The congenital long-QT syndrome (LQTS) is characterized by recurrent syncope, prolonged QT intervals, QT interval lability, polymorphic ventricular tachycardia, and sudden death. We report a case of congenital long QT syndrome in a 28-day-old male infant who presented with syncope, bradycardia with 2: 1 pseudo-atrioventricular block and a markedly prolonged QT inteval. One episode occured after crying and degenerated into ventricular fibrillation and terminated after cardioversion. A VVI type cardiac pacemaker was implanted. Subsequently, the infant's heart rate was over 110/min and 2: 1 AV block and any other arrhythmia were absent. The infant recovered from the accompanied pneumonia and sepsis and was discharged 47 days after adrnission. However, 13 days after discharge, the infant returned to our hospital ER with syncope. Ventricular fibrillation ceased after cardioversion. Despite medication with propranolol, ventricular tachycardia persisted. The infant expired the day after he was discharged against medical advice.
Arrhythmias, Cardiac ; Atrioventricular Block* ; Bradycardia ; Crying ; Death, Sudden ; Electric Countershock ; Heart Rate ; Hospitals ; Humans ; Infant ; Long QT Syndrome ; Male ; Pneumonia ; Propranolol ; Sepsis ; Syncope ; Tachycardia, Ventricular ; Ventricular Fibrillation

No abstract available.
Histiocytosis, Sinus* ; Lip*

Short umbilical cord syndrome, also known as the limb-body wall malformation complex and the body stalk anomaly, is a poorly defined sporadic group of congenital anomaly charaterized by a complex set of disruptive abnormalities having in common the failured closure of the ventral body wall. This disorder is charaterized by a short or absent umbilical cord and disruption of the lateral body wall, spine, limbs, face, and cranium, isolated or in combination. Recently, we present a case of short umbilical cord syndrome which found in a term baby, so we report a case of short umbilical cord syndrome with brief review of literature.
Extremities ; Skull ; Spine ; Umbilical Cord*

Objectives : The purpose of this study is to know the shisho constitutional distribution of outpatients at the Center for Kampo Medicine, Keio University School of Medicine (the Keio Kampo Center, herein) and to find out if there are differences in the type of diseases and symptoms according to shisho constitution.
Methods : We collected data from 366 outpatients at the Keio Kampo Center. All did a SSCQ-P (shisho Constitution Questionnaire for Patients) to obtain a shisho constitution diagnosis. We then classified their shisho constitutions and surveyed disease and symptom prevalence according to same.
Results : 1 : Among the 366 outpatients, distribution rates for Taiyojin, Syoyojin, Taiinjin, and Syoinjin were 0.8%, 27.3%, 28.7%, and 43.2% respectively. 2 : And the prevalence of V. Mental and behavioral disorders, XI. Diseases of the digestive system, XV. Pregnancy, childbirth and the puerperium, and Feeling of coldness (under XVIII. Symptoms, signs and abnormal clinical and laboratory findings, NEC) items for Shoinjin were significantly higher than those for the other constitutions. (p < 0.05)
Conclusions : The distribution rate for shisho in 366 outpatients at the Keio Kampo Center was different from that in Korea, with the rate for shoinjin in these outpatients at the Center especially, being significantly higher than that for Koreans. There were also significant differences in the prevalence of some diseases and symptoms in shoinjin group.

BACKGROUND: It was recognized that the intake of the whole cow's milk(WCM) early (before 1 year old of age) and large amount in early childhood is related to the iron deficiency, sometimes the iron deficiency anemia in advanced state. To verify the relationship between the variables in the WCM intake and the iron status, we carried out this study. METHODS: Thirty subjects(1 year to 3 years old of age) were classified as a WCM large intake group and a WCM small intake group. The relationship between the WCM commencing age and the iron status(serum ferritin, transferrin saturation and hemoglobin) in the WCM large intake group was evaluated and analyzed by the linear regression. Futhermore, the relationship between the value of WCM intake amountxduration/10 and the iron status(serum ferritin, transferrin saturation and hemoglobin) in the WCM large and small intake groups was evaluated and analyzed by the linear regression. RESULTS: As the WCM commencing age was earlier, the values of serum ferritin, transferrin saturation and hemoglobin decreased significantly(P<0.05). The values of serum ferritin and transferrin saturation decreased significantly as the value of WCM intake amountxduration/10 increased(P<0.05). But the value of hemoglobin decreased insignificantly as the value of WCM intake amountxduration/10 increased(P>0.1). CONCLUSION: The variables in the intake of WCM influence upon the iron status in early childhood. The early commencing of the WCM(before 1 year old of age) is the worst variable that influences adversely upon the iron status in early childhood. The amount of WCM intake and the duration influence upon the storage iron(serum ferritin and transferrin saturation), but not hemoglobin. In this regard, it is necessary to inform the parents of the correct WCM intake ways to avoid the irreversible recognition impairment and behavioral change.
Anemia, Iron-Deficiency ; Child, Preschool ; Ferritins ; Humans ; Iron* ; Linear Models ; Milk* ; Parents ; Transferrin

The purpose of this study was to investigate the characteristics of craniofacial morphology in adult with crowded dentition. The craniofacial morphology associated with normal and crowded of mandibular dentition was studied in lateral cephalometric radiograph obtained from 145 subjects grouped 44 males and 40 females in normal group, 33 males and 28 females in crowded group. The conclusions were as follows: Means, standard deviations and facial polygons from measurements were obtained from normal and crowded groups. Palatal plane to SN, occlusal plans to SN, mandibular plane to SN, mandibular plane to palatal plane and gonial angle measurements of crowded group were significantly different from normal group and were larger than normal group. SNA, SNB measurements of crowded group were significantly different from normal group and were smaller than normal group. Significant differences were observed between normal group and crowded groups on the basis of SN/1, PP/1, MP/1, and compared with normal group, upper incisors of crowded group were inclined more labially, whereas lower incisors of crowded group were inclined more lingually. Posterior facial height and ramal height were shorter in males of crowded group than those of normal group. Posterior facial height, mandibular length and posterior cranial base length were shorter in females of crowded group than those of normal group. Anterior facial height was longer in females of crowded group than those of normal group.
Adult* ; Dentition* ; Female ; Humans ; Incisor ; Male ; Skull Base

Entrapment of the extensor pollicis longus tendon is reported rarely on Smith's fractures in children. In our case, a 15 year old boy with Smith's fracture received treatment of closed reduction at another hospital. When he visited our hospital, a wide gap at the fracture site was detected on radiograph and the thumb movement was limited. We have doubt the entrapment of the soft tissue, especially the tendon. We decided on open reduction. In the operation field, entrapment of the extensor pollicis longus tendon at the gap of the fracture site was found through dorsal approach. In addition, fracture treatment with K-wire fixation after reduction of extensonr pollicis longus tendon reduction was done. Therefore, we report this case with a review of the literatures.
Child ; Humans ; Tendons ; Thumb

BACKGROUND: Tumor markers have been used in diagnosis, predicting the extent of disease, monitering recurrence after therapy and prediction of prognosis. But the utility of markers in lung cancer has been limited by low sensitivity and specificity. TPA-M is recently developed marker using combined monoclonal antibody of Cytokeratin 8, 18, and 19. This study was conducted to evaluate the efficacy of new tumor marker, TPA-M by comparing the estabilished markers 8CC, CEA, Cyfra2 I - I in hmg cancer. METHODS: An immunoradiometric assay of serum CEA, SCC, Cyfra2l-I, and TPA-M was performed in 49 pathologically confirmed lung cancer patients who visited Keimyung University Hospital from April 1996 to August 1996, and 29 benign lung diseases. Commercially available kits, Ab bead CM (Eiken) to CEA, SCC RIA BEAD (DAINABOT) to SCC, CA21-1 (TEE) to Cyfra21-1, arid TPA-M (DAIICHI) to TPA-M were used for this study. RESULTS: The mean serum values of lung cancer group and control group were 10.05 +/- 38.39 micro/L, l.59+/-0.94 micro/L in CEA, 3.04+/-5.79 micro/L, 1.58+/-2.85 micro/L in SCC, 8.27+/-11.96 micro/L, 1.77+/-2.72 micro/L in Cyfra21-1, and 132.02+/-209.35 U/L, 45.86+/-75.86 U/t in TPA-M respectively. Serum values of Cyfra21-1 and TPA-M in lung cancer group were higher than control group (p<0.05). Using cutoff value recommended by the manufactures, that is 2.5 micro/L, in CEA, 3.0 micro/L in Cyfra21-1, 70.0 U/L in TPA-M, arid 2.0 micro/L in SCC, sensitivity and specificity of lung cancer were 33.3%, 786% in CEA, 50.0%, 89.7% in Cyfra2l-l, 52.3%, 89.7% in TPA-M, 23.8%, 89.3% in SCC. Sensitivity and specificity of nonsmall cell lung cancer were 36.1%, 78.1% in CIA, 50.1%, 89.7% in Cyfra2l-1, 53.1%, 89.7% in TPA-M, 33.8%, 89.3% in SCC. Sensitivity and specificity of small cell king cancer were 25.0%, 78.5% in CEA, 50.0%, 89.6% in Cyfra2l-1, 50.0%, 89.6% in TPA-M, 0%, 89.2% in SCC. Cutoff value according to ROC(Receiver operating characteristics) curve was l.25 micro/L in CEA, 1.5 micro/L in Cyfra2l-1, 35 U/L in TPA-M, 0.6 micro/L in SCC. With this cutoff value, sensitivity, specificity, accuracy and kappa index of Cyfra21-1 and TPA-M were Letter than CEA and SCC. SCC only was related with statistic significance to TNM stages, dividing to operable stages(TNM stage I to IIIA) and inoperable stages (IIIB and IV) (p<0.05). But no tumor markers showed any correlation with significance with tumor size(p>0.05). CONCLUSION: Serum TPA-M and Cyfra21-1 shows higher sensitivity and specificity than CEA and SCC in overall lung cancer and nonsmall cell lung cancer those were confirmed pathologically. SCC has higher specificity in nonsmall cell lung cancer. And the level of serum SCC are significantly related with TNM staging.
Carcinoma, Non-Small-Cell Lung ; Diagnosis ; Humans ; Immunoradiometric Assay ; Keratin-8 ; Lung Diseases ; Lung Neoplasms* ; Lung* ; Neoplasm Staging ; Prognosis ; Recurrence ; Biomarkers, Tumor

Nitric oxide (NO) is known to mediate penile erection by activating intracellular cyclic GMP pathway. It is also suggested that cGMP pathway, on penile erection, has dominant role over the other secondary messenger pathway with cAMP, etc. Based on the hyposthesis that activation of NO-cGMP pathway could represent a more physiologic and effective approach in the treatment of erectile dysfunction, several NO donors and activator of cGMP have been used in human and animal studies of impotence. However the efficacy of those remains debatable. In the present study, we investigated the effect of NO donor [linsidomine chlorhydrate (SIN-1), S-nitroso-N-acetylpcnicillamine (SNAP), sodium nitroprusside (SNP)] alone and in combination with zaprinast (cGMP specific phosphodiesterase inhibitor) on penile erection in rats. NO donors used in this study, except SNP, did not induce penile erection sufficiently. SNP-induced penile erection is comparable to the erection induced by cavernosal nerve stimulation. However, direct applicaion of SNP in the treatment of impotence may not be acceptable as it causes a marked hypotension. Zaprinast given intracayernously either alone or in combination with NO donor may not be clinically effective. Combination with zaprinast is not shown to enhance the effect of NO donor on penile erection. Therefore, combination of NO donor with other drugs modulating different pathway may be a therapeutic approach to erectile dysfunction worthy of further investigation.
Animals ; Cyclic GMP ; Erectile Dysfunction ; Humans ; Hypotension ; Male ; Nitric Oxide* ; Nitroprusside ; Penile Erection* ; Rats* ; Tissue Donors*

Congenital hypertrophic pyloric stenosis is the most common intra-abdominal disease required surgery during the first few months of life. The expression of pyloric stenosis is dependent upon the genetic influence of ancestors affected with the disease, as well as unknown environmental influences in the postnatal period. Pyloric stenosis has been reported in multiple sibs in a family, which suggests the genetic influence on the expression of this disease. Until now, the genetic influence is thought a sex-modified polygenic or multifactorial background which facilitates the expression of a common dominant gene. We experienced a case of congenital hypertrophic pyloric stenosis in a two siblings. The siblings suffered projectile vomiting for 2~4 days at 16 days old of age and 15 days old of age. After we confirmed the diagnosis by upper gastrointestinal series and abdominal sonogram, the Fredet-Ramstedt pyloromyotomy was done successfully. This case suggests the genetic influence on the expression of this disease.
Diagnosis ; Genes, Dominant ; Humans ; Pyloric Stenosis ; Pyloric Stenosis, Hypertrophic* ; Siblings* ; Vomiting