The purpose of the present study was to investigate the coordination and correlation of growth pattern between craniomaxillary complex and mandible, and among the craniofacial region, body-weight and stature 14 boys and 16 girls between 6 and 12 years of age were used in this study. The result were as follows, 1. Total increments and maximum increment in mandible is higher than in craniomaxillary complex during given period and no significant sexual difference existed. 2. The annual growth of craniofacial region did not assume an aspect of constant growth, periodically. 3. Craniofacial growth pattern was mterrelatcd with statute more than with body-weight. 4. The growth behavior of body-weight and stature coincided with the growth of craniofacial region or preceded it in time.
Child* ; Female ; Humans ; Longitudinal Studies* ; Mandible*

Polycystic kidney disease is an autosomal dominant disease that may be associated with liver and pancretic cysts. Mitral valve prolapse and intracranial berry aneurysms are also well-known manifestations of autosomal dominant polycystic kidney disease (ADPKD). Cystadenocarcinoma of the pancreas is uncommon and accounts for only 1% of primary pancreatic malignancies. Few cases were reported to have an association of ADPKD and pancreatic malignancies. We report a 63-year-old man with ADPKD who was admitted to our hospital with anorexia and severe weight loss. After abdominal CT and histologic examination, he was diagnosed as pancreatic cystadenocarcinoma with lung, spleen, and liver metastasis. To prolong the life of the patient, we tried gemcitabine and cisplatin combination chemotherapy. But the patient died 2 months after diagnosis due to the disease progression.
Anorexia ; Cisplatin ; Cystadenocarcinoma* ; Diagnosis ; Disease Progression ; Drug Therapy, Combination ; Humans ; Intracranial Aneurysm ; Liver ; Lung ; Middle Aged ; Mitral Valve Prolapse ; Neoplasm Metastasis ; Pancreas* ; Pancreatic Neoplasms ; Polycystic Kidney Diseases ; Polycystic Kidney, Autosomal Dominant* ; Spleen ; Tomography, X-Ray Computed ; Weight Loss

Within the past decade it has been said that progress it pediatric anesthesia is especially the result of clinical application of developments in baic medicine, added to the anesthetist's technical improvements and experience in the use of apparatus and the knowledge of respiratory control. And so anesthetists sholud perform good pediatric anesthesia after understanding pediatric anatomy, physiology and the phamacology of all the drugs used in pediatric anesthesia. 803 cases (under 15years) of pediatric anesthesia were analyzed statistically according to sex, age, disease, department, physical status, premedication, anesthetic technic, anesthetic agents, length of anesthesia duration of Hospitalization, re-operation incidence, complications and mortality in the Department of Anesthesiology, Hanyang University, College of Medicine from August of 1979 to December 1980. The conclusions are as follows: 1) Sex and Age: 91 cases(11.33%) under 1year and 95 cases(11.83%) under 7years are of interest. The comparison of male (542 cases) to female (261 cases) is 2:1. 2) Departmente: 257 cases(32.00%) from ENT, and 217 cases(27.02% from general surgery were noted ont of the total. 3) Disease: It divided into two groups. 204 cases(25.40%) were congenital disease which comprised mainly of 70 cases of inguinal hernia and 45 cases of eleft lip and palate. 599 cases (74.60%) were acquired disease comprised mainly of 237 cases of tonsillitis and 43 cases of appendicitis. 4) Physical status: The time of operation divided into two groups. One group contained 657 cases of elective surgery and the other 146 cases of emergency surgery. According to the ASA classification of physical status, 741 cases(92.28%) were class 1 and 2, and 52 cases (64.8%) were class 3, and 10 cases(1.24%) were in class 4. 5) Premedication: 400 cases(49.81%) were premedicated with atropine sulfate and valium 161 cases(20.05%) were not premedicated because the patients had fever, dehydration, or tachycadia. 6) Anesthetic method and agents: they were divided into three groups. In the frist group using general inhalation, 206 cases(2565%) ont of 761, had non-rebreathing anesthesia and 555 cases(69.12%) had semiclosed circle technique anesthesia. In the second group 41 cases(5.10%) had intramuscular and intravenous anesthesia. Thirty 1 case(0.12%) was given spinal anesthesia. 711 cases(88.54%) received halothane + nitrone oxide _ oxygen. 7) Incidence of reoperation: 32 cases(3.98%) were reoperated and included colostomy repari, abdominal flap detachment, pin removal, skin graft, post operative bleeding control, remove of laryngeal papilloms, and urethral dilatation. 8) Complications: 20 cases which were made up of 8 cases of pneumonia, 5 cases of wound infection, 3 cases of post operative bleeding control, 2 cases of fistula formation, 1 case of gastroenteritis, 1 case of intestinal obstruction. The relationship between length of anesthesia and complications is statistically in significant(p<0.01). 9) Motality: 10 cases died 3 from respiratory insufficiency, 3 cases of sepsis, 1 case of intestinal obstruction and 3 cases of high intracranial pressure. There is statistical significance in the relationship between the anesthetic time, physical status, and mortality.(p<0.01).
Anesthesia* ; Anesthesia, Intravenous ; Anesthesia, Spinal ; Anesthesiology ; Anesthetics ; Appendicitis ; Atropine ; Classification ; Colostomy ; Dehydration ; Diazepam ; Dilatation ; Emergencies ; Female ; Fever ; Fistula ; Gastroenteritis ; Halothane ; Hemorrhage ; Hernia, Inguinal ; Hospitalization ; Humans ; Incidence ; Inhalation ; Intestinal Obstruction ; Intracranial Pressure ; Lip ; Male ; Mortality ; Oxygen ; Palate ; Palatine Tonsil ; Physiology ; Pneumonia ; Premedication ; Reoperation ; Respiratory Insufficiency ; Sepsis ; Skin ; Tonsillitis ; Transplants ; Wound Infection

Previous studies have demonstrated that rottlerin, a specific PKCdelta inhibitor, potentiates death receptor- mediated apoptosis through a cytochrome c-dependent or -independent pathway. However, its ability to regulate necrotic cell death, as well as the underlying mechanism, remains unknown. We found that in murine fibrosarcoma L929 cells, treatment with rottlerin protected the cells against TNF-induced necrosis, whereas it sensitized the cells to apoptosis induced by co-treatment with Hsp90 inhibitor geldanamycin and TNF, in a manner independent of its ability to inhibit PKC-delta. TNF treatment induced rapid accumulation of mitochondrial superoxide (O2") through the Nox1 NADPH oxidase when cells undergo necrosis. Moreover, pretreatment with rottlerin failed to induce the GTP-bound form of small GTPase Rac1 by TNF treatment, and subsequently suppressed mitochondrial O2(-) production and poly(ADP-ribose) polymerase activation, thus inhibiting necrotic cell death. Therefore, our study suggests that Nox1 NADPH oxidase is a new molecular target for anti-necrotic activity of rottlerin upon death-receptor ligation.
Acetophenones/*pharmacology ; Animals ; Benzopyrans/*pharmacology ; Cell Death/*drug effects ; Cell Line, Tumor ; Mice ; Protein Kinase Inhibitors/*pharmacology ; Superoxides/metabolism ; Tumor Necrosis Factor-alpha/*antagonists & inhibitors/pharmacology

Kawasaki disease (KD) is an acute pediatric vasculitis that affects genetically susceptible infants and children. To identify coding variants that influence susceptibility to KD, we conducted whole exome sequencing of 159 patients with KD and 902 controls, and performed a replication study in an independent 586 cases and 732 controls. We identified five rare coding variants in five genes (FCRLA, PTGER4, IL17F, CARD11, and SIGLEC10) associated with KD (odds ratio [OR], 1.18–4.41; p = 0.0027–0.031). We also performed association analysis in 26 KD patients with coronary artery aneurysms (CAAs; diameter > 5 mm) and 124 patients without CAAs (diameter < 3 mm), and identified another five rare coding variants in five genes (FGFR4, IL31RA, FNDC1, MMP8, and FOXN1), which may be associated with CAA (OR, 3.89–37.3; p = 0.0058–0.0261). These results provide insights into new candidate genes and genetic variants potentially involved in the development of KD and CAA.