Bioclinical features of haemophilia patients in Benin in 2023: Towards better care.

Objective: To analyse the demographic, clinical and laboratory data of Beninese patients with haemophilia.

Method: A prospective survey was conducted in three different hospitals of Benin from April 2021 to March 2022, to analyse clinical and biological features of patients with haemophilia previously diagnosed or identified based on personal/family history.

Results: A total of 101 patients were studied, 97 with haemophilia A and 4 with haemophilia B, including 26 new cases identified after family investigation.

Urine Protein/Creatinine Ratio in Thrombotic Microangiopathies: A Simple Test to Facilitate Thrombotic Thrombocytopenic Purpura and Hemolytic and Uremic Syndrome Diagnosis.

Background: Early diagnosis of thrombotic thrombocytopenic purpura (TTP) versus hemolytic and uremic syndrome (HUS) is critical for the prompt initiation of specific therapies.

Objective: To evaluate the diagnostic performance of the proteinuria/creatininuria ratio (PU/CU) for TTP versus HUS.

Patients/methods: In a retrospective study, in association with the "French Score" (FS) (platelets < 30 G/L and serum creatinine level < 200 µmol/L), we assessed PU/CU for the diagnosis of TTP in patients above the age of 15 with thrombotic microangiopathy (TMA).

Real Life Population Pharmacokinetics Modelling of Eight Factors VIII in Patients with Severe Haemophilia A: Is It Always Relevant to Switch to an Extended Half-Life?

We retrospectively analysed the data files of 171 adults and 87 children/adolescents with severe haemophilia, except for 14 patients (moderate; minor) (1), to develop a global population pharmacokinetic (PK) model for eight factors VIII (FVIII) that could estimate individual PK parameters for targeting the desired level of FVIII activity (FVIII:C); and (2) to compare half-life (HL) in patients switching from a standard half-life (SHL) to an extended half-life (EHL) and evaluate the relevance of the switch. One-stage clotting assay for the measurement of FVIII activity (FVIII:C, IU/mL) was used for population PK modelling. The software, Monolix version 2019R1, was used for non-linear mixed-effects modelling.

Multicentre pharmacokinetic evaluation of rFVIII-Fc (efmoroctocog alfa) in a real life and comparison with non-extended half-life FVIII concentrates.

The use of enhanced half-life (EHL) FVIII has improved the quality of prophylaxis in haemophilia A, but with a benefit that may vary from one patient to another. We analysed the pharmacokinetic data obtained with efmoroctocog alfa (rFVIII-Fc) in 114 patients and, in 47 cases, compared them to those previously measured with non-EHL FVIII. The in vivo recovery (IVR) of rFVIII-Fc measured with one stage clotting assay (OSA) and chromogenic assay (CSA) was 2.

Pain requires processing - How the experience of pain is influenced by Basic Body Awareness Therapy in patients with long-term pain.

Background And Purpose: Long-term pain is common and entails large costs to society. One physiotherapy treatment with documented positive effects for patients with long-term pain is Basic Body Awareness therapy (BBAT). However, studies are lacking about patients' experience of BBAT's influence on their pain.

Incidence of obstetrical thrombotic thrombocytopenic purpura in a retrospective study within thrombocytopenic pregnant women. A difficult diagnosis and a treatable disease.

Background: Thrombotic thrombocytopenic Purpura (TTP) defined as ADAMTS-13 (A Disintegrin And Metalloprotease with ThromboSpondin type 1 domain 13) activity <10 % is a rare aetiology of thrombocytopenia during pregnancy, although the precise incidence is unknown. During pregnancy, the diagnosis of TTP is crucial as it has high feto-maternal morbidity-mortality and requires urgent plasma exchange. The purpose of this study was to assess the incidence of TTP retrospectively and to describe case presentations and follow-up.

Severe transient ADAMTS13 deficiency in pneumococcal-associated hemolytic uremic syndrome.

Thrombotic microangiopathies comprise different entities, including hemolytic uremic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP), and several other conditions. TTP is characterized by hemolytic anemia, thrombocytopenia, and multiorgan failure. TTP is the result of severe von Willebrand factor multimer cleaving protease (ADAMTS13) deficiency that is either inherited or the result of acquired autoantibodies.

Successful treatment with rituximab for acute refractory thrombotic thrombocytopenic purpura related to acquired ADAMTS13 deficiency: a pediatric report and literature review.

Objective: To report the case of a child with severe autoimmune thrombotic thrombocytopenic purpura (TTP) resistant to plasma exchange and steroids who was successfully treated with rituximab.

Design: Case report and review of the literature on pediatric acquired TTP. The report was approved by an independent local ethics committee.

Optimal management of an aneurysmal subarachnoid hemorrhage in a patient with known factor XI deficiency: a case report.

The authors report a rare case of an acute cerebral aneurysm rupture in a patient with a known factor XI deficiency. Aneurysmal subarachnoid hemorrhage (SAH) accounts for a high mortality and morbidity rate. When SAH is associated with an inherited coagulation disorder such as hemophilia C, an unexpected and possible increase in hemorrhagic stroke and increase in bleeding during surgery and in the postoperative period could lead to an extremely bad outcome.

Postoperative thrombotic microangiopathy following radical cystectomy for bladder cancer.

This report describes the perioperative management of disseminated intravascular coagulation occurring abruptly during a planned cystectomy for non-metastatic bladder papillary carcinoma. Peroperatively, profuse bleeding and an acute decrease in blood pressure were effectively treated by blood transfusions and fresh frozen plasma. Haematological tests indicated the presence of disseminated intravascular coagulation.

Occipital hypoperfusion on SPECT in dementia with Lewy bodies but not AD.

Objective: To compare regional cerebral blood flow (rCBF) changes using 99mTc-hexamethylpropyleneamine oxime (99mTc-HMPAO) SPECT in subjects with dementia with Lewy bodies (DLB) and AD and in normal age-matched control subjects; to examine the utility of SPECT changes in the differential diagnosis of AD and DLB.

Method: Whole-brain SPECT scans were acquired using a single-headed rotating gamma camera (IGE CamStar XR/T) in elderly subjects with consensus criteria DLB (n = 23; mean age = 79.4 years), National Institute of Neurological and Communicative Disorders and Stroke-Alzheimer's Disease and Related Disorders Association AD (n = 50; 81.

Does the ICD 10 classification accurately describe subtypes of borderline personality disorder?

The aim of the paper is to explore whether the division of borderline personality disorder, as described in the DSM classification, into impulsive and borderline subtypes of emotionally unstable personality disorder in the ICD classification of personality disorder, is a valid division. The self-report questionnaire responses of 288 referrals to a personality disorder service were rated on each of the eight criteria for DSM-III-R diagnosis of borderline personality disorder. Factor analysis identified two factors; factor one closely corresponds with the borderline subtype of ICD10, whilst factor two closely corresponds with the impulsive subtype of ICD10.

A voxel-based analysis of cerebral perfusion in dementia and depression of old age.

Thirty-nine elderly depressed patients as well as 15 demented patients with Alzheimer's disease and 11 healthy volunteers were imaged at rest with a high resolution single-slice 12-detector head scanner (SME-Neuro 900) and the cerebral perfusion marker 99mTc-Exametazime (HM-PAO). Statistical parametric maps were computed to compare early- and late-onset depressed, Alzheimer patients and healthy volunteers and to examine associations between regional perfusion and clinical and MRI variables. Patients with late-onset depression showed reductions in temporal lobe perfusion compared with early-onset depression and controls.

Autoantibodies directed against phospholipids or human beta 2-glycoprotein I in HIV-seropositive patients: relationship with endothelial activation and antimalonic dialdehyde antibodies.

Background: We investigated the possible role of antiphospholipid (APA) and anti-human 2-glycoprotein I (beta2-GPI) antibodies (Ab) in thrombosis and atherosclerosis in human immunodeficiency (HIV)-positive patients, in whom they seem to be more frequent.

Methods: We measured APA and anti-beta2-GPI Ab in 58 HIV-positive patients together with markers of disease progression, circulating beta2-GPI, plasma lipids, biological markers of endothelial activation and integrity (plasma thrombomodulin, von Willebrand factor, vascular cell adhesion molecule 1) and with antimalonic dialdehyde antibodies (anti-MDA Ab).

Results: We found a 41% frequency of IgG APA in the HIV-positive patients.

Temporal lobe abnormalities in dementia and depression: a study using high resolution single photon emission tomography and magnetic resonance imaging.

Objectives: Perfusion SPECT and MRI were used to test the hypothesis that late onset depression is associated with brain abnormalities.

Methods: Forty depressed patients (DSM-III-R major depressive episode, not demented at two year follow up) were recruited who were either drug free, or on a stable dose of antidepressants for at least three weeks, as well as 22 demented patients (DSM-IIIR and NINCDS/ADRDA criteria for probable Alzheimer's disease). Patients were imaged at rest with a high resolution single slice 12 detector head scanner (SME-Neuro 900) and the cerebral perfusion marker 99mTc-exametazime (HM-PAO).

Performance on the delayed word recall test (DWR) fails to differentiate clearly between depression and Alzheimer's disease in the elderly.

Background: The differential diagnosis of early dementia of the Alzheimer's type from depression in the elderly is often made difficult by the presence of significant memory impairment in depressed patients. The Delayed Word Recall test (DWR) was developed to facilitate the early diagnosis of Alzheimer's disease. The DWR involves: (a) repeated elaborate encoding of ten separate words; (b) a filled delay; (c) delayed free recall.