Primary endobronchial non-hodgkins lymphoma: description of a case and review of the literature]

Photosensitive epilepsy is relatively rare. However, a large proportion of individuals with epilepsy perceive that they are at an increased risk of a seizure whilst exposed to specific photic material. The difference between perceived and real risk may be due to inadequate education and misinformation. One half of the participants in the present survey could not recall being informed of the result of the 'gold standard' test for photosensitivity--intermittent photic stimulation during an electroencephalogram. Furthermore, approximately one-third of our sample were apparently given inaccurate and overcautious advice about their everyday exposure to photic material. Better information and advice is crucial to improve this situation in the future. The majority of people with epilepsy (>95%) who are not photosensitive can pursue activities that involve flickering or patterned light, encompassing educational, employment and leisure opportunities, without undue concern.     

Convulsions in relation to polycythemia: literature review and case description

We investigated the ability of polyethylene glycol 4000 to accelerate thrombin generation in a mixture of prothrombin and factor X at concentrations of 1-30%. In the presence of 5 mM of CaCl2, polyethylene glycol 4000 promoted prothrombin activation at concentrations above 1%. The peak of activation was seen at levels of 14 and 20% of polyethylene glycol 4000. The effect of the polyethylene glycol was remarkably dependent on its molecular weight; molecular weights greater than 2000 were required for accelerating thrombin generation. Under optimal conditions, polyethylene glycol 4000, in the presence of CaCl2, promoted conversion of all of the prothrombin into thrombin and its derivatives. We conclude that polyethylene glycol 4000, at concentrations ranging from 14 to 20%, effectively accelerates thrombin generation in the presence of 5 mM of CaCl2. This new method for preparing thrombin is based on the use of polyethylene glycol 4000 and CaCl2 and is applicable to the manufacture of thrombin.     

Lingual osseous choristoma: a study of eight cases and review of the literature

A choristoma is a tumor-like mass of normal tissue in an "abnormal" location. Lingual osseous choristoma, previously known as osteoma of the tongue, is a rare entity: we found documentation of eight cases at our center during the 10-year period between 1987 and 1996. These cases were analyzed along with 50 others reported in the English language literature between 1913 and 1996. Lingual osseous choristoma frequently occurs during the third and fourth decades of life, and predominantly affects women (81%). Most of the osseous choristomas in our review were located in the posterior third of the tongue, primarily at or close to the foramen cecum or circumvallate papillae (87.9%). While 39.7% of the patients were asymptomatic, the remaining patients complained of symptoms including a lump in the throat (25.8%), dysphagia (6.9%), gagging (5.1%), nausea (3.4%) and irritation (3.4%). Treatment of lingual osseous choristoma consists of simple excision. The tumor's origin has been discussed elsewhere.     

Metastatic follicular thyroid carcinoma to the mandible: a case report and review of the literature

OBJECTIVES: To describe the patterns of protective equipment use by in-line skaters in Winnipeg, Manitoba and nearby rural communities. METHODS: In-line skaters were observed for three months in 1996 at 190 urban and 30 rural sites selected using a formal sampling scheme. Age, gender, protective equipment use, skating companions, correct helmet use, and use of headphones were recorded. RESULTS: Altogether 123 in-line skaters were observed at 61 sites, including one rural site. No skaters were observed at the remaining sites. There were 37 adults and 86 children; 56% were male. Helmet use was 12.2% (95% confidence interval (CI) = 6.4% to 18.0%), wrist guard use was 16.3% (95% CI = 9.7% to 22.8%), knee pad use was 9.8% (95% CI = 5.2% to 16.4%), and elbow pad use was 7.3% (95% CI = 3.4% to 13.4%). Children were more likely to wear a helmet than teens 12-19 years of age (relative risk (RR) = 30, 95% CI = 4.01 to 225). Adults were more likely to wear wrist guards than children (RR = 4.32, 95% CI = 1.87 to 9.94). No gender differences were found. Incorrect helmet use was documented in four skaters; three skaters were wearing headphones. CONCLUSIONS: Low rates of protective equipment use were documented in our region, significantly lower than those reported in the literature. Barriers to equipment use are not known, and should be examined by further study. In-line skating safety programs should be developed, promoted, and evaluated. Teens should be targeted for future preventive efforts.     

Von-Voss-Cherstvoy syndrome (DK-Focomelia): description of the first case in Spain and a literature review

Because hypercellularity is an important feature in acute serum sickness (AcSS), we quantified glomerular proliferation with immunoperoxidase staining using anti-proliferating cell nuclear antigen (PCNA Mab) and studied its relationship with lymphocyte infiltration (M108 Mab). AcSS was induced in 31 New Zealand White rabbits; group A (n = 14), with proteinuria, sacrificed 6-8 days after immunization; group B (n = 10), without proteinuria, sacrificed 6-7 days after immunization; group C (n = 7), sacrificed prior to development of AcSS. Four normal rabbits were included as controls. Intraglomerular proliferation (PCNA-positive cells/glomerular cross section) was increased in group A (12.2 +/- SEM, 1.84) but not in groups B (0.93 +/- 0.17) and C (0.37 +/- 0.05), which were similar to controls (0.66 +/- 0.06). Lymphocyte infiltration (lymphs/glomerular cross section) increased with time and was more prominent in rabbits with proteinuria (1.9 +/- 0.21, P < 0.001 vs controls). Lymphocyte infiltration was correlated with proliferative activity (Spearman correlation, r = 0.67, P < 0.0001). There was correlation between the severity of glomerular deposition of IgG and C3 and glomerular proliferation and proteinuria. These studies demonstrate a chronological association between lymphocyte infiltration and proliferative activity in AcSS.     

Recto-cutaneous penile fistula. A description of a clinical case and review of the literature

This describes a newborn with an imperforate anus associated to a fistulous track extending along the scrotal raphe and opening at the penis top. This variant appears to be a low anomaly. The surgical treatment of this pathology is discussed.     

Insular carcinoma of the thyroid. Apropos of a case and review of the literature

We present a new case report of "Insular" thyroid carcinoma. The first series was published, in 1984, by Carcangiu et al. The propensity for local recurrence and early distant metastases explains the poor prognosis of this carcinoma and demands aggressive therapy at the time of diagnosis. This includes radical surgery, radiotherapy with possible chemotherapy. Clinical and pathological arguments suggest that this carcinoma could be an intermediate stage in the process of loss of differentiation of some thyroid neoplasms.     

Papillary and follicular thyroid carcinoma metastatic to the skin: a case report and review of the literature

Cutaneous metastases from thyroid cancers are rare. We report the case of an otherwise asymptomatic 81-year-old woman with an enlarging scalp lesion. Her solitary skin metastasis was the presenting feature of thyroid carcinoma. Routine histopathology of the lesion was notable for an atypical clear cell neoplasm. Immunohistochemistry was positive for thyroglobulin. Subsequent resection of the thyroid gland identified separate foci (< 1 cm) for both papillary and follicular carcinoma. Although such immunohistochemical staining has been used previously, it has never been reported to provide the definitive diagnosis for a solitary cutaneous metastasis from the thyroid. Previous tumors had anatomic features in a clinical context that permitted identification by routine light microscopy. Clear cell features found in the follicular focus of carcinoma in the thyroid suggest that it is the primary. A worldwide literature review reveals that follicular carcinoma has a greater preponderance than papillary carcinoma for cutaneous metastasis and that the majority of skin metastases from either papillary or follicular thyroid cancer are localized to the head and neck.     

Ectopic intrathyroidal thymus diagnosed as a solid thyroid lesion: case report and review of the literature

The authors report a case of a 6-year-old boy in whom aberrant thymic tissue was misdiagnosed as an intrathyroidal solid lesion. The patient underwent hemithyroidectomy and did well. Because cervical ectopic thymus rarely produces symptoms, the true incidence in children is unclear. Surgeons need to consider this rare entity, not to mistake it as a thyroid neoplasm, and not to perform extensive thyroid resection.     

Gallbladder diverticulum: a case report and review of the literature

BACKGROUND: The clinical significance of the 14 and 6 positive spikes (PS) electroencephalographic (EEG) phenomenon is not well established. This study was performed to provide further data regarding the clinical correlates of the PS, particularly attention-deficit/hyperactivity and somatic symptoms. METHODS: Diagnostic information gathered through structured interviews was compared among four groups of psychiatric inpatients aged 4-17 years who underwent an EEG examination over a 2-year period. Groups consisted of patients with: 1) 14 and 6 PS (n = 25); 2) epileptic discharges (n = 29); 3) slow-wave abnormalities (n = 23); and 4) a normal EEG group age and sex matched to the 14 and 6 PS group (n = 25). RESULTS: Attention-deficit hyperactivity disorder (ADHD) symptoms were significantly more frequent in the PS group (chi 2 = 2.96, p < .05) compared to the other three groups combined. Although somatic symptoms were not increased in the 14 and 6 PS group, anxiety symptoms tended to occur more in this group (chi 2 = 3.50, p < .06). CONCLUSIONS: The PS profile appears associated with ADHD symptoms. Possible treatment implications of this finding (e.g., use of anticonvulsants in ADHD patients with PS) need to be explored.     

Bilateral elastofibroma: a case report and review of the literature

Elastofibroma, or elastofibroma dorsi as it was first described, is an uncommon tumor-like process that characteristically appears as an ill-defined mass in the infrascapular region of elderly patients. This lesion can occur bilaterally and may appear asynchronously. The occurrence of elastofibroma in other anatomic sites has been reported, although with much less frequency. Elastofibromas display typical diagnostic histologic, cytologic, and electron microscopic features. The use of magnetic resonance imaging can lead to a presumptive diagnosis in elderly individuals with suprascapular lesions. It is important to differentiate this lesion from other soft-tissue lesions, such as sarcomas and desmoid tumors. This study presents a case of bilateral elastofibromas in a 72-year-old man and a review of the literature.     

Atelencephalic microcephaly: a case report and review of the literature

Rapid changes in the circulating blood volume or hemoglobin level during apheresis may pose a risk for healthy individuals donating allogeneic PBSC. In this study, a real-time noninvasive monitor CRIT-LINE was used for continuous monitoring of hematocrit values in a total of 16 aphereses performed in 4 adult (median age 30 years) and 4 pediatric donors (4 years). Donors received recombinant G-CSF (10 microg/kg s.c. for 5 days) for mobilization of PBSC. A CS3000 plus blood cell separator (Baxter) was used in two different procedures. Adults donors were subjected to modified program 1-120 using a combination of the granulocyte chamber and the small volume collection chamber (SVCC), and pediatric donors were subjected to specialized program 4 with a combination of the newly developed small volume separation chamber holder (SVSCH) and SVCC. In all of the procedures for children, the extracorporeal line was primed with 400 ml leukocyte-depleted allogeneic RBC or 200 ml autologous RBC after regular priming with normal saline, whereas none of the adult donors received this treatment. We found a marked contrast in the hematocrit kinetics during apheresis in the two cohorts/procedures. In adults, the initiation of apheresis was followed by an immediate decline in the hematocrit value over the initial 10 min until a stable plateau level was reached (7% decrease). In children, the values decreased slowly but progressively throughout the entire procedure to finally reach a 9% decrease at the completion of apheresis. These data may suggest that the use of SVSCH plus SVCC or priming with RBC can eliminate the abrupt decline in blood hemoglobin levels that occurs during apheresis.     

Riedel's thyroiditis: a case report and review of the literature

Riedel's thyroiditis is a very rare disease of unknown aetiology, occasionally associated with retroperitoneal and mediastinal fibrosis. It is a benign condition, but may be confused with an anaplastic carcinoma of the thyroid. The differential diagnosis with anaplastic carcinoma is assured only by intraoperative biopsy. The Authors report a clinical case: symptoms were a progressive enlargement of the thyroid gland, left recurrential palsy, dyspnoea and dysphagia. The surgical treatment was total thyroidectomy, performed with bilateral neurolysis of recurrent nerves. The patient was also under adjuvant corticosteroid treatment.     

Cryptococcal myositis: a case report and review of the literature

Only rare cases of cryptococcal myositis have been previously reported in the literature. All of these cases have occurred in the setting of human immunodeficiency virus (HIV) infection. We report a case of cryptococcal myositis diagnosed premortem on a needle biopsy in a heart transplant patient undergoing immunosuppressive therapy.     

Duodenal phytobezoar: a case report and review of the literature

Phytobezoars are an unusual cause of small bowel obstruction. We report a case of small bowel obstruction due to phytobezoar in a 63 year-old female patient who had undergone gastric surgery (truncal vagotomy with pyloroplasty) for duodenal ulcer disease complicated by gastric outlet obstruction 10 years ago. We diagnosed this bezoar case by radiologic methods and these methods keep their importance for the diagnosis of small bowel obstruction with phytobezoars.     

Gorham's disease: a literature review and case reports

Gorham's disease (disappearing bone disease, massive osteolysis, idiopathic osteolysis, essential osteolysis, progressive atrophy of bone, spontaneous absorption of bone, phantom bone, hemangiomatosis/lymphangiomatosis of bone, progressive osteolysis) is an extremely rare occurrence. There are fewer than 150 reported cases in the literature. This disorder can be characterized by spontaneous or posttraumatic progressive resorption of bone. The etiology is still very speculative, the prognosis unpredictable, and any effective therapy still unknown. This paper presents a review of the literature and two case reports of suspected Gorham's disease of the bones of the foot.     

Lingual thyroid: report of three cases

Lingual thyroid is a rare developmental disorder and is more frequent in women. The pathogenesis is unclear but may be related to the presence of maternal blocking autoantibodies against the thyroid. Treatment of this disorder includes the use of levothyroxine in order to correct the hypothyroidism, which is very frequent and to induce the shrinkage of the gland. When symptoms of obstruction or bleeding appear, ablative therapy by means of surgery or radioiodine is warranted. We report three cases and discuss the approach to diagnosis and a strategy for management.     

Familial insensitivity of the pituitary and periphery to thyroid hormone: a case report in two generations and a review of the literature

A clinically euthyroid 2-yr-old girl was found to have diffuse goiter that measured 3 X 5.5 cm with a prominent systolic bruit. Serum free T4 (3.4 ng/dl) and serum T3 (360 ng/dl) remained elevated for the next 10 months even though she remained clinically euthyroid. Elevation of serum free T4 (3.0 ng/dl) and serum T3 (265 ng/dl) was also present in the 24-yr-old nongoitrous mother who had symptoms and signs of hypothyroidism. Following intravenous injection of TRH, basal TSH levels of 2.7 and 2.8 microunits/ml increased to peak values of 17 and 21 microunits/ml at 30 min in the daughter and mother, respectively. Administration of exogenous T3 followed by sequential testing with boluses of TRH revealed retention of TSH responsiveness in both daughter and mother during pretreatment with dosage regimens of T3 below 125 micrograms daily. Maintenance of TSH responsiveness to TRH in the presence of elevated levels of serum free T4 and serum T3 indicates relative pituitary insensitivity to thyroid hormone which could be overridden by increasing the circulating levels of serum T3 three to fivefold over the already elevated basal levels. The absence of clinical signs of thyrotoxicosis indicates peripheral insensitivity to thyroid hormone with elevated circulating concentrations presumptively compensating for the defect. Resistance to thyroid hormone in two generations of the same family suggests genetic inheritance, and is concordant with four earlier reports of familial aggregation in this syndrome.     

Congenital microgastria: a case report and review of literature

Congenital microgastria is an uncommon result of impairment of normal foregut development. To date, only 39 cases have been described in the literature. We report a boy born with microgastria and bilateral hypoplastic kidneys who had feeding problems, resulting in failure to thrive and growth retardation. After a short period of conservative management, he was operated upon at the age of 11 months. A Hunt-Lawrence pouch was created, leading to toleration of increasing amounts of oral feeding. Although his feeding problems have decreased, his height and weight are below normal (<10th percentile). The embryology, clinical presentation, diagnostic procedures, associated anomalies, and management are discussed.