It remains challenging to adequately assess abnormalities in water and electrolyte balance and to determine what type of intravenous fluids is favorable for sick children. The use of hypotonic fluids has been reported to be a cause of hospital-acquired hyponatremia and isotonic fluids are recommended as maintenance intravenous fluids by clinical guidelines. However, it is not shown that hypotonic fluids significantly increase symptomatic hyponatremia or mortality compared with isotonic fluids. Therefore, it remains unclear whether isotonic fluids should be used in all children who need maintenance intravenous fluids. It is highly advised to monitor plasma electrolytes after initiation of intravenous fluids based on the assessment of the risk of nonosmotic secretion of anti-diuretic hormone. Concurrently, the assessment of total exchangeable cations and total body water is quite helpful in the management of sick children with abnormalities in water and electrolyte balance.
Cytokines are multifunctional proteins which play various roles including cell growth, differentiation, migration and metabolism in vivo. Cytokines regulate inflammatory reactions to maintain homeostasis. In pediatric inflammatory diseases, homeostatic balance maintained by cytokine network is broken down by excessive immune response. Furthermore, overproduction of cytokines increase severity in these inflammatory diseases. Cytokine profile analysis which evaluates multiple cytokines simultaneously reflects immune response and inflammatory condition in each patient. Therefore, cytokine profile analysis is a very useful approach for the evaluation of patientʼs condition, disease activity, severity and also differential diagnosis. It is desired that cytokine profile analysis is more widely used in a variety of ways in clinical practice.
Children with congenital anomalies of the kidney and urinary tract (CAKUT) have been seen by pediatricians and pediatric urologists. The JSPU (Japanese Society of Pediatric Urology) developed the guideline of treatment for cryptorchidism in 2005. Because the guideline was appreciated by many doctors who were engaged in pediatric urology, patients with undescended testicles have been treated according to the Japanese guideline. In Japan patients with congenital hydronephrosis and vesicoureteral reflux (VUR) have been treated according to the guideline of western countries. However, the guidelines for the Japanese patients of these disorders are required, because the medical insurance system is different from that in western countries. In 2016, the guidelines for patients with congenital hydronephrosis and VUR were developed in Japan by the academic committee of the JSPU. According to these guidelines, laparoscopic surgeries have become widespread as minimally invasive surgery. In addition, robotic surgery has just started in the field of pediatric urology in Japan. In conclusion, urological reconstruction surgery for children could be replaced with robot-assisted surgery, which may be proved to be minimally invasive surgery in the near future.
To assess impact of multi-disciplinary therapy for nephrotoxicity, including hematopoietic stem cell transplantation (HSCT), among childhood hematology and oncology survivors, we conducted retrospective longitudinal study. Thirty-eight survivors (HSCT [N=14] and non-HSCT [N=24] groups) were recruited. The 50%ile standard serum creatinine*100/serum creatinine (CrM1) was assessed before treatment (T1); upon therapy completion (T2); and 1 (T3), 3 (T4), and 5 (T5) years after therapy completion. We analyzed correlations between cumulative dose of anticancer drugs and CrM1 over time. We found that average CrM1 level decreased especially in HSCT group from T1 to T5 (HSCT: T1 112⇒T5 98, Non-HSCT: T1 133⇒T5 111). Twenty-one percent of HSCT survivors showed grade 2 or higher nephrotoxicity on T2 through T5, whereas only 4% (T4) of non-HSCT survivors showed grade 2 kidney dysfunction. Surprisingly, the CrM1 of neuroblastoma survivors in the HSCT group significantly decreased from T1 to T5 (108 to 85). Pearson's correlation analyses revealed strong correlations between CrM1 and cumulative doses of cyclophosphamide (T3 γ=−0.47, p<0.01) and cisplatin (T3 γ=−0.56, p<0.01). Multi-disciplinary therapy, including HSCT, could be a risk factor for long-term nephrotoxicity. The most severe nephrotoxicity were found in survivors with neuroblastoma who had received HSCT. Medical team should conduct risk-based follow-up for renal function for hemato-oncology survivors.
A 9-year-old girl visited our hospital complaining of proteinuria, which was identified during her school urinary screening. She had mild proteinuria (1+, 29 mg/dl, protein/creatinine ratio =0.9 g/gCre), without hematuria or deterioration of kidney function. Initially, a kidney biopsy was performed, which showed only minor glomerular abnormalities. Angiotensin II receptor blocker was prescribed, but her proteinuria increased over the next 5 years. A second biopsy was performed when the patient was 14 years old. Histologic examination of biopsy specimen showed focal segmental glomerular sclerosis and granular swollen epithelial cells in the tubular epithelium. The presence of granular swollen epithelial cells suggested that she was affected by mitochondrial disease. Screening of the m.3243A>G mitochondrial DNA mutation revealed a heteroplasmy level of 49% in the peripheral blood. This was diagnosed as focal segmental glomerular sclerosis due to mitochondrial disease. Mitochondrial disease should be thought as a differential diagnosis, even if the patient has no neurologic abnormality, familial diabetes or maternal inheritance.
We report a case involving a 21-year-old woman with membranoproliferative glomerulonephritis (MPGN) who had exacerbation of the urinary findings due to repeated tonsillitis. She underwent tonsillectomy, which resulted in clinical and histological remission. The patient was under observation for poststreptococcal glomerulonephritis at the age of 11 years, but no improvement in urinary findings or hypocomplementemia was observed after 5 months, and a kidney biopsy led to the diagnosis of MPGN Type-I. Urinary findings improved once by multi-drug therapy including methylprednisolone, mizoribine, angiotensin converting enzyme inhibitor and angiotensin II receptor blocker. However, hypocomplementaemia and proteinuria were detected again at the age of 12 years. We performed tonsillectomy at the age of 17 years for chronic tonsillitis because she showed exacerbation of the clinical findings. Subsequently, urinary findings showed improvement and serum complement levels normalized. Histological improvement was also found in the fifth kidney biopsy performed at the age of 18 years. This suggested that tonsillectomy could be an effective treatment option for intractable MPGN with repeated exacerbation of clinical findings due to repeated tonsillitis.
We report a case of tubulointerstitial nephritis and uveitis (TINU) syndrome. A 15-year-old girl developed uveitis 2 years after onset of tubulointerstitial nephritis (TIN). She had fever for two weeks. A germ cell tumor was found by computed tomography. After one month, the tumor was completely removed. However, she had fever again one month later. Moreover, she had proteinuria, hematuria and renal dysfunction. Renal biopsy showed interstitial infiltration of mononuclear cells. She did not develop uveitis yet. Therefore, she was diagnosed with TIN. She was treated with 2 courses of methylprednisolone pulse therapy followed by 40 mg/day of prednisolone and her symptoms improved soon. We tapered prednisolone to 1 mg/day for 23 months. Subsequently she had photophobia, eye pain and redness. She was diagnosed with uveitis. Therefore, she was diagnosed with TINU syndrome for the first time. This case suggests that uveitis in TINU syndrome may develop more than 1year after onset of nephritis, or that steroid might prevent the onset of uveitis. Patients with TIN should have an eye examination regularly or at the time they complain of eye symptoms.
Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a syndrome characterized by double uterus, blind hemivagina and ipsilateral renal agenesis. In addition, multiple renal arteries are characterized by the presence of more than one renal artery in one of the kidneys and are often combined with essential hypertension. We report the case of an 11-year- and 6-months-old female. At the age of 8 years, she was indicated unilateral left renal agenesis and uterine hypoplasia. At the age of 9 years and 8 months, she developed headache and blood pressure of 154/82 mmHg, and was admitted to hospital for further evaluation and medical treatment. Antihypertensive treatment was carried out and completed in 4 months, but her blood pressure increased again one year later. Abdominal MRI revealed unilateral left renal agenesis, double uterus and left hemivaginal obstruction, therefore diagnosed as OHVIRA syndrome. In addition, there were two right renal arteries, suggesting right multiple renal artery. In females, unilateral renal agenesis requires further evaluation because of its high rate of association with uterovaginal abnormalities. Further, in this study, hypertension may have developed as a result of a congenital solitary kidney with multiple renal artery.