Eosinophilic cystitis in children. Study of 4 cases

OBJECTIVE: To describe the development and initial psychometric properties of a new outcome measure to assess satisfaction with prosthesis in children with limb deficiencies. DESIGN: Parents of children with limb deficiency were surveyed during routine outpatient clinic visits. SETTING: Two outpatient pediatric clinics. PARTICIPANTS: Ninety-seven parents of children with limb deficiency aged 1 to 17 years. MAIN OUTCOME MEASURE: The newly developed Child Amputee Prosthetics Project-Prosthesis Satisfaction Inventory (CAPP-PSI). RESULTS: Internal consistency reliability is high. Zero-order correlations with prosthesis wear/use patterns and with parent ratings of prosthesis appearance provide support for the construct validity of the CAPP-PSI. CONCLUSION: The CAPP-PSI is a promising, brief, parent-administered inventory for the assessment of prosthesis satisfaction in children with limb deficiency. It may be useful in future research for predicting prosthesis wear and use patterns in this population.     

Droxicam-induced hepatitis. Description of 3 new cases and review of the literature

Three new cases of cholestatic hepatitis caused by droxicam are described, along with a revision of the other eight cases published to date. Itching, asthenia, and jaundice were the most common symptoms. Average age was 62.8 years (range: 45-82 years), and the median time of exposition was 22.7 days (range: 5-50 days). Biochemistry of the liver showed primarily cholestasis and in 4/11 cases hypereosinophilia. Two patients presented elevated levels of cholesterol and triglycerides which disappeared within the month. Clinical manifestations persisted in one patient for eight weeks after the cessation of treatment. The three patients presented in the present series presented alteration in the biochemistry of the liver two months after initiation. Liver biopsy in three patients showed centrozonal cholestasis associated with portal inflammatory activity and presence of granulomas consistent with toxic hepatitis.     

Unilateral amnesic stroke. Six new cases and a review of the literature

BACKGROUND AND PURPOSE: Although persistent amnesia due to bilateral limbic system infarction is well described, reports of amnesic syndromes due to unilateral stroke have appeared infrequently and unsystematically. We report six new cases and review previously published reports to expand and consolidate knowledge regarding amnesic stroke. CASE DESCRIPTIONS: Six patients developed acute amnesia associated with unilateral stroke. Brain computed tomography or magnetic resonance imaging revealed lesions in limbic structures, which accounted for the memory deficit. Based on these as well as similar cases in the literature, we delineate three distinct syndromes of unilateral amnesic stroke involving the territories of the posterior cerebral, anterior choroidal, and thalamic penetrating arteries. Eighty-five percent of reported cases have involved the left hemisphere. CONCLUSIONS: Persistent as well as transient amnesia may be the sole or primary manifestation of unilateral hemispheric stroke. The vascular mechanisms of amnesic stroke are diverse. Patients presenting with acute amnesia possibly related to cerebral ischemia should be classified by documented or presumed lesion site as well as the involved vascular territory. Left amygdalohippocampal or diencephalic dysfunction may produce a particular vulnerability to global amnesia.     

Gangrenous cystitis: case report and review of the literature

A case of gangrenous cystitis in a 67-year-old woman is reported. Associated etiological factors include invasive recurrent carcinoma of the cervix, radiotherapy and atherosclerosis. Primary infection was not a feature. The patient was treated with antibiotics, bladder drainage and total cystectomy with complete recovery. The etiology and management of this unusual condition are discussed.     

Formalin treatment for intractable hemorrhagic cystitis: a review of the literature with 16 additional cases

Acute, exsanguinating hemorrhagic cystitis secondary to cyclophosphamide therapy, radiation therapy, or an infiltrating bladder tumor may be managed successfully with intravesical Formalin therapy. The indications for its use, the technique, success rates, and complications are discussed. This treatment was effective in 14 of 16 patients in the present series and 79 of 90 cases reported in the literature. Dilutions of 4% or less were as effective as a 10% dilution and were associated with far fewer complications. The early use of Formalin in the treatment of intractable hemorrhagic cystitis is recommended.     

Mesenchymal chondrosarcoma of the orbit. Report of three new cases and review of the literature

BACKGROUND: Extraskeletal mesenchymal chondrosarcoma is a rare tumor characterized by undifferentiated mesenchymal cells with islands of mature hyaline cartilage. Considering all sites, long-term survival is approximately 30%. Only seven cases of orbital mesenchymal chondrosarcoma have been reported. METHODS: The records of three cases of orbital mesenchymal chondrosarcoma treated at the Columbia-Presbyterian Medical Center, and the seven previously reported cases of this tumor were reviewed to determine clinical characteristics and appropriate therapy. RESULTS: Nine of the 10 patients were female; age of onset ranged from 10 to 35 years. Of eight patients with at least 2 years of observation, five survived 5 or more years after resection, or after resection plus adjuvant therapy. Two patients died of metastatic disease 2 and 5 years, respectively, after the initial treatment, and one died of pneumonia 6 months after surgery. Review of the histology of the three cases treated at Columbia-Presbyterian Medical Center did not identify distinct histologic types that might guide therapy. Presenting symptoms were typical of symptoms of an orbital mass: proptosis, pain, diplopia, change in visual acuity, ptosis, and tearing. CONCLUSION: The small number of reported cases of mesenchymal chondrosarcoma of the orbit prevents definitive conclusions, but it appears that resection is adequate therapy in some cases. Extraskeletal mesenchymal chondrosarcoma of the orbit may have a better prognosis than tumors in other sites.     

Merkel cell tumor of the head and neck. Five new cases with literature review

Merkel cell carcinoma (MCC) of the skin is an uncommon, but highly aggressive neoplasm with a marked propensity for local and distant metastasis. Despite the fact that more than half of the 600 cases of MCC reported in the literature involved primary sites in the head and neck, MCC has rarely been discussed in otolaryngology publications. We present five new cases of MCC of the head and neck and summarize 89 additional cases from the literature in which detailed treatment and survival data were given. Our findings again emphasize the difficulty in making the initial histopathologic diagnosis of MCC and demonstrate the necessity of early diagnosis and multimodality treatment.     

Antley-Bixler syndrome. Description of two new cases and review of the literature. Prognostic and therapeutic aspects

Taxol cytotoxicity was evaluated in human head and neck squamous carcinoma cell lines growing as multicellular tumor spheroids (MTS) and compared with monolayered culture using conventional clonogenic assays. End points were respectively the concentration inhibiting 50% of the cellular growth (IC50) in clonogenic assays and the concentration required to induce a 50% decrease in the MTS volume (ID50) or number in the overall spheroid population (SCC50). A significant difference was observed when the cells were exposed for 10 days to Taxol as a consequence of the different growth kinetics of the spheroids. After 16 day exposure of spheroids to Taxol, no difference remained between ID50 and IC50. In addition, a significant correlation was found between individual spheroid sensitivity to Taxol (ID50) and the spheroid population sensitivity (SCC50). Both parameters (ID50 and SCC50) defined in cell models appear useful for the evaluation of chemosensitivity of three-dimensional structures known to be closer to in vivo tumor models.     

Appendicular mucocele. Clinical cases and review of the literature

The authors report three cases of mucocele of the appendix. They review the literature on this argument and discuss the hypotheses on etiology, pathologic and clinical classification, problems in diagnosis, principles of therapy and prognosis.     

Nonossifying fibroma. Four cases and review of the literature

Nonossifying fibroma is a benign, lytic lesion that occurs in young children and adolescents. Radiographically, the lesion is multilocular and sharply demarcated. It often occurs at the metaphyseal region of long bones of the lower extremity and is usually eccentrically located. Four cases of nonossifying fibroma occurring during the past 7 years are presented with a review of the literature.     

Dens evaginatus: review of the literature and report of several clinical cases

Dens evaginatus (DE) presents as an innocuous looking tubercle of enamel on the occlusal surface of a tooth, most commonly a bicuspid. Problems can arise when the tubercle is either worn, ground, or fractured off, resulting in pulpal exposure and possible loss of vitality of the tooth. Dentists who perform orthodontic treatment should be aware of this dental anomaly, which occurs in at least two per cent of the Asian and Native Indian populations. Bicuspid extraction cases should involve the extraction of the anomalous premolars rather than the normal ones. In addition, the dentist should be mindful of occlusal changes that may occur during treatment or occlusal equilibration, both of which can jeopardize the vitality of teeth with DE. Pulp capping or partial pulpotomy has been postulated to be one of the most reliable forms of vital tooth treatment when pulp exposure is encountered following the sterile removal of the tubercle. When pulp exposure is not encountered, preventive resin composite sealing of the dentin or class I amalgam cavity preparation seems to be the treatment of choice.     

Acanthamoebiasis in Korea: two new cases with clinical cases review

OBJECTIVE: To compare mortality rates on hemodialysis (HD) to rates on continuous ambulatory/cyclic peritoneal dialysis (CAPD/CCPD), to contrast our results with those of other recent investigations, and to discuss reasons for discrepancies. DATA SOURCES: Patient-specific data obtained from the Canadian Organ Replacement Register on patients initiating renal replacement therapy (RRT) between 1 January 1990 and 31 December 1995 (n = 14 483). Recent mortality comparisons of CAPD and HD. MAIN OUTCOME MEASURES: Mortality rate ratio (RR) based on "as-treated" (AT) analysis incorporating treatment modality switches and adjusting for age, primary renal diagnosis, and comorbid conditions using Poisson regression. Hazard ratios (HR) were estimated using Cox regression and based on an "intent-to-treat" (ITT) analysis wherein patients were classified based on dialytic modality received on follow-up day 90. RESULTS: Adjusted mortality rates were significantly decreased on CAPD/CCPD relative to HD [RR = 0.73, 95% confidence interval (CI) = (0.69, 0.77)] based on the AT analysis. Most of the protective effect of CAPD/CCPD was concentrated in the first 2 years of follow-up post-RRT initiation. Based on the ITT analysis, the estimated CAPD/ CCPD effect was greatly reduced, with HR = 0.93 (0.87, 0.99). CONCLUSIONS: We provide further evidence that CAPD/CCPD is not an inferior dialytic modality to HD, particularly in the short term. Comparing mortality rates on CAPD/CCPD and HD is inherently difficult due to the potential for bias. Discrepancies between our results and those of previous investigations, and variability in findings among previous studies, relate to differences in clinical and demographic setting, patient populations, study design, statistical methods, and interaction between the dialytic modality effect and various other covariables.     

Scleroderma renal crisis. 7 cases and review of the literature

We report a series of seven patients who had scleroderma renal crisis. Their primary clinical and laboratory features along with the details of their management were compared with those of similar cases from the literature. The seven patients died within one to four months of the diagnosis with a pattern of acute renal failure, left ventricular failure and malignant hypertension. Histopathologic examination was performed in four of the patients, in two of whom it revealed thickening of the wall of the interlobular arteries related to the scleroderma, and in the other two patients nonspecific lesions of malignant hypertension. This histopathologic particularity led us to propose, on the basis of multiple renal biopsies performed in patients with scleroderma, a lesion chronology of the kidney in patients with scleroderma. Nevertheless, the triggering factors and pathophysiologic mechanisms of scleroderma renal crisis remain unclear and its prognosis is severe. Early treatment with angiotensin-converting enzyme inhibitors and other vasodilatators administered intravenously can prevent death and dialysis.     

Delayed delivery. Two cases, and review of the literature

Delayed deliveries are rarely encountered in multiple pregnancies. They are useful in case of premature fetus. We report two case of delayed delivery; the first one of 23 days in a twin pregnancy, and the second one of 4 days in a triplet pregnancy. In both cases, the first delivered fetus died, and the later one or ones survived. Delayed delivery is a good management practice for multiple pregnancies in case of prematurity if no contraindications exist.     

Recurrent transverse myelitis. 2 cases and review of the literature

Recurrent transverse myelitis is a rare inflammatory demyelinating disorder, which is distinguished from acute monophasic transverse myelitis and from MS. We present two patients with recurrent transverse myelitis, who developed an acute remitting tetraparesis and sensible deficits with a cervical level. They were followed for 4 and 5 years. MRI showed an isolated lesion with contrast enhancement in the spinal cord but no pathological cranial findings. Oligoclonal bands were negative in repeated lumbar punctures. One patient showed lymphocytic pleocytosis during the first and second attack. Somatosensory and motor evoked potentials were abnormal during relapses while visually and brainstem acoustically evoked potentials (VEP and BAEP) stayed within limits. Laboratory examinations for bacterial, viral or parasitic infections, antinuclear antibodies, Angiotensin-converting enzyme and the sedimentation rate were also normal. The differential diagnosis will be discussed in view of previously reported series. We give a review of the current literature and discuss the differential diagnoses.     

Calcification of the basal ganglia and Fahr disease. Report of two clinical cases and review of the literature

gamma-Aminobutyric acidA (GABAA) receptors are linked to ion channels which mediate many aspects of neural inhibition. Although the effects of phosphorylation on GABAA receptor function have been widely studied, the actual role of phosphorylation in the regulation of these receptors still remains controversial. In recent reports, we have described the effects of phosphorylating/dephosphorylating enzymes on the regulation of GABAA receptors in a rat cortical slice preparation (Shaw et al., Mol. Neuropharmacol., 2 (1992) 297-302; Shaw and Lanius, Dev. Brain Res., 70 (1992) 153-161; Pasqualotto et al., Neuroreport, 4 (1993) 447-450) and predicted that ionic co-factors are involved in mediating the regulation of GABAA receptors by kinases and phosphatases. In the present report, the effects of chloride, sodium, potassium, and calcium were examined alone and in the presence of cAMP-dependent protein kinase (protein kinase A) or alkaline phosphatase. The results showed a decrease in [3H]SR 95531 (GABAA receptor antagonist) binding after incubation with chloride alone; this decrease was further enhanced in the presence of protein kinase A. Both effects could be blocked by a protein kinase A inhibitor. Conversely, an increase in [3H]SR 95531 binding was observed after incubation with sodium alone; this increase was further enhanced in the presence of alkaline phosphatase. In both cases these increases in binding could be blocked by sodium orthovanadate, a phosphatase inhibitor. Potassium was ineffective under all conditions; calcium showed enzyme-independent effects at low concentrations only. These results suggest the existence of a novel chloride-dependent protein kinase which may have significant sequence homology to protein kinase A, and a novel sodium-dependent phosphatase.(ABSTRACT TRUNCATED AT 250 WORDS)     

Cytogenetic analysis of hypophyseal adenoma. Study of 9 cases and review of the literature]

We present an easy alternative to enhance rhinoplasty in patients with wide nasofrontal angles. By implanting PTFE (GORE-TEX) in the glabella region, we obtained a significant change in nasal shape. Twenty-seven patients were treated with this technique with no complications and with good results. The material showed excellent biocompatibility, matching the host tissues perfectly.     

Ophthalmoplegia-ataxia-areflexia in pediatrics. Three new patients and review of the literature

OBJECTIVE: The objective of this study was to investigate if pediatric patients with benign brainstem encephalitis (Bickerstaff Syndrome) or with Miller-Fisher Syndrome are the extremes of the same nosological entity which, in adults, has been named ophalmoplegia-ataxia-areflexia syndrome. PATIENTS AND METHODS: The subjects included in the study were three patients of our institution and 24 patients found in the revision of the English and Spanish pediatric literature who fulfilled the diagnostic criteria of ophtalmoplegia-ataxia-areflexia syndrome. The topographical location of the lesion in the nervous system was based on previously established criteria by using clinical and complementary studies. RESULTS: Of the 27 patients included in the study we were able to reach an accurate topographical diagnosis in 9. None had an exclusive involvement of the peripheral nervous system, (6) had exclusively central nervous system involvement and 2 showed involvement of both system. In 12, the topographical location of the lesion could be only ascertained as probable; 3 of them in the peripheral nervous system, 2 in the central nervous system and mixed involvement in 7. In the remaining 7 patients there were insufficient clinical data to allow topographical classification. CONCLUSIONS: The ophtalmoplegia-ataxia-areflexia syndrome can also be found in pediatric patients. The lesion in the majority of patients in this age group is located in the central nervous system, either alone or combined with peripheral nervous system involvement.     

Neurosurgical aspects of fronto-ethmoidal osteomas; report of 6 cases and review of the literature

The cases of six patients with fronto-ethmoidal osteomas which have produced intracranial complications are reported. The patients have been divided in two groups depending on their clinical aspects, from cranio-facial deformities to exophtalmus, rinorrhea, pneumocephalus, meningitis and mucocele. A review of the literature is made and the importance of such lesions is stressed together with a critical analysis of the methods proposed for their surgical treatment. The results obtained with a frontal approach and a plastic closure of the dura were uniformelly good with restoration of functions in all patients.