Over the past two decades it has become clear that the glomerular podocyte is a key cell in preventing albuminuria, kidney failure and cardiovascular morbidity. Understanding the key pathways that protect the podocyte in times of glomerular stress, which can also be therapeutically manipulated, are highly attractive. In the following review we assess the evidence that the peroxisome proliferator activating receptor (PPAR) agonists are beneficial for podocyte and kidney function with a focus on PPAR-γ. We explain our current understanding of the mechanisms of action of these agonists and the evidence they are beneficial in diabetic and non-diabetic kidney disease. We also outline why these drugs have not been widely used for kidney disease in the past but they may be in the future.
The effects of the calcimimetic drug Cinacalcet were assessed in six children with uncontrolled hyperparathyroidism secondary to stage 5 chronic kidney disease (CKD). Data were collected retrospectively regarding bone biochemistry and medications. Patients were between the ages of 11 months and 14 years on commencing Cinacalcet at initial doses of 0.4-1.4 mg/kg. Treatment, which was well tolerated in the majority and still on going in five patients, was for periods ranging between 3 months and 3 years. All six cases saw at least an 86% reduction in serum parathyroid hormone (PTH). Hypophosphataemia and/or hypocalcaemia were observed in three cases. Overall, achievement of UK Renal Association targets for corrected calcium (Ca), phosphate (P) and the calcium x phosphate product (Ca x P) were unaffected. We conclude that Cinacalcet is an effective treatment for correcting and sustaining correction of uncontrollable PTH levels seen in a difficult group of patients. Importantly, it has allowed the avoidance of parathyroidectomy for a significant time period in all cases. There remain questions about the effect of Cinacalcet on linear growth amongst paediatric dialysis patients, and future studies should aim to address this.
In cases of congenital lymphoedema the finding of ulceration, violaceous nodules or papules, or apparent traumatic ecchymoses should act as a diagnostic beacon warning of dangers. A case is reported of a high-grade angiosarcoma developing in a patient with congenital hereditary lymphoedema (Milroy's disease). This is the second paper to report this complication, the third case report and the first case in which the diagnosis is substantiated by immunohistochemistry and lectin histochemistry. A review of cases of angiosarcoma complicating congenital hereditary and non-hereditary lymphoedema is also presented.
The results indicate significant shortcomings in the implementation of NICE guidance on childhood UTI in England. The guidance is complex and this makes its implementation challenging. It was difficult to identify children presenting with nonspecific fever from clinical data systems. Adequate IT systems throughout the NHS are a key step to improving implementation of this and other NICE guidance.
Earlier studies have indicated a role for serum lactic dehydrogenase (LDH) in haematological and other malignancies. As this is still not a routine test in many units, its value has been re-examined in a retrospective analysis of serum LDH levels in 278 patients with haematological malignancies managed in a single unit. Abnormal levels at diagnosis were found in 42% of these disorders; yet analysis of over 23,000 routine requests for a biochemical screen showed only 4% with an abnormal LDH level. The estimation was also valuable in follow-up of those haematological patients, with rising levels often indicating progressive or relapsing disease even in cases which had shown a normal LDH at diagnosis.
Aims To describe the aetiologies of paediatric rhabdomyolysis and explore the medium‐term renal consequences. Methods Retrospective, single‐centre review of children with rhabdomyolysis. Results Two hundred and thirty‐two children met inclusion criteria for the analysis. Mean age at presentation was 8.4 (SD ± 5.5) years. The commonest aetiology was infection (28%), with viral myositis making up the clear majority (75%). Trauma was identified as a cause in 18% of children, seizures in 10% and immune‐mediated mechanisms in 8%. Acute kidney injury (AKI) was present in 32% of the cases overall. Children with AKI tended to be younger, with higher peak creatine kinase (CK) and active urinary sediment on urinalysis at presentation. AKI and the need for renal replacement therapy (RRT) were associated with a prolonged hospital stay (15 (interquartile range, IQR 6.5–33) vs. 2 (IQR 0–7) days). A total of 18 children and young people required RRT, with a mean duration of 7.1 ± 4.3 days. Those who received RRT were more likely to have abnormalities on urinalysis at presentation (46% vs. 5%). Over the period of the study, 9% of children died and 2% met criteria for a diagnosis of chronic kidney disease. Conclusions This large paediatric rhabdomyolysis case series provides new and unique insights into the condition. Our results highlight the common aetiologies and provide evidence of good renal recovery overall, even in the most severely affected cases. Abnormalities of urinalysis appear to be important in predicting the development of AKI and the need for RRT.
Summary The presenting features of 120 consecutive cases of myeloma diagnosed in a single unit were examined. Screening tests on routine laboratory samples increased the detection rate but did not significantly reduce the percentage of cases presenting with late stage disease. Morbidity was closely related to the incidence of bone involvement already present at diagnosis. About one fifth of cases had reported suspicious symptoms for some considerable time before further investigation was forthcoming. Almost half had ‘benefitted’ by having concurrent disease which often led to the discovery of myeloma at an early stage. These findings, together with the wide variety of symptomatology and few physical signs, stressed the critical importance of having a high index of suspicion and thus taking suitable blood samples without unnecessary delay.
We present the case of a young boy with Dent's disease, identified as having a mutation in the kidney-specific chloride-proton antitransporter CLCN5 during investigation for nephrotic-range proteinuria. He went on to develop growth hormone deficiency and was treated with recombinant growth hormone. He later presented acutely with hepatorenal failure and thrombotic occlusion of the middle and right hepatic veins consistent with a diagnosis of Budd-Chiari syndrome, which required a prolonged period of intensive care. The diagnosis of Dent's disease should be considered early in boys with nephrotic-range proteinuria in the absence of clinical oedema and hypoalbuminaemia to allow for the timely introduction of strategies, such as a high-citrate diet, to preserve renal function. The measurement of urinary β-2 microglobulin has been shown by this case to be a more reliable and specific marker of tubular dysfunction than the urinary retinol-binding protein.
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