No abstract available.
Hodgkin Disease*

No abstract available.
Hyperplasia* ; Islets of Langerhans* ; Nesidioblastosis*

No abstract available.
Proteinuria*

A patient with Down syndrome has hematologic and oncologic disorders of medical complication. Down syndrome shows that characteristic hematologic and oncologic abnormalities and developments of disorders are different at ages. Many hematologic disorders were related to Down syndrome. There are diseases of red blood cells, white blood cell disorders, platelet and bleeding disorders, aplastic anemia, and transient myeloproliferative disease. Acute myeloid leukemia, acute lymphoblastic leukemia, and rarely solid tumors (Neuroblastoma, Wilm's tumor, Hodgkin's lymphoma) are also associated with Down syndrome. We checked clinical manifestations of each disorder and we should make standard hematologic index of different age groups. In addition, the relations between chromosome 21 and hemato-oncologic disorders should be found. We need to investigate potential therapeutic interventions that can improve quality of life and life expectancy in patients with Down syndrome.
Anemia, Aplastic ; Blood Platelets ; Chromosomes, Human, Pair 21 ; Down Syndrome* ; Erythrocytes ; Hemorrhage ; Humans ; Leukemia, Myeloid, Acute ; Leukocytes ; Life Expectancy ; Myeloproliferative Disorders ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Quality of Life ; Wilms Tumor

Myosin heavy chain 9 (MYH9) related diseases are autosomal dominant diseases characterized by macrothrombocytopenia and inclusion bodies in leukocyte, which can be accompanied by extra-hematologic symptoms such as sensorineural hearing loss, renal dysfunction, and cataract. They are often diagnosed incidentally in adulthood or misdiagnosed as idiopathic thrombocytopenic purpura (ITP), leading to unnecessary treatment with intravenous high-dose gamma-globulin, steroids, or splenectomy. Here, we report the case of a brother and sister confirmed to have a MYH9 gene mutation during follow-up. An 8-year-old boy was confirmed to have thrombocytopenia at birth and treated with intravenous gamma-globulin under suspicion of ITP or sepsis. He was discharged after showing an increase in platelet count. Subsequently, during outpatient workup, he exhibited thrombocytopenia, large platelets, and neutrophilic inclusion bodies. His 10-year-old sister also presented with the same findings. In 2021, DNA analysis revealed that they share a mutation (c.4270G> A, p. Asp1424Asn), a pathogenic variant associated with MYH9-related disorder.

OBJECTIVE: Atopic dermatitis (AD) is a chronic inflammatory skin disease with significant morbidity, and for which there is a need for safe and effective alternative therapies. Although a few observations on the efficacy of intravenous immunoglobulin (IVIG) in AD have been reported, clinical evidence of effectiveness from controlled trials is lacking. Therefore, the purpose of this study was to clarify whether IVIG therapy (1.0 g/kg body weight at each monthly visit for 6 months) is effective in childhood atopic dermatitis and to analyze the clinical characteristics of IVIG responses in this disease. METHODS: Forty three atopic dermatitis patients who had characteristic clinical features of atopic dermatitis were included in this study. The patients received an injection of IVIG at 1.0 g/kg body weight at each monthly visit for 6 months. Laboratory tests were performed for blood chemistry, total immunoglobulin E, immunoglobulin G/immunoglobulin A/immunoglobulin M, blood eosinophil count, and C-reactive protein. RESULTS: In total forty three atopic dermatitis patients, only 14 patients completely underwent 6 cycles, but other 29 patients incompletely (1-5 cycles). In the 14 patients, there were just 13 records of scoring atopic dermatitis (SCORAD) index. The mean SCORAD score in the 13 patients was 39.6+/-24.4. SCORAD score decreased significantly (initial SCORAD, 39.6+/-24.4; final SCORAD, 21.3+/-15.6; P=0.016). CONCLUSION: IVIG therapy may be recommended in the treatment of recalcitrant atopic dermatitis. In addition, further investigation on predictive markers for responses of IVIG therapy in atopic dermatitis may be needed.
Body Weight ; Child ; Complementary Therapies ; Dermatitis, Atopic ; Eosinophils ; Humans ; Immunization, Passive ; Immunoglobulin E ; Immunoglobulins ; Immunoglobulins, Intravenous ; Skin Diseases

We present a study on hospital stay in 1000 consecutive head injuries. The mean hospital stay and standard deviation were calculated in a given condition according to some variables, such as sex, age, Glasgow Coma Score on admission, skull fracture, CT findings, and treatment. Usually, standard deviation was greater than the mean value in a given condition and hospital stay varied in a wide range, representing that the duration of treatment is related not only to the severity but also various individual properties. Since the associated injuries were variable in location, type and severity, they altered hospital stay greatly. Thus hospital stay was analyzed in patients without associated injuries. Hospital stay was largely dependant on three variables. Low Glasgow Coma Score on admission, presence of intracranial mass lesion or diffuse axonal injury, and operative treatment prolonged hospital stay. Skull fracture also lengthened hospital stay but only in patients without associated injuries. Duration of treatment should be recorded in all medical certificates related to the injury. For the proper estimation of duration of treatment, more reports are needed in this field.
Coma ; Craniocerebral Trauma* ; Diffuse Axonal Injury ; Head* ; Humans ; Length of Stay* ; Skull Fractures

We report a case of bilateral chronic subdural hematoma (CSDH) in a 75-year-old man after exercise using a vibrating belt machine on the head. He suffered from headache and intermittent left side numbness for ten days. He denied any head injuries except eccentric exercise using a vibrating belt on his own head for 20 days. An MRI revealed bilateral CSDH. The hematoma was isodense on the CT scan. We made burr-holes on the both sides under local anesthesia. We identified the neomembrane and dark red subdural fluid on both sides. In the postoperative CT scan, we found an arachnoid cyst on the left temporal pole. Although the arachnoid cyst itself is asymptomatic, trivial injury such as vibrating the head may cause a CSDH.
Aged ; Anesthesia, Local ; Arachnoid ; Craniocerebral Trauma ; Head ; Headache ; Hematoma ; Hematoma, Subdural, Chronic* ; Humans ; Hypesthesia ; Vibration

No abstract available.
Hyperprolactinemia*

We report a case of ectopic thymus in the left submandibular area of a two-month-old boy. On US and CT scans, a well-marginated, 3 x 2cm-sized solid mass along the left carotid sheath, anteromedial to the sternocleidomastoid muscle and posterior to the submandibular gland, was seen. CT attenuation of the mass showed that it was similar to that of normal thymus in the anterior mediastinum. Although a rare disease, ectopic thymus should be included in the differential diagnosis of cervical masses along the carotid sheath in infants.
Diagnosis, Differential ; Humans ; Infant* ; Male ; Mediastinum ; Rare Diseases ; Submandibular Gland ; Thymus Gland* ; Tomography, X-Ray Computed