Case 3

DG is a 7 yr old male with severe cerebral palsy, neurologic impairment, and seizure disorder secondary to congenital CMV (cytomegalovirus) infection. He presented to the pediatric clinic with a chief complaint from his mother that “his chest fell in.” X-ray studies obtained in the clinic revealed several rib fractures and severe osteoporosis and osteomalacia (ie, both decreased bone mineral density and abnormal bone matrix). Patient was admitted to hospital for treatment and short-term rehabilitation.

Weight: 5.6 kg
Length: 91 cm
Head circumference: 37 cm
Vital signs: Temp: 35 Heart rate: 45 Blood pressure: 100/67 Resp rate: 26
Exam (brief): Severely cachectic white male, minimally responsive to environment

Skin: dry; no rash, no petechiae or purpuric lesions; slight pallor
HEENT: sclerae w/o icterus, conjuntivae w/o injection or pallor; mouth w/o lesions except + gingival hypertrophy; dry lips
LUNGS: coarse breath sounds, fair air exchange
CV: Reg rate & rhytym, no murmur; adequate peripheral pulses, slightly prolonged capillary refill & cool extremities
ABDOMEN: slightly distended w/ palpable stool; no organomegaly
EXTREMITIES: markedly decreased subcutaneous fat and muscle mass, no edema
NEURO: minimally responsive, normal deep tendon reflexes

Labs: Low phosphorus and calcium, high alkaline phosphatase
Normal electrolytes, normal albumin
See below for progressive changes in labs

 

LABORATORY STUDIES:

Electrolytes: Complete Blood Count:
Sodium 133 White blood cells 3,700
Potassium 3.9 (25 S 5 B 62 L )
Chloride 101 Hemoglobin 12.6g/dl
Bicarb 21 Hematocrit 36.8%
Blood urea N 3 (low ) MCV 100 ( ­ )
Creatinine 0.2
Liver Function tests: normal except: albumin 3.2 (3.2-5.5)
alk phos 686 (110-400)

Calcium Phosphorus Magnesium Alk Phos
(4.4-5.4) (4.0-6.0) (1.4-2.0) (110-460)
mg/dl mg/dl mg/dl IU/I
1/16 2.8 1.2 1.4 686
1/17 2.8 0.9 1.2
1/18 2.9 1.2 1.3
1/18 1.0
1/19 1.8 1.3 659
1/20 1.5 1.3
1/21 5.8 1.1
1/22 3.0 1.1 516
1/24 2.9 5.0 1.2 467

 

Question 1: What nutritional history you would want to know (specifically re intake, possible losses, requirements)?

  1. Obtain a delailed diet history, including what he consumes in a typical day as well as a 24 hour recall. Get specific amounts and types of foods and liquids. How is he fed? Does he participate?
  2. What are his cues that he is hungry or full? How long do meals take, and what is the usual eating schedule?
  3. Does he have any food intolerances or allergies? Are any food groups excluded?
  4. Does he have trouble chewing or swallowing?
  5. Is he taking any vitamin, mineral, or herbal supplements?
  6. What medications if any is he taking? (some meds especially anti-seizure meds can be vitamin D antagonists).
  7. Does he have problems with constipation, diarrhea, or vomiting?
  8. Does he receive physical therapy? Do the caregivers have any concerns about his intake or with feeding him?
  9. How has he been growing and gaining weight?

 

Question 2: What is the patient’s weight status? Is he marasmic or with kwashiorkor? What supports that diagnosis? What percent of ideal body weight is he?

  1. He is severely marasmic, with % of ideal body weight at 45%. Ideal body weight is estimated at 12 – 12.5 kg (weight for height at 10-25th% given dx of CP). He is cachectic, with markedly decreased subcutaneous fat and muscle, and with no edema. Albumin level is low normal, consistent with a picture of starvation.

 

Question 3: The x-ray findings and labs confirm a diagnosis of rickets. What labs and what others might you want to assess this child?

  1. Would want to obtain 25,OH vitamin D and 1, 25 OH vitamin D levels. Would continue to follow serum calcium, phosphorus, magnesium, and alkaline phosphatase levels. Could also consider checking a parathyroid level (would expect it to be elevated).

 

Question 4: How would you approach nutritional rehabilitation in this child?

  1. Given the degree of current malnutrition, he will require supplemental enteral nutrition support for repletion. With his underlying condition, he most likely will need permanent enteral access. However, with his level of fragility, surgical intervention should wait until he has had some nutritional rehabilitation to reduce the risk of surgical morbidity and mortality. A nasogastric tube could be used to provide him with continuous drip feedings, advancing to bolus feedings as tolerated. Calories should be started at maintenance requirements (about 100 kcal/kg current weight), and be increased very gradually.
  2. He is at high risk for refeeding syndrome, and feeding should be advanced cautiously – watching for precipitous drops in potassium, magnesium, and phosphorus. Because of his underlying condition, calorie needs may be difficult to estimate. Increase feeds slowly until he demonstrates consitent weight gain, which may require > 150 – 200 kcal/kg current weight.
  3. Because of the care required for this child, social services should assess the family situation as well as support options for the family.
  4. Physical therapy should consult to address positioning, muscle strengthening and range of motion exercises, etc. Patient is at high risk for fractures.
  5. Feeding therapy (occupational or speech language pathology) should assess whether it is appropriate and safe for him to take anything by mouth.

 

Question 5: Why did the Ca, P, & Mg stay low despite supplementation?

  1. Calcium can often take weeks to return to normal once treatment for rickets is begun. In DG’s case, he was likely both vitamin D and calcium deficient. During initial treatment for rickets, the bones act like “sinks” and rapidly take up the supplemented calcium.
  2. His hypophosphatemia is multifactorial and is related to his severe malnutrition and evidence of refeeding syndrome, compounded by the vitamin D deficiency. Upon refeeding there is a significant intracellular shift of phosphorus as it is used during energy metabolism. The “starved” bones also rapidly take up phosphorus.
  3. The hypomagnesemia is also related to his malnutrition/refeeding as well as the vitamin D deficiency and hypocalcemia. Huge amounts of magnesium supplements can be required during rickets treatment, especially during the initial phases.

 

Question 6: Why did he become Vitamin D deficient?

  1. He became so vitamin D deficient likely from a combination of a vitamin D deficient diet (in the setting of severe malnutrition) as well as minimal sunlight exposure because of his underlying condition. He may have also had drug-nutrient interactions if he was taking anti-convulsant medications (interfering with vitamin D metabolism).