MORNING REPORT

Brittany Esty

History of Present Illness

10 year old male with history of Autism Spectrum Disorder, sensory integration dysfunction, presenting with right leg pain Patient started limping 3 days prior to admission 2 days prior to admission limping progressed, taken to PCMC ED and XR of right ankle noted osteopenia but no cause for pain. The following day, he was complaining what seemed more like right knee pain per mother. He was taken to PCP. Xray at PCP office of the hip did not reveal any acute concerns. He went back home The next morning, patient refused to bear weight that prompted mother to bring in for repeat evaluation

Review of systems

20-pound loss September-August. Severely Restricted diet and eating smaller portions at mealtime. Patient refused to eat on the day of admission Rash that began in his upper arms (September) and progressed to involve forearms and thighs (April) to now (August) spreading to legs, back, chest, neck and cheeks Significant bruises and "wakes up every morning with a new bruise" per mom Chronic diarrhea Mood changes, more irritable No mental status changes, fever, night sweats, dysphagia, nausea, emesis, dyspnea, cough, or change in urinary or bowel habits Otherwise ROS negative

PHI
PAST MEDICAL HISTORY: Autism (diagnosed before he turned 2) Sensory Integration Dysfunction, oral aversion Developmental Delay, language Chronic diarrhea Chronic Sinusitis Hospitalized for dehydration, rotavirus, cellulitis PAST SURGICAL HISTORY: Dental procedures: tooth extraction (2-3 months ago), routine cleanings, fillings

PHI continued
MEDICATIONS: Vitamin D drops, does not tolerate (refusing meds by mouth) Sertraline 10mg daily, but not taking as refusing meds by mouth ALLERGIES: no known allergies FAMILY HISTORY: Mom denies family history of eczema, psoriasis or mental health problems. SOCIAL HISTORY: Lives with mom, stepdad and younger brother. Has a dog at home. Mom reports many stressors in the last 5 years (changing homes, changing schools and teachers, grandparents divorcing and then remarrying).

PHI continued
DIET: Mostly consists of Cheetos, grilled cheese, chocolate chip, cheeseballs, chocolate pudding, chicken nuggets, Cheez-Its and Sprite. Per mom, he never eats fruits or vegetables and she has been unable to give him a multivitamin. Mom reports decrease quantity of food intake starting around April, as well as an even more restricted diet only Cheez-Its and Sprite recently.

PHYSICAL EXAM
VITALS: Wt: 35.6kg (64%ile, was 96%ile in 2011), Height 137cm (31%ile, was 90%ile in 2011), BP: 113/75, HR:96, RR:16, T: 37.6 GENERAL: Patient laying in bed, awake, minimal verbal response, no acute distress, thin appearing but not obviously cachectic. HEAD: Normocephalic and atraumatic, hair slightly dry, not obviously brittle EYES: Pupils round, reactive to light, no conjunctivitis, mucous or discharge. EOM intact. EARS: No pits or tags. Could not examine TM due to patient sensitivity NOSE: No mucous crusts or blood OROPHARYNX: Mild hypertrophy and bleeding in lower and upper gums. No erythema, swelling or lesions. Tongue looks slightly atrophied with small papillae NECK: No anterior/posterior lymphadenopathy, full ROM CARDIOVASCULAR: Normal rate and rhythm. No murmurs or gallop. Strong peripheral pulses, Capillary refill <2 sec LUNGS: Clear bilateral posterior lung fields, no retractions

PHYSICAL EXAM
ABDOMEN: Soft, non-tended, non-distended. No pain to palpation. Bowel sounds present. No HSM. EXTREMITIES: Warm, well perfused. No cyanosis, clubbing or edema, nails not brittle, big toe nails with slight koilonchyia. Generally decreased muscle mass. Pain to palpation over right right distal femur/right knee but no surrounding swelling or areas of trauma. Patient with knee in flexed position and refusal to extend knee joint. No tenderness to palpation over the right ankle. Unable to assess hip range of motion but no tenderness to palpation over hip joint. No joint effusions. GENITOURINARY: Descended testicles. No pubic hair. Circumcised. SKIN: Multiple ecchymosis in bilateral shins and knees at various stages of healing. Multiple non-blanching red and purple, hyperkeratotic papullar lesions around hair follicles over anterolateral legs thigh, lateral arms, back, chest, neck and cheeks. Normal turgor. NEURO: no distress, normal gross motor movements, normal fine motor movements. responding to questions appropriately although not very verbal. Gait: Bears minimal weight on the right leg. Knee remained in flexed position and allows leg to briefly touch without weight. No ataxia. Unable to illicit patellar reflex on right, but right Achilles reflex normal. Sensation intact bilaterally.

Case Summary
10 year old male with history of Autism Spectrum Disorder, sensory integration dysfunction, with right leg pain (with progressive decrease in ambulation), rash, weight loss, and gingival hyperplasia

Differential diagnosis
ID

ENDO: Infection Osteomyelitis Septic arthritis Myositis Lyme disease Chronic recurrent multifocal osteomyelitis
Toxic synovitis

Hyperparathyroidism Hematologic diseases Sickle cell disease , Hemoglobinopathy Bone tumor

HEME

ONCO

Chondroma or sarcoma Osteoblastoma

RHEUM:

Systemic lupus erythematosus

Juvenile rheumatoid arthritis Spondyloarthropathy

Ewings or other sarcoma

Leukemia Lymphoma GuillainBarre syndrome Heavy-metal

LeggCalvePerthes disease

NEURO:

Connective-tissue disorders Dermatomyositis HenochSchonlein purpura Kawasakis disease Polyarteritis nodosa Inflammatory bowel disease

OTHER:

Truama Vitamin A intoxication Vitamin deficiency Rickets Scurvy Abuse

GI

Ddx of Gingival Swelling

Phenytoin exposure Pyogenic granuloma Aphthous ulcers Infectious gingivitis Crohns disease Behcets disease Dental abscess Scurvy

Labs

CRP 2.0 ESR 13 CBC: WBC 4.4 (39N, 48L, 8M, NC 1700), Hgb 11, Hct 33.3, MCV 76, MCH 27, MCHC 35, RDW 13.5, PLTS 177 CMP: Alk phos 85, otherwise normal PT:13.7, INR:1, PTT:31, vWF:norm Zn: 61 (low normal) Vit D: 16 (low) Immunoglobulins: IgM 40, all others normal Lymphocyte subset panes normal Plasma AA and Urine OA normal TTG 3, total IgA107 T4 3.09 Fe: 57, TIBC: 254, transferrin: 22%

Labs

Vitamin C: <0.1 (undetectable)

Vitamin C Deficiency: Scurvy

Scurvy caused by deficiency in Vitamin C (ascorbic acid) that is require for the synthesis of collagen in humans Reversible reducing agent that is an essential cofactor for the hydroxylation of proline to hydroxyproline in collagen synthesis and for the hydroxylation of the neurotransmitter dopamine to noradrenaline. Does not occur in most animals because they can synthesize their own vitamin C. BUT, humans and other higher primates (monkeys, apes), guinea pigs, bats, birds, and fish lack L gulonolactone oxidase enzyme needed for synthesis of Vit C. Must be obtained through diet.

Scurvy symptoms

Many signs and symptoms of vitamin C deficiency relate to its essential role in collagen synthesis
Dermatologic symptoms: petechia, ecchymoses, corkscrew hairs, hyperkeratosis, and perifollicular hemorrhages Bone disease: typically subperiosteal bleeding

Neurologic symptoms: depression and vasomotor instability Systemic symptoms: Malaise and fatigue Oral symptoms: Gingival swelling and hemorrhage

Scurvy symptoms

Early symptoms: malasie and lethargy. After 1-3 months, bone pain, skin changes with roughness, easy bruising, petechiae, gum disease, poor wound healing and emotional changes

Skeletal changes in Scurvy

Most severe in young children (like our patient) as their skeletal tissue is still growing, and the periosteum is not as tightly bound to the surface of the cortex as it is in adults Deficient collagen in subperiosteal blood vessels leads to rupture and hemorrhage mechanically lifts the periosteum from the underlying cortex Reparative osteoblasts deposit reactive bone that can be seen on plain radiography. Similar findings occur in the metaphysis at the base of the growth plate reduced collagen production results in decreased bone deposition structural weakness hemorrhage and fractures with minimal stress With treatment these changes of scurvy will resolve

Body supply can be depleted in 1-3 months Risk factors:

smoking

and medications (aspirin, sulfinpyrazone, indomethacin, phenylbutazone, tetracycline, oral contraceptives, corticosteroids, chlorcyclizine) increase the body's need for vitamin C intestinal malabsorption syndromes, pregnancy and lactation, and depressed socioeconomic or refugee status.

Rash in Scurvy

Follicular hyperkeratosis (corkscrew hair)

Vitamin C Deficiency: Scurvy

ASD and Food Selectivity

Is it real? Why does it happen? Does it matter? What do we do?

Study out of the UK (Cornish) examined the diets of 17 children with autism spectrum disorders (ages 3 to 10 years)

10 of 17 children (59%) ate fewer than 20 different foods

Klein and Nowak study via survey to parents of 43 children and adolescents with ASD (ages 4 to 26 years) assessing dental treatment, oral hygiene behavior, and nutrition, including food preferences and eating patterns

found that 53% of the participants were reported to be reluctant to try new foods.

Factors influencing food sensitivity

Survey study done by Williams looked at 100 parents of children with ASD (ages 22 months to 10 years)

67% of the parents reported that their child was a picky eater BUT nearly three quarters (73%) reported that their child had a good appetite for foods that they liked The factors parents felt influenced food selectivity were:

texture (69%) appearance (58%) taste (45%) smell (36%) temperature (22%)

Texture makes all the difference

Schmitt and colleagues surveyed parents of 20 boys with ASD and 18 typically developing boys (ages 7 to 10 years) to complete a questionnaire on eating behaviors and food preferences and a 3-day food record.
Boys with autism spectrum disorders ate a smaller variety of foods than controls more often made their food choices based on texture than did the boys in the control group. Seventy percent of children with autism chose their food based on texture, compared to 11% of children without autism.

Does it matter?

Restricted food intake can lead to nutritional insufficiency making food selectivity a potential health risk. Multiple studies have been done to assess nutrient intake of children with ASD, but they have produced conflicting results. Some studies indicate that the nutrient intakes of children with autism are below that of non ASD children while others do not demonstrate any differences in nutritional intake

+ inadequate nutrient intake

Cornish study (3 day dietary recall and food frequency check list of 17 children with ASD revealed inadequate nutrient intake

There was an inverse relationship between variety and nutritional adequacy; as the daily variety decreased, the number of nutrient intakes that fell below the recommended amount increased. Intake of protein, vitamin A, thiamin, vitamin B-12, folic acid, sodium, potassium, magnesium, phosphorous, and copper were determined to be adequate for all children. Inadequate intakes of iron, vitamin D, vitamin C, niacin, riboflavin, and zinc were found in one or more children.

Summary

Feeding problems in ASD are complex and multifactorial Food selectivity is a major problem in children with ASD One of the consistent themes in the food selectivity literature relates to food textures There is high variability within this population and we must consider and treat each individual patient

Treatment

Close follow up and appropriate interdisciplinary approach.

Nutritionist/registered

dietitian (RD) Occupational therapist Behavioral psychologist

Consider use of food records or 24-hour diet recalls Laboratory screening in those at risk for nutritional deficiencies

References

Duggan C. A 9-Year-Old Boy with Bone Pain, Rash, and Gingival Hypertrophy. N engl j med 357;4: 392-400. Cermak S et al. Food Selectivity and Sensory Sensitivity in Children with Austism Spectrum Disorders. J Am Diet Assoc. 2010; 110:238-246 Cornish E. A balanced approach towards healthy eating in autism. J Hum Nutr Diet. 1998;11:501509. Schmitt L, Heiss CJ, Campbell EE. A comparison of nutrient intake and eating behaviors of boys with and without autism. Top Clin Nutr. 2008;23:23-31. Williams PG, Dalrymple N, Neal J. Eating habits of children with autism. Pediatr Nurs. 2000;26:259-264. Klein U, Nowak AJ. Characteristics of patients with autistic disorder (AD) presenting for dental treatment: A survey and chart review. Spec Care Dentist. 1999;19:200-207. Schreck KA, Williams K. Food preferences and factors influencing food selectivity for children with autism spectrum disorders. Res Dev Disabil. 2006;27:353-363. Field D, Garland M, Williams K. Correlates of specific childhood feeding problems. J Paediatr Child Health. 2003;39:299-304. Levy SE, Souders MC, Ittenbach RF, Giarelli E, Mulberg AE, Pinto- Martin JA. Relationship of dietary intake to gastrointestinal symp- toms in children with autistic spectrum disorders. Biol Psychiatry. 2007;61:492-497.