Treatment problem of anephric patients with Wilms tumor

Bilateral nephroblastoma is rare and is about 5 % of the total number of patients. The modern strategy of treatment children with bilateral Wilms tumor requires a comprehensive approach. An important task is to preserve as much of the renal parenchyma as possible, which will be sufficient for normal kidney function and development of the child. However, in subtotal kidney damage organ-preserving treatment is not always possible. In this case, the only curative option is bilateral nephrectomy followed by continuous renal replacement therapy. To date, the literature describes isolated cases of adjuvant chemotherapy in children on peritoneal dialysis. The article presents a clinical case of specific therapy with topotecan for a child after bilateral nephrectomy, a carrier of peritoneal dialysis catheter on the background of progression of the underlying disease.

Long-term clinical spectrum and circulating RAS evaluation of anephric patients undergoing hemodialysis: A report of four cases and literature review

Introduction: Bilateral nephrectomy leads to a short-term reduction in blood pressure. This is mainly due to a sharp change in the circulating renin-angiotensin system (RAS), but data on the long-term outcomes of their clinical status and further changes in circulating RAS are rare. Materials and methods: We enrolled four Chinese patients who had both of their kidneys removed two (1), six (1) and eight (2) years prior to this study, respectively. Their clinical data were collected retrospectively and circulating RAS was evaluated by radioimmunoassay. Results: Hypotension after surgery occurred in two patients who suffered thrombosis of the arteriovenous fistula, but no life-threatening complications occurred. The average hemoglobin level was 103.3±12.3 g/l. Two patients without hemorrhage received intravenous erythropoietin (EPO) of 4500–8000 iu/week. Extremely low plasma renin activity (PRA) of 0.08±0.03 ng/ml (normal range 0.93–6.56 ng/ml) showed in the patients. Surprisingly, plasma angiotensin II concentration (71.37±8.28 pg/ml) and aldosterone level (0.17±0.02 ng/mlng/ml) were within the normal range. Conclusions: The four anephric individuals did not suffer life-threatening complications while their hypotension gradually subsided and their EPO dosage was relatively low. Although their PRA level was extremely low, they produced normal levels of angiotensin II and aldosterone in plasma, which indicates the kidney-independent mechanism of angiotensin II production likely compensated in the long term.

Immunoglobulin Free Light Chain Dimers in Human Diseases

Immunoglobulin free light chain (FLC) kappa (κ) and lambda (λ) isotypes exist mainly in monomeric and dimeric forms. Under pathological conditions, the level of FLCs as well as the structure of monomeric and dimeric FLCs and their dimerization properties might be significantly altered. The abnormally high fractions of dimeric FLCs were demonstrated in the serum of patients with multiple myeloma (MM) and primary systemic amyloidosis (AL), as well as in the serum of anephric patients. The presence of tetra- and trimolecular complexes formed due to dimer-dimer and dimer-monomer interactions was detected in the myeloma serum. Analysis of the amyloidogenic light chains demonstrated mutations within the dimer interface, thus raising the possibility that these mutations are responsible for amyloidogenicity. Increased κ monomer and dimer levels, as well as a high κ/λ monomer ratio, were typically found in the cerebrospinal fluid from patients with multiple sclerosis (MS). In many MS cases, the elevation of κ FLCs was accompanied by an abnormally high proportion of λ dimers. This review focuses on the disease-related changes of the structure and level of dimeric FLCs, and raises the questions regarding their formation, function, and role in the pathogenesis and diagnosis of human diseases.