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Lupus nephritis (LN) is a common and severe manifestation of pediatric-onset systemic lupus erythematosus (pSLE). It is one of the major causes of long-term glucocorticoid/immune suppressants use in pSLE. It causes long-term glucocorticoid/immune suppressants use and even end-stage renal disease (ESRD) in pSLE. It is now well known that high chronicity, especially the tubulointerstitial components in the renal biopsy, predicts a poor renal outcome. Interstitial inflammation (II), a component of activity in LN pathology, can be an early predictor for the renal outcome. With the advent of 3D pathology and CD19-targeted CAR-T cell therapy in the 2020s, the present study focuses on detailed pathology and B cell expression in II. We recruited 48 pSLE patients with class III/IV LN to analyze the risk of ESRD based on different II scores. We also studied 3D renal pathology and immunofluorescence (IF) staining of CD3, 19, 20, and 138 in patients with a high II score but low chronicity. Those pSLE LN patients with II scores of 2 or 3 showed a higher risk for ESRD (p = 0.003) than those with II scores of 0 or 1. Excluding patients with chronicity >3, high II scores still carried a higher risk for ESRD (p = 0.005). Checking the average scores from the renal specimens from different depths, the II, and chronicity showed good consistency between 3D and 2D pathology (interclass correlation coefficient [ICC], II = 0.91, p = 0.0015; chronicity = 0.86, p = 0.024). However, the sum of tubular atrophy plus interstitial fibrosis showed no good consistency (ICC = 0.79, p = 0.071). The selected LN patients with negative CD19/20 IF stains showed scattered CD3 infiltration and a different IF pattern of Syndecan-1 expression. Our study provides unique data in LN, including 3D pathology and different in situ Syndecan-1 patterns in LN patients.

The elderly population is expanding rapidly, and that has become a major healthcare burden in terms of chronic kidney disease. The distribution patterns of kidney diseases in these elderly patients remain largely unclear. Here, we compared biopsy-based renal disease patterns between elderly and nonelderly patients. We performed a single-center, retrospective study (1992-2008) on biopsy-proven renal diseases to compare results between geriatric patients (age ≥ 65 years; n = 254) and nongeriatric patients (18 ≤ age 65 years; n = 2592). Renal pathology was interpreted by pathologists based on light microscopy, immunofluorescence, and electron microscopy. The ages of the geriatric and nongeriatric groups were 71.8 ± 4.5 (65.1-87.3) and 39.7 ± 17.6 (18-64.9) years, respectively, and 74% and 41% of them, respectively, were men. In the geriatric group, the most frequent diagnosis was membranous nephropathy (46.1%), followed by minimal change disease/focal segmental glomerulosclerosis (16.9%), diabetic nephropathy (8.3%), hypertensive nephrosclerosis (7.5%), and IgA nephropathy (5.9%). The geriatric group had more membranous nephropathy and less lupus nephritis and IgA nephropathy than the nongeriatric group. Furthermore, the 5-year survival rate of the geriatric group was significantly low. Our results demonstrated the different distributions of renal biopsy patterns in geriatric patients diagnosed with acute or chronic progressive kidney injury and proteinuria through renal biopsy.

BACKGROUND In recent years, the progress and generalization surrounding portable ultrasonic probes has made ultrasound a useful tool for physicians when making a diagnosis. With the advent of machine learning and deep learning, the development of a computer-aided diagnostic system for screening renal ultrasound abnormalities can assist general practitioners in early detection of pediatric kidney diseases. OBJECTIVE We therefore sought to evaluate the diagnostic performance of deep learning techniques to classify kidney images as normal and abnormal. METHODS We chose 330 normal and 1,269 abnormal pediatric renal ultrasound images for establishing a model for artificial intelligence (AI). The abnormal images involved hydronephrosis, cysts, stones, hyper-echogenicity and space occupying lesions. We performed pre-processing of the original images for subsequent machine learning. To classify both diseased and normal kidneys, support vector machine classifiers were built on the extracted features using transfer learning from a pre-trained deep learning model. The classifier and its diagnosis performance were measured using an area under the receiver operating characteristic curve (AUC), accuracy, specificity and sensitivity. RESULTS The deep learning model 94M parameters in size based on ResNet-50 was built for classifying normal and abnormal images. The accuracy of the validated images of normal, hydronephrosis, cyst, stone, hyperechogenicity and space occupying lesions were 90.9 %, 94.17%, 91.6%, 93.17%, 89.92% and 91.3%, respectively. CONCLUSIONS We have established the usefulness of a computer-aided model for automatic classification of pediatric renal ultrasound images in terms of normal and abnormal categories.

Background: Invasive candidiasis (IC) is a major cause of morbidities and mortality in patients hospitalized with major burns. This study investigated the incidence of IC in this specific population and analyzed the possible risk factors. Materials and Methods: We retrospectively analyzed data from the National Health Insurance Research Database (NHIRD) of Taiwan. We identified 3582 patients hospitalized with major burns on over 20% of their total body surface area (TBSA) during 2000–2013; we further analyzed possible risk factors. Result: IC was diagnosed in 452 hospitalized patients (12.6%) with major burns. In the multivariate analysis, patients older than 50 years (adjusted odds ratio (OR) = 1.96, 95% confidence interval (CI) 1.36–2.82), those of female sex (adjusted OR = 1.33, 95% CI 1.03–1.72), those with burns on the head (adjusted OR = 1.33, 95% CI 1.02–1.73), and those with burns over a greater TBSA had higher risks of IC. Conclusion: Treating IC is crucial in healthcare for major burns. Our study suggests that several risk factors are associated with IC in patients hospitalized with major burns, providing reliable reference value for clinical decisions.

Frasier syndrome is a rare disease that affects the kidneys and genitalia. Patients who have Frasier syndrome develop nephrotic syndrome (NS) featuring focal segmental glomerulosclerosis (FSGS) that is resistant to steroid treatment in early childhood. Male patients can have female external genitalia (pseudo-hermaphroditism) at birth and develop gonado-blastoma in their adolescence. Frasier syndrome is caused by mutations in the splice donor site at intron 9 of the Wilms’ tumor WT1 gene; these mutations result in an imbalanced ratio of WT1 protein isoforms and affect the development of the urogenital tract, podocyte function, and tumor suppression. Here, we report on a patient with long-term refractory NS who developed a malignant mixed germ cell tumor arising in a gonado-blastoma of the ovary 8 years after the onset of proteinuria.

Malignant atrophic papulosis (MAP), also known as Degos disease, is an extremely rare disease that is characterized by its unique skin presentation (namely, central, porcelain-white atrophic lesions with a telangiectatic rim). MAP has the following 2 variants: cutaneous MAP is manifested in the skin alone, whereas systemic MAP affects the gastrointestinal tract, central nervous system, lungs, and other internal organs. Some patients who presented with only cutaneous symptoms at first may develop systemic symptoms several years later. Although the exact pathologic mechanisms are unclear, Magro et al suggested in a recent study that MAP is a vascular injury syndrome that involves complement component C5b–9 complex deposition and high expression of interferon-α. The prognosis of systemic MAP is poor and typically fatal within a few years. Nonetheless, because the C5b–9 complex is detected in MAP, some researchers have suggested combined treatment with eculizumab (a humanized monoclonal antibody against C5) and treprostinil (a prostacyclin analog). Here, we report on a girl with systemic MAP who had severe central nervous system involvement and responded to eculizumab.

Background: Antipyretics are frequently used in pediatric practice. Both acetaminophen and nonsteroidal anti-inflammatory drugs (NSAIDs) have been reported to increase the risk of asthma exacerbation. The study investigated antipyretic use during respiratory infection in children and analyzed the risk of acetaminophen and NSAID for severe asthma exacerbation (AE) in asthmatic children in Taiwan. Methods: We used the data from the National Health Insurance Research Database in 2005. There were 27,095 pediatric asthmatic patients having at least one respiratory infection episode, and 27,095 age- and sex-matched non-asthmatic children with respiratory infection served as controls. These patients were divided into groups with acetaminophen use, NSAID cyclooxygenase-1 (COX-1) use, and no antipyretic use. The rate of AE occurrence within the first 7 days after respiratory infection diagnosis was compared among the groups. Results: During a single episode of respiratory infection, asthmatic patients used fewer antipyretics than controls (48.51% vs. 55.50%, p

As life expectancy increases in persons with hemophilia (PWH), more age-related diseases such as cancer emerge among this patient group. The aim of this study was to investigate incidence and survival of cancers among PWH in Taiwan. We analyzed data of 1,054 PWH retrieved from Taiwan's National Health Insurance Research Database between 1997 and 2010, by comparing variables to 10540 age- and gender-matched healthy individuals from the general population. There were 43 PWH and 178 individuals of general population with newly diagnosed cancer (RR 2.42, 95% CI 1.74–3.35). The cumulative incidences of cancer in PWH and the general population were 4.7 and 1.9%, respectively. Hepatocellular carcinoma (HCC) was the major type of cancer (17 cases) in PWH; cancer rate was still increased when HCC and HIV-related cancers were excluded (RR 1.66, 95% CI 1.06–2.59). There was no significant difference observed in lung, colorectal, or prostate cancer occurrence. Compared to the general population, PWH were younger at the time of cancer diagnosis (45.1 vs. 57.2 years old, P value