IgM nephropathy; can we still ignore it

Vanikar, Aruna V

doi:10.5812/nephropathol.9872

Cited by 17 publications

(25 citation statements)

References 28 publications

(36 reference statements)

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“…This is in accordance with our study (8.69%). Abnormal T-cell function with hyper functioning suppressor T-cells are believed to be responsible for this disease entity and approximately one third of the patients are steroid responders where as the remaining two thirds are steroid resistant or dependent [19] with a range of 0-52% being quoted by studies before 1988 [22] . This is not as per our study as we had a majority of cases with SDNS (65%) with 30% having SRNS.…”

Section: Discussionmentioning

confidence: 99%

“…IgMN is an important and rather neglected pathology responsible for renal morbidity in children and adults in developing countries as compared to developed nations with incidence of 2-18.5% of native biopsies [19] . This is in accordance with our study (8.69%).…”

Section: Discussionmentioning

confidence: 99%

“…Therapeutic trials including cell therapies targeting suppressor T-cells are required [19] . IgMN is treated in the same manner as MCD or FSGS.…”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Clinical Profile &Treatment Outcome in Children with IgM Nephropathy

Mutalik¹,

Pradhan²,

Satapathy³

2017

IOSR JDMS

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Clinical Profile &Treatment Outcome in Children with IgM Nephropathy

Mutalik¹,

Pradhan²,

Satapathy³

2017

IOSR JDMS

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“…[7][8][9] Women with hematuria have a favorable prognosis, and age appears to have no role as a prognostic indicator in this disease. 3 Our patient presented primarily with nonspecific complaints of epigastric discomfort and generalized malaise. Her abdominal imaging including direct endoscopic Electron microscopic study showing normal glomerular basement membrane, mild mesangial sclerosis, and segmental effacement of foot processes (electron microscopy, x 8000).…”

Section: Discussionmentioning

confidence: 99%

“…Little is known about the pathogenesis and treatment of this disease. 1,3 We describe a patient who presented with nonspecific symptoms of epigastric pain, nausea, and early satiety. Abdominal imaging and endoscopies were unremarkable.…”

Section: Introductionmentioning

confidence: 99%

Acute Cerebellar Thrombosis in an Adult Patient with IgM Nephropathy

Adike

Cherry

Awar

2015

Methodist DeBakey Cardiovascular Journal

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A 70-year-old female with a past medical history of hypertension, type 2 diabetes, and hypothyroidism presented with a 2-month history of epigastric pain associated with abdominal distention, nausea, and generalized malaise. She also noted facial and bilateral leg swelling. Despite reporting decreased appetite, she had gained approximately 20 pounds in 1 month. She denied hematuria, fevers, night sweats, joint pain, vomiting, diarrhea, or changes in stool color or caliber. Admission vital signs were within normal range. Physical examination was remarkable for a mildly distended abdomen without evidence of ascites or peritoneal signs. Her lungs were clear and she had no jugular venous distention. There was 3+ pretibial pitting edema in her lower extremities bilaterally. Periorbital edema was also noted.Laboratory tests showed a normal electrolyte panel with a blood urea nitrogen of 2 mg/dL and creatinine of 0.7 mg/dL. Liver function tests were within normal limits. Serum albumin and protein were significantly depressed at 1.1 and 4.1 g/dL, respectively. Computed tomography of the abdomen was only remarkable for diverticulosis. Urinalysis showed 3+ protein, < 1 red blood cells, and no casts, and 24-hour urine protein was significantly elevated at 6426 mg/vol. Total cholesterol was elevated at 246 mg/dL, with an elevated triglyceride of 254 mg/dL. Hemoglobin A1c was 6.7%. Autoimmune workup was negative and systemic lupus erythematosus was excluded. Complement C3 was 126 mg/dL (normal 85-200 mg/dL) and complement C4 was 24 mg/dL (normal 17-46 mg/dL). Viral panel including acute hepatitis panel, HIV, cytomegalovirus, and herpes simplex virus was also negative. Immunoglobulin panel was checked and showed an immunoglobulin A level of 172 mg/dL (normal 85-350), immunoglobulin M (IgM) level of 99 mg/dL (normal 60-300), and immunoglobulin G level of 193 mg/dL (normal 696-1488). Doppler analysis of the renal vasculature was unremarkable. For work-up of her abdominal pain, esophagogastroduodenoscopy showed a normal esophagus and gastritis with prominent gastric folds. Gastric mucosa showed no evidence of inflammation or dysplasia but was remarkable for edema and congestion. Colonoscopy revealed diverticulosis without diagnostic mucosal alteration. Ultimately, biopsy of the left kidney was performed. Renal pathology was remarkable for mesangial proliferation and mild segmental sclerosis on light microscopy (Figure 1), diffuse IgM deposits in the mesangial area on immunofluorescence with areas of focal C1q, and diffuse C4d deposits in the mesangium (Figure 2). No light chain deposits were identified. Electron microscopy was remarkable for effacement of visceral epithelial cells (Figure 3). C A S E R E P O R T AbstractIgM nephropathy is a relatively rare cause of idiopathic nephrotic syndrome.

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A novel MTX2 gene splice site variant resulting in exon skipping, causing the recently described mandibuloacral dysplasia progeroid syndrome

Doğan

Günay

Ada

et al. 2022

American J of Med Genetics Pt A

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Until recently, mandibuloacral dysplasia (MAD) with type A and type B lipodystrophy was the first to come to mind in the association of mandibular hypoplasia, lipodystrophy, and acro‐osteolysis. However, it has recently been added to the differential diagnosis of MAD, a newly defined syndrome, called MDPS. MDPS is a skeletal dysplasia characterized by postnatal growth retardation, hypotonia, generalized lipodystrophy, skin changes, progeroid traits, and dysmorphic facial features, including prominent eyes, long pinched nose, mandibular hypoplasia, and a small mouth. Biallelic null variants of the MTX2 gene are responsible for this syndrome. We performed whole‐exome sequencing (WES) in a 6‐year‐old patient with skeletal dysplasia. WES revealed a novel homozygous c.543+1G>T splice site variant in the MTX2 gene. We also extracted total RNA from peripheral blood and used reverse transcription‐polymerase chain reaction to generate cDNA. Sanger sequencing from cDNA showed that exon 8 of MTX2 was skipped. This study adds to the genetics and phenotype of MDPS and underlines the importance of comprehensive clinical and molecular research.

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IgM nephropathy; can we still ignore it

Cited by 17 publications

References 28 publications

Clinical Profile &Treatment Outcome in Children with IgM Nephropathy

Clinical Profile &Treatment Outcome in Children with IgM Nephropathy

Acute Cerebellar Thrombosis in an Adult Patient with IgM Nephropathy

A novel MTX2 gene splice site variant resulting in exon skipping, causing the recently described mandibuloacral dysplasia progeroid syndrome

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