Background
Hypogammaglobulinemia is a major adverse event after rituximab treatment; however, the precise incidence and risk factors are unclear in complicated steroid-dependent or frequently ...relapsing nephrotic syndrome (SDNS/FRNS) patients.
Methods
This was a single-center, retrospective, observational study. Patients who received a single dose of rituximab for complicated SDNS or FRNS between February 2007 and May 2019 were enrolled. Serum IgG levels were plotted, and their trends were evaluated after rituximab treatment. The incidence of transient and persistent hypogammaglobulinemia was examined, and risk factors were calculated by multivariate analysis using logistic regression.
Results
We enrolled 103 patients who received 238 single doses of rituximab. Hypogammaglobulinemia was observed in 58.4% of the patients at least once after a single dose of rituximab treatment and 22.3% developed persistent hypogammaglobulinemia. Serum IgG levels gradually increased during B-cell depletion, and patients with low serum IgG levels at rituximab treatment had persistent hypogammaglobulinemia. Repeated courses of rituximab treatment increased the incidence of hypogammaglobulinemia. A past history of steroid-resistant nephrotic syndrome (SRNS) (odds ratio OR = 10.02; 95% confidence interval CI = 2.65–37.81;
P
< 0.001) and low serum IgG levels at rituximab treatment (OR = 7.63; 95% CI = 2.10–27.71;
P
= 0.002) was significantly associated with hypogammaglobulinemia in multivariate analysis.
Conclusions
Hypogammaglobulinemia is a frequent adverse event after rituximab treatment, although IgG levels slightly increase during B-cell depletion. Low serum IgG levels at rituximab treatment and a past history of SRNS are significant risk factors for the development of hypogammaglobulinemia after rituximab treatment.
Background
Congenital nephrotic syndrome of the Finnish type (CNF) caused by
NPHS1
mutations is a rare disease. Infants with CNF can develop many complications, but hepatobiliary complications are ...uncommon. CNF is not reported as a risk factor of cholelithiasis, which is also a rare disease in the pediatric population. We herein present the cases of three infants with CNF diagnosed with gallstones among a total of seven children with CNF who were treated between 2010 and 2020 in our hospital.
Case-Diagnosis/Treatment
The gestational ages, birth weights, and ages at CNF diagnosis of cases 1, 2, and 3 were 38, 37, and 38 weeks; 2450, 2374, and 3120 g; and 25, 3, and 31 days of age, respectively. Imaging studies at the time of CNF diagnosis revealed asymptomatic gallstones in each of them. Case 1 patient developed incarcerated choledocholithiasis and presented with vomiting, white stool, and critically prolonged coagulation at 7 months of age. The gallstones disappeared at 3 months of age in case 2, although they remained in cases 1 and 3 at the last observation.
Conclusions
Infants with CNF can develop cholelithiasis and should undergo hepatobiliary ultrasonography at the time of diagnosis. Patients with gallstones should be evaluated for signs of potential gallstone incarceration because of the risk of hemorrhagic complications due to cholestasis with subsequent vitamin K deficiency and treatment with anticoagulation agents.
Background
Practice patterns and bleeding complications of percutaneous native kidney biopsy (PNKB) have not recently been investigated and the Japanese Society of Nephrology performed a nationwide ...questionnaire survey in 2018.
Methods
The survey consisted of nine sections about PNKB: (1) general indications; (2) indications for high-risk patients; (3) informed consent; (4) pre-biopsy evaluation; (5) procedures; (6) sedation; (7) post-biopsy hemostasis, bed rest, and examinations; (8) bleeding complications; and (9) specimen processing. A supplementary survey examined bleeding requiring transcatheter arterial embolization (TAE).
Results
Overall, 220 directors of facilities (nephrology facility NF, 168; pediatric nephrology facility PF, 52) completed the survey. Indications, procedures, and monitoring protocols varied across facilities. Median lengths of hospital stay were 5 days in NFs and 6 days in PFs. Gauge 14, 16, 18 needles were used in 5%, 56%, 33% in NFs and 0%, 63%, 64% in PFs. Mean limits of needle passes were 5 in NFs and 4 in PFs. The bed rest period was 16–24 h in 60% of NFs and 65% of PFs. Based on 17,342 PNKBs, incidence rates of macroscopic hematuria, erythrocyte transfusion, and TAE were 3.1% (NF, 2.8%; PF, 6.2%), 0.7% (NF, 0.8%; PF, 0%), and 0.2% (NF, 0.2%; PF, 0.06%), respectively. Forty-six percent of facilities processed specimens all for light microscopy, immunofluorescence, and electron microscopy, and 21% processed for light microscopy only. Timing of bleeding requiring TAE varied among PNKB cases.
Conclusion
Wide variations in practice patterns of PNKB existed among facilities, while PNKBs were performed as safely as previously reported.
Patients receiving immunosuppressive agents are at risk of life-threatening infections. However, live vaccines are generally contraindicated in them. We conducted a prospective study regarding live ...attenuated vaccines for them. Patients elder than one year of age with immunosuppressive agents who showed negative or borderline antibody titers (virus-specific IgG levels < 4.0) against one or more of measles, rubella, varicella, and mumps and fulfilled the criteria (CD4 cell counts ≥ 500/mm3, stimulation index of lymphocyte blast transformation by PHA ≥ 101.6, serum IgG level ≥ 300 mg/dl, no steroid use or prednisolone < 1 mg/kg/day or < 2 mg/kg/2 days, trough levels of tacrolimus or cyclosporine were < 10 ng/ml or < 100 ng/ml and under good control of primary disease) were enrolled. Sixty-four vaccinations were administered to 32 patients. The seroconversion rates for measles, rubella, varicella, and mumps were 80.0%, 100.0%, 59.1%, and 69.2%, respectively. No life-threatening adverse events were observed, although one patient suffered from vaccine-strain varicella who showed cellular and humoral immunodeficiency (CD4 cell counts = 511/mm3, stimulation index of lymphocyte blast transformation by PHA = 91.1, serum IgG level = 208 mg/dl). This girl was immunized before we established the criteria for vaccination. Immunization with live attenuated vaccines for patients receiving immunosuppressive agents might be effective and safe if their cellular and humoral immunological parameters are within normal levels. However, determining the criteria for vaccination by immunological parameters should be established to guarantee the safety of live vaccines in the future. Clinical Trial Registration: UMIN Clinical Trials Registry (UMIN-CTR) UMIN000007710. The date of registration: 2012/4/13.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Background
Eosinophilic peritonitis (EP) is sometimes difficult to distinguish from bacterial peritonitis (BP) at onset, as they are often overlapping. Previous reports show EP occurs more frequently ...in infants, although the reason is unknown.
Methods
The study population was 77 pediatric patients receiving chronic peritoneal dialysis (PD) in our center. We compared clinical and laboratory data at onset of EP with those of BP. We also investigated age distribution at onset of EP and PD-related surgery.
Results
Eleven patients developed EP (18 episodes) and 19 patients developed BP (38 episodes). EP patients showed lower rate of cloudy dialysate (44.4% vs. 74.4%;
p
= 0.04), lower rate of fever (38.9% vs. 56.4%), lower frequency of abdominal pain (16.7% vs. 38.5%), higher peripheral blood eosinophil counts (/μL) (514 vs. 160;
p
< 0.001), and lower serum C-reactive protein level (mg/dL) (0.4 vs. 4.7;
p
< 0.001) than BP patients. Thirteen EP events were observed after 169 surgical interventions. Age at surgery-related EP was similar to age at surgery without EP (2.6 vs. 2.1;
p
= 0.65). There was no significant difference in postoperative EP occurrence between groups <2 years and ≥ 2 years (6.2% vs. 9.1%;
p
= 0.48). However, infants received more operations than older children.
Conclusion
Clinical symptoms in children and laboratory data of EP in children were less severe than those of BP. As incidence of postoperative EP did not differ by age, we speculate that higher incidence of EP in infants might be associated with higher incidence of surgery, although further validation is necessary.
Background
Rituximab is effective against complicated childhood steroid-dependent nephrotic syndrome (SDNS). Peripheral blood B-lymphocyte (B-cell) depletion is strongly correlated with persistent ...remission, relapse rarely occurring during B-cell depletion; however, we have encountered several such patients.
Methods
We retrospectively analyzed the characteristics and clinical course of 82 patients with SDNS treated with rituximab from January 2007 to December 2012 in our institution.
Results
Six of 82 patients (7.3%) had relapses during B-cell depletion after receiving rituximab (relapsed group). The remaining 76 patients did not have relapses during B-cell depletion (non-relapsed group). The median time to initial relapse during B-cell depletion was 85 days after receiving rituximab, which is significantly shorter than in the non-relapsed group (410 days,
p
= 0.0003). The median annual numbers of relapses after receiving rituximab were 2.5 and 0.9 in the relapsed and non-relapsed groups, respectively (
p
< 0.0001). Five patients in the relapsed group also had a total of 10 relapses after B-cell recovery; their median time from B-cell recovery to initial relapse was significantly shorter than in the non-relapsed group (31 vs. 161 days,
p
= 0.014). Number of relapses before rituximab, history of steroid resistance, onset age, previous treatment, time to ceasing steroids after rituximab, and duration of B-cell depletion did not differ between the two groups.
Conclusion
Relapse during B-cell depletion after receiving rituximab suggests that various pathophysiological mechanisms play a part in childhood nephrotic syndrome.
Background
Rituximab is effective for maintaining remission in patients with complicated nephrotic syndrome, although a history of steroid-resistant nephrotic syndrome (SRNS) is a risk factor for ...early relapse. We investigated the efficacy of prophylactic rituximab treatment for maintaining remission after B cell recovery.
Methods
Patients with complicated steroid-dependent or frequently relapsing nephrotic syndrome with history of SRNS who received a single dose of rituximab (375 mg/m
2
) and continued immunosuppressive agents were enrolled in this retrospective study. Patients were divided into two groups: a prophylaxis group, which received additional rituximab treatment at B cell recovery and a non-prophylaxis group. The relapse-free period from the last rituximab infusion (the second treatment in prophylaxis group and the first treatment in non-prophylaxis group) was compared between two groups using the Kaplan-Meier method, and risk factors for early relapse were calculated using multivariate analysis by Cox proportional hazards model.
Results
Sixteen patients in the prophylaxis group and 45 in the non-prophylaxis group were enrolled. Fifty-percent relapse-free survival after the last rituximab treatment was 667 days in the former and 335 days in the latter (
p
= 0.001). Multivariate analysis showed that additional rituximab treatment was the only significant negative factor for early relapse, with a hazard ratio of 0.40 (
p
= 0.02). Fifty-percent relapse-free survival after B cell recovery was much longer in the prophylaxis group (954 vs. 205.5 days,
p
= 0.003).
Conclusions
Additional rituximab treatment at B cell recovery can maintain prolonged remission even after B cell recovery in patients with complicated nephrotic syndrome with history of SRNS.
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