Decoupling shear stress and pressure effects in the biomechanics of autosomal dominant polycystic kidney disease using a perfused kidney-on-chip.

Kidney tubular cells are submitted to two distinct mechanical forces generated by the urine flow: shear stress and hydrostatic pressure. In addition, the mechanical properties of the surrounding extracellular matrix modulate tubule deformation under constraints. These mechanical factors likely play a role in the pathophysiology of kidney diseases as exemplified by autosomal dominant polycystic kidney disease, in which pressure, flow and matrix stiffness have been proposed to modulate the cystic dilation of tubules with mutations.

Urinary collagen peptides: Source of markers for bone metabolic processes in kidney transplant recipients.

Introduction: Kidney transplant recipients (KTRs) are at an increased risk of fractures. Total urinary hydroxyproline excretion served as marker for bone resorption (BR) but was replaced by β-CrossLaps (CTX), a C-terminal collagen α-1(I) chain (COL1A1) telopeptide. We investigated the low-molecular-weight urinary proteome for peptides associated with changes in bone metabolism after kidney transplantation.

Allograft function and muscle mass evolution after kidney transplantation.

Background: Advanced chronic kidney disease is associated with muscle wasting, but how glomerular filtration rate (GFR) recovery after kidney transplantation is associated with muscle mass is unknown.

Methods: We took advantage of the simultaneous measurement of GFR (using iohexol plasma clearance; ioGFR) and creatinine excretion rate (a surrogate marker of muscle mass; CER) performed 3 months after transplantation and at a later time point at our institution to investigate the interplay between allograft function, muscle mass, and outcome in kidney transplant recipients.

Results: Between June 2005 and October 2019, 1319 successive kidney transplant recipients (mean age 50.

Efficacy of Prolonged Antibiotic Therapy for Renal Cyst Infections in Polycystic Kidney Disease.

Objective: To determine the impact of antibiotic therapy (ATBT) on outcomes of renal cyst infection (CyI) in patients with polycystic kidney disease.

Patients And Methods: We undertook a single-center retrospective study of CyI in autosomal dominant polycystic kidney disease (January 1, 2000, through December 31, 2018). Cyst infections were classified as definite (microbiologically proven), probable (radiologic signs), or possible (clinical or biologic signs only).

Aortic root thrombus causing non-ST elevation myocardial infarction after left ventricular assist device.

A 61-year-old male presented with a history of ischemic cardiomyopathy requiring left ventricular assist device (LVAD) implantation 9 months prior to presentation. The patient was on aspirin and warfarin as part of his LVAD management. The patient had chest pain and was found to have non-ST elevation myocardial infarction.

Diagnostic Overshadowing and Pain Insensitivity in a Schizophrenic Patient With Perforated Duodenal Ulcer.

Some patients with schizophrenia and psychotic illnesses have reduced pain perception, and others have decreased pain expression. The diagnosis of the acute abdomen can be delayed, and its outcomes can be worse in psychiatric patients than in non-psychiatric patients. We present a case of perforated peptic ulcer (PPU) in a schizophrenic woman and discuss how the phenomenon of pain insensitivity and diagnostic overshadowing-a process in which a person with mental illness receives inadequate treatment due to a misattribution of physical symptoms to their mental illness-nearly contributed to a missed diagnosis.

The renal inflammatory network of nephronophthisis.

Renal ciliopathies are the leading cause of inherited kidney failure. In autosomal dominant polycystic kidney disease (ADPKD), mutations in the ciliary gene PKD1 lead to the induction of CCL2, which promotes macrophage infiltration in the kidney. Whether or not mutations in genes involved in other renal ciliopathies also lead to immune cells recruitment is controversial.

Long-term renal outcome in methylmalonic acidemia in adolescents and adults.

Background: Chronic kidney disease (CKD) is one of the main long-term prognosis factors in methylmalonic acidemia (MMA), a rare disease of propionate catabolism. Our objective was to precisely address the clinical and biological characteristics of long-term CKD in MMA adolescent and adult patients.

Patients And Methods: In this retrospective study, we included MMA patients older than 13 years who had not received kidney and/or liver transplantation.

The spectrum of kidney function alterations in adolescents with a solitary functioning kidney.

Background: A precise assessment of glomerular filtration rate is key to delineate the care of children with a solitary functioning kidney (SFK). Data regarding measured GFR (mGFR) in this population is restricted to a single study of 77 individuals, which suggested that a GFR estimation (eGFR) method based on creatinine and cystatin C (eGFR-CKiD2) performed better than Schwartz's equation (eGFR-Schwartz).

Methods: We measured GFR in 210 consecutive adolescents (7 to 22 years old) with an SFK referred to our institution between 2014 and 2019 and in 43 young candidates for kidney donation (18 to 25 years old).

Association between 25(OH) vitamin D and graft survival in renal transplanted children.

Background: In children, vitamin D deficiency is common after renal transplantation. Besides promoting bone and muscle development, vitamin D has immunomodulatory effects, which could protect kidney allografts. The purpose of this study was to assess the association between vitamin D status and the occurrence of renal rejection.

mTOR and S6K1 drive polycystic kidney by the control of Afadin-dependent oriented cell division.

mTOR activation is essential and sufficient to cause polycystic kidneys in Tuberous Sclerosis Complex (TSC) and other genetic disorders. In disease models, a sharp increase of proliferation and cyst formation correlates with a dramatic loss of oriented cell division (OCD). We find that OCD distortion is intrinsically due to S6 kinase 1 (S6K1) activation.

Tubular STAT3 Limits Renal Inflammation in Autosomal Dominant Polycystic Kidney Disease.

Background: The inactivation of the ciliary proteins polycystin 1 or polycystin 2 leads to autosomal dominant polycystic kidney disease (ADPKD). Although signaling by primary cilia and interstitial inflammation both play a critical role in the disease, the reciprocal interactions between immune and tubular cells are not well characterized. The transcription factor STAT3, a component of the cilia proteome that is involved in crosstalk between immune and nonimmune cells in various tissues, has been suggested as a factor fueling ADPKD progression.

Association of blood bicarbonate and pH with mineral metabolism disturbance and outcome after kidney transplantation.

In kidney transplant recipients (KTRs), scarce evidence has associated low blood bicarbonate levels with mineral metabolic disturbance and reduced allograft survival. However, the contribution of the blood pH to these observations remains unassessed. Equally, little is known about the influence of the blood provenance (arteriovenous fistula vs peripheral vein) on bicarbonate values.

[Renal transplantation: Procedure and early follow-up].

More than fifty years after the success of the two first renal transplantations in Boston and in Necker hospital in Paris, renal transplantation became the treatment of choice of end stage renal failure, because it improves not only the quality of life of the patients but also their long-term survival. In France, more than 3,700 kidney transplantations are performed every year and more than 40,000 patients are living with a functioning kidney allograft. This treatment of end stage renal disease requires a fine-tuned pre-transplant evaluation and a multidisciplinary post-transplant care in order to prevent, to detect and to treat comorbidities and complications of immunosuppression.

Osmoregulation Performance and Kidney Transplant Outcome.

Background: Kidney transplant recipients have an impaired ability to dilute urine but seldom develop baseline hyponatremia before ESRD. Although hyponatremia is a risk factor for adverse events in CKD and in kidney transplant recipients, it remains unclear whether subtler alterations in osmoregulation performance are associated with outcome.

Methods: We studied a single-center prospective cohort of 1258 kidney transplant recipients who underwent a water-loading test 3 months after transplant to determine osmoregulation performance.

Primary pulmonary Kaposi Sarcoma in a newly diagnosed cisgender heterosexual HIV positive patient presenting before cutaneous manifestations.

AIDS-related Kaposi sarcoma (KS) is a vascular malignancy that usually presents with mucocutaneous lesions. Bronchopulmonary involvement as an initial manifestation is a rare phenomenon. This case describes a young male presenting with pulmonary symptoms mimicking HIV-related opportunistic infection who was eventually diagnosed with primary pulmonary KS.

Cilia-localized LKB1 regulates chemokine signaling, macrophage recruitment, and tissue homeostasis in the kidney.

Polycystic kidney disease (PKD) and other renal ciliopathies are characterized by cysts, inflammation, and fibrosis. Cilia function as signaling centers, but a molecular link to inflammation in the kidney has not been established. Here, we show that cilia in renal epithelia activate chemokine signaling to recruit inflammatory cells.