Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome.

Camptodactyly-arthropathy-coxa vara-pericarditis syndrome (CACP) is a rare autosomal recessive disease caused by mutation in proteoglycan 4 (PRG4) gene on chromosome 1q25-q31. We faced a dilemma and delay in diagnosis in two sisters. The elder sister had pericardial effusion with constrictive pericarditis, underwent pericardiectomy and received empirical treatment for suspected tuberculosis. After 2 years, she developed bilateral knee swelling with restriction of movement. At the same time, her younger sister also presented with bilateral knee swelling which aroused the suspicion of genetic disease. The whole-genome sequencing revealed homozygous PRG4 mutation suggestive of CACP syndrome.

Cross-Cultural Adaptation and Validation of a Marathi Version of the Versus Arthritis Musculoskeletal Health Questionnaire (MSK-HQ).

Introduction Musculoskeletal (MSK) well-being plays a crucial role in determining one's quality of life. Musculoskeletal Health Questionnaire (MSK-HQ) score is a tool recently developed by the Versus Arthritis group of Oxford University in English to measure MSK health. Marathi is a regional language in western India spoken by more than 100 million people. There is a scarcity of valid and reliable tools to measure MSK health in this language. Hence, we decided to cross-culturally adapt and translate MSK-HQ to Marathi. Method We translated MSK-HQ score to Marathi (MSK-HQ-Ma) as per the International Society for Pharmacoeconomics and Outcomes Research (ISPOR) guidelines. We tested its internal consistency, construct validity and reproducibility. It was compared with other health status scores EQ-5D-5L and overall health using the Visual Analogue Scale (VAS). Test-retest reliability was tested in those subjects who were having stable MSK health after two weeks. Results We recruited 158 consecutive subjects attending musculoskeletal clinics who had Marathi as their native language. Mean age was 44.8±17 years, females were 78 (49%). It showed good internal consistency (Cronbach's alpha = 0.95). For construct validity we found a strong correlation between MSK-HQ-Ma and EQ-5D-5L values (Spearman's r = 0.82, p<0.001). There was also a good correlation between MSK-HQ-Ma and overall health by VAS (Spearman's r = 0.76, p<0.001). An excellent test-retest reliability (Spearman's r = 0.94, p<0.001) was seen in 105 subjects who had stable MSK condition after two weeks of first appearance. Conclusion The MSK-HQ-Ma instrument has demonstrated good consistency, reliability and construct validity when evaluating the musculoskeletal health of individuals who can understand the Marathi language. Hence it can be used as a validated tool for the evaluation of musculoskeletal health in western India where Marathi is a commonly used language.

Hansen's Disease, the Perpetual Impersonator ("The Behroopiya") of Rheumatology Practice: A Case Series.

We encountered five patients with Rheumatic symptoms, suspected to have five different clinical diagnoses: Systemic vasculitis, Gout, Systemic Sclerosis, Rheumatoid Arthritis, and Spondyloarthropathy. However, all were finally diagnosed with the same illness: Hansen's Disease.

Off-label Drug Prescription Pattern and Related Adverse Drug Reactions in the Medical Intensive Care Unit.

Introduction: The utilization of prescription drugs as off-label is common. While this practice can be beneficial to some patients, it can raise a safety concern when scientific evidence is lacking; hence, this study was conducted to evaluate the off-label drug consumption and its adverse drug reactions (ADRs) in the medical intensive care unit (ICU). Materials and methods: In the prospective cohort study conducted for a duration of 6 months, data pertaining to ICU patients' (age ≥18 years) demography, diagnosis, treatment, and laboratory investigation were collected to assess for off-label use as well as the strength of evidence and the occurrence of ADRs by using MICROMEDEX 2017 version (Healthcare Series Thomson Reuter, Greenwood, CO). Results: Of total 3574 drugs prescribed, 1453 (41%) were off-label indications and 65 (1.81%) were off-label dose. On the evaluation of off-label indication use, 1279 (88%) were evidence-based and 174 (12%) were low/no evidence-based medications (EBMs); 59 (91%) were evidence-based and 6 (9%) were low/no EBMs for off-label dose. Most commonly prescribed evidence-based off-label drug belonged to the gastrointestinal class while low/no evidence drugs were mostly of anti-infective class. A total of 383 ADRs were identified and 139 (36.2%) were implicated due to off-label medications, of which ADRs with evidence off-label medications (87.8%) were higher than low/no evidence off-label medication (12.2%) (P < 0.001). Conclusion: Widespread presence of off-label use was observed in medical ICU. Although incidence of ADRs was similar to the FDA-approved use, ongoing monitoring of such practice is needed. How to cite this article: Raut A, Krishna K, Adake U, Sharma AA, Thomas A, Shah J. Off-label Drug Prescription Pattern and Related Adverse Drug Reactions in the Medical Intensive Care Unit. Indian J Crit Care Med 2021;25(8):872-877.

Methemoglobinemia - The Cryptic Cause of Dyspnoea.

Methemoglobinemia is a life threatening condition that can be difficult to diagnose. It can be congenital or, more often an adverse drug effect. A good, detailed history taking and thorough knowledge of drugs and toxins is the secret to early diagnosis. We present two interesting cases of methemoglobinemia. First was phenol poisoning with G6PD deficiency leading to hemolysis and methemoglobinemia and second was phenol induced methemoglobinemia. Here we discuss the diagnosis and management of a patient with acquired methemoglobinemia.

Jaundice Heralding the Onset of Thyrotoxic Crisis.

Thyrotoxic crisis is a life threatening medical condition that requires urgent diagnosis and treatment. Because of the wide variety of presenting symptoms, its diagnosis can be difficult in some cases and a high index of suspicion is required for diagnosis. We present a case of 53 year old patient who presented with fever, jaundice and passage of loose stools. Upon investigations other etiologies for hepatic injury were ruled out and she was found to be in thyrotoxic crisis causing liver injury.

Behcet's Disease: The Darker side !

Behcet's disease (BD) is a chronic inflammatory disease affecting blood vessels throughout the body mainly veins and clinically characterized by oro-genital aphthae ,ophthalmologic involvement ,cutaneous lesions ,articular, neurological involvement and less commonly by gastrointestinal manifestations which may have dreadful complications. Here we report a case of 58 year male with gastrointestinal complications of BD and a rapidly worsening course.

Acute Myocardial Infarction in Nephrotic Syndrome.

A 28 year old male, known case of nephrotic syndrome since 12 years, hypertensive presented with acute myocardial infarction (AMI) and accelerated hypertension. Coronary angiography revealed 100% thrombotic occlusion of mid left anterior descending artery, treated with thrombus aspiration and intracoronary tirofiban and nitroglycerine. He was stabilized within 24 hours. The pathogenesis of AMI in nephrotic syndrome has been discussed with this case report.

Acute organophosphorus poisoning complicated by acute coronary syndrome.

We report a case of 30 year old alcoholic male admitted with vomiting, drowsiness, limb weakness and fasciculations after alleged history of consumption of 30 ml of chlorpyriphos insecticide. He had low serum cholinesterase levels. With standard treatment for organophosphorus poisoning (OPP), he improved gradually until day 5, when he developed neck and limb weakness and respiratory distress. This intermediate syndrome was treated with oximes, atropine and artificial ventilation. During treatment, his ECG showed fresh changes of ST elevation. High CPK & CPK-MB levels, septal hypokinesia on 2D echo suggested acute coronary syndrome. Coronary angiography was postponed due to his bedridden and obtunded status. The patient finally recovered fully by day 15 and was discharged. Acute coronary syndrome is a rare occurrence in OP poisoning. The present case thus emphasises the need for careful electrocardiographic and enzymatic monitoring of all patients of organophosphorus poisoning to prevent potential cardiac complication which can prove fatal.

Knowledge and Awareness of HPV Infection and Vaccination Among Urban Adolescents in India: A Cross-Sectional Study.

AIM OF THE STUDY: To assess the level of awareness and knowledge of HPV infection and vaccination among 1,000 adolescent girls from secondary schools and colleges in five metro cities of India-Ahmedabad, Cuttack, Lucknow, Gwalior, and Visakhapatnam. To evaluate participants own interest and barriers toward HPV vaccination for cervical cancer prevention. MATERIALS: This cross-sectional study was conducted by Adolescent Health Committee of FOGSI from April 2009 to March 2010 under the project "protecting young girls." Girls of 13-19 years, with an average of 16 years are targeted. METHODS: A written questionnaire with two parts has been applied. A preliminary written questionnaire included questions of knowledge on cancer cervix and HPV awareness. Then, health talk on HPV is given by the researcher and group discussions lasting for 20 min. Second questionnaire was then applied to evaluate effectiveness of the talk. RESULTS: The study group participants are poorly aware about HPV infection and vaccination but are intensely willing to know about it and get vaccinated. 72 % of them did not know about cervical cancer or HPV. 77.2 % were not aware of the virus that causes cancer cervix. After the health talks. there is an overall significant positive improvement in both knowledge and awareness. 74.4 % of them agreed to get vaccinated. CONCLUSIONS: This study brings out the unawareness about HPV infection and vaccination in urban adolescent girls in five metro cities in India. Results show a changing positive trend of acceptance toward HPV vaccination. Adolescent understanding of HPV is needed to have successful vaccination programs in India.

Evan's syndrome revisited.

A female aged 43 years presented with acute per vaginal bleeding since six days, severe thrombocytopenia and anaemia, she responded partially to platelets and blood transfusion initially. Four days later she started bleeding from nose, intravenous access sites, developed right sided hemiparesis and subsequently died. Her investigations were suggestive of Idiopathic Thrombocytopenia Purpura (ITP) and Autoimmune Haemolytic Anaemia (AIHA). So a diagnosis of Evan's syndrome was made.

Lemierre's syndrome--the syndrome quite forgotten.

A 17 year old male presented with seizures, headache, and fever and left chronic suppurative otitis media. A 35 year old male presented with headache, giddiness, vomiting, pain in eyes, diplopia and right chronic suppurative otitis media. Brain imaging in both revealed thrombosis of lateral and sigmoid sinus and also of internal jugular vein on the left and right side respectively. A diagnosis of Lemierre's syndrome was made in both. They were treated with antibiotics and anticoagulants, and they responded to treatment. We want to report this case as we feel, that with the advent of the antibiotic era, this syndrome has become rare; and so "quite forgotten" or overlooked, by many physicians.

Levetiracetam.

Levetiracetam (LEV) is a novel antiepileptic drug (AED) which was discovered in early 1980s and soon, in 1999 FDA approved LEV for the management of partial onset seizure. In India, LEV tablet was approved in April 2005. It acts by binding to the synaptic vesicle protein SV2A, which is present on synaptic vesicles and some neuroendocrine cells. Pharmacokinetics of LEV such as, less protein binding and lack of hepatic metabolism makes LEV less susceptible to drug interactions with other anticonvulsants. Evidence also suggests that LEV is much better than other AEDs in the way of broad therapeutic window, convenient dosing and less adverse effect. Besides the pharmacological effects, pharmacoeconomically also, LEV is a beneficial drug. All these valuable pharmacological and pharmacoeconomic aspect makes LEV an important option in management of various types of epilepsy.

Robotic sacrocolpopexy: an observational experience at mayoclinic, USA.

Although there are many studies the ongoing debate on the management of posthysterectomy vault prolapse whether it should be abdominal, vaginal, or laparoscopic still continues. However there is no clear consensus. Though the incidence of vaginal vault prolapse is said to range from 0.2 to 45%, the choice of the optimal treatment depends on the surgeon's experience, suitability for surgery, age, symptoms, quality of life impairment, and prolapse grade. Abdominal sacrocopopexy (ASCP) with mesh interposition is the traditional surgical procedure for treating pelvic organ prolapse and has been shown to have one of the highest long-term success rates for vaginal vault prolapse. The laparoscopic approach offers reduced morbidity, shorter hospitalization, and decreased post operative pain. The disadvantages of the laparoscopic approach include longer operating time and need for advanced laparoscopic surgical skills including suturing. Robot-assisted laparoscopic procedure allows the performance of complex laparoscopic maneuvers with less difficulty, and thereby simplifies the complex procedure. The aim is to describe and demonstrate the use and benefit of robot-assisted laparoscopic sacrocolpopexy in the treatment of posthysterectomy vaginal vault prolapse in obese patients along with mid-urethral sling application.

Paget-Schroetter syndrome.

A case of primary upper-extremity deep vein thrombosis (UEDVT) or Paget-Schroetter syndrome is reported. It is effort thrombosis usually affecting young healthy individuals and is a rare condition. Our patient was an elderly male with history of exertion using the left upper limb. He responded to limb elevation and anticoagulation. No other cause for thrombosis was found.

Dermoid of the conus medullaris.

Dermoids are rare midline tumours. When involving the spinal cord they usually present early, in the first decade of life. We present a case of an elderly male with paraparesis. His MRI showed cord expansion at the tip of the conus medullaris; contrast enhancement with Gadolinium revealed a cystic lesion and histopathology confirmed the diagnosis of a dermoid cyst.