Is edema in minimal change disease of childhood really hypovolemic?

OBJECTIVES: In this study, we aimed to find out whether children with minimal change disease can be classified as hypervolemic by objective measures. METHODS: Eighteen children with minimal change disease diagnosed at our department between November 2005 and May 2007 were included in this study. All patients were newly diagnosed or relapsed but were steroid free for at least 6 months. In the first week of edema and when edema resolved (5-7 days after initiation of therapy), weight, height and blood pressure were obtained from all patients. Serum and plasma samples were taken following a starvation period of 12-14 h. The volume load of all patients was evaluated, measuring the inferior vena cava indices in each stage by echocardiography. RESULTS: Average weight at presentation was 8.5% higher than the ideal (dry) weight. There were significant differences between the first and post-treatment body weights, abdomen circumference, and systolic and diastolic blood pressure values (P < 0.05 for each). The inferior vena cava index (IVCI) values decreased significantly after diuretic treatment (P < 0.001), while inferior vena cava collapsibility index (IVCCI) values increased in the post-treatment period (P < 0.001). CONCLUSION: We believe that a close follow-up of hypervolemic children with MCD, treated solely with easy-to-handle diuretics instead of I.V. albumin and diuretics may properly solve the edematous state in these patients.     

Is extracranial-intracranial bypass surgery effective in certain patients?

Cerebral revascularization can be performed through a variety of extracranial-intracranial (EC-IC) bypass operations, using several different donor and recipient vessels, interposition grafts, and anastomotic techniques. The choice of bypass option is dependent on many factors, including the goals of the operation and the availability and accessibility of particular donor and recipient vessels. Potential indications for EC-IC bypass fall into two major categories: (1) flow replacement, in the treatment of complex aneurysms or tumors that require vessel sacrifice and (2) flow augmentation, for treatment of cerebral ischemia. The effectiveness of EC-IC bypass for these indications is reviewed in this article.     

Is subtotal bilateral thyroidectomy still indicated in patients with Grave's disease?

AIM OF THE STUDY: To evaluate the morbidity and the functional results of subtotal bilateral thyroidectomy in patients (TST) with Graves' disease. PATIENTS AND METHOD: A retrospective study was performed in 128 patients. They were 23 males and 105 females with a median age of 34 years (range: 14-68). Weight of remnant tissue was between 4 and 5 g. Thyroid functional status was evaluated, at 3 months and after a follow-up period ranged from 1 to 5 years, by measurement of serum concentration of free T4 and/or free T3 and TSH. RESULTS: They were no post-operative death. Surgical complications were 2 vocal cord palsies and 17 hypocalcemia (inf. to 2 mmol/L). After a median follow-up of 2 years, they were no longer any cases of vocal cord dysfunction and no case of permanent hypoparathyroidism. Functional results were established in 118 patients: 46 patients had clinical hypothyroidism (39%), 64 patients had latent hypothyroidism or euthyroidism (54.2%), and 8 had recurrent hyperthyroidism (6.8%). CONCLUSION: These results suggest that TST with a remnant mass inferior to 5 g provides a low level of recurrent hyperthyroidism and allows to give no drug therapy to half patients. In our opinion, TST is still indicated in Graves' disease.     

Is lower extremity revascularization worthwhile in patients with end-stage renal disease?

BACKGROUND: The purpose of this study was to review the results of lower extremity revascularization in patients with end-stage renal disease and to determine in these patients the functional benefit and cost of an aggressive approach to limb preservation. METHODS: During a 5-year period at our institution, 33 bypass operations were performed on 31 limbs of 23 dialysis-dependent patients. Indications for revascularization were limited (18) or extensive (12) tissue loss or ischemia without tissue loss (3). Procedures included aortobifemoral bypass (1), femoropopliteal bypass (10), and femorotibial/pedal bypass (22). A digital or transmetatarsal amputation was performed in 57% of limbs. RESULTS: The 30-day primary patency was 100%. Cumulative primary and secondary patency rates at 2 years were 65% and 79%, respectively. Limb salvage was 67% and 59% at 1 and 2 years, respectively. Patient survival was poor (47% at 2 years). Peritoneal dialysis was predictive of poor survival (P <.001). Four of 5 patients on peritoneal dialysis died within 3 months of intervention. Extensive tissue loss was predictive of a diminished rate of limb salvage (P =.027). Only 39% of limbs with extensive tissue loss were salvaged at 1 year compared with 78% and 100% of limbs with limited and no tissue loss, respectively. The average hospital cost was $44,308 per year of limb salvage. CONCLUSIONS: Although revascularization of ischemic limbs in dialysis patients can be achieved with an excellent initial graft patency and reasonable limb salvage, patient survival is poor and costs are high. A selective approach to revascularization in these complicated patients may be indicated. For patients treated with peritoneal dialysis and for those with extensive tissue loss, primary amputation may be the preferred approach.     

Mesangial IgA deposition in minimal change nephrotic syndrome: coincidence of different entities or variant of minimal change disease?

BACKGROUND: Mesangial deposition of IgA (MCA) is a very rare finding in minimal change disease and has previously been considered a pure coincidence. In the U.S. and Europe only anecdotal case reports exist. To date, there has been no consensus on nomenclature and categorization of this entity. We describe 2 cases of MCA with analogue histological findings but relevant differences in clinical presentation, and we discuss the clinical implications of mesangial IgA deposition in minimal change nephrotic syndrome. PATIENTS: A 47-year-old female was admitted to hospital with nephrotic syndrome, microscopic hematuria, arterial hypertension and slight impairment of renal function 3 weeks after an unspecific upper airway infection. A 42-year-old male presented with nephrotic syndrome, microscopic hematuria, normotension and normal renal function. Both of the nephrotic syndromes were steroid-responsive and steroid-dependent. FINDINGS: The clinical presentation of the male patient was consistent with the features of minimal change glomerulopathy, whereas the female patient combined signs of minimal change disease and IgA nephropathy. Light microscopy revealed mesangial IgA immune deposits and slight mesangial hypercellularity. Electron microscopic studies of MCA patients disclose diffuse effacement of glomerular foot processes. CONCLUSION: Our cases and a review of the literature indicate that the histological diagnosis of MCA may comprise different pathogenetic entities. From the clinical point of view, MCA has to be regarded as a minimal change nephrotic syndrome with symptomatic or asymptomatic mesangial IgA deposition. IgA deposition constitutes a risk factor for impairment of renal function and indicates a frequently relapsing course.     

Is deep dorsal vein arterialization effective in elderly patients?

Objective Arterialization operations of the penis are recommended for young patients with erectile dysfunction (ED). In this study, we investigated the efficiency of deep dorsal vein arterialization (DDVA) in carefully selected healthy elderly patients Patients and methods An initial extensive evaluation with corpus cavernosum electromyography, cavernosometry, and penile doppler ultrasonography was performed for 43 elderly patients with ED for whom the presence of risk factors (hypertension, diabetes, hyperlipidemia, smoking habit, psychiatric or neurologic disorders, liver or kidney failure, and history of major trauma) had been ruled out. All patients underwent to DDVA using the Furlow–Fisher technique. Surgical outcome was tested postoperatively by use of the fifteen-item International Index of Erectile Function questionnaire (IIEF-15). Surgical success was assumed if the score in the five-item version of the IIEF (IIEF-5) had increased by at least five points. Results Of the patients, 21 were detected to have caverno-occlusive disease, 13 had arteriogenic disease, and 9 had both caverno-occlusive and arteriogenic disease. The mean age of the patients was 59.7 ± 4.6 years and the mean follow-up time was 22.1 ± 7.1 months. The operation was successful in 26 cases (60.5%) according to IIEF-5. Total IIEF-15 score was increased from 19.2 ± 5.0 to 28.5 ± 9.4 (P < 0.05). Significant increases were observed in the erectile function, intercourse satisfaction, and overall satisfaction domains of IIEF-15. The preoperative degree of ED or the etiology of ED had no impact on the surgical results. Percent changes in the total IIEF-15 score and in its domains were no different between patients aged <60 and those aged ≥60. Conclusion DDVA could successfully be performed for carefully selected elderly patients as long as the presence of risk factors for ED are ruled out.     

Do C1q or IgM nephropathies predict disease severity in children with minimal change nephrotic syndrome?

Background

It has been suggested that C1q and immunoglobulin M (IgM) nephropathy are variants of minimal change nephrotic syndrome (MCNS). Many researchers believe that these two conditions signify a worse prognosis for children with MCNS in comparison with immunofluorescence (IF)-negative MCNS. The aim of our study was to determine the prognostic significance of C1q nephropathy and IgM nephropathy in children with MCNS.

Methods

Fifty-five children with MCNS who had been biopsied over the course of 24 years at our institution were retrospectively categorized into three groups on the basis of IF microscopy findings: IF-negative MCNS (29/55 patients), MCNS with IgM nephropathy (19/55 patients), and MCNS with C1q nephropathy (7/55 patients). Clinical characteristics at disease presentation, clinical course, and renal outcome were compared between groups during the median follow-up period of 16.9 years (minimum 1.0, maximum 31.1 years).

Results

No statistically significant differences in clinical characteristics at disease presentation, clinical course, and renal outcome were found. Children with IgM nephropathy, C1q nephropathy, and IF-negative MCNS were clinically indistinguishable.

Conclusions

We concluded that C1q or IgM nephropathy variants do not seem to signify a worse prognosis in children with MCNS in comparison with IF-negative MCNS.     

Intravenous pulse cyclophosphamide—is it effective in children with steroid-resistant nephrotic syndrome?

Treatment of steroid-resistant nephrotic syndrome (SRNS) remains a challenge to pediatric nephrologists. Recently, intravenous cyclophosphamide (IV-CPM) infusion was shown to be effective, safe, and economical for the treatment of SRNS, particularly minimal change disease (MCD), as it results in more sustained remissions, longer periods without proteinuria, and fewer significant side effects at a lower cumulative dose. A prospective study was conducted to evaluate IV-CPM infusions in the management of children with SRNS secondary to MCD or IgM nephropathy. Five patients with SRNS (4 IgM nephropathy and 1 MCD) received six monthly IV-CPM infusions at a dose of 500 mg/m². No patient achieved complete or sustained remission. Three patients attained partial remission, which was not sustained for more than 1 month post therapy. One patient progressed rapidly to end-stage renal disease during treatment. Side effects included vomiting in four patients and alopecia in one patient. Conclusion: IV-CPM pulse therapy at a dose of 500 mg/m² is unsuccessful in obtaining complete or sustained remission in children with SRNS secondary to IGM nephropathy or MCD. Further randomized controlled studies with higher doses are required.     

Diabetes treatment in patients with renal disease: Is the landscape clear enough?

Diabetes is the most important risk factors for chronic kidney disease(CKD). The risk of CKD attributable to diabetes continues to rise worldwide. Diabetic patients with CKD need complicated treatment for their metabolic disorders as well as for related comorbidities. They have to treat, often intensively, hypertension, dyslipidaemia, bone disease, anaemia, and frequently established cardiovascular disease. The treatment of hypoglycaemia in diabetic persons with CKD must tie their individual goals of glycaemia(usually less tight glycaemic control) and knowledge on the pharmacokinetics and pharmacodynamics of drugs available to a person with kidney disease. The problem is complicated from the fact that in many efficacy studies patients with CKD are excluded so data of safety and efficacy for these patients are missing. This results in fear of use by lack of evidence. Metformin is globally accepted as the first choice in practically all therapeutic algorithms for diabetic subjects. The advantages of metformin are low risk of hypoglycaemia, modest weight loss, effectiveness and low cost. Data of UKPDS indicate that treatment based on metformin results in less total as well cardiovascular mortality. Metformin remains the drug of choice for patients with diabetes and CKD provided that their estimate Glomerular Filtration Rate(eGFR) remains above 30 mL/min per square meter. For diabetic patients with eGFR between 30-60 mL/min per square meter more frequent monitoring of renal function and dose reduction of metformin is needed. The use of sulfonylureas, glinides and insulin carry a higher risk of hypoglycemia in these patients and must be very careful. Lower doses and slower titration of the dose is needed. Is better to avoid sulfonylureas with active hepatic metabolites, which are renally excreted. Very useful drugs for this group of patients emerge dipeptidyl peptidase 4 inhibitors. These drugs do not cause hypoglycemia and most of them(linagliptin is an exception) require dose reduction in various stages of renal disease.     

Rituximab: effective treatment for severe steroid-dependent minimal change nephrotic syndrome?

Sir, Minimal change disease (MCNS) accounts for 70–90% of thecases of idiopathic nephrotic syndrome in children. Most patientsrespond to steroid therapy. However, the relapse rate is highand 50% of patients are steroid-dependent or frequent-relapsers[1]. These patients are often treated with other immunosuppressiveagents, such as cyclophosphamide or ciclosporin (CsA). Ultimately75% of these patients will develop a long-lasting remission.However, 25% of patients will experience a relapsing courseof the disease and many of them need immunosuppressive maintenance     

Is laparoscopic antireflux surgery safe and effective in obese patients?

Background  

It is not clear whether obesity has any negative impact on the results of laparoscopic antireflux surgery (LARS). In this prospective study we investigated the effect of body mass index (BMI) on the surgical outcome of LARS.