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Thrombocytopenia with elevated serum IgA and renal disease

MedGen UID:
374149
•Concept ID:
C1839162
•
Disease or Syndrome
Synonym: Thrombocytopenia with Elevated Serum Iga and Renal Disease
 
Monarch Initiative: MONDO:0010744
OMIM®: 314000

Clinical features

From HPO
Hematuria
MedGen UID:
5488
•Concept ID:
C0018965
•
Disease or Syndrome
The presence of blood in the urine. Hematuria may be gross hematuria (visible to the naked eye) or microscopic hematuria (detected by dipstick or microscopic examination of the urine).
See: Feature record | Search on this feature
Thrombocytopenia
MedGen UID:
52737
•Concept ID:
C0040034
•
Disease or Syndrome
A reduction in the number of circulating thrombocytes.
See: Feature record | Search on this feature
Abnormal bleeding
MedGen UID:
264316
•Concept ID:
C1458140
•
Pathologic Function
An abnormal susceptibility to bleeding, often referred to as a bleeding diathesis. A bleeding diathesis may be related to vascular, platelet and coagulation defects.
See: Feature record | Search on this feature
Glomerulonephritis
MedGen UID:
6616
•Concept ID:
C0017658
•
Disease or Syndrome
Inflammation of the renal glomeruli.
See: Feature record | Search on this feature
Increased circulating IgA level
MedGen UID:
66800
•Concept ID:
C0239984
•
Finding
An abnormally increased level of immunoglobulin A in blood.
See: Feature record | Search on this feature
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Recent clinical studies

Etiology

Saliva IgM and IgA are a sensitive indicator of the humoral immune response to Escherichia coli O157 lipopolysaccharide in children with enteropathic hemolytic uremic syndrome.
Ludwig K, Grabhorn E, Bitzan M, Bobrowski C, Kemper MJ, Sobottka I, Laufs R, Karch H, Müller-Wiefel DE
Pediatr Res 2002 Aug;52(2):307-13. doi: 10.1203/00006450-200208000-00026. PMID: 12149511
Hemolytic uremic syndrome associated with immunoglobulin A nephropathy: a case report and review of cases of hemolytic uremic syndrome with glomerular disease.
Morita S, Sakai T, Okamoto N, Funabiki A, Okada Y, Hasegawa Y, Amano K, Yoshikawa N, Kasuga M
Intern Med 1999 Jun;38(6):495-9. doi: 10.2169/internalmedicine.38.495. PMID: 10411356
The role of Escherichia coli O 157 infections in the classical (enteropathic) haemolytic uraemic syndrome: results of a Central European, multicentre study.
Bitzan M, Ludwig K, Klemt M, König H, Büren J, Müller-Wiefel DE
Epidemiol Infect 1993 Apr;110(2):183-96. doi: 10.1017/s0950268800068102. PMID: 8472763Free PMC Article

Diagnosis

Staphylococcus Infection-Associated Glomerulonephritis in a Kidney Transplant Patient: Case Report.
Cascais de Sá D, Rodrigues L, Santos L, Romãozinho C, Macário F, Marinho C, Pratas J, Alves R, Figueiredo A
Transplant Proc 2018 Apr;50(3):853-856. doi: 10.1016/j.transproceed.2018.02.005. PMID: 29661452
Saliva IgM and IgA are a sensitive indicator of the humoral immune response to Escherichia coli O157 lipopolysaccharide in children with enteropathic hemolytic uremic syndrome.
Ludwig K, Grabhorn E, Bitzan M, Bobrowski C, Kemper MJ, Sobottka I, Laufs R, Karch H, Müller-Wiefel DE
Pediatr Res 2002 Aug;52(2):307-13. doi: 10.1203/00006450-200208000-00026. PMID: 12149511
De novo hemolytic uremic syndrome after kidney transplantation in patients treated with cyclosporine-sirolimus combination.
Langer RM, Van Buren CT, Katz SM, Kahan BD
Transplantation 2002 Mar 15;73(5):756-60. doi: 10.1097/00007890-200203150-00017. PMID: 11907423
IgA nephropathy and idiopathic thrombocytopenic purpura with splenectomy: a case report.
Morino M, Inami K, Shibuya A, Sasaki N
Pediatr Nephrol 1994 Jun;8(3):345-6. doi: 10.1007/BF00866358. PMID: 7917864
Renal hypertension in children.
Olson DL, Lieberman E
Pediatr Clin North Am 1976 Nov;23(4):795-805. doi: 10.1016/s0031-3955(16)33361-2. PMID: 995444

Therapy

Staphylococcus Infection-Associated Glomerulonephritis in a Kidney Transplant Patient: Case Report.
Cascais de Sá D, Rodrigues L, Santos L, Romãozinho C, Macário F, Marinho C, Pratas J, Alves R, Figueiredo A
Transplant Proc 2018 Apr;50(3):853-856. doi: 10.1016/j.transproceed.2018.02.005. PMID: 29661452
Saliva IgM and IgA are a sensitive indicator of the humoral immune response to Escherichia coli O157 lipopolysaccharide in children with enteropathic hemolytic uremic syndrome.
Ludwig K, Grabhorn E, Bitzan M, Bobrowski C, Kemper MJ, Sobottka I, Laufs R, Karch H, Müller-Wiefel DE
Pediatr Res 2002 Aug;52(2):307-13. doi: 10.1203/00006450-200208000-00026. PMID: 12149511
De novo hemolytic uremic syndrome after kidney transplantation in patients treated with cyclosporine-sirolimus combination.
Langer RM, Van Buren CT, Katz SM, Kahan BD
Transplantation 2002 Mar 15;73(5):756-60. doi: 10.1097/00007890-200203150-00017. PMID: 11907423
IgA nephropathy and idiopathic thrombocytopenic purpura with splenectomy: a case report.
Morino M, Inami K, Shibuya A, Sasaki N
Pediatr Nephrol 1994 Jun;8(3):345-6. doi: 10.1007/BF00866358. PMID: 7917864
Renal hypertension in children.
Olson DL, Lieberman E
Pediatr Clin North Am 1976 Nov;23(4):795-805. doi: 10.1016/s0031-3955(16)33361-2. PMID: 995444

Prognosis

Hemolytic uremic syndrome associated with immunoglobulin A nephropathy: a case report and review of cases of hemolytic uremic syndrome with glomerular disease.
Morita S, Sakai T, Okamoto N, Funabiki A, Okada Y, Hasegawa Y, Amano K, Yoshikawa N, Kasuga M
Intern Med 1999 Jun;38(6):495-9. doi: 10.2169/internalmedicine.38.495. PMID: 10411356
Renal hypertension in children.
Olson DL, Lieberman E
Pediatr Clin North Am 1976 Nov;23(4):795-805. doi: 10.1016/s0031-3955(16)33361-2. PMID: 995444

Clinical prediction guides

The role of Escherichia coli O 157 infections in the classical (enteropathic) haemolytic uraemic syndrome: results of a Central European, multicentre study.
Bitzan M, Ludwig K, Klemt M, König H, Büren J, Müller-Wiefel DE
Epidemiol Infect 1993 Apr;110(2):183-96. doi: 10.1017/s0950268800068102. PMID: 8472763Free PMC Article

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