Severe vitamin deficiencies in pregnancy complicated by progressive familial intrahepatic cholestasis

2021 ◽  
Vol 14 (3) ◽  
pp. e240248
Author(s):  
Amanda Caroline Mahle ◽  
Bernard David Morris ◽  
Zane Frazer ◽  
Christopher Novak
Keyword(s):  
Vitamin A ◽  
Intrahepatic Cholestasis ◽  
Vitamin A Deficiency ◽  
Nutritional Deficiencies ◽  
Primiparous Woman ◽  
Progressive Familial Intrahepatic Cholestasis ◽  
Vitamin Deficiencies ◽  
Visual Deficits ◽  
Severe Vitamin

Progressive familial intrahepatic cholestasis (PFIC) is a rare disease of impaired bile acid excretion which can lead to nutritional deficiencies. Vitamin deficiencies during pregnancy can result in adverse maternal and fetal outcomes. A 20-year-old primiparous woman at 30 4/7 weeks with PFIC type 2 presented with worsening cholestasis, coagulopathy and fat-soluble vitamin deficiency. She developed visual deficits and was found to have severe vitamin A deficiency. Her coagulopathy and visual deficits improved following vitamin K and A supplementation, respectively. She delivered at 32 2/7 weeks following preterm labour. This case highlights several unique aspects in the care of pregnant women with liver disease. These patients are at risk for fat-soluble vitamin deficiencies which can result in significant coagulopathy and rarely, visual deficits due to vitamin A deficiency. Prompt treatment is necessary to prevent permanent sequelae.

scholarly journals Severe Vitamin A Deficiency After Biliopancreatic Diversion

2019 ◽  
Vol 7 ◽  
pp. 232470961988805 ◽  
Author(s):  
Lauren M. Lemieux ◽  
Vijaya Surampudi
Keyword(s):  
Bariatric Surgery ◽  
Weight Loss ◽  
Vitamin A ◽  
Biliopancreatic Diversion ◽  
Unique Feature ◽  
Vitamin A Deficiency ◽  
Case Series ◽  
Nutritional Deficiencies ◽  
Severe Vitamin ◽  
Deficiency Screening

Biliopancreatic diversion is a surgical procedure that causes weight loss via volume restriction and malabsorption. It is now rarely performed due to the risk of severe nutritional deficiencies including vitamin A. We report a case of severe vitamin A deficiency due to malabsorption from a biliopancreatic diversion procedure for weight loss. By the time the patient presented to our department, she had developed blindness refractory to parenteral vitamin A treatment. A unique feature of her case is the development of a rash with vitamin A injections. This reaction has only been reported in one case series of 3 patients in the published literature. Her case highlights the importance of vitamin deficiency screening in patients after bariatric surgery, and her skin reaction to the injections is a unique side effect that is not frequently observed.

scholarly journals Two Cases of Progressive Familial Intrahepatic Cholestasis Type 2 Presenting with Severe Coagulopathy without Jaundice

2014 ◽  
Vol 2014 ◽  
pp. 1-4
Author(s):  
Eric Tibesar ◽  
Christine Karwowski ◽  
Paula Hertel ◽  
Ann Scheimann ◽  
Wikrom Karnsakul
Keyword(s):  
Bile Acid ◽  
Bile Salt ◽  
Intrahepatic Cholestasis ◽  
Growth Failure ◽  
Acid Transport ◽  
Progressive Familial Intrahepatic Cholestasis ◽  
Bile Acid Transport ◽  
Vitamin Deficiencies ◽  
Severe Coagulopathy

Progressive familial intrahepatic cholestasis (PFIC) type 2 results from a mutation in the bile salt exporter pump, impeding bile acid transport. Patients usually present with jaundice, pruritus, growth failure, and fat soluble vitamin deficiencies. We present two patients diagnosed with PFIC type 2 due to severe coagulopathy and bleeding without jaundice.

scholarly journals Glycerol Phenylbutyrate Therapy in Progressive Familial Intrahepatic Cholestasis Type 2

2020 ◽  
Vol 70 (6) ◽  
pp. e139-e140
Author(s):  
Marion Almes ◽  
Agathe Jobert ◽  
Martine Lapalus ◽  
Elodie Mareux ◽  
Emmanuel Gonzales ◽  
...  
Keyword(s):  
Intrahepatic Cholestasis ◽  
Progressive Familial Intrahepatic Cholestasis

Improved Liver Function and Relieved Pruritus after 4-Phenylbutyrate Therapy in a Patient with Progressive Familial Intrahepatic Cholestasis Type 2

2014 ◽  
Vol 164 (5) ◽  
pp. 1219-1227.e3 ◽  
Author(s):  
Sotaro Naoi ◽  
Hisamitsu Hayashi ◽  
Takeshi Inoue ◽  
Ken Tanikawa ◽  
Koji Igarashi ◽  
...  
Keyword(s):  
Liver Function ◽  
Intrahepatic Cholestasis ◽  
Progressive Familial Intrahepatic Cholestasis

scholarly journals Successful mutation-specific chaperone therapy with 4-phenylbutyrate in a child with progressive familial intrahepatic cholestasis type 2

2012 ◽  
Vol 57 (3) ◽  
pp. 695-698 ◽  
Author(s):  
Emmanuel Gonzales ◽  
Brigitte Grosse ◽  
Doris Cassio ◽  
Anne Davit-Spraul ◽  
Monique Fabre ◽  
...  
Keyword(s):  
Intrahepatic Cholestasis ◽  
Progressive Familial Intrahepatic Cholestasis

scholarly journals Normal levels of serum pancreatic enzymes in patients with progressive familial intrahepatic cholestasis type 2.

2010 ◽  
Vol 57 (4) ◽  
Author(s):  
Jaroslaw Walkowiak ◽  
Irena Jankowska ◽  
Aleksandra Lisowska ◽  
Maciej Biczysko ◽  
Aldona Wierzbicka ◽  
...  
Keyword(s):  
Intrahepatic Cholestasis ◽  
Elevated Serum ◽  
Normal Serum ◽  
Pancreatic Enzymes ◽  
Serum Lipase ◽  
Progressive Familial Intrahepatic Cholestasis ◽  
Elastase 1 ◽  
Pancreatic Damage ◽  
High Prevalence

High prevalence of elevated serum pancreatic enzymes in children with cholestasis with normal fecal elastase-1 concentrations has been documented. However, this state is related predominantly to biliary atresia. Therefore, we aimed to assess pancreatic damage by measuring serum pancreatic enzymes in patients with progressive familial intrahepatic cholestasis type 2 (PFIC type 2). Twenty PFIC type 2 patients with normal serum bilirubin and bile acid concentrations were included in the study. Thirty pancreatic insufficient cystic fibrosis (PI-CF) patients, thirty patients with acute pancreatitis (AP) and thirty healthy subjects (HS) served for the purpose of comparison. In all subjects, serum lipase and elastase-1 levels were measured. In all but one PFIC type 2 patients and all HS normal lipase activities were found. Serum elastase-1 concentrations were normal in all PFIC type 2 patients and HS. The enzyme levels were very similar in both groups studied. Lipase activities in PFIC type 2 patients were significantly higher than in PI-CF patients (p < 0.00001) and lower than in patients with AP (p < 0.00001). Serum elastase-1 levels in PFIC type 2 patients were significantly lower than in patients with AP (p < 0.00001) and not different from those in PI-CF patients. In conclusion. serum pancreatic enzymes in patients with PFIC type 2 are normal. No pancreatic damage in these patients could be detected.

Vision Loss Due to Severe Vitamin A Deficiency After Biliopancreatic Diversion

2021 ◽  
Author(s):  
Fulya Calikoglu ◽  
Ozlem Soyluk Selcukbiricik ◽  
Serife Bayraktar ◽  
Umut Barbaros
Keyword(s):  
Vitamin A ◽  
Biliopancreatic Diversion ◽  
Vision Loss ◽  
Vitamin A Deficiency ◽  
Severe Vitamin

scholarly journals Severe Vitamin a Deficiency After Biliopancreatic Diversion (P12-051-19)

2019 ◽  
Vol 3 (Supplement_1) ◽  
Author(s):  
Lauren Lemieux ◽  
Vijaya Surampudi
Keyword(s):  
Vitamin A ◽  
Injection Site ◽  
Biliopancreatic Diversion ◽  
Vision Loss ◽  
Vitamin A Deficiency ◽  
Skin Testing ◽  
Injection Site Reaction ◽  
Case Series ◽  
Night Vision ◽  
Nutritional Deficiencies

Abstract Objectives Introduction: Biliopancreatic diversion (BPD) is a surgical procedure that causes weight loss via volume restriction and malabsorption. It is now rarely performed due to the risk of severe nutritional deficiencies including Vitamin A (1). Methods Case Description: A 68-year-old female status-post BPD in 1987 was referred for vitamin A deficiency. She initially had diminished night vision that progressed to left eye blindness by the time of her diagnosis in 2013. She was treated with oral vitamin A 100,000 IU daily; however, levels did not normalize, and her retinopathy progressed. She was later treated with intramuscular vitamin A and developed injection site rashes (Figure 1) thought to be due to an injection site reaction versus type IV hypersensitivity. Over time her vitamin A levels improved but her vision did not. Results Vitamin A deficiency is a preventable complication of BPD and can lead to permanent vision loss. A study of 376 BPD patients found that 1 year after surgery vitamin A levels were low in 52% and 4 years this increased to 69% despite supplementation compliance (2). Vitamin A injection site rashes have been reported in one case series of 3 patients (3), and skin testing revealed sensitivity to polysorbate 80. This is an emulsifier found in injectable vitamin A palmitate, other parental medications and some vaccines (3). Conclusions Vitamin screening is important in post-bariatric surgery patients. Rash following intramuscular vitamin A is uncommon and should be closely monitored for progressive allergic reaction and potential for reactions to other medications that contain similar components. Funding Sources None. Supporting Tables, Images and/or Graphs

scholarly journals Isolated and combined prevalence of anemia, vitamin A deficiency and zinc deficiency in preschool children 12-72 months for the government of Paraíba

2014 ◽  
Vol 27 (3) ◽  
pp. 301-310 ◽  
Author(s):  
Dixis Figueroa Pedraza ◽  
Márcia Cristina Sales
Keyword(s):  
Vitamin A ◽  
Zinc Deficiency ◽  
School Children ◽  
Vitamin A Deficiency ◽  
Serum Zinc ◽  
Nutritional Deficiencies ◽  
Simultaneous Occurrence ◽  
Micronutrient Deficiencies ◽  
Serum Retinol ◽  
Prevalence Of Anemia

OBJECTIVE: To estimate the isolated and combined prevalence of anemia, vitamin A deficiency and zinc deficiency in pre-school children, as well as the distribution of isolated deficiencies according to gender, age and prior supplementation with vitamin A. METHODS: Cross-sectional study with pre-school children in the state of Paraíba, Brazil. Analysis of the average concentrations of hemoglobin, serum retinol and serum zinc, according to gender, age and previous vitamin A supplementation of children were carried out as well as the risk of simultaneous occurrence of micronutrient deficiencies in the presence of these deficiencies. RESULTS: The prevalence of anemia, vitamin A deficiency and zinc deficiency were 15.4%, 23.3% and 13.8%, respectively. The anemia was significantly associated with age (p<0.01). Children previously supplemented by vitamin A had higher serum retinol concentrations than children without supplements, an effect that was not observed for concentrations of hemoglobin or serum zinc. The prevalence of anemia associated with vitamin A deficiency was 5.8%, with the chance of vitamin A deficiency and anemia coexist 2.21 times (95%CI=1.03-4.84) higher in the case of vitamin A deficiency or anemia rather than in the absence of these conditions. CONCLUSION: The high prevalence of micronutrient deficiencies which are important in child growth, as well as the coexistence of nutritional deficiencies, point out the need to strengthen nutrition intervention strategies that consider this issue.

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