Sphenoid sinus brown tumor, hypercalcemia, and blindness: an unusual presentation of primary hyperparathyroidism

This is a first report of primary hyperparathyroidism (HPT) masquerading as a destructive fibrous sphenoid sinus "Brown tumor" associated with progressive blindness and hypercalcemia. Diagnosis of a Brown tumor was delayed despite serial computerized tomography of the head and repeated transnasal and transethmoid sphenoid biopsies demonstrating diffuse fibrosis. Only detection and medical evaluation of hypercalcemia, demonstrating elevation of both serum calcium and C-terminal parathyroid hormone with an elevated chloride/phosphate ratio, prompted neck exploration, thus confirming a solitary left superior parathyroid adenoma. Postoperative normocalcemia occurred synchronously with the return of light perception and the arrest of sphenoid sinus and parasellar erosion. Although maxillary Brown tumors of secondary HPT have been reported, this is the first report of osteitis fibrosa of the sphenoid sinus. Differential diagnosis of an erosive sphenoid lesion with cranial nerve dysfunction, exclusive of inflammatory or vascular disease, should include sarcoidosis, primary and metastatic sphenoid carcinoma, fibrous dysplasia, giant cell reparative granuloma, midline lethal granuloma, chordoma, and chondrosarcoma. Furthermore, the bony destructive lesions with concomitant hypercalcemia of sarcoidosis and HPT are distinguishable by radiographic and laboratory analyses and by the Dent corticosteroid suppression test. Hypercalcemia of primary HPT is associated with elevated serum C-terminal parathormone, osteitis fibrosa, a negative Dent test, and a chloride/phosphate ratio greater than 33 in 94% of primary HPT patients. Hypercalcemia of sarcoidosis is associated with a normal or decreased C-terminal parathormone assay and a positive Dent test, as well as elevated serum immunoglobulins and erythrocyte sedimentation rate, and a positive angiotensin-converting enzyme assay.(ABSTRACT TRUNCATED AT 250 WORDS)     

Race as a risk factor in the severity of infragenicular occlusive disease: study of an urban hospital patient population

We retrospectively studied the arteriograms of 135 men admitted for evaluation of lower extremity ischemia to examine whether race influences the severity of infragenicular occlusive disease. The scoring system prepared by the Ad Hoc Committee on Reporting Standards for the Society for Vascular Surgery and the International Society for Cardiovascular Surgery was used to grade the severity of stenosis in each of the upper, middle, and lower thirds of the anterior and posterior tibial and peroneal arteries (collectively called "infragenicular" arteries). The patients were divided into two groups: 83 blacks (140 arteriogram limbs) and 52 whites (87 arteriogram limbs). Disease severity scores between the groups were compared, and the existence of five known risk factors for atherosclerosis were considered for poststratification adjustment. Results showed that higher disease scores, indicating more severe disease, were found in the black population in every segment of the infragenicular arteries. The mean (+/- SE) score for all the infragenicular segments in blacks was significantly higher than that in the whites (2.08 +/- 0.05 vs 1.57 +/- 0.06, p less than 0.001). The black and white groups were comparable in terms of age (65.2 vs 64.6 years), prevalence of diabetes (20% vs 25%), smoking history (93% vs 90%), and hypercholesterolemia (51% vs 63%). Hypertension was more prevalent among the black patients (51% vs 27%, p less than 0.001). When only the non-hypertensive patients in both groups were considered, however; the mean severity score was still significantly higher in blacks (2.10 +/- 0.06 vs 1.42 +/- 0.06, p less than 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)     

Photodynamic therapy for treatment of head and neck cancer

Since 1975, photodynamic therapy has reportedly been effective in a variety of head and neck malignancies that failed traditional (conventional) therapy, including surgery, cryotherapy, chemotherapy, hyperthermia, and radiation therapy. Photodynamic therapy consists of the intravenous administration of (di)hematoporphyrin ether, a chemosensitizing drug selectively retained by neoplastic and reticuloendothelial tissues which, when exposed to a 630-nm argon laser, catalyzes a photochemical reaction to release free oxygen radicals, "the cytotoxic" agents responsible for cell death and tumor necrosis. Preliminary investigations have assessed the efficacy of photodynamic therapy in treatment of: (1) superficial "condemned mucosa" or "field cancerization" of the oral cavity and (2) stage III and IV head and neck carcinomas that had unsuccessful conventional therapy. Complete and/or partial remissions were obtained in 11 of 12 patients (16 treatments) with a variety of carcinomas of the nasopharynx, palate and uvula, retromolar trigone, temporal bone, cervical esophagus, and AIDS-related Kaposi's sarcoma of the oral cavity.     

Allelic loss on chromosome-13 can preceed histological-changes in head and neck-cancer

Seventy paired tumor and blood samples from patients with upper aerodigestive tract squamous cell carcinoma (UADT SCC) were tested for allelic loss on chromosome 13. Increased loss of heterozygosity (LOH) was observed at 11 of 13 different highly polymorphic microsatellite 'CA' dinucleotide repeat-containing loci. Increasing percent LOH correlated with lymph node metastasis (N Stage) (p=0.016). LOH was also detected in 10 of 16 (63%) informative samples of histologically normal mucosa adjacent to the tumors. These findings demonstrate that allelic loss on chromosome 13 is a frequent event in UADT SCC. Furthermore, these genetic alterations can be detected prior to histological changes in normal mucosa adjacent to these tumors.     

New instruments, bone liners, and tray for finger joint arthroplasty

Titanium circumferential grommets have been developed for finger joint arthroplasty that fit on the base of the silicone implant stems to protect the flexible hinge from tearing and fracture. To facilitate grommet insertion, new intramedullary bone rasps have been devised with a reverse cutting tooth pattern, an extended shaft for an improved view of the surgical field, and a redesigned cutting head to allow for grommet insertion. Surgical accessibility and ease of recognition have been facilitated by the development of color-coded sizers. All of the instruments necessary for finger joint surgery are available in a molded tray that has also been redesigned with a transparent lid and clearly labeled compartments for accurate determination of instruments and implant sizers.     

Internal derangements of the shoulder: decision tree and cost-effectiveness analysis of conventional arthrography, conventional MRI, and MR arthrography

Purpose. In a patient with internal derangement of the shoulder, the diagnostic method of choice is controversial. Conventional arthrography can diagnose most rotator cuff tears accurately; however, in many institutions MR arthrography is usually necessary to diagnose labral tears. We utilized decision tree methodology to compare the cost- effectiveness of conventional arthrography and conventional MRI with a hypothetical algorithm in which a patient underwent arthrography, performed with admixed gadolinium, which if negative, was followed by MRI. Design. The use of double-contrast arthrography alone, conventional MRI alone, and gadolinium-enhanced MRI used as an adjunct to conventional arthrography were modeled for the diagnosis of full-thickness rotator cuff tears (RCT), partial RCTs, labral tears, and the absence of cuff/labral tears using decision analysis methodology. English language medical publications were searched to determine the base probabilities for the accuracy of the diagnostic tests. The outcome utilities ranged from –1 to +1 to reflect the value of correct diagnostic evaluation. Charges for diagnostic tests and appropriate surgical treatments were based on 1997 Medicare reimbursement rates for professional fees and hospital charges in an outpatient setting. Sensitivity analyses were performed to evaluate the effects of uncertainty regarding the prevalence of each disease state and the accuracy of several diagnostic tests. Results. In the base-case analysis, the average effectiveness of double-contrast arthrography alone, MRI alone and arthrography selectively followed by MRI were 0.6610, 0.6715, and 0.7204, respectively. The average costs for each of these strategies were $1090, $2033, and $2339, respectively. Conclusion. Arthrography performed with admixed diluted gadolinium, which if negative is immediately followed by MRI, was somewhat more expensive than conventional MRI. However, because of much greater effectiveness, cost-effectiveness was significantly higher for our proposed algorithm. Conventional arthrography without gadolinium, although less expensive, had severely limited effectiveness. Received: 4 June 1999 Revision requested: 6 August 1999 Revision received: 30 August 1999 Accepted: 1 September 1999     

Long-term outcome in 134 patients with galactosaemia

In a retrospective study 134 galactosaemic patients, born between 1955 and 1989 in the Federal Republic of Germany were traced and their long-term outcome evaluated. We investigated 83 galactosaemic patients (78 homozygotes, 5 compound heterozygotes) by clinical, psychometric and laboratory testing; 31 patients were evaluated by medical history, the remaining 20 patients had died due to sequelae of the underlying disease. In 48 out of 78 classical galactosaemia patients galactose-free therapy had been started before the 15th day, in 19 between days 15 and 56 and in 11 patients after the 56th day. Physical findings revealed that puberty was delayed in 1 out of 18 males and 6 out of 11 females. Neurological abnormalities included ataxia (n=6), intention tremor (n=11) and microcephaly (n=10). Speech abnormalities were found in 43 out of 66 patients over 3 years of age and disturbance of visual perception and/or arithmetic deficits in 29. Intelligence declined with age, i.e., a DQ or IQ less than 85 was found in 4 out of 34 patients less than 6 years of age (12%), in 10 out of 18 between 7 and 12 years (56%) and in 20 out of 24 older than 12 years (83%). Metabolite patterns (RBC galactose-1-phosphate and UDP-galactose, plasma and urinary galactitol) did not correlate with DQ or IQ. Dietary compliance was good in almost all patients. Compound heterozygotes (n=5) had normal mental and growth development and all laboratory parameters were in the normal range. The cause of the unsatisfactory outcome of well-treated galactosaemic patients with disturbances in long-term development remains unclear. This could be due to a chronic intoxication of galactose metabolites or a deficiency of UDP-galactose or galactose-containing glycoproteins or glycolipids.This study was supported by a grant from the Stifterverband für die Deutsche Wissenschaft (Huebner-stiftung), grant TS 114/18/89 and by the Arbeitsgemeinschaft für Pädiatrische Stoffwechselstörungen     

The cost of being perfect: perfectionism and suicide ideation in university students

OBJECTIVE: The aim of the study was to assess the relationship between dimensions of perfectionism and suicide ideation in a tertiary student population in Australia. METHOD: The methodology involved 405 students completing the General Health Questionnaire (GHQ-28) which includes a subset of questions which can be used to assess suicide ideation, and the Multidimensional Perfectionism Scale. RESULTS: The presence of suicide ideation was associated with higher scores on total perfectionism and two perfectionism dimensions, and total GHQ scores. There were significant differences between participants with high levels of perfectionism and participants with moderate to low levels of perfectionism on a measure of suicide ideation. Neither gender nor age were associated with differences in the scores, with results indicating high levels of perfectionism may indicate a vulnerability to suicide ideation. CONCLUSIONS: Perfectionism is a valued attribute in high-achieving populations. The question needs to be asked, however, at what cost? The findings indicate that high levels of perfectionism may be associated with an increased vulnerability to suicide ideation. Future research is needed to gain a better understanding of the complex interrelationship between personality and temperament, environmental factors and self-destructive behaviour.     

Man with cherubism

Cherubism was diagnosed in a male when he was 6 years old. Cherubism is a benign, bilateral, painless lesion. It is commonly located in the mandible but in a minority of patients also in the maxilla. Cherubism is a familial condition with an autosomal dominant pattern of inheritance. At least one member of the family of the patient described had cherubism. The disease becomes manifest during early childhood and progresses until puberty when it spontaneously regresses. In the majority of patients no treatment is required. However, in the patient presented surgical procedures and odontological corrections were necessary. Due to a flare-up of the disease at the age of 22 years, the patient was treated with calcitonin for 1 year followed by bisphosphonates. During these treatments the disease symptoms diminished.     

Utilization of "used" vials: cost-effective technique for MR arthrography

Because full vials of commercially available MR arthrographic contrast are expensive, we hypothesized that the small residual contrast in a "used" vial would be adequate for MR arthrography. After sterility testing and quantity analysis of the residual contrast in 28 vials, this method was successfully used in 10 patients. J. Magn. Reson. Imaging 2000;12:953-955.