BACKGROUND Suprasellar germinomas are rare intracranial tumors frequently associated with permanent endocrine disorders.We present the clinical picture,treatment,and complications of suprasellar germinoma at pediatric...BACKGROUND Suprasellar germinomas are rare intracranial tumors frequently associated with permanent endocrine disorders.We present the clinical picture,treatment,and complications of suprasellar germinoma at pediatric age which,besides being lifethreatening,has lifelong endocrinological consequences.CASE SUMMARY A 12-year-old female patient was presented having had intensive headaches for three weeks and visual disturbances for six months.An ophthalmological examination revealed bilateral papilledema and a marked loss of vision.Emergency brain magnetic resonance imaging(MRI)showed a suprasellar tumor,involving the infundibulum and the optic chiasm,extending to the third ventricle.Laboratory tests confirmed decreased levels of thyroxine,cortisol,gonadotropins,and insulin-like growth factor 1.Maximal tumor reduction was performed,and immunohistopathology established the diagnosis of suprasellar germinoma.MRI of the spine and cerebrospinal fluid cytology confirmed the localized disease.Adjuvant chemotherapy and radiotherapy were performed according to the SIOP CNS GCT II protocol.A post-treatment MRI showed no residual tumor,but pituitary function had not recovered.Three and a half years after the end of the treatment,the patient is in a complete remission,requiring hormonal replacement therapy,continuous education,and psychological support.CONCLUSION This complex case highlights the importance of timely diagnosis,a multidisciplinary approach,and close follow-up in children with suprasellar germinomas.展开更多
Acute pancreatitis(AP) is common gastrointestinal disease of varied aetiology. The most common cause of AP is gallstones, followed by alcohol abuse as an independent risk factor. With the increased need for invasive t...Acute pancreatitis(AP) is common gastrointestinal disease of varied aetiology. The most common cause of AP is gallstones, followed by alcohol abuse as an independent risk factor. With the increased need for invasive techniques to treat pancreatic and bile duct pathologies such as endoscopic retrograde cholangiopancreatography(ERCP), AP has emerged as the most frequent complication. While severe AP following ERCP is rare(0.5%), if it does develop it has a greater severity index compared to non-ERCP AP. Development of a mild form of AP after ERCP is not considered a clinically relevant condition. Differences in the clinical presentation and prognosis of the mild and severe forms have been found between non-ERCP AP and postendoscopic pancreatitis(PEP). It has been proposedthat AP and PEP may also have different immunological responses to the initial injury. In this review, we summarise the literature on clinical and inflammatory processes in PEP vs non-ERCP AP.展开更多
BACKGROUND Diaphragmatic hernia(DH)is extremely rarely described during pregnancy.Due to the rarity,there is no diagnostic or treatment algorithm for DH in pregnancy.AIM To summarize and define the most appropriate di...BACKGROUND Diaphragmatic hernia(DH)is extremely rarely described during pregnancy.Due to the rarity,there is no diagnostic or treatment algorithm for DH in pregnancy.AIM To summarize and define the most appropriate diagnostic methods and therapeutic options for DH in pregnancy based on scarce literature.METHODS Literature search of English-,German-,Spanish-,and Italian-language articles were performed using PubMed(1946–2021),PubMed Central(1900–2021),and Google Scholar.The PRISMA protocol was followed.The search terms included:Maternal diaphragmatic hernia,congenital hernia,pregnancy,cardiovascular collapse,mediastinal shift,abdominal pain in pregnancy,hyperemesis,diaphragmatic rupture during labor,puerperium,hernie diaphragmatique maternelle,hernia diafragmática congenital.Additional studies were identified by reviewing reference lists of retrieved studies.Demographic,imaging,surgical,and obstetric data were obtained.RESULTS One hundred and fifty-eight cases were collected.The average maternal age increased across observed periods.The proportion of congenital hernias increased,while the other types appeared stationary.Most DHs were left-sided(83.8%).The median number of herniated organs declined across observed periods.A working diagnosis was correct in 50%.DH type did not correlate to maternal or neonatal outcomes.Laparoscopic access increased while thoracotomy varied across periods.Presentation of less than 3 days carried a significant risk of strangulation in pregnancy.CONCLUSION The clinical presentation of DH is easily confused with common chest conditions,delaying the diagnosis,and increasing maternal and fetal mortality.Symptomatic DH should be included in the differential diagnosis of pregnant women with abdominal pain associated with dyspnea and chest pain,especially when followed by collapse.Early diagnosis and immediate intervention lead to excellent maternal and fetal outcomes.A proposed algorithm helps manage pregnant women with maternal DH.Strangulated DH requires an emergent operation,while delivery should be based on obstetric indications.展开更多
Post-endoscopic retrograde cholangiopancreatographypancreatitis(PEP) is the most common complication of endoscopic retrograde cholangiopancreatography. The incidence of post-endoscopic retrograde cholangiopancreatogra...Post-endoscopic retrograde cholangiopancreatographypancreatitis(PEP) is the most common complication of endoscopic retrograde cholangiopancreatography. The incidence of post-endoscopic retrograde cholangiopancreatography(ERCP) pancreatitis varies substantially and is reported around 1%-10%, although there are some reports with an incidence of around 30%. Usually, PEP is a mild or moderate pancreatitis, but in some instances it can be severe and fatal. Generally, it is defined as the onset of new pancreatictype abdominal pain severe enough to require hospital admission or prolonged hospital stay with levels of serum amylase two to three times greater than normal, occurring 24 h after ERCP. Several methods have been adopted for preventing pancreatitis, such as pharmacological or endoscopic approaches. Regarding medical prevention, only non-steroidal anti-inflammatory drugs, namely diclofenac sodium and indomethacin, are recommended, but there are some other drugs which have some potential benefits in reducing the incidence of post-ERCP pancreatitis. Endoscopic preventive measures include cannulation(wire guided) and pancreatic stenting, while the adoption of the early pre-cut technique is still arguable. This review will attempt to present and discuss different ways of preventing post-ERCP pancreatitis.展开更多
Adult patients with developmental dysplasia of the hip develop secondary osteoarthritis and eventually end up with total hip arthroplasty(THA) at younger age. Because of altered anatomy of dysplastic hips, THA in thes...Adult patients with developmental dysplasia of the hip develop secondary osteoarthritis and eventually end up with total hip arthroplasty(THA) at younger age. Because of altered anatomy of dysplastic hips, THA in these patients represents technically demanding procedure. Distorted anatomy of the acetabulum and proximal femur together with conjoined leg length discrepancy present major challenges during performing THA in patients with developmental dysplasia of the hip. In addition, most patients are at younger age, therefore, soft tissue balance is of great importance(especially the need to preserve the continuity of abductors) to maximise postoperative functional result. In this paper we present a variety of surgical techniques availablefor THA in dysplastic hips, their advantages and disadvantages. For acetabular reconstruction following techniques are described: Standard metal augments(prefabricated), Custom made acetabular augments(3D printing), Roof reconstruction with vascularized fibula, Roof reconstruction with pedicled iliac graft, Roof reconstruction with autologous bone graft, Roof reconstruction with homologous bone graft, Roof reconstruction with auto/homologous spongious bone, Reinforcement ring with the hook in combination with autologous graft augmentation, Cranial positioning of the acetabulum, Medial protrusion technique(cotyloplasty) with chisel, Medial protrusion technique(cotyloplasty) with reaming, Cotyloplasty without spongioplasty. For femoral reconstruction following techniques were described: Distraction with external fixator, Femoral shortening through a modified lateral approach, Transtrochanteric osteotomies, Paavilainen osteotomy, Lesser trochanter osteotomy, Double-chevron osteotomy, Subtrochanteric osteotomies, Diaphyseal osteotomies, Distal femoral osteotomies. At the end we present author's treatment method of choice: for acetabulum we perform cotyloplasty leaving only paper-thin medial wall, which we break during acetabular cup impacting. For femoral side first we peel of all rotators and posterior part of gluteus medius and vastus lateralis from greater trochanter on the very thin flake of bone. This method allows us to adequately shorten proximal femoral stump, with possibility of additional resection of proximal femur. Furthermore, several advantages and disadvantages of this procedure are also discussed.展开更多
A pancreatic pseudocyst(PPC) is typically a complication of acute and chronic pancreatitis, trauma or pancreatic duct obstruction. The diagnosis of PPC can be made if an acute fluid collection persists for 4 to 6 wk a...A pancreatic pseudocyst(PPC) is typically a complication of acute and chronic pancreatitis, trauma or pancreatic duct obstruction. The diagnosis of PPC can be made if an acute fluid collection persists for 4 to 6 wk and is enveloped by a distinct wall.Most PPCs regress spontaneously and require no treatment, whereas some may persist and progress until complications occur. The decision whether to treat a patient who has a PPC, as well as when and with what treatment modalities, is a difficult one. PPCs can be treated with a variety of methods: percutaneous catheter drainage(PCD), endoscopic transpapillary or transmural drainage, laparoscopic surgery, or open pseudocystoenterostomy. The recent trend in the management of symptomatic PPC has moved toward less invasive approaches such as endoscopic- and image-guided PCD. The endoscopic approach is suitable because most PPCs lie adjacent to the stomach. The major advantage of the endoscopic approach is that it creates a permanent pseudocysto-gastric track with no spillage of pancreatic enzymes. However, given the drainage problems, the monitoring, catheter manipulation and the analysis of cystic content are very difficult or impossible to perform endoscopically, unlike in the PCD approach. Several conditions must be met to achieve the complete obliteration of the cyst cavity.Pancreatic duct anatomy is an important factor in the prognosis of the treatment outcome, and the recovery of disrupted pancreatic ducts is the main prognostic factor for successful treatment of PPC, regardless of the treatment method used. In this article, we review and evaluate the minimally invasive approaches in the management of PPCs.展开更多
Polycystic ovary syndrome(PCOS)is the most common endocrinopathy in women of reproductive age associated with long-term metabolic and cardiovascular consequences.A plethora of symptoms and their severity differentiate...Polycystic ovary syndrome(PCOS)is the most common endocrinopathy in women of reproductive age associated with long-term metabolic and cardiovascular consequences.A plethora of symptoms and their severity differentiate on an individual level,giving the syndrome numerous phenotypes.Due to menstrual cycle abnormalities,women suffer from irregular menstrual bleeding,difficulty in conception,and infertility.Furthermore,the risk of pregnancy complications such as gestational diabetes mellitus,hypertensive disorders of pregnancy,and preterm birth are higher in women with PCOS than in the general population.Often,women with PCOS have comorbidities such as dyslipidemia,obesity,glucose intolerance or diabetes type 2,non-alcoholic fatty liver disease,and metabolic syndrome,which all influence the treatment plan.Historic insulinsensitizing agents,although good for some of the metabolic derangements,do not offer long-term cardiovascular benefits;therefore,new treatment options are of paramount importance.Sodium-glucose co-transporter-2(SGLT-2)inhibitors,a new class of antidiabetic agents with beneficial cardiovascular,bodyweight,and antihyperglycemic effects,although not approved for the treatment of PCOS,might be an attractive therapeutic addition in the PCOS armamentarium.Namely,recent studies with SGLT-2 inhibitors showed promising improvements in anthropometric parameters and body composition in patients with PCOS.It is important to further explore the SGLT-2 inhibitors potential as an early therapeutic option because of the PCOS-related risk of metabolic,reproductive,and psychological consequences.展开更多
Irritable bowel syndrome(IBS)is considered a biopsychosocial disorder,whose onset and precipitation are a consequence of interaction among multiple factors which include motility disturbances,abnormalities of gastroin...Irritable bowel syndrome(IBS)is considered a biopsychosocial disorder,whose onset and precipitation are a consequence of interaction among multiple factors which include motility disturbances,abnormalities of gastrointestinal sensation,gut inflammation and infection,altered processing of afferent sensory information,psychological distress,and affective disturbances.Several models have been proposed in order to describe and explain IBS,each of them focusing on specific aspects or mechanisms of the disorder.This review attempts to present and discuss different determinants of IBS and its symptoms,from a cognitive behavioral therapy framework,distinguishing between the developmental predispositions and precipitants of the disorder,and its perpetuating cognitive,behavioral,affective and physiological factors.The main focus in understanding IBS will be placed on the numerouspsychosocial factors,such as personality traits,early experiences,affective disturbances,altered attention and cognitions,avoidance behavior,stress,coping and social support.In conclusion,a symptom perpetuation model is proposed.展开更多
AIM To investigate the effect of clonidine on the cutaneous silent period(CSP) during spinal anesthesia. METHODS A total of 67 adult patients were included in this randomized, prospective, single-center, double-blind ...AIM To investigate the effect of clonidine on the cutaneous silent period(CSP) during spinal anesthesia. METHODS A total of 67 adult patients were included in this randomized, prospective, single-center, double-blind trial. They did not have neurological disorders and were scheduled for inguinal hernia repair surgery. This trial was registered on ClinicalTrials.gov(NTC03121261). The patients were randomized into two groups with regards to the intrathecally administered solution:(1) 15 mg of 0.5% levobupivacaine with 50 μg of 0.015% clonidine, or(2) 15 mg of 0.5% levobupivacaine alone. There were 34 patients in the levobupivacaine-clonidine(LC) group and 33 patients in the levobupivacaine(L) group. CSP and its latency were measured four times: prior to the subarachnoid block(SAB), after motor block regression to the 0 level of the Bromage scale, with ongoing sensory blockade, and both 6 and 24 h after SAB.RESULTS Only data from 30 patients in each group were analyzed. There were no significant differences between the groups investigated preoperatively and after 24 h. The CSP of the L group at the time point when the Bromage scale was 0 was 44.8 ± 8.1 ms, while in the LC group it measured 40.2 ± 3.8 ms(P = 0.007). The latency in the L group at the time point when the Bromage scale was 0 was 130.3 ± 10.2 ms, and in the LC group it was 144.7 ± 8.3 ms(P < 0.001). The CSP of the L group after 6 h was 59.6 ± 9.8 ms, while in the LC group it was 44.5 ± 5.0 ms(P < 0.001). The latency in the L group after 6 h was 110.4 ± 10.6 ms, while in LC group it was 132.3 ± 9.7 ms(P < 0.001).CONCLUSION Intrathecal addition of clonidine to levobupivacaine for SAB in comparison with levobupivacaine alone resultsin a diminished inhibitory tonus and shortened CSP.展开更多
In the last two decades,advances in immunosuppressive regimens have led to fewer complications of acute rejection crisis and consequently improved shortterm graft and patient survival.In parallel with this great succe...In the last two decades,advances in immunosuppressive regimens have led to fewer complications of acute rejection crisis and consequently improved shortterm graft and patient survival.In parallel with this great success,long-term posttransplantation complications have become a focus of interest of doctors engaged in transplant medicine.Metabolic syndrome(MetS)and its individual components,namely,obesity,dyslipidemia,diabetes,and hypertension,often develop in the post-transplant setting and are associated with immunosuppressive therapy.Nonalcoholic fatty liver disease(NAFLD)is closely related to MetS and its individual components and is the liver manifestation of MetS.Therefore,it is not surprising that MetS and its individual components are associated with recurrent or“de novo”NAFLD after liver transplantation(LT).Fibrosis of the graft is one of the main determinants of overall morbidity and mortality in the post-LT period.In the assessment of post-LT steatosis and fibrosis,we have biochemical markers,imaging methods and liver biopsy.Because of the significant economic burden of post-LT steatosis and fibrosis and its potential consequences,there is an unmet need for noninvasive methods that are efficient and cost-effective.Biochemical scores can overestimate fibrosis and are not a good method for fibrosis evaluation in liver transplant recipients due to frequent post-LT thrombocytopenia.Transient elastography with controlled attenuation parameter is a promising noninvasive method for steatosis and fibrosis.In this review,we will specifically focus on the evaluation of steatosis and fibrosis in the post-LT setting in the context of de novo or recurrent NAFLD.展开更多
Dear Editor,Obesity has nowadays become a global public health challenge due to its rapidly growing prevalence and interconnection with a wide spectrum of comorbidities.
Patients with complete transposition of the great arteries(TGA)treated by the Senning procedure have a higher risk of developing heart failure due to:a)additional work load of the systemic(morphologic right)ventricle(...Patients with complete transposition of the great arteries(TGA)treated by the Senning procedure have a higher risk of developing heart failure due to:a)additional work load of the systemic(morphologic right)ventricle(sRV),b)arrhythmias,mainly caused by surgical implications at the atria as well as c)worsening of systemic tricuspid regurgitation.We present a unique case of a female patient who developed all these complications,who was successfully treated and was able to carry out a twin pregnancy.This breakthrough approach was based on:1.detecting reversibility potential of myocardial systolic dysfunction in a severe valvular lesion combined with continuous systemic afterload settings and permanent tachyarrhythmia,and 2.prevention of subsequently iatrogenic worsening of systemic ventricular function due to permanent pacing.Surgical replacement of systemic tricuspid valve(sTV)and cardiac resynchronization device(CRT)implantation after nodal ablation resulted in recovering of the systolic function and a positive remodeling of the sRV.The reversal of a further decline in systolic function was achieved by permanent arrhythmia control,synchronous pacing with epicardial leads of CRT,sTV replacement as well as echocardiographic monitoring during pregnancy to determine the right time for delivery.Two years after delivery,the patient remains in NYHA Class I.展开更多
This opinion review summarizes comparison of clinical presentation and immunology of post-endoscopic pancreatitis and acute pancreatitis(AP) of other etiology.The rationale for this topic was found in studies that men...This opinion review summarizes comparison of clinical presentation and immunology of post-endoscopic pancreatitis and acute pancreatitis(AP) of other etiology.The rationale for this topic was found in studies that mention differences in clinical presentation between these entities,stating that severe form of AP after endoscopic retrograde cholangiopancreatography was more severe than AP of other etiology.Found difference in clinical presentation may have a background in different immunology that needs to be further investigated.展开更多
Bariatric surgery offers the best health results in overweight and obese patients but is not a risk and/or complication-free treatment.In cases with additional hyperglycemia,the burden of surgery can be even higher an...Bariatric surgery offers the best health results in overweight and obese patients but is not a risk and/or complication-free treatment.In cases with additional hyperglycemia,the burden of surgery can be even higher and alter both shortterm and long-term outcomes.Although bariatric surgery offers glycemic improvements and in the case of early onset diabetes disease remission,weight loss results are lower than for obese patients without diabetes.Different multimodal programs,usually including interventions related to patients’performance,nutritional and psychological status as well as currently available pharmacotherapy before the surgery itself might considerably improve the immediate and late postoperative course.However,there are still no clear guidelines addressing the prehabilitation of obese patients with dysglycemia undergoing bariatric surgery and therefore no unique protocols to improve patients’health.In this minireview,we summarize the current knowledge on prehabilitation before bariatric surgery procedures in patients with obesity and dysglycemia.展开更多
Structured training in endonasal endoscopic sinus surgery(EESS) and skull base surgery is essential considering serious potential complications. We have developed a detailed concept on training these surgical skills o...Structured training in endonasal endoscopic sinus surgery(EESS) and skull base surgery is essential considering serious potential complications. We have developed a detailed concept on training these surgical skills on the lamb's head. This simple and extremely cheap model offers the possibility of training even more demanding and advanced procedures in human endonasal endoscopic surgery such as: frontal sinus surgery, orbital decompression, cerebrospinal fluid-leak repair followed also by the naso-septal flap, etc. Unfortunately, the sphenoid sinus surgery cannot be practiced since quadrupeds do not have this sinus. Still, despite this anatomical limitation, it seems that the lamb's head can be very useful even for the surgeons already practicing EESS, but in a limited edition because of a lack of the experience and dexterity. Only after gaining the essential surgical skills of this demanding field it makes sense to go for the expensive trainings on the human cadaveric model.展开更多
BACKGROUND Donor-origin cancer is a well-recognized but rare complication after liver transplantation (LT). The rise in the use of extended criteria donors due to the current shortage of organs increases the risk. Dat...BACKGROUND Donor-origin cancer is a well-recognized but rare complication after liver transplantation (LT). The rise in the use of extended criteria donors due to the current shortage of organs increases the risk. Data on donor-origin neuroendocrine neoplasms (NENs) and the most appropriate treatment are scarce. Here, we report a case of a patient who developed a NEN confined to the liver after LT and was treated with liver re-transplantation (re-LT). CASE SUMMARY A 49-year-old man with no other medical co-morbidities underwent LT in 2013 for alcoholic liver cirrhosis. The donor was a 73-year-old female with no known malignancies. Early after LT, a hypoechogenic (15 mm) lesion was detected in the left hepatic lobe on abdominal ultrasound. The lesion was stable for next 11 mo, when abdominal magnetic resonance identified two hypovascular lesions (20 and 11 mm) with atypical enhancement pattern. Follow-up abdominal ultrasound revealed no new lesions for the next 2.5 years, when magnetic resonance showed a progression in size and number of lesions, also confirmed by abdominal computed tomography. Liver biopsy proved a well-differentiated NEN. Genetic analysis of the NEN confirmed donor origin of the neoplasm. As NEN was confined to liver graft only, in 2018, the patient underwent his second LT. At 12 mo after re-LT the patient is well with no signs of NEN dissemination. CONCLUSION The benefits of graft explantation should be weighed against the risks of re-LT and the likelihood of NEN dissemination beyond the graft.展开更多
Spontaneous rupture of the spleen (SRS) is a rare clinical entity with a potentially poor medical outcome. In most cases, SRS is caused by neoplastic disorder. Acute promyelocytic leukaemia is a rare but important cau...Spontaneous rupture of the spleen (SRS) is a rare clinical entity with a potentially poor medical outcome. In most cases, SRS is caused by neoplastic disorder. Acute promyelocytic leukaemia is a rare but important cause of SRS that physicians are required to assess for. We present a 28-year-old woman with APL and COVID-19 pneumonia, who successfully underwent embolisation of the splenic artery for spontaneously occurring splenic rupture during induction chemotherapy. After the intervention the patient completed induction chemotherapy and achieved complete remission. Our case demonstrates that emergent transcatheter arterial embolisation can be lifesaving even in the unfavourable condition of a patien</span></span><span style="font-family:Verdana;"></span><span style="font-family:Verdana;"><span style="font-family:Verdana;">t with severe immune deficiency.展开更多
A candidate identification questionnaire (CIQ) was tested to determine its predictive value for patient-reported satisfaction in patients switched from once-weekly or once-daily treatment with a bisphosphonate to once...A candidate identification questionnaire (CIQ) was tested to determine its predictive value for patient-reported satisfaction in patients switched from once-weekly or once-daily treatment with a bisphosphonate to once-monthly dosing. This was a prospective, open-label, multicenter international study in patients with postmenopausal osteoporosis who had been receiving once-daily or once-weekly alendronate or risendronate for at least 3 months. Patients completed a CIQ, then commenced 150 mg monthly ibandronate for 6 months. Patients completed the Osteoporosis Patient Satisfaction Questionnaire (OPSAT-QTM) at baseline for 6 months. Scores were converted to composite satisfaction scores (CSS, scale 0-100). Totally 677 patients completed a CIQ, 645 were enrolled in the treatment phase and comprised the intent-to-treat (ITT) population, and 630 completed the study. In the ITT population, 68.1% patients answered “yes” to one or more CIQ questions. OPSAT-Q scores increased for the convenience, quality of life and overall satisfaction domains (p scores for the side effects domains were significant (p < 0.001) in the CIQ “yes” group, but not for the degree of bother (decrease in mean of 0.1 points, p = 0.50) or duration (no change, p = 0.84) of non-gastrointestinal side effects. Of 638 patients who completed the preference questionnaire, 93.0% of patients preferred the once-monthly dosing schedule and 563 patients (90.7%) found it more convenient. The most common adverse events were dyspepsia (1.9%), nausea (1.1%), and upper abdominal pain (0.9%). Patients are likely to prefer treatment with monthly ibandronate to a weekly or monthly bisphosphonate irrespective of their stated preference before switching treatment.展开更多
I read with interest the article by Mantadakis et al entitled"Intravenous iron sucrose for children with iron deficiency anemia:a single institution study".[1]The article has raised a very interesting issue....I read with interest the article by Mantadakis et al entitled"Intravenous iron sucrose for children with iron deficiency anemia:a single institution study".[1]The article has raised a very interesting issue.There are very few published studies on parenteral iron administration in pediatric practice for nonrenal indication.[2]Besides,because of reported hypersensitivity,pediatric hematologists are usually reluctant to give parenteral iron formulations.Finally,either European Medicines Agency or Food and Drug Administration does not approve most intravenous iron agents for pediatric use.Thereby,as opposed to countless oral iron preparations,there are only few intravenous iron products for children.展开更多
文摘BACKGROUND Suprasellar germinomas are rare intracranial tumors frequently associated with permanent endocrine disorders.We present the clinical picture,treatment,and complications of suprasellar germinoma at pediatric age which,besides being lifethreatening,has lifelong endocrinological consequences.CASE SUMMARY A 12-year-old female patient was presented having had intensive headaches for three weeks and visual disturbances for six months.An ophthalmological examination revealed bilateral papilledema and a marked loss of vision.Emergency brain magnetic resonance imaging(MRI)showed a suprasellar tumor,involving the infundibulum and the optic chiasm,extending to the third ventricle.Laboratory tests confirmed decreased levels of thyroxine,cortisol,gonadotropins,and insulin-like growth factor 1.Maximal tumor reduction was performed,and immunohistopathology established the diagnosis of suprasellar germinoma.MRI of the spine and cerebrospinal fluid cytology confirmed the localized disease.Adjuvant chemotherapy and radiotherapy were performed according to the SIOP CNS GCT II protocol.A post-treatment MRI showed no residual tumor,but pituitary function had not recovered.Three and a half years after the end of the treatment,the patient is in a complete remission,requiring hormonal replacement therapy,continuous education,and psychological support.CONCLUSION This complex case highlights the importance of timely diagnosis,a multidisciplinary approach,and close follow-up in children with suprasellar germinomas.
基金Supported by an education grant from Unicersity of Rijeka,No.963./2018
文摘Acute pancreatitis(AP) is common gastrointestinal disease of varied aetiology. The most common cause of AP is gallstones, followed by alcohol abuse as an independent risk factor. With the increased need for invasive techniques to treat pancreatic and bile duct pathologies such as endoscopic retrograde cholangiopancreatography(ERCP), AP has emerged as the most frequent complication. While severe AP following ERCP is rare(0.5%), if it does develop it has a greater severity index compared to non-ERCP AP. Development of a mild form of AP after ERCP is not considered a clinically relevant condition. Differences in the clinical presentation and prognosis of the mild and severe forms have been found between non-ERCP AP and postendoscopic pancreatitis(PEP). It has been proposedthat AP and PEP may also have different immunological responses to the initial injury. In this review, we summarise the literature on clinical and inflammatory processes in PEP vs non-ERCP AP.
文摘BACKGROUND Diaphragmatic hernia(DH)is extremely rarely described during pregnancy.Due to the rarity,there is no diagnostic or treatment algorithm for DH in pregnancy.AIM To summarize and define the most appropriate diagnostic methods and therapeutic options for DH in pregnancy based on scarce literature.METHODS Literature search of English-,German-,Spanish-,and Italian-language articles were performed using PubMed(1946–2021),PubMed Central(1900–2021),and Google Scholar.The PRISMA protocol was followed.The search terms included:Maternal diaphragmatic hernia,congenital hernia,pregnancy,cardiovascular collapse,mediastinal shift,abdominal pain in pregnancy,hyperemesis,diaphragmatic rupture during labor,puerperium,hernie diaphragmatique maternelle,hernia diafragmática congenital.Additional studies were identified by reviewing reference lists of retrieved studies.Demographic,imaging,surgical,and obstetric data were obtained.RESULTS One hundred and fifty-eight cases were collected.The average maternal age increased across observed periods.The proportion of congenital hernias increased,while the other types appeared stationary.Most DHs were left-sided(83.8%).The median number of herniated organs declined across observed periods.A working diagnosis was correct in 50%.DH type did not correlate to maternal or neonatal outcomes.Laparoscopic access increased while thoracotomy varied across periods.Presentation of less than 3 days carried a significant risk of strangulation in pregnancy.CONCLUSION The clinical presentation of DH is easily confused with common chest conditions,delaying the diagnosis,and increasing maternal and fetal mortality.Symptomatic DH should be included in the differential diagnosis of pregnant women with abdominal pain associated with dyspnea and chest pain,especially when followed by collapse.Early diagnosis and immediate intervention lead to excellent maternal and fetal outcomes.A proposed algorithm helps manage pregnant women with maternal DH.Strangulated DH requires an emergent operation,while delivery should be based on obstetric indications.
文摘Post-endoscopic retrograde cholangiopancreatographypancreatitis(PEP) is the most common complication of endoscopic retrograde cholangiopancreatography. The incidence of post-endoscopic retrograde cholangiopancreatography(ERCP) pancreatitis varies substantially and is reported around 1%-10%, although there are some reports with an incidence of around 30%. Usually, PEP is a mild or moderate pancreatitis, but in some instances it can be severe and fatal. Generally, it is defined as the onset of new pancreatictype abdominal pain severe enough to require hospital admission or prolonged hospital stay with levels of serum amylase two to three times greater than normal, occurring 24 h after ERCP. Several methods have been adopted for preventing pancreatitis, such as pharmacological or endoscopic approaches. Regarding medical prevention, only non-steroidal anti-inflammatory drugs, namely diclofenac sodium and indomethacin, are recommended, but there are some other drugs which have some potential benefits in reducing the incidence of post-ERCP pancreatitis. Endoscopic preventive measures include cannulation(wire guided) and pancreatic stenting, while the adoption of the early pre-cut technique is still arguable. This review will attempt to present and discuss different ways of preventing post-ERCP pancreatitis.
文摘Adult patients with developmental dysplasia of the hip develop secondary osteoarthritis and eventually end up with total hip arthroplasty(THA) at younger age. Because of altered anatomy of dysplastic hips, THA in these patients represents technically demanding procedure. Distorted anatomy of the acetabulum and proximal femur together with conjoined leg length discrepancy present major challenges during performing THA in patients with developmental dysplasia of the hip. In addition, most patients are at younger age, therefore, soft tissue balance is of great importance(especially the need to preserve the continuity of abductors) to maximise postoperative functional result. In this paper we present a variety of surgical techniques availablefor THA in dysplastic hips, their advantages and disadvantages. For acetabular reconstruction following techniques are described: Standard metal augments(prefabricated), Custom made acetabular augments(3D printing), Roof reconstruction with vascularized fibula, Roof reconstruction with pedicled iliac graft, Roof reconstruction with autologous bone graft, Roof reconstruction with homologous bone graft, Roof reconstruction with auto/homologous spongious bone, Reinforcement ring with the hook in combination with autologous graft augmentation, Cranial positioning of the acetabulum, Medial protrusion technique(cotyloplasty) with chisel, Medial protrusion technique(cotyloplasty) with reaming, Cotyloplasty without spongioplasty. For femoral reconstruction following techniques were described: Distraction with external fixator, Femoral shortening through a modified lateral approach, Transtrochanteric osteotomies, Paavilainen osteotomy, Lesser trochanter osteotomy, Double-chevron osteotomy, Subtrochanteric osteotomies, Diaphyseal osteotomies, Distal femoral osteotomies. At the end we present author's treatment method of choice: for acetabulum we perform cotyloplasty leaving only paper-thin medial wall, which we break during acetabular cup impacting. For femoral side first we peel of all rotators and posterior part of gluteus medius and vastus lateralis from greater trochanter on the very thin flake of bone. This method allows us to adequately shorten proximal femoral stump, with possibility of additional resection of proximal femur. Furthermore, several advantages and disadvantages of this procedure are also discussed.
文摘A pancreatic pseudocyst(PPC) is typically a complication of acute and chronic pancreatitis, trauma or pancreatic duct obstruction. The diagnosis of PPC can be made if an acute fluid collection persists for 4 to 6 wk and is enveloped by a distinct wall.Most PPCs regress spontaneously and require no treatment, whereas some may persist and progress until complications occur. The decision whether to treat a patient who has a PPC, as well as when and with what treatment modalities, is a difficult one. PPCs can be treated with a variety of methods: percutaneous catheter drainage(PCD), endoscopic transpapillary or transmural drainage, laparoscopic surgery, or open pseudocystoenterostomy. The recent trend in the management of symptomatic PPC has moved toward less invasive approaches such as endoscopic- and image-guided PCD. The endoscopic approach is suitable because most PPCs lie adjacent to the stomach. The major advantage of the endoscopic approach is that it creates a permanent pseudocysto-gastric track with no spillage of pancreatic enzymes. However, given the drainage problems, the monitoring, catheter manipulation and the analysis of cystic content are very difficult or impossible to perform endoscopically, unlike in the PCD approach. Several conditions must be met to achieve the complete obliteration of the cyst cavity.Pancreatic duct anatomy is an important factor in the prognosis of the treatment outcome, and the recovery of disrupted pancreatic ducts is the main prognostic factor for successful treatment of PPC, regardless of the treatment method used. In this article, we review and evaluate the minimally invasive approaches in the management of PPCs.
文摘Polycystic ovary syndrome(PCOS)is the most common endocrinopathy in women of reproductive age associated with long-term metabolic and cardiovascular consequences.A plethora of symptoms and their severity differentiate on an individual level,giving the syndrome numerous phenotypes.Due to menstrual cycle abnormalities,women suffer from irregular menstrual bleeding,difficulty in conception,and infertility.Furthermore,the risk of pregnancy complications such as gestational diabetes mellitus,hypertensive disorders of pregnancy,and preterm birth are higher in women with PCOS than in the general population.Often,women with PCOS have comorbidities such as dyslipidemia,obesity,glucose intolerance or diabetes type 2,non-alcoholic fatty liver disease,and metabolic syndrome,which all influence the treatment plan.Historic insulinsensitizing agents,although good for some of the metabolic derangements,do not offer long-term cardiovascular benefits;therefore,new treatment options are of paramount importance.Sodium-glucose co-transporter-2(SGLT-2)inhibitors,a new class of antidiabetic agents with beneficial cardiovascular,bodyweight,and antihyperglycemic effects,although not approved for the treatment of PCOS,might be an attractive therapeutic addition in the PCOS armamentarium.Namely,recent studies with SGLT-2 inhibitors showed promising improvements in anthropometric parameters and body composition in patients with PCOS.It is important to further explore the SGLT-2 inhibitors potential as an early therapeutic option because of the PCOS-related risk of metabolic,reproductive,and psychological consequences.
基金Supported by Ministry of Science,Education and Sport,Republic of Croatia,No.009-0092660-2655
文摘Irritable bowel syndrome(IBS)is considered a biopsychosocial disorder,whose onset and precipitation are a consequence of interaction among multiple factors which include motility disturbances,abnormalities of gastrointestinal sensation,gut inflammation and infection,altered processing of afferent sensory information,psychological distress,and affective disturbances.Several models have been proposed in order to describe and explain IBS,each of them focusing on specific aspects or mechanisms of the disorder.This review attempts to present and discuss different determinants of IBS and its symptoms,from a cognitive behavioral therapy framework,distinguishing between the developmental predispositions and precipitants of the disorder,and its perpetuating cognitive,behavioral,affective and physiological factors.The main focus in understanding IBS will be placed on the numerouspsychosocial factors,such as personality traits,early experiences,affective disturbances,altered attention and cognitions,avoidance behavior,stress,coping and social support.In conclusion,a symptom perpetuation model is proposed.
文摘AIM To investigate the effect of clonidine on the cutaneous silent period(CSP) during spinal anesthesia. METHODS A total of 67 adult patients were included in this randomized, prospective, single-center, double-blind trial. They did not have neurological disorders and were scheduled for inguinal hernia repair surgery. This trial was registered on ClinicalTrials.gov(NTC03121261). The patients were randomized into two groups with regards to the intrathecally administered solution:(1) 15 mg of 0.5% levobupivacaine with 50 μg of 0.015% clonidine, or(2) 15 mg of 0.5% levobupivacaine alone. There were 34 patients in the levobupivacaine-clonidine(LC) group and 33 patients in the levobupivacaine(L) group. CSP and its latency were measured four times: prior to the subarachnoid block(SAB), after motor block regression to the 0 level of the Bromage scale, with ongoing sensory blockade, and both 6 and 24 h after SAB.RESULTS Only data from 30 patients in each group were analyzed. There were no significant differences between the groups investigated preoperatively and after 24 h. The CSP of the L group at the time point when the Bromage scale was 0 was 44.8 ± 8.1 ms, while in the LC group it measured 40.2 ± 3.8 ms(P = 0.007). The latency in the L group at the time point when the Bromage scale was 0 was 130.3 ± 10.2 ms, and in the LC group it was 144.7 ± 8.3 ms(P < 0.001). The CSP of the L group after 6 h was 59.6 ± 9.8 ms, while in the LC group it was 44.5 ± 5.0 ms(P < 0.001). The latency in the L group after 6 h was 110.4 ± 10.6 ms, while in LC group it was 132.3 ± 9.7 ms(P < 0.001).CONCLUSION Intrathecal addition of clonidine to levobupivacaine for SAB in comparison with levobupivacaine alone resultsin a diminished inhibitory tonus and shortened CSP.
文摘In the last two decades,advances in immunosuppressive regimens have led to fewer complications of acute rejection crisis and consequently improved shortterm graft and patient survival.In parallel with this great success,long-term posttransplantation complications have become a focus of interest of doctors engaged in transplant medicine.Metabolic syndrome(MetS)and its individual components,namely,obesity,dyslipidemia,diabetes,and hypertension,often develop in the post-transplant setting and are associated with immunosuppressive therapy.Nonalcoholic fatty liver disease(NAFLD)is closely related to MetS and its individual components and is the liver manifestation of MetS.Therefore,it is not surprising that MetS and its individual components are associated with recurrent or“de novo”NAFLD after liver transplantation(LT).Fibrosis of the graft is one of the main determinants of overall morbidity and mortality in the post-LT period.In the assessment of post-LT steatosis and fibrosis,we have biochemical markers,imaging methods and liver biopsy.Because of the significant economic burden of post-LT steatosis and fibrosis and its potential consequences,there is an unmet need for noninvasive methods that are efficient and cost-effective.Biochemical scores can overestimate fibrosis and are not a good method for fibrosis evaluation in liver transplant recipients due to frequent post-LT thrombocytopenia.Transient elastography with controlled attenuation parameter is a promising noninvasive method for steatosis and fibrosis.In this review,we will specifically focus on the evaluation of steatosis and fibrosis in the post-LT setting in the context of de novo or recurrent NAFLD.
文摘Dear Editor,Obesity has nowadays become a global public health challenge due to its rapidly growing prevalence and interconnection with a wide spectrum of comorbidities.
文摘Patients with complete transposition of the great arteries(TGA)treated by the Senning procedure have a higher risk of developing heart failure due to:a)additional work load of the systemic(morphologic right)ventricle(sRV),b)arrhythmias,mainly caused by surgical implications at the atria as well as c)worsening of systemic tricuspid regurgitation.We present a unique case of a female patient who developed all these complications,who was successfully treated and was able to carry out a twin pregnancy.This breakthrough approach was based on:1.detecting reversibility potential of myocardial systolic dysfunction in a severe valvular lesion combined with continuous systemic afterload settings and permanent tachyarrhythmia,and 2.prevention of subsequently iatrogenic worsening of systemic ventricular function due to permanent pacing.Surgical replacement of systemic tricuspid valve(sTV)and cardiac resynchronization device(CRT)implantation after nodal ablation resulted in recovering of the systolic function and a positive remodeling of the sRV.The reversal of a further decline in systolic function was achieved by permanent arrhythmia control,synchronous pacing with epicardial leads of CRT,sTV replacement as well as echocardiographic monitoring during pregnancy to determine the right time for delivery.Two years after delivery,the patient remains in NYHA Class I.
文摘This opinion review summarizes comparison of clinical presentation and immunology of post-endoscopic pancreatitis and acute pancreatitis(AP) of other etiology.The rationale for this topic was found in studies that mention differences in clinical presentation between these entities,stating that severe form of AP after endoscopic retrograde cholangiopancreatography was more severe than AP of other etiology.Found difference in clinical presentation may have a background in different immunology that needs to be further investigated.
文摘Bariatric surgery offers the best health results in overweight and obese patients but is not a risk and/or complication-free treatment.In cases with additional hyperglycemia,the burden of surgery can be even higher and alter both shortterm and long-term outcomes.Although bariatric surgery offers glycemic improvements and in the case of early onset diabetes disease remission,weight loss results are lower than for obese patients without diabetes.Different multimodal programs,usually including interventions related to patients’performance,nutritional and psychological status as well as currently available pharmacotherapy before the surgery itself might considerably improve the immediate and late postoperative course.However,there are still no clear guidelines addressing the prehabilitation of obese patients with dysglycemia undergoing bariatric surgery and therefore no unique protocols to improve patients’health.In this minireview,we summarize the current knowledge on prehabilitation before bariatric surgery procedures in patients with obesity and dysglycemia.
文摘Structured training in endonasal endoscopic sinus surgery(EESS) and skull base surgery is essential considering serious potential complications. We have developed a detailed concept on training these surgical skills on the lamb's head. This simple and extremely cheap model offers the possibility of training even more demanding and advanced procedures in human endonasal endoscopic surgery such as: frontal sinus surgery, orbital decompression, cerebrospinal fluid-leak repair followed also by the naso-septal flap, etc. Unfortunately, the sphenoid sinus surgery cannot be practiced since quadrupeds do not have this sinus. Still, despite this anatomical limitation, it seems that the lamb's head can be very useful even for the surgeons already practicing EESS, but in a limited edition because of a lack of the experience and dexterity. Only after gaining the essential surgical skills of this demanding field it makes sense to go for the expensive trainings on the human cadaveric model.
文摘BACKGROUND Donor-origin cancer is a well-recognized but rare complication after liver transplantation (LT). The rise in the use of extended criteria donors due to the current shortage of organs increases the risk. Data on donor-origin neuroendocrine neoplasms (NENs) and the most appropriate treatment are scarce. Here, we report a case of a patient who developed a NEN confined to the liver after LT and was treated with liver re-transplantation (re-LT). CASE SUMMARY A 49-year-old man with no other medical co-morbidities underwent LT in 2013 for alcoholic liver cirrhosis. The donor was a 73-year-old female with no known malignancies. Early after LT, a hypoechogenic (15 mm) lesion was detected in the left hepatic lobe on abdominal ultrasound. The lesion was stable for next 11 mo, when abdominal magnetic resonance identified two hypovascular lesions (20 and 11 mm) with atypical enhancement pattern. Follow-up abdominal ultrasound revealed no new lesions for the next 2.5 years, when magnetic resonance showed a progression in size and number of lesions, also confirmed by abdominal computed tomography. Liver biopsy proved a well-differentiated NEN. Genetic analysis of the NEN confirmed donor origin of the neoplasm. As NEN was confined to liver graft only, in 2018, the patient underwent his second LT. At 12 mo after re-LT the patient is well with no signs of NEN dissemination. CONCLUSION The benefits of graft explantation should be weighed against the risks of re-LT and the likelihood of NEN dissemination beyond the graft.
文摘Spontaneous rupture of the spleen (SRS) is a rare clinical entity with a potentially poor medical outcome. In most cases, SRS is caused by neoplastic disorder. Acute promyelocytic leukaemia is a rare but important cause of SRS that physicians are required to assess for. We present a 28-year-old woman with APL and COVID-19 pneumonia, who successfully underwent embolisation of the splenic artery for spontaneously occurring splenic rupture during induction chemotherapy. After the intervention the patient completed induction chemotherapy and achieved complete remission. Our case demonstrates that emergent transcatheter arterial embolisation can be lifesaving even in the unfavourable condition of a patien</span></span><span style="font-family:Verdana;"></span><span style="font-family:Verdana;"><span style="font-family:Verdana;">t with severe immune deficiency.
文摘A candidate identification questionnaire (CIQ) was tested to determine its predictive value for patient-reported satisfaction in patients switched from once-weekly or once-daily treatment with a bisphosphonate to once-monthly dosing. This was a prospective, open-label, multicenter international study in patients with postmenopausal osteoporosis who had been receiving once-daily or once-weekly alendronate or risendronate for at least 3 months. Patients completed a CIQ, then commenced 150 mg monthly ibandronate for 6 months. Patients completed the Osteoporosis Patient Satisfaction Questionnaire (OPSAT-QTM) at baseline for 6 months. Scores were converted to composite satisfaction scores (CSS, scale 0-100). Totally 677 patients completed a CIQ, 645 were enrolled in the treatment phase and comprised the intent-to-treat (ITT) population, and 630 completed the study. In the ITT population, 68.1% patients answered “yes” to one or more CIQ questions. OPSAT-Q scores increased for the convenience, quality of life and overall satisfaction domains (p scores for the side effects domains were significant (p < 0.001) in the CIQ “yes” group, but not for the degree of bother (decrease in mean of 0.1 points, p = 0.50) or duration (no change, p = 0.84) of non-gastrointestinal side effects. Of 638 patients who completed the preference questionnaire, 93.0% of patients preferred the once-monthly dosing schedule and 563 patients (90.7%) found it more convenient. The most common adverse events were dyspepsia (1.9%), nausea (1.1%), and upper abdominal pain (0.9%). Patients are likely to prefer treatment with monthly ibandronate to a weekly or monthly bisphosphonate irrespective of their stated preference before switching treatment.
文摘I read with interest the article by Mantadakis et al entitled"Intravenous iron sucrose for children with iron deficiency anemia:a single institution study".[1]The article has raised a very interesting issue.There are very few published studies on parenteral iron administration in pediatric practice for nonrenal indication.[2]Besides,because of reported hypersensitivity,pediatric hematologists are usually reluctant to give parenteral iron formulations.Finally,either European Medicines Agency or Food and Drug Administration does not approve most intravenous iron agents for pediatric use.Thereby,as opposed to countless oral iron preparations,there are only few intravenous iron products for children.