Waldenstrom macroglobulinemia - indolent disorder or slow killer?

Waldenström macroglobulinemia (WM) is an indolent (slow-growing) subtype of non-Hodgkin lymphoma that affects small lymphocytes. The risk of WM increases with age with median age at diagnosis of 63 years. Men are more likely than women to develop the WM. The overall incidence of WM is approximately five cases per one million persons per yea

Delayed Onset Of Acute Abdomen Revealing Foreign Body Ingestion

Foreign body ingestion is a common issue in the daily practice of dentistry and can lead to life-threatening complications such as intestinal obstruction, perforation, fistulas, peritonitis and sepsis. In the majority of cases, the foreign object will pass through the gastrointestinal tract without any complications, 10% of cases will require endoscopic treatment and in 1% of cases surgical removal is necessary

External and a four-act internal pelvic fixation in a patient with polytrauma

Fractures of the pelvic ring carry a high mortality and morbidity rate due to possibly great blood loss and abdominal organ trauma. However, external pelvic fixation can stabilize the patient enough to undergo emergency surgeries when treating a patient with multiple life-threatening injuries

100 BLOOD TRANSFUSIONS IN 10 MONTHS DUE TO HEYDE SYNDROME

Heyde syndrome is a multisystem disorder characterized by an association between gastrointestinal bleeding and aortic stenosis. High shear stress on stenotic valves leads to acquired coagulopathy due to proteolysis of von Willebrand factor and thus leads to bleeding from angiodysplasias of the digestive system

Pregnancy outcomes in women with Fontan circulation

The Fontan operation is a life-saving procedure performed on pediatric patients diagnosed with univentricular heart disease. The Fontan circulation is established by redirecting blood flow directly to the pulmonary circulation without passing through a ventricle. As more women with Fontan circulation reach adulthood and become pregnant, it is important to recognize obstetrical risks and provide optimal care for this rare condition

COVID-19 i kolorektalni karcinom – toksične poveznice i kako ih prekinuti: KBC Zagreb, iskustvo tercijarnog centra

Colorectal cancer (CRC) is one of the most prevalent oncological diseases globally, taking 3rd place in incidence in the general population. High in mortality, it is also a form of cancer whose outcome is highly dependable on its stage at diagnosis. Therefore, many countries have adopted a more or less successful screening process to ensure early diagnosis and, in turn, higher survival rates and better results overall. The COVID-19 pandemic has altered the established medical routines worldwide, with massively postponing diagnostic procedures and elective surgeries. This study aims to measure the effect the pandemic has had on colorectal cancer treatment in our Institution. Variables such as deferral time from diagnosis to commencement of treatment, lapse of time between different phases of the treatment process, time of presentation (elective versus emergent surgery), the physical status of the patient at the time of surgery (ASA classification) and metastatic index (positive lymph node ratio), were taken into account. We juxtaposed data from patients treated at the Surgical Department of Clinical Hospital Center in Zagreb in 2019 and 2020, the latter being heavily affected by the pandemic. In 2019 and 2020, 347 and 314 patients, respectively, with C18-C20 diagnoses (International Statistical Classification of Diseases and Related Health Problems ICD-10), have been treated at our Hospital. With exclusion criteria applied, the patient count falls to 173 for 2019 and 157 for 2020. These numbers include operated cases with or without an anastomosis formation and with or without neoadjuvant chemotherapy applied. From the analysis we excluded patients with recurrent colorectal tumors, synchronous and metachronous tumors, and patients treated palliatively. Furthermore, colorectal adenomas were also excluded from the study. Our data shows significant difference between observed variables in the two patient groups, attributed to the COVID-19 pandemic. Since there is still no reliable way to predict the duration of this global health crisis, it is imperative to implement strategies to lessen the damaging effect the pandemic has had on favourable oncosurgical treatment outcomes in colorectal cancer patients.Kolorektalni karcinom jedna je od najčešćih onkoloških bolesti u svijetu. Uz visoki mortalitet obilježena je i ovisnošću ishoda liječenja o stadiju bolesti u trenutku dijagnoze. Mnoge su države stoga usvojile više ili manje uspješne programe probira kako bi osigurale ranu dijagnozu, bolje stope preživljenja te generalno optimalnije ishode liječenja. Pandemija COVID-19 u kratkom je roku promijenila temelje medicinske svakodnevice uz nemale odgode dijagnostičkih procedura i elektivnih zahvata. Cilj ovog rada jest procijeniti utjecaj koji je pandemija imala na liječenje kolorektalnog karcinoma u našoj ustanovi. Uspoređivana su dva jednogodišnja razdoblja – 2019. i 2020. od kojih je potonja godina bila značajno pogođena COVID 19 pandemijom. Uspoređivali smo podatke pacijenta liječenih na Zavodu za kirurgiju Kliničkog bolničkog Centra Zagreb, vođenih pod MKB dijagnozama C18-C20. U 2019. godini na našem je Zavodu liječeno 347 pacijena s kolorektalnim karcinom, po primjeni kriterija isključenja ta brojka pada na 173. Bolesnika s operiranim karcinomom koloektuma u 2020. godini u našoj je ustanovi bilo 314, po primjeni kriterija isključenja 157. Praćeni parametri uključivali su vremensku odgodu od incijalne dijagnoze do početka liječenja, vrijeme proteklo između različitih etapa onko kirurškog liječenja, odnos elektivnih i hitnih zahvata, fizičku spremnost pacijenata u vrijeme operacije (ASA klasifikacija) te metastatski index (udio pozitivnih metastatskih limfnih čvorova u dobivenim preparatima). U studiju su uključeni bolesnici neovisno o primjeni neoadjuvantne kemoterapije te neovisno je li intraoepracijski uspostavljen kontinuitet probavne cijevi. Recidivni tumori, metakroni i sinkroni tumori, palijativno liječeni pacijenti, reoperirani te pacijenti operirani uslijed kolorektalnog adenoma nisu uključeni u ovu studiju. Naši podaci pokazuju značajne razlike među praćenim parametrima u dva razdoblja te se iste uvelike pripisuju utjecaju COVID-19 pandemije. Kako su budućnost i trajanje ove medicinske krize još uvijek neizvjesni, potrebno je što prije usvojiti strategije kojima bi se smanjio razorni utjecaj pandemije na ishode liječenja bolesnika sa kolorektalnim karcinomom

Potpuni patološki odgovor nakon neoadjuvantne kemoradioterapije lokalno uznapredovalog karcinoma rektuma

Background: The prognosis of rectal cancer has improved with neoadjuvant treatment for locally advanced disease. Twenty percent of patients respond to treatment with complete pathological regression, which is clinically estimated with magnetic resonance imaging. Aim: describe the properties of the pathological complete response group of patients at our institution Materials and methods: All selected patients received LCCRT at the University Hospital for Tumors Sestre milosrdnice University Hospital Center, Zagreb, between January 2014 and December 2019 and were later surgically treated at the same facility. Results: We identified 23 patients with complete pathological responses, of which, despite surgery, seven progressed. We recorded a higher proportion of female patients in that group and younger age of onset. MRI preoperatively was not yet predictive of a complete pathological response. Conclusion: The proportion of patients with a complete pathological response is 16% in this cohort. All patients underwent surgery but did not receive consolidating therapy. About 30% progressed during the observed period.Uvod: Prognoza raka rektuma poboljšana je neoadjuvantnim liječenjem lokalno uznapredovale bolesti. Dvadeset posto pacijenata reagira na liječenje potpunom patološkom regresijom, što se klinički procjenjuje magnetskom rezonancijom(MR). Cilj: opisati svojstva skupine pacijenata s patološkim potpunim odgovorom u našoj ustanovi Materijali i metode: Svi odabrani pacijenti primili su LCCRT u KBC-u Sestre milosrdnice, Zagreb, između siječnja 2014. i prosinca 2019. te su kasnije kirurški liječeni u istoj ustanovi. Rezultati: Identificirali smo 23 pacijenta s potpunim patološkim odgovorom, od kojih je, unatoč operaciji, sedam imalo progresiju bolesti. U toj skupini bilježimo veći udio bolesnica i mlađu dob pri dijagnozi. Magnetska rezonanca prije operacije nije bila pouzdan pokazatelj potpunog patološkog odgovora. Zaključak: Udio pacijenata s potpunim patološkim odgovorom je 16% u ovoj kohorti. Svi pacijenti su operirani, ali nisu primili konsolidirajuću kemoterapiju. Oko 30% je imalo progresiju bolesti tijekom promatranog razdoblja

The course and outcome of pregnancies complicated with myasthenia gravis

Mijastenija gravis (MG) je stečena autoimuna bolest koja nastaje kao posljedica stvaranja protutijela na acetilkolinske receptore (AChR) završne motorne ploče čime se narušava prijenos signala neuromuskularne spojnice. Usprkos nepoznavanju točnog uzroka smatra se da je bolest multifaktorijalne etiologije. Dobna raspodjela MG koja svoj vrh doseže u reproduktivnoj dobi žene zahtjeva pojačanu prekoncepcijsku, antenatalnu i postpartalnu skrb. Prema raširenosti simptoma MG razlikuju se okularni oblik s razvojem ptoze i diplopije te generalizirani oblik koji uključuje slabost ekstraokularnih mišića. Mijastenička kriza sa zatajenjem disanja predstavlja hitno neurološko stanje, a često je provocirana trudnoćom i postpartalnim razdobljem. Dijagnoza MG se postavlja na temelju anamneze, fizikalnog pregleda, serologije te dodatnih farmakoloških i elektrofizioloških ispitivanja. Liječenje uključuje inhibitore acetilkolinesteraze, imunosupresivnu terapiju, timektomiju te intravenske imunoglobuline i plazmaferezu. Prekoncepcijsko savjetovanje žena je neophodno kako bi se osigurala kontrola majčine bolesti, a istovremeno izbjegle teratogene posljedice imunosupresivne terapije na fetus. Trudnoća ima različite učinke na tijek MG, a moguće su egzacerbacije, remisije ili nepromijenjen tijek bolesti. Tijekom antenatalne skrbi provode se redoviti testovi plućne funkcije, individualna prilagodba terapije te fetalni ultrazvuk u svrhu određivanja pokreta ploda. Zbog hemodinamskih promjena prilagođava se doza lijeka, a odabir sigurne terapije u trudnoći može predstavljati izazov. Prilikom odabira načina porođaja u većini slučajeva se pristupa spontanom vaginalnom porođaju u epiduralnoj anesteziji. Dojenje se podupire uz praćenje stanja novorođenčeta i majke zbog mogućnosti postpartalne egzacerbacije u 30% slučajeva. Tranzitorna neonatalna mijastenija gravis (TNMG) nastaje kao posljedica transplacentarnog prijenosa majčinih protutijela, a prepoznaje se unutar prvih 12-48 h praćenjem kvalitete disanja novorođenčeta.Myasthenia gravis (MG) is an acquired autoimmune disease which occurs from the production of antibodies targeting the acetylcholine receptors on the motor end plate, thereby disrupting signal transmission of neuromuscular junction. Even though the exact cause is still unknown, MG is considered to have multifactorial etiology. The age distribution of MG that reaches its peak in the female's reproductive age indicates the need for enhanced preconceptional, antenatal and postpartum care. According to the distribution of symptoms, MG is classified into the ocular MG with the development of ptosis and diplopia and the generalized MG which includes extraocular muscles weakness. Myasthenic crisis with respiratory failure presents urgent neurological condition and is frequently provoked during pregnancy and postpartum period. The diagnosis is based on medical history, physical examination, serology, and additional pharmacological and electrophysiological testing. Treatment includes acetylcholinesterase inhibitors, immunosuppressive therapy, thymectomy, intravenous immunoglobulins and plasmapheresis. Preconception counseling is mandatory in an attempt to ensure disease control and to prevent teratogenic effect of immunosuppressants at the same time. Pregnancy has various effects on the course of the disease. Remission and exacerbation are possible while the severity of the disease may remain unchanged. Antenatal care includes regular pulmonary function tests, individual dose adjustment and fetal ultrasound to determine fetal movements. Due to hemodynamic changes during pregnancy, drug dose adjustment and deciding on a safe therapy in pregnancy can be very challenging. Spontaneous vaginal delivery under epidural anesthesia is usually encouraged while choosing the mode of delivery. Breastfeeding is recommended with continuous monitoring of the newborn and mother due to the possibility of postpartum exacerbation in 30% of the cases. Transient neonatal myasthenia gravis (TNMG) occurs as a result of transplacental transmission of maternal antibodies and is recognized within the first 12-48 h when it is important to monitor the quality of respiration

The course and outcome of pregnancies complicated with myasthenia gravis

Mijastenija gravis (MG) je stečena autoimuna bolest koja nastaje kao posljedica stvaranja protutijela na acetilkolinske receptore (AChR) završne motorne ploče čime se narušava prijenos signala neuromuskularne spojnice. Usprkos nepoznavanju točnog uzroka smatra se da je bolest multifaktorijalne etiologije. Dobna raspodjela MG koja svoj vrh doseže u reproduktivnoj dobi žene zahtjeva pojačanu prekoncepcijsku, antenatalnu i postpartalnu skrb. Prema raširenosti simptoma MG razlikuju se okularni oblik s razvojem ptoze i diplopije te generalizirani oblik koji uključuje slabost ekstraokularnih mišića. Mijastenička kriza sa zatajenjem disanja predstavlja hitno neurološko stanje, a često je provocirana trudnoćom i postpartalnim razdobljem. Dijagnoza MG se postavlja na temelju anamneze, fizikalnog pregleda, serologije te dodatnih farmakoloških i elektrofizioloških ispitivanja. Liječenje uključuje inhibitore acetilkolinesteraze, imunosupresivnu terapiju, timektomiju te intravenske imunoglobuline i plazmaferezu. Prekoncepcijsko savjetovanje žena je neophodno kako bi se osigurala kontrola majčine bolesti, a istovremeno izbjegle teratogene posljedice imunosupresivne terapije na fetus. Trudnoća ima različite učinke na tijek MG, a moguće su egzacerbacije, remisije ili nepromijenjen tijek bolesti. Tijekom antenatalne skrbi provode se redoviti testovi plućne funkcije, individualna prilagodba terapije te fetalni ultrazvuk u svrhu određivanja pokreta ploda. Zbog hemodinamskih promjena prilagođava se doza lijeka, a odabir sigurne terapije u trudnoći može predstavljati izazov. Prilikom odabira načina porođaja u većini slučajeva se pristupa spontanom vaginalnom porođaju u epiduralnoj anesteziji. Dojenje se podupire uz praćenje stanja novorođenčeta i majke zbog mogućnosti postpartalne egzacerbacije u 30% slučajeva. Tranzitorna neonatalna mijastenija gravis (TNMG) nastaje kao posljedica transplacentarnog prijenosa majčinih protutijela, a prepoznaje se unutar prvih 12-48 h praćenjem kvalitete disanja novorođenčeta.Myasthenia gravis (MG) is an acquired autoimmune disease which occurs from the production of antibodies targeting the acetylcholine receptors on the motor end plate, thereby disrupting signal transmission of neuromuscular junction. Even though the exact cause is still unknown, MG is considered to have multifactorial etiology. The age distribution of MG that reaches its peak in the female's reproductive age indicates the need for enhanced preconceptional, antenatal and postpartum care. According to the distribution of symptoms, MG is classified into the ocular MG with the development of ptosis and diplopia and the generalized MG which includes extraocular muscles weakness. Myasthenic crisis with respiratory failure presents urgent neurological condition and is frequently provoked during pregnancy and postpartum period. The diagnosis is based on medical history, physical examination, serology, and additional pharmacological and electrophysiological testing. Treatment includes acetylcholinesterase inhibitors, immunosuppressive therapy, thymectomy, intravenous immunoglobulins and plasmapheresis. Preconception counseling is mandatory in an attempt to ensure disease control and to prevent teratogenic effect of immunosuppressants at the same time. Pregnancy has various effects on the course of the disease. Remission and exacerbation are possible while the severity of the disease may remain unchanged. Antenatal care includes regular pulmonary function tests, individual dose adjustment and fetal ultrasound to determine fetal movements. Due to hemodynamic changes during pregnancy, drug dose adjustment and deciding on a safe therapy in pregnancy can be very challenging. Spontaneous vaginal delivery under epidural anesthesia is usually encouraged while choosing the mode of delivery. Breastfeeding is recommended with continuous monitoring of the newborn and mother due to the possibility of postpartum exacerbation in 30% of the cases. Transient neonatal myasthenia gravis (TNMG) occurs as a result of transplacental transmission of maternal antibodies and is recognized within the first 12-48 h when it is important to monitor the quality of respiration

The course and outcome of pregnancies complicated with myasthenia gravis

Mijastenija gravis (MG) je stečena autoimuna bolest koja nastaje kao posljedica stvaranja protutijela na acetilkolinske receptore (AChR) završne motorne ploče čime se narušava prijenos signala neuromuskularne spojnice. Usprkos nepoznavanju točnog uzroka smatra se da je bolest multifaktorijalne etiologije. Dobna raspodjela MG koja svoj vrh doseže u reproduktivnoj dobi žene zahtjeva pojačanu prekoncepcijsku, antenatalnu i postpartalnu skrb. Prema raširenosti simptoma MG razlikuju se okularni oblik s razvojem ptoze i diplopije te generalizirani oblik koji uključuje slabost ekstraokularnih mišića. Mijastenička kriza sa zatajenjem disanja predstavlja hitno neurološko stanje, a često je provocirana trudnoćom i postpartalnim razdobljem. Dijagnoza MG se postavlja na temelju anamneze, fizikalnog pregleda, serologije te dodatnih farmakoloških i elektrofizioloških ispitivanja. Liječenje uključuje inhibitore acetilkolinesteraze, imunosupresivnu terapiju, timektomiju te intravenske imunoglobuline i plazmaferezu. Prekoncepcijsko savjetovanje žena je neophodno kako bi se osigurala kontrola majčine bolesti, a istovremeno izbjegle teratogene posljedice imunosupresivne terapije na fetus. Trudnoća ima različite učinke na tijek MG, a moguće su egzacerbacije, remisije ili nepromijenjen tijek bolesti. Tijekom antenatalne skrbi provode se redoviti testovi plućne funkcije, individualna prilagodba terapije te fetalni ultrazvuk u svrhu određivanja pokreta ploda. Zbog hemodinamskih promjena prilagođava se doza lijeka, a odabir sigurne terapije u trudnoći može predstavljati izazov. Prilikom odabira načina porođaja u većini slučajeva se pristupa spontanom vaginalnom porođaju u epiduralnoj anesteziji. Dojenje se podupire uz praćenje stanja novorođenčeta i majke zbog mogućnosti postpartalne egzacerbacije u 30% slučajeva. Tranzitorna neonatalna mijastenija gravis (TNMG) nastaje kao posljedica transplacentarnog prijenosa majčinih protutijela, a prepoznaje se unutar prvih 12-48 h praćenjem kvalitete disanja novorođenčeta.Myasthenia gravis (MG) is an acquired autoimmune disease which occurs from the production of antibodies targeting the acetylcholine receptors on the motor end plate, thereby disrupting signal transmission of neuromuscular junction. Even though the exact cause is still unknown, MG is considered to have multifactorial etiology. The age distribution of MG that reaches its peak in the female's reproductive age indicates the need for enhanced preconceptional, antenatal and postpartum care. According to the distribution of symptoms, MG is classified into the ocular MG with the development of ptosis and diplopia and the generalized MG which includes extraocular muscles weakness. Myasthenic crisis with respiratory failure presents urgent neurological condition and is frequently provoked during pregnancy and postpartum period. The diagnosis is based on medical history, physical examination, serology, and additional pharmacological and electrophysiological testing. Treatment includes acetylcholinesterase inhibitors, immunosuppressive therapy, thymectomy, intravenous immunoglobulins and plasmapheresis. Preconception counseling is mandatory in an attempt to ensure disease control and to prevent teratogenic effect of immunosuppressants at the same time. Pregnancy has various effects on the course of the disease. Remission and exacerbation are possible while the severity of the disease may remain unchanged. Antenatal care includes regular pulmonary function tests, individual dose adjustment and fetal ultrasound to determine fetal movements. Due to hemodynamic changes during pregnancy, drug dose adjustment and deciding on a safe therapy in pregnancy can be very challenging. Spontaneous vaginal delivery under epidural anesthesia is usually encouraged while choosing the mode of delivery. Breastfeeding is recommended with continuous monitoring of the newborn and mother due to the possibility of postpartum exacerbation in 30% of the cases. Transient neonatal myasthenia gravis (TNMG) occurs as a result of transplacental transmission of maternal antibodies and is recognized within the first 12-48 h when it is important to monitor the quality of respiration