Receptor expression in orbital inflammatory syndromes and implications for targeted therapy

To investigate the expression of multiple therapeutic targets in tissue specimens from patients with orbital inflammatory syndromes, the clinical records of 16 patients treated for orbital inflammation between January 2003 and November 2005 for whom tissue blocks were available were reviewed retrospectively. Immunohistochemical staining was performed on archived specimens using commercially available monoclonal antibodies against CD3, CD20, CD22, CD23, CD25, and CD52 antigens. The histologic diagnoses were confirmed, and the immunohistochemical staining patterns were agreed upon by both collaborating pathologists (JLJ and PC-B). The study included 13 women and 3 men who ranged in age from 4 to 79 years (mean, 46 years). The histologic diagnoses were as follows: orbital pseudotumor in six patients; sarcoidosis, three; eosinophilic granuloma, one; necrobiotic xanthogranuloma, one; nonspecified granulomatous inflammation, one; Graves' ophthalmopathy, one; Wegener's granulomatosis, one; and reactive lymphoid hyperplasia, two. One orbital lymphoma specimen and one foreign body reaction specimen were used as controls. CD20 was strongly expressed in all specimens except three (Wegener's granulomatosis, eosinophilic granuloma, and nonspecified granulomatous inflammation specimens), and CD25 was strongly expressed in all specimens except the Wegener's granulomatosis specimen, in which this antigen was only moderately expressed. CD20 and CD25 were strongly or moderately expressed in most of the tested specimens of orbital inflammation. If our findings are confirmed in a larger study, rituximab, which targets CD20, and denileukin diftitox (ONTAK), which targets CD25, should be considered for future clinical trials for orbital inflammatory syndromes.     

Primary orbital meningioma: a study of six cases at a single institution

Primary orbital meningioma is a rare tumor of the anterior visual pathway and constitutes approximately 2% of all orbital tumors and 1-2% of all meningiomas. The differentiation from secondary orbital meningioma of intracranial origin is sometimes difficult on image. As the tumor often leads to visual loss if left untreated and surgical intervention inevitably causes morbidity, the timing and modality of treatment are very important. We carried out the study involving six cases (mean age: 42.7 years, male to female ratio: 1:5) of primary orbital meningioma to further elucidate its behavior. The clinical signs and symptoms, diagnosis, treatment strategies, and follow-up information are recorded for all cases. The most frequent initial symptoms were visual complaints (100%) and proptosis (67%). In five cases, the diagnosis was based on pathologic findings and the tumors were all grade I meningiomas. In one case, however, the diagnosis was based on radiographic and clinical findings, lacking histologic confirmation. Five patients were operated on, four underwent tumor removal, and one received eyeball exenteration. One patient was treated with Novalis radiotherapy. The mean follow-up period was 8.8 years (range from 9 months to 15 years). All patients experienced loss of vision during the course without exception. No recurrent tumor was found in five cases during follow-up. In case 5, whose eyeball was exenterated, developed recurrent meningioma 7 years later. She received radiotherapy but the tumor was out of control. She expired 8 years after eyeball exenteration. The primary orbital meningioma is aggressive in behavior despite its benign histopathologic features. Loss of vision is frequently seen even after treatment. The tumor could be fatal if surgery and radiotherapy fail to control its intracranial extension.     

Clinical analysis of computed tomography-staged orbital cellulitis in children.

BACKGROUND AND PURPOSE: Bacterial infection of the orbital structures can affect all age groups, but is more frequent in pediatric populations. Prompt recognition, correct diagnosis, and adequate management are important if serious complications are to be avoided. This study sought to delineate the clinical, bacteriological and radiological findings, management and outcome of orbital cellulitis. METHODS: This retrospective study reviewed 80 children admitted to Chang Gung Children's Hospital with a diagnosis of orbital cellulitis who were staged by computed tomography (CT), between January 1999 and August 2005. The staging classification was as follows: stage I, inflammatory edema (preseptal); stage II, subperiosteal phlegmon and abscess; stage III, orbital cellulitis; stage IV, orbital abscess; and stage V, ophthalmic vein and cavernous sinus thrombosis. The patients were categorized into 2 groups: preseptal (stage I) and postseptal (stage II-V). RESULTS: Of the 80 children, 50 were male and the mean age was 6.8 years. Sinusitis and upper respiratory tract infection were the most common predisposing factors. Forty one percent of patients in stage I presented with symptoms that indicated postseptal involvement. The patients with postseptal involvement had a significantly higher rate of proptosis and limitation of extraocular motility. Bacterial pathogens were identified in 31 patients (39%), the 2 most common pathogens being Staphylococcus and Streptococcus. Ten patients (13%) had polymicrobial infection. Twenty three patients underwent sinus and/or orbital and/or intracranial surgery, including all 5 patients (100%) in stage IV, 3 of 6 patients (50%) in stage III, 13 of 35 patients (37%) in stage II, and 2 of 34 patients (6%) in stage I. Complete resolution without complication was achieved in 72 children. Eight patients had complications, including intracranial infection in 3, recollection of abscess in 2, ophthalmoplegia in 2, and corneal scar in 1. CONCLUSIONS: Proptosis and limitation of extraocular motility may be considered the most important signs on CT examination in children with suspicious orbital cellulitis. Given that polymicrobial infection is common, broad-spectrum antibiotics are indicated initially. Surgery should be considered not only when an abscess is demonstrated by CT scan but also if clinical deterioration occurs within 24 to 36 h of adequate intravenous antibiotic treatment.     

Orbital solitary fibrous tumor: encompassing terminology for hemangiopericytoma, giant cell angiofibroma, and fibrous histiocytoma of the orbit: reappraisal of 41 cases

Hemangiopericytomas and solitary fibrous tumors are uncommon neoplasms found in many locations, including the orbit. Both mesenchymal neoplasms share several clinicopathologic features, thus prompting intense debate as to whether they are variants of the same entity or merit separate designations in the orbit. These 2 entities, with the addition of giant cell angiofibroma of orbit, are of benign- to uncertain-behavior, CD34-positive, collagen-rich, specialized fibroblastic tumors, which may have overlapping or histologically identical features. In addition, so-called fibrous histiocytoma of orbit, a previous designation, has overlapping morphologic features with these tumors. To date, a large series of these collagen-rich fibroblastic tumors of the orbit has not been fully explored. Forty-one fibroblastic orbital tumors, originally diagnosed as hemangiopericytomas (n = 16), fibrous histiocytomas (n = 9), mixed tumors (hemangiopericytomas/fibrous histiocytoma) (n = 14), and giant cell angiofibromas of orbit (n = 2) between 1970 and 2009, were retrieved from our consultation files, the Ophthalmic Registry, at the Armed Forces Institute of Pathology. Slides and clinical records were reviewed, analyzed, and compared. Immunochemistry was performed for CD34, CD99, Bcl-2, Ki-67, and p53. Upon histologic review, all cases were reclassified as solitary fibrous tumor (41/41). The patients included 23 (56%) males, 17 (41%) females, and 1 unknown, with a mean age at presentation of 40.7 years (range, 16-70 years). The sites of involvement were the right orbit in 18 (44%) cases and the left in 16 (39%) cases. Tumors ranged in size from 0.4 to 5.0 cm (mean, 2.2 cm). Seventeen (41%) patients presented with an orbital mass, 8 (20%) with proptosis, 2 (5%) with painful mass, and 2 (5%) with painless mass. Duration of symptoms ranged from 3 to 96 months, with a mean of 23 months (median, 9 months). Microscopically, all lesions showed considerable similarity, varying in degree of cellularity, stromal collagen, and the presence of giant cells. Overlapping features with soft tissue giant cell fibroblastoma were observed. Immunochemistry revealed positivity for CD34 in all cases (100%), p53 in 85%, CD99 in 67.5%, and Bcl-2 in 47.5%. Although Ki-67 labeling was seen in all cases, it ranged from less than 1% in 54.3% of cases to 5% to 10% in 20% of cases. Taken together, the findings of this study suggest that orbital hemangiopericytoma and some cases previously designated as fibrous histiocytoma, giant cell angiofibroma of orbit, and solitary fibrous tumor have overlapping morphologic and immunohistochemical features and should be designated as solitary fibrous tumor. Adipocytes and unusual multivacuolated adipocytic cells may be present in these tumors, as well stromal myxoid change; and even stromal intramembranous ossification can be observed. There are overlapping features of orbital solitary fibrous tumor with another CD34-positive specialized fibroblastic tumor of soft tissue, giant cell fibroblastoma. Morphologic criteria for uncertain behavior to low-grade malignant ocular solitary fibrous tumors can be made by cytologic atypia and increased mitotic activity, but overall outcome for malignant solitary fibrous tumors of the eye should be further explored.     

Primary liposarcoma of the orbit: a clinicopathologic study of seven cases

Liposarcoma, the most common soft tissue sarcoma in adults, will rarely involve the orbit, either primarily or as a metastasis. We describe seven primary orbital liposarcomas, representing the largest documented series of primary orbital liposarcoma to date. Affected patients were three males and four females ranging in age from 28 to 69 years (median, 51 years). Five patients presented with painless proptosis, one patient had painful proptosis, and no details of presenting symptoms are available in one case. The site distribution was retrobulbar (3 cases), lateral orbital wall (2 cases), medial wall (1 case), and unspecified (1 case). Radiologic impression included hemangioma, lipoma, and an inflammatory process. Lesional size ranged from 2.8 to 4 cm. Five liposarcomas were purely well-differentiated, one was dedifferentiated, and one was pleomorphic in type. The well-differentiated cases comprised the following subtypes: spindle cell (2 cases), adipocytic (2 cases), and combined adipocytic/sclerosing/inflammatory (1 case). Five patients underwent orbital exenteration (one followed by radiation) and two had marginal/partial excision of their tumors. Follow-up was available for five patients, ranging from 13 to 204 months (median 65 months). Four patients showed no evidence of recurrence, including the patient with pleomorphic liposarcoma who had a long, disease-free survival (65 months) following marginal excision. One patient has had multiple recurrences following initial partial excision. One patient died of an unrelated cause with no clinical evidence of recurrence. Despite the difficulty in obtaining wide surgical margins, the small tumor size at presentation and the apparent predominance of the well-differentiated type means that the prognosis for orbital liposarcoma is generally good. In view of the various morphologic patterns that may occur, liposarcoma should be considered in the differential diagnosis of any histologically unusual mesenchymal lesion in the orbit.     

Fibrous histiocytoma of the orbit. A clinicopathologic study of 150 cases

This is a clinicopathologic study of 150 cases of fibrous histiocytoma of the orbit. The tumors occurred in adults (median age, 43 years). The upper and nasal portions of the orbit were the most common sites of involvement. The most common signs and symptoms were proptosis (60 per cent), mass (46 per cent), and decreased vision (25 per cent). Based on the histopathologic features, the tumors were classified in three groups: benign (94 cases), locally aggressive (39 cases), and malignant (17 cases). The biological behavior correlated well with the duration of symptoms, the size and margins of the mass, and the histologic classification. The rate of recurrence was 31 per cent for the benign tumors, 57 per cent for the locally aggressive tumors, and 64 per cent for the malignant tumors. Follow-up data were obtained for 123 patients, with a mean duration of seven years. The ten-year survival of patients with benign, locally aggressive, and malignant fibrous histiocytoma was 100 per cent, 92 per cent, and 23 per cent, respectively. Nine patients died as a result of the tumor, six from local invasion of adjacent structures and three from metastatic disease. Fibrous histiocytoma is the most common primary mesenchymal orbital tumor of adults. The origin of the neoplasm is probably a primitive mesenchymal cell. Complete surgical excision appears to be the treatment of choice.     

Corticosteroid effect on immunoglobulins

The corticosteroid (prednisone) effect on serum immunoglobulins in 9 atopic asthmatic patients who required corticosteroids for the control of asthma was evaluated. Serum immunoglobulins were determined before corticosteroids were administered, an average of 15 days while on corticosteroids, and again an average of 22 days after corticosteroids were discontinued. While on corticosteroids (averaging 16.8 mg prednisone daily) for 15 days, mean serum IgG was significantly decreased (?22%, p ≤ 0.01), mean serum IgA tended to be decreased (?10%), and mean serum IgM was essentially unchanged. Serum IgE was initially significantly increased (p < 0.01) when compared to levels of other serum immunoglobulins (IgG,A,M). An average of 22 days after corticosteroids were discontinued, mean serum IgG was still significantly decreased (p < 0.05), and mean serum IgA again tended to be decreased. Serum IgM remained unchanged and mean serum IgE now was significantly decreased (p < 0.01). Corticosteroids appear to have a significant effect on levels of some serum immunoglobulins.     

Primary ear and temporal bone meningiomas: a clinicopathologic study of 36 cases with a review of the literature.

"Primary" ear and temporal bone meningiomas are tumors that are frequently misdiagnosed and unrecognized, resulting in inappropriate clinical management. To date, a large clinicopathologic study of meningiomas in this anatomic site has not been reported. Thirty-six cases of ear and temporal bone meningiomas diagnosed between 1970 and 1996 were retrieved from our files. Histologic features were reviewed, immunohistochemical analysis was performed (n = 19), and patient follow-up was obtained (n = 35). The patients included 24 females and 12 males, aged 10-80 years (mean, 49.6 years), with female patients presenting at an older age (mean, 52.0 years) than male patients (mean, 44.8 years). Patients presented clinically with hearing changes (n = 20), otitis (n = 7), pain (n = 5), and/or dizziness/vertigo (n = 3). Symptoms were present for an average of 24.6 months. The tumors affected the middle ear (n = 25), external auditory canal (n = 4), or a combination of temporal bone and middle ear (n = 7). The tumors ranged in size from 0.5 to 4.5 cm in greatest dimension (mean, 1.2 cm). Radiographic studies demonstrated a central nervous system connection in 2 patients. Histologically, the tumors demonstrated features similar to those of intracranial meningiomas, including meningothelial (n = 33), psammomatous (n = 2), and atypical (n = 1). An associated cholesteatoma was identified in 9 cases. Immunohistochemical studies confirmed the diagnosis of meningioma with positive reactions for epithelial membrane antigen (79%) and vimentin (100%). The differential diagnosis includes paraganglioma, schwannoma, carcinoma, melanoma, and middle ear adenoma. Surgical excision was used in all patients. Ten patients developed a recurrence from 5 months to 2 years later. Five patients died with recurrent disease (mean, 3.5 years), and the remaining 30 patients were alive (n = 25, mean: 19.0 years) or had died (n = 5, mean: 9.5 years) of unrelated causes without evidence of disease. We conclude that extracranial ear and temporal bone meningiomas are rare tumors histologically similar to their intracranial counterparts. They behave as slow-growing neoplasms with a good overall prognosis (raw 5-y survival, 83%). Extent of surgical excision is probably the most important factor in determining outlook because recurrences develop in 28% of cases.     

53例SARS早期患者的临床分析

目的研究SARS早期的临床特点.方法对53例SARS早期病例的流行病学、临床、实验室及影像学资料进行回顾性分析.结果53例患者中男性24例,女性29例,平均年龄(38±16.7)岁(10～85岁),发病年龄以20～50岁患者居多(67.9%),平均潜伏期(7.3±7.0)d(3～14 d),职业分布居多者为职员(11例,占20.8%),其次是医护人员(9例,17.0%).临床症状以发热(100%),咳嗽(49.1%),肌肉酸痛(24.5%),乏力(17.0%),胸闷憋气(20.8%),腹泻(5.7%)等为主要表现.在病程的第1～5天患者白细胞＜4.0×109/L 33例(62.3%),(4.0～10.0)×109/L 18例(34.0%),67.9%患者淋巴细胞计数减少,13.2%患者血小板计数减少.X线胸片主要病变呈肺部斑片状渗出性阴影,其中双侧受累占15.1%,单侧受累75.4%.血气分析示PO2＜90mm Hg(1kPa=7.5 mm Hg)26例(占49.1%),出现酸碱平衡紊乱者9例(17.0%),其中代谢性酸中毒4例(10.8%)及代谢性碱中毒合并呼吸性酸中毒4例(10.8%).SARS常合并多脏器损伤,其中肝功能(ALT、AST)异常占37.7%,肾功能(BUN、SCR)损伤占11.3%,心肌酶(LDH、CK、HBDH)异常占43.4%.结论本病临床表现复杂,密切监测血常规、胸片及血气分析对于早期诊断意义重大.     

Catastrophic antiphospholipid syndrome (CAPS): Descriptive analysis of a series of 280 patients from the “CAPS Registry”

ObjectiveTo describe the clinical and laboratory features, as well as the precipitating factors, treatment and outcome of patients with catastrophic antiphospholipid syndrome (APS).MethodsWe analyzed the 280 patients included until September 2008 in the website based international registry of patients with catastrophic APS (“CAPS Registry”) (http://www.med.ub.es/MIMMUN/FORUM/CAPS.HTM).ResultsThe entire series includes 201 (72%) female and 79 (28%) male patients with a mean age of 37 ± 14 years (range, 11–60 years). A total of 129 (46%) patients suffered from primary APS, 112 (40%) from systemic lupus erythematosus, 14 (5%) from lupus-like disease, and 25 (9%) from other autoimmune diseases. The catastrophic episode was the first manifestation of the APS in 129 (46%) patients. A precipitating factor was reported in 53% of the patients. The first clinical manifestation at the time of the catastrophic episode was a pulmonary complication in 24% of the cases, a neurologic feature in 18% and a renal feature in 18%. During the catastrophic episode, intraabdominal involvement was identified in the majority of patients, mainly consisting of renal (71%), hepatic (33%), gastrointestinal (25%), splenic (19%), adrenal (13%), and pancreatic (8%) manifestations. 123 (44%) patients died at the time of the catastrophic APS event but the higher recovery rate was achieved by the combination of anticoagulants plus corticosteroids plus plasma exchange (PE) and/or intravenous immunoglobulins (IVIG) (69% versus 54%).ConclusionsThe catastrophic APS is an uncommon but potentially life-threatening condition that needs high clinical awareness. The therapeutical connotation is that this may be corrected with the combination of anticoagulation plus steroids plus attempts at achieving a prompt reduction of antiphospholipid antibody titer (i.e. PE and/or IVIG).     

Dural cavernous sinus fistula: an unusual presentation.

This article describes a 22-year-old man who presented to the Howard University Hospital emergency room with acute onset of swelling, proptosis, and decreased vision in the right eye preceded by 24 hours of nausea and vomiting. The patient''s visual acuity was count fingers in the involved eye with marked proptosis and limitation of ocular motility. There was no history given of any ocular or head trauma. A computed tomography scan of the orbits showed diffuse symmetric enlargement of the extraocular muscles of the right eye, felt to be consistent with an orbital inflammatory pseudotumor. The patient was treated with intravenous steroids initially, then placed on oral prednisone. After minimal improvement on the steroids, a selective external carotid angiogram showed a moderate-sized dural cavernous sinus fistula. The patient underwent selective embolization of the fistula with rapid resolution of periorbital edema and proptosis. Visual acuity was stabilized at 20/200 in the right eye. The differential diagnosis and pathogenesis of carotid cavernous sinus fistulas and the likely pathogenesis of the fistula in this case are discussed.     

Allergic fungal sinusitis in children.

BACKGROUND: Allergic fungal sinusitis (AFS) has been characterized in adults presenting with chronic sinusitis. Rare reports allude to a similar disease in children. OBJECTIVE: To characterize the features of AFS in children. METHODS: Children referred to otolaryngology clinics at Arkansas and LeBonheur Children's Hospitals for chronic sinusitis during a 12-year period were studied. This retrospective analysis reviews the following: clinical presentation, laboratory evaluations, radiographic and pathologic findings, and surgical intervention. Twenty patients (age range, 7-18 years; mean age, 12.5 years; median age, 16 years) met previously published criteria for AFS. Thirteen patients were male and 7 were female. Thirteen were African American and 7 were white. RESULTS: Presentation at diagnosis included the following: atopy (n = 20), nasal symptoms (n = 20), recurrent sinusitis (n = 18), nasal polyps (n = 18), recurrent headaches (n = 12), asthma (n = 11), proptosis (n = 10), and ocular symptoms (n = 10). All had radiographic evidence of sinusitis and allergy to fungal organisms. IgE levels were elevated in 8 of 9 patients, and 10 of 15 patients had eosinophilia. Surgical specimens demonstrated allergic mucin (n = 11), Charcot-Leyden crystals (n = 2), hyphae or fungal debris (n = 9), and fungal growth (n = 17). All patients underwent endoscopic sinus surgery, with 11 requiring multiple surgical procedures. Postoperatively, 19 patients received intranasal and oral steroids, and all had nasal saline washes. Eleven patients (9 who had undergone multiple surgical procedures) were treated with immunotherapy. Relapse was seen in 55% of patients at 1 year of follow-up. CONCLUSION: AFS presents with a higher incidence of proptosis in children when compared with adults. Typically, AFS occurs in atopic children with refractory sinus disease, requiring a high index of suspicion for evaluation and aggressive treatment.     

Clinical features in 27 patients with Angelman syndrome resulting from DNA deletion.

We report the clinical features in 27 Australasian patients with Angelman syndrome (AS), all with a DNA deletion involving chromosome 15(q11-13), spanning markers from D15S9 to D15S12, about 3 center dot 5 Mb of DNA. There were nine males and 18 females. All cases were sporadic. The mean age at last review (end of 1994) was 11 center dot 2 years (range 3 to 34 years). All patients were ataxic, severely retarded, and lacking recognisable speech. In all patients, head circumference (HC) at birth was normal but skewed in distribution, with 62 center dot 5% at the 10th centile. At last review HC was around the 50th centile in three patients (12 center dot 5%) while 15 had poor postnatal head growth. Short stature was not invariable, 5/26 (19%) were on or above the 50th centile. Hypotonia at birth was recorded in 15/24 (63%) and neonatal feeding difficulties were recorded in 20/26 (77%). Epilepsy was present in 26/27 (96%) with onset by the third year of life in 20 patients (83%). Improvement in epilepsy was reported in 11/16 patients (69%) with age. An abnormal EEG was reported in 25/25 patients. Hypopigmentation was present in 19/26 (73%). One patient had oculocutaneous albinism. Five patients could not walk independently. Of the remaining 22 who could walk, age of onset of walking ranged from 2 to 8 years. Disrupted sleep patterns were present in 18/21 patients (86%), with improvement in 9/12 patients (75%) over 10 years of age. The clinical features in this group of deletional AS patients were similar to previous reports, but these have not separated patients into subgroups based on DNA studies. In our group of deletional cases, 100% showed severe mental retardation, ataxic movements, absent language, abnormal EEG, happy disposition (noted in infancy in 95%), normal birth weight and head circumference at birth, and a large, wide mouth. These features occurred with a higher frequency than in AS patients as a whole. Our study also provided information on the evolution of the phenotype. The data can act as a benchmark for comparisons of AS resulting from other genetic mechanisms.     

Thoratec ventricular assist devices in pediatric patients: update on clinical results

Particularly in pediatric patients, mechanical circulatory support remains a clinical challenge. We analyzed the Stanford experience with use of the Thoratec ventricular assist device (VAD) in children and adolescents and data from the company's voluntary database. Through January 2005, 209 patients up to 18 years of age have been supported with the Thoratec VAD worldwide. Mean age was 14.5 years (range 5-18 years), mean weight was 57 kg (range 17-118 kg), and mean body surface area was 1.6 m2 (range 0.7-2.3 m2). The majority of patients were supported for cardiomyopathies (55%) and acute myocarditis (25%). A minority (6%) was treated for end-stage congenital heart disease. Average duration of support was 44 days (0-434 days). Overall survival to transplantation or weaning off the device was 68%. Survival rates were higher for patients with cardiomyopathies (74%) and acute myocarditis (86%) compared with patients with congenital heart disease (27%). We performed a subanalysis in small children with a body surface area of less 1.3 m2. This subgroup had a higher incidence of congenital heart disease and a slightly lower survival (52%). Aspects of the particular risks and device management in these small patients are discussed.     

Persistent airflow obstruction in asthma of patients with Churg–Strauss syndrome and long-term follow-up

Background:  Little is known about the long-term outcome of airflow obstruction in asthma of patients with Churg–Strauss syndrome (CSS).
Methods:  We conducted a retrospective study of 24 consecutive patients (aged 41.1 ± 13.5 years) with CSS in a single center. All had asthma (starting 8.1 ± 9.5 years prior to the diagnosis of CSS), blood eosinophilia (6.1 ± 4.4 × 10⁹/l) and systemic manifestations of CSS. Antineutrophil cytoplasmic antibodies were found in 7 of 22 tested patients. Seven patients had smoked (a mean of 10 pack-years). All patients received oral corticosteroids, 11 cyclophosphamide and 23 inhaled corticosteroids.
Results:  Airflow obstruction was found in 14 patients (70%) at diagnosis, and in 11 of 22 patients (50%) at the time of the clinical remission of CSS. The mean postbronchodilator FEV1/FVC and FEV1 were 69 ± 12% and 74 ± 21% of predicted at diagnosis ( n  = 20); 71 ± 10% and 92 ± 19% of predicted at the clinical remission ( n  = 22); and 64 ± 13% and 80 ± 21% at last visit ( n  = 13), respectively. During follow-up, postbronchodilator FEV1 increased by 30 ± 28% in six patients with FEV1/FVC < 70% despite inhaled therapy who received higher dose of oral corticosteroids. At last visit, 5 of 13 patients (38%) with more than 3 years of follow-up had persistent airflow obstruction as defined by postbronchodilator FEV1/FVC < 70% and FEV1 < 80% of predicted.
Conclusion:  Airflow obstruction due to uncontrolled asthma is present despite corticosteroids in many patients at diagnosis and at clinical remission of CSS, and during follow-up. It may be still partly reversible with increased oral corticosteroid treatment.     

Three vs. 10 days of amoxycillin–clavulanic acid for type 1 acute exacerbations of chronic obstructive pulmonary disease: a randomised, double-blind study

The optimal duration of antibiotic treatment for acute exacerbations of chronic obstructive pulmonary disease (AECOPD) is unknown. This study compared the outcome of treatment for 3 vs. 10 days with amoxycillin-clavulanic acid of hospitalised patients with AECOPD who had improved substantially after initial therapy for 3 days. Between November 2000 and December 2003, 56 patients with AECOPD were enrolled in the study. Unfortunately, because of the low inclusion rate, the trial was discontinued prematurely. Patients were treated with oral or intravenous amoxycillin-clavulanic acid. Patients who showed improvement after 72 h were randomised to receive oral amoxycillin-clavulanic acid 625 mg or placebo, four times daily for 7 days. The primary outcome measure of the study was clinical cure after 3 weeks and 3 months. Of 46 patients included in the final analysis, 21 were in the 3-day treatment group and 25 were in the 10-day treatment group. After 3 weeks, 16 (76%) of 21 patients in the 3-day treatment group were cured, compared with 20 (80%) of 25 in the 10-day treatment group (difference -3.8%; 95% CI -28 to 20). After 3 months, 13 (62%) of 21 patients were cured, compared with 14 (56%) of 25 (difference 5.9%; 95% CI -23 to 34). Microbiological success, symptom recovery, the use of corticosteroids, the duration of oxygen therapy and the length of hospital stay were comparable for both treatment groups. It was concluded that 3-day treatment with amoxycillin-clavulanic acid can be a safe and effective alternative to the standard 10-day treatment for hospitalised patients with AECOPD who have improved after initial therapy for 3 days.     

Neuropathology associated with ventricular assist devices: an autopsy series of 33 patients

The ventricular assist device (VAD) is a mechanical pump that has been shown to be an effective modality of cardiac support in patients with heart failure refractory to pharmacologic intervention and who are awaiting cardiac allograft transplantation. Neuropathologic findings in these patients have not been well described. We retrospectively reviewed 2,632 autopsy reports (between 1990 and 2000) and found 64 patients who received VADs. Of these 64 patients, brain and spinal cord tissue was available for review in 33 patients (25 males and eight females; age range, 4 to 69 years; mean age, 52 years). The study group was composed of these 33 patients. Ventricular assist devices were in place from one to 603 days (mean 49 days). Twenty-five patients had left VAD, three had right VAD, and five had biventricular VADs. Brain weights ranged from 928 g to 1,740 g (mean 1,325 g). The most common central nervous system pathologic findings included infarct (N = 23; 70%), acute neuronal necrosis (N = 22; 67% focal and N = 1; diffuse anoxic encephalopathy), hemorrhage (N = 14; 42%), and herniations (N = 7; 21%). Two patients had no neuropathologic findings at autopsy. Cause of death was central nervous system-related in eight patients (24%) including six with massive parenchymal hemorrhage and herniations, one with brainstem infarction, and one with air embolism (radiographically diagnosed). The most common causes of death in the remaining 25 patients included sepsis (n = 10; 30%), pneumonia (n = 4; 12%), and embolic events with widespread infarcts (n = 4; 12%). The most common neuropathologic findings in patients with VAD were related to ischemia and infarction. In a significant subset of patients, central nervous system pathology, particularly hemorrhage with herniation, was the primary cause of death. Ann Diagn Pathol 5:67-73, 2001.     

Clinical profile of abdominal tuberculosis in children

Aim: A retrospective study was conducted in children, suffering from abdominal TB, attending Pediatric TB clinic from 2007 to 2009. Materials And Methods: Age-wise distribution and type of abdominal TB were analyzed with clinical features. Results : Out of 285 children with TB, 32 (11.2%) had abdominal tuberculosis. Male: Female ratio was 2.1:1. 7 (21.9%) children were < 5 years of age, 15 (46.9%) children were 5 - 10 years of age, and 10 (31.2%) children were > 10 years of age. The most common clinical features were fever in 24 (75%), pain in abdomen in 17 (53.1%), loss of weight in 15 (46.9%), raised ESR in 14 (43.8%), and loss of appetite in 13 (40.6%) children. TB contact was present in 10 (31.2%), and 7 (21.9%) children had tuberculosis in the past. 28 (87.5%) children had received BCG vaccine, and 17 (53.1%) had a positive Mantoux test. Extra-abdominal tuberculosis was found in 17 patients (53.1%). Duration of fever was more in children less than 5 years of age (127 ± 66 days) than that in children between 5 -10 years (37 ± 30 days) and in > 10 years of age (73 ± 66 days), which is statistically significant (P = 0.0228). Lymph node TB (17 patients, 53.1%) was found to be the commonest, followed by intestinal (10 patients, 31.2%) and peritoneal TB (4 patients, 12.5%). 18 (56.2%) of the total patients had recovered, 7 (21.9%) of all patients failed first line therapy and had to be started on second line drugs, of which 4 (12.5%) were proven to have drug-resistant TB. Conclusion : Abdominal TB is seen in 11.2% of children affected with TB, of which over 53% will have extra-abdominal manifestations. Common clinical and laboratory features include fever, pain in abdomen, loss of weight, loss of appetite, and raised ESR. The duration of fever is more in children of younger age group. Lymph node TB is the most common type of abdominal TB. Drug-resistant TB is seen in at least 12.5% of the patients.     

Painful ophthalmoplegia--Tolosa-Hunt syndrome

Tolosa Hunt syndrome is a rare disorder caused by nonspecific inflammation in the cavernous sinus/superior orbital fissure and/or orbital apex. It is clinically characterized by alternating remissions and exacerbations, and manifested as diplopia associated with unilateral periorbital hemicranial headache. The symptoms include blepharoptosis, which is usually mild if present, bulbomotor paresis involving the pupil, and loss of sensation in the area supplied by the first division of the trigeminal nerve. Therapy for Tolosa-Hunt syndrome are systemic steroids. The course of disease in a 25-year-old man hospitalized for painful ophthalmoplegia and diplopia is presented. The history included severe pain on rightward eye movement and parabulbarly on the right, considerable defect in the area supplied by the first division of the trigeminal nerve, right hemicrania, and diplopia on looking to the left, right, upward and downward that developed after four days. A month before, the patient was observed at neurology department for severe right hemicrania. Current status included severe pain parabulbarly on the right, discrete proptosis with mild ptosis on the right eye, restricted right eye bulbar motoricity on looking nasally, nasally upward and downward, and loss of sensation in the area supplied by the first division of the trigeminal nerve. Pupilar motoricity was normal. Upon admission, neuroradiologic examination (orbit CT) and brain MR were performed, and therapy with systemic corticosteroids was initiated ex iuvantibus, in consultation with a neurologist. At 24 hours of corticosteroid therapy, the pain subsided, whereas diplopia disappeared almost completely after 5 days, now being only occasionally recorded on looking to the left and upward.     

Adult non-viral lymphocytic meningitis in Abidjan (Côte d'Ivoire)

Our prospective and longitudinal study aimed to analyse the aetiologies, clinical features and prognostic of non viral lymphocytes meningitis (NVLM). We recruited 131 patients, 77 males (59%) and 54 females (41%) sex-ratio 1.4. The mean age was 35 years [15-67]. 117 patients were HIV positive (89%) and 14 (11%) were HIV negative. Feverish meningoencephalitis was present in 85% of cases, with 7 days for mean delay of admission into hospital. 80 germs were found in the C.S.F. 70 Cryptococcus neoformans, 4 Mycobacterium tuberculosis, 3 Streptococcus pneumoniae, 1 Candida albicans, 1 Neisseria meningitis and 1 Trypanosoma gambiense. 63 aetiologies were linked to lymphocytes meningitis by indirect deduction: 41 cases of tuberculous meningitis with lung X-ray anomalies and M. tuberculosis in sputum (11 times), 11 cases of cerebral malaria with Plasmodium falciparum in blood, 11 cases of cerebral toxoplasmosis by significant features with cerebral tomodensitometry. Letality was 53%, 35% of patients improved and 12% were lost to follow-up. Our study shows the difficulties in the management of the NVLM, due to the delay of diagnosis, particularly for tuberculous meningitis.