Recent advance in the treatment of acute lymphoblastic leukemia (ALL) in children has attained more than 70% of event free survival for long term in children with ALL, which is considered curable. The advance depends on suitable therapeutic regimen for risk groups, improving chemotherapies with multi-drug agents and developing supportive cares. Identification of prognostic factors which include age, white blood cell count, immunophenotype and so on has allowed classification into standard and high risk group. The treatment regimen of standard risk children consists of remission induction therapy with vincristine, prednisolone and L-asparaginase, central nervous system prophylaxis with cranial irradiation and intrathecal chemotherapy, consolidation and maintenance therapy. The high risk children are treated furthermore with intense multi-drug chemotherapy, especially in early remission period. Intensive chemotherapy can be tolarated under supportive cares, such as prevention of enfective complication, of drug toxicities and of bleding, and hematopoietic growth factors. Bone marrow transplantaion has been increasingly used as a potentially curative therapy in children with refractory ALL. Devices of less toxic therapeutic regimens in standard risk ALL, and of more effective regimens in high risk ALL are expected

View full abstract

From 1983 to 1987, 140 cranioplasties following decompressive craniectomy were performed. The cranioplasty infection rate was found to be 7% with the prominent organisms being Staphylococcus aureus and Staphylococcus epidermidis (50%). We found that all of these organisms were resistant to the standard therapy of penicillin and first or second generation cephalosporins. We felt that this resistance was secondary to preoperative use of antibiotics.
In a small study of 17 patients, we preoperatively performed skin cultures at surgical site. We found that of the patients who received multiple antibiotics or long term antibiotic therapy (group I), all were found to harbor organisms resistant to penicillin and first or second generation cephalosporins, while there was no resistant organisms found in those patients who were not treated preoperatively with antibiotics or were treated for less than 7 days (group II).
We conclude that preoperative use of antibiotics increases the risk of Staphylococci resistance to standard therapy of penicillin and first or second generation cephalosporins, and that preoperative skin culture taken from the operative site aids in the early identification of resistance

View full abstract

The effect of hemodialysis on left ventricular performance in 10 uremic patients was studied utilizing echocardiography. Left ventricular function curves constructed by a lower body negative pressure method were compared before and after dialysis. Although hemodialysis caused a decrease in both left ventricular end-diastolic volume index from 93±27 to 87±30ml/² and stroke volume index from 55±17 to 50±20 ml/², the left ventricular function curve did not show any detectable shift. Thus, a decrease in stroke volume after dialysis was attributable to the reduction in preload. It is concluded that changes in cardiac performance secondary to dialysis are due mainly to the Frank-Starling mechanism and the contractile state of the left ventricle remains unchanged

View full abstract

It is quite difficult to judge whether various ECG abnormalities in asymptomatic alcoholics reflect latent stage of alcoholic cardiomyopathy, because several deterioration observed at alcoholic withdrawal stage modify ECG findings. To assess the ECG abnormalities during withdrawal stage, we studied the consecutive changes of ECG abnormalities after abstinence in 48 asymptomatic alcoholics admitted within 48 hours of last drink. The incidence of high QRS voltage (Sv₁+Rv₆36mm, Sv₁+Rv₅>40mm, and Sv₂+Rv₅>45mm) were 25%, 10%, and 13%, QTc prolongation (QTc>0.43 sec), 71%, 27%, and 15% on admission, 7th, and 30th hospital day, respectively. The consecutive changes of their correlations with drinking profile, electrolyte imbalance, blood pressure, heart rate, cardiac autonomic nerve dysfunction assessed by heart rate variability were studied by linear regression analyses. Heavy daily alcohol consumption, electrolyte imbalance, cardiac autonomic nerve dysfunction were related with ECG abnormalities at early withdrawal stage. With the improvement of ECG abnormalities after abstinence, these relationship became poorer.
These results suggest ECG abnormalities in alcoholics are affected by several deterioration at early withdrawal stage

View full abstract

Recently the number of cases of colorectal cancer has been gradually increasing in Japan and there is a close relationship between colorectal cancer and polyp. With the improvement of endoscopic polypectomy, further informations concerning colorectal polyps such as diagnosis, treatment and histopathology were obtained. But long term courses after colorectal polypectomy still remained uncertain. And now we investigated the cases of colorectal polyps which were followed up after polypectomy and analyzed new polyp genesis. From 1983 to 1987, we followed up 88 cases with 145 polyps after polypectomy at our department. There was a tendency to finding new polyps in cases when over 3 polyps were polypectomized at initial polypectomy, and also in cases that were followed up over 3 years after initial polypectomy. Regarding the site, new polyps were frequently found in the sigmoid colon. Careful and strict follow up observation of the sigmoid colon seemed necessary

View full abstract

Nineteen cadaveric renal transplantation were performed or mediated in a local renal transplantation center of the Nagasaki Chuo National Hospital from October 1983 to April 1988. We investigated renal function, graft survival and complications in these cases.
Kidneys were perfused with cold Euro-Collins' Solution in situ after cardiac arrest and performed nephrectomy. Mean warm ischemic time (WIT) was 19.6 minutes. All cases but one with renal arterial thrombus exhibited renal function and seceded from dyalysis.
Immuno-suppressive methods consisted of conventional therapy (Cony group) in 5 cases and ciclosporin therapy (CsA group) in 14 cases. Total graft survival was 73.6% at 6 months and 70.5% at 1 year after transplantation.
Complications were more frequent in poor renal function group. Candidiases and viral infection were found more frequently in CsA group than in Cony group

View full abstract

Feeding functional disorders are prevalent among severely handicapped children. Since 1981 we have been running a feeding training programme for severely handicapped children institutionalized in our hospital. Developmental levels of feeding functions of 27 children were examined in 1983 and 1987, and changes in the levels during the 5-year-period were evaluated.
The following results were obtained:
1) Lip closing functions were significantly developed.
2) Tongue protrusions were markedly decreased.
3) Food manipulation skills inside the mouth were also significantly improved. Cases with reverse swallow were totally disappeared. Chewing movements and more matured swallowing functions were acquired in many cases.
4) As a whole, it was believed that the feeding training had been beneficial even for the severely handicapped children

View full abstract

The Palliative Care Unit (PCU) of Matsudo Byoin National Sanatorium opened in October 1987. This PCU which is not based on any specific religious background aims at palliating cancer patients' pain and supporting them to be comfortable and have a qualified human life with their family members. This PCU is the first PCU set up by the Ministry of Health and Welfare.
We analyzed the data of the PCU inpatients from October 1987 to September 1988. There were 71 patients, 54 men and 17 women. (1) The route of admission to the PCU 21 patients were transferred from other wards into the PCU. 18 patients entered into the PCU after being outpatients. 32 patients entered the PCU directly from their home or other hospitals. (2) Patient information about diagnosis and prognosis:27 patients were informed of their diagnosis and prognosis. 42 patients were not informed, and for 2 patients, their information could not be assessed. (3) The inpatients' religious background:50 patients didn't have any specific religious background, 10 patients were Buddhists. 1 patient was Christian. 5 patients belonged to recently founded religions. For 5 patients, their religions were unknown.
In this PCU, an appropriate medication of analgesics make the physical pain lesser and the patients' dignities remain until the time of their death. We belive that it is unneces-sary for palliative care to have a specific religious background. Palliative Care Units appears to be the best system for the terminal stage of cancer patients

View full abstract

Three cases of plunging ranula were reported and the literature reviewed. A mucous extravasation from the sublingual gland caused by trauma or obstruction of its duct appears to be the most likely cause of ranula. Plunging ranula probably occur via a hiatus in the mylohyoid muscle and appears clinically as swelling of the floor of the mouth exhibiting submandibular, cervical and/or parapharyngeal extension.
Since 1978, various procedures such as marsupialization or enucleation of the cyst, excision of the sublingual and/or submandibular gland and the combination of those proce-dures in 57 cases with plunging ranula have been reported in Japanese literature on oralmaxillofacial surgery and the oto-rhino-laryngeal division. The recurrence rate was 20% after enucleation of the cyst and excision of the submandibular gland, 15% after marsupi-alization and 0% after excision of the sublingual gland.
On the bases of concept that plunging ranula is a pseudocyst with no epithelial lining and a wall consisting of compressed fibrous connective tissue, the 3 cases presented were treated by intraoral extirpation of the sublingual gland and oral drainage of the cervical cyst. There has been no evidence of recurrence for 4-7 years after surgery. We recommended the intra-oral excision of the sublingual gland with oral drainage of the cervical cyst with no necessity of excising the cervical extension as an adequate treatment for plunging ranula. Care must be exercised to avoid damage to the lingual and hypoglossal nerves and the submandibular gland and duct

View full abstract

A high incidence of interstitial pulmonary fibrosis (IPF) in patients with neurofibroma-tosis has been reported since 1963, however, precise pathophysiology of this lung change is not clear. We examined a case of IPF with neurofibromatosis by means of CT of the lung, bronchof iberscope (BF), transbronchial lung biopsy (TBLB), bronchoalveolar lavage (BAL).
A 58-year-old woman was admitted to the hospital with a chief complaint of exertional dyspnea. The diagnosis of neurofibromatosis was made in childfood on the basis of multiple cafe-au-lait spots, biopsy of cutaneous neurofibromata, and similar features in her father. Laboratory data were as follows; Hb 12.3g/dl, WBC 8470/mm³, CRP(-), RA(++), ADNA(-), ANF(-), IgG 1820mg/dl, IgA 515mg/dl, IgM 443mg/dl, IgA 46 IU, BSR 82mm/h, PPC in sputum (-), Tubercukus bacilli (-), Tuberculin reaction (2×3mm), Arterial blood gas:pH 7.394, PO₂ 63.8 Torr, PCO₂ 42.6 Torr, Lung function:FVC 1340ml, %FVC 57.7% FEV1.0 1200ml, FEV1.0% 89.5%, RV 580ml, TLC 1990ml, RV/TLC 29.1, %DLCO 26.7%.
Chest X ray film showed diffuse ground glass like and fine nodulous shadow on both middle to lower fields. No bullous change was detected.
CT scans of thorax showed generally increased interstitial shadow, irregular thickened line and soft patchy lesions mainly in the lower field.
BAL showed an increased cell count (6×10⁵/ml), normal OKT4/OKT8 ratio (1.86) (OKT4 62.3%, OKT8 33.5%, OKIA1 76.3%).
Histological findings of TBLB were compatible with interstitial pulmonary fibrosis. Alveolar septa were thikened with infiltration of lymphocytes, and enlarged type II alveolar cells were noted.
It is known that neurofibromatosis can be associated with high incidence of IPF (7-15%), though the direct relationship between these two diseases is not clear. Genetic factor is considered from occurence in mother and son. IPF may be important in considering clinical course and prognosis of neurofibromatosis

View full abstract

Eight adults were admitted to the Seiranso Byoin National Sanatorium with the diagnosis of acute nontuberculous pleural empyema during a three year period (Sept., 1985-Sept., 1988). Six of the patients were men, and two were women. Their ages ranged from 32 to 64 years; six of them were older than 50. These eight patients had no history of tuberculosis. Their common presenting symptoms were high fever (39°C) and chest or back pain. Their chest films showed air-fluid collection, with different air-fluid levels on frontal, lateral, and decubitus projections. The right pleural space was more affected than the left.
All eight patients underwent closed chest tube thoracostomy and systemic chemotherapy immediately after pleural empyema was diagnosed. When the chest x-ray showed different air-fluid levels on a single projection, the empyema cavity was composed of multiple pockets within the pleural space. Even when the empyema cavity appeared to be a single round space, it was, in some cases, composed of discrete pockets which were noted to interconnect on computed tomography of the chest. Chest computed tomography was helpful for proper chest tube placement and consequent successful empyema drainage.
Pleural fluid cultures grew anaerobic bacteria (Peptostreptococcus) in two cases and aerobic bacteria in three (Streptococcus pneumoniae in one patient and an a-Streptococcus other than Streptococcus pneumoniae in two patients).
Six patients of the eight have diabetes mellitus. Two of these six were known diabetics before admission but not treated. Four were diagnosed as having diabetes during this admission. Diabetes mellitus is thus strongly considered a predisposing factor for pleural empyema

View full abstract

A 74-year-old male was admitted for The further examination of general fatigue and pitting edema in the lower extremities. At the time of his hospitalization, high perme-ability of the left lung on his chest x-ray film was pointed out and he underwent a further_ examination.
Lung function tests showed a restrictive pulmonary functional disorder. A perfusion lung scan and an inhalation lung scan showed a remarkable decrease in left pulmonary artery flow and ventilation. Cardiac catheterization and pulmonary angiography was done. Pulmonary artery pressure was normal and left pulmonary artery was markedly narrow. But any thrombus in pulmonary artery and collateral formation was not detected. Bronchography showed narrowing of segmental bronchus on the left side and especially of the lower lobe gathering of bronchus resulting in the loss of lung volume. Bronchoscopy showed no abnormalities. By these lung examinations we diagnosed as a case of Swyer-James' syndrome. As he did not have special respiratory problems, he hasn't been pointed out the abnormality of his left lung till the age of 74. It is considered that the cause of this disease is serious infection of respiratory system in the childhood and in this case at the age of 6 he had a history of serious pleuritis. His renal biopsy demonstrated the findings of membranous nephropathy (stage 1) which caused his nephrotic syndrome. After the administration of prednisolone 40mg/day he made a favorable progress and he wento into remission

View full abstract

A 16-year-old boy with Duchenne muscular dystropy developed adult respiratory distress syndrome subsequent to trauma in the chest. A mechanical ventilator was fitted immediately and a drip infusion of prednisolone and gabexate were started. Findings of arterial blood gas analysis and chest X-ray film showed transient improvement, but alveolar airarterial blood Poz difference increased and respiratory failure progressed. We performed the pulse therapy by drip infusion of methylprednisolone, 1g daily for 3 days, starting on the 4th hospitalday. His respiratory failure improved rapidly without remaining any compli-cations or residual signs and symptoms

View full abstract

We reported a case of Budd-Chiari syndrome without stenosis of inferior vena cava. The case was a 33 years old man who consulted a doctor because of general fatigue and liver dysfunction pointed out at the time of health examination. Ultrasonography was performed for screening of liver dysfunction and we found an obstruction of left hepatic vein and recognized a strong echo at the outflow portion of right hepatic vein. He was admitted to our hospital on October 8, 1986. By this time he hada moderate hepatomegaly, but he had no ascites, no edema in legs, and no venous dilatation in the abdominal wall. We did hepatic vein catheterization and confirmed the stenosis of right hepatic vein at the portion of just before the IVC, flowing of the mid hepatic vein into right hepatic vein, obstruction of the left hepatic outflow, and no stenosis at the hepatic portion of the IVC. We diagnosed the case as Budd-Chiari syndrome without stenosis at the hepatic portion of IVC (Chiari disease).
In this case, an aggressive surgery was carried out. The stenosis was removed under the direct visualization, and the membranous obstruction of the right hepatic vein was seen macroscopically and histologically. There was a small hole, 2mm in diameter, in the center of the membrane. Presently, 2 years after the operation, he is healthy and has no abnormal findings in medical examinations and no ultrasonic findings.
In Japan, Chiari disease is very rare. We reported this case with 12 other Japanese cases of Chiari disease reported in the literature after 1970

View full abstract

The uterus, which was about to rupture, was excised by laparotomy from a 39-year-old housewife. The patient was diagnosed as having a right interstitial pregnancy and an invasive mole. The diagnosis was arrived at from abnormal vaginal bleeding in the ninth week of pregnancy, continuous right hypogastric pain, and moderate hyperemesis gravidarum. Examination also presented an ultrasonic echo and a high urine hCG value. A histological inspection was made of the endometrium.
The postoperative course was uneventful, and the urine hCG value has been maintained an LH level. This is a very rare case of an invasive mole that originated in the right interstitium

View full abstract

Adrenoleukodystrophy is characterized by progressive central demyelination and adrenal cortical deficiency.
In this case the hyperpigmentation was present at birth. He visited our hospital at the age of six, and we diagnosed this case as adrenocortical hypofunction using biochemical and endocrinological examinations; the high levels of plasma ACTH and β-MSH were suppressed by the dexamethasone test. The aldosterone levels in plasma and urine were elevated and the Na level in urine was decreased by a low NaCl diet and the urinary aldosterone level was increased by the administration of ACTH, which suggested normal mineral cortical function, and familiar glucocorticoid insufficiency. Hydrocortisone had been administered. After the patient's adrenal insufficiency had been improving for about 10 years, he developed neurologic abnormalities such as disturbance of gait, motor disturbance, and difficulty in school work at the age of sixteen, and left the senior high school. The brain computed tomography revealed large low density areas and calcifications around the bilateral posterior corn in the temporal region. The electroencephalogram showed spiky waves in the bilateral occipital areas. Very long chain fatty acid levels in plasma were high, and the diagnosis of adrenoleukodystrophy was made

View full abstract

A 37-year-old male gardener developed a trauma on the right middle finger tip in August 1987. There occured a severe pain associated with this lesion in January, 1988. When he first visited our hospital in February 29, 1988, a necrotic ulcer, 7 mm in diameter, with surrounding granulation tissue was seen on his right finger tip. Gram stain of this necrotic tissue was negative for bacteria and potassium hydroxide preparations of this material were positive for structures thought to be hyphae.
The debridement of the necrotic tissue was done, but new lesion invaded into the finger nail, which was removed. Fungus cultures from the necrotic tissue as well as the nail grew out typical Fusarium colonies and Candida yeasts. These yeasts were determined as Candida parapsilosis. Microscopically small uniseptate microconidia were seen, and typical multiseptate crescent-shaped macroconidia were produced as the culture matured. A biopsy from the necrotic tissue revealed amorphous eosinophilic lamelated substance containing numerous thick walled rather large PAS-positive plemorphic fungla hyphae.
Topical griseofulvin and miconazole were of no benefit. Good response was achieved with Amphotericin B by systemic administration and local application

View full abstract