A digest of the Evidence-Based Clinical Practice Guideline for Nephrotic Syndrome 2020

Clinical and Experimental Nephrology -     

Inhaled nitric oxide therapy via nasopharyngeal tube in an infant with end-stage pulmonary hypertension

Abstract The delivery of nitric oxide (NO) via a nasopharyngeal tube is an alternative to endotracheal intubation. A male infant with end-stage pulmonary hypertension (PH) due to a severe hypoplastic lung developed a PH crisis on day 145 and received NO inhalation via a nasopharyngeal tube. Clinical improvement was maintained for 7 days with18–22ppm NO inhalation. The patient remained in close physical contact with his parents without the use of sedation. Blood methemoglobin levels remained below 1%. The environmental NO levels were less than 0.06 ppm and NO₂ less than 0.3 ppm throughout the treatment, well within the safety margin. On day 152, the patient succumbed to hypoxemia and heart failure. The use of a nasopharyngeal NO delivery system without sedation, as an alternative to endotracheal intubation with sedation, was a practical method in treating a patient with PH while maintaining a certain quality of life for the patient and the family.     

Contrast-enhanced high-resolution MRI of invasive breast cancer: correlation with histopathologic subtypes

OBJECTIVE: We sought to determine whether contrast-enhanced MRI could aid in the identification of the histopathologic subtypes of invasive ductal carcinoma. MATERIALS AND METHODS: We evaluated the contrast-enhanced MR images obtained in 62 women with invasive ductal carcinoma of no special type. The presence or absence of three distinct MRI findings-linear enhancement, a serrated border, and delayed rim enhancement-was evaluated. Classification and regression tree analyses were performed to construct the most efficient algorithm for predicting histopathologic subtype on the basis of dynamic MRI features. RESULTS: Histopathologic subtypes of the invasive ductal carcinomas were scirrhous carcinoma in 22 patients, solid tubular carcinoma in 14, and papillotubular carcinoma in 26. A lesion with a serrated border was observed in 28 (45.2%) of the 62 patients. Delayed rim enhancement was seen in 23 (37.1%) and linear enhancement in 20 (32.3%). Scirrhous carcinomas were closely associated with a serrated border (20/22 or 90.9%, p < 0.0001). Delayed rim enhancement was frequently observed in solid tubular carcinomas (12/14 or 85.7%, p < 0.0001) but was not typically seen in scirrhous carcinomas (1/22 or 4.5%, p < 0.0001). Linear enhancement showed relatively high prevalence in papillotubular carcinomas (13/26 or 50%) and low prevalence in solid tubular carcinomas (1/22 or 7%, p < 0.02). Histopathologic subtypes of invasive breast carcinoma of no special type could be correctly identified in 47 (75.8%) of 62 lesions using the diagnostic algorithm generated by the classification and regression tree analyses. CONCLUSION: MRI features showed a close relationship with histopathologic subtypes of invasive ductal carcinoma of no special type. Contrast-enhanced MRI can be a noninvasive diagnostic tool for histopathologic subtypes of invasive breast cancer.     

ACTN4 copy number increase as a predictive biomarker for chemoradiotherapy of locally advanced pancreatic cancer

Background:

Several clinical trials have compared chemotherapy alone and chemoradiotherapy (CRT) for locally advanced pancreatic cancer (LAPC) treatment. However, predictive biomarkers for optimal therapy of LAPC remain to be identified.We retrospectively estimated amplification of the ACTN4 gene to determine its usefulness as a predictive biomarker for LAPC.

Methods:

The copy number of ACTN4 in 91 biopsy specimens of LAPC before treatment was evaluated using fluorescence in situ hybridisation (FISH).

Results:

There were no statistically significant differences in overall survival (OS) or progression-free survival (PFS) of LAPC between patients treated with chemotherapy alone or with CRT. In a subgroup analysis of patients treated with CRT, patients with a copy number increase (CNI) of ACTN4 had a worse prognosis of OS than those with a normal copy number (NCN) of ACTN4 (P=0.0005, log-rank test). However, OS in the subgroup treated with chemotherapy alone was not significantly different between patients with a CNI and a NCN of ACTN4. In the patients with a NCN of ACTN4, the median survival time of PFS in CRT-treated patients was longer than that of patients treated with chemotherapy alone (P=0.049).

Conclusions:

The copy number of ACTN4 is a predictive biomarker for CRT of LAPC.     

Late gadolinium-enhanced magnetic resonance imaging in acute and chronic myocardial infarction. Improved prediction of regional myocardial contraction in the chronic state by measuring thickness of nonenhanced myocardium

OBJECTIVES: We sought to determine serial changes of enhanced and nonenhanced tissue on late gadolinium-enhanced cardiac magnetic resonance (CMR) imaging in patients with a myocardial infarction (MI) and to assess whether thickness of nonenhanced myocardium can improve the detection of preserved contractile function in the chronic state. BACKGROUND: Previous studies demonstrated that enhancement on late gadolinium-enhanced CMR images indicates myocardial necrosis, and nonenhancement shows the presence of viable myocardium. METHODS: The CMR studies were performed within one week (scan 1) and more than five months (scan 2) after the onset of MI in 18 patients. The area and mean thickness of enhanced tissue and nonenhanced myocardium were measured by using a 30-segment model. Systolic wall thickening on cine CMR at scan 2 was assessed for evaluating regional contractile function. RESULTS: The amount of enhanced tissue significantly decreased from scan 1 to 2 (22.1 +/- 14.0 ml vs. 15.0 +/- 9.3 ml, p < 0.001). The averaged thickness of nonenhanced myocardium in the infarct segments significantly increased from scan 1 to 2 (5.2 +/- 3.0 mm vs. 6.6 +/- 3.2 mm, p < 0.001). Receiver operating characteristic analysis demonstrated that the measurement of thickness of nonenhanced myocardium, compared with measurement of percent transmural enhancement, had better diagnostic accuracy for predicting improved systolic wall thickening form scan 1 to 2 in dysfunctional segments (Az 0.650 vs. 0.594, p < 0.05). CONCLUSIONS: The amounts of enhanced tissue and nonenhanced myocardium significantly altered from the acute to chronic state in MI patients. The diagnostic performance of CMR imaging for detection of preserved contractile function can be significantly improved by measuring thickness of nonenhanced myocardium in MI patients.     

Characterization of alkaline phosphatase in human urinary bladder carcinoma cell lines and enzyme regulation with prednisolone or sodium chloride

In order to characterize newly established human urinary bladder carcinoma cell lines (JTC-29, JTC-30, JTC-31, JTC-32, JTC-33 and JTC-34) by Kakuya et al. [In Vitro, 19, 591-599 (1983)], we investigated alkaline phosphatase in these cell lines. Relatively high alkaline phosphatase activity was found in JTC-29 and JTC-32 cells, but the enzyme activity in the other cell lines was very low. Prednisolone induced alkaline phosphatase activity even at 0.005 micrograms/ml (14 nM) and the effect was maximal at 0.05 micrograms/ml. NaCl also induced alkaline phosphatase activity at 50 mM. A further increase in the activity was observed at 0.1 M NaCl, but at 0.2 M all cells came off from plastic culture dishes without an increase in the enzyme activity during the incubation for 24 h at 37 degrees C. Both actinomycin D and cycloheximide abolished the increases in the enzyme activity with prednisolone or NaCl. The Lineweaver-Burk plot showed that the increases in the enzyme activity are due to the increases in Vmax, but not in Km. Alkaline phosphatase in JTC-32 cells and prednisolone-treated JTC-32 cells was heat stable and 100% of the initial enzyme activity remained after 30 min of incubation at 56 degrees C. However, the enzyme in JTC-29 cells was heat labile and its activity was reduced to less than 50% after 20 min of incubation at 56 degrees C. L-Phenylalanine was the strongest inhibitor for the enzyme reactions in JTC-32 cell homogenates among L-phenylalanine, L-leucine and L-homoarginine, whereas L-homoarginine was the strongest for the enzyme reactions in JTC-29 cell homogenates.(ABSTRACT TRUNCATED AT 250 WORDS)