It is known that ROS causes mitochondrial damage and plays an important role for the death of activated T cells 27. TSC1KO T cells display increased ROS production, but decreased mitochondrial content, number, and membrane potential. Since the ROS scavenger NAC can reduce the death of TSC1KO T cells and can increase mitochondrial membrane potential, it suggests that

TSC1 may promote T-cell survival mainly through the inhibition Selleckchem SAHA HDAC of ROS production to maintain mitochondrial integrity. Of note, CD28 mediated co-stimulation, but not rapamycin treatment, can reduce TSC1KO T-cell death correlated with reduced ROS production and increased mitochondrial potential, but without obvious increase of Akt activity. Thus, TSC1 may inhibit ROS production in T cells and promote T-cell survival through mTOR-independent mechanisms. Further studies are needed to determine the mechanisms by which TSC1 regulates ROS production and mitochondrial homeostasis. The TSC1flox/flox and CD4-Cre transgenic mice were

purchased from Jackson Laboratory and Taconic Farm, respectively 38, 39. All experiments were performed in accordance with protocols approved by the Duke University Institutional Animal Care and Use Committee. Single-cell suspension of thymocytes, splenocytes, and LN cells in IMDM medium supplemented with 10% FBS, penicillin/streptomycin, and 2-mercaptoethanol (IMDM-10) were made according to standard protocols. Purification of T cells was achieved using either the Mouse T Cell Enrichement Kit (STEMCELL Omipalisib Technologies) or the LD depletion columns (Miltenyi Biotech) and purities of ≥90% were achieved. Thymocytes, splenocytes, and purified T cells (5–20×106

cells/mL in PBS) were left Bumetanide un-stimulated or stimulated with 5 μg/mL of anti-CD3ε (500A2; BD Pharmingen) for different times. Cells were lysed in 1% Nonidet P-40 Lysis solution (1% Nonidet-40, 150 mM NaCl, and 50 mM Tris, pH 7.4) with freshly added protease and phosphatase inhibitors. Proteins were resolved by SDS-PAGE and were transferred to a Trans-Blot Nitrocellulose membrane (Bio-Rad Laboratories). The blots were probed with specified antibodies and detected by ECL. Antibodies for TSC1 (♯4906), TSC2 (♯3612), p-Foxo1 (♯9461S), p-ERK1/2 (♯91015), p-p70 S6K (♯9204S), p70 S6K (♯9202), p-4EBP1 (♯2855S), 4EBP1 (♯9644), Cleaved Caspase-3 (♯9661), Cleaved Caspase-9 (♯9509), p-Akt T308 (♯9275S), p-Akt S473 (♯9271S), Puma (♯4976), Bid (♯2003), Bax (♯2772), Bim (♯4582), Bcl-xL (♯2762), Mcl-1 (♯4572), Akt (♯2938), Foxo1a (♯94545), S6K1(♯9202) were purchased from Cell Signaling Technology. Bcl-2 (♯554087) antibody was purchased from BD. Noxa (♯2437) was purchased from ProSci Inc. Anti-β-actin antibody was from Sigma-Aldrich (A1978). Cells were stained with fluorescence-conjugated antibodies specific for CD4, CD8, CD25, CD44, and CD69 (eBioscience and BioLegend) at 4°C for 30 min. Dying cells were identified using 7AAD, annexin V, or the Violet Live/Dead cell kit (Invitrogen).

Methods: An experimental study was conducted for 30 days at hemodialysis unit Dr. Soetomo Hospital, Surabaya. Twenty-three patients

were enrolled in this study and divided into two groups of NAC capsules (11 patients) and effervescent tablets (12 patients). Statistical analysis was conduced with paired t-test (in normally distributed data) or Wilcoxon test (in abnormally distributed data). Results: The results showed insignificant homocysteine decrease of 10.99% (p = 0.072) and in the capsule and significant see more homocysteine decrease of 13.21% (p = 0.024) in the effervescent group There were no significant difference (p = 0.067) in mean serum homocysteine between groups using the NAC capsules and effervescent tablets. No difference in NAC side effects was found in both treatment groups. Conclusion: In group receiving capsules, mean homocysteine level decreased insignificantly, while in group receiving effervescent tablets homocysteine decrease was significant. There was no significant difference in mean serum homocystein between group receiving NAC capsule and group receiving effervescent tablet. NAC side effects in both groups were not significantly different. Key words: N-acetylcysteine, NAC, hyperhomocysteinaemia HANAFUSA NORIO1, HAMASAKI YOSHIFUMI1, Cell Cycle inhibitor KINUGASA SATOSHI2, NOIRI EISEI2, NANGAKU MASAOMI2 1Division of Total Renal Care Medicine, the University of Tokyo

Hospital, Tokyo, Japan; 2Department of Hemodialysis and Apheresis, the University of Tokyo Hospital, Tokyo, Japan Introduction: Carnitine deficiency is popular among hemodialyzed population, which is supposed due to elimination during hemodialysis procedure as well as several other factors. Although kinetics of carnitine during hemodialysis procedure has been investigated, the actual amount of carnitine eliminated during hemodialysis remains unclear. We measured the actual amount of eliminated carnitine with use of continuous syringe extract method (CSEM) during Ribose-5-phosphate isomerase hemodialysis. Methods: Chronic hemodialysis patients as inpatient settings at our hospital were investigated. All were treated with hemodialysis of 4 hour session with high-flux dialyzer. Carnitine

was measured in both serum and dialysate. A portion of dialysate at the outlet of dialyzer was collected by CSEM. We calculated total amount of carnitine loss into dialysate, the clearance at the middle of sessions, and cleared space during beginning, latter half or entire session. Factors that affected the amount of removal were also investigated. The entire protocol had been approved by the ethical committee of our facility (approval number #3658). Results: Thirty patients were finally included into the present study. Their ages were 64.1 ± 8.6 years. Seven patients were female. Thirteen patients were diabetic. Median dialysis vintages were 8.1 (IQR 4.2–14.0) years. Predialytic total carnitine concentration was 44.9 ± 11.5 μmol/l (mean ± standard deviation).

5, 3.6, 2.4, and 2.9 for T0, T1, T2, and T3, respectively). In this study, the volunteers were all selected to be above 70 years of age as a model of immune-compromised subjects. Furthermore, all volunteers were living

in the same elderly home. This was expected to reduce differences in the diet and environmental conditions, leading to reduced inter-individual variability during the study. As shown in this study, the probiotic combination tested showed a significant improvement in NK cell ability to kill target tumor cells and the phagocytosis activity of granulocytes and monocytes. This may be of practical benefit to the health of the elderly population. A previous study reported an enhancement of immune parameters to be more pronounced in volunteers aged 70 years or more (Gill et al., 2001). Our results support the earlier studies Selleckchem PLX3397 selleck screening library demonstrating an enhancement of natural and acquired immunity indices in mice and in elderly populations (Gill et al., 2000; Gill et al., 2001; Sanders & Klaenhammer, 2001). In addition, this study verified that the reported enhancement of immune indices

could also be achieved when the probiotic bacteria are embedded in a cheese matrix, while earlier studies used reconstituted fat-free milk as a carrier (Gill et al., 2000; Gill et al., 2001; Sheih et al., 2001). In the present study, there was no significant association between the probiotic-induced enhancement of cytotoxicity and any of the lymphocyte subsets. This is in accordance

with the observations by Gill and colleagues (Gill et al., Olopatadine 2001; Morimoto et al., 2005; Takeda & Okumura, 2007) with elderly volunteers. On the other hand, no significant correlation was found between the increase of NK cytotoxicity after the intervention and age in contrast to that observed by the authors (Gill et al., 2001). The significant negative association between the cytotoxicity values after the intervention and that at the baseline indicates that the increase of cytotoxicity is higher for volunteers with lower baseline cytotoxicity. This suggests that the consumption of these probiotics may benefit mostly those with reduced immune functions. Because the significant reduction in the relative proportion of the CD3−CD56− level after the run-in and the intervention was not accompanied by a significant increase in at least some of the other cell types (CD3−CD56+, CD3+CD56+, and CD3+CD56−), this shows that the expected increase was distributed between those three types of cells. The weak, but significant, association between the cytotoxicity vs. NK, NKT, and CD3+CD56− cells indicates that these cells may be the main contributors to the cytotoxicity observed.

17 Conversely, check details the 2A peptide linker results in a single mRNA molecule, but during translation ribosomal skipping generates two separate proteins from the single mRNA.18 The majority of constructs currently in clinical and preclinical development use the 2A sequence to link the TCR-α and TCR-β chains as a result of the improved equimolar expression of both genes, compared to vectors with an IRES element separating the TCR genes. Importantly, it has been shown by ourselves and others that T cells transduced with constructs containing the TCR genes linked by a 2A sequence express higher levels of cell-surface TCR and demonstrate improved antigen-specific function, as measured by IFN-γ secretion,

compared with constructs containing identical TCR sequences

separated by an IRES element.19 Efficient cell-surface TCR expression requires the formation of a stable TCR–CD3 complex.11 In MI-503 cell line the absence of CD3, TCRs do not assemble properly and are degraded. Therefore, the availability of CD3 molecules for TCR–CD3 complex assembly is a major rate-limiting effect when introducing additional exogenous TCRs into T cells. Competition may reduce cell-surface expression of the introduced TCR and impair the avidity of antigen recognition of the transduced cells. We have recently demonstrated that the double transduction of CD8+ T cells with a vector encoding the desired TCR-α and TCR-β chain genes, together with a second vector encoding the CD3 gamma, delta, epsilon and zeta genes (linked by 2A sequences), can enhance the avidity of CD8+ T cells (King J, Ahmadi M, personal communication). This may be a mechanism to enhance the functional avidity of transduced T cells expressing low-affinity TCRs. It is common for the introduced TCRs to be expressed at lower levels than the endogenous TCRs, which may impair the ability of the transduced T cell to respond to low concentrations of the TCR-recognized antigen, as

discussed above. This observation is consistent with the introduced TCR competing with the endogenous TCR for limited CD3 molecules. Heemskerk et al.20 Baricitinib have recently shown that the expression levels of the introduced TCR can be influenced by the ‘strength’ of the endogenous TCR by introducing the same TCR into different antigen-specific T-cell clones. It is currently unclear whether TCR-specific molecular motifs exist to determine the ‘competitiveness’ of a given TCR-αβ chain. Primary T cells transduced with exogenous TCRs have the potential to express four different TCR-αβ heterodimers on the recipient T-cell surface: (i) the endogenous αβ heterodimer; (ii) the introduced αβ heterodimer; (iii) the endogenous α chain paired with the introduced β chain; and, finally, (iv) the introduced β chain paired with the endogenous α chain. These possibilities are indicated in the schematic diagram shown in Fig. 2.

Throughout the book, the authors are on the side of the reader

as they explain neuropathology and toxicology from a very practical point of view. In Part 1, on the fundamentals of neurobiology, the book begins with a section defining the spectrum of neurotoxicology and the importance of neurotoxicological research. The history of neurotoxicology is outlined with particular mention of the important contributions of the founding editor of Neuropathology and Applied Neurobiology, John B Cavanagh, who devised many of the routine morphological approaches to neurotoxicology that are in use today. A valuable set of 10 principles is propounded that impress on the reader the importance of grasping nomenclature in the field, and recognizing the restricted SAHA HDAC nature of responses in the nervous system and its selective vulnerability. Each principle has a memorable title such as Principal 4: ‘what gets

wrecked depends on when it gets whacked!’ Principles for assessing acute pathological lesions in the nervous system, the use of special stains, an inbuilt scepticism with regard to what you see down the microscope and the value of a wider knowledge of neurology are all discussed. Finally, the necessity of good planning for screening studies in neurotoxicology Omipalisib price and in experimental neuropathology is emphasized; the advisability of adhering to standard study designs and protocols is discussed under Principle 10: ‘garbage in, garbage out!’ The eight ensuing chapters cover

functional and comparative neuroanatomy, development, localization of neuropathological lesions, ageing, behavioural systems and cognitive assessment, mainly in relation to neurotoxicology, but the general principles expounded in these chapters have general applicability to the whole spectrum of neuroscience. Part 2 of the book deals with the techniques involved in the investigation of the central and peripheral nervous systems, cerebrospinal fluid and muscle. There is a very interesting chapter on fluoro-Jade dyes describing the use of fluorochromes for localizing degenerating neurones. There is a chapter on imaging that includes ultrasound, magnet resonance imaging, positron emission tomography and single-photon emission Bumetanide computed tomography (SPECT) techniques that are used in human medical practice, and non-invasive bioluminescent imaging (BLI) that is not used in humans. BLI detects light emitted endogenously via a chemical reaction driven by the enzyme luciferase. This section of the book also includes chapters on histological artefacts in nervous system tissues and on molecular techniques. Part 3 covers the practice of toxicological neuropathology and its applications; the actions of toxins on the central nervous system, retina, ear, peripheral nervous system and the olfactory nervous system are clearly reviewed.

Attitudes toward genetic diagnosis and prenatal diagnosis for Chinese AS families were investigated. Attitudes toward genetic diagnosis and prenatal diagnosis

in Chinese XLAS families were evaluated in the current study. Methods:  A total of 160 XLAS patients and their 126 healthy family members in China were interviewed. After providing background knowledge counselling and education on AS, their attitudes toward genetic diagnosis and prenatal diagnosis were evaluated by multiple-choice questionnaire. selleck kinase inhibitor Results:  Majority of the respondents cared mostly about the prognosis and treatment effects of AS (89.9% vs 81.1%) since they considered that the worst outcome of XALS was renal insufficiency (92.3%). Of all

the interviewees, 99.3% were interested in genetic research for the discovery of better treatments and more appropriate diagnostic tools (positive attitudes) (89.5% vs 73.2%). About 80% of the participants would accept prenatal testing and subsequent termination of pregnancy in cases of affected foetuses (boys: 86.8% and girls: 74.6%, respectively). Conclusion:  Most Chinese XLAS families show positive attitudes and desire new discoveries in treatment and diagnosis. About 80% of respondents would approve prenatal testing with a desire for selective termination of pregnancy rather than predicting the health of a future child. “
“Published literature on fracture in dialysis selleck products patients seldom addressed the effect of co-morbidity and malnutrition. In this study, we reported the incidence and risk factors for fracture in peritoneal dialysis patients. Peritoneal dialysis

patients who had fractures between 2006 and 2011 were recruited. Demographic data, details of fracture, Charlson Co-morbidity Index (CCI) and biochemical parameters were also collected. Non-fracture controls, matched for age, gender and duration of dialysis, were also recruited at ratio 1:1 for fracture risk analysis. The incidence of fracture was 1 in 37 patient-years. The commonest site of fracture was neck of femur (n = 16, 55.2%). Twenty-four patients (82.8%) developed fracture after slip and fall injury. Eight out of 17 self-ambulatory filipin patients (47.1%) became non-ambulatory after fracture. Infection was the commonest complication during hospitalization. Univariant analysis demonstrated high CCI (P = 0.001), hypoalbuminaemia (P < 0.001), loss of self autonomy (P = 0.006) and non-ambulatory state (P = 0.011) significantly associated with increased fracture risk. However, only CCI (odds ratio (OR) 1.373, P = 0.028) and albumin (OR 0.893, P = 0.025) increased fracture risk significantly on multivariant analysis. Bone profile and parathyroid hormone were not significant risk factors. To conclude, fracture associated with adverse outcome in peritoneal dialysis patients. High CCI score and hypoalbuminaemia significantly increase risk of fracture.

However, primary renal diseases for ESRD are different by race and area and the incidence, prevalence and mortality of CKD vary accordingly.14 Consequently, the CKD screening and prevention programs requires different approaches depending on the patient’s race, habitual and socioeconomic status and be modified in response GPCR Compound Library ic50 to the situations where they would be conducted. The authors thank Dr Hung-Chun Chen and the organizing committee for providing this opportunity to share experience on prevention and management of CKD. Dr Nan Chen’s work was supported in part by grants from the Leading Academic Discipline Project of Shanghai Health

Bureau (05III001), the Shanghai Leading Academic Discipline Project (T0201) and the Science and Technology Commission of Shanghai Municipality (08dz1900502). The Authors state that there is no conflict of interest regarding the material discussed in the manuscript. “
“Date written: July 2008 Final submission: October 2008 No recommendations possible based on Level I or II evidence (Suggestions

are based on Level III and IV evidence) Ulixertinib research buy As dialysis is an accepted and available mode of treatment for end-stage kidney disease (ESKD) in Australia and New Zealand, the decision concerning acceptance onto a dialysis programme should be made on the basis of the patient’s need. The cardinal factor for acceptance onto dialysis or continuation 2-hydroxyphytanoyl-CoA lyase of dialysis is whether dialysis is likely to be of benefit to the patient.* *Additional notes: 1 Lack of certainty about whether the treatment will be of benefit to the patient may suggest the use of temporary dialysis or a ‘trial’ so

that dialysis as a treatment option can be evaluated. Survey individual unit documentation of implementation of the above ‘Suggestions for Clinical Care’ and rates of insertion and completion of the checklist titled ‘Approaching ESKD’ (Appendix) in patient notes. These draft guidelines do not refer to temporary dialysis, but expressly consider acceptance onto long-term dialysis, which would be terminated only by the death of the patient, successful renal transplantation, inability to maintain successful dialysis or elective withdrawal of dialysis by the patient. There is broad consensus in Australia and New Zealand that people in our society regardless of age, race, gender, religion and underlying disease have equal rights to access health facilities. Unless the patient has chosen to accept only supportive treatment, individuals and society at large expect that ESKD should not, except in unusual circumstances, be the primary cause of death.

Surface Vip (Lmo0320), a bacterial cell wall-anchored protein, also seems to be an important candidate in late stages of the infectious process. Endoplasmic reticulum resident chaperone Gp96 has been identified as a cellular receptor for Vip (Cabanes et al.,

2005). Gp96 is employed in the modulation learn more of the immune response by affecting the cellular trafficking of several molecules, including Toll-like receptors. It is predicted that Vip may not only use Gp96 as a receptor for invasion but may also sequester Gp96 to subvert immunological response. Earlier, researchers predicted the induction and thus the involvement of FAK and PI 3-kinase in the Listeria cell invasion as a consequence of Vip–Gp96 binding, as it occurs in E. coli invasion. However, later studies showed RXDX-106 clinical trial that Listeria interaction with cells does not seem to induce FAK activation for cytoskeletal rearrangements. Similarly, no involvement of the Vip in the increase in tyrosine phosphorylation of protein associated with p85α or Gp96 has been reported elsewhere (Cabanes et al., 2005). Thus, the role of Vip–Gp96 interaction in the Listeria cell entry might be through other signal transduction events associated with Gp96 responses that remain to be elucidated. Another mechanism of BBB translocation, a Trojan horse, needs internalization/phagocytosis of the pathogen by monocytes wherein InlA and InlB play a

crucial role. These internalins and P60 protein bind specific receptors (like

complement Dichloromethane dehalogenase receptor) on phagocytic cells and trigger the internalization of bacteria through a variety of opsonin-dependent and opsonin-independent mechanisms. Internalization allows persistence in a shielded niche, concealed from circulating antibodies. Listeria, in its intracellular form, stimulates NF-κB and secretion of cytokines IL-1α, IL-1β, IL-6, and TNF-α in phagocytes. Listeria-infected monocytes further upregulate E-selectin, ICAM-1, P-selectin, and VCAM-1, which leads to the adherence to BMECs. The mechanism for this endothelial activation involves listeriolysin O-dependent triggering of NF-κB nuclear translocation in cerebral vessels (Kayal et al., 1999). Infected phagocytes may adhere to endothelium and thus bacteria can invade ECs by cell-to-cell spread in an hly- and actA-dependent process (Greiffenberg et al., 1998; Drevets, 1999). Infected phagocytes then cross the endothelial barrier, and infection can spread to the brain parenchyma cells or subarachnoid space and ventricles (Drevets & Leenen, 2000). As an alternative to adhering to and infecting the endothelium, infected phagocytes could transmigrate and enter the brain tissue. In this case, bacteria contained within phagocytes could spread to cells such as neurons and microglia (Dramsi et al., 1998). Interestingly, pneumococcus, meningococcus, and H. influenzae adhere to the BMECs via 37/67-kDa laminin receptor (LR).

We

speculated that the mechanism was as follows: The PHB expression in the GU group was weakened, which induced the generation of ROS. The increased ROS might upregulate the expression of TGF-βl.48,49 The disorder of TGF-βl might induce the expressions of Col-IV and FN,50–52 and the overexpression TGF-βl could upregulate the expression of Caspase-3.53–55 The increased Caspase-3 was associated with cell apoptosis.37,38 So, the over-accumulation of ECM was observed and index of RIF and the number of apoptotic cells were increased. Interestingly, in our investigation, we found that PHB and Caspase-3 mainly located in RTEC, and the apoptotic cell was mainly derived from RTEC. We speculated that the injury of RTEC was an early event and might play a pivotal role in the progression of RIF in UUO rats. So, how to protect the RTEC against injury was very important in the prevention Cell Cycle inhibitor of RIF. More attention should be paid to the event of impaired RTEC in future study. Furthermore, in our study, we also found that the PHB mainly located in RTEC, and there was only a minimal expression in mesangial cells of glomerulus. The PHB expression in glomerulus was markedly weak when compared that in renal interstitium in UUO rats (figure and data not shown). The location of PHB was similar to that in Guo et al.18 It might give us some new

insights to explore the association of PHB with renal disease. However, there was click here Suplatast tosilate a limitation in our study. In this observational study, we only found that the PHB was associated with caspase-3 expression/cell apoptosis. Cell culture using RTEC

in vitro and transfection with small inhibitory RNA of PHB to decrease the PHB gene expression might be needed in future to investigate the effect of PHB on caspase-3/cell apoptosis in UUO rats. In conclusion, less expression of PHB was associated with the increased expression of Caspase-3/cell apoptosis in RIF rats, although the detailed mechanisms were not fully elucidated. So, how to upregulate the expression of PHB is very important for prevention of RIF, and PHB might be a potential therapeutic target for prevention of the cell injury. However, cells culture in RTEC and so on, and inhibition of signalling pathway of PHB need to be conducted to explore its detailed mechanism in the further. This study was supported by the Nature Science Foundation of China (no. 81060061), the Natural Science Foundation of the Guangxi Zhuang Autonomous Region (no. 0832121) and the Health Department of Guangxi Zhuang Autonomous Region (no. 200917). The authors would like to gratefully acknowledge the most helpful comments on this paper received from Professor Liang Rong, Department of Pediatric-Neonatology, Baylor College of Medicine, Houston, Texas, USA.

Moreover, the onset in most cases is several months or even years after the inciting delivery, so it is often misrecognized INCB024360 molecular weight and not adequately treated. Hyponatremi and hypoglicemi that have been rarely reported in the literature. Case Report: A 47-year-old woman, a housewife, was admitted because disturbed consciousness. She had a history of postpartum hemorrhage which had occurred 15 years previous. Amenorrhea and failure to lactate developed thereafter. Fatigue and dry skin were also found. Physical examination revealed a chronically ill looking. She was drowsy, her fluid status was euvolemic, and her conjunctiva appeared anemic. Laboratory data were as follows:

hemoglobin 7, 8 g/dl, the random blood glucose 40 g/dl and the serum sodium 108 meq/L with low serum osmolality and elevated urine sodium. Moreover, the investigations also showed a low of FSH, LH and prolactin. Magnetic Resonance Imaging

of the brain showed an “empty sella” appearance. Thus, a diagnosis of Sheehan BYL719 molecular weight syndrome was made. Hyponatremia and hypoglycemia that was improved after replacement with glucocorticoids. Conclusions: This case illustrates that Sheehan’s syndrome whose first presentation was with hyponatraemia and hypoglycaemia that have been rarely reported in the literature. Early diagnosis and appropriate treatment are necessary to reduce the morbidity and mortality of patients. Key words: Sheehan Syndrome, Hyponatremia and Hypoglycemia, Empty sella. 283 MILD PERSISTENT HYPERKALEMIA: AN IMPORTANT DIAGNOSTIC CLUE IN SHORT STATURE S CAMPBELL, A WALKER, J KAUSMAN, C QUINLAN Royal Children’s Hospital – Nephrology Department, Melbourne, Australia Aim: The case is of a 10-year-old female who presented

as a diagnostic dilemma to multiple paediatric physicians with key features short stature & hyperkalemia. Background: She initially presented with Perthes disease of both hips was then noted to have a height on the 3rd centile, with mid-parental height expectation of a 10th centile. She was found to be normotensive (50th centile), and without dysmorphic features. Investigations revealed a persistent hyperkalemia (average = 6.2 (3.5–5.5 mmol/L)), in the presence of low/normal aldosterone level (55U/L), and low renin ≤0.2 (1.0–4.0). Branched chain aminotransferase Plasma creatinine was normal (36 mmol/L) as was urinary potassium excretion (91 mmol/L). A venous gas demonstrated a mild metabolic acidosis (pH 7.32, BE = −4). Methods: A diagnostic trial of hydrochlorothiazide was successful in resolving her hyperkalemia. Results: The clinical & biochemical picture is consistent with that of Type II pseudohypoaldosteronism (PHAII), specifically Spitzer-Weinstein syndrome. Conclusions: A rare disorder, inherited in an autosomal dominant manner involving the WNK1 and WNK4 genes. WNK kinases are named so due to a lack of lysine in the ATP binding cassette of the catalytic region.