Few cases of ichthyosis bullosa of Siemens(IBS) have been reported since 1939, as a distinct entity from bullous congenital ichthyosiform erythroderma(BCIE). IBS can be differentiated from BCIE by the absence of congenital erythroderma and a different distribution of involved skin area. It's characteristic features include blistering, superficial erosion or moulting of the outer skin. Histological features are tonofilaments aggregation confined to the granular and upper spinous layer of the epidermis. However, in BCIE these findings are present in the whole suprabasal compartment. The original reports of Siemens and cases from other authors showed an autosomal dominant inheritance. Our patient developed IBS sporadically without a familial background.
Blister ; Dermatitis, Exfoliative ; Epidermis ; Humans ; Ichthyosis Bullosa of Siemens* ; Ichthyosis* ; Intermediate Filaments ; Molting ; Skin ; Wills

No abstract available.
Catheterization* ; Catheters* ; Hemothorax* ; Subclavian Vein*

One of the important functions of nervous system is to supply the information related to the injury. Therefore, for various kinds of injuries, to feel pain is thought to be the part of this important function. There are A-fiber (Type I AMHs, Type II AMHs) & C-fiber (CMHs) which respond to the mechanical & thermal stimuli as the nerve fibers of peripheral unit performing this function. The phenomena expressed to us by these stimuli are those reaction like the in- crease of heart rate and the elevation of blood pvessure by stimulation of the autonomic nervous system as well as pain. As it is, using these reactions which are the most popular parameter of pain expression during anesthesia, we make the depth of anesthesia deeper or use analgesics as adjuvants. In general, there are many kinds of analgesics for adjuvants of anesthesia or for elimination of postoperative pain. To compare the efficiency of those analgesics toward pain, the authors studied the inhibitory effects of analgesics on the increase of pulse rate and mean arterial pressure evoked by the thermal and mechanical stimulations in rats. We used the agents popularly used in nowadays 1) Tramadol HC1, known as a potent analgesics, 2) Nalbuphine, antagonist of opioids, 3) Meperidine and Morphine, opioids to com- pare the efficiency of hemodynamic augumentation by the thermal and mechanical stimulations with the control group. The results were as follows', 1) The inhibitory effects on the increase in pulse rate and mean arterial pressure evoked by the stimulations were in the decsing order of morphine, meperidine, nalbuphine, and tramadoL 2) The inhibitory effect of morphine on the incmnent of pulse rate and mean arterial pressme was evident. 3) Meperidine and nalbuphine, not so much potent as morphine in inhibitory also inhibit to a considerable degree. 4) In the case of tramadol, the increament of pulse rate evoked by the stimulations were inhibited to some extent, but it was proven not to inhibit the elevation of mean arterial pressure.
Analgesics* ; Analgesics, Opioid ; Anesthesia ; Animals ; Arterial Pressure ; Autonomic Nervous System ; Heart Rate ; Hemodynamics* ; Meperidine ; Morphine ; Nalbuphine ; Nerve Fibers ; Nervous System ; Pain, Postoperative ; Rats* ; Tramadol

One of the important functions of nervous system is to supply the information related to the injury. Therefore, for various kinds of injuries, to feel pain is thought to be the part of this important function. There are A-fiber (Type I AMHs, Type II AMHs) & C-fiber (CMHs) which respond to the mechanical & thermal stimuli as the nerve fibers of peripheral unit performing this function. The phenomena expressed to us by these stimuli are those reaction like the in- crease of heart rate and the elevation of blood pvessure by stimulation of the autonomic nervous system as well as pain. As it is, using these reactions which are the most popular parameter of pain expression during anesthesia, we make the depth of anesthesia deeper or use analgesics as adjuvants. In general, there are many kinds of analgesics for adjuvants of anesthesia or for elimination of postoperative pain. To compare the efficiency of those analgesics toward pain, the authors studied the inhibitory effects of analgesics on the increase of pulse rate and mean arterial pressure evoked by the thermal and mechanical stimulations in rats. We used the agents popularly used in nowadays 1) Tramadol HC1, known as a potent analgesics, 2) Nalbuphine, antagonist of opioids, 3) Meperidine and Morphine, opioids to com- pare the efficiency of hemodynamic augumentation by the thermal and mechanical stimulations with the control group. The results were as follows', 1) The inhibitory effects on the increase in pulse rate and mean arterial pressure evoked by the stimulations were in the decsing order of morphine, meperidine, nalbuphine, and tramadoL 2) The inhibitory effect of morphine on the incmnent of pulse rate and mean arterial pressme was evident. 3) Meperidine and nalbuphine, not so much potent as morphine in inhibitory also inhibit to a considerable degree. 4) In the case of tramadol, the increament of pulse rate evoked by the stimulations were inhibited to some extent, but it was proven not to inhibit the elevation of mean arterial pressure.
Analgesics* ; Analgesics, Opioid ; Anesthesia ; Animals ; Arterial Pressure ; Autonomic Nervous System ; Heart Rate ; Hemodynamics* ; Meperidine ; Morphine ; Nalbuphine ; Nerve Fibers ; Nervous System ; Pain, Postoperative ; Rats* ; Tramadol

PURPOSE: Puberty is a rapid changing periods of body composition and endocrine system. Already leptin was known to be closely related to body composition and was known to change according to Tanner stage. Ghrelin, a natural GH secretagogue, was also known to be related with weight change, fat utilization, insulin secretion, and influenced by sex steroid. But the relationship between ghrelin and other parameter according to pubertal development is not established yet. METHODS: Cross-sectional study of 101 peripubertal children (54 males and 47 females) aged 8.5 to 17.0 years. We examined body composition (free fat mass, fat mass, % body fat) by bioelectric impendence measurements. Total ghrelin, leptin, insulin was measured by radioimmunoassay (RIA). Relationship between ghrelin, leptin, insulin and body composition data was analysed according to Tanner stage controlling gender using SPSS ver 11.0. RESULTS: Ghrelin concentration is only correlated with Tanner stage (r=-0.21, P<0.05) and GIR (r=0.29, P<0.01). When we divided subjects by sex, even though there was no difference of ghrelin level between both sex, only male is correlated with Tanner stage (r=-0.28, P<0.05) and GIR (r=0.36, P<0.01). There was no difference of ghrelin level between each Tanner stage and no correlation was observed with other body composition and endocrine parameter including leptin. CONCLUSION: These data suggest that Ghrelin levels decrease during puberty at least in boys as pubertal development proceeds but it is minimal. During puberty, ghrelin levels are stable. Ghrelin may have no relationship with other pubertal change. The relation of ghrelin and insulin sensitivity need to be further investigated.
Adolescent ; Body Composition ; Child ; Cross-Sectional Studies ; Endocrine System ; Ghrelin* ; Humans ; Insulin ; Insulin Resistance ; Leptin ; Male ; Puberty* ; Radioimmunoassay

PURPOSE: Transcatheter arterial chemoembolization (TACE) and subsequent percutaneous ethanol injection (PEi) was attempted in 8 patients with 9 hepatocellular carcinomas (HCCs) for complete tumor necrosis of HCCs less than 5cm in greatest diameter. MATERIALS AND METHODS: PEI was performed with 2-8ml of absolute (99.9%) ethanol two weeks after TACE under CT or ultrasound guidance. For each patient PEI was done twice to four times within 4-10 days of each procedure. After completion of a series of PEI, follow up examination (range:3 months-l.5 year period) was done with angiography, CT or ultrasound and correlated with serum alpha-fetoprotein (AFP) level. RESULTS: On follow up anglograms, the lesions completely disappeared or decreased in size without tumor vessels or staining in 5 of 6 patients. On follow up CT of 6 patients, the lipiodol-laden HCCs were surrounded by non-enhancing low density and the losion sizes were slightly decreased or not changed. These are suggestive of necrosis of tumor itself and adjacent liver parenchyma. The tumors could not be detected on follow up ultrasound examination in 2 patients. Serum AFP was decreased in 7 patients and was well corresponded to the results of imaging modalities. CONCLUSION: The authors concluded that the combined TACE and PEI is an appropriate treatment for small HCCs having high surgical risks.
alpha-Fetoproteins ; Angiography ; Carcinoma, Hepatocellular* ; Ethanol* ; Follow-Up Studies ; Humans ; Liver ; Necrosis ; Ultrasonography

Background: The goal of this study was to evaluate the clinical and radiological outcomes of arthroscopic partial repair with medialization of the attachment site of the rotator cuff tendon and to identify prognostic factors affecting rotator cuff healing in patients with irreparable large to massive posterosuperior rotator cuff tears. Methods: Between July 2012 and March 2016, 42 patients with irreparable large to massive posterosuperior rotator cuff tears underwent an arthroscopic partial repair with medialization of the attachment site of the rotator cuff tendon. All patients had a minimum of 2-year follow-up (mean, 35.4 ± 7.3 months). Clinical evaluation was performed using the visual analog scale, the University of California, Los Angeles shoulder rating scale, Constant score, and active range of motion. Radiological evaluation was performed using magnetic resonance imaging and simple radiography. Results: Clinical outcomes at the final follow-up improved significantly compared with the preoperative values (all p < 0.001). The failure rate was 23.8% (10/42); however, clinical outcomes significantly improved regardless of cuff healing (all p < 0.001). The mean acromiohumeral distance was 6.5 ± 1.7 mm (range, 3.2–9.7 mm) before surgery and 6.3 ± 1.6 mm (range, 2.8–9.5 mm) at the final follow-up. Preoperative acromiohumeral distance was associated with failure of cuff healing in the univariate analysis (p = 0.043) and multivariate analysis (p = 0.048). A receiver operating characteristic curve was used to determine the predictive cutoff value for the smallest preoperative acromiohumeral distance for successful healing, which was calculated as 5.3 mm. Conclusions Despite healing failure, arthroscopic partial repair with medialization can be a possible treatment option for irreparable large to massive posterosuperior rotator cuff tears because of the improvement in clinical outcome. The shorter preoperative acromiohumeral distance was the single most important factor negatively affecting cuff healing, and the likelihood of success of healing might be improved if a repair is performed when the preoperative acromiohumeral distance is < 5.3 mm.

Immunoglobulin G4 (IgG4)-related hypertrophic pachymeningitis, defined as focally or diffusely thickened dura mater and lymphoplasmacytic infiltration with increased IgG4 bearing plasma cells, is a rare disease. Moreover, cases involving bone are even rarer. In this report, the authors describe a case of IgG4-related hypertrophic pachymeningitis involving the skull in a 65-year-old man presenting with generalized tonic seizures. There is a 2.4 cm diameter extra-axial mass at the vertex of the left frontal convexity and thickened dura mater with contrast enhancement on magnetic resonance (MR) imaging. In addition, the skull adjacent to the mass was focally enhanced. He underwent surgical resection of the enhanced mass and skull. Histopathological findings revealed chronic inflammation with fibrosis, and idiopathic hypertrophic intracranial pachymeningitis was considered. However, eight months after surgery, partial seizures developed and brain MR imaging revealed a recurrence adjacent to the previous mass. We decided to perform additional immunohistochemical staining of the previous specimen, instead of a re-excision. Immunohistochemical staining showed markedly increased IgG4 (+) plasma cells. Consequently, IgG4-related hypertrophic meningitis was confirmed. Since then, steroids and immunosuppressant medications were started. Follow-up MR imaging at 3 months after medication initiation demonstrated complete remission. In conclusion, IgG4-related hypertrophic pachymeningitis should be considered in the differential diagnosis of hypertrophic cranial pachymeningitis.
Aged ; Brain ; Diagnosis, Differential ; Dura Mater ; Fibrosis ; Follow-Up Studies ; Humans ; Immunoglobulin G ; Immunoglobulins* ; Immunosuppressive Agents ; Inflammation ; Magnetic Resonance Imaging ; Meningitis* ; Plasma Cells ; Rare Diseases ; Recurrence ; Seizures ; Skull* ; Steroids

PURPOSE: To evaluate the influence of gestational age(GA) and disease on thyroid hormone concentration in preterm neonates(preterm), we measured thyroxine (T4) and thyroid stimulating hormone(TSH) concentrations and analyzed the relation to GA and diseases. Additionally, we calculated the reference ranges of T4 in preterm for future investigation. METHODS: Serum T4 and TSH were measured by radioimmunoassay for 107 preterm who admitted neonatal intensive care unit during 1994. We sampled from preterm on 5th day of life. We analysed the relationship of GA and birth weight with the levels of T4 and TSH, and prospectively compared them with each neonatal disease. RESULTS: Serum T4 concentration correlated positively with GA(r=0.62, P<0.001) and birth weight(r=0.29, P<0.01). After controlling GA, birth weight did not correlate to the levels of T4. But after controlling birth weight, GA had correlation with T4(r=0.58, P<0.001). In preterm less than 32 weeks of GA, there was no difference of T4 level between healthy and the diseased(respiratory distress syndrome, patent ductus arteriosus, sepsis, intraventricular hemorrhage, and cerebral palsy). Group of bronchopulmonary dysplasia had significant lower level of T4 compared control group(P<0.01). The reference range of T4 in GA 32-36 weeks is 5.56-15.58 microgdL (9.82+/-.40 microgdL). CONCLUSION: GA positively correlated with serum T4 in preterm, but not to TSH. The measurement of TSH level, using in most of the neonatal care unit as neonatal thyroid screening test, in Korea, is not an adequate test to diagnosis hypothyroidism early in preterm. We recommend serial follow up of TSH and T4 in preterm.
Birth Weight* ; Bronchopulmonary Dysplasia ; Diagnosis ; Ductus Arteriosus, Patent ; Gestational Age* ; Hemorrhage ; Humans ; Hypothyroidism ; Infant, Newborn ; Infant, Premature* ; Intensive Care, Neonatal ; Korea ; Mass Screening ; Parturition* ; Prospective Studies ; Radioimmunoassay ; Reference Values ; Sepsis ; Thyroid Gland* ; Thyroxine

A 42-year-old male was hospitalized with abdominal pain, dyspnea, and turbid peritoneal fluid. He was diagnosed with hypertension, diabetes and started continuous ambulatory peritoneal dialysis (CAPD) 11 months ago. He was treated with intraperitoneal cefazolin and ceftazidime, and then white blood cell counts of dialysate decreased. Incidentally, liver abscess was found in chest CT performed for the evaluation of dyspnea, and patient was febrile persistently. So percutaneous abscess drainage was done by pigtail catheter. We changed the antibiotics to ceftriaxone and metronidazole, and hemodialysis was started. Klebsiella pneumoniae was cultured from peritoneal fluid and blood simultaneously. We concluded that liver abscess is a primary cause of CAPD peritonitis.
Abdominal Pain ; Abscess ; Adult ; Anti-Bacterial Agents ; Ascitic Fluid ; Catheters ; Cefazolin ; Ceftazidime ; Ceftriaxone ; Drainage ; Dyspnea ; Humans ; Hypertension ; Klebsiella ; Klebsiella pneumoniae ; Leukocyte Count ; Liver ; Liver Abscess ; Male ; Metronidazole ; Peritoneal Dialysis, Continuous Ambulatory ; Peritonitis ; Renal Dialysis ; Thorax