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Juvenile xanthogranuloma-related central diabetes insipidus and pregnancy: Case report and literature review
1 Department of Obstetrics and Gynecology, University Hospital of Brasilia, Brazil
2 Departmen of Pathology, University Hospital of Brasilia, Brazil
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Ana Luiza Pessoa De Resende
Department of Obstetrics and Gynecology, University Hospital of Brasilia,
Brazil
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Article ID: 101043Z01AR2019
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De Resende ALP, França PS, Takano GHS, Neto FFC. Juvenile xanthogranuloma-related central diabetes insipidus and pregnancy: Case report and literature review. Int J Case Rep Images 2019;10:101043Z01AR2019.ABSTRACT
No Abstract
Keywords: Diabetes insipidus, Gestational diabetes insipidus, Juvenile xanthogranuloma, Touton giant cell
Case Report
A 27-year-old patient, G1P0A0, presented with diabetes insipidus caused by juvenile xanthogranuloma, diagnosed at 11 years of age due to the appearance of xanthomas in the skin and central nervous system (CNS). Skin biopsy evidenced the presence of numerous foamy cytoplasm cells and Touton giant cells, expressing CD-68 and S100 protein (Figure 1) and (Figure 2). She was followed up at the High-Risk Prenatal Care Unit of the University of Brasília Hospital, together with the Endocrinology Team. On the eighth month of gestation, it was necessary to increase the number of nasal desmopressin puffs from three to seven per day (0.1–2.5 mg/ml); no other alterations were observed in the pre-natal period. A C-section delivery was performed at 39 weeks and 3 days, due to fetal tachysystole observed at cardiotocography. The child was born at term, with Apgar score of 8/8 and weighing 4,015 g. The anatomopathological assessment of the placenta did not evidence any alterations. After delivery, the desmopressin dose was reduced back to three puffs per day. In the post-delivery consultation, the patient was clinically stable.
Discussion
Diabetes insipidus is a rare condition in pregnancy that affects approximately two to four women per 100,000 pregnancies [1],[2],[3]. In the past 30 years, few cases have been reported; some cases probably go unnoticed because the symptoms are confused with those of a normal gestation [1],[4].
In pregnancy, there is a decrease in plasma osmolality of about 10 mOsm/kg, which in turn results from the adjustment of the thirst threshold [1]. The metabolic clearance of ADH increases by up to six times between the 8th and the 20th week of pregnancy. Furthermore, the placental syncytiotrophoblast plays a significant role in the production of vasopressinase (aminopeptidase), which degrades ADH. The action of this enzyme is directly proportional to the weight of the placenta. It increases gradually during gestation, reaching its peak in the third trimester, as does antidiuretic hormone (ADH) [3],[4].
The treatment of choice is desmopressin (DDAVP), an analog of ADH with a different N-terminal chain, which prevents it from being degraded by placental vasopressinase [2],[5]. It can be administered through several routes; the nasal route is preferred due to its rapid absorption and convenience [2],[5].
Conclusion
Gestational diabetes insipidus does not appear to entail serious complications throughout pregnancy. With early diagnosis, correct treatment, and good prenatal follow-up, the prognosis is favorable, leading to a pregnancy with low maternal and fetal morbimortality.
REFERENCES
1.
Chanson P, Salenave S. Diabetes insipidus and pregnancy. Ann Endocrinol (Paris) 2016;77(2):135–8. [CrossRef]
[Pubmed]
2.
Quigley J, Shelton C, Issa B, Sripada S. Diabetes insipidus in pregnancy. The Obstetrician & Gynaecologist 2018;20(1):41–8. [CrossRef]
3.
Marques P, Gunawardana K, Grossman A. Transient diabetes insipidus in pregnancy. Endocrinol Diabetes Metab Case Rep 2015;2015:150078. [CrossRef]
[Pubmed]
4.
Aleksandrov N, Audibert F, Bedard MJ, Mahone M, Goffinet F, Kadoch IJ. Gestational diabetes insipidus: A review of an underdiagnosed condition. J Obstet Gynaecol Can 2010;32(3):225–31. [CrossRef]
[Pubmed]
5.
Del Carpio-Orantes L. Transient central diabetes insipidus associated with pregnancy. Case report and bibliography review. [Article in Spanish ]. Rev Med Inst Mex Seguro Soc 2017;55(5):672–5.
[Pubmed]
SUPPORTING INFORMATION
Author Contributions
Ana Luiza Pessoa De Resende - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Paulo Sergio França - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Gustavo Henrique Soares Takano - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Florêncio Fiqueiredo Cavalcante Neto - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Guarantor of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2019 Ana Luiza Pessoa De Resende et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
