Management of hereditary angioedema in resource-constrained settings: A consensus statement from Indian subcontinent.

Hereditary angioedema (HAE) is an uncommon disorder characterized clinically by recurrent episodes of nonitchy subcutaneous and/or submucosal swellings. The estimated prevalence of HAE is ~ 1: 10,000 to 1: 50,000. There are no prevalence data from India, however, estimates suggest that there are 27,000 to 135,000 patients with HAE in India at present. The majority of these, however, remain undiagnosed. Replacement of plasma-derived or recombinant C1-esterase inhibitor (C1-INH) protein, administered intravenously, is the treatment of choice during the management of acute episodes of angioedema (i.e., "on-demand treatment") and is also useful for short-term prophylaxis (STP) and long-term prophylaxis (LTP). This has been found to be effective and safe even in young children and during pregnancy. Until recently, none of the first-line treatment options were available for "on-demand treatment," STP or LTP in India. As a result, physicians had to use fresh frozen plasma for both "on-demand treatment" and STP. For LTP, attenuated androgens (danazol or stanozolol) and/or tranexamic acid were commonly used. These drugs have been reported to be useful for LTP but are associated with a significant risk of adverse effects. Intravenous pd-C1-INH, the first-line treatment option, is now available in India. However, because there is no universal health insurance, access to pd-C1-INH is a significant challenge. HAE Society of India has developed these consensus guidelines for India and other resource-constrained settings where plasma-derived C1-INH therapy is the only available first-line treatment option for the management of HAE and diagnostic facilities are limited. These guidelines have been developed because it may not be possible for all patients to access the recommended therapy and at the recommended doses as suggested by the international guidelines. Moreover, it may not be feasible to follow the evaluation algorithm suggested by the international guidelines.

A unique bilateral accessory forearm flexor muscle.

Muscular and neurovascular variations in the upper extremity are of utmost clinical significance. Here we report a unique bilateral accessory muscle in the forearm and palm of an 89-year-old male cadaver. The accessory muscle presented two bellies on the right side, one in the forearm, innervated by the anterior interosseous nerve, and the other in the palm, innervated by a branch of the median nerve. A long tendon interconnected the two bellies. On the left side, the muscle had a single belly in the palm, which began at the end of a long tendon that extended from the forearm. However, on both sides, the muscle originated from the posterior surface of the flexor digitorum superficialis belly and inserted along with the first lumbrical muscle into the dorsal digital expansion of the index finger. The proximal parts of the variant muscles were sandwiched between the flexor digitorum muscles. The palmar bellies coursed distally through the carpal canal and lay deep to the superficial palmar arch, and superficial to the first lumbrical, between the thenar muscles and the lateral-most tendon of the flexor digitorum superficialis. Arguably, the accessory muscle might be a variant of a lumbrical muscle, as reported before, but innervation of the proximal belly by the anterior interosseous nerve suggests that the muscle may well be a deep accessory muscle at the forearm, probably appeared as a diverted part of the flexor digitorum profundus. Its space-occupying course through the forearm and palm, especially through the carpal canal, might be clinically significant as it might contribute to nerve compression pathologies in the upper extremity. This accessory muscle also indicates the complex nature of individual muscle formation and evolution of the upper extremity with constant changes in the morphology of muscles based on their changing functions.

Intussusception: Single Center Experience of 10 Years.

OBJECTIVE: To analyze the association between the clinical presentation, clinical course, management and outcome in intussusception with emphasis on safety of saline hydrostatic reduction. METHODS: This retrospective study included 375 patients of intussusception diagnosed between March 2007 to February 2017. Symptoms at presentation, mode of reduction of intussusception and associated complications were recorded. RESULTS: 336 (89.6%) patients were aged below 3 years. Classical triad of abdominal pain, vomiting and red stools was present in 111 (29.6%) patients. While 64 (17.1 %) patients had spontaneous resolution, hydrostatic reduction and surgery cured 283 (75.5 %) and 28 (7.4 %) patients, respectively; overall recurrence rate was 13.1%. Among the patients who underwent operative reduction, blood in stools was present in 15 (53.6%) patients. CONCLUSIONS: Hydrostatic reduction of intussusception is effective irrespective of duration of symptoms and number of recurrences.

Airway Diseases Education and Expertise (ADEX ) in Pediatrics: Adaptation for Clinical Practice in India.

JUSTIFICATION: Asthma and allergic rhinitis together are part of the concept of one airway, one disease or united airway disease. The management of allergic airway diseases should address this united concept and manage the issue by educating the patients and their parents and health care providers, along with environmental control measures, pharmacotherapy and immunotherapy. Here, we present recommendations from the module of Airway Diseases Education and Expertise (ADEX) that focused on allergic rhinitis, asthma and sleep disorder breathing as a single entity or Allergic Airway Disease. PROCESS: A working committee was formed by the collaboration of Pediatric Allergy Association of India (PAAI) and Indian Academy of Pediatrics (IAP) Allergy and Applied Immunology chapter to develop a training module on united airway disease. OBJECTIVE: To increase awareness, understanding and acceptance of the concept of United Airway disease and to educate the primary health care providers for children and public health officials, in the management of united airway diseases. RECOMMENDATIONS: Recommendations for diagnosis, management and follow-up of Allergic airway disease are presented in this document. A better compliance by linking education of child, parent, grandparents and other health care providers, and scientific progress by collaboration between practitioners, academicians, researchers and pharmaceutical companies is suggested.

Validation of R wave voltage endomyocardial mapping to assess myocardial fibrosis: comparison with thallium and dobutamine echocardiography in a swine model.

The first aim of this study was to validate R wave voltage values measured with a catheter-based three-dimensional mapping system (NOGA, Biosense) against myocardial fibrosis in a swine model of ameroid placement. The second aim was to correlate the UPV maps with thallium uptake and low-dose dobutamine echocardiography. The electromechanical catheter mapping system can be used to guide endomyocardial laser therapy and direct gene delivery to the myocardium. The accuracy of R wave voltage in identifying myocardial fibrosis and its comparison with other imaging technologies against histopathology has not been fully investigated in an animal model. Ameroid constrictors were placed on 24 vessels in 14 swine. Wall motion abnormalities by echocardiography were detected in distribution of the constrictors at 22 +/- 10 days. Animals underwent rest and delayed thallium imaging, low-dose dobutamine echocardiography, and R wave voltage mapping. Animals were sacrificed, hearts were removed, 4-micron slices were stained, and fibrosis was quantified. Five control animals were studied to obtain segment normalization. There was significant agreement between each imaging modality and fibrosis by chi-square analysis. The correlation for segment normalized thallium uptake and segment normalized UPV score versus fibrosis were both significant (P < 0.001) with an r2 value of 0.362 vs. thallium, and r2 = 0.445 vs. UPV. The correlation was improved using log of UPV vs. fibrosis (r = 0.532). These results help validate R wave voltage mapping as an imaging technique to identify myocardial fibrosis.

Agreement among Ayurvedic practitioners in the identification and treatment of three cases of inflammatory arthritis.

OBJECTIVE: To conduct a preliminary investigation into the consistency of approach between three Ayurvedic medicine experts on treatments for inflammatory polyarthritis. METHODS: A convenience sample of three experienced Ayurvedic practitioners was recruited. These practitioners independently assessed three subjects with inflammatory polyarthritis for health status, treatment history, and lifestyle, conducted a physical examination, and then independently determined the treatment plan. The treatment plan was recorded on standardized collection forms. The subject examination order was randomized for each practitioner. Following completion of the assessments, a facilitated discussion among the practitioners permitted each to discuss all aspects of the recommended therapies. Proceedings were audio-taped and the content analyzed. RESULTS: All three practitioners agreed upon a unified concept of Ayurvedic disease origin, disease diagnosis, and treatment approach for each patient. Seven specific treatment groupings (i.e. modalities) emerged: diet, exercise, relaxation, analgesic, anti-inflammatory, immune-enhancing, and detoxification/cleansing. Based on the single visit, the practitioners agreed upon 17 of 21 treatment groups for the three patients. CONCLUSION: Despite Ayurvedic medicine's individualized approach, considerable agreement existed among the practitioners studied. The identified Ayurvedic treatment approaches require investigation in a controlled clinical setting.

A clinical profile of mitral valve prolapse syndrome.

Clinical, radiologic, electrocardiographic and 2-dimensional echocardiographic findings of fifty cases of mitral valve prolapse syndrome attending the Pediatric Cardiology clinic of I.C.H. and Childrens Hospital, Medical College, Kottayam over a period of ten years from 1980-1989 are presented. Mitral valve prolapse syndrome (MVPS) accounted for 2% of cardiac problems attending our pediatric cardiology clinic. Isolated MVPS constituted 64% of the cases. The common causes of secondary MVPS were Marfan Syndrome-18%, Atrial Septal Defect-10% and Rheumatic fever-8%. Associations of MVPS included Pectus excavatum (8%), Isolated arachnodactyly (2%) and Straight back syndrome (2%). 2-D echocardiogram demonstrated prolapse of both leaflets of Mitral valve in 44%, Isolated posterior mitral valve leaflet prolapse in 32% and Isolated anterior mitral valve leaflet prolapse in 24% of cases. No complications were seen during follow up.