National Survey of CT Colonography Practice in Ireland.

CT Colonography was first introduced to Ireland in 1999. Our aim of this study is to review current CT Colonography practices in the Republic of Ireland. A questionnaire on CT Colonography practice was sent to all non-maternity adult radiology departments in the Republic of Ireland with a CT scanner. The results are interpreted in the context of the recommendations on CT Colonography quality standards as published by the European Society of Gastrointestinal and Abdominal Radiology (ESGAR) consensus statement in the journal of European Radiology in 2013. Thirty centres provide CT Colonography; 21 of which responded (70%). Each centre performs median 90 studies per year; the majority follow accepted patient preparation and image acquisition protocols. Seventy-six percent of the centres repsonded that the majority of patients imaged are symptomatic. Of the 51 consultant radiologists reading CT Colonography, 37 (73%) have attended a CT Colonography course. In 17 (81%) of the centres the studies are single read although 81% of the centres have access to a second radiologist's opinion. Fourteen (67%) of the centres reported limited access to CT scanner time as the major limiting factor to expanding their service. CT Colonography is widely available in Ireland and is largely performed in accordance with European recommendations.

Subclassification of small bowel Crohn's disease using magnetic resonance enterography: a review using evidence-based medicine methodology.

Magnetic resonance enterography (MRE) has a growing role in imaging small bowel Crohn's disease (SBCD), both in diagnosis and assessment of treatment response. Certain SBCD phenotypes respond well to biologic therapy and others require surgery; MRE has an expanding role in triaging these patients. In this review, we evaluate the MRE signs that subclassify SBCD using evidence-based medicine (EBM) methodology and provide a structured approach to MRE interpretation.

Risk factors for the development of stress fractures during fluoride therapy for osteoporosis.

We attempted to identify risk factors for the development of lower limb stress fractures during fluoride therapy for osteoporosis (OP). We compared 18 patients who developed 41 such fractures (26 periarticular, 6 femoral neck, 5 long bone shaft, 1 greater trochanter and 3 pubic rami fractures) during fluoride therapy, with 24 similarly treated patients who did not develop stress fractures. Treatment consisted of sodium fluoride 0.99 mg/kg per day, elemental calcium 1 g/day, and vitamin D. We obtained a previous fracture history, annual radiographs of the spine (fractures), hands (metacarpal cortical index, MCI) and pelvis (Singh index, femoral cortical index), three-monthly serum fluoride and alkaline phosphatase levels, and pretreatment transiliac bone biopsies (routine histomorphometry). The stress fracture group was found to have, before treatment: lower MCI (p less than 0.05), lower trabecular bone volume (p less than 0.05), a lower number of trabeculae (p less than 0.05), greater trabecular separation (p less than 0.05), less extensive eroded surfaces (p less than 0.05), a lower double/single tetracycline label ratio (p less than 0.05); and during treatment: more new spinal fractures (p less than 0.05) and higher serum alkaline phosphatase levels (p less than 0.01). We conclude that stress fracture patients had more severe trabecular and cortical OP and possibly a poorer bone-forming capacity before therapy than patients without stress fractures. We suspect that fluoride therapy may temporarily further weaken bone and so lead to stress fractures in severely osteoporotic patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Iliac bone biopsies at the time of periarticular stress fractures during fluoride therapy: comparison with pretreatment biopsies.

We attempted to establish whether systemic changes in trabecular bone explain the development of stress fractures in the lower limbs during fluoride therapy for osteoporosis. To this end we compared transiliac bone biopsies obtained before treatment with those taken around the time of stress fractures after 14.3 +/- 10.9 (SD) months of therapy in six patients (group A). Biopsies from a comparable group of six patients without stress fractures at the time of the second biopsy (after 11.9 +/- 2.7 months of treatment) served for comparison (group B). The biopsies were processed undecalcified and examined by routine histomorphometry. The second biopsies did not show any significant improvement in mean bone volume or trabecular architecture. Although the second biopsies in group A had increased erosion surfaces (p less than 0.05) and greater osteoid volume (p less than 0.05), group B biopsies showed no difference in erosion surfaces but an increase in all osteoid parameters: osteoid volume (p less than 0.05), osteoid surface (p less than 0.05), and osteoid seam thickness (p less than 0.01). We reached the following conclusions: (1) the combination of increased erosion and replacement of removed bone by as yet unmineralized osteoid in the stress fracture group must have weakened bone and allowed the development of stress fractures. (2) Stress fracture patients may have mounted a less vigorous osteoblast response to fluoride than non-stress fracture patients. Under these conditions microfractures are likely to heal poorly and propagate to develop into full stress fractures. (3) Renal failure is a contraindication to fluoride therapy.

Using a self-reported functional score to assess disease progression in systemic sclerosis.

OBJECTIVES: This study compares the scleroderma Functional Score (FS) with the validated Disability Index of the Health Assessment Questionnaire (HAQ-DI) and other outcome measures. The aim is to determine if the FS is useful as an objective assessment tool for longitudinal evaluation of the functional impact of systemic sclerosis (SSc). METHODS: A cohort of 135 patients was studied (M:F, 15:120), with a mean age of 45.7 (S.D. = 13.2) at SSc disease onset. 69 (51%) had diffuse cutaneous scleroderma (dcSSc) and 66 (49%) had limited disease (lcSSc). The mean interval between the two assessments was 1.8 yrs (S.D. = 1.2). Functional impact was determined by evaluating archived self-reported questionnaires (FS, HAQ and scleroderma-VAS). Concurrent evaluation of the disease severity score was derived from clinical data stored in the hospital database and from medical case note reviews. RESULTS: At baseline, the mean FS was 11.0 (S.D. = 9.0) and at reassessment 12.0 (S.D. = 9.2). The mean absolute change in FS between the two assessments was 4.1 (S.D. = 4.9). With time 49% (n = 66) showed a clinically significant change in their functional ability with regard to FS, of these 29% (n = 39) worsened and 20% (n = 27) improved. There was an excellent cross-sectional correlation between the FS and the HAQ-DI (rho = 0.90; P < 0.0001). Also, a strong correlation between longitudinal change in these two outcome measures (rho = 0.59, P < 0.0001) was observed. CONCLUSIONS: This is the first longitudinal study of the scleroderma FS. It demonstrates that the FS can capture bidirectional and clinically significant changes in SSc related disability over time. The concurrent validity of the FS is asserted through its strong correlation with the HAQ-DI. The FS is a disease-specific, inexpensive and practical instrument for assessing functional status in SSc. It is a promising self-administered assessment tool for use in evaluating new SSc treatments.

Digital vascular responses and serum endothelin-1 concentrations in primary and secondary Raynaud's phenomenon.

OBJECTIVE: To determine circulating endothelin-1 levels (ET-1) in patients with primary or secondary associated Raynaud's phenomenon (RP) under resting conditions and in response to cold provocation. METHODS: Patients were categorised as primary RP (18) or scleroderma associated RP (14). Finger blood flow was measured by venous occlusion plethysmography at finger temperatures of 32 degrees C and 24 degrees C. Vasospasm was detected as a finger systolic pressure of 0 mm Hg after standardised provocative cooling. Severity of vasospasm was assessed by the level of cooling required to provoke spasm. Plasma ET-1 levels were measured in antecubital blood withdrawn under baseline conditions (finger 32 degrees C) and at the point of vasospasm. Measurements were also made in 19 matched control subjects. RESULTS: Finger blood flow was lower in patients with RP than in controls, with no difference between the two RP groups. Vasospasm occurred in all patients with RP but not in any control subjects and a grading system of severity was established. Baseline plasma ET-1 levels were similar in patients with RP and controls. Increases in ET-1 levels at the point of vasospasm in patients or corresponding timepoint in controls were also similar. There was no significant difference between the ET-1 levels in the two RP subgroups when the fingers were warm or when vasospasm was present. CONCLUSIONS: These results do not support the hypothesis that ET-1 plays a part in the pathogenesis of RP. Objective testing is a useful adjunct to the clinical diagnosis of RP and allows assignment of a severity grade.

A case-control study of candidate vasoactive mediator genes in primary Raynaud's phenomenon.

OBJECTIVES: To elucidate possible genetic factors involved in the pathogenesis of primary Raynaud's phenomenon (RP) and to determine the demographic features. METHODS: The allele frequencies of known polymorphisms in four vasoactive candidate genes, eNOS, BKRG, ET-1 and the ETA receptor genes, were compared in a phenotypically homogeneous group of patients with primary RP and a normal control population. RESULTS: In patients with primary RP, there was a higher reporting of both a family history of RP than in controls (45.3% vs 3.1%; P<0.0001) and a personal history of migraine (32.6% vs 7.2%; P<0.0001). No significant differences in allele frequencies of the candidate genes were found. CONCLUSIONS: These findings support the concept that genetic susceptibility exists in primary RP. The high prevalence of migraine suggests that primary RP is part of a more widespread disorder of vascular tone. These findings do not suggest that common molecular variants of these candidate genes are involved in primary RP.

Prevalence of hypocitraturia and hypopyrophosphaturia in recurrent calcium stone formers: as isolated defects or associated with other metabolic abnormalities.

Several underlying metabolic abnormalities may be present in patients with recurrent calcium calculus disease (RCCD). The aim of this study was to determine the prevalence of deficiencies of 2 well-known potent inhibitors of crystal formation and growth, citrate and pyrophosphate, in the various metabolic subgroups and as single defects. In 107 patients with RCCD, urinary citrate was significantly decreased in all metabolic subgroups with 49% of patients having hypocitraturia (2.53 +/- 1.19 mmol/24 h) versus controls (3.44 +/- 0.96 mmol/24 h; p less than 0.001). Reduced pyrophosphate:creatinine ratios were present in all the patient subgroups, and 48% of all patients had reduced ratios (1.68 +/- 1.68 vs. 3.10 +/- 2.66 in controls; p less than 0.01). There was no correlation between citrate and pyrophosphate concentration. Isolated hypocitraturia was found in 11.2%, reduced pyrophosphate:creatinine ratios as the single defect in 11.2% and a combination of both in 12.1% of patients. Thus inhibitor defects play an important role in patients with RCCD and frequently occur as isolated biochemical defects.

Limitations of faecal chymotrypsin as a screening test for chronic pancreatitis.

Faecal chymotrypsin was measured in patients with chronic pancreatitis and in healthy black urban and rural control subjects. In the patients, significantly lower values of faecal chymotrypsin were obtained (mean (SD) 2.4 1.79 U/g stool) whereas in urban control subjects, values were within the normal range (mean (SD) 13.2 (11.9)). In rural black control subjects, however, the faecal chymotrypsin value was significantly lower (mean (SD) 7.1 (5.1)) than in urban black control subjects. It is suggested that faecal pH may influence faecal chymotrypsin values. The mean faecal pH in rural black subjects (pH 6.14) was significantly lower than that in urban control subjects (pH 6.77) and in patients with chronic pancreatitis (pH 6.61). Moreover, mean faecal chymotrypsin is high (20.0 U/g stool) at a pH greater than 7. Between pH 6 and 7 the mean value drops to 8.6 U/g stool and below pH 6 mean faecal chymotrypsin is in the abnormal range (4.4 U/g stool). Hence, low values for faecal chymotrypsin may be due to lower faecal pH (less than 6) in healthy control subjects. For diagnostic purposes, the faecal pH value should be determined if a low faecal chymotrypsin value is obtained.

Histomorphometry of iliac crest bone in 346 normal black and white South African adults.

We examined undecalcified transiliac bone samples from 346 normal black and white South African adults (age range 21-83 years) by routine histomorphometry. The results were analysed for race-, age- and sex-dependent characteristics of trabecular microstructure (bone volume, trabecular thickness, trabecular number, trabecular separation) and static bone turnover variables (osteoid surface, osteoid volume, osteoid thickness, erosion surface). Trabecular thickness was greater in blacks than in whites, and bone volume was greater in black males, but not in black females, than in their white counterparts. Values for osteoid surface, volume and thickness, and for erosion surface were greater in blacks than in whites. Age-related changes were: a decline in bone volume in all race/sex groups; a decline in trabecular thickness in all groups except black males; a decline in trabecular number in all groups except black females; and a rise in trabecular separation in all groups except black females. There was an increase with age in osteoid surface in all groups except white males, in osteoid volume in all groups, and in erosion surface in blacks only. When correcting for age there were no sex-dependent differences in microstructure but values of some osteoid variables were greater in males than in females. If the greater osteoid and erosion values in blacks reflect greater bone turnover, then trabecular bone in blacks would be renewed more frequently, be subjected to fewer loading cycles and be less prone to fatigue failure. Blacks may thus have trabecular bone of better quality and sturdier microarchitecture. These features could contribute to the lower spontaneous fracture rate in blacks.

A cross-sectional comparison of three self-reported functional indices in scleroderma.

OBJECTIVES: In scleroderma, outcome measures such as skin score provide only limited information about the functional impact of the disease. The requirement for validated and convenient instruments that reliably reflect disease morbidity is now recognized. This study compares the Disability Index of the Health Assessment Questionnaire (HAQ-DI) with two more recently developed scleroderma-specific tools: scleroderma-visual analogue scales (scleroderma-VAS) and the UK scleroderma Functional Score (UKFS). In addition, the use of clinical and laboratory measures as predictors of disease severity have been examined. METHODS: One hundred and fifteen consecutive patients were studied. Subjects completed the 20-item HAQ-DI, the scleroderma-VAS and a questionnaire related to hand and muscle function (UKFS). Clinical details, measurement of maximal hand-spread, fist-closure and investigations for internal organ involvement were recorded. RESULTS: Over 68% of patients with diffuse disease had moderate to severe disease on the UKFS, compared with 44% with limited disease. The mean UKFS in diffuse disease was 14.7 (s.d. 9.1) and 10.6 (s.d. 8.5) in the limited subset (P=0.02). The mean HAQ-DI in diffuse disease was 1.23 (s.d. 0.77) and 0.79 (s.d. 0.75) in the limited subset (P=0.005). The HAQ-DI showed significant correlation with UKFS (r=0.9; P < 0.001). Several clinical and laboratory measures were associated with higher HAQ-DI and UKFS. CONCLUSIONS: This is the first comparative study of the UKFS and the HAQ-DI. These data show a strong correlation between assessment methods. Higher scores correlated with clinical and laboratory indicators of severe disease. Used together, these inexpensive tools assess general and organ-specific symptoms, as well as functional limitation.

Serum amylase levels after obstetric and gynecologic operations.

The incidence of postoperative hyperamylasemia was evaluated in 131 patients who underwent obstetric and gynecologic procedures. Preoperative and postoperative serum amylase levels were determined in 178 patients who underwent routine surgical procedures. In our sample, we could not document any elevations in serum amylase levels after operations. These findings contradict those of previous reports of a high incidence of postoperative hyperamylasemia after surgical procedures except those performed upon the gastrointestinal tract. Furthermore, in spite of the fact that the female internal genitalia is rich in amylase and that pregnancy is considered a predisposing condition for the development of postoperative pancreatitis, the preoperative and postoperative serum amylase levels were consistently within normal range. We would like to conclude that the manipulation of female internal genitalia, pregnant or not, does not induce hyperamylasemia. Therefore, hyperamylasemia in postoperative gynecologic and obstetric patients should alert the clinician to the possibility of postoperative pancreatitis. We believe that our findings should be confirmed on large samples of patients.

Interferon-gamma receptor deficiency mimicking Langerhans' cell histiocytosis.

Two patients who were initially given a diagnosis of Langerhans' cell histiocytosis on the basis of the clinical, radiologic, and biopsy findings had mycobacterial infection subsequently identified. The correct diagnosis of dominant partial interferon-gamma receptor deficiency was established.