1/4. Seckel syndrome with polyarteritis nodosa.Seckel syndrome is a rare genetic disorder with a typical "bird-headed" appearance. It could affect many organ systems but renal involvement is uncommon. polyarteritis nodosa is systemic vasculitic disorder which also involves kidneys. We report a case of Seckel syndrome in a 9 year-old boy with renal involvement due to polyarteritis nodosa. According to the literature, this is the first report of polyarteritis nodosa in Seckel syndrome.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
2/4. Successful combined liver and kidney transplant for COACH syndrome and 5-yr follow-up.The COACH syndrome is a very rare disorder with cerebellar vermis hypoplasia, oligophrenia, ataxia, coloboma, and hepatic fibrosis. Nineteen cases with COACH diagnosis have been reported. Neurologic abnormalities are the first symptoms in most cases. Complications of the hepatopathy [portal hypertension, esophageal varices, and gastrointestinal (GI) bleeding] contribute extensively to the morbidity and lethality in the course of the disease. We describe a 28-yr-old female with COACH syndrome resulting in chronic renal and hepatic insufficiency. The patient was found to have significant mental retardation, truncal ataxia, motor abnormality and occulomotor abnormality. She began to develop GI bleeding and encephalopathy because of biopsy-confirmed cirrhosis. We performed combined liver and kidney transplant after challenging discussion. Her postoperative course was uneventful, and she was discharged on the ninth postoperative day (POD). She has not had any problems at 1, 3 and 5-yr follow-up with excellent liver and renal function. This is the first description of successful combined liver and kidney transplant with long-term follow-up. The decision for transplant is challenging because COACH syndrome is rare with only descriptive characterization and patients have non-progressive ataxia and mental retardation. However, our case shows that liver and kidney transplant can be medically successful, and the individuals achieve long-term success if they have a stable neurological condition and an excellent support system.- - - - - - - - - - ranking = 7keywords = kidney (Clic here for more details about this article) |
3/4. Optimizing lithium dosing in hemodialysis.We studied a 62-year-old female hemodialysis patient during initiation and maintenance of lithium carbonate therapy. Three different methods were applied to estimate the regimen: a scenario based on volume of distribution (V(d)), a scenario based on glomerular filtration rate (GFR), and a scenario in which we developed an algorithm based on a 2-compartment distribution without elimination. The GFR estimate led to plasma concentrations 3-4 times lower than those anticipated. In contrast, the estimates based on V(d) and the algorithm derived from pharmacokinetic modeling led to comparable loading dose estimates. Furthermore, the maintenance dose estimated from the central compartment (V1) led to plasma concentrations within the therapeutic range. Thus, a regimen where 12.2 mmol lithium was given after each hemodialysis session resulted in stable between-dialysis plasma lithium concentrations in this patient with no residual kidney function. We did not observe adverse effects related to this regimen, which was monitored from 18 days to 8 months of therapy, and the patient experienced relief from her severe depressive disorder. In conclusion, dialysis patients may be treated with lithium administrated immediately postdialysis. Further observations are necessary to obtain robust long-term safety data and to optimize the monitoring schedule.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
4/4. Applying the Growth Failure in CKD consensus Conference: evaluation and treatment algorithm in children with chronic kidney disease.Growth failure is a common and significant clinical problem for children with chronic kidney disease (CKD), particularly those with chronic renal insufficiency (CRI). Children with CRI (typically defined by a glomerular filtration rate [GFR] <75 mL/min/1.73 m2) who have growth impairment exhibit a variety of medical and psychological problems in addition to increased mortality. Growth failure in children with CKD is usually multifactorial in etiology, including abnormalities in the growth hormone (GH)-insulin-like growth factor (IGF)-I axis and a variety of nutritional and metabolic concerns characteristic of CKD. Proper management of these factors contributes to better growth in affected children. Although the safety and efficacy of recombinant human GH (rhGH) therapy in promoting growth in children with CKD are well established, recent data indicate that the use of rhGH administration in children with CKD and growth failure remains low. Recently, guidelines were developed by the consensus Conference for Evaluation and Treatment of Growth Failure in Children with CKD. This paper focuses on the application of these guidelines to children with CKD.- - - - - - - - - - ranking = 1404.337339475keywords = kidney disease, kidney (Clic here for more details about this article) |