Objective To elucidate the features of clinicopathology and mutation in Fabry disease complicated with thin basement membrane nephropathy (TBMN), and to investigate the kindred. Methods Data of clinicopathology and gene mutation of a female patient of Fabry disease complicated with TBMN admitted to the Department of Nephro]ogy in our hospital were analyzed. Members of her kindred were investigated simultaneously. Results Proband was a 41-year-old Chinese woman who presented syndrome of Fabry disease and TBMN including angiokeratomas, chronic pain, tinnitus, vertigo, left ventricular hypertrophy and nephropathy as proteinuria, microscopic hematuria and hypertension. A percutaneous renal biopsy was performed on the proband, which was consistent with FSGS and vaculization of podocytes by light microscopy.Electron microscopy showed concentric lamellated inclusions in some podocytes. Diffuse thinning of glomerular basement membrane (GBM) with a mean thickness of (216±31) nm was found. The diagnosis of TBMN with suspected Fabry disease was identified. Family screening showed that her daughter had microscopic hematuria, tinnitus and neuropathic pain. One of her sisters only had microscopic hematuria. The activity of α-galacsidase A (α-Gal A )enzyme in the proband and her daughter was 33 units and 75 units respectively (the normal range is 100 to 500 units). They all carried the novel GLA mutation 1208 ins 21 bp and COL4A3 SNP c: 3627G＞A(p:M1209I). While her sister who only had microscopic hematuria just carried the variant of COL4A3 gene-c:3627G ＞A (p:M1209I), and had the normal activity of α-Gal A with no mutation of GLA.Conclusion TBMN should be considered in the patients of Fabry disease with the condition of benign familial hematuria.

Seventy-five preschool children with hemoglobin content below 11 g/dl in kindergartens and nurseries were divided into five groups. The first, second and third groups were given 10 mg, 20 mg and 30 mg of iron in the form of ferrous sulfate syrup respectively every day for two months, while the fourth and fifth groups were given 100 mg ascorbic acid and 100 mg ascorbic acid plus 10 mg iron respectively every day for one month. The average hemoglobin content in the order of the five groups were 9.6?1.16, 10.6?1.34, 9.7?0.91, 9.7?0.85 and 11.0?1.86 g/dl respectively before treatment. After 1-2 months of iron or ascorbic acid therapy, the average hemoglobin contents raised to 12.0?.31, 12.0?1.06, 12.0?0.79, 11.0?1.86 and 12.7?1.37 g/dl respectively. There were highly significant differences (P

Based on the theory of ascending and descending of center qi， it is believed that yang deficiency and water dampness， as well as abnormal circulation of center qi， are the core pathogenesis of psoriasis. The common pathogenic evolution of psoriasis includes wood constraint and blood stagnation， lung metal fluid exhaustion. In the later stage of psoriasis， a mixture of deficiency and excess patterns， as well as cold and heat in complexity， are often observed. Treatment focuses on warming yang and resolving dampness to restore proper qi ascending and descending， and the therapeutic formulas such as Ganjiang Lingzhu Decoction （甘姜苓术汤）， Zexie Decoction （泽泻汤）， Zhenwu Decoction （真武汤）， and Linggui Zhugan Decoction （苓桂术甘汤） are suggested based on the nature and location of the disease. To unblock the qi movement through discharging the liver and rectifying the lung， Xiaochaihu Decoction （小柴胡汤） and Danzhi Xiaoyao Powder （丹栀逍遥散） are commonly used as the foundation with modifications. To balance yin and yang through clearing the heart and nourishing the kidneys， a self-designed Changzhong Decoction （畅中汤） is utilized. Following the theory of ascending and descending of center qi， it is suggested to combine cold and warm medicinals and mediate the center earth， so as to promote the circulation of the center qi， restore the clear and the turbid， and keep four-dimensional smooth flow of qi， providing a reference for traditional Chinese medicine treatment for psoriasis.

Objective:To evaluate the clinical value of different shimming methods at 7.0 T MR in two-dimensional (2D) and three-dimensional (3D) T 2WI. Methods:Totally 23 healthy volunteers were prospectively recruited from the First Medical Center of PLA General Hospital from November, 2022 to May, 2023, including 12 volunteers who underwent 2D shimming mode and 14 volunteers who underwent 3D shimming mode. 2D shimming mode included patient-specific (PS) mode, direct signal control (DSC) mode, the standard circularly polarized (CP) mode, and volume-specific (VS) mode. 3D shimming mode included universal pulses (UP) mode and CP mode. The image quality for the subtentorial and supratentorial region was assessed by the subjective image quality score and signal-to-noise ratio. Comparisons of quantitative indices between multiple groups were performed using repeated-measures ANOVA or Friedman′s test; comparisons of quantitative indices between 2 groups were performed using paired-samples t test or Wilcoxon signed-rank test. Results:The image quality of subtentorial region and SNR was significant differences in 2D T 2WI with PS mode, DSC mode, CP mode and VS mode ( F=26.74, P<0.001; F=28.24, P<0.001), and the image quality score and SNR of PS mode, DSC mode, VS mode were better than CP mode ( P<0.05). In 2D T 2WI, there was no significant difference in image quality score and SNR of supratentorial region in PS mode, DSC mode, CP mode ( P>0.05). Besides, in 3D T 2WI, the image quality score for subtentorial and supratentorial region of UP mode were better than those of CP mode ( Z=-2.74, P=0.006; Z=-3.24, P=0.001); SNR of subtentorial region was significantly better in UP mode than those in CP mode ( t=3.49, P=0.004). But there was no significant difference in SNR of supratentorial region between the UP mode and CP mode in 3D T 2WI ( P>0.05). Conclusion:T 2WI with different shimming methods at 7.0 T MR can provide data support for the clinical application, which is helpful for the accurate diagnosis of patients with subtentorial lesions.

BACKGROUNDThe high blood homocysteine (Hcy) levels found in patients with hyperhomocysteinemia (HHcy) have been implicated in an increased risk of cardiovascular disease morbidity and mortality in end-stage renal disease (ESRD). This study investigated the association of HHcy with progression of IgA nephropathy.

METHODSWe analyzed 108 participants newly diagnosed with IgA nephropathy between August 2005 and August 2007 in the Department of Nephrology, Chinese People's Liberation Army General Hospital. The association between clinicopathological factors and the Hcy levels were analyzed by Logistic regression and those with ESRD risk were analyzed by Cox regression.

RESULTSPatients were aged (35.71 ± 10.73) years and included 45.71% women and 12.04% patients with HHcy. In multivariate Logistic regression analysis, HHcy was associated with arterial lesions (OR 2.60; 95% CI 1.55 ± 4.34; P < 0.001) even when age, body mass index, estimated glomerular filtration rate, mean arterial pressure, and initial proteinuria were taken into account. Mean follow-up was (67.37 ± 16.21) months. HHcy was also associated with worse ESRD-free survival (HR 4.71; 95% CI 1.45 to 15.31; P = 0.010).

CONCLUSIONHHcy is associated with the risk of intrarenal arterial lesions and may be useful for estimating the prognosis of IgA nephropathy.

Adult ; Body Mass Index ; Female ; Glomerular Filtration Rate ; physiology ; Glomerulonephritis, IGA ; etiology ; physiopathology ; Humans ; Hyperhomocysteinemia ; complications ; physiopathology ; Logistic Models ; Male ; Middle Aged ; Retrospective Studies