Efficacy of Intravenous Lignocaine 2% Versus Oropharyngeal Topical 10% Xylocaine Spray Before Induction of Anaesthesia in Attenuating the Pressor Response to Direct Lryngoscopy and Endotracheal Intubation

The current Study was done to compare the efficacy of intravenous lignocaine 2% versus oropharyngeal topical 10% xylocaine spray before induction of anaesthesia in attenuating the pressor response to direct laryngoscopy and endotracheal intubation. A total of 60 patients were divided randomly into two groups of 30 patients each. Group I received intravenous lignocaine 2% @ 1.5 mg/kg. Group II received topical 10% xylocaine spray @ 1.5 mg/kg body weight just before induction of anaesthesia. All hemodynamic parameters were measured immediately after laryngoscopy and intubation and at 1, 3, 5 minutes after laryngoscopy and intubation in both the groups. Mean values of haemodynamic parameters in Group I increased after intubation and then started declining but did not reach the baseline even at 5 minutes. In Group II all mean values of haemodynamic parameters increased after intubation and then started declining to almost baseline at 5 minutes. The differences in mean values of haemodynamic between the two groups immediately after intubation and at 1, 3 and 5 minutes thereafter were statistically highly significant (p<0.001). Oropharyngeal topical xylocaine 10% when applied before induction of general anaesthesia is more effective method for attenuating the pressor response to direct laryngoscopy and endotracheal intubation when compared to intravenous lignocaine 2%

Metaplastic Carcinoma of Breast - Squamous Variant.

Metaplastic carcinoma of breast is a rare neoplasm. Although it is a tumor of ductal type, the predominant component may have appearance other than the glandular pattern and usually runs an aggressive course.

Primary Pleomorphic Adenoma of External Auditory Canal.

Primary pleomorhic adenoma (Chondroid syringoma) arising from the external auditory canal is very rare and because of the unremarkable clinical presentation, the diagnosis is often made after microscopic examination. We reviewed the worldwide literature and found that only forty one cases have been previously reported. In this report, we describe one such unusual case. The treatment of choice is wide local excision without esthetic or functional disruption of surrounding structures, followed by careful long-term post-operative observation of the clinical course.

Lupus Vulgaris of external nose with septal perforation - A rarity in antibiotic era.

Lupus vulgaris (LV) is the commonest morphological variant of cutaneous tuberculosis. Case of LV of external nose extending to internal nose causing septal perforation is documented here. Histopathology of biopsy taken confirmed the diagnosis of LV. Patient responded well to Anti-tubercular therapy (ATT).

Giant retropharyngeal abscess secondary to tubercular spondylitis.

Chronic retropharyngeal abscess secondary to tubercular spondilitis is a rare phenomenon. Anatomical location of this abscess makes it a life threatening condition requiring prompt diagnosis and treatment thus preventing morbidity and mortality. Authors are documenting a case of tubercular spondylitis of cervical region in a 12-year old male child with huge retropharyngeal abscess extending to superior mediastinum. Clinically, patient had respiratory distress but no neurological deficit. USG (Ultrasonography) guided aspirate of abscess sent for microscopy and culture showed acid fast bacilli. Multiple USG guided aspirations under antibiotic and antitubercular cover (Category I) were done. Patient is doing well at three month follow-up.

The 2006 Dengue Outbreak in Delhi, India.

Dengue is a worldwide condition spread throughout the tropical and subtropical zones between 30 degrees north and 40 degrees south. It is endemic in South East Asia, the pacific, East and West Africa, the Caribbean and the Americas. Dengue outbreaks are occurring almost every three yearly in Delhi for the last 12 years. The latest outbreak was in the year 2006, which started late in August, peaked in the month of October and lasted till late November. We describe here the clinical, hematological and biochemical data of 70 patients of dengue fever diagnosed as per WHO criteria in Lok Nayak Hospital, New Delhi during this outbreak. Hematological parameters were estimated by automated counter and dengue serology was done by capture ELISA technique. The results displayed widespread effect of dengue fever on hematological and biochemical profile. Some of our patients also had atypical dengue manifestations. These results suggest that dengue fever is a major public health problem which can lead to significant morbidity and can even be fatal at times. All efforts should be made to prevent it.

Branchio-oto-renal syndrome.

Branchio-oto-renal syndrome (Melnick-Fraser syndrome) is a rare autosomal dominant disorder characterized by syndromic association of branchial cysts or fistulae along with external, middle & inner ear malformations and renal anomalies. Authors are reporting a 19 year male patient, who presented with profound deafness & low set "lop-ear" with right sided preauricular pit. USG abdomen revealed agenesis of the left kidney.

Multi-minicore disease: a rare form of myopathy.

BACKGROUND: Multi-minicore disease is a rare form of myopathy characterized by slowly progressive or nonprogressive muscle weakness and characteristic multiple cores within the muscle fibers. To the best of our knowledge, this is first documentation of the clinicopathological features of this rare entity from India. MATERIALS AND METHODS: A ll cases of multi-minicore disease diagnosed in our laboratory were retrieved. Clinical and pathological features were reviewed. RESULT: During a period of two years (January 2004 to December 2005), we received 985 muscle biopsies for various reasons. Of which, 15 were diagnosed as myopathies and four of which were of multi-minicore disease. Thus, multi-minicore disease comprises 0.40% of all muscle diseases and 26.6% of all myopathies. All were male and presented in early childhood (first decade of life) with generalized hypotonia and muscle weakness. All of them had dysmorphic facies and three had high arched palate. CPK levels were normal and EMG was myopathic except in one patient. Microscopic examination revealed minimal changes with Type I fibers' predominance but characteristic multiple cores in the myofibers. Ultrastructural examination showed both structured and unstructured cores. CONCLUSIONS: Multi-minicore disease, although a rare form of myopathies, should be suspected in children who present with generalized hypotonia and slowly progressive muscle weakness along with dysmorphic facies.

Nemaline rod myopathy: a rare form of myopathy.

Nemaline rod myopathy (NM) is a rare form of congenital myopathy characterized by slowly progressive or nonprogressive muscle weakness and pathognomonic rod-like structures within the muscle fibers. To the best of our knowledge, this is first documentation of the clinicopathological features of this rare entity from India. All cases of NM diagnosed in our laboratory were retrieved. Clinical and pathological features were reviewed. During a period of 1.5 years (Jan 2004 to June 2005), we received 750 muscle biopsies for various reasons. Of which, 15 were diagnosed as congenital myopathies and four as nemaline rod myopathies. Thus, NM comprises 0.53% of all muscle diseases and 22.6% of all congenital myopathies. All of them presented in childhood (first five years of life) with generalized hypotonia, feeding problems, repeated respiratory infections and muscle weakness. Both males and females were equally affected. The CPK levels were normal and EMG was myopathic. Microscopic examination revealed minimal changes but characteristic red-colored material was seen on modified Gomori trichrome staining which was immunopositive to alpha actinin. Ultrastructural examination confirmed this material to be nemaline rods. NM, although a rare form of congenital myopathies, should be suspected in children who present with generalized hypotonia, repeated chest infections and slowly progressive muscle weakness. This report highlights the importance of histochemistry and ultrastructural examination in the diagnosis of this entity, in the absence of the availability of methodology for genetic studies.

Angiotensin converting enzyme gene polymorphism in Indian children with steroid sensitive nephrotic syndrome.

CONTEXT: Nephrotic syndrome is one of the commonest renal problem encountered in children. It is difficult to predict at onset, the clinical course in terms of steroid responsiveness or resistance. Angiotensin converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism has been studied as a predictor of clinical course in common multi-factorial diseases including focal segmental glomerulosclerosis. There is no study available from our country till date to find out any correlation of the steroid response in idiopathic nephrotic syndrome and ACE gene polymorphism. AIM: To study distribution and correlation of ACE gene I/D polymorphism in idiopathic steroid sensitive nephrotic syndrome. SETTINGS & DESIGN: Case-control retrospective study. SUBJECTS & METHODS: We studied ACE gene polymorphism in 90 consecutive patients (82% males, 18% females) with steroid sensitive idiopathic nephrotic syndrome and 300 normal controls (NC). STATISTICAL ANALYSIS: Chi-square test and Fisher's exact test (for cases with insufficient expected cell frequencies). RESULTS: The mean age of onset was 5.3 +/- 4 years. Steroid sensitive (SS) patients showed II (SS-48%, NC-26%) genotype was more frequent than normal controls (p=0.002). There was no significant difference in genotype frequencies among steroid SS subgroups. CONCLUSIONS: In our study II genotype was more frequent in steroid sensitive nephrotic syndrome children in comparison to normal controls. Further functional studies with large number of children are required to investigate the role of II genotype in steroid sensitive nephrotic syndrome. Comparison of the genotypic frequency with steroid resistant patients may provide information, which might be useful in clinical practice.

Sarcoglycanopathies: a clinicopathological study of 13 cases [corrected]

BACKGROUND: Limb girdle muscular dystrophy (LGMD) is a phenotypic expression of a heterogeneous group of diseases and sarcoglycanopathy is one of the causes of LGMD. There is only one study on sarcoglycanopathies in the Indian literature. No data is available from northern India. MATERIALS AND METHODS: All cases of muscular dystrophies, which were diagnosed in our laboratory in the last six years, were reviewed. Immunohistochemistry for various sarcoglycan proteins was done. Clinical features and pathological findings of the cases that were diagnosed as sarcoglycanopathies were reviewed. RESULTS: In the last 6 (1/2) years (1998-June 2004), we received 1435 muscle biopsies, of which 498 cases were of muscular dystrophies, and 13 cases were of sarcoglycanopathies (8 of gamma, 3 of alpha, 1 of both alpha and gamma, and 1 with absence of all four sarcoglycans). Sarcoglycanopathies comprised 2.6% of all muscular dystrophies, 11.8% of LGMD and 0.90% of all muscle diseases diagnosed in our laboratory. The mean age of onset was 7.2 years and the M:F ratio was 1.1:1. Most of them presented with difficulty in getting up, climbing stairs, calf hypertrophy and markedly raised CPK levels. Histological features were like dystrophinopathies. CONCLUSION: Sarcoglycanopathies are a relatively rare cause of LGMD and should be confirmed by immunohistochemistry as it will facilitate counseling and also prognostification. Although rare, in patients with muscle weakness, calves hypertrophy and raised CPK levels this possibility should be considered and needs to be differentiated from dystrophinopathies.

Congenital fiber type disproportion: a rare type of congenital myopathy: a report of four cases.

Congenital fiber type disproportion is a rare type of congenital myopathy which presents as hypotonia, delayed motor milestones and dysmorphic facies. During the past 2 years we received 449 muscle biopsies, of which 4 cases were diagnosed as congenital fiber type disproportion (CFTD). In addition to CFTD, one case also had centronuclear features. Three of them were females and one was a male child. Although rare, it should be considered in the differential diagnosis of childhood muscle diseases. Histochemical staining is necessary for the diagnosis of this entity.

Tubercular Osteomyelitis of nasal bones A rare entity.

A case of tubercular osteomyelitis of nasal bones in a 10 year old child is being reported because of its extremely rare occurrence.

Merosin negative congenital muscular dystrophy: a short report.

We report a rare case of an infant with congenital muscular dystrophy who presented at birth with marked generalized hypotonia and normal mental development. Creatinine phosphokinase (CPK) level was markedly raised; however no white matter abnormalities were detected by brain imaging techniques. Immunohistochemical staining for merosin (laminin alpha 2) was negative, thereby confirming merosin-deficient congenital muscular dystrophy.

Giant cell reparative granuloma of the base of the skull presenting as a parapharyngeal mass.

Giant cell reparative granuloma is an infrequent non-tumoral lesion affecting the maxillary and mandibular bones and only rarely, the cranial bones. We report a case of giant cell reparative granuloma of the base of the skull in a 12-year-old female who presented with a parapharyngeal mass. Computerized Tomography (CT) showed the intracranial extension and destruction of surrounding bones.

Groundnut as the commonest foreign body of tracheobronchial tree in winter in Northern India. An analysis of fourteen cases.

In Northern India, in an institution like ours which receives quite a number of patients from surrounding rural areas, we found groundnut as the most common foreign body of tracheobronchial tree in children particularly in winter months of October to January. This article attempts to address the potential hazard of groundnut inhalation in children. In Northern India, in an institution like ours which receives quite a number of patients from surrounding rural areas, we found groundnut as the most common foreign body of tracheobronchial tree in children particularly in winter months of October to January. This article attempts to address the potential hazard of groundnut inhalation in children.

An unusual case of neonatal anuria.

Neonatal anuria is not an uncommon problem in neonates. Here, we report an unusual case of neonatal anuria due to renal tubular dysgenesis, secondary to the use of angiotensin converting enzyme inhibitor (ACEI) during pregnancy. ACEI remains one of the most commonly used antihypertensive drug at present. A greater awareness needs to be created in the medical fraternity especially among pediatricians, gynecologists and internists that ACE inhibitors should not be prescribed during any trimester of pregnancy.