Many kinds of hemoglobin (Hb) variants and thalassemia syndrome were reported during 40 years search for hemoglobinopathy in the world. Studies on their molecular pathology and gene abnormalities have elucidated the effects of base deletion, substitution and insertion in the genomic DNA. The expression of the abnormal gene products trans-formes in a graded manner as follows: hemoglobinopathies due to thalassemia syndrome without abnormal gene product-thalassemic expression of Hb variants-hyperunstable Hb disorder-unstable Hb disorder-stable Hb variant.

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A consecutive series of 60 patients with acutely obstructing carcinomas of the colon and rectum were encountered during the last ten years from 1979 to 1988. The clinicopathological analysis was perfomred according to the general rules of colorectal cancer edited in Japan.
The leison was frequently located in the sigmoid colon but rare in the lower parts of the rectum. The resectability rate was 86.7% and curability rate was 65.0%, both of them being significantly lower than the rates of 436 non-obstructed cases (93.3% and 81.9%, respectively). The cumulative five-year survival rate of the curative case was significantly low in the obstructed cases (47.9%) compared with non-obstructed group (71.6%).
Histologically, moderately and poorly differentiated adenocarcinoma were predominant in the obstructed cases. Moreover, histological venous invasion (v) and lymphatic invasion (ly) were both predominant in the obstructed group.
One stage operation for the obstructing carcinomas of left colon and rectum was per-formed in 67.8% of total cases and 80% of the cases since 1986. The results of the one stage operation have been improved because of the progress of the preoperative managements using intravenous hyperalimentation (IVH) and a long ileus tube. The authors propose it as the first emergency management for the acute obstructing colorectal carcinoma.

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Seventeen patients from whom Clostridium difficile toxin (D-1 toxin) was detected in their stool by latex agglutination test were investigated to elucidate their clinical features by reviewing their charts retrospectively. Patients were distributed over five of fifteen wards of our institution. On one ward where the incidence of Clostridium difficile colitis was the highest, 7 of 988 admitted patients (0.7%) were found positive for D-1 toxin.
They were ten males and seven females. Average age was 65.5 years. Most of the patients were gravely ill, although one patient was a healthy male who developed toxin positive diarrhea after corrective operation of pectus excavatum.
With regard to the relationship to the antibiotic usage, fifteen patients were still on antibiotics when they developed toxin positive stool and two were 4 days and 20 days after cessation of antibiotics respectively.
Clinical symptoms were scrutinized in fifteen patients: forteen of fifteen had typical foul smelling diarrhea which led to the toxin investigation, six of them had preceding anorexia prior to the development of diarrhea, other gastrointestinal symptoms such as abdominal pain, nausea and vomiting were not impressive, typical body temperature pattern was a low grade fever mixed with episodic spiking as high as 38-39°C, and lastly it is note-worthy that one patient had hematochezia.
Laboratory data were also reviewed in the same fifteen patients: all patients had in-creased serum mucoprotein and C-reactive protein, eight of fifteen had increased total WBC count although increased neutrophils were seen in twelve of thirteen, ESR was in-creased in four of nine, serum albumin was below normal range in twelve of fifteen, and lastly decreased Na⁺ and/or K⁺ were seen in nine of fifteen.
It took average 9.6 days to reach the diagnosis of Clostridium difficile colitis.
With regard to the treatment, twelve patients were treated by vancomycin, one by metronidazole, one by vancomycin followed by metronidazole and one by cessation of an antibiotic only, and all patients except one on whom evaluation was impossible because of multiple complications showed remarkable improvement in average 4.1 days.
Fairly high rate of relapse, as high as 38% (five out of thirteen that could be followed), was seen. It is important to note that four of five relapsed despite no usage of antibiotics after the first successful treatment. One patient experienced the third episode of toxin positive diarrhea. Average duration from the completion of the treatment to relapse was 26.3 days.
A case was presented which went through a debilitating clinical course because the diagnosis was delayed. This case clearly demonstrates the importance of early diagnosis of Clostridium difficile colitis which can be easily accomplished by latex agglutination test.

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On the basis of the questionnaire to 130 patients followed up more than 10 years after open heart corrections of congenital heart diseases, the following results were obtained.
1) Two third of school students with right ventricle pressure overload such as tetralogy of Fallot, had slight symptoms and limitation in sports during school life.
2) The educational status of 55 patients who were employed seems to be equivalent to that of general population.
3) The occupational status of 55 patients had a full-time work without any limitation.
4) It was noted that 53% of young females beyond 15 years old were conscious about operation scars with mid sternal incision.
5) Generally, all of 130 patients, except for one patient with Down's disease and tetralogy of Fallot, followed a normal pattern of life on daily activities, parenthood, and family life. Such a result is considered to be satisfactory.

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The relationship between type of hemorrhage on CT scan and outcome was examined in 32 patients (23 men and 9 women) with primary pontine hemorrhage, ranging in age from 34 to 85, with a mean of 55.4. Type of hemorrhage on CT scan was divided into the following three types: 1) massive type (13 cases), 2) basis tegmentum type (7 cases) and 3) tegmentum type (12 cases). All patients with massive type died in the acute stage. Of five cases with bilateral basis tegmentum type, two died and three were vegetative. In twelve cases with tegmentum type, outcome was excellent as full work (ADL) in five cases, recovery to daily life for oneself in four cases and recovery to daily life with an assistance in two cases. There was exact correlation between type of hemorrhage and outcome in primary pontine hemorrhage. We discussed the indication of stereotactic surgery for pontine hemorrhage.

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For diagnosis, severity and prognosis of neonatal central nervous system (CNS) diseases, we made a study of spinal fluid NSE, LDH and CPK, and serum CPK and CPK-BB in neonates. Examination of overall CNS diseases including intracranial hemorrhage, asphyxia neonatorum, convulsion, meningitis, etc, demonstrated that the values of the markers in the disease groups were significantly higher than those in the normal group. In terms of individual diseases, the intracraneal hemorrhage group showed significantly high values for spinal fluid NSE and LDH and serum CPK, likewise the asphyxia neonatorum group for spinal fluid LDH, and the convulsion group for spinal fluid LDH and serum CPK, in comparison with the normal group. In relation to prognosis, the cerebral palsy group had significantly high spinal fluid NSE and LDH values. In the mental retardation group, the marker values were higher but showed no significant differences from those in the normal group. From all these findings, spinal fluid NSE and LDH were considered to be very effective markers in the diagnosis of and the determination of prognosis of neonatal CNS diseases.

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A retrospective study was made on 7 cases of post-traumatic syringomyelia, which included 4 male and 3 female patients. Initially 5 cases were incomplete paraplegic and two other cases showed complete transverse lesions of the cervical cord in one case and of thoracic spinal cord in another case.
The period between the time of initial trauma and the time of the onset of syringomyelia ranged from 1.5 years to 22 years and diagnosis were made at 3 months to 4 years after the onset of initial symptoms, which were brought by straining, coughing or other stressful activity. Initially four patients complained of pain above their original spinal levels. Dissociated sensory loss and flaccid motor weakness were characteristically noticed mainly in their unilateral upper extremities. Sensory impairment in the trigeminal areas was found in 5 cases.
Delayed CT scanning after myelography and magnetic resonance images (M. R. I) were extremely useful in diagnosing syringomyelia. Especially MRI was useful not only to determine the site of myelotomy but also to evaluate the post-operative results. In all seven cases bi-directional syrinx-subarachnoid shunting was carried out to improve the circulatory dynamics of cerebrospinal fluid at the site of primary spinal cord lesions. Neurological improvement, especially motor recovery was satisfactory and no serious complication was noticed postoperatively.
Pathogenesis of this condition was discussed and the partial meningeal adhesion at the originally injured site was thought to be a major causative foctor.
This surgical procedure was thought to be a choice of treatment to manage the neurological symptoms in this condition.

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Mass-screening for neuroblastoma using infant's urine has been carried out in Sapporo City for seven years, and 22 patients with this tumor were found to be treated. The incidence of detection was 1: 5, 500 babies tested. Patients in early stages I, II IV-S amounted to 786, and the total number of the patients under the age of 4 years old showed no change before and after the start of mass-screening. It indicates that the tumors were detected in early stages and younger ages.
On analysis, amplifications of N-myc gene were not found in any case. However, there were many types of chromosome pattern, as well as clinical features, suggesting heterogenous character of those neuroblastomas.

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The Prader-Willi syndrome is a disorder characterized by hypotonia and feedig difficulty in infancy followed by polyphagia, obesity, short stature, hypogonadism and mental retardation throughout the remainder of life. The etiology of the syndrome remains controversial. With a recent developed high-resolution chromosome banding technique, Ledbetter et al. (1981, 1982)successfully demonstrated an interstitial deletion of a chromosome 15 affecting the q11-12 region in 23 of their 45 patients with Prader-Willi syndrome.
In order to investigate the relationship between Prader-Willi syndrome and the 15q deletion, we performed high-resolution chromosome analyses in two diagnosed patients with the Prader-Willi syndrome and three suspected infants.
High-resolution chromosome analyses with a modified ethidium bromide technique revealed an interstitial deletion of the proximal 15q in the both diagnosed patients and in all of the three suspected infants. The affected region of all the patients was a sub-band 15q 11.2. These findings indicate that the frequency of the 15q deletion in strictly defined patients with the Prader-Willi syndrome is very high, and that the deletion is etiologically related to the syndrome.
The early diagnosis of Prader-Willi syndrome is important clinically and may have merit in helping prevent patients from developing obesity and diabetes. However, it is often difficult to establish the diagnosis in infancy, and in most cases the diagnosis can-not be confirmed until early childhood, when the clinical picture is fully developed. At this point, chromosome analysis with a high-resolution banding technique is a useful method in the early diagnosis in young infants.

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(1) According to year-specific changes in the numbers of patients with eating disorders, who consulted the Department of Neuropsychiatry at Keio University School of Medicine Hospital for the first time during the last 14 years, the number of cases with these disorders has increased every year to become almost nine-fold over this period. In addition, the percentage of patients with eating disorders increased from 0.2% to 2.6% of the total patients who consulted the department for the first time. (2) Concerning the rate of this disease in female patients who consulted this department for the first time in 1986, this disease accounted for in 12.5% of 15-to 24-year-old females. This disease occurs most frequently in this age group, especially in females, and 15-to 24-year-old patients accounted for up to 77.4% of the total patients with this disease. (3) One hundred and fourteen patients with this disease were treated over 3 years at the special dispensary for eating disorders at Keio University established in January, 1985. There is so much need for this dispensary. (4) The 54 patients with eating disorders, who were treated at the Department of Psychiatry at the Second Tokyo National Hospital between February 1986 and January 1987, comprised of 24 patients with anorexia nervosa and 30 patients with bulimia. There were more patients with bulimia than those with anorexia nervosa. Almost 60% of patients with anorexia nervosa requried hospitalization, but in contrast, less than 30% of patients with bulimia required hospitalization.

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Adjustment disorders with obsessive compulsive state, withdrawal or depressed mood, accelarated by the social factors, have been increasing in number, among from younger population through mid-, or late adulthood. Behind them as described in this paper, unsolved conflicts between parents and children were found in great degree which led us to consider further upon the problems of the reconciliation between them. Many suggestive evidences were brought about to us from the related areas, i.e. linguistics, antholopology, psychopathology and others. Going back to our history, traditions, and early religions, we aimed to explore the archetype of the reconciliation and the meaning of the burden accompanying with it. The thema “reconciliation- sacrifice-revival” might be one of the archetypes we are interested in. Though various factors to be resolved as in our cases, reconciliation should have more basic role in our society. Reconciliation is not a passive ceremony to pass through, but it should be the active or positive field we have to face and get over for the succession of the law we have to carry on and develop.

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A case of a 60 year old patient with Buerger disease who had an ulcer in the right third finger is reported. He had been smoking 60 pieces for 30 years. We found the thrombosis in his ulnar artery by arteriography. We let him to stop smoking and infuse prostaglandin E₁ into vein. We think it rare that the patient with Buerger disease has an ulcer in only one finger.

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Interferon therapy has been reported to have beneficial effect mainly on HBe-antigen-positive chronic hepatitis type B. However, some patients with anti-HBe-positive chronic hepatitis type B had positive DNAP and HBV DNA in their serum as a marker of HBV replication. Several investigators suggested that interferon therapy might be also effective in such patients.
We treated 2 patients who had anti-HBe-positive chronic hepatitis type B with beta interferon in total doses of 84 million units and 102 million units respectively. Both patients had positive DNAP, HBV DNA in their serum and elevated serum aminotransferase levels. Biopsy specimens in 2 patients obtained before beta interferon therapy showed chronic active hepatitis in hepatic histology.
In these patients, elevated serum aminotransferase levels decreased and DNAP, HBV DNA disappeared from their serum during beta interferon therapy although in one patient DNAP and HBV DNA became positive transiently after beta interferon therapy. These findings implied that further follow-up study was necessary to decide whether the effect of interferon therapy was transient or not. In conclusion, these preliminary findings, although not adequately controlled, suggest that beta interferon therapy may be effective in controlling the disease activity in patients with anti-HBe-positive chronic hepatitis type B.

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The patient, 74-year-old man who had been followed up for hypertension, ischemic heart disease and emphysema, was admitted to the hospital because of intractable diarrhea continued one month. His hepatic duct and choledocus had been detected to be dilated by echography for years. On admission he was thin (reduced weight 9 kg), had no joundice, no anemia, no liver swelling nor Courvoisier's sign. Laboratory tests showed elevation of CEA (9.1U/ml) and serum amylase (414U/L).
ERCP revealed anomalous connection of the pancreatic and biliary ducts. He was emaciated and became icteric. PTCD was done. Adenocarcinoma cells were shown by cytological examination of PTCD fluid. Angiography showed the tumor invasion to gastro-duodenal and gastroepiploic arteries. We had to give up operative resection.
Autopsy findings showed that the pancreatic duct connected to the biliary duct on 3 cm apart from papilla Vateri and occuluded completely 1 cm from the connective portion. The pancreas was replaced by duct cell carcinoma from the head to the tail, and in-filtrated to backward and laterally. The tumor pressed portal vein and inferior mesenteric vein and infiltrated to para-pancreatic, paracholecystic lymphnodes. Thus, diarrhea had occured. As the cancers of biliary duct are common in ACPB, we were suspicious of it in this case, but the duct cell carcinoma of the pancreas, which produced DEA, was moderately differenciated and scirrhous, was shown.

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This case is a 38-year-old woman who had first noticed a painless breast lump two days prior to a visit to our hospital when it was about 5cm oval-shaped, firm mobile mass located in the upper outer quadrant of her right breast. On mammographic and sonographic study the oval smooth contour with lobulation of the tumor was not characteristic of the usual carcinoma. Findings from a fine needle aspiration biopsy were consistent with lymphoma. Gallium scan showed high uptake only in the mass. Results of blood studies were within normal limits except for slightly abnormal LDH. Patey's modified mastectomy was performed. Regional lymphnodes were without metastasis histologically. Subsequent evaluation, including whole body CT scan, bone scan, liver ultra-sound, bone marrow aspiration, GI-series and indirect laryngoscope did not reveal other foci of lymphoma. Postoperatively she was given three courses of chemotherpy with epi-VEPA (epiADM 50mg, CPA 500mg, VCR 1mg and PDN 40mg 1-4d) in one month and another six courses at outpatient clinic as once in every month schedule. She is free of the disease at present time, one and half years after the operation.
Clinical diagnosis of breast lymphoma and the prognosis of this particular case is discussed.

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Meningiomas within the lateral ventricle in children are extremely rare. The present case was a 14-year-old girl, who had convulsion as an initial symptom. On her admission, clinical features were the right mild hemiparesis and slow speech. A CT scan and MRI revealed a homogeneously-enhanced mass at the left trigone. Cerebral angiography showed enlargement and displacement of the anterior and posterior choroidal arteries. On March 1, 1989, an operation was performed with the left middle temporal gyrus approach, and the tumor was totally removed. A histological diagnosis of meningothelial meningioma was made. The postoperative course was uneventful and she recovered almost completely.
Incidence of lateral ventricle meningioma is 1-2% of all meningiomas. Patients with meningiomas of the ventricles are usually asymptomatic until it reaches to some size. The CT scan, MRI and angiography are quite useful for the diagnosis of intraventricular meningiomas. Early diagnosis and surgical removal permits better results.

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Four cases of spasmodic torticollis were treated surgically. In all cases, vascular compression to the spinal accessory nerve at the level of C₁-C₂ was denied. The accessory nerve was severed at the level of Cl in 2 cases, at the level of C₁ and jugular foramen in 1 case, and at the level of jugular foramen in 1 case. In all cases, the symptoms improved after the operation. However, the patients' satisfaction was not enough except in 1 case, in whom there was no spastic movement at all.

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In order to study the cause of hematuria, ultrasonography and computed tomography were performed on the 13 cases of essential hematuria, and 17 cases of asymptomatic microhematuria with no urologic abnormalities. Ultrasonographic images in essential hematuria showed 8 cases of “Nutcracker syndrome” like congestive changes in left renal venous system, and one case of retroaortic left renal vein which was one of congenital anomalies of renal venous system. Same congestive changes were found in the 13 cases out of 17 cases of asymptomatic microhematuria. These results coincided with the results of CT images. These results suggest that there are lot of cases of “Nutcracker syndrome” like changes in the cases of hematuria. Ultrasonic examination of the renal venous system is very useful to study the cause of hematuria.

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