Medical nutrition therapy for a rare disease (Gullian barre syndrome)

doi:10.15406/emij.2018.06.00196

eISSN: 2473-0815

Endocrinology & Metabolism International Journal

Proceeding Volume 6 Issue 4

Medical nutrition therapy for a rare disease (Gullian barre syndrome)

Ushakiran Sisodia,¹

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Nirva Desai,² Bhavika Singhvi³

¹HOD Diet, Nutrition NSSH, Lifestyle Medicine Clinician, India
²Dietician NSSH, P, G, Dip in Dietetics, India
³Nutrition, Dietetics, India

Correspondence: Ushakiran Sisodia, HOD Diet, Nutrition NSSH, RD CDE, Msc, FN, Lifestyle Medicine Clinician, India

Received: July 11, 2018 | Published: August 6, 2018

Citation: Sisodia U, Desai N, Singhvi B. Medical nutrition therapy for a rare disease (Gullian barre syndrome). Endocrinol Metab Int J. 2018;6(4):313. DOI: 10.15406/emij.2018.06.00196

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Keywords

hospital, electromyography, injections of immunoglobulins, dyspnoea hypotonia

Abbreviations

EMG, electromyography; AMAN, acute motor axonal neuropathy

Proceeding

10year boy admitted in hospital underweight. BMI 14.9kg/m^² (between 10^th and 25^th percentile) diagnosed Gullian Barre Syndrome a rare serious autoimmune disorder, with present complaint vomiting low grade fever, pain in left hand ,inability to move lower limb, dyspnoea hypotonia (muscle weakness) of 4limbs. High platelet count and low sodium levels. MRI showed demyelinating polyneuropathy, characteristic of GBS Electromyography (EMG) and Abnormalities in NCS that are consistent with dymelination are sensitive, acute motor axonal neuropathy (AMAN) as seen in patient Blood smears and ET secretions showed presence of Pseudomonas aeroginosa, cause for the syndrome. Lumbar puncture showed significant rise in protein without pleocytosis. Injections of Immunoglobulins @ 1gm/kg/day twice a day, Amlodipin were added in view of hypertension (dysautonomia). Child on ventilator for 20 days after which extubated reintubated. Tracheotomy removed gradually power and movement, of left side improved. Anti hypertensive were tapered. MNT sips of liquids, which patient tolerated well. As gag and cough reflux improved oral feeds started and RT was removed. Shifted from ICU to paediatric ward. home diet recall vegetarian 1340 calories and 33gms of proteins liked fruits, poha, dal and rice, disliked vegetables and upma, having dry fruits and chocolates rarely. The RDA 2190 kcal and 39.9gm of protein on discharge hemodynamically stable, No respiratory distress, neurologically improved, Voice quality improved high protein 70gms. RT plan s high calorie, high protein fussy eater so lot of psychological counselling done started with intake of, approx 1000kcals and 30gms protein, 1400 cal average and 60gms of protein. Improvement in the child’s muscle strength, discharged could stand until 250 counts.

PES statement (Problem etiology and signs and symptoms): Gullian Barre Syndrome due to Pseudomonas infection as evidenced by weakness, difficulty in breathing, areflexia, quadraparesis and weight loss. The search for more effective treatment based on a complete understanding of the molecular pathogenesis of GBS continues. I describe my third encounter with a GBS patient that prompted me share my experience in 17 years of research life to elucidate the pathogenesis of GBS .last two GBS which been living normal healthy life .playing sports ,dancing shows .Its will power combined with good nutritional support therapy with lot of patience in handling such cases.