Cushing's syndrome in pregnancy. Laparoscopic adrenalectomy during pregnancy: the mainstay treatment.

INTRODUCTION: Cushing's syndrome (CS) is a rare disease in pregnancy and its differential diagnosis is complicated. It is usually confused with complicated pregnancy cases, such us preeclampsia and gestational diabetes. This usually leads to a delayed diagnosis and is also associated with severe materno-foetal complications. PURPOSE: We present a case of a pregnant woman in her third trimester of pregnancy with CS secondary to an adrenal adenoma without response to medical treatment who underwent laparoscopic adrenalectomy without complications. DISCUSSION: Most authors consider adrenalectomy as the first-choice treatment, since it presents higher rate of successful pregnancies. However, previously published cases did not reflect this stance. These publications prefer to maintain medical treatment, allowing pregnancy to go to term and delaying adrenalectomy after childbirth. We consider that experience in laparoscopic surgery, obstetric knowledge and resources now available, all offer sufficient guarantees to carry out the laparoscopic procedure to treat CS in pregnancy even in the third trimester. Laparoscopic adrenalectomy for CS in pregnancy is a safe and efficacious procedure allowing stopping the disease and curbing the dreadful consequences it may have for both mother and foetus.

Clinical and genetic factors involved in Porto-sinusoidal vascular disorder after oxaliplatin exposure.

BACKGROUND AND AIMS: Oxaliplatin (OX) has been described as a potential etiologic agent for porto-sinusoidal vascular disorder (PSVD). Our aim was to describe the natural history of PSVD due to OX in colon cancer (CRC) and identify risk factors for its development. METHODS: We made a multicenter retrospective case-control (ratio 1:3) study with patients diagnosed of PSVD-OX. Baseline data, end of treatment, years of follow-up and diagnosis of PSVD were collected and compared to controls (without PSVD). Besides, 16 different SNPs were selected from bibliography and analyzed by genotyping in the case group to identify potential genetic risk factors. RESULTS: 41 cases were identified, with a median time to PSVD diagnosis after the end of OX of 34 months. Spleen diameter was the strongest predictor of PSVD during treatment (OR 43.94 (14.48-133.336); p < 0.0001). Additionally, thrombocytopenia (<150 × 10^9) at one year was a significant disease risk marker (OR 9.35; 95% CI: 3.71-23.58; p = 0.001). We could not establish any significant association between the selected SNPs and PSVD diagnosis. CONCLUSION: The increase of spleen diameter is the strongest predictor of PSVD in patients treated with OX for CRC. These patients could be candidates for a specific follow-up of portal hypertension-related complications.

[Failed back surgery, spinal cord stimulation and pregnancy: presentation of a case]. / Descripción de un caso de síndrome de cirugía fallida de espalda, estimulación medular y embarazo.

Spinal cord stimulation is increasingly used to manage chronic pain syndromes, such as complex regional pain syndrome, chronic back pain, refractory angina pectoris or peripheral vascular diseases, which are unresponsive to other common less aggressive treatment methods. The early use of this technique in the aforementioned diseases makes it suitable in young women of childbearing age and who wish to become pregnant. We report the case of a 33-year-old woman who became pregnant 4 months after having undergone posterior cord stimulation, and we review the approach to this situation and the perioperative management during the perinatal period.

Indications and results of sentinel lymph node biopsy in breast cancer: Changes observed in 2018 compared to 2012.

BACKGROUND AND OBJECTIVE: Over the last 15 years, breast cancer (BC) treatment has undergone numerous changes, which have also affected the indications for Sentinel Lymph Node Biopsy (SLNB) as well as the procedures depending on its outcome. The aim of this study is to compare the results of the SLNB carried out at our Center during 2012, when we stopped performing an axillary lymph node dissection (ALND) after the finding of a micrometastasis, with those conducted in 2018, when we started applying Z011 criteria. MATERIALS AND METHODS: We have performed a comparative retrospective observational study, including cN0 BC patients that underwent a SLNB in 2012 versus those that underwent this procedure in 2018. RESULTS: 174 patients from 2012 and 165 from 2018 were studied. We found significant differences between the two groups: in 2018 there were fewer lobular invasive cancers (14 vs 28) (P < .05), a smaller mean pathological size (P < .001), a higher proportion of HER2 and triple negative tumors (28 vs 49) (P < .01) and, finally, an increase in use of neoadjuvant treatments (42.0% vs 6.7%). Regarding axillary involvement, we observed a decrease in both the presence of a positive sentinel node (24.1% in 2018 vs 42.4% in 2012) (P < .0001) as well as in the proportion of ALND performed (12.6% in 2018 vs 21.2% in 2012) and the presence of positive non sentinel nodes after a ALND (59.1% vs74.3%) (ns) CONCLUSION: Although the 2018 cohort that underwent SLNB had more aggressive tumors, there were fewer positive SN and ALND performed. This is probably due to a more accurate radiological diagnosis with ultrasound, that enables to detect cN1 cases before surgery, and to the increasing use of neoadjuvant treatments that may downstage the axilla.

[99mTc-MAA peritoneal scintigraphy in pleuroperitoneal comunication in peritoneal dialysis patients]. / Gammagrafía peritoneal con 99mTc-MAA en las comunicaciones pleuroperitoneales en pacientes en diálisis peritoneal.

Peritoneal dialysis is a fully-contrasted alternative for the treatment of end-stage renal disease although it is not exempt of complications. Peritonitis and exit-site infections are among the most frequent complications found. Pleural effusion secondary to pleuroperitoneal communication (PPC) is a serious and uncommon complication in these patients. We present the case of a 50-year old man diagnosed of end-stage renal disease undergoing treatment with peritoneal dialysis who presented progressive dyspnea and right pleural effusion. The peritoneal scintigraphy with (99m)Tc-MAA makes it possible to confirm communication of intraperitoneal dialysis fluid to the pleural cavity.

Numerical simulation of the aqueous humor flow in the eye drainage system; a healthy and pathological condition comparison.

The present work describes the motion of aqueous humor through the anterior chamber and the trabecular drainage system, considering several distributions of the collector channels. The 3D computational model, implemented into the open-source software, was reconstructed from an optical coherence tomography. The model has been employed to simulate the aqueous humor dynamics considering buoyancy effects. The presence of the anterior chamber, the trabecular meshwork, and the Schlemm's canal were taken into consideration with 14 different distributions of collector channels. The influence of collector channels position on the intraocular pressure and shear stress has been analyzed, for a healthy and a glaucomatous condition. Aqueous humor velocity, pressure, temperature, wall shear stress, skin friction coefficient and Nusselt number, are presented for the different cases. The results indicate that the position of the collector channels has a strong influence on the wall shear stress on the Schlemm's canal and collector channels.

Impact of immune-mediated diseases in inflammatory bowel disease and implications in therapeutic approach.

Inflammatory bowel diseases (IBD) belong to the group of immune-mediated diseases (IMIDs). The effect of associated IMIDs in the prognosis in IBD is nowadays unknown. To describe IMIDs associated to IBD patients and evaluate differences linked to the presence or absence of IMIDs. A unicentric retrospective descriptive study was designed. A cohort of 1,448 patients were categorized according to the presence of IMIDs. Clinical characteristics were obtained from IBD database. Univariate and multivariate analysis were performed. 385 patients were diagnosed with associated IMIDs while 1,063 had no associated IMIDs. A prevalence of 26.6% IMIDs associated to IBD was observed. Asthma, skin psoriasis and rheumatoid diseases were most commonly found. Factors associated to the presence of IMIDs were women (OR 1.48; 95 CI 1.17-1.87) and Crohn's disease (OR 1.35; 95 CI 1.07-1.70). Patients with associated IMIDs required more immunomodulator (OR 1.61; 95 CI 1.27-2.43) and biological treatment (OR 1.81; 95 CI 1.47-2.43). More surgical risk was observed in multivariate analysis in those patients diagnosed with IMIDs prior to the onset of IBD (OR 3.71; 95% CI 2.1-6.56). We considered the presence of IMIDs a poor prognostic factor and suggest a closer monitoring of these patients.

[Accumulated experience in the analysis of quality indicators in a haemodialysis unit]. / Experiencia acumulada en el análisis de indicadores de calidad en una unidad de hemodiálisis.

INTRODUCTION: The object of this work was to review our use of various indicators of haemodialysis within the guidelines of good clinical practice. MATERIALS AND METHODS: The study includes all patients from our haemodialysis program from June 2005 to February 2008. The indicators we evaluated included various areas: anaemia, bone-mineral metabolism, dialysis dose, cardiovascular risk, vascular access and morbidity/ mortality. The measurement intervals varied according to the parameter being evaluated. RESULTS: We gathered those indicators in which we found a difference between our results and the targets set. It is possible to reach a haemoglobin of > or = 11 g/dl in more than 85% of the patients, although more than 20% of them showed > 13 g/dl. We were able to stay on target with phosphorous (> 85%) but not with calcium (72.7%) or PTH (38.8%) although average values were improved. The incorporation of new patients to the haemodialysis programme, some previously unknown, limited our possibilities of achieving > or = 85% with a Kt/V > or = 1.3. Only 62.2% of the patients had a systolic blood pressure of < or = 140 mmHg. The percentage of patients dialysed by catheter (objective < 10%) was only achieved in five out of the eleven measures. The hospitalisation rate was between 0.49 and 0.71 episodes/patient/year. The patient survival rates coincide with those of the Comunidad Valenciana Register. CONCLUSION: the use of clinical performance measures has improved our results, whilst in some cases it has raised doubts over their definition and established targets. In general we feel that they should be revised and redefined where necessary in an attempt to avoid variability, iatrogenia, and increased costs. The use of only those indicators in which a clear scientific basis is evident, should be considered.

[Spinal granuloma in a patient receiving a spinal infusion of morphine and clonidine]. / Granuloma intradural en un paciente con infusión espinal de morfina y clonidina.

Patients treated with long-term spinal infusion of high doses of morphine develop a granuloma at the location of the catheter tip. Diagnosis is based on a steady increase in intrathecal morphine dosage after a relatively prolonged period of stability, on the gradual development of neurologic signs and symptoms suggesting radicular or spinal cord compression, and on magnetic resonance images. We describe a man with central neuropathic pain after removal of a tumor. The presence of all 3 of the aforementioned diagnostic criteria led to suspicion of a spinal granuloma.

[Effect of posture on spinal cord stimulation in patients with chronic pain syndromes: analysis of energy requirements in different patient postures]. / Efecto de la postura en la estimulación medular en pacientes con síndromes de dolor crónico. Análisis del gasto de energía dependiendo de la postura del paciente.

BACKGROUND AND OBJECTIVE: Patients being treated with spinal cord stimulation for chronic pain complain of variable paresthesias, particularly in relation to changes in posture. Such changes affect the great majority of patients with implantable pulse generators, requiring them to use the external programmer for avoidance of painful paresthesias or even to disconnect the generator, leading to loss of pain relief. The aim of this study was to determine the relationship between the pulse charge needed for stimulation and the patient's different postures. MATERIAL AND METHODS: Observational study of 70 patients treated with spinal cord stimulation in the following postures and situations: decubitus position, standing, seated, and walking. With the patients standing, we analyzed the thresholds of perception, pain, and pain relief, as well as the therapeutic range. Studies were performed in all patients. Later, data were analyzed by anatomical positioning of the stimulator (cervical, thoracic, sacral, occipital, or subcutaneous). RESULTS: In the analysis of the therapeutic range in the overall group we identified statistically significant differences between decubitus and standing positions and between decubitus position and walking. At the level of the thoracic spine differences were identified between all positions except between standing and walking and between seated and decubitus positions. At the level of the cervical spine, no significant differences were detected. Analysis of the pulse charge showed a significant difference in the decubitus position, in which less charge was needed to achieve satisfactory stimulation. When electrodes implanted at the cervical and thoracic levels were compared, differences were found between standing and seated positions (P=.04) but none between decubitus position or walking and the other positions. CONCLUSION: Stimulation systems are not currently designed to adapt to changes in distance between the electrodes and nerve fibers. Improvements are required in this respect.

Numerical model of aqueous humor drainage: effects of collector channel position.

Glaucoma is a progressive and a degenerative eye disease that gradually deteriorates the vision. The origin of glaucoma is still under debate. Recent studies report that 50% of the aqueous humor outflow resistance resides on collector channels and Schlemm's canal (SC). This paper provides a descriptive analysis of the aqueous humor outflow through the anterior chamber, the trabecular meshwork (TM) and the SC. The aim of this work is to determine the influence of the collector channels position on the intraocular pressure (IOP) and its contribution to the development of glaucoma. Pressure and wall shear stress distributions are presented for four cases. The first case has an arrangement of collector channels according to micro CT (Gong and Francis, 2014). The remaining cases have an symmetrical distribution; case 2 has all open quadrants (AOQ), cases 3 have three quadrants completely open and just one quadrant semi closed (SCQ), and finally cases 4 consider that a quadrant is completely closed (CQ). Symmetrical and micro CT cases have 29 collector channels. The results show that the position and the opening of the collector channels have a strong influence on the IOP.

[Use of rechargeable stimulators in 2 cases of cauda equina syndrome]. / Utilización de sistemas de estimulación recargables. A propósito de dos casos de síndrome de cola de caballo.

The first of the 2 cases of cauda equina syndrome we report occurred following phenolization of sacral roots after a series of operations for Hirsprung disease. The second occurred after trauma from a bullet that hit the spine. Fecal and urinary incontinence and neuropathic pain in the lower extremities were present in both cases. After various treatments failed, both patients were given dual spinal stimulation for integrated treatment of both incontinence and neuropathic pain. We applied a novel approach, connecting both stimulators to a single rechargeable generator, reducing medium- and long-term costs. Fewer generator replacements (every 2-3 years with the standard approach) and fewer generator implantations (2 each time with the usual system) are required when rechargeable generators are used. Patient quality of life is thus improved. The overall cost of the implants is reduced from their second year of use.

[Vascular access outcome in patients aged over 75 years]. / Resultados del acceso vascular en mayores de 75 anos.

UNLABELLED: We report a retrospective study on the results of 132 native fistulas, 12 grafts and 27 tunneled catheters followed during 30 months in 144 patients on hemodialysis. The results were compared according to patient age: 75 years or over (n = 58, 80.3 +/- 3.5) vs below 75 years (n = 86, 59.5 +/- 13.3). Gender, presence of diabetes and type of fistula were also included in the analysis. RESULTS: There were no statistically significant differences between both groups in the use of tunneled catheters or grafts (8.6% vs 5.8% y 5.2% vs 10.5% respectively), primary failure of native fistulas (7.1% in those aged 75 years or over vs 25.5% in patients below 75 years), rate of thrombosis (0.03 vs 0.09/patient year at risk respectively) or number of percutaneous or surgical procedures in order to maintain the fistula patency (0.11 vs 0.16/patient year at risk respectively). At the same time no differences were seen in the primary, primary assisted and secondary patency of the native fistulas. The mean age of the patients when the first access fistula was created was different according to the area of surgery (74.9 +/- 9.3 for the elbow vs 64.9 +/- 16.2 years for the forearm, p < 0.005). Diabetes was an unfavourable factor for primary (HR Cox 2.08, p < 0.05) or secondary (Log Rank, p <0.05) patency. CONCLUSION: The vascular access for hemodialysis in elderly patients presents a similar evolution to that seen in younger populations if the access creation is based on an exhaustive study, including ecodoppler of the vascular map and the use of more proximal fistulas if necessary. Therefore the more frequent use of grafts or catheters in elderly patients is not justified.

[Enzyme replacement therapy in Fabry's disease]. / Terapia de sustitución enzimática en la enfermedad de Fabry: evaluación clínico-histológica tras 18 meses de terapia.

We report a 56-year-old man with history of chronic renal failure, who was diagnosed to have Fabry's disease after performing a percutaneous kidney biopsy. The diagnosis was confirmed by the deficient level of activity of alpha-galactosidase A and by the identification of the mutation. A enzime replacement therapy with alpha-galactosidase A was administered. After 18 months of treatment, a second kidney biopsy was performed showing renal deposits of globotriaosylceramide (we did not evaluate the percentage of histologic clearance of the deposits). Six months after the end of the therapy, a reduction in the impairment of renal function is observed, and the classic manifestations of the disease are absent.

Emerging Targets for Therapeutic Development in Diabetes and Its Complications: The RAGE Signaling Pathway.

Types 1 and 2 diabetes are on the rise worldwide. Although the treatment of hyperglycemia has benefited from recent advances, aggressive efforts to maintain euglycemia may be fraught with risk, especially in older subjects or in subjects vulnerable to hypoglycemic unawareness. Hence, strategies to prevent and treat the complications of hyperglycemia are essential. In this review we summarize recent updates on the biology of the receptor for advanced glycation endproducts (RAGE) in the pathogenesis of both micro- and macrovascular complications of diabetes, insights from the study of mouse models of obesity and diabetic complications, and from associative studies in human subjects. The study of the mechanisms and consequences of the interaction of the RAGE cytoplasmic domain with the formin, mDia1, in RAGE signal transduction, will be discussed. Lastly, we review the "state-of-the-art" on RAGE-directed therapeutics. Tackling RAGE/mDia1 may identify a novel class of therapeutics preventing diabetes and its complications.

Prevalence of osteomyelitis in non-healing diabetic foot ulcers: usefulness of radiologic and scintigraphic findings.

The study was conducted in order to assess the prevalence of osteomyelitis and the predictive value of radiographic (xR) and combined Tc 99-bone and leukocyte scanning (CS) findings in diabetic foot ulcers that met criteria for hospital admission (FUH). Out of 150 episodes of ulceration managed in an outpatient basis, 33 (in 28 NIDDM patients) requiring admission were evaluated. In all cases plain xR and CS were carried out. Seventeen episodes (51.5%) had a good outcome (healed or improving, at the time of the last follow up). Osteomyelitis was found in 21 episodes and 14 (66.6%) of them required an amputation. In 13 cases where xR showed characteristic radiologic changes of osteomyelitis (11 of them had a positive CS) 11 (84.6%) underwent an amputation. However, when osteomyelitis was diagnosed only by a positive CS, only 3/8 (37.5%) required a toe amputation. Severe peripheral vasculopathy was present in 44% of cases who required amputation and only in 17.6% of those who did not. We conclude that in FU underlying osteomyelitis is frequent and associated to a higher amputation rate than when no bone infection is identified (66.6 vs 17%), even when corrected for vascular status (OR 11, CI 95% 1.65-74.2), with a worse outcome when xR changes are already present.

Lymphangioma circumscriptum of the vulva.

Lymphangioma circumscriptum is a benign disease of the lymph ducts and an unusual pathologic process that rarely affects the vulva. The etiology of this lesion is not clear, but obstruction of the lymph vessels has been suggested as a possible cause in some cases. We report the case of a 44-year-old woman with lesions similar to lymphangioma circumscriptum of the vulva and chronic idiopathic lymphedema of the lower right limb. Because there was no obvious cause, we propose that the lymphangioma was caused by the lymphedema.

[Successful pregnancy in a patient with systemic lupus erythematosus on hemodialysis]. / Gestación con éxito en una paciente con lupus eritematoso sistémico en hemodiálisis.

We report a twenty-nine-year-old patient, on renal replacement treatment (including 2 kidney transplants) since she was 11 years old, because of systemic lupus erythematosus. The patient became pregnant on hemodialysis, concluding the pregnancy successfully. The evolution and management of pregnancy were described and the related literature was revised. We concluded that, although pregnancy on hemodialysis and/or systemic lupus erythematosus patients involves a high risk for the mother and the fetus that makes advisable contraception, an increase in success in pregnancies in systemic lupus erythematosus patients on remission during the previous months, has been recently observed. Moreover, the increase of frequency and duration of hemodialysis sessions, has improved the neonatal survival around the 50% in pregnancies on dialysis. We emphasize the difficulty to maintain the mother's hemoglobin at an adequate target, as well as the high frequency of fetal prematury.

[G-CSF versus GM-CSF in the treatment of neutropenia in a patient with Felty's syndrome on hemodialysis]. / G-CSF versus GM-CSF en el tratamiento de la neutropenia de un paciente con síndrome de Felty en hemodiálisis.

We describe a patient with Felty's syndrome and chronic renal failure due to secondary amyloidosis in a periodic haemodialysis programme, who was successfully treated for neutropenia with sequential administration of colony-stimulating factors: granulocyte colony-stimulating factor and granulocyte macrophage colony-stimulating factor.

[Distribution of hepatitis C virus genotypes among the hemodialysis population in the province of Alicante]. / Distribución de los genotipos del virus C en la población de hemodiálisis de la provincia de Alicante.

UNLABELLED: Hepatitis C virus (HCV) genotypes are irregularly distributed among the different geographic area and groups at risk. OBJECTIVE: To study the different HCV genotypes and subtypes of hemodialyzed patients from Alicante. METHODS: We studied 640 patients on haemodialysis (HD) and we determined the RNA-HCV and the genotypes in the 120 patients with antibodies against HCV (HCV-Ab). We compared the results with the genotypes of 1,370 patients from other groups at risk in the same geographic area. RESULTS: RNA-HCV was not found in the serum in 15% (18/120) of the patients on HD who were HCV-Ab positive. Prevalence of the different genotypes in the 102 patients with positive viral RNA was the following: 1b: 56.8% (58/102), 1a: 19.6% (20/102), 3: 17% (17/102), 2a-2c: 1.9 (2/102), 2b: 0.9% (1/102) 4: 2.9 (3/102), 5: 0.9% (1/102). In conclusion, the genotype 1b was the most frequent in the patients studied in all these areas, and was the same as in the rest of the country. This genotype has been associated with the most severe hepatic disease and poor response to treatment, affecting the prognosis of these patients. The most frequent genotypes in HD in Alicante were 1b, 3 and 1a. HCV genotypes distribution among the HD units was not uniform in the different geographic areas. HCV genotypes distribution in the HD population is similar to other groups at risk from the same geographic area.