Objective: The aim of this study is to validate preventive medication and radiological examinations in management of children with pyelonephritis.
 Methods and Results: The medical records of one hundred sixty four patients (121 males and 43 females) were reviewed retrospectively. They were managed according to the Practice Parameter recommended by the American Academy of Pediatrics. Voiding cystourethrography were inspected after the first pyelonephritis. In nine of 32 patients (28%) without vesicoureteral reflux (VUR) who had no preventive medication, pyelonephritis relapsed. While a recurrence was observed in 2 of 15 patients (13%) with the grade I to III of VUR and in 5 of 10 patients (50%) with the grade IV and V of VUR who had preventive medication. The VUR detection rate of DMSA scintigraphy in patients with the degree III-V of VUR remained at 73% (8 of 11 patients). DMSA scintigraphy showed the defect even in 6 of 15 patients who received medication within 24 hours after onset of fever. The grade of VUR was improved from over III to under I in 7 of 11 infants.
 Conclusions:Effectiveness of prophylaxis was uncertain in this study. However, there are some infants with high grade VUR who showed an improving trend. So, prophylaxis seemed usefull in such patients. DMSA scintigraphy was not an alternative to VCUG in acute phase. There seemed to be no inevitability of changing the Practice Parameter from our data. Large-scale research would be needed considering the age and the degree of reflux.

View full abstract

 Alport syndrome (AS) is a hereditary and progressive glomerular nephritis that is frequently associated with sensorineural hearing loss and less ocular changes. It usually presents in children as haematuria and proteinuria, and progresses to end-stage renal disease (ESRD) increasing with age.
 AS is caused by abnormal type IV collagen which is composed of three α chains complex (α1-α1-α2 and α3-α4-α5, α5-α5-α6). The α3-α4-α5 component is expressed in the glomerular basement membrane (GBM). The α5-α5-α6 component is expressed in the Bowman's capsule but not in the GBM.
 AS is genetically heterogeneous and X-linked (XLAS), with both autosomal recessive (ARAS), and autosomal dominant (ADAS) has been clarified.
 XLAS accounts for approximately 85% of affected AS patients. It is caused by mutations in the COL4A5 gene on the X chromosome. XLAS is reported that 90% of male patients are at risk of developing ESRD before the age of 40, and females typically show milder clinical symptoms, although the probability of developing ESRD before the age of 40 year is 12%.
 ARAS accounts for about 15% of patients with AS. It is caused by homozygous mutations or compound heterozygous mutations in the COL4A3 or COL4A4 genes.
 ARAS usually presents most severe clinical findings. In our study, about 70% of ARAS from eight families) 71.4% of patients had reached ESRD until 15 years old. ARAS is suggested by presence of one or more of following criteria: (i) severe early disease in both females and males; (ii) parental consanguinity; (iii) absence of severe renal disease in the parents. Recently heterozygous mutations in COL4A3 or COL4A4 have been detected in 40% of families with thin basement membrane nephropathy (TBMN). Today understanding of the mode of inheritance for TBMN and ARAS is that heterozygotes with TBMN can be considered carriers for ARAS, and the offspring from a couple in which both parents have TBMN can be expected to get ARAS. We underline that when you examine the patient whose father and mother have histories of TBMN, should rule out ARAS.
 ADAS has been observed in a few families. It is caused by heterozygous mutations in the COL4A3 or COL4A4. Both female and male patients show a high clinical variability with renal phenotypes ranging from isolated haematuria to late onset ESRD. When EM findings show TBMN in young patients, it is not able to distinguish ADAS or TBMN. Immunohistochemical study of renal GBMs shows normal pattern in both ADAS and TBMN. Therefore we need the detailed family histories and to follow up the patients whether decrease renal function or not.
 In conclusion, we described mainly about the autosomal AS. Recently relations between ARAS and TBMN have revealed, AS may be the more frequent disease. It is not revealed what is the difference between ARAS and ADAS in molecular aspects, they have mutations in the same genes of COL4A3 or COL4A4. We will conduct molecular analysis in AS and reveal the mechanism and clinical findings of AS.

View full abstract

 There were some clinico-pathological grading systems of IgA nephropathy. The Committee of the Special Study Group on Progressive Glomeular Diseases, Ministry of Health, Labor and Welfare of Japan (MHLW) revised the clinical guidelines for IgA nephropathy, in 2002. The newly guideline of MHLW was in course of preparation, for adult. However, the old and new guidelines had not been verified enough, especially for the pediatric IgA nephropathy.
 This time, over 150 renal biopsies of pediatric IgA nephropathy were accumulated by corporation of The Chugoku-shikoku society for pediatric nephrology and analyzed by pathological guidelines. We explained the new and old guidelines of IgA nephropathy in Japan, and examined whether they were suitable or insufficient for the pathological evaluation of pediatric IgA nephropathy, including our data.

View full abstract

 We report the cases of two patients with nephropathy that occurred secondary to congenital cyanotic heart disease (CCHD). Few reports have described nephropathy in CCHD (NCCHD) because CCDH patients are usually not followed carefully for urinary abnormalities. Case 1, a 15-year-old girl, was cyanotic and diagnosed as pulmonary atresia immediately after birth. Suspected proteinuria was noted from 9 years old. At age 15, she received renal biopsy. She was diagnosed as NCCHD with segmental lesions caused by microthrombi. Case 2, that of a 14-year-old girl, was diagnosed as complexity of cardiac abnormality by nature and has remained in a condition of cyanosis. Suspected proteinuria was noted from age 12. She received renal biopsy at age 14. This case was diagnosed as NCCHD with intraglomerular megakaryocytes.
 As the major pathophysiology of NCCHD, hyperviscosity or polycythemia attributable to hypoxia causes an increase of intraglomerular pressure, leading to capillary dilatation or glomerular swelling. Glomerular disorders in CCHD are also reported in association with megakaryocyte infiltration. Usually, megakaryocytes pass through bone marrow sinusoids, enter the systemic venous flow, circulate to the lungs, and shed platelets at the lung. However, megakaryocytes and platelets transported via right to left shunt form microthrombi at glomeruli, release PDGF and or TGF-beta, and increase in the juxtaglomerular cellularity, mesangial cells, and mesangial matrix in CCHD patients. Actually, both our cases revealed segmental glomerular lesions, which are probably associated with megakaryocytes or microthrombi at glomeruli. Our two cases were under hypoxic conditions, but they showed improvement in their urinalysis results and retained normal renal function with treatment using antiplatelet agents and a renin-angiotensin system inhibitor. Risk factors for NCCHD remain indistinct because the backgrounds of underlying diseases, hemodynamics, and medicines vary among CCHD patients. We reported clinicopathological findings related to megakaryocytes or microthrombi in NCCHD. Long-term prognosis of CCHD has improved. The management of NCCHD is important to improve the quality of life in CCHD patients.

View full abstract

 We described the case of a 9-year-old girl with MPO-ANCA-associated glomerulonephritis, who developed acute pancreatitis, perforated duodenal ulcer and posterior reversible encephalopathy syndrome after steroid pulse therapy. She was suffering from mild proteinuria and hematuria. Laboratory examination showed a serum creatinin level of 0.71 mg/dl, and MPO-ANCA level of 198 EU. Renal biopsy revealed crescentic glomerulonephritis. On hospital day 8, we performed steroid pulse therapy on the patient with methylpredonisolone at 30mg/kg/day. On 2 days after the pulse therapy, she had abdominal pain with acute pancreatitis and we diagnosed her as having perforated duodenal ulcer on the basis of operative findings. On the next day she had a generalized seizure. We diagnosed her as having posterior reversible encephalopathy syndrome on the basis of head MRI findings. There is no report of children who developed acute pancreatitis with MPO-ANCA-associated glomerulonephritis, but are a few cases of adult who after steroid pulse therapy. We must take great care over another organs except kidney in case of MPO-ANCA-associated glomerulonephritis.

View full abstract

 We report a girl born at extremely-low birth (ELBW) developed proteinuria at six years of age. She was born at 25 weeks and one day of gestation with a birth weight of 624g. Proteinuria was found at six years of age by routine school urinalysis. Renal biopsy was performed at nine years of age. The renal biopsy findings showed diffuse increase in glomerular size without mesangial proliferation or glomerular sclerosis. Recently, increased risk of hypertension and microalbuminuria caused by the decreased number of glomerulus were reported in adult persons born at low birth-weight (LBW). We speculated that proteinuria was developed in early childhood because of the smaller number of glomerulus in this case born at ELBW as compared to the persons born at LBW. As the number of survivor of ELBW increases coupled with the advance in medicine, it is suspected that number of the cases who developed proteinuria at early childhood increases.

View full abstract

 Anticoagulant therapy is a very important strategy for children with nephrotic syndrome, because of their hyper-coagulated condition. For patients with steroid-resistant nephrotic syndrome who show poor response to initial steroid treatment, renal biopsy is preferably required for diagnosis and treatment. At the time of renal biopsy, however, anticoagulant therapy is usually discontinued to reduce the hemorrhagic risk for those with steroid-resistant nephrotic syndrome. In addition, it has been stated that children with steroid-resistant nephrotic syndrome have usually hypercoagulation state.
 We here report a 6-year-old boy with steroid-resistant nephrotic syndrome who developed deep venous thrombosis (DVT) in his left subclavian vein after renal biopsy. He needed the central venous catheter, because of the difficulty of blood drawing and establishment of an intravenous line for severe edema. At renal biopsy temporal discontinuation of anticoagulant therapy resulted in the production of DVT in his left subclavian vein. We immediately started warfarin administration and checked the affected vein periodically with vein echogram. DVT gradually disappeared and blood flow was finally reconstituted two months later.
 We can conclude that for steroid-resistant nephrotic syndrome, the usage of the anticoagulation therapy and central venous catheter should be carefully considered to prevent the deep venous thrombosis, especially at the time of renal biopsy.

View full abstract

 The patient was a 13-year-old male diagnosed to have nephrotic syndrome based on facial edema, proteinuria (4+) and hypoproteinemia (Total protein 4.8 g/dl, Alb 2.3 g/dl). He was referred for steroid resistant nephrotic syndrome. A renal biopsy revealed a diagnosis of focal segmental glomerulosclerosis. He achieved an incomplete remission with the administration of methylprednisolon (mPSL) pulse therapy (three days/week, 5 weeks), oral predonisolone (PSL), cyclosporin A (CyA) and lisinopril. However, he experienced a recurrence of edema and hypoproteinemia 7 months after the original onset. Finally, plasma exchange was performed because the administration of additional mizoribin proved to be ineffective. The proteinuria decreased at the time of PEX (three days/week), and the patient achieved a state of remission within three days. Steroid pulse therapy (three days/week) was thereafter administered in addition to PEX for two weeks. He remained in remission for 13 months after PEX. This patient achieved an incomplete remission with immune-suppressive therapy, but the introduction of PEX for the treatment of recurrence eventually allowed him to achieve a complete remission. The criteria for the selection of PEX remain to be clearly elucidated, but early induction was found to be very effective.

View full abstract

[in Japanese]

View full abstract

[in Japanese]

View full abstract