Evidence map›Paper›PMID 39734506›Full record

ArticleAACE clinical case reports

Management of Dysglycemia in a Pregnancy Complicated by Fanconi-Bickel Syndrome.

Emily D Szmuilowicz, Ellen Fruzyna, Nigel Madden, Janelle R Bolden, Anne Kozek, Erika Vucko, Cybele Ghossein, Grant Barish

Abstract readCase Reports
In one paragraph

Article in AACE clinical case reports. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

2 citing papers in PubMed.

  1. Article
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4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

8 authors.

Emily D SzmuilowiczDivision of Endocrinology, Metabolism, and Molecular Medicine, Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
Ellen FruzynaDivision of Endocrinology, Metabolism, and Molecular Medicine, Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
Nigel MaddenDivision of Maternal-Fetal Medicine, Department of Obstetrics & Gynecology, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
Janelle R BoldenDivision of Maternal-Fetal Medicine, Department of Obstetrics & Gynecology, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
Anne KozekDivision of Pediatric Genetics, Genomics and Metabolism, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois.
Erika VuckoDivision of Pediatric Genetics, Genomics and Metabolism, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois.
Cybele GhosseinDivision of Nephrology and Hypertension, Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
Grant BarishDivision of Endocrinology, Metabolism, and Molecular Medicine, Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Background/Objective: Fanconi-Bickel Syndrome (FBS) is an inherited disorder of glucose metabolism resulting from functional loss of glucose transporter 2 characterized by fasting hypoglycemia oscillating with postprandial hyperglycemia. Dysglycemia treatment strategies during FBS pregnancy have not been reported, and insulin therapy carries significant risk due to fasting hypoglycemia in FBS. We report for the first time: (1) glycemic profiles obtained via continuous glucose monitoring (CGM), (2) CGM-guided strategies for cornstarch and nutritional therapy for fasting hypoglycemia and postprandial hyperglycemia, respectively, and (3) placental glucose transporter 2 isoform expression in a pregnant individual with FBS. Case Report: A 27-year-old woman with FBS presented at 6 weeks gestation for management of fasting hypoglycemia and postprandial hyperglycemia. Cornstarch therapy for fasting hypoglycemia and nutritional therapy for postprandial hyperglycemia were iteratively adjusted across gestation based on CGM-derived glycemic patterns. Pregnancy-specific glycemic targets were successfully achieved, and she delivered a healthy term infant. Glucose transporter 2 isoform was not detected in placental tissue. Discussion: We report for the first time glycemic patterns across gestation in a pregnant individual with FBS. Glycemic targets were achieved through stepwise optimization of nutritional and cornstarch therapy, both guided by CGM data. Our approach obviated the need for insulin therapy, which carries amplified risk in FBS. Conclusion: Fasting hypoglycemia and postprandial hyperglycemia can be effectively treated through CGM-guided adjustment of both nutritional and glucose polymer therapies in FBS pregnancy. More broadly, our case highlights a novel application for CGM in the management of uncommon glucose metabolism disorders during pregnancy.

Indexed as

continuous glucose monitoringcornstarch therapyFanconi–Bickel Syndromeglucose transporter 2pregnancy

Identifiers

PMID39734506
PMCPMC11680753

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.