Retroperitoneal Fibrosis Is Still an Underdiagnosed Entity with Poor Prognosis.

BACKGROUND: Retroperitoneal fibrosis (RPF) is a rare disease characterized by the presence of inflammatory and fibrous retroperitoneal tissue that often encircles abdominal organs including the aorta and ureters. Data on the incidence of this disease are limited. SUMMARY: The disease may be idiopathic or secondary to infections, malignancies, drugs, or radiotherapy. The idiopathic form is an immune-mediated entity and a part of the broader spectrum of idiopathic diseases termed chronic periaortitis, characterized by a morphologically similar fibroinflammatory changes in the aorta and surrounding tissues. Taking into account the dominant symptoms and clinical characteristics of patients with periaortitis, 2 subtypes of disease could be distinguished. The vascular subtype includes patients with nondilated aorta or with inflammatory abdominal aortic aneurysm, both with and without involvement of adjacent structures and with numerous risk factors for atherosclerosis. In the renoureteral subtype, obstructive uropathy manifesting with hydronephrosis and acute kidney injury is the predominant finding. Due to the variety of symptoms, diagnosis of RPF remains challenging, difficult, and often delayed. A series of diagnostic tests should be performed, in order to confirm the diagnosis idiopathic RPF. Laboratory workup includes evaluation of inflammatory indices and immunological studies. A biopsy and histopathological evaluation may be necessary to confirm diagnosis and differentiate the disease. Computed tomography, magnetic resonance imaging, and positron emission tomography are the modalities of choice for the diagnosis and follow-up of this disease. Management of ureteral obstruction, hydronephrosis, and aortic aneurysms often requires surgical evaluation and treatment. The pharmacological treatment of RPF has been evaluated in a few randomized trials and is mainly based on observational studies. Steroid therapy remains the gold standard of treatment. KEY MESSAGES: Nowadays, multidisciplinary team approach with clinical and diagnostic experience in both primary and secondary RPF as well as 2 major subtypes should be offered. Centers specialized in rare diseases with collaboration with other units and referral system yield the best possible outcomes.

Acid-base balance in hemodialysis patients in everyday practice.

INTRODUCTION: Abnormalities in blood bicarbonates (HCO3-) concentration are a common finding in patients with chronic kidney disease, especially at the end-stage renal failure. Initiating of hemodialysis does not completely solve this problem. The recommendations only formulate the target concentration of ≥22 mmol/L before hemodialysis but do not guide how to achieve it. The aim of the study was to assess the acid-base balance in everyday practice, the effect of hemodialysis session and possible correlations with clinical and biochemical parameters in stable hemodialysis patients. MATERIAL AND METHODS: We enrolled 75 stable hemodialysis patients (mean age 65.5 years, 34 women), from a single Department of Nephrology. We assessed blood pressure, and acid-base balance parameters before and after mid-week hemodialysis session. RESULTS: We found significant differences in pH, HCO3- pCO2, lactate before and after HD session in whole group (p < 0.001; p < 0.001; p < 0.001; p = 0.001, respectively). Buffer bicarbonate concentration had only statistically significant effect on the bicarbonate concentration after dialysis (p < 0.001). Both pre-HD acid-base parameters and post-HD pH were independent from buffer bicarbonate content. We observed significant inverse correlations between change in the serum bicarbonates and only two parameters: pH and HCO3- before hemodialysis (p = 0.013; p < 0.001, respectively). CONCLUSIONS: Despite the improvement in hemodialysis techniques, acid-base balance still remains a challenge. The individual selection of bicarbonate in bath, based on previous single tests, does not improve permanently the acid-base balance in the population of hemodialysis patients. New guidelines how to correct acid-base disorders in hemodialysis patients are needed to have less 'acidotic' patients before hemodialysis and less 'alkalotic' patients after the session.

Retroperitoneal fibrosis, a rare entity with urorenal and vascular subtypes - preliminary data.

INTRODUCTION: Retroperitoneal fibrosis (RPF) is a rare disease associated with the formation of hard inflammatory and fibrous tissue in the retroperitoneum. Taking into consideration the fact that RPF is a rare disease with different subtypes, we compared the basal clinical and biochemical characteristics of the vascular and urorenal subtypes. PATIENTS AND METHODS: From January 2005 until December 2021, 27 patients were identified as vascular subtype (18 males) and 11 as urorenal subtype (9 males). RESULTS: Patients with a primary urorenal origin had significantly worse kidney function as reflected by serum creatinine and eGFR (both p < 0.001); they also had higher serum cholesterol (p < 0.01). Hypertension, diabetes, hyperlipidemia and nicotinism were significantly more prevalent in vascular subtype (all p < 0.001). CONCLUSION: Vascular subtype is more prevalent in our study with more cardiovascular risk factor present. Due to the diversity of symptoms, diagnosis of RPF becomes a challenge for specialists as well as therapy.

CANCER IN DIALYSIS PATIENTS.

Cancer in dialysis patients is a common problem and is one of the most common reasons of mordibity and mortality in developed countries. An impaired renal function leads to the accumulation in the blood products of nitrogen transformation, which negatively affect organ function, especially the immune system. The Standardized Cancer Incidence is higher in patients with end-stage kidney failure than in the general population and reaches 1,18-1,42. The cancer risk is three times higher in dialysis patients over age 65 years and is more common among hemodialysis than peritoneal dialysis patients. The most common type of cancer occurring in this group of patients is urinary tract cancer which often develops on the basis of acquired cysts and in patients previously treated with cyclophosphamide. Nonetheless, patients with kidney problems are not regularly tested for these diseases and the only group that is screened for cancer are patients tested for kidney transplantation. Some problems in this topic are briefly presented in this article.

[Asymptomatic bacteriuria in pregnancy].

Bacteriuria is a presence of bacteria in urine. It may remain asymptomatic or elicit acute, symptomatic urinary tract infection. In pregnancy, due to physiological changes in the woman organism it increases risk of serious infectious and obstetric complications and therefore requires a different approach than in general population.

[Common errors in management of urinary tract infections].

Urinary tract infections seems to be simple diseases. However, inappropriate diagnostic and therapeutic procedures may lead to serious complications such as systemic infection, while on the other hand contribute to increasing antimicrobial resistance of uropathogens which is a growing threat to global public health. Due to more frequent occurrence of multidrug resistance bacteria, popularization of guidelines and correct schemes among doctors of all specialities. In the following article the commonest errors made by doctors in their clinical practice that seem to cause a lot of problems are shortly reviewed.

[Complement-mediated hemolytic uremic syndrome: a diagnostic approach].

Complement-mediated hemolytic uremic syndrome (a-HUS), an uncommon variant of thrombotic microangiopathy, is characterized by hemolytic anemia, thrombocytopenia and renal impairment. This disorder might be inherited or/and acquired and leads to dysregulation of the alternative complement pathway at the endothelial cell surface and formation of microvascular thrombi. The differential diagnosis includes other forms of hemolytic syndrome (eg. Shiga-toxin-producing E.coli or S. dysenteriae -associated HUS - STEC-HUS), thrombotic thrombocytopenic purpura (TTP) and congenital errors of vitamin B12 metabolism. The diagnostic approach is presented below.

[Diagnostic problems in retroperinoneal fibrosis].

Retroperitoneal fibrosis (RPF) is a uncommon disease characterized by the presence of inflammatory and fibrous retroperitoneal tissue that often encircles the ureters or abdominal organs. The disease may be idiopathic or secondary to infections, malignancies, certain drugs or radiotherapy. Idiopathic form is an immune-mediated entity and a part of the broader spectrum of idiopathic diseases termed chronic periaortitis, characterized by a morphologically similar fibroinflammatory changes in aorta. In the article the most important diagnostic problems of RPF are reviewed.

Clinicopathologic correlations of renal pathology in the adult population of Poland.

BACKGROUND: This is the first report on the epidemiology of biopsy-proven kidney diseases in Poland. METHODS: The Polish Registry of Renal Biopsies has collected information on all (n = 9394) native renal biopsies performed in Poland from 2009 to 2014. Patients' clinical data collected at the time of biopsy, and histopathological diagnoses were used for epidemiological and clinicopathologic analysis. RESULTS: There was a gradual increase in the number of native renal biopsies performed per million people (PMP) per year in Poland in 2009-14, starting from 36 PMP in 2009 to 44 PMP in 2014. A considerable variability between provinces in the mean number of biopsies performed in the period covered was found, ranging from 5 to 77 PMP/year. The most common renal biopsy diagnoses in adults were immunoglobulin A nephropathy (IgAN) (20%), focal segmental glomerulosclerosis (FSGS) (15%) and membranous glomerulonephritis (MGN) (11%), whereas in children, minimal change disease (22%), IgAN (20%) and FSGS (10%) were dominant. Due to insufficient data on the paediatric population, the clinicopathologic analysis was limited to patients ≥18 years of age. At the time of renal biopsy, the majority of adult patients presented nephrotic-range proteinuria (45.2%), followed by urinary abnormalities (38.3%), nephritic syndrome (13.8%) and isolated haematuria (1.7%). Among nephrotic patients, primary glomerulopathies dominated (67.6% in those 18-64 years of age and 62.4% in elderly patients) with leading diagnoses being MGN (17.1%), FSGS (16.2%) and IgAN (13.0%) in the younger cohort and MGN (23.5%), amyloidosis (18.8%) and FSGS (16.8%) in the elderly cohort. Among nephritic patients 18-64 years of age, the majority (55.9%) suffered from primary glomerulopathies, with a predominance of IgAN (31.3%), FSGS (12.7%) and crescentic GN (CGN) (11.1%). Among elderly nephritic patients, primary and secondary glomerulopathies were equally common (41.9% each) and pauci-immune GN (24.7%), CGN (20.4%) and IgAN (14.0%) were predominant. In both adult cohorts, urinary abnormalities were mostly related to primary glomerulopathies (66.8% in younger and 50% in elderly patients) and the leading diagnoses were IgAN (31.4%), FSGS (15.9%), lupus nephritis (10.7%) and FSGS (19.2%), MGN (15.1%) and pauci-immune GN (12.3%), respectively. There were significant differences in clinical characteristics and renal biopsy findings between male and female adult patients. CONCLUSIONS: The registry data focused new light on the epidemiology of kidney diseases in Poland. These data should be used in future follow-up and prospective studies.

[MGRS - monoclonal gammopathy of renal significance].

MGRS represents a group of kidney disorders caused by monoclonal gammapathy that is secreted by B cell or plasma cell clone. Kidney biopsy is required in the diagnosis, in the treatment - chemotherapy. When the hematology remision is reached, kidney remision is possible. In case of end-stage kidney disease despite hematologic remission - kidney transplantation may be considered.

[Spectrum of monoclonal gammopathies].

The monoclonal gammopathies are defined as heterogenous group of diseases characterized by proliferation of a single clone of plasma cells, producing immunoglobulin or light (rarely heavy) chains, which can be detected in blood or urine as monoclonal (M) protein. The most common among them is monoclonal gammopathy of undetermined significance (MGUS), the asymptomatic benign disorder, present in ~ 3% of the population aged ≥50 years. However MGUS is a pre malignant condition and may progress to symptomatic multiple myeloma or related malignancies, with annual rate of approximately 1%. The clone may also produce kidney damage, resulting from just the protein M, with different patterns of renal disease. Since the lesions are progressive and may be severe leading to a significant morbidity the term "monoclonal gammopathy of renal significance (MGRS)" has been recently introduced.

[Systemic lupus erythematosus - news 2017].

Systemic lupus erythematosus is a chronic autoimmune disease with diverse manifestations and clinical course, required multidisciplinary treatment. The etiology, pathogenesis and optimal treatment are still unknown. There are two summaries on SLE, published in 2017, presented below.

Kidney disease in the elderly: biopsy based data from 14 renal centers in Poland.

BACKGROUND: Longer life expectancy is associated with an increasing prevalence of kidney disease. Aging itself may cause renal damage, but the spectrum of kidney disorders that affect elderly patients is diverse. Few studies, mostly form US, Asia and West Europe found differences in the prevalence of some types of kidney diseases between elderly and younger patients based on renal biopsy findings, with varied proportion between glomerulopathies and arterionephrosclerosis as a dominant injury found. Here, for the first time in Eastern Europe we analyzed native kidney biopsy findings and their relationship to clinical characteristics at the time of biopsy in elderly individuals (aged ≥65) in comparison to younger adults (aged 18-64). METHODS: Biopsy and clinical data from 352 patients aged ≥65 were retrospectively identified, analyzed and compared with a control group of 2214 individuals aged 18-64. All kidney biopsies studied were examined at Medical University of Warsaw in years 2009-14. RESULTS: In elderly patients the leading indication for biopsy was nephrotic range proteinuria without hematuria (34.2%) and the most prevalent pathologic diagnoses were: membranous glomerulonephritis (MGN) (18.2%), focal segmental glomerulosclerosis (FSGS) (17.3%) amyloidosis (13.9%) and pauci immune glomerulonephritis (12.8%). Hypertension and age-related lesions very rarely were found an exclusive or dominant finding in a kidney biopsy (1.7%) and a cause of proteinuria (1.1%) in elderly individuals. There were 18.2% diabetics among elderly individuals, and as much as 75% of them had no morphologic signs of diabetic kidney disease in the renal biopsy. Amyloidosis, MGN, pauci immune GN, crescentic GN and light and/or heavy chain deposition disease (LCDD/HCDD) were more frequent whereas IgA nephropathy (IgAN), lupus nephritis (LN) and thin basement membrane disease (TBMD) were less common among elderly than in younger patients. CONCLUSIONS: Proteinuria, a dominating manifestation in elderly patients subjected to kidney biopsy was most commonly related to glomerulopathies. The relatively high prevalence of potentially curative kidney diseases in elderly individuals implicates the importance of renal biopsy in these patients.

[IgA nephropathy - prognostic factors and treatment].

IgA nephropathy is the most common cause of primary glomerolunephritis. Patients who have isolated hematuria, little or no proteinuria (<0,5 g/d) and present with normal renal function usually have a low risk of progression. In patients with greater proteinuria and/or an elevation in the serum creatinine concentration, different rate of progression to end-stage renal disease has been observed. The approach to the management consists of nephroprotective therapies based on renin-angiotensin-aldosterone system blockade, which aim to slow progression of kidney damage, and, in some patients, immunosuppression.

[IgA vasculitis (Henoch-Schönlein purpura)].

IgA vasculitis (Schönlein-Henoch purpura) is a systemic inflammation of the small vessels associated with the deposition of IgA antibodies in the vascular wall. Typical clinical symptoms are: skin lesions (purpura), joint pain, abdominal discomfort and renal disorder (most common haematuria/proteinuria). The disease affects usually the pediatric population; in those patients its course is benign and usually not associated with permanent complications. However, adults often present severe symptoms of organ damage, and the risk of complications, especially chronic renal failure is high. The diagnosis is based on clinical signs and skin biopsy. In mild cases the treatment is symptomatic. In cases with severe clinical symptoms corticosteroids are the basis of the therapy.

[Kidney disease in HIV-infected patient].

In the developed countries with an access to highly active antiretroviral therapy (HAART), the spectrum of renal complications observed in patients infected with HIV has shifted from HIV-associated kidney diseases to medications-related nephrotoxicities. In this article all types of these disorders, ranging from HIV-associated nephropathy (HIVAN), immune mediated glomerulopathies (HIVICK), and thrombotic microangiopathies to induced by HAART tubulopathies, acute toxic tubular necrosis, acute interstitial nephritis, crystal nephropathy, and chronic kidney disease have are shortly reviewed.

[Nephrological problems in pneumonology - pulmonary renal syndrome]. / Problemy nefrologiczne na oddziale pneumologii - zespól plucno-nerkowy.

Pulmonary-renal syndrome is rare disease. The term describes acute syndrome is ANCA associated vasculitis. The patients need aggressive therapy (induction and maintenance phase), in some cases accompanied by plasmapheresis. They often have respiratory failure and require dialysis because of acute kidney injury.

[Renal dysfunction in liver disease]. / Zaburzenia czynnosci nerek w chorobach watroby.

In patients with liver disease various acute and chronic kidney disorders may occur, the most severe being a hepatorenal syndrome. It is usually found in patients with decompensated cirrhosis and the only effective treatment is liver transplantation. The other common causes of renal dysfunction are: prerenal (dehydration, hypovolemia) and renal (ischemia or nephrotoxicity) acute kidney injury. The most frequent chronic nephropathies are: glomerulonephritis and mixed cryoglobinemia due to viral hepatitis C and B. Finally, chronic liver disease has been associated with an increased risk of diabetic nephropathy.

[Renovascular hypertension]. / Nadcisnienie naczyniowo-nerkowe.

Renovascular hypertension (RVH) is one of the most common causes of secondary hypertension. In about 90% of cases it is due to atherosclerotic renal artery stenosis, often accompanied by severe occlusive disease in the other vessels, and as such carries a bad prognosis. In the remaining 10% patients (usually young women) the underlying vascular lesion is fibromuscular dysplasia. A presence of RVH should be suspected in patients with severe or resistant hypertension, sudden decline of renal function, sudden development or worsening of hypertension, flash pulmonary edema, impairment of renal function after treatment with renal-angiotensin-aldosterone system (RAAS) antagonists. Those patients should be screened with Doppler ultrasound, followed by computer tomography or magnetic resonance angiography. In most cases intensive and well-controlled medical treatment with RAAS blockers, aldosterone and/or calcium antagonists, with an addition of statins and platelet-inhibiting drugs is succesful. However in a selected cases renal revascularization may be necessary.

[Anemia in chronic kidney disease--reasons and treatment]. / Przyczyny i leczenie niedokrwistosci w przewleklej chorobie nerek.

Anemia in chronic kidney disease (CKD) may affect up to 90% of the patients. It is one of the non typical risk factors of cardiovascular disease, specific for this population. The main reasons of the anemia in CKD are iron and erythropoietin deficiency. It is recognized in women with hemoglobin concentration < 11 g/dl and in men and postmenopausal women with hemoglobin concentration < 12 g/dl. Other potentially reversible reasons of anemia should be excluded in differential diagnosis. Iron and erythropoiesis stimulating agents (ESA) constitute the main treatment of anemia of CKD.