Clinical Profile of Sharp Syndrome: Is it rare in India?

Background: In 1972, Dr Sharp and colleagues described a new connective tissue disease, characterized by overlapping features of systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and polymyositis/ dermatomyositis (PM/DM) and by the presence of antibodies against the U1 small nuclear ribonucleoprotein autoantigen (U1 snRNP). This condition was termed mixed connective tissue disease (MCTD) and proposed as a distinct disease. Later, after observing the clinical evolution of MCTD patients, Sharp himself agreed that the original concept of MCTD had to be modified and that Internal organs were at risk for serious complications; patients were not always steroid responsive; prognosis was not always benign. Materials and methods: Patients in the age group of 15-50 years diagnosed to have connective tissue diseases were included. 8 patients in the age group of 15-50 admitted in Medicine department were taken and they were evaluated for the clinical profile of sharp syndrome by thorough clinical examination, routine laboratory tests and special investigations depending on the clinical profile. Results: 8 patients with connective tissue disease attending the medicine OPD were studied. Of these 8 patients were female patients. The median age of onset was 36 years, 8 patients met criteria by sharp and Alarcon-Segovia. The clinical features of patients at presentation are Raynaud抯 phenomena, Puffy fingers, esophagus dysmotility, skin rash, interstitial lung disease, arthritis, pulmonary hypertension, myositis, anemia. Conclusion: SHARP syndrome is a rare condition, as evidenced by the small series of cases reported to date. Diagnosis is based on clinical and paraclinical criteria. The evolution can be interspersed with various complications that can affect the short, medium and long-term prognosis

Management of internal hemorrhoids by Kshara karma: An educational case report.

A 66-year-old male patient came to the anorectal clinic, Outpatient department, AVT Institute for Advanced Research, Coimbatore, Tamil Nadu, with complaints of prolapsing pile mass during defecation and bleeding while passing stool. The case was diagnosed as “Raktarsha” - 11 & 7 ‘o’ clock position II degree internal hemorrhoids, deeply situated, projecting one and caused by pitta and rakta; with bleeding tendency. Kshara karma (application of caustic alkaline paste) intervention was done in this case to internal hemorrhoids under local anesthesia. The pile mass and per rectal bleeding resolved in 8 days and the patient was relieved from all symptoms within 21 days. No complications were reported after the procedure. The patient was followed up regularly from 2004 onward till date and proctoscopic examination did not reveal any evidence of recurrence of the hemorrhoids.

Synthesis and antimicrobial evaluation of some novel sulfur incorporated 7-substituted chromones..

We report herein the design and synthesis of five 2-(phenylthio) methylchromone (4a-d and 5), from 2-bromomethylchromones (3) which were obtained on refluxing 2-methylchromone with N-bromosuccinimide in carbon tetrachloride. The title compounds were characterised by spectral data (IR and NMR). All the compounds have been screened for antimicrobial activity.

Synthesis and antimicrobial evaluation of 3-thiocyanato-7-hydroxy-2-aryl-4h-chromen-4-one.

3-Thiocyanato-7-hydroxy-2-aryl-4H-chromen-4-one 5 a-e have been synthesized from 1- (2,4-dihydroxyphenyl)-2-chloroethanone 1; this reacted with thiocyanate anion afforded 1-(2,4– dihydroxyphenyl)–2-thiocyanatoethanone 2. Reaction of 2 with substituted benzoyl chlorides under modified Baker-Venkataraman conditions directly afforded the diketones 3 a-g, which on cyclization furnished 4 a-g, saponification of which afforded 3-thiocyanato-7-hydroxy-2-aryl-4H-chromen-4-one 5 ag. All of the synthesized compounds have been established by elemental analysis, IR and NMR spectral data. Compounds 5 a-g was screened for antibacterial activity.

Benign superior mediastinal syndrome due to intrathoracic tuberculosis in a child-association with endobronchial tuberculosis.

A 5-year-old boy presented with fever, cough, breathlessness and facial swelling. He was pale with distended veins over neck and chest, cervical and axillary lymphadenopathy and bilateral expiratory wheeze. Chest radiographs showed superior mediastinal widening. A computed tomography scan of the chest revealed mediastinal lymph nodes compressing superior vena cava and trachea. Bronchoscopy revealed nodular lesions in trachea and bronchi and compression of trachea. Broncho-alveolar lavage revealed acid fast bacilli. Diagnosis of superior mediastinal syndrome (SMS) secondary to tuberculosis was made and child was treated with antitubercular treatment along with oral prednisolone with good response.

Fiberoptic bronchoscopy in children an audit from a tertiary care center.

A retrospective review of 529 fiberoptic bronchoscopies was done with an aim to identify conditions where it will be most useful. The common indications were persistent/ recurrent pneumonia, persistent collapse, stridor and pulmonary hemorrhage. The diagnostic yield was maximum when it was performed to identify structural abnormalities and the yield was relatively poor in suspected drug resistant tuberculosis, and interstitial lung diseases. Serious complications were seen in children with pulmonary arterial hypertension. Fiberoptic bronchoscopy is an important tool for management of respiratory problems but should be performed with caution in children with pulmonary arterial hypertension.

Post obstructive pulmonary edema in a child who attempted suicidal hanging.

Postobstructive pulmonary edema occurs rarely in children. We describe here a child who attempted suicide by hanging and developed postobstructive pulmonary edema and was successfully managed. There was a rapid response to management with morphine and supportive care, enabling extubation by 30 hours of ventilatory support. The case highlights an unusual cause of postobstructive pulmonary edema.

Hepatopulmonary syndrome, an unusual cause of hypoxemia.

A six-year-old boy presented with cough, cyanosis and clubbing. Investigations revealed hypoxia (PaO2 53 mm Hg on room air) which was only partially correctable (PaO2 73 mm Hg) with 100% oxygen administered through a non re breathing face mask. Liver function tests showed elevated total bilirubin, and transaminases, liver biopsy confirmed chronic hepatitis and endoscopy showed grade three varices. A contrast enhanced echocardiography (bubble study) revealed pulmonary arterio-venous communication. A diagnosis of hepatopulmonary syndrome was made based on the triad of hypoxemia, liver disease and intra pulmonary vascular communications.

Toothbrush selection: a dilemma?

The toothbrush is a fundamental tool used for accomplishing plaque control. Various types of toothbrushes available in the market keep the buyer in a state of dilemma as to which one to choose, due to lack of information about the "quality" of it. This study has been designed to understand whether all the products available in the market adhere to the specifications laid down by the ADA, which is universally accepted in standardization of a tooth brush. 95% of the brushes surveyed contain less number of bristles per tuft than specified and a large percentage deviate from the standard prescribed value for their bristle diameter and other dimensions. Few brushes labeled as "Soft" were "Medium" and labeled "Medium" were "Hard". The study will bring to light the quality of toothbrushes available in India for a rationalised selection of appropriate toothbrush by the community at large.