Congenital dyserythropoietic anemia type II — a case report of two siblings in a family

Two children out of three siblings of a family presented with tiredness, fatigue, and breathlessness for more than 6 months. Examination of peripheral blood smear, bone marrow aspirate, and a positive acidified serum test (HEMPAS) revealed these children to be a case of congenital dyserythropoietic anemia type II. This case is reported because of its rarity.     

Editorial

Evidence-Based Integrative Medicine -     

Clinical Impact of Community-Acquired Respiratory Viruses on Bronchiolitis Obliterans After Lung Transplant

Community-acquired viral respiratory tract infections (RTI) in lung transplant recipients may have a high rate of progression to pneumonia and can be a trigger for immunologically mediated detrimental effects on lung function. A cohort of 100 patients was enrolled from 2001 to 2003 in which 50 patients had clinically diagnosed viral RTI and 50 were asymptomatic. All patients had nasopharyngeal and throat swabs taken for respiratory virus antigen detection, culture and RT-PCR. All patients had pulmonary function tests at regular intervals for 12 months. Rates of rejection, decline in forced expiratory volume (L) in 1 s (FEV-1) and bacterial and fungal superinfection were compared at the 3-month primary endpoint. In the 50 patients with RTI, a microbial etiology was identified in 33 of 50 (66%) and included rhinovirus (9), coronavirus (8), RSV (6), influenza A (5), parainfluenza (4) and human metapneumovirus (1). During the 3-month primary endpoint, 8 of 50 (16%) RTI patients had acute rejection versus 0 of 50 non-RTI patients (p=0.006). The number of patients experiencing a 20% or more decline in FEV-1 by 3 months was 9 of 50 (18%) RTI versus 0 of 50 non-RTI (0%) (p=0.003). In six of these nine patients, the decline in FEV-1 was sustained over a 1-year period consistent with bronchiolitis obliterans syndrome (BOS). Community-acquired respiratory viruses may be associated with the development of acute rejection and BOS.     

Celiac disease in India.

This review of the current scenario of celiac disease (CD) in India covers both pediatric and adult CD. CD is primarily reported from northern India with isolated case reports from the rest of the country. CD cases among Indian children are associated with multiple DR3-DQ2 haplotypes. Delay in diagnosis is contributed by multiple factors including atypical presentations. Use of serological tests, IgA EMA and anti-tTG antibodies, along with modified ESPGHAN criteria provides a definitive diagnosis of CD. Dietary management is often difficult due to non-availability of labeled and marketed gluten-free foods. A majority of children with CD show normalization of nutrition, substantial improvement in growth parameters and attainment of healthy percentile curves on gluten-free diet. Small bowel histology remarkably improves but does not normalize even after 2-3 years on gluten-free diet. The true burden of the disease should be addressed by large epidemiological studies.     

Airway obstruction after extubation following use of transesophageal echocardiography for posterior fossa surgery in the sitting position.

We report respiratory obstruction following surgery in the sitting position with tracheal intubation and placement of a transesophageal echocardiography probe. Obstruction was due to pharyngeal oedema, which resolved with 24 hours. The mechanisms of this complication are discussed.     

Expanding the Spectrum of Acquired Cerebral Hemiatrophy

The authors report a 50-year-old man with progressive left hemispheric atrophy, cognitive decline, infrequent seizures, and spells suggestive of complicated migraine. They discuss the association between migraine and cerebral hemiatrophy and suggest that the spectrum of acquired cerebral hemiatrophy may be broader than what has been commonly recognized.     

Pleomorphic adenoma of the nasal septum

Despite the common occurrence of pleomorphic adenoma of major salivary glands, intranasal pleomorphic adenoma are rare. We present a ease of pleomorphic adenoma of the nasal septum along with a brief review of literature. The histological nature of this lesion in comparison to other salivary gland tumours and the importance of an accurate diagnosis has been stressed.     

Conventional 4-field box radiotherapy technique for cancer cervix: potential for geographic miss without CECT scan-based planning

BACKGROUND: The advantage of 4-field radiation to the pelvis is that the use of lateral portals spares a portion of the small bowel anteriorly and rectum posteriorly. The standard lateral portals defined in textbooks are not always adequate especially in advanced cancer cervix. METHODS: An analysis was done to determine adequacy of margins of standard lateral pelvic portals with CECT defined tumor volumes. The study included 40 patients of FIGO stage IIB and IIIB treated definitively for cancer cervix between 1998 and 2000. An inadequate margin was defined if the cervical growth and uterus were not encompassed by the 95% isodose. RESULTS: An inadequate posterior margin was common with bulky disease (P = 0.06) and with retroverted uterus (P = 0.08). Menopausal status, FIGO stage, associated myoma, and age were of no apparent prognostic significance. Bulk retained significant on multivariate analysis. An inadequate anterior margin was common in premenopausal (P = 0.01); anteverted uterus (P = 0.02); associated myoma (P = 0.01); and younger patients (P = 0.03). It was not influenced by bulk or stage. Menopausal status and associated myoma retained significant on multivariate analysis. CONCLUSION: Without the knowledge of precise tumor volume, the 4-field technique with standard portals is potentially risky as it may under dose the tumor through lateral portals and the standard AP/ PA portals are a safer option.