Oncogenic osteomalacia: clinical presentation, densitometric findings, and response to therapy.

A 72-yr-old white female who had previously enjoyed excellent health presented with global bone and muscle pain, and chronic fatigue. Her evaluation revealed an increased sedimentation rate and mild anemia, and a diagnosis of polymyalgia rheumatica was made. Prednisone therapy was of little benefit. A laboratory evaluation revealed mild hypocalcemia, marked hypophosphatemia, elevated alkaline phosphatase, normal 25- hydroxyvitamin D, and undectable 1,25-dihydroxyvitamin D. A diagnosis of oncogenic osteomalacia was made and the patient received calcitriol and neutraphos therapy. The patient's initial bone density by dual energy X-ray absorptiometry of the lumbar spine was 0.847 g/cm2 (T score -1.96) and of the femoral neck was 0.669 gm/cm2 (T score -2.89). After 40 mo of treatment with calcitriol and neutraphos, the bone mineral density of the lumbar spine and hip rose dramatically by 47.8 and 59.1%, respectively. Although oncogenic osteomalacia is a very rare metabolic bone disease, its recognition and appropriate treatment can have a dramatic effect not only on the bone mineral density of the patient, but also on the patient's general health and feeling of well-being.     

Correlation of clinical examination,ultrasound sonography,and magnetic resonance imaging findings with arthroscopic findings in relation to acute and chronic lateral meniscus injuries

Purpose

The aim of this study was to evaluate whether ultrasound sonography (USS) performed by orthopedic surgeons is a reliable method of investigating lateral meniscus (LM) knee lesions as compared to magnetic resonance imaging (MRI), and arthroscopy, which is the gold standard in clinical practice.

Methods

In total, 107 patients were involved in this study. They were hospitalized for arthroscopy due to LM injury of the knee. Clinical examination (McMurray’s, Apley’s, and joint line tenderness tests), USS, and MRI were performed prior to arthroscopy. We compared the results of clinical examination, USS, and MRI with the arthroscopic findings for the knee, which were considered the reference values.

Results

McMurray’s clinical test, which is the most sensitive method of detecting lateral meniscus lesions, gave the same sensitivity rate for both acute and chronic LM injuries: 65 %. USS was observed to be more sensitive and specific for chronic LM injuries (85 and 90 %, respectively) than for acute LM injuries (71 and 87 %). MRI also yielded higher values of sensitivity and specificity for chronic lateral meniscus injuries (75 and 95 %, respectively) than for acute LM injuries (68 and 87 %).

Conclusions

The accuracy of ultrasound examination is demonstrated by the high reliability of this method in the diagnosis of lateral meniscus lesions of the knee, and the evaluation performed in this study showed that ultrasound is a useful clinical tool for diagnosing knee pathology.     

Testicular microlithiasis, a premalignant condition: prevalence, histopathologic findings, and relation to testicular tumor

Objectives. To perform a retrospective analysis concerning the prevalence of testicular microlithiasis (TM). In patients with TM, the association of TM with testicular tumor, histopathologic findings, and follow-up were studied.Methods. During a 6-year period at the Central Military Hospital or the University Medical Center in Utrecht, The Netherlands, ultrasonography of the testis was performed in 1535 patients. Patient records, ultrasound images, and histopathologic specimens were reviewed. Follow-up was performed in patients with TM.Results. In 63 patients (4.1%), with a mean age of 35.4 years (range 19 to 74), TM was diagnosed at ultrasonography. In 29 of these patients (46%), a concomitant testicular tumor was diagnosed. A statistically significant correlation was found between TM and the presence of a testicular tumor (P <0.001; chi-square test). No significant correlation was found concerning the respective positions of the TM and the tumor in the testis, type of calcification, and histologic type of the tumor. In 34 patients, TM was found without a malignancy at diagnosis (mean age 39.2 years; range 19 to 69). Follow-up was possible in 31 patients. During the follow-up period (median 61.8 months), 1 patient developed a testicular tumor.Conclusions. A correlation was found between TM and testicular tumor. Because an increasing number of studies have reported patients with TM who developed a testicular tumor, TM should be regarded as a premalignant condition, which necessitates follow-up. Urologists should consider testis biopsy in patients with TM.     

Outcomes after partial neurectomy of Morton's neuroma related to preoperative case histories, clinical findings, and findings on magnetic resonance imaging scans.

Our purpose was to evaluate the reliability of preoperative case histories, clinical findings, and magnetic resonance imaging (MRI) scans in substantiating the diagnosis of Morton's neuroma and in predicting clinical outcomes after surgical intermetatarsal neurectomy. We studied 19 consecutive patients with histologically proved Morton's neuroma. All had preoperative MRI of the forefoot. Partial neurectomy was performed when there was forefoot pain with transmetatarsal compression and positive findings on MRI scans. Case histories, clinical findings, and findings on MRI scans were correlated with clinical outcomes. Preoperative clinical findings including localization correlated with intraoperative findings in 11 of 19 patients (58%), and MRI scans correlated in 16 of 19 patients (84%). Of the 19 patients, 74% achieved satisfactory outcomes. Neither reliable clinical findings or findings on MRI scans nor confirmation of clinical findings by MRI correlated with a superior result, but 77% of patients with neuromas measuring more than 5 mm in transverse measurement on MRI scans had good outcomes; only 17% with neuromas measuring 5 mm or less had good outcomes. Preoperative localization and diagnosis of Morton's neuroma is better achieved with MRI than through clinical findings. A more favorable clinical outcome can be expected after surgical intermetatarsal neurectomy when a Morton's neuroma has a transverse measurement larger than 5 mm on MRI scans.     

Cricoarytenoid subluxation, computed tomography, and electromyography findings

A case of cricoarytenoid subluxation secondary to endotracheal intubation and documented by computed tomography (CT) and electromyography (EMG) is reported. Successful endoscopic reduction of the displaced arytenoid is confirmed by CT. The normal anatomy and physiology of the cricoarytenoid joint is presented and the literature regarding this rarely reported injury is reviewed. Based on this review and the case reported, a treatment plan is proposed.     

Induced sputum in children: feasibility, repeatability, and relation of findings to asthma severity

BACKGROUND: The collection of induced sputum provides a non-invasive method of investigating airway inflammation. Few studies have been performed in children, so a study was undertaken to determine the feasibility of sputum induction, the repeatability of eosinophil counts and sputum eosinophil cationic protein (ECP) levels, and the relation of these to current asthma severity. For comparison, serum ECP levels were also measured. METHODS: In a cross sectional study of children aged 5-15 years, 27 healthy children and 60 with asthma underwent sputum induction using inhaled nebulised hypertonic saline. The whole sputum sample was used for analysis. Ten children with stable asthma repeated the procedure within 10 days. RESULTS: A satisfactory sample (>500 non-squamous cells) was obtained in 61% of children with asthma and in 60% of healthy controls. The limits of agreement within subjects ranged from a 0.68 to 2.8 fold difference for eosinophil differential counts and from 0.38 to 4.4 fold for sputum ECP. Despite a median of 42% squamous cells, significant differences were found between asthma and healthy controls for the eosinophil differential count (p = 0.0004), total eosinophil counts (p = 0.03), and sputum ECP level (p = 0.0001). Overall, there was no correlation between any marker of airway inflammation and asthma severity, however expressed, including lung function. CONCLUSIONS: Sputum induction is only possible in a proportion of children. The repeatability of sputum cell counts and ECP levels, measured in a small number of children, was similar to that reported in adults. Sputum analysis revealed no evidence of airway inflammation in a number of highly symptomatic children with asthma.     

Clinical, radiological, and laboratory findings in Lebanese spondylarthropathy patients according to HLA-B27 status

To evaluate clinical, radiologic, and laboratory features in Lebanese spondylarthropathy patients according to HLA-B27 status. METHODS: We retrospectively compared demographic, clinical, radiologic, and severity data in 40 HLA-B27-positive and 58 HLA-B27-negative patients. All 98 patients met Amor's or European Spondylarthropathy Study Group criteria for spondylarthropathy, and 51.7% met New York modified criteria for ankylosing spondylitis. RESULTS: Onset before 16 years of age, hip involvement, and an elevated mean erythrocyte sedimentation rate were significantly associated with the presence of the HLA-B27 (32.5 vs 13.8%, P=0.02; 45 vs 7.5%, P=0.001; and 47.7 vs 25.4, P=0.02; respectively). The two groups were comparable for age, sex ratio, prevalence and distribution of spondylarthropathy types, family history, sacroiliitis, bamboo spine, syndesmophytes, peripheral joint involvement, enthesopathies, extra-articular involvement, response to nonsteroidal anti-inflammatory drugs, and need for other medications. CONCLUSION: In Lebanon, spondylarthropathy patients positive for HLA-B27 experience disease onset at an earlier age, are more likely to develop hip involvement, and have laboratory evidence of more severe inflammation than their HLA-B27-negative counterparts. None of the other clinical and radiologic parameters are modified by HLA-B27 status.     

Hyperphosphatasemia in an adult. Clinical, roentgenographic, and histomorphometric findings and comparison to classical Paget's disease

A 54-year-old man with short stature, diffuse skeletal abnormalities, and elevated alkaline phosphatase was evaluated by bone biopsy with undecalcified sections and morphometry analysis. Microscopically the bone changes were identical to those of classic Paget's disease. Histomorphometric analysis of bone demonstrated a high remodeling activity with increased mineralization rate similar to Paget's disease. However, the early age at onset and severity suggest that this man suffers from hyperphosphatasemia, a different and distinct condition.     

Chemonucleolysis. The relationship of the physical findings, discography, and myelography to the clinical result

Fifty consecutive patients undergoing chemonucleolysis with chymopapain at William Beaumont Hospital were analyzed with special reference to the following factors: the physical examination, the dye pattern noted on discogram, and the size of the preoperative myelographic defect. Discogram pattern was divided in four types: normal disc, degenerative pattern, degenerative pattern with extravasation, and annular injection. The myelograms were graded into a mild defect, a moderate defect, or a severe defect. Follow-up averaged 20 months. Conclusions of this study were Chymopapain can be considered as an alternative to lumbar laminectomy for relief of sciatica secondary to herniated disc. Statistically significant improved postinjection results were noted when patients presented with three out of four objective physical findings consisting of positive straight leg raising, reflex change, dermatomal paresthesia pattern, and/or mild motor weakness. Placement of the needle within the nucleus leads to a statistically significant improved result over placement of the needle into the annulus. A severe myelographic defect greater than 50% dura sac compression is a relative contraindication to the injection of chymopapain.     

Iliac artery stenosis proximal to a kidney transplant: clinical findings,duplex-sonographic criteria,treatment, and outcome

BACKGROUND: Stenosis of the iliac segment proximal to the transplant renal artery (Prox-TRAS) is an uncommon cause of graft dysfunction and hypertension. We assessed the role of duplex sonography (DS) in regard to clinical and angiographic findings and followed the patients after percutaneous transluminal angioplasty (PTA), PTA stenting (PTAS), or surgery. METHODS: From January 1988 to August 2001, 97 of 1,064 kidney recipients underwent angiography for clinical or Doppler-sonographic suspicion of vascular problems. Kidney function, blood pressure, medication, and DS findings after renal transplantation (RTx) at the time of diagnosis of Prox-TRAS and after treatment were evaluated. RESULTS: Prox-TRAS was diagnosed in 16 patients (1.5%) (49.6+/-6.9 years). Four patients demonstrated early presentation of Prox-TRAS 1 to 7 days after RTx (group A), leading to acute renal failure but without hypertension. In all patients, DS revealed pulsus parvus et tardus, low pulsatility index (PI) (<1.0), and a pathologic flow profile in the iliac artery proximal and distal to the graft. After treatment (surgery in two patients, PTA in one patient, PTAS in one patient), all patients developed good renal function (creatinine 1.7+/-0.9 mg/dL). PI increased from 0.9+/-0.1 to 1.2+/-0.1 (P=0.04), and flow profile within the iliac artery distal to the graft normalized. Late presentation (3-209 months after RTx) of Prox-TRAS was observed in 12 patients (group B), causing an increase of creatinine in 11 patients (two patients receiving dialysis treatments), impairment of blood pressure (141+/-15 and 80.7+/-7 to 160+/-18 and 85+/-7 mm Hg, P=0.009), and an increase in antihypertensive drugs (2.1+/-1.1 and 4.3+/-1, P=0.003) in all patients. The PI was decreased when compared with values early after RTx (1.6+/-0.4 to 1.2+/-0.3, P=0.007), and flow profile in the iliac artery was pathologic. All patients except one were managed by surgery (n=6), PTA (n=1), or PTAS (n=4). Creatinine (2.7+/-1.4 to 1.8+/-0.4 mg/dL, P=0.02) and blood pressure (160+/-18/85+/-7 mm Hg to 138+/-7/82+/-9, P=0.018) improved. Antihypertensive drugs were reduced to 2.8+/-0.8 (P=0.01). PI increased from 1.2+/-0.3 to 1.9+/-0.5 (P=0.01). Flow profile within the iliac artery distal to the graft anastomosis normalized. Kidney function, blood pressure, and PI remained unchanged during follow-up (82+/-69.9 months) in both groups. CONCLUSIONS: Prox-TRAS is rare. Because clinical symptoms are similar to those of transplant renal artery stenosis, DS is a valuable tool for diagnosis and follow-up for this type of vascular lesion. Selective treatment with PTA, PTAS, or surgery improves kidney function and hypertension.     

Paravaginal defect: anatomy,clinical findings,and imaging

Introduction and hypothesis

The paravaginal defect has been a topic of active discussion concerning what it is, how to diagnose it, its role in anterior vaginal wall prolapse, and if and how to repair it. The aim of this article was to review the existing literature on paravaginal defect and discuss its role in the anterior vaginal wall support system, with an emphasis on anatomy and imaging.

Methods

Articles related to paravaginal defects were identified through a PubMed search ending 1 July 2015.

Results

Support of the anterior vaginal wall is a complex system involving levator ani muscle, arcus tendineus fascia pelvis (ATFP), pubocervical fascia, and uterosacral/cardinal ligaments. Studies conclude that physical examination is inconsistent in detecting paravaginal defects. Ultrasound (US) and magnetic resonance imaging (MRI) have been used to describe patterns in the appearance of the vagina and bladder when a paravaginal defect is suspected. Different terms have been used (e.g., sagging of bladder base, loss of tenting), which all represent changes in pelvic floor support but that could be due to both paravaginal and levator ani defects.

Conclusion

Paravaginal support plays a role in supporting the anterior vaginal wall, but we still do not know the degree to which it contributes to the development of prolapse. Both MRI and US are useful in the diagnosis of paravaginal defects, but further studies are needed to evaluate their use.

     

Cholecystosonography: Accuracy,pitfalls and unusual findings

Gray scale cholecystosonograms in 200 patients were reviewed. The findings in 133 of the patients were proved correct at surgery or autopsy. The overall accuracy for the detection of gallstones was 92 percent, with a false-negative rate of 4 percent. A false-positive diagnosis of cholelithiasis was made in three patients, two of whom proved to have extensive cholesterolosis. Causes of false-negative studies were the presence of a single small calculus, obesity and a large distended gallbladder. Ultrasound was specific but insensitive in the detection of a thickened gallbladder wall. The significance of a nonvisualized gallbladder by ultrasound and the role of ultrasound in the diagnosis of gallbladder disease are discussed.     

Familial Mediterranean fever, clinical and laboratory findings

Familial Mediterranean fever is the best known of the recurrent hereditary autoinflammatory diseases. It predominantly affects subjects of Mediterranean origin, Sephardic Jews in particular. Its gene, MEFV, is located on chromosome 16 and has autosomal recessive transmission, with incomplete penetration. It codes for a protein called pyrin or marenostrin, which is probably involved in the inflammatory process. In most cases, the first episodes appear before the age of 20 years and very rarely after the age of 40. Episodes usually last a few days, although they may extent over several weeks when localized in joints. Fever, occasionally pseudo-malaria, may accompany various symptoms, the most frequent of which are abdominal, articular, pleural or cutaneous. The abdomen is the classic site of this disease, and acute abdominal flares masquerade as abdominal emergencies. Musculoskeletal involvement is revealed by episodes of inflammation of the joints (more often mono- than oligoarthritis) and muscle pain. The flares are usually brief and totally reversible. Flares of thoracic pain corresponding to pleural inflammation and erysipelas-like skin eruptions have been observed. Acute symptoms disappear between flares, but hepatic splenomegaly, swollen lymph nodes or abnormal fundus of the eye may persist. Laboratory findings are typical of nonspecific inflammation, accompanied by moderate hyperleukocytosis during the flares.     

The relation of preoperative coagulation findings to diagnosis, blood usage, and survival in adult liver transplantation

A group of 70 adults with end-stage liver disease received 87 homologous liver transplants from 7/11/81 and 7/11/83. The recipients fell into the following diagnostic categories: postnecrotic cirrhosis (PNC) in 22, primary biliary cirrhosis (PBC) in 18, cancer or neoplasia (CA) in 11, sclerosing cholangitis (SC) in 8 and miscellaneous (MISC) in 11. Survival for six months or longer was 46%: survival by group was PBC = 67%, CA = 55%, PNC = 45%, SC = 25%, and MISC = 18%. Preoperative coagulation profiles were evaluated on 64 of the 70 first transplant patients by assigning a score derived from one point per abnormality in each of 8 tests. Mean coagulation abnormality scores (CAS) were strikingly elevated in the PNC and MISC groups. Mean intraoperative blood product usage was 43 units of RBCs, 40 units of fresh frozen plasma (FFP), 21 units of platelets, and 9 bags of cryoprecipitate. Direct correlations were found between CAS and RBC usage (+0.454, P = less than .001), CAS, and survival of 6 months or longer (-0.281, P = less than .02), and RBC usage and survival (-0.408, P = less than .001). These findings indicate that the degree of coagulation abnormality and the type of liver disease may be predictive of intraoperative blood usage and survival in liver transplantation in adults.