Congenital Renal Anomalies Research Articles

A Literature Review: Radiological Computed Tomography Assessment for Congenital Renal Anomalies

A Literature Review: Radiological Computed Tomography Assessment for Congenital Renal Anomalies

Evaluating iron replacement therapies in pediatric CKD: A comparative analysis of oral liposomal iron, iron supported lactoferrin and IV iron Dextran

CKD is defined as longstanding kidney damage and reduction in renal function. The global burden of CKD in the pediatric population is 15 per million, with congenital renal anomalies and hereditary disorders being the primary causes. Of these, 10% of patients require RRT. The development of anemia in chronic disease is a significant concern as the disease progresses. This condition exacerbates the pathological state by increasing the risk of infection, inevitably resulting in mortality. The study aims to establish the comparative analysis of three types of iron replacement therapies including oral liposomal iron, iron supported lactoferrin and IV iron dextran by evaluating their safety and efficacy corresponding to the different stages of CKD in pediatric population. A comparison between all three variants of iron replacement therapy shows oral liposomal iron provides higher bioavailability and tolerance but effective only in lower CKD stages. In IV iron dextran, rapid iron store replenishment has been observed, but with significant side effects, including the risk of severe hypersensitivity reaction. Conversely, oral iron-supported lactoferrin has demonstrated a maximal iron recovery response after six months of therapy, but with frequent gastrointestinal side effects.

Lymphedema in Turner syndrome: correlations with phenotype and karyotype.

Lymphedema (LD) in Turner syndrome (TS) is a commonly reported comorbidity, though its associations with karyotype and other comorbidities are poorly understood. Characteristics of patients with TS and LD, including correlation with phenotype and karyotype, are described. Medical records of patients with TS seen in two pediatric institutions from 2002 to 2020 were retrospectively reviewed. Demographic data (age, presentation onset, clinical features, genetics, LD presence, investigations, treatments) were collected. 393 girls with TS with mean age of 12.5 years (SD: 5.7) were identified. LD was noted in 37 % of patients (n=146). Among the 112 patients with TS and documentation of onset of LD, LD was noted within the first year of life in 78.6 % (n=88). 67.6 % (n=96) of total patients with TS and LD had non-mosaic 45, X karyotype. Frequency of webbed neck was significantly greater in girls with TS and LD compared with girls without LD (58 vs. 7 %, p<0.001). Congenital heart anomalies, hypertension, and renal anomalies were also more common in girls with LD. Nail abnormalities with presence of hypoplastic or dysplastic nails were significantly associated with LD (OR: 6.784, 95 % CI 4.235-11.046). The number of girls reporting presence of LD decreased with age. LD in TS often occurs within the first year of life, is less prevalent in older children and adolescents, and issignificantly associated with 45, X karyotype, presence ofwebbed neck, nail changes, congenital heart anomalies, and renal anomalies.

Pancake kidney – a rare form of renal malformation in a 7-year-old girl. A case report and a literature review

Renal ectopia and renal fusion belong to a group of congenital anomalies of the kidney and urinary tract that mostly remain asymptomatic and are diagnosed incidentally. Patients with congenital renal anomalies are prone to urinary tract infections and stone formation, and ultimately more likely to develop chronic kidney disease. Pancake kidney is one of the rarest types of renal anomaly, with complete fusion of the superior and inferior poles as well as the middle parts of both kidneys in the pelvic cavity into a pancake-like mass. Imaging studies play a key role in the diagnosis of this anomaly. We present a case of a girl diagnosed with pancake kidney at the age of 8 years. The patient did not present with any alarming symptoms and the suspicion of renal malformation was raised after an abdominal ultrasound, which shew that the left kidney was missing from its typical location. Ultimately, computed tomography allowed for precise imaging of the urinary tract and establishing the diagnosis.

A Comparative Study between Ultra mini Percutaneous Nephrolithotomy versus Stented Extracorporeal Shock Wave Lithotripsy for Treatment of Renal Stones in Egypt

Abstract Background Nephrolithiasis is the third most common disease of the urinary tract with a 1-year recurrence rate of 7% and 10-year recurrence rate of 50%. Shock wave lithotripsy (SWL), retrograde intrarenal surgery (RIRS), and percutaneous nephrolithotomy (PCNL) are different surgeries to treat renal stones. Percutaneous nephrolithotripsy (24- 30F) remains the standard procedure for treating large renal calculi. While achieving high SFR, it also has many drawbacks such as bleeding, postoperative pain, and a long recovery period due to its large access tract, so the Ultra mini percutaneous nephrolithotripsy (12-14F) with a smaller tract size came into existence to reduce renal parenchymal injury associated with standard PCNL. ESWL is a minimally invasive intervention with good patient tolerance; it is regarded as the first line treatment for nephrolithiasis &amp;lt; 20 mm in size. Aim andObjectives Compare results, safety and outcome of Ultra mini PCNL versus stented extracorporeal shock wave lithotripsy (ESWL) for the management of renal calculi from 10 – 20 mm. Materials and Methods This study was conducted at Urology department, Faculty of Medicine, Ain Shams University. Patients were recruited from the outpatient urology clinic between March 2021 till September 2022. Inclusion criteria included patients between 18 and 60 years, having radioopaque renal stones ranging from 10-20 mm with BMI not exceeding 40. Exclusion criteria were patients who had radiolucent stones, smaller than 10 mm or larger than 20 mm, with congenital renal anomalies or spinal deformity or BMI exceeding 40. Patients with uncorrected bleeding diathesis or pregnant females or untreated UTI were also excluded. After meeting our inclusion and exclusion criteria, 90 patients were thoroughly informed about the study, feeling well about participating in it, an informed written consent was taken from them. Patients were randomized to either Ultra-Mini-Percutaneous nephrolithotomy group or stented SWL group via the closed envelope method(each group 45 patients). Results Stone free rate was higher in the Ultra-mini-PCNL group compared to the stented ESWL group with no statistically significant difference with P-value = 0.316. As for the need for retreatment was slightly higher in the stented ESWL group compared to the Ultra-mini-PCNL group yet this difference was statistically insignificant with P-value 0.681. No statistical significant difference between both groups regarding post operative complications fever, hematuria and need for blood transfusion respectively. Operative time and hospital stay were significantly higher in the ultra-Mini-PCNL group compared to the stented ESWL group with P-value &amp;lt;0.001 for both. Conclusion Both stented ESWL and Ultra-Mini-PCNL are good treatment choices for renal stones less than 2 cm with low complication rates. Denying Ultra-Mini-PCNL from patients with medium sized stones for fear of complications is considered unwarranted. Stone size indices were significant predictor for the need of retreatment. Further studies to compare stone free rate based on stone size in both interventions are needed.

A literature review: Radiological computed tomography assessment for congenital renal anomalies

A literature review: Radiological computed tomography assessment for congenital renal anomalies

Correlation Diagnostic Contribution of SPECT/CT Imaging to 99mTc-DTPA Dynamic Scintigraphy in Patients with Different Renal Pathologies

Introduction: Dynamic renal scintigraphy with 99mTechnetium diethylenetriaminepentaacetic acid (99mTc-DTPA) is a widely performed imaging method to evaluate kidney function of patients with various renal pathologies. However the one-stop-shop combination with subsequent SPECT/CT imaging is a rarely discussed topic. Objective: The aim of the study was to evaluate the diagnostic benefits of SPECT/CT imaging to 99mTc-DTPA dynamic renal scintigraphy. Materials and Methods: A group of 32 patients (21 females and 11 males) who underwent dy- namic nephroscintigraphy with subsequent SPECT/CT was retrospectively studied. A dose of 185MBq (5mCi) 99mTc-DTPA was intravenously applied. A low-dose non-contrast CT protocol was used for attenuation correction and anatomical correlation. Results: Impaired relative renal function in various degrees of left kidney was detected in 18 patients (56%) and of right kidney in 14 patients (44%). SPECT/CT imaging identified the particular cause for the renal dysfunction in the analyzed subjects. The hybrid imaging demonstrated urolithiasis in 11 patients, postoperative ureteral strictures in 5 patients, diagnosed urological cancer in 4 patients and external compression of ureter in 2 patients as a possible obstructive cause. Congenital renal anomalies were the identified causes of impaired renal function in 10 patients – hypoplastic kidney in 4 patients, duplex kidney in 2 patients, multiple cortical cysts in 2 patients, aberrant vessel obstructing the pyeloureteral segment in 1 patient and horseshoe kidney in 1 patient. Conclusion: Dynamic renal scintigraphy combined with SPECT/CT is a very useful clinical tool, by providing not only functional but also anatomical information of the urinary tract, especially in the evaluation of obstructive uropathies and congenital renal abnormalities, that has the potential to change clinical management.

Evaluation of Routine Urine Analysis in Diagnosing Urinary Tract Infection among Pediatric Population: A Validation Study

Background: Urinary tract infection (UTI) stands out as one of the most common bacterial infections affecting children below the age of five. A valid diagnostic test for UTI is of utmost importance for avoiding bacterial resistance.Objectives: To assess the diagnostic efficacy of routine urine examinations in detecting urinary tract infections in pediatric patients, employing urine culture as the reference standardMaterial and Methods: This diagnostic study was conducted at leady reading hospital, Peshawar. The study employed a non-probability consecutive sampling technique and included 130 children between the ages of 1 and 5 years, regardless of gender, who exhibited symptoms suggestive of urinary tract infections. Exclusion criteria comprised children who had taken antibiotics within the past 48 hours, those with congenital renal anomalies such as renal or renal tumors, renal tract obstruction, and acute renal failure. All participants underwent urine regular examination and urine culture. Sensitivities, specificities, and accuracy were calculated, and the data was further stratified based on gender and age.Results: The mean age of the participants was 2.646±1.32 years. The males were predominant (n-86, 66.2%). The urine examination demonstrated a specificity of 90.91% and a sensitivity of 58.06% in detecting urinary tract infections. A statistically significant difference was observed when comparing the diagnosis of Urinary tract infections using urine culture and urine examination.Conclusion: While urine examinations exhibit satisfactory specificity, their sensitivity is insufficient to be utilized as a reliable diagnostic test for detecting urinary tract infections in children. Clinicians are advised to depend on urine culture for accurate diagnosis in such cases.Keywords: Diagnostic accuracy, Pediatrics, Urine Microbiology, Urinalysis, Urinary tract infection.

Bilateral Renal Ectopia-Prenatal Diagnosis.

This report explores the diverse spectrum of congenital anomalies of the kidney and urinary tract (CAKUT), ranging from asymptomatic presentations to the most severe form characterized by bilateral renal agenesis. Genitourinary anomalies, a prevalent subset within this domain, account for a significant proportion, constituting 15-20% of anomalies identified during prenatal screening. An ectopic kidney is defined by the presence of an empty renal fossa and the displacement of the kidney from the lumbar region to alternative locations, with the pelvic region emerging as the most prevalent site. The reported case involves bilateral renal ectopia with unilateral duplex kidney. Initial suspicions of a renal anomaly arose during the first trimester, leading to a definitive diagnosis in the second trimester. The patient underwent regular monitoring every four weeks, ultimately delivering a healthy baby at term. This case underscores the frequency of renal anomalies, emphasizing that a considerable proportion remains asymptomatic. These findings contribute to a broader understanding of congenital renal anomalies, their varied manifestations, and the importance of vigilant prenatal screening for early detection and management.

The Outcomes of Cardiac Surgery in Children With DiGeorge Syndrome in a Single Center Experience: A Retrospective Cohort Study.

Background DiGeorge syndrome, a common genetic microdeletion syndrome, is associated with multiple congenital anomalies, including congenital cardiac diseases. This study aims to identify the short and midterm outcomes of cardiac surgery performed on children with DiGeorge syndrome. Methods A retrospective cohort study was conducted between the period of 2018-2022, which included children divided into two groups with a 1:2 ratio. Group one included DiGeorge syndrome patients who were diagnosed using fluorescence in situ hybridization (FISH). Group two included the control group of patients who were clear of genetic syndromes. The two groups were matched based on similar cardiac surgery, age of surgery, and Risk Adjustment in Congenital Heart Surgery (RACHS-1) score. The two groups were compared based on the demographical data and postoperative complications. Results The study consisted of 81 children; 27 were DiGeorge syndrome patients, and 54 were in the control group. DiGeorge syndrome patients showed an increase in mechanical ventilation duration (p=0.0047), intensive care unit (ICU) length of stay (p=0.0012), and hospital length of stay (p=0.0391). Moreover, they showed an increased risk for bacteremia (p=0.0414), ventilator-associated pneumonia (VAP; p=0.0036), urinary tract infections (UTI; p=0.0064), and surgical site infection (SSI; p≤0.0001). They were also more susceptible to postoperative seizures (p=0.0049). Furthermore, patients with DiGeorge syndrome had a higher prevalence of congenital renal anomalies. However, there was no mortality in either group. Conclusion This study shows a variability in the postoperative outcomes between the two groups. The study demonstrates that patients with DiGeorge syndrome have higher risks of infections and longer hospital stayduring the postoperative period. Further research with a larger sample is needed to confirm our findings.

Optimizing management of congenital renal and urinary tract anomalies in children – efficient algorithms

Optimizing management of congenital renal and urinary tract anomalies in children – efficient algorithms

Autonomous and non-cell autonomous role of cilia in structural birth defects in mice.

Ciliopathies are associated with wide spectrum of structural birth defects (SBDs), indicating important roles for cilia in development. Here, we provide novel insights into the temporospatial requirement for cilia in SBDs arising from deficiency in Ift140, an intraflagellar transport (IFT) protein regulating ciliogenesis. Ift140-deficient mice exhibit cilia defects accompanied by wide spectrum of SBDs including macrostomia (craniofacial defects), exencephaly, body wall defects, tracheoesophageal fistula (TEF), randomized heart looping, congenital heart defects (CHDs), lung hypoplasia, renal anomalies, and polydactyly. Tamoxifen inducible CAGGCre-ER deletion of a floxed Ift140 allele between E5.5 to 9.5 revealed early requirement for Ift140 in left-right heart looping regulation, mid to late requirement for cardiac outflow septation and alignment, and late requirement for craniofacial development and body wall closure. Surprisingly, CHD were not observed with 4 Cre drivers targeting different lineages essential for heart development, but craniofacial defects and omphalocele were observed with Wnt1-Cre targeting neural crest and Tbx18-Cre targeting epicardial lineage and rostral sclerotome through which trunk neural crest cells migrate. These findings revealed cell autonomous role of cilia in cranial/trunk neural crest-mediated craniofacial and body wall closure defects, while non-cell autonomous multi-lineage interactions underlie CHD pathogenesis, revealing unexpected developmental complexity for CHD associated with ciliopathies.

Clinical application of 99mTc-DTPA dynamic renal scintigraphy with consequent SPECT/CT imaging in patients with different nephrological conditions

Introduction: Dynamic renal scintigraphy with 99mTechnetium diethylenetriaminepentaacetic acid (99mTc DTPA) is a generally used imaging technique for evaluating renal function of patients with various nephro-urological conditions. Clinical application of SPECT/CT nephroscintigraphy has rarely been discussed. Objective: The aim of the study was to evaluate the added value of SPECT/CT imaging to dynamic renal scintigraphy with 99mTc DTPA. Materials and Methods: Thirty two patients (66% female and 34% male) who underwent dynamic nephroscintigraphy with subsequent SPECT/CT with mean age 55.9±18.25 years was observed. The CT scan was performed with a low dose protocol. A dose of 185MBq (5mCi) 99mTc DTPA was administered intravenously. Results: Our analysis demonstrated impaired relative renal function in various degrees of left kidney in 18 patients (56%) and of right kidney in 14 patients (44%). SPECT/CT imaging revealed the specific cause, responsible for the renal dysfunction in the cases. The hybrid imaging demonstrated urolithiasis in 11 patients, postopertative strictures of ureter in 5 patients, diagnosed urological cancer in 4 patients, external compression of ureter from ovarian cyst in 1 patient and compression of ureter due to nephroptosis in 1 patient as a possible obstructive cause. In 10 patients congenital renal anomalies were the identified causes of impaired renal function – hypoplastic kidney in 4 patients, duplex kidney in 2 patients, multiple cortical cysts in 2 patients, aberrant vessel obstructing the pyeloureteral segment in 1 patient and horseshoe kidney in 1 patient. Conclusion: The combination of conventional 99mTc DTPA dynamic renal scintigraphy with subsequent SPECT/CT could provide not only functional but also anatomical information of the urinary tract, especially in the evaluation of obstructive uropathies and congenital renal abnormalities, that can change the clinical management

Challenges in diagnosing vaginal mass in a pregnant woman with an underlying duplex collecting system disease: a case report.

In this case, a 23-year-old pregnant woman at 32 weeks came to the clinic for a routine check-up. A vaginal mass had been noticed during the examination and was punctured, revealing unknown fluid. Further investigations revealed left duplex collecting system consisted of an upper moiety opening with a ureterocele in the anterior wall of the vagina and a lower moiety ending with an ectopic orifice near the right ureter orifice. Therefore, the modified Lich-Gregoir procedure was done to reimplant the ureter of the upper renal moiety. Postoperative following-up investigations affirmed improvement without complications. The duplex collecting system disease may remain asymptomatic until adulthood or present with unexpected symptoms. The subsequent workup in the duplex kidney disease depends on the moieties' function and the ureter orifice opening site. Although the Weigert-Meyer rule is usually used to describe the typical pattern of duplex collecting system ureters opening sites, it has many expectations in the literature. This case shows how some common symptoms may lead to finding an unexpected urinary tract abnormality.

Duplicated Inferior Vena Cava in a 69-Year-Old White Female Donor

While relatively uncommon, a duplication of the inferior vena cava is moderately well-discussed in the literature. This anatomical variation was noted in a 69-year-old white female donor. This variation is typically asymptomatic; however, it can be associated with complications, such as confusion with a mediastinal mass, increased risk for thromboembolism, and hemorrhage during surgery. It is also associated with a handful of comorbidities, including, but not limited to, congenital renal anomalies such as horseshoe kidney or fused crossed kidney. Research supports that the variation of a duplicated IVC (DIVC) can be due to a failure of the left supracardinal vein to regress during embryonic development.

Unfavorable factors in accessing the pelvicalyceal system during retrograde flexible ureteroscopy (fURS).

Flexible ureteroscopy (fURS) is a well-established procedure for treating multiple upper-urinary tract pathologies, particularly renoureteral lithiasis. Endoscopes have undergone significant advancements, including miniaturization, improved optics, and increased maneuverability. In addition, advancements in accessory instruments, such as the performance of laser fibers, guidewires, and extraction probes, have played a significant role in improving the overall performance of flexible ureteroscopy procedures. However, despite these advancements, unique circumstances can make achieving optimum results during flexible ureteroscopy challenging. These include congenital renal anomalies (horseshoe kidneys, ectopic kidneys, rotation anomalies), as well as the unique intrarenal anatomy (infundibulopelvic angle, infundibular length) or the specifications of the endoscope in terms of maneuverability (active and passive deflection). This review explored challenging scenarios during flexible ureteroscopy procedures in the pyelocaliceal system.

Prevention of ureteral injury during laparoscopic colorectal cancer surgery with horseshoe kidney using fluorescent ureteral catheters: a case report

BackgroundHorseshoe kidney is one of the most common congenital renal fusion anomalies and is characterized by abnormalities in the position, rotation, vascular supply, and ureteral anatomy of the kidney. When performing surgery for colorectal cancer in patients with horseshoe kidneys, anatomical identification is important to avoid organ injuries. Several reports on surgery for colorectal cancer with horseshoe kidneys have described the usefulness of three-dimensional (3D) computed tomography (CT) angiography for detecting abnormalities in vascular supply. However, few reports have focused on the prevention of ureteral injury in surgery for colorectal cancer with horseshoe kidney, despite abnormalities in the ureteral anatomy. Here, we report a case in which laparoscopic sigmoid colon resection for sigmoid colon cancer with a horseshoe kidney was safely performed using fluorescent ureteral catheters.Case presentationA 60-year-old Japanese man presented to our hospital testing positive for fecal occult blood. Colonoscopy revealed sigmoid colon cancer, and CT confirmed a horseshoe kidney. The 3D-CT angiography showed aberrant renal arteries from the aorta and right common iliac artery, and the left ureter passed across the front of the renal isthmus. A fluorescent ureteral catheter was placed in the left ureter before the surgery to prevent ureteral injury. Laparoscopic sigmoid colon resection with D3 lymph node dissection was performed. The fluorescent ureteral catheter enabled the identification of the left ureter that passed across the front of the renal isthmus and the safe mobilization of the descending and sigmoid colon from the retroperitoneum. The operative time was 214 min, with intraoperative bleeding of 25 mL. The patient’s postoperative course was good: no complications arose and she was discharged on the seventh postoperative day.ConclusionIn patients with horseshoe kidney, the use of fluorescent ureteral catheters and 3D-CT angiography enables safer laparoscopic surgery for colorectal cancer. We recommend the placement of fluorescent ureteral catheters in such surgeries to prevent ureteral injury.

Pattern of urinary tract and surgical site infection in allograft renal recipient during early postoperative period

A kidney transplant (KT) offers a significant survival benefit and is the only curative treatment for patients with ESRD. Infectious complications are still a leading cause of morbidity and death among transplant patients. This study aimed to assess the organisms and risk factors responsible for urinary tract and surgical site infection in allograft live-related renal transplant recipients during the early post-transplant period. This observational follow-up study was conducted among 20 purposively selected patients who underwent renal allograft transplant surgery from July 2019 to December 2020 in the Department of Urology, BSMMU, Dhaka. Patients with a history of renal transplant recipients or congenital renal anomalies and any urological malignancy were excluded from the study. After kidney transplant, UTI developed in 6 (30%) renal transplant recipients where all were complicated types and occurred between 10 to 14 days postoperatively. UTI was caused by E. coli in 3 (15%) patients, while Klebsiella and Enterococci were causative organisms for 1 (5%) and 2 (10.0%) patients, respectively. SSI developed in 3 (15%) renal transplant recipients where two were superficial, and one was deep. SSI occurred between 5-8 days postoperatively. SSI was caused by S. aureus in 2 (10%) patients and Pseudomonas in 1 (5%) patient. UTI was significantly more in older patients and the patients with a longer duration of catheterization. Older patients and overweight patients had significantly higher rates of SSI. In total, 33% of the recipient during the early hospital stay developed UTIs, all of which were complicated types. SSI developed in 15%, where 2/3rd was superficial and 1/3rd was deep. Older age with a longer duration of catheterization and DJ stent was found to be associated with the occurrence of UTI, while older age and high BMI were related to the event of SSI. BSMMU J 2022; 15(2): 127-131

HNF1B Alters an Evolutionarily Conserved Nephrogenic Program of Target Genes.

Mutations in hepatocyte nuclear factor-1 β ( HNF1B ) are the most common monogenic causes of congenital renal malformations. HNF1B is necessary to directly reprogram fibroblasts to induced renal tubule epithelial cells (iRECs) and, as we demonstrate, can induce ectopic pronephric tissue in Xenopus ectodermal organoids. Using these two systems, we analyzed the effect of HNF1B mutations found in patients with cystic dysplastic kidney disease. We found cross-species conserved targets of HNF1B, identified transcripts that are differentially regulated by the patient-specific mutant protein, and functionally validated novel HNF1B targets in vivo . These results highlight evolutionarily conserved transcriptional mechanisms and provide insights into the genetic circuitry of nephrogenesis. Hepatocyte nuclear factor-1 β (HNF1B) is an essential transcription factor during embryogenesis. Mutations in HNF1B are the most common monogenic causes of congenital cystic dysplastic renal malformations. The direct functional consequences of mutations in HNF1B on its transcriptional activity are unknown. Direct reprogramming of mouse fibroblasts to induced renal tubular epithelial cells was conducted both with wild-type HNF1B and with patient mutations. HNF1B was expressed in Xenopus ectodermal explants. Transcriptomic analysis by bulk RNA-Seq identified conserved targets with differentially regulated expression by the wild-type or R295C mutant. CRISPR/Cas9 genome editing in Xenopus embryos evaluated transcriptional targets in vivo . HNF1B is essential for reprogramming mouse fibroblasts to induced renal tubular epithelial cells and induces development of ectopic renal organoids from pluripotent Xenopus cells. The mutation R295C retains reprogramming and inductive capacity but alters the expression of specific sets of downstream target genes instead of diminishing overall transcriptional activity of HNF1B. Surprisingly, targets associated with polycystic kidney disease were less affected than genes affected in congenital renal anomalies. Cross-species-conserved transcriptional targets were dysregulated in hnf1b CRISPR-depleted Xenopus embryos, confirming their dependence on hnf1b . HNF1B activates an evolutionarily conserved program of target genes that disease-causing mutations selectively disrupt. These findings provide insights into the renal transcriptional network that controls nephrogenesis.

A multi-institutional study of renal outcomes and renal-related pregnancy outcomes in uterus transplant recipients

Uterus transplantation (UTx) is an effective treatment option for uterine factor infertility. However, the need for immunosuppression and congenital renal anomalies that coexist with uterine agenesis in about 30% of women with Mayer-Rokitansky-Kuster-Hauser syndrome create a risk for renal dysfunction. We therefore examined renal function trajectory and related pregnancy complications in an international cohort of 18 UTx recipients from September 2016-February 2020 who had at least one live birth. All UTx recipients had a diminution in their renal function that was apparent starting at 30 days posttransplant and in half the reduction in eGFR was at least 20%; the decrease in eGFR persisted into the early post-partum period. Half met criteria for Stage 1 acute kidney injury (AKI) as defined by the AKI Network criteria during their pregnancy. Overall, 28% of UTx recipients developed pre-eclampsia. eGFR was lower at embryo transfer and throughout pregnancy among those who developed pre-eclampsia, reaching statistical significance at week 16 of pregnancy. This effect was independent of tacrolimus levels. Mean eGFR remained significantly lower in the first 1-3months after delivery. In the subgroup who reached 12 months of postpartum follow up and had a graft hysterectomy (n= 4), there was no longer a statistical difference in eGFR (pretransplant 106.7ml/m ± 17.7 vs. 12 mos postpartum 92.6ml/m ± 21.7, p= .13) but the number was small. Further study is required to delineate long term renal risks for UTx recipients, improve patient selection, and make decisions regarding a second pregnancy.