Kysyntä-toimitusketjun synkronointi epävarman kysynnän ja tarjonnan toimintaympäristössä

Kysynnän ja tarjonnan epävarmuudet ovat nykyisin arkipäivää useilla toimialoilla. Elämme epävarmuuden suhteen ennen näkemättömiä aikoja, minkä on arvioitu jatkuvan myös tulevaisuudessa. Yritysten tilauskannat ovat lyhyitä, ja tilaukset viivästyvät tai peruuntuvat kokonaan. Toisaalta tarjonnan epävarmuudet aiheuttavat asiakasyrityksille haasteita esimerkiksi toimitusmyöhästymisten muodossa. Tuotannon ollessa hajaantunut verkostoihin yksittäisten yritysten toiminta ja päätökset vaikuttavat toisten verkostoyritysten toimintaan. Tämän takia epävarmuuden aiheuttamista muutoksista ja poikkeamista tulisi tiedottaa kumppaniyrityksiä, jotta kaikki pysyisivät samalla kellotaajuudella. Operatiivisen ja taktisen tiedon jakaminen on nykyisissä toimitusketjuissa jo arkipäivää, mutta yritysten välisistä rajapinnoista löytyy edelleen kehitettävää. Riittävästä ei kiinnitetä huomiota vastaanottajan kykyyn ja tapaan hyödyntää informaatiota – varsinkaan muutostilanteissa. Ajan/nopeuden ollessa yhä tärkeämpi kilpailutekijä informaation ajoituksella on kriittinen merkitys kysyntä-toimitusketjujen kokonaissuorituskykyyn. Toisin sanoen, millä ajanhetkellä tietoa tulisi jakaa, jotta kumppani pystyisi hyödyntämään saamaansa tietoa mahdollisimman hyvin. Kysyntä-toimitusketjun synkronoinnilla tarkoitetaan tässä väitöstutkimuksessa nimenomaan aikatekijään keskittymistä yritysten välisessä päätöksenteossa ja informaation jakamisessa toimitusketjun kokonaissuorituskyvyn parantamiseksi. Tutkimus kytkeytyy toimitusketjukoordinoinnin tieteelliseen keskusteluun. Koordinointiteorian keskeinen osa ovat riippuvuussuhteet, joita johdetaan koordinointimekanismien avulla. Kysyntätoimitusketjun synkronointia on mallinnettu aikaisemmin VOP-OPP-mallin (Value Offering Point – Order Penetration Point) ja sen johdannaisten avulla. Näissä malleissa asiakasyrityksen kysyntäketju ja toimittajayrityksen toimitusketju ovat keskinäisessä riippuvuussuhteessa, jota johdetaan päätöksenteon synkronoinnin ja informaation jakamisen koordinointimekanismeilla. VOP-OPP-malli johdannaisineen eivät kuitenkaan huomioi epävarman toimintaympäristön vaikutuksia synkronointiin. Näissä malleissa informaation ainoana laatudimensiona tarkasteltava aikatekijä on liian kapea-alainen näkökulma synkronointiin epävarmassa ympäristössä. Lisäksi nämä mallit keskittyvät vain yksisuuntaiseen, kysyntälähtöiseen, synkronointiin jättäen huomioimatta tarjontalähtöisen synkronoinnin. Aikatekijä- ja kokonaissuorituskykypainotustensa takia VOP-OPP-malli tarjosi kuitenkin hyvän lähtöfilosofian uusien synkronointimallien kehittämiseen. Väitöstutkimus toteutettiin hypoteettis-deduktiivisena tapaustutkimuksena, jossa ensin luotiin kirjallisuuden perusteella uudet teoreettiset synkronointimalliehdotukset. Tämän jälkeen ehdotusten toimivuutta arvioitiin käytännön kysyntä-toimitusketjuissa. Tutkimuksen uutuusarvo liittyy kysyntä-toimitusketjun synkronoinnin keskeisten piirteiden systeemiseen mallintamiseen epävarmassa toimintaympäristössä. Kontribuutiona esitetään kysyntä-toimitusketjun synkronoinnin moniulotteinen kokonaismalli, joka sisältää koordinointimekanismeina päätöksenteon synkronoinnin, informaation läpinäkyvyyden sekä asiakas- ja toimittajapään joustot. Tiedon vaihtoa mallissa tarkastellaan kaksisuuntaisesti – kysyntä- ja tarjontalähtöisesti. Informaation laatudimensioina mallissa ovat informaation ajoitus, luotettavuus ja tarkkuus. Kokonaismalli sisältää kolme alimallia: Demand Visibility Point – Demand Penetration Point (DVP-DPP) on kysyntälähtöisen synkronoinnin malli, Supply Visibility Point – Supply Information Penetration Point (SVP-SIPP) on tarjontalähtöisen synkronoinnin malli ja Integroitu DVP-DPP - SVP-SIPP-malli kytkee edellä mainitut mallit toisiinsa. Näissä alimalleissa informaation eri luokkia ovat tilausta edeltävä, tilaukseen liittyvä, tilauksen jälkeinen ja sovitun toimitusajankohdan jälkeinen kysyntä- ja tarjontatieto. Käytännön hyödyntämisen näkökulmasta mallit toimivat ns. mentaalitason koordinointimekanismeina, joiden tarkoitus on herättää toimitusketjukumppanit tavoittelemaan kokonaissuorituskyvyn parantamista oman edun tavoittelemisen sijasta. Tutkimuksen päärajoitteena on sen keskittyminen ainoastaan kahdenvälisiin yhteistyösuhteisiin, mikä tarjoaa nykyisessä verkostoituneessa toimintaympäristössä varsin kapean kuvan käytännön synkronointihaasteisiin.

Production of Chemicals from Woody Biomass by Wet Oxidation

The production of chemicals from sawdust by wet oxidation has been investigated. Two different concentrations of sawdust; 54054 mg/l and 32683 mg/l were used in the study. The wet oxidation operating conditions were; 175 deg.C – 225 deg.C, 1MPa Oxygen, and 40 minutes to 120 minutes reaction time. Carboxylic acids were among the chemicals produced in the process. The total yield of carboxylic acids was found to increase with temperature. Also, higher yields of carboxylic acids were observed at a lower sawdust concentration. This was probably due to the high oxygen-biomass ratio at lower sawdust concentration. Higher oxygen availability at low sawdust concentration resulted in increased conversion of the sawdust; hence the higher yields of carboxylic acids. At lower sawdust concentration, a total carboxylic acid yield of 25.59 wt% was attained at 200 deg.C and 40 minutes reaction time. At higher sawdust concentration, a total carboxylic acid yield of 15.57 wt% was attained at 200 deg.C and 40-minutes reaction time. The carboxylic acids identified include formic acid, acetic acid, succinic acid and oxalic acid. The optimum temperature for the production of formic acid was found to be 200 deg.C, while the optimum temperature for the production of acetic acid was found to be 225 deg.C. A temperature of 225 deg.C and relatively short reaction time of 10 minutes was found to be the optimal condition for the production of succinic acid. Formic acid was produced in the highest yield, with an optimal yield of 13.69wt %, when the reaction temperature and time are 200 deg.C and 40 minutes respectively. The yield of formic acid was found to decrease significantly when further increasing the temperature to 225 deg.C. This was presumably due to thermal decomposition of formic acid at relatively higher temperature. However, the yield of acetic acid was found to steadily increase with temperature. This is because acetic is more thermally stable than formic acid. The yield of acetic acid did not decrease after the temperature was increased to 225 deg.C. Optimal yield of acetic acid (7.98wt %) was achieved at; 225 deg.C, and 40 minutes reaction time. Succinic acid was produced only at temperatures of 200 deg.C and 225 deg.C. Optimal yield of succinic acid (5.66wt %) was attained under the following conditions; 32683 mg/l, 225 deg.C, 1MPa O2, and 10-minutes reaction time. Oxalic acid was produced in the lowest yield and, less frequently. The optimal yield of oxalic acid (4.02 wt%) was attained at 175 deg.C and 80-minutes of reaction time The Total Organic Carbon (TOC) is found to be higher when increasing the operating temperature, thus suggesting that more organic compounds are formed at higher temperatures. The identified carboxylic acids could only account for less than 30% of the measured COD content of the various wet oxidation samples. This implies that some other unidentified compounds (reaction products) must have been present. In general, wet oxidation seems to be an effective method for converting lignocellulosic biomass into useful chemicals. Relatively higher temperatures have been found to favor the production of carboxylic acids from sawdust.

Insiders´ views as a predictor of stock performance : can insider trading be used to predict stock performance on the United States stock market?

This thesis studies the possibility of using information on insiders’ transactions to forecast future stock returns after the implementation of Sarbanes Oxley Act in July 2003. Insider transactions between July 2003 and August 2009 are analysed with regression tests to identify the relationships between insiders’ transactions and future stock returns. This analysis is complemented with rudimentary bootstrapping procedures to verify the robustness of the findings. The underlying assumption of the thesis is that insiders constantly receive pieces of information that indicate future performance of the company. They may not be allowed to trade on large and tangible pieces of information but they can trade on accumulation of smaller, intangible pieces of information. Based on the analysis in the thesis insiders’ profits were found not to differ from the returns from broad stock index. However, their individual transactions were found to be linked to future stock returns. The initial model was found to be unstable but some of the predictive power could be sacrificed to achieve greater stability. Even after sacrificing some predictive power the relationship was significant enough to allow external investors to achieve abnormal profits after transaction costs and taxes. The thesis does not go into great detail about timing of transactions. Delay in publishing insiders’ transactions is not taken into account in the calculations and the closed windows are not studied in detail. The potential effects of these phenomena are looked into and they do not cause great changes in the findings. Additionally the remuneration policy of an insider or a company is not taken into account even though it most likely affects the trading patterns of insiders. Even with the limitations the findings offer promising opportunities for investors to improve their investment processes by incorporating additional information from insiders’ transaction into their decisions. The findings also raise questions on how insider trading should be regulated. Insiders achieve greater returns than other investors based on superior information. On the other hand, more efficient information transfer could warrant more lenient regulation. The fact that insiders’ returns are dominated by the large investment stake they maintain all the time in their own companies also speaks for more leniency. As Sarbanes Oxley Act considerably modified the insider trading landscape, this analysis provides information that has not been available before. The thesis also constitutes a thorough analysis of insider trading phenomenon which has previously been somewhat separated into several studies.

Effects of different ammoniacal nitrogen sources on N-metabolism of the atmospheric bromeliad Tillandsia pohliana Mez

Increasing levels of atmospheric ammonia from anthropogenic sources have become a serious problem for natural vegetation. Short-term effects of different ammoniacal sources on the N metabolism of Tillandsia pohliana, an atmospheric bromeliad, were investigated. One-year-old, aseptically grown plants were transferred to a modified Knudson medium lacking N for three weeks. Plants were subsequently transferred to Knudson media supplemented with 0.5, 1.0, or 1.5 mM of N in the forms of NH3 or NH4+ as the sole N source. The activities of glutamine synthetase (GS) and glutamate dehydrogenase (GDH-NADH) were determined after 40 h. The GS activity was stimulated significantly by increasing the levels of the gaseous form. The GDH-NADH activity increased significantly under increasing N concentrations with NH3, while no significant differences were observed with NH4+ as a N source. These results may reflect a faster NH3 absorption by T. pohliana compared to NH4+ uptake. The increased activity of GDH-NADH in NH3 treatment may play a role in protecting the cells from the toxic effects of increased endogenous level of free ammonium. A raise in the concentration of N, especially in the form of NH3, greatly increased the content of free amino acids and soluble proteins. A possible utilisation of T. pohliana to evaluate the changes of atmospheric gaseous ammonia is proposed.

Megaspore germination and initial development of Regnellidium diphyllum Lindman (Pteridophyta, Marsileaceae) sporophytes in the presence of cadmium

Regnellidium diphyllum has its distribution restricted to Southern Brazil and adjoining localities in Uruguay and Argentina. Currently it is on the list of threatened species of Rio Grande do Sul. The conversion of wetlands into agricultural areas or soil contamination by the introduction of waste products and fertilizers may compromise the establishment and survival of this species. Among the pollutants are heavy metals, such as cadmium (Cd). Megaspores were germinated in liquid culture medium, with concentrations 0 (control), 0.39; 0.78; 1.56; 3.12; 6.25; 12.5; 25; 50 and 100 mg L-1 of Cd, starting from a standard solution of Titrisol® at 1000 mg L-1. The increase of Cd in the growth medium to 50 mg L-1 resulted in low germinability (58%), and no germination was observed on 100 mg L-1. In apomictical sporophytes, the growth of primary root and leaf was significantly reduced and no secondary leaf was formed at Cd concentrations of 12.5 and higher than this. The results indicated that R. diphyllum is tolerant to the presence of Cd up to considerably higher concentrations (0.78 mg L-1) than that normally found in unpolluted aquatic ecosystems (0.01 mg L-1), although the sensitivity to higher concentrations might endanger the establishment and permanence of this species in habitats exposed to contamination with this metal.

Creating risk measurement model to project portfolio management in construction company

This thesis was carried out as a case study of a company YIT in order to clarify the sev-erest risks for the company and to build a method for project portfolio evaluation. The target organization creates new living environment by constructing residential buildings, business premises, infrastructure and entire areas worth for EUR 1.9 billion in the year 2013. Company has noted project portfolio management needs more information about the structure of project portfolio and possible influences of market shock situation. With interviews have been evaluated risks with biggest influence and most appropriate metrics to examine. The major risks for the company were evaluated by interviewing the executive staff. At the same time, the most appropriate risk metrics were considered. At the moment sales risk was estimated to have biggest impact on company‟s business. Therefore project port-folio evaluation model was created and three different scenarios for company‟s future were created in order to identify the scale of possible market shock situation. The created model is tested with public and descriptive figures of YIT in a one-year-long market shock and the impact on different metrics was evaluated. Study was conducted using con-structive research methodology. Results indicate that company has notable sales risk in certain sections of business portfolio.

A comparative study of eleven protein systems in tamarins, genus Saguinus (Platyrrhini, Callitrichinae)

The genetic variability of six tamarin taxa, genus Saguinus, was analyzed comparatively using protein data from eleven systems coded by 15 loci. S. fuscicollis weddelli and S. midas midas were the most polymorphic taxa, and S. bicolor the least. The results of the phylogenetic analyses (UPGMA and neighbor-joining) and the genetic distances between taxa were generally consistent with their geographic and probable phylogenetic relationships. Analyses of the S. bicolor and S. midas populations suggested that they represent no more than three subspecies of a single species, S. midas, with the bicolor forms belonging to a single subspecies, S. midas bicolor. If supported by additional studies, this would have important implications for the conservation of the bicolor form, which is endangered with extinction. The genetic similarity of S. fuscicollis and S. mystax was also consistent with their geographical and morphological proximity, although more data from a larger number of taxa will be required before the taxonomic relationships within the genus can be defined.

Cell lineage relationship in the stomach of normal and genetically manipulated mice

The oxyntic mucosa of the mouse stomach is lined with a heterogeneous population of cells that form numerous short pits continuous with long tubular glands. Tritiated thymidine radioautography has made it possible to pinpoint the origin of all cell types and to follow the differentiation/migration of different cell lineages along the pit-gland unit. The proliferating multipotent stem cells functionally anchored in the upper glandular region, the isthmus, give rise to three main lineage precursors: 1) pre-pit cells, which migrate upward to the pit while differentiating into mucus-producing pit cells; 2) pre-neck cells, which migrate downward to the glandular neck while differentiating into mucus-producing neck cells that, by approaching the glandular base, gradually change their phenotype into pepsinogen- and intrinsic factor-producing zymogenic cells; 3) pre-parietal cells, which differentiate into acid-producing parietal cells in the isthmus and then undergo bipolar migration towards the pit and the glandular base. Thus, parietal cells are the only cells that complete their differentiation in the isthmus and then migrate to be scattered throughout the pit-gland unit. To determine whether parietal cells play a role in controlling decisions about cell fate within the pit-gland unit, the gastric epithelium has been examined in transgenic mice expressing the H,K-ATPase ß-subunit-1035 to +24/simian virus 40 large T antigen fusion gene. The blockade in parietal cell differentiation in these mice produces an amplification of lineage precursors, a marked depletion of zymogenic cells and an increase in pit cell census. Ablation of parietal cells in another transgenic mouse model expressing the H,K-ATPase ß-subunit-1035 to +24/diphtheria toxin fragment A fusion gene also produces amplification of lineage precursors, and similar effects on zymogenic and pit cell census. These findings strongly suggest that parietal cells produce regulatory signals that control the cellular differentiation program of both pit and zymogenic cell lineages, and would hopefully improve our ability to identify the cellular pathways leading to malignant transformation

Perturbation of EGF-induced MAP kinase activation by TGF-ß1

TGF-ß1 regulates both cellular growth and phenotypic plasticity important for maintaining a growth advantage and increased invasiveness in progressively malignant cells. Recent studies indicate that TGF-ß-1 stimulates the conversion of epitheliod to fibroblastoid phenotype which presumably leads to the inactivation of growth-inhibitory effects by TGF-ß1 (Portella et al. (1998) Cell Growth and Differentiation, 9: 393-404). Therefore, the investigation of TGF-ß1 signaling that leads to altered growth and migration may provide novel targets for the prevention of increased cell growth and invasion. Although much attention has been paid to TGF-ß1 responses in epithelial cells, the above studies suggest that examination of signal transduction pathways in fibroblasts are important as well. Data from our laboratory are consistent with the concept that TGF-ß1 can act as a regulatory switch in density-dependent C3H 10T1/2 fibroblasts capable of either promoting or delaying G1 traverse. The regulation of this switch is proposed to occur prior to pRb phosphorylation, namely prior to activation of cyclin-dependent kinases. The current study is concerned with the evaluation of a key cyclin (cyclin D1) which activates cdk4 and p27KIP1 which in turn inhibit cdk2 in the proliferative responses of epidermal growth factor (EGF) and platelet-derived growth factor (PDGF) and their modulation by TGF-ß1. Although the molecular events that lead to elevation of cyclin D1 are not completely understood, it appears likely that activation of p42/p44MAPK kinases is involved in its transcriptional regulation. TGF-ß1 delayed EGF- or PDGF-induced cyclin D1 expression and blocked the induction of active p42/p44MAPK. The mechanism by which TGF-ß1 induces a block in p42/p44MAPK activation is being examined and the possibility that TGF-ß1 regulates phosphatase activity is being tested.

A receptor for infectious and cellular prion protein

Prions are an unconventional form of infectious agents composed only of protein and involved in transmissible spongiform encephalopathies in humans and animals. The infectious particle is composed by PrPsc which is an isoform of a normal cellular glycosyl-phosphatidylinositol (GPI) anchored protein, PrPc, of unknown function. The two proteins differ only in conformation, PrPc is composed of 40% a helix while PrPsc has 60% ß-sheet and 20% a helix structure. The infection mechanism is trigged by interaction of PrPsc with cellular prion protein causing conversion of the latter's conformation. Therefore, the infection spreads because new PrPsc molecules are generated exponentially from the normal PrPc. The accumulation of insoluble PrPsc is probably one of the events that lead to neuronal death. Conflicting data in the literature showed that PrPc internalization is mediated either by clathrin-coated pits or by caveolae-like membranous domains. However, both pathways seem to require a third protein (a receptor or a prion-binding protein) either to make the connection between the GPI-anchored molecule to clathrin or to convert PrPc into PrPsc. We have recently characterized a 66-kDa membrane receptor which binds PrPc in vitro and in vivo and mediates the neurotoxicity of a human prion peptide. Therefore, the receptor should have a role in the pathogenesis of prion-related diseases and in the normal cellular process. Further work is necessary to clarify the events triggered by the association of PrPc/PrPsc with the receptor.

Designing of Balanced Performance Measurement System to Purchasing Process

The performance measurement produces information about the operation of the business process. On the basis of this information performance of the company can be followed and improved. Balanced performance measurement system can monitor performance of several perspectives and business processes can be led according to company strategy. Major part of the costs of a company is originated from purchased goods or services are an output of the buying process emphasising the importance of a reliable performance measurement of purchasing process. In the study, theory of balanced performance measurement is orientated and framework of purchasing process performance measurement system is designed. The designed balanced performance measurement system of purchasing process is tested in case company paying attention to the available data and to other environmental enablers. The balanced purchasing performance measurement system is tested and improved during the test period and attention is paid to the definition and scaling of objectives. Found development initiatives are carried out especially in the scaling of indicators. Finally results of the study are evaluated, conclusions and additional research areas proposed.

Psychometric properties of the Portuguese version of the State-Trait Anxiety Inventory applied to college students: factor analysis and relation to the Beck Depression Inventory

The psychometric properties of the Portuguese version of the trait form of the State-Trait Anxiety Inventory (STAI-T) and its relation to the Beck Depression Inventory (BDI) were evaluated in a large Brazilian college student sample containing 845 women and 235 men. STAI-T scores tended to be higher for women, singles, those who work, and subjects under 30 years. Factor analysis of the STAI-T for total sample and by gender yielded two factors: the first representing a mood dimension and the second being related to worrying or cognitive aspects of anxiety. In order to study the relation between anxiety and depression measures, factor analysis of the combination of the 21 BDI items and the 20 STAI-T items was also carried out. The analysis resulted in two factors that were analyzed according to the tripartite model of anxiety and depression. Most of the BDI items (measuring positive affectivity and nonspecific symptoms of depression) were loaded on the first factor and four STAI-T items that measure positive affectivity. The remaining STAI-T items, all of them measuring negative affect, remained in the second factor. Thus, factor 1 represents a depression dimension and factor 2 measures a mood-worrying dimension. The findings of this study suggest that, although widely used as an anxiety scale, the STAI-T in fact measures mainly a general negative affect.

Insights into the physiological function of cellular prion protein

Prions have been extensively studied since they represent a new class of infectious agents in which a protein, PrPsc (prion scrapie), appears to be the sole component of the infectious particle. They are responsible for transmissible spongiform encephalopathies, which affect both humans and animals. The mechanism of disease propagation is well understood and involves the interaction of PrPsc with its cellular isoform (PrPc) and subsequently abnormal structural conversion of the latter. PrPc is a glycoprotein anchored on the cell surface by a glycosylphosphatidylinositol moiety and expressed in most cell types but mainly in neurons. Prion diseases have been associated with the accumulation of the abnormally folded protein and its neurotoxic effects; however, it is not known if PrPc loss of function is an important component. New efforts are addressing this question and trying to characterize the physiological function of PrPc. At least four different mouse strains in which the PrP gene was ablated were generated and the results regarding their phenotype are controversial. Localization of PrPc on the cell membrane makes it a potential candidate for a ligand uptake, cell adhesion and recognition molecule or a membrane signaling molecule. Recent data have shown a potential role for PrPc in the metabolism of copper and moreover that this metal stimulates PrPc endocytosis. Our group has recently demonstrated that PrPc is a high affinity laminin ligand and that this interaction mediates neuronal cell adhesion and neurite extension and maintenance. Moreover, PrPc-caveolin-1 dependent coupling seems to trigger the tyrosine kinase Fyn activation. These data provide the first evidence for PrPc involvement in signal transduction.

Evidence for a modulation of neutral trehalase activity by Ca2+ and cAMP signaling pathways in Saccharomyces cerevisiae

Saccharomyces cerevisiae neutral trehalase (encoded by NTH1) is regulated by cAMP-dependent protein kinase (PKA) and by an endogenous modulator protein. A yeast strain with knockouts of CMK1 and CMK2 genes (cmk1cmk2) and its isogenic control (CMK1CMK2) were used to investigate the role of CaM kinase II in the in vitro activation of neutral trehalase during growth on glucose. In the exponential growth phase, cmk1cmk2 cells exhibited basal trehalase activity and an activation ratio by PKA very similar to that found in CMK1CMK2 cells. At diauxie, even though both cells presented comparable basal trehalase activities, cmk1cmk2 cells showed reduced activation by PKA and lower total trehalase activity when compared to CMK1CMK2 cells. To determine if CaM kinase II regulates NTH1 expression or is involved in post-translational modulation of neutral trehalase activity, NTH1 promoter activity was evaluated using an NTH1-lacZ reporter gene. Similar ß-galactosidase activities were found for CMK1CMK2 and cmk1cmk2 cells, ruling out the role of CaM kinase II in NTH1 expression. Thus, CaM kinase II should act in concert with PKA on the activation of the cryptic form of neutral trehalase. A model for trehalase regulation by CaM kinase II is proposed whereby the target protein for Ca2+/CaM-dependent kinase II phosphorylation is not the neutral trehalase itself. The possible identity of this target protein with the recently identified trehalase-associated protein YLR270Wp is discussed.

Molecular identification of Sicilian (dß)º-thalassemia associated with ß-thalassemia and hemoglobin S in Brazil

We describe the clinical and molecular characteristics of two unrelated Brazilian families with an association of the Sicilian form of (dß)º-thalassemia with hemoglobin S and ß-thalassemia. Direct sequencing of the ß-globin gene showed only the hemoglobin S mutation in patient 1 and the ß-thalassemia IVS1-110 in patient 2. The other allele was deleted in both patients and PCR of DNA samples of the breakpoint region of both patients showed a band of approximately 1,150 bp, expected to be observed in the DNA of carriers of Sicilian (dß)º-thalassemia. The nucleotide sequence of this fragment confirmed the Sicilian deletion. There are few reports concerning the Hb S/(dß)º-thalassemia association and patient 2 is the first reported case of Sicilian type of (dß)º-thalassemia in association with ß-thalassemia documented at the molecular level.