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Volume 27, Number 7—July 2021
Research

Shiga Toxin–Associated Hemolytic Uremic Syndrome in Adults, France, 2009–2017

Benoît Travert1, Antoine Dossier1, Matthieu Jamme, Aurélie Cointe, Yahsou Delmas, Sandrine Malot, Alain Wynckel, Amélie Seguin, Claire Presne, Miguel Hie, Ygal Benhamou, David Ribes, Gabriel Choukroun, Steven Grangé, Alexandre Hertig, Emilie Cornec Le Gall, Lionel Galicier, Eric Daugas, Lila Bouadma, François-Xavier Weill, Elie Azoulay, Fadi Fakhouri, Agnès Veyradier, Stéphane Bonacorsi, Julien Hogan, Véronique Frémeaux-Bacchi, Eric Rondeau, Patricia Mariani-Kurkdjian, Paul CoppoComments to Author , and Centre de Référence des Microangiopathies Thrombotiques2
Author affiliations: Centre de Référence des Microangiopathies Thrombotiques, Paris, France (B. Travert, A. Dossier, M. Jamme, Y. Delmas, S. Malot, A. Wynckel, A. Seguin, C. Presne, M. Hie, Y. Benhamou, G. Choukroun, S. Grangé, A. Hertig, L. Galicier, E. Azoulay, F. Fakhouri, A. Veyradier, V. Frémeaux-Bacchi, E. Rondeau, P. Coppo); Université de Paris, Paris (B. Travert, A. Dossier, A. Cointe, L. Galicier, E. Daugas, L. Bouadma, E. Azoulay, A. Veyradier, S. Bonacorsi, J. Hogan, V. Frémeaux-Bacchi, P. Mariani-Kurkdjian); Hôpital Bichat—Claude Bernard, Paris (B. Travert, A. Dossier, E. Daugas, L. Bouadma); Sorbonne-Université, Paris (M. Jamme, M. Hie, A. Hertig, E. Rondeau, P. Coppo); Hôpital Tenon, Paris (M. Jamme, E. Rondeau); Hôpital Robert-Debré, Paris (A. Cointe, S. Bonacorsi, J. Hogan, P. Mariani-Kurkdjian); Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France (Y. Delmas); Hôpital Maison Blanche, Reims, France (A. Wynckel); Centre Hospitalier Universitaire de Caen, Caen, France (A. Seguin); Centre Hospitalier Universitaire d’Amiens, Amiens, France (C. Presne, G. Choukroun); Groupement Hospitalier Pitié-Salpêtrière, Paris (M. Hie, A. Hertig); Centre Hospitalier Universitaire de Rouen, Rouen, France (Y. Benhamou, S. Grangé); Centre Hospitalier Universitaire de Toulouse, Toulouse, France (D. Ribes); Centre Hospitalier Universitaire de Brest, Brest, France (E. Cornec-Le Gall); Hôpital Saint-Louis, Paris (L. Galicier, E. Azoulay); I; nstitut Pasteur, Paris (F.-X. Weill); Centre Hospitalier Universitaire de Nantes, Nantes, France (F. Fakhouri); Hôpital Lariboisière, Paris (A. Veyradier); Hôpital Européen Georges Pompidou, Paris (V. Frémeaux-Bacchi); Hôpital Saint Antoine, Paris (P. Coppo)

Main Article

Table 1

Characteristics of adults with Shiga toxin–associated hemolytic uremic syndrome, France, 2009–2017*

Characteristic Value
Median age, y (IQR)
 60.5 (47.00–71.00)
Sex
M 35 (36.5)
F
 61 (63.5)
Median age-weighted Charlson Comorbidity Index (IQR) 2.00 (1.00–4.25)
Tobacco use within previous 3 y
 12 (12.5)
>1 underlying condition 69 (71.9)
Cardiovascular disease 48 (50.0)
Arterial hypertension 38 (39.6)
Diabetes mellitus 12 (12.5)
Venous thromboembolic disease 11 (11.5)
Heart disease† 20 (20.8)
CKD‡ 15 (15.6)
History of kidney transplant 5 (5.2)
Stage 2 CKD 4 (4.2)
Stage 3 CKD 8 (8.3)
Stage 4 CKD 3 (3.1)
Digestive disorder§ 29 (30.2)
Gastrointestinal disorder 18 (18.8)
Biliopancreatic disorder 9 (9.4)
Hepatic disorder 4 (4.2)
Autoimmune or inflammatory disease¶ 11 (11.5)
Immunodeficiency 27 (28.1)
History of bone marrow or solid organ transplant# 8 (8.3)
Hematologic disease** 8 (8.3)
Active cancer†† 8 (8.3)
HIV‡‡ 3 (3.1)
Primary immunodeficiency§§ 2 (2.1)
Neuropsychiatric disorder¶¶
 18 (18.8)
Treatment
Immunosuppressive treatment 12 (12.5)
Corticosteroids 11 (11.5)
Calcineurin inhibitors 7 (7.3)
Azathioprine or mycophenolate mofetil 7 (7.3)

*Values are no. (%) patients except as indicated. CKD, chronic kidney disease; IQR, interquartile range. †8 patients had hypertensive disease, 4 had ischemic disease, 4 had hypertensive and ischemic disease, 2 had valvular cardiopathy, 1 had pulmonary hypertension, and 1 had unspecified heart disease. ‡According to Kidney Disease Improving Global Outcomes guidelines (15). §8 patients had gastric, small bowel, or colonic resection; 2 had history of bariatric surgery; 3 had chronic diarrhea from diverticulosis; 1 had graft-versus-host disease; 1 had colonic endometriosis; 1 had microscopic colitis; 1 had AA amyloidosis; 1 had neurovegetative disorder (1 each); 3 had recurrent pyogenic cholangitis; 1 had sclerosing cholangitis; 2 had a double kidney-pancreas transplantation; 3 had chronic pancreatitis; 2 had cirrhosis; 1 had history of liver transplant; and 1 had autoimmune hepatitis. ¶2 patients had mixed connective tissue disease, 1 had systemic sclerosis, 1 had sclerosing cholangitis, 1 had microscopic polyangiitis, 3 had type 1 diabetes, 1 had multiple sclerosis, and 2 had psoriasis. #3 patients had a history of kidney, 2 of double kidney–pancreas, 2 of bone marrow, and 1 of liver transplant. **1 patient had acute myeloid leukemia, 1 had chronic lymphocytic leukemia, 1 had Hodgkin’s lymphoma, 1 had clonal B-cell lymphocytosis, 1 had monoclonal gammopathy of undetermined significance, 1 had Waldenström’s disease, 1 had myeloproliferative disorder, and 1 had myelodysplastic syndrome. ††3 patients had breast cancer, 1 had metastatic lung cancer, 1 had a gastrointestinal stromal cell tumor, 1 had bladder cancer, 1 had cervical cancer, and 1 had gastric cancer. ‡‡3 patients had AIDS, including 2 patients who received HIV diagnoses during treatment. §§2 patients had hypogammaglobulinemia, including 1 patient who had ICF1 syndrome caused by a DNMT3b germinal mutation. ¶¶5 patients had stroke sequelae, 3 had Parkinson’s disease, 1 had multiple sclerosis, 4 had cognitive impairment (including 1 patient who had Korsakoff syndrome and 1 who had vascular dementia), 1 had epilepsy, 1 had chronic polyradiculoneuropathy, and 5 had major depressive or bipolar disorder.

Main Article

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1These first authors contributed equally to this article.

2Members of this group are listed at the end of this article.

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Page updated: June 16, 2021
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