Orofacial anatomical and occlusal changes in patients with sickle cell disease in Kuwait.

Objective: Sickle cell disease (SCD) is the most common hemoglobinopathy where morphologic changes to red blood cells affect the development of hard and soft tissues. The purpose of this study is to identify the craniofacial characteristics and maxillomandibular relationship in SCD patients and compare with unaffected subjects, through cephalometric radiographic assessment.

Materials And Methods: The study included 44 Kuwaiti SCD patients (20 female, 24 male) as well as 44 age and gender matched controls.

Pattern of Renal Blood Flow and Renovascular Parameters in Adult Patients With Sickle Cell Disease.

Objectives: To evaluate renal blood flow patterns and renovascular parameters in adult patients with sickle cell disease (SCD) without laboratory evidence of renal impairment.

Methods: Sixty-five steady-state adult patients with SCD (50 hemoglobin SS [HbSS], 12 HbSβ , and 3 HbSD) and 30 age- and sex-matched healthy controls were studied. The kidney length, echo pattern, peak systolic velocity (PSV), end-diastolic velocity, renal-to-aortic ratio, resistive index (RI), acceleration time (AT), and renal vein velocity were acquired, recorded, and analyzed with a 1-5-MHz curvilinear transducer through the abdomen.

Right Testicular Infarction Secondary to Spontaneous Testicular Vein Thrombosis in a Child: Case Report and Literature Review.

Objectives: To describe a case of right testicular vein thrombosis in a child and to review the literature describing similar cases and their management.

Methods: An extensive literature review of the English language literature was conducted using the following databases: PubMed, ScienceDirect and Google Scholar.

Results: Six cases were reported previously and summarized in this article.

HWA: Hypoferritinemia without anemia a hidden hematology disorder.

Introduction: Anemia is a condition, in which the number of red blood cells (RBC) and the hemoglobin (Hb) is insufficient to meet the body's physiologic needs. Hypoferritinemia without anemia (HWA) could cause masked clinical manifestations as general weakness, easy fatigability, depressed mode, and hair loss but with normal complete blood count (CBC) parameters. HWA is deferent from iron deficiency anemia (IDA) because there is no reduction in the RBC, Hb, or any of the other parameters of the CBC.

Long Standing Eculizumab Treatment without Anticoagulant Therapy in High-Risk Thrombogenic Paroxysmal Nocturnal Hemoglobinuria.

Paroxysmal nocturnal hemoglobinuria (PNH) is an ultra-orphan disease affecting all hematopoietic cell types. The abnormality of red blood cells in this disease predisposes to intravascular complement-mediated hemolysis. Eculizumab is an orphan drug used to treat this rare disease.

Drug-Immune Thrombocytopenia with Thrombosis versus Heparin-Induced Thrombocytopenia: A Critical Clinical Controversy.

Heparin-induced thrombocytopenia (HIT) is a type of drug-induced immune thrombocytopenia (DITP). DITP is a rare and challenging clinical issue, especially when it is associated with thrombosis. A 62-year-old woman was admitted to our institution with end-stage renal failure.

Precision of SPECT/CT Allows the Diagnosis of a Hidden Brodie's Abscess of the Talus in a Patient with Sickle Cell Disease.

Brodie's abscess is a rare subacute osteomyelitis that can be found in sickle cell disease along with other bone complications. A 21-year-old female with sickle cell disease was presenting frequently to the medical casualty department for painful vasoocclusive crises and for persistent ankle pain and swelling. Hybrid imaging with single-photon emission computed tomography-computed tomography (SPECT-CT) incidentally revealed Brodie's abscess in the talus bone of the ankle, causing persisting long-standing pain.

Successful nilotinib treatment in a child with chronic myeloid leukemia.

A 16-year-old female was diagnosed incedentally with chronic myeloid leukemia (CML) in the chronic phase. She showed complete remission after 3 months of nilotinib treatment. CML is a rare malignant neoplasm in pediatric age.

Successful management of severe refractory acquired immune bleeding disorder: Prior to insisting surgery.

Introduction: Acquired bleeding disorders are rare and may be missed before surgery. Additionally, they may be refractory to conventional treatments.

Presentation Of Case: A 50-year-old patient experienced prolonged post-operative bleeding when his bleeding disorder was missed prior to his undergoing inguinal herniorrhaphy.

Severe thrombocytopenia in a patient with hepatitis C treated with eltrombopag from off-label drug use to on-label drug use: a case report.

Introduction: Off-label drug use refers to drug use beyond the specifications authorized for marketing. Eltrombopag is a new thrombopoietin receptor agonist which was used successfully in this critical case of thrombocytopenia associated with hepatitis C infection before it became an approved drug for such cases.

Case Presentation: A 56-year-old Kuwaiti woman with hepatitis C virus infection was treated with pegylated interferon α-2a and ribavirin, laboratory test results prior to therapy were within normal values.

Splenectomy complicated by sustained extreme thrombocytosis and extensive portosplenomesenteric vein thrombosis in pyrimidine 5'-nucleotidase deficiency.

Reactive and redistributional thrombocytosis is a well-known postsplenectomy occurrence .Usually it is transient and it rarely reaches extreme levels. We report a rare case of haemolytic anaemia where splenectomy was carried out following trauma to a massively enlarged spleen and was followed by extreme sustained thrombocytosis associated with extensive portal, splenic and mesenteric vein thrombosis despite standard antithrombotic prophylaxis.

Can lenalidomide play a role in the management of scleritis?

Lenalidomide is an immunomodulatory agent that was approved for the treatment of a monoclonal bone marrow disorders, myelodysplastic syndrome del(5q)(MDS del(5q)), in 2005; the drug was subsequently also approved for the treatment of refractory multiple myeloma, a bone marrow malignancy of the B-lymphocyte lineage. The purpose of this study is to report a case of MDS del(5q) in a female patient, which was most likely secondary to the immunosuppressive drugs that the patient was taking for scleritis. After lenalidomide treatment, the patient's haematological symptoms rapidly resolved and she became transfusion independent, with normal haemoglobin levels.

Complete Remission of del(5q) Myelodysplastic Syndrome after 7 Days of Lenalidomide Therapy Gives an Alert!

Myelodysplastic syndrome (MDS) refers to a group of haematological, monoclonal disorders. A 50-year-old woman was diagnosed with MDS 5q deletion syndrome [del(5q)], becoming dependent on blood transfusion after long-term treatment with cytotoxic drugs for chronic scleritis. Lenalidomide therapy (10 mg/day) led to profound pancytopaenia, followed by recovery of her blood cell counts.

Use of romiplostim in a hemodialysis patient with primary immune thrombocytopenia.

Objective: To present the case of a patient with primary immune thrombocytopenia (ITP), renal impairment, and chronic hepatitis C virus (HCV) infection who was treated with platelet transfusions, intravenous immunoglobulin (IVIG), corticosteroids, eltrombopag, rituximab, and romiplostim in an attempt to raise platelet counts to a clinically acceptable level.

Case Summary: A 71-year-old man with end-stage renal disease (ESRD) was on maintenance hemodialysis and had long-term diabetes mellitus, chronic obstructive pulmonary disease, and other comorbidities. He was admitted with epistaxis, severe thrombocytopenia, and a platelet count of 4 × 10(9)/L.

Lymphocyte subsets in healthy adult Kuwaiti Arabs with relative benign ethnic neutropenia.

Relative and absolute neutropenia is frequently seen in the healthy adult Kuwaiti Arab population. Fluorescent monoclonal antibody labelling followed by flow cytometry was used to determine the lymphocyte subsets in 48 normal healthy individuals in the Kuwaiti adult population (24 males and 24 females, age 17-59 years) with relative or absolute neutropenia, and this was compared to age-matched controls (64 males and 63 females). The mean haemoglobin levels were 13.