A skillful research presentation can be compared to good sushi. The fresh sushi toppings are interesting topics, and an appropriate quantity of high-quality rice is necessary and sufficient data. However, even if you have the fresh sushi toppings and appropriate quantity of high-quality rice, you cannot make “delicious sushi” without perfect shaping. Perfect shaping includes the design of presentation slides. In this review, I will discuss tips and tricks for presentation slide design that can be immediately put into practice in your talk.

Although our country’s neonatal and infant mortality rates are the lowest in the world, the psychological health of children is rated by UNICEF as poor among OECD countries. Health is defined as a state of physical, psychological and social well-being. The number of children and adolescents with chronic physical, developmental, behavioral or mental health problems and in need of some form of medical care or support is increasing; the number of children in medical care is increasing; and the number of children with low birth weight and proportion of low birth weight children is increasing. The number of medical check-ups is low among developed countries, and school health check-ups do not evaluate and support children psychologically and socially. It is necessary to establish a system to examine and support children physically, psychologically and socially. We hope that the Basic Act on Child Development, the Basic Act on Children and Families and the Children and Families Agency will help to solve the problems of pediatric medicine and health in Japan.

Considering the reclassification of COVID-19 and gradual recovery of economic activities in society, we hosted the 58th Annual Meeting of the Japanese Society for Pediatric Nephrology as a face-to-face meeting for the first time in a long time since the 54th Annual Meeting. The theme of the meeting was “Serendipity—Treasure every encounter”. The “encounters” mentioned here refer not only to encounters with people, including patients (children), teachers, colleagues who provide medical care together, and junior colleagues who follow in one’s footsteps, but also to encounters with new knowledge, awareness and research to verify awareness. We believe that these various encounters have been the driving force behind the progress of medicine to date. I would like to look back on the various encounters and experiences I have had as a clinician, express my gratitude and thanks to all the doctors who took care of me, and send messages to young pediatric nephrologists who will lead the next generation.

Serum calcium (Ca) and phosphorus undergo regulation in the intestine, kidney, and bone. The movement within and outside cells also influences serum phosphorus levels. Hormones play a crucial role in controlling serum Ca and phosphorus to keep them within the normal range. Considering disorders of calcium and phosphorus metabolism, it is vital to assess the in-out balance of Ca and phosphorus in organs and the impact of controlling hormones to identify the root cause. Potential causes include chronic kidney diseases like renal dysfunction and renal tubular abnormalities, along with conditions such as vitamin D deficiency or excess, parathyroid hormone deficiency or excess, and hereditary hypophosphatemic rickets. Metabolic disorders involving Ca and phosphorus can manifest as symptomatic or asymptomatic, with prompt intervention necessary in symptomatic cases. In managing chronic illnesses, attention should be given to treatment-related events, as well as symptoms and signs of the diseases. This article delves into the etiology, pathophysiology, clinical manifestations, diagnostic methods, and treatment approaches for disorders of calcium and phosphorus metabolism.

Focal segmental glomerulosclerosis (FSGS) is recognized as a common cause of steroid and immunosuppressive drugs-resistant nephrotic syndrome and is a major cause of kidney failure in both children and adults. In this review, causes of FSGS, clinicopathological characteristics and differential diagnosis of primary, secondary and genetic FSGS, a possible role of anti-nephrin antibodies in post-transplant FSGS recurrence, and therapies for FSGS patients with anti-nephrin antibodies were described.

The kidney is an extremely complex organ derived from the intermediate mesoderm, and the development and maintenance of its function involve a multitude of genes. Due to abnormalities in these genes, various types of hereditary kidney diseases can occur, resulting in a wide range of genetic kidney disorders. Advances in genome analysis technology in recent years have enabled the diagnosis of many patients with hereditary kidney diseases, leading to the identification of new causative genes. In the field of kidney disease management, genomic data is increasingly utilized in medical practice, promoting the advancement of genomic medicine. However, the promotion of genomic medicine is recommended to be accompanied by genetic counseling. To advance genomic medicine in the field of kidney diseases, it is essential to establish a collaborative framework involving nephrologists, clinical geneticists, certified genetic counselors, basic researchers, and professionals from a wide range of fields.

We discuss the viral infection control and the timing of vaccination in patients with childhood-onset kidney disease who re receiving immunosuppressive drugs, with a focus on patients who underwent or awaited kidney transplantation. This review also addresses the monitoring and treatment of infectious diseases caused by cytomegalovirus, BK virus, and Epstein-Barr virus. In addition, vaccines and treatments for COVID-19 control are outlined. Specific response methods for real clinical situations are described based on the policies of the Department of Pediatric Nephrology at Tokyo Women's Medical University.

Background and Purpose: Tubulointerstitial nephritis (TIN) can present with a variety of symptoms. The relationship between initial symptoms and renal prognosis is still unknown. Methods: We performed a retrospective analysis of the clinical course and renal outcome of 14 patients diagnosed with TIN in our hospital. Initial symptoms were classified into three groups: proteinuria detected by school urinalysis screening in 4 patients, ocular symptoms in 7 patients, and fever in 3 patients. Results: The median follow-up period was 27.4 months (range: 5.6–86.6 months). At the time of diagnosis, the median estimated glomerular filtration rate (eGFR) was 76.2, 103.1, and 83.1 mL/min/1.73 m², in school urinalysis screening, ocular symptoms, and fever groups, respectively. The school urinalysis screening and fever groups had lower eGFRs than the ocular symptoms group. At the last observation, the median eGFR was 86.7, 107.8, and 90.9 mL/min/1.73 m², respectively, with the school urinalysis screening group having a lower eGFR than the ocular symptoms group. Conclusion: Patients in school urinalysis screening group may have longer duration from symptom onset to diagnosis and treatment. The duration from symptom onset to treatment may affect the renal prognosis.

Urinary metabolite screening is indicated for children with urolithiasis because they are likely to have metabolic abnormalities. Although 24-h urine collection is useful for evaluation, it is difficult for infants because it requires hospitalization and catheterization. We investigated the usefulness of a single 24-h urine collection test in estimating stone constituents. Of the seven patients with calcium stone, two had hypercalciuria, one had hypocitraturia, and one had hypomagnesuria. Two patients with L-cystine stones had hypercystinuria in both cases. Urinary metabolite abnormalities identified by 24-h urine collections were associated with stone formation in five of nine patients (56%), including duplicates. Two patients with hypercystinuria on 24-h urine collections had hypercystinuria in all spot urine tests. A single 24-h urine collection can be insufficient to estimate metabolic abnormalities in approximately 50% of pediatric patients with urolithiasis. Because spot urine test can be used as a surrogate for several diseases, 24-h urine collection should be performed in selected cases.

We have examined clinically five cases of acute kidney injury in pediatric idiopathic nephrotic syndrome (NSAKI) that required hemodialysis (HD) at onset. This cohort comprised four boys and a girl aged between one and three years. HD was indicated in all cases due to oliguria/anuria and in two cases due to respiratory failure. The average weight gain rate was 46%. Two cases started HD within 2 weeks of NS onset, and three cases started approximately one month after. Renal histological findings were minimal-change type in three cases and focal segmental glomerulosclerosis (FSGS) in one case. The average duration of HD was 16 days, ranging from 6 to 42 days. Two patients transitioned from HD to peritoneal dialysis (PD), but one patient could be withdrawn from PD and had no renal dysfunction at final observation. A patient with FSGS developed end-stage renal failure, and underwent renal transplant. It must be noted that there are two timings for the onset of NSAKI: the early episode and the refractory stages of steroid therapy. Furthermore, it’s suggested in NSAKI that the prompt introduction of HD might be able to prevent permanent renal dysfunction by focusing on urine volume and weight gain rate rather than serum creatinine levels.

We encountered two cases of a 1-year-old boy with refractory steroid-resistant nephrotic syndrome (SRNS). Their treatment regimens included prednisolone and cyclosporine, but did not result in complete remission. After receiving rituximab, the boy in case 1 was able to maintain an incomplete remission with low-dose cyclosporine alone, and the boy in case 2 was able to maintain a complete remission without any medication. In both cases, no genetic abnormalities were found in the analysis. Rituximab may be effective in refractory SRNS without genetic abnormalities.

An 8-year-old boy with persistent nocturnal enuresis was admitted to the authors’ hospital. We suspected diabetes insipidus because his urine was hypotonic, and he had both polydipsia and polyuria. Magnetic resonance imaging (MRI) of the head revealed an 8-mm Rathke’s cyst within a sella turcica, and a water restriction test confirmed the diagnosis of partial central diabetes insipidus. When oral desmopressin was initiated, the nocturnal enuresis, polydipsia, and polyuria all resolved. Two years later, head MRI showed that the Rathke’s cyst had disappeared; however, oral medication was still required. After 2.5 years, his urine volume did not increase, even when he forgot to take his medicine; therefore, the medication was gradually reduced and stopped after 3 years. An improvement of symptoms after surgery has been reported in some cases of acute onset diabetes insipidus due to Rathke’s cyst. However, in cases with a chronic onset caused by an inflammatory spread from the cyst, an improvement after surgery has only rarely been reported. In this case, the diabetes insipidus improved and the patient’s height increased due to the spontaneous regression of the Rathke’s cyst, thus suggesting that the pressure caused by the cyst had most likely been the main cause of his symptoms.

Herein, we report the case of an adolescent patient with nephrotic-range proteinuria and hypoproteinemia caused by tiopronin. The patient was a 13-year-old adolescent girl who had been diagnosed with cystinuria at the age of 9-years. Initial treatment comprised urinary alkalization and tiopronin. Urinalysis showed 3+ proteinuria 43 months after the initiation of tiopronin treatment. Proteinuria was accompanied by 2.2 g/dL of hypoalbuminemia, hyperlipidemia, and high adiponectin levels. Considering the adverse effects of tiopronin, it was immediately withdrawn. Ten days after withdrawal of the drug, proteinuria and hypoproteinemia had completely resolved, and steroid therapy was therefore not required. These findings are consistent with those of nephrotic syndrome secondary to medication in our patient. Tiopronin, which is a common treatment for cystinuria, may cause nephrotic-range proteinuria and hypoproteinemia. Therefore, periodic urine analyses and patient follow-ups are warranted during tiopronin therapy for cystinuria.

We report four girls with unilateral renal aplasia complicated by genital anomalies. The first case was diagnosed with molimina menstrualia at the age of 12 years. The second case was diagnosed with genital anomalies at the age of 12 years when an infection was associated with retention of blood in the right uterus. Since this time, magnetic resonance imaging was performed at the age of 6 years in girl with unilateral renal aplasia, and a genital organ abnormality was confirmed. Third and fourth cases were diagnosed with OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly syndrome) at the age of 6 years. They had gynecological consultations and have progressed without complications. Genitourinary abnormalities associated with unilateral renal aplasia, such as OHVIRA syndrome, are diagnosed at different times depending on age. If the diagnosis is delayed, endometriosis may develop from molimina menstrualia and lead to infertility. Therefore, diagnosing reproductive organ abnormalities early and preventing complications through proactive examinations are important.

Congenital nephrotic syndrome (CNS) due to ARHGDIA gene mutation is rare and few cases have been reported. A boy who developed CNS at 1 month of age presented with rapidly progressing renal dysfunction. He was transferred to our hospital and treated with maintenance peritoneal dialysis following continuous hemodialysis. In addition to nephrotic syndrome and renal dysfunction, he also had microcephaly, micrognathia, and strabismus. During his hospitalization, He also developed epilepsy. Genetic testing revealed a homozygous novel nonsense variant in the ARHGDIA gene (c.153C>G: (p.Tyr51*)) . His mother had the same heterozygous mutation, and an uniparental disomy from his mother was suspected as the cause of his homozygous mutation. In previous reports, nephrotic syndrome caused by this gene develops in the CNS and steroid-resistant nephrotic syndrome in early childhood, both of which lead to end-stage renal failure. The complications included intellectual disability, seizures, and visual and hearing impairments. There were no cases showing microcephaly or micrognathia like this case. Case series are needed to clarify the phenotype of ARHGDIA gene mutation.

We report a case of an eight-year-old boy who developed immune complex-mediated membranoproliferative glomerulonephritis (MPGN), seven years after liver transplantation. He underwent Kasai operation at the age of three months, followed by liver transplantation at the age of 19 months for congenital biliary atresia. Since then, he has experienced protein-losing enteropathy due to hepatic vein stenosis and portal hypertension. At the age of eight years, he was referred to our department due to sudden onset of severe proteinuria and hematuria. Renal biopsy revealed mesangial proliferative lesion with double contours of the glomerular basement membrane under light microscopy. Immunofluorescence demonstrated a full house pattern with positive staining for IgG, IgA, IgM, C3c, C4, and C1q. Additionally, electron microscopy revealed subendothelial and mesangial deposits. Considering the absence of clinical signs such as negative antinuclear antibodies and any symptoms indicative of systemic lupus erythematosus, we diagnosed him with immune complex-mediated MPGN, resulting from hepatic vein stenosis and subsequent portal hypertension. After initiating induction therapy with prednisolone, lisinopril hydrate proved to be effective for maintenance. This case underscores the significant concern about the development of immune complex-mediated glomerulonephritis in children, several years after liver transplantation.