Benefica chirurgia. A global surgery project focusing on hernia surgery.

BACKGROUND: The aim of international health cooperation projects is to alleviate the deficiencies in the area of health in low resource settings. Hernia surgery is a procedure that is well suited to these missions, due to its low morbidity, the fact that it can be performed on an outpatient basis, and the improvement in quality of life that it provides. OBJECTIVE: To describe the results of Benefica Chirurgia (BC), a Spanish non-profit humanitarian association in hernia pathology. METHODS: Five one-week surgical campaigns were carried out in Ecuador between 2015 and 2019, involving anesthetists, general and pediatric surgeons. Surgical and medical equipment was provided and transported by BC. ASA I/II patients underwent surgery. RESULTS: Surgery was performed on 240 patients with hernia pathology on 27 days. Sixty-three per cent of patients were male and the mean age was 48.2 years (range: 1-83). Hernia location was inguinal in 113 patients, umbilical in 101, and other in 26. The anesthetic technique used was spinal in 185 patients (77.1%), local plus intravenous sedation in 31 (12.9%), and general in 24 (10%). The surgical technique used was hernioplasty in 191 patients, herniorrhaphy in 31, incisional hernia repair in 15 and herniotomy in three. Surgery was performed on an outpatient basis in 98.4% of cases. Morbidity was 2%. Long-term postoperative evaluation is very complex. CONCLUSION: These campaigns make a significant contribution to health in low resource settings and provide great personal satisfaction for those involved. Standards achieved in the immediate postoperative period were similar to those obtained at the surgeons' centers in Europe. However, it is difficult to establish the rates of recurrence and chronic pain.

[How to organize a Fetal Medicine Unit in the context of COVID-19 pandemic. Safe measures for obstetric scans and equipment cleaning]. / Cómo reestructurar una Sección de Medicina Fetal en tiempos de pandemia COVID-19. Medidas de seguridad y limpieza de los equipos ecográficos.

During a pandemic, the three basic principles are. to prioritize medical resources, ensure patients' lockdown in order to avoid community transmission and prevent healthcare collapse, and keep the number of visits to an absolute minimum to avoid patient exposure and safeguard healthcare workers. Antenatal care must be maintained during a health crisis, regardless of the COVID-19 state of alert. Routine and specialist obstetric ultrasound scans are essential for clinical decision-making during pregnancy, as it has a direct impact on the management of mothers and fetuses and on the perinatal outcome. In an attempt to minimize in-person visits, these will be organized according to the established ultrasound schedule. Based on scientific evidence, and on existing main national and international guidelines, this document has been prepared, in which proposals and options are provided for managing pregnant women in the context of the SARS-CoV-2 pandemic. It includes how a Fetal Medicine Unit facing this health crisis should be restructured, what safety measures should be followed in the performance of obstetric scans and invasive procedures, and how ultrasound rooms, equipment and transducers should be cleaned and disinfected. These recommendations should be adapted to different units based on their resources and infrastructure.

[The role of fetal magnetic resonance imaging in the study of congenital diaphragmatic hernia]. / El papel de la resonancia magnética fetal en el estudio de la hernia diafragmática congénita.

INTRODUCTION AND OBJECTIVES: Different echographic and fetal magnetic resonance (MRI) measurements have been described in the diagnosis of associated malformations and the prognosis of congenital diaphragmatic hernia (CDH). We have reviewed our experience searching for useful isolated or combined parameters and how MRI can complement ultrasound. MATERIAL AND METHODS: We evaluated 29 fetuses with CDH. We examined ultrasonography: Lung to Head (LHR o/e) and in MRI: ipsilateral lung volume (IPV) and total expressed as percentage of observed / expected lung volume (VPT o/e) and percentage of herniated liver (PHH). We studied: survival, ECMO and associated malformations. RESULTS: LHR o/e was the measure that best predicted survival (p< 0.05). VPT o/e did not predict survival or the need of ECMO (p> 0.05). PHH ≥19% was related to the need of ECMO. IPV < 2 cc required ECMO more frequently (p< 0.018) and when it was 0 cc in all cases. No combination of MR measurements was superior to LHR o/e in prediction of survival. MRI complemented the ultrasound in 4 cases: diaphragmatic eventration diagnosed with HDC, right HDC with fluid in the sac that suggested thoracic cyst, differentiation between spleen and lung that measured together overestimated the LHR and/or suspicion of Cornelia de Lange due to facial malformations. CONCLUSIONS: Not a single or combined MRI measurement exceeds LHR o/e in survival prediction. MRI is related to prognosis and can be used to support ultrasound in making decisions. MRI occasionally provides complementary morphological information.

INTRODUCCION Y OBJETIVOS: Distintas medidas ecográficas y de resonancia magnética fetal (RM) han sido descritas en el diagnóstico de malformaciones asociadas y el pronóstico de la hernia diafragmática congénita (HDC). Hemos revisado nuestra experiencia buscando parámetros aislados o combinados útiles y cómo la RM puede complementar a la ecografía. MATERIAL Y METODOS: Evaluamos 29 fetos con HDC. Revisamos en ecografía: Lung to Head Ratio observado/esperado (LHR o/e) y en RM: volumen pulmonar ipsilateral (VPI) y total expresado como porcentaje del volumen pulmonar observado/esperado (VPT o/e) y porcentaje de hígado herniado (PHH). Estudiamos: supervivencia, oxigenación con membrana extracorpórea (ECMO) y malformaciones asociadas. RESULTADOS: El LHR o/e fue la medida que mejor predijo supervivencia (p< 0,05). El VPT o/e no predijo supervivencia ni la necesidad de ECMO (p> 0,05). El PHH ≥19% se relacionó con necesidad de ECMO. El VPI menor de 2 cc requirió ECMO más frecuentemente (p< 0,018) y cuando fue de 0 cc en todos los casos. Ninguna combinación de medidas de RM fue superior al LHR o/e en predicción de supervivencia. La RM complementó a la ecografía en 4 casos: eventración diafragmática diagnosticada como HDC, HDC derecha con líquido en el saco que sugería quiste torácico, diferenciación entre bazo y pulmón que medidos juntos sobreestimaba el LHR o/e y sospecha de síndrome de Cornelia de Lange por malformaciones faciales. CONCLUSIONES: Ninguna medida aislada o combinada de RM supera al LHR o/e en la predicción de supervivencia. La RM se relaciona con el pronóstico y puede usarse como apoyo de la ecografía en la toma de decisiones. La RM aporta ocasionalmente información morfológica complementaria.

[Study of pulmonary hypertension and long-term respiratory clinic in children with congenital diaphragmatic hernia]. / Estudio de la hipertensión pulmonar y la clínica respiratoria a largo plazo en los niños con hernia diafragmática congénita.

INTRODUCTION AND OBJECTIVES: The aim of this study is to identify respiratory clinic and pulmonary arterial hypertension (PAH) in congenital diaphragmatic hernia (CDH) and whether these could be predicted by prenatal measures. MATERIAL AND METHODS: We studied fetal ultrasound: Observed/expected Lung to Head Ratio (O/E LHR) and classified patients according to their outcome (group 1: O/E LHR <25%, group 2: 26-35%, group 3: 36-45%, group 4: >55%) as well as the severity of PAH (group 0: non-PAH, group 1: mild, group 2: moderate, group 3: severe) in echocardiograms at birth, 1st, 6th, 12th and 24 months of life. We also evaluated gestational age, weight, bronchodilator treatment and number of hospital admissions. RESULTS: 58 patients with CDH, 13 without prenatal diagnosis. 36 patients out of 45 had O/E LHR calculated at 22.4 ± 5.8 weeks. O/E LHR had significant association with the severity of PAH at birth and in the 1st, 6th, 12th and 24th months (p <0.05). At 6 months, only 30.4% had PAH without any association with a higher risk of hospital admission [OR 1.07 (0.11-10.1)] and only three patients (5.1%) required bronchodilator treatment. CONCLUSION: In CDH, PAH and the respiratory clinic improve over time, being uncommon the need for treatment as of the 6th month. O/E LHR predicts the presence and severity of PAH in short and long term.

INTRODUCCION Y OBJETIVOS: Nuestro objetivo es estudiar la presencia en hernia diafragmática congénita (HDC) de clínica respiratoria e hipertensión pulmonar (HTP) a largo plazo y si estas pueden predecirse prenatalmente. MATERIAL Y METODOS: Estudiamos en ecografía fetal: Lung to Head Ratio observado/esperado (LHR O/E) y clasificamos a los pacientes según su resultado (grupo 1: LHR O/E <25%, grupo 2: 26-35%, grupo 3: 36-45%, grupo 4: >55%) así como la gravedad de HTP (grupo 0: no HTP, grupo 1: leve, grupo 2: moderada, grupo 3: grave) en los ecocardiogramas al nacimiento, 1º, 6º, 12º y 24º meses de vida. Estudiamos también edad gestacional, peso, tratamiento broncodilatador y número de ingresos hospitalarios. RESULTADOS: Se identificaron 58 pacientes con HDC, 13 de ellos sin diagnóstico prenatal. De los 45 restantes, 36 tenían calculado el LHR O/E registrado a las 22,4 ± 5,8 semanas. El LHR O/E se relacionó significativamente con la gravedad de la HTP al nacimiento y en los meses 1º, 6º, 12º y 24º (p <0,05). A los 6 meses únicamente el 30,4% presentaban HTP sin que ello asociara más riesgo de ingresos hospitalarios [OR 1,07 (0,11-10,1)] y siendo solo n = 3 (5,1%) los que precisaban algún tipo de tratamiento broncodilatador. CONCLUSION: En HDC, la HTP y la clínica respiratoria mejoran con el tiempo, siendo infrecuente la necesidad de tratamiento a partir del 6º mes. El LHR O/E predice la presencia y gravedad de HTP a corto y largo plazo.

Dichorionic twin pregnancy discordant for anencephaly: two cases with different management.

BACKGROUND: Prevalence ofanencephaly in dichorionic twins is higher than in singleton pregnancies. The authors report two cases with two different management strategies. CASE 1: Spontaneous dichorionic diamniotic twin pregnancy with the second twin diagnosed with anencephaly at 12 weeks gestation. Selective feticide was performed at the age of 13.2 weeks. Vaginal delivery occurred at 39 weeks, and birth weight was 2,850 g. CASE 2: Dichorionic diamniotic twin pregnancy discordant for anencephaly in the second twin was diagnosed at 13 weeks gestation. An expectant management was decided. Preterm delivery occurred at 35 weeks due to hydramnios of the affected fetus, delivering a healthy newborn weighing 2,300 g and an anencephalic neonate who died immediately after delivery. CONCLUSION: Anencephaly should be diagnosed as soon as possible, idealistically at 11-13+6 weeks ultrasound (US) scan, in order to offer the most appropriate counselling to the parents, ranging from selective feticide or expectant management. This short series suggests that selective early feticide may increase gestational age and birth weight.

Varicella zoster central nervous system vasculitis after allogeneic hematopoietic stem cell transplant successfully treated with cyclophosphamide.

We report the first case of varicella zoster central nervous system vasculitis, to our knowledge, which responded to intravenous pulses of cyclophosphamide in an immunocompromised child with severe and progressive disease, without sequelae.

Effects of different cryopreservation methods on DNA integrity and sperm chromatin quality in men.

BACKGROUND: Cryopreserved human sperm are used in assisted reproductive technology. However, the effect of cryopreservation on sperm DNA integrity is unclear. OBJECTIVES: The objectives of this study were to: (i) determine the impact of semen cryopreservation on human sperm DNA integrity and chromatin structure; (ii) test if parameters obtained from TUNEL and SCSA® correlate; and (iii) verify correlation between sperm motility, morphology and viability with TUNEL and SCSA® parameters. MATERIALS AND METHODS: Men attending a fertility clinic were recruited and grouped according to their sperm parameters (n = 9/group): normozoospermia, oligoasthenoteratozoospermia and teratozoospermia. Each semen sample was processed as follow: (i) directly frozen at -80 °C; (ii) diluted in Sperm Maintenance Medium, cooled for 30 min at 4 °C and frozen at -80 °C; (iii) diluted in Sperm Maintenance Medium; or (iv) in SpermFreeze. Each mixture from method (iii) and (iv) was then suspended for 30 min in liquid nitrogen vapor and plunged into liquid nitrogen. After at least two months of storage, samples were thawed at room temperature and analyzed for motility and viability, TUNEL and SCSA® assays. RESULTS: Progressive motility and viability decreased after freeze-thawing. TUNEL scores increased significantly in all samples after freezing-thawing while no significant change in the DNA fragmentation index (DFI) from SCSA® was observed. No change in the percentage high DNA stainability (HDS) was observed in normozoospermic samples; however it was significantly increased in all the methods in oligoasthenoteratozoospermic and in the methods (ii)-(iv) in teratozoospermic samples. The DFI and TUNEL scores correlated significantly with each other and inversely with sperm motility, viability and morphology. DISCUSSION AND CONCLUSION: Cryopreservation seems to be deleterious for the integrity of human sperm DNA and compaction. However, the sperm DFI was not affected during cryopreservation under the various methods of storage tested. Clinicians and investigators should take this information into consideration when using cryopreserved sperm for assisted reproduction.

Prognostic factors and outcomes for pediatric patients receiving an haploidentical relative allogeneic transplant using CD3/CD19-depleted grafts.

Haploidentical hematopoietic stem cell transplantation using T-cell-depleted grafts is a valid option for pediatric patients with hematological malignancies in need of an allogeneic transplantation and lacking an HLA-identical donor. Seventy-five transplantations were performed in 70 patients. Thirty-eight patients had ALL, 32 had AML, 3 had advanced myelodysplastic syndromes and 2 juvenile myelomonocytic leukemia; 19 were in first CR, 30 in second CR, 12 in greater than second CR and 14 were considered to be in refractory disease at time of transplantation. Four patients developed graft failure. Among engrafted patients, the median time to neutrophil and platelet recovery was 13 (range 8-20) and 10 days (range 8-70), respectively. In 64 (85%) cases, ⩾1 infections were diagnosed after transplant. The probability of nonrelapse mortality by day +100 after transplantation was 10±4%. With a median follow-up of 22 months, the probability of relapse was 32±6% and disease-free survival was 52±6%. Haploidentical transplantation using CD3/CD19 depletion is associated with encouraging results especially in patients in early phase of disease. Killer-cell Ig-like receptor B haplotype donors confer a rapid natural killer cells expansion early after transplantation, resulting in lower probability of relapse and suggesting a GvL effect apart from graft-versus-host reactions. Donor infusion of high numbers of CD34+ cells is recommended in order to improve T-cell reconstitution.

Synthesis and structure-activity relationships of dequalinium analogues as K+ channel blockers. Investigations on the role of the charged heterocycle.

Small conductance Ca(2+)-activated K+ (SKCa) channels occur in many cells but have been relatively little studied. Dequalinium, a bis-quinolinium compound, has recently been shown to be the most potent nonpeptidic blocker of this K+ channel subtype. This paper examines the importance of the quinolinium rings for blocking activity. Analogues of dequalinium were synthesised in which one quinolinium group was removed (1 and 2) or replaced by a triethylammonium group (3). They have been assayed in vitro for their ability to block the after-hyperpolarization (mediated by the opening of SKCa channels) that follows the action potential in rat sympathetic neurones. The compound having one quinolinium and one triethylammonium group (3) showed reduced activity, and it is suggested that the stronger binding to the channel of the quinolinium relative to the triethylammonium group may be related to differences in their electrostatic potential energy maps. Two monoquaternary compounds (1 and 2) were tested, but they exhibited a different pharmacological profile that did not allow definite conclusions to be drawn concerning their potency as blockers of the SKCa channel. Replacement of both quinolinium groups by pyridinium, acridinium, isoquinolinium, or benzimidazolium reduced but did not abolish activity. These results show that compounds having a number of different heterocyclic cations are capable of blocking the SKCa channel. However, among the heterocycles studied, quinoline is optimal. Furthermore, charge delocalization seems to be important: the higher the degree of delocalization the more potent the compound.

Prospective biochemical and scintigraphic evaluation of autografted normal parathyroid glands in patients undergoing thyroid operations.

BACKGROUND: Parathyroid autotransplantation has been widely used in thyroid operations. Few studies have assessed the function of the autotransplanted glands. The aim of this study was to evaluate the success rate, pattern of biochemical function, and scintigraphic appearance of parathyroid autografts in patients undergoing thyroid operations. METHODS: Twelve patients in whom 1 parathyroid gland was removed during thyroid operation were included. Glands were fragmented and autografted into individual pockets in the brachioradialis muscle of the nondominant forearm. Parathyroid hormone levels were measured in both arms the day of autotransplantation and 2 weeks and 1, 2, 3, and 6 months after operation. Serum calcium levels were also measured at each interval. Sestamibi scanning was performed 6 months after operation in 7 patients. RESULTS: All patients were women with a mean age of 50 +/- 15 years. Serum calcium levels were normal during follow-up. A gradient of parathyroid hormone level of 1.5 or greater between the autotransplanted and nontransplanted arm was found in 10 patients. Isotope uptake at the site of the parathyroid autotransplant was demonstrated in the 7 patients evaluated. CONCLUSIONS: Biochemical function of autotransplanted normal parathyroid tissue was documented in 83% of the patients. Sestamibi scintiscans were able to identify the autotransplanted tissue.

Weekly variation of fungal colonies in the atmosphere of Palencia (Spain) throughout the year 1992.

We have carried out a qualitative and quantitative study of the fungal colonies developed in two different culture media: Czapecdox and Sabouraud, throughout the year 1992 in Palencia city. A volumetric trap was used. We collected daily samples of aerovagans spore from the atmosphere through a cellulose esther filter, half of which was cultivated on Petri dishes. The following genera were identified: 26 Deuteromycetes (54%), four Zygomycetes (28%), and three bacteria, which along with Actinomycetes, reached 18% of all the registered colonies. Fifty-two percent of the colonies were developed in Czapecdox culture medium and 48% in Sabouraud medium. Most of the bacteria were grown in Sabouraud medium. The highest number of colonies recorded belonged to the following three genera: Mucor (25%), Aspergillus (23%) and Penicillium (16%). Most colonies were grown in autumn (32%), while spring was the second most frequent season when 28% of the colonies were registered.

[Susac syndrome as a cause of sensorineural hearing loss]. / Síndrome de Susac como causa de hipoacusia neurosensorial.

Susac's syndrome is an extremely rare clinical manifestation characterized by the triad of fluctuating sensorineural hearing loss, sudden visual loss and encephalopathy. Probably underdiagnosed, it affects young women who start the clinical history with headache, visual and hearing disturbances, with neurological findings in MRI. With unknown aetiology, pathogenesis is based on arteriolar microinfarcts in retina, cochlea, and grey and white matter in the brain. Treatment is, as stated in the bibliography and our experience, intravenous high doses of steroids followed by oral steroids together with hyperbaric oxygen to minimize ischaemic lesions. Aspirin associate to nimodipine has been useful to date in the treatment of our patient. We present a case and review the existing literature.

[Transformation of myelodysplastic syndrome to acute lymphoblastic leukemia: 2 new cases]. / Transformación de síndrome mielodisplásico a leucemia linfoblástica aguda: 2 casos nuevos.

Myelodysplastic syndromes (MDS) are clonal disorders of hematopoietic stem cells, with a variable risk of transformation to acute myeloid leukemia. Progression into acute lymphoblastic leukemia (ALL) is an extremely rare event, with very few cases published in children. In this report, we describe two cases of myelodysplastic syndromes that progressed to ALL. Moreover, we review previously reported cases of MDS transformation to acute lymphoblastic leukemia in the pediatric population whose prognosis seems to be similar to that for adults.

[Ophthalmological manifestations in Fabry's disease. Four clinical cases showing deficient alpha-galactosidase-A activity]. / Manifestaciones oftalmológicas en la enfermedad de Fabry. A propósito de 4 casos con actividad deficiente de a-galactosidasa A.

CASE REPORT: Fabry's disease is an illness produced by an alteration in the catabolism of the glycosphingolipids. We report ophthalmologic findings in 4 people, detected after 113 patient evaluations from an analytical, cardiological and genetic point of view. DISCUSSION: Fabry's disease is uncommon and shows variable ophthalmologic affectation. Some patients with Fabry's disease do not present ocular affectation, while, on the other hand, healthy carriers with important ocular alterations have been described. The deposit of glycosphingolipids produces affectation at the corneal, crystalline, vascular and retinal levels. The vascular alterations affect not only the veins but also the arteries, as we report in our patients.

Adynamic bone lesion in renal transplant recipients with normal renal function.

Adynamic bone lesion has been defined as low bone turnover, normal or low osteoid volume and decreased bone formation rate (BFR). A prospective cross-sectional study was performed in 16 asymptomatic post-transplant kidney patients with normal renal function, to evaluate low bone mineral density. The mean age of the nine women and seven men was 33.9 +/- 7.3 years, the mean serum creatinine was 1.1 +/- 0.2 mg/dl and the mean creatinine clearance 71.5 +/- 13.8 ml/min/1.73 m2. Six patients received triple immunosuppressive therapy for a period of 10.3 +/- 3.7 months and nine received double therapy. Eighty-four months after renal grafting, we carried out bone densitometry, biochemical markers and bone biopsy. Bone densitometry showed 78 +/- 8.7% and 80.4 +/- 8% for hip and lumbar spine, with a mean Z score of 1.79 +/- 0.72 and 1.88 +/- 0.78 (SD), significantly less than normal in the Hispanic young population for those two regions. Serum PTH (0.83 +/- 0.23 microgram/ml normal range 0.32-0.65), urine cAMP (4.1 +/- 1.3, normal range 0.5-4.7 nmol/mg Cr) and total and nephrogenic fraction (3.1 +/- 1.1, normal range 0.29-2.9 nmol/100 ml GFR) were significantly greater than normal (P < 0.01). The bone biopsy in 12/16 patients showed decreased percentage osteoid area (1.59 +/- 0.86% vs 3.19 +/- 0.82%), percentage mineralized area (13 +/- 4.7% vs 21.03 +/- 3.36%) and bone formation rate (505 +/- 237 vs 1275 +/- 168 microns2/mm2/day), with a P value < 0.05 compared with 10 normal bone biopsies. The remaining four patients exhibited low bone turnover image with normal bone formation rate (1442 +/- 206 microns2/mm2/ day). Iron deposits were demonstrated at the mineralization front in 10/16 patients. No aluminium or amyloid deposits were observed. The histomorphometric results showed the presence of adynamic bone lesion in 12 renal transplant recipients with normal renal function and osteopenia, which explains the low bone density. The long-term use of glucocorticoids and the presence of iron deposits may contribute to this bone lesion. The biochemical markers of bone remodelling showed abnormalities compatible with moderate increase in parathyroid function. The adynamic lesion in the presence of hyperparathyroid function may suggest down-regulation of PTH bone receptors, alterations of the bone microenvironment or both.

Myelodysplastic syndrome with erythroid hypoplasia/aplasia: a case report and review of the literature.

Myelodysplastic syndrome (MDS) with erythroid hypoplasia/aplasia has not yet been clearly defined, and in most patients it is mistaken for acquired pure red cell aplasia (PRCA). We report a patient with severe transfusion-dependent anemia (Hb 6.9 g/dl) and reticulocytopenia. WBC and platelet counts were normal. Bone marrow examination showed a marked trilineage dysplasia and a low percentage of erythroid precursors (3%). A diagnosis of MDS (refractory anemia according to FAB classification) with erythroid hypoplasia/aplasia was made. Repeated cytogenetic analysis of bone marrow showed normal karyotypes. Moreover, serial IgM serology and DNA analysis of the patient's sera for B19 parvovirus were negative. Other conditions known to be associated with erythroid aplasia were also absent. The patient failed hematinics and prednisone therapy. He next received r-HuEPO (200 U/kg three times weekly). This form of therapy achieved a rapid and complete erythroid response. He has remained in complete erythroid response after a 7-month period on maintenance therapy of 100 U/kg three times weekly. A review of the literature revealed only 15 well-documented cases of MDS with erythroid hypoplasia/aplasia. All had morphological evidence of myelodysplasia. These patients were predominantly elderly males, all required regular packed red cell transfusions, and had an unfavorable prognosis, mainly because of a high rate of blastic transformation (frequently preceded by a myeloproliferative phase). The mechanism of erythroid hypoplasia in this subgroup of MDS remains uncertain. However, laboratory and clinical data suggest the existence of an intrinsic stem cell defect. None of the patients received hematopoietic growth factors. To our knowledge, our patient is the first case of MDS with erythroid hypoplasia where r-HuEPO was successfully attempted. The description of more cases is necessary to delineate the value of r-HuEPO therapy in this rare variant of MDS.