scholarly journals Membranous Nephropathy With Monoclonal IgM Lambda Deposits in a Patient With IgM Monoclonal Gammopathy: A Case Report

2021 ◽  
Vol 8 ◽  
Author(s):  
Go Hirose ◽  
Takahiro Uchida ◽  
Aki Kojima ◽  
Kentaro Sugisaki ◽  
Muneharu Yamada ◽  
...  
Keyword(s):  
Renal Biopsy ◽  
Membranous Nephropathy ◽  
Monoclonal Gammopathy ◽  
Rare Condition ◽  
Japanese Woman ◽  
Immunofluorescence Staining ◽  
First Case ◽  
Old Japanese ◽  
Repeat Renal Biopsy ◽  
Igm Deposits

We report a case of membranous nephropathy with monoclonal immunoglobulin (Ig)M lambda deposits in a patient with IgM monoclonal gammopathy, in whom histological changes were observed on repeat renal biopsy. A 72-year-old Japanese woman was referred to our hospital because of massive proteinuria. A prominent increase in monoclonal IgM lambda level was identified, and she was diagnosed as having IgM monoclonal gammopathy of undetermined significance. Renal biopsy showed glomerular subepithelial electron-dense deposits that were found to be granular deposits of IgM lambda but not kappa or IgG by immunofluorescence staining, resulting in a diagnosis of membranous nephropathy with monoclonal IgM deposits. The second biopsy, which was performed 2 years later because of exacerbation of her nephrotic syndrome, demonstrated less immunofluorescence staining of IgM, and dominant IgG2 deposition without light chain restriction. Interestingly, immunostaining for thrombospondin-type-1-domain-containing-7A was positive in both renal biopsy tissues, although the second biopsy showed clearly stronger immunoreactivity. The effect of steroid therapy was limited; however, rituximab treatment improved both the hematological and renal abnormalities. Solitary deposition of IgM in membranous nephropathy is a quite rare condition. To our knowledge, this is the first case of monoclonal gammopathy of renal significance presenting as membranous nephropathy with monoclonal IgM deposits, in which chronological immunohistochemical changes were observed and rituximab therapy was effective.

scholarly journals Tissue Hypoperfusion, Hypercoagulopathy, and Kidney and Liver Dysfunction after Ingestion of a Naphazoline-Containing Antiseptic

2017 ◽  
Vol 2017 ◽  
pp. 1-5
Author(s):  
Yuko Ono ◽  
Nozomi Ono ◽  
Kazuaki Shinohara
Keyword(s):  
Liver Dysfunction ◽  
Laboratory Data ◽  
Japanese Woman ◽  
Over The Counter ◽  
Tissue Hypoperfusion ◽  
First Case ◽  
Old Japanese ◽  
History Of ◽  
History Of Depression ◽  
Adrenergic Receptor Agonist

Naphazoline is a peripheral α2-adrenergic receptor agonist commonly used as a topical decongestant. In Japan, over-the-counter antiseptics often contain naphazoline to effect local hemostasis. We present the first case involving the development of hypercoagulopathy, with kidney and liver dysfunction, following a naphazoline overdose. A 22-year-old Japanese woman with a history of depression ingested 160 mL of a commercially available antiseptic containing 0.1% naphazoline. Three days later, she was brought to the emergency department because of general fatigue, nausea, and vomiting. Physical examination revealed cool, pale extremities. Laboratory data showed evidence of severe kidney and liver dysfunction (creatinine, 9.2 mg/dL; alanine aminotransferase, 2948 IU/L), hypercoagulation (D-dimers, 58.3 μg/mL), and thrombocytopenia (platelet count, 90,000/μL). After infusion of normal saline, intravenous administration of alprostadil, and hemodiafiltration, her organ function completely recovered. Because both the kidney and liver express α2-adrenergic receptors, their failure was likely associated with naphazoline overdose-induced hypoperfusion. The most plausible causes of hypercoagulation are peripheral low perfusion and subsequent microthrombus formation. This case illustrates that severe organ dysfunction can occur following over-the-counter antiseptic ingestion and serves as a caution for both drug manufacturers and healthcare professionals.

scholarly journals Sub-Tenon Injections of Triamcinolone Acetonide Had Limited Effect on Cystoid Macular Edema Secondary to Nanoparticle Albumin-Bound-Paclitaxel (Abraxane)

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Naoki Matsuoka ◽  
Hiruma Hasebe ◽  
Tetsuji Mayama ◽  
Takeo Fukuchi
Keyword(s):  
Macular Edema ◽  
Triamcinolone Acetonide ◽  
Cystoid Macular Edema ◽  
Treatment Options ◽  
Time Lag ◽  
Japanese Woman ◽  
First Case ◽  
Old Japanese ◽  
Long Term Follow Up ◽  
Near Future

Purpose. To report the first case of cystoid macular edema (CME) induced by nanoparticle albumin-bound- (nab-) paclitaxel treated with sub-Tenon injections of triamcinolone acetonide (STTA) with detailed long-term follow-up.Case. A 39-year-old Japanese woman with breast cancer presents with decreased vision in both eyes while receiving nab-paclitaxel. Two STTA treatments were administered for persistent CME in her right eye. Central retinal thickness (CRT) of the treated eye decreased after the first STTA, but there was no change after the second STTA. CRT of the other eye and bilateral visual acuity (VA) showed no change after each treatment. However, this patient experienced gradual recovery of visual function after nab-paclitaxel treatment was completed, 3 months after the second STTA. Improvements in VA and CRT did not overlap in time. Moreover, there was a big improvement time lag in VA between the eyes.Conclusion. Cessation of nab-paclitaxel could lead to resolution of CME more than STTA, although STTA had some effect. Since nab-paclitaxel has been recently approved for treating more types of malignancies, the number of the patients with this CME is expected to increase in the near future. Patients and physicians should understand this side effect and prepare for other treatment options.

scholarly journals A Case of Cornelia de Lange Syndrome: Difficulty in Prenatal Diagnosis

2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Tadatsugu Kinjo ◽  
Keiko Mekaru ◽  
Miyuki Nakada ◽  
Hayase Nitta ◽  
Hitoshi Masamoto ◽  
...  
Keyword(s):  
Body Weight ◽  
Prenatal Diagnosis ◽  
Ultrasound Examination ◽  
Fetal Mri ◽  
Rare Condition ◽  
Japanese Woman ◽  
Cornelia De Lange Syndrome ◽  
Intrauterine Fetal Death ◽  
Old Japanese ◽  
Cornelia De Lange

We report a case of Cornelia de Lange syndrome (CdLS) where prenatal diagnosis was not made even with major anomaly. A 33-year-old Japanese woman was referred to our institution at 23 weeks of gestation because of fetal forearm defect. Ultrasound examination revealed short forearms and short humeri and femurs (–2.1 SD). The fetal estimated body weight was 450 g (–1.3 SD). Fetal MRI at 26 weeks of gestation revealed short forearms and hypoplasty of hand fingers. Fetal growth restriction became evident thereafter, leading to intrauterine fetal death occurring at 29 weeks of gestation. A stillbirth baby was of 798 g in body weight and 33.0 cm in length. External examination showed a low hairline, synophrys, low-set ear, hypertrichosis, and smooth long philtrum with thin lips. The neck appeared short and broad. Finally, CdLS was diagnosed. The prenatal diagnosis might be possible as the arm findings were totally characteristic in a small fetus, regardless of whether an overhanging upper lip was identified. Because CdLS is a rare condition, it is important to consider its possibility as a part of differential diagnosis.

scholarly journals Preservation of renal function by intensive glycemic control

2018 ◽  
Vol 2018 ◽  
Author(s):  
Naoya Toriu ◽  
Masayuki Yamanouchi ◽  
Rikako Hiramatsu ◽  
Noriko Hayami ◽  
Junichi Hoshino ◽  
...  
Keyword(s):  
Blood Glucose ◽  
Glycemic Control ◽  
Renal Biopsy ◽  
Japanese Woman ◽  
Vascular Lesions ◽  
List Type ◽  
Intensive Glycemic Control ◽  
Old Japanese ◽  
Glomerular Lesions ◽  
Intensive Control

Summary We report the case of a 67-year-old Japanese woman with type 1 diabetes mellitus. At 47 years of age, her hemoglobin A1c (HbA1c) was 10.0%, and she had overt nephropathy. The first renal biopsy yielded a diagnosis of diabetic nephropathy. Intensive glycemic control was initiated and her HbA1c improved to 6.0%. Renal dysfunction showed no progression for 15 years. At 62 years of age, a second renal biopsy was performed. Glomerular lesions did not show progression but tubulointerstitial fibrosis and vascular lesions showed progression compared with the first biopsy. Intensive glycemic control can prevent the progression of glomerular lesions, but might not be effective for interstitial and vascular lesions. Learning points: Intensive control of blood glucose can prevent the progression of glomerular lesions. Intensive control of blood glucose may not be able to prevent progression of interstitial and vascular lesions. CSII reduces HbA1c without increasing the risk of hypoglycemia.

scholarly journals The first case report of polymerase proofreading-associated polyposis in POLD1 variant, c.1433G>A p.S478N, in Japan

2020 ◽  
Vol 50 (9) ◽  
pp. 1080-1083
Author(s):  
Tetsuya Ito ◽  
Tadashi Nomizu ◽  
Hidetaka Eguchi ◽  
Nao Kamae ◽  
Sariya Dechamethakun ◽  
...  
Keyword(s):  
Endometrial Cancer ◽  
Brain Tumours ◽  
Rare Condition ◽  
Missense Variant ◽  
Japanese Woman ◽  
Colorectal Carcinomas ◽  
Bilateral Oophorectomy ◽  
First Case ◽  
Increased Risk ◽  
Paternal Grandfather

Abstract Polymerase proofreading-associated polyposis, caused by germline variants in the exonuclease domains of POLD1 and POLE, is a dominantly inherited rare condition characterized by oligo-adenomatous polyposis and increased risk of colorectal cancer, endometrial cancer and brain tumours. We report the first Japanese case of polymerase proofreading-associated polyposis carrying a POLD1 variant. The proband was a Japanese woman who had undergone resections of early colorectal carcinomas repeatedly and a hysterectomy with bilateral oophorectomy for endometrial cancer, all of which were diagnosed within 2 years after the first colectomy at 49 year old. Colonoscopic examinations demonstrated at least 14 non-cancerous polypoid lesions, some of which were histologically confirmed to be adenoma. Multigene panel sequencing identified a missense variant in POLD1 (c.1433G>A). Although her relatives did not undergo genetic testing, her father and paternal grandfather died of brain tumours at 53 and ~30 years of age, respectively.

scholarly journals A Case of Sublingual Ranula That Responded Successfully to Localized Injection Treatment with OK-432 after Healing from Drug Induced Hypersensitivity Syndrome

2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
Kunio Yoshizawa ◽  
Akinori Moroi ◽  
Shuichi Kawashiri ◽  
Koichiro Ueki
Keyword(s):  
Oral Treatment ◽  
Hypersensitivity Syndrome ◽  
Treatment Method ◽  
Japanese Woman ◽  
Drug Induced ◽  
Diaminodiphenyl Sulfone ◽  
Injection Treatment ◽  
First Case ◽  
Old Japanese ◽  
Generalized Lymphadenopathy

A ranula is a mucus retention cyst or pseudocyst caused by leakage of mucus from the sublingual gland and generally occurs in the oral floor. In addition, drug induced hypersensitivity syndrome (DIHS) is a rare but well-recognized serious adverse effect characterized by fever, skin rashes, generalized lymphadenopathy, hepatitis, and hepatosplenomegaly and oral stomatitis. This paper presents the first case of successfully treated sublingual ranula with localized injection of OK-432 after healing from drug induced hypersensitivity syndrome, which has previously been unreported in the literature. We present the case of a 38-year-old Japanese woman with sublingual ranula that responded successfully to localized injection treatment with OK-432 after healing from drug induced hypersensitivity syndrome. She was affected with cutaneous myositis and interstitial lung disease when she was 26 years old. At the age 34 years, she received additional oral treatment of diaminodiphenyl-sulfone due to deterioration of the cutaneous myositis, which resulted in drug induced hypersensitivity syndrome (DIHS) with severe oral stomatitis. Local injection of OK-432 to the ranula may be a very safe and useful treatment method even if the patient has a history of drug allergy and has connective tissue disease such as cutaneous myositis.

scholarly journals Podocyte Infolding Glomerulopathy, First Case Report From North America

2021 ◽  
Vol 8 ◽  
pp. 205435812110483
Author(s):  
Julie Anne Ting ◽  
Wayne Hung ◽  
Susanna A. McRae ◽  
Sean J. Barbour ◽  
Michael Copland ◽  
...  
Keyword(s):  
Case Report ◽  
Autoimmune Disease ◽  
North America ◽  
Hepatitis B ◽  
Renal Biopsy ◽  
Membranous Nephropathy ◽  
Interval Change ◽  
Raas Blockade ◽  
First Case ◽  
Podocyte Infolding

Rationale: Podocyte infolding glomerulopathy (PIG) is a newly described condition with only 37 cases reported worldwide. Due to its rarity, the pathogenesis and evolution of this disease is unclear. This case report contributes to our collective knowledge about the clinical and histological progression of this disease. Presenting concerns of the patient: Over the course of a year, a 52-year-old Malaysian woman with no known prior medical history developed progressively worsening edema and other findings consistent with nephrotic syndrome. Diagnosis: Unlike most patients with PIG, this patient did not have any autoimmune disease. She was Hepatitis B core antibody positive with a Hepatitis B surface antibody >1000, suggesting prior Hepatitis B infection with immunity. A renal biopsy was performed which was consistent with PIG. A second renal biopsy was done 2 years later which again showed characteristic findings of PIG with worsened podocyte effacement but no interval change in chronicity. Interventions: The patient was treated with blood pressure control and renin-angiotensin-aldosterone system (RAAS) blockade with irbesartan and spironolactone. She was also treated with prednisone at 1 mg/kg for 2 months followed by a taper for a total of 7 months of prednisone treatment. Outcomes: The patient had a partial response to a course of prednisone. However, since stopping steroids, her proteinuria and renal function has been gradually worsening. Teaching points: PIG is mostly found in patients of East Asian descent. It presents as proteinuria and is often associated with autoimmune disease but can be idiopathic. It is characterized on renal biopsy by infolding or protrusion of podocyte cytoplasm into glomerular basement membrane, as well as intramembranous cytoplasmic microspherules or microtubules. Atypical membranous nephropathy should be ruled out prior to diagnosis. Unlike membranous nephropathy, PIG usually responds at least partially to steroid monotherapy. To our knowledge, this is the first reported case of PIG from North America. Furthermore, it is the first case of PIG with repeat biopsy showing interval worsening of PIG rather than either resolution of PIG or transformation of PIG to a different diagnosis.

scholarly journals Two Cases of Leiomyoma in the Colon Masquerading as Other Types of Colonic Pedunculated Polyps

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Ailee Ikeda ◽  
Masaya Iwamuro ◽  
Takehiro Tanaka ◽  
Toshihiro Inokuchi ◽  
Asuka Nakarai ◽  
...  
Keyword(s):  
Endoscopic Resection ◽  
Preoperative Diagnosis ◽  
Muscle Layer ◽  
Japanese Woman ◽  
Hepatic Flexure ◽  
Submucosal Tumors ◽  
Colonic Involvement ◽  
First Case ◽  
Old Japanese ◽  
Pedunculated Polyp

We describe two cases of leiomyoma in the colon that were diagnosed histologically after endoscopic resection. The first case was a 79-year-old Japanese woman who presented with a pedunculated polyp of 14 mm length at the splenic flexure. Preoperative diagnosis suggested a colonic mucosubmucosal elongated polyp. The second case was a 29-year-old Japanese woman who presented with a pedunculated polyp of 40 mm length at the hepatic flexure and had an ulcer on top of the polyp. Preoperative diagnosis suggested an inflammatory fibroid polyp. A pathological diagnosis of colonic leiomyoma was made after endoscopic resection in both cases. Both tumors were confirmed to originate, not from the proper muscle layer, but from the muscularis mucosae. These cases underscore that although colonic involvement is infrequent, leiomyomas can display pedunculated morphology in the colon rather than the typical gross appearance of gastrointestinal submucosal tumors seen with sessile morphology.

scholarly journals Light and heavy chain deposition revealed by repeat renal biopsy after inconclusive initial biopsy

2019 ◽  
Vol 5 ◽  
pp. 205930071984499 ◽  
Author(s):  
Ramy M Hanna ◽  
Marina Barsoum ◽  
Andrae Vandross ◽  
Jonathan Zuckerman ◽  
Brian Cone ◽  
...  
Keyword(s):  
Chronic Kidney Disease ◽  
Bone Marrow ◽  
Kidney Disease ◽  
Renal Disease ◽  
Renal Biopsy ◽  
Bone Marrow Biopsy ◽  
Immunoglobulin G ◽  
Monoclonal Gammopathy ◽  
Monoclonal Immunoglobulin ◽  
Repeat Renal Biopsy

Monoclonal gammopathy is a premalignant condition associated with an abnormal circulating immunoglobulin indicative of an expanded B cell clone. Apart from monitoring, no other intensive management is prescribed in these cases. Periodic bone marrow biopsies when free light chain imbalances are detected are used to spot incidental cases of multiple myeloma. New reports have suggested the existence of a monoclonal gammopathy of renal significance where a circulating antibody and normal bone marrow biopsy results may be associated with proteinuric renal disease due to monoclonal immunoglobulin deposition. We report a case of a 65-year-old male with an immunoglobulin G kappa monoclonal gammopathy of undetermined significance, chronic kidney disease, proteinuria, and an initial inconclusive renal biopsy. His chronic kidney disease worsened with persistence of 0.5 g of proteinuria. Given the finding of ongoing Bence Jones proteinuria, a repeat renal biopsy was done revealing monoclonal immunoglobulin G kappa deposition disease. Bone marrow biopsy showed 20% plasma cells that could be consistent with smoldering myeloma. The patient’s renal disease has stabilized after starting treatment for the monoclonal gammopathy of renal significance. This case illustrates the importance of renal biopsy in making the diagnosis of monoclonal immune deposition disease and monoclonal gammopathy of renal significance.

Severe Antithrombin III Deficiency in an Infant Associated with Multiple Arterial and Venous Thromboses

1976 ◽  
Vol 36 (03) ◽  
pp. 495-502 ◽  
Author(s):  
Geoffrey Mendelsohn ◽  
Edward D. Gomperts ◽  
Dennis Gurwitz
Keyword(s):  
Antithrombin Iii ◽  
Adult Life ◽  
Rare Condition ◽  
Male Infant ◽  
Liver Cells ◽  
Liver Pathology ◽  
Fixed Tissue ◽  
Formalin Fixed Tissue ◽  
First Case ◽  
At Iii

SummaryInherited antithrombin III (AT-II, heparin cofactor) deficiency is a rare condition, presenting with thrombotic disease in adult life. This paper reports an 8 months old South African Black male infant with multiple large vessel venous and arterial thromboses, and E. coli septicaemia. This was associated with an extremely low plasma AT-II level. Micronodular cirrhosis and intracytoplasmic hyaline globules in the liver cells were present. These globules were eosinophilic, and PAS-positive after diastase. They measured approximately 5 μ to 30 μ in diameter, occurred singly in the liver cells and were located mainly in the periportal areas. The histological findings in the liver are similar to those observed in α1-antitrypsin (AAT) deficiency in which the intracytoplasmic globules represent accumulation of altered AAT. Immunochemical studies carried out on formalin fixed tissue failed to detect cross reaction material with anti-α1 antitrypsin or anti-AT III antiserum. This is the first case report of AT-III deficiency presenting in infancy. It is also the first case associated with distinctive liver pathology.The available data presented are insufficient to distinguish between an inborn defect and acquired causes of the severely depressed AT-III plasma level and the distinctive liver pathology.

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