[Soft-tissue reconstruction of the dorsum of the hand and finger to cover the extender tendons]. / Weichteilrekonstruktion am hand- und fingerrücken über dem streckapparat.

Small defects of extensor tendons can mostly be covered by local flaps. For larger defects the groin flaps, pedicled flaps from the forearm and free flaps are indicated. Between January 2001 and December 2005, we performed the following pedicled and free flaps to reconstruct the soft tissue defects of the hand or distal forearm: 23 pedicled flaps: 15 posterior interosseus artery or perforator flaps, 8 radial forearm flaps; 26 free flaps: 4 radial forearm flaps, 12 lateral upper arm flaps, 10 latissimus, serratus fascia or scapula flaps. With this study we want to illustrate the different procedures, compare their surgical techniques and outcomes and finally to rate the results. In the context of security, morbidity of the donor site and functional outcomes, our favourite flap is the lateral upper arm flap.

Costello syndrome and hyperinsulinemic hypoglycemia.

Costello syndrome is characterized by mental retardation, loose skin, coarse facies, skeletal abnormalities, cardiovascular abnormalities (congenital heart defects, cardiomyopathy, rhythm disturbances), and predisposition to neoplasia. Endocrine abnormalities including growth hormone deficiency, adrenal insufficiency, glucose intolerance, parathyroid adenoma with hyperprolactinemia and hypoglycemia have been described. Hypoglycemia has been documented due to growth hormone and cortisol deficiency. We report on two patients with Costello syndrome and persistent hyperinsulinemic hypoglycemia and review the endocrine manifestations of Costello syndrome. Both patients required diazoxide therapy to stop the unregulated insulin secretion and maintain normoglycemia. The mechanism of persistent hyperinsulinism in patients with Costello syndrome is unclear.

[The treatment of carpal collapse: proximal row carpectomy or limited midcarpal arthrodesis? A comparative study]. / Der fortgeschrittene karpale Kollaps: Resektion der proximalen Handwurzelreihe (PRC) oder mediokarpale Teilarthrodese? Eine vergleichende Studie.

Between July 2000 and February 2002, 26 wrists were treated because of SLAC, SNAC and radiocarpal arthrosis after fracture of the distal radius. In 14 cases proximal row carpectomy (PRC) and in twelve cases limited midcarpal arthrodesis (LWF) was performed. Follow-up was after a mean time of 16.8 (PRC) and 13.7 months (LWF) on ten wrists each. There was one complication in the PRC group and two in the LWF group. In all cases significant reduction of extension/flexion was observed with a mean range of motion of 69 degrees (PRC) and 71 degrees (LWF). The reduction of pain postoperatively was almost identical in both groups. Comparing grip-strength to the unoperated hand, we measured 26 compared to 39 kg in the PRC group and 28 compared to 46 kg in the LWF group, respectively. The DASH-score for the LWF group was 30 points and therefore better than the 36 points for the PRC group.

Successful pregnancies following remission in childhood acute lymphoblastic leukaemia--case report.

We report two successful pregnancies in a 17-year-old Arab girl who had received modern combination chemotherapy and central nervous system minimal disease therapy for childhood acute lymphoblastic leukaemia at the age of 9.5 years.

Design and implementation of a cost-accounting system in hospital pharmacy.

The design and implementation of a cost-accounting system in a hospital pharmacy department is described. Pharmacy resource use (labor, drugs, supplies, and overhead), or pharmacy's intermediate products, was clearly defined in terms of dosage forms (10 groupings representing variable labor and supplies) and drug products (more than 100 categories that incorporate cost and volume of use for 3000 line items). Costs were defined as variable or nonvariable (fixed), based on whether they were related to a specific medication order. Labor was divided into variable and fixed components. Time standards were developed using time and motion studies. Variable labor hours were determined as follows: specified hours (the volume of each dosage form multiplied by the standard time for each dosage form); nonspecified hours (time not directly associated with production); hours worked (specified plus nonspecified hours); and hours paid (hours worked plus sick leave and vacation). A standard cost for each drug product was based on the weighted average of volume and cost of the individual line items. The total drug budget was constructed by multiplying the standard cost for each drug product times the projected volume for each drug product. The pharmacy budget was developed by calculating the number and mix of pharmacy products used in association with the projected number and type of cases for the fiscal year. The monthly pharmacy budget reports were assembled with data from the payroll, billing, and cost-accounting systems.(ABSTRACT TRUNCATED AT 250 WORDS)

Elevated red cell adenosine deaminase activity: a marker of disordered erythropoiesis in Diamond-Blackfan anaemia and other haematologic diseases.

Red-cell adenosine deaminase (ADA) activity in children with Diamond-Blackfan anaemia is significantly increased (1.91 +/- 0.90 U/g Hb) compared to that seen in transient erythroblastopenia of childhood (0.80 +/- 0.16 U/g Hb) or normal individuals (0.61 +/- 0.13 U/g). These data thus further support that measurement of this purine metabolic enzyme is useful in diagnosing the cause of pure RBC aplasia in children. Of interest, however, elevated RBC-ADA activity also is seen in some children with acute leukaemia and other haematologic disorders. In children with acute lymphoblastic leukaemia (ALL), the increase in RBC-ADA activity is proportional to the degree of anaemia. However, the elevated RBC-ADA activity in this leukaemic population is not related to the fetal haemoglobin concentration. These data suggest increased RBC-ADA activity may be a non-specific manifestation of abnormal erythroid stem cell function, an alteration distinct from that seen with reactivation of fetal erythropoiesis. However, since almost all patients with Diamond-Blackfan anaemia manifest elevated RBC-ADA activity, this chemical alteration yet may reflect the specific erythroid differentiation lesion in this disorder.

A first-year medical school pilot program for early clinical exposure.

A weekly out-patient elective in a medical oncology clinic was established for first- and some second-year medical students. Interviews were carried out immediately after the elective experience and two years later to evaluate the degree of success in achieving specific goals in this elective. Such clinical experience appeared to introduce the medical students to some of the major medical and psychosocial issues involved in the clinical care of patients, to be highly motivating for the students with regard to their standard concurrent basic science curriculum, and to relieve the impatience that first-year medical students have for clinical exposure. The usefulness of the elective, as perceived by the participating students, appeared to increase from the first to the second interview, that is as the students entered the clinical teaching program. The clinical setting was perceived by the students as more useful than either their increased understanding of medicine or observation of physicians as role models. The students' perception and understanding of physician-patient communication, and of the problems facing an oncology patient, appeared to be more favorably influenced than increasing confidence in the ability of the student to deal with patient interaction. Thus, a clinical oncology out-patient experience for first-year medical students appears to be sufficiently rewarding to justify the time and effort to develop such an elective.

Comparative activity of erythrocyte adenosine deaminase and orotidine decarboxylase in Diamond-Blackfan anemia.

It previously has been reported that red blood cells (RBC) of patients with Diamond-Blackfan syndrome (DBS) have increased activity of orotidine decarboxylase (ODC) and adenosine deaminase (ADA). The studies reported here compared the activity of these two enzymes in DBS erythrocytes, cord blood, and reticulocytes. The activity of ODC, although increased in some DBS erythrocytes, was not significantly different from that seen in cord RBC or reticulocytes. In contrast, RBC-ADA activity was increased in 23 of 26 DBS patients; and this enzyme elevation was distinct from that seen in cord blood and reticulocytes. Moreover, ADA activity was normal in 26 of 27 patients with transient erythroblastopenia of childhood (TEC). Taken together, these data indicate RBC-ADA activity is more sensitive than ODC as a marker of DBS. In addition, RBC-ADA activity continues to be useful for distinguishing DBS and TEC in most patients with RBC hypoplasia.

Monovalent cation changes in sickle erythrocytes: a direct reflection of alpha-globin gene number.

It has been suggested that the less severe anemia that exists in patients with sickle cell anemia who also have alpha-thalassemia is related to an effect on the sickling of erythrocytes. To test this hypothesis, we used as a measure of sickling the change in sickle erythrocyte sodium and potassium content that occurs during deoxygenation. Sickle cells from individuals with four, three, or two alpha-globin genes were deoxygenated, and the change in monovalent cation content measured to determine whether a relationship exists between alpha-globin gene number and sickling. In samples of cells depleted of irreversibly sickled cells, the alpha-globin gene number was directly related to the magnitude of mean cation change and inversely related to the ratio of membrane surface area to cell volume. These data indicate that alpha-thalassemia is associated with a diminished degree of sickling in individuals with sickle cell anemia. They also suggest that excess cell membrane may play a role in this protective effect.

Elevated erythrocyte adenosine deaminase activity in congenital hypoplastic anemia.

Abnormalities of adenosine deaminase, a critical enzyme of the purine salvage pathway, have been reported in association with immune dysfunction, acute leukemia, and hereditary hemolytic anemia. We report data showing that erythrocyte adenosine deaminase activity is also abnormal in congenital hypoplastic anemia (the Diamond-Blackfan syndrome). Adenosine deaminase activity in erythrocytes from 12 patients (mean +/- S.D., 2.20 +/- 0.77 IU per gram of hemoglobin) was substantially greater than that observed in 50 controls (0.62 +/- 0.13 IU per gram). Enzyme activity in affected patients was also greater than that seen in cord blood or in erythrocytes from patients with hemolytic anemia, acquired aplastic anemia, Fanconi's hypoplastic anemia, acquired pure red-cell aplasia, or transient erythroblastopenia of childhood. These observations indicate that erythrocyte adenosine deaminase activity may be a unique marker for identifying congenital hypoplastic anemia.