Relapse of nephrotic syndrome with unusual thromboembolic event: A case report.

Key Clinical Message: Most children with nephrotic syndrome heal without any sequelae. However, rare life-threatening complications such as thromboembolism may occur in pediatric nephrotic syndrome and should be considered in those with a new-onset neurologic deficit. Abstract: The thromboembolism (TE) as a complication of nephrotic syndrome (NS) is rare and serious, and may involve renal, cerebral, pulmonary, or peripheral venous and/or arterial thrombosis. Here, we describe a 4.5-year-old male with a history of nephrotic syndrome, who developed hemorrhagic stroke in the territory of middle cerebral artery (MCA).

Acute Kidney Injury in Pediatric Patients with COVID-19; Clinical Features and Outcome.

INTRODUCTION: Renal disorders have been reported as the underlying cause as well as complications of critical COVID-19 in pediatric patients. The purpose of this study was to investigate the pattern of kidney involvement, particularly acute kidney injury (AKI), among pediatric patients with COVID-19. METHODS: In this prospective study, hospitalized pediatric patients with a clinical diagnosis of COVID-19 were enrolled. Demographic, clinical, and laboratory findings were collected and analyzed using a mixed method of qualitative and quantitative approaches and descriptive statistics. RESULTS: One hundred and eighty-seven patients, including 120 (64.2%) males and 67 (35.8%) females with COVID-19 with a median age (interquartile range) of 60 (24 to 114) months were enrolled in this study. Most patients (n = 108, 58.1%) had one or two underlying comorbidities, mainly malnutrition (77.4%), neurologic/learning disorders (21.4%), and malignancy (10.2%). According to the Kidney Disease Improving Global Outcomes (KDIGO) classification, AKI was detected in 38.5% of patients (stage 1: 55.6%, stage 2: 36.1%, and stage 3: 8.3%) at presentation or during hospitalization. Nine patients (4.8%) required hemodialysis and 16 (8.6%) eventually died. There was no significant association between AKI and admission to the pediatric intensive care unit (PICU) (P > .05), a multisystem inflammatory syndrome in children (MIS-C) (P > .05), comorbidities (P > .05), and mortality rate (P > .05). CONCLUSION: Kidneys are among the major organs affected by COVID-19. Although kidney abnormalities resolve in the majority of pediatric COVID-19 infections, particular attention should be paid to serum creatinine and electrolyte levels in patients affected by COVID-19, particularly children with a history of malnutrition and kidney disorders.  DOI: 10.52547/ijkd.7151.

Sphingosine phosphate lyase insufficiency syndrome: a systematic review.

BACKGROUND: Sphingosine-1-phosphate lyase insufficiency syndrome (SPLIS) or nephrotic syndrome type-14 is caused by biallelic mutations in SGPL1. Here, we conducted a systematic review to delineate the characteristics of SPLIS patients. METHODS: A literature search was performed in PubMed, Web of Science, and Scopus databases, and eligible studies were included. For all patients, demographic, clinical, laboratory, and molecular data were collected and analyzed. RESULTS: Fifty-five SPLIS patients (54.9% male, 45.1% female) were identified in 19 articles. Parental consanguinity and positive family history were reported in 70.9% and 52.7% of patients, respectively. Most patients (54.9%) primarily manifested within the first year of life, nearly half of whom survived, while all patients with a prenatal diagnosis of SPLIS (27.5%) died at a median [interquartile (IQR)] age of 2 (1.4-5.3) months (P = 0.003). The most prevalent clinical feature was endocrinopathies, including primary adrenal insufficiency (PAI) (71.2%) and hypothyroidism (32.7%). Kidney disorders (42, 80.8%) were mainly in the form of steroid-resistant nephrotic syndrome (SRNS) and progressed to end-stage kidney disease (ESKD) in 19 (36.5%) patients at a median (IQR) age of 6 (1.4-42.6) months. Among 30 different mutations in SGPL1, the most common was c.665G > A (p.Arg222Gln) in 11 (20%) patients. Twenty-six (49.1%) patients with available outcome were deceased at a median (IQR) age of 5 (1.5-30.5) months, mostly following ESKD (23%) or sepsis/septic shock (23%). CONCLUSION: In patients with PAI and/or SRNS, SGPL1 should be added to diagnostic genetic panels, which can provide an earlier diagnosis of SPLIS and prevention of ESKD and other life-threatening complications.

Serological response to one intradermal or intramuscular hepatitis B virus vaccine booster dose in human immunodeficiency virus-infected nonresponders to standard vaccination.

PURPOSE: Hepatitis B virus (HBV) vaccination is recommended for all human immunodeficiency virus (HIV)-infected patients without HBV immunity. However, serological response to standard HBV vaccination is frequently suboptimal in this population and the appropriate strategy for revaccination of HIV-infected nonresponders remained controversial. We aimed to determine the serological response to one booster dose of HBV vaccine given by intradermal (ID) or intramuscular (IM) route in HIV-positive nonresponders to standard HBV vaccination. MATERIALS AND METHODS: In this study, 42 HIV-infected nonresponders were enrolled. We randomized them to receive either 10 µg (0.5 mL) for ID (20 cases) or 20 µg (1 mL) for IM (22 cases) administration of HBV vaccine as a one booster dose. After 1 month, anti-HBs titer was checked in all cases. A protective antibody response (seroconversion) defined as an anti-HBs titer ≥10 IU/L. RESULTS: Seroconversion was observed in 47.6% of subjects after 1 ID dose. A total of 30% showed antibody titers above 100 IU/L. Except one case, all responders had CD4(+) >200 cells/mm(3). Mean anti-HBs titer was 146.5 ± 246 IU/L. After the one IM booster dose, seroconversion was observed in 50% of cases. A total of 36.3% of subjects had anti-HBs ≥100 IU/L. All responders had CD4(+) >200 cells/mm(3), except one case. Mean anti-HBs titer was 416.4 ± 765.6 IU/L. Responders showed significantly higher CD4(+) cell counts, in comparison to nonresponders (P < 0.001). CONCLUSIONS: One booster dose administered IM or ID to HIV-infected nonresponders resulted in similar rates of seroconversion, overall response rate 50%. However, higher anti-HBs titers observed more frequently in IM group.

Relationship of generalized joint hypermobility with vesicoureteral reflux and urinary tract infection.

INTRODUCTION: Generalized joint hypermobility is deemed to be an underlying risk factor for many clinical conditions. The goal of this study was to determine the prevalence of generalized joint hypermobility in patients with vesicoureteral reflux. MATERIALS AND METHODS: This was a cross-sectional study on 313 children, 3 to 15 years old, with a history of urinary tract infection. Generalized joint hypermobility was evaluated according to the Beighton scores. Urinary tract ultrasonography and cystography were done if indicated. Participants were divided into 2 groups, group 1 without urinary tract abnormality and group 2 with primary vesicoureteral reflux, which were compared with the control group. RESULTS: Generalized joint hypermobility was documented in 37.2% of the children in the control group and 45.7% of those in group 1. This rate was 62.3% in group 2 (odds ratio, 2.79; 95% confidence interval, 1.61 to 4.82). Generalized joint hypermobility was seen in 44.1% of the children with mild vesicoureteral reflux, 60.5% of those with moderate vesicoureteral reflux, and 86.2% of those with severe vesicoureteral reflux. There was a significant relationship between the hypermobility incidence and the urinary reflux severity (P = .003). CONCLUSIONS: This study showed the prevalence of generalized joint hypermobility in children with vesicoureteral reflux was more than that in the general population, and the prevalence of hypermobility syndrome increased with the reflux severity.

ELISA Cut-off Point for the Diagnosis of Human Brucellosis; a Comparison with Serum Agglutination Test.

BACKGROUND: Brucellosis is a world-wide disease, which has a diverse clinical manifestation, and its diagnosis has to be proven by laboratory data. Serum agglutination test (SAT) is the most-widely used test for diagnosing brucellosis. The enzyme linked immunosorbent assay (ELISA) can also determine specific antibody classes against brucella. It is a sensitive, simple and rapid test, which could be an acceptable alternative to SAT with fewer limitations, however, like any other new test it should be further evaluated and standardized for various populations. This study was planned to determine an optimal cut-off point, for ELISA which would offer maximum sensitivity and specificity for the test when compared to SAT. METHODS: Four hundred and seven patients with fever and other compatible symptoms of brucellosis were enrolled in the study. Serum agglutination test, 2-Mercaptoethanol test, and ELISA were performed on their sera. RESULTS: The cut-off point of 53 IU/ml of ELISA-IgG yielded the maximal sensitivity and specificity comparing to the other levels of ELISA-IgG, and was considered the best cut off-point of ELISA-IgG to diagnose acute brucellosis. At this cut-off, the sensitivity, specificity, positive predictive value, negative predictive value, positive likelihood ratio, and negative likelihood ratio were 84.09%, 85.38%, 62.20, 94.90, 5.75, 0.18, respectively. CONCLUSION: The best cut-off point of ELISA-IgG is 53 IU/ml, which yields the maximal sensitivity and specificity to diagnose acute brucellosis.

Persistence of antibody to hepatitis B surface antigen among vaccinated children in a low hepatitis B virus endemic area.

BACKGROUND: A potential problem of hepatitis B immunization is that vaccine-induced antibody to hepatitis B surface antigen (anti-HBs) declines to low levels with age. This study investigated the persistence of anti-HBs in vaccinated children in a low hepatitis B virus (HBV) endemic area. METHODS: Plasma samples of 938 children between ages of 8 months and 15 years were tested for the presence of anti-HBs. RESULTS: The seroprotection rate was 60%. Protective antibody level was detected in 65% of children one year after vaccination, and in 30%, 29% and 24% 5, 10 and 15 years after vaccination, respectively. The mean anti-HBs titer declined with post-vaccination time (to 66 mIU/mL in 1 year, 60 mIU/mL in 5 years, 40 mIU/mL in 10 years to 37 mIU/mL in 15 years after vaccination). CONCLUSIONS: Children vaccinated against HBV during infancy may show low levels of antibody during adolescence. Our data suggest that a booster dose of vaccine may be required in low HBV endemic areas.