• Childhood Kidney Diseases
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• v.2 no.2
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• pp.187-191
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• 1998

Yersinia pseudotuberculosis, a gram-negative coccobacillus, is a member of the enterobacteriaceae family, and the illness has fever, abdominal pain, diarrhea, mesenteric lymphadenitis, terminal ileitis, arthropathy, erythema nodosum. Specially in children, it is known as an important cause of acute renal failure due to acute tubulointerstitial nephritis. It has been recognized in a worldwide distribution. For the first time, the cases of acute renal failure caused by this organism were reported in Japan in 1980's. In Korea, the first case accompanying with ARF was reported in 1988. Since that, many cases have been reported in Seoul area, but no case in other places. So we report 2 cases of Y.P. infection accompanying with ARF in Pusan area, for the first time in other place than Seoul area.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.251-258
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• 2002

We report the experiences of pump-driven continuous venovenous hemofiltration therapy in three children with acute renal failure. The all three patients required mechanical ventilation and needed the support of vasopressors. Renal replacement therapy was needed to meet the metabolic and fluid balance, but intermittent hemodialysis and peritoneal dialysis were not feasible because of hemodynamic instability and concurrent infection. We instituted pump-driven continuous venovenous hemofiltratlon (CVVH), and immediate improvement of pulmonary edema and successful removal of retained fluid were observed. Urea clearance also was satisfactory. During the filter running time, significant thromboembolic event or rapid drop of systemic blood pressure were absent. We concluded that the CVVH is an effective and safe method of renal support for critically ill pediatric patient.

• Childhood Kidney Diseases
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• v.18 no.1
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• pp.13-17
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• 2014

Continuous renal replacement therapy (CRRT) has become the preferred dialysis modality to support critically ill children with acute kidney injury. As CRRT technology and clinical practice advances, experiences using CRRT on small infants and neonates have increased. In neonates with hyperammonemia or acute kidney injury during extracorporeal membrane oxygenation (ECMO) therapy, CRRT can be a safe and effective technique. However, there are many limitations of CRRT in neonates, including vascular access, bleeding complications, and lack of neonatespecific devices. This review discusses the basic principles of CRRT and the special considerations when using this technique in neonates and infants.

• Childhood Kidney Diseases
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• v.8 no.1
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• pp.86-90
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• 2004

Hemolytic uremic syndrome(HUS) is characterized by acute renal failure, microangiopathic hemolytic anemia, and thrombocytopenia and the most common pathogen is Escherichia coli (E. coli) O157 : H7. Ischemic colitis, which rarely occurs in children, is due to the reduced local blood flow to the intestine, tissue necrosis and secondary bacterial infection. We describe a patient who was admitted with abdominal pain, vomiting and hematochezia, and diagnosed as ischemic colitis by barium enema. This patient showed hemolytic anemia, thrombocytopenia and progressive renal failure and was subsequently diagnosed as hemolytic uremic syndrome. After hemodialysis, the patient showed improvement of symptoms and resolution of renal failure and ischemic colitis.

• Childhood Kidney Diseases
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• v.14 no.2
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• pp.236-239
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• 2010

Typhoid fever is a systemic infectious disease which affects many organs. In children, few cases have been reported of acute nephritic syndrome in typhoid fever. We report an immunocompetent 9-year old girl with typhoid fever complicated by acute tubulointerstitial nephritis who presented with prolonged fever and acute renal failure.

• Childhood Kidney Diseases
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• v.14 no.2
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• pp.240-245
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• 2010

Nutcracker syndrome refers to the compression of the left renal vein between the abdominal aorta and superior mesenteric artery which can lead to renal vein hypertension. Patients can present with hematuria, proteinuria, dysuria or abdominal pain. In diagnosing the nutcracker syndrome, measurement of the peak velocity ratio of aorto-mesenteric border versus renal hilum by Doppler sonography is useful. Currently, there are few reports about the genetic correlation in this syndrome. We report two cases of the nutcracker syndrome found in male siblings with hematuria.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.223-228
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• 2003

Oligmeganephronia is congenital hypoplasia of kidney with renal pathology showing very small number of nephrons with compensatory hypertrophy of the remaining glomeruli. A 7-year-old girl was referred to our nephrology clinic due to hematuria detected on school screening urinalysis and diagnosed as chronic renal failure and oligomeganephronia on renal biopsy. We are reporting the clinical and histomorphometric changes for the four years follow-up with review of literatures.

• Childhood Kidney Diseases
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• v.23 no.2
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• pp.124-127
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• 2019

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly of the genitourinary tract comprising uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Patients with HWW syndrome usually present symptoms such as dysmenorrhea, abdominal pain, pelvic mass, and purulent vaginal discharge. If not treated at an appropriate time, complications such as infertility, endometriosis, pyosalpinx, and subsequent pelvic adhesions may occur. Here, we report a case of HWW syndrome in a 7-year-old-girl who was also diagnosed as having central precocious puberty. She was brought to the pediatric department with chief complaints of lump in her breast and vaginal discharge. When she was around 2 months old, she was confirmed to have a single kidney on ultrasonography. We checked her past medical history and diagnosed her as having HWW syndrome based on the results of imaging studies, including abdominal ultrasonography and pelvic magnetic resonance imaging. She underwent treatment with gonadotropin-releasing hormone analogue for 2 years. During 24 months of follow-up, she showed no serious problems or complications. If renal anomalies are identified immediately after birth or in infancy, further screening tests should be conducted prior to menstruation for determining congenital abnormalities of the reproductive tract and vice versa.

• Yonsei Medical Journal
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• v.59 no.9
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• pp.1015-1025
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• 2018

Kidney diseases including acute kidney injury and chronic kidney disease are among the largest health issues worldwide. Dialysis and kidney transplantation can replace a significant portion of renal function, however these treatments still have limitations. To overcome these shortcomings, a variety of innovative efforts have been introduced, including cell-based therapies. During the past decades, advances have been made in the stem cell and developmental biology, and tissue engineering. As part of such efforts, studies on renal cell therapy and artificial kidney developments have been conducted, and multiple therapeutic interventions have shown promise in the pre-clinical and clinical settings. More recently, therapeutic cell-secreting secretomes have emerged as a potential alternative to cell-based approaches. This approach involves the use of renotropic factors, such as growth factors and cytokines, that are produced by cells and these factors have shown effectiveness in facilitating kidney function recovery. This review focuses on the renotropic functions of bioactive compounds that provide protective and regenerative effects for kidney tissue repair, based on the available data in the literature.

• Childhood Kidney Diseases
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• v.26 no.1
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• pp.63-67
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• 2022

Nephrocalcinosis often occurs in infants and is caused by excessive calcium or vitamin D supplementation, neonatal primary hyperparathyroidism, and genetic disorders. Idiopathic infantile hypercalcemia (IIH), a rare cause of nephrocalcinosis, results from genetic defects in CYP24A1 or SLC34A1. Mutations in CYP24A1, which encodes 25-hydroxyvitamin D 24-hydroxylase, disrupt active vitamin D degradation. IIH clinically manifests as failure to thrive and hypercalcemia within the first year of life and usually remits spontaneously. Herein, we present a case of IIH wih CYP24A1 mutations. An 11-month-old girl visited our hospital with incidental hypercalcemia. She showed failure to thrive, and her oral intake had decreased over time since the age of 6 months. Her initial serum parathyroid hormone level was low, 25-OH vitamin D and 1,25(OH)₂ vitamin D levels were normal, and renal ultrasonography showed bilateral nephrocalcinosis. Whole-exome sequencing revealed compound heterozygous variants in CYP24A1 (NM_000782.4:c.376C>T [p.Pro126Ser] and c.1310C>A [p.Pro437His]). Although her hypercalcemia and poor oral intake spontaneously resolved in approximately 8 months, we suggested that her nephrocalcinosis and renal function be regularly checked in consideration of potential asymptomatic renal damage. Hypercalcemia caused by IIH should be suspected in infants with severe nephrocalcinosis, especially when presenting with failure to thrive.