Renal Abnormalities In Patients Research Articles

Horseshoe kidney presenting as recurrent urinary tract infections with severe hydronephrosis in a 10-month-old baby: a case report

Introduction : Horseshoe kidney (HSK) is type of congenital anomalies of the kidneys and urinary tracts (CAKUT), which fusion anomalies happened in early embryonic period partially. One-third of the patients with horseshoe kidney are usually asymptomatic and diagnosed incidentally. It can be presented with other renal and extrarenal abnormalities. Here we report a case of horseshoe kidney in infant presenting as recurrent urinary tract infections with severe hydronephrosis. Case Presentation: A 10-months old boy with multiple congenital anomalies admitted with a history of recurrent urinary tract infection for 3-month and progressive abdominal enlargement. His physical examination revealed an abdominal distention, no-tender, dullness on percussion, a palpable mass on the right flank, and normal bowel sound. The genitalia examination revealed penile-type hypospadia and bilateral undescended testis. Further examination with a contrast computed tomography scan showed HSK with severe right hydronephrosis with thin parenchyma due to UPJO. The heminephrectomy procedure was performed successfully. There is no recurrent UTI and no deterioration in renal function after 3 years of evaluation. Conclusion: It is important to investigate renal abnormalities in patients with recurrent UTI. HSK is one of the prevalent findings. Therefore, proper diagnostic investigation tailored to the clinical presentation in a stepwise approach aiming for prompt and optimal patient care is essential.

Significance of reduced renal function in patients with chronic myelomonocytic leukemia.

In aretrospective study, we analyzed the prevalence of increased creatinine levels in 166 patients with chronic myelomonocytic leukemia (CMML), their potential prognostic impact, and potential correlations with laboratory and molecular features. Increased creatinine values (> 1.1 mg/dl) were found in 71of 166 (43%) patients. The median survival of patients with increased creatinine values was significantly shorter than in patients without impairment of renal function (20vs. 52months, p < 0.001). Patients with increased creatinine values were older, were more often male, had higher leukocyte counts, higher monocyte counts, and higher lactatdehydrogenase (LDH) values. There was atrend toward ahigher prevalence of CBL and ASXL1 mutations in patients with renal impairment. Our findings show ahigh prevalence of renal abnormalities in patients with CMML. Increased creatinine values were identified as anew prognostic marker. These findings may be important for the individualized management of this heterogenous group of patients.

Neutrophil Gelatinase-Associated Lipocalin Profile in Critically Ill Patients with Decreased of Consciousness

Background&#x0D; Neutrophil Gelatinase-Associated Lipocalin (NGAL) is an early marker of renal tubules damage. In critically ill patients,there are significant oxygenation disruptions to many organs particularly the kidneys and the brain. Early recognition of renal abnormalities in patients with a decreased of consciousness may improve the outcomes of these patients.&#x0D; &#x0D; Objective&#x0D; To observe the profile of Neutrophil Gelatinase-Associated Lipocalin in critically ill children with a decreased level of consciousness in Hasan Sadikin Hospital Bandung.&#x0D; &#x0D; Method&#x0D; A cross-sectional study was performed on critically ill children with a decreased of consciousness in Hasan Sadikin Hospital, Bandung.&#x0D; &#x0D; Result&#x0D; Fifty-nine patients aged 2-15 years old were included in the study. There were thirty-seven males (62.7%) and twenty-two females (37.2%). In this study, all critically ill patients had elevated urinary NGAL levels with an average value of 606.95 ng/mL (1.20 -24629.59 ng/mL). We found that 30% of these patients developed sepsis caused by various etiologies which mostly were malignancy in 22 patients (37.3%) and pneumonia in 14 patients (23.7%). Of all the patients, 16.9% showed clinical improvement.&#x0D; &#x0D; Summary&#x0D; Neutrophil Gelatinase-Associated Lipocalin level is elevated in critically ill patients with a decreased of consciousness.

Bone Marrow Findings in Renal Patients: A Single Renal Specialist Center Experience.

ObjectiveThis study evaluated the importance of bone marrow aspiration and trephine biopsy (BM) for the diagnosis of underlying hematological abnormalities in renal patients.MethodsThis cross-sectional study on BM was carried out between August 2010 and April 2019, in our specialist renal center for various unexplained hematological abnormalities in patients with renal diseases [chronic kidney disease (CKD), end-stage renal disease (ESRD) requiring maintenance hemodialysis (MHD), patients with normal renal function but other nephrology and urology issues like stone disease and nephrotic syndrome].ResultsOut of 176 reported BM examinations, 48 (27.3%) were done on ESRD patients on MHD (CKD-D), and 69 (39.2%) on CKD patients not on MHD (CKD-nD). Fifty-nine (33.5%) BM were done on patients with normal renal function (n-CKD). The indication for BM was pancytopenia 50 (28.4%), unexplained anemia 39 (22.2%), and unexplained thrombocytopenia 43 (24.4%). In 91 (51.7%) patients BM was normal. In 30 (17%) patients multiple myeloma (MM) was diagnosed on BM, out of which 18 (26.1%), nine (18%), three (5.3%) were CKD-nD, CKD-D, and n-CKD patients, respectively. In 11 (6.3%) patients BM was suggestive of myelodysplasia (MD), out of these 11 patients, five (10%) were CKD-D patients.ConclusionBM is an underutilized method of diagnosis of hematological abnormalities in renal patients. Our study revealed the importance of BM examination, especially in patients with CKD.

Clinical and pathological findings of SARS-CoV-2 infection and concurrent IgA nephropathy: a case report

BackgroundSince the Coronavirus Disease 2019 (COVID-19) outbreak, there is accumulating data on the clinical characteristics, treatment strategies and prognosis of COVID-19 in patients with concurrent renal disease. Postmortem investigations reveal renal involvement in COVID-19, and most recently, several biopsy researches reveal that acute tubular injury, as well as glomerular nephropathy such as collapsing glomerulopathy were common histological findings. However, to our best knowledge, there is limited data regarding IgA nephropathy in the setting of COVID-19.Case presentationIn the present case, we report a 65-year old Chinese woman who presented with dark-colored urine, worsening proteinuria and decreased renal function after COVID-19 infection. She received a renal biopsy during COVID-19 infection. The renal biopsy revealed IgA nephropathy without any evidence for SARS-Cov-2. The findings suggest that the renal abnormalities were a consequence of exacerbation of this patient’s underlying glomerular disease after COVID-19 infection. After a regimen of 3-day course of glucocorticoid and angiotensin II receptor blocker therapy, the patient recovered and remained stable upon follow-up.ConclusionsIt is important to consider the underlying glomerular disease exacerbation as well as virus induced injury when dealing with renal abnormalities in patients with COVID-19. A kidney biopsy may be indicated to exclude a rapidly progressive glomerular disease.

Mutation profile of BBS genes in patients with Bardet\u2013Biedl syndrome: an Italian study

BackgroundBardet–Biedl syndrome (BBS) is a rare inherited multisystemic disorder with autosomal recessive or complex digenic triallelic inheritance. There is currently no treatment for BBS, but some morbidities can be managed. Accurate molecular diagnosis is often crucial for the definition of appropriate patient management and for the development of a potential personalized therapy.MethodsWe developed a next-generation-sequencing (NGS) protocol for the screening of the 18 most frequently mutated genes to define the genotype and clarify the mutation spectrum of a cohort of 20 BBS Italian patients.ResultsWe defined the causative variants in 60% of patients; four of those are novel. 33% of patients also harboured variants in additional gene/s, suggesting possible oligogenic inheritance. To explore the function of different genes, we looked for correlations between genotype and phenotype in our cohort. Hypogonadism was more frequently detected in patients with variants in BBSome proteins, while renal abnormalities in patients with variations in BBSome chaperonin genes.ConclusionsNGS is a powerful tool that can help understanding BBS patients’ phenotype through the identification of mutations that could explain differences in phenotype severity and could provide insights for the development of targeted therapy. Furthermore, our results support the existence of additional BBS loci yet to be identified.

Atteintes rénales de la trisomies 21

Down syndrome (DS) is often associated with cardiac malformations, so that kidney damage is little known. The objective of this study was to present the diversity of renal abnormalities and their potential progression to chronic renal failure. Among congenital abnormalities of the kidney and urinary tract (CAKUT) abnormalities appear to be frequent: pyelectasis, megaureters, posterior urethra valves, as well as renal malformations such as renal hypoplasia, horseshoe kidney, or renal ectopia. Contributing factors to acute kidney failure have been described in patients with DS: bilateral lesions and minor renal injury, such as glomerular microcysts, tubular dilation, and immature glomeruli. Histological lesions can be found, albeit nonspecific; they occur earlier than in the general population. Two metabolic specificities have also been described: decreased clearance of uric acid and a hypercalciuria by passive hyperabsorption. End-stage renal disease can occur, thus raising the problem of the best choice of management. In conclusion, renal abnormalities in patients in DS should be known so as to preserve a good renal functional prognosis: systematic screening with renal ultrasound can be proposed.

Renal function in hepatosplenic schistosomiasis--an assessment of renal tubular disorders.

BackgroundRenal involvement in Schistosoma mansoni infection is not well studied. The aim of this study is to investigate the occurrence of renal abnormalities in patients with hepatosplenic schistosomiasis (HSS), especially renal tubular disorders.MethodsThis is a cross-sectional study with 20 consecutive patients with HSS followed in a medical center in Maceió, Alagoas, Brazil. Urinary acidification and concentration tests were performed using calcium chloride (CaCl2) after a 12-h period of water and food deprivation. The biomarker monocyte chemoattractant protein 1 (MCP-1) was quantified in urine. Fractional excretion of sodium (FENa+), transtubular potassium gradient (TTKG) and solute-free water reabsorption (TcH2O) were calculated. The HSS group was compared to a group of 17 healthy volunteers.ResultsPatients' mean age and gender were similar to controls. Urinary acidification deficit was found in 45% of HSS patients. Urinary osmolality was significantly lower in HSS patients (588±112 vs. 764±165 mOsm/kg, p = 0,001) after a 12-h period of water deprivation. TcH2O was lower in HSS patients (0.72±0.5 vs. 1.1±0.3, p = 0.04). Urinary concentration deficit was found in 85% of HSS patients. The values of MCP-1 were higher in HSS group than in control group (122±134 vs. 40±28 pg/mg-Cr, p = 0.01) and positively correlated with the values of microalbuminuria and proteinuria.ConclusionsHSS is associated with important kidney dysfunction. The main abnormalities found were urinary concentrating ability and incomplete distal acidification defect, demonstrating the occurrence of tubular dysfunction. There was also an increase in urinary MCP-1, which appears to be a more sensitive marker of renal damage than urinary albumin excretion rate.

Glycosuria-mediated urinary uric acid excretion in patients with uncomplicated type 1 diabetes mellitus.

Plasma uric acid (PUA) is associated with metabolic, cardiovascular, and renal abnormalities in patients with type 2 diabetes but is less well understood in type 1 diabetes (T1D). Our aim was to compare PUA levels and fractional uric acid excretion (FEUA) in patients with T1D vs. healthy controls (HC) during euglycemia and hyperglycemia. PUA, FEUA, blood pressure (BP), glomerular filtration rate (GFR-inulin), and effective renal plasma flow (ERPF-paraaminohippurate) were evaluated in patients with T1D (n = 66) during clamped euglycemia (glucose 4-6 mmol/l) and hyperglycemia (9-11 mmol/l), and in HC (n = 41) during euglycemia. To separate the effects of hyperglycemia vs. increased glycosuria, parameters were evaluated during clamped euglycemia in a subset of T1D patients before and after sodium glucose cotransporter 2 (SGLT2) inhibition for 8 wk. PUA was lower in T1D vs. HC (228 ± 62 vs. 305 ± 75 μmol/l, P < 0.0001). In T1D, hyperglycemia further decreased PUA (228 ± 62 to 199 ± 65 μmol/l, P < 0.0001), which was accompanied by an increase in FEUA (7.3 ± 3.8 to 11.6 ± 6.7, P < 0.0001). In T1D, PUA levels correlated positively with SBP (P = 0.029) and negatively with ERPF (P = 0.031) and GFR (P = 0.028). After induction of glycosuria with SGLT2 inhibition while maintaining clamped euglycemia, PUA decreased (P < 0.0001) and FEUA increased (P < 0.0001). PUA is lower in T1D vs. HC and positively correlates with SBP and negatively with GFR and ERPF in T1D. Glycosuria rather than hyperglycemia increases uricosuria in T1D. Future studies examining the effect of uric acid-lowering therapies should account for the impact of ambient glycemia, which causes an important uricosuric effect.

Alteraciones glomerulares en la hipertensión pulmonar e hipertrofia ventricular derecha

El objeto del presente trabajo fue estudiar las alteraciones renales en los pacientes portadores de hipertensión arterial pulmonar, cualquiera que fuera su origen (siempre que no existiera evidencia de lesión mitral alguna), así como en aquellos que, no teniéndose evidencia plena de hipertensión pulmonar, se encontrara presente la hipertrofia del ventrículo derecho. Poco se ha escrito sobre este tópico, más pobres son aun las conclusiones obtenidas, no conociéndose hasta ahora los factores patogénicos de las alteraciones renales; menos todavía su significación fisiopatológica.

Paraclinical Evolutions Regarding Liver and Renal Abnormalities of Kawasaki Disease in the Southeast of Iran

Background: Kawasaki disease (KD) is a vasculitis affecting multi-organ systems including liver and kidneys. KD is diagnosed by some clinical criteria including sterile pyuria, microscopic hematuria, proteinuria due to renal involvement, liver abnormalities manifesting as abnormal liver function tests, gallbladder hydrops, and hypoalbuminemia. Objectives: The aim of the study was to determine the frequency of liver and renal abnormalities in patients with KD, hospitalized in Ali-Ebne-Abitaleb Hospital of Zahedan during 2006 - 2013. Materials and Methods: Paraclinical findings including serum and urine tests as well as gallbladder ultrasonography of 47 patients with KD hospitalized in Ali-Ebne-Abitaleb Hospital of Zahedan during 2006 - 2013 were reviewed retrospectively. Results: Liver abnormalities were found in 22 (46.8%) cases and impaired liver function tests were more common than other liver abnormalities in this cross-sectional study. The incidence of abnormal liver function tests in this study was about 42%. Gallbladder hydrops were found in 6 (12.7%) patients and 18 (38.3%) had renal involvement. Sterile pyuria was the most common urine abnormality with incidence of 36.2%. Microscopic hematuria and proteinuria were rarely found; in addition, serum urea nitrogen and creatinine had normal levels in all patients. Conclusions: The possibility of KD should be considered in any infants or children with abnormal results in liver or renal function tests. Paraclinical findings of liver and renal involvements of all patients were partly similar to the previous studies.

Checking and correcting compliance: focusing renal patients

Compliance is a behaviour resulting from a specific set of cues and consequences. It is a self-care behaviour which entails obedience to a directive whereas noncompliance is self-care deficit, which calls for rejection of particular behaviour which may result in dissonance and may lead to the development of metabolic abnormalities in renal patients. To understand compliance, it is necessary to look for modifying and enabling factors affecting readiness to undertake recommended behaviour. It has been recognised that poverty, denial of illness, lack of control over life, non-supportive environment, old age, female gender and lower socioeconomic status, affect compliance, which is assumed to be a major obstruction to the effective management of disease and therapeutic disciplines. Diverse direct as well as indirect methods have been identified to measure compliance. Out of numerous methods, none of the methods appear to be completely reliable and valid, although biological assay is considered most accurate among all, as it is not affected by human judgements. To prevent complications due to noncompliance, measures should be adopted for improvement which not only entails role of physician and dietician but also of family. In health care system, compliance check is of prime importance, while aiming for better quality of care and management of patients.

Targeted Exome Sequencing Integrated with Clinicopathological Information Reveals Novel and Rare Mutations in Atypical, Suspected and Unknown Cases of Alport Syndrome or Proteinuria

We applied customized targeted next-generation exome sequencing (NGS) to determine if mutations in genes associated with renal malformations, Alport syndrome (AS) or nephrotic syndrome are a potential cause of renal abnormalities in patients with equivocal or atypical presentation. We first sequenced 4,041 exons representing 292 kidney disease genes in a Caucasian woman with a history of congenital vesicoureteral reflux (VUR), recurrent urinary tract infections and hydronephrosis who presented with nephrotic range proteinuria at the age of 45. Her biopsy was remarkable for focal segmental glomerulosclerosis (FSGS), a potential complication of longstanding VUR. She had no family history of renal disease. Her proteinuria improved initially, however, several years later she presented with worsening proteinuria and microhematuria. NGS analysis revealed two deleterious COL4A3 mutations, one novel and the other previously reported in AS, and a novel deleterious SALL2 mutation, a gene linked to renal malformations. Pedigree analysis confirmed that COL4A3 mutations were nonallelic and compound heterozygous. The genomic results in conjunction with subsequent abnormal electron microscopy, Collagen IV minor chain immunohistochemistry and progressive sensorineural hearing loss confirmed AS. We then modified our NGS approach to enable more efficient discovery of variants associated with AS or a subset of FSGS by multiplexing targeted exome sequencing of 19 genes associated with AS or FSGS in 14 patients. Using this approach, we found novel or known COL4A3 or COL4A5 mutations in a subset of patients with clinically diagnosed or suspected AS, APOL1 variants associated with FSGS in African Americans and novel mutations in genes associated with nephrotic syndrome. These studies demonstrate the successful application of targeted capture-based exome sequencing to simultaneously evaluate genetic variations in many genes in patients with complex renal phenotypes and provide insights into etiology of conditions with equivocal clinical and pathologic presentations.

Long term follow up of congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) on hemodialysis for 19 years: a case report

BackgroundThrombotic thrombocytopenic purpura (TTP) is frequently associated with renal abnormalities, but there have been few reports about renal abnormalities in patients with hereditary TTP. In particular, little is known about the long-term prognosis of patients with childhood-onset congenital TTP.Case presentationWe report a Japanese patient with congenital TTP (Upshaw–Schulman syndrome) who was followed for 19 years after initiation of hemodialysis when he was 22 years old. At the age of 6 years, the first episode of purpura, thrombocytopenia, and proteinuria occurred without any precipitating cause. He underwent living-related donor kidney transplantation from his mother, but the graft failed after 5 months due to recurrence of TTP. Even after resection of the transplanted kidney and resumption of regular hemodialysis, TTP became refractory to infusion of fresh frozen plasma (FFP). Therefore, splenectomy was performed and his disease remained in remission for 10 years. However, TTP recurred at the age of 39 years. Plasma activity of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type I domain 13) was less than 3%, while ADAMTS13 inhibitor was not detected (< 0.5 Bethesda units/mL). The patient died suddenly after hemodialysis at the age of 41 years. Subsequent genetic analysis of this patient and his parents revealed two different heterozygous mutations of ADAMTS13, including a missense mutation in exon 26 (c.T3650C causing p.I1217T) inherited from his father and a missense mutation in exon 21 (c.G2723A causing p.C908Y) inherited from his mother. The former mutation has not been detected before in Japan, while the latter mutation is common in Japan. A retrospective review showed that serum C3 levels were consistently low while C4 levels were normal during follow-up, and C3 decreased much further during each episode of TTP.ConclusionCongenital TTP was diagnosed from the clinical, biochemical, and genetic findings. Infusion of FFP controlled each thrombotic episode, but the effect was limited and of short duration. Review of the complement profile in this patient suggested that a persistently low serum C3 level might be associated with refractory TTP and a worse renal prognosis.

Definition of a critical genetic interval related to kidney abnormalities in the Potocki–Lupski syndrome

Potocki-Lupski syndrome is a genomic disorder caused by duplication of 17p11.2. It is characterized by failure to thrive, intellectual disability, hypotonia, and behavioral difficulties. Structural renal anomalies have been observed in <10% of affected individuals. We present detailed clinical and molecular data on six patients with Potocki-Lupski syndrome, two of whom had renal abnormalities, and investigate the prevalence of kidney abnormalities in the mouse model for the syndrome. In contrast to affected humans, the mouse model does not demonstrate a renal phenotype. Comparison of the duplicated segment in patients with Potocki-Lupski syndrome and the renal phenotype and the syntenic duplicated region in the mouse model allowed us to suggest a 0.285 Mb critical region, including the FLCN gene that may be important for development of renal abnormalities in patients with this duplication.

The spectrum of renal abnormalities in patients with psoriasis

Kidney involvement secondary to psoriasis is a controversial issue. In this study, we evaluated the prevalence of urinary abnormalities in patients with psoriasis. Forty-five psoriasis patients (28 women, 17 men, mean age 44 ± 14 years) and 45 age- and gender-matched control subjects without hypertension or diabetes were enrolled in the study. Psoriasis area and severity index (PASI) was used to assess the severity of psoriasis. Urinalysis by dipstick and microscopic evaluation and 24-h proteinuria and albuminuria were measured in all patients and controls. Pathologic albuminuria was defined as albumin excretion of more than 30 mg/24 h. Renal biopsy was performed in psoriasis patients with urinary abnormalities. Patients with psoriasis and controls were not significantly different with respect to the prevalence of abnormal urinalysis (17.7% vs. 13.3%, P = 0.56), mean 24-h proteinuria (145 ± 66 mg/24 h vs. 141 ± 71 mg/24 h, P = 0.54), and albuminuria (21 ± 34 mg/24 h vs. 8 ± 9 mg/24 h, P = 0.31). However, patients with psoriasis had an increased prevalence of pathologic albuminuria compared with controls (24% vs. 2%, P = 0.005). PASI scores in psoriasis patients correlated significantly with 24-h albuminuria (r = 0.458, P = 0.007). Of the eight patients with psoriasis who had urinary abnormalities, four underwent renal biopsy. Two of them had biopsy-proven glomerulonephritis: mesangial proliferative glomerulonephritis in one and IgA nephropathy in the other. The presence of abnormal urinalysis was not more common in patients with psoriasis than in controls. However, the increased prevalence of pathologic albuminuria and its positive correlation with psoriasis severity may suggest subclinical glomerular dysfunction in these patients.

Abnormal Cystatin C Levels in Two Patients with Bardet-Biedl Syndrome

Bardet-Biedl syndrome (BBS) is an autosomal recessive disorder characterized by central obesity, mental impairment, rod-cone dystrophy, polydactyly, hypogonadism in males, and renal abnormalities. The causative genes have been identified as BBS1-14. In the Western countries, the prevalence of this disease ranges from 1/13,500 to 1/160,000, while only a few Japanese patients have been reported in the English-language literature. The incidence of renal dysfunction or anomalies in previous reports varies considerably ranging from ∼20% to universal occurrence. We here report that two Japanese patients who had BBS with normal BUN and creatinine levels had elevated levels of cystatin C, a sensitive marker of glomerular filtration rate. A urine albumin level increased only in the elder patient. Thus, cystatin C may be useful for detecting renal abnormalities in patients with an apparent normal renal function. Because this disease is diagnosed by accumulation of symptoms, such a sensitive marker might help early diagnosis of BBS.

Coronary Flow Reserve Is Impaired in Hypertensive Patients With Subclinical Renal Damage

Renal dysfunction is relatively common in patients with primary hypertension (PH). A reduction in coronary vasodilator capacity has recently been reported in patients with renal damage undergoing coronary angiography. We investigated the relationship between coronary flow reserve (CFR) and early renal abnormalities in patients with PH and normal serum creatinine. Seventy-six untreated patients were studied. Albuminuria was measured as the albumin-to-creatinine ratio and glomerular filtration rate (eGFR) was estimated by the Cockroft-Gault formula. Chronic kidney disease (CKD) was defined as an eGFR <60 ml/min/1.73 m(2) and/or in the presence of microalbuminuria. Coronary blood flow velocities (cm/s) were measured by Doppler ultrasound at rest and after adenosine administration. CFR was defined as the ratio of hyperemic-to-resting diastolic peak velocities. Prevalence of reduced eGFR, microalbuminuria, CKD, and left ventricular (LV) hypertrophy was 8, 10, 16, and 31%, respectively. Overall, 10% of patients showed impaired CFR (i.e., <2.0). Patients with CKD were more likely to be older (P < 0.05) and of female gender (P < 0.01) and showed higher LV mass index (LVMI) (P < 0.05), lower CFR (P < 0.05; analysis of covariance, P < 0.05), and CFR/LVMI (P < 0.05) than patients with normal renal function. Conversely, patients with impaired CFR showed a significantly higher prevalence of reduced eGFR (chi(2) 5.2, P < 0.05), microalbuminuria (chi(2) 10.2, P < 0.01), and CKD (chi(2) 9.2.1, P < 0.01). Even after adjustment for gender, the presence of CKD entailed a sevenfold higher risk of having impaired CFR (confidence interval 1.17-40.9, P < 0.05). Early renal abnormalities are associated with reduced CFR in PH.

Magnetic Resonance Imaging of Renal Abnormalities in Patients with Congenital Osseous Anomalies of the Spine

Background: Patients with congenital osseous anomalies of the spine are known to have a high prevalence of abnormalities in the renal system and of the spinal cord. Today, the screening tools of choice to detect these abnormalities include ultrasonography of the kidneys and collecting system and magnetic resonance imaging of the spine. A single screening tool that can identify both renal and intraspinal anomalies would be ideal. Methods: Imaging studies of all patients with a congenital osseous anomaly of the spine seen at our institution during a ten-year period were retrospectively reviewed. Only patients who had had both a sonogram of the renal system and a magnetic resonance imaging study of the entire spine were included in the investigation. All studies were reviewed blindly by a pediatric radiologist for this study. Results: One hundred and fifty-three patients met the criteria for inclusion in the study. Forty-one patients (27%) had a total of forty-seven renal abnormalities noted on both the sonogram and the magnetic resonance imaging scan. In no instance was a renal anomaly seen on one study and not on the other. Conclusions: When properly performed, screening magnetic resonance imaging scans of the spine can show renal abnormalities, thus obviating the need for a separate screening renal ultrasound study. Level of Evidence: Diagnostic Level IV. See Instructions to Authors for a complete description of levels of evidence.

Magnetic Resonance Imaging of Renal Abnormalities in Patients with Congenital Osseous Anomalies of the Spine

Patients with congenital osseous anomalies of the spine are known to have a high prevalence of abnormalities in the renal system and of the spinal cord. Today, the screening tools of choice to detect these abnormalities include ultrasonography of the kidneys and collecting system and magnetic resonance imaging of the spine. A single screening tool that can identify both renal and intraspinal anomalies would be ideal. Imaging studies of all patients with a congenital osseous anomaly of the spine seen at our institution during a ten-year period were retrospectively reviewed. Only patients who had had both a sonogram of the renal system and a magnetic resonance imaging study of the entire spine were included in the investigation. All studies were reviewed blindly by a pediatric radiologist for this study. One hundred and fifty-three patients met the criteria for inclusion in the study. Forty-one patients (27%) had a total of forty-seven renal abnormalities noted on both the sonogram and the magnetic resonance imaging scan. In no instance was a renal anomaly seen on one study and not on the other. When properly performed, screening magnetic resonance imaging scans of the spine can show renal abnormalities, thus obviating the need for a separate screening renal ultrasound study.