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7/29/2019 Malnutrition with Thiamine Deficiency
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MR 1/2/13Anna Petersen, MD
PGY-3
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12 y.o. M, with history ofchoking at age 4 on piece ofmeat, o/w healthy, presents asa direct admission from
neurology clinic.
Approximately 4 weeks ago hehad a "virus" with sore throat andupset stomach x 4 days.Grandmother endorses somelower extremity weakness butthought it then seemed toresolve.
2 weeks PTA, felt fatigued anddeveloped pain and weakness inboth lower extremities. The painand weakness has gottenprogressively worse. He has spent
the last 2 weeks in bed watchingTV.
Presented to PCP for difficultywalking--he describes this ashaving to kick his feet out towalk. His grandmother endorses awide based gait and says that he
has been taking ibuprofenregularly for pain. He getsbullied by his older brother forthis.
Seen in neurology clinic,who noted significantfoot drop and decreasedreflexes in both lowerextremities, R>L, and had
him directly admitted forfurther workup at PCMC.
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PMH: Term birth, nl. development & milestones. Had a chokingincident at age 4 with red meat. Per Grandmother, he developedanorexia to meat and chewy food items thereafter.
PSH: none
FHx:
- Father RLE neuropathy due to crush injury
- Mother IDDM, hypotensive episode with 2o TBI, mitral valveprolapse, diabetic neuropathy
- PGT died at age 55 w/w pulmonary artery rupture
- MGF Diabetic peripheral neuropathy
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Social: Lives part-time with MGM and aunt; part-timewith father and fathers girlfriend. There disagreementfrom guardians about the best living situation andschool environment for patient and brother. Motherhad a TBI 2/2 diabetic coma, now is trach-ventdependent and living in a rehab facility.
Meds: Ibuprofen
Behavioral Told he had ADHD previously, not on anymedications right now. Does have frequent screamingfights with grandmother and dad.
Exposures - No recent travel outside of state. Spendstime outdoors with brother. No risk of ingestion, pergrandmother.
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ROS:
foot weakness limp, tingling sensationConstipation
Cranky per MGM, not following directions
Negatives:
No feversNo altered mental status
No recent illnesses
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PE: T 36.7; HR 88; RR 20; BP 109/71
Gen pale, thin boy, alert, awake, difficult to talk to,seems mad to be in the hospital
HEENT Slightly thin temporal sides, TMs nl, necksupple, EOMI/PERRL, NP/OP nl
CV RRR, S1/S2 no murmur, normal pulsesRESP CTAB, no distress
ABD thin and concave , normal BS, no HSMappreciated
Ext No rashes, no edema, no clubbing
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NEURO EXAM: BLE proximal muscle weakness
was noted from the thighs distally to feet.- In his lower extremities, he had bilateral
decreased sensations from knees and knee and ankle jerkreflexes were absent.
- No upper-extremity abnormalities andintact cranial nerves
Gait Ataxic, unsteady without a hand-hold.
Significant right-sided foot drop andTrendelenburg gait.
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Thoughts?Polyneuropathy in a pediatrics patient:
INFECTIOUS: INFLAMMATORY PARANEOPLASTIC TOXIC
-Lyme Dz
-Leprosy-HIV-Diptheria-Mononucleosis
-CIDP
-AIDP-Guillain-Barre
-TUMORS spinal
-- CIDP
-- Lead ingestion
-- Arsenic-- Medications
NUTRITIONAL: DIABETES: Other:
--Alcohol-- B12, B1, B6deficiencies--Vit E def
- Chronichyperglycemicdamage
-- Amyloidosis-- Porphyria-- Lupus, Sjogrens-- Vasculitis-- Uremia
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EMG Abnormal conduction consistent with axonal injury
(normal conduction, decreased amplitude)
LP and spinal fluid analysis were normal. Clear, 3 WBC(Lymphs/Monos), nl protein/glucose; no oligoclonal bands
CMP, CBC, CPK, magnesium, TSH CRP/ESR were all normal.
UA = nl; heavy metals in urine = neg
Vitamin levels, Copper, Pyruvate, PENDING
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And now,
The rest of the storyOn HD #1, NGT placed and slow rehydration begun,due to patient refusing to eat and poor urine output
By evening of HD #2, patient had more fatigue andslight tachycardia
AM HD #3, NS bolus x 2 was given due to concerns ofdehydration.
Tachycardia and hypotension ensued
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Admitted to PICUBNP of >2000 and Troponin 0.18
Milrinone drip started
STAT ECHO obtained LVH and impaired LV relaxation
Given Lasix and weaned off Milrinone slowly
Myocarditis workup initiated: Cardiology believes heart
failure not related to neuropathy
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Labs return:
B 12 = normal; B 6 = normal;
B1 =
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A Very-Very
Beri-Beri ProblemThiamine, vitamin B1-- serves as a coenzyme in the oxidation of alpha-keto acids and 2-keto sugars. It is critical to pyruvate metabolism and is necessary
for the synthesis of acetylcholine.
-- Because of its use in a variety of metabolic processes and itslimited storage in the body, there is a constant daily need for
-- It is rapidly absorbed in the proximal jejunum by activetransport and passive diffusion
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Wet/Cardiac
Cardiac Beri-Beri
Occurs when the impaired pyruvate metabolismleads to a decrease in the amount of acetly-CoA in
Krebs cycleEnergy deprivation to heart muscle => cellbreakdown
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Dry/Neuronal
Neuronal Beri-Beri
Occurs most commonly with distal polyneuropathy,both affecting sensory nerves and motor function due
to affected axonal injury.Usually irreversible
Etiology not fully understood
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Treatment
IV loading dose of 100mg x 1
IV Thiamine @ 25 mg daily
Switched to PO with 25mg, twice daily, for severalweeks
Added a multivitamin as well.
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Prognosis
Foot Drop
Guarded, most likely poor. Unsure of potential use.
Heart Failure
Improved, likely full recovery
BehavioralAdmitted to UNI Child Psychiatry unit
Nutritional
Dependent on the behavioral