Malnutrition with Thiamine Deficiency

7/29/2019 Malnutrition with Thiamine Deficiency

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MR 1/2/13Anna Petersen, MD

PGY-3


2/20

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.


3/20

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


4/20

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.


5/20

ROS:

foot weakness limp, tingling sensationConstipation

Cranky per MGM, not following directions

Negatives:

No feversNo altered mental status

No recent illnesses


6/20

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


7/20

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.


8/20


9/20

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


10/20

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


11/20

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


12/20

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


13/20

Labs return:

B 12 = normal; B 6 = normal;

B1 =


14/20

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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17/20

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


18/20

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


19/20

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.


20/20

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

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Malnutrition with Thiamine Deficiency