Seven cases of hereditary spherocytosis (HS) treated by splenectomy at Sagamihara National Hospital between 1985 and 1988 were studied to evaluate the effectiveness of splenectomy against hemolytic anemia and jaundice.
The age range of the patients in this series was 6 to 56 years (mean age, 23.3 years), four being under 15. When these patients were reviewed, familial evidence of HS was confirmed in 4 children. Five (71.4 per cent) of the 7 patients had concomitant cholelithiasis, and so cholecystectomy was performed with the splenectomy, except in one patient, whose gall bladder had been removed at another hospital 23 years before because of cholelithiasis. The analysis of the gallstones was performed by spectrum analysis of infrared rays. In three cases, pigment gallstones were identified ; in two, calcium bilirubinate stones; and one had black stones.
The number of red blood cells (RBC) and the level of serum total bilirubin during the period from 2 months to 3 years after splenectomy were compared with the preoperative data. The mean (±SD) number of RBC was 361.0±24.3×10⁴/mm³ before splenectomy, 501.8±41.9×10⁴/mm³ at 1 month after operation and 543.3±35.1×10⁴/mm³ at 1 year after operation. The mean (±SD) level of serum total bilirubin was 7.47±2.54mg/dl before splenectomy, 1.27±0.59mg/dl at 1 week after operation and 1.18±0.45mg/dl at 1 year after operation. For HS patients, the splenectomy was very effective in improving anemia and relieving jaundice. The immediate postoperative course in all patients was uneventful; but late postsplenectomy fulminant infection was seen from 7 months to 3 years and 8 months after operation.

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For localization of parathyroid lesions, neck palpation, ultrasonography (7.5MHz), thallium 201-technetium ^99m(²⁰¹T1-^99mTc) scintigraphy and computed tomography (CT) were carried out in 24 surgically verified primary hyperparathyroidism (1° HPT). Seven patients (29%) were associated with thyroid diseases (follicular adenoma in 3 patients, adenomatous goiter in 2, papillary carcinoma in 1, and Hashimoto's disease in 1). In patients with palpable parathyroid glands, serum calcium level was very high (13.0±1.7mg/dl, mean±SD), and the mean weight of their tumors was greater than 2.0g (6.1±3.8g). Ultrasonography could detect all parathyroid tumors in group A (parathyoid gland ≥0.5g), while only 4 were detected in group B (parathyroid <0.5g). Serum calcium level (12.8±1.3mg/dl) in group A was significantly higher than that (11.3±0.6mg/dl) in group B (p<0.01). False positive results occurred in 3 patients. The errors were due to two thyroid nodules and one esophagus. ²⁰¹T1-^99mTc scintigraphy identified 93% of parathyroid lesions in group A, and 50% of those in group B. False positive image was produced by thyroid nodule in one patient. CT gave the lowest sensitivity among these image diagnostic methods. From these results, the ultrasonography and ²⁰¹T1-^99mTc scintigraphy were recommended as useful tools to detect the parathyroid gland greater than 0.5g. However, more sensitive methods are necessary to identify the small parathyroid galnds less than 0.5g.

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Values for serum tumor markers (CEAZ, LEAS, SLX, SCC, NSE, CA19-9, CA125, and TPA) were measured and compared each other in 95 cases of primary lung cancer. The positive rates of serum CEAZ and TPA were greater than 50%. The positive rates of SLX, SCC and NSE were higher in patients with adenocarcinoma, squamous cell carcinoma and small cell carcinoma, respectively. Concerning the relationship between clinical stage and the levels of tumor markers, the levels of each marker except for SCC increased in proportion as the stage advanced. The serum level of SCC was not related to the clinical stage and relatively high even in the early stage. The positive rates were increased by simultaneous assay of CEAZ and TPA (81.7%), and CEAZ, SLX, SCC and NSE (767%). We conclude that simultaneous assay of CEAZ, SLX, SCC, NSE was useful in the diagnosis of primary lung cancer.

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Our analysis and evaluation of various factors affecting the postoperative pyuria were based on observation of 476 patients who underwent prostatectomy at Atami National Hospital in the eleven years between 1977 and 1987. Among these patients, 380 underwent TUR-P and 96 underwent open prostatectomy.
Possible factors affecting the duration of pyuria included surgical method, age of patients, preoperative urinary infection, weight of resected prostatic tissue and postoperative complications. From our analysis, we concluded that each of the factors affected the duration of pyuria.
1. The average postoperative duration of pyuria among patients who underwent open prostatectomy was 76.6 days, and 65.9 for those who underwent TUR-P. The difference between the two groups was statistically significant (p<0.05).
2. The duration of pyuria tended to be longer in older patients. There was a statistically significant difference between those below 69 years of age and those over 80.
3. For those patients without preoperative urinary tract infection, the normalization period of urinalysis was shorter compared to those with preoperative pyuria.
4. As to the weight of prostate tissue resected in TUR-P, there was a statistically significant difference between a weight of over 20 grams and tissue weighing less than 20 grams. The heavier the tissue, the longer it took for urinalysis to normalize.
5. For both open prostatectomy and TUR-P groups, postoperative complications delayed the normalization of pyuria.

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In a retrospective psychological evaluation using WTSC (Wechsler Intelligence Scale of Children), WAIS (Wechsler Adult Intelligence Scale), and Yatabe Guilford personality inventory test for patients with Duchenne progressive muscular dystrophy (DMD) since Hara Byoin National Sanatorium became involved in inpatients. The following results were obtained. 1) Those with IQ less than 75 occupied 35% among 105 cases, which was compatible with general frequency, however, 22% of dead cases showed higher VIQ than PIQ. These patients showed relatively gradual progression except for those with heart failure onset. 2) Y-G test done in 157 cases showed the tendency of Calm type (emotionally stable, socially adaptable, passive type) comparing with healthy senior high school students. However, director type (emotionally stable, active type) was found to be significantly fewer. With lapse of time, blast type (emotionally instsble, socially inadaptable, active type) decreased and average type increased. 3) The results of personality, which were derived from analyzing eight factors in 83 dead cases suggested the following. 4) The relation between physical findings (obesity, scoliosis), cause of death and age of death against IQ and factors of personality was statistically analyzed in 83 cases. Scoliosis was found to be slightly correlated with age of death. 5) By preliminary study regarding a life worth living those with high IQ were found to show much more motivation and activity of daily living. We are planning not to test psychologically but to do by preliminary method in order to analyze the psychologic status deeply and more delicately as well.

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Two hundred adult patients with epilepsy were studied using a questionnaire with respect to their prolems. The problems were classified into the following 8 types.
(1) Problems directly related to seizures, which were further classified into 3 subtypes: (i) unpredictability of the seizure occurrence, (ii) social disadvantage caused by seizures and (iii) physical concerns, (2) social stigma, (3) marriage, (4) employment, (5) side effects of medication, (6) burdens caused by medication continuation, (7) personality and social relationship, (g) daily living and family relationship (arranged in order of frequency).
The relation of these problems to the medicosocial conditions and other related responses was analyzed to discuss psychosocial background of patients with epilepsy.

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We studied the clinical profile of the inpatients with intractable neurologic disorders in Tokushima Byoin National Sanatorium from 1984 to 1988. The total number of the inpatients was 148 during these 5 years and the number has been increasing year by year. At present we have 54 patients. The mean numbers of the new inpatients and the discharged patients for one year were 25 and 19, respectively. The mean period of admisson about 94 discharged cases and 54 cases now in admission was 13 and 38 months, respectively. There were 26 dead cases and 9 autopsied cases (35%) for these 5 years. There were 44 (30%) cases of Parkinson disease (PD), 28 (19%) spinocerebellar degeneration (SCD), 19 (13%) amyotrophic lateral sclerosis (ALS), 21 (14%) motor neuron disease except ALS (MND) and others.
At admission to our hospital, the number of bed-ridden patients was 97 (65%). In activities of daily living during admissoin, 13 patients out of 33 PD patients showed improvement, 13 out of 15 ALS patients showed progression and 12 of them died, and 9 out of 12 SCD patients and 11 out of 14 MND patients did not show any changes.

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Recently, therapeutic drug monitoring (TDM) has been introduced to many hospitals to control drug therapy appropriately. In this study, we have presented the TDM at Kure National Hospital.
If a doctor wants to know the drug concentration of in or outpatient, and then he brings the whole blood of the patient to the pharmacist office, a pharmacist assays the sample at any time. This is a strong point in this hospital. The data is sent to the doctor as soon as possible. Routinely, we have assayed the concentration of phenytoin, phenobarbital, carbamazepine, sodium valproate, digoxin, theophylline, gentacin, amikacin and lithium.
We have done data analysis according to, the general therapeutic range and analyzed by microcomputer and have determined the dosage after conference with the doctor with consideration of clinical state of the patient.
We have given the doctor the predictive concentration after changing dosage form or administration route. We are presently studying this correlation of drug. Concentration by different administration routes using experimental animals. It is important to communicate with medical members, doctors and nurses to further develop the TDM system.

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One hundred and eighteen patients with end-stage renal disease under 18 years old in twenty-seven national sanatoriums up to August 1987 were studied through questionnaires. Their original diseases consisted of primary (61 cases) or secondery (11 cases) glomerular diseases, congenital renal and/or urinary tract anomalies (19 cases), Alport's syndrome (10 cases), chronic pyelonephritis (8 cases) and others (7 cases).
The onset and/or detection of the original disease was most frequent at the age from 6 to 9 years old (37 cases) and about 30 percent of patients were detected by mass-screening. Replacement therapy was started with hemodialysis in 71 patients, intermittent peritoneal dialysis in 28 and CAPD in 15, the latter having been increasing rapidly in recent years. Thirty-six patients had been introduced to renal transplantation (32 living, 3 cadaveric). At the last check up, 22 patients were alive with transplanted kidney, 61 were on dialysis, 17 were died and 16 were unknown. The survival rate at 1, 3 and 5 years was 91.6%, 81.6% and 78.8%, respectively. The major complications consisted of anemia (78.8%), hypertension (28.7%) and osteodystrophy (25.5%). The most common cause of death was cerebral hemorrhage (7 cases) and the prognosis of patients with lupus nephritis was poor. We pointed out some problems about early detection, diagnosis and treatment.

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We report a case of a revascularized isolated jejunal segment for the immediate reconstruction of cervical esophagus. A 73-year-old female was admitted to our hospital complaining of dysphagia and weight loss.
Esophagogram, CT scan, esophageal fiberscope and bronchofiberscope provided signs of mass located in the larynx and cervical esophagus.
The patient was treated by laryngoesophagectomy combined with bilateral modified neck dissections. The resected portion of esophagus was reconstructed with revascularized isolated jejunal segment and a permanent tracheostoma was performed.
In the case, jejunal vessels were anastomosed to the common carotid artery and internal jugular vein, respectively. Other details of the operative technique were essentially the same as described by other authors.
No serious postoperative complications were associated with this operative procedure and postoperative digestive functions of this patient was satisfactory.
We confirmed that a revascularized isolated jejunal segment was a reliable and effective method reconstructing the cervical esophagus with a high succcess rate and low mortality,

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OK-432 was intratumorally administrated at a dose of 10 KE to 11 patients with gastric cancer through an endoscope 4N7 days before surgery (treated group).
During surgery proximal and distal lymphnodes were aseptically extirpated to examine the antitumor immunological response of regional lymphnode on production of and response to Interleukin-2 (IL-2) and T-cell subsets.
Control were 15 patients with gastric cancer not given OK-432 (untreated group).
In the proximal lymphnode, proportion of CD4⁺4B4⁺ cells was increased in the treated group (p<0.05). In the distal lymphnode, production of and response to IL-2 were increased in the treated group (p<0.05).
These results indicated that the intratumor administration of OK-432 through an endoscope enhanced the antitumor immunological response of regional lymphnode in gastric cancer, and suggested that this method could be a clinically useful technique.

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Calcified gall bladder is known as porcelain gall bladder, and complicated frequently with carcinoma of gall bladder. But the concept of calcified gall bladder contains of calcifying carcinoma of gall bladder, that is calcified only in the cancer tissue. It is distinguished from so called porcelain gall bladder. We report a case of calcifying carcinoma.
Eighty-year-old man was admitted due to jaundice. Patty calcification was seen on the scout film of the abdomen. PTCD was performed because of obstructive jaundice. So his general condition was improved for about one year. But, jaundice recurred and he was hospitalized again. At this time, his condition was already inoperable, and died from cachexia.
Autopsy revealed calcifying carcinoma of the gall bladder that invaded directly into the hepatic hilum. And histological examination revealed that calcification existed only in the cancer tissue and metastatic lymph nodes.

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We experienced 9 cases of liver injury that were studied by abdominal CT scanning on arrival at the Nagasaki Chuo National Hospital, between April 1985 and October 1985. Four of them underwent emergency operation because of the progressive abdominal condition and/or the shock unresponsive to rapid fluid transfusion. The others were treated conservatively, and made a complete recovery and were discharged. Delayed rupture occurred in one case, necessitating TAE (transcatheter arterial embolization) to stop bleeding. Another case developed biloma, but the term of cure was shortened by percutaneous transhepatic drainage. Abdominal CT findings did not necessarily correlate well with the patient's condition. Decisionof operative versus nonoperative management should be based on the full evaluation of clinical symptoms of patients.

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A sixteen year old man was admitted to our hospital for further examination of marked weight loss, abdominal pain and nausea. After admission, he developed elevation of body temperature and palpable mass. Small bowel series showed Crohn's disease. After examination, he felt rebound tenderness on lower part of the abdominal wall. Emergency laparotomy was performed and huge inflammatory mass consisted with small intestines with pin hole perforation was demonstrated. Some literatures were also reviewed.

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We present a case of idiopathic retroperitoneal fibrosis demonstrating acute renal failure. The patient, a 53-year-old man, complained of lumbago and weight loss. He was referred to our hospital because of acute renal failure, on July 24, 1987. Serum creatinine, blood urea nitrogen, potassium, phosphate and erythrocyte sedimentation rate was elevated, and both CRP and antismooth muscle antibody were positive. Retrograde pyelogram revealed bilateral hydron'ephrosis due to the stenosis of bilateral ureters at the level of the 5th lumbar spine. Then construction of right percutaneous nephrostomy was done on the day of his admission, and that of the left was on August 6, 1987. Soon his renal function improved. Abdominal CT revealed a large retroperitoneal mass with the density of soft tissue. Based upon these findings, we made a diagnosis of idiopathic retroperitoneal fibrosis. After confirming histological diagnosis by biopsy from the mass, we performed bilateral ureterolysis and intraabdominal transposition. Seven days after operation, he began to be treated with steroid administration. Twenty-five days after operation, antegrade pyelogram demonstrated that bilateral hydronephrosis was reduced. We reviewed the association of autoimmune disease with idiopathic retroperitoneal fibrosis and recent therapeutic modalities for this disease.

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A 74-year-old lady had been given 30 mg of thyroid sicca daily since 1973 when the diagnosis of primary hypothyroidism was made. In April 1988, she was anorexic and felt sick. She was told by others that her face become dark, She visited a hospital and was suspected to have Addison's disease because of low blood cortisol and high ACTH levels. She entered our hospital for further examination acid medical treatment. The cause of her Addison's disease was thought to be tuberculous from reasons described below. Firstly bilateral adrenal glands were involved and both the cortex and medulla were impaired. Secondly, the strongly positive tuberculin reaction and the calcification in the lung on the chest X-ray film were observed. Moreover, the measurement of antibody against adrenal gland was negative. Daily dose of 25 mg of hydrocortisone was started in addition to the thyroid hormone. Blood ACTH levels, then, began to decrease and the pigmentation became less, She was fine again after the administration of hydrocortisone. Although it is considered to be rare that the tuberculous Addison's disease occurred with primary hypothyroidism, it is unknown whether these two conditions occurred coincidentally or some specific mechanisms are involved in this process.

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Chromosome analysis of the mentally and physically handicaped children revealed extra chromosome material on the short arm of chromosome no. 18 (46XX. 18p+) in a mentally retarded adult female with mildly dysmorphic features. The phenotypically normal father had a balanced translocation between the long arm of chromosome no. 1 and the short arm of chromosome no. 18. Thus this patient was trisomic for the long arm segment of chromosome no. 1, distal to q32.

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A 13-year-old boy of the Schönlein-Henoch purpura with generalized seizures and high frequency of sister chromatoid exchanges is reported.
He suffered from abdominal pain and nausea in July 1986 and after a few days developed blepharoedema, facial exanthema, hematuria, bloody stool and generalized seizures.
He was admitted to our hospital on September 18, 1986. His family history was negative. Urinalysis revealed hematuria and proteinuria. EEG showed diffuse paroxysmal slow waves. Needle biopsy of a kidney revealed mesenchymal proliferation and deposits of IgA, IgM and fibrinogen. Chromosome analysis showed high frequency of sister chromatoid exchanges. His parent had high frequency, too.
It is not known why generalized seizures occur in the Schönlein-Henoch purpura.
This may be the first case report of the Schönlein-Henoch purpura that showed high frequency of sister chromatoid exchanges.
This suggest that etiology of generalized seizures may be associated with genetic factors.

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We describe the results of FAB/MS analysis of urine sediments from a patient, 12-year-old boy, who had peripheral neuralgia, impediment in perspiration and angiokeratoma of his skin. Glycosphingolipids in urine sediments from the patient were extracted with chloro-form-methanol by the method of Folch's partition. Ceramide trihexoside (CTH) fraction in the glycosphingolipids was purified by silicic acid column chromatography and thin layer chromatography. The purified CTH fraction was analyzed by positive FAB/Mass spectrometry. The condition of FAB/MS analysis was as follows; first beam was Xe gas, emission current and acceleration voltage were 20mA, 2KV, respectively. JEOL JMS-DX 300 mass spectrometer was used in this study.
Glycerine, triethanolamine and triethanolamine-added NaOH were tested as matrices. As glycerine matrix was used, molecular ions, [CTH+H]+, weakly detected at m/z 1136 (composed fatty acid C24:0), 1134 (C24:1), 1108 (C22:0) and fragment ions were shown as the results from sugar chain cleavage, dehydration at m/z 1118, 1116 and 632. As triethanol-amine-added NaOH matrix was used, [CTH+Na]+ were strongly detected at 1158, 1156, 1130, respectively and S/N ratio was significantly made improvement than glycerine matrix. By the results of FAB/MS analysis of glycosphingolipids, chemical diagnosis of Fabry's disease could be made. This method could be applied to the detection of abnormal metabolic substances which are caused by hemi or heterozygotes with many inherited lysosomal enzyme deficiencies. FAB/MS method will be widely used to analyze many valuable substances in the section of clinical chemistry.

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