Pitfall in diagnosis of Crohn's disease in a cystic fibrosis patient

Cystic fibrosis (CF) and Crohn's disease may both present as failure to thrive and recurrent intestinal obstruction. Proper treatment and adequate nutrition may reverse these manifestations and improve the patient's quality of life. We describe a girl with CF who, despite appropriate management, failed to grow and had several episodes of bowel obstruction. After the additional diagnosis of Crohn's disease was reached, the patient improved on antiinflammatory and nutritional therapy. This patient illustrates the pitfall in the diagnosis of Crohn's disease in a CF patient due to the clinical overlap between the two conditions. We suggest that therapeutic failure in a chronic disease justifies additional diagnostic efforts resulting in a completion of diagnosis and significant changes in management.     

Intracranial ependymoma in children: analysis of prognostic factors

Summary Between 1955 and 1986, 25 children (aged 2 weeks to 15 years) were treated for intracranial ependymoma at M.D. Anderson Cancer Center. Nine patients had supratentorial primaries (5 high-grade, 4 low-grade), and 16 had infratentorial primaries (9 high-grade, 7 low-grade). Five patients had gross complete resection and 20 had incomplete resection. Seven patients received craniospinal irradiation (25–36 Gy to the neuroaxis, 45–55 Gy to tumor bed), 12 received local field irradiation (29–60 Gy, median 50 Gy). Five infants had adjuvant chemotherapy without radiotherapy, and 6 children had postradiotherapy adjuvant chemotherapy, and 12 patients had salvage chemotherapy with various agents and number of courses. Eight patients are alive, disease-free and without relapse from 1 year to 12 1/2 years from diagnosis (median 42 months). The primary failure pattern was local recurrence. The data suggest that 1) the long-term cure rate of children with ependymoma is suboptimal; 2) histologic grade may be of prognostic importance for supratentorial tumors; 3) prognosis appears worse for girls and infants under 3 years of age; 4) in well-staged patients routine spinal irradiation could be omitted; 5) the role of adjuvant chemotherapy is unclear. Address for offprints: Shiao Y. Woo, Department of Radiation Oncology, 6565 Fannin, M.S. DB1-37, Houston, Texas 77030, USA     

Duplication of distal 22q

We report on three patients with duplication of distal 22q. One patient is a de novo carrier of the translocation t(21;22) (p13;q11), the other two are offspring of a translocation carrier t(10;22) (q26;q12). The clinical manifestations of these patients demonstrate the variability of the dup(22q) syndrome.     

Fast neutron radiotherapy for locally advanced prostate cancer: results of an RTOG randomized study

Between June 1977 and April 1983, the Radiation Therapy Oncology Group (RTOG) sponsored a Phase III randomized study investigating fast neutron radiation therapy in the treatment of patients with locally advanced (Stage C and D1) adenocarcinoma of the prostate gland. Patients were randomized to receive either conventional photon radiation therapy or fast neutron irradiation used in a mixed-beam treatment schedule (neutron/photon). A total of 91 analyzable patients were entered in the study; 78 of them were treated without major protocol deviations. The two treatment groups were balanced in regard to all major prognostic variables. Actuarial curves for "overall" survival, "determinantal" survival and local/regional control are presented both for the entire group of 91 patients and the 78 patients treated within protocol guidelines. The overall local/regional tumor recurrence rate is 7% for the mixed-beam treated group of patients and is 22% for the photon (X ray) treated group of patients. The difference is statistically significant at the p = 0.05 level. For the entire group of 91 evaluable patients, the 5-year "overall" survival rate is 62% for the mixed-beam-treated group and 35% for the photon-treated group. This difference is also statistically significant (p less than 0.05). However, this statistical significance is lost when the smaller number of patients treated strictly within protocol guidelines is considered. The significance is regained (p less than 0.02) when one looks at "determinantal" survival, which uses active cancer at time of death as the failure endpoint. This study demonstrates that a regional treatment modality, in this case mixed-beam irradiation, can influence both local/regional tumor control and survival in patients with locally-advanced adenocarcinoma of the prostate gland.     

Lack of effect of cannabinoids on carbonic anhydrase

Summary Cannabinoids have significant anticonvulsant effects, the mechanism of which is obscure. In addition, these agents reduce intraocular pressure. Inhibitors of carbonic anhydrase also share these two pharmacological actions. In order to investigate whether these actions of Cannabinoids are mediated through inhibition of carbonic anhydrase (CA), commercial solutions of the enzyme were exposed to ⁹-tetrahydrocannabinol and cannabidiol. No inhibition was observed at concentrations of 10^–7–10^–6 M.     

YKL-40 expression is associated with poorer response to radiation and shorter overall survival in glioblastoma.

PURPOSE: YKL-40 is a secreted protein that has been reported to be overexpressed in epithelial cancers and gliomas, although its function is unknown. Previous data in a smaller sample set suggested that YKL-40 was a marker associated with a poorer clinical outcome and a genetically defined subgroup of glioblastoma. Here we test these findings in a larger series of patients with glioblastoma, and in particular, determine if tumor YKL-40 expression is associated with radiation response. EXPERIMENTAL DESIGN: Patients (n=147) with subtotal resections were studied for imaging-assessed changes in tumor size in serial studies following radiation therapy. An additional set (n=140) of glioblastoma patients who underwent a gross-total resection was tested to validate the survival association and extend them to patients with minimal residual disease. RESULTS: In the subtotal resection group, higher YKL-40 expression was significantly associated with poorer radiation response, shorter time to progression and shorter overall survival. The association of higher YKL-40 expression with poorer survival was validated in the gross-total resection group. In multivariate analysis with both groups combined (n = 287), YKL-40 was an independent predictor of survival after adjusting for patient age, performance status, and extent of resection. YKL-40 expression was also compared with genetically defined subsets of glioblastoma by assessing epidermal growth factor receptor amplification and loss at chromosome 10q, two of the common recurring aberrations in these tumors, using fluorescent in situ hybridization. YKL-40 was significantly associated with 10q loss. CONCLUSIONS: The findings implicate YKL-40 as an important marker of therapeutic response and genetic subtype in glioblastomas and suggest that it may play an oncogenic role in these tumors.     

Guidance of Post Myocardial Infarction Ventricular Septal Defect and Pseudoaneurysm Closure

Left ventricular pseudoaneurysm and ventricular septal defect are rare but devastating complications of myocardial infarction. With medical treatment alone, the majority of patients will die from these complications. Until recently, the recommended treatment was surgical closure. These surgeries carried extreme risk due to abnormal hemodynamics, necrotic substrates and the comorbidities of these patients. Recently, trans-catheter closure was shown to be an acceptable alternative to open surgical intervention. 3D echocardiography identifies the location, size, and shape of the defect and can assess, guide, and follow up the closure procedure.     

Asistencia al niño y adolescente con diabetes. Unidades de referencia en diabetes pediátrica

Intensive treatment of type 1 diabetes mellitus (DM1) delays and slows down the progression of chronic diabetes complications (DCCT 1993).This type of treatment in children and adolescents with DM1 has a different complexity to other stages of life and therefore, needs specialized care units. Various documents and declarations of diabetic patient's rights are evaluated, and the need for an adequate health care is emphasized.In the last decade, several projects have been developed in Europe to create a benchmark treatment of pediatric diabetes, with the aim of establishing hospitals with highly qualified healthcare to control it.The Diabetes Working Group of the Spanish Society for Pediatric Endocrinology (SEEP) has prepared this document in order to obtain a national consensus for the care of children and adolescents with type 1 diabetes in specialist Pediatric Diabetes Units, and at the same time advise Health Care Administrators to establish a national healthcare network for children and adolescents with diabetes mellitus, and organize comprehensive pediatric diabetes care units in hospitals with a reference level in quality of care.     

Exposure to Family Violence of Israeli Inmates: Does Sex Make a Difference?

The purpose of the current study was to examine differences by sex (a) in history of child abuse and neglect (CAN) and exposure to parental partner violence (PPV); and (b) in the association of CAN and PPV with violent offenses, substance use, suicidal behavior, and psychiatric problems. This cross-sectional study investigated sex differences in CAN and exposure to PPV in a sample of 290 Israeli inmates (65 women and 225 men). Participants completed a self-report measures of the Childhood Trauma Questionnaire, and the revised Conflict Tactics Scale to measure PPV. The findings indicated that the female inmates had experienced significantly more sexual abuse (p?<?.001), and more exposure to PPV (p?=?.030), compared with male inmates. Female inmates who had experienced CAN were at higher risk of committing violent offenses than male inmates and female inmates were also more likely than male inmates to have been engaged in suicidal behaviors (39.3% and 18.5%, respectively), and to have had psychiatric problems (40% and 14.2%, respectively). The findings also revealed that psychiatric problems and suicidal behavior can be predicted by CAN and family substance use. The findings highlight the effects of the adverse childhood experience of exposure to CAN and PPV on behavioral problems. Appropriate treatment requires simultaneous intervention that targets all three issues of childhood trauma, substance use disorders, and psychiatric problems for both sexes.