Background: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired hematologic condition which could be revealed by deep venous thrombosis. It could be fatal unless correctly treated.
Case report: We report here the case of a 28 year-old male with no medical history who was admitted to the emergency room for severe abdominal pain. Computerized Tomography angiography (CT) scan revealed portal vein thrombosis. Laboratory findings showed pancytopenia with severe regenerative normocytic anemia resulting in PNH. Because of the lack of Eculizumab, treatment was first based on curative anticoagulation until bone marrow transplant, with no success.
Conclusion: PNH remains a severe disease with bad prognosis unless treated with Eculizumab.
Pierre Maria and Sainton syndrome or cleido-cranial dysplasia (CCD) is a rare syndrome presenting an autosomal pattern of inheritance, characterized by characterized by a triad: clavicular aplasia, delayed ossification of the fontanelles and sutures of the vault of the skull. To these may be added multiple dental inclusions.
The measures put in place by many governments around the world to fight the spread of COVID-19 have drastically reduced visits to diabetes centres. To keep providing people with diabetes the professional support they need and reduce the inconvenience caused by the interruption of traditional assistance, the typical control visit can be carried out by virtual visit, telemedicine (TM) that should have the necessary characteristics to provide a correct execution. Especially this is important for pregnant women first diagnosed with gestational diabetes mellitus (GDM), they have the necessity to frequent checks in a short time due to the pregnancy.
Pachydermoperiostosis, also known as Primary Hypertrophic Osteoarthropathy (PHO), is a rare genetic disorder. The three main features are: enlarged fingertips (clubbing), thickened facial skin (pachydermia), and excessive sweating (hyperhidrosis). PHO is characterized by problems with skin and bone growth. Patients with PHO usually have coarse facial features with oily, thick, grooved skin on the face, joint pain, enlarged fingertips and toes, and hyperhidrosis of the hands and feet. Symptoms vary individually; however, men generally present with more severe manifestations. X-rays can help check for features that are not noticeable to the naked eye. There are two genes that are associated with PHO: the HPGD gene, located on the long arm of chromosome 4 at 4q34.1, and the SLCO2A1 gene, located on the long arm of chromosome 3 at 3q22.1 - q22.2. Mutations in the HPGD gene are inherited in an autosomal recessive manner, and the condition is sometimes abbreviated as PHOAR1 or Touraine-Solente-Gole syndrome.
Objectives: Type-2 diabetes mellitus, caused by impaired secretion of insulin, is becoming one of the health hazardous threats to human lives across the world. Its prevalence is rising with time. In this study, 2750 phytochemicals, that are considered to have great ability to eliminate diseases caused by different viruses and bacteria, are obtained from different medicinal plants and discovery of inhibitors through in silico method was performed against Dipeptidyl peptidase-4 (DPP4).
Method: The pharmacological assessment and pharmacokinetics of phytochemicals, molecular docking and density functional theory (DFT) analysis helped to explore the inhibitory action of phytochemicals against DPP4. Total forty-nine phytochemicals were screened initially to reduce the number of compounds to be analyzed further based on a threshold of binding affinity ≥ -5.5 kcal/mol and were considered for further computational studies to analyze their inhibitory effects for DPP4. For comparison and validation of the results of present study, various previously reported and experimentally validated compounds were docked with the DPP4. For these dockings, binding affinity was predicted and compared with those of phytochemicals to check if these phytochemicals are competent enough to be used as an inhibitor in the treatment of diabetes mellitus in the future.
Results: Only four phytochemicals showed binding affinity greater than those of experimentally validated compounds. These included two phytochemicals from Silybum marianum, i.e. Diprenyleriodictyol and Taxifolin and while other two phytochemicals from Santolina insularis and Erythrina Varigatae i.e. Papraline and Osajin respectively. The reactivity levels for these four phytochemicals with the binding site residues of DPP4 were obtained by DFT based analysis, in which ELUMO, EHOMO and band energy gap were computed.
Conclusion: Based on these results, it is concluded that these four phytochemicals, after passing through in vitro and in vivo validation, can be utilized as potential DPP4 inhibitors as they have strong properties as compared to those of various experimentally validated inhibitors.
Nuclear Medicine is an integral part of modern healthcare. The use of radioactive nuclides tagged biomolecules, evaluating their distribution in human bodies by SPECT or PET systems, provides longitudinal sets of volumetric and quantitative images that can be used to diagnose a wide range of disease and/or assess response to disease specific treatments [1].
Background: The treatment of chronic lymphoid leukemia currently uses news drugs which are more expensive in our countries. Its why, the results of chemotherapy remains a challenge in our sector.
Aims: To evaluate the place of polychemotherapy in the treatment of chronic lymphoid leukemia in black Africa.
Methods: It was a prospective, descriptive, analytic and non-comparative study, concerning the records of patients with chronic lymphoid leukemia treated and followed at the department of clinical hematology in Abidjan.
Results: We included 56 patients. The average age was 62 years with extremes of 38 and 84 years. The sex ratio was 0.8 in favor of female. The clinical signs noted a tumor syndrome among which splenomegaly, classified stage III (46, 43%) and adenopathy (64, 29%). Biologically, we observed a blood lymphocytosis (50%), an anemia (39.29%) and a thrombocytopenia (62.50%). The majority of patients were classified stage A of BINET (51.79%). The COP protocol (44.64%) and the monochemotherapy with chlorambucil (39.29%) were the most used. The therapeutic response of polychemotherapy was low (12.5%) compared to 35, 71% for monochemotherapy (p = 0.0001) with overall survival significantly better in monochemotherapy. The outcome of patients used polychemotherapy were more adverse that of patients used chlorambucil alone (p = 0,003). The overall probability of survival at 12 months was 90, 9% for patients who used monochemotherapy and 63, 4% for polychemotherapy.
Conclusion: Polychemotherapy in chronic lymphoid leukemia of black African has an adverse therapeutic response hence the interest of using new therapeutic possibilities.
A 42 years old gentleman who was a known case of Psoriasis vulgaris since last 5 years presented to the Rheumatology clinic with inflammatory arthritis predominantly involving the joints of the upper extremities. Musculoskeletal examination of both hands revealed dactylitis and distal interphalangeal joint arthritis. He had a shortened right ring finger with excessive transverse skin folding suggestive of an Opera-Glass hand
Background: Tetanus continues to threaten the survival of children in spite of it being a vaccine preventable disease. The objective of this study was to determine the prevalence of post-neonatal tetanus, review the vaccination of affected children, complications encountered and the outcome among affected children in a tertiary health institution in southwestern Nigeria.
Methods: The study was a retrospective study. Case notes of children outside neonatal life admitted to the Paediatric ward with clinical diagnosis of tetanus between January 2012 and October 2018 were retrieved and evaluated to identify socio-demographic and clinical characteristics. A review of the immunization history and cards was done where the immunization cards were available.
Results: 21children with post-neonatal tetanus were admitted over a period of six years (November 2012 to October 2018) with a prevalence of 0.3%. The M:F was 3.2:1. The mean age in years was 10.14 ±3.44 while the age range of the subjects was 4 to 16years. None of the patients had booster doses of tetanus toxoid (TT) outside the infancy period. Nine (42.9%) subjects had no previous TT vaccination, 2 (9.5%) had 3 doses of TT vaccine in infancy but developed tetanus at age ≥9 years, 1(4.8%) subject had a dose of TT while the remaining 9subjects had no proof of previous TT vaccination. The percentage mortality was 19% (4 out of 21). All the patients that died had no prior record of TT vaccination. Complications identified included laryngeal spasm and autonomic dysfunction.
Conclusion: Post-neonatal tetanus is still common in our locality because booster doses of Tetanus Toxoid are not part of the national immunization schedule. Complete dose of tetanus toxoid vaccination during infancy and booster doses at school entry is necessary and should be part of school health programme to forestall post-neonatal tetanus
Background: Congenital Vascular malformation relatively rare and extremely varied clinical presentations. The purpose of our study was to present our initial experience of embolization in a series of 26 patients with congenital vascular malformation to assess retrospectively the results and the complications of ethanol and coils embolization treatment of these patients.
Methods: Retrospective trial, the study group consisted of 26 patients with congenital vascular malformations. Transcatheter arterial embolization by ethanol or coils were performed, Therapeutic outcomes were established by evaluating the clinical outcome of symptoms and signs, as well as the degree of devascularization at follow-up angiography.
Results: Between November 2014 and March 2018, 26 consecutive patients (3 male, 23 female) at Alshifa Hospital - Cardiac Catheterization Center with congenital vascular malformations in the body and extremities underwent staged ethanol or coils embolization. The mean age of the patients was 25 years (age range, 6– 59 years). Ethanol embolization was administrated in 16 patients, coil embolization in 9 patients and graft stent in one patient. The side effect such as pain, pulsation, and bruit in most of the patients were obtained. The reduction of redness, swelling, and warmth was achieved in all of the patients, According to the angiographic findings, congenital vascular malformation were devascularized 100% in 12 patients, 50% to 99% in 11 patients, less than 50% in 3 patients. The most common complications were reversible skin necrosis.
Conclusion: Transcatheter embolization by ethanol or coils has proved efficacious and safe in the treatment of congenital vascular malformation of the body and extremities but with acceptable risk of complications
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