Assessing fluid volume and determining outcomes of acute heart failure using plasma human atrial natriuretic peptide.

BACKGROUND: The post-dialysis plasma level of human atrial natriuretic peptide (hANP) reflects the fluid volume in patients on hemodialysis. The threshold hANP level is reportedly 100 pg/mL; however, the clinical usefulness of the threshold hANP level for volume control has not been sufficiently studied. METHODS: We conducted a single-center, retrospective, observational study that included 156 hemodialysis patients without atrial fibrillation. First, we examined the usefulness of the threshold hANP level (100 pg/mL) for predicting hypoxemia due to congestion in a short-term observational study from December 30, 2015 to January 5, 2016. Subsequently, we conducted a 5-year follow-up study wherein the outcomes were hospitalization due to acute heart failure (AHF), development of cardiovascular diseases (CVD), and all-cause death. Finally, we collected echocardiography data to investigate the relationship between cardiac function and hANP. RESULTS: Our short-term observational study showed that patients with an hANP level ≥ 100 pg/mL developed hypoxemia due to congestion (odds ratio, 3.52; 95% confidence interval, 1.06-11.71; P = 0.040). At the 5-year follow-up, patients with an hANP level ≥ 100 pg/mL had significantly higher rates of hospitalization due to AHF, CVD, and all-cause death based on the log-rank test (P = 0.003, P = 0.019, P < 0.001, respectively). Cardiac disfunctions were significantly associated with the high hANP level. CONCLUSIONS: The hANP level is indicative of both fluid volume and cardiac dysfunction. A threshold hANP level of 100 pg/mL can serve as a predictive marker for AHF and a practical indicator for volume control.

Aortic Angiosarcoma in Association with Endovascular Aneurysm Repair: Case Report and Review of the Literature.

BACKGROUND Primary aortic sarcoma often poses diagnostic challenges for pathologists and clinicians because of a very low incidence and controversy over nomenclature and definition. We report a case of aortic angiosarcoma in association with a graft. We also conducted a clinicopathological review of cases of primary aortic sarcomas associated with implanted grafts. CASE REPORT The patient was an 82-year-old woman. She underwent thoracic endovascular aneurysm repair (TEVAR) at age 78 because of an aneurysm in the descending aorta. Approximately 4 years after the TEVAR, computed tomography revealed a type II endoleak and expansion of the aneurysm. Her c-reactive protein level rose to 34 mg/dL, and Ga scintigraphy showed 67Ga accumulation at the aneurysm. She had fever up to 39°C, and a stent graft infection was suspected. Despite administration of antibiotics, her condition deteriorated, and she died. Postmortem examination identified epithelioid aortic angiosarcoma at the aorta with aneurysm repair and the graft, and the aortic angiosarcoma invaded the left lower lobe of the lung. CONCLUSIONS Our clinicopathological review revealed that the proper clinical diagnosis was very difficult owing to confusion of aortic sarcoma after the implantation with the infected graft, atypical endoleak, or pseudoaneurysm. The histological diagnosis was ambiguous because immunohistochemical and genetic studies were not adequately conducted. Overall prognosis of aortic sarcoma is poor as most patients die within a year, with no effective treatments. It is hoped that recent projects for genomic medicine will provide useful insights about the diagnosis and treatment of these cancers.

An Autopsy Case of Mesenteric Panniculitis with Massive Pleural Effusions.

BACKGROUND Mesenteric panniculitis (MP) is an idiopathic chronic inflammatory condition of the mesentery. The main symptoms include abdominal pain, abdominal distention, weight loss, fever, nausea, and vomiting. The patients also present with chylous ascites in 14% of the cases and chylous pleural effusion (CPE) in very rare occasions. Despite the previous view of excellent prognosis of MP, two recent papers reported several fatal cases. However, there are still only a few autopsy case reports that describe the macroscopic and histological details of MP cases. CASE REPORT The patient was an 81-year-old Japanese woman. She complained of edema of her lower legs and face, general fatigue, and dyspnea. She was overweight and had type 2 diabetes (T2D). Computerized tomography (CT) demonstrated massive bilateral pleural effusions, with mild pericardial effusion and mild ascites. There was no pulmonary, cardiac or hepatic condition to explain the effusions. However, MP was suspected based on her CT. She gradually deteriorated into respiratory failure. The autopsy revealed CPEs (left 1,300 mL, right 1,400 mL) and MP in the mesentery of the small intestine. Neither neoplasia nor inflammatory conditions other than MP were detected. CONCLUSIONS In rare occasions, patients with MP present with CPE or chylothorax. We thought that a possible mechanism of the CPEs was a diaphragmatic defect. We suspected that being overweight and T2D had an etiological relationship with MP in our patient's case. Adipose tissue of the mesentery is the main focus of MP. We believed that MP would be the best umbrella term of the many synonyms.

Kidney morphological parameters measured using noncontrast-enhanced steady-state free precession MRI with spatially selective inversion recovery pulse correlate with eGFR in patients with advanced CKD.

BACKGROUND: It is well known that atrophic renal changes are associated with chronic kidney disease (CKD) progression, but conventional diagnostic imaging methods such as noncontrast-enhanced computed tomography and magnetic resonance imaging (MRI) have been insufficient for precisely assessing kidney function because they cannot clearly distinguish between the medulla and cortex. Hence, here we used noncontrast-enhanced steady-state free precession (SSFP) MRI with a spatially selective inversion recovery (IR) pulse to improve visibility for renal corticomedullary differentiation and evaluated the association between morphological parameters and kidney function in patients with CKD. METHODS: Kidney corticomedullary contrast ratio, cortical and medullary areas, and minimal cortical thickness of 107 patients with CKD G1-G5 were measured using SSFP MRI with a spatially selective IR pulse and the association between these morphological parameters and kidney function were evaluated. RESULTS: Corticomedullary contrast ratio was significantly improved on SSFP MRI compared with conventional in-phase T1-weighted gradient-echo MRI and positively correlated with estimated glomerular filtration ratio (eGFR), raw eGFR, and 24-h creatinine clearance. The medullary and cortical areas and minimal cortical thickness also positively correlated with those of kidney functional markers and the age. In patients with CKD and diabetes mellitus (DM), the correlation coefficients between raw eGFR and morphological parameters were higher than those in patients without DM, while minimal cortical thickness was larger in CKD patients with DM with a raw eGFR ≥ 45 mL/min. CONCLUSION: Kidney morphological parameters measured with SSFP MRI were clearly correlated with kidney function in patients with CKD, including those with advanced kidney dysfunction.

Comparison of methods of steroid administration combined with tonsillectomy for IgA nephropathy patients.

BACKGROUND: IgA nephropathy (IgAN) is a chronic glomerular disease that causes end-stage renal disease in 20-40 % of patients within 20 years. The efficacy of tonsillectomy combined with steroid pulse (SP) administration (TSP) for clinical remission of IgAN has been reported. Particularly in Japan, TSP has been performed widely. However, the optimum method for steroid administration in TSP has not been established. METHODS: We retrospectively compared clinical remission in IgAN patients treated with tonsillectomy combined with two different steroid administration methods: (1) three courses of SP therapy and oral prednisolone administered on alternate days (group 3A; n = 25); and (2) one course of SP therapy and oral prednisolone administered on consecutive days (group 1C; n = 22). RESULTS: There was no significant difference in the clinical remission rates between the two groups at 12 (48.0 vs. 40.9 %, P = 0.77) and 24 months after starting treatment (68.0 vs. 72.7 %, P = 0.76) and at the final observation (76.0 vs. 81.8 %, P = 0.73). The mean period from starting treatment to remission of hematuria in group 3A was significantly shorter than that in group 1C (5.7 ± 4.4 vs. 9.9 ± 5.9 months, P = 0.03). Dyslipidemic patients treated for the first time with statin after the SP therapy were more present in group 3A at 24 months (P = 0.02). CONCLUSIONS: In IgAN patients, treatment of group 3A may be effective for inducing rapid remission of hematuria. Further studies are needed to establish an appropriate protocol for TSP.

Acute tubulointerstitial nephritis with multiple organ involvement including fatal adrenalitis: a case report with autopsy findings.

A 68-year-old woman with Alzheimer's disease developed renal dysfunction after starting carbamazepine for epilepsy. Although Ga-67 citrate scintigraphy strongly suggested interstitial nephritis, renal biopsy was not possible due to her overall state. At 61 days after admission, she died of unexplained shock. At autopsy, severe infiltration of T lymphocytes was noted, not only in the renal interstitium but also in the liver, lungs, and adrenal glands. Adrenal failure was a possible cause of shock. In carbamazepine-induced interstitial nephritis, multiple organ involvement including fatal adrenalitis should be considered.

Systemic capillary leak syndrome.

A 40-year-old woman was referred to our hospital with severe hypovolemic shock and anasarca. The laboratory findings showed marked hemoconcentration and a decrease in total serum protein with the presence of monoclonal IgG-lambda. She had had a similar episode of generalized edema 2 years previously. We diagnosed the patient as having typical systemic capillary leak syndrome (SCLS) and she improved gradually after infusion of albumin-containing fluid. SCLS is a very rare condition caused by unexplained episodic capillary hyperpermeability. Its treatment has remained largely supportive and the prognosis is generally poor. Awareness of SCLS is necessary for improvement of the outcome.