Thelazia callipaeda, parasitic in the eye of 24 years old Korean male, was extracted from the left eye of the patient in Seoul, Korea. We confirmed that as T. callipeada morphologically, based on the descriptions of Hsu and other literature. We presents the 7th human thelaziasis case in Korea with the historical review of the literature.
parasitology-helminth-nematoda ; Thelazia callipaeda ; thelaziasis ; case report

Cranial fasciitis is a rare fibroblastic tumor which shows a predilection for the scalp of young children. We present a child with a rapidly growing mass and lytic skull lesion which on pathologic evaluation was diagnosed as cranial fasciitis. Histologically this lesion was identical to nodular fasciitis which was typically found in the trunk and extremities of adults. Cranial fasciitis is unique in that it may present as a lytic lesion in the skull, but this disease entity is not widely known to pathologists and radiologists, and should be included in the differential diagnosis of fibroblastic lesion occurring in the cranium of young children.
Adult ; Child ; Diagnosis, Differential ; Extremities ; Fasciitis* ; Fibroblasts ; Humans ; Scalp ; Skull

No abstract available.
Aorta, Thoracic* ; Blood Flow Velocity* ; Female ; Hypertension, Pregnancy-Induced* ; Pregnancy ; Umbilical Arteries*

Authors experienced a case of A3B in a 46-year-old patient with liver cirrhosis and two cases of A3 in her children by family study. A3 subgroups were confirmed by delayed and weak positive with anti-A and anti-A,B, negative in anti-A1 lectin, adsorption-elution test, and family study. We report a family case of A3B and A3 with brief review of literatures.
Child ; Humans ; Liver Cirrhosis ; Middle Aged

Merkel cell carcinoma (MCC) is an uncommon but potentially aggressive neuroendocrine carcinoma of the skin. It typically develops on sun-exposed areas of the head and neck, particularly the eyelid, periorbital region, and extremities. We report a case of unusually located MCC, presenting as a conjunctival mass, which has only been reported once in the English literature. An 83-year-old male presented with a 0.8 x 0.7 cm sized mass protruding from the lower fornix of the right conjunctiva. The mass was excised. The tumor was located in the submucosa without connection to the overlying mucosa. Light microscopic findings showed a carcinoma with undifferentiated small round cells and the presence of cytokeratin (CK AE1/3, CK7) and neuroendocrine markers. We established a diagnosis of MCC. As reported in the literature, the majority of MCCs are positive for CK20 but negative for CK7. But, this case showed an uncommon cytokeratin immunohistochemical profile of positive for CK7 and negative for CK20, suggesting a new immunophenotypic MCC variant.
Aged, 80 and over ; Carcinoma, Merkel Cell ; Carcinoma, Neuroendocrine ; Conjunctiva ; Extremities ; Eyelids ; Head ; Humans ; Keratins ; Light ; Male ; Mucous Membrane ; Neck ; Neuroendocrine Tumors ; Skin

BACKGROUND: Platelet refractoriness has been reported to occur in 30-70% of multitransfused patients. This can result by either immune or nonimmune mechanisms. The predominant immune cause of platelet refractoriness is alloimmunization to HLA class I antigens. Recently, acid-treated platelets have been used in a few patients with platelet refractoriness due to HLA alloantibodies. However, the effect of acid-treated platelets has not been consistent. The aim of this study was to evaluate the in vivo survival of acid-treated, HLA-eluted platelets in HLA-immunized rabbits. METHODS: For in vivo survival test, 14 New Zealand White rabbits were studied. Four rabbits were in the nonimmunized control and 10 were immunized by weekly transfusions of human pooled platelets for six weeks. The HLA-immunized group was separated into two groups with transfusion of acid-treated platelets and untreated platelets. The survival of transfused platelets in rabbits with immunization and control group was estimated by a flow cytometer using FITC-labeled anti-CD42a. We also examined the HLA re-expression in acid-treated platelets due to regeneration and adsorption of HLA from human plasma. RESLUTS: The half-life of untreated platelets in nonimmunized rabbits was 11.8 +/- 3.7 hr. The half-life of acid-treated platelets in rabbits with HLA antibodies was 9.5 +/- 5.5 hr and the half-life of untreated platelets in rabbits with HLA antibodies was 5.9 +/- 2.9 hr. The difference between untreated platelets in the nonimmunized control group and acid-treated platelets in rabbits with HLA antibodies was statistically insignificant (p=0.221). Re-expression of HLA-A,B,C by endogenous resynthesis occurred continuously, and after 24 hrs it reached 84% of pre-elution level. Adsorption of HLA antigens from human plasma was completed within four hrs. CONCLUSIONS: Acid-treated, HLA-eluted platelets may be applicable for the patients with refractoriness to platelet transfusion, especially, in case of unavailability of HLA-compatible donors and fatal bleeding such as intracranial hemorrhage and pulmonary hemorrhage. However, the post-transfusion increment of the platelet count could not be maintained over 24 hrs because of the endogenous resynthesis of HLA antigens.
Adsorption ; Antibodies ; Blood Platelets ; Half-Life ; Hemorrhage ; Histocompatibility Antigens Class I ; HLA Antigens ; Humans ; Immunization ; Intracranial Hemorrhages ; Isoantibodies ; Plasma ; Platelet Count ; Platelet Transfusion ; Rabbits* ; Regeneration ; Tissue Donors

Glutaric aciduria type 1(GA1) is an autosomal recessive disorder of the lysine, hydroxylysine and tryptophan metabolism caused by the deficiency of mitochondrial glutaryl-CoA dehydrogenase. This disease is characterized by macrocephaly at birth or shortly after birth and various neurologic symptoms. Between the first weeks and the 4-5th year of life, intercurrent illness such as viral infections, gastroenteritis, or even routine immunizations can trigger acute encephalopathy, causing injury to caudate nucleus and putamen. But intellectual functions are well preserved until late in the disease course. We report a one-month-old male infant with macrocephaly and hypotonia. In brain MRI, there was frontotemporal atrophy(widening of sylvian cistern). In metabolic investigation, there were high glutarylcarnitine level in tandem mass spectrometry and high glutarate in urine organic acid analysis, GA1 was confirmed by absent glutaryl-CoA dehydrogenase activity in fibroblast culture. He was managed with lysine free milk and carnitine and riboflavin. He developed well without a metabolic crisis. If there is macrocephaly in an infant with neuroradiologic sign of frontotemporal atrophy, GA1 should have a high priority in the differential diagnosis. Because current therapy can prevent brain degeneration in more than 90% of affected infants who are treated prospectively, recognition of this disorder before the brain has been injured is essential for treatment.
Atrophy ; Brain ; Carnitine ; Caudate Nucleus ; Diagnosis, Differential ; Fibroblasts ; Gastroenteritis ; Glutaryl-CoA Dehydrogenase ; Humans ; Hydroxylysine ; Immunization ; Infant ; Lysine ; Macrocephaly* ; Magnetic Resonance Imaging ; Male ; Metabolism ; Milk ; Muscle Hypotonia ; Neurologic Manifestations ; Parturition ; Putamen ; Riboflavin ; Tandem Mass Spectrometry ; Tryptophan

C-kit-negative gastrointestinal stromal tumors (GISTs) are uncommon, and there have been few reports about the diagnosis and treatment of c-kit-negative GISTs in the stomach. We report the case of a patient who was diagnosed with a huge and atypical GIST in the stomach. The GIST was completely resected and finally diagnosed as c-kit-negative GIST based on immunohistochemical staining of tumor cells, which were negative for CD117 and CD34 and positive for Discovered on GIST-1 (DOG1). C-kit-negative GISTs could be treated by complete resection and/or imatinib, which is the same treatment for c-kit-positive GISTs.
Diagnosis ; Gastrointestinal Stromal Tumors* ; Humans ; Proto-Oncogene Proteins c-kit ; Stomach* ; Imatinib Mesylate

Kawasaki disease is an acute vasculitis of unknown causes that occurs predominantly in infants and young children and produces coronary artery aneurysm. We have recently experienced a case of persistent Kawasaki disease in a 24 month-old-girl improved with pulsed doses of methylprednisolone. Even with an administration of intravenous gamma globulin(IVIG), she repeated the course of recovery and aggravation. After four times repeated doses of IVIG, additional intravenous methylprednisolone pulse therapy was tried and resulted in remarkable improvement. We reported the case with a brief review of the related literature.
Aneurysm ; Child ; Coronary Vessels ; Humans ; Immunoglobulins, Intravenous ; Infant ; Methylprednisolone* ; Mucocutaneous Lymph Node Syndrome* ; Vasculitis