Complications of uveitis in a Spanish population, UveCAM study.

OBJECTIVE: To estimate the population frequency of uveitis complications and to evaluate their main risk factors in the patients with uveitis from the UVECAM study. PATIENTS AND METHODS: Development of complications in 386 patients with uveitis in the whole health area of the province of Toledo (UVECAM study) during a period of 1 year. Description of complications and study of their possible determinants by means of multivariate regression models. RESULTS: Information on the development of complications was available in 371 of the 386 patients of the UVECAM study. The most frequent complications were posterior synechiae (19.0%), ocular hypertension (14.0%), macular edema (7.5%), epirretinal membrane (6.9%), glaucoma (6.6%), iridian atrophy (5.6%) and cataract (5.5%). The risk of complications increases with age, intermediate and panuveitis locations, and those of chronic or recurrent evolution. CONCLUSION: Uveitis is associated with a high frequency of complications, especially in older patients, with intermediate or posterior localization of the inflammatory process and chronic or recurrent evolution.

[Macroadenoma of the non-pigmented ciliary epithelium]. / Macroadenoma del epitelio no pigmentado del cuerpo ciliar.

CASE REPORT: We report the clinical features and surgery of a patient with an adenoma of the non-pigmented ciliary epithelium. The adenoma measured 5 × 7 mm. The patient underwent radical ocular surgery consisting of partial iridocyclectomy associated to lamellar sclerouvectomy. DISCUSSION: Adenomas of ciliary body can mimic clinically amelanotic melanomas. We present details of the patient's medical records and review the literature. Clinically, adenoma in ciliary body can mimic amelanotic melanomas. Conservative surgery of the eye allows diagnosis and treatment, maintaining visual function.

Amblyopia secondary to iris cyst.

CLINICAL CASE: A 5 year-old child diagnosed with moderate anisometropic amblyopia secondary to primary cyst of iris pigment epithelium. He was evaluated with ultrasound biomicroscopy (BMU) and optical coherence tomography (OCT) of anterior segment. DISCUSSION: The OCT, although with some limitations, is a useful tool to study the anterior segment. It is probably more recommendable than BMU in the childhood.

[Technique for removing donor sclera by eyeball extrusion]. / Técnica de extracción de esclera donante mediante extrusión del globo ocular.

PURPOSE: To describe a surgery technique for removing donor sclera tissue after corneo-scleral button excision. RESULTS: The extrusion technique is easy to perform. It allows the complete scleral extraction its total clean up to be performed, as well as making easier to isolate the retina and uveal tissue. This technique could have an important role in the anatomical and morphological study of ocular structures.

[Hypothalamic hamartoma in paediatric patients: clinical characteristics, outcomes and review of the literature]. / Hamartoma hipotalámico en la edad pediátrica: características clínicas, evolución y revisión de la literatura.

OBJECTIVE: To describe the epidemiological and clinical-electroencephalographic characteristics, and associated morbidity of patients with hypothalamic hamartoma, as well as the treatment followed and outcomes PATIENTS AND METHODS: We have retrospectively reviewed the medical histories of 10 patients diagnosed with hypothalamic hamartoma by magnetic resonance imaging over the last 20 years. RESULTS: The age of onset of epilepsy in patients with hypothalamic hamartoma in our series was between the first days of life and 2 years. Of the 10 total patients, 8 had epileptic seizures during its progress. All of them had gelastic seizures, in addition to other types of seizures, with the most common being partial simple seizures. The electroencephalographic findings recorded were highly variable. One of the patients developed epileptic encephalopathy. Five patients had some kind of conduct disorder. Five patients had cognitive problems. At least 2 different antiepileptic drugs were measured in 8 of the patients who had seizures, and in 6 of these some type of non-pharmacological treatment had been used with the objective of seizure control. Only in 3 of 8 patients has been achieved Acceptable control of epilepsy had only been achieved in 3 out the 8 patients. Five patients of the series developed precocious puberty. The average time of follow-up of the series was approximately 6 years. CONCLUSIONS: Epilepsy is the most frequent manifestation of hypothalamic hamartomas. Most cases were drug-resistant, which led to difficulties in the management of these patients, requiring surgery for their control on many occasions. Psychiatric comorbidity and cognitive impairment is common.

Panayiotopoulos syndrome: probable genetic origin, but not in SCN1A.

Panayiotopoulos syndrome is encompassed in the classification of the ILAE in idiopathic focal epilepsies. Mutations in the SCN1A gene have been associated with the development of this syndrome. We present two cases of Panayiotopoulos syndrome in two monozygotic twins, who underwent a molecular analysis of SCN1A, but no alteration was found. These cases suggest a genetic origin, and SCN1A appears to be associated with the outcome but not with the development of this syndrome.

[Argon laser iridoplasty as treatment of plateau-like iris configuration secondary to multiple ciliary body cysts: long-term follow-up by ultrasound biomicroscopy]. / Iridoplastia con láser argón como tratamiento del iris plateau secundario a múltiples quistes del cuerpo ciliar: seguimiento a largo plazo con biomicroscopía ultrasónica.

CASE REPORT: We present a 37 year-old man with secondary angle closure by neuroepithelial ciliary body cysts. An argon laser peripherical iridoplasty was performed and ultrasound biomicroscopy (UBM) was employed to follow-up. Initially, the patient had a favorable response to treatment, but six months later the cysts and the secondary closed angle reappeared. DISCUSSION: Argon laser peripheral iridoplasty has been proposed as treatment of plateau-like iris configuration resulting from neuroepithelial ciliary body cysts. However, the cysts and, the secondary closed angle could recur in a long-term (Arch Soc Esp Oftalmol 2009; 84: 569-572).

[Arterial ischaemic stroke in children with cardiac diseases]. / Ictus arteriales isquémicos en niños con cardiopatías.

INTRODUCTION: The incidence of cerebral stroke in children has significantly increased in last years. Heart diseases are one of the best known risk factors in pediatric stroke. AIM. To describe the characteristics of the cerebral stroke in children with heart diseases, emphasizing in predisposing conditions, time to diagnosis, management and follow-up of patients. PATIENTS AND METHODS: We performed a retrospective study in children suffering from heart diseases with cerebral stroke admitted to our hospital that comprised 10 years. Type of cardiopathy, associated factors like surgery or catheterization, study of thrombophilia, clinical findings, methods and time to diagnosis, primary and secondary prophylaxis, treatment and long-term neurologic outcome were analyzed. RESULTS: Twenty patients were included in our study (age: from neonatal period to 15 years), eighteen of them were children with congenital cardiac disease. Eleven patients had antecedent of surgery and/or catheterism in the previous month. The most common clinical presentation was focal seizures. Time from clinical onset to diagnosis of stroke was longer than 24 hours in 60% of our patients. Fifty per cent of our patients did not receive any primary prophylaxis. Treatment was initiated in 70% of patients, but no one received thrombolysis. CONCLUSIONS: It is crucial to consider stroke when children with heart diseases show any neurologic symptom. Optimal diagnostic strategies must be established to low the delay of diagnosis in these patients, as well as randomised clinical trials in order to establish uniform guidelines in pediatric stroke.

Recurrent finding of the FIP1L1-PDGFRA fusion gene in eosinophilia-associated acute myeloid leukemia and lymphoblastic T-cell lymphoma.

The FIP1L1-PDGFRA fusion gene has been described in patients with eosinophilia-associated myeloproliferative disorders (Eos-MPD). Here, we report on seven FIP1L1-PDGFRA-positive patients who presented with acute myeloid leukemia (AML, n=5) or lymphoblastic T-cell non-Hodgkin-lymphoma (n=2) in conjunction with AML or Eos-MPD. All patients were male, the median age was 58 years (range, 40-66). AML patients were negative for common mutations of FLT3, NRAS, NPM1, KIT, MLL and JAK2; one patient revealed a splice mutation of RUNX1 exon 7. Patients were treated with imatinib (100 mg, n=5; 400 mg, n=2) either as monotherapy (n=2), as maintenance treatment after intensive chemotherapy (n=3) or in overt relapse 43 and 72 months, respectively, after primary diagnosis and treatment of FIP1L1-PDGFRA-positive disease (n=2). All patients are alive, disease-free and in complete hematologic and complete molecular remission after a median time of 20 months (range, 9-36) on imatinib. The median time to achievement of complete molecular remission was 6 months (range, 1-14). We conclude that all eosinophilia-associated hematological malignancies should be screened for the presence of the FIP1L1-PDGFRA fusion gene as they are excellent candidates for treatment with tyrosine kinase inhibitors even if they present with an aggressive phenotype such as AML.

[Exploration of the anterior segment by optical coherence tomography-3]. / Exploración del segmento anterior mediante tomografía óptica de coherencia 3.

PURPOSE: To show the utility of optical coherence tomography (OCT) for studying the anterior segment and to explain its potential advantages as compared to ultrasonic biomicroscopy (UBM). MATERIALS AND METHODS: We have described the findings in 5 patients with different pathologies of the anterior segment, all of whom were studied with UBM and OCT-3 adjusted for the anterior segment. There were 2 cases of an acute attack of closed angle glaucoma, 1 case of rubeosis iridis, 1 case of penetrating ocular trauma and 1 case of a primary stromal iris cyst. RESULTS: OCT was found to be as useful as UBM in detecting angle closure in patients affected by an acute attack of glaucoma. In addition, OCT was more comfortable for the patient and faster than UBM in obtaining images, with the exploration mean time using OCT being less than five minutes while that with UBM was over ten minutes. OCT has also been demonstrated to be a safe and valuable non-contact examination in other iris pathologies such as rubeosis iridis, for checking the permeability of iridotomies and even for studying iris masses such as primary stromal iris cysts. In these entities OCT may reach a higher resolution than ultrasonic biomicroscopy. CONCLUSION: OCT-3, with an adjustable focus, can obtain images from the scleral angle, as well as from other ocular structures like the iris, thus assisting in the diagnosis of numerous pathologies.

Phase III study of all-trans retinoic acid in previously untreated patients 61 years or older with acute myeloid leukemia.

The purpose of our study was (i) to evaluate the impact of all-trans retinoic acid (ATRA) given as adjunct to chemotherapy and (ii) to compare second consolidation vs maintenance therapy in elderly patients with acute myeloid leukemia (AML). A total of 242 patients aged >or=61 years (median, 66.6 years) with AML were randomly assigned to ATRA beginning on day +3 after the initiation of chemotherapy (ATRA-arm, n=122) or no ATRA (standard-arm, n=120) in combination with induction and first consolidation therapy. A total of 61 patients in complete remission (CR) were randomly assigned to second intense consolidation (n=31) or 1-year oral maintenance therapy (n=30). After induction therapy the intention-to-treat analysis revealed a significant difference in CR rates between the ATRA- and the standard-arm (52 vs 39%; P=0.05). Event-free (EFS) and overall survival (OS) were significantly better in the ATRA-compared to the standard-arm (P=0.03 and 0.01, respectively). OS after second randomization was significantly better for patients assigned to intensive consolidation therapy (P<0.001). The multivariate model for survival revealed lactate dehydrogenase, cytogenetic risk group, age, and first and second randomization as prognostic variables. In conclusion, the addition of ATRA to induction and consolidation therapy may improve CR rate, EFS and OS in elderly patients with AML.

[Monolayered amniotic membrane transplantation as a palliative treatment for bullous keratopathy]. / Injerto de membrana amniótica monocapa como tratamiento paliativo de la queratopatía bullosa.

PURPOSE: To determine the efficacy of monolayered amniotic membrane transplantation for short and medium-term symptomatic improvement in those cases of bullous keratopathy where a penetrating keratoplasty is not indicated. METHODS: Amniotic membrane transplantation was performed in five patients with symptomatic bullous keratopathy in which penetrating keratoplasty was not indicated. Data about bandage contact lens need before and after surgery, existence of ocular pain, occurrence and extension of bullae, epithelial defects, and persistence of amniotic membrane, were obtained at days 4 and 14, and months 1, 2, 4 and 6 after surgery. RESULTS: No pain, bullae or epithelial defects were present at day 14 in any patient. However, progressive dissolvement of the amniotic membrane was accompanied with more bullae and pain. At 6th month, 4 of 5 patients required bandage contact lens. CONCLUSION: Amniotic membrane transplantation alleviates short-term symptomatic bullous keratopathy and preserves corneal anatomy. Six months after surgery, anatomical and symptomatic improvement does not avoid the need of bandage contact lens.

Risk-adapted postremission therapy in acute myeloid leukemia: results of the German multicenter AML HD93 treatment trial.

The objective of the AML HD93 treatment trial was to evaluate the outcome in young adults with acute myeloid leukemia (AML) after postremission therapy was stratified according to cytogenetically defined risk. The rationales for the study design were based (i) on previous favorable results with high-dose cytarabine in AML with t(8;21), inv/t(16q22) and in AML with normal karyotype, and ii) on encouraging results obtained in several phase II trials using autologous stem cell transplantation (SCT). Between July 1993 and January 1998, 223 eligible patients, 16-60 years of age with newly diagnosed AML other than French-American-British type M3/M3v, were entered into the trial. Risk groups were defined as follows: low risk: t(8;21) or inv/t(16q22); intermediate risk: normal karyotype; high risk: all other chromosomal abnormalities. Following intensive double induction therapy with idarubicin, cytarabine and etoposide, all patients in complete remission (CR) received a first consolidation therapy with high-dose cytarabine and mitoxantrone (HAM). A second consolidation therapy was stratified according to the risk group: low risk: HAM; intermediate risk: related allogeneic SCT or sequential HAM; high risk: related allogeneic or autologous SCT. Double induction therapy resulted in a high CR rate of 74.5%, and 90% of the responding patients were eligible for consolidation therapy. Survival for all 223 trial entrants was 40%, and for the 166 patients who entered CR, disease-free (DFS) and overall survival were 40 and 51% after 5 years, respectively. Within the low-, intermediate- and high-risk groups, DFS and survival after 5 years were 62.5 and 87, 40 and 49 and 17 and 26% respectively, without advantage for allogeneic transplantation in the intermediate- and high-risk groups. Postremission therapy-related mortality was 0, 7 and 14%, respectively. This study demonstrates the feasibility of cytogenetically defined risk-adapted consolidation therapy. The overall trial results are at least equivalent to those of published trials supporting the risk-adapted treatment strategy.

[Analytic management in ophthalmology: towards a cost per hospital out- and in-patient]. / La contabilidad analítica en oftalmología: hacia el coste por paciente hospitalario.

PURPOSE: To determine the total annual cost of the activities in the Ophthalmology Department at the Hospital La Mancha-Centro, Homogeneous functional group (HFG) of the INSALUD, and to estimate the cost per patient/process of both ambulatory patients and hospital in-patients. METHODS: The study was designed over 1 year period, from January to December 1997, and applied by using analytic accounting in 17,857 patients seen at the out-patient ophthalmology clinic and 973 hospital in-patients who were classified by diagnosis-related groups (DRG) and sorted out by analysis of Case Mix index. RESULTS: We have determined the annual cost of our activity in both the ambulatory patient group and the hospital in-patient group classified by DRG and obtained the cost per process. CONCLUSIONS: To know systematically the annual cost of our medical and surgical activity is nowadays a necessary tool in the framework of the Total Quality Plan developed by the INSALUD's SIGNO Group since 1991. This leads us to the analytic management area and places us in the never ending search for the ideal model of cost per process determination.

Primary gastrointestinal non-Hodgkin's lymphoma: I. Anatomic and histologic distribution, clinical features, and survival data of 371 patients registered in the German Multicenter Study GIT NHL 01/92.

PURPOSE: The study was initiated to obtain epidemiologic data and information on anatomic and histologic distribution, clinical features, and treatment results in patients with primary gastrointestinal non-Hodgkin's lymphomas (PGI NHL). PATIENTS AND METHODS: Between October 1992 and November 1996, 371 PGI NHL patients were eligible to evaluate clinical features. Radiotherapy and chemotherapy were stratified according to histologic grading, stage, and whether surgery had been carried out or not. RESULTS: A total of 74.8% patients had gastric NHL (PGL). Within the intestine, the small bowel and the ileocecal region were involved in 8.6% and 7.0% of the cases, respectively. Multiple GI involvement (MGI) was 6.5%. Approximately 90% of the GI NHL were in stages IE/IIE. Aggressive NHL accounted for the majority, with a distinguishable pattern in several sites. Forty percent of PGL were of low-grade mucosa-associated lymphatic tissue type. One third of large-cell lymphomas had low-grade components. Most intestinal NHL were germinal-center lymphomas. The site of origin was prognostic. In gastric and ileocecal lymphoma, event-free (EFS) and overall survival (OS) were significantly higher as compared with the small intestine or MGI (median time of observation, 51 months). In PGL, localized disease was prognostic for EFS and OS. Histologic grade influenced only EFS significantly. Numbers in intestinal lymphomas were too small for subanalyses. CONCLUSION: PGI NHL are heterogeneous diseases. The number of localized PGL allowed for detailed analyses. Larger studies are needed for stages III and IV and for intestinal NHL. A uniform reporting system for PGI NHL, in terms of definitions and histologic and staging classifications, is needed to facilitate comparison of treatment results.

Primary gastrointestinal non-Hodgkin's lymphoma: II. Combined surgical and conservative or conservative management only in localized gastric lymphoma--results of the prospective German Multicenter Study GIT NHL 01/92.

PURPOSE: The aim of the study was to obtain data on anatomic and histologic distribution, clinical features, and treatment results of patients with primary gastrointestinal non-Hodgkin's lymphomas, particularly combined surgical and conservative treatment (CSCT) versus conservative treatment (CT) alone for primary gastric lymphoma (PGL) in localized stages. PATIENTS AND METHODS: Whether the treatment included surgery was left to the discretion of each participating center. Radiotherapy (Rx) and chemotherapy were stratified according to histologic grading, stage, and the inclusion or omission of surgery as follows: patients with low-grade PGL were treated with extended-field (EF) Rx (30 Gy). In case of residual tumor after surgery or in case of CT only (in stage IIE after six cycles of cyclophosphamide, vincristine, and prednisone), an additional boost of 10 Gy was given. All patients with high-grade PGL were treated with four (stage IE) or six (stage IIE) cycles of cyclophosphamide, doxorubicin, vincristine, and prednisone followed by EF Rx (stage IE) or involved-field (IF) Rx (stage IIE). Rx dosage corresponded to low-grade NHL. RESULTS: Between October 1992 and November 1996, 106 patients had CT only. The survival rate (SR) after 5 years was 84.4% and was influenced neither by patients' characteristics nor by stage or histologic grade. Seventy-nine patients had CSCT. Their SR was 82.0%. Complete resection of the tumor (R0) was prognostic for the overall survival (P =.0165) as compared with incomplete resection. CONCLUSION: Although the study was not randomized, a stomach-conserving approach may be favored.