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.     

Plantar pressure analysis after percutaneous repair of displaced intra-articular calcaneal fractures

BACKGROUND: Clinical results for the treatment of displaced intra-articular calcaneal fractures are mainly expressed using disease-specific outcome scores, physical examination and radiographs. We hypothesized that plantar pressure and foot position analysis is a valuable tool in assessing foot function in patients with a unilateral displaced intra-articular calcaneal fracture treated percutaneously. MATERIALS AND METHODS: With a followup of at least one year, 21 patients with a unilateral displaced intra-articular calcaneal fracture treated percutaneously participated in the study. The pedobarographic measurements in the injured foot were compared with the contralateral control foot. Correlations between the ratios (injured/control) of plantar pressure and foot position variables and outcome scores, the physical exam items ratios, the fracture classification, and the radiological parameters were calculated. RESULTS: Statistically significant differences between the injured and the control foot were found for the weight distribution (p = 0.002), total contact time (p < 0.001), and the maximum pressure under the first metatarsal (p = 0.02) after a median followup of 18 months. Of all correlations calculated, only the heel time ratio correlated significantly with the heel width ratio (p = 0.004). CONCLUSION: Significant differences in plantar pressure distribution between the injured and uninjured foot were found, indicating that plantar pressure analysis and foot position analysis is an objective test to assess deviations in foot function. Plantar pressure data revealed limited correlation with outcome scores. Therefore, plantar pressure analysis should not be used instead of but in addition to established outcome scores.     

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     

The knowledge,awareness, and practice patterns of dermatologists toward psychocutaneous disorders: results of a survey study

Background To assess the level of training in, and awareness and attitude about, psychocutaneous disorders among dermatologists. Methods A mail‐in survey was sent to all members of Washington State Dermatology Society, who were requested to provide information on demographic variables; level of training, skills, and degree of comfort in managing psychodermatologic disorders; referral patterns, knowledge of patient and family resources on psychodermatology; and interest in continuing medical education on psychocutaneous disorders. Results Of 237 mailed surveys, 102 were returned for analysis. Only 18% of dermatologists reported a clear understanding of psychodermatology, and 42% of the respondents reported being very comfortable in diagnosing and treating psychocutaneous disorders. Acne, atopic dermatitis and psoriasis were reported as the most common diagnoses associated with psychiatric manifestations. Delusion of parasitosis, neurotic excoriations, and trichotillomania were the most common conditions wherein patients were referred by dermatologists to psychiatrists. About 90% of the survey respondents were not aware of any patient or family resources on psychodermatology. Overall, 39% of the dermatologists expressed interest in attending any kind of continuing medical education activity on psychodermatologic disorders. Conclusion Survey results showed that knowledge about the diagnosis, treatment and/or appropriate referral for psychocutaneous disorders is lacking. Significant information gaps were also identified in the knowledge of patient or family resources on psychocutaneous disorders. We recommend the incorporation of formal training and didactics on psychodermatology in dermatology residency programs and regular CME events. Dermatology–Psychiatry liaison services and psychodermatology clinics will prove helpful in the management of these patients in clinical settings.     

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.     

Social competence and social support as mediators between comorbid depressive and conduct problems and functional outcomes in middle school children

This study examined the roles of social competence and social support as potential mediators of the association between psychopathology and functional outcomes in a middle school sample (n=521). Participants were stratified into four psychopathology risk groups (depression only, conduct problems only, comorbid depression and conduct problems, low symptoms) based on screening during early 6th grade. Functional outcomes were 6th grade point average (GPA) and parent rating of global adaptive functioning in their 7th grade student. Low levels of social competence were found to mediate the association between symptoms and both lower grades and global functioning for adolescents with depressive symptoms alone and with comorbid symptoms, but not for those with conduct problems alone. Lack of social support mediated the association between psychiatric symptoms and lower grades for adolescents with depression alone and comorbid symptoms, but not for those with conduct problems alone. These findings suggest that intervention to improve social competence and social support may enhance functional outcomes, especially for youth with depressive symptoms or comorbid depressive and conduct symptoms.     

What explains the relation between family poverty and childhood depressive symptoms?

The relation between low socioeconomic status (SES) and depression has been well documented in adult populations. A number of studies suggest that family SES may be associated with depression among children and adolescents as well, although the evidence is mixed. We assessed the relation between family income and depressive symptoms among 457 children aged 11-13 years and examined pathways that may explain this relation. In-person interviews of children and their caregivers were conducted, including assessment of family income and administration of the Computer-based Diagnostic Interview Schedule for Children (C-DISC). Family income was significantly associated with depressive symptoms, with children in the lowest income group (<$35,000) reporting a mean of 8.12 symptoms compared to 6.27 symptoms in the middle income group ($35,000-$74,999) and 5.13 symptoms in the highest income group (> or = $75,000; p<0.001). Controlling for the number of stressful life events experienced in the past 6 months attenuated the effect of low family income on depressive symptoms by 28%. Indicators of the family environment explained 45% and neighborhood median household income and aggravated assault rate explained 12% of the relation. The family environment, including parental divorce or separation and perceived parental support, appears to explain most of the relation between low family income and childhood depressive symptoms. Further exploration of the pathways between family SES and depression may suggest potential interventions to reduce the occurrence and persistence of depressive symptoms in children.