Renal infarction in vascular Ehlers–Danlos syndrome masquerading as pyelonephritis

Dousa, Khalid M; Khan, Kashif; Alencherry, Ben; Deng, Lin; Salata, Robert A.

doi:10.1002/ccr3.1639

Cited by 3 publications

(2 citation statements)

References 12 publications

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“…She also underwent surgical interventions twice. Renal infarction could be present, but the association was previously established for vEDS [ 91 ]. Evidence supports the presence of gynecologic disorders, such as incontinence, endometriosis (also present in this patient), dyspareunia, etc., in EDS [ 92 ].…”

Section: Discussionmentioning

confidence: 99%

Ehlers-Danlos: A Literature Review and Case Report in a Colombian Woman with Multiple Comorbidities

Fajardo-Jiménez

Moreno

Mejía-García

et al. 2022

Genes

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Ehlers-Danlos syndromes (EDS) are a heterogeneous group of genetically transmitted connective tissue disorders that directly affect collagen synthesis, with a broad range of symptoms. Case presentation: This study presents a clinical case of a Colombian woman with myopathic EDS and multiple comorbidities taking 40 years of medical history to make the right diagnosis. This article also presents a review of the current literature on EDS, not only to remind the syndrome but also to help the clinician correctly identify symptoms of this diverse syndrome. Conclusion: A multidisciplinary approach to the diagnosis of the patient, including clinical and molecular analysis, and neuropsychological and psychological assessment, is important to improve the treatment choice and the outcome prediction of the patients.

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Section: Discussionmentioning

confidence: 99%

Ehlers-Danlos: A Literature Review and Case Report in a Colombian Woman with Multiple Comorbidities

Fajardo-Jiménez

Moreno

Mejía-García

et al. 2022

Genes

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“…A high frequency of esophagitis, bowel diseases and gastroduadenitis in connective tissue dysplasia has been revealed. Nephraptoses and kidney dystopathies most often exhibit changes from the urinary system in children with CTD [5]. Significant severity of bone mineral density reduction is observed in children with CTD.…”

Section: Introductionmentioning

confidence: 99%

The Results of Laboratory Studies of Connective Tissue Dysplasia in Children Living in Yakutia

Nokhsorova¹,

Борисова²,

Ammosova³

2022

Proceedings of the Conference on Health and Wellbeing in Modern Society (CHW 2021)

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Visceral disorders of various organs and systems in children with connective tissue dysplasia (CTD) living in extreme climatogeographic conditions according to laboratory tests have been studied. The children (35 schoolchildren) aged 10 to 17 years old were divided into mild, moderate and pronounced CTD degrees. Selection of children was performed by CTD phenotypic signs with score assessment and definition of CTD severity. The results of laboratory studies revealed more than 90 % cardiovascular system disorders in children with CTD, about 85 % of digestive system disorders and 70 % of visual disorders, 50 % of urinary disorders were detected in half of the children, mineral metabolism was impaired in 44 % of the children. The connective tissue dysplasia in 70 % of the children causes changes of 2 or more systems and organs, and in half (55 %) causes 3 or more systems and organs disorders. When examining children with EchoCG, almost the majority of Cardiovascular Development Abnormalities (CDA) were detected. In more than half of children, 65.8 %, i.e., 25 children were diagnosed with mitral valve prolapse (MVP). Mitral valve regurgitation was found in 28 children (73.8 %), MVP of the 1st degree was noted in half of children with CTD, and the second degree was noted in 16 % of the children and adolescents. In ten children (26 %), abnormally located chords (ALC) were diagnosed, 13 children (34 %) showed additional trabecules. The compaction of the calices-pelvis system walls occupies a special place in children with CTD.

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Peripartum considerations and challenges in the management of type IV Ehlers-Danlos syndrome

et al. 2021

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Ehlers-Danlos syndrome (EDS) is an uncommon heterogenous autosomal dominant multisystemic connective tissue disorder, which may potentially present with life-threatening emergencies. The prevalence of EDS of all subtypes is from 1 in 10 000 to 1 in 20 000. Pregnancy in women with type IV EDS poses significant challenges, necessitating the care of multidisciplinary team. Potential complications of type IV EDS include arterial dissection, arterial aneurysm, visceral rupture, excessive bleeding, tissue fragility and delayed wound healing. We present an interesting case of a pregnant woman with type IV EDS who had previous cerebrovascular complications and had a successful delivery in our tertiary centre following a collaborative approach to her care.

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Renal infarction in vascular Ehlers–Danlos syndrome masquerading as pyelonephritis

Cited by 3 publications

References 12 publications

Ehlers-Danlos: A Literature Review and Case Report in a Colombian Woman with Multiple Comorbidities

Ehlers-Danlos: A Literature Review and Case Report in a Colombian Woman with Multiple Comorbidities

The Results of Laboratory Studies of Connective Tissue Dysplasia in Children Living in Yakutia

Peripartum considerations and challenges in the management of type IV Ehlers-Danlos syndrome

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