Hypokalemia in a young man think Bartter syndrome type 3.

Background: Bartter syndrome is an autosomal recessive salt reabsorption disorder that results in decreased extracellular fluid volume with low/normal blood pressure. Case presentation: A 17-year-old boy with polydipsia, polyuria, weakness in the lower limbs, and ataxic gait. His Laboratory test shows hypokalemia; hypochloremia, hypomagnesemia and metabolic alkalosis. The authors' patient was managed by fluid and electrolyte replacement, which is essential in emergency management. Conclusion: Bartter syndrome is difficult to treat, and currently, there is no complete cure. The overall prognosis depends on the extent of receptor dysfunction, and despite these facts, most patients can live a normal life if they strictly follow their treatment plan.

Determinants of hyperphosphatemia and its relation with hemoglobin levels among hemodialysis patients in two hospitals from Syria: a prospective cross-sectional study.

Hyperphosphatemia and anemia were both associated with several complications in chronic kidney disease (CKD) patients. This study aimed to determine the risk factors of hyperphosphatemia and its relation with anemia among hemodialysis (HD) patients. Secondly, it aimed to determine the prevalence of hyperphosphatemia and anemia. Material and methods: A prospective cross-sectional study was conducted among 146 HD patients from two HD centers in Syria, between June 2021 and March 2022. All patients at least 18 years old on maintenance HD were enrolled. The threshold of phosphorus (phos) level was divided by the upper normal range among HD patients (5.5 mg/dl). We used parametric and nonparametric statistics, the Pearson and Spearman correlations with simple and multiple linear regressions between study variables. Results: 36.9% of patients had a serum phos level of 5.5 or less (norm phos group), and 63.1% of patients had a serum phos level higher than 5.5 (high phos group). Also, 60.9% of patients had hemoglobin (Hb) less than 10 g/dl, and 40.4% of patients had Hb at least 10 g/dl. Age, type of HD access, phos binders (P-binders), parathyroid hormone (PTH), and calcium (Ca) showed significant effects on phos levels. Most patients were using arteriovenous fistula (AVF) (89.7%) as a HD access, and the meantime on HD was higher in the norm phos group compared to the high phos group. In a multivariate and univariate logistic regression analysis, hyperphosphatemia increased with increasing urea (Ur) and creatinine (Cr) levels, while the odds declined with increasing time on HD. Hb did not show a significant relation with phos by using several statistical methods. Discussion/Conclusion: A high prevalence of hyperphosphatemia and anemia was encountered among this sample of HD patients from Syria. There was no correlation between phos and Hb levels in contrast to previous conflicting studies, which mandates future studies to evaluate this correlation and further efforts to determine the range of phos that could have a benefit on anemia with respect to other comorbidities.

Cyclosporine treatment for steroid-resistant nephrosis complicated with acquired hemophilia A: a case report.

Acquired hemophilia refers to the development of a clotting factor deficiency typically, when autoantibodies are produced against coagulation factor and tend to be in the elderly and rare in children. Case report: A 12-year-old girl with steroid-resistant nephrosis (SRN) was admitted complaining of pain in her right leg and an ultrasound showed a hematoma in her right calf. The coagulation profile revealed partial thromboplastin time prolongation and high titers (156 BU) of anti-factor VIII inhibitors were observed. Where half of the patients with antifactor VIII inhibitors were associated with underlying disorders, additional tests were performed that rule out secondary causes. This patient with long-standing SRN, who was on a maintenance dose of prednisone for six years, was complicated with acquired hemophilia A (AHA). In different to the last recommendations of AHA treatment, we preferred using cyclosporine; which is considered the initial second-line therapy for children with SRN. Complete remission was achieved of both disorders after a month with no recurrence of nephrosis or bleeding events. Conclusion: To our knowledge, nephrotic syndrome with AHA was reported in only three patients, two cases after remission and one during a relapse but no one were treated with cyclosporine. The authors encountered the first case of cyclosporine treatment for AHA in a patient with SRN. This study supports the use of cyclosporine to treat AHA, especially with nephrosis.

Delayed acyclovir therapy for disseminated varicella zoster in an adult kidney transplant recipient: a case report and literature review.

Kidney transplant recipients are at increasing risk for reactivation of varicella-zoster virus (VZV) infection. Presentation of case: A 31-year-old male was admitted with fever, chest pain, and dyspnea. Also, the complaints accompanied by generalized maculopapular, vesicular, hemorrhagic, itching, and painful rash with pustules and crusts on an erythematous base fill the entire body for the last 10 days. Chest computed tomography scan showed diffuse miliary and ground-glass opacities. The patient had a previous history of chickenpox infection in childhood, no recent contact with individuals suffering from VZV infection, and no known pretransplant serology for VZV. Due to the high clinical suspicion of reactivated VZV with pneumonitis and severe disseminated form, we started the treatment with intravenous acyclovir (ACV) for 10 days followed by oral ACV for a total of 21 days, along with stopping mycophenolate mofetil and increasing the prednisolone dose to 10 mg/d. The clinical status was improved and the rash receded with a flaked surface for old lesions. Conclusion: We experienced a successful ACV treatment for delayed and severe VZV infection with a literature review of VZV pneumonitis among kidney transplant recipients. To the best of our knowledge, this is the first case that presented a disseminated skin form with pneumonitis of VZV from Syria. This case supports the initiation of antiviral therapy for transplant patients even after 72 hours the onset of the rash despite the lack of evidence in these circumstances.

MELAS syndrome with rare manifestations misdiagnosed as vasculitis in the absence of lactic acidosis: A case report.

Introduction: The syndrome of mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) is one of the most common inherited mitochondrial disorders. Presentation of case: A 33- year-old male was admitted due to edema, urinary retention, and reduce urinary output. The medical history included a pigmentary retinopathy (PR) at age of 22 and uveitis at age of 30, which were both treated with prednisolone. At age of 32, unapparent bilateral sensorineural hearing loss (SNHL) and symmetric basal ganglia calcifications were observed in neurologic study, and received prednisolone for the diagnosis of migraine and undefined vasculitis. Also, he described a right transient ischemic stroke (TIA) in the past 4 months. His family history included a dead brother, who had nearly similar components. Physical exam on admission corresponded with parkinsonism. The status points to MELAS but the genetic test was not available. Additional tests were applied, excluding all other disorders. Lactate was normal in serum and CSF. Kidney tests revealed a nephrotic syndrome and glomerulopathy, and the biopsy showed a single hyalinized glomerulus, which most likely suggests focal segmental glomerulosclerosis (FSGS). Muscle biopsy showed ragged red fibers. Conclusion: Here, we report a challenging case of MELAS syndrome with rare manifestations including uveitis, PR, parkinsonism, and FSGS in the absence of lactic acidosis with unapparent muscle or hearing impairments. Since, clinicians might misdiagnose MELAS as vasculitis or other disorders due to its heterogeneous presentations, a proper investigations should guide the diagnosis of these conditions to reduce the delay of diagnosis and ineffective treatments.

Primary membranoproliferative glomerulonephritis in a child with down syndrome complicated with CVA: A case report.

Introduction: Down syndrome (DS) is a genetic disorder that affects multiple organs but glomerular lesions were reported only in case reports such as focal segmental glomerulosclerosis (FSGS), and Membranoproliferative glomerulonephritis (MPGN). Case presentation: A 14-year-old male child with DS was presented with generalized edema over three months. Laboratory tests revealed nephrotic syndrome (NS) and urinary tract infection (UTI). Renal ultrasound consisted with CKD. Kidney biopsy corresponded with MPGN. Also, all investigations for secondary underlying disorders came back negative suggesting the idiopathic form. Moreover, the status complicated with cerebrovascular accident (CVA), which has not been described in a DS- patient with glomerulonephritis. Discussion/conclusion: The relationship between DS and the incidence of glomerulonephritis is unclear. we suggest regular monitoring of renal function and urinalysis in different-age patients with Down syndrome, because early detection of renal disorders may prevent or slow down the progression and could be beneficial for increasing survival.

Ischemic Hepatitis Induced by Uremic Cardiac Tamponade in a Patient with Underlying Hepatitis C with a Review of the Literature.

Ischemic hepatitis is a rare cause of acute liver injury (ALI) and is associated with various etiologies including cardiac failure, trauma, hemorrhage, and respiratory failure that all result in poor perfusion and oxygen delivery to the liver. A 30-year-old patient complained of orthopnea with a history of hepatitis C treatment and is currently on hemodialysis (HD) due to chronic allograft rejection. Also, he had previous pericardial effusion (PEFF) due to inadequate dialysis. Laboratory tests on admission revealed urinary tract infection, HCV PCR positive, and high blood urea nitrogen. Computed tomography of the chest showed massive PEFF. Echocardiography revealed a massive PEFF that measured 3.6 cm on the apical four-chamber window, and the inferior vena cava diameter was 27 mm with a decreased collapsibility of ˂20% in inspiration. The patient was treated for UTI and started the treatment for HCV. Also, increased HD sessions with minimal heparinization of the dialyzer circuit were obtained along with daily monitoring of PEFF by echocardiography. At first, echocardiography did not reveal frank signs of cardiac tamponade, but after 2 sessions of HD, the patient developed chest pain, worsening orthopnea, JVP elevation, and dropping of the systolic BP. Echocardiography showed specific signs of cardiac tamponade, which included an increased effusion to 4.4 cm and changes in velocities of the mitral valve and tricuspid valve during the respiratory cycle by more than 25% and 40%, respectively. The patient was transmitted to ICU, and pericardiocentesis was obtained. Two days later, asymptomatic ALI was noticed by elevation of the following tests: ALT, AST, LDH, PT, and INR. However, ALI exhibits a rapid and spontaneous resolution to nearly normal tests after 10 days. Although the patient was hemodynamically stable, the liver injury occurred and might be attributed to ESRD and hypertension that caused thickened heart walls, diastolic dysfunction, and subsequently hepatic congestion, in addition to previous liver injury due to HCV. We present a rare case of ALI caused by uremic pericardial tamponade with an overview of the current literature with regard to this entity. So, we emphasize monitoring liver function tests in the context of PEFF, especially in patients with chronic kidney disease.

Percutaneous transluminal interventions of transplant renal artery stenosis: A case series study.

Introduction: and importance: Transplant renal artery stenosis (TRAS) is a well-recognized vascular complication after kidney transplant that can lead to graft loss, when it is diagnosed early and treated appropriately it may prevent kidney damage and related systemic squeals. Case presentation: This case-series represents our center experience in managing TRAS using percutaneous transluminal angioplasty [either balloon angioplasty (PTA) or stent placement (PTAS)] in 11 patients. Clinical discussion: All treated patients experienced immediate total recovery of renal function and normalization of arterial blood pressure without any drug or reducing the number of drugs used; no complications related to the intervention were reported. Conclusion: PTA or PTAS of TRAS can be considered safe and effective when it diagnosed and treated early.

Posterior Reversible Encephalopathy Syndrome Induced by Hypomagnesemia due to Clostridium Difficile in a Patient with Kidney Transplant.

Hypomagnesemia is found in 12% of hospitalized patients and up to 60% of intensive care unit patients and is associated with a variety of organ dysfunction. Posterior reversible encephalopathy syndrome is a neurologic hyperperfusion disorder that mostly affects posterior portions of the brain. Various theories were proposed to explain whether hypomagnesemia is etiology or associated with posterior reversible encephalopathy syndrome (PRES). A patient with kidney transplantation suffered from fatigue and reduced urine output due to chronic diarrhea induced by Clostridium difficile. Hypoparathyroidism in addition to persistent hypocalcemia and hypokalemia was observed and suggested magnesium depletion with normal serum levels. Thereafter, the status was complicated with delirium, seizures, and coma. Neurological status rapidly improved after adding intravenous magnesium sulfate to antiepileptic drugs. The second magnetic resonance imaging (MRI) showed vasogenic edema compatible with posterior reversible encephalopathy syndrome. Therefore, magnesium depletion, with normal serum levels, was considered the most implicated etiology of the syndrome in this patient. Also, hypomagnesemia during the acute phase of the syndrome and excluding all other etiology support this theory. Our case highlights hypomagnesemia-induced PRES, despite the normal serum level. Serum magnesium dropped during the acute phase of PRES, and magnesium should be maintained at the high normal limit, regardless of normal serum level. MRI findings might present after few days of symptoms; this might delay appropriate treatment.

Successful Kidney Transplant for Nephropathic Cystinosis in a Patient with Von Willebrand Disease Type III: The First Case Report.

Nephropathic cystinosis (NC) is a rare autosomal recessive disease, which causes cysteine-crystals accumulation with progression to end-stage renal disease (ESRD). Von willebrand disease (VWD) type III is a rare subtype of von willebrand factor (VWF) abnormality, which is characterized by severe reduction of VWF and factor VIII activity. A 16-year-old patient with NC and VWD type III presented with uremic symptoms due to ESRD. Dialysis access was inserted and followed by hemodialysis (HD) for 4 months with a proper infusion of blood products. While renal transplant remains the treatment of choice of NC and superior to chronic HD, bleeding complications were a major concern in this case with coexisting VWD type III. However, with the meticulous implementation of the Hematology team's daily recommendations, renal transplantation was successfully performed. This is the first case that mentions a new association between two inherited rare disorders, NC and VWD type III, and this entity has not been reported before. Moreover, successful kidney transplantation in our patient supports the possibility of these procedures in hereditary clotting disorders.

Liver-kidney recipients with chronic viral hepatitis C treated with interferon-alpha.

Antiviral therapy with interferon-alpha (IFN-alpha) and pegylated IFN-alpha (PEG-IFN-alpha) for chronic hepatitis C (HCV)-infected kidney recipients remains controversial. IFN-alpha is not recommended in most cases because it induces severe acute graft rejection. However, IFN-alpha, as PEG-IFN-alpha, is associated with a more pronounced immune response, and is well tolerated in HCV-infected liver recipients without causing graft rejection. In combined liver-kidney transplant (LKT) recipients, IFN-alpha has been occasionally used and appears to be well tolerated. All LKT recipients with a functioning kidney and liver having a HCV replication and who needed IFN-alpha therapy have been included in the study. The occurrence of liver and/or renal acute rejection as well as the HCV replication has been collected. A total of 12 LKT patients treated with PEG-IFN-alpha plus ribavirin have been studied. No acute rejection was observed. Renal function remained stable during and after discontinuing treatment, without any graft dysfunction. Two patients had a partial viral response and four had a sustained viral response. All patients, whatever their viral response, had decreased liver-enzyme levels. Response to PEG-IFN-alpha therapy was correlated with steroid dose and transaminase level when PEG-IFN-alpha was started. These data suggest that the combination therapy of PEG-IFN-alpha plus ribavirin did not have a higher risk of acute kidney-graft rejection after liver-kidney transplantation.