New Computerized Planning Algorithm and Clinical Testing of Optimized Nuss Bar Design for Patients with Pectus Excavatum.

BACKGROUND Computer-aided design (CAD) has been used in the Nuss procedure to determine the bar length and shape. Despite computer aid, the shape and design remain quite intuitive. We tested a new algorithm to determine the optimal bar shape. MATERIAL AND METHODS The normal sterno-vertebral distance was defined on computed tomography (CT) scans of patients without pectus excavatum (PEx) at the same level where the deepest depression was found on CT scans of 97 patients with PEx. Four points were marked on the CT scan of 60 patients with PEx at the deepest deformity: P1: edge of the vertebra; P2: edge of the deformity; P3: the expected contact point of the bar and the rib; and P4: the expected end of the bar. The algorithm generated 3 circles upon these points, and the fusion of the arcs drew the line of the ideal bar. Corrected and normal sterno-vertebral distance values were compared with the Mann-Whitney U test. Ten bars were bent manually guided by a 1: 1 printout of the designed bar and were implanted in 10 adolescents. RESULTS The shortest sterno-vertebral distance was 3 cm below the intermammillary line in PEx patients. The normal mean sterno-vertebral distance at this level was 10.16±1.35 cm in non-PEx patients. The mean virtually corrected sterno-vertebral distance was 10.28±1.27 cm. No significant difference was found (P=0.44). The bars were seamless and were successfully implanted. No bar needed adjustment, the operation time was shorter, and the patient satisfaction score was 9.4/10. CONCLUSIONS With our new algorithm, an optimal Nuss bar can be designed.

Modern understanding of intussusception and recent trends in management / A gyermekkori invaginatio modern értelmezése és kezelése

Intussusception is one of the most common abdominal emergencies in children. The understanding of its aetiology and management has changed significantly over the last decades. Earlier, the hypertrophic Peyer's patches and polyps were considered responsible, but with the knowledge obtained from the lipopolysaccharide-induced animal model of intussusception, the rotavirus vaccination, the seasonality and the postnatal changes of the enteric nervous system it became clear that the intestinal motility plays a key role in the aetiology. The efficacy of non-operative management is continuously improving. The radiologists initially moved from the hydrostatic X-ray-controlled reduction towards the air enema (pneumatic reduction), however, nowadays, there is a shift back to hydrostatic procedures but under ultrasound guidance to reduce radiation exposure. In many institutions, intussusception is managed as day-case rather than as an inpatient case. The role of medications like glucagon and cyclo-oxygenase inhibitors used during reduction manoeuvres and prevention of recurrence is still controversial. Surgical management is shifting towards laparoscopy. The authors herein reviewed the current literature to present recent insights into understanding the pathogenesis and management updates. Orv Hetil. 2020; 161(32): 1331-1338.

Determining Projections of Grain Boundaries from Diffraction Data in Transmission Electron Microscope.

Grain boundaries (GB) are characterized by disorientation of the neighboring grains and the direction of the boundary plane between them. A new approach presented here determines the projection of GB that can be used to determine the latter one. The novelty is that an additional parameter of GB is quantified in addition to the ones provided by the orientation maps, namely the width of the projection of the GB is measured from the same set of diffraction patterns that were recorded for the orientation map, without the need to take any additional images. The diffraction patterns are collected in nanobeam diffraction mode in a transmission electron microscope, pixel-by-pixel, from an area containing two neighboring grains and the boundary between them. In our case, the diffraction patterns were recorded using the beam scanning function of a commercially available system (ASTAR). Our method is based on non-negative matrix factorization applied to the mentioned set of diffraction patterns. The method is encoded in a MATLAB environment, making the results easy to interpret and visualize. The measured GB-projection width is used to determine the orientation of the GB-plane, as given in the study by Kiss et al.

Highlighting material structure with transmission electron diffraction correlation coefficient maps.

Correlation coefficient maps are constructed by computing the differences between neighboring diffraction patterns collected in a transmission electron microscope in scanning mode. The maps are shown to highlight material structural features like grain boundaries, second phase particles or dislocations. The inclination of the inner crystal interfaces are directly deduced from the resulting contrast.

A Tool for Local Thickness Determination and Grain Boundary Characterization by CTEM and HRTEM Techniques.

A new approach for measurement of local thickness and characterization of grain boundaries is presented. The method is embodied in a software tool that helps to find and set sample orientations useful for high-resolution transmission electron microscopic (HRTEM) examination of grain boundaries in polycrystalline thin films. The novelty is the simultaneous treatment of the two neighboring grains and orienting both grains and the boundary plane simultaneously. The same metric matrix-based formalism is used for all crystal systems. Input into the software tool includes orientation data for the grains in question, which is determined automatically for a large number of grains by the commercial ASTAR program. Grain boundaries suitable for HRTEM examination are automatically identified by our software tool. Individual boundaries are selected manually for detailed HRTEM examination from the automatically identified set. Goniometer settings needed to observe the selected boundary in HRTEM are advised by the software. Operation is demonstrated on examples from cubic and hexagonal crystal systems.

Laparoscopic versus open surgery for complicated appendicitis: a randomized controlled trial to prove safety.

BACKGROUND: To date, no randomized control trial has been performed comparing open appendectomy (OA) to laparoscopic appendectomy (LA) in complicated appendicitis. A systematic review and meta-analysis in 2010 concluded LA is advantageous to OA with less surgical site sepsis in complicated appendicitis; however, the level of evidence is weak (level 3a). The aim of the study was to determine whether LA is safe in the treatment of complicated appendicitis. Primary outcome included all-cause mortality and procedure-related mortality; secondary outcomes included intra-operative duration, rates of wound sepsis and re-intervention, length of hospital stay and re-admission rates. METHODS: One hundred and fourteen patients were randomized prospectively to either OA or LA using a computer-generated blind method. Patients who were either less than 12 years of age, had previous abdominal surgery or were pregnant were excluded. A team of senior surgeons capable of doing both OA and LA performed all procedures. RESULTS: The intra-operative duration, the rate of wound sepsis, the number of re-operations, the length of hospital stay and the rate of re-admissions between the OA and LA groups did not differ statistically. CONCLUSION: Laparoscopic appendectomy is safe in complicated appendicitis. Current Control Trials (ISRCTN92257749).

[Questions and dilemmas in the management of hypospadias]. / A hypospadiasis ellátásában felmerülo kérdések, dilemmák.

Hypospadias is the second most common congenital malformation in males. Etiology remains unknown in about 70% of the cases. Distal hypospadias is considered not only developmental abnormality of the urethra in males, but it may also constitute a mild form of sexual development disorder in 46,XY males. Most urologists and endocrinologists consider that it is necessary to perform a detailed investigation of children presenting with proximal hypospadias associated with a small phallus or poorly developed scrotum and undescended testes. Currently, there is no generally accepted recommendation for the preoperative evaluation of hypospadias and, therefore, masculinizing surgery without preoperative evaluation is performed in these children. The authors summarize the international literature data and their own experience for the assessment and management of hypospadias concerning questions and problems related to preoperative investigation, masculinizing surgery and additional surgery. A detailed algorithm is presented for preoperative evaluation of both proximal and distal hypospadias.

[Prevalence and possible causes of hypospadias]. / A hypospadiasis gyakorisága és lehetséges okai.

Hypospadias is the most common congenital malformation of the male external genitalia. After the heart and circulatory system, it is the second most common developmental disorder in males. It is due to a midline fusion defect of the male urethra, which results in a misplaced urethral meatus. Hypospadias may be distal, medial and proximal. It may occur as an isolated defect or it may develop together with other genital disorders (retention of testes in one or both sides, microphallus, bifid scrotum) or with malformation of other organs. In some cases syndromic forms may also occur. Genetic factors play a crucial role in the occurrence of early developmental defect, but endocrine and environmental factors may also be important in the aetiology of hypospadias. It may be associated with various sex and autosomal chromosomal abnormalities. Monogenic and chromosomal causes of hypospadias accounts for about in 30% of all cases, while genetic factors remain unknown in 70% of cases. The authors summarize the development of the male external genitalia, the prevalence and possible causes of hypospadias. They propose that better understanding of the pathogenesis of the disease may contribute to the prevention and decreased prevalence of the disease.

[Dysgenetic male pseudohermaphroditism]. / Dysgeneticus pseudohermaphroditismus masculinus.

The authors report a case of a dysgenetic male pseudohermaphroditism with a 45,X/46,XY karyotype in a mosaic form, which was diagnosed in an infant. The one-week-old infant was evaluated because of proximal hypospadias and retention of the right testis. The results of hormonal tests were the followings: serum FSH 5.2 mU/ml; LH: 2.0 mU/ml; testosterone: 144.3 ng/dl; androstendione: 0.42 µg/l; 17-hydroxyprogesterone: 1.12 ng/ml. Chromosomal analysis revealed 45,X/46,XY karyotype. Fluorescent in vitro hybridization showed that 51% of the lymphocytes had the Y chromosome and the SRY gene. Analysis of the SRY showed no deletion in the AZF a,b,c regions. Pelvic magnetic resonance imaging indicated the presence of vagina between the bladder and the rectum, and it showed a mass measuring 15×8 mm in the right inguinal canal as well as an oval gonadal mass with a size of 13×7 mm in the left scrotum. During surgical intervention, performed at the age of one, the right gonad was removed and biopsy of the scrotal testis was performed. Histological examination revealed dysgenetic testis in both sides. The authors emphasize the necessity of cytogenetic and endocrinological investigations of newborns with perineoscrotal hypospadia and bilateral or unilateral maldescent testes immediately after birth. Surgical removal of the dysgenetic testicular tissue located in the abdominal cavity and its histological evaluation provides separation of mixed gonadal dysgenesis, dysgenetic male pseudohermaphroditism, bilateral gonadal dysgenesis and ovotestis in the 45,X/46,XY mosaic cases. An accurate evaluation is necessary for a correct sex assignment and for surgical intervention to prevent neoplastic degeneration of the dysgenetic gonad.

[Pure 46,XY gonadal dysgenesis]. / 46,XY tiszta gonáddiszgenezis.

The authors report a rare case of pure 46,XY gonadal dysgenesis (Swyer syndrome). Swyer syndrome is associated with 46,XY karyotype, primary amenorrhea as well as the presence of female internal genital tract and bilateral streak gonads in a phenotypic female. The genetic background of this syndrome includes mutations of several genes involved in the testis differentiation cascade. Mutation of the SRY gene accounts for only 10-15% of all 46,XY gonadal dysgenesis cases while the majority cases may be linked to other deficient genes involved in the sex differentiation pathway. The patient was a 16-year-old female who was referred for endocrinological evaluation because of primary amenorrhea. Physical examination revealed a phenotypic female, height 166 cm, weight: 56.5 kg, breast and pubic hair development were Tanner I. and II, respectively. She had female external genitalia. Pelvic magnetic resonance imaging showed a hypoplastic uterus and ovaries at both sides measuring 5×10 mm in size. Chromosomal analysis revealed 46,XY karyotype. Analysis of the SRY and SF1 genes showed no mutations. Serum follicle-stimulating hormone and luteinizing hormone were elevated. Serum tumor marker concentrations were normal. Prophylactic bilateral gonadectomy was performed and histological examination showed bilateral streak gonads. Hormone replacement therapy produced development of secondary sexual characters and 1.5 years after treatment the patient had menarche. Authors conclude that karyotype analysis should be performed in adolescent with primary amenorrhea. After establishment of the diagnosis, dysgenetic gonads should be removed because of the high risk of gonadal neoplasia.

Severe dystrophy in DiGeorge syndrome.

We present the case history of a 3-year-old girl who was examined because of severe dystrophy. In the background, cow's milk allergy was found, but her body weight was unchanged after eliminating milk from her diet. Other types of malabsorption were excluded. Based on nasal regurgitation and facial dysmorphisms, the possibility of DiGeorge syndrome was suspected and was confirmed by fluorescence in situ hybridization. The authors suggest a new feature associated with DiGeorge syndrome.

[Mixed gonadal dysgenesis associated with an isodicentric Y chromosome]. / Izodicentrikus Y-kromoszómához társuló kevert gonáddiszgenezis.

Authors report a case of mixed gonadal dysgenesis with a karyotype containing an isodicentric Y chromosome in mosaic form, which was diagnosed in an infant. They emphasize the necessity of the special investigations of newborn with perineoscrotal hypospadia and bilateral or unilateral maldescent testes immediately after birth. The result of accurate evaluation provides correct sex assignment and the prevention of the neoplastic degeneration of a dysgenetic gonad.

Extralobar pulmonary sequestration in the right upper thoracic region.

We present an extremely rare case of extralobar pulmonary sequestration in the right upper thoracic region, with a wide tracheal communication and a right subclavian arterial blood supply. The MRI scan suggested a bronchogenic cyst. Although preoperative color Doppler and angiography were not performed, successful resection was carried out. The histology indicated a diagnosis of extralobar pulmonary sequestration.