Rheumatoid Arthritis- Unique Combination of Rheumatic, Cardiac and Pulmonary Manifestation of a Multisystem Disorder

Rheumatic arthritis is a common inflammatory disease but combination of cardiac valvular dysfunction involving aortic valve calcification, tricuspid regurgitation and right atrioventricular enlargement with interstitial involvement of lung is very rare manifestation of this multisystem disorder.

Consensus & Evidence-based INOSA Guidelines 2014 (First edition).

Obstructive sleep apnoea (OSA) and obstructive sleep apnoea syndrome (OSAS) are subsets of sleep-disordered breathing. Awareness about OSA and its consequences amongst the general public as well as the majority of primary care physcians across India is poor. This necessiated the development of the INdian initiative on Obstructive Sleep Apnoea (INOSA) guidelines under the auspices of Department of Health Research, Ministry of Health & Family Welfare, Government of India. OSA is the occurrence of an average five or more episodes of obstructive respiratory events per hour of sleep with either sleep related symptoms or comorbidities or >15 such episodes without any sleep related symptoms or comorbidities. OSAS is defined as OSA associated with daytime symptoms, most often excessive sleepiness. Patients undergoing routine health check-up with snoring, daytime sleepiness, obesity, hypertension, motor vehicular accidents and high risk cases should undergo a comprehensive sleep evaluation. Medical examiners evaluating drivers, air pilots, railway drivers and heavy machinery workers should be educated about OSA and should comprehensively evaluate applicants for OSA. Those suspected to have OSA on comprehensive sleep evaluation should be referred for a sleep study. Supervised overnight polysomnography (PSG) is the “gold standard” for evaluation of OSA. Positive airway pressure (PAP) therapy is the mainstay of treatment of OSA. Oral appliances are indicated for use in patients with mild to moderate OSA who prefer oral appliances to PAP, or who do not respond to PAP or who fail treatment attempts with PAP or behavioural measures. Surgical treatment is recommended in patients who have failed or are intolerant to PAP therapy.

Bariatric surgery: An overview.

Obesity is a global epidemic. Its prevalence is increasing rapidly and India is no exception.1 Obesity is not only a cosmetic problem but is associated with a number of diseases including type 2 diabetes mellitus (DM), hypertension, obstructive sleep apnoea syndrome (OSAS), polycystic ovarian disease syndrome (PCOS) and non-alcoholic fatty liver disease (NAFLD). It is also a risk factor for a number of malignancies. Obese persons tend to have a shorter life span when compared to an age-matched normal population. Among the options for weight reduction, dietary and lifestyle modifications are generally advised as the first line of treatment. A large amount of scientific literature has suggested beyond doubt that non-surgical treatment of morbid obesity does not lead to a meaningful weight loss.2–5 Almost all (90%–95%) those who do lose significant weight regain it.3 As far as pharmacotherapy is concerned, there is almost nihilism. Most of the drugs have been withdrawn due to side-effects. Even after discounting for side-effects, these medicines lead to a nominal weight loss of only 5–10 kg. In 10-year data reported by the Swedish Obese Subject (SOS) study, weight loss in the nonsurgical group was minimal compared to that in the surgical group (1.5% v. 25%).4 This non-randomized study included over 4000 patients and the 5-year mortality rate in the surgical group was significantly lower than that in the non-surgical group (0.68% v. 6.17%).4 We often come across patients who initially refuse surgery due to fear of complications but later seek surgery. During this period they gain more weight and the comorbid conditions worsen. Bariatric surgery provides a means for considerable weight control and has also been effective in the resolution of obesity-related comorbid conditions.5 The long-term mortality due to obesity also decreases after bariatric surgery.4

Conventional renal cell carcinoma with renal capsular leiomyoma: A diagnostic dilemma.

Primary renal leiomyomas are very rare benign tumors of the kidney, arising from smooth muscle cells of the renal capsule, pelvis or blood vessels. Simultaneous occurrence of a leiomyoma with conventional renal cell carcinoma (RCC) in the same kidney is an extremely rare incidence. Herein we describe a case, which to the best of our knowledge is the second reported case in the English literature. This 70-year-old female presented in surgical out-patient department with the complaints of hematuria and abdominal pain. Subsequently, on computed tomography a multifocal mass was noted in the right kidney, measuring 4.5 cm × 3 cm × 2 cm and 1 cm × 0.5 cm × 0.5 cm respectively. A radical nephrectomy was performed with the clinical diagnosis of a multifocal RCC. Histopathological examination revealed a conventional RCC in the larger nodule, whereas the smaller nodule showed a sub capsular leiomyoma, which was immune-positive for smooth muscle actin and HMB45. In the index case, radiology could not differentiate between the RCC and leiomyoma. Apart from being a radiological diagnostic dilemma, rare HMB45 immunostain positivity in capsular leiomyoma may create diagnostic dilemma for histopathologists too. Sole HMB45 positivity should not distract one to diagnose this lesion as an angiomyolipoma, unless the other components are seen.

Beta-sarcoglycanopathy.

Sarcoglycanopathies are relatively rare progressive muscular dystrophies with autosomal recessive inheritance; which belong to the group of limb girdle muscular dystrophies. The phenotype resembles dystrophinopathies due to proximal muscle weakness and calf hypertrophy. Reports from the Indian subcontinent are scarce. The authors report a case of primary beta-sarcoglycanopathy and describe literature pertaining to this rare entity.

Infant gastroesophageal reflux disease score: reproducibility and validity in a developing country.

A 25-point infant gastro-oesophageal reflux disease (GERD) score based on 11 signs and symptoms of gastrooesophageal reflux (GER), to diagnose GERD has been suggested in infant. We carried out this study to test the reproducibility and validity of this scoring system in the cross-cultural settings of Indian infants. Caretakers of 610 apparently healthy infants, between the ages of 1 month and 24 months were administered the Orenstein's infant GER questionnaire and assigned a GERD score. Of these, 95 infants were taken up for a 24-hours oesophageal pH monitoring study. Before the pH study, each subject was again tested by the infant GER questionnaire by another independent observer and assigned an infant GERD score. The 24-hours oesophageal pH study was done using the Synectics Digitrapper MK III portable pH recording device. Reflux index (RI) >10% in infants up to 1 year of age and >5% in children more than 1 year of age was taken as pathological. Upper gastrointestinal endoscopy and oesophageal biopsies were performed in 35 cases, after taking informed consent. A good correlation was seen between the scores evaluated independently by the two workers, with a Pearson correlation coefficient of 0.906. The mean GERD score in infants with GER (as diagnosed by pH-metry) was 4.64 +/- 3.99 compared to 3.54 +/- 3.96 in those with no documented GER (p>0.05). A GERD score of 5 had a sensitivity of 43% and specificity of 79%, compared to 86% and 85% observed by Orenstein et al. in their series. The infant GER Questionnaire is easily adaptable and reproducible in the settings of developing countries. However, its diagnostic validity appears to be much less than that obtained by Orenstein et al. in their study on American infants.

Xanthogranulomatous pancreatitis: mass lesion of the pancreas simulating pancreatic carcinoma--a report of two cases.

Xathogranulomatous inflammation is well known in the gall bladder and kidney. Xanthogranulomatous pancreatitis has not been previously described. We report two cases of this new clinicopathologic entity. The first was a 50 years old male with cholelithiasis and progressive obstructive jaundice for 5 months. Radiology was suggestive of carcinoma head of pancreas and a Whipples procedure was performed. The second was a 36 years old male with choledocholithiasis and features of chronic pancreatitis. During pancreaticojejunostomy, a mass was found in the tail of pancreas, which was excised with a suspicion of carcinoma. Gross examination of both specimens showed firm grey white masses, demarcated from the surrounding pancreas but with infiltrative margins, and were thought to be carcinoma. Histopathological examination showed localized inflammation with numerous foamy histiocytes along with dilated ducts and microabscesses. A diagnosis of xanthogranulomatous pancreatitis was made in both instances. In view of clinical, radiological, operative and gross appearances of our cases simulating carcinoma, recognition of xanthogranulomatous chronic pancreatitis as a distinct clinicopathological entity seems important, analogous to similar lesions of the kidney and gall bladder.

Portal vein thrombosis complicating neonatal hepatic abscess.

Hepatic abscess in a neonate is a rare but serious disorder. Diagnosis of hepatic abscess requires a high index of suspicion in any septic neonate. CT scan and ultrasound of liver are the most sensitive diagnostic tests in detection of hepatic abscess. Portal vein thrombosis and portal cavernoma formation is hitherto unreported complication of neonatal hepatic abscess in English literature. Present case report highlights the difficulty in diagnosis of neonatal hepatic abscess and describes the development of portal vein thrombosis and cavernoma during its treatment.