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.     

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.     

Pulse pressure is more susceptible to the white coat effect than is systolic blood pressure: observations from real-life ambulatory blood pressure monitoring

BACKGROUND: Pulse pressure is a derivative of arterial stiffness. We have previously demonstrated ambulatory pulse pressure to be relatively independent from the blood pressure (BP) lowering during sleep, and thus of a neurogenic effect. On the other hand, white coat BP effects are thought to involve neurogenic activation. The aim of this work was to analyze white coat induced variability in pulse pressure. METHODS: Percent clinic-awake differences in systolic BP (SBP) and pulse pressure (white coat effects) were calculated for 688 consecutive subjects (mean age 60 +/- 16 years, 58% female). Of the subjects, 23% had controlled hypertension, 45% uncontrolled hypertension, 8% normotension, and 4% isolated office hypertension; all were referred to our unit for 24 h ambulatory BP monitoring. RESULTS: Pulse pressure highly correlated with SBP (r = 0.82, P <.00001). We found a larger white coat effect on pulse pressure than on SBP (8.3% and 5.2%, respectively, P < or =.0001). This was true in all subgroups except in normotensive subjects. Specifically, the magnitude of the white coat effect on pulse pressure was greater than on SBP in subjects with treated hypertension, untreated hypertension, and isolated office hypertension, and in young hypertensive subjects, older subjects, and those with diabetes. CONCLUSIONS: Although pulse pressure is related to the mechanical properties of large arteries, it is also influenced by the white coat effect, a neurogenic process. Furthermore, in hypertensive but not in normotensive subjects, the white coat effect on pulse pressure is significantly more pronounced than on SBP.     

Acute renal embolism. Forty-four cases of renal infarction in patients with atrial fibrillation

Acute renal embolus is rarely reported in the medical literature; thus, accurate data regarding presentation, laboratory tests, diagnostic techniques, and treatment are lacking. To better define this condition, we examined the medical records of all patients admitted to Kaplan Medical Center and Sheba Medical Center in central Israel from 1984 to 2002 who had a diagnosis of renal infarction and atrial fibrillation. We noted demographic, clinical, and laboratory parameters; method of diagnosis; treatment received; and patient outcome. We identified 44 cases of renal embolus: 23 females and 21 males, with an average age of 69.5 +/- 12.6 years. Thirty (68%) patients had abdominal pain, and 6 (14%) had a previous embolic event. Nine patients were being treated with warfarin on admission, 6 (66%) of whom had an international normalized ratio (INR) < 1.8. Hematuria was present in 21/39 (54%), and 41 (93%) patients had a serum lactate dehydrogenase (LDH) level > 400 U/dL. The mean LDH was 1100 +/- 985 U/dL. Diagnostic techniques included renal isotope scan, which was abnormal in 36/37 cases (97%); contrast-enhanced computed tomography (CT) scan, which was diagnostic in 12/15 cases (80%); and ultrasound, which was positive in only 3/27 cases (11%). Angiography was positive in 10/10 cases (100%). Twenty-three (61%) of 38 patients had normal renal function on follow-up. The 30-day mortality was 11.4%. Renal embolus was diagnosed mainly in patients aged more than 60 years, some of whom had a previous embolic event. Most of those receiving anticoagulant therapy had a subtherapeutic INR. Abdominal pain was common, as well as hematuria and an elevated LDH. These patients are at risk of subsequent embolic events to other organs. The most sensitive diagnostic technique in this population is a renal isotope scan, but contrast-enhanced CT scan requires further assessment.     

Advanced atrioventricular conduction block in acute rheumatic fever

We carried out a retrospective case control analysis to evaluate the outcome, and the need for treatment, of problems with atrioventricular conduction occurring during an acute attack of rheumatic fever, assessing the occurrence of second and third atrioventricular block versus first degree block. We reviewed and analysed the clinical, electrocardiographic and echocardiographic records of all children diagnosed in a single institute as having acute rheumatic fever during a period of seven consecutive years. During the period from October, 1994, through October, 2001, 65 children meeting the modified Jones criterions for acute rheumatic fever were hospitalized in the Soroka University Medical Center, Israel. First-degree atrioventricular block was identified in 72.3% of the children, and resolved with no specific treatment other than non-steroidal anti-inflammatory medications. Second-degree atrioventricular block of Mobitz type I, was observed in one child (1.5%), which progressed from first-degree block, and subsequently resolved. Complete atrioventricuar block was found in 3 children (4.6%), one progressing from Mobitz type I second-degree block, and two being seen as the first presentation. Of the three children with complete atrioventricular block, one patient was not treated, the second was treated with aspirin, and the final one with combined aspirin and steroids. The disturbances of conduction resolved in all three. We conclude that advanced atrioventricular block is rare during acute rheumatic fever. If occurring, block appears to be temporary, and resolves with conventional anti-inflammatory treatment. Specific treatment, such as insertion of a temporary pacemaker, should be considered only when syncope or clinical symptoms persist.     

No association between histo-blood group antigens and susceptibility to clinical infections with genogroup II norovirus

Noroviruses (NoVs) are a leading cause of viral gastroenteritis in humans. In the present study, the association between NoV susceptibility and the ABO histo-blood group was studied during 2 outbreaks of acute gastroenteritis in military units in Israel caused by genogroup II (GII) NoVs. The findings demonstrate that, unlike for genogroup I of NoV, there is no association between the ABO histo-blood group and clinical infection with GII NoVs. This is the largest study to test the association between NoVs, proven clinical infection with GII, and the ABO histo-blood group.     

Short-term course and outcome of treatments of pleural empyema in pediatric patients: repeated ultrasound-guided needle thoracocentesis vs chest tube drainage

BACKGROUND: Several reports have suggested that early chest tube drainage (CTD) may not be necessary in the treatment of severe pleural empyema (PE) in pediatric patients if appropriate antibiotic therapy and supportive care are provided. OBJECTIVES: A prospective open study to compare the short-term course of two treatment protocols of severe PE in pediatric patients. STUDY DESIGN: One group of 32 patients was treated with early insertion of a chest tube for CTD, and a second group of 35 patients was treated by a repeated ultrasound-guided needle thoracocentesis (RUSGT). The severity of the empyema was assessed by chest radiograph, the amount of fluid drained, the number of days the patient had experienced a fever, and the duration of antibiotic treatment. RESULTS: No significant differences were found between the two groups (RUSGT vs CTD) in all of the following measurements: mean (plus minus SD) duration of a temperature > or = 39 degreesC, 6.2 +/- 2.4 vs 6.5 +/- 1.8 days, respectively; mean duration of a temperature > or = 38 degreesC, 9 +/- 3.9 vs 8.2 +/- 4.5 days, respectively; fluid drained, 35.1 + 23.8 vs 30 +/- 28.2 mL/kg, respectively; duration of antibiotic treatment, 30 +/- 13.2 vs 30.2 +/- 7.3 days, respectively; and length of hospitalization and home IV treatment, 22 +/- 7.6 vs 24.2 +/- 7.5 days, respectively. A failure to respond to treatment occurred in three patients in the RUSGT-treated group and in five patients in the CTD-treated group. The failure to respond occurred in the RUSGT-treated group only in those patients with very large empyemas that caused mediastinal deviation. CONCLUSION: The treatment of PE by RUSGT is as efficacious as CTD, unless PE causes mediastinal deviation.