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 -     

Laparoscopic transhiatal surgery of the esophagus.

OBJECTIVE: Esophagectomy is an operation with high morbidity and mortality. Its adoption as a minimally invasive operation worldwide has been slow, but the potential benefits of reducing the trauma of surgery need to be considered. Our 30-month experience with transhiatal esophagectomy in a district general hospital is presented herein. METHODS: Patients were considered for surgery after radiological staging had excluded inoperable disease. Laparoscopic staging was initially performed. Patients with tumors of the esophagus and high-grade dysplasia in a Barrett's esophagus were included. RESULTS: Twenty-nine patients were referred for consideration for resectional surgery. Nine underwent outpatient laparoscopy only. Twenty patients (age range, 34 to 78, 15 males:5 females) underwent resectional surgery. Seventeen transhiatal resections were completed, 2 were converted to open procedures, and 1 transhiatal resection of a benign tumor was performed. Median time of surgery was 415 minutes (range, 320 to 480) and blood loss was 300 mL (range, 200 to 350). The median length of post-operative ventilation and critical care stay were 1 (range, 1 to 4) and 4 (range, 2 to 8) days. Median duration of hospitalization was 17 days (range, 10 to 28). Thirty-day mortality was 0; 1 patient who was converted to an open procedure died after a cerebrovascular event on day 34. CONCLUSION: A zero mortality rate for laparoscopic resection and a low-morbidity rate compare well with morbidity and mortality in reported series using this method and open surgery. Laparoscopic transhiatal esophagectomy is an advanced, complex procedure that can be performed safely in a district general hospital setting.     

A Pelvic Ring Fracture Complicated by Acute Traumatic Obstructed Direct Inguinal Hernia

Abstract A young male motorcyclist presented to the Pelvic Unit with an open-book fracture of his pelvis and an acute obstructed direct inguinal hernia. He was managed operatively and made an excellent recovery. Traumatic abdominal wall hernias are rare. This is a unique combination of injuries that the trauma surgeon should be aware of.     

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.