Delayed diagnosis and exacerbation of hyperlipidemia in idiopathic nephrotic syndrome in children during the COVID-19 pandemic.

BACKGROUND: During the coronavirus disease-2019 (COVID-19) pandemic, there was a lack of access to outpatient facilities for other diseases. Conversely, few studies have reported changes in clinical features of idiopathic nephrotic syndrome (INS) in children before and after the COVID-19 pandemic. METHODS: Thirty-two children with primary INS, who were admitted to four Showa University-affiliated hospitals between January 2017 and December 2022, were enrolled in this retrospective study. Children were divided according to the onset of INS into a post-COVID-19 group (onset in 2020-2022, n = 25) and a pre-COVID-19 group (onset in 2017-2019, n = 32). We compared the clinical characteristics and features of initial INS between two groups. RESULTS: In the post-COVID-19 group, these patients had interval between noticing symptoms of INS, such as edema and INS diagnosis was significantly longer (7 days versus 3.5 days; p = 0.0047), and had significantly raised serum LDL cholesterol levels at the time of INS diagnosis than in the pre-COVID-19 group (314 mg/dL versus 260 mg/dL; p = 0.028). Likewise, steroid-resistant nephrotic syndrome was significantly more common in the post-COVID-19 group [32% (n = 8) versus 6% (n = 2); p = 0.016]. A correlation analysis revealed a moderate positive correlation between the interval from symptom to diagnosis and LDL cholesterol (r = 0.460015, p = 0.0003). CONCLUSIONS: Children with INS after the COVID-19 pandemic showed a longer time between noticing symptoms of INS and diagnosis, increased serum LDL cholesterol and more steroid resistance than before the pandemic.

Antimicrobial Stewardship Program for Patients in the Hematological Department Receiving Carbapenem Therapy: A Single-Center and Interrupted Time Series Analysis.

As antibiotic resistance has become a global problem, the intervention of an antimicrobial stewardship team (AST) is warranted. In hematological disorders, infectious complications are crucial owing to abnormal neutrophil function and decreased cell-mediated immunity. Despite the widespread implementation of AST intervention, the effectiveness of stewardship practices for immunocompromised patients remains uncertain. We determined the effect of AST interventions on carbapenem therapy in the department of hematology. Patients admitted to the department and undergoing carbapenem therapy were enrolled. We compared carbapenem use between the pre-AST (April 2016-March 2018) and post-AST (April 2018-March 2021) periods. Factors associated with long-term carbapenem therapy were investigated. Overall, 515 episodes of carbapenem therapy in 264 patients in the department were evaluated. According to the interrupted time series analysis, the number of days of therapy decreased with AST intervention (ß = -0.263, p = 0.011). In multivariate analysis, predictive factors associated with long-term carbapenem therapy (>8 days) were outpatient onset, chronic obstructive pulmonary disease, acute myeloid leukemia, multiple myeloma, and infection with resistant bacteria (such as extended spectrum ß-lactamases and AmpC) (95% confidence interval, 1.030-2.818, 1.067-66.667, 1.057-2.782, 0.168-0.742, and 1.382-5.750, respectively). The AST intervention reduced carbapenem use in patients with hematological disorders.

Sleep features of nocturnal enuresis: relationship between rapid eye movement sleep latency prolongation and nocturnal enuresis.

Nocturnal enuresis, or bed wetting, is the involuntary urination during sleep. One of its causes is difficulty awakening during sleep, suggesting a relationship between Nocturnal enuresis (NE) and sleep. However, no studies have yet clarified the relationship between NE and sleep, and the effects of sleep structure in NE children are not yet known. Assuming that changes in sleep structure are related to NE, there would be a difference in sleep structure between days with and without NE. We measured the sleep electroencephalograms of 27 at home patients aged 6-16 years, evaluated the differences between days with and without NE, and examined the NE-associated sleep characteristics associated. The evaluation items were total sleep time, sleep efficiency, the ratio of rapid eye movement (REM) to non-REM sleep, REM sleep latency, and non-REM sleep latency. Factors influencing NE were examined by logistic regression analysis, with NE presence/absence as the dependent variable and each evaluation item as the independent variable. Given that 2-6 measurements were made for each patient, Generalized Estimating Equations was used in the analysis. NE positively correlated with prolonged REM sleep latency, but no significant differences were found in other sleep structures. A positive correlation exists between NE and prolonged REM sleep latency. Changes in sleep structure in the early stages of sleep may lead to increased nocturnal urine volume and increased NE frequency.

Comparison of wireless and wired alarm devices for nocturnal enuresis treatment.

BACKGROUND: In monosymptomatic nocturnal enuresis (MNE) treatment, enuretic alarm devices are the first recommended treatment option. This study aimed to compare retrospectively the effectiveness of wearable wireless and wired alarm devices for MNE treatment in children aged 6-14 years. METHODS: All children aged 6-16 with MNE who underwent alarm therapy as outpatients were included. A wired alarm device was used from 2012 to 2015, and a wireless alarm device was used from 2016 to 2019. The primary outcomes were the dropout rates during therapy and at last follow up. The full response(14 consecutive dry nights) and the partial response rate during therapy were also assessed. RESULTS: Of the 173 patients enrolled, 75 and 98 used a wired and a wireless alarm device, respectively. The dropout rate at the last visit was significantly lower in the wireless alarm group than that in the wired alarm group (6.1% vs. 20.0%; P = 0.006). The full response(FR) rate was significantly higher in the wireless alarm group than these in the wired alarm group at 4, 12, 24 weeks (4 weeks: 11.2% vs. 1.3%, P = 0.011; 12 weeks: 31.9% vs. 13.5%, P = 0.005; 24 weeks: 72.9% vs. 39.7%, P < 0.0001). CONCLUSIONS: Wireless alarm therapy for MNE had lower attrition rates and a higher rate of FR than wired alarm therapy.

Fecal impaction detected by imaging predicts recurrent urinary tract infection.

BACKGROUND: Few studies have evaluated the efficacy of ultrasonography (US) and abdominal radiography in assessing bladder and bowel dysfunction in children aged <24 months. We aimed to investigate the association between the risk of urinary tract infection (UTI) recurrence and fecal impaction using imaging findings. METHODS: The medical records of 121 children (aged <24 months) with initial febrile UTI (fUTI) who were admitted to the authors' institution from January 2004 to September 2019 were reviewed retrospectively. We evaluated the rectal diameters of children with suspected fecal impaction that were measured using transabdominal US, or the rectal diameters divided by the distance between the ischial spines that were measured using abdominal radiography. Based on previous reports, we defined fecal impaction as a transabdominal US score of >30 mm or an abdominal radiography score of >0.5. The definition of functional constipation was based on the child/adolescent Rome IV criteria - i.e., a maximum stool frequency of twice per week. RESULTS: The median age at initial fUTI diagnosis was 4 months. The occurrence of fecal impaction identified via imaging was significantly greater in patients with UTI recurrence than in those without recurrence: yes/no: 17/9 (65.4%) versus 35/60 (36.8%); P = 0.013. On the other hand, the occurrence rates of constipation based on stool frequency did not differ between the two groups. In multiple logistic analyses, fecal impaction detected via imaging was identified as an independent risk factor for fUTI recurrence. CONCLUSIONS: Fecal impaction observed via US and abdominal radiography may be useful in predicting the recurrence of fUTI in children.

Complete resolution of urinary incontinence with treatment improved the health-related quality of life of children with functional daytime urinary incontinence: a prospective study.

BACKGROUND: To assess the health-related quality of life (HRQOL) of children with daytime urinary incontinence (DUI) based on pre- and post-treatment self-reports and parent proxy-reports. METHODS: The study population comprised 117 children with at least one episode of DUI per week and their caregivers as well as 999 healthy children (control group). The Pediatric Quality of Life Inventory 4.0 (PedsQL) questionnaire was administered to assess the HRQOL of children. To assess the degree of improvement in HRQOL, we categorized children into two groups: group A achieved complete response (CR) to treatment within 12 months and group B did not achieve CR within 12 months. CR was defined as the complete resolution of symptoms or alleviation of symptoms to < 1 DUI episode/month. RESULTS: Valid responses were collected from 84 children [53 boys and 31 girls; mean age: 7.9 ± 1.5 years (range, 6-12)]. Sixty-two patients (73.8%) were classified into group A and 22 (26.1%) into group B. Based on self-reports, significant post-treatment improvement was observed in the scores of all PedsQL items (mean total score: 82.2 ± 11.3 vs. 87.2 ± 9.8; P = 0.003). Group A showed significant improvement in the scores of all PedsQL items after achievement of CR based on child self-reports; however, this was improvement not observed in group B. CONCLUSIONS: To the best of our knowledge, this is the first study to demonstrate the complete resolution of DUI with treatment for improving the HRQOL of these children.

Clinical features of, and association of bladder ultrasound and uroflowmetry with, overactive bladder recovery period in children.

BACKGROUND: Overactive bladder (OAB) is a symptomatic syndrome defined by urinary urgency, usually accompanied by increased urination frequency and nocturia, with or without urinary incontinence. The prevalence of pediatric OAB in 5-13 year olds is as high as 16.6%, but the pathophysiology and epidemiology have not been sufficiently elucidated. METHODS: We retrospectively reviewed medical records in 117 children with OAB aged between 5 and 15 years during the years 2012-2016. At initial presentation, abdominal ultrasound and uroflowmetry were performed, and behavioral modifications, such as timed voiding, and constipation therapy were initiated. If there was no response after 4 weeks, antimuscarinic treatment was added. We evaluated the clinical features of OAB and factors related to the recovery period, which was defined as the period from the start of behavioral modifications to cure. RESULTS: The average recovery period was 11.9 ± 9.73 months. There was no significant difference in the recovery period according to age, gender, percentage of urination frequency, nocturnal enuresis, or constipation. The recovery period was significantly shorter in the group with bladder wall thickness ≥5 mm than with bladder wall thickness <5 mm. Children with a tower-shaped curve on uroflowmetry had a significantly shorter recovery period than those with a bell-shaped curve. CONCLUSIONS: Bladder wall thickness and uroflow curve shape are related to the recovery period of pediatric OAB.

Rituximab-associated agranulocytosis in children with refractory idiopathic nephrotic syndrome: case series and review of literature.

BACKGROUND: Agranulocytosis has been reported as a delayed-onset complication of rituximab treatment. However, the exact incidence and risk factors of this complication in patients with nephrotic syndrome remain unknown. METHODS: Records of 213 rituximab treatments for 114 patients with refractory nephrotic syndrome between February 2006 and April 2013 were reviewed to identify episodes of agranulocytosis (defined as an absolute neutrophil count of <500 mm(3)). RESULTS: Eleven episodes of agranulocytosis were detected in 11 patients. Median time of onset of agranulocytosis was 66 days (range, 54-161 days) after rituximab treatment. Nine patients experienced acute infections and received antibiotics. All but one patient received granulocyte colony-stimulating factor. Agranulocytosis resolved in all cases within a median of 3 days. The incidence of agranulocytosis was 9.6% in total patients and 5.2% in all treatments. Median age of the 11 patients who developed agranulocytosis was 6.4 years at the first rituximab treatment, significantly younger than the median age of the 103 patients who did not (median, 12.5 years; P = 0.0009). Five patients received re-treatment with rituximab. No recurrence of agranulocytosis was observed in any patient. CONCLUSIONS: It is important to pay extra attention to this clinically serious delayed-onset complication as it may be accompanied by life-threatening infections such as sepsis. Further clinical studies are needed to clarify its pathogenesis.

[A case of focal segmental glomerulosclerosis with nephrotic syndrome after hematopoietic stem cell transplantatation for aplastic anemia].

We report a case of pediatric severe aplastic anemia (SAA), where the patient underwent allogenic bone marrow transplantation (BMT) from an HLA mismatched family donor and developed focal segmental glomerulosclerosis (FSGS). An 11-year-old girl, who had SAA, was admitted to our hospital in 200X. Complete remission was not attained after immunosuppressive therapy with rabbit-antithymocyte globulin, prednisolone (PSL), and cyclosporine A (CsA). Eight months after being diagnosed with SAA, she underwent an allogenic BMT from her mother. We used a combination of 2-Gy total body irradiation, fludarabine, and cyclophosphamide as a preparative regimen prior to the BMT. CsA and PSL were used as prophylaxis against GVHD. Since the BMT did not lead to successful engraftment, the patient required two peripheral blood stem cell transplantations (PBSCT). Engraftment was sustained and no acute or chronic GVHD was observed. Six months after the first BMT, she developed clinical nephrotic syndrome despite the continuous PSL and CsA treatments. Renal biopsy revealed a total of 12 glomeruli, one of which showed segmental sclerosis. Electron microscopy revealed diffuse effacement of the foot processes. These findings were consistent with FSGS, and she was treated with mycophenolate mofetil (MMF) in combination with PSL instead of CsA, which greatly reduced her proteinuria. In general, the most common type of nephropathy after HSCT is GVHD-related nephrotic syndrome, and the most common pathological finding is membranous nephropathy or minimal change. FSGS without GVHD after HSCT, such as that observed in our case, is rare. In this case, the renal damage appears to have been caused by the effect of circulating permeability factors with immunity change after HSCT. This case demonstrates the importance of renal biopsy as a guide to determine the extent of renal damage and as an aid to determine the possible response to therapy.