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Kronična bubrežna bolest (KBB) sistemska je bolest povezana s nizom komplikacija koje naposljetku dovode do povećanog pobolijevanja i smrtnosti bolesnika. Poremećaj koštano-mineralnog metabolizma (CKD-MBD) počinje u ranim stadijima KBB-a nakupljanjem fosfora u organizmu s posljedičnim pokretanjem niza zbivanja koja rezultiraju razvojem sekundarnog hiperparatiroidizma s promjenama na kostima i izvankoštanim tkivima. Najvažnija i klinički najbitnija posljedica CKD-MBD-a jesu vaskularne kalcifikacije koje pridonose povećanju smrtnosti od srčano-krvožilnih bolesti. Potrebno je prepoznati bolesnike s povećanim rizikom od razvoja CKD-MBD-a i terapijski intervenirati. Prevencija je najbolji ­pristup zbrinjavanju bolesnika. Prvi korak u liječenju jest nutritivno savjetovanje uz nadoknadu vitamina i ispravljanje poremećaja minerala. Napredovanjem KBB-a prema završnom stadiju povećava se potreba za medikamentnom terapijom. Istodobno treba liječiti i ostale probleme poput anemije i acidoze. Potrebno je pravodobno započeti s nadomještanjem bubrežne funkcije uz osiguranje odgovarajuće doze dijalize. Idealno je omogućiti preemptivnu transplantaciju u bolesnika koji nemaju kontraindikacija za primjenu imunosupresijske terapije., Chronic kidney disease (CKD) is a systemic disease with numerous complications associated with increased morbidity and mortality. Chronic kidney disease-metabolic bone disease (CKD-MBD) starts at early stages of CKD with phosphorus accumulation and consequent initiation of numerous events that result with the development of secondary ­hyperparathyroidism with changes on bones and extraskeletal tissues. The most important and clinically most relevant consequences of CKD-MBD are vascular calcifications which contribute to cardiovascular mortality. Patients with the ­increased risk for the development of CKD-MBD should be recognized and treated. Prevention is the most important therapeutic option. The first step should be nutritional counseling with vitamin supplementation if necessary and correction of mineral status. Progression of CKD requires more intensive medicamentous treatment with the additional correction of metabolic acidosis and anemia. Renal replacement therapy should be timely initiated, with the adequate dose of dialysis. Ideally, preemptive renal transplantation should be offered in individuals without contraindications for immunosuppressive therapy

Fabryjeva bolest (Anderson-Fabryjeva bolest) jedna je od najčešćih lizosomskih bolesti nakupljanja (nakon Gaucherove bolesti) uzrokovana smanjenom aktivnošću enzima α-galaktosidaze A (α -Gal A) uz posljedično nakupljanje globotriaozilceramida u različitim stanicama, ponajprije u endotelnim i vaskularnim glatkim mišićnim stanicama uz posljedične multisistemske manifestacije. Pojavnost bolesti u muškaraca je 1:40.000-60.000, dok je u općoj populaciji oko 1 : 117.000. Bol je najčešće prvi simptom bolesti u 60-80 % djece, kao i simptomi probavnog sustava, oftalmološki simptomi, gubitak sluha. Smanjenje bubrežne funkcije, hipertrofična miokardiopatija ili moždani udar mogu se iskazati kao izolirani simptomi bolesti. U bolesnika s Fabryjevom bolešću skraćuje se očekivani životni vijek i to u muškaraca za oko 20 godina, a u žena za 10-15 godina, pa je stoga uvođenje nadomjesne enzimske terapije nužno u svih bolesnika koji zadovoljavaju kriterije za terapiju ove bolesti bez obzira na dob i spol., Fabry disease (Anderson-Fabry disease) is one of the most common lysosomal storage diseases (after Gaucher disease) caused by deficient activity of the α-galactosidase A (α-Gal A) enzyme, which leads to progressive accumulation of globotriaosylceramide in various cells, predominantly in endothelium and vascular smooth muscles, with multisystem clinical manifestations. Estimates of the incidence range from one per 40,000 to 60,000 in males, and 1:117,000 in the general population. Pain is usually the first symptom and is present in 60%-80% of affected children, as well as gastrointestinal disturbances, ophthalmologic abnormalities and hearing loss. Renal failure, hypertrophic cardiomyopathy, or stroke as the presenting symptom may also be found even as isolated symptoms of the disease. Life expectancy is reduced by approximately 20 years in males and 10-15 years in females, therefore enzyme replacement therapy should be introduced in patients of any age and either sex, who meet treatment criteria for Anderson-Fabry disease.

The increasing number of possible recipients for kidney transplantation and relatively unchanged number of organ donors has led to consideration of alternative strategies and expansion of deceased donor criteria in order to expand donor pool. Previously, kidneys from expanded criteria donors (ECD) were strongly underestimated because of the conventional opinion suggesting these kidneys to have a higher rate of preservation injury, delayed graft function, rejection and nonfunction. Reducing the difference between graft outcome in patients transplanted from ECD and standard criteria donor (SCD) is one of the goals of many respectable kidney transplantation centers. This assignment includes major concern about reduction of cold ischemia time, recipient selection, novel and adapted immunosuppressive regimens, increased nephron mass by dual kidney transplantation, and using histologic criteria for marginal donor graft selection. There are not many reports on the outcome of kidneys transplanted from donors with acute renal failure and high terminal creatinine. This review presents the exact definition of marginal donor, especially donor with acute renal failure. The management of such grafts during preimplantation and implantation period, outcomes and post-transplantation care are the main assignments for transplantation teams. Recipients of such grafts should be well informed about the possibilities and potential complications and sign their informed consent thereafter. Some respectable studies have shown that under certain, highly controlled conditions, these kidneys can be used safely, with excellent short- and long-term outcomes.

Hrvatske konsenzus konferencije o virusnim hepatitisima održane su 2005. i 2009. g. (1). S obzirom na brojne nove spoznaje o epidemiologiji, dijagnostici i liječenju virusnih hepatitisa (poglavito kroničnog hepatitisa C genotipa 1) u protekle četiri godine, 28. veljače 2013. g. održana je nova Hrvatska konsensus konferencija o virusnim hepatitisima u Zagrebu. Sažeti tekst ove Hrvatske konsenzus konferencije o virusnim hepatitisima sadrži prikaz novih spoznaja o epidemiologiji virusnih hepatitisa, serološkoj i molekularnoj dijagnostici virusnih hepatitisa, određivanju polimorfizma promotora gena za IL-28, procjeni stadija fibroze, algoritmu dijagnostičkog praćenja bolesnika, liječenju kroničnog hepatitisa C (genotipovi 1-6) i hepatitisa B, liječenju specijalnih populacija (djeca, bolesnici na dijalizi, bolesnici liječeni transplantacijom, osobe s HIV/HCV koinfekcijom) i nuspojavama liječenja.

Metabolic acidosis is an inevitable complication associated with progressive loss of kidney function. It appears when glomerular filtration rate (GFR) falls below 25 ml/min/ 1.73 m² . Metabolic acidosis arises from the difference between the excretion of hydrogen and the synthesis of ammonia ions. Damaged renal tubules cannot contribute in maintaining the acid base balance by reabsorbing the daily filtrated HCO3- and synthesizing new HCO3... ions. Although usually mild to moderate, it may lead to several metabolic complications. Metabolic acidosis is one of the important causes of protein energy wasting (PEW) and can trigger muscle loss in patients dealing with chronic kidney disease (CKD). Furthermore, it contributes to the development of chronic kidney disease-metabolic bone disease by increasing the bone resorption and inhibiting the bone formation. It has been shown that the acidic environment could play an important role in regulation of the hepcidin homeostasis and thus, be one of the factors contributing to the etiology of the anemia of chronic disease. It is also known that metabolic acidosis can severely affect kidney function by causing progressive tubulointerstitial injury and decline in glomerular filtration rate. All these metabolic effects are complex but can be successfully managed. Series of studies provide evidence that bicarbonate therapy is beneficial in these patients. It helps in achieving the balance between calcium and phosphate. Furthermore, it improves dietary protein intake and lean body mass and slows down the decline in glomerular filtration rate. Thus, metabolic acidosis needs to be monitored and carefully corrected.

A 21-year-old female patient was diagnosed with horseshoe kidney at the age of 10. She had been treated with peritoneal dialysis from 2005 to 2009, when she received kidney from a deceased donor. The posttransplant course was complicated by development of Pseudomonas aeruginosa and Candida sepsis. Reduced immunosuppression resulted in acute rejection, which demanded graphtectomy 2 months after transplantation. She restarted peritoneal dialysis for additional 2 years. In March 2011, she received her second transplant with excellent function. Nine months after the transplantation, she developed ascites, with early satiety and vomiting. MSCT revealed severe encapsulating sclerosing peritonitis. Her overall condition deteriorated, so she underwent adhesiolysis with resection of incarcerated terminal ileum. Due to acute allograft rejection, urgent graphtectomy was performed. Currently, she is receiving everolimus and dialysis successfully, with excellent overall status.

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Renal transplantation is the method of choice for renal replacement therapy in the majority of patients. Immunosuppressive drugs may increase the risk of developing malignancies. We present a case of a patient having undergone different renal replacement methods over 25 years. He had good graft function at 12 years of transplantation. Development of spontaneous retroperitoneal hematoma caused by rupture of the native kidney oncocytoma was complicated with sepsis and deterioration of graft function. We emphasize the importance of regular ultrasonography follow-up of native kidney, which is challenging because of fibrous changes.

Aging is a natural process that occurs in all tissues and organs resulting in a decreasing functional capacity. Aging of the population results in an increased number of elderly patients who require replacement of renal function. Renal transplantation is the method of choice for this group of patients if they have no contraindications for immunosuppressive therapy. The lack of donors is the main obstacle for renal transplantation. However, the use of organs from elderly donors for transplantation in elderly recipients is an appropriate method of renal replacement therapy in this group of patients.

Renal failure is a rare complication of hereditary coagulopathies. However, when it occurs, it rapidly progresses to a stage that requires replacement of renal function. Major problems include the choice of dialysis method, prevention of complications and supplementation of deficient factor. In hemodialysis, it is challenging to prevent system clotting and avoid bleeding. We present a case of polytraumatized male patient with hemophilia A, who developed compartment syndrome with acute renal failure. Continuous venovenous hemodialysis (CWHD) improved his condition and he recovered his kidney function. However, over the next few days he developed severe sepsis with deterioration of renal function. CWHDF (hemodiafiltration) was restarted. Several large hematomas were found in the abdominal cavity and in the inguinal region, one of them inducing compartment syndrome with leg necrosis. The patient died from cardiorespiratory arrest.

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The balance and quality of different renal replacement treatment modalities used in the elderly with end-stage renal disease vary between countries depending on economic resources, distribution of renal units, number of specialists, and patterns of reimbursement of both hospitals and physicians. Elderly patients with end-stage renal disease need detailed assessment of medical, psychological, motor, and social factors in order to choose an appropriate renal replacement treatment option. Presence or absence of significant comorbidity is much more important than the chronological age. The choice between hemodialysis and continuous ambulatory peritoneal dialysis is largely dependant on preferences of the local team and the patient. Patients with adequate cardiovascular systems are generally considered to be more suitable for hemodialysis. Hemoglobin should be optimized (Hb between 100 and 120 g/L) in all patients. Poor cardiac status and/or angina will require assessment, medical treatment, and, if necessary, surgical treatment or angioplasty. Transplantation should be considered in all reasonably fit and carefully selected patients older than 65 or even 70 years. Only the permanent shortage of suitable kidneys limits our ability to treat all those who could benefit from this type of treatment. Renal transplant recipients may benefit in terms of both survival and quality of life even if older than 70 years and beyond. Immunosuppressive therapy in elderly patients should be moderate.

Posljednjih nekoliko godina pojavio se pozitivan trend u liječenju bolesnika oboljelih od neuroimunoloških poremećaja intravenskom primjenom imunoglobulina. Unatoč učestaloj i širokoj upotrebi imunoglobulina intravenskim putem nema zadovoljavajućeg konsenzusa o njihovoj optimalnoj primjeni. U cilju definiranja intravenske upotrebe imunoglobulina u neurologiji temeljene na znanstvenim dokazima i u skladu sa smjernicama EFNS-a donesene su preporuke za konsenzus. Učinkovitost intravenske upotrebe imunoglobulina dokazana je u liječenju bolesnika s Guillain- Barréovim sindromom (stupanj A), kroničnom upalnom demijelinizirajućom polineuropatijom (stupanj A), multifokalnom mononeuropatijom (stupanj A), akutnom egzacerbacijom mijastenije gravis te kao kratkotrajna terapija u bolesnika s teškim oblikom mijastenije gravis (stupanj A). Kao druga linija liječenja intravenski imunoglobulini preporučuju se u liječenju bolesnika oboljelih od dermatomiozitisa, ali u kombinaciji s prednizonom (stupanj B) te u liječenju polimiozitisa. Kao druga ili treća linija liječenja intravenski imunoglobulini uzimaju se u obzir kod bolesnika s relapsno-remitirajućom multiplom sklerozom pri nepodnošenju konvencionalne imunomodulatorne terapije (stupanj B) te u relapsima za vrijeme trudnoće ili u postpartalnom razdoblju (rezultat dobre kliničke prakse). Naposljetku, doima se da bi upotreba intravenskih imunoglobulina također mogla imati povoljan učinak u liječenju sindroma ukočene osobe (stupanj A), nekih paraneoplastičnih neuropatija (stupanj B) i akutno demijelinizirajućih bolesti te refraktorne epilepsije u djetinjstvu (rezultat dobre kliničke prakse).

Bone morphogenetic proteins (BMPs) are a large group of growth factors. More than 20 members of BMP family have been identified to date. Based on their structural and functional properties, BMPs can be divided into 4 subgroups: BMP 2/4, BMP 5/6/7/8a/8b, BMP 9/10 and BMP 12/13/14. Each BMP has a unique structural feature that differentiates it from all other family members. BMPs take part in different stages of kidney development. For example, BMP-2 inhibits branching morphogenesis in ureteric buds ; BMP-4 is expressed in metanephritic mesenchyme along the Wolff canal before kidney development, its expression is also found in the mesenchyme surrounding the ureteric bud before the invasion of the mesenchymal cells in the ureteric stem and induction completion ; BMP-7 is the only bone morphogenetic protein that is crucial in kidney development. BMP family members are also included in maintaining normal kidney structure and function. Experimental models have shown that BMP- 7 prevents ischemia/reperfusion damage. Expression of BMP-7 is reduced in the samples of kidney tissue with diabetic nephropathy. Also, BMP-7 mRNA is reduced in clear cell kidney carcinomas, indicating its protective effects in maintaining normal kidney structure and function. Bone morphogenetic proteins are some of the key players in regulating normal kidney development, but their role also extends into maintaining normal kidney structure and function.

WNT 4 is a secreted glycoprotein that is critical for nephrogenesis during mesenchymal to epithelial transformation. Lately there are some experimental modles witch confirm a role of Wnt 4 during regeneration process in acute renal failure. On the other hand there are some evidence that Wnt 4 plays important role in renal fibrosis during experimental renal injury in rats that provide tubuloinerstitial fibrosis. When will Wnt 4 have a protective role or when will induce fibrosis still is not known and therefore futher studies will be necessary to gain a more precise understanding.

The use of intravenous immunoglobulin (IVIg) in the management of patients with neuroimmune disorders has shown a progressive trend over the last few years. Despite the wide use of IVIg, consensus on its optimal use is deficient. The European Federation of Neurological Societies (EFNS) guidance regulations offer consensus recommendations for optimal use of IVIg. The effectiveness of IVIg has been proven in Guillain- Barré syndrome (level A), chronic inflammatory demyelinating polyradiculoneuropathy (level A), multifocal mononeuropathy (level A), acute exacerbations of myasthenia gravis and short- term treatment of severe myasthenia gravis (level A). As a second-line treatment, the use of IVIg is recommended in dermatomyositis in combination with prednisone (level B) and is considered as a treatment option in polymyositis (level C). As a second- or even third-line therapy, the use of IVIg should be considered in patients with relapsing-remitting multiple sclerosis if conventional immunomodulatory therapies are not tolerated (level B) and in relapses during pregnancy or post-partum period (good clinical practice point). Finally, it appears that the use of IVIg has a beneficial effect also in stiff-person syndrome (level A), some paraneoplastic neuropathies (level B), and some acute-demyelinating diseases and childhood refractory epilepsy (good practice point).

Patients with chronic kidney disease (CKD) and specially end-stage renal disease (ESRD) have markedly advanced vascular disease when compared to the general population. In particular, several authors have reported more severe atherosclerotic disease of the carotid arteries among ESRD patients than in subjects with normal renal function. This accelerated disease of the cerebral vasculature could increase the risk of ischemic stroke in these patients. Additionally, ESRD is associated with hypertension, a bleeding diathesis, and the routine administration of heparin during hemodialysis, which could increase the risk of hemorrhagic stroke. Dialytic support, including both hemodialysis (HD) and peritoneal dialysis, have been shown to be risk factors for stroke. No studies have assessed stroke risk in renal transplant recipients. Although there are some epidemiologic data for stroke in patients with CKD, there are fewer data for stroke treatment in patients with CKD. In primary and secondary prevention of stroke even in the CKD patients, it has been well established that improved outcomes occur by correcting reversible risk factors, including treating hypertension, secondary hyperparathyroidisam, anemia, dyslipidemia, coagulation abnormalities, malnutrition, inflammation, controlling diabetes, and cardiac disease.

The link between the kidney and hypertension has been considered a villain-victim relationship because of the potential two-way causality between high blood pressure (BP) and chronic kidney disease (CKD). Arterial hypertension (AH) per se, but also together with diabetes mellitus, is the most important cause of CKD and end-stage renal disease (ESRD) in the developed world. Pathophysiologicaly, the increment in systemic BP leads to the rise in glomerular pressure. Glomerular hypertension results in glomerular capillary wall stretch, endothelial damage and a rise in protein glomerular filtration. These processes, in turn, cause changes of mesangial and proximal tubular cells, ultimately resulting in the replacement of functional by non-functional connective tissue and the development of fibrosis. One of the most important factors in the progression of CKD is activation of the renin- angiotensin system (RAS). Its effect is not only elevated BP, but also the promotion of cell proliferation, inflammation and matrix accumulation. The terms that clinicians use to identify renal damage associated with hypertension are nephrosclerosis, benign nephrosclerosis, hypertensive kidney disease, or nephroangiosclerosis. Many studies, first in experimental animals and later in humans, have shown that the lowering of BP (and proteinuria) is associated with a slower progression of CKD. It seems that angiotensin-converting enzyme inhibitors (ACEI's) are more renoprotective than other antihypertensives (the protection beyond the antihypertensive effect), although some studies have also confirmed a comparatively beneficial effect of non-dihydropiridine calcium channel blockers (CCBs) and angiotensin II receptor blockers (ARBs). Moreover, it seems that a combination of antihypertensives (e.g. ACEI, CCB, and ARB) has a more effective action than either of the drugs alone. The effects depend first on the degree of BP reduction. The strict BP control has been considered the basis of therapy for slowing renal deterioration.

The principal iron storage protein is ferritin, which is primarily present in cytoplasm. The most common cause of hyperferritinemia is iron overload, which is either primary or secondary. Hyperferritinemia is commonly found in patients with chronic kidney disease regardless of their hemoglobin level and is often considered to be related to chronic inflammatory status as well as malnutrition and neoplasias. We present a case of a kidney transplant patient that developed severe hyperferritinemia associated with liver dysfunction. In our patient, high hyperferritinemia was detected a year after transplantation, when she had no signs of inflammation. Malignancies, chronic viral hepatitis, and chronic inflammatory disease were also excluded as the causes of hyperferritinemia. Since high serum ferritin levels were combined with increased transferrin saturation and mildly elevated plasma iron concentrations, we presume that the most probable cause of hyperferritinemia in our patient was iron overload.