Posttransplant epithelioid inflammatory myofibroblastic sarcoma: A case report

Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare entity and a novel variant of inflammatory myofibroblastic tumor (IMT), usually seen in children and nonsmoking young adults. Their occurrence in a posttransplant setting is still rare. These tumors are characterized by prominent epithelioid morphology, large histiocytoid “Reed Sternberg”-like cell, unique pattern of ALK immuno-reactivity, and aggressive clinical behavior. Their etiology and metastatic potential is controversial. In a post-transplant setting, many factors such as trauma, infections with EBV, HIV, Hepatitis C, mycobacteria, fungus, and chemotherapy-induced immunosuppression have been implicated in their etiology. We present the case of a 2-year-old female child who developed multiple omental and mesenteric tumor nodules, 8 months post liver transplant for progressive familial intrahepatic cholestasis (PFIC). Following a histopathological diagnosis of “mesenchymal neoplasm of possible malignant nature” on a trucut biopsy and frozen section, tumor debulking was performed. A final histological diagnosis of EMIS was made on the completely resected tumor. The patient remains in remission nearly 7 months after presentation, without any follow-up systemic chemotherapy. IMT after a solid organ transplant is rare, only 5 cases have been reported in the literature until now. Similar phenomenon has also been noted with hematopoietic stem cell transplant. However, to our knowledge, this case of EMIS in a post liver transplant patient is first of its kind.

Antithrombotic Therapy in Patients Postcardiac Interventions.

Many primary care doctors need to know exactly how to manage antithrombotic medication of patients who have earlier undergone a cardiac intervention or surgery, if they come for a noncardiac problem. This article addresses this important issue.

Risk factors and survival analysis of the esophageal cancer in the population of Jammu, India.

Objective: To identify the risk factors of esophageal cancer and study their effect on the survival rates patients of Jammu region, India. Materials and Methods: Detailed information was collected on socio-demographic, dietary and clinico-pathological parameters for 200 case control pairs. Discrete (categorical) data of 2 independent groups (control and cases) were summarized in frequency (%) and compared by using Chi-square (χ2 ) test. The mean age of two independent groups was compared by independent Student's t-test. To find out potential risk factor (s), the variable (s) found significant in univariate analysis were further subjected to multivariate logistic regression analysis. The association of potential risk factors with patients survival (3-year overall survival) was done by Kaplan-Meier survival curve analysis using Log-rank test. A 2-tailed (a = 2) P < 0.05 was considered statistically significant. Results: Out of the 63 response parameters, seven were found highly significant on multivariate analysis. The mean (± SD) age was 56.74 ± 10.76 years, the proportions of males were higher than females, mostly illiterate and lower income group. Among dietary characteristics, snuff was highest (OR = 3.86, 95% CI = 2.46-6.08) followed by salt tea (OR = 2.53, 95% CI = 1.49-4.29), smoking (OR = 1.97, 95% CI = 1.18-3.30), sundried food (OR = 1.77, 95% CI = 1.10-2.85) and red chilly (OR = 1.76, 95% CI = 1.07-2.89). Probability of survival lowered significantly (P < 0.05 or P < 0.01 or P < 0.001) in those consuming tobacco in the form of snuff (Log-rank c 2 = 24.62, P = 0.000) and smoking (Log-rank c 2 = 5.20, P = 0.023) as compared to those who did not take these. Conclusions: The analysis finally established snuff (smokeless tobacco) as the most powerful risk factor of esophageal cancer in Jammu region, followed by the salt tea, smoking and the sundried food.

Prosthetic rehabilitation of an adolescent with hypohidrotic ectodermal dysplasia with partial anodontia: case report.

Ectodermal dysplasia is a hereditary syndrome characterized by dysplasia of tissues of ectodermal origin (hair, skin, nails, and teeth) and occasionally, dysplasia of mesodermally derived tissues. The triad of nail dystrophy (onychodysplasia), alopecia, or hypotrichosis (scanty, fine, light hair on the scalp and eyebrows) and palmoplantar hypohidrosis is usually accompanied by lack of sweat glands and partial or complete absence of primary and permanent dentition. Hypohidrotic ectodermal dysplasia usually has an X-linked inheritance and affects only males severely, while female heterozygotes show only minor defects. The clinical management of children with ectodermal dysplasia provides a unique opportunity for cooperative effort between the pedodontist and the prosthodontist. The following case report discusses the management of a young boy with hypohidrotic ectodermal dysplasia. Removable prostheses were employed in the treatment. The aim was to rehabilitate the adolescent prosthodontically and boost him psychologically.

One year trends in the gram-negative bacterial antibiotic susceptibility patterns in a medical intensive care unit in South India.

PURPOSE: To describe the changes in antibiotic susceptibility patterns of common intensive care unit pathogens with time from the medical intensive care unit of a tertiary care hospital. METHODS: A prospective observational study was conducted in the medical intensive care unit (MICU) of a 2100 bed tertiary care hospital in South India. All data regarding patient characteristics, disease characteristics, infective agents, identified along with their antibiotic sensitivity patterns and patient outcomes were prospectively recorded in MICU data base. Various bacterial pathogen antibiotic sensitivity patterns from August 2004 to May 2005 were prospectively documented. During this period 491 patients were admitted to the MICU. Data were analyzed using excel spreadsheets. RESULTS: Ceftazidime resistance reduced in Klebsiella spp. while cefotaxime resistance increased. In E. coli however, ceftazidime and cefotaxime resistance increased. Klebsiella resistance to cefotaxime and ceftazidime ranged from 25-50% and 14-91%, while E. coli resistance to these antibiotics ranged from 50-70% and 50 to 80% respectively. In Pseudomonas and the non-fermenting gram-negative bacteria (NFGNB) ceftazidime resistance decreased. Third generation cephalosporin resistance seemed to be reducing in the NFGNB, however, carbapenem resistance appeared to be increasing, possibly due to their increasing use. CONCLUSIONS: This study demonstrates the trend in antibiotic susceptibility pattern (AST) of common gram negative infections seen in intensive care units. It demonstrates the changes seen especially after a change in the protocol antibiotic. Changes in the AST patterns of Klebsiella, E. coli, Pseudomonas and non-fermenting gram negative bacteria were seen. The data on the changing antibiotic susceptibility trends we believe is an important pillar in our efforts at infection control especially in intensive care settings.

Surgical reconstruction for absence of sternum and pericardium in a newborn.

We report the surgical management of a 3 day old baby with absent sternum and pericardium with impending rupture of the skin cover. The repair described is innovative and may be used in place of primary repair by bringing the ribs together. Review of embryology and various modalities of closure are provided.

Seasonal fluctuations of dengue fever vector, Aedes aegypti (Diptera: Culicidae) in Delhi, India.

Studies on the seasonal fluctuation of Aedes aegypti were undertaken in different localities of Delhi, during 2000. The Aedes aegypti population was found to be prevalent in all the localities in Delhi. Water coolers and tires were found to be the preferred breeding habitats of Aedes mosquitos in the city. Aedes aegypti, being hygroscopic, showed a phenomenon of annual pulsation. It tends to move to mother foci in the central areas of the city, which are humid in the dry season, and spread out during the wet season. Out of 103,778 houses surveyed, 20,513 houses and 3,547 containers were reported positive for Aedes aegypti. The house container, and Breteau indices were very high during the post-monsoon season. The container indicies was very high (17.7%) in the defence area in September 2000. The container index in the areas of the Municipal Corporation of Delhi (MCD) and the New Delhi Municipal Committee (NDMC) were found to be high during the same period. The house index forAedes aegypti ranged from 0.1 to 7.4, 0.1 to 11.3, and 0.1 to 11.1 in the MCD, NDMC, and Defence areas, respectively.

Subtypes of Ischemic Stroke in a Metropolitan City of South India (One year data from a hospital based stroke registry).

Limited data exists on the subtypes of ischemic stroke from the Indian subcontinent. The present study was aimed to investigate the frequency, spectrum and risk factors of various subtypes of ischemic stroke in a south Indian referral center. The study was conducted on consecutive patients of ischemic stroke, fully investigated to determine the underlying mechanism and enrolled in the stroke registry of Nizam's Institute of Medical Sciences between Ist February 2000 to 31st January 2001. There were 282 men and 110 women (mean age 54 years; range 2-97 years). Of all ischemic stroke patients, 41%, 18%, 10%, 4%, and 27% were classified as large-artery atherosclerosis, lacunae, cardioembolism, other determined etiology and undetermined etiology respectively. The most notable difference of this registry from western registries was the predominance of intracranial rather than extracranial location of the large artery atherosclerosis. Hypertension, diabetes and smoking were the common risk factors among all the subtypes. Coronary artery disease and rheumatic heart disease were responsible for most of the cardioembolic strokes.

Critical care management of acute stroke.

Treatment of acute stroke in an intensive care unit improves the outcome by reducing the mortality and morbidity. In addition to the primary neurological condition that affects the outcome, the secondary complications and associated co-morbid conditions also influence the outcome. It is essential to monitor and maintain the general physiological conditions in the neurologically sick patients. Maintenance of a clear airway is important to prevent hypoxic insult to the brain in stroke patients whose airway is compromised. Mean arterial blood pressure should be maintained > 110mm of Hg. The choice of antihypertensive is labetolol. Drugs causing raised intracranial pressure (ICP) should be generally avoided. Cardiac arrhythmias should be monitored and treated. Treatment of ICP by osmotic agents is the usual practice. Hyperventilation is reserved for patients who are coning and is only a temporizing measure prior to surgery. Hemicraniectomy in malignant middle cerebral artery territory infarcts reduces the mortality significantly. Insulin therapy may be required to maintain a normoglycemic state for preserving the functionally impaired neurons. Infections should be treated by appropriate antibiotics. Care should be taken to prevent aspiration, and deep vein thrombosis. Intravenous thrombolysis for the eligible candidates and Aspirin for the rest is the recommended therapy for acute ischemic stroke.

Cognitive rehabilitation in stroke : therapy and techniques.

Cognitive deficits following stroke are common and interfere with recovery. Cognitive rehabilitation incorporates principles of restorative neurology and neuropsychology and has now become an integral component of stroke rehabilitation strategy. The process of cognitive rehabilitation involves assessment of cognitive functions, identification of specific areas of impairment, goal setting and institution of appropriate rehabilitation techniques. Currently, there is enough evidence supporting the effectiveness of cognitive rehabilitation of neglect and aphasia in stroke. Apraxia, inattention and executive dysfunction may also improve with specific intervention. Compensatory strategies are the mainstay of managing patients with memory disturbances. In addition to specific cognitive deficits, physical and emotional disturbances as well as social support affect functional recovery. Comprehensive and holistic cognitive rehabilitation programs are necessary to improve daily life function in stroke patients.

Myelopathy during treatment of autoimmune hemolytic anaemia.

A specific neurological syndrome resembling, subacute combined degeneration of spinal cord, has been observed to occur during treatment of autoimmune hemolytic anaemia (AHA). We propose that folate administration can precipitate this neurological syndrome in patients with subclinical deficiency of vitamin B12, a phenomenon more likely to occur in tropical countries. Such cases have not been previously reported.

Sleep apnoea syndromes : clinical and polysomnographic study.

Sleep apnoea syndromes have been known since long, and frequently the presenting symptoms are neurological in nature. However, these disorders have not been systematically studied and reported in the Indian literature. Out of 12,000 neurology outpatients seen by authors in 2 years, 60 had primary sleep disturbances. All these 60 patients underwent clinical evaluation and video EEG polysomnography. In 8 out of 60 (13%) patients, sleep apnoea was documented. Five patients had obstructive sleep apnoea, 3 had mixed sleep apnoea and none had pure central sleep apnoea syndrome. Three-fourths of the patients were obese (mean weight 82kg) middle aged males (mean age 46.3 years). The main symptoms encountered were excessive daytime somnolence and snoring. The symptom severity was found to correspond directly with the duration of symptoms as well as obesity. Sleep apnoea syndromes must be seriously considered and documented in all patients complaining of excessive daytime somnolence.

Carotid artery disease and ocular vascular disorders.

The carotid artery supplies blood to the structures of the eye; therefore a variety of ocular vascular disturbances can occur secondary to abnormalities in the parent vessels. The ocular manifestations of chronic carotid artery abnormalities may often be subtle and overlooked or they may be acute, where the contribution of the carotid arteries in the aetiology may not be apparent on routine clinical evaluation. Ophthalmologists and neurologists working independently may not recognise the effects of carotid artery disturbances on the eye and missed diagnoses are not infrequent. In our experience of almost a decade in a tertiarycare referral hospital, we have not recorded any patient referred to us from a primary referring physician (either ophthlamologist or neurologist) as a case of ocular manifestation of a carotid artery disease. This brief review highlights the important clinical features, diagnostic modalities and therapeutic options in this group of disorders.

Primary fibrosarcoma of brain.

This is a case presentation of a young patient with an intracranial space-occupying lesion following multiple episodes of generalised tonic clonic seizures for the last 20 years. Such a long latency period between the onset of fits and the discovery of an intracranial lesion is highly unusual in malignant brain tumours. This lesion was excised completely and proved to be a primary lesion of the brain - fibrosarcoma. These rare tumours of mesenchymal origin in the central nervous system are very rare.

Clinical features of cardioembolic strokes identified by transesophageal echocardiography.

Clinical and CT scan features predictive of a cardiac source of embolism (CSOE) are helpful in planning appropriate investigations in ischaemic strokes. The currently described predictors of CSOE were determined before the availability of trans esophageal echocardiography (TEE). After the advent of TEE, many new CSOE were discovered. The present study was planned to investigate if the previously described predictors of CSOE are also valid for patients with CSOE detectable only with TEE (TEE-detected CSOE). From 1992-1995, 485 consecutive patients of ischemic stroke were enrolled in the Maryland Stroke Data Bank (MSDB). Patients with CSOE identified only by TEE and not by clinical, electrocardiographic or transthoracic echocardiographic (TTE) examination were compared to patients with a CSOE with respect to the features of the history, neurologic examination and CT scan. Of 485 patients with cerebral infarction, 132 (27%) patients had CSOE. In 21/132 (16%), diagnosis of high risk CSOE could be established only by TEE. The most discriminating clinical findings in TEE-detected CSOE patients were visual field deficit (OR 2.9; 95% CI, 1.1-7.4) and neglect (OR 3.4; 95% CI,1.2-9.3). Less strong associations were also found with other clinical features described previously for CSOE. No significant differences were found for features of the initial CT scan. In summary, presence of visual field defect and hemineglect may suggest a higher likelihood of finding a CSOE by TEE, even if the clinical cardiac examination and TTE are normal.

Spinal extradural abscess following local steroid injection.

A case of spinal extradural abscess following local steroid injection for back ache, in a 26 years old male is described. The abscess presented as a swelling externally. MRI localised the lesion to L2-S1 segments. Mechanism of such an infection has been discussed.