Enhanced S-cone syndrome: Clinical spectrum in Indian population

Purpose: Enhanced S-cone syndrome (ESCS), a rare disorder, is often misdiagnosed as other forms of retinal degenerations, which have a poorer prognosis than ESCS. The aim of this study is to report the varied clinical features of ESCS and distinguish it from other similar disorders. Methods: We retrospectively scrutinized the records of patients with confirmed diagnosis of ESCS and analyzed the findings. Results: We included 14 patients (age range 4–39 years) who were confirmed to have ESCS according to pathognomonic electroretinography (ERG) showing reduced photopic, combined responses, and 30 Hz flicker with reduced L, M cone responses and supernormal S cone responses. The disease presented in the 1st decade with night blindness and was almost stationary or minimally progressive. Mid-peripheral fundus changes in form of nummular pigmentary alterations, yellow punctate lesions, and macular schisis were noted. The vision ranged from 6/6 to 6/36 with follow-up ranging from 1month to 22 years. Conclusion: ESCS shows varied clinical features ranging from unremarkable fundus to pigment clumping and atrophic lesions. It has good prognosis with patients mostly maintaining their vision. ERG is diagnostic. More awareness and knowledge about this entity can help to differentiate it from other forms of night blindness.

Endoscope-assisted harvest of autogenous fascia lata in frontalis suspension surgery: A minimally invasive approach revisited

Purpose: To report endoscope-assisted fascia lata harvest (EAFH) as a minimally-invasive technique for correction of severe blepharoptosis. Methods: This was a retrospective case series between January 2013 and April 2017. Medical records of all consecutive patients who underwent frontalis suspension by EAFH in the study period were reviewed and outcome was analyzed. Results: Fourteen patients (10 males) were included in the study. Mean age of the group was 18.14 + 17.03 years (range 4-65 years) and 11 patients had simple congenital blepharoptosis. Blepharophimosis syndrome was seen in 3 patients. Eleven patients had bilateral blepharoptosis. The mean preoperative and postoperative MRD1 was –1.60 ± 0.87 mm and +2.12 ± 1.37 mm respectively. Mean lengths of the incision and fascial harvest were 2.25 ± 0.43 cm and 13.0 ± 2.35 cm (range 10-17 cm) respectively. The median follow-up of patients was 4.57 + 4.03 months (range 1-15 months). Complications included a wound dehiscence in two patients and these were resutured. The donor sites healed well in all patients leaving a small thigh scar and none needed scar revision. Conclusion: EAFH is a promising minimally-invasive technique performed with a small incision and achieved adequate length of fascial harvest.

Gelastic syncope: An unusual condition.

Gelastic syncope or laughter-induced syncope is a rare disease often misdiagnosed as narcolepsy or cataplexy. We report a 54-year-old man with syncopal episodes. Each episode started after laughter, leading to light-headedness with blurring of vision and loss of consciousness for a few seconds. The episodes resolved spontaneously. The treatment of gelastic syncope is the same as that for neurally mediated syncope.

Hospitalisation due to infectious and parasitic diseases in District Civil Hospital, Belgaum, Karnataka.

To assess the burden of infectious and parasitic diseases on hospital services at District Civil Hospital (DCH) Belgaum, a retrospective study was carried out using discharge records concerning 8506 inpatients due to infectious and parasitic diseases among 95,655 patients admitted for all causes during the reference period 2000-2003. Out of the 21 causes of infectious and parasitic diseases, only 5 contributed maximally towards hospital admission. The most frequent cause was intestinal infections (44.0%) followed by tuberculosis (35.4%). 57.5% of these admissions were from the productive age group of 20-54 years. Tuberculosis is the most important disease in terms of hospital bed days (59.7%). Tuberculosis and intestinal infectious diseases represent more than three-fourth of the overall burden in terms of hospital bed days.

Long QT syndrome revisited.

Congenital Long QT Syndrome (cLQTS) is an inherited disease in children and adolescents who have structurally normal hearts but present with sudden death in a high proportion of untreated patients. More than 300 mutations have been identified in 7 LQT genes. Diagnosis still depends on ECG, clinical presentations and family history. Molecular genetic testing is useful to unravel borderline family members of LQT probands, but it continues to be a research tool at present. Beta blockers remain the mainstay of treatment. ICDs are highly effective in reducing SCD for high risk patients. Gene based therapy is still preliminary. Considerable thought is needed to address and treat the asymptomatic LQT family members. The main cause of Acquired LQTS is inhibition of Ikr current, usually by drugs. Care must be taken to avoid further exposure to QT prolonging drugs or conditions. Physicians need to be aware of the pharmacodynamic and pharmacokinetic interactions of various important drugs.

A descriptive clinical evaluation of arthroscopic synovectomy in rheumatoid knees: a prospective study.

OBJECTIVE: To evaluate the clinical outcome of arthroscopic synovectomy for persistent rheumatoid synovitis of the knee joints. DESIGN: Prospective clinical study. SETTING: Kasturba Medical College Hospital, Manipal. PATIENTS: Fifty two knee joints in 46 patients. MEASUREMENTS: The effect of the procedure and its influence in the progression of the disease process on knee joints were assessed in terms of reduction of pain, improvement in range of motion, improvement in functional activity and recurrence of synovitis with effusion. RESULTS: During the average follow up period of 5 years, the patients showed appreciable improvement (90% of knee joints) until 3 years of follow up. At the end of 5 years of follow up, it reduced to about 75%. CONCLUSIONS: Arthroscopic synovectomy along with medical treatment can control the disease process and preserve the knee joint function for up to 3 years.

Acute viral E hepatitis with chronic liver disease (autoimmune hepatitis).

A 36 years old male presented with anorexia, jaundice and ascites. He was suffering from acute viral E hepatitis. In view of ascites, he was investigated for associated asymptomatic chronic liver disease (CLD). The CLD was diagnosed as cirrhosis with autoimmune hepatitis and was treated with steroid with good response. He is maintaining good health with low dose steroid, on follow up for 1 year.

Cost Awareness and Containment : OPD Pharmaceutical Services of a Speciality Hospital

A major share of the hospital budget gets consumed in maintaining its pharmacy services. The cost consumption pattern of different group of medicines is directly related to the prescription load and prescription pattern. The Medical Officers and specialists of the hospital have got all important role in rational prescription in term of current therapeutics and saving on over prescription. A study carried out in a tertiary level super-speciality hospital indicated that the total cost of medicine per OPD day and cost of antibiotics work out to be Rs. 45291 and Rs. 11974 on an average, respectively. The average cost per prescription of OPD was up to Rs. 123.75.

A double-blind study of oral salbutamol supplement and repeat sputum smear microscopy in enhancing diagnosis of smear-negative pulmonary tuberculosis in south India.

Background: As routine culture facilities are not available in TB control programme in low income countries like India, there is an urgent need to improve the sensitivity of sputum microscopy, especially in diagnosis of smear negative pulmonary TB. Methodology: In a double blind placebo controlled study, the role of repeat sputum microscopy after antibiotics and oral salbutamol supplement in improving the diagnosis of smear negative TB suspects was investigated in an urban TB clinic. We undertook culture examinations for all study patients to find out proportions of TB cases in this series. Results: Of 206 enrolled, (101 salbutamol (S), 105 placebo (P) groups) 26 were positive by repeat sputum smear examination; similar in two groups (S 16, P 10, p = 0.25). In all, 40 (S 23, P 17) including 26 smear- positives, were culture -positive for M. tuberculosis. Conclusions: Two thirds of initially smear negative but culture positive TB patients were smear positive on repeat sputum examination. Thus, repeat sputum smear microscopy for TB suspects improved the diagnosis, nevertheless oral salbutamol therapy was not beneficial. In resource poor settings, repeat sputum smear microscopy after a trial of antibiotics, could significantly improve the diagnosis of smear-negative PTB patients.

Effects of alkaloidal extract of Phyllanthus niruri on HIV replication.

Phyllanthus niruri has been found to exhibit marked inhibitory effect on hepatitis B virus evident by its exhaustive utility in cases of chronic jaundice. However, till date, research has not been focused on identification and validation of active pharmacophores of Phyllanthus niruri responsible for the reported inhibitory effect of its aqueous extract on anti-human immunodeficiency virus. The present investigation examines the anti-HIV effects of the alkaloidal extract of Phyllanthus niruri in human cell lines. The inhibitory effect on HIV replication was monitored in terms of inhibition of virus induced cytopathogenecity in MT-4 cells. The alkaloidal extract of Phyllanthus niruri showed suppressing activity on strains of HIV-1 cells cultured on MT-4 cell lines. The CC50 for the extract was found to be 279.85 microgmL(-1) whereas the EC50 was found to be 20.98 microgmL(-1). Interestingly the Selectivity Index (SI) was found to be 13.34, which showed a clear selective toxicity of the extract for the viral cells. The alkaloidal extract of Phyllanthus niruri was thus found to exhibit sensitive inhibitory response on cytopathic effects induced by both the strains of human immunodeficiency virus on human MT-4 cells in the tested concentrations.

Effect of a non-hormonal feed additive, Cholymbi on growth, body composition and digestive enzyme activity of common carp, Cyprinus carpio.

Supplementation of pelleted feeds (30% crude protein) consisting of practical ingredients with 250, 375 and 500 mg Cholymbi/kg diet resulted significantly improved the growth of common carp, Cyprinus carpio. Highest final average weight was recorded with diet containing 500 mg Cholymbi/kg. Feeding Cholymbi to fish showed increased carcass protein and fat. Gut protease and hepatopancreas amylase activity were the highest with 500 mg Cholymbi/kg incorporated diet (T3). The results of the present study suggest that Cholymbi, a non-hormonal growth promoter could be effectively used in the diet of common carp. Further investigations are required to work out the optimal dosage required in the diet of common carp.

Comparison of efficacy of intravenous diltiazem and esmolol in terminating supraventricular tachycardia.

OBJECTIVE: Paroxysmal supraventricular tachycardia (PSVT) can be effectively terminated by the intravenous administration of adenosine or verapamil. However adenosine is expensive and injectable verapamil currently is scarcely available. While intravenous diltiazem has been shown to be useful for terminating PSVT, the efficacy of esmolol in this regard has not been evaluated previously. Hence these latter two drugs were studied for their efficacy in terminating PSVT. METHODS: A prospective, randomised, crossover study was undertaken in patients presenting with hemodynamically tolerated PSVT to the ICCU. While 50 patients had been planned for the trial, the study had to be prematurely terminated after 32 patients had been enrolled due to the marked superiority of diltiazem. Two sequential doses with a 5 minute interval of either drug were administered before crossover. Diltiazem was given in a dose of 0.25 mg/kg while the esmolol dose was 0.5 mg/kg. RESULTS: Diltiazem terminated PSVT in all the 16 patients in whom it was given as the first drug. The 12 patients who did not respond to esmolol were also effectively treated with diltiazem. Thus totally 28/28 patients responded to diltiazem while only 4/16 patients responded to esmolol (p < 0.001). Of the 28 patients who responded to diltiazem, in 13 patients the second bolus of diltiazem worked after the first one had failed. No significant adverse effects were seen. CONCLUSION: Intravenous diltiazem is highly effective and safe for terminating PSVT. When the first bolus is ineffective, the second bolus given after 5 minutes usually succeeds. Esmolol in the dose of 0.5 mg/kg has poor efficacy for terminating PSVT, even when 2 boluses are administered.

Growth response of African catfish, Clarias gariepinus (Burchell) to varied protein and lipid levels.

To study the growth response of African catfish (Clarias gariepinus) to the diets containing two protein (30 and 35%) and two lipid levels (12 and 18%) in a closed recirculatory system for 60 days, fish were fed to satiation twice daily. The experiment was carried out in triplicate groups. Fortnightly sampling of fish and water samples was carried out. The best result in terms of growth was obtained with the diet containing 34.62% protein and 12.12% lipid. The protein to lipid ratio was 18.57 in the diet that gave best percentage weight gain. However best survival, food conversion ratio and protein efficiency ratio were recorded in the diet containing 29.86% protein and 18.08% lipid.

Age at onset, gender distribution and intraarterial blood pressure changes in atrioventricular node-dependant tachycardias.

OBJECTIVE: Atrioventricular nodal reentrant tachycardia (AVNRT) and atrioventricular reentrant tachycardia (AVRT) utilising accessory pathways constitute the vast majority of paroxysmal supraventricular tachycardia (SVT). We studied the age at onset, the gender distribution and the intraarterial hemodynamics of these tachycardias. METHODS: The data of 224 patients who underwent electrophysiology study (EPS) and radiofrequency ablation was analysed. The age at onset of tachycardia was assessed by a careful history. The intraarterial BP was noted during sinus rhythm (SR), immediately after tachycardia onset (T0) and 15 seconds after the onset of tachycardia (T15). RESULTS: The age at onset of tachycardia was a decade later for AVNRT (48 +/- 10 years) than for AVRT (37 +/- 11 years). There was no gender preponderance in the AVNRT group (60 males, 56 females) while a male preponderance was seen in the AVRT group (71 males, 37 females, p < 0.01). There was a marked fall in the intraarterial systolic BP in both groups at the onset of tachycardia, from 143 +/- 24 mm Hg to 108 +/- 16 mm Hg (p < 0.05) for AVNRT and from 139 +/- 25 mm Hg to 107 +/- 18 mm Hg (p < 0.05) for AVRT. There was no correlation between the rate of tachycardia and the extent of fall of BP. CONCLUSION: Hospital-based data in an Indian setting found a similar pattern of age of onset of AV node-dependant tachycardia as in Western literature. However, unlike in Western studies, no female preponderance was seen in the AVNRT group. The fall in systolic BP at the onset of tachycardia is significant, similar in the two groups and independent of the rate of tachycardia.

Arrhythmogenic right ventricular dysplasia: electrophysiologic and morphologic features in nine patients.

Arrhythmogenic right ventricular dysplasia is a potentially life-threatening disorder, not previously well described in India. We analysed the electrocardiographic, electrophysiologic, angiographic, signal-averaged electrocardiogram and magnetic resonance imaging features of nine patients having arrhythmogenic right ventricular dysplasia at our centre. There were seven males and two females, aged 25 to 55 years. Eight patients presented with sustained monomorphic ventricular tachycardia. The electrocardiogram showed prolonged QRS duration in four, T inversion in leads V2-V3 in four, and epsilon wave in two patients. Abnormal late potentials were present in eight patients. During electrophysiologic study in seven patients, 13 different VTs were induced. Seven patients had right ventricular dilatation and dysfunction and left ventricular involvement was seen in three patients. Eight patients were treated with amiodarone, including one who received an implantable cardioverter-defibrillator. At a follow-up of 8.6 +/- 5.3 months, there was no death, while one patient had recurrence of ventricular tachycardia. Arrhythmogenic right ventricular dysplasia seems to predominantly affect middle-aged men, the presentation most often being sustained monomorphic ventricular tachycardia. Depolarisation and/or repolarisation abnormalities in the electrocardiogram are commonly present. Multiple ventricular tachycardia morphologies during electrophysiologic study and abnormal right ventricular angiograms are usually observed.

Scope and efficacy of complex radiofrequency ablation.

Radiofrequency ablation is an effective treatment for various tachycardias. In some patients undergoing radiofrequency ablation, there are additional complexities. Of 254 consecutive patients (161 males, 93 females, aged 38 +/- 15 years) who underwent radiofrequency ablation, two groups were identified. Patients with structural heart disease, multiple accessory pathways, multifocal idiopathic ventricular tachycardia, parahisian accessory pathways, and multiple mechanisms of tachycardia were considered as complex radiofrequency ablation (Group I) and the remaining as non-complex radiofrequency ablation (Group II). There were 23 patients in Group I. The overall success rate was not different in the two groups: 20/23 (87%) vs 208/231 (90%) in groups I and II, respectively. The procedure and fluoroscopy time (212 +/- 91 min vs 136 +/- 45 min; p = 0.0001; and 55 +/- 31 min vs 31 +/- 21 min; p = 0.001, respectively) were significantly longer in Group I. There was a higher recurrence rate in Group I (6/20, 30% vs 20/208, 9.6%; p = 0.02); repeat radiofrequency ablation was successfully performed in four patients with recurrence in Group I. Complications were rare in both the groups. There was no mortality. Thus radiofrequency ablation in complex situations was effective, though more demanding than non-complex radiofrequency ablation and associated with higher recurrence.

Is haemorrhagic myocardial infarction more common with streptokinase?

Autopsy reports and clinical data of 226 consecutive myocardial infarction deaths in whom postmortem studies could be carried out during the period 1980 to 1996 were analyzed retrospectively for the presence of haemorrhagic myocardial infarction (HMI). Of 53 autopsies done from 1980 to 1986 [prior to use of streptokinase (SK) therapy in our institution] none of the specimens showed haemorrhagic infarction. Of 173 autopsies done from 1987 to 1996 (intravenous SK therapy was utilised in this period), 20 specimens showed haemorrhagic infarctions. Sixteen of these 20 patients had received SK, while 66 of the remaining 153 non-haemorrhagic myocardial infarction patients received SK (statistically significant association of SK with HMI, p < 0.005). Acute mechanical complications [ventricular septal rupture (n = 10), papillary muscle rupture (n = 2), cardiac free wall rupture (n = 7)] were seen in 19 cases. Of these, 16 were HMIs and 14 of these patients had received streptokinase. These observations suggest a strong association of HMI with SK therapy and with acute mechanical complications.