Bronchial lobar obstruction from a sucralfate tablet: a rare cause of acute respiratory insufficiency]

In 1995, the Committee on Sports Medicine and Fitness of the American Academy of Pediatrics (AAP) published a position paper on atlantoaxial instability in children with Down syndrome in which a previous statement on the same subject published in 1984 (Table) was retired. The 1995 statement includes several arguments that disfavor screening of children with Down syndrome for atlantoaxial instability. Whereas some of these arguments are well founded, other lack substantive evidence that would support the statement. In the following discussion, I attempt to analyze some of these arguments made in the 1995 statement and provide a viewpoint that favors radiologic examinations of the cervical spine of children with Down syndrome.     

Cutaneous CD30+ lymphoproliferative disorders: expression of bcl-2 and proteins of the tumor necrosis factor receptor superfamily

The spectrum of CD30+ cutaneous lymphoproliferative disorders is characterized by the histology of a high-grade lymphoma but frequent clinical regression of skin lesions in lymphomatoid papulosis (LyP) and occasional regression in CD30+ large cell lymphomas (LCLs). A recent study shows that apoptosis may be a significant mechanism of regression of LyP (Arch Dermatol 133:828-833, 1997). Therefore, we studied expression of proteins that induce apoptosis, including CD27, CD40, CD95, and nerve growth factor receptor (NGF-R), as well as anti-apoptotic protein bcl-2 in skin lesions from 25 patients within the spectrum of CD30+ cutaneous lymphoma. Our results show consistent expression of CD95 (APO-1/Fas), but rare or absent expression of CD27, CD40, and NGF-R on tumor cells from both regressing LyP lesions and nonregressing CD30+ lymphomas. Bcl-2 was expressed at low levels in LyP and at high levels in pleomorphic CD30+ lymphomas. These results indicate that, in addition to CD30, CD95 expression is preferentially expressed at high levels in all cutaneous CD30+ lymphomas and suggest that CD95 may play a role in the regression of CD30+ skin lesions. Expression of bcl-2 appears to protect tumor cells from apoptosis in CD30+ lymphoproliferative disorders.     

Outcome of 1000 consecutive clinic- and hospital-based cataract surgeries in a Danish county

PURPOSE: To evaluate the homogeneity and quality of cataract surgery in a Danish county. SETTING: Four private eye clinics and the Department of Ophthalmology, Hiller?d Hospital, Frederiksborg County, Denmark. METHODS: This prospective study comprised 1012 consecutive cases of age-related cataract; 48% of the surgeries were performed in the hospital and 52%, at 1 of 4 clinics. Demographic and clinical data were recorded on standardized data sheets at referral, surgery, and final refraction. Main outcome measures were change in best corrected visual acuity (BCVA), refractive power at final refraction, surgery-related complications, and waiting time for surgery and final refraction. RESULTS: The hospital group had greater dispersion of age (P < .001) and higher frequency of general health problems (P < .005) and glaucoma (P < .01) than the clinic group. Fifty-four percent of surgeries were by phacoemulsification and 46%, by extracapsular cataract extraction (ECCE). In general, phacoemulsification was prevalent at the hospital and ECCE at the clinics. No difference was found between groups in visual acuity at final refraction. Of all patients, 87.1% attained a BCVA of 0.5 or better and of the best cases, 96.2%. Zonule or capsule rupture with or without vitreous loss occurred more often in the hospital group (P < .05), while the incidence of postoperative complications was identical in the 2 groups. One cases of retinal detachment was found. Time from referral to final refraction was shorter at clinics, while waiting time from surgery to final refraction was shorter at the hospital (P < .001). CONCLUSION: Dividing cataract surgery between hospital and private clinics seems to be a satisfactory model for meeting the increasing demand for cataract surgery.     

Case report: secondary biliary cirrhosis possibly related to congenital hepatic fibrosis. Evidence for decreased number of portal branch veins and hypertrophic peribiliary vascular plexus

A 30-year-old man with presinusoidal portal hypertension was transplanted for cryptogenic cirrhosis. On the explanted liver, few intrahepatic stones, biliary cirrhosis, chronic cholangitis of the large bile ducts and a peculiar proliferation of small dilated bile ducts at the periphery of the portal tracts led to the diagnosis of secondary biliary cirrhosis and cholangitis, possibly linked to ductal plate malformation, including congenital hepatic fibrosis associated with a minor form of Caroli's disease. Ex vivo portogram and histology showed the paucity of portal vein branches and the hypertrophy of the peribiliary vascular plexus. This hypertrophy, which has been reported in livers with presinusoidal hypertension, is another indirect argument to suggest the diagnosis of congenital hepatic fibrosis.     

Increased intraventricular velocities: an unrecognized cause of systolic murmur in adults

OBJECTIVES: The purpose of this study was to determine the frequency, clinical features and echocardiographic characteristics of increased intraventricular velocities (IIVs) in patients referred to the echocardiography laboratory for systolic murmur. BACKGROUND: A subset of patients referred to the echocardiography laboratory for evaluation of a systolic murmur have IIVs in the absence of other recognized causes of systolic murmur. METHODS: We prospectively studied echocardiograms from 108 consecutive patients referred for evaluation of a systolic murmur. Clinical data were obtained from patient examinations and medical records. RESULTS: The sole explanation for systolic murmur was IIVs in 16.7% of referred patients. Compared with those without IIVs, patients with IIVs had a higher ejection fraction (EF) (58.7+/-7.8% vs. 51.1+/-12.5%, p < 0.001), percent fractional shortening (42.3+/-9.7% vs. 31.0+/-11.4%, p < 0.0001), left ventricular (LV) mass index (181+/-70 vs. 152+/-48 g/m2, p=0.046) and prevalence of hypertension (73.3% vs. 51.7%, p=0.043) and a lower prevalence of segmental wall motion abnormalities (2.2% vs. 39.3%, p < 0.001). CONCLUSIONS: Increased intraventricular velocities are a common cause of systolic murmur in this group of patients and should be included in the differential diagnosis of systolic murmurs in adults. The association of IIVs with LV hypertrophy should be a clinical consideration when these murmurs are identified.     

Coreceptor usage of human immunodeficiency virus type 2 primary isolates and biological clones is broad and does not correlate with their syncytium-inducing capacities

Entry of human immunodeficiency virus type 1 (HIV-1) into target cells is mediated by binding of the surface envelope glycoprotein to the CD4 molecule. Interaction of the resulting CD4-glycoprotein complex with alpha- or beta-chemokine receptors, depending on the biological phenotype of the virus, then initiates the fusion process. Here, we show that primary HIV-2 isolates and biological clones, in contrast to those of HIV-1, may use a broad range of coreceptors, including CCR-1, CCR-3, CCR-5, and CXCR-4. The syncytium-inducing capacity of these viruses did not correlate with the ability to infect via CXCR-4 or any other coreceptor. One cell-free passage of the intermediate isolates in mitogen-stimulated, CD8+ cell-depleted peripheral blood mononuclear cells resulted in the outgrowth of variants with CCR-5 only, whereas the coreceptor usage of late and early isolates did not change. Since HIV-2 is less pathogenic in vivo than HIV-1, these data suggest that HIV pathogenicity in vivo is not directly related to the spectrum of coreceptors used in in vitro systems.     

Survival and functional status after resection of recurrent glioblastoma multiforme

OBJECTIVE: To determine the selection factors for and results of second resections performed to treat recurrent glioblastoma multiforme (GM), we studied 301 patients with GM who were treated from the time of diagnosis using two prospective clinical protocols. METHODS: The patients were prospectively followed from the time of diagnosis, using clinical and radiographic criteria after maximal surgical resection and external beam radiotherapy with or without adjuvant chemotherapy. Resection of recurrent GM was performed at the recommendation of the treating clinicians. The results of the second resections were retrospectively reviewed and analyzed using multivariate logistic regression, Kaplan-Meier-Turnbull survival analysis, Cox regression, and propensity score stratification. RESULTS: Forty-six patients underwent second resections during the study period. The actuarial rate of the second resections was 15% of the patients 1 year after diagnosis and 31% 2 years after diagnosis. Younger age (P = 0.01) and more extensive initial resection (P = 0.02), but not Karnofsky Performance Scale (KPS) score at the time of diagnosis or recurrence, predicted a higher chance of selection for reoperation after initial tumor recurrence. Twenty-eight percent of the patients had improved KPS scores after undergoing reoperation, 49% were stable, and 23% had declines in KPS scores of 10 to 30 points. There was no operative mortality. After reoperation, 85% of the patients received chemotherapy, 11% received brachytherapy or underwent stereotactic radiosurgery, and 17% underwent third resections. The median survival period after reoperation was 36 weeks. Higher preoperative KPS scores predicted longer survival periods after reoperation (P = 0.03). Age and interval since diagnosis were not significant prognostic factors. The median high-quality survival period (KPS score, > or =70) was 18 weeks. The median survival period after first tumor progression was 23 weeks for 130 patients treated using the same protocols who did not undergo reoperations. Patients who did undergo reoperations experienced clinically and statistically significantly longer survival periods. However, this was determined to be partially because of selection bias. CONCLUSION: Survival after resection of recurrent GM remains poor despite advances in imaging, operative technique, and adjuvant therapies. High-quality survival after resection of recurrence to treat GM seems to have increased significantly since an earlier report from our institution.     

Novel hepatoselective insulin analogues: studies with covalently linked thyroxyl-insulin complexes

To explore the possibility that insulin analogues designed to have restricted access to peripheral tissues may display relative hepatoselectivity in vivo, Nalphabeta1-thyroxyl-insulin (B1-T4-Ins) and Nalphabeta1-thyroxyl-aminohexanoyl insulin (B1-T4-AHA-Ins) were synthesized. These insulin analogues bind thyroid hormone binding proteins to form high molecular weight complexes. Effects of intravenous infusions of B1-T4-Ins; B1-T4-AHA-Ins; combined thyroxine binding globulin (TBG) and B1-T4-Ins and combined TBG and B1-T4-AHA-Ins were compared with those of insulin infusion in hyperinsulinaemic euglycaemic clamp protocols in anaesthetized beagles (n=4 and n=3 for combined TBG infusions). Glucose turnover rates were measured using D-[3-3H]glucose infusion. With all 5 protocols the rate of glucose disappearance (Rd) was increased and the rate of endogenous glucose production (Ra) decreased from basal level 13.53+/-0.60 micromol kg(-1) min(-1)(p<0.05). Insulin-like activity for Ra and Rd was calculated as the area between the basal values of each variable and the subsequent values plotted graphically against time (AUC). For insulin, B1-T4-Ins, B1-T4-AHA-Ins, combined infusions of TBG+B1-T4-Ins, and TBG+B1-T4-AHA-Ins, respectively, AUC for Rd values were 6.30+/-0.69, 3.35+/-0.53, 4.40+/-0.64, 2.82+/-0.40 and 3.46+/-0.95 (mmol kg(-1)), all analogue infusions being different from insulin (p<0.05). AUC for Rd was further reduced by addition of TBG to B1-T4-AHA-Ins (p<0.05). In contrast the effect of all analogues on AUC for Ra was similar to that of insulin. These observations are compatible with the suggestion that insulin analogues which bind to thyroid hormone binding proteins retain access to hepatic insulin receptors which primarily control Ra. The reduced peripheral insulin-like effect (Rd) could be due to reduced transcapillary access to peripheral insulin receptor sites.     

Gallbladder volume and contractility in term and preterm neonates: normal values and clinical applications in ultrasonography

BACKGROUND: Normovolemic hemodilution is a well-accepted method for intraoperative blood salvage. However, some controversy exists concerning the possible risk of myocardial fiber injury as consequence of the reduced oxygen content. Laboratory diagnosis of perioperative myocardial fiber injury is difficult, since biochemical markers are elevated postoperatively due to the surgical trauma. Cardiac troponin I (cTnI) is a new, highly sensitive and specific marker for the detection of myocardial injury. The aim of our study was to investigate whether normovolemic hemodilution in patients with major orthopedic surgery (13 hemodiluted patients, 15 control) induces a release of cTnI. METHODS: cTnI as a highly specific and sensitive cardiac parameter, as well as total creatine kinase (CK), creatine kinase isoenzyme MB mass (CKMB mass) and myoglobin were measured after induction of anesthesia, after normovolemic hemodilution, prior to retransfusion of blood components, 3 h after surgery, and on the first and third postoperative days. RESULTS: Prior to retransfusion of blood components the hematocrit was decreased to 25.4 +/- 1.2% (mean +/- SEM; range: 18%-34%) in the control group and to 20.2 +/- 0.8% (mean +/- SEM; range: 17%-24%) in the hemodilution group. Total CK, CKMB mass as well as myoglobin concentration increased significantly in both groups, reaching their maxima within the first day of surgery. In contrast, cTnI was below the detection limit of assay (< 0.5 micrograms/L) at any time. CONCLUSIONS: We suggest that pre- and intraoperative hemodilution to a hematocrit of approximately 20% by maintaining normovolemia does not induce myocardial fiber injury in patients without preexisting cardiac diseases.