Lipoprotein glomerulopathy with markedly increased arterial stiffness successfully treated with a combination of fenofibrate and losartan: a case report.

BACKGROUND: Lipoprotein glomerulopathy (LPG) is a apolipoprotein E (ApoE)-related glomerular disease and has been associated with type III hyperlipidemia. Without appropriate treatment, chronic kidney disease (CKD) caused by LPG progresses, and approximately half of the patients develop end-stage kidney disease within 1-27 years of disease onset. However, few studies have highlighted the clinical course of cardiovascular diseases (CVDs) in patients with LPG. Herein, we report the first case of LPG in which the CVD risk was assessed using arterial stiffness. CASE PRESENTATION: A 32-year-old Japanese man was referred to our hospital due to persistent proteinuria. Kidney biopsy showed markedly dilated capillary lumens containing pale-stained thrombi, which stained positively with Oil Red O. Electron microscopy revealed the presence of thrombi in the capillary lumen with low electron density and vacuoles of various sizes in part of the thrombi. Toluidine blue and Sudan IV stains were used to stain the thin sections of Epon-embedded tissue samples for electron microscopy. Sudan IV-positive droplets were observed in the capillary lumens, vascular walls, and cytoplasm of tubular cells. Increased serum ApoE concentration was observed. Liquid chromatography-tandem mass spectrometry of laser-microdissected glomeruli from paraffin sections revealed an increase in ApoE. Direct deoxyribonucleic acid sequencing of ApoE revealed a heterozygous ApoE Sendai mutation (Arg145Pro). The patient was finally diagnosed with LPG with heterozygosity for ApoE-Sendai mutation (Arg145Pro). Notably, at the time of diagnosis, he had markedly increased arterial stiffness for his age. Arterial stiffness was measured using brachial-ankle pulse wave velocity (baPWV), which was equivalent to that of a 56-year-old man. After three months of treatment with fenofibrate and losartan, a significant reduction in proteinuria was achieved along with an improvement in baPWV. Furthermore, these effects were maintained despite the lack of decrease in serum ApoE levels. CONCLUSION: Herein, we report the case of a patient with LPG with markedly increased arterial stiffness at the time of diagnosis, in whom combination therapy with fenofibrate and losartan successfully improved proteinuria and arterial stiffness. To the best of our knowledge, this is the first case report of LPG in which CVD risk was assessed using arterial stiffness.

[A Case of Concentrated Ascites Reinfusion Therapy(CART)for Abundant Refractory Ascites in Cecal Cancer with Peritoneal Dissemination Followed by the Readministration of Chemotherapy].

A 66-year-old man underwent laparoscopic ileocecal resection for cecal cancer with liver metastasis(cT3N1M1a, cStage Ⅳa). One month later, combination chemotherapy with capecitabine, oxaliplatin, and bevacizumab was administered for liver metastasis. However, during the treatment, peritoneal dissemination and abundant diuretic-resistant ascites was revealed, resulting in poor dietary intake. One year and 11 months after the surgery, the chemotherapy was interrupted and cell-free and concentrated ascites reinfusion therapy(CART)was undergone as palliative care. The initial volume of retrieved ascites was 6,500 mL, and the volume was increased gradually to a maximum of 14,020 mL without hemodynamic instability. Totally CART was administered 10 times during 7 months without any complications: mean volume of retrieved ascites; 9,780 mL/unit, the interval between therapies; 2-3 weeks. Serum albumin level did not decrease since CART administration. His oral intake and daily activities were improved by CART. These clinical outcomes contributed to the readministration of chemotherapy. We present a recent case of safe and periodical CART for abundant refractory ascites in cecal cancer with peritoneal dissemination, resulting in the improvement of QOL and the readministration of chemotherapy.

Different Clinical Courses of Nephronophthisis in Dizygotic Twins.

Siblings with nephronophthisis occasionally show different clinical courses; however, the reasons for this remain unclear. We herein report cases of nephronophthisis in a pair of dizygotic twins with different clinical courses. The brother developed end-stage kidney disease at 17 years old; however, his sister did not show kidney insufficiency. Kidney biopsies revealed severe tubulointerstitial damage at 14 and 22 years old in the brother and sister, respectively. Both had a homozygous NPHP1 deletion with different heterozygous mutations related to hereditary cystic kidney disease. Since the dizygotic twins were exposed to similar environmental factors, genetic factors may have influenced their clinical course more strongly than environmental factors.

[A Long-Term Survival Case of HER2-Positive Gastric Cancer with Distant Lymph Node Metastasis Receiving Multidisciplinary Therapy].

An 82-year-old man presented with right cervical swelling. Cervical ultrasonography revealed several swollen lymph nodes which were diagnosed with adenocarcinoma by fine needle aspiration cytology. Computed tomography showed right axillary lymph nodes were also swelling. Upper and lower gastrointestinal endoscopy found type 0-Ⅱa gastric cancer located at the posterior wall of the middle region. Pathology was HER2-positive moderately differentiated tubular adenocarcinoma. Doublet chemotherapy with S-1 and cisplatin was administered for unresectable gastric cancer(cT1bN0M1, cStage Ⅳb). One month later, doublet chemotherapy was changed to triplet chemotherapy with trastuzumab, capecitabine, and cisplatin. A month later, complete response(CR)was achieved. After 8 courses of triplet chemotherapy, we changed to doublet chemotherapy with trastuzumab and capecitabine due to impaired kidney function 8 months. Two months later from that, endoscopic mucosal dissection was performed for gastric cancer as local therapy(pathology: well differentiated tubular adenocarcinoma, pT1a, ly0, v0). Two years and 2 months after the beginning of chemotherapy, the right axillary lymph nodes were enlarged again and surgically resected(pathology: HER2-positive poorly differentiated adenocarcinoma). He had CR for 8 years and 2 months, and chemotherapy was canceled due to his decision. During 1 year and 7 months, disease progression was not observed. We present a long-term survival case of HER2-positive gastric cancer with distant lymph node metastasis receiving multidisciplinary therapy.

[A Case of Descending Colon Cancer with Long-Term Maintenance of QOL by Multidisciplinary Treatment Approach for Locally Recurrent Lesion with Hydronephrosis].

We report a case of recurrent descending colon cancer in which QOL was maintained for a long period by performing resection with intestinal reconstruction, chemotherapy, and radiotherapy for local recurrence with hydronephrosis. A man in his 60s with good ADL underwent laparoscopic left hemicolectomy for descending colon cancer. After 4.5 years postoperatively, computed tomography and positron emission tomography showed a local recurrence of 32 mm contacting with the left external iliac artery and sigmoid colon, and CAPOX plus BEV was started. When cholecystitis developed after 5 chemotherapy courses, the recurrent lesion was resected simultaneously. After 8 months, repeated recurrent lesion with a major axis of 13 mm with left hydronephrosis was observed at the same site. After 3 years of chemotherapy after placing the left ureteral stent, CEA level gradually increased, and tumor growth was observed. Because of the aggressive chemotherapy limitation due to high proteinuria, 66 Gy/22 Fr radiotherapy was performed. After 1 month of radiotherapy, the CEA level decreased and proteinuria improved in that period. Radiotherapy for local recurrence can be a useful interval for chemotherapy and effective local control.

[Postoperative Severe Dysphagia Due to Hypophosphatemia in a Patient with Peritoneal Dissemination of Gastric Cancer].

An 83-year-old man presented with melena and weight loss. Upper gastrointestinal endoscopy showed type 3 advanced gastric cancer with pyloric stenosis. Surgical findings revealed numerous peritoneal dissemination, then gastro-jejunum anastomosis was performed. The oral diet was resumed on POD4, however severe dysphagia occurred immediately on POD6. There were no specific findings on MRI/MRA and nasal endoscopy. Serum antibodies related to neuromuscular diseases and connective tissue diseases were also negative. Despite the rehabilitation, the dysphagia remained. Before total parenteral nutrition on POD16, hypophosphatemia was discovered(1.4 mg/dL). His dysphagia disappeared with the improvement in the serum phosphate level. Hypophosphatemia might be caused by an inadequate intake as urine phosphate, serum calcium and serum PTH levels were normal. We present a recent case of severe dysphagia due to hypophosphatemia in a patient with peritoneal dissemination of gastric cancer.

[Primary Biliary Cholangitis Diagnosed with Spontaneous Bacterial Peritonitis after Gastrectomy for Gastric Cancer-A Case Report].

Spontaneous bacterial peritonitis is defined as an ascitic fluid infection without an evident intra-abdominal surgically treatable source. The diagnosis is established by a positive ascitic fluid bacterial culture and an ascitic fluid absolute polymorphonuclear leukocyte(PMN)count≥250 cells/µL. Here we report the case of 81-year-old female patient who was diagnosed with spontaneous bacterial peritonitis after gastrectomy for gastric cancer. The laparoscopic distal gastrectomy and D1+ lymph node dissection were performed for Stage Ⅰ gastric cancer, and the postoperative course was uneventful. The patient presented with abdominal pain and was hospitalized again on the third day from the discharge. Computed tomography showed an accumulation of ascites, and the ascitic fluid polymorphonuclear leukocyte count was 9,973 cells/µL. The patient was diagnosed with spontaneous bacterial peritonitis, and antibacterial agent was performed. Abdominal pain and accumulation of ascites had been improved, and the ascitic fluid polymorphonuclear leukocyte count had decreased clearly. The patient discharged on the 57th day from the operation. Spontaneous bacterial peritonitis after gastrectomy for gastric cancer was rare. We report this rare case, along with a discussion of the literature.

[A Case of Portal Vein Thrombosis Found during Postoperative Adjuvant Chemotherapy(CAPOX)after Laparoscopic Surgery for Rectal Cancer].

Portal vein thrombosis after laparoscopic colorectal cancer surgery is rare and sometimes lethal. We report a case of asymptomatic portal vein thrombosis found during postoperative adjuvant chemotherapy(CAPOX)after laparoscopic surgery for rectal cancer. A male patient in his 60s underwent postoperative adjuvant chemotherapy( CAPOX). The elevation of liver enzyme before the chemotherapy was moderate enough to start. The liver enzyme was increased mildly during the chemotherapy. Computed tomography 27 weeks after the operation revealed the thrombus from the main portal vein to the right branch and posterior branch, and atrophy of the lateral segment with narrowed left branch. Blood flow was confirmed to be maintained by ultrasonic Doppler. We decided to discontinue the chemotherapy and started anticoagulant therapy with Warfarin. Thrombosis was disappeared 2 weeks later, and liver function went back to normal range after 8 weeks. Liver dysfunction during chemotherapy should be noted not only for drug-induced liver damage, but also for the possibility of postoperative asymptomatic portal vein thrombosis.

Plasma exchange combined with bortezomib-based chemotherapy is effective for early renal recovery in a patient with IgD-λ type multiple myeloma.

The immunoglobulin (Ig) D type is a rare variant of multiple myeloma (MM), that accounts for 1-2% of all cases. Compared to the more common types of MM, IgD MM is known to have more severe symptoms at presentation, and a poorer prognosis. A woman was admitted to our hospital for severe acute kidney disease and disorder (AKD) and back pain, and was started on hemodialysis. The renal biopsy revealed light chain cast nephropathy. She was diagnosed with IgD-λ MM based on Bence-Jones protein expression and high IgD serum levels, and started bortezomib therapy with plasma exchange (PE). After three sessions of PE, the serum free light chain levels decreased by 92%, and she was withdrawn from dialysis. The patient underwent autologous transplantation and is still in remission, demonstrating the benefits of a bortezomib-based regimen in combination with PE for IgD MM with AKD.

Maternally inherited diabetes and deafness complicated by mesangial galactose-deficient IgA1 deposits: a case report.

BACKGROUND: Maternally inherited diabetes and deafness (MIDD), a mitochondrial genetic disorder, typically affects the kidneys and results in end-stage renal disease. Early diagnosis of MIDD is challenging when renal manifestations precede other key clinical features such as diabetes and deafness and/or when the disease is complicated by other renal pathologies. CASE PRESENTATION: Here, we present the case of a 33-year-old Japanese woman who had initially been diagnosed with IgA nephropathy but was found to have MIDD 6 years later. Two renal biopsies were conducted six years apart. While assessment of the first biopsy specimen with the monoclonal antibody (KM55) revealed mesangial IgA deposits (containing the galactose-deficient IgA1 variant [Gd-IgA1]), examination of the second specimen showed no mesangial IgA deposits and newly-developed glomerular global scleroses and tubular damage. Granular swollen epithelial cells (GSECs), characterised by abnormal mitochondria, were observed among the tubules and collecting ducts in both biopsy specimens. Mitochondrial DNA analysis revealed an m.3243A > G mutation. CONCLUSIONS: We rediscovered the usefulness of GSECs as a pathologically distinctive feature of mitochondrial nephropathy and reviewed the literature regarding MIDD complicated by mesangial IgA deposition. Furthermore, we demonstrate that the mesangial IgA deposits in this patient consisted of the galactose-deficient IgA1 variant. The monoclonal antibody (KM55) might be a useful tool to distinguish IgAN from latent IgA deposits.

Ischemia/reperfusion of unilateral kidney exaggerates aging-induced damage to the heart and contralateral kidney.

AIMS: We aimed to determine the impact of aging on ischemic acute kidney injury, especially in terms of the pathological mechanisms of kidney and heart crosstalk. METHOD: The effects of 45 min of unilateral ischemic reperfusion (IR) of the renal artery on the contralateral kidney and heart were histologically assessed in 7- and 40-week-old SD rats after 7 days. RESULTS: Glomerular sclerosis, interstitial fibrosis and numbers of ED1 cells were significantly increased in the contralateral kidneys of the 40-, but not the 7-week-old rats. The numbers of ED1 cells in the heart significantly and similarly increased in both groups, but reactive fibrosis after IR was significant only in the 40-week-old rats. The exaggerated profibrotic response induced by aging seemed to be closely associated with the increased number of ED1 cells in the affected area. CONCLUSION: Aging could play a major role in exaggerating the pathological processes of inflammation to fibrosis in remote organs including the heart and the nonischemic kidney after IR stimulation of the unilateral kidney.