Normal Social Life Research Articles

Longterm outcomes and quality of life after Z-shaped anastomosis for Hirschsprung’s disease

Background: Z-shaped anastomosis is one of the modifications of Duhamel’s procedure that was designed to eliminate the blind rectal pouch and to achieve complete resection of the colorectal septum. It has been the most widely performed operation in Japan for many years. The longterm postoperative function of evacuation and quality of life of the patients are considered important to evaluate this procedure. Methods: At Kyushu University Hospital, from 1963 to 1997, 127 patients with Hirschsprung’s disease underwent Z-shaped anastomosis. As a result, 122 out of 127 patients (96%) survived. The present status and symptoms, and anorectal functions, including a manometric study and barium enema, were evaluated during the clinical followup. Results: A total of 99 of the 122 surviving patients (81%) were available for this study, and the mean postoperative period was 16 years. Evacuation scores in all patients were as follows; excellent, 62.2%; good, 28.6%; fair, 8.2%; and poor, 1.0%. The percentage of the patients who showed severe symptoms was 4.1% for diarrhea, 3.1% for constipation, 5.1% for incontinence, and 7.1% for soiling. The evacuation score improved chronologically and tended to reach a plateau at 10 to 15 years after operation, at which time 73% of the patients showed excellent outcomes and 95% were satisfactory (good or excellent). The appearance of a sense of defecation and an increase in the pressure difference between the anal canal and the rectum substantially contributed to the improvement in the defecation score. The appearance of the rectosphincteric reflex, including the atypical one, was seen in 40.5% of patients, but the appearance of a reflex did not seem to be related to the clinical status of defecation. Twenty-two of 30 patients older than 20 years were married, and 8 patients had children. Conclusions: The evacuation scores of in-patients undergoing Z-shaped anastomosis improved with age and were satisfactory (good or excellent) in most patients at least 10 years after operation. Most of the patients adapted to a normal social life.

PERCUTANEOUS DILATATION TECHNIQUE FOR POSTOPERATIVE BENIGN BILIARY STRICTURES

Effect of percutaneous dilatation technique using a stent tube obtainable internal and external biliary drainage indwelling for management of postoperative bengin biliary strictures was evaluated in this study. Among 24 cases of postoperative benign biliary stricture encountered in Teikyo university hospital at Mizonokuchi for the past 15 years, 19 cases treated with percutaneous dilatation technique were involved in this study. The route for percutaneous cholangioscopy were PTBD fistula in 15 cases (87.9%) and T-tube fistula in four cases (21.1%). In the patients with biliary fistula, the stent tube was in place after mechanical dilatation of the fistula under fluoroscopy. Dilatation of stricture site was also mechani-cally performed along a guidewire inserted under an endoscopic control. The size of the stent tube used ranged from 12 Fr to 18 Fr in diameter. The mean period of indwelling time of the stent tube was 125.1 days. Regarding complications, two patients had liver dysfunction and one patient had cholangitis, but the former two patients were treated conservatively. Two patients with re-stenosis and one patient of repeated cholangitis required surgical treatment. The remaining 16 patients (84.2%) had been leading a normal social life without any symptom of stenosis of the biliary duct. In conclusion, percutaneous biliary dilatation technique using a stent tube obtainable internal and external biliary drainage seemed to be an effective procedure for postoperative benign biliary strictures, because it can be treated without any foreign body left behind.

A CASE OF BRONCHIAL ARTERY-PULMONARY ARTERY COMMUNICATION

We report a case of bronchial artery-pulmonary artery communication. A 36-year-old man was seenat our hospital because of a chief complaint of hemoptysis. CT scanning revealed cylindrical brow chiectasis, but bronchoscopic and cytologic examination presented neither endoscopic nor cytologic abnormalities. He was followed-up, but then admitted to our hospital because of massive hemoptysis. Bronchial angiography revealed a communication between the bronchial and pulmonary arteries. Bron-chial artery-pulmonary artery communication was considered to be derived from bronchiectasis. There-fore, right middle lobectomy was performed. He is now leading a normal social life two years after the surgery without hemoptysis. Communications between the bronchial and pulmonary arteries due to bronchiectasis are very rare, and only 14 cases including ours have been reported in the Japanese literature.

Classification and Treatment of Vertebral Dissecting Aneurysm

Background For many years, dissecting aneurysms of the intracranial vertebral artery were believed to be quite rare. In recent years, because vascular disorders have been studied more thoroughly by three dimensional-computed tomography (3D-CT), angiographically and pathologically, these aneurysms are being reported with more frequency. Methods Among the 45 patients diagnosed to have aneurysms arising from the vertebral artery or its branches over a 20-year period, 16 had dissecting aneurysms. The authors present their therapeutic strategy for these patients. Surgery was performed in the 16 patients, the most common technique being clip-occlusion or trapping of the parent artery wherever feasible, in an attempt to optimize cerebral blood flow. The dissecting aneurysms of the vertebral artery were classified into two groups for the purpose of determining a therapeutic approach, namely unilateral and circumferential groups. In the unilateral group, the dissection seemed to involve only on one side of the vessel according to the conventional cerebral angiogram. These patients underwent surgical reconstruction of the vertebral artery by direct clipping. In the circumferential group, the dissection was all around the artery. Proximal clipping or trapping was performed in this group. Results In six out of eight patients with unilateral dissecting aneurysms, vascular reconstruction was possible by direct clipping. Of these six patients, the surgical outcome was considered excellent in four, fair in one, and one patient died of cardiac failure after 12 days as his preoperative morbid condition remained the same after surgery. Two other patients with unilateral dissecting aneurysms were treated with trapping technique and the surgical outcome was excellent in one patient and good in the other patient. Both patients resumed a normal social life. In five out of eight patients with circumferential dissecting aneurysms, trapping or proximal clipping was performed and the surgical outcome was excellent in two patients, good in one and fair in one patient. One patient with preoperative brain stem infarction died of aspiration pneumonitis after 8 months. Two patients who were noted to have an increase in the size of aneurysm during follow-up angiography underwent a craniotomy with clipping and wrapping of the aneurysm. There was a favorable surgical outcome in both patients. The remaining three patients had Grade IV subarachnoid hemorrhage (SAH) prior to surgery and at autopsy a disturbed vascular wall was detected. Conclusion The authors’ experience suggests that when surgically feasible, direct clipping is an effective alternative approach in the treatment of dissecting aneurysms of the vertebral artery in which blood flow in the parent artery is to be preserved.

Long-term results of treatment for craniopharyngioma in children.

Results of the treatment of 27 children with craniopharyngioma are reported. A subfrontal pterional approach was used in 55.6% of cases, a transsphenoidal and a transcallosal or transcortical approach in 25.9% and 18.5% of cases, respectively. Radical removal was the goal of surgery and was achieved in 70.8% of cases treated as primary surgery. The operative mortality was 3.7% and was due to hypothalamic failure. Most (81.4%) patients were followed up, for a mean of 7 years. Patients were evaluated according to a functional evaluation scale and outcome categories proposed by us. The scale takes account of tumor (recurrences and their eventual evolution); visual functions; endocrine functions (mainly hypothalamic), attainment of endocrine balance and drug regimen; headache; and psychosocial function. Recurrences were observed in 17.6% of patients treated with radical surgery and in 42.8% of those treated with limited surgery plus radiotherapy. A progressive amelioration of visual, endocrine and neuropsychosocial functions from the intervention to follow-up was observed in the majority of patients. Complete tumor excision was associated in 85% of cases with a low score on the functional scale, reflecting a high functional performance. Adequate substitution therapy maintained endocrine balance in 81% of patients. Since the intervention a progressive decrease in the number and dosages of medications has been observed. The majority of patients were again able to lead a normal social life. Small stature, obesity, headache, and emotional and sexual disturbances were frequent cause of long-term disability even despite adequate drug regimens. The functional evaluation scale we propose is a simple and effective tool that can be easily used during routine evaluation of patients with craniopharyngioma.

Teaching young people to respect human dignity: Contribution of the International Red Cross and Red Crescent Movement

Young people are the focus of special interest in studies on the humanitarian, social and political situation throughout the world. As “victims”, their plight attracts particular attention on account of their vulnerability, which is recognized in all cultures everywhere—albeit with considerable variation in views as to the age of reaching adulthood. If they belong to “deviant” groups such as street children, criminals, children outside the school system or child soldiers, they are treated as victims, permanent outcasts or a threat, depending on where they are and what they do. Lastly, those who belong to a “controlled” group, in other words those enjoying a normal social and/or school life, are subject to demands which are all the greater given their elders' own disarray in face of the accelerated pace of change at the turn of the century, and the adults' desire to prepare the rising generation to cope with an uncertain future.

チタン合金髄内釘を使用した大腿骨・下腿骨骨折の治療経験

We have been treating femoral and tibial fractures using the interlocking titanium nail since 1992. We investigated the results of 14 fractures in 14 patients. The 14 patients were 9 males and 5 females with an average age at the time of surgery of 48 years (range: 15 to 75 years).There were 2 fractures with delayed union. One was a crush fracture of the femoral shaft and another involved a large fragment. All other others fractures achieved good bone union. There were no infections.Partial weight bearing began 1-8 weeks after operation (average time 2.9 weeks), and full weight bearing began 2 to 12 weeks after surgery (average 4.8 weeks). Patients were able to return to their normal social life at an early stage.

Ocular non-Hodgkin’s lymphoma: a clinical study of nine cases

BACKGROUNDPrimary oculocerebral large cell malignant non-Hodgkin’s lymphoma, formerly called ocular reticulum cell sarcoma, runs a uniformly fatal course. Once the central nervous system (CNS) is involved, survival without treatment is...

Epilepsy and everyday life risks. A case-referent study: rationale, study design, and preliminary results. Risk in Epilepsy Study Group.

Despite its favorable outcome in up to 90% of patients, epilepsy is still considered to carry a higher than normal risk of morbidity and accidents. Knowledge of the everyday life risks (illnesses, accidents) in patients with epilepsy is mostly anecdotal. For these reasons, a multinational cohort study has been started in 9 European countries to investigate the incidence and number of medical events and accidents in children and adults with unprovoked epilepsy (diagnosed in the recruiting centers in the previous 10 years) and in age- and sex-matched controls (relatives or friends). The 2 groups are being followed for at least 12 months. Using patients' diaries, data are being collected regarding the number and type of illnesses and accidents, the number of medical contacts and the actions taken, the number of hospital admissions, and the total number of days off work/school. In a preliminary report on 833 patients and 833 controls, the cases reported slightly less illnesses, medical contacts, and actions taken by doctors. Accidents and related events occurred in similar proportions in the 2 groups. Based on the results of the pilot study, idiopathic, cryptogenic and remote symptomatic epilepsy may be compatible with an at least fairly normal social life.

An Alternative View of Outcome in Autism

Parents often rely on the knowledge and experience of others to explain the longitudinal outcomes in autism. Unfortunately, limitations keep parents from obtaining accurate and relevant information. First, professionals with limited experiences are not likely to understand the range of outcomes for persons with autism. Second, previous studies have used narrow explanations to define outcomes. Past researchers have generally defined good outcome as the development of a normal social life and independence by adulthood; and they have defined the best predictors as verbal and IQ levels. Because most individuals with autism continue to struggle with social relationships and need individualized supports, this outcome is rare. Parents, educators, and researchers need other frameworks now to characterize and conceptualize “good outcome.” Data on 46 individuals with autism will be used to present an alternative view of outcome. Some who would have been predicted to do poorly as adults because of their relatively low verbal, cognitive, and adaptive behavior levels were found to be leading satisfactory lives. To illustrate the multitude of variables that have an impact on outcome and to propose the claim that competency and quality of life are the best criteria for judging outcomes, we present four case studies.

A CASE OF ANGIOSARCOMA OF THE BREAST

A case of angiosarcoma of the brest which is a very rare entity with poor prognosis is presented in this paper. A 24-year-old female was admitted to the hospital because of a rapidly enlarging tumor of the right breast. A 10×10cm elastic soft and illdefined tumor was palpated in the right breast. The skin over the tumor was bluish in color. Histological study of open biopsied specimen revealed angiosarcoma. Modified radical mastectomy was performed. In this particular case, 700, 000 unit IL-2 was intravenously administered for 14 days as postoperative adjuvant chemotherapy. She is leading a normal social life 10 months after the surgery without any recurrent signs. A case of angiosarcoma of the brest is very rare and only 18 cases including our case have been reported in the Japanese literature. A careful follow-up study is necessary, because the prognosis of this entity is extremery poor, although histological findings only show a mild atypism.

Orthotopic liver transplantation for Crigler-Najjar type I disease in six children.

Hyperbilirubinemia in Crigler-Najjar disease type I (CN) can be partially controlled by daily phototherapy, but these children remain at permanent risk of developing brain damage due to kernicterus. Because liver transplantation is the only available curative treatment for liver-based inborn errors of metabolism, orthotopic liver transplantation (OLT) was performed in six patients with CN. Mean age at surgery was 52.5 months (range 27 to 100). Despite a mean daily phototherapy of 12.4 +/- 0.8 hr, mean bilirubin of the 6 patients was 388 microM/L (range 175 to 703) before OLT; one of them was also being treated with tin-protoporphyrin. All 6 had elevated AST/ALT, ranging from 1.4 to 6 times upper normal values. Complications occurred in three patients after OLT, including miliary tuberculosis in one, graft rejection and retransplantation in one, and hepatic artery thrombosis in one. All patients survive with normal serum bilirubin level (follow up 6 to 116 months). Four have normal enzymes on post-OLT follow-up (30 to 95 months), follow a normal education program, and have a normal social life. One recently transplanted patient has progressively normalizing liver function tests 6 months after OLT. One patient transplanted at 8 y.o. (now 116 months post-OLT) has moderate neurological delay due to pretransplant kernicterus, and posttransplant chronic persistent hepatitis. Our series shows that OLT cures hyperbilirubinemia in CN patients, with an excellent survival prospect. The procedure should be decided upon before neurological sequelae occur, since these persist after transplantation.

L'insertion professionnelle des jeunes transplantés rénaux

When young patients suffer from a chronic disease their mothers tend to be hyperprotective, doctors tend to prescribe unnecessary restrictions to their activities and fathers to think that all this is mothers' and doctors' concern. This keeps young patients away from the normal social life of their age. After analysing interviews of young patients of kidney transplants, we suggest, as a hypothesis, that this situation is an important negative factor resulting in bad adaptation to working conditions and excessive dependence on social funding.

An investigation of some implications of coeliac disease

Following even ‘simple’ dietary advice for long periods is problematic and substantial disruption of home and social life is likely. The variety and extent of problems faced by coeliacs has received little attention compared, for example, to that given to diabetics. Therefore the inconvenience caused to coeliacs by their diets was investigated using a simple questionnaire. Usable replies were obtained from 46 adult coeliacs and were compared to information from 46 matched ‘control’ subjects. There was evidence that coeliac disease did restrict activities and was a constant source of worry. About a quarter of coeliacs thought that it was impossible to have a normal social life. It is possible that women in particular, showed indications of an unacceptable level of anxiety. The Coeliac Society is an excellent vehicle for help but greater awareness of such problems amongst health‐care professionals would also be beneficial.

Pulmonary function one year after extracorporeal lung assist. A long-term follow-up of patients with acute adult respiratory distress syndrome

This retrospective follow-up studied lung function and reconvalescence in 38 young patients without primary lung disease, who suffered from severe ARDS and survived by means of extracorporeal lung assist (ELA) treatment. Over a period of 3, 6, 9 and 12-20 months dynamic and static lung volumes and the results of X-ray and CT scan of the thorax were studied. Within the third and sixth month the forced vital capacity, FEV1, and arterial blood gases reached the lower range of normal values. Obvious relative emphysema RV/TLC was observed at the time of discharge from hospital and during the first four months. This was found to reverse during the following months. After a period of 12-20 months all patients had an abnormal diffusion capacity (TLCO) but with normal transfer coefficients (TLCO/VA). Even though ARDS did not induce obstructive changes, the diminished diffusion capacity and the slightly reduced expiratory peak flow in 25-75% of FVC (FEF 25-75) indicates slight changes in the small airways. Following up the X-ray and CT results we found extraordinary morphological restitution. Spiroergometry results showed a normal cardiopulmonary pattern for untrained individuals. 36 of the 38 patients were integrated in normal working and social life within 12-20 months.

An appraisal of pediatric liver transplantation from living relatives. Initial clinical experiences in 20 pediatric liver transplantations from living relatives as donors.

The authors performed 20 liver transplantations from living related donors between June 1990 and July 1991. The 20 pediatric patients (14 biliary atresia, two Budd-Chiari syndrome, one liver cirrhosis after hepatitis C viral infection (HCV hepatitis), 1 progressive intrahepatic cholestasis, 1 liver cirrhosis, 1 protoporphyria) were transplanted with 11 left lobes, eight left lateral segments, and one right lobe. The choice of donors was restricted to the parents of the recipients. The immunosuppressive treatment consisted of FK 506 and steroids. Seventeen recipients are alive, 15 of whom are well and at home. Two recipients, who underwent emergency transplantation, died of postoperative complications. Another recipient died of accidental asphyxia at 6 months after the transplantation. All 20 donors had uneventful postoperative courses and were able to resume their normal social lives. The arterial ketone body ratio (AKBR) increased to above 1.0 within 2 days after the transplantation in all cases. Relatively mild rejection episodes were encountered in only two cases transplanted with ABO-compatible grafts, and these were treated successfully with steroids and FK 506.

Middle-aged women with Turner's syndrome. Medical status, hormonal treatment and social life

A study of 49 middle-aged (greater than 35 years old) women with Turner's syndrome was performed to evaluate medical status, hormonal treatment and social life. Most of the women lived a normal social life in stable relationships and all were employed. Some had adopted children and 4 had children of their own. They had all been informed about Turner's syndrome at time of diagnosis, but after the induced puberty they did not know who to turn to with their variety of medical problems. They were healthy except for reduced hearing, which in many cases required hearing aid. Elevated liver enzymes were found in almost all the women. The mechanism behind this finding is unclear, but it does not seem to imply severe liver damage why the indicated estrogen therapy should not be withdrawn from these women. Today amniocentesis and chorionic villus biopsies are commonly used to detect chromosome abnormalities. It is our duty as counsellors to give adequate information on the prognosis of a specific finding in the fetus to help future parents in their decision.

Acute revascularization for progressing stroke

The effectiveness of acute stage vascular reconstruction in cases of progressing stroke is reported. The clinical material consists of 28 cases of progressing stroke in the anterior circulation upon which vascular reconstruction was performed. Following admission, brain protective substances (500 ml mannitol, 500 mg vitamin E, 500 mg phenytoin) and dextran, were administered to the patients, and induced hypertension was also performed. Changes in symptoms were then observed and vascular reconstruction was carried out in cases where symptoms progressed. The vascular lesion was on the internal carotid artery in 8 cases and on the middle cerebral artery in 20 cases. Complete disappearance of neurological symptoms was obtained in 12 cases, whereas only mild neurological symptoms remained but a return to normal social life was possible in 11 other cases. Symptoms remained in three cases and there were two fatalities. In a long-term follow-up study, there were no cases of aggravation of symptoms due to ischaemic stroke. Moreover, the reconstruction was judged as effective on the basis of cerebral blood flow and metabolism. We concluded that acute vascular reconstruction for progressing stroke under the administration of brain protective substances is effective in preventing the progression of neurological symptoms.

Lateral meniscal cyst

Thirty-six patients underwent arthroscopic cystectomy and partial meniscectomy for a lateral meniscal cyst. In two other patients, an open cystectomy was performed following a diagnostic arthroscopy. The patients were reviewed at an average of 39 months (range 8-82) after the operation, and recurrence was found in four cases. Five patients, all keen sportsmen, were dissatisfied with the operation. Four patients, with arthroscopic signs of early degenerative arthritis, were still experiencing some pain, but were able to lead a normal working and social life. Arthroscopy is safe, giving low morbidity and recurrence. In the cases treated by arthroscopy, a horizontal meniscal tear was always found. The question whether this tear and the resulting meniscal changes are traumatic or degenerative in origin still remains; and, to some extent, neither hypothesis can be readily discarded. This study supports the use of arthroscopy alone in the treatment of lateral meniscal cysts.

Clinical review 23: The use of the long-acting somatostatin analog octreotide in the treatment of gut neuroendocrine tumors.

The SRIF analog octreotide (SMS 201-995) has been in clinical use for over 6 yr in the treatment of acromegaly and metastatic endocrine pancreatic and carcinoid tumors. The use of the analog in the treatment of acromegaly and TSH-secreting tumors is beyond the scope of this clinical review. Patient acceptance of the analog, given chronically by the sc route, has been excellent and side effects have been few with the exception of the development of gallstones. In endocrine pancreatic and carcinoid tumors the hypersecretion of hormones such as VIP, glucagon, and gastrin and the secretory products of carcinoid tumors (e.g. 5-hydroxytryptamine and tachykinins) and their clinical effects may be successfully blocked. This allows excellent palliation of such tumors and often enables the patients to return home and lead normal social lives. Initial hopes that long-term octreotide therapy would be an effective antitumor drug, reducing tumor growth, based on experimental animal models and human tumor cell lines, have not been born out in clinical practice. A reduction in gut tumor bulk due to octreotide, rarely or never occurs as a sustained phenomenon. Eventually a decrease in, and finally an absence of, clinical effectiveness occurs despite the reintroduction of other treatment modalities.