Isolated unilateral proximal focal femoral deficiency presenting in a young woman

Proximal focal femoral deficiency (PFFD) is a rare congenital skeletal abnormality characterised by the partial absence of the proximal femoral segment with shortening of the entire lower extremity. It typically presents as a shortened thigh, which is flexed, externally rotated and abducted. Some other skeletal anomalies, especially fibular hemimelia, usually accompany the disorder. The diagnosis of PFFD is made in early childhood and based mainly on conventional radiography. However, the radiographic appearance of the disease evolves as the child grows. In addition, treatment procedures applied to the child affect the radiographic presentation of the disease in adulthood. Almost all cases of PFFD reported to date are in young children and in the current literature, there is a paucity of radiographic images of PFFD in mature skeleton. The aim of this case report is to present the radiographic appearance of PFFD in an adult patient and to increase awareness among radiologists about this disease.</jats:p

The Effects of Bougie Diameters on Tissue Oxygen Levels After Sleeve Gastrectomy: A Randomized Experimental Trial

Background:Staple-line leak is the most frightening complication of laparoscopic sleeve gastrectomy and several predisposing factors such as using improper staple sizes regardless of gastric wall thickness, narrower bougie diameter and ischemia of the staple line are asserted. Aims:To evaluate the effects of different bougie diameters on tissue oxygen partial pressure at the esophagogastric junction after sleeve gastrectomy. Study Design:A randomized and controlled animal experiment with 1:1:1:1 allocation ratio. Methods:Thirty-two male Wistar Albino rats were randomly divided into 4 groups of 8 each. While 12-Fr bougies were used in groups 1 and 3, 8-Fr bougies were used in groups 2 and 4. Fibrin sealant application was also carried out around the gastrectomy line after sleeve gastrectomy in groups 3 and 4. Burst pressure of gastrectomy line, tissue oxygen partial pressure and hydroxyproline levels at the esophagogastric junction were measured and compared among groups. Results:Mortality was detected in 2 out of 32 rats (6.25\%) and one of them was in group 2 and the cause of this mortality was gastric leak. Gastric leak was detected in 2 out of 32 rats (6.25\%). There was no significant difference in terms of burst pressures, tissue oxygen partial pressure and tissue hydroxyproline levels among the 4 groups. Conclusion:The use of narrower bougie along with fibrin sealant has not had a negative effect on tissue perfusion and wound healing

Incidence and clinical characteristics of acute rheumatic fever in Turkey: Results of a nationwide multicentre study

© 2021 Paediatrics and Child Health Division (The Royal Australasian College of Physicians)Aim: To evaluate the incidence and clinical features of acute rheumatic fever (ARF) in Turkey, following the revised Jones criteria in 2015. Methods: This multicentre study was designed by the Acquired Heart Diseases Working Group of the Turkish Pediatric Cardiology and Pediatric Cardiac Surgery Association in 2016. The data during the first attack of 1103 ARF patients were collected from the paediatric cardiologists between 1 January 2016 and 31 December 2016. Results: Turkey National Institute of Statistics records of 2016 were used for the determination of ARF incidence with regard to various cities and regions separately. The estimated incidence rate of ARF was 8.84/100 000 in Turkey. The ARF incidence varied considerably among different regions. The highest incidence was found in the Eastern Anatolia Region as 14.4/100 000, and the lowest incidence was found in the Black Sea Region as 3.3/100 000 (P < 0.05). Clinical carditis was the most common finding. The incidence of clinical carditis, subclinical carditis, polyarthritis, aseptic monoarthritis, polyarthralgia and Sydenham's Chorea was 53.5%, 29.1%, 52.8%, 10.3%, 18.6% and 7.9%, respectively. The incidences of clinical carditis, subclinical carditis, polyarthritis and polyarthralgia were found to be significantly different among different regions (P < 0.05). Conclusion: The findings of this nationwide screening of ARF suggest that Turkey should be included in the moderate-risk group

Clinical and molecular evaluation of MEFV gene variants in the Turkish population: a study by the National Genetics Consortium

Familial Mediterranean fever (FMF) is a monogenic autoinflammatory disorder with recurrent fever, abdominal pain, serositis, articular manifestations, erysipelas-like erythema, and renal complications as its main features. Caused by the mutations in the MEditerranean FeVer (MEFV) gene, it mainly affects people of Mediterranean descent with a higher incidence in the Turkish, Jewish, Arabic, and Armenian populations. As our understanding of FMF improves, it becomes clearer that we are facing with a more complex picture of FMF with respect to its pathogenesis, penetrance, variant type (gain-of-function vs. loss-of-function), and inheritance. In this study, MEFV gene analysis results and clinical findings of 27,504 patients from 35 universities and institutions in Turkey and Northern Cyprus are combined in an effort to provide a better insight into the genotype-phenotype correlation and how a specific variant contributes to certain clinical findings in FMF patients. Our results may help better understand this complex disease and how the genotype may sometimes contribute to phenotype. Unlike many studies in the literature, our study investigated a broader symptomatic spectrum and the relationship between the genotype and phenotype data. In this sense, we aimed to guide all clinicians and academicians who work in this field to better establish a comprehensive data set for the patients. One of the biggest messages of our study is that lack of uniformity in some clinical and demographic data of participants may become an obstacle in approaching FMF patients and understanding this complex disease

Nationwide prospective audit for the evaluation of appendicitis risk prediction models in adults: Right iliac fossa treatment (RIFT) - Turkey

Background: Appendicitis is the most prevalent surgical emergency. The negative appendicectomy rate and diagnostic uncertainty are important concerns. This study aimed to assess the effectiveness of current appendicitis risk prediction models in patients with acute right iliac fossa pain. Methods: A nationwide prospective observational study was conducted, including all consecutive adult patients who presented with right iliac fossa pain. Diagnostic, clinical and negative appendicectomy rate data were recorded. The Alvarado score, Appendicitis Inflammatory Response (AIR), Raja Isteri Pengiran Anak Saleha Appendicitis (RIPASA) and Adult Appendicitis Score systems were calculated with collected data to classify patients into risk categories. Diagnostic value and categorization performance were evaluated, with use of risk category-based metrics including 'true positive rate' (percentage of appendicitis patients in the highest risk category), 'failure rate' (percentage of patients with appendicitis in the lowest risk category) and 'categorization resolution' (true positive rate/failure rate). Results: A total of 3358 patients from 84 centres were included. Female patients were less likely to undergo surgery than men (71.5% versus 82.5% respectively; relative risk 0.866, 95% c.i. 0.834 to 0.901, P 0.001); with a three-fold higher negative appendicectomy rate (11.3% versus 4.1% respectively; relative risk 2.744, 95% c.i. 2.047 to 3.677, P 0.001). Ultrasonography was utilized in 56.8% and computed tomography in 75.2% of all patients. The Adult Appendicitis Score had the best diagnostic performance for the whole population; however, only RIPASA was significant in men. All scoring systems were successful in females patients, but Adult Appendicitis Score had the highest area under the receiver operating characteristic curve value. The RIPASA and the Adult Appendicitis Score had the best categorization resolution values, complemented by their exceedingly low failure rates in both male and female patients. Alvarado and AIR had extremely high failure rates in men. Conclusion: The negative appendicectomy rate was low overall, but women had an almost three-fold higher negative appendicectomy rate despite lower likelihood to undergo surgery. The overuse of imaging tests, best exemplified by the 75.2% frequency of patients undergoing computed tomography, may lead to increased costs. Risk-scoring systems such as RIPASA and Adult Appendicitis Score appear to be superior to Alvarado and AIR. © 2024 The Author(s). Published by Oxford University Press on behalf of BJS Foundation Ltd.Tibbi Akademi