The natural history of hidradenitis suppurativa

AIM: To investigate aspects of the natural history of hidradenitis. BACKGROUND: The natural history of hidradenitis suppurativa (HS) is not well known. There is incomplete published data on the average age of disease onset, progression of the disease, average monthly incidence and duration of boils, and factors that relieve or exacerbate disease symptoms. STUDY DESIGN: Questionnaire-based survey among HS patients identified from hospital records of three hospitals in Nottinghamshire, UK. RESULTS: One hundred and ten of 156 questionnaires (70.5%) were returned, 93 from females and 17 from males. The average patient's age was 40.1 years and the average reported age of disease onset was 21.8 years. At the time of the survey patients had suffered an average disease duration of 18.8 years. Most patients (98 of 110) still had experienced active disease within the past year. There was some evidence that in women the condition has a tendency to ease or subside after the menopause. Forty-four per cent of women felt that their condition was aggravated by menstruation. Thirty-eight per cent of patients gave a positive family history of the disorder. The average duration of painful boils was 6.9 days. In addition, 62% of patients acknowledged the presence of permanently painful boils that failed to subside. Patients developed a median of two boils per month. Factors that could aggravate the condition were primarily sweating or heat, stress or fatigue and tight clothing or friction. Factors that could improve the condition consisted largely of a variety of medical treatments and a number of life-style measures, such as swimming or baths. Twenty-four per cent of patients had failed to find anything at all to help their condition, despite an average disease duration of almost 19 years. CONCLUSIONS: The study highlights several of the factors that make HS one of the most distressing dermatological diseases, such as the average monthly incidence of painful lesions, their average duration and the chronicity of the disease. It seems striking that the mean duration of an HS boil (6.9 days) roughly equals the duration of an average course of antibiotics. The postulated response of HS to oral antibiotics may thus simply have its explanation in the natural history of the condition itself.     

Autoimmune hemolytic anemia in Kawasaki disease: a case report

A 3-year-old boy presented with the fever, conjunctivitis, rash, and lymphadenopathy diagnostic of Kawasaki disease. Treatment with antibiotics, aspirin, and intravenous immunoglobulin was instituted. The hematocrit decreased from 35 percent on admission to 11 percent by hospital Day 10, and the white cell count had increased from 13.7 to 42 × 10(3) per microL, and the patient had a leukoerythroblastic blood smear. The direct antiglobulin test demonstrated IgG but not complement on the red cell (RBC) surface. An acid eluate reacted (titer of 4) with all panel cells in the antiglobulin phase. Intravenous immunoglobulin from the same lot used for treatment did not contain antibody that reacted with the patient's group O RBCs or a panel of group O RBCs, but did contain IgG anti-A and -B (titer of 4). The patient received a transfusion and was given methylprednisone. The direct antiglobulin test and acid eluate were negative 4 days later. The patient had an uneventful recovery. The distinction between antibody-mediated hemolytic anemia and autoimmune hemolytic anemia is important in the treatment of this disease.     

示指桡侧指背动脉逆行岛状皮瓣移植修复示指缺损

目的:观察示指桡侧指背动脉逆行岛状皮瓣移植对示指指端缺损的修复效果。方法:选择2002-08/2005-09南华大学附属第一医院急诊收治的外伤性示指末节部分缺损患者13例,手术方法的选择在术前均得到患者同意,且得到医院伦理道德委员会批准。组织缺损大小在1.5cm×1.0cm～2.0cm×1.5cm之间,所有缺损均有指骨和(或)大部分的指腹组织缺损,采用示指桡侧指背动脉逆行岛状皮瓣移植修复,皮瓣设计:旋转点位于近侧指间关节的近端,皮瓣轴为示指的桡背侧,皮瓣部分设计在第2掌指关节的近侧,蒂宽约0.8mm。术后定期随访,主要观察皮瓣质地和感觉的恢复情况,将感觉恢复的评估标准分为5级:S1:无感觉;S5:在神经单一分布区恢复两点鉴别能力。结果:13例患者全部进入结果分析,无脱落。①术后随访三四个月者10例,随访五六个月者3例。②术后9例皮瓣完全成活;4例皮瓣远端部分坏死,经换药表皮爬行创面愈合。③外形基本满意,皮瓣色泽、质地良好。④术后1个月随访时,3例患者两点辨别觉大于6.0mm,感觉恢复S4;术后五六个月随访时,3例患者两点辨别觉4.0～6.0mm,感觉恢复S5。结论:示指桡侧指背动脉逆行岛状皮瓣设计的旋转点位于近侧指间关节的近端,可以简化手术而不影响皮瓣存活,是示指部分缺损修复的可选方法。     

Erythrocytapheresis therapy to reduce iron overload in chronically transfused patients with sickle cell disease

Chelation therapy with deferoxamine is effective in preventing the risk of transfusional iron overload, but treatment failure is common because of noncompliance. To reduce the transfusional iron load, we have evaluated longterm erythrocytapheresis in 14 subjects with sickle cell disease and stroke (11) or other complications (3) as an alternative to simple transfusion. Subjects were treated with erythrocytapheresis using the Haemonetics V50 (Haemonetics Corp, Braintree, MA) to maintain the target pretransfusion hemoglobin S (Hb S) level less than 50% for 6 to 71 months. The transfusional iron load and the donor blood usage were analyzed for a 6- to 36-month study period and were compared with similar data from a subset of 7 subjects previously treated with conventional (target Hb S < 30%) and modified (target Hb S < 50%) simple transfusion protocols. The effect of erythrocytapheresis on iron accumulation was determined by assessment of serum ferritin levels in the absence of iron chelation. The mean transfusional iron load and donor blood usage with erythrocytapheresis were 19 +/- 14 mg iron/kg/yr (range, 6 to 50) and 188.4 +/- 55.2 mL packed-red blood cells (RBC)/kg/yr (range, 107 to 281), respectively. Of 6 subjects receiving no iron chelation therapy, 5 maintained normal or nearly normal serum ferritin levels during 11 to 36 months of erythrocytapheresis. In comparison with conventional simple transfusion and modified simple transfusion, erythrocytapheresis reduced iron loading by 87% (P < .01) and 82% (P < .01), respectively, but increased donor blood usage by 23% and 73%, respectively. Subjects with pre-erythrocytapheresis Hb levels > or = 8.0 g/dL had lower iron accumulation (P < .001) and less donor blood usage (P < .005) than subjects with Hb levels < or = 8.0 g/dL. Although donor blood usage is increased in comparison with simple transfusion, long-term erythrocytapheresis markedly reduces or prevents iron accumulation. This form of transfusion therapy allows the cessation of iron chelation in well-chelated subjects and, if used as the initial form of transfusion therapy, may prevent long-term complications of sickle cell disease without risk of iron overload and the need for chelation therapy.     

Single-strand conformation polymorphism analysis is a rapid and effective method for the identification of mutations and polymorphisms in the gene for glycoprotein IIIa

Glanzmann thrombasthenia (GT) is the most common inherited disorder of platelets. Most of the molecular defects previously identified in GT have been caused by point (or other small) mutations in the genes for glycoprotein (GP) IIb or GPIIIa. We have used single-strand conformation polymorphism (SSCP) analysis to rapidly identify single- base changes in the GPIIIa gene. Using genomic DNA from normal individuals and patients with GT, each GPIIIa exon and a short stretch of flanking intronic sequence was amplified, heat-denatured, and separated in nondenaturing acrylamide gels. Only those fragments with an abnormal migration pattern were isolated and the nucleotide sequence determined. Using SSCP, we detected the polymorphism in the HPA-1 (P1A) system and all three known silent polymorphisms in the GPIIIa gene. Screening 14 GPIIIa exons from 5 patients with GT, one mutant allele was identified. The nucleotide sequence of the abnormal 240-bp SSCP fragment was determined and a G-->A substitution in the splice donor site of exon iv was identified. Analysis of platelet RNA resulting from this mutation showed two mRNA species: one contained a deletion of exon iv, whereas the other had a 27-bp addition to exon iv due to the use of a cryptic splice site in the downstream intron. Single-base substitutions are the most common mutation in GT and often result in abnormal mRNA splicing. SSCP is a rapid and sensitive technique for identifying mutations or polymorphisms in the GPIIIa gene.     

Bedside filtration of blood products in the prevention of HLA alloimmunization--a prospective randomized study. Alloimmunisation Study Group

To test the efficacy of poststorage bedside leucodepletion of blood products in the prevention of primary HLA alloimmunization and its clinical sequelae, 172 patients with hematologic malignancy requiring intensive red blood cell and platelet support were randomized to receive either standard or filtered red blood cells and platelets. Quality control of bedside filtration was explored by sequential sampling downstream of the filter, but this did not predict the total number of leucocytes transfused. After exclusions, 123 evaluable patients were assessed every two weeks until the end of therapy. HLA antibodies developed in 21 of 56 (37.5%) nonfilter (NF) and 15 of 67 (22%) filter (F) patients (risk ratio estimate, 0.60 [95% confidence interval, 0.34 to 1.05]; P = .07). Patients with acute myeloid leukemia (AML; n = 53) had higher alloimmunization rates in both arms of the study, with a greater effect of filtration (62.5% NF and 31.0% F; P = .025). Bedside filtration did not affect the overall incidence of febrile transfusion reactions (FTRs; 37% NF and 34% F; P = .71) or of platelet refractoriness assessed in 50 patients (30% NF and 26% F), despite an association between broad HLA reactivity and both FTRs and refractoriness. However, FTRs were also seen in 28 patients without HLA antibodies. Five alloimmunized refractory patients (2 F and 3 NF) required HLA-selected platelets. This report, the first prospective study of bedside filtration, has failed to show clear clinical benefit. Methodological limitations may account in part for this failure, notably the difficulties in accurately assessing the number of leucocytes transfused.