JenniferH: Created page with "''(adapted from PowerPoint Presentation by Drs. Lescher and Singleton)''
'''Pathophysiology:''' Failure of mineralization of growing bone and cartilage A state of extre..."

2020-09-24T10:23:13Z

Created page with "''(adapted from PowerPoint Presentation by Drs. Lescher and Singleton)'' <br/>'''Pathophysiology:''' *Failure of mineralization of growing bone and cartilage *A state of extre..."

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''(adapted from PowerPoint Presentation by Drs. Lescher and Singleton)''
<br/>'''Pathophysiology:'''
*Failure of mineralization of growing bone and cartilage
*A state of extreme vitamin D deficiency
*Peak incidence between 3 and 18 months of age
'''Risk Factors:'''
*insufficient dietary intake and sun exposure
*darker skin color and use of sunscreen
*breastfeeding exclusively without Vitamin D supplementation
*Northern Latitudes (above 37 deg Latitude)
*Anticonvulsants, Antifungals, glucocorticoids
*Limited intake of foods high in Vitamin D (very few exist naturally; however, the Native diet is high in Vitamin D, but the number of children on an exclusive Native diet is decreasing)
*Obesity
'''Signs/Symptoms:'''
*Irritability, pain, gross motor developmental delay, poor growth
*Widening of the wrists and ankles, Genu varum or valgum, Prominent costochondral junction (rachitic rosary), Delayed closure of fontanels, Craniotabes, Frontal bossing
*Delayed tooth eruption, increased risk of caries
*Increased susceptibility to infections
*Severe hypocalcemia—tetany, seizures (more often infancy or adolescence with increased growth velocity). usually asymptomatic until serum Ca<7.5mg/dl
'''Diagnosis:'''
*Radiologic studies: Wrist or Knee XRays
**Osteopenia, cortical thinning of long bones
**Stress fractures
**Metaphyseal widening and fraying, splaying, cupping
*Laboratory studies: Alk Phos is a good screen for rickets; 25OHD level is needed for assessment of Vitamin D status
**Hypophosphatemia, varying degrees of hypocalcemia
**Increased alkaline phosphatase
**Increased PTH
**Low 25OHD levels
'''Management:'''
*Replace vitamin D and calcium (Proposed treatment plans by AAP):
**Pharmacological doses of vitamin D: 1000-10,000 IU per day for 8-12 weeks depending on age of the child, then maintain at 400-1000 IU per day
***1000-5000 IU/day up to age 1, >5000 IU/day after age 1
**Stoss therapy: 100,000 – 600,000 IU vitamin D orally, over 1-5 days, then maintain at 400-1000 IU vitmain D per day or 50,000 IU vitmain D2 weekly for 8 weeks (teens and adults only)
**Calcium: 30-75 mg/kg/day elemental Ca in 3 divided doses (start at higher dose, then wean down to lower end of the range over 2-4 weeks
**May also need Calcitriol (1,25D) if hypocalcemic
*Monitoring of therapy (proposed, by AAP)
**At 1 month: measure Ca, Phos, Alk Phos
**At 3 months, measure Ca, Phos, Mg, Alk Phos, iPTH, 25OHD, urine Ca/Cr and recheck X-rays
**At 1 year and annually, measure 25OHD
*If symptomatic from severe hypocalcemia
**Slow (<1 ml/min) IV infusion 10% Ca gluconate 1 ml/kg
***100 mg/ml Ca Gluconate = 9 mg/ml elemental Ca
***Cardiac monitoring (bradycardia, shortened QTc due to IV Ca); close attention to infusion site if not central IV (risk of tissue necrosis if peripheral IV infiltration)
**If Mg low, replace with 0.1-0.2 ml/kg 50% Mg Sulfate
'''Critical Times for Affected Patients (When to refer to Endocrinology):'''
*If no healing after 3 months of Vit D and Ca replacement
**Concern for malabsorption, liver disease, adherence
*When considering other causes of rickets (not Vit D deficiency)
**Rickets <6 months old or between 3 and 10 years old
**Xrays that show periostal reaction and moth-eaten metaphysis rather than splaying, cupping, etc.
**Normal levels of AlkP, 25OHD, very low or very high levels of 1,25OHD, high BUN and Cr
*Severe hypocalcemia
'''Resources:'''
*Vitamin D Supplementation and Screening for the Prevention of Rickets and Osteomalacia in Alaska Recommendations from the Alaska Vitamin D Workgroup [http://www.epi.alaska.gov/bulletins/docs/rr2018_04.pdf]
*Singleton, R. et al. [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4678029/ Rickets and Vitamin D Deficiency in Alaska Native Children]. J Pediatrc Endocrinol Metab. 2015 Jun: 28(0): 815-823. doi: 10.1515/jpem-2014-0446.
*[http://alaskamchconference.org/2016_assets/archives/2014/E1%20-%20Rickets%20and%20Vitamin%20D%20-%20Lescher.pdf Rickets and Vitamin D Deficiency in Children] Powerpoint Presentation by Drs. Lescher and Singleton

[[Practicing Medicine in Bush Alaska—Some ABCs|Common/Unique Medical Diagnoses]]

Rickets and Vitamin D Deficiency - Revision history

JenniferH: Created page with "''(adapted from PowerPoint Presentation by Drs. Lescher and Singleton)'' '''Pathophysiology:''' *Failure of mineralization of growing bone and cartilage *A state of extre..."

JenniferH: Created page with "''(adapted from PowerPoint Presentation by Drs. Lescher and Singleton)''
'''Pathophysiology:''' Failure of mineralization of growing bone and cartilage A state of extre..."