Anti-HLA antibody related neonatal thrombocytopenia is an uncommon disorder caused by platelet antigen incompatibility between mother and fetus in Korea. Mothers who lack the specific platelet antigen produce the IgG against the platelet antigen which the fetus inherits from the father. These IgG antibodies are then transported across the placenta into the fetal circulation where they lead to the destruction of fetal platelets. We report a case of neonatal alloimmune thrombocytopenia related with anti-HLA antibody in second baby of dizygotic twin who had petechia on trunk and platelet count 43,000/mm. Initially, mother and twin showed the positives in antiplatelet antibodies. In microlym- phocytotoxic test at 6 months of age, anti-HLA antibodies was negative in twins but anti-HLA A2, A24 was positive in their mother. The patient was treated with intravenous immunoglobulin and clinically improved and her platelet count was norrnalized.
Acrocephalosyndactylia* ; Antibodies ; Blood Platelets ; Fathers ; Fetus ; Humans ; Immunoglobulin G ; Immunoglobulins ; Infant, Newborn ; Korea ; Mothers ; Placenta ; Platelet Count ; Thrombocytopenia, Neonatal Alloimmune ; Twins ; Twins, Dizygotic

Partial trisomy 10q syndrome is a rare chromosome anomaly characterized by severe mental and growth retardation, craniofacial dysmorphia with prominent forehead, fine arched eyebrows, deep set small eyes and micrognathia, In addition, other physical manifestations have been reported as skeletal anomaly, congenital heart disease, inguinal hernia, and so on. We report a case of partial trisomy 10q syndrorne with certain stigmata which confirmed by chromosome analysis.
Christianity ; Eyebrows ; Forehead ; Heart Defects, Congenital ; Hernia, Inguinal ; Trisomy*

The morphologic abnormalities of the endocrine pancreas that underlie persistent neonatal hyperinsulinemic hypoglycemia and are included under the heading "nesidioblastosis" appears to be heterogeneous. This characteristic morphologic finding is ductuloinsular complexes showing endocrine cells budding off the ductoepithelium and merging with adjacent endocrine cell clusters. A case of nesidioblastosis associated with hyperinsulinemic hypoglycemia occurred in a 6/365 year-old male neonate. Microscopic finding of near totally resected pancreas revealed irregular sized islets and ductuloinsular complexes, both of which contained hypertrophied B cells with a few mitosis. Because of persistent hypoglycemia after first operation, he received second operation 8 days after. This histologic finding was more severe comparative to that of first operation. According to these findings, the pathogenesis of nesidioblastosis may be congenital or developmental defect of a kind of compensatory mechanism by unknown stimuli to acquire persistent hypoglycemia.
Male ; Infant, Newborn ; Humans

OBJECTIVE: Internal carotid artery (ICA) trunk aneurysms are rare constituting about 0.9 to 6.5% of all ICA aneurysms. They may arise from medial, lateral, ventral (posterior) or dorsal (anterior) walls of ICA. The most frequent site of origin is dorsomedial, followed by dorsal, dorsolateral and ventromedial wall. ICA dorsal wall aneurysms can be divided into the saccular type and blister type, which have different shapes, wall histological features and surgical tactics. The authors report an analysis of 27 cases of ICA trunk aneurysms treated with surgical clipping or GDC embolization. METHODS: Of 145 cases of ICA aneurysms from May 1998 to December 2005, we found 27 cases (17.5%) of such unusual aneurysms located at nonbranching sites of the intradural ICA. Features of neuroimagings and medical records were analyzed. RESULTS: Out of 27 aneurysms, 10 were located at ICA dorsal wall, 8 aneurysms were developed at ICA medialwall. Seven aneurysms originated from ICA ventral wall. Seven cases presented with subarachnoid hemorrhage and 20 cases were found unruptured. Two patients had blister-like aneurysm, and the others had saccular aneurysms. All patients were treated successfully with microsurgical clippings or GDC embolization. Microsurgical clipping were performed in 14 cases and among them, removal of anterior clinoid process was done in 12 cases. Eleven aneurysms were occluded with detachabel coil. Two patients underwent balloon occlusion of ICA. Blister aneurysm was clipped including a portion of the normal ICA wall. Good outcomes were obtained in 25 patients, and two patients were dead. CONCLUSION: ICA trunk aneurysms are developed at any site of ICA circumference. Therefore we consider a variety of treatment strategies. On clipping of aneurysms at proximal ICA trunk, removal of anterior clinoid process is important. Endovascular treatment may be good an alternative. However, surgical treatment is essential for blister like aneurysms.
Aneurysm* ; Balloon Occlusion ; Blister ; Carotid Artery, Internal* ; Humans ; Intracranial Aneurysm ; Medical Records ; Subarachnoid Hemorrhage ; Surgical Instruments

A female newborn had the following characteristics; a congenital localized absence of skin over the lower extremities; blistering of the skin or mucous membrane, incited by trauma, which heals without scarring; and congenital absence or deformity of the nails. In respect to the characteristic manifestation, clinical course and electron microscopic features, our patient seems to fit well into Bart's syndrome. The inheritance pattern appears to be autosomal dominant but, as in this report, isolated cases have been recognized. As the review of the literatures, congenital localized absence of skin has been observed in various subsets of inherited E.B. But, we believe that the term Bart's syndrome should be used to identify patients with good prognosis as the Bart's initial description To the best of our knowedge, this is the first reported case of Bart's syndrome in the korean literature.
Blister ; Cicatrix ; Congenital Abnormalities ; Female ; Humans ; Infant, Newborn ; Inheritance Patterns ; Lower Extremity ; Mucous Membrane ; Prognosis ; Skin

No abstract available.
Child* ; Humans ; Reye Syndrome*

No abstract available.
Hemolytic-Uremic Syndrome*

BACKGROUND: An increase in oxidative stress has been suggested to play major roles in the complications of diabetes. The bulk of the experimental data favors enhanced free radicals in diabetes and antioxidant defense mechanisms may be reduced in diabetes. Melatonin, the major secretory product of the pineal gland has been shown to be a potent and specific hydroxyl radical scavenger. The purpose of our study was to determine the antioxidative effeet of melatonin in streptozotocin-induced diabetic rats. METHODS: Sprague-Dawley rats weighing 200-240 g were divided into 3 groups: normal controls(n-7), diabetic contmls(n-9), melatonin-treated diabetic animals(n-9). Diabetes was induced by intraperitoneal injection of streptozotoein(55 mg/kg body weight) and melatonin(6 mg/kg body weight) was orally administered for 20 days. At day 20 after streptozotocin administration, blood was collected for the assay of glucose, albumin and cholesterol. Erythrocyte membrane lipid peroxidation was determined by malonyldialdehyde(MDA) reactivity. RESULTS: 1) The MDA resctivity of erytbrocyte membrane in melatonin-treated diabetic animals (meanstandard deviation: 5.52+-1.52nmol/ml packed cells) were lower(p<0.05) than that in diabetic controls(7.68+-1.16nmol/mL packed cells). But, there was no significant difference between melatonin-treated diabetic animals and normal contls(4.93+-1.19 nmol/mL packed cells). 2) There were no significant differences of blood glucose and body weight between diabetic controls and melatonin-treated diabetic animals. CONCLUSION: These results show the antioxidative effect of melatonin in streptozotocin-induced diabetic rats. Further clinical and long-term experimental studies are needed to assess the effect of melatonin on development and progression of diabetic complications.
Animals ; Blood Glucose ; Body Weight ; Cholesterol ; Defense Mechanisms ; Diabetes Complications ; Erythrocyte Membrane ; Free Radicals ; Glucose ; Hydroxyl Radical ; Injections, Intraperitoneal ; Lipid Peroxidation ; Melatonin* ; Membranes ; Oxidative Stress ; Pineal Gland ; Rats* ; Rats, Sprague-Dawley ; Streptozocin

Although uterine arterovenous malformation(AVM) is a rare cause of menorrhagia or postmenopausal bleeding, it is important to consider that curettage may precipitate life-threatening hemorrhage and therefore it is contraindicated when AVM is suspected. Special investigations such as hysteroscopy, Doppler flow ultrasound and angiography are important diagnostic tools, and transarterial embolization(TAE) has replaced hysterectomy as the treatment of choice in women who wish to retain their fertility. But due to the high incidence of collateral vessels, recurrence of the vascular malformation after TAE is common. We have experienced two cases of AVM, one of which was managed by TAE in combination with surgical arterial ligation.
Angiography ; Arteriovenous Malformations* ; Curettage ; Female ; Fertility ; Hemorrhage ; Humans ; Hysterectomy ; Hysteroscopy ; Incidence ; Ligation* ; Menorrhagia ; Recurrence ; Ultrasonography ; Vascular Malformations

OBJECTIVE: Adult onset Still s disease is an acute systemic inflammatory disorder. There are no pathognomonic symptoms or specific laboratory abnormalities. In recent reports, serum ferritin concentration is increased in active disease phase and decreased after defervescence. Our purpose was to determine the clinical significance of serum ferritin as an indicator for disease activity. METHODS: Seven patients who were diagnosed as adult onset Still s disease at Samsung Medical Center between October 1994 and March 1997, were reviewed. In these patients we checked leukocyte count, ESR, CRP and serum ferritin concentrations at the time of diagnosis and during follow-up periods and recorded febrile events during follow-up periods. RESULTS: At the time of diagnosis and during febrile periods, the concentrations of ferritin were extremely high(927ng/ml to 96,650ng/ml normal 10-290.8 ng/ml). The values were unrelated to other manifestations of the disease or laboratory findings. The ferritin concentrations decreased rapidly after adequate treatment. Eleven febrile reattacks happened in 7 patients. Serum ferritin concentrations were increased in 8 febrile attacks, while leukocyte count, ESR, and CRP were increased in 5, 5, 6 febrile attacks respectively, There were 10 events of increased serum ferritin concentrations in 7 patients during follow-up periods and 8 events were related with fever. The increases of other laboratory tests were similar. CONCLUSIONS: In all patients, serum ferritin concentrations were increased at the time of diagnosis and closely related to fever. During follow-up periods, serum ferritin concentrations are helpful in monitoring disease activity and guiding decisions about treatment.
Adult* ; Diagnosis ; Ferritins* ; Fever ; Follow-Up Studies ; Humans ; Leukocyte Count ; Still's Disease, Adult-Onset*