72 research outputs found
Situs inversus viscerum and renal agenesis in a newborn
Situs inversus viscerum is a rare congenital anomaly (incidence 1/15–20 000) in which organs are mirrored from their normal positions. It is called situs inversus viscerum totalis when there is a total transposition of abdominal and thoracic viscera. Incomplete situs inversus viscerum involves transposition only of abdominal organs and is more frequently associated with congenital cardiac defects (90%–95% vs 5%–10% in situs inversus totalis). About 25% of individuals with situs inversus have an underlying condition known as Kartagener Syndrome. As a result of benign pathology, patients with situs inversus viscerum can live normal healthy lives. Situs viscerum inversus can be associated with defects in various organ systems (respiratory, GI tract, genitourinary). In literature there’s evidence of cases of situs inversus associated with renal malformations (dysplasia, hypoplasia, ectopia, polycystic kidney, horseshoe kidney). Association with renal agenesis is rare. We report the case of S., born at 40 weeks, eutocic delivery. APGAR 8/9, weight 3350 g. Normal fetal US. In the third day of life, heart sounds better heard on the right side of the chest. Cardiac US: situs inversus viscerum, dextrocardia, subaortic stenosis, restrictive ventricular septal defect, atrial septal defect ostium secundum. In the fourth day of life, abdominal US findings were suggestive of right renal agenesis. In the 20th day of life, right reducible inguinal hernia. The little patient underwent cystography, negative for vesicoureteral reflux, and static renal scintigraphy with evidence of absent activity in right renal space. Renal function, cerebral US, audiologial assessment and karyotype were normal. No respiratory distress reported. Mucociliary clearance test and a regular follow-up are scheduled
Sifilide congenita: causa di grave sofferenza perinatale
Si evidenziano i segni e sintomi clinici e soprattutto l'importanza di verificare i fattori di rischio per riconoscere e diagnosticare correttamente una infezione da spirocheta causa di sifilide congenita nel neonato, come patologia riemergente
A case of allergic enterocolitis to milk protein in a down baby
Anna, a term baby born by Caesarean section, firstborn, Apgar 8/9, born SGA 2.630 g weight; performed in karyotype of Down syndrome suspected ’free trisomy of chromosome 21’, echocardiography and abdominal ultrasound normal, ABR REFER, discharged in 8th day of life with diet of mother’s milk integrated with zero infant formula.
Hospitalisation at one month of life due to serious clinical conditions (dehydration, hypovolemic shock, metabolic acidosis, PCR 67,4 mg/L, PCT 4,45 ng/ml, leukocytosis 63870/microL, weight 2447 g); negative blood, liquor, urine and stool cultures; Chest and Abdomen X-ray showed meteoric distension of the intestinal loops. She began antibiotic (Ampicillin+Sulbactam, Netilmicin, Ceftazidime) and immunoglobulin therapy with improvement and reduction of inflammatory markers; feeding with zero infant formula.
After the fourth day of hospitalisation spontaneous emission of greenish bloody stools and then liquid stools, hypotonus, metabolic acidosis, bulbous abdomen, fever, increased inflammatory markers and leukocytosis occurred despite antibiotic therapy. New negatives cultures tested, negative parasitological faecal examination, positive faecal calprotectin (702.59 mg/kg); lymphocyte subpopulations and normal immunoglobulins. Anaemia with need of blood transfusion. Therapy replaced with Meropenem and Vancomycin and diet with hydrolyzed infant formula with slight improvements.
The appearance of two similar to previous episodes in the following days suggested to treat with antibiotics and immunoglobulins. After gastroenterological consulting, in allergic enterocolitis to milk protein suspect, it replaced diet with hydrolyzed infant formula with amino acids. The following days progressive clinical improvement noted, weight increase, normal inflammatory markers, good conditions and subsequently discharge. Three weeks later, during medical check-up, she appeared in good clinical condition, increase in weight of 650
Unilateral renal agenesis: three case reports
Unilateral renal agenesis (incidence of 1/500–1000 newborns), can be isolated or associated with other urological/extra-urological abnormalities. Unilateral renal agenesis (URA) may be suspected after a renal US and confirmed by static renal scintigraphy. We describe 3 cases among the 1299 children born in our Centre in the first half of 2015. S., born at 40 weeks, eutocic delivery. APGAR 8/9, weight 3350 g. In the third day of life a diagnosis of situs inversus viscerum totalis, subaortic stenosis, restrictive ventricular septal defect and atrial septal defect ostium secundum has been made. In the fourth day of life, abdominal US findings were suggestive of right renal agenesis. The patient underwent cystography, negative for reflux, and static renal scintigraphy which confirmed diagnosis. Renal function, cerebral US, audiologial assessment and karyotype were normal. M., born at 27,5 weeks by C-section, APGAR 6/8, weight 1240 g. Mother with gestational diabetes. In the first day of life, patent ductus arteriosus and patent foramen ovale were detected. After 2 months US before discharge revealed right renal agenesis. Cystography was negative and renal scintigraphy confirmed diagnosis. B., born at 34,6 weeks by eutocic delivery. APGAR 7/8, weight 2240 g. As an imperforate anus was detected, the patient underwent renal US which revealed pyelectasis and empty left renal space. Diagnostic management was incomplete because the patient was transported to another Health Care Centre
Occasional diagnosis in newborn of systemic venous return with unexpected persistence of vena cava upper left and absence of vena cava superior right – case report
Congenital abnormalities of the systemic venous return (SVR) are rare, most often asymptomatic and discovered occasionally in the course of imaging studies. The persistence of the left superior vena cava (PLSVC) is the most common thoracic venous anomaly. The prevalence is 0.1%–0.3% in the general population, more common in individuals with congenital heart anomalies (CHA). The PLSVC with the absence of the right superior vena cava (ARSVC) is extremely rare, with a prevalence of 0.09% to 0.13% in patients with other CHA. The PLSVC is given by the persistence of the Marshall vein, counterpart of the superior vena cava in the early stages of embryonic development, which usually regresses during intrauterine development. The PLSVC determines venous return into the right atrium through the coronary sinus (CS), consequently dilated. Patients with abnormal SVR are at greater risk of developing arrhythmias due to possible aberrations in the cardiac conduction tissue in the early stages of development. In fact, the conduction tissue originates from two sites close to the superior vena cava ancestors. Furthermore, arrhythmias could result from structural abnormalities, such as right atrial dilation or CS dilation. We report the clinical case of M.C., female, premature of 33 weeks, birth weight 2450 g, APGAR score 8–9, silent prenatal history. On the second day of life, an echocardiography was performed, since a systolic murmur 1/6 in mesocardium was noticed. In addition to a restrictive interventricular muscle defect, the examination showed the PLSVC with a significant increase of the CS (5 mm), draining into the right atrium, and the ARSVC. During hospitalisation M.C. was subjected to electrocardiogram, oxygen saturation monitoring and heart rate, resulted in the normal ranges. M.C. was discharged on the 12th day of life in good general conditions with a cardiology follow up planned at 1 year of age
Toxoplasmosi congenita: mai abbassare la guardia
Descrizione dell'importanza di un corretto screening per T.Gondi in gravidanza ed una precoce diagnosi feto-neonatale per la corretta identificazione e gestione della Toxoplasmosi congenita
Also adipose tissue pays consequencies of perinatal asphyxia
D.R. is born at term of physiological pregnancy by caesarean section. Birth weight was 3520 g, APGAR 7/9. At birth he presented axial hypotonia, hyporeactivity, groan, mild respiratory distress. Started Non Invasive Ventilation for 2 days with good improvement of outcome.
At 4 day life, we started antimicrobial therapy and immunoglobulins ev because of elevated inflammatory markers and low platelet counts (PLT 47000/mcl) confirmed at the blood smear.
After 2 weeks, on the neck and on the right cheek, finding of erythematous nodules of a few millimetres of diameter. In the days after, the lesions are also extended to the left cheek and became larger up to about 3 cm.
At the ultrasound they appeared as ‘delimited echogenic areas of 19 × 14 mm at left, 17 × 12 mm at right whit intrinsic vascularisation and of irregular structure. Laboratory pannels (hepatic, renal, coltural exams, procalcitonin) were negative, ESR 24 mm/h, CRP 23.6 mg/L.
About 30 days after, we see a reduction of the lesions in spite of a persistent increase of inflammatory markers. Therefore, a bacterial aetiology appeared ulikely saw that antimicrobial therapy so soon established, had no effects on them.
Subcutaneous fat necrosis of the newborn is a relatively rare and transient condition that appeared in the first weeks of life in term infants with a perinatal suffering.
This condition is caractherized by single or multiple subcutaneous nodules, isolated or confluent in plaques, erythematous/purplish, sometimes with little depressions, calcifications or necrosis inside; symmetrically distributed on the back, shoulders, buttocks, cheeks and at the root of the limbs; often painful at palpation. These nodules grow for some weeks and completely resolve within few months.
Etiopathogenesis is probably linked to ischaemic injury, hypoxia, hypothermia and/or stress damage on the immature adipose tissue in infants with perinatal asphyxia.
Maternal hypertension, gestational diabetes, family history of thrombophilia and dyslipidemia are considered risk factors. The diagnosis is clinical; the cutaneous biopsy is of support. Important for diagnosis are also the Ultrasonography and RMN.
Hypercalcemia is the most dangerous complication; less frequent consequencies are thrombocytopenia, metabolic disorders (hypertriglyceridemia, hypoglycemia) and atrophic development of the nodular lesions
Workflow Pattern Analysis in Web Services Orchestration: the BPEL4WS Example
The work of this paper intends to analyze in-depth the Business Process Execution Language for Web Services (BPEL4WS), presenting a new methodology used to state in a formal way which workflow patterns can be executed by using the BPEL4WS constructs
Structural Performance-Based Design Optimisation of a Secondary Mirror for a Concentrated Solar Power (CSP) Plant
Concentrated Solar Power (CSP) plants use mirrors to reflect and concentrate sunlight onto a receiver, to heat a fluid and store thermal energy, at high temperature and energy density, to produce dispatchable heat and/or electricity. The secondary mirror is a critical component in the optical system of certain Solar Power Tower plants (SPT), as it redirects the concentrated sunlight from the primary mirror onto the receiver, which can be arranged at ground level. In this study, we propose a design optimisation for the secondary mirror of a CSP plant. The design optimisation method consists of two steps. The first step involves the use of the finite element simulation software Abaqus 2022 to analyse the structural performance of the secondary mirror under thermal loads and wind. The second step consists of the use of simulation results to identify the combination of design parameters and best performances, with respect to both design constraints and structural safety. This is carried out by developing an algorithm that selects those configurations which satisfy the constraints by using safety coefficients. The proposed optimisation method is applied to the design of a potential configuration of a secondary mirror for the beam-down of the CSP Magaldi STEM® technology, although the methodology can be extended to other components of CSP plants, such as primary mirrors and receivers, to further enhance the structural performance of these systems
Lengua y derechos lingüísticos en Arán: régimen jurídico de la oficialidad del aranés
This work deals with the legal framework of the official status of Aranese language within the territory of Aran and in the rest of Catalonia. For that purpose, the author explains, firstly, which has been the path followed until the full recognition of the said language as an official language, which took place with the adoption of the Statute of Autonomy of Catalonia in 2006. Secondly, the author analyses how the ordinary Catalan legislator has developed the statutory provisions regarding the official status of the Aranese language. In that regard, it has made a clear distinction between the effects of such declaration in the territory of Aran and in the rest of Catalonia, but outside Aran. Finally, the paper also deals with the preferential use of Aranese language, established in the Catalan Law on Aranese language, and questions its conformity with the Spanish Constitution.El presente estudio analiza de forma sistemática y detallada el régimen jurídico de la oficialidad del aranés en Arán y en la Comunidad Autónoma de Cataluña. Para ello, expone, en primer lugar, cuál ha sido la evolución normativa hasta llegar a la declaración de oficialidad en el Estatuto Autónomo de Cataluña de 2006. Seguidamente, se estudia cómo se ha desarrollado este mandato estatutario de oficialidad del aranés, tanto en el ámbito territorial de Arán como en el resto del territorio de Cataluña. Finalmente, se analiza también la regulación establecida en relación con el uso preferente del aranés en el ámbito institucional y público y su encaje en el modelo lingüístico constitucional
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