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Sarmad Said,1 Haider Alkhateeb,1 Chad J Cooper,1 Emmanuel Rodriguez,1 Remi Trien,1 German T Hernandez,1,2 Hasan S Salameh1,2 1Department of Internal Medicine, 2Department of Internal Medicine, Division of Nephrology and Hypertension, Paul L Foster School of Medicine, Texas Tech University Health Sciences Center at El Paso, El Paso, TX, USA Introduction: Idiopathic cluster of differentiation 4 (CD4+) T-cell lymphocytopenia (ICL) is a rare non human immunodeficiency virus (HIV)-related syndrome with unclear natural history and prognosis that was first reported and defined in 1992. ICL has been observed in patients after the onset of an opportunistic infection without known immunosuppression. Case presentation: A 20-year-old Hispanic male patient without significant past medical history presented with progressive shortness of breath and cough for 3 weeks. Chest computed tomography showed bilateral cavitary lesions in the upper lung lobes. The HIV rapid screening test as well as the sputum acid-fast bacilli test were both positive. The patient was started on antituberculosis therapy. The CD4 count was noticed to be low. However, the HIV Western blot test was negative, and the HIV viral load was within normal limit. Further radiologic studies, hemato-oncologic, and autoimmune workups were normal. The patient was discharged on the treatment for tuberculosis. Follow-up after 8 weeks revealed a persistent low CD4+ count, and the repeated HIV tests were negative. Conclusion: The clinical features of ICL range from an asymptomatic condition to life-threatening complications that imitate the clinical course of HIV-infected patients. The differential diagnosis in adults comprises primarily HIV infection and other diseases or drug side effects. ICL is very rare and should be considered in the absence of any defined immunodeficiency or therapy associated with depressed levels of CD4+ T-cells. Early detection and recognition of the disease allow purposeful and systemic treatment approach and screening for the affected patients. Keywords: CD4 count, immunodeficiency, lymphocytopenia, opportunistic infection

Background: Vadadustat is an oral hypoxia-inducible factor prolyl hydroxylase inhibitor for treating anemia in chronic kidney disease (CKD). This study investigated safety and efficacy of once-daily and 3-times-weekly dosing in patients with dialysis-dependent (DD)-CKD compared with darbepoetin alfa (DA)., Methods: This phase 3b, randomized (1:1:1; vadadustat once-daily [starting dose: 300 or 450 mg], vadadustat 3-times-weekly [starting dose: 600 or 750 mg], DA), open-label, active-controlled, noninferiority trial included conversion (weeks 0-20) and maintenance (weeks 20-52) periods. Primary and secondary efficacy endpoints were mean change in hemoglobin from baseline during the primary (PEP, weeks 20-26) and secondary (SEP, weeks 46-52) evaluation periods. Other endpoints included proportion of patients requiring ESA rescue (hemoglobin <9.5 g/dL or with increases in dose ≥50% or ≥100% in DA group). Safety endpoints included treatment-emergent adverse events (TEAEs) and serious AEs (SAEs)., Results: Least squares (LS) mean treatment difference between vadadustat once-daily and DA from baseline to PEP was -0.27 g/dL (95% CI, -0.55 to 0.01); the lower bound met the noninferiority threshold (-0.75 g/dL). The LS mean treatment difference between vadadustat 3-times-weekly and DA from baseline to PEP was -0.53 g/dL (95% CI, -0.80 to -0.25), which did not meet the lower bound noninferiority threshold. The LS mean change from baseline to the SEP between DA and vadadustat once-daily was -0.40 (95% CI, -0.79 to -0.02), and for vadadustat 3-times-weekly was -0.42 (95% CI, -0.81 to -0.02). Proportion of patients who received ESA rescue during weeks 2-52 was higher in the DA group than vadadustat groups. Similar TEAEs and treatment-emergent SAEs were observed across groups., Conclusions: Vadadustat once-daily, but not 3-times-weekly, was noninferior to DA in the correction and maintenance of hemoglobin in patients with DD-CKD converted from an ESA; safety profiles were similar across groups., Trial Registration: EudraCT 2019-004851-36/ClinicalTrials.gov identifier: NCT04313153., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Society of Nephrology.)

Background: Hemodialysis patients are at increased risk of hepatitis B virus (HBV) infection and are poorly responsive to HBV vaccines. Current vaccine recommendations for hemodialysis patients utilize more than twice the amount of hepatitis B surface antigen (HBsAg) used for healthy adults and achieve lower immune responses., Methods: An open-label, single-arm, multicenter trial was conducted among adults 18 years of age and older who were initiating or undergoing hemodialysis who had not previously received hepatitis B vaccine. Participants received four doses of HepB-CpG (HEPLISAV-B®) (20 mcg rHBsAg + 3000 mcg CpG 1018, a Toll-like receptor 9 agonist) administered at 0, 4, 8, and 16 weeks. Participants are being followed for 68 weeks. This paper reports the final immunogenicity analysis of the primary endpoint at study week 20 and an interim safety analysis., Results: We enrolled 119 participants receiving hemodialysis who were followed for a median of 47.4 weeks. Of the 119 participants, 75 were in the per-protocol population. At week 20, the seroprotection rate (% with antibodies to hepatitis B surface antigen [anti-HBs] ≥ 10 mIU/mL) was 89.3% and the percentage of participants with anti-HBs ≥ 100 mIU/mL was 81.3%. The anti-HBs geometric mean concentration was 1061.8 mIU/mL. HepB-CpG was well tolerated with no observed safety concerns., Conclusion: In patients receiving hemodialysis, HepB-CpG given as four doses was well tolerated and induced very high anti-HBs concentrations and seroprotection in a very high proportion of recipients., Competing Interests: Declaration of Competing Interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Randall Hyer, Fang Xie, Kimberly Erby, and Robert Janssen were employed by Dynavax Technologies Corporation at the time of study conduct. No other author reported competing interests., (Copyright © 2021 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

The impact of age, race/ethnicity, healthcare insurance, and selected clinical variables on statin-preventable ASCVD were quantified in adults aged 21 to 79 years from National Health and Nutrition Examination Surveys 2007-2012 using the 2013 American College of Cardiology/American Heart Association guideline on the treatment of cholesterol. Among ≈42.4 million statin-eligible, untreated adults, 52.6% were hypertensive and 71% were younger than 65 years. Of ≈232 000 statin-preventable ASCVD events annually, most occur in individuals younger than 65 years, with higher proportions in blacks and Hispanics than whites (73.0% and 69.2% vs 56.9%, respectively; P<.01). Among adults younger than 65 years, the ratio of statin-eligible but untreated to statin-treated adults was higher in blacks and Hispanics than whites (3.0 and 2.9 vs 1.3, respectively; P<.01), and blacks, men, hypertensives, and cigarette smokers were more likely to be statin eligible than their statin-ineligible counterparts by multivariable logistic regression. Two thirds of untreated statin-eligible adults had two or more healthcare visits per year. Identifying and treating more statin-eligible adults in the healthcare system could improve cardiovascular health equity., (©2017 Wiley Periodicals, Inc.)

Background: Healthy People 2020 aim to reduce fatal atherosclerotic cardiovascular disease (ASCVD) by 20%, which translates into 310 000 fewer events annually assuming proportional reduction in fatal and nonfatal ASCVD. We estimated preventable ASCVD events by implementing the American College of Cardiology/American Heart Association (ACC/AHA) 2013 Cholesterol Guideline in all statin-eligible adults. Absolute risk reduction (ARR) and number needed-to-treat (NNT) were calculated., Methods and Results: National Health and Nutrition Examination Survey data for 2007-2012 were analyzed for adults aged 21 to 79 years and extrapolated to the US population. Literature-guided assumptions were used including (1) low-density lipoprotein cholesterol falls 33% with moderate-intensity statins and 51% with high-intensity statins; (2) for each 39 mg/dL decline in low-density lipoprotein cholesterol, 10-year ASCVD10 risk would fall 21% when ASCVD10 risk was ≥20% and 33% when ASCVD10 risk was <20%; and (3) either all statin-eligible untreated adults or all with ASCVD10 risk ≥7.5% would receive statins. Of 175.9 million adults aged 21 to 79 years not taking statins, 44.8 million (25.5%) were statin eligible. Treating all statin-eligible adults would prevent an estimated 243 589 ASCVD events annually (ARR 5.4%, 10-year NNT 18). Treating all statin-eligible adults with ASCVD10 risk ≥7.5% reduces the number treated to 32.2 million (28.2% fewer), whereas ASCVD events prevented annually fall only 10.5% to 217 974 (6.8% ARR, NNT 15)., Conclusions: Implementing the ACC/AHA 2013 Cholesterol Guideline in all untreated, statin-eligible adults could achieve ≈78% of the Healthy People 2020 ASCVD prevention goal. Most of the benefit is attained by individuals with 10-year ASCVD risk ≥7.5%., (© 2016 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.)

A 2014 hypertension guideline raised goal systolic blood pressure (SBP) from <140 mm Hg to <150 mm Hg for adults 60 years and older without diabetes mellitus (DM) or chronic kidney disease (CKD). The authors aimed to define the status of hypertension in black adults 60 to 79 years from the National Health and Nutrition Examination Survey 2005-2012 and provide practical guidance. Black patients were more often aware and treated (P≤.005) for hypertension than whites and had higher rates of DM/CKD (P<.001), similar control to <140/<90 mm Hg with DM/CKD (P=.59), and lower control without DM/CKD (<140/<90 mm Hg and <150/<90 mm Hg, P≤.01). Limited awareness (<30%) and infrequent health care (>30% 0-1 health-care visits per year) occurred in untreated black and white hypertensive patients without DM/CKD and BP ≥140/<90 mm Hg. The literature suggests benefits of treated SBP <140 mm Hg in adults 60 to 79 years without DM/CKD. The International Society of Hypertension in Blacks recommends: (1) continuing efforts to achieve BP <140/<90 mm Hg in those with DM/CK, and (2) identifying hypertensive patients without DM/CKD and BP ≥140/<90 mm Hg and treat to an SBP <140 mm Hg in black adults 60-79 years., (© 2015 Wiley Periodicals, Inc.)

Kidney disease disproportionately affects racial and ethnic minority populations, the poor, and the socially disadvantaged. The excess risk of kidney disease among minority and disadvantaged populations can only be partially explained by an excess of diabetes, hypertension, and poor access to preventive care. Disparities in the environmental exposure to nephrotoxicants have been documented in minority and disadvantaged populations and may explain some of the excess risk of kidney disease. High-level environmental and occupational exposure to lead, cadmium, and mercury are known to cause specific nephropathies. However, there is growing evidence that low-level exposures to heavy metals may contribute to the development of CKD and its progression. In this article, we summarize the excess risk of environmental exposures among minority and disadvantaged populations. We also review the epidemiologic and clinical data linking low-level environmental exposure to lead, cadmium, and mercury to CKD and its progression. Finally, we briefly describe Mesoamerican nephropathy, an epidemic of CKD affecting young men in Central America, which may have occupational and environmental exposures contributing to its development., (Copyright © 2015 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.)

Context: It is well known that patients with chronic kidney disease (CKD) have a strong risk of cardiovascular disease (CVD). However, the excess risk of cardiovascular disease in patients with CKD is only partially explained by the presence of traditional risk factors, such as hypertension and diabetes mellitus., Evidence Acquisitions: Directory of Open Access Journals (DOAJ), Google Scholar, PubMed, EBSCO and Web of Science has been searched., Results: Chronic kidney disease even in its early stages can cause hypertension and potentiate the risk for cardiovascular disease. However, the practice of intensive blood pressure lowering was criticized in recent systematic reviews. Available evidence is inconclusive but does not prove that a blood pressure target of less than 130/80 mmHg as recommended in the guidelines improves clinical outcomes more than a target of less than 140/90 mmHg in adults with CKD., Conclusions: The association between CKD and CVD has been extensively documented in the literature. Both CKD and CVD share common traditional risk factors, such as smoking, obesity, hypertension, diabetes mellitus, and dyslipidemia. However, cardiovascular disease remains often underdiagnosed und undertreated in patients with CKD. It is imperative that as clinicians, we recognize that patients with CKD are a group at high risk for developing CVD and cardiovascular events. Additional studies devoted to further understand the risk factors for CVD in patients with CKD are necessary to develop and institute preventative and treatment strategies to reduce the high morbidity and mortality in patients with CKD.

Moyamoya disease was first described in 1957 as hypoplasia of the bilateral internal carotid arteries, the characteristic appearance of the associated network of abnormally dilated collateral vessels on angiography was later likened to something hazy, like a puff of cigarette smoke, which, in Japanese, is moyamoya. This paper describes two cases of moyamoya presentations, including moyamoya disease and moyamoya syndrome. Moyamoya may rarely occur in North American Hispanic patients. The presentation can vary significantly and ranges bwtween fulminant outcome and prolonged survival. Awareness about moyamoya and its different presentations may be beneficial for the patients and can improve the outcome.

Patient: Female, 60 FINAL DIAGNOSIS: Bird fanciers' lung Symptoms: Cough productive • hypoxia • short of breath • substernal chest pain, Medication: - Clinical Procedure: - Specialty: -, Objective: Rare disease., Background: Hypersensitivity pneumonitis (HP) is a group of inflammatory interstitial lung diseases caused by hypersensitivity reactions from repeated insults of inhalation of fine particulate organic dusts derived from environmental sources. Bird fanciers' lung (BFL) is the most common form of HP, with an estimated prevalence of 0.5-7.5% and is observed in individuals who develop a hypersensitivity response to avian droppings or antigens on bird feathers., Case Report: A 60-year-old woman presented to our care with shortness of breath with exertion. She was hypoxic with oxygen saturation of 70% on room air. The CTA of the chest revealed a diffuse bilateral ground glass density in the lung parenchyma with a mosaic attenuation pattern. On further questioning she explained that she collected many duck and goose feathers she found on the ranch and placed them in a vase at home. Transbronchial lung biopsy revealed non-caseating granulomas, aggregates of epithelioid macrophages, and patchy mononuclear cell infiltration with lymphocytes and fibrotic tissue. The patient clinically improved and was discharged home on the 6(th) hospital day with prednisone 20 mg daily, with clinical improvement noted on subsequent follow up visits., Conclusions: There is no specific clinical manifestation; abnormal laboratory test results help establish a definitive diagnosis. The best diagnostic tool is the correlation of symptom onset with the environmental exposure. The prognosis is excellent after a single episode of HP, but continuous re-exposure carries the risk of progressive pulmonary impairment.

Patient: Female, 66 FINAL DIAGNOSIS: Chorea • hyperglycemia • Basal Ganglia Syndrome (C-H-BG) Symptoms: Hemibalism • hemichorea, Medication: - Clinical Procedure: - Specialty: Endocrinology and Metabolic., Objective: Challenging differential diagnosis., Background: Hemichorea-hemiballism (HCHB) is a spectrum of involuntary, continuous non-patterned movement involving 1 side of the body. Possible causes of HCHB include hemorrhagic or ischemic stroke, neoplasm, systemic lupus erythematosus, HHNK, Wilson's disease, and thyrotoxicosis. This case illustrates the need to be aware of hyperglycemia as a cause of hemiballism/hemichorea, which is now referred to in the medical literature as C-H-BG (chorea, hyperglycemia, basal ganglia) syndrome., Case Report: A 66-year-old Hispanic woman presented to our care with hemiballism/hemichorea of the right arm and leg of 1 week duration. She had been admitted 3 months prior with toxic metabolic encephalopathy secondary to hyperosmolar hyperglycemic non-ketotic syndrome with a blood glucose level of 984 mg/dL. Her blood glucose level was normal but hemoglobin A1C was 12.2%. A brain MRI revealed an asymmetric T1 hyperintensity of the left putamen. This specific finding was compatible with hyperglycemia-induced hemichorea hemiballism syndrome. The hemiballism/hemichorea slowly improved over the course of the hospitalization with strict glycemic control. At the 3-month follow-up visit she had no involuntary movements of her extremities, and she had well controlled blood glucose levels and a hemoglobin A1C of 9.0., Conclusions: In a patient with normal glycemic levels but a history of uncontrolled diabetes, C-H-BG syndrome should be on the top of the differential list when the characteristic MRI findings of a hyperintensity in the basal ganglia are observed. This is a rare disease that deserves attention because it is reversible with correction of hyperglycemia. Thus, prompt recognition and treatment is essential to avoid adverse outcomes.

Patient: Male, 47 FINAL DIAGNOSIS: Porphyria cutanea tarda Symptoms: Chills • cough dry • thumb swelling, Medication: - Clinical Procedure: - Specialty: Metabolic Disorders and Diabetics., Objective: Challenging differential diagnosis., Background: Porphyria cutanea tarda (PCT) is the most common type of the porphyria. It occurs due to the deficiency of enzyme uroporphyrinogen decarboxylase (UROD), which is the fifth enzyme in the biosynthesis of heme and catalyzes the conversion of uroporphyrinogen to coproporphyrinogen. The risk factors for PCT include hereditary hemochromatosis, hepatitis C infection, ethanol abuse, estrogen use, HIV, smoking, chlorinated polycyclic aromatic hydrocarbons, and hemodialysis., Case Report: A 47-year-old Hispanic man presented with right thumb swelling, redness, and pain for approximately 1 week. Past medical history included HIV/AIDS, hepatitis C infection, alcohol abuse, heroin abuse, and CMV retinitis. Skin examination revealed blistering and hypo/hyper pigmented lesions over the dorsal aspects of the hands and other sun-exposed areas. Serum porphyrins were discovered to be elevated. The quantitative urine porphyrins revealed elevation of uroporphyrins, heptacarboxyl-porphyrins, hexacarboxy-porphyrins, pentacarboxyl-porphyrins and coproporphyrin. Genetic mutation of UROD was not detected. Due to the classic cutaneous lesions, laboratory findings, and associated risk factors, we were able to confirm our suspicion of the sporadic (type 1) form of PCT., Conclusions: A strong correlation has been demonstrated between the sporadic (type 1) form of PCT and hepatitis C virus (HCV) infection in multiple studies. The mechanism through which HCV infection may cause or trigger PCT is unknown. PCT has been described for many years, but still eludes the differential diagnosis in a patient with cutaneous findings. The uniqueness of our case is the possibility that combined risk factors have an effect on PCT.

Dipeptidyl peptidase-4 inhibitors (DPP-4 inhibitors) are effective and safe oral incretin-based antihyperglycemic drugs. In preclinical studies, DPP-4 inhibitors have demonstrated cardiovascular benefits, independent of glycemic control, in patients with type-2 diabetes mellitus. Since 2005, various DPP-4 inhibitors (sitagliptin, linagliptin and saxagliptin) have been clinically available in the United States. Since 2013, alogliptin is the 4(th) approved DPP-4 inhibitor available on the market. Studies have shown that alogliptinis non-inferior to comparator drugs among newly diagnosed type 2 diabetes mellitus patients. Alogliptin can effectively be used as a single agent or as an add-on drug in combination with other glucose lowering drugs with favorable outcomes on glycemic control and HbA1C levels.

Background. Hyper IgE is a rare systemic disease characterized by the clinical triad of high serum levels of IgE (>2000 IU/mL), eczema, and recurrent staphylococcal skin and lung infections. The presentation of hyper IgE syndrome is highly variable, which makes it easy to confuse the diagnosis with that of severe atopy or other rare immunodeficiency disorders. Case Report. A 23-year-old Hispanic presented with history of frequent respiratory and gastrointestinal infections as a child and multiple episodes of skin and lung infections (abscess) with Staphylococcus aureus throughout his adult life. He had multiple eczematous lesions and folliculitis over his entire body, oral/esophageal candidiasis, and retention of his primary teeth. The IgE was elevated (>5000 IU/mL). Genetic mutation analysis revealed a mutation affecting the transactivation domain of the STAT3 gene. Conclusion. The hallmark of hyper IgE syndrome is serum IgE of >2000 IU/mL. Hyper IgE syndrome is a genetic disorder that is either autosomal dominant or recessive. A definite diagnosis can be made with genetic mutation analysis, and in this case, it revealed a very rare finding of the transactivation domain STAT3 mutation. Hyper IgE syndrome is a challenge for clinicians in establishing a diagnosis in suspected cases.

Background: Cocaine is a potent sympathomimetic agent associated with the development of possible fatal cardiovascular complications. Dysrhythmias, acute myocardial infarction, hypertension and dilated cardiomyopathy are just some of many cardiovascular effects related to the abuse of cocaine., Case Presentation: A 38-year-old Hispanic male with a past medical history of hypertension presented with a chief complaint of progressive shortness of breath. The patient confessed to the use of cocaine for almost 18 years once per week. On examination he was hypertensive and tachycardic with a systolic murmur over the 5th intercostal space at the level of the left mid-clavicular line. Laboratory workup revealed an elevated Brain natriuretic peptide; urine toxicology was positive for cocaine. 2D-echocardiogram showed dilated cardiomyopathy. Cardiac catheterization excluded angioischemic cause. He was managed medically and subsequently discharged with drug rehabilitation. On follow-up diagnostic evaluation after 5 months of cocaine cessation, his ejection function improved significantly., Conclusion: The exact incidence of cocaine related cardiomyopathy is unknown and likely underreported. The clinical course is abrupt and comparatively similar to other types of cardiomyopathy. The management is like other forms of cardiomyopathy; however β-blockers should be avoided. The myocardial dysfunction is reversible with abstaining from additional cocaine ingestion. Non-invasive testing should be performed after several months to re-evaluate the treatment response.

Patient: Male, 22 FINAL DIAGNOSIS: Cardiomyopathy Symptoms: Shortness of breath • dispnoea • chest discomfort, Medication: - Clinical Procedure: Echocardiogram • cardiac MRI Specialty: Cardiology., Objective: Challenging differential diagnosis., Background: Non-compaction cardiomyopathy (NCM) is a rare congenital cardiomyopathy characterized by increased trabeculation in one or more segments of the ventricle. The left ventricle is most commonly affected. However, biventricular involvement or right ventricle predominance has also been described. Clinical features of NCM are non-specific and can range from being asymptomatic to symptoms of congestive heart failure, arrhythmia, and systemic thromboembolism., Case Report: 22-year-old Hispanic male presented with two month history of chest discomfort. Laboratory workup revealed an elevated brain-natriuretic-peptide of 1768 pg/ml. ECG and chest x-ray was nonspecific. Transthoracic echocardiogram revealed prominent trabeculae and spongiform appearance of the left ventricle (LV) with an ejection-fraction of 15-20%; 5 of 9 segments of the LV were trabeculated with deep intertrabecular recesses also involving the right ventricle (RV) with demonstrated blood flow in these recesses on color-doppler. The biventricular spongiform appearance was morphologically suggestive for NCM with involvement of the RV. Confirmatory cardiac MRI was performed, demonstrating excessive trabeculation of the left-ventricular apex and mid-ventricular segments. Hypertrabecularion was exhibited at the apical and lateral wall of the RV. Cardiac catheterization showed an intact cardiac vessel system. The patient was discharged on heart failure treatment and was placed on the heart transplantation list., Conclusions: NCM is a unique disorder resulting in serious and severe complications. The majority of the reported cases describe the involvement of the left ventricle. However, the right ventricle should be taken into careful consideration. The early diagnosis may help to increase the event-free survival.

Unlabelled: Patient Male, 32 FINAL DIAGNOSIS: Dural arterio-venous fistula Symptoms: Eye redness • post-trauma headache • tinnitus, Medication: - Clinical Procedure: Fistula embolization Specialty: Neurology., Objective: Mistake in diagnosis., Background: A dural arteriovenous fistula (DAVF), is an abnormal direct connection (fistula) between a meningeal artery and a meningeal vein or dural venous sinus. The pathogenesis of DAVF still remains unclear. Sinus thrombosis, head trauma, chronic central nervous system, hypercoagulable state, surgery, and hormonal influence are the pre-disposing factors that initiate this disease. The symptoms experienced by the patient will depend on the location of the fistula., Case Report: Thirty-two year old Hispanic male who presented one day after a rear ended motor vehicle collision (MVC) with a severe throbbing headache in the left parietal region, left eye redness but no retro-orbital pain and tinnitus in the left ear. He was initially misdiagnosed to have a carotid-cavernous fistula but upon cerebral angiogram was actually diagnosed with a dural arterio-venous fistula in the posterior fossa venous system followed by successful embolization of the fistula., Conclusions: A cerebral angiography is the gold standard for detection and characterization of a DAVF and will distinguish it from a CCF. Endovascular surgery involves a catheter-based technique for embolization of the lumen of arteries feeding the DAVF, or directly into the vein draining the DAVF. It is very important to recognize the typical findings of patients presenting with a DAVF then quickly proceeding with a cerebral angiogram to determine the exact location of the fistula and the appropriate treatment plan. By diagnosing and treating a DAVF as early as possible, the associated fatal complications can be averted.

Patient: Female, 21 FINAL DIAGNOSIS: Multicystic Dysplastic Kidney Disease complicated by pyelonephritis Symptoms: Left flank pain (CVAT) • dysuria • fever, Medication: Levofloxacin Clinical Procedure: Dimercaptosuccinic acid scan • voiding cystouretrogram Specialty: Nephrology., Objective: Rare disease., Background: Multicystic dysplastic kidney (MCDK) is a renal dysplasia characterized by the presence of multiple cysts that are non-communicating, separated by dysplastic parenchyma that consumes the renal cortex resulting in a nonfunctional kidney. MCDK has an incidence of 1: 4300 of live births and is usually unilateral, most commonly occurring in the left kidney. Simple MCDK is defined as unilateral dysplasia with a normal contralateral kidney but with compensatory hypertrophy of the contralateral kidney, and no associated genitourinary anomalies., Case Report: A 21 year old Hispanic American female, presented with intermittent, sharp, severe left flank pain, fever and dysuria for two days but had gradually worsened within the last 24 hours prior to presentation. Previous history of multicystic dysplastic kidney, diagnosed four years ago. No pertinent physical examination findings except left costovertebral angle tenderness (CVAT). Urinalysis findings were positive for infection and urine culture grew pan sensitive Escherichia coli. A CT scan of abdominal and pelvis without contrast revealed a normal right kidney and left kidney had multiple non-communicating dilated cystic spaces, but no hydronephrosis, left ureteropelvic junction obstruction and finding were consistent with multicystic dysplastic kidney and also noted perinephric stranding., Conclusions: VUR is the most common renal abnormality in patients with MCDK, occurring in about 25% of contralateral kidney. Infections involving the MCDK are rare. In fact, cases of infections such as pyelonephritis or an infected renal cyst of MCDK are almost non-existent in the current literature. This patient presented with findings consistent with MCDK complicated by pyelonephritis.

Patient: Female, 50 FINAL DIAGNOSIS: Double superior vena cava Symptoms: -, Medication: - Clinical Procedure: - Specialty: Nephrology., Objective: Anatomical anomaly/variation., Background: Superior vena cava anomalies are caused by variations in the development of the embryonic thoracic venous system. Duplication of the superior vena cava is a rare anomaly with an incidence in the general population of 0.3%. The majority of cases are asymptomatic and diagnosed incidentally by imaging done for another reason., Case Report: A fifty year old female patient presented to our care with a history of end stage renal disease that has been recently started on dialysis. Procedures performed included a fistulogram, PermaCath placement and angiogram of internal jugular vein. Angiogram was done and showed that the patient has a good sized cephalic vein which is wide open and mild to moderate stenosis at the subclavian area which proved to be a dual superior vena cava., Conclusions: Double SVC is a rare congenital anomaly. The literature available on this congenital anomaly is sparse. The majority of cases are diagnosed incidentally on imaging for other reasons, which can alert the physician of other congenital abnormalities that will need further work up. However, these venous anomalies should be recognized, as they can have significant clinical implications, especially during central venous catheter placement, radiofrequency ablation, pacemaker insertion or coronary artery bypass graft.

The sodium glucose cotransporter 2 (SGLT2) is expressed primarily in the kidneys and is involved in the reabsorption of filtered glucose in the renal tubule. Clinical trials of SGLT2 inhibitors in patients with type 2 diabetes mellitus demonstrate a significant clinical effect in decreasing serum glucose, hemoglobin A1C, body weight, systolic blood pressure, improving β-cell function, and minimizing the risk of hypoglycemia. This report reviews the potentially beneficial effects of SGLT2 inhibitors in type 2 diabetes mellitus, specifically focusing on canagliflozin, the only SGLT2 inhibitor approved for use in the United States.

An estimated 11% of the U.S. population has chronic kidney disease (CKD). Cardiovascular morbidity and mortality remain high among individuals with CKD and the higher mortality from cardiovascular disease persists even after adjusting for most of the traditional risk factors, suggesting the possible contributions of uremia-related, nontraditional risk factors. This has led to the current understanding that the pathophysiology of cardiovascular disease in CKD involves a complex interplay of both the traditional as well as nontraditional, uremia-related risk factors. Given the high cardiovascular morbidity and mortality, patients with CKD should be a target for aggressive cardiovascular risk reduction.

Background: The validity of serum parathyroid hormone (PTH) as a surrogate marker of bone turnover in chronic kidney disease (CKD) is limited by several factors such as relative resistance of bone to PTH, hyperphosphatemia, diabetes, gender, age, race and vitamin D analog action on the PTH-bone axis. Urinary collagen N-terminal telopeptide X (NTx), a bone collagen degradation product, expressed as bone collagen equivalents (BCE) per mM of creatinine (NTx/Cr ratio), is routinely used to estimate bone turnover in osteoporosis. The purpose of this study is to evaluate NTx as a marker of bone turnover in CKD., Methods: We studied the relationship between bone-specific alkaline phosphatase (BSAP), PTH and urine NTx/Cr in 37 CKD out-patients., Results: In a multivariate model, PTH had a positive correlation with BSAP (r=0.44, P<0.19) and U-NTx/Cr (r=0.55, P<0.30), after adjusting for age, gender, estimated glomerular filtration rate (GFR), serum phosphorus, corrected calcium, and race. However, the strongest correlation was found between the two direct markers of bone resorption and formation (U-NTx vs. BSAP; r=0.80; P<0.0001), suggesting a tight coupling of bone resorption and formation in CKD. The effect of gender on U-NTx/Cr was studied in a multivariate model after adjusting for age, race, GFR, serum calcium, phosphorus and PTH. Females had a higher U-NTx/Cr than males., Conclusion: Our findings indicate that urinary NTx, a promising marker of bone resorption in CKD patients, exhibits a strong positive correlation with other markers used to assess renal osteodystrophy i.e. PTH and BSAP. Unlike PTH and BSAP, urine NTx also measures bone loss secondary to osteoporosis., (Published by Elsevier B.V.)

Background: End-stage renal disease disproportionately affects black persons, but it is unknown when in the course of chronic kidney disease racial differences arise. Understanding the natural history of racial differences in kidney disease may help guide efforts to reduce disparities., Methods: We compared white/black differences in the risk of end-stage renal disease and death by level of estimated glomerular filtration rate (eGFR) at baseline in a national sample of 2,015,891 veterans between 2001 and 2005., Results: Rates of end-stage renal disease among black patients exceeded those among white patients at all levels of baseline eGFR. The adjusted hazard ratios for end-stage renal disease associated with black versus white race for patients with an eGFR > or = 90, 60-89, 45-59, 30-44, 15-29, and <15 mL/min/1.73 m2, respectively, were 2.14 (95% confidence interval [CI], 1.72-2.65), 2.30 (95% CI, 2.02-2.61), 3.08 (95% CI, 2.74-3.46), 2.47 (95% CI, 2.26-2.70), 1.86 (95% CI, 1.75-1.98), and 1.23 (95% CI, 1.12-1.34). We observed a similar pattern for mortality, with equal or higher rates of death among black persons at all levels of eGFR. The highest risk of mortality associated with black race also was observed among those with an eGFR 45-59 mL/min/1.73 m2 (hazard ratio 1.32, 95% CI, 1.27-1.36)., Conclusion: Racial differences in the risk of end-stage renal disease appear early in the course of kidney disease and are not explained by a survival advantage among blacks. Efforts to identify and slow progression of chronic kidney disease at earlier stages may be needed to reduce racial disparities.

Background: A 60-year-old man with a history of diabetes and hypertension was referred to a nephrology clinic for investigation of his elevated serum creatinine level., Investigations: Physical examination; laboratory investigations, including measurement of whole-blood lead level, body lead burden and urine albumin:creatinine ratio; history of lead exposure and use of herbal medical products; and renal ultrasonography., Diagnosis: Stage 3 chronic kidney disease that was probably worsened by consumption of lead in the form of an Ayurvedic herbal remedy., Management: Cessation of the herbal product, followed by lead-chelation therapy with calcium disodium ethylenediaminetetraacetic acid. The patient's whole-body lead burden and blood lead level decreased to acceptable levels and his serum creatinine value was within the normal range at final follow-up.

Background: A 32-year-old African American man with HIV infection presented with hemoptysis, shortness of breath and renal insufficiency. Serologic testing revealed the presence of anti-glomerular basement membrane antibodies and equivocal levels of anti-myeloperoxidase antibodies., Investigations: Physical examination, urine and blood analysis, kidney ultrasound, chest radiograph, sputum cultures, bronchoscopy and renal biopsy., Diagnosis: Reactivation of tuberculosis infection, immune complex glomerulonephritis, and 'false-positive' anti-glomerular basement membrane and anti-myeloperoxidase antibodies., Management: Directly observed therapy with four-drug anti-tuberculosis therapy and conservative management of chronic kidney disease.

Introduction: Asians, Pacific Islanders, and Native Americans are a potentially high-risk group for dietary exposure to methylmercury through fish consumption. However, blood mercury levels in this group have not been identified in recent reports of the National Health and Nutrition Examination Survey (NHANES) for the years 1999-2002., Methods: We used NHANES data from 1999-2002 to obtain population estimates of blood mercury levels among women of childbearing age classified as belonging to the "other" racial/ethnic group (Asian, Pacific Islander, Native American, and multiracial; n = 140). Blood mercury levels in this group were compared with those among all other women participants, classified as Mexican American, non-Hispanic black, non-Hispanic white, and "other" Hispanic., Results: An estimated 16.59 +/- 4.0% (mean +/- SE) of adult female participants who self-identified as Asian, Pacific Islander, Native American, or multiracial (n = 140) had blood mercury levels > or = 5.8 microg/L, and 27.26 +/- 4.22% had levels > or = 3.5 microg/L. Among remaining survey participants (n = 3,497), 5.08 +/- 0.90% had blood mercury levels > or = 5.8 microg/L, and 10.86 +/- 1.45% had levels > or = 3.5 microg/L., Conclusions: Study subjects in NHANES who self-identified as Asian, Pacific Islander, Native American, or multiracial had a higher prevalence of elevated blood mercury than all other racial/ethnic participants in the survey. Future studies should address reasons for the high mercury levels in this group and explore possible interventions for lowering risk of methylmercury exposure in this population.

Background: Although Latinos constitute the largest and fastest growing minority group in the United States, little is known about the prevalence of renal disease among different Latino subgroups., Methods: We used data from the Hispanic Health and Nutrition Examination Survey (HHANES) to compare serum creatinine measurements among Mexican Americans, mainland Puerto Ricans, and Cuban Americans. We compared estimated creatinine clearance across Latino subgroups adjusted for demographic, clinical, and socioeconomic characteristics (including known predictors of chronic kidney disease) using survey logistic regression analysis., Results: Cuban Americans had higher mean serum creatinine levels than the other groups across both gender and age categories. In multivariable analysis, Puerto Ricans [odds ratio (OR) 1.74, 95% confidence interval (CI) 1.16 to 2.60] and Cuban Americans (OR 4.59, CI 2.53 to 8.31) were more likely than the referent category of Mexican Americans to have an estimated creatinine clearance < 60 mL/min/1.73 m2., Conclusion: Serum creatinine levels differ substantially among Latino subgroups, suggesting national origin needs to be taken into consideration in studies of renal disease in Latinos. In addition, our findings highlight the need for more contemporary studies directly comparing both incidence rates of end-stage renal disease and measured renal function among Latino subgroups, perhaps leading to subgroup-specific prediction equations.

Herlitz junctional epidermolysis bullosa is an autosomal recessive disorder characterized by generalized blistering at the lamina lucida of the cutaneous basement membrane. The monoclonal antibody GB3 has been used as a diagnostic probe because of its lack of reactivity in patient skin. The antigen recognized by GB3 has been identified as laminin-5, a glycoprotein consisting of three subunits (alpha 3, beta 3 and gamma 2). To identify the laminin-5 protein chain that contains the epitope recognized by GB3 and to determine if chain assembly is required for antibody recognition, we expressed a gamma 2 protein constructed from a full-length gamma 2 cDNA. Radioimmunoprecipitation of the culture medium from 293 cells revealed that both GB3 and anti-gamma 2 polyclonal antibodies were capable of directly precipitating recombinant gamma 2 without coprecipitation of other proteins. In immunodepletion experiments, each antibody removed most of the protein that was reactive with the other antibody. The epitope recognized by GB3 is present only when the complex is in the native conformation because GB3 reacted only with the non-reduced laminin-5, but not the reduced laminin-5 in immunoblots. Moreover, because GB3 reacted with laminin-5 of SCC25 cells (gamma 2 in the heterotrimer) but not recombinant gamma 2 in 293 cells (gamma 2 alone) during indirect immunofluorescence staining, this epitope may be dependent upon a less stable conformation of gamma 2. We conclude that GB3 recognizes the gamma 2 chain of laminin-5 and that the epitope is entirely contained in the native form of the gamma 2 chain.