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Background and Aims High-flux dialyzer membrane material has been shown to improve mortality in hemodialysis (HD) patients. However, little is known about the impact of surface area of dialyzer membrane on prognosis in HD patients. A retrospective analysis was undertaken to investigate whether the difference in high-flux dialyzer membrane area affects survival rate. Method We observed 119 patients (43 females, mean age; 68 years, and HD duration; mean 84.4 months) maintained on HD for up to 5 years and 7 months. All patients were divided into two groups according to the surface area of dialyzer membranes; S group, 39 patients treated with the dialyzer membrane area of Results The patients were treated with four types of high-flux dialyzer membranes. S group included 21 patients with cellulose triacetate (CTA), 17 patients with polysulfone (PS) and 1 patient with polymethylmethacrylate. L group included 2 patients with CTA, 71 patients with PS and 7 patients with polyethersulfone (PES). Thirty-two patients, including 12 patients in S group and 20 patients in L group, had diabetes mellitus (DM). Survival rate was compared between the two groups, patients treated with CTA, PS and PES, and those with and without DM by Kaplan-Meier method. Type of dialyzer membranes used did not affect survival rate at the final observation (65.2% in CTA, 75% in PES and 50% in PS, p=0.26). The surface area of dialyzer membrane did not have a significant impact on mortality; survival rate at 3-year for S group, 71% vs L group, 77%, at 5-year for S group, 51% vs L group, 59%, and at the final observation for S group, 48%, vs L group, 58% (p=0.352). Among 32 diabetic patients, survival rate at the final observation did not differ between the two groups (S group, 33%, vs L groups, 45%, p=0.477). Among 87 non-diabetic patients (S groups; 27 patients), survival rate did not differ between the two groups at the final observation (S group, 56%, vs L group, 63%, p=0.575). Conclusion We conclude that difference in high-flux dialyzer membrane area is unlikely to affect survival rate in HD patients.

No literature review exists on Mycoplasma pneumoniae-associated mild encephalitis/encepharopathy with a reversible splenial lesion (MERS). M.pneumoniae-associated MERS cases were searched till August 2016 using PubMed/Google for English/other-language publications and Ichushi ( http://www.jamas.or.jp/ ) for Japanese-language publications. Inclusion criteria were children fulfilling definition for encephalitis, M.pneumoniae infection, and neuroimaging showing hyperintensity in the splenium of the corpus callosum (SCC) alone (type I) or SCC/other brain areas (type II). We described two children with type I and II M.pneumoniae-associated MERS. Thirteen cases found by the search and our 2 cases were reviewed. Mean age, male/female ratio, duration of prodromal illness was 8.3 years, 1.5 and 3.5 days. The most common neurological symptom was drowsiness, followed by abnormal speech/behavior, ataxia, seizure, delirium, confusion, tremor, hallucination, irritability, muscle weakness, and facial nerve paralysis. Fever was the most common non-neurological symptom, followed by cough, headache, gastrointestinal symptoms, headache, lethargy and dizziness. Seizure and respiratory symptoms were less common. All were diagnosed for M.pneumoniae by serology. Cerebrospinal fluid (CSF) M.pneumoniae was undetectable by PCR in the 3 patients. Three patients were clarithromycin-resistant. Leukocytosis, positive C-reactive protein, hyponatremia, CSF pleocytosis and slow wave on electroencephalography frequently occurred. All except 2 were type I MERS. Neuroimaging abnormalities disappeared within 18 days in the majority of patients. All type I patients completely recovered within 19 days. Two type II patients developed neurological sequelae, which recovered 2 and 6 months after onset. Prognosis of M.pneumoniae-associated MERS is excellent. Type II MERS may increase a risk of neurological sequelae.

Iron deficiency anemia (IDA) is a major problem in chronic kidney disease (CKD), causing increased mortality. Ferritin stores iron, representing iron status. Hepcidin binds to ferroportin, thereby inhibiting iron absorption/efflux. Inflammation in CKD increases ferritin and hepcidin independent of iron status, which reduce iron availability. While intravenous iron therapy (IIT) is superior to oral iron therapy (OIT) in CKD patients with inflammation, OIT is as effective as IIT in those without. Inflammation reduces predictive values of ferritin and hepcidin for iron status and responsiveness to iron therapy. Upper limit of ferritin to predict iron overload is higher in CKD patients with inflammation than in those without. However, magnetic resonance imaging studies show lower cutoff levels of serum ferritin to predict iron overload in dialysis patients with apparent inflammation than upper limit of ferritin proposed by international guidelines. Compared to CKD patients with inflammation, optimal ferritin levels for IDA are lower in those without, requiring reduced iron dose and leading to decreased mortality. The management of IDA should differ between CKD patients with and without inflammation and include minimization of inflammation. Further studies are needed to determine the impact of inflammation on ferritin, hepcidin and therapeutic strategy for IDA in CKD.

Background: We determined optimal serum ferritin for oral iron therapy (OIT) in hemodialysis (HD) patients with iron deficiency anemia (IDA)/minor inflammation, and benefit of intravenous iron therapy (IIT) for OIT-nonresponders. Methods: Inclusion criteria were IDA (Hb 3 months before the study. ΔHb ≥ 20 g/L above baseline or maintaining target Hb (tHB; 120–130 g/L) was considered responsive. Fifty-one patients received OIT (ferrous fumarate, 50 mg/day) for 3 months; this continued in OIT-responders but was switched to IIT (saccharated ferric oxide, 40 mg/week) in OIT-nonresponders for 4 months. All received continuous erythropoietin receptor activator (CERA). Hb, ferritin, hepcidin-25, and CERA dose were measured. Results: Demographics before OIT were similar between OIT-responders and OIT-nonresponders except low Hb and high triglycerides in OIT-nonresponders. Thirty-nine were OIT-responders with reduced CERA dose. Hb rose with a peak at 5 months. Ferritin and hepcidin-25 continuously increased. Hb positively correlated with ferritin in OIT-responders (r = 0.913, p = 0.03) till 5 months after OIT. The correlation equation estimated optimal ferritin of 30–40 ng/mL using tHb (120–130 g/L). Seven OIT-nonresponders were IIT-responders. Conclusions: Optimal serum ferritin for OIT is 67.4–89.9 pmol/L in HD patients with IDA/minor inflammation. IIT may be a second line of treatment for OIT-nonreponders.

We present 32- and 38-year-old males with Schizophyllum commune-induced allergic fungal rhinosinusitis (AFRS). S. commune-induced AFRS was diagnosed by clinical and radiographic findings, positive specific IgE antibodies against S. commune as measured by the ImmunoCAP system, and sequencing analysis of the fungus. Our two cases with S. commune-induced AFRS for the first time showed evidence for type 1 hypersensitivity to S. commune as determined by using specific IgE antibodies against S. commune, and the fungus was identified by sequence analysis.

Iron is an essential element for all living organisms, but produces toxic oxidants. Thus, iron homeostasis is tightly regulated in mammals. Hepcidin-25 (hepcidin) has emerged as a molecule that regulates iron metabolism. Binding of hepcidin to its receptor, ferroportin, inhibits intestinal iron absorption and iron efflux from hepatocytes and macrophages. Decreased hepcidin enhances iron absorption and efflux. Hepcidin could be predictive of iron status and the response to iron supplementation or erythropoietin-stimulating agents. Monitoring hepcidin is helpful for the management of anemia. Thus, it is urgent to obtain normal reference values in a large population of healthy subjects and to standardize various hepcidin assays, which enables to compare the data measured by different methods. Anemia is an important and common problem associated with chronic kidney disease (CKD), which is caused by erythropoietin deficiency, iron-restricted erythropoiesis, inflammation, hypoxia, vitamin D deficiency, hyperparathyroidism, and obesity. Anemia causes poor quality of life, progression of CKD, increased risk of cardiovascular events, and mortality. Besides its role in anemia, recent evidence suggests that hepcidin-25 plays a role in the pathogenesis and progression of kidney injury via modulation of iron-mediated oxidant injury. Despite accumulating experimental data, information about clinical significance of hepcidin-25 for anemia and kidney injury in CKD patients is scarce, especially in children. This review summarizes the current knowledge of the role of hepcidin-25 in the regulation of anemia and kidney injury in children and adults with CKD. Strategy for modulating hepcidin-25 to prevent anemia and kidney injury associated with CKD is also discussed.

The benefit of oral iron therapy (OIT) and factors predictive of OIT response are not established in hemodialysis (HD) patients with iron deficiency anemia (IDA). We examined the values of hepcidin-25, mean corpuscular volume (MCV), and ferritin as predictors of OIT response. Oral ferrous fumarate (50 mg/day, 8 weeks) was given to 51 HD patients with IDA (hemoglobin (Hb) &lt, 12 g/dL, ferritin &lt, 100 ng/mL) treated with an erythropoietin activator. Sixteen patients were responders (improvement of Hb (ΔHb) ≥ 2 g/dL) and 35 were non-responders (ΔHb &lt, 2g/dL). Baseline Hb, MCV, serum hepcidin-25, ferritin, iron parameters, and C-reactive protein (CRP) before and ΔHb after OIT were compared between groups. Hepcidin-25, MCV, ferritin, and transferrin saturation were lower in the responders than in the non-responders. Hepcidin-25 positively correlated with ferritin. Hepcidin-25, MCV, and ferritin positively correlated with baseline Hb and negatively correlated with ΔHb. Despite normal CRP levels in all patients, CRP correlated positively with hepcidin-25 and ferritin. Stepwise multiple linear regression analysis and receiver operating characteristics curve analysis revealed that hepcidin-25, MCV, and ferritin could predict OIT response. We conclude that hepcidin-25, MCV, and ferritin could be useful markers of iron storage status and may help predict OIT response in HD patients.

Background/Aims: We examined whether regulation of hepcidin-25 by short- or long-acting recombinant human erythropoietin (rhEPO) is dependent on ferritin and predicts the response to rhEPO in hemodialysis (HD) patients. Methods: Two studies with rhEPO were performed in 9 HD patients with a 2-year interval. Serum hepcidin-25 was measured at 0-18 h after intravenous epoetin-β (EPO) or methoxy polyethylene glycol-epoetin-β (PEG-EPO) administration and on days 3-7 after PEG-EPO. Hemoglobin (Hb), serum ferritin, transferrin, C-reactive protein (CRP), and interleukin (IL)-6 were analyzed before hepcidin measurement and 6 months after rhEPO. Based on the serum ferritin levels before hepcidin measurement, the patients in the two studies with EPO or PEG-EPO were combined into low (11; serum ferritin of Results: The serum hepcidin-25 levels rose at 6-9 h and returned to the baseline at 18 h after EPO. They rose at 6-9 h, returned to the baseline at 18 h, and decreased on day 5-7 after PEG-EPO. Serum hepcidin-25 levels were low (Conclusion: Regulation of hepcidin-25 by rhEPO may be dependent on ferritin, affecting the response to rhEPO in HD patients.

It remains elusive what factors induce growth without growth hormone (GWGH) in children after neurosurgery of brain tumors. Growth velocity and endocrinological data were compared between the patients with and without GWGH. We experienced three patients with GWGH (median, 12 years; 2 germinoma and 1 craniopharyngioma; three females; group 1) and 11 patients without (12 years; 8 craniopharyngioma, 2 germinoma and 1 medulloblastoma; 7 males; group 2) after neurosurgery. All patients in group 2 received GH replacement therapy. Growth velocity and endocrinological data were compared. Median height velocity was normal in group 1 (5.5 cm/year), but low in group 2 (2.2 cm/year), which improved after GH replacement therapy (7.0 cm/year). Median serum insulin level was increased in group 1 (87.0 μU/ml, P < 0.05) compared with normal level in group 2 (10.0 μU/ml). Despite hyperinsulinemia, serum glucose level was normal in group 1. Three of 5 with hyperinsulinemia and 2 of 9 without were obese or overweight, but the difference was not significant. Current body mass index and serum levels of IGF-1, IGFBP-3, leptin, and prolactin were similar between groups. Serum estradiol was prepuberty level in group 1. Hyperinsulinemia may induce GWGH in children with brain tumors after neurosurgery.

Neurogenic-mediated inflammation may be associated with several inflammatory skin diseases including atopic dermatitis. However, age-dependent differences in neurogenic-mediated skin responses are not fully understood. We compared skin plasma leakage in rats aged 2 and 8 wk, which was induced by topical capsaicin, topical formalin, and intracutaneous substance P, whose effects are mediated via tachykinin NK1 receptors. Evans blue dye extravasation served as an index of the increase in skin vascular permeability. Capsaicin, formalin, and substance P caused a skin response in a dose-dependent manner in both age groups. However, the skin response was much greater in adults than in pups. In addition, the localization of sensory C-fibers and tachykinin NK1 receptors in the skin was investigated by immunofluorescent staining with antisubstance P and antitachykinin NK1 receptor antibodies, respectively. Substance P-immunoreactive nerves were detected throughout the dermis and tachykinin NK1 receptors were mainly detected in blood vessel walls in the dermis in both age groups. However, they were more sparsely distributed in pups. In conclusion, the weak neurogenic-mediated skin inflammation in pups is probably because of immature neural mechanisms associated with skin inflammation such as reduced innervation of sensory C-fibers and low expression of tachykinin NK1 receptors.

Race, young age, male gender, severe dehydration, metabolic acidosis, drowsiness/unconsciousness and shock are associated with gastroduodenal perforation/ulcer (GDPU) in childhood rotaviruses and noroviruses gastroenteritis. Awareness of the risk of GDPU prevents lethal outcome in these common infections among young children., Background. There is no literature review on gastroduodenal perforation or ulcer (GDPU) with rotavirus (RV) and norovirus (NoV) gastroenteritis. Methods. Pediatric cases of GDPU or upper gastrointestinal bleeding with RV and NoV gastroenteritis were searched from September 1974 until October 2015 using PubMed, Google for English, other-language-publications, and Ichushi (http://www.jamas.or.jp) for Japanese-language publications. All reports confirming GDPU or upper gastrointestinal bleeding with RV and NoV gastroenteritis were eligible for inclusion in the study. In addition, clinical characteristics were reviewed. Results. A boy with duodenal ulcer (DU) and NoV gastroenteritis was described. There were 32 GDPU cases (23 RVs and 9 NoVs cases), including our case; with the exception of 1 case, all were Japanese. Mean age, male/female ratio, and symptoms' duration before admission were 21.6 months, 2.2, and 4.0 days, respectively. Vomiting was the most common symptom, followed by diarrhea, lethargy, fever, abdominal distension, and convulsion. Dehydration, hematemesis, melena, drowsiness or unconsciousness, shock, metabolic acidosis, leukocytosis, anemia, positive C-reactive protein, high blood urea nitrogen, and hyponatremia commonly occurred. Helicobacter pylori was a minor cause of GDPU. Duodenal (DP) or gastric perforation (GP) developed in 14 cases (10 DP/RVs, 1 GP/RV, and 3 DP/NoVs). Duodenal ulcer or gastric ulcer (GU) developed in 18 cases (10 DU/RVs, 4 DU/NoVs, 1 GU/RV, 1 GU + DU/NoV, and 2 upper gastrointestinal bleeding/RVs). The predominant perforation or ulcer site was in the duodenum. With the exception of 2 deaths from DU, all cases recovered. Conclusions. Race, young age, male, severe dehydration, metabolic acidosis, drowsiness and unconsciousness, and shock may be potential risk factors of GDPU associated with RV and NoV gastroenteritis. Limitation of this descriptive study warrants further investigations to determine the risk factors in these infections that could be associated with GDPU.

BACKGROUND: Aplasia cutis congenita (ACC) is a condition in which localized or widespread areas of skin are absent at birth. Defective lesions show complete absence of all layers of skin, occasionally extending to skull or dura. ACC is etiologically heterogeneous; many different etiologies including teratogens have been documented. CASE: We describe the first reported case of a monozygotic twin with ACC after exposure to methimazole in utero. The female patient was born at 36 weeks gestation as the first child of monozygotic twins. The mother received methimazole between the 11th and 17th weeks of pregnancy because of transient hyperthyroidism. The second child did not have ACC. The patient had defects of the scalp, skull, and dura (7 × 5 cm) on the sagittal line of the parieto-occipital region. No other malformations were noted. The scalp defect has been treated daily with sterile physiological saline and petrolatum dressing in addition to intravenous antibiotics. Trafermin, a recombinant human fibroblast growth factor, was sprayed from day 6 to promote epithelialization of the scalp defect. On day 21, she had high fever due to infection of the defect lesion, which was controlled by povidone iodine dressing and intravenous antibiotics. The defect of the scalp was well healed after 6 weeks, but the skull defect remained unclosed. CONCLUSIONS: We describe a rare case of a monozygotic twin with ACC and skull defect after methimazole exposure in utero. The findings of our case suggest that methimazole is a potential teratogen of ACC. Birth Defects Research (Part A) 2007. © 2007 Wiley-Liss, Inc.

Key Clinical Message Norovirus (NoV) and rotavirus (RV) gastroenteritis are usually self-limiting. However, few pediatric cases of bowel perforation and no duodenal perforation with NoV gastroenteritis were reported. We describe two children with duodenal perforation due to NoV or RV gastroenteritis. Suspicion for this association enables prompt intervention, preventing lethal outcomes of these common infections.

Little is known about the risk factors for long-term poor outcome in pediatric renal transplantation. Between 1973 and 2010, 111 renal transplants (92 living donations) were performed in 104 children (56 males, mean age, 12.5 yr) at the Social Insurance Chukyo Hospital, and followed-up for a mean period of 13.6 yr. The patient survival at 1, 5, 10, 15, 20 (living- and deceased-donor transplants), and 30 yr (living-donor transplants only) was 98.1%, 92.8%, 87.8%, 84.9%, 82.6%, and 79.3%. The graft survival at 1, 5, 10, 15, 20, and 30 yr was 92.0%, 77.3%, 58.4%, 50.8%, 38.5%, and 33.3%. The most common cause of graft loss was CAI, AR, death with functioning, recurrent primary disease, ATN, and malignancy. Donor gender, ATN, malignancy/cardiovascular events, and eras affected patient survival. AR and CAI were the risk factors for graft loss. The evolved immunosuppression protocols improved the outcome by reducing AR episodes and ATN but not CAI, suggesting CAI as the major risk factor for graft loss. CAI was correlated with AR episodes, CMV infection, and post-transplant hypertension. Strategies for preventing the risk factors for malignancy/cardiovascular events and CAI, including hypertension/infection, are crucial for better outcomes.

Hypothalamo-pituitary dysfunction is commonly seen in children with brain tumors after neurosurgery, resulting in endocrine disorders, including growth hormone deficiency (GHD). GHD causes growth failure, and thus the majority of the patients need GH replacement therapy. Despite GHD, some patients grow normally or excessively, which has been recognized as growth without GH (GWGH). Since hyperinsulinemia is highly associated with the patients with GWGH, it has been considered to play an important role for promoting linear growth. However, the causes of hyperinsulinemia associated with GWGH and the mechanisms of GWGH remain elusive. The data to date collectively suggest that the location of brain tumors and/or the mode of surgery, insulin resistance associated with obesity, insulin-like growth factor (IGF)-I, IGF binding protein (IGFBP)-1 and −3, leptin and prolactin (PRL) may contribute to the regulation of insulin secretion in patients with GWGH. Besides its metabolic effects, insulin exerts the diverse effects as a growth factor, including proliferation and differentiation of osteoblasts, leading to acceleration of linear growth. Interaction between insulin- and IGF-I-induced signaling pathways and of insulin with other hormonal factors such as leptin or PRL may play a crucial role for promoting linear growth in patients with GWGH. Despite normal or excessive linear growth, patients with GWGH still have metabolic abnormalities and retarded bone maturation. It remains elusive whether GH replacement therapy solves these problems and enables the patients to reach normal height in adulthood. Better understanding for the mechanisms of GWGH is essential to improve the quality of life of patients with GWGH. Because of a lack of accumulating data, further studies would be necessary to clarify the clinical characteristics and the mechanisms of GWGH in more detail in the future.

Sequential neuroimaging before and after seizures has not been reported in patients with nephrotic syndrome and recurrent posterior reversible encephalopathy syndrome (PRES). We report a 13-year-old nephrotic female patient with recurrent PRES during and after cessation of cyclosporine A (CyA). She had headache, visual disturbance and acute hypertension, followed by seizures. The brain magnetic resonance imaging (MRI) before seizures revealed a high signal intensity area on fluid-attenuated inversion recovery and diffusion-weighted images and a low signal intensity area on T1-weighted images in bilateral parieto-occipital and left temporal lobes. Cessation of CyA resulted in clinical improvement. The follow-up MRI 2 months after the initial episode showed a complete resolution. Six months later, she had similar symptoms, edema, severe hypoalbuminemia, renal insufficiency, and acute pancreatitis before seizures. The brain MRI after seizures showed similar abnormalities in the same regions, which completely resolved 2 months later. Recurrence of acute severe hypertension, nephrotic state (edema/ hypoalbuminemia), and renal insufficiency may lead to recurrent PRES and thus early treatment of trigger factors, especially of hypertension, is important to reduce the episodes of PRES.

It remains elusive what factors affect posterior reversible encephalopathy syndrome (PRES). Eleven PRES children, all with acute glomerulonephritis, Alport syndrome, and lupus nephritis, 5 with nephrosis, and 3 renal transplant recipients, were studied. PRES recurred in 1 patient. Neurological symptoms were graded as 1: mild (headache, nausea/vomiting, or tremor), 2: moderate (vision change), and 3: severe (mental dysfunction, cerebellar symptoms, seizures, recurrence of seizures, and coma). Magnetic resonance imaging was graded as 1: subtle change, 2: abnormal large areas, and 3: complete involvement of the regions. The common symptoms were seizures (100%), headache (82%), nausea/vomiting (73%), coma (55%), and vision change (46%). Seizures recurred in 7 (64%). All but one (91%) developed hypertension and 7 (64%) received calcineurin inhibitors (CNI). Edema occurred in 7 (64%) and renal insufficiency/end-stage renal disease (ESRD) in 4 (36%). Seizures recurred frequently in younger patients. Symptoms were severe in girls. Duration or severity of the condition with predisposing factors (hypertension, CNI, nephrosis or renal insufficiency/ERSD) did not make a difference in the symptoms and neuroimaging. Two patients developed chronic epilepsy. Age and gender may affect PRES symptoms. Our results are limited by small sample size and should be determined using larger numbers of patients.

Effect of corticosteroids (steroids) on some hemostatic parameters was serially studied in 23 children with minimal change nephrotic syndrome (MCNS). Increased platelet count, erythrocyte sedimentation rate (ESR), cholesterol, fibrinogen, fibrin(ogen) degradation products (FDP), alpha 2-macroglobulin (alpha 2M), alpha 2-antiplasmin (alpha 2AP) and protein C, and reduced antithrombin III (ATIII) and plasminogen (Plg) were noted in relapse before steroid therapy began. With institution of oral prednisolone, FDP started to fall, and platelet count, cholesterol, alpha 2M, ATIII, Plg, alpha 2AP and protein C started to increase despite unchanged nephrotic state from that before the therapy. In remission induced by prednisolone, platelet count, cholesterol, alpha 2M, ATIII, Plg, alpha 2AP and protein C were still increased, but normalized off therapy. ESR, fibrinogen, FDP, alpha 2M and protein C correlated inversely with serum albumin and directly with cholesterol and urine protein excretion. In contrast, ATIII and Plg correlated directly with serum albumin and inversely with cholesterol and urine protein excretion. A direct correlation was only noted between alpha 2AP and the dose of prednisolone. The data indicate that steroids appear to be a thrombogenic factor by accerelating thrombocytosis and hyperlipidemia, and by reducing plasma fibrinolysis in children with MCNS.

Sir, Here, we report a neonate with severe oligo-anuric renal failure during multi-organ failure who survived with prolonged renal replacement therapy. A female neonate, born at a gestational age of 39 weeks and 3 days, weighing 2780 gram, presented cyanosis on Day 15. She developed anuria and respiratory arrest, requiring mechanical ventilation. Blood pressure was 39/24 mmHg. She was diagnosed as having interrupted aortic arch, requiring surgery (Figure 1). Serum creatinine increased from 0.34 to 1.26 mg/dL. Continuous-flow peritoneal dialysis (PD), using two catheters for dialysate infusion and drainage simultaneously, was started. She had septicemia, which was controlled with antibiotics. After surgery, chylous effusion occurred in the thorax, resulting in pleural effusion and edema. At ∼2 months, she passed small volumes of urine output with renal and dialytic clearances of 0.98 and 11.45 mL/min/1.73 m2, respectively. Ligation of the thoracic duct was performed at 2.5 months. Overfluid status continued till 3 months, requiring PD and mechanical ventilation. Fig. 1. Course of the illness in the neonate. Cdial, dialytic clearance; Cr, creatinine; Crenal, renal clearance; Inf, influenza A virus infection; PN, peritonitis; RS, respiratory syncytial virus infection; RV, rotavirus infection; UV, urine volume. Because of frequent vomiting, she was fed with an intestinal tube at 4.5 months, resulting in severe diarrhea for 1 month. She remained anuric at 5 months despite decreased serum creatinine (1.36 mg/dL) due to malnutrition. Renal clearance was 1.68 mL/min/1.73 m2 at 6 months when urine output was redetected. She had episodes of peritonitis and virus infections, resulting in pleural effusion, requiring antibiotics, supportive therapy and PD. Renal clearance increased to 8.44 mL/min/1.73 m2 at 8 months. Urine output increased with serum creatinine of 0.81–1.2 mg/dL and renal clearance of 10.4 mL/min/1.73 m2 at 10 months, representing almost 50% of total clearance. Dialysis was stopped 1 year after surgery. Her growth and development are normal with glomerular filtration rate of 24.7 mL/min/1.73 m2. Prolonged oligo-anuria correlates with the final outcome [1]. Renal function recovered after 6–14 months of oliguria in two children with hemolytic uremic syndrome (HUS), in whom plasma infusion prolonged acute renal failure (ARF) [2], and after 23 days of oliguria in an infant with HUS, at the age of 23 weeks [3]. Younger age is another poor prognostic factor of ARF [4]. Neonates have a decreased number and function of nephron, resulting in severe renal insult and poor outcome [5]. ARF occurs in 8% of neonates in the intensive care unit, and its mortality rate is 48% [4], depending on the underlying disease and prognostic factors, including respiratory support, hypotension, cardiac disorders, surgery, oliguria/anuria, need for vasopressor drugs or dialysis, sepsis or multi-organ failure [1]. Despite these factors, the renal function of our patient returned to an extent that allowed cessation of dialysis after many months although PD might have been stopped late. Our patient still has significant chronic kidney disease and will require renal replacement therapy again in the future. Our observation suggests that neonates with severe oligo-anuric renal failure during multi-organ failure may survive with prolonged renal replacement therapy.

It has been generally accepted that a catastrophic breakdown of regulated cellular homeostasis, known as necrosis, is the mode of cellular injury in various forms of acute renal failure. One of the major advances in our understanding of cell death has been the recognition that the pathways traditionally associated with apoptosis as described in the landmark study by Kerr, Wyllie, and Currie in 1972 may be very critical in the form of cell injury associated with necrosis. The pathway that is followed by the cell varies with both nature and severity of insults and may evolve from an apoptotic to a necrotic form of cell death. It is also likely that there are some common pathways that are shared and regulated in the two modes of cell death. In this review, we first describe evidence for the role of apoptotic pathways in ischemic acute renal failure, and then consider the potential mechanisms that may participate in this model of acute renal tubular injury. We then summarize the current information of apoptotic pathways related to other common causes of acute renal failure including endotoxin-induced, toxic acute renal failure and transplant rejection. A better understanding of the mechanisms of apoptosis could lead to safer and more specific therapeutic interventions for acute renal failure.

Over the last decade, there is accumulating evidence for a role of reactive oxygen metabolites in the pathogenesis of a variety of renal diseases, including gentamicin, glycerol, cisplatin, and cyclosporine A models of toxic acute renal failure. Gentamicin has been shown both in in vitro and in vivo studies to enhance the generation of reactive oxygen metabolites. Iron is important in models of tissue injury, presumably because it is capable of catalyzing free-radical formation. Gentamicin has been shown to cause release of iron from renal cortical mitochondria. Scavengers of reactive oxygen metabolites as well as iron chelators provide protection in gentamicin-induced nephrotoxicity. In glycerol-induced acute renal failure, an animal model of rhabdomyolysis, there is enhanced generation of hydrogen peroxide, and scavengers of reactive oxygen metabolites and iron chelators provide protection. Although the dogma is that the myoglobin is the source of iron, recent studies suggest that cytochrome P450 may be an important source of iron in this model. In addition, there are marked alterations in antioxidant defenses, such as glutathione, as well as changes in heme oxygenase. Several recent in vitro and in vivo studies indicate an important role of reactive oxygen metabolites in cisplatin-induced nephrotoxicity. Thus, catalytic iron is increased both in vitro and in vivo by cisplatin, and iron chelators as well as hydroxyl radical scavengers have been shown to be protective. Recent studies indicate that cytochrome P450 may also be an important source of the catalytic iron in cisplatin nephrotoxicity. Cyclosporine A has been shown to enhance generation of hydrogen peroxide in vitro and enhance lipid peroxidation in vitro and in vivo. Antioxidants have been shown to be protective in cyclosporine A nephrotoxicity. This collective body of evidence suggests an important role for reactive oxygen metabolites in toxic acute renal failure and may provide therapeutic opportunities of preventing or treating acute renal failure in humans.

Oral iron administration (OIA) is little benefit, and I.V. iron injection (IVI) is recommended. IVI increases high serum ferritin (ferr) levels, increaseing serum hepcidin-25(hepc) levels, which inhibits intestinal iron absorption. In a state of low hepc levels, intestinal iron absorption can be well preserved. We examined whether smaller or normal MCV affects the therapeutic effect of OIA in HD patients with low hepc levels. Serum hepc were measured in 66 patients on HD. Of these, oral iron was immediately administered in 14 cases who had low serum hepc levels (

The clinicopathologic features and the response to corticosteroid therapy were compared in 9 patients with minimal change nephrotic syndrome (MCNS) and diffuse mesangial IgM deposits (Group I) and in 32 of those without IgM deposits (Group II). However, serum IgM levels in Group I in both relapse and remission were significantly higher than those of Group II and controls (p

The effect of dipyridamole on proteinuria was studied in 60 children with various renal diseases. A significant decrease in 24-hour urine protein excretion was observed within a few months after treatment in 32 (53%) of the patients with minimal or moderate mesangial proliferation. The effect was reproducible and parallelled by a reduction in plasma levels of beta-thromboglobulin. Renal function in patients significantly improved with the therapeutical effect. The appropriate dosage was 4-10 mg/kg daily and no serious toxicity was seen despite large dosage and even in long-term application. The data suggest that dipyridamole treatment appears safe and has a beneficial effect on proteinuria dependent on its effect on platelets in renal disease.

Forty-six children with steroid-responsive nephrotic syndrome were randomly allocated to receive two different prednisolone regimens for initial therapy. Twenty-nine children (group 1) received an intermittent regimen (60 mg/m2/day for 4 weeks, followed by 40/mg/m2/day on 3 days a week for 4 weeks); 17 children (group 2) had a long-term regimen (60 mg/m2/day for 4 weeks, followed by the same dose on alternate days for 4 weeks and the doses tapered by 10 mg/m2, given on alternate days every 4 weeks for 5 months). There was no difference between the two groups in the regimen used to treat relapses, steroid responsiveness, number of patients with relapses, and frequency of toxic reactions to steroids. However, the number of patients with a relapse within 6 months after initial therapy and the number of those with frequent relapses or steroid dependence were significantly higher in group 1 than in group 2 (P less than 0.05 for both). The data indicate that the long-term tapering regimen appears to be both safe and preferable to the intermittent regimen for initial therapy in children with idiopathic nephrotic syndrome.

Prothrombin time (PT), activated partial thromboplastin time (APTT) and plasma procoagulant activities were studied in 38 children with nephrotic syndrome in the presence or absence of prednisolone therapy. PT was normal but APTT was prolonged during relapse in untreated patients. Increased factors V, VII, VIII, XI and XIII in both treated and untreated and factor IX in treated patients, as well as decreased factors X and XII in untreated patients, were observed during relapse. These coagulation factor changes were unrelated either to the dose of prednisolone or underlying renal histology and normalized with clinical remission. However, plasma levels of factors II, V, VIII, IX, X and XI were still increased in treated patients. The data suggest that corticosteroids shorten APTT, raise both intrinsic and extrinsic factors, and therefore have favorable and unfavorable effects on the coagulation system in children with nephrotic syndrome.

Two children, one with renal manifestations of systemic lupus erythematosus and the other with idiopathic nephrotic syndrome, were treated with methylprednisolone pulses. Neither had previous evidence of underlying cardiac disease. Within 24 h of pulse therapy, they complained of palpitations and developed atrial fibrillation which reversed spontaneously or after anti-arrhythmic therapy. Subsequent serial electro- and echocardiograms were normal. We propose that the arrhythmias were a complication of steroid pulse therapy.