MONOCLONAL PURIFIED FACTOR VIII:C (MONOCLATE) TREATMENT IN A PREVIOUSLY UNTREATED HAEMOPHILIA

1987 ◽  
Author(s):  
L Parapia ◽  
A Minford ◽  
J B Hamilton

Monoclate is a new generation of Factor VIII concentrate produced by purification using mouse monoclonal anti-Factor VIII:R antibody. As the- Factor VIII:C does not interact with the antibody it can be eluted by disrupting the Factor VIII:C - Factor VIII:R complex using a high concentration of calcium ions. The eluted Factor VIII:C is concentrated and purified. The method of manufacture has demonstrated efficacy in the elimination of infectious viral particles.The first “virgin” haemophiliac to be treated by this has completed 20 weeks follow-up. The patient, a child of 18 months with a Factor VIII:C level of 2.8%, was treated with 190 × 4 units of the Factor VIII concentrate for a severe cut of the lower lip.The HIV status has remained negative. The AST and ALT enzymes have remained within normal limits. Other parameters which have remained normal are Gamma GT, WBC and lymphocyte counts, T cell subsets and B cell ratios.The patient has remained well and no side effects have been noted. Mouse antibody titres are being carried out and the results will be presented at the conference.

1987 ◽  
Author(s):  
E D GOMPERTS ◽  
K WEINBERG

Three children with severe inherited bleeding disorders have been followed for a number of years at this center. One child (DOB 3/71) initially presented with mild hemophilia A, (Factor VIII 6%). He subsequently developed an inhibitor to Factor VIII (maximum 45 B. U.) and seroconverted to HIV+ Status 12/83. In 12/86 he had virtually lost his antibody response to infused Factor VIII (previously withheld), with a maximum increase in inhibitor titre to1 B. U. on challenge. In addition, his antitetanus antibody titre was very low at 0.01 u/ml earlier in theyear. His absolute T4 cell number at this time was very low at 64 and did not respond to skin antigen testing to PPD, tetanus and Candida.The second patient (severe hemophilia A DOB 7/76) had seroconvertedto HIV+ Status in 9/78. This child has lost his a-HBs seropositive status with an absolute T4 count of 239. His current anti-tetanus titre is 0.01 u/ml.The third patient (von Willebrand disease, Type III, DOB 7/74) seroconverted to HIV+E status by 5/83. His T4 absolute numbers have fallen to 53. His anti-tetanus antibody titre has fallen to extremely low levels (0.01 u/ml), and this failed to respond to re-immunization with tetanus toxoid. These three patients indicate that previously immunized children may lose their immune status and their ability to respond to recall antigens. It is pertinent to note that lymphocytes from all 3 patients failed to respond mitogenically in vitro to tetanus antigen pari passu with the observed very low anti-tetanus antibody titres. These phenomena would indicate that these patients are probably susceptible to previously preventable infectious agents including poliovirus, measles, mumps, rubella, diphtheria, tetanus and hepatitis B virus.


1985 ◽  
Vol 54 (03) ◽  
pp. 665-668 ◽  
Author(s):  
F Rodeghiero ◽  
G C Castaman ◽  
T Chisesi ◽  
A De Rossi ◽  
O Dalla Gassa ◽  
...  

SummaryA 1-year follow-up study of the T-cell subset abnormalities was carried out in 16 severe haemophilia A patients, treated “on demand” with an average amount of 500 U/kg/yr of factor VIII concentrate (group A) and in 15 mild haemophiliacs or von Willebrand patients treated only sporadically with less than 3000 U of factor VIII and no longer exposed to any other blood component in the 2 years preceding the beginning of the study (group B).In group A, 50% and 70% of patients showed a reduced or inverted T 4/T 8 ratio, respectively, at the beginning and at the end of follow-up. These values were of 30% and 20% in patients of group B, suggesting a long-lasting effect of concentrate therapy on T-cell subsets. The low T 4/T 8 ratio was mainly due to an increase of suppressor cells. None of the patients was found positive for anti HTLV-I, whereas 3 patients, all belonging to the group A, showed antibodies against HTLV-III. Thus, in these patients, HTLV-III seems not to be the only cause of low T 4/T 8 ratio.


1976 ◽  
Vol 35 (03) ◽  
pp. 510-521 ◽  
Author(s):  
Inga Marie Nilsson

SummaryThe incidence of living haemophiliacs in Sweden (total population 8.1 millions) is about 1:15,000 males and about 1:30,000 of the entire population. The number of haemophiliacs born in Sweden in 5-year periods between 1931-1975 (June) has remained almost unchanged. The total number of haemophilia families in Sweden is 284 (77% haemophilia A, 23% haemophilia B) with altogether 557 (436 with A and 121 with B) living haemophiliacs. Of the haemophilia A patients 40 % have severe, 18 % moderate, and 42 % mild, haemophilia. The distribution of the haemophilia B patients is about the same. Inhibitors have been demonstrated in 8% of the patients with severe haemophilia A and in 10% of those with severe haemophilia B.There are 2 main Haemophilia Centres (Stockholm, Malmo) to which haemophiliacs from the whole of Sweden are admitted for diagnosis, follow-up and treatment for severe bleedings, joint defects and surgery. Minor bleedings are treated at local hospitals in cooperation with the Haemophilia Centres. The concentrates available for treatment in haemophilia A are human fraction 1-0 (AHF-Kabi), cryoprecipitate, Antihaemophilic Factor (Hyland 4) and Kryobulin (Immuno, Wien). AHF-Kabi is the most commonly used preparation. The concentrates available for treatment in haemophilia B are Preconativ (Kabi) and Prothromplex (Immuno). Sufficient amounts of concentrates are available. In Sweden 3.2 million units of factor VIII and 1.0 million units of factor IX are given per year. Treatment is free of charge.Only 5 patients receive domiciliary treatment, but since 1958 we in Sweden have practised prophylactic treatment of boys (4–18 years old) with severe haemophilia A. At about 5-10 days interval they receive AHF in amounts sufficient to raise the AHF level to 40–50%. This regimen has reduced severe haemophilia to moderate. The joint score is identical with that found in moderate haemophilia in the same age groups. For treatment of patients with haemophilia A and haemophilia B complicated by inhibitors we have used a large dose of antigen (factor VIII or factor IX) combined with cyclophosphamide. In most cases this treatment produced satisfactory haemostasis for 5 to 30 days and prevented the secondary antibody rise.


Infection ◽  
2021 ◽  
Author(s):  
Ali Hamady ◽  
JinJu Lee ◽  
Zuzanna A. Loboda

Abstract Objectives The coronavirus disease 2019 (COVID-19), caused by the novel betacoronavirus severe acute respiratory syndrome 2 (SARS-CoV-2), was declared a pandemic in March 2020. Due to the continuing surge in incidence and mortality globally, determining whether protective, long-term immunity develops after initial infection or vaccination has become critical. Methods/Results In this narrative review, we evaluate the latest understanding of antibody-mediated immunity to SARS-CoV-2 and to other coronaviruses (SARS-CoV, Middle East respiratory syndrome coronavirus and the four endemic human coronaviruses) in order to predict the consequences of antibody waning on long-term immunity against SARS-CoV-2. We summarise their antibody dynamics, including the potential effects of cross-reactivity and antibody waning on vaccination and other public health strategies. At present, based on our comparison with other coronaviruses we estimate that natural antibody-mediated protection for SARS-CoV-2 is likely to last for 1–2 years and therefore, if vaccine-induced antibodies follow a similar course, booster doses may be required. However, other factors such as memory B- and T-cells and new viral strains will also affect the duration of both natural and vaccine-mediated immunity. Conclusion Overall, antibody titres required for protection are yet to be established and inaccuracies of serological methods may be affecting this. We expect that with standardisation of serological testing and studies with longer follow-up, the implications of antibody waning will become clearer.


2021 ◽  
Vol 7 (1) ◽  
Author(s):  
George Deryabin ◽  
Simonas Grybauskas

Abstract Background The purpose of this study was to analyze medium-to-long-term implant success and survival rates, and lower lip sensory disturbance after placement of dental implants with simultaneous inferior alveolar nerve (IAN) repositioning. Methods Fifteen patients (3 men, 12 women) treated in two centers were included in this retrospective study. The ages of the participants ranged from 19 to 68. A total of 48 dental implants were placed in 23 posterior mandibular segments simultaneously with IAN transposition or lateralization. The residual bone above the IAN ranged from 0.5 to 7.0 mm. Crestal bone changes were measured using cone beam computed tomography (CBCT) images. Disturbance of the IAN was evaluated subjectively using a modified questionnaire. Results The healing process was uneventful in fourteen patients. In one patient, spontaneous fracture of the operated mandible occurred on tenth day after the surgery. The implant in the fracture line was removed at the time of open reduction and fixation. One more implant was lost after 5 years of loading. Therefore, the overall dental implant survival rate was 95.8%, whereas all implants in function were judged as successful after a follow-up period of 1 to 10 years. Transient neurosensory disturbances (ND) were observed in all patients who underwent IAN lateralization and IAN transposition. At follow-up times of 3 years, 5 years, and 10 years, weak hypoesthesia remained in two subjects treated with IAN transposition. None of the patients developed neuropathic pain after the procedure. Conclusions Within the limitations of this study, we conclude that reconstruction of severely resorbed mandibles with dental implants in conjunction with IAN repositioning is an effective and reliable technique. Although neurosensory disturbances are the most common complication after surgery, they tend to resolve over time. Advanced surgical skills are required to perform this technique.


2021 ◽  
Vol 11 (13) ◽  
pp. 5819
Author(s):  
Gianluca Botticelli ◽  
Marco Severino ◽  
Gianmaria Fabrizio Ferrazzano ◽  
Pedro Vittorini Velasquez ◽  
Carlo Franceschini ◽  
...  

Oral mucocele is a benign cystic exophytic lesion affecting the minor salivary gland and is especially present in pediatric patients (3% under 14 years). It is characterized by an extravasation or retention of fluid or mucus in the submucosal tissue of the minor salivary glands. Several surgical techniques have been proposed over the years, including the excision of the mucocele by using the injection of a hydrocolloid impression material in the light of the cyst to prevent the collapse of the cystic wall and solidify the lesion, resulting in a better cleavage plan. The combined clinical approach between the combination of Shira’s technique and the surgical excision of the cystic lesion results in a conservative surgical removal of the lesion. Here, we reported the removal of a labial mucocele in a 14-year-old male patient, using the injection of a hydrocolloid impression material. At a 12 months follow up, the patient showed complete healing of the surgical site, showing a pinkish lip lining mucosa without scarring or recurrence of the primary lesion. The combined therapeutic approach between Shira’s technique and surgical excision allows a safe and predictable excision of the labial mucocele, minimizing the risk of recurrence.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
T Jimba ◽  
M Ikutomi ◽  
D Nishijyo ◽  
M Yamasaki ◽  
A Shindou ◽  
...  

Abstract Background Edge restenosis still occurs after stent implantation, even by using new generation drug-eluting stents (DES) considered to have favorable biomechanical properties. Mechanical stress imposed on the stent edge are thought to be aggravated by hinge motion at a point between the stented and unstented segments, inducing chronic local inflammation and neointimal overgrowth. Purpose The aim of this study was to investigate the association between the development of edge restenosis and hinge motion in right coronary artery (RCA) where the excessive vessel movement is commonly observed. Methods Among consecutive 650 lesions in RCA where new generation DESs were implanted between 2009 and 2019, 427 serial lesions with sets of angiographies at baseline and follow-up (6–18 month) were included. In addition to conventional quantitative angiography analysis, hinge angle at stent edges was measured (Fig. 1). All the appropriate data for intravascular imaging were analyzed for both stent edges and reference segments. Results Binary restenosis occurred in 43 lesions, and 39 of them were referred to re-intervention. Fifty five percent of them were related to stent edges (15 at proximal and 9 at distal edges). Classical risk factors including diabetes and hemodialysis were more prevalent in the restenosis group (p<0.05). Hinge angle was statistically larger in edge restenosis group than body restenosis or no restenosis group (17.3° vs 11.6° vs 10.6°, p<0.001, Fig. 2). In per-edge analysis, hinge angle, dissection and residual plaque ratio were the independent predictors for binary restenosis (Table 1) with the optimal cut-off value of hinge angle 11.5°. The coexistence of excessive hinge angle and residual plaque burden had an amplified effect on the angiographic stenotic progression at stent edge (p for interaction <0.001) and the incidents of binary restenosis (16.7% vs 1.7% p<0.01, Figs. 3,4). Conclusion Substantial stress determined by angulation at the stent edge and its interaction with residual plaque can be considered as one of the plausible mechanisms for edge restenosis. For tortuous RCA lesions, it would be important to decide the stent-landing zone for minimizing hinge motion and optimize the future stent design. Funding Acknowledgement Type of funding source: None


2010 ◽  
Vol 22 (3-4) ◽  
pp. 131-139 ◽  
Author(s):  
Paolo Caffarra ◽  
Letizia Concari ◽  
Simona Gardini ◽  
Sabrina Spaggiari ◽  
Francesca Dieci ◽  
...  

A patient who suffered a transient global amnesia (TGA) attack underwent regional cerebral blood flow (rCBF) SPECT imaging and neuropsychological testing in the acute phase, after one month and after one year. Neuropsychological testing in the acute phase showed a pattern of anterograde and retrograde amnesia, whereas memory was within age normal limits at follow up. SPECT data were analysed with a within subject comparison and also compared with those of a group of healthy controls. Within subject comparison between the one month follow up and the acute phase detected increases in rCBF in the hippocampus bilaterally; further rCBF increases in the right hippocampus were detected after one year. Compared to controls, significant hypoperfusion was found in the right precentral, cingulate and medial frontal gyri in the acute phase; after one month significant hypoperfusion was detected in the right precentral and cingulate gyri and the left postcentral gyrus; after one year no significant hypoperfusion appeared. The restoration of memory was paralleled by rCBF increases in the hippocampus and fronto-limbic-parietal cortex; after one year neither significant rCBF differences nor cognitive deficits were detectable. In conclusion, these data indicate that TGA had no long lasting cognitive and neural alterations in this patient.


2018 ◽  
Vol 12 (supplement_1) ◽  
pp. S137-S138
Author(s):  
B Roosenboom ◽  
C Smids ◽  
P Wahab ◽  
M Groenen ◽  
E Van Koolwijk ◽  
...  

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