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Dissertation
Autosomal dominant polycystic kidney disease : from bedside to bench and back.
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ISBN: 9789090272801 Year: 2012 Publisher: Leuven KU Leuven. Faculteit Geneeskunde. Departement Cellulaire en moleculaire geneeskunde

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Dissertation
HNF1β mutation in 2 children with abdominal pain: case report and literature review

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Mutations in HNF1β predominantly result in renal malformations and are associated with Maturity Onset Diabetes of the Young type 5 (MODY5). In addition to pancreatic dysfunction and renal malformations, the spectrum of HNF1β-associated disease also includes abnormal liver function, genital tract abnormalities, hypomagnesemia, hyperuricemia and early-onset of gout. Although renal dysfunction is one of the most frequent presenting symptoms of HNF1β-associated disease, endocrinologists and paediatricians should be made aware about this entity.

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Dissertation
Cytopenia in Autosomal Dominant Polycystic Kidney Disease
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Year: 2020 Publisher: Leuven KU Leuven. Faculteit Geneeskunde

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Autosomal dominant polycystic kidney disease (ADPKD), the most common monogenic inherited kidney disease, is associated with cytopenias in observational studies. Especially lymphopenia is more common in patients with ADPKD than in patients with other chronic kidney diseases. Although the underlying mechanism of this association is unclear, it has been hypothesized to be secondary to sequestration of white blood cells in cystic organs or related to the uremic proinflammatory environment in chronic kidney disease. However, since mutations in polycystin-1 and -2 affect several immunomodulating pathways, cytopenia may well be an unrecognized extrarenal manifestation of ADPKD. Furthermore, many important questions on the clinical implications of this finding and the effect on the disease course in these patients are unanswered. In this review, we provide an overview of the current evidence on cytopenia in ADPKD and explore the underlying mechanisms of this association and its potential prognostic implications.

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Dissertation
Kidney cystic diseases in children
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Year: 2023 Publisher: Leuven KU Leuven. Faculteit Geneeskunde

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Kidney cystic diseases comprise a broad group of heterogeneous diseases, that highly differ in age at onset, disease manifestation, systemic involvement, disease progression and long term prognosis. As our understanding of these diseases continues to evolve and new treatment strategies continue to emerge, correctly differentiating and diagnosing these diseases becomes increasingly important. In this review, we aim to highlight the key features of the most relevant cystic kidney diseases, underscore important diagnostic characteristics of each disease and present specific management options if applicable.

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Dissertation
The anteroposterior diameter and the relative renal function are good predictors for pyeloplasty in low and normal kidney function
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Year: 2021 Publisher: Leuven KU Leuven. Faculteit Geneeskunde

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Introduction The management of hydronephrosis and a suspicion of a ureteropelvic junction obstruction with a different than normal functioning kidney is controversial. Is a supranormal relative function a good or rather a bad sign? In the event of a lower-than-normal value, is there a cut off value where a reconstructive pyeloplasty is useless? In hydronephrosis, besides the excretion curve, the relative renal function is an important decision factor for surgery, and it is determined by using a radio-isotope exam, most frequently MAG-3. However, several factors may influence the accuracy of this study. In this retrospective study the preoperative anteroposterior diameter (APD) and the relative function of the hydronephrotic kidney is observed postoperatively and the effect of a dismembered pyeloplasty in children is analyzed. Methods A total of 146 cases of children younger than 16yr old with diagnosis of unilateral ureteropelvic junction obstruction underwent an open pyeloplasty between 1997-2019. Children with bilateral diseases, redo-pyeloplasty, laparoscopic or robotic pyeloplasty were excluded. Follow-up evaluation was made by ultrasound pre- and post-operatively at 6 weeks, 6 months, 12 months and more than one year, specifically by reviewing the APD. The relative and differential renal function (DRF) was individually calculated pre- and in some patients post-operatively by diuretic renography such as DTPA, DMSA, MAG3 or EC scan. The patients were categorized in 3 groups, group I with a DRF <30%, group II 30-50% and group III >50%. We evaluated the value and the postoperative course of ultrasound findings and renal differential function before and after a reconstructive pyeloplasty. Clinical variables that may have an impact on these changes were examined. Results Patients with poorly functioning kidneys (<20%), we noted a change of renal function of 2.80 % (p = 0.259) with reduced APD in more than 6 months. The highest functional improvement of DRF (>5%) was seen in group II (mean postoperative change of 2.32 %, P = 0.05). The supranormal kidneys (≥55%) had a decreased change in DRF with a decreased mean change of APD from 6 weeks. Multivariate logistic regression identifies that baseline DRF (odds ratio [OR] = 0.96, P = 0.014) and APD (OR = 1.61, P = 0.013) were the only factors predicting improvement in postoperative DRF greater than 5%. Conclusion This study revealed that improvement in DRF after surgery was significant in normal or moderately impaired preoperative renal function (group II). Baseline DRF and APD were the only predictive factors for prediction of improvement in renal function after surgery. In poorly functioning kidneys no improvement in DRF was observed, but also no further deterioration. The kidneys with a supranormal DRF showed a postoperative slightly reduction of kidney function. In all 3 groups of patients, the APD reduced postoperatively and remained stable as of 6 months postoperatively.

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Dissertation
The value of ambulatory blood pressure monitoring in children with autosomal dominant polycystic kidney disease
Authors: --- --- ---
Year: 2021 Publisher: Leuven KU Leuven. Faculteit Geneeskunde

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Background The importance of ambulatory blood pressure monitoring (ABPM) for diagnosing hypertension in children with autosomal dominant polycystic kidney disease (ADPKD) has recently been demonstrated. However, extensive data on hypertension profiles in ADPKD children are lacking. This study is the first to focus on the prevalence of masked hypertension, white coat hypertension and sustained hypertension on ABPM in ADPKD children. Methods We conducted a retrospective study on ABPM data in a pediatric ADPKD cohort (<19 years). Demographics and clinical data were collected together with the blood pressure measurements from the outpatients clinic (BPc) and from ABPM. The hypertension phenotypes were diagnosed by comparing BPc to ABPM findings. Repetitive ABPM records from the same patients were also analyzed. Results 59 patients with a mean age of 10.8 ± 3.8 years were included. On ABPM data, hypertension was diagnosed in 19% of patients. Comparing ABPM data to BPc showed white coat hypertension, masked hypertension and sustained hypertension in 10%, 12% and 7% of all patients respectively. Repetitive ABPM records were performed in 30 patients (51%). White coat, masked and sustained hypertension newly emerged on these records in 14%, 7% and 11% of patients, respectively. Conclusion ABPM is the golden standard for the correct diagnosis and monitoring of hypertension in ADPKD children. Moreover, we demonstrated that ABPM is also very important in detecting masked hypertension and white coat hypertension which can influence the treatment of these children. Repetitive ABPM records should be considered, since new hypertension phenotypes may arise at later records.

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Dissertation
A Clinicopathologic Review of Renal Cell Carcinoma Diagnosis in Tuberous Sclerosis Complex

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Tuberous Sclerosis Complex (TSC) is a genetic disorder characterized by benign tumours in several organs, including renal angiomyolipoma in up to 80%. However, renal cell carcinoma (RCC) is diagnosed in up to 5% of patients with TSC. Fat-poor angiomyolipoma can mimic RCC on imaging, and clinicians rely on histopathological evaluation for definitive diagnosis.. However, in the context of TSC, angiomyolipoma can possess characteristics that complicate the distinction between both entities. Literature on this difference is very scarce. We retrospectively reviewed the clinical data of all TSC patients who had a diagnosis of RCC and re-analysed prospectively the pathology of the renal lesions. Seven patients underwent 5 partial nephrectomies and 5 total nephrectomies (n=10) at an average age of 34.0 (range 1.3-66.2) years. A tentative diagnosis of RCC was made in all the patients based on imaging, none had a needle biopsy prior to surgery. One original histological RCC diagnosis was ’downgraded’ to the diagnosis of angiomyolipoma. One sample consisted of a solitary fibrous tumour and RCC. The other samples contained angiomyolipoma with or without epithelial tumour components with atypical and/or discordant morphological and immunohistochemical features. At last follow-up of 7.8 years (range 1.0-16.8), no metastasis has been documented. This small cohort study illustrates the complexity of the histopathological diagnosis and classification of tumours with epithelial features in TSC. In order to avoid unnecessary nephrectomies, correlation of the pathological findings with imaging data, as well as further research on discriminating tissue markers and biological behaviour is mandatory.

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Dissertation
Rituximab in children with steroid dependent nephrotic syndrome experience of a tertiary center and review of the literature

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Objectives: Rituximab (RTX) is a new treatment option in children with difficult-to-treat steroid-dependent nephrotic syndrome (SDNS). We evaluated the experience of our tertiary center and reviewed the current literature. Methods: This is a retrospective single-center study evaluating the efficacy and safety of RTX in children with difficult-to-treat SDNS. Age at diagnosis, type and duration of immunosuppression, age at administration, dose of RTX, possible adverse events, number of relapses, duration of remission, and B-cell count after administration of RTX were analyzed. Results: Nine children with a median age at diagnosis of nephrotic syndrome of 4.75 (range 1.33–11.33) years and a median age at administration of RTX of 16.08 (range 3.33–19.25) years were included. Before administration of RTX they had a median number of relapses per year of 1.70 (range 0.82–4.80). At last follow-up (median 2.75 years, range 0.58–3.92), a reduction in the number of relapses per year to 0.26 (range 0–2.18) was noted, despite cessation or lowering the dose of immunosuppressive therapy. Four patients achieved complete remission after the first administration of RTX, four more patients after subsequent doses of RTX. No severe adverse events were noted. Conclusion: RTX was an effective and safe therapeutic option in our cohort of children with difficult-to-treat SDNS, resulting in a significant reduction of yearly relapses in the absence of severe adverse events and facilitating the reduction of other immunosuppressive medication.

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Dissertation
Hypertension in Tuberous Sclerosis complex: the usefulness of screening

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Background: Tuberous sclerosis complex (TSC) is an autosomal dominant genetic syndrome characterized by growth of benign tumours (hamartomas) in multiple organs, including the kidneys. Although renal complications are an important cause of morbidity and mortality, until now, very little is known about the natural history of these renal lesions and co-morbidities such as hypertension in TSC patients. We hypothesize that hypertension may represent an important risk factor for morbidity in TSC patients and that its detection and treatment in an early stage may be beneficial for long term renal outcome. Aims: To use ambulatory blood pressure measurements to define the blood pressure profile in a small cohort of TSC patients and to detect true hypertension in this population. Methods: A prospective, cross-sectional study in a small TSC cohort, in which conventional and ambulatory blood pressure measurement, blood sampling and ultrasound of the kidneys were performed. Results: Fifteen TSC patients (6 younger than 18 years) were included. Hypertension was observed in 6/15, white coat hypertension in 3/15, and normotension in 6/15. No masked hypertension was detected. The prevalence of nocturnal non-dipping was 4/15. Conclusion: In a small TSC cohort, we demonstrated that ambulatory blood pressure measurement detected both nocturnal non-dipping and white coat hypertension. These findings have to be further investigated in a large cohort of TSC patients in order to better define the prevalence of true hypertension and to develop appropriate guidelines for screening, follow-up and treatment of these patients. In the meanwhile, we advise annual screening in TSC patients by ambulatory blood pressure measurement and early treatment of hypertension.

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Dissertation
An interesting case “out of paper” with celiac disease leading to xylophagia: case report and review of literature

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We describe a young girl presenting with the intriguing symptom of xylophagia, subsequently being diag- nosed with celiac disease. Our goal is to review literature on prevalence and etiology of pica and to raise awareness on the less obvious presentations of celiac disease and its possible consequences. In this case, di- agnosis was established and treatment was initiated rapidly after first atypical presentation on consultation, with good clinical outcome. We want to stress the need for further population-based research on pica, given the potential and dangerous consequences of ingestion of nonnutritive substances.

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