Objectives of study: The adolescent population signifies the transitory period where the frequent occurrence of different histopathological lesions in patients with nephrotic syndrome (NS) is different from that seen in young children as well as that seen in adults. This study aimed to analyze the clinical features and histopathological spectrum of adolescent-onset NS. Material and methods: We retrospectively evaluated clinical features, biochemical investigations and histopathology of 103 children with idiopathic NS referred to the Pediatric Department, County Hospital of Târgu-Mureş. Fourteen patients with congenital-, infantil- and secondary NS were excluded from this study. Results: The patients were divided into 2 groups: in group A we included 69 patients diagnosed with NS diagnosed before 10 years-old, with a median age of 3.76±1.96 years, majority males (59.42%) and presenting the pure form of NS. On the other hand, in group B we included 20 adolescents having the median age at the onset of the disease 13.61±2.18 years, with equal distribution of the sexes and presenting the impure form in 65% of cases. The majority of the patients in both groups (68.11% and 70% respectively) responded to steroid therapy. The commonest histopathological subtype in both groups was focal segmental glomerulosclerosis. Conclusions: The incidence of nephrotic syndrome has increased in the last years. The impure form of NS is more frequent in the adolescents than in younger patients. Adolescents with impure and steroid-resistant NS at presentation have other lesions than minimal change disease. The early genetic diagnosis in NS is important for proper clinical management of the patients, prognosis and genetic counseling of the families.
Category Archives: Number
Rare Case of Immature Gastric Teratoma
Introduction: Teratomas are rare and complex tumors with components from more than one of the three germ cell layers. Teratomas range from benign, well-differentiated (mature) cystic lesions to those that are solid and malignant (immature). The incidence of all teratomas is estimated at 1:10,000–1:20,000 newborns. Gastric teratomas represent only 1-2% of all teratomas.
Case presentation: We report a case of gastric teratoma of a 2 month-old boy who presented with abdominal distension. Diagnosis was established by physical examination, ultrasonography and computed tomography. The tumor measured 13/10/5.5 cm and weighted 390 grams and was surgically excised. Histological examination revealed an immature gastric teratoma. We also reviewed existing clinical and genetic data on gastric teratomas. Association of gastric teratomas with other congenital anomalies or tumors is very rare. Reported cases include: Beckwith-Wiedemann syndrome (involved:11p15, IGF2), Hodgkin disease (developed 3 yrs. post-resection) and focal neuroblastoma. Recent theories include extraembryonic cells; also have been hypothesized to originate from pluripotent cells present in the gastric wall.
Conclusion: Gastric teratomas are extremely rare tumors. Complete resection induce a good outcome.
Development of a HPLC-UV Method for Determination of Meloxicam in Human Plasma and Pharmaceutical Dosage Forms
Objectives: A simple, quick and low cost HPLC-UV method for assay of meloxicam in plasma and pharmaceutical dosage forms was developed.
Methods: Separation and assay of meloxicam, using a simple reverse phase HPLC-UV method was achieved using an Agilent Zorbax SB C18 column, with methanol and 1% aqueous solution of glacial acetic acid as mobile phase. Elution was performed with composition gradient, meloxicam being detected at 355 nm with a 5 minutes analysis time. The method was tested on human plasma and pharmaceutical dosage forms.
Results: The retention time of the meloxicam was 3,7 minutes. Regression analysis showed good linearity, with correlation coefficient R= 0,9997; linear regression equation: y = 206,1x –77,5 over the 20-2000 ng/ml concentration range. Limit of detection was determined to be 5 ng/ml and limit of quantification was set at 15 ng/ml. The recovery of the analyte in human plasma was low: 30,50%, however it was reproducible, with a coefficient of variation of 4,83%. The analysis of the tablets resulted in a 85,82% of meloxicam compared to the declared concentration.
Conclusions: The method proposed is quick, simple and adequate for detecting the meloxicam in human plasma. Although the recovery rate was low, it was reproducible, which leads to the fact, that improving extraction procedure can optimize the method.
New Perspectives in the Management of Aortic Intramural Hematoma – A Literature Review
Aortic intramural hematoma frequently appear in elderly hypertensive patients who suffered a vasa vasorum rupture into the media, presenting clinical symptoms similar to aortic dissection. The current available data suggest a similar treatment strategy as in aortic dissection, although intramural hematoma is a different pathophysiological entity. The issue of the vulnerable contact of the intraaortic plaque, which is prone to rupture and to trigger the formation of an intramural hematoma, has not been elucidated so far. We present a brief literature review regarding complex plaque analysis, which opens a new area in identification of vulnerable patients with intramural hematoma, important for management of these patients and optimization of their treatment in order to avoid complications.
Evidence-based medicine
Evidence-based medicine can be defined as the well-conceived and beneficial use of current research knowledge in making decisions concerning patient care. The concept of evidence-based medicine has two fundamental principles, it is based on best available research studies and the subsequent transfer of their results to use in practice. It follows that an evidence-based approach has several advantages. Patients are better served because only tested procedures will be endorsed. The standing of the profession will be enhanced because only proven treatments will be offered.
Evidence hierarchies classify the importance and robustness of diverse types of biomedical research. There is no universally accepted hierarchy of evidence, though there is broad agreement on the relative strength of the principal types of research, or epidemiological studies. Randomized controlled trials (RCTs) rank above observational studies, while expert opinion and anecdotal experience are ranked at the bottom. Nonetheless, RCTs are not always the ‘ideal’ way of conducting clinical research. The “ladder of evidence” was developed, to a large extent, for questions related to interventions or therapies. For questions related to the cause, diagnosis or prognosis of a disease, cohort studies or case-control studies will often be more appropriate. It is useful to think of the various study designs, not as a hierarchy but as categories of evidence which will allow the strongest possible, practical and ethical study-design to be chosen.[More]
Cephalic Duodeno-Pancreatectomy With Pancreatic-Gastric Anastomosis With Double Purse String, in Patient with Lithiasis and Tumoral Jaundice – Case Report
Introduction: One of the most feared complications after cephalic duodeno-pancreatectomy remains pancreatic fistula. In recent years, various methods of pancreatico-digestive reconstruction were performed in order to reduce the rate of pancreatic fistula. One of these methods is pancreatico-gastric reconstruction by using two purse string threads.
Case report: We present in this article a patient with jaundice with mixed etiology: tumoral and lithiasic. Subjectively, the patient accused sclerose-skin-jaundice, right upper quadrant and epigastric pain, nausea and vomiting. Computed tomography revealed dilatation of intra- and extrahepatic bile ducts, a dilated Wirsung duct and a tumor at the biliopancreatic confluence, leading to a suspicion of vaterian ampulom. Upper endoscopy revealed a tumor protruding in the descending duodenal segment. Intraoperatively a tumor suggestive of vaterian ampulom and duct stones was shown. Surgical treatment consisted of coledocolitotomy, cephalic duodeno-pancreatectomy with pancreatic-gastric anastomosis, performed by using two purse string threads. The postoperative evolution was favorable.
Conclusion: Pancreatico-gastric anastomosis using two purse string threads is a simple, safe and quick procedure, avoiding the application of sutures through the pancreatic parenchyma and thus reducing the rate of pancreatic fistula.
Surgical treatment of anogenital hemangiomas of infancy
Introduction Infantile hemangiomas (IHs) are the most common benign tumors of the soft tissue in infants and children and they often represent a serious challenge for the treating physician. Hemangiomas located in the anogenital region represent only about 1% of all IHs, but raise special concerns as they have the propensity to ulcerate. This condition may appear spontaneously, or could result from therapeutic procedures. Ulceration is extremely painful and takes many weeks of conservative therapy to heal.
Material and Methods. The aim of this study is to present the surgical approach of the IHs located in the anogenital area and the outcomes of this treatment option.
Results. During a period of 36 months, 11 children (nine girls, two boys) were referred to our plastic surgery department with hemangiomas involving the anogenital, groin and perineum areas. The average follow-up period was of 8 months, during which 82% of cases experienced complications, especially ulceration. All the target hemangiomas were removed through a lenticular excision and the wound closed with a linear suture.
Conclusions. Our study has shown that surgical excision of a complicated anogenital hemangioma or of a “healthy” hemangioma at high risk for ulceration in the anogenital region is an effective treatment, with fast healing and complete resolution of the pathogenic condition. Lenticular excision and linear closure represent a convenient surgical technique that can be performed as early surgery, during the proliferative stage, or at any time later, when the patient needs treatment, in safety conditions and with good results.
Anatomical and Surgical Basis for Adult Living Donor Liver Transplantation with the Right Liver Lobe
Liver transplantation is now a standard procedure for the treatment of end stage liver diseases. Since 1968 until 2012, a number of 113,627 liver transplantations were performed in Europe, in 28 countries and 153 institutions. Despite these impressive figures the waiting list is growing every year. Transplant surgeons were preoccupied to find new ways to increase the donor pool. Among them: reduced size liver transplantation, split liver technique and more recently living donor liver transplantation. At first in the early `90, living donor liver transplantation was used for pediatric patients because the left lateral hepatic segments were harvested. This graft is too small for the metabolic demands of an adult patient. So the next step was the harvesting of the right liver lobe from the donor and transplantation to adult patients. Living donor liver transplantation has gained fast a wide acceptance but there are a few issues to discuss. The main concern is about the donor safety which is a healthy person undergoing major surgery with potential risks. Also the surgical technique evolved due to a better understanding of the anatomy and physiology of the liver and the right liver graft. We discuss here the anatomical and surgical basis for living donor liver transplantation with the right liver lobe.
Meyer’s Loop Anatomy Demonstrated Using Diffusion Tensor MR Imaging and Fiber Tractography at 3T
Objective: The ultimate anatomy of the Meyer’s loop continues to elude us. Diffusion tensor imaging (DTI) and diffusion tensor tractography (DTT) may be able to demonstrate, in vivo, the anatomy of the complex network of white matter fibers surrounding the Meyer’s loop and the optic radiations. This study aims at exploring the anatomy of the Meyer’s loop by using DTI and fiber tractography.
Methods: Ten healthy subjects underwent magnetic resonance imaging (MRI) with DTI at 3 T. Using a region-of-interest (ROI) based diffusion tensor imaging and fiber tracking software (Release 2.6, Achieva, Philips), sequential ROI were placed to reconstruct visual fibers and neighboring projection fibers involved in the formation of Meyer’s loop. The 3-dimensional (3D) reconstructed fibers were visualized by superimposition on 3-planar MRI brain images to enhance their precise anatomical localization and relationship with other anatomical structures.
Results: Several projection fiber including the optic radiation, occipitopontine/parietopontine fibers and posterior thalamic peduncle participated in the formation of Meyer’s loop. Two patterns of angulation of the Meyer’s loop were found.
Conclusions: DTI with DTT provides a complimentary, in vivo, method to study the details of the anatomy of the Meyer’s loop.
Fanconi Anemia — Case Report of Rare Aplastic Anemia at Child
Introduction: Fanconi anemia is an autosomal recessive disease characterized by congenital abnormalities, defective haematopoiesis, and a high risk of developing acute myeloid leukaemia, myelodysplastic syndrome and cancers. FA was first described in 1927 by the Swiss pediatrician Guido Fanconi. The diagnosis is based on morphological abnormalities, hematologic abnormalities (pancytopenia, macrocytic anemia and progressive bone marrow failure) and genetic tests (cariograma).
Case report: We present the case of a child with Fanconi anemia. Although skin and bone morphological abnormalities were present from birth, diagnosis was suspected at 11 years old.
Conclusions: Fanconi anemia is a heterogeneous condition that can present a variety of congenital defects but invariably results in defective haemopoiesis, which is the major cause of morbidity and mortality.