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7/27/2019 Scurvy and Autism Spectrum Disorder
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MORNING REPORT
Brittany Esty
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History of Present Illness
10 year old male with history of Autism SpectrumDisorder, sensory integration dysfunction, presentingwith right leg pain
Patient started limping 3 days prior to admission
2 days prior to admission limping progressed, taken toPCMC ED and XR of right ankle noted osteopenia butno cause for pain.
The following day, he was complaining what seemedmore like right knee pain per mother. He was taken to
PCP. Xray at PCP office of the hip did not reveal anyacute concerns. He went back home
The next morning, patient refused to bear weight thatprompted mother to bring in for repeat evaluation
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Review of systems
20-pound loss September-August.
Severely Restricted diet and eating smaller portions at meal-time. Patient refused to eat on the day of admission
Rash that began in his upper arms (September) andprogressed 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 newbruise" per mom
Chronic diarrhea
Mood changes, more irritable
No mental status changes, fever, night sweats, dysphagia,nausea, emesis, dyspnea, cough, or change in urinary orbowel habits
Otherwise ROS negative
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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
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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 5years (changing homes, changing schools and teachers,grandparents divorcing and then remarrying).
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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.
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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 acutedistress, 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 withsmall papillae
NECK: No anterior/posterior lymphadenopathy, full ROM
CARDIOVASCULAR: Normal rate and rhythm. No murmurs or gallop. Strongperipheral pulses, Capillary refill
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PHYSICAL EXAM
ABDOMEN: Soft, non-tended, non-distended. No pain to palpation. Bowel soundspresent. 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 topalpation over right right distal femur/right knee but no surrounding swelling orareas 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 ofhealing. Multiple non-blanching red and purple, hyperkeratotic papullar lesionsaround hair follicles over anterolateral legs thigh, lateral arms, back, chest, neckand cheeks. Normal turgor.
NEURO: no distress, normal gross motor movements, normal fine motor movements.
responding to questions appropriately although not very verbal. Gait: Bears minimalweight on the right leg. Knee remained in flexed position and allows leg to brieflytouch without weight. No ataxia. Unable to illicit patellar reflex on right, but right
Achilles reflex normal. Sensation intact bilaterally.
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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 gingivalhyperplasia
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Differential diagnosis
ID
Infection Osteomyelitis
Septic arthritis
Myositis
Lyme disease
Chronic recurrent multifocal osteomyelitis
Toxic synovitis
RHEUM:
Systemic lupus erythematosus
Juvenile rheumatoid arthritis
Spondyloarthropathy
Connective-tissue disorders
Dermatomyositis
HenochSchnlein purpura
Kawasakis disease
Polyarteritis nodosa
GI
Inflammatory bowel disease
ENDO:
Hyperparathyroidism Hematologic diseases
HEME
Sickle cell disease , Hemoglobinopathy
ONCO
Bone tumor
Chondroma or sarcoma
Osteoblastoma
Ewings or other sarcoma
Leukemia Lymphoma
NEURO:
GuillainBarre syndrome
Heavy-metal
LeggCalvPerthes disease
OTHER:
Truama
Vitamin A intoxication
Vitamin deficiency Rickets
Scurvy
Abuse
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Ddx of Gingival Swelling
Phenytoin exposure
Pyogenic granuloma
Aphthous ulcers
Infectious gingivitis
Crohns disease
Behets disease
Dental abscess
Scurvy
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Labs
CRP 2.0
ESR 13
CBC: WBC 4.4 (39N, 48L, 8M, NC 1700), Hgb 11, Hct 33.3, MCV 76, MCH27, 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%
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Labs
Vitamin C:
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Vitamin C Deficiency: Scurvy
Scurvy caused by deficiency in Vitamin C (ascorbicacid) that is require for the synthesis of collagen inhumans
Reversible reducing agent that is an essential cofactor
for the hydroxylation of proline to hydroxyproline incollagen synthesis and for the hydroxylation of theneurotransmitter dopamine to noradrenaline.
Does not occur in most animals because they cansynthesize their own vitamin C. BUT, humans and
other higher primates (monkeys, apes), guinea pigs,bats, birds, and fish lack L gulonolactone oxidaseenzyme needed for synthesis of Vit C. Must beobtained through diet.
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Scurvy symptoms
Many signs and symptoms of vitamin C deficiencyrelate 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 vasomotorinstability
Systemic symptoms: Malaise and fatigue
Oral symptoms: Gingival swelling andhemorrhage
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Scurvy symptoms
Early symptoms:malasie andlethargy.
After 1-3 months,bone pain, skinchanges withroughness, easy
bruising, petechiae,gum disease, poorwound healing andemotional changes
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Skeletal changes in Scurvy
Most severe in young children (like our patient) as theirskeletal tissue is still growing, and the periosteum is not astightly bound to the surface of the cortex as it is in adults
Deficient collagen in subperiosteal blood vessels leads torupture 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 thegrowth 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
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Body supply can be depleted in 1-3 months
Risk factors:
smoking and medications (aspirin, sulfinpyrazone,
indomethacin, phenylbutazone, tetracycline, oralcontraceptives, corticosteroids, chlorcyclizine)
increase the body's need for vitamin C
intestinal malabsorption syndromes, pregnancy
and lactation, and depressed socioeconomic orrefugee status.
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Rash in Scurvy
Follicular hyperkeratosis (corkscrew hair)
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Vitamin C Deficiency: Scurvy
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ASD and Food Selectivity
Is it real?
Why does it happen?
Does it matter?
What do we do?
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Study out of the UK (Cornish) examined the dietsof 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 26years) assessing dental treatment, oral hygienebehavior, and nutrition, including food preferences
and eating patterns found that 53% of the participants were reported to be
reluctant to try new foods.
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Factors influencing food sensitivity
Survey study done by Williams looked at 100 parentsof children with ASD (ages 22 months to 10 years)
67% of the parents reported that their child was a pickyeater
BUT nearly three quarters (73%) reported that their childhad 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%)
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Texture makes all the difference
Schmitt and colleagues surveyed parents of 20boys with ASD and 18 typically developing boys(ages 7 to 10 years) to complete a questionnaireon 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% ofchildren without autism.
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Does it matter?
Restricted food intake can lead to nutritionalinsufficiency making food selectivity apotential health risk.
Multiple studies have been done to assessnutrient intake of children with ASD, but theyhave produced conflicting results. Somestudies indicate that the nutrient intakes of
children with autism are below that of non ASDchildren while others do not demonstrate anydifferences in nutritional intake
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+ inadequate nutrient intake
Cornish study (3 day dietary recall and food frequencycheck list of 17 children with ASD revealed inadequatenutrient intake
There was an inverse relationship between variety and
nutritional adequacy; as the daily variety decreased, thenumber of nutrient intakes that fell below therecommended amount increased.
Intake of protein, vitamin A, thiamin, vitamin B-12, folicacid, 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.
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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 populationand we must consider and treat each
individual patient
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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
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References
Duggan C. A 9-Year-Old Boy with Bone Pain, Rash, and Gingival Hypertrophy. N engl j med357;4: 392-400.
Cermak S et al. Food Selectivity and Sensory Sensitivity in Children with Austism SpectrumDisorders. J Am Diet Assoc. 2010; 110:238-246
Cornish E. A balanced approach towards healthy eating in autism. J Hum Nutr Diet. 1998;11:501-509.
Schmitt L, Heiss CJ, Campbell EE. A comparison of nutrient intake and eating behaviors of boyswith 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 dentaltreatment: A survey and chart review. Spec Care Dentist. 1999;19:200-207.
Schreck KA, Williams K. Food preferences and factors influencing food selectivity for childrenwith autism spectrum disorders. Res Dev Disabil. 2006;27:353-363.
Field D, Garland M, Williams K. Correlates of specific childhood feeding problems. J PaediatrChild Health. 2003;39:299-304.
Levy SE, Souders MC, Ittenbach RF, Giarelli E, Mulberg AE, Pinto- Martin JA. Relationship ofdietary intake to gastrointestinal symp- toms in children with autistic spectrum disorders. BiolPsychiatry. 2007;61:492-497.