focus on preventive efforts including child-rearing information to parents. Parenting has an immense impact on emotional, social and cognitive development and also plays a role in the later occurrence of mental illness, educational failure and criminal behavior. Creating the right conditions for early childhood development is likely to be more effective and less costly than addressing problems at a later age.

Television Viewing and Development

Television viewing in younger children has been shown to retard language development. It is a passive mode of entertainment and impairs children's ability to learn and read, and also limits creativity. Children can pick up inappropriate language and habitsby watching TVshows andcommercials. Violence andsexualityon televisioncan have alastingimpact on the child's mind. Parents need to regulate both the quantity and quality of TV viewing, limiting the time to 1-2 hr per day and ensuring that the content they see is useful.

Some Useful Internet Resources

http://www.nlm.nih.gov/medlineplus/child development. html http://kidshealth.org/parent/growth/ http://www.nichd.nih.gov/ http://www.med.umich.edu/yourchild/ http://www.bridges4kids.org/disabilities/SU.html http://www.zerotothree.org/

Suggested Reading

Developmental surveillance and screening of infants and young chil­ dren. American Academy of Pediatrics, Committee on Children with Disabilities. Pediatrics 2001;108:192---6

Engle PL, Black MM, Behrman JR, et al. Strategies to avoid the loss of developmental potential in more than 200 million children in the de­ veloping world. Lancet 2007;369:229-42

Grantham-McGregor S, Cheung Y, Cueto S, et al. Developmental potential in the first 5 yr for children in developing countries. Lancet 2007;369:60-70

BEHAVIORAL DISORDERS

Anorexia Nervosa

This eating disorder is characterized by: (i) body weight <85% of expected weight for age and height; (ii) intense fear of becoming fat even though underweight; (iii) disturbed body image and denial that the current body weight is low; and, (iv) in postmenarcheal girls, amenor­ rhea. It is most common among 15-19-yr-old.

Two clinicalsubtypesare recognized. Somepatientslose weight through excessive dietary restrictions and increased physical activity (restricting type), while others resort to vomiting and the use of laxatives or diuretics. Anorexia is commonly associated with depression, anxiety, suicidal ideation and/or obsessive compulsive disorder. Profoundweightloss mayresult in hypothermia, hypotension, dependent edema, bradycardia and metabolic changes. Hypokalemic metabolic alkalosis may occur due to vomiting and use of diuretics or purgatives. Mortality is attributed to cachexia and suicide.

Psychotherapy, including individual and family the­ rapy, and in some cases, group therapy, are required to establish appropriate eating patterns and restore normal perceptions of hunger and satiety. A nurturing emotional environment is essential. Severely undernourished patients require nutritional rehabilitation targeting normal weight for height. While oral supervised feeding is preferred, some patients require nasogastric or parenteral nutrition. Antidepressant and antipsychotic drugs are prescribed as required.

Bulimia

Bulimia nervosa is characterized by (i) recurrent episodes of binge eating characterized by eating in a discrete period of time an amount of food that is definitely more than what normalindividualseat during a similar time period, without control over eating during the episode; and (ii) recurrent inappropriate compensatorybehaviorto prevent weight gain, such as self-induced vomiting, misuse of laxatives, diuretics, enemas, fasting, or excessive exercise. Binge eating and inappropriate compensatory behavior both occur, on average, at least twice a week for 3 months. There is undue influence of body shape and weight on self-evaluation. The disorder ismorecommonamonggirls between 10-19 yr of age. Many affected patients have comorbidities like depression and other psychoses. Management includes a combination of psychotherapy (specifically, cognitivebehaviortherapy)and anti-depres­ sant medications (such as fluoxetine). Active followup

needs to be maintained to ensure motivation and adhe­ rence to therapy.

Pica

Pica is the persistent ingestion of non-nutritive substances such as plaster, charcoal, paint and earth for at least 1 month in a manner that is inappropriate for the develop­ mental level, is not part of a culturally sanctioned practice and is sufficiently severe to warrant independent clinical attention. It is a common problem in children less than 5 yr of age. Factors speculated to predispose to pica include mental retardation, psychosocial stress (maternal deprivation, parental neglect and abuse) and other beha­ vioral disorders. Poor socioeconomic status, malnutrition and iron deficiency are commonly associated with pica but their etiologic significance has not been established. Children with pica are at an increased risk for lead poison­ ing, iron deficiency anemia and parasitic infestations. Management comprises behavior modification, alleviating the psychosocial stress if any, and iron supplementation if deficiency is present.

Food Fussiness

Food fussiness is a common problem in young children. It often reflects an excessive need for control on the part of the parents about what the child eats. Management involves examining the child for any nutritional deficiencies and counseling the parents regarding the nor­ mal growth pattern and dietary requirements of children. Useful behavioral strategies include establishing regular meal timings, ensuring a pleasant atmosphere, offering a variety of foods and setting an example of enjoying the same food themselves. Offering small servings at a time, reducing between meal caloric intake, not force feeding the child, presenting the food in an interesting manner and praise for good eating behavior are other helpful strategies. Parents should resist the temptation of offering sugary or fatty snacks as substitute or reward for eating healthy food.

Difficulties with Toilet Training

Refusal to defecate in the toilet with development of constipation is a common problem in children and is a cause for parental frustration and increased stress for the child. The most common setting is a power struggle between the child and parents ensuing from toilet training that is begun before the child is developmentally ready to be trained. Toilet training should be started after 2 yr of age, when the child has spontaneously started indicating bladder and bowel fullness, and is able to follow simple instructions. The general ambience should be conducive to learning and free from pressure. Use of a toddler-size seat that can be placed on top of the regular toilet seat helps the child feel more secure and not afraid of falling in. Consistency in the parents' approach and positive reinforcement help in achieving the normal pattern.

Temper Tantrums

Temper tantrums include behaviors that occur when the child responds to physical or emotional challenges by drawing attention to himself and can include yelling, biting, crying, kicking, pushing, throwing objects, hitting and head banging. Tantrums typically begin at 18-36 months of age. Inability to assert autonomy or perform a complex task on his/her own causes frustration to the child which cannot be effectively communicated due to limited verbal skills. The frustration therefore is acted out as undesired behaviors. Such behavior peaks during second and third year of life and gradually subsides by the age of 3-6 yr as the child learns to control his negativism.

Parents should be asked to list situations where disruptive behavior are likely to occur and plan strategies to avoid these. For example, they should ensure that the child is rested and fed, and should carry a snack for the child when going for an outing. During a tantrum, the parents' behavior should be calm, firm and consistent and they should not permit the child to take advantage from suchbehavior. The child should be protected from injuring himself or others. At an early stage, distracting his attention from the immediate cause and changing the environment can abort the tantrum. A 'time out', i.e. asking the child to stay alone in a safe and quiet place for a few minutes, is useful.

Breath Holding Spells

Breath holding spells are reflexive events typically initiated by a provocative event that causes anger, frustration or pain causing the child to cry. The crying stops at full expiration and the child becomes apneic and cyanotic or pale. In some cases the child may lose consciousness, become hypotonic and fall. If the spell lasts for more than a few seconds, brief tonic-clonic seizure may occur. Breath­ holding spells always revert on their own within several seconds, with the child resuming normal activity or falling asleep for some time. Breath holding spells are rare before 6 months of age, peak at 2 yr and abate by 5 yr of age.

Diagnosis is based on the setting and the typical seq­ uence of crying, cyanosis or pallor with or without brief loss of consciousness. The differential diagnoses include seizures, cardiac arrhythmias or brainstem malformation. The history of provoking event, stereotyped pattern of events and presence of color change preceding the loss of consciousness help in distinguishing breath holding spells from seizures. In case the spells are associated with pallor, an electrocardiogram may be done to rule out cardiac arrhythmias and long QT syndrome.

After a thorough examination of the child, the parents should be reassured. They are explained that the apneic spells are always self-limited and do not lead to brain injury or death. The family should be advised to be consistent in their behavior with the child, remaining calm

during the event, avoid picking the child up (since this decreases blood flow to the brain) and to turn him to the side so that secretions can drain. As the child recovers, they should avoid exhibiting undue concern nor give in to his demands if the spell was provoked by anger or frustration. Children with iron deficiency should receive iron supplementation.

HABIT DISORDERS AND TICS

Habit disorders include repetitive pattern of movements such as head banging, rocking of body, thumb sucking, twisting of hair and grinding of teeth. Such movements are seen frequently in normally developing children between 6 months to 2 yr and are benign and generally self limited. These movements seem to serve as a means of discharging tension in the children or providing extra self-nurturance.Asthesechildrenbecome older, theylearn to inhibit some of their rhythmic habit patterns, partic­ ularly in social situations. Undue attention from parents and forcing the child to give up the behavior often leads to reinforcement of such behavior and their persistence for a longer period.

Nail biting is a common stress-relieving habit. It includes biting the cuticle and soft tissue surrounding the nail as well as the nails. It is the most common among 'nervous' habits that include thumb-sucking, nose-picking, hair­ twisting. Although seen most commonly in school-age children, it is frequent at all ages. Nail biting increases the risk for infections around the nail beds and for infections transmitted by feco-oral route. Soreness of fingertips may cause occasional bleeding from cuticles. Nail biting may interfere with normal nail growth and lead to deformed nails.

If nail biting is occasional and transient, occurring only in situations of stress (e.g. while learning something new, attending a party with many strangers), it should be ignoredas this is a comforting mechanism. If nail biting is more persistent, parents hould try to identify the source of persistent stress (e.g. bullying at school) and help the child in resolving it. Measures like wearing gloves or adhesive bandages and conscious substitution of nail­ biting by other activities (e.g. squeezing a rubberball) are helpful. The nails should be trimmed regularly and the childshould be offered positive reinforcement or rewards for allowing the nails to grow.

Thumb sucking is normal behavior in infants and toddlers. It peaks between the ages of 18-21 months and mostchildrenspontaneouslydrop the habit by 4 yr of age. Its persistence in older children is socially unacceptable and can lead to dental malalignment. Parents should be reassured and asked to ignore the habit if the child is younger than 4 yr of age. If it persists beyond the age of 4-5 yr, the parents shouldmotivate the child to stop thumb sucking and encourage him when he restrains himself

from sucking the thumb. Application of noxious agents over the thumb is useful as an adjunctive second-line treatment.

Tics are involuntary and purposeless movements or utterancesthatare sudden, spasmodicandrepetitive.They usuallyinvolve themusclesof eyes, mouth, face andneck, and can range from blinking of eyes, facial twitching, shrugging or throat clearing toextreme forms like obscene gestures and vocalization (coprolalia). The disorder is seen in 1-2% children, particularly in school-aged boys.

Attention Deficit Hyperactivity Disorder

Attention deficit hyperactivity disorder (ADHD) is the most common neurobehavioral disorder in children, estimated to affect 3-5% of school-aged children, parti­ cularly boys. It is characterized by difficulty in paying attention, difficulty in controlling behavior and hyper­ activity.

Etiology

For most children, no etiology is identified. Both genetic and environmental factors play a role. Studies have identified abnormalities in dopamine transporter and thyroid receptor beta genes in some patients.

Clinical Features

Examples of inattentive, hyperactive and impulsive behavior included within the criteria for diagnosis of ADHD are listed in Table 3.7. For making the diagnosis, the behavior must begin before 7 yr of age, be present for

Table 3.7: Examples of inattentive and hyperactive/ impulsive behavior included within the criteria for diagnosis of ADHD

Inattentive behavior

l.Early distraction by extraneous stimuli

2.Often makes careless mistakes in schoolwork or other activities

3.Often has difficulty sustaining attention in tasks or play

4.Often forgetful in daily activities

5.Does not seem to listen to what is being said to him

6.Often fails to finish schoolwork or other chores

7.Daydreams, becomes easily confused, and move slowly

8.Difficulty in processing information as quickly and accurately as others

Hyperactive behavior

l.Runs about or climbs excessively in situations where it is inappropriate

2.Fidgets with hands and feet and squirms in seat

3.Talks nonstop

4.Has trouble sitting still during dinner, school, and story time

5.Has difficulty doing quiet tasks or activities.

Impulsive behavior

1.Has difficulty awaiting turn in games or group situations

2.Blurts out answers to questions

3.Often interrupts conversations or others' activities

at least 6 months, be pervasive (present in at least 2 different settings) and impair the child's ability to func­ tion normally. The symptoms should not be secondary to another disorder. Three subtypes are known:

Predominantly hyperactive-impulsive. Most symptoms (6 or more) are in the hyperactivity-impulsivity categories, and less than 6 symptoms of inattention are present.

Predominantly inattentive. The majority of symptoms (6 or more) are in the inattention category and less than 6 symptoms of hyperactivity-impulsivity are present, although hyperactivity-impulsivity may still be present to some degree. Parentsor teachersmaynotreadily recognize these children as having a problem.

Combined hyperactive-impulsive and inattentive. These children have six or more symptoms each of inattention and hyperactivity-impulsivity. Most children have the combined type of ADHD.

Diagnosis is primarily clinical, using thorough clinical interview of parents and use of behavior rating scales. Physical examination includes direct observation of the child and ruling out chronic systemic illnesses that affect child's attention span. Neuropsychological evaluation using standard tests of general intelligence and edu­ cational achievement help to exclude learning disorders or mental retardation.

Management

The management of ADHD should begin with educating the parents about ADHD and helping them in setting realistic goals of treatment. The treatment involves a combination of behavioral therapy and medications.

Usefulbehavioralstrategiesinclude: (i) clearandexplicit instructions to the child about desirable and nondesirable behavior; (ii) positive reinforcement of desirable behavior by praise or small tangible rewards; (iii) punishment strategies like verbal reprimand, nonverbal gestures or 'time out' for undesirable behaviour; and (iv) extinction technique, i.e. systematic ignoring of undesirable beha­ vior; and (v) providing a well-structured and organized routine for the child at home as well as school. At school, giving brief and consistent instructions to the child, clear and consistent response to the child's behavior, seating in an area with few distractions, and allowing the child to changeactivities and move about periodicallyare helpful.

Stimulants, e.g. methylphenidate, amphetamine and their derivatives, areeffectivein ameliorating inattention, hyperactivity and impulsivity in 70-80% children. How­ ever, academic achievement or social skills do not improve. Adverse effectsaremildandinclude abdominal discomfort, loss of appetite, headache and sleep distur­ bances. Atomoxetine, a selective norepinephrine reuptake inhibitor, and extended-release preparations of selective a.-adrenergic agonists (e.g. guanfacine, clonidine) have also demonstrated efficacy in reducing core symptoms.

Learning Disabilities

Learning disabilities arise from specific neurodevelop­ mentaldysfunctionsthatprevent expectedlearning in one or more academic areas. The important defining principle is that such disabilities are unexpected when considering the overall intellectual functioning of the child. These disordersarenottheresult of global developmental delay, majorvisionor hearinghandicaporconsequencesofmajor social or emotional stress. Dyslexia constitutes 80% of all cases. Othersaredysgraphia(difficultyinwriting),reading comprehensiondifficulty(inabilityto comprehendwhatis read) and dyscalculia (difficulty in performing mathe­ matical operations).

Dyslexia It is a receptive language-based learning dis­ ability that is characterized by difficulties with decoding, fluent word recognition, and/or reading comprehension skills. Word decoding is the ability to apply principles of phoneticstosoundthe words,i.e.understandingthat each letter or lettercombination inthe word has asound and by combining these, the word can be read and spelled. Secon­ dary consequences include reduced reading experience that can impede growthof vocabulary, writtenexpression and background knowledge. Dyslexic children read very slowlyandmakemanymistakesinreading.Theyalsohave difficulty in spelling because of underlying problem with word decoding. The difficulty in reading impairs their ability to cope up withthe academic syllabus and is often associated with low self-confidence and feeling of frustra­ tion, which increases the risk of developing psychological and emotional problems. Listening comprehension is typically normal in the affected children.

Dyslexia may co-exist with ADHD in 15-40% of children. Genetic factors are recognized to play a strong role in the etiology of dyslexia. Up to 50% of children of a dyslexic parent and50% of siblingsof adyslexicchildhave dyslexia.

Diagnosis of dyslexia is clinical, based on presence of unexpected difficulties in reading at the level of phono­ logic processing of words. Standardized tests are used to test speed, accuracy and comprehension in reading and spelling ability, in relation to the age and school grade.

In younger children, the focus of management is on remediation. Affected children are best taught in small groups by teachers trained in theprincipleof phonics. The children are taught how letters are linked to sounds. The stressis onimprovingphonemic awareness, i.e.the ability to focus on and manipulate phonemes (speech sounds) in spoken syllables and words. Usually these programs improve the reading accuracy significantly and fluency to a lesser extent. If the child also has ADHD, this should be managed with pharmacotherapy. For older children, the management stresses more on accommodation rather than remediation, e.g. use of laptops with spell-check, recorded books, giving extra time for writing tests or use of multiple choice questions.

Stuttering

Stuttering is a defect in speech characterized by hesitation or spasmodic repetition of some syllables with pauses. There is difficulty in pronouncing the initial consonants caused by spasm of lingual and palatal muscles. It is a common problem affecting up to 5% of children between 2-5 yr of age, a period in which there is non-fluency of speech.Environmentalandemotional stress or excitement may exacerbate stuttering.

Parents of a young child with primary stuttering should be reassured that stuttering between the age of 2-5 yr usually resolves on its own. Making the child conscious of his stutter or pressurizing him to repeat the word without stuttering will further increase the stress and the stutter. Children who continue to have significant stuttering require referral to a speech therapist. In older children with late onset of stuttering, the help of a child psychologist should be sought.

Suggested Reading

Subcommittee on Attention-Deficit/Hyperactivity Disorder, Steer­ ing Committee on Quality Improvement and Management. ADHD: Clinical Practice Guideline for the Diagnosis,EvaluationandTreatment of Attention-Deficit/Hyperactivity Disorder in Children and Adoles­ cents. Pediatrics 2011:128; 1007

Snowling MJ, Hulme C. Interventions for children's language and literacy difficulties. Int J Lang Commun Disord 2012;47:27-34

Autistic Disorder

Autistic disorder is classified asone of the pervasive deve­ lopmental disorders, also called as autistic spectrum disorders,aclusterofsyndromesthatsharemarkedabnor­ malities in the development of social and communicative skills (Table 3.8). Data from developed countries suggest that 1 in 150 children is affected by such disorders.

Children with autistic disorders (AD) show severe and pervasive impairmentsinreciprocal socialinteraction and communicationand exhibit stereotypedbehaviors, as well as restricted interests and activities. To meet full criteria for diagnosis, a child must demonstrate the following symptoms: (i) qualitative impairmentin socialinteraction as manifested by two of the following: impairment in the use of multiple nonverbal behaviors (e.g. eye gaze, facial expression, body postures); failure to develop peer relationships; lack of sharing of enjoyment; and lack of social or emotional reciprocity; (ii) qualitative impairment in communication in at least one of the following areas: delayor total lack ofspoken language; markedimpairment in the ability to initiate or sustain a conversation with others; stereotyped or repetitive use of language; and lack

Table 3.8: Pervasive developmental disorders

Autistic disorder

Rett syndrome

Asperger syndrome

Childhood disintegrative disorder

of varied spontaneous play; (iii) restricted repetitive and stereotyped patterns of behaviors, interests, and activities as manifested by preoccupation with one or more restric­ ted patterns of interests; inflexible adherence to nonfunc­ tional routines or rituals; repetitive motor mannerisms (such as rocking, hand flapping, finger flicking); and pre­ occupation with parts of objects. Intelligence is variable,

although most children fall in the functionally retarded I category by conventional psychological testing. Some

children show an isolated remarkable talent.

Diagnosis of AD is clinical, guided by the application of diagnostic tools, such as Autism Diagnostic Observational Schedule and the Autism Diagnostic Interview-Revised. Testing for associated neurological disorders such as tuberous sclerosis and fragile X is recommended. Conditions that should be differentiated from AD include mental retardation, deafness, selective mutism and ADHD.

Treatment. The primarymanagementisthrough 'intensive behavioral therapy', starting before 3 yr of age, applied at home as well as school and focusing on speech and languagedevelopmentandgoodbehavioralcontrol. Older children andadolescentswithrelativelyhigherintelligence but poor social skills and psychiatric symptoms (e.g. depression, anxietyandobsessive-compulsivesymptoms) may require psychotherapy and pharmacotherapy.

Prognosis. Factors associated with better prognosis are early diagnosis, intensive behavioral therapy, higher intelligence level and presence of functional speech. Children with better prognostic factors may grow up to be self-sufficient and employed, though socially isolated. On the other hand, those with poor prognosis remain dependent on family or require placement in facilities outside home.

Munchausen by Proxy

Munchausen syndrome by proxy is a disorder in which a caregiver, usually mother deliberately makes up a history of illness in her child and/or harms the child to create illness. The name is derived from the adult 'Munchausen syndrome' in which a person self-induces or acts out illness to gain medicalattention.In Munchausen byproxy, the abusing caregiver gains attention from the relation­ ships formed withhealthcareproviders, or her ownfamily as a result of the problems created.

Most commonly, the victims are infants and young preverbal children. The child's symptoms, their pattern or response to treatment may not conform to any recognizable disease and always occurs when mother is with the child. Apnea, seizures (which may be induced by suffocating the child or injecting insulin), fever, diarrhea and skin conditions arethe common symptoms. Confirmation of diagnosis needs careful history and reviewing of pastandcurrent hospital records.Monitoring

by hidcien teievis10n cameras in the ward may be useful. Once the diagnosis is made, the offending caregiver should be confronted, separated from the child and provided psychotherapy.

Suggested Reading

Martinez-Pedraza F, Carter AS. Autism spectrum disorders in young children. child Adolesc Psychiatr Cl.in N Arn 2009;18:645-63

Mitchell I, Brummet J, De Forest J. Apnea and factitious illness (Munchausen syndrome) by proxy. Pediatrics 1993;92:810-5

Disruptive Behavior Disorders

This term encompasses a broad range of behaviors that bring children into conflict with their environment.

Oppositional Defiant Disorder

Oppositional defiantdisorderis a repetitive andpersistent pattern of opposition, defiant, disobedient and disruptive behaviors towards authority figures persisting for at least 6 months. Examples of such behaviors are: (i) persistent stubbornness and refusal to comply with instructions or unwillingness to compromise with adults or peers;

(ii)deliberate and persistent testing of the limits;

(iii)failing to accept responsibility and blaming othersfor one's mistakes; (iv) deliberately annoying others; and

(v)frequently losing temper. Although the disorder does not include the more aggressive aspects of conduct disorder, many children go on to be later diagnosed with conduct disorders.

Oppositional defiant disorder is thought to result from interplay of factors in the child's characteristics, parental interactions and environmental factors. Serious conflict between the parents and family history of mental health problems such as depression, ADHD or antisocial perso­ nality disorder is often present.

The management should focus on alleviating known risk factors or stresses that might be contributing to the development of oppositional behavior. Interventions are directed towards enhancing parents' skills in conflict resolution and communication and the child's skills in effective communication and anger management. Use of stimulant medication is effective in patients with ADHD.

Conduct Disorder

Conduct disorder is characterized by aggressive and destructive activities that cause disruptions in the child's natural environments such as home, school, or the neigh­ borhood. The overriding feature is the repetitive and persistent pattern ofbehaviorsthat violate societal norms and the rights of other people, for a period of at least one year. Prevalence is estimated at 9% for boys and 2% for girls.

The specific behaviors necessary to make a diagnosis of conduct disorder are: (i) aggressive conduct that causes or threatens physical harm to other people or animals; (ii) non-aggressive behavior that causes property loss or damage; (iii) deceitfulness or theft; and (iv) serious violations of rules. The diagnosis of conduct disorder is made if three or more of the above behaviors are present, with at least one having taken place in the previous six months.

Variouschildbehavior managementtechniques,such as positive reinforcement to increase desirablebehavior, and extinction and time out to decrease problem behavior, are taught to the parents. The children are taught anger­ coping, peer coping and problem-solving skills, so that theyareabletodealbetterwithproblematic interpersonal situations. They are trained to not misjudge others' intent as hostile to avoid precipitating aggressive behavior.

Juvenile Delinquency

Children who show oppositional defiant behavior or conduct disorder and come into conflict with the juvenile justice systembecause ofsuchbehavior are called juvenile delinquents. The term refers to a person under 18 yr who is brought to the attention of the juvenile justice system for committing a criminal act or displaying a variety of other behaviors not allowed under the law, such as, truancy, use of alcohol or illicit drugs.

Family and parenting interventions have been shown toreducetherate ofre-incarcerationandcriminal behaviour byjuvenile delinquents. In some cases, placement infoster care is recommended with similar interventions being administered by the foster family.

Adolescence is a stage of transition from childhood to adulthood. During this stage of life, a youth undergoes rapid changes in body structure, mediated by the sex hormones. The appearance of sexualcharacters is coupled with changes in cognition and psychology. Whereas adolescence refers to this entire process, puberty refers to the physical aspect. The age group 10-19 yr is considered as the period of adolescence, and puberty marks the early half of adolescence. Though it is a continuous process, for convenience sake, adolescence is generally divided into three phases: early (10-13 yr), mid (14-16 yr) and late (17-

19 yr) puberty.

PHYSICAL ASPECTS

The activation of the hypothalamo-pituitary-gonadal axis leads to the production of gonadotropins, luteinizing hormone (LH), follicle stimulating hormone (FSH), sex steroids, estrogen and testosterone. Gonadal sex steroids bring about secondary sexual characters (breast develop­ ment, increase in penile and testicular size andmenarche), whereas adrenal androgens cause development of sexual hair, acne and underarm odor. The details of hormonal mechanisms of onset andprogression of puberty are dealt with in Chapter 17.

Onset and Sequence of Puberty

Pubertyin girls starts with breast development (thelarche) any time between 8 and 13 yr (Fig. 4.1). This is followed by appearance of pubic hair (pubarche) and subsequently menstruation (menarche), occurring at an average of

12.6 yr (range 10-16 yr). However, many experts believe that the normal age of menarche is advancing to as early as 9 yrin manypopulations.Menarcheusuallyoccursafter

2-21h yr of thelarche. The breast buds may be tender and there may be asymmetry in the breast size during early phases of puberty.

In boys, the earliest change is increase in testicular size (testicular volume reaching 4 ml or length 2.5 cm) and

Tushar R. Godbole, Vijayalakshmi Bhatia

this occurs between 9 and 14 yr (Fig. 4.2). This is followed by appearance of pubic hair and lengthening of the penis.

Spermarcheor theproductionof spermsstarts duringmid adolescence. Laryngeal growth, manifesting as cracking of voice, begins in boys in rnidpuberty under androgenic stimulus; deepening of voice is complete by the end of puberty. Mild degree of breast enlargement is normally seen in more than half of boys in early puberty which subsides spontaneously over several months. The onset of puberty is highly variable in both sexes.

Physical Growth and Nutritional Requirements

During puberty, boys gain about 20-30 cm andgirls about 16-28 cm. Peak growth velocity in girls occurs before attainment of menarche (stage 3) in girls whereas boys have their peak growth velocity during later stages of puberty (stages 4-5). The growth spurt affects the distal skeleton first, hence enlargement of limb and extremities is followed by increase in trunk size.

During pubertal development there is increase in muscle mass and bone diameter, particularly inboys, and total bone mass in both the sexes. Lean body mass increases duringtheearlystages in both the sexes;fat mass increases in girls at later stages of puberty. Rapid calcium accretionoccursduringpuberty.Almost50% of adult bone mass is achieved during the adolescent period. Estrogen and androgen enhance calcium accretion by bone but favorearlyfusion of epiphyses. Increase in bodystructure isparalleledby increase inbloodvolumeandmusclemass.

With commencement of menstruation, nutritional requirements of iron are higher.

COGNITIVE AND SOCIAL DEVELOPMENT

Volumetricandfunctionalimagingtechniquesshowthatthe adolescent brain undergoes subtle structural changes and differential growth. Though the exact implications of these changes are largely unknown, these probably indicate the

Prepubertal

Reddening of scrotum, testicular volume reaching 4 ml or length 2.5 cm Scanty hair at penile base

Fig. 4.2:

Increase in length of penile shaft

Further increase in testicular volume

Hair begin to curl and darken

Increase in girth of penis and glans Darkening of scrotum

Coarse, abundant and curly hair, less than in adult

Adult size scrotum and penis

Adult type hair, spreading over to medial thighs

Sexual maturity rating (1-5) in boys