Acute renal failure with severe loin pain and patchy renal vasoconstriction in a patient with march hemoglobinuria.

Acute renal failure (ARF) with severe loin pain and patchy renal vasoconstriction is a rare syndrome described as an anaerobic exercise-induced acute kidney injury (AKI) without rhabdomyolysis. The characteristic computed tomography (CT) finding is multiple patchy wedge-shaped delayed contrast enhancement in the kidney. The syndrome is named as a clinical syndrome of ARF with severe loin pain after anaerobic exercise (ALPE). A 16-year-old Japanese boy was admitted to our hospital for vomiting and severe loin pain after Japanese fencing. He had kidney dysfunction with slightly increased serum creatine phosphokinase and was eventually diagnosed with ALPE based on the characteristic finding on delayed renal CT scans. He had no predisposing factors for ALPE, such as renal hypouricemia or analgesic use prior to the exercise; however, he had pigmenturia at the onset, which is an unusual finding for ALPE. The pigmenturia proved to be hemoglobinuria due to intravascular hemolysis, indicated by increased serum levels of indirect bilirubin and lactate dehydrogenase, an undetectable level of serum haptoglobin, and absence of red blood cells and myoglobin in the urine. The hemolysis following strenuous steps on a hard floor suggested that the phenomenon was march hemoglobinuria. Our patient is the first reported example in which ALPE developed in the setting of hemoglobinuria due to mechanical intravascular hemolysis.

Oxidized trilinoleate and tridocosahexaenoate induce pica behavior and change locomotor activity.

Pica behavior, a behavior that is characterized by eating a nonfood material such as kaolin and relates to the degree of discomfort in animals, and the variations of locomotor activity of rats after eating deteriorated fat and oil extracted from instant noodles were examined in our previous study. The result shows that oxidized fat and oil with at least 100 meq/kg in peroxide value (PV) increase pica behavior and decrease locomotor activity. In the present study, the same two behaviors were measured using autoxidized trilinoleate (tri-LA) and tridocosahexaenoate (tri-DHA) as a model of vegetable and fish oil, respectively, to compare fatty acid differences against the induction of two behaviors. The oxidized levels of tri-LA and tri-DHA were analyzed with PV and p-anisidine value (AnV), the method to analyze secondary oxidized products. The oxidation levels of respective triacylglycerol (TAG) samples were carefully adjusted to make them having almost the same PV and AnV. As the results, 600 or more meq/kg in PV of both TAGs significantly increased the consumption of kaolin pellets compared to the control group. Furthermore, 300 or more meq/kg in PV of tri-LA and 200 or more meq/kg in PV of tri-DHA demonstrated significant decrease in locomotor activity compared to control group. These results would indicate that the oxidized TAG having the same PV and/or AnV would induce the same type of pica behavior and locomotor activity. Furthermore, that the structure of oxidized products might not be important and the amount of hydroperoxide group and/or aldehyde group in deteriorated fats and oils might affect the pica behavior and locomotor activity were thought.

The clinical characteristics of vitamin D deficiency in childhood: a systematic literature review of Japanese patients.

To describe the characteristics of children with vitamin D deficiency, we reviewed the reports of vitamin D deficiency among Japanese children that were published between 1989 and 2008. We identified 25 patients with vitamin D deficiency in 9 published studies and evaluated their clinical characteristics together with those of 3 patients we recently treated. The patients were distributed in two distinct age groups at diagnosis: < 1 year old and > or = 1 year old. The main symptom of the < 1 year old age group was hypocalcemic convulsions and that of the > or = 1 year old age group was bowed legs. Serum calcium, intact PTH, and 1,25(OH)2D levels were significantly lower in the < 1 year age group than in the > or = 1 year age group. It would be useful to find and make early interventions in cases of children at a high-risk of vitamin D deficiency.

HDM2 impairs Noxa transcription and affects apoptotic cell death in a p53/p73-dependent manner in neuroblastoma.

HDM2, a human homologue of MDM2, is a major negative regulator of p53 function, and increased expression of HDM2 by its promoter polymorphism SNP309 resulted in p53 inactivation and an increased risk of several tumours, including neuroblastoma (NB). Herein, we show that increased expression of HDM2 is related to a worse prognosis in MYCN-amplified NB patients. HDM2 plays an important role in the expression of Noxa, a pro-apoptotic molecule of the Bcl-2 family, which induces NB cell apoptotic death after doxorubcin (Doxo) treatment. Knockdown of HDM2 by siRNA resulted in the upregulation of Noxa at mRNA/protein levels and improved the sensitivity of Doxo-resistant NB cells, although these were not observed in p53-mutant NB cells. Noxa-knockdown abolished the recovered Doxo-induced cell death by HDM2 reduction. Intriguingly, resistance to Doxo was up-regulated by over-expression of HDM2 in Doxo-sensitive NB cells. By HDM2 expression, p53 was inactivated but its degradation was not accelerated, suggesting that p53 was degraded in a proteasome-independent manner in NB cells; downstream effectors of p53, p21(Cip1/Waf1) and Noxa were suppressed by HDM2. Noxa transcription was considerably regulated by both p53 and p73 in NB cells. Furthermore, in vivo binding of p53 and p73 to Noxa promoter was suppressed and Noxa promoter activation was inhibited by HDM2. Taken together, our results may indicate that the HDM2-related resistance to chemotherapeutic drugs of NB is regulated by p53/p73-dependent Noxa expression in NB.

Effects of blue light deficiency on acclimation of light energy partitioning in PSII and CO2 assimilation capacity to high irradiance in spinach leaves.

Blue light effects on the acclimation of energy partitioning characteristics in PSII and CO2 assimilation capacity in spinach to high growth irradiance were investigated. Plants were grown hydroponically in different light treatments that were a combination of two light qualities and two irradiances,i.e. white light and blue-deficient light at photosynthetic photon flux densities (PPFDs) of 100 and 500 micromol m(-2) s(-1). The CO2 assimilation rate, the quantum efficiency of PSII(PhiPSII) and thermal dissipation activity (F(v)/F(m)-F'(v)/F'(m)) in young, fully expanded leaves were measured under 1,600 micromol m(-2) s(-1) white light. The CO2 assimilation rate and (PhiPSII) were higher, while F(v)/F(m)-F'(v)/F'(m) was lower in plants grown under high irradiance than in plants grown under low irradiance. These responses were observed irrespective of the presence or absence of blue light during growth. The extent of the increase in the CO2 assimilation rate and PhiPSII and the decrease in F(v)/F(m)-F'(v)/F'(m) by high growth irradiance was smaller under blue light-deficient conditions. These results indicate that blue light helps to boost the acclimation responses of energy partitioning in PSII and CO2 assimilation to high irradiance. Similarly, leaf N, Cyt f and Chl contents per unit leaf area increased by high growth irradiance, and the extent of the increment in leaf N, Cyt f and Chl was smaller under blue light-deficient conditions. Regression analysis showed that the differences in energy partitioning in PSIIand CO2 assimilation between plants grown under high white light and high blue-deficient light were closely related to the difference in leaf N.

Novel compound heterozygous mutation of the MC2R gene in a patient with familial glucocorticoid deficiency.

Familial glucocorticoid deficiency (FGD) is an autosomal recessive disorder characterised by glucocorticoid insufficiency without mineralocorticoid deficiency. Here, we report a 2 year-old girl with FGD, showing tall stature and skin pigmentation, but no abnormalities of the external genitalia. Serum sodium, potassium and chloride levels were within normal ranges. Endocrinological analysis revealed low serum cortisol (<5.5 nmol/1), elevated plasma ACTH (875.2 pmol/1) and low 17alpha-hydroxyprogesterone (< 0.303 nmol/l). We suspected the patient of having FGD type 1. Direct and allele-specific sequence analyses of the melanocortin 2 receptor gene (MC2R) revealed compound heterozygous mutations (C21Y and R146H) in the MC2R gene. Her father and mother each had heterozygous C21Y and R146H mutations, respectively, without symptoms of glucocorticoid deficiency. This is the first report of FGD associated with a compound heterozygous mutation of C21Y and R146H in the MC2R gene.

Effect of parathyroid hormone administration in a patient with severe hypoparathyroidism caused by gain-of-function mutation of calcium-sensing receptor.

Hypoparathyroidism caused by gain-of-function mutations of the calcium-sensing receptor (CaR) in the transmembrane domain is usually severe and difficult to manage. A patient with severe hypoparathyroidism, caused by CaR activating mutation F821L, was treated for 3 days (Day 1 to Day 3) with synthetic human parathyroid hormone 1-34 (teriparatide, PTH). An Ellsworth-Howard test of the patient revealed normal responses of urine phosphate and cyclic AMP excretion, indicating that the patient's renal tubules normally responded to extrinsic PTH. On Day 1 to Day 3, 0.9 microg/kg/day of PTH was administered subcutaneously twice daily at 0800 and 2000. On Day 1, the serum calcium level that was 1.8 mmol/l before PTH administration increased to 2.1 mmol/l at 1200, and gradually decreased to 1.8 mmol/l at 2000. On Days 2 and 3, the maximum calcium levels were 2.5 and 2.4 mmol/l, respectively, at 1200. At 2000, they returned to or below basal levels at 0800. On Day 4 without PTH administration, the calcium levels were maintained at the basal levels at Day 0. The urine calcium/creatinine (Ca/Cr) ratio that was high (>0.4) before PTH injection decreased after PTH administration (0.4>). Changes in the ionized calcium levels were almost parallel with the total calcium levels. The serum inorganic phosphate (IP) level decreased to 2.4 mmol/l at 1000, but gradually increased before the second PTH injection to the level at 0800 on Day 1. The minimum IP level on Days 2 and 3 was 2.1 mmol/l and 2.0 mmol/l, respectively. In contrast to the remarkable changes in the serum calcium level by PTH treatment, the serum magnesium levels showed few changes. These results indicate that PTH therapy could be effective in correcting serum and urine calcium and the phosphate levels in hypoparathyroidism caused by activating mutation of CaR.

Static suspension culture of carrot somatic embryos.

Three culture methods of carrot somatic embryos were compared: conventional shake flask culture, static suspension culture using Erlenmeyer flasks, and static suspension culture using petri dishes. Compared with the former two cultures, the last one resulted in an enhanced ratio of torpedo-shaped embryos and suppressed cotyledonary-stage embryo formation.

Photosynthetic characteristics of rice leaves grown under red light with or without supplemental blue light.

In rice plants grown under red light supplemented with blue light (red/blue-light PPFD ratio was 4/1), photosynthetic rates per unit leaf area measured under white light at 1,600 and 250 micromol m-2) s-1 were higher than those in the plants grown under red light alone. The higher photosynthetic rates were associated with higher total N content of leaves, which was accompanied by larger amounts of key components of photosynthesis-limiting processes, including Rubisco, Cyt f, Chl and LHCII. These results suggested that the increase in total N content of leaves induced by supplemental blue light enhanced both light-saturated and light-limited photosynthesis.

Dynamic dissolved oxygen concentration control for enhancing the formation rate of torpedo-stage embryos in carrot somatic embryo culture.

In utilizing somatic embryogenesis for transplant production, torpedo-stage embryos are harvested. In order to enhance the formation rate of torpedo-stage embryos to total embryos of all developmental stages in the culture at the time of harvest, a dynamic dissolved oxygen concentration (DO) control algorithm is proposed. The algorithm is based on the difference in developmental response of somatic embryogenesis to DO level depending on developmental stages, and the culture period was divided into three phases of different DO levels. The timing of the phase change was determined based on the formation rate of the embryos in each developmental stage in the suspension assessed by noninvasive monitoring of the culture. The induction of carrot (Daucus carroa L.) somatic embryogenesis resulted in doubled formation rate of torpedo-stage embryos by dynamic DO control compared to the 20% oxygen gas aeration, and 1.4 times higher formation rate compared to 6% oxygen gas aeration, while the total number of embryos did not differ among DO treatments. Plant conversion rate of torpedo-stage embryos cultured by the dynamic DO control was 70%, and was approximately the same as that in the control cultures. The relations between variations of medium pH and somatic embryogenesis were also analyzed.