Search

/

/

/

There is little data on the spectrum of renal diseases among children in Croatia. A renal diseases registry has been set up recently in an attempt to address this issue nationwide. We report the results of a retrospective analysis of clinical and pathohistological data.

Fibrillary glomerulonephritis(FGN)-do we know enough to make firm conclusions?

Isnose se podaci o biopsijom dokaznim bubrežnim bolesti djece u Hrvatskoj.

We present a case of 5-yrs old girl with interstitial lung disease which is important cause of respiratory impairment in adults, but very rare in children. She was initially healthy. The first clinical manifestations she developed 3 months before the first hospitalization and included cough, tachypnea, abdominal pain and failure to thrive. She was admitted to our clinic as acute ill child. Chest radiograph (CR) showed bilateral basal infiltrates as acute bacterial bronchopneumonia (BP), control CR showed the residuum of previous BP. She was treated with antibiotics, parenteral and inhaled steroids. After 3 weeks she was discharged in better condition.Two months later she became prostrated, with poor appetite, febrile.Laboratory findings: Leucocytes:24.3 G/L, 91% granulocytes, ABS at admission in refferent ranges, but after 5 days Sat.75%, pO2 5.6 kPa, pH 7.303, pCO2 2.4 kPa, CR right basal infiltrates as BP, control CR bilateral basal infiltrates with suspect pleural effussion. The sixth day of admission she presented severe respiratory failure and died (six months after the first symptoms).Pathologic findings: diffuse interstitial pneumonitis.The point of this case report is to present diagnostic and clinical dilemmas in children with clinical manifestations of interstitial lung disease as cough, tachypnea, respiratory distress and failure to thrive mimicking asthma or recurrent bronchopneumonia.

Ultrazvučni probir dojenčadi radi otkrivanja anomalija mokraćnog sustava.

/

Expression of intermediate filaments and desmosomal proteins during differentiation of the human spinal cord

/

/

Pretraga ultrazvukom potvrdila se kao sigurna, neinvazivna i pouzdana u ranom otkrivanju prirođenih AMS. Zahvaljujući njoj indikacije za invazivne i toksične metode, posebice rentgenske, u novorođenačkom periodu bitmo su reducirane ili postponirane. Prikazan je slučaj ženskog novorođenčeta s dvostrukim kanalnim sustavom lijevog bubrega. Gornji kanalni sustav bio je enormno dilatiran, kao i pripadajući mu ureter, koji je završavao ureterocelom. Donji kanalni sustav bio je vidljiv, ali ne i jače proširen. Desni kanalni sustav bio je dvostruk, bez dilatacije. Devetog dana života izvršena je perkutana drenaža gornjeg kanalnog sustava lijevo zbog pionefrosa i piouretera. Trinaestog dana života ekscidirana joj je ureterocela uz neoimplantaciju oba ušća s lijeve strane. Tek u dobi od tri mjeseca učinjena je i daljnja obrada(IVU ; MCUG i dinamička scintigrafija bubrega) kojom je potvrđeno prije navedeno, ali i funkcija gornjeg dijela lijevog bubrega te se učini parcijalna nefrektomija s pripadajućim ureterom. Temeljem opisanog vidljivo je da smo UZ pretragom utbrdili prirodu anomalije, a zatim temeljem UZ nalaza učinili korektivni kirurški zahvat. Tek kasnije smo komplementarnim dijagnostičkim postupcima dopunuli spoznaje o bolesti. Ovaj slučaj potvrđuje da je u rukama iskusnog ultrasonoskopičara UZ objektivna i pouzdana dijagnostička metoda u otkrivanju AMS.

Heterogeneity of the syndrome of renal tubular acidosis and nerve deafness

Dr.Jakov Miličić (1876.-1958.) bio je liječnik koji je najveći dio svog radnog vijeka proveo u Splitu. Po specijalnosti bio je kirurg i ginekolog, a bio je umjetnik, kipar i slikar. Zadužio je Hrvatski liječnički zbor svojom velikodušnom donacijom, jer je još za života svoj stan i dio sanatorija darovao Hrvatskom liječničkom zboru. Djelujući u Splitu, godine 1933. otvorio je ginekološki sanatorij sa 15 bolesničkih postelja, operaciojskom dvoranom i rendgenom. U svojoj privatnoj ordinaciji siromahe je besplatno liječio. Tijekom života izradio je 53 kiparske radnje, 15 kipova i 38 reljefa. Godine 1957. izložio je te radove u svom stanu sa željom da tu budu trajno izloženi i poslije njegove smrti. Sve radove je također nesebično darovao Splitskoj podružnici Zbora liječnika Hrvatske, u čijim se prostorijama i sada nalaze.

/

/

/

We report a case of ANCA associated nephritis in one 15-year old boy.

/

/

/

McCune - Albright sindrom - osteitis fibrosa cystica, MIM 17048 je sporadično multisistemno oboljenje, nazivaju ga bolešću postzigotične somatske mutacije, nasljeđuje se autosomno dominantno. Misteruja etiologije i patogeneze MAS je riješena sa identifikacijom mutacije u tzv. GNAS1 genu 20. kromosoma, u egzonu 8 alf podjedinice G proteina (Gs) što vodi povećanoj aktivnosti cAMP formacija. Mutacija ovog gena u ranoj embriogenezi rezultira mozaičkom populacijom u normalnim i mutacijom zahvaćenim tkivima što je podloga kliničke manifestacije bolesti. Bolest uključuje slijedeće major znakove: fibroznu poliostotičnu displaziju, iregularnu kožnu pigmentaciju te endokrine poremećaje. Skeletne promjene našeg 14 godišnjeg muškog pacijenta su unilateralne i opsežne, zahtijevaju stalni nadzor kirurga i ortopeda. Kožne promjene nalazimo samo na licu, glavi. Operirao je hipospadiju. Za sada nema znakova moguće endokrinopatije. Ultrazvučne promjene na bubrezima odgovaraju odlaganju kalcija u stijenke jrvnih žila bubrega, uz promjene parenhima ( početna nefrokalcinoza ?). Ima oscilatornu hipertenziju, hiperlipoproteinemiju II B, laboratorijski blagu hiperkalcemiju, jednom potvrđenu hiprekalciuriju. Dječak se liječi diskontinuirano kalcitoninom, koji je preve ntivni faktor patoloških fraktura. Rezultat liječenja zadovoljava.

Opisan je bolesnik s "Prune belly" sindromom koji se izuzetno rijetko susreće. U radu se raspravlja o mogućoj etiologiji i patogenezi "Prune belly" sindroma. Pokušalo se donekle protumačiti pojavu tog sindroma embriološkim teorijama o induktivnij reakciji tkiva, teorijom polja, te nešto pobliže reći o mogućem naslijeđivanju. Potrebno je naglasiti da u slučaju našeg bolesnika nema pojave "Prune belly" sindroma u obitelji niti se moglo utvrditi izlaganje majke nekoj teratogenoj noksi za vrijeme trudnoće, a ultrazvučni pregled nije pokazao intrauterini edem ploda.

Introduction: Blood pressure is controlled by kallilkrein-kinin system (KKS) and renin- angiotenzin-aldosteron system (RAAS). In developing kidneys, renin progenitor cells appear in the kidney arterial tree and juxtaglomerular apparatus (JGA), while kallikrein expression characterizes distal nephron. Both renin and kallikrein seem to influence kidney development and maturation. Data on kallikrein expression are conflicting, especially during human kidney development. In this study, we analyze the spatiotemporal and functional relationship between kidney renin and kallikrein during human development, in the postnatal period, and in the nephrotic syndrome of the Finnish type (CNF). Material and methods: The spatiotemporal expression of renin and kallikrein was analyzed in human kidneys by double immunofluorescence techniques in paraffin sections of 10 human conceptuses 8 to 38 weeks old, in 1, 5-year-old healthy kidney tissues and in the CNF. Results: In the embryonic period, renin - kallikrein expression characterized metanephric cup cells, S- form nephrons, immature glomeruli, and collecting ducts (CD). In fetal and postnatal kidneys, kallikrein was partly expressed in distal tubules (DT) and CD, and strongly along connecting tubules (CT), while renin expression characterized DT and CD, and partly CT. Their co-expression was observed in small areas of DT and CT. In CNF kidneys, renin and kallikrein co-expression was stronger and with longer overlapping areas than in the healthy kidneys. Conclusions: Our data suggested the interaction of renin and kallikrein during kidney maturation and in the blood pressure control. The two proteins were predominantly expressed in neighboring structures, while their co-expression characterized CT region. In CNF, their changed expression indicated increased efforts of affected kidneys in the blood pressure control.

Introduction: Primary cilia are non-motile sensory organelles involved in the control of different cellular processes, including cell differentiation. The role of primary cilia has been implicated in numerous cystic kidney diseases. By now, primary cilia have been detected on the surfaces of tubular cells and collecting ducts in developing and postnatal human kidneys. In the present study we analyze appearance and percentage of primary cilia on the surfaces of developing human healthy and nephrotic podocytes. Material and methods: Kidney tissue were dissected from human conceptuses 8th-38th postovulatory week old, postnatal healthy and nephrotic syndrome of the Finnish type (CNF) kidneys. They were stained by double immunofluorescent technique, using α- tubulin as primary cilia marker and DAPI as nuclear marker. Cilia were counted on cell surfaces and their percentage was calculated using Kruskal–Wallis test, followed by Dunns post-hoc test. Ultrastructural characteristics of podocytes were analyzed by transmission electron microscopy. Results: Between the 8th and 10th developmental week, population of metanephric cup cells contained 24% of ciliated cells, immature glomeruli 47% and Bownam’s capsule cells (parietal podocytes) 26%. Percentage of ciliated podocytes decreased to 10% in the 38th week and then to 3% in postnatal period. Number of ciliated cells in Bowman’s capsule first increased to 32% by the 22nd developmental week, and postnatally decreased to 16%. Compared to healthy kidneys, CNF podocytes showed increased number of ciliated podocytes (12%) and Bowman’s capsule cells (19%). Conclusions: Prospective human podocytes contain primary cilia already at the metanephric cup stage. During development, number of ciliated podocytes decreases in accordance with podocyte differentiation, while Bowman’s capsule cells retain higher percentage of ciliated cell, thus indicating their potential in podocyte regeneration. Compared to healthy kidneys, both cell populations of CNF kidneys display higher percentage of primary cilia, pointing to their incomplete maturation.