Pediatric early-phase anorexia nervosa without hypokalemia exhibiting acute tubular injury: a case report.

Anorexia nervosa can lead to kidney complications. Few studies reported kidney biopsy results in young adults, most of whom had chronic anorexia nervosa, and kidney biopsy findings in pediatric patients with early-phase anorexia nervosa are rarely reported. A 14-year-old girl who lost weight due to excessive exercise and reduced diet was admitted for kidney dysfunction. She was 147 cm tall and weighed 32.9 kg, with a body mass index of 15.2 kg/m2. She was 39 kg about a year earlier. Her heart rate and blood pressure were 30-40 beats/min and 108/68 mmHg, respectively. She had kidney dysfunction (estimated glomerular filtration rate, 56.6 mL/min/1.73 m2). Urine ß2-microglobulin was slightly elevated (393 µg/L), and percent tubular phosphate reabsorption was low (75.2%), suggesting tubular damage; however, hypokalemia was absent. Kidney dysfunction did not improve with fluid loading. Kidney biopsy revealed that all glomeruli were intact, with no vasculitis, interstitial inflammation or fibrosis on light microscopy. However, proximal tubular epithelial walls were flattened and the brush border was absent, suggesting acute tubular injury. Immunofluorescent staining was negative for immunoglobulins and complement proteins, and electron microscopy showed no significant electron-dense deposition. The patient's serum creatinine gradually declined, normalizing on the 17th day of admission. Unlike previous reports in young adults, kidney dysfunction was observed even in the absence of hypokalemia in the current pediatric patient with early-phase anorexia nervosa. Proximal tubular injury in early-phase anorexia nervosa may be caused by bradycardia without hypokalemia, leading to subsequent kidney dysfunction.

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.

Differences in the Clinical Characteristics of Kawasaki Disease Between Older and Younger Children (2015-2019): A Single-Center, Retrospective Study.

OBJECTIVE: The objective of this study was to investigate the differences in the clinical characteristics of Kawasaki disease between older and younger children. STUDY DESIGN: This retrospective study examined 405 children with Kawasaki disease admitted to Showa University Northern Yokohama Hospital between 2015 and 2019. RESULTS: Eligible patients were classified into the older (≥3.0 years of age, n = 169) and younger (<3.0 years of age, n = 236) groups. Skin rash was found in significantly fewer cases (112 [66.3%] vs 229 [97.0%], P < .001 in the younger group). Cervical lymphadenopathy was more common in older children (153 [90.5%] vs 165 [69.9%], P < .001) and in incomplete Kawasaki disease (3 or 4 findings) (34 [20.1%] vs 25 [10.6%], P = .0078). The diagnosis was more delayed in older children (median: 5.0 days vs 4.0 days, P = .003) than the younger group. Additionally, fever nonresponsive to a single intravenous immunoglobulin was more common, and the duration of fever was significantly longer in the older group (48 [28.4%] vs 47 [19.9%], P = .0479). CONCLUSIONS: Kawasaki disease should be suspected in children aged >3.0 years with cervical lymphadenopathy and fever, despite the absence of skin rash. Additionally, incomplete Kawasaki disease, fever unresolved by a single intravenous immunoglobulin infusion, and the tendency to delay treatment initiation are more common in children aged >3.0 years.

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.

Meningitis due to Enterobacter aerogenes in the community associated with congenital dermal sinus in a Japanese infant.

Congenital dermal sinus is associated with meningitis caused by atypical pathogens. Although nosocomial infections with Enterobacter aerogenes in limited settings have been reported, community-acquired infections associated with congenital dermal sinus are rarely observed. We present the first non-neonatal case of a 3-month-old boy with meningitis due to Enterobacter aerogenes associated with congenital dermal sinus. The patient visited our hospital with fever and a skin dimple with lumbosacral hemangioma. He was diagnosed with meningitis based on cerebrospinal fluid (CSF) examination, which showed a cell count of 5717/µL. Subsequently, antimicrobial therapy with meropenem, cefotaxime (CTX), and vancomycin was initiated. His fever subsided, and the number of CSF cells decreased. Magnetic resonance imaging was performed for the dimple of the lumbosacral region, revealing the congenital dermal sinus. Enterobacter aerogenes was isolated from CSF and stool cultures, and treatment was adjusted to CTX alone based on susceptibility testing. However, the CSF culture remained positive. Although CTX was effective, the response to treatment was partial, and a switch to meropenem was required to achieve negative CSF cultures. In conclusion, Enterobacter aerogenes, although atypical, can cause community-acquired meningitis associated with congenital dermal sinus. Consistent with previous reports, in this case, a hemangioma on the back led to the diagnosis of congenital dermal sinus. Hence, systemic examination, including the back, is important. In addition, use of a third-generation cephalosporin (e.g., CTX) may not negate the CSF culture, even if it is effective. Thus, a switch to another drug (e.g., carbapenem) may be required.

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.

Distribution of serum adiponectin isoforms in pediatric patients with steroid-sensitive nephrotic syndrome.

BACKGROUND: Serum adiponectin circulates in three multimeric isoforms: high-molecular-weight (HMW), middle-molecular-weight (MMW), and low-molecular-weight (LMW) isoforms. Potential change in the circulating adiponectin levels in patients with nephrotic syndrome (NS) remain unknown. This study aimed to assess the levels of total adiponectin and the distribution of its isoforms in pediatric patients with NS. METHODS: We sequentially measured total adiponectin and each adiponectin isoform levels at the onset of NS, initial remission, and during the remission period of the disease in 31 NS patients. We also calculated the ratios of HMW (%HMW), MMW (%MMW), and LMW (%LMW) to total adiponectin incuding 51 control subjects. RESULTS: The median of total serum adiponectin levels in patients were 36.7, 36.7, and 20.2 µg/mL at the onset, at initial remission, and during the remission period of NS, respectively. These values were significantly higher than those in control subjects. The median values of %HMW, %MMW, and %LMW values were 56.9/27.0/14.1 at the onset, 62.0/21.8/13.4 at the initial remission, and 58.1/21.7/17.5 at during the remission period of NS, respectively. Compared with control subjects, %HMW at initial remission and %MMW at the onset were high, and the %LMW values at the onset and at initial remission were low. CONCLUSIONS: In patients with NS, total serum adiponectin levels increase at the onset of the disease, and the ratio of adiponectin isoforms changes during the course of the disease. Further studies are needed to delineate the mechanisms between proteinuria and adiponectin isoforms change.

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.

Increased renal concentrating ability after long-term oral desmopressin lyophilisate treatment contributes to continued success for monosymptomatic nocturnal enuresis.

OBJECTIVES: To investigate renal concentrating ability after long-term fast-melting oral desmopressin lyophilisate treatment in children with monosymptomatic nocturnal enuresis. METHODS: The present retrospective study involved 58 children (43 boys, 15 girls; aged 6-12 years) with nocturnal enuresis receiving oral desmopressin lyophilisate. After treatment for 4 weeks with a complete response, patients were placed on a reduced dose of 120 µg on alternate days. Moring urine osmolality was measured using urine samples obtained after medication and non-medication dry nights. Patients who experienced ≥1 wet nights/month during alternate-day oral desmopressin lyophilisate treatment or within 6 months after its cessation were assigned to the relapse group, whereas those who experienced <1 wet night/month were assigned to the continued success group. RESULTS: The continued success and relapse groups included 41 and 17 patients, respectively. The mean duration of treatment was 18.5 and 18.3 months in the continued success group and relapse group, respectively. There was no significant difference in morning urine osmolality after medication nights between the continued success and relapse groups; however, morning urine osmolality after non-medication nights was significantly higher in the continued success group than in the relapse group (P < 0.0001). Similarly, nocturnal urine volume was significantly higher in the relapse group than in the continued success group (P = 0.046). CONCLUSIONS: These results suggest that patients receiving long-term oral desmopressin lyophilisate treatment develop increased nocturnal renal concentrating ability, which results in sustained dryness even after treatment cessation.

Optimal urinary protein-to-creatinine ratio as a renal biopsy criterion in children with asymptomatic proteinuria.

BACKGROUND: This retrospective case series aimed at exploring the optimal urinary protein-to-creatinine ratio (uP/uCr) cut-off value to determine the need for renal biopsy in pediatric patients with isolated asymptomatic proteinuria (ASP). METHODS: Data from 32 patients (16 boys, 16 girls) with persistent isolated ASP treated between January 2001 and September 2010 were analyzed. The uP/uCr cut-off value at which a renal biopsy is indicated was determined using the minimum p-value approach. An "optimal" cut-off value was selected to distinguish significant and non-significant glomerular changes. RESULTS: The minimum p-value approach using the χ2-test resulted in a peak uP/uCr of 0.5 g/g x Cr, which was then used to divide the patients into a low-proteinuria group and a high-proteinuria group. The proportion of significant glomerular changes was marginally higher (p = 0.097) in the high-proteinuria group than in the low-proteinuria group after adjustment for multiple tests. In addition, the number of patients with severe proteinuria at the most recent followup was higher in the high-proteinuria group than in the low-proteinuria group. CONCLUSION: The use of a uP/uCr >= 0.5 g/g x Cr may be a reasonable criterion for renal biopsy aimed at distinguishing renal outcomes in patients with persistent isolated ASP.

Posterior reversible encephalopathy syndrome with exercise-induced acute kidney injury in renal hypouricemia type 1.

Renal hypouricemia type 1 is caused by mutations in the SLC22A12 gene, whereas type 2 is caused by defects in the SLC2A9 gene. Although both subtypes predispose to exercise-induced acute kidney injury (EIAKI), posterior reversible encephalopathy syndrome (PRES) occurring with this disorder is an uncommon phenomenon that has only been reported to date in a patient with renal hypouricemia type 2. We describe a 13-year-old boy with renal hypouricemia type 1 (serum uric acid, 0.9 mg/dL) with a homozygous W258X mutation in the SLC22A12 gene, presenting with EIAKI and PRES. On admission, his body weight was 61 kg (11 kg above the dry weight), and blood pressure was 153/88 mmHg. Cranial magnetic resonance imaging revealed high-intensity areas in the cortical and subcortical white matter of the occipital lobe. After admission, the patient responded well to a combination of hemodialysis and intravenous nicardipine. This is the first case of concurrent PRES and EIAKI in a patient with renal hypouricemia type 1. We suggest that PRES is not due to severe hypouricemia caused by SLC2A9 mutation but is a manifestation of severe EIAKI associated with renal hypouricemia.