Kronični dugotrajni neliječeni giht s istodobnom dilatacijskom kardiomiopatijom i iznimno opsežnom anasarkom

Gout is the most common type of inflammatory arthritis in man caused by deposition of urate crystals into the joints as the result of elevated serum urate levels. A case of a 59-year-old patient with untreated, long-lasting gout and clinical manifestation of decompensated global dilated cardiomyopathy is presented. Examination revealed generalized pitting edema extending from both lower extremities to the sacrum, abdominal, and thoracic wall, with scrotal swelling and upper extremity involvement, an exceptionally vast generalized edema, i.e. anasarca. Proximal and distal interphalangeal joints of the hands and feet were swollen and deformed, with marked yellow tophi nodules. Laboratory studies revealed high serum uric acid concentration (546 μmol/L), decreased creatinine clearance (0.8 mL/s) and albumin concentration (27.4 g/L), as well as increased total urine protein mass (0.35 g/24 h). X-rays of the affected feet and fists showed punched-out lesions of the subchondral bone with overhanging bony margins in the first metatarsophalangeal, proximal, and distal interphalangeal joints of both hands. The extreme clinical presentation resolved upon intravenous administration of diuretics and pleurocentesis, followed by oral medications including furosemide, angiotensin-converting enzyme inhibitor, spironolactone and digoxin. Since serum urate level has been identified as an independent risk factor for the development of ischemic heart and chronic kidney disease, regulation of urate concentration is necessary, especially in patients diagnosed with gout.Giht je najčešći tip upalnog artritisa, a uzrokovan je nakupljanjem kristala urata u zglobovima kao rezultat povišene razine urata u serumu. Prikazan je slučaj 59-godišnjeg bolesnika s neliječenim uričnim artritisom i kliničkom manifestacijom globalno dekompenzirane dilatacijske kardiomiopatije. Kliničkim pregledom dominirala je anasarka, tj. opsežan generalizirani tjestasti edem oba gornja i donja ekstremiteta, skrotalni otok, edem trbušne i prsne stijenke. Proksimalni i distalni interfalangealni zglobovi šaka i stopala bili su otečeni i deformirani, uz vidljive žute čvoričaste tofe. Laboratorijski testovi pokazali su visoku koncentraciju mokraćne kiseline u serumu (546 μmol/L), smanjeni klirens kreatinina (0,8 mL/s) i koncentraciju albumina (27,4 g/L), te povišenu ukupnu masu bjelančevina u mokraći (0,35 g/24 h). Rendgenske snimke zahvaćenih stopala i šaka pokazale su destrukcije i rubne uzure s uzdignućem korteksa u prvom metatarzofalangealnom, proksimalnom i distalnom interfalangealnom zglobu obiju šaka. Ova ekstremna klinička slika riješena je intravenskom primjenom diuretika i pleurocentezom, nakon čega je slijedila oralna terapija uključujući furosemid, inhibitor enzima za konverziju angiotenzina, spironolakton i digoksin. Kako su serumske razine urata utvrđene kao neovisan čimbenik rizika za razvoj ishemijske srčane i kronične bubrežne bolesti, regulacija koncentracije urata je neophodna, naročito kod bolesnika s dijagnosticiranim gihtom

Endocrinological outcomes of pure endoscopic transsphenoidal surgery: a Croatian Referral Pituitary Center experience

AIM: To analyze early remission, complications, and pituitary function recovery after pure endoscopic endonasal transsphenoidal surgery (PEETS), a novel method in pituitary adenoma treatment. ----- METHODS: Testing of all basal hormone values and magnetic resonance imaging (MRI) were performed preoperatively and postoperatively (postoperative MRI only in nonfunctioning adenomas) in 117 consecutive patients who underwent PEETS in the period between 2007 and 2010. The series consisted of 21 somatotroph adenomas, 61 prolactinomas, and 4 corticotroph and 31 nonfunctioning adenomas. Sixty-three were macroadenomas and 54 were microadenomas. Remission was defined as hormonal excess normalization on the seventh postoperative day in functioning adenomas and as normal MRI findings approximately four months postoperatively in nonfunctioning adenomas. The presence of hypogonadism, growth hormone deficiency, and hypothyroidism was assessed on the seventh postoperative day. Hypocortisolism was assessed through necessity for replacement therapy within 18 months postoperatively. ----- RESULTS: Remission was achieved in 84% of patients: in 100% of microadenoma and 70% of macroadenoma patients (P<0.001, odds ratio [OR], 28.16, 95% confidence interval [CI], 1.61-491.36), respectively. Endocrinological complications occurred in 17.1% of patients: in 9% of microadenoma and 24% of macroadenoma patients (P=0.049, OR, 3.06; 95% CI, 1.03-9.08). Duration of empirical hydrocortisone replacement therapy was significantly shorter in microadenoma patients (P<0.001). Thirty-five percent of preoperatively present hormonal deficiencies improved after the surgery. Between tumor types there were no significant differences in remission, complications, and normal pituitary function recovery. ----- CONCLUSION: Patients with microadenomas had higher remission and lower complication rates following PEETS, emphasizing the necessity for early detection and treatment of pituitary adenomas. PEETS is a discussion-worthy method for microprolactinoma treatment

Ksantogranulom selarnog područja u bolesnika sa sarkoidozom

Xanthogranuloma of the sellar region is a very rare brain tumor with favorable prognosis and without reported relapses of purely xanthogranulomatous lesion after complete resection. A case is presented of a 40-year-old male diagnosed with and treated for sarcoidosis,complaining of headache, photophobia and loss of libido. Physical examination revealed generally scarce hairiness, while laboratory investigations showed panhypopituitarism. Expansive sellar and suprasellar mass compressing the floor of the third ventricle and optical chiasm was confirmed by cranial multi-slice computerized tomography (MSCT). Complete resection of the tumor mass using trans-sphenoidal approach was performed. Histopathologic analysis revealed cholesterol clefts, sparse lymphoplasmacellular infiltrates, macrophages, siderophages and foreign body giant cells around cholesterol clefts confirming the diagnosis of xanthogranuloma of the sellar region. Since preoperative diagnosis of xanthogranuloma is very difficult, therapeutic algorithm does not differ from other expansive lesions of the sellar region, but pituitary involvement should always be considered inpatient with sarcoidosis since therapeutic management is non-surgical. Follow up MSCT imaging after 6 months revealed a solid, contrast-enhanced mass at the posterior base of the sella.Ksantogranulom selarnog područja je vrlo rijedak tumor mozga s povoljnom prognozom i bez opisanih recidiva čisto ksantogranulomatozne lezije nakon potpunog odstranjenja. Prikazuje se slučaj 40-godišnjeg bolesnika s dijagnosticiranom i liječenom sarkoidozom koji se tužio na glavobolju, fotofobiju i gubitak libida. Fizikalni pregled je pokazao oskudnu dlakavost, dok su laboratorijske pretrage otkrile panhipopituitarizam. Ekspanzivnu selarnu i supraselarnu tvorbu koja je pritiskala dno trećeg ventrikla i optičkog hijazma potvrdila je višeslojna kompjutorizirana tomografija (MSCT). Tumorska tvorba je odstranjena u potpunosti transsfenoidnim pristupom. Histopatološka analiza je pokazala nakupine kolesterola,rijetke infiltrate limfoplazmatskih stanica, makrofage, siderofage i divovske stanice stranog tijela oko nakupina kolesterola, potvrđujući dijagnozu ksantogranuloma selarnog područja. Kako je prijeoperacijsku dijagnozu ksantogranuloma vrlo teško postaviti, terapijski algoritam se ne razlikuje od onoga za druge ekspanzivne lezije selarnog područja, no zahvaćenost hipofize treba uvijek imati na umu u bolesnika sa sarkoidozom, jer je liječenje neoperacijsko. Kontrolni MSCT nakon 6 mjeseci pokazao je čvrstu, kontrastom pojačanu tvorbu na stražnjoj bazi sele

Slučajno otkriveni miolipom učvršćen pedunklom u hernijskoj vreći: prikaz slučaja

A case of a very rare adipocytic tumor found during corrective surgery for incisional abdominal hernia is presented. Because of uterine leiomyomas the patient underwent total abdominal hysterectomy 14 years before. During surgery of incisional hernia, a part of small intestine along with a pedunculated tumor was found in hernial sac. The tumor was attached to the medial intra-abdominal peritoneum. On examination, the tumor presented as a totally encapsulated dimorphic benign neoplasm composed of mature adipocytes and well-differentiated smooth muscle cells. Lipoblasts, floret-like giant cells, nuclear atypia, mitosis or proliferation of medium-sized arteries with thick muscular walls were not observed. Tumor cells were negative for HMB45, estrogen and progesterone. The diagnosis of myolipoma was established. This tumor commonly presents as a large quiescent mass in retroperitoneum in adult females and to our knowledge this is the first report of myolipoma in hernial sac. Characteristic findings, differential diagnosis, prognosis and the possible origin of such a neoplasm are discussed.Prikazuje se slučaj vrlo rijetkog adipocitnog tumora koji je otkriven tijekom korektivne operacije incizijske trbušne hernije. Bolesnica je zbog leiomioma maternice 14 godina ranije podvrgnuta potpunoj abdominalnoj histerektomiji. Tijekom sadašnje operacije zbog incizijske hernije u hernijskoj vreći je otkriven dio tankog crijeva i tumor s peteljkom. Tumor je bio učvršćen za medijalni intraabdominalni peritoneum. Za vrijeme pregleda tumor se prikazao kao potpuno enkapsulirana dimorfna dobroćudna novotvorina sastavljena od zrelih adipocita i dobro diferenciranih glatkomišićnih stanica. Nisu nađeni lipoblasti, divovske stanice nalik cvjetiću, kao ni jezgrena atipija, mitoza ili proliferacija srednje velikih arterija sa zadebljanim mišićnim stijenkama. Stanice tumora bile su negativne na HMB45, estrogen i progesteron. Postavljena je dijagnoza miolipoma. Ovaj tumor se obično pojavljuje kao velika mirujuća masa u retroperitoneumu u odraslih žena i, prema našem saznanju, ovo je prvi prikaz slučaja miolipoma u hernijskoj vreći. Raspravlja se o znakovitim nalazima, diferencijalnoj dijagnostici, prognozi te mogućem podrijetlu ovakve novotvorine

Hipopituitarizam uzrokovan metastazom supraglotičnog karcinoma larinksa u hipofizu: prikaz slučaja

Intracranial metastases from laryngeal carcinoma are rarely clinically diagnosed. To our knowledge, this is the first report of hypopituitarism due to pituitary metastasis from laryngeal carcinoma. We report on a 70-year-old man who had a supraglottic squamous cell carcinoma, which was resected surgically followed by local full dose radiation therapy. Four months later, he presented with a sudden onset of diplopia, syncope, headache, general malaise and loss of appetite. Magnetic resonance imaging of the brain revealed a tumorous process of the sellar region. Endocrinological tests disclosed the presence of hypopituitarism. The tumor was subtotally resected endoscopically via endonasal transsphenoidal approach. Histopathology of tumor specimens indicated squamous cell carcinoma. Tumors of the sellar and parasellar region as in the case presented may easily be confused with pituitary adenoma. Pituitary metastases should be considered on differential diagnosis of unusual pituitary tumors, especially in patients with as well as in those without a history of malignant disease.Intrakranijske metastaze karcinoma larinksa se rijetko dijagnosticiraju. Prema našim spoznajama, ovo je prvi zabilježeni slučaj hipopituitarizma uzrokovanog metastazom karcinoma larinksa u hipofizu. Prikazujemo slučaj muškarca u dobi od 70 godina kojemu je dijagnosticiran supraglotički planocelularni karcinom larinksa koji je kirurški uklonjen te je provedena lokalna radioterapija u punoj dozi. Četiri mjeseca kasnije javljaju se naglo nastale dvoslike, glavobolja, sinkopa, opća slabost i gubitak apetita. Učinjena je MR mozga te je otkriven tumorski proces selarne i paraselarne regije. Endokrinološkim testovima otkriveno je postojanje hipopituitarizma. Tumor je djelomice odstranjen endonazalnim transsfenoidnim pristupom. Patohistološki nalaz pokazao je planocelularni karcinom. Tumori selarne i paraselarne regije, kao u ovom slučaju, lako se zamijene za adenom hipofize. Metastaze u hipofizu bi trebalo razmotriti u diferencijalnoj dijagnozi neobičnih tumora hipofize, pogotovo u bolesnika s poviješću maligne bolesti, ali također i u onih bez nje

Giant "Lipoma-like" Liposarcoma of the Retroperitoneum: A Case Report

Prikazan je slučaj 37-godišnjeg muškarca s palpabilnom tvorbom u abdomenu koja je odgovarala velikom retroperitonealnom liposarkomu koji je zahvatio silazni dio kolona. Učinjena je potpuna kirurška resekcija tumorske tvorbe s djelomičnom kolektomijom. Odstranjena tvorba težila je ukupno 10,7 kg, a patohistološki je odgovarala dobro diferenciranom ("lipomu sličnom") liposarkomu. Uvidom u rezultate objavljenih istraživanja smatra se da je agresivna kirurška resekcija metoda izbora u terapiji retroperitonealnog liposarkoma, dok se upotreba adjuvantne kemoterapije preporuča u liječenju proširene bolesti.A giant retroperitoneal liposarcoma that incorporated the descending colon was presented clinically as a palpable abdominal mass in a 37-year-old male patient. Complete surgical resection with adjacent partial colectomy was performed. Total tumor mass was 10.7 kg and pathological examination revealed well differentiated ("lipoma-like") liposarcoma. Literature review shows that aggressive surgical resection remains the mainstay of treatment for retroperitoneal liposarcoma, although there are some promising results that support the use of adjuvant combination chemotherapy in advanced metastatic disease

Kvaliteta života bolesnika s epilepsijom - naša iskustva

A prospective study was carried out at the Zagreb University Hospital Centre to evaluate the relationship between epilepsy, antiepileptic drugs (AEDs) and quality of life (QoL) in patients with epilepsy (PE), and its association with depressive symptoms and sexual dysfunction (SD). QoL was assessed by use of the Quality of Life in Epilepsy-31 Inventory (QOLIE-31), SD by the Arizona Sexual Experiences Scale (ASEX), and depressive symptoms by the Hamilton Rating Scale for Depression (HAM-D17). The study included 108 PE (women 63% and men 37% men), mean age 39.54±15.91 years. Focal type epilepsy was diagnosed in 14.8%, generalized type in 35.2%, and both types were present in 40.7% of study patients. Drug-resistant epilepsy (DRE) was present in 44/108 and vagus nerve stimulation (VNS) was implanted in 27/44 patients. The mean response on QOLIE-31 was 62.88±17.21 with no significant differences according to gender, type of epilepsy, and age. A statistically significantly lower QoL was found in the ‘Overall QoL’ domain (35-55 vs. <35 age group). Patients taking both types of AEDs had a significantly lower QoL compared to those on newer types of AEDs. Higher QoL was associated with less pronounced depressive symptoms (p=0.000). Significant correlations were found between lower QoL and SD (p=0.001). In 27 patients with DRE having undergone VNS, a favorable effect of VNS implantation on the QoL and mood was observed as compared with 18 patients without VNS (p=0.041).Provedeno je prospektivno istraživanje u KBC-u Zagreb s ciljem procjene povezanosti epilepsije, antiepileptičkih lijekova (antiepileptic drug, AED) i kvalitete života (quality of life, QoL) u bolesnika s epilepsijom, kao i učestalosti depresije i seksualne disfunkcije (SD). QOLIE-31 (Quality of Life in Epilepsy-31 Inventory) je primijenjen za procjenu QoL-a, ASEX (Arizona Sexual Experiences Scale) za SD i HAM-D17 (Hamilton Rating Scale) za depresiju. Uključeno je 108 bolesnika s epilepsijom (63% žena, 37% muškaraca; srednja dob 39,54±15,91 godina). Žarišnu epilepsiju imalo je 14,8% i generaliziranu 35,2% bolesnika, dok je obje vrste epilepsije imalo 40,7% bolesnika. Farmakorezistentnu epilepsiju (drug-resistant epilepsy, DRE) imalo je 44/108 bolesnika, a kod njih 27/44 ugrađen je stimulator vagusnog živca (vagus nerve stimulation, VNS). Srednji odgovor na QOLIE-31 bio je 62,88±7,21 bez značajnih razlika u odnosu na spol, vrstu epilepsije i dob. Statistički značajno niži QoL nađen je u domeni ‘Sveukupni QoL’ (dobna skupina 35-55 godina u odnosu na dobnu skupinu <35). Bolesnici koji su uzimali obje vrste AED imali su značajno niži QoL u usporedbi s onima na novijim AED. Viši QoL bio je povezan s manje izraženim simptomima depresije (p=0,000). Pronađene su značajne korelacije između nižeg QoL-a i SD (p=0,001). U bolesnika s DRE utvrđen je pozitivan utjecaj ugradnje VNS-a na QoL i raspoloženje (27 bolesnika s VNS-om u usporedbi s 18 bolesnika bez VNS-a, p=0,041)

DEPRESSION AND QUALITY OF LIFE IN PATIENTS WITH EPILEPSY - SINGLE CENTRE EXPERIENCE

Background: Patients with epilepsy commonly report depressive symptoms. The main aim of this study was to evaluate the relationship between epilepsy, antiepileptic drugs (AEDs) and depression. We also wanted to evaluate possible association between depressive symptofigms in patients with epilepsy with the quality of life (QoL). Material and methods: This was a prospective cross-sectional study carried out at the tertiary teaching hospital (University Hospital Centre Zagreb, Croatia) with Ethics committee approval. Questionnaires evaluating depressive symptoms and QoL were administered to consecutive patients treated in the Referral Centre of the Ministry of Health of the Republic of Croatia for Epilepsy. Depressive symptoms were evaluated using Hamilton Rating Scale for Depression (HAM-D17). Quality of life was assessed using Quality of life in epilepsy-31 inventory (QOLIE-31) Results: 108 patients (63% women, 37% men; mean age 39.54±15.91 years, range 18-80 years) with epilepsy were included. 14.8% of patients had focal, 35.2% generalised and 40.7% both types of epilepsy. Majority of patients (65.74%) were on two and more AEDs and quarter was on monotherapy (25%); 42% were on newer, 19% on older and 39% on both AEDs. Mean total score on HAM-D17 was 9.94±8.18 (men - mean total score 10.16±8.85, women - mean total score 9.81±7.84). There were no significant differences on HAM- newer AEDs, or both types AEDs) and results on HAM-D17, nor between the type of epilepsy and results on HAM-D17. We found strong negative correlation between the higher QoL and HAM-D17 (p=0.000). Conclusions: Results of this study evaluating depressive symptoms in patients with epilepsy demonstrate that our patients mainly experience mild depressive symptoms, with no significant differences on HAM-D17 regarding gender and age. Patients with epilepsy with less pronounced depressive symptoms were found to have higher QoL. We did not find statistically significant differences regarding the type of epilepsy and results on HAM-D17, nor between the AEDs (older vs. newer AEDs, or both types AEDs) and results on HAM-D17

Kronični dugotrajni neliječeni giht s istodobnom dilatacijskom kardiomiopatijom i iznimno opsežnom anasarkom

Gout is the most common type of inflammatory arthritis in man caused by deposition of urate crystals into the joints as the result of elevated serum urate levels. A case of a 59-year-old patient with untreated, long-lasting gout and clinical manifestation of decompensated global dilated cardiomyopathy is presented. Examination revealed generalized pitting edema extending from both lower extremities to the sacrum, abdominal, and thoracic wall, with scrotal swelling and upper extremity involvement, an exceptionally vast generalized edema, i.e. anasarca. Proximal and distal interphalangeal joints of the hands and feet were swollen and deformed, with marked yellow tophi nodules. Laboratory studies revealed high serum uric acid concentration (546 μmol/L), decreased creatinine clearance (0.8 mL/s) and albumin concentration (27.4 g/L), as well as increased total urine protein mass (0.35 g/24 h). X-rays of the affected feet and fists showed punched-out lesions of the subchondral bone with overhanging bony margins in the first metatarsophalangeal, proximal, and distal interphalangeal joints of both hands. The extreme clinical presentation resolved upon intravenous administration of diuretics and pleurocentesis, followed by oral medications including furosemide, angiotensin-converting enzyme inhibitor, spironolactone and digoxin. Since serum urate level has been identified as an independent risk factor for the development of ischemic heart and chronic kidney disease, regulation of urate concentration is necessary, especially in patients diagnosed with gout.Giht je najčešći tip upalnog artritisa, a uzrokovan je nakupljanjem kristala urata u zglobovima kao rezultat povišene razine urata u serumu. Prikazan je slučaj 59-godišnjeg bolesnika s neliječenim uričnim artritisom i kliničkom manifestacijom globalno dekompenzirane dilatacijske kardiomiopatije. Kliničkim pregledom dominirala je anasarka, tj. opsežan generalizirani tjestasti edem oba gornja i donja ekstremiteta, skrotalni otok, edem trbušne i prsne stijenke. Proksimalni i distalni interfalangealni zglobovi šaka i stopala bili su otečeni i deformirani, uz vidljive žute čvoričaste tofe. Laboratorijski testovi pokazali su visoku koncentraciju mokraćne kiseline u serumu (546 μmol/L), smanjeni klirens kreatinina (0,8 mL/s) i koncentraciju albumina (27,4 g/L), te povišenu ukupnu masu bjelančevina u mokraći (0,35 g/24 h). Rendgenske snimke zahvaćenih stopala i šaka pokazale su destrukcije i rubne uzure s uzdignućem korteksa u prvom metatarzofalangealnom, proksimalnom i distalnom interfalangealnom zglobu obiju šaka. Ova ekstremna klinička slika riješena je intravenskom primjenom diuretika i pleurocentezom, nakon čega je slijedila oralna terapija uključujući furosemid, inhibitor enzima za konverziju angiotenzina, spironolakton i digoksin. Kako su serumske razine urata utvrđene kao neovisan čimbenik rizika za razvoj ishemijske srčane i kronične bubrežne bolesti, regulacija koncentracije urata je neophodna, naročito kod bolesnika s dijagnosticiranim gihtom