Thrombotic microangiopathy (TMA) reflects a syndrome of endothelial injury characterised by microangiopathic haemolytic anaemia (nonimmune), thrombocytopenia, and often end-organ dysfunction. TMA disorders are well-recognised in kidney transplant recipients, often due to an underlying genetic predisposition related to complement dysregulation, or de novo due to infection, immunosuppression toxicity, or antibody-mediated rejection. In pregnancy, TMA disorders are most commonly due to severe pre-eclampsia or HELLP, but may also be due to thrombotic thrombocytopenic purpura (TTP) or complement-mediated (atypical) haemolytic uremic syndrome (aHUS). Complement dysregulation is being recognised as playing a role in the development of preeclampsia and HELLP syndrome in addition to aHUS. Due to overlapping clinical and laboratory features, diagnosis can be difficult and delays in treatment can be life-threatening for both mother and fetus. This report describes a 32 year-old female who had two successive wanted pregnancies. The first pregnancy was terminated at 22 weeks gestation due to presumed severe preeclampsia and fetal growth restriction in the context of known chronic kidney failure due to reflux nephropathy. A living-related kidney transplant was performed to improve the chances of pregnancy resulting in a live birth. A subsequent pregnancy was complicated by progressive kidney impairment and hypertension at 22 weeks gestation. Kidney biopsy showed TMA, but the etiology was unclear. This report highlights the diagnostic dilemma of TMA in a pregnant kidney transplant recipient and a role for the anti-C5 terminal complement blockade monoclonal antibody eculizumab, in pregnancy-associated TMA, especially at a peri-viable gestation.

A. Krelle

S. Price

M. M. Law

S. Kranz

P. Shamdasani

S. Kane

J. Unterscheider

P. Champion de Crespigny

The Challenges of Distinguishing Different Causes of TMA in a Pregnant Kidney Transplant Recipient

Background. The syndrome of tubulointerstitial nephritis and uveitis (TINU) is a rare oculorenal condition, mainly seen in children and women. The underlying cause of this disease is unknown. Case Presentation. We report a 24-year-old male without any past medical history, diagnosed with bilateral uveitis and azotemia. Biopsy revealed tubulointerstitial nephritis, consistent with TINU syndrome. Fluorescein angiogram revealed peripheral retinal vasculitis. Discussion. TINU is a rare disorder that needs to be distinguished from sarcoidosis, Sjogren’s disease, and tuberculosis. Treatment is indicated in patients with progressive renal insufficiency, consisting of steroid therapy. Most patients recover kidney function. Its early recognition is important to offer the best chance of organ preservation.

Juan Montejo-Hernández

Jorge Rico-Fontalvo

Jose Cabrales

Shuchi Anand

María Cristina Martínez-Ávila

Claudia Duran-Merino

Luis Arias-Restrepo

Camilo Andrés Gómez Duran

TINU: A Multisystemic Inflammatory Disorder—Case Report and Literature Review

Introduction. Longer survival in dialysis led to a higher incidence of vascular access complications and failure. With the limited access to kidney transplantation programs and peritoneal dialysis, exhaustion of vascular access for hemodialysis is an increasingly common situation. Among the available options, atrial tunneled dialysis catheter (ATDC) has been reported as an effective vascular access in this population. Methodology. We report the experiences of two nephrology centers in Tunis with ATDC as an ultimate vascular access for dialysis. Case Reports. Two patients with exhausted vasculature underwent ATDC insertion in 2020 and 2022, respectively, as a vascular access of last resort. Both patients underwent CRBI, which resolved with favorable outcomes. One case was complicated by post-operative thrombosis and was successfully treated with thrombolysis. Both patients are currently on dialysis via their ATDC with a catheter patency of 29 months. Conclusion. ATDC is a life-saving and safe vascular access in cases of depleted vasculature. Little more than 50 cases have been reported in the literature during the last 30 years. As the frequency of vasculature exhaustion is expected to increase, preservation of veinous access in patients at risk of chronic kidney disease have never been more crucial.

Meriam Hajji

Salah Saied

Ikram Mami

Yassine Khadhar

Tasnim Ben Ayed

Imen Gorsane

Fethi Ben Hamida

Jalel Ziadi

Mohamed Karim Zouaghi

Ezzeddine Abderrahim

The Tunnelled Atrial Catheter: A Promising Solution for Vascular Capital Depletion in Dialysis despite Associated Thrombi

Acute kidney injury (AKI) poses a substantial challenge in the management of lymphoma patients and is frequently associated with diverse causative factors. Herein, we report an illustrative case involving a 47-year-old male with influenza A infection who developed severe AKI, which was incongruent with his medical history. Laboratory investigations disclosed aberrant immunoglobulin levels and urinary protein excretion, prompting further evaluation. A renal biopsy revealed the presence of infiltrating lymphoid cells and cast nephropathy, raising suspicion of an underlying hematological disorder. A comprehensive diagnostic workup, including positron emission tomography imaging and bone marrow biopsy, culminated in the definitive diagnosis of splenic marginal zone lymphoma. This case highlights the crucial significance of including lymphoma-associated kidney disorders in the evaluation of unexplained AKI, particularly when encountering unconventional clinical and laboratory results. Swift and precise intervention is of utmost importance in attaining positive results in these rare and complex clinical situations. This study underscores the persistent concern of AKI in lymphoma patients, with lymphocytic infiltration and cast nephropathy as notable elements contributing to the intricate nature of this condition.

Wan-Ching Lee

Chun-Kuang Tsai

Szu-Yuan Li

Unusual Coincidence: Concurrent Cast Nephropathy and Lymphoma Infiltration in an Influenza A-Associated Acute Kidney Injury

Congenital nephrotic syndrome is an autosomal recessive inherited disorder that manifests as steroid-resistant massive proteinuria in the first three months of life. Defects in the glomerular filtration mechanism are the primary etiology. We present a child who developed severe nephrotic syndrome at two weeks of age and eventually required a bilateral nephrectomy. Genetic testing revealed compound heterozygous variants in NPHS1 including a known pathogenic variant and a missense variant of uncertain significance. Light microscopy revealed crescent formation—an atypical finding in congenital nephrotic syndrome caused by nephrin variants—in addition to focal segmental and global glomerulosclerosis. Electron microscopy showed diffuse podocyte foot process effacement. Confocal and Airyscan immunofluorescence microcopy showed aggregation of nephrin in the podocyte cell body that is not a result of diffuse podocyte foot process effacement as seen in minimal change disease. These findings confirm the novel variant as pathogenic.

Kyle N. Goodman

Pongpratch Puapatanakul

Kevin T. Barton

Mai He

Jeffrey H. Miner

Joseph P. Gaut

A Case of Congenital Nephrotic Syndrome with Crescents Caused by a Novel Compound Heterozygous Pairing of NPHS1 Genetic Variants

Background. Urinary lithiasis constitutes a recurrent pathology affecting a relatively young population. The risk of progression to chronic renal failure and the cost of treatment are the most important issues. Primary hyperparathyroidism (PHPT) is responsible for urolithiasis and nephrocalcinosis in 7% of patients, and it represents the 7th cause of urolithiasis in Tunisia. Unfortunately, it remains an underdiagnosed pathology although it is curable. We aim to determine the clinical, biological, therapeutic, and evolutionary particularities of urinary lithiasis associated with PHPT in a nephrology setting. Methods. This is a monocentric, retrospective, descriptive study which took place in our nephrology department during the period from January 2010 to January 2023. Ten patients were included. All of them underwent blood and urine tests and a morphoconstitutional study of the urinary stones if possible. Results. The median age at diagnosis of PHPT was 42 years (34–54). The median time from the onset of kidney stones to the diagnosis of PHPT was 6.2 years (1–17). The male/female gender ratio was 0.66. Five patients had hypertension, two patients had obesity, one patient had diabetes, and three patients had urinary tract infections. Kidney stones were bilateral in eight cases and unilateral in two cases. Nine patients underwent urological intervention: surgery in 5 cases associated with nephrectomy in one case, extracorporeal lithotripsy in 4 cases, and percutaneous nephrolithotomy in two cases. The diagnosis of PHPT was retained with high or uncontrolled PTH associated with hypercalcemia in 8 cases and normocalcemic PHPT was found in 2 patients. Two patients had parathyroid adenoma and one patient had mediastinal adenoma. Radiology exploration was normal for the others patients. Surgery was performed in 7 patients and histology revealed an adenoma in 5 cases and hyperplasia in one case. The predominant urinary risk factors in our study were hypercalciuria in 6 cases and insufficient diuresis in 4 cases. Conclusion. This study underlines the role of the nephrologist in the exploration of urinary lithiasis and the prevention of recurrences, especially as PHPT is a curable aetiology of urolithiasis and affects a relatively young population. The determination of the epidemiological profile of patients with stones associated with primary PHPT and lithogenic risk factors allows the primary and secondary prevention of stone formation.

Hajji Meriam

Hayet Kaaroud

Rahma Karray

Kahena Bouzid

Recurrent Urolithiasis Revealing Primary Hyperparathyroidism in a Nephrology Department

Myoglobin cast nephropathy occurs in cases of acute renal injury in which large amounts of myoglobin accumulate in the renal tubules, presenting as muscle pain, reddish-brown urine, and elevated creatine kinase levels. Our case describes a 60-year-old male who came to the emergency department with fevers, mild abdominal pain, and constitutional symptoms one day after returning to the United States from a trip to Nigeria. Initial workup demonstrated an acute kidney injury and elevated aminotransferase levels and the patient was started onatovaquone-proguanil for possible malaria given a recent diagnosis in Nigeria. Two days later, the patient was found to have rhabdomyolysis, resulting in a renal biopsy that showed myoglobin cast nephropathy. Previous literature has suggested mechanisms for the development of rhabdomyolysis in malarial infection, including inflammatory processes, direct effect of parasite accumulation, and drug-induced toxicity. Our case further implicates antimalarial therapy as a cause of rhabdomyolysis and increases awareness of myoglobin cast nephropathy as a potential complication of malaria.

Ramya Varadarajan

Ashmi Patel

Haneen Salah

Neil Sutaria

Roberto Barrios

Luan Truong

Lillian Gaber

Ziad M. El-Zaatari

Myoglobin Cast Nephropathy Diagnosed on Renal Biopsy in a Patient Treated for Malarial Infection

Peritonitis is the major complication of peritoneal dialysis (PD) patients. Staphylococcus is the leading causative organism of PD-related peritonitis. However, there were more reports of unusual organisms causing peritonitis. Clinical features, management, and outcome of peritonitis from unusual organisms are important information. We reported herein a 72-year-old female patient who presented with fever, abdominal pain, and cloudy dialysate for 3 days. Upon admission, ceftazidime and vancomycin were given intraperitoneally. A preliminary report of blood and PD fluid culture showed the presence of Gram-positive bacilli. Her clinical status improved 48 hours after the commencement of the antibiotics. Subsequently, culture reports of blood and PD fluid showed Lysinibacillus sphaericus which was susceptible to vancomycin at a minimal inhibitory concentration of less than 0.25 μg/mL. The patient was given intraperitoneal vancomycin for a total of 14 days. Then, the PD effluent was clear, and its cell count was below 100 cells/mm3 in 3 days. The patient did not have a recurrence of peritonitis after antibiotic discontinuation. The possibility of this organism infection is environmental contamination related to the patient’s gardening activities.

Teerawat Thanachayanont

Pailin Mahaparn

Tanyarat Teerapornlertratt

Teerachai Chantarojanasiri

Kriang Tungsanga

Peritoneal Dialysis-Related Peritonitis Caused by Lysinibacillus sphaericus

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Case Reports in Nephrology publishes case reports and case series focusing on the prevention, diagnosis, and management of kidney diseases and associated disorders, including cancer. The journal also focuses on advances in transplantation techniques.

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Case Reports in Nephrology - Table of contents 2024

title

Case Reports in Nephrology publishes case reports and case series focusing on the prevention, diagnosis, and management of kidney diseases and associated disorders, including cancer. The journal also focuses on advances in transplantation techniques.

Case Reports in Nephrology