Correlates of medical compliance among hemophilic boys.

Twenty-nine males with hemophilia completed the Medical Compliance Incomplete Stories Test (M-CIST), and their scores were correlated with health care professionals' ratings of four aspects of medical compliance, along with measures of possible moderating variables. The results indicated that significant associations were found between most of the M-CIST category scores, particularly the Compliance/coping subscale, and the health care specialists' ratings of how well the children exhibited compliant responses to bleeding episodes, and inverse associations with the incidence of monthly bleeding episodes. The findings suggested that the M-CIST continues to demonstrate promise as an instrument to be used in studies of compliance among pediatric chronic illness patients.

MR imaging of the pituitary gland in infants and children: changes in size, shape, and MR signal with growth and development.

It has been reported that on MR images of the brain in neonates the entire pituitary gland is hyperintense. This is quite different from the well-established observation that portion is isointense. To better understand the development of the neonatal pituitary gland, we studied the timing and magnitude of changes in the size, shape, and MR signal of the pituitary gland in early childhood and infancy. The study included 76 infants and children ages 3 days to 4 years old. In each MR study, the signal intensity of the pituitary gland on T1-weighted coronal (n = 24) and sagittal (n = 76) images was compared with the signal intensity of the pons by using region-of-interest methods. The size and shape of the pituitary glands were recorded. Statistical comparisons were made for signal intensity and size of the pituitary gland between boys and girls and among age groups. The results showed that the MR signal of the posterior lobe remained hyperintense relative to the pons for all age groups represented in our study (through 4 years). However, the signal intensity of the anterior lobe gradually diminished and by approximately age 2 months was isointense with the pons. Thus, by approximately age 2 months the MR signal intensity of the infant's pituitary gland has changed from being entirely hyperintense (as seen in neonates) to isointense in the anterior lobe and hyperintense in the posterior lobe (as seen in adults). In the first 2 months of life the pituitary gland is bulbous in shape; later it has a flatter upper surface. We found no significant changes in the length or signal intensity of the pituitary gland; no differences among sexes in size, shape, or signal intensity were demonstrated. Findings of a bulbous pituitary gland and high MR signal of the entire pituitary are normal in neonates up to 2 months of age. After that, the gland gradually flattens and the MR signal of the anterior lobe decreases until it has characteristics similar to the adult pituitary. Lack of high signal or discovery of a small pituitary gland in a neonate should alert the radiologist to the possibility of pituitary malformation or dysfunction.

Factors in the diagnostic delays of small bowel malignancy.

The overall survival from primary malignancies of the small intestine has not changed over more than four decades. This generally is ascribed to delays in establishing the diagnosis and the advanced stage of the disease when treatment is begun. There has been no critical analysis of these delays. To answer these questions, we reviewed the records of all patients diagnosed with small bowel malignancy from 1967 to 1988 at the Methodist Hospital of Indiana. The onset and duration of symptoms, first medical consultation, time of performance of diagnostic procedures and surgery were verified in 77 patients with histologically confirmed small bowel malignancies. The longest delay occurs from the time medical help is sought to the time the diagnosis is made, not from the onset of symptoms to the first medical consultation. The small bowel should always be considered as a source of unexplained, persistent subtle abdominal symptoms.

The role of the physician in the late diagnosis of primary malignant tumors of the small intestine.

Survival from primary malignancies of the small intestine has not improved during the last four decades. One reason for this is the advanced stage of disease at the time of surgery. In order to determine why diagnosis is made late, we reviewed the records of all patients with small bowel malignancy diagnosed between 1967 and 1988. The time from the onset of symptoms to the first medical contact and the time from medical contact until diagnosis were evaluated in 77 patients. The average delay in diagnosis attributable 1) to the patient failing to report symptoms was less than 2 months, 2) to the physician not ordering the appropriate diagnostic test was 8.2 months, and 3) to the radiologist failing to make the diagnosis was 12 months. Thus, the major delay in diagnosis was after medical help was sought and not from the onset of symptoms to first medical consultation. Physicians must increase their sensitivity to the subtle but persistent symptoms that necessitate a small bowel evaluation.

Traumatic knee injuries: the accuracy of MRI compared with arthroscopy.

Forty-three knees in 43 patients were evaluated preoperatively with magnetic resonance imaging (MRI). Both menisci and cruciate ligaments subsequently were examined directly with arthroscopy. A grading scale was used to evaluate intrameniscal signal intensity and to predict the presence of meniscal tear using MR. Compared with arthroscopy, the sensitivity, specificity and accuracy of MRI were, respectively, 100%, 88% and 93% for tears of the medial meniscus; 72.7%, 93.7% and 88.4% for tears of the lateral meniscus; 100%, 96.7% and 97.7% for tears of the anterior cruciate ligament. There were no posterior cruciate ligament tears, and none were suggested from the images. Our results show that MRI is a valuable diagnostic aid in the management of traumatic knee injury.

Primary malignant tumors in the small bowel: a comparison of the small-bowel enema and conventional follow-through examination.

Of 71 patients diagnosed with primary mesenteric malignant tumors in the small bowel over a 21-year period in a community/teaching hospital, 14 underwent small-bowel follow-through, 16 underwent small-bowel enema (enteroclysis), and four patients underwent both studies preoperatively. In a retrospective study, the sensitivity of both the small-bowel enema and the conventional small-bowel follow-through examination were compared on the basis of the original radiologic interpretation. The studies were ordered by clinicians in a clinical setting. Results of the small-bowel follow-through were abnormal in 11 of 18 patients for a sensitivity of 61%, and small-bowel enema showed abnormalities in 19 of 20 patients for a sensitivity of 95% (p = .0165). The actual tumor was shown in six (33%) of 18 small-bowel follow-through studies and in 18 (90%) of 20 small-bowel enemas (p = .0005). In four patients, normal findings on conventional small-bowel follow-through were followed by abnormal findings on small-bowel enema done for the same reason. This experience suggests that the small-bowel enema is more sensitive than the conventional follow-through examination for the detection of small-bowel cancers.

School functioning and disease severity in boys with hemophilia.

There have been few recent studies investigating the scholastic progress of children with hemophilia. The advent of PL94-142, which ensures an appropriate educational program based on a child's specific learning disabilities, and home Factor VIII therapy, which increases the hemophiliac's school attendance and sense of freedom and control, might have had a positive impact on the academic performance of these patients. We investigated past and current educational functioning of 26 boys with hemophilia, using both parental and teacher reports. Twenty-two of these children were administered reading and math achievement tests. Results indicate that school absenteeism is a continuing problem for these children, with the average hemophiliac missing 18 school days in an academic year (median = 11 school days missed); one child had missed 77% of the year. Although these boys had normal intelligence and made average grades in school, six of the 22 tested performed more than two grade levels below expectations in reading and 10 performed greater than two grade levels below expectations in math. School underachievement did not necessarily correlate with disease severity (as indicated by serum factor level), school absenteeism, or grade point average. Although eight parents indicated that their children had past or current school problems, and teachers reported that greater than 50% of the group received special tutoring or classes for learning disabled students, six out of 22 (27%) performed below expectations in either math or reading and yet had no current individual educational remediation plan.(ABSTRACT TRUNCATED AT 250 WORDS)

Double-contrast barium enema study: simple conversion to CO2.

The use of CO2 instead of room air for double-contrast barium enema (DCBE) studies has been shown to decrease discomfort after the study. An inexpensive system assembled from materials currently used to perform pneumocolon examinations is described, which simplifies conversion to CO2 use for the DCBE study. No changes in currently used pneumocolon techniques are necessary with the use of CO2.

Selection of replacement therapy for patients with severe factor VII deficiency.

The risk of significant bleeding in patients with severe Factor VII (FVII) deficiency is well documented. However, controversy regarding management exists not only for the choice of FVII replacement product, but also for the desired hemostatic level of FVII. Two case reports are presented where fresh frozen plasma (FFP) and several Factor IX (FIX) complex concentrates were used to achieve hemostasis. Significantly higher FVII levels were achieved with Proplex (FIX Complex, Hyland Therapeutics) than with other products. Although this and other reports indicate that minor bleeding can be controlled with levels of FVII under 15%, life-threatening hemorrhages require higher FVII levels that can be attained only by using a product with a significant FVII content. It is important to know the FVII content of FIX concentrates in order to plan appropriate therapy for patients with FVII deficiency.