Treatment of rickets and dyslipidemia in twins with progressive familial intrahepatic cholestasis type 2

Sura, Sunitha; Germain‐Lee, Emily L.

doi:10.1186/s13633-020-00079-1

Cited by 1 publication

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References 20 publications

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“…Another previous study using high doses of vitamin D therapy (vitamin D2 50,000 IU, three times a week over 10 weeks) led to the improvement of serum 25-OHD levels and resolution of rickets in PFIC type 2. [ 29 ] The pathogenesis of cholestasis in PFIC type 2, which is associated with impaired function of hepatocyte bile salt export pump [ 30 ], differs from that of biliary atresia, which is mainly caused by anatomical bile duct obstruction. This may explain the better outcomes of vitamin D treatment in patients with PFIC type 2 compared with the present study.…”

Section: Discussionmentioning

confidence: 99%

Determination of Optimal Vitamin D Dosage in Children with Cholestasis

Chongthavornvasana,

Lertudomphonwanit,

Mahachoklertwattana

et al. 2023

BMC Pediatr

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Background Vitamin D deficiency in patients with cholestasis is due to impaired intestinal vitamin D absorption, which results from decreased intestinal bile acid concentration. Patients with cholestasis usually do not achieve optimal vitamin D status when a treatment regimen for children without cholestasis is used. However, data on high-dose vitamin D treatment in patients with cholestasis are limited. Methods This study is a prospective study that included pediatric patients with cholestasis (serum direct bilirubin > 1 mg/dL) who had vitamin D deficiency (serum 25-hydroxyvitamin D, 25-OHD, < 20 ng/mL). In Phase 1, single-day oral loading of 300,000 IU (or 600,000 IU if weight ≥ 20 kg) of vitamin D2 was administered, followed by an additional loading if serum 25-OHD < 30 ng/mL, and 4-week continuation of treatment using a vitamin D2 dose calculated based on the increment of 25-OHD after first loading. In Phase 2, oral vitamin D2 (200,000 IU/day) was administered for 12 days, followed by 400,000 IU/day of vitamin D2 orally for another 8 weeks if serum 25-OHD < 30 ng/mL. Results Phase 1: Seven patients were enrolled. Three out of seven patients had a moderate increase in serum 25-OHD after loading (up to 20.3–27.2 ng/mL). These patients had conditions with partially preserved bile flow. The remaining four patients, who had biliary atresia with failed or no Kasai operation, had low increments of serum 25-OHD. Phase 2: Eleven patients were enrolled. Eight out of 11 patients had a moderate increase in serum 25-OHD after 200,000 IU/day of vitamin D2 for 12 days. Serum 25-OHD continued increasing after administering 400,000 IU/day of vitamin D2 for another 8 weeks, with maximal serum 25-OHD of 15.7–22.8 ng/mL. Conclusion Very high doses of vitamin D2 (200,000 and 400,000 IU/day) partly overcame poor intestinal vitamin D absorption and resulted in moderate increases in serum 25-OHD in pediatric patients with cholestasis, particularly when cholestasis was caused by uncorrectable bile duct obstruction.

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Section: Discussionmentioning

confidence: 99%