Quantification of liver herniation in fetuses with isolated congenital diaphragmatic hernia using two-dimensional ultrasonography.

OBJECTIVES: To describe a method of quantifying the amount of liver herniation in fetuses with isolated congenital diaphragmatic hernia (CDH) using two-dimensional ultrasonography and to correlate this finding with neonatal outcome. METHODS: Ultrasound images obtained from 77 consecutive fetuses that presented with isolated CDH between January 2004 and July 2012 were reviewed. Liver herniation and thoracic area were measured in a cross-sectional plane of the fetal chest at the level of the four-chamber view of the heart (the same section as is used to measure the lung area-to-head circumference ratio) and the ultrasound-derived liver-to-thoracic area ratio (US-LiTR) was calculated by dividing the liver herniation area by the thoracic area. Receiver-operating characteristics (ROC) curve analysis was used to evaluate the performance of US-LiTR in predicting neonatal outcome (survival to 6 months after delivery and need for extracorporeal membrane oxygenation (ECMO)). In addition, the US-LiTR was compared with the magnetic resonance imaging (MRI)-derived volume ratio (MRI-LiTR) and percentage of liver herniation (MRI-%LH). RESULTS: The overall neonatal mortality in the 77 cases with isolated CDH was 20.8% (16/77). ECMO was needed in 35.5% (27/76) of the newborns, with a survival rate of 52%. The US-LiTR was associated statistically with mortality (P < 0.01) and with the need for ECMO (P < 0.01). Good correlations were observed between US-LiTR and MRI-LiTR (r = 0.87; P < 0.001) and between US-LiTR and MRI-%LH (r = 0.90; P < 0.001). Based on ROC curve analysis, all three parameters had similar accuracy in predicting mortality (US-LiTR: area under the ROC curve (AUC), 0.78 (95% CI, 0.65-0.92), P < 0.01; MRI-LiTR: AUC, 0.77 (95% CI, 0.63-0.90), P < 0.01; MRI-%LH: AUC, 0.79 (95% CI, 0.65-0.92), P < 0.01, respectively) as well as the need for ECMO (US-LiTR: AUC, 0.72 (95% CI, 0.60-0.84), P < 0.01; MRI-LiTR: AUC, 0.73 (95% CI, 0.60-0.88), P < 0.01; MRI-%LH: AUC, 0.77 (95% CI, 0.64-0.89), P < 0.01, respectively). CONCLUSIONS: Two-dimensional ultrasound measurement of the amount of liver herniation in fetuses with isolated CDH is feasible and demonstrates a predictive accuracy for neonatal outcome similar to that of MRI.

Congenital heart anomaly in newborns with congenital diaphragmatic hernia: a single-center experience.

OBJECTIVE: To evaluate the impact of the presence of a congenital heart anomaly (CHA) and its potential contribution to morbidity and mortality in infants with congenital diaphragmatic hernia (CDH). METHODS: In this retrospective cohort study, prenatal and postnatal data of all newborns diagnosed with CDH between January 2004 and December 2012 in a single center were reviewed. Cases were classified into two groups: those with 'isolated' CDH and those with both CDH and CHA. Patients with CHA were further subclassified into those with a major or minor CHA based on the Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1), and the Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery (STS-EACTS) scoring systems. Patients with associated non-cardiac anomalies, including 'syndromic cases', were excluded from the analysis. Primary and secondary outcomes were survival up to 1 year of age and a need for extracorporeal membrane oxygenation (ECMO), respectively. RESULTS: Of the 180 infants with CDH, 41 were excluded because of the presence of non-cardiac associated anomalies, 118 had isolated CDH and 21 had CDH with CHA (16 with minor and five with major CHA). Receiver-operating characteristics curve analysis demonstrated that the best cut-off for survival was when the score for CHA was ≤ 2 for both RACHS-1 (area under the curve (AUC), 0.74 (P = 0.04); sensitivity, 80.0%; specificity, 87.5%) and STS-EACTS (AUC, 0.83 (P = 0.03); sensitivity, 100%; specificity, 87.5%). Survival rate at 1 year was significantly lower in those with CHD and a major CHA (40.0%; P = 0.04) than in those with isolated CDH (77.1%) and those with CDH and a minor CHA (81.3%). We found no significant differences among the groups with regard to the need for ECMO. CONCLUSIONS: In general, a milder form of CHA does not appear to have a negative impact on the survival of infants with CDH. However, mortality appears to be significantly higher in infants with CDH and a major form of CHA. The scoring systems appear to be useful as predictors for classifying the effects of CHA in this population of patients.

Fetal lung volume and quantification of liver herniation by magnetic resonance imaging in isolated congenital diaphragmatic hernia.

OBJECTIVE: To determine associations between fetal lung and liver herniation volumes measured by magnetic resonance imaging (MRI) and mortality/need for extracorporeal membrane oxygenation (ECMO) in cases of isolated congenital diaphragmatic hernia (CDH). A secondary objective was to compare prenatal MRI parameters with two-dimensional ultrasound lung measurements. METHODS: A retrospective review of medical records of all fetuses with isolated CDH evaluated between January 2004 and July 2012 was performed. The following MRI parameters were measured at 20-32 weeks: observed/expected total fetal lung volume (o/e-TLV), predicted pulmonary volume (PPV), percentage of liver herniated into the fetal thorax (%LH) and the liver/thoracic volume ratio (LiTR). These were compared with the ultrasound-determined lung-to-head ratio (LHR) and the observed/expected LHR (o/e-LHR) in the same cohort. The predictive value of MRI and ultrasound parameters for mortality and the need for ECMO was evaluated by univariate, multivariate and factor analysis and by receiver-operating characteristics curves. RESULTS: Eighty fetuses with isolated CDH were evaluated. Overall mortality was 18/80 (22.5%). Two newborns died a few hours after birth. ECMO was performed in 29/78 (37.2%) newborns, with a survival rate of 48.3% (14/29). The side of the diaphragmatic defect was not associated with mortality (P = 0.99) or the need for ECMO (P = 0.48). Good correlation was observed among o/e-TLV, PPV, LHR and o/e-LHR as well as between %LH and LiTR (r = 0.89; P < 0.01); however, fetal lung measurements and measures of liver herniation were not correlated (all P > 0.05). All parameters were statistically associated with mortality or the need for ECMO. The best combination of measurements to predict mortality was o/e-TLV and %LH, with 83% accuracy. CONCLUSION: Mortality and the need for ECMO in neonates with isolated CDH can be best predicted using a combination of MRI o/e-TLV and %LH.

Evaluating executive function in schoolchildren with symptoms of attention deficit hyperactivity disorder.

OBJECTIVE: To identify impairment of executive functions (EF) in children with attention deficit hyperactivity disorder (ADHD). SUBJECTS AND METHODS: A case-control study was performed on a sample of schoolchildren with low socioeconomic levels in Bogota, Colombia. ADHD was diagnosed using the DSM IV checklist and the Behavior Assessment System for Children scale. Children with cognitive deficits were excluded. We evaluated scores from six measurements of executive function (EF). We conducted a bivariate statistical analysis to compare the variables, a multivariate study controlled by sex and age, and a logistic regression analysis. RESULTS: The study sample included 119 children with ADHD and 85 controls, all aged between 6 and 12 years. Controlling by sex, age, and type of school showed that EF measurements in children with ADHD were significantly more impaired than in controls, especially for measurements of verbal and graphic fluency, Rey-Osterrieth Complex Figure, and cognitive flexibility. Comparison of ADHD subgroups showed that results in children with multiple deficits were similar to those in the global ADHD group. Graphic fluency impairment was the sole impairment in cases with only attention deficit or only hyperactivity-impulsivity manifestations. CONCLUSIONS: EF measures in children with ADHD revealed more problems, particularly those having to do within planning, inhibition, working memory and cognitive control. Age and sex may affect the degree of EF impairment.

[Early physiotherapy in lower trapezius transfer for massive and irreparable rotator cuff injuries. A case report]. / Fisioterapia precoz en transferencia del trapecio inferior para lesiones masivas e irreparables del manguito rotador. A propósito de un caso clínico.

Massive and irreparable lesions of the rotator cuff are frequent between people over 60 years old. Recently, inferior trapezium transference with Achilles tendon allograft has demonstrated very good results to restore shoulder's function. For this kind of surgery, it is recommended an absolute immobilization in shoulder abduction at 90° and maximal external rotation between 6-8 weeks. A 57-year-old woman underwent surgery with a lower trapezius transfer to repair the rotator cuff due to a massive and irreparable tear. She started aquatic physiotherapy 15 days after surgery and conventional physiotherapy treatment at 25 days, with a follow-up of 12 months. Good functional results of the operated shoulder were obtained, which were maintained in the postoperative follow-up. In this case early physiotherapy did not result in any risk of suture dehiscence, loosen or distressing of the allograft.

Diagnosis of chikungunya virus infection in Baja California Sur, Mexico.

Chikungunya virus infection had not been reported by the National Secretary of Health of the State of Baja California Sur, Mexico before 2015, and until now, no information of the disease has been published. Thus, the objective of this study was to assess the tendency that the disease has shown since its introduction. The total number of cases in the state was analyzed, and the incidence rate of infection was evaluated in the general population, age groups, and gender. From 2015-2019, the year with the highest number of reported cases was 2016 with 210 cases, but no cases were reported from 2018-2019. The gender with the highest number of cases was female. The most affected age group was adults 45-64 years of age.

Renal donor implication in the origin of BK infection: analysis of genomic viral subtypes.

UNLABELLED: BK virus (BKV) reactivation in immunocompromised kidney transplant patients can produce a tubulointerstitial nephropathy (BKVN). Molecular tools that test for DNA-BKV provide early detection and assist in management, but some aspects of the pathogenesis of this infection, such as donor causality, remain unclear. MATERIALS AND METHODS: Between November 2004 and January 2006, 55 Spanish kidney donors were studied for BK infection. A quantitative PCR assay was performed on urine and serum to detect BKV. To determine the origin of the viral infection, a transcription control region of the BK polymorphism sequence was designed to identify the viral subtype. RESULTS: Fifteen of 55 (27%) donors were BK-PCR positive: 13 in urine and 2 in serum and urine. Moreover, monitoring of recipient pairs detected BK-PCR positivity in 14 of 73 recipients. We studied eight BK-PCR positive recipients (corresponding to four pairs) and their respective donors. The same viral genome was observed in the four pairs, namely, the A250-1-a, WW-like, AS, and JL genotypes. Interestingly, one of the four pairs showed the donor and the two recipients to display exactly the same JL genotype. CONCLUSION: On the basis of our preliminary results analyzing the molecular fingerprints of donor and recipient pairs, we have presented new data implicating the donor, in at least some cases, as the source of BK infection.

Polymerase chain reaction detection of BK virus and monitoring of BK nephropathy in renal transplant recipients at the University Hospital La Fe.

INTRODUCTION: Reactivation of BK infection occurs in immunocompromised hosts causing tubulointerstitial nephropathy (BKVN). Approximately 5% of kidney transplant recipients (KTR) develop BKVN, special half of whom lose their grafts. However, BKVN morphologic diagnosis on a renal biopsy is complicated, because the cytopathic changes can sometimes mimic rejection. Thus, BKV DNA-polymerase chain reaction (PCR) assay on serum, urine, and renal tissue is useful for early detection and monitoring of BKV. MATERIALS AND METHODS: We performed routine monthly urine cytologies looking for decoy cells as a marker of virus replication. Then, we performed a qualitative PCR on urine and serum in all recipients (independently of positive or negative cytology). We amplified 3 BK viral genome regions, LT (early transcription region) and VP1 (late transcription region) seeking a more accurate virus detection, and the TCR (control transcription region) region to perform a polymorphism sequence analysis to identify the BK genomic variant. Finally, the BKVN diagnosis was confirmed using renal biopsy. RESULTS: At present, 132 patients have been monitored. Thirteen of 40 (33%) were PCR-urine-positive cases (5 LT+/VP1- and 8 LT+/VP1+), and 10 of 132 (7.5%) were PCR-serum-positive cases (7 LT+/VP1- and 3 LT+/VP1+). When we compared PCR-urine and cytology results, 11 of 40 (27.5%) patients showed a positive cytology, 6 of whom were PCR- urine-positive (1 LT+/VP1- and 5 LT+/VP1+); whereas, 29 patients showed a negative cytology, 7 of whom were PCR-urine-positive(3 LT+/VP1- and 4 LT+/VP1+). Thus, comparison of PCR- urine and cytology results revealed false-positive and false-negative cases. Finally, TCR sequence analysis was performed in 9 patients to identify the BK genomic variants. CONCLUSION: Testing for BKV DNA in urine and serum is a noninvasive early detection assay and monitoring tool.

Analysis of selective regions in the active sites of human cytochromes P450, 2C8, 2C9, 2C18, and 2C19 homology models using GRID/CPCA.

This study demonstrates a selectivity analysis using the GRID/CPCA strategy on four human cytochrome P450 2C homology models (CYP2C8, 2C9, 2C18, and 2C19). Although the four enzymes share more than 80% amino acid sequence identity, the substrate specificity differs. To investigate the selectivity of the enzymes and the amino acids that determine the specificity of each CYP2C enzyme, a selectivity analysis was made using GRID/CPCA. In the GRID calculations 10 probes were used covering hydrophobic, steric, and hydrogen bond acceptor and donor interactions. The selectivity analysis showed that the most important determinants of selectivity among the CYP2C models are the geometrical features of the active sites and the hydrophobic interactions. The selectivity analysis singled out CYP2C8 as the most different of the four CYP2C enzymes with amino acids with distinct properties in positions 114, 205, and 476 (Ser, Phe, and Ile, respectively) compared to the other enzymes. An inverse pharmacophore model for CYP2C9 was constructed from the selective regions, and the model agreed with the docking of diclofenac where the properties of the ligand overlapped with the pharmacophoric points in the model.

Premature cataracts in a family with hidrotic ectodermal dysplasia.

In a family with hidrotic ectodermal dysplasia affecting five members in three generations, bilateral premature cataracts have developed in four of the five affected individuals. To our knowledge, this represents the first report of a family in which bilateral premature cataracts appear to be inherited with hidrotic ectodermal dysplasia.

Synthesis and molecular modeling: related approaches to progress in brassinosteroid research.

In the field of brassinosteroids, which are potent plant growth regulators, we have developed a quantitative structure-activity relationship study to develop knowledge from a structural point of view and to find out new requirement definitions. This will help identify other suitable active brassinosteroid derivatives with a good activity/synthetic cost ratio for further application in agriculture. The methodology used to achieve this goal represents a multidisciplinary study involving synthesis, molecular modeling calculations, and bioactivity evaluation. The influence of different molecular properties in the bioactivity of a set of synthetic compounds (i.e., molecular electrostatic potential and the ability to form H bonds) is discussed. The molecular electrostatic potential is expressed in terms of the electrostatic Carbó similarity index (CI) between brassinolide (1) and other brassinosteroids. We have found that the electrostatic charges of the functional groups play an important role in the description of the activity, as evidenced by its good correlation with the CI in most cases. Deviation from this rule could be explained by the H bonding abilities of some of these compounds, which we believe may play an essential role in binding to the natural receptors.

Congenital thymic cyst in the neck.

Cervical thymic cysts are rare. Thirty-four cases are reported in the English literature. Persistence of a part of thymopharyngeal duct may lead to the cyst formation in the neck. The majority of the cysts are seen in children under 10 years of age. There is marked variation in the size of the cysts. The majority of them are multilocular and the color of the fluid varies widely. The epithelial lining is variable; the majority of the cysts show stratified squamous epithelium. The characteristic histological features are Hassal's corpuscles, cholesterol crystals and aggregates of lymphocytes. Our patient was a four and a half year old female; she presented with a four month history of a painless swelling in the neck. The multilocular bluish-black cyst excised from the left side of the neck had all the histological features of a thymic cyst. A pre-operative diagnosis of such a cyst can be made only by bearing in mind the possibility of thymic remnants in the neck.

[Mesangial glomerulonephritis and intermediate uveitis]. / Glomerulonefritis mesangial asociada a uveítis intermedia (pars planitis).

Uveitis in children are less frequent than in adults. Their prognosis is variable because it may be found as an isolated and idiophatic condition or in association with definite clinical entities. The associated noninfectious diseases with predominantly renal involvement are tubulointerstitial nephritis and uveitis syndrome (TINU syndrome), mesangial glomerulonephritis isolated or in association with Behçet's disease. A case of 14-years-old girl with intermediate uveitis (pars planitis) and mesangial glomerulonephritis is presented. The ocular symptoms was eye redness and ocular pain and she has snow-banks in pars plana. She showed microscopic hematuria and intermitent proteinuria that increased during the ocular clinical exacerbation. Renal biopsy revealed both mild mesangial matrix increase and mesangial celullarity with normal tubulointerstitial structure and mesangial deposition of IgA and IgG immunoglobulins. This case is de first pediatric patient report in the literature with intermediate uveitis and mesangial glomerulonephritis with immune deposition. Mesangial glomerulonephritis were observed in patients whit Behçet disease, known etiological cause of uveitis in adults and children. These findings may suggest that uveitis and glomerulonephritis have common immunological pathogenesis including circulatory immune complexes. In uveitis patients, screening for associated extra-ocular and renal manifestations is mandatory and should have careful long-term follow-up with regular systemic evaluation.

[Glomerulocystic kidney disease and hemolytic-uremic syndrome: clinicopathological case]. / Enfermedad glomeruloquística renal y síndrome hemolítico-urémico: observación clinicopatológica.

Glomerulocystic kidney is a heterogeneous group of conditions morphologically characterised by multiple cortical cysts apparently originated from a cystic dilation of the filtration space with atrophy of the glomerular tufts. We report a case of glomerulocystic kidney affecting a 13-year-old boy who underwent renal transplantation for end-stage renal disease following a haemolytic-uraemic syndrome diagnosed nine years ago. The absence of other stigmas (urinary obstruction, extrarenal congenital abnormalities and family history of cystic kidney disease) suggest that our observation is apparently a sporadic and acquired glomerulocystic kidney following a haemolytic-uraemic syndrome, an infrequent association previously reported only twice. Our histological and immunohistochemical findings suggest that the cysts in this rare condition are really of glomerular origin but the pathogenesis of cyst development remains unknown.

[Cytomegalovirus nephrotic syndrome]. / Síndrome nefrótico infantil por citomegalovirus.

The case of a 5 months old infant with a nephrotic syndrome after neonatal cytomegalovirus infection is reported. Genomic amplification nested-PCR for CMV was positive in renal biopsy. Treatment with gancyclovir was effective to maintain nephrotic syndrome remission. We stresses the importance to discharge an infections cause of the nephrotic syndrome of newborns and infants due to the possibility on curative treatment.

[Anti-CD25 monoclonal antibody against polyclonal antibodies in pediatric renal transplantation]. / Anticuerpo monoclonal anti-CD25 frente a anticuerpos policlonales en el trasplante renal pediátrico.

UNLABELLED: Although usually reversible, acute rejection of kidney graft is a negative factor in long-term graft survival. Commonly used in pediatric renal transplantation, immunosuppresive induction therapy is established to prevent it. New immunosuppressive agents have been developed in recent years and among them anti-CD25 monoclonal antibody appears to be specially interesting. AIM: To evaluate efficacy and safety of anti-CD325 monoclonal antibody (basiliximab) versus polyclonal antibodies as induction therapy in renal transplantation. MATERIAL AND METHODS: Thirty consecutive kidney transplants performed in children 4-16 years age in Hospital Infantil La Fe through 1997-2000. The first 15 patients received polyclonal antibodies as induction therapy, and 15 consecutive ones received monoclonal anti-CD25 antibodies. Receptor, donor and graft characteristics were similar in both groups. Also, maintenance immunosuppression was the same. RESULTS: The follow-up was over one year in all patients. Four patients in the polyclonal antibody group suffered one acute rejection episode and four other patients had some drug reaction. In the anti-CD25 treatment group there was one episode of acute graft rejection and no collateral effects were observed. Glomerular filtration rate, proteinuria, hypertension, infection episodes, graft and patient survival were similar in both groups. CONCLUSIONS: Induction therapy for pediatric renal transplantation with anti-CD25 antibody has been effective and safe. Compared with polyclonal antibodies as standard treatment, basiliximab reduced acute rejection episodes and had no collateral side effects. Graft and patient one year survival were identical in the two groups.

[Menarche, menstrual and sociodemographic characteristics of Puerto Rican female athletes in the XV and XVI Central American and Caribbean Games]. / Menarquia, características menstruales y sociodemográficas de mujeres atletas puertorriqueñas en los XV y XVI Juegos Centroamericanos y del Caribe.

UNLABELLED: This investigation described maturation, menstrual and socio-demographic characteristics of 65 Puerto Rican women athletes that were interviewed during the XVI Central American and Caribbean Games (CACG), Mexico City in 1990. The results were compared with those of Puerto Rican women athletes (n = 52) at the XV CACG, Santiago Dominican Republic, 1986. The quantitative variables (age, age at initiation of training, years of training, age at menarche, birth order, and family size) were not statistically different (t-independent, p > or = 0.05). The observed frequencies for the qualitative variables (menstrual characteristics, degree of certainty in the recall of age of menarche, use of oral contraceptives, and marital status) were very similar. IN CONCLUSION: the women at the XVI CAC in Mexico demonstrated similar maturational, menstrual and socio-demographic characteristics to the those athletes evaluated four years earlier in Santiago and based on their long history of training, both samples were representative of athletically mature athletes. The findings were very similar to those reported for olympic athletes and such data expands the available information on Puerto Rican women athletes.

[Diagnostic and therapeutic problems in mucocele]. / Problemas diagnósticos y terapéuticos en los mucoceles.

As mucoceles are manifested usually during the exteriorization phase by compression of surrouding structures, the patient often consults other specialists before seeing an ear nose and throat specialist. We report the case of a female patient first seen in the neurology and ophthalmology departments. The diagnostic procedures and treatment protocols are analyzed. Endoscopic surgery is a good option that requires adequate patient selection.

[Solitary extramedullary plasmacytoma of the larynx. Apropos of a case]. / Plasmocitoma extramedular solitario de laringe. A propósito de un caso.

The present paper shows a case of solitary extramedullary plasmocytoma of the larynx. It is important to know the limits of this process in order to rule out a multiple myeloma. After a literature review, emphasis is made in the necessity of a very long term patient's follow up, since recurrences may occur some years after the initial diagnostic.

[Relapsing polychondritis. Apropos of a case]. / Policondritis recidivante. A propósito de un caso.

Relapsing polychondritis is not a very frequent systemic disease. Nevertheless, it has to be borne in mind since very often the first symptoms appear in the sphere of otorhinolaryngology. We are faced with the problem of differential diagnosis in a case which in the beginning seemed to be a neurological pattern. The definitive diagnosis was not established until 15 months after the appearance of the symptoms when, after several outbreaks, the clinical pattern was clearer. As we have to deal with a recurrent and systemic pathology whose diagnosis is eminently clinical, long-term pursuit and valuation are necessary to allow us to know the natural history of the disease as well as the reaction to the treatment.