A Case of Miescher Syndrome with Insulin-Resistant Diabetes Mellitus

Miescher syndrome comprises congenital acanthosis nigricans, hypertrichosis, failure to thrive and short stature, dysmorphism especially of the jaws and oral cavity, insulin-resistant diabetes mellitus, and a characteristic general appearance. This report concerns a rare case of 12-year-old girl having insulin resistant diabetic mellitus with Miescher syndrome. The relevant literature was reviewed.

A Case of Type V Hyperlipoproteinemia

The hyperlipoproteinemias are the disturbance of lipid transport resulted from accelerated synthesis or retarded degradation of lipoproteins that transport cholesterol and trigycerides through plasma. These diseases are classified as type I, type IIa, type IIb, type III, type IV, type V, and hyper- -lipoproteinemia by lipoprotein phenotype. Type V hyperlipoproteinemia is uncommon in childhood and characterized by elevation of triglyceride levels due to increases in both VLDL and chylomicrons and lipoprotein electrophoresis shows increased pre- , , and chylomicron bands. We experienced a case of hyperlipoproteinemia type V in a 12 year old male who presented no specific signs and symptoms. So, we report a case of hyperlipoproteinemia type V with brief review of the literatures.

A Case of Congenital Hypoprothrombinemia

Congenital hypoprothrombinemia is a rare congenital coagulation defect. The clinical signs are manifestation of generalized tendency such as; mucosal bleeding, hypermenorrhea and post tooth extraction hemorrage. It is associated with prolongation of PT and PTT with normal thrombin time and decreased serum prothrombin level. A case with congenital hypoprothrombinemia was experienced by the authors. A 36 days old male baby was admitted with palor skin and vomiting started one day before admission. Right side brain parenchymal hemorrhage and left shift of lateral ventricle were on brain CT scan. Prologation of prothrombin time and partial prothrombin time with decreased serum prothrombin level were resulted. Serum factor I, V, VII, VIII, IX and X were within normal level. We report a case congenital hypoprothrombinemia with a brief review of relaed literatures.

A clinical study on early diagnosis in neonatal sepsis

Neonatal sepsis is a major contributing factor for increased mortality and morbidity in the newbron and its successful outcome depends on early detection and prompt institution of an effective antimic robial therapy. Accurate diagnosis of the neonatal septicemia, however, is quite difficult because its early signs and symptoms are rather subtle. This study was conducted in an attempt to search for useful early markers for the clinical entity A total of 224 newborns, over a 17 month pcriod (1/90 5/91), who were evaluated for probable neonatal sepsis were analysed. The laboratory parameters that showed highest correlation with bacteriologically proves sopsis were as follows: Total WBC counts (0.2), elevated ESR(>15mm/hr), CRP(>6ng/ml). Of the 224 study subjects, 41 had two or more parameters of which 12 subjects had positive blood cultures(positive predictive value:29%), only 4 of the 183 newborns who had less than 2 parameters had positive blood cultures(false negative:2%). It is concluded that the presence of two or more of the above parameters can be used as a strong indicator for neonatal sepsis and its sensitivity is 75% while specificity is 86%.

T-lymphocyte subsets in nephrotic syndrome / 대한신장학회잡지

No abstract available.

Sphenoid Sinus Mucocele with Intracranial Extension: Case Report

A case of mucocele of the sphenoid sinus with blurred vision and headache is presented. Mucocele of the sphenoid sinus is relatively rare and benign. Accurate diagnosis is possible through Brain CT scan and MRI. But it should be included in the differential diagnosis of malignancy or pituitary tumor, and unnecessary treatment such as radiotherapy or craniotomy should not be performed. In this case, mucocele is extended into sella turcica which is markedly destroyed and eroded. We removed it by sublabial transseptal sphenoidal sinusotomy and drainage without difficulties and postoperative complications.

Clinical Study of Normal Pressure Hydrocephalus

Since the discovery of CT scan for brain, the detection of hydrocephalus became easier than before. Of those hydrocephalus, non-parenchymatous in origin, in order words, normal pressure hydrocephalus can be differentiated by RISA cisternography. The etiology, symptomatology and surgical outcome of 16 cases of normal pressure hydrocephalus were presented. Followings are the results. 1) All of the cases revealed ventriculomegaly in brain CT scan and prolonged ventricular retention of dye in RISA cisternogram. 2) Ventriculoperitoneal shunt was done on all of the patients and outcome was excellent:3, good:6, fair:3 and poor:4. 3) It is thought that the prognosis is much related duration of clinical manifestation; the earlier surgery, the better outcome.

Multiple Intracranial Dural Sinus Thrombosis associated with Polycythemia: Case Report

Cerebral venous thrombosis is commonly related to infectious processes, trauma, neoplasm, puerperium, and the use of oral contraceptives, etc., but this case was associated with a very rare condition, "polycythemia". This case was confirmed by hematologic study, brain C-T, conventional angiography, and digital subtraction angiography(DSA). The patient was treated by venesection, the use of urokinase and dicumarol, and lumbo-peritoneal shunt(L-P shunt), etc. Post-treatment DSA showed the complete recanalization of all dural sinuses except the left transverse sinus, suggesting normal variant.