Analysis of surgical outcome and review of literature of schwannomas arising from the extremities

Background: Schwannoma is a benign peripheral nerve sheath tumour derived from Schwann cells. Also known as Neurilemoma, it can affect any nerve in the body. They usually present as a painless swelling or paresthesia over the sensory distribution of the affected nerve. Although it is classically described that schwannomas are well encapsulated and can be completely enucleated during excision, many of them have fascicular involvement and could not be completely shelled out. The aim of this work is to present our experience in operative management of schwannomas located in extremities.Methods: Authors conducted a retrospective review for 18 adult patients with schwannoma, from June 2012 to June 2018.  There were 10 men and 8 women, ranging from 20 to 68 years of age, with a mean age of 46 years old. All patients had excision done for the tumour and histopathological examination confirmed schwannoma. All patients were preoperatively evaluated both clinically and radiologically. FNAC was also done to confirm the origin of the swelling.Results: The mean follow up period has been 2 years. Complete excision with preservation of nerve was done in all cases except for one case in which nerve graft was used.Conclusions: Use of preoperative MRI, magnification and good surgical technique will help to enucleate the tumour completely without any collateral damage or recurrence. The possibility and option of nerve graft should be discussed with patients prior to schwannoma excision, so that nerve grafting could be directly proceeded with patient consent in case there is fascicular involvement of tumour found intraoperatively.

Adult glomerular diseases in east zone and zonal prevalence in India: an Omnium Gatherum

Background: India is a vast country with four geographical zones. Zonal heterogeneity amongst prevalent adult glomerular diseases is expected and has not been analysed in past studies.Methods: We conducted clinico-histological correlation of 290 kidney biopsies for adult glomerular diseases (GD) at tertiary teaching hospital in Eastern India between January 2013 and December 2015 and compared our data with biopsy data from other geographical zones in India to evaluate zonal variability (intra/inter) of adult glomerular diseases.Results: Males dominated all clinical syndromes except subnephrotic proteinuria (SbNP). IgA Nephropathy (IgAN, 41.1%) and Focal Segmental glomerulosclerosis (FSGS, 17.3%) were prevalent primary GD whereas Lupus nephritis (LN, 52.2%) and diabetic nephropathy (DN, 23.9%) were prevalent secondary GD. IgAN (44.4%) and LN (33.2%) dominated SbNP group whereas FSGS (30.2%) and Membranous nephropathy (MGN, 22.3%) dominated nephrotics. Mean eGFR (CKD-EPI) amongst EyRD and RPRF was 39.6±12.9 and 6.2±2.9 ml/min/1.73m2 respectively. In contrast, biopsies from East India showed MCD prevalence, followed by FSGS. Kidney biopsy data from West India showed MCD prevalence whereas Northern India and South India studies showed FSGS and MCD prevalence, but later data showed an IgAN emergence, as in our data.Conclusions: There is considerable heterogeneity in prevalent adult glomerular diseases in different geographical zones (inter and intra) in India. FSGS and MCD were the most prevalent in all zones. Our study showed IgAN prevalence in East Zone, similar to South India. Reason was, increased number of kidney biopsies in EyRD (eGFR 30-60 ml/min) and subnephrotic proteinuria.

Assessment of Knowledge and Attitude among Dental Care Workers towards Patients Affected with HIV/AIDS in a Private Dental College in India.

Aims: HIV and AIDS is a major public health challenge and India alone accounts for 2.5 million of the Asian sub continent 2009 estimated 4.89 million people were living with HIV/AIDS. The oral manifestations are often among the first symptoms of HIV/AIDS and thus can be useful in early detection of the disease. Therefore it is professional and ethical responsibility of the dentist to provide treatment to such patients. Therefore, the present study was carried out to assess the knowledge and attitude of dentist and Dental auxiliary staff in a private Dental College. Study Design: Descriptive cross sectional study. Methods: A descriptive cross sectional study was carried out for one month among the dentists and dental auxiliary staff. Information, thus obtained from above questionnaire was entered in MS excel spreadsheet and the percentage was calculated. Results: Fifteen (30%) of the dentists had excellent knowledge regarding modes of transmission of HIV/AIDS while 18 (36%) of nurses had extremely poor knowledge. Thirty (60%) of the dentists had a positive attitude towards HIV/AIDS patients while 25 (50%) of the dental nursing staff and 9 (45%) of the Lab Technicians had negative attitudes towards them. Recommendation: Regular continuous medical examination of dental health workers is advised for the up gradation of their knowledge and building of strong positive attitudes towards HIV/AID patients.

Structural studies on a non-toxic homologue of type II RIPs from bitter gourd: Molecular basis of non-toxicity, conformational selection and glycan structure.

The structures of nine independent crystals of bitter gourd seed lectin (BGSL), a non-toxic homologue of type II RIPs, and its sugar complexes have been determined. The four-chain, two-fold symmetric, protein is made up of two identical two-chain modules, each consisting of a catalytic chain and a lectin chain, connected by a disulphide bridge. The lectin chain is made up of two domains. Each domain carries a carbohydrate binding site in type II RIPs of known structure. BGSL has a sugar binding site only on one domain, thus impairing its interaction at the cell surface. The adenine binding site in the catalytic chain is defective. Thus, defects in sugar binding as well as adenine binding appear to contribute to the non-toxicity of the lectin. The plasticity of the molecule is mainly caused by the presence of two possible well defined conformations of a surface loop in the lectin chain. One of them is chosen in the sugar complexes, in a case of conformational selection, as the chosen conformation facilitates an additional interaction with the sugar, involving an arginyl residue in the loop. The N-glycosylation of the lectin involves a plant-specific glycan while that in toxic type II RIPs of known structure involves a glycan which is animal as well as plant specific.

Unique morphology of intratubular light chain casts in multiple myeloma: The amyloid cast nephropathy.

Cast nephropathy is the most frequent pattern of renal involvement in multiple myeloma characterized by presence of tubular casts with characteristic morphology that are composed of monotypic (either kappa or lambda) light chains as seen by immunofl uorescence microscopy. Rarely these casts may show evidence of amyloidogenesis and assume a unique morphology, which needs to be appreciated for arriving at accurate diagnosis. We present the case of an elderly male presenting with features of acute kidney injury and detected with extensive inspissation of intratubular casts with lambda light chain restriction and a unique morphology with spiculated congophilic periphery. Further investigations confi rmed the presence of systemic myeloma. Presence of intratubular amyloid casts is a rare occurrence which needs to be recognized by the pathologist and forms a vital element in timely diagnosis of the systemic disease which often presents with renal involvement.

Autologous Bone Marrow Stem Cell Therapy Shows Functional Improvement in Hemorrhagic Stroke.

In hemorrhagic stroke, damage to the brain tissue is inevitable and no effective treatment for functional improvement is currently available except neurorehabilitation. Stem cell therapy is a rapidly growing field and has recently opened new avenues for brain repair strategies. We present a case study of a 69-year-old female treated with stem cell therapy for right-sided hemiplegia caused due to left thalamic hemorrhagic stroke. Inspite of regular physiotherapy, the patient had constant residual neurodeficit, one year after the stroke, which was severely incapacitating. In view of the same, the patient was given intrathecal autologous bone marrow derived stem cell therapy as part of the neuroregeneration and rehabilitation therapy (NRRT) along with rehabilitation. After the therapy, patient showed functional as well as neurological improvements (cognition and motor strength) without any side effects. There is accumulating experimental data showing the benefits of cell transplantation on functional recovery after hemorrhagic stroke. This case study supports the concept of neuroregeneration with bone marrow stem cells as a novel strategy having great therapeutic potential. However, large clinical studies are needed to further investigate autologous bone marrow stem cell therapy in addition to neurorehabilitation for treating the disability in hemorrhagic stroke.

Retrospective study of chikungunya outbreak in urban areas of India.

Background & objectives: A retrospective study on chikungunya outbreak in India in five States viz. Delhi, Madhya Pradesh, Orissa, Maharashtra and Kerala was conducted in 2007-2008 to know the distribution and determinants of chikungunya fever outbreak in India. Methods: On the basis of high and low incidence of chikungunya fever, two districts from each State and two wards from the selected district were taken for random selection of 1000 households from 10 districts and 5 States. Semi-structured questionnaires were administered to individuals, patients, qualified health professionals and to stakeholders for collecting information. Results: The educational background and occupation of the respondents showed variations across the study States. Only in high incidence ward of Maharashtra, water storage period for 3-6 days and emptying, drying of water containers on weekly basis was noted. The study through knowledge, attitude, belief, practice (KABP) obtained individual's perception of chikungunya fever, its prevention and control. Patients’ expenditure on treatment was mainly recorded less than Rs 500 across study States. Health facility survey obtained an overview of the capacity of local health facilities. Stakeholders’ perception regarding chikungunya fever was also noted. Interpretation & Conclusions: The study revealed differences in awareness of chikungunya, cause of the disease, vector responsible, mode of transmission, biting time and elimination of breeding of mosquitoes statistically significant among high and low incidence wards of all the States. Expenditure on treatment was independent of economically active status and loss of man-days across all the States. Education and occupation did not have any relation with emptying/drying of water containers in high incidence wards. Strengthening of surveillance, information, education and communication (IEC) activities along with case management facilities may be provided by the State health department for prevention of chikungunya outbreaks in future. Stakeholders should be more involved in outbreak management and future planning.

Quaternary association in β-prism I fold plant lectins: Insights from X-ray crystallography, modelling and molecular dynamics.

Dimeric banana lectin and calsepa, tetrameric artocarpin and octameric heltuba are mannose-specific β-prism I fold lectins of nearly the same tertiary structure. MD simulations on individual subunits and the oligomers provide insights into the changes in the structure brought about in the protomers on oligomerization, including swapping of the N-terminal stretch in one instance. The regions that undergo changes also tend to exhibit dynamic flexibility during MD simulations. The internal symmetries of individual oligomers are substantially retained during the calculations. Energy minimization and simulations were also carried out on models using all possible oligomers by employing the four different protomers. The unique dimerization pattern observed in calsepa could be traced to unique substitutions in a peptide stretch involved in dimerization. The impossibility of a specific mode of oligomerization involving a particular protomer is often expressed in terms of unacceptable steric contacts or dissociation of the oligomer during simulations. The calculations also led to a rationale for the observation of a heltuba tetramer in solution although the lectin exists as an octamer in the crystal, in addition to providing insights into relations among evolution, oligomerization and ligand binding.

Crescentic glomerulonephritis: A clinical and histomorphological analysis of 46 cases.

Background: Crescentic glomerulonephritis (CrGN), defined as crescents involving more than 50% of the glomeruli, includes pauci-immune, immune complex-mediated and anti-glomerular basement membrane disease. Objectives: The present study was aimed at evaluating the various clinical, biochemical and histological parameters in CrGN with respect to these categories and clinical outcome. Materials and Methods: Renal biopsies diagnosed as CrGN between Jan 2008 and Feb 2010 were included. Clinical and laboratory parameters were retrieved along with the therapeutic approach and clinical outcome, wherever available. Renal biopsy slides were evaluated for various glomerular, tubulo-interstitial and arteriolar features. Appropriate statistical tests were applied for significance. Results: A total of 46 cases of CrGN were included; majority (71.7%) of cases were pauci-immune (PI) while 28.3% were immune complex-mediated (IC). Among clinical features, gender ratio was significantly different between PI and IC groups (P = 0.006). The various histological parameters, including proportion of cellular crescents, tuft necrosis and Bowman's capsule rupture, were similar in both the groups. Four unusual associations, including idiopathic membranoproliferative glomerulonephritis (MPGN), multibacillary leprosy, acute lymphoblastic leukemia and C1q nephropathy were detected. Adequate follow-up information was available in 21 (46%) of the patients. Of these, 11 (52.4%) were dialysis-dependent at the last follow-up. Adult patients required renal replacement therapy more frequently than pediatric cases (P = 0.05). Presence of arteriolar fibrinoid necrosis also showed association with poor clinical outcome (P = 0.05). Conclusions: Crescentic glomerulonephritis remains one of the main causes of acute renal failure with histological diagnosis. Immunohistologic examination is essential for accurate classification into one of the three categories. This condition should be considered in rare causal associations like leprosy or MPGN with renal failure, to allow for timely performed renal biopsy and appropriate aggressive therapy.

Calcineurin inhibitor toxicity in renal allografts: Morphologic clues from protocol biopsies.

Background: Calcineurin inhibitors (cyclosporine and tacrolimus) are important constituents of post renal transplant immunosuppression. However, renal toxicity limits their utility. Histological features of calcineurin inhibitor toxicity (CNIT) have been the subject of few studies using protocol biopsy samples, and consensus on diagnostic criteria is still evolving. Aims: To analyze the spectrum of histological changes in protocol renal allograft biopsies with evidence of CNIT and identify additional features that are likely to help the pathologist in arriving at a diagnosis. Materials and Methods: One hundred and forty protocol allograft biopsies performed at 1, 6 and 12 months post renal transplant were studied. The defining features of CNIT included: isometric vacuolization of proximal tubular cells, arteriolar hyalinosis with medial/peripheral nodules and striped pattern of tubular atrophy/interstitial fibrosis. Other features such as global glomerulosclerosis, vacuolization of smooth muscle cells of arterioles, tubular microcalcinosis, ischemic shrinkage of glomeruli and hyperplasia of juxtaglomerular apparatus (JGA) were also analyzed and graded semiquantitatively. Results: CNIT was seen in 17/140 protocol biopsies (12.1%). In addition to the diagnostic criteria, arteriolar hyalinosis, smooth muscle cell vacuolization of arterioles and hyperplasia of JGA were found to be useful indicators of CNIT. Conclusions: There is a relatively high incidence of CNIT in protocol allograft biopsies. A critical analysis of renal biopsy in adequate number of serial step sections to identify these features is mandatory, as many of these features are subtle and are likely to be missed if not specifically sought.

Primary neuroendocrine carcinoma of thymus: A rare cause of Cushing's syndrome.

Thymomas constitute majority of the thymic neoplasms. In contrast, neuroendocrine tumors (carcinoid and neuroendocrine carcinoma) of thymus are extremely rare. Thymic carcinoids may present rarely with Cushing's syndrome due to the ectopic production of adrenocorticotropic hormone (ACTH). Recognition of this association is imperative for appropriate management of patients. We describe three cases of rare atypical carcinoid tumor (neuroendocrine carcinoma) of the thymus. Case 1, of a 26-year-old man presenting with Cushing's syndrome, case 2 - a 23-year-old female with Cushingoid features, and Case 3 - a 39-year-old man complaining of progressively worsening dyspnea. Computed tomography (CT) scans of chest in all three patients revealed anterior mediastinal mass. Excision of tumors and histological examination of the three tumors showed a carcinoid tumor with nuclear pleomorphism, increased mitotic activity and focal necrosis. The features suggested a diagnosis of atypical carcinoid tumor in all the three cases. The tumor cells in Cases 1 and 2 showed focal immunohistochemical staining for ACTH. Atypical carcinoid (neuroendocrine carcinoma, well-differentiated and moderately-differentiated) of the thymus is a rare thymic tumor which carries a worse prognosis compared to thymoma and requires aggressive therapy. Hence, an accurate diagnosis is essential.

Invasive papillary carcinoma of male breast.

Breast carcinoma is uncommon in males and constitutes less than one per cent of all cancers in men. Invasive papillary carcinoma is a rare morphological type of breast cancer. Since papillary carcinoma has a favorable prognosis as compared to other histopathological subtypes, an accurate diagnosis is essential. We report two cases of this rare histological type of male breast cancer. A 62-year-old man presented with a lump in the central quadrant of right breast and underwent simple mastectomy. Histological examination showed features of invasive papillary carcinoma. The other case was of an 81-year-old male patient with a subareolar mass in the right breast. Wide local excision of the lump showed features of an intracystic invasive papillary carcinoma. The patient subsequently underwent simple mastectomy, however, no residual tumor was found in the resection specimen. Both the patients were free of disease at one year of follow-up. Invasive papillary carcinoma is an uncommon morphological type of breast cancer in males. The intracystic variant of papillary carcinoma is extremely unusual and may be missed on cytological examination. A thorough sampling is essential for an accurate diagnosis of invasion in these cases.

Application of autologous bone marrow stem cells in giant axonal neuropathy.

Giant axonal neuropathy is a rare disorder of autosomal recessive inheritance, morphologically characterized by accumulation of neurofilaments in enlargements of preterminal regions of central and peripheral axons. We present a 7-year-old girl with thick and tightly curled lackluster hair suffering from giant axonal neuropathy. The diagnosis was confirmed on the brain MRI which showed white matter abnormalities in the anterior and posterior periventricular regions as well as the cerebellar white matter. In view of the same, the patient was given intrathecal autologous bone marrow-derived stem cell therapy as part of the neuroregenerative rehabilitation therapy protocol. The patient showed functional improvements in her disability after receiving the therapy. A detailed case report is presented here with.

Diffuse lipomatosis of the thyroid gland: a pathologic curiosity.

We report the case of a 45-year-old man who presented with a 4-year history of midline neck swelling associated with recent onset respiratory distress. Local examination showed a lobulated diffuse thyroid enlargement. A subtotal thyroidectomy was performed. The right lobe weighed 225 g and the left lobe weighed 130 g. Multiple sections from both the lobes revealed diffuse infiltration of the stroma by mature adipose tissue. There was no evidence of amyloid deposits or papillary carcinoma. A final diagnosis of diffuse lipomatosis of the thyroid gland was rendered. Lipomatosis or adenolipomatosis of the thyroid gland is an extremely rare entity. The various differential diagnoses of fat in the thyroid include benign entities like amyloid goiter, adenolipoma, lymphocytic thyroiditis, intrathyroid thymic or parathyroid lipoma and malignant tumors like liposarcoma and encapsulated papillary carcinoma. Adequate clinical details and a thorough histopathological examination are mandatory for diagnosis.

Primary hyperoxaluria type 1 with a novel mutation.

Primary hyperoxaluria type 1 [PH1] is an autosomal recessive disorder caused by a deficiency of alanine-glyoxylate aminotransferase AGT, which is encoded by the AGXT gene. We report an Indian family with two affected siblings having a novel mutation in the AGXT gene inherited from the parents. The index case progressed to end stage renal disease at 5 months of age. His 4 month old sibling is presently under follow up with preserved renal function.