Hemoglobin M equon beta 41 (C7) phenylalanine leads to tyrosine

A severe hemolytic crisis was observed in a 34-yr-old female of English- Irish extraction following a viral illness treated with acetaminophen. Heinz bodies and heat instability were present only during a transient hemolytic event. A challenge dose of acetaminophen caused no detectable hematologic abnormality. Structural studies of the hemoglobin during hemolysis and again after complete recovery localized the abnormality to tryptic peptide beta Tp-5, and automated sequencing of I 125-labeled beta chains indicated a replacement of phenylalanine (C7) beta 41 by tyrosine. Substitution of the next residue, phenylalanine (CD1) beta 42 by serine (Hb Hammersmith), has resulted in chronic severe Heinz body hemolytic anemia. The lack of chronic anemia in the present disorder may reflect the different relationships of beta41 and beta 42 and/or the similarities in volume and hydrophobicity of tyrosine and phenylalanine. It is suggested that substitution of tyrosine for phenylalanine in Hb Mequon may disturb the critical environment around the heme group and render it susceptible to oxidative denaturation in the presence of infections and/or drugs.     

Single-layer uninterrupted suture at the surgery of chronic pancreatitis

Single-layer uninterrupted suture is uncommon at the pancreatic surgery. This method has been used at 44 patients during pancreatic resection, cysto-enterostomy, pancreato-enterostomy and pancreato-cysto-enterostomy. Techniques of suture is described and illustrated. There were no cases of anastomosis insufficiency or other anastomosis complications. Method is simple, reliable and may be interesting for surgeons operating on pancreas.     

Blood Transfusion Practices in Military Medicine

Background

This observational study was conducted in a small, 45 bed border static hospital, located in a field area, where no blood bank facilities were available. The present study was conducted to elucidate the blood transfusion practices of this hospital. Methods: A retrospective analysis of all blood transfusions performed in this hospital between Dec 2004 and Dec 2006 was carried out. The data collection included blood group patterns, common indications, haemoglobin levels and complications of blood transfusion. Inferences were based on available data and relevant statistical analysis.

Result

A total of 246 blood transfusions were administered to 79 recipients during the study period. Only one patient had an Rh negative blood group. The most frequently transfused blood group was A Rh positive. Majority of transfusions were administered to surgical cases and the commonest indication was gunshot wounds with haemorrhagic shock. The mean haemoglobin at admission was 8.93 g/dl. The mean number of blood transfusions per patient was 3.13. No haemolytic or other transfusion reactions occurred in any of the transfusions.

Conclusion

This study demonstrates that blood transfusions can be safely administered in field conditions despite constraints of not having a blood bank.Key Words: Blood transfusion practices, Haemoglobin, Anaemia     

Sarcomas in the groin and inguinal canal �C often missed and difficult to manage

INTRODUCTION

This is a 7-year retrospective review summarising the North of England Bone and Soft Tissue Tumour Service''s experience of managing 13 cases of groin sarcoma requiring soft tissue flap reconstruction. This study was performed to try to identify where national referral guidelines in sarcoma management had been followed and reasons for any delays. The study also includes outcome data relating to these patients.

PATIENTS AND METHODS

A retrospective, case-note review was undertaken using the local sarcoma database to identify approriate patients.

RESULTS

In nine patients, national referral guidelines were not followed. This resulted in a mean delay of presentation to the multidisciplinary team of 4.4 months. Ten patients had unplanned excision or exploration of tumours before referral. There were no lower limb amputations. All patients with narrow margins or high grade tumours were referred for radiotherapy. Four patients died; three as a result of distant metastases and one as a result of local recurrence.

CONCLUSIONS

Despite delays in referral, treatment by wide excision and plastic surgical reconstruction allowed for local control of these tumours with functional limb salvage. Implementation of National Institute for Health and Clinical Excellence (NICE) guidelines and local strategies could improve the expedient management of these patients.