Endobronchial lipoma: review of 64 cases reported in Japan

BACKGROUND: Several recent studies discuss bronchoscopic techniques for treating endobronchial lipoma, an extremely rare benign tumor. OBJECTIVES: To describe the epidemiology of endobronchial lipoma and to propose appropriate therapeutic policies for treating this tumor. METHODS: We reviewed 64 cases of endobronchial lipoma: 33 cases previously reported in 30 different articles, and 31 case reports presented at thoracic meetings in Japan. RESULTS: Of the 64 patients included in this study (50 male and 14 female; mean age, 60 years), 40 patients had endobronchial lipoma in the right lung and 23 patients had it in the left lung. The overwhelming majority of the tumors (n = 61) were found in the first three subdivisions of the tracheobronchial tree. Forty-eight patients (75%) were symptomatic, and their symptoms included cough, sputum, hemoptysis, elevated temperature, and dyspnea. Additionally, abnormal radiographic findings were reported for 51 patients (80%): 18 patients had atelectasis, 14 patients had infiltration or consolidation, 6 patients showed volume loss of the lung, and mass shadow was identified in 9 patients, and another abnormality including pleural effusion was found in 4 patients. Forty patients underwent surgical resection: 4 pneumonectomies, 24 lobectomies, 8 bilobectomies, and 4 resections by bronchotomy. Bronchoscopic resection was carried out in 17 cases: 7 cases by Nd-YAG laser, 5 cases by electrosurgical snaring forceps, and another 5 cases with a combined therapy using both procedures. CONCLUSIONS: Bronchoscopic resection should be considered as the first choice of treatment for endobronchial lipoma; however, surgical therapy is indicated for patients who show the possibility of a complicated malignant tumor, who have destructive peripheral lung disease, who have extrabronchial growth, or who may have technical difficulties during the bronchoscopic procedure.     

Diagnosis and treatment of chronic gastroparesis and chronic intestinal pseudo-obstruction

Chronic gastroparesis and CIP are debilitating disorders that are difficult to treat with currently available therapies. Failure of proper migration and differentiation of enteric neurons or ICC can result from specific genetic mutations and lead to phenotypes of CIP with or without concomitant gastroparesis. Intestinal dysfunction in diabetes may reflect a depletion of NO production (and perhaps other neurotransmitters or modulators), which is manifest as a syndrome of gastroparesis, diarrhea, or constipation in individual patients. As the key molecular changes underlying these disorders are defined, clinicians will begin to understand their precise etiology and rational medical therapy may become possible. In the future, testable hypotheses regarding the etiology of other functional bowel disorders (e.g., functional dyspepsia, irritable bowel syndrome, and so forth) may be developed.     

Infantile chronic idiopathic intestinal pseudo-obstruction: the role of small intestinal manometry as a diagnostic tool and prognostic indicator.

BACKGROUND: Chronic idiopathic intestinal pseudo-obstruction (CIIP) presenting in infancy is a rare but serious condition of heterogeneous aetiology often with an uncertain outcome. AIM: To assess whether intestinal manometry in the first two years of life can help define a neuropathic or myopathic aetiology or clinical outcome, or both, in cases of infantile CIIP. SUBJECTS AND METHODS: 14 consecutive children who presented in the first year of life with CIIP were studied histologically and by small intestinal manometry. RESULTS: Five had a myopathic disorder, four were neuropathic, and five unclassified following histological investigation of full thickness intestinal biopsy specimens. Analysis of fasting phase III activity showed four patterns: (1) (n = 4) no detectable motor activity, (2) (n = 5) low amplitude phase III activity, (3) (n = 3) poorly formed phase III complexes of short duration, (4) (n = 2) well formed cyclical phase III activity with abnormal propagation. The seven children with low amplitude phase III: motility index (MI) < 10 KPa/min, all had a poor outcome (death or dependence on parenteral nutrition) after 1-10 years follow up, compared with two of seven of those with a MI > 10 KPa/min. Of the five with myopathic histology, four had a MI < 10 KPa/min. CONCLUSION: These results show that small intestinal manometry is useful not only as an aid in diagnosing the aetiology of CIIP presenting in infancy, but also in predicting outcome.     

Effects of erythromycin in chronic idiopathic intestinal pseudo-obstruction

The prokinetic effects of erythromycin, a macrolide antibiotic, on the gastrointestinal tract as a motilin receptor agonist and its potential value for the treatment of gastrointestinal motility disorders have recently attracted interest. The effects of erythromycin on the clinical symptoms and gastrointestinal motility of patients with chronic idiopathic pseudo-obstruction have not been investigated extersively. We presented a case of chronic idiopathic intestinal pseudo-obstruction, in a 67-year-old man in whom oral erythromycin (900 mg/day) dramatically improved postprandial abdominal distention, nausea, and vomiting. Other agents with prokinetic effects on intestinal motility, i.e., cisapride, domperidone, metoclopramide, and trimebutine maleate did not have a favorable effect. Gastric emptying, measured by the sulfamethizole method; and intestinal transit, evaluated using radio-opaque markers, were markedly improved by treatment with erythromycin. Our experience suggests that the prokinetic effects of erythromycin may be of therapeutic value in chronic idiopathic intestinal pseudo-obstruction.     

慢性假性肠梗阻的临床特征和诊断

目的 分析慢性假性肠梗阻（CIP）的临床特征和诊断方法。方法 对北京协和医院1978年11月至2000年12月诊断的23例CIP的临床资料进行了分析,并结合文献,总结CIP的临床特征和诊断方法。结果 23例CIP患者中男性9例,女性14例,年龄4-75岁（中位数50岁）。主要症状有腹胀（87%）、腹痛（57%）、腹泻（65%）、便秘（48%）、呕吐（39%）和体重下降（83%）。CIP患者腹胀突出而腹痛相对较轻,伴有腹泻、体重明显下降和消化道其他部位动力的异常;很少出现机械性肠梗阻的体征;腹部平片除存在液气平面（17/20,85%）外,可见肠管普遍扩张的结肠积气（13/20,65%）;消化道钡造影显示肠管明显扩张（19/23,83%）,肠蠕动减慢和消失（17/23,74%）;有关检查或（和）手术探查均未发现机械性肠梗阻的证据。病变主要累及小肠（83%）和结肠（39%）。18例为慢性特发性假性肠梗阻,其中1例为家庭性内脏肌病,5例继发于结缔组织病。结论 CIP的临床表现具有一定的特征性,凭借腹部平片的特点,有针对性地选择消化道钡剂造影,结合胃肠道动力检查的异常发现可对CIP做出正确诊断。     

Quality of life outcomes in congenital chronic intestinal pseudo-obstruction

The goal of this study was to assess the quality of life for children with chronic intestinal pseudoobstruction. We used a retrospective chart review to identify children with congenital chronic intestinal pseudoobstruction, then a structured telephone interview with parents that included the Child Health Questionnaire to gather information about the current status and quality of life for each patient and family. Children with chronic intestinal pseudo-obstruction had less freedom from pain, depression, and anxiety than healthy children or children with juvenile rheumatoid arthritis (P < 0.05 for all three parameters). Parents of children with chronic intestinal pseudo-obstruction had poorer emotional status than parents of healthy children or children with juvenile rheumatoid arthritis. The time required for parents to care for children with chronic intestinal pseudo-obstruction was greater than the time required to care for healthy children or children with juvenile rheumatoid arthritis (P < 0.01). In conclusion, the quality of life for children with chronic intestinal pseudo-obstruction lags behind that of healthy children and children with another chronic illness. Appropriate treatment of chronic pain may improve the quality of life for children with chronic intestinal pseudo-obstruction and their families. Moreover, attention to reducing each family's burden of time and emotional distress may help them cope better with their chronically ill child.     

Cronkhite-Canada syndrome. A case report and analytical review of 23 other cases reported in Japan

A case study is presented of a 57-year-old male who showed typical clinical features of Cronkhite-Canada syndrome. Numerous polypoid lesions were found in the stomach, duodenum, ileum, colon and rectum accompanied with characteristic ectodermal changes. Tests indicated a protein-losing gastroenteropathy. Intestinal lactase deficiency was demonstrated by the lactose tolerance test. Scanning electronmicroscopy of the gastric and colonic mucosa revealed prominent secretion of mucoid substances and distortion in the gastric pits and colonic crypts. These abnormal findings were interpreted as having a direct relationship to the loss of protein into the gastrointestinal tract.     

Liver cell adenoma: A case report and a review of 30 cases reported in Japan

A 38 year old man with a huge liver cell adenoma extending to the pelvis was treated by left lateral lobectomy. The tumour, which was well-encapsulated by fibrous membrane, was 18 × 19 × 6 cm in size and weighed 1500g. Histopathologically, this tumour was composed of well-differentiated liver cell trabeculae and a small number of pseudobiliary ducts, but no Glisson sheaths. For the past 4 years 8 months he has remained asymptomatic. Thirty cases of liver cell adenoma found in Japanese literature are reviewed here and possible pathogenic implications are discussed.     

Clinical characteristics of chronic idiopathic intestinal pseudo-obstruction in adults

Background—Chronic idiopathic intestinalpseudo-obstruction, a syndrome of ineffectual motility due to a primarydisorder of enteric nerve or muscle, is rare.
Aims—To determine the clinical spectrum,underlying pathologies, response to treatments, and prognosis in aconsecutive unselected group of patients.
Methods—Cross sectional study of all patientswith clinical and radiological features of intestinal obstruction inthe absence of organic obstruction, associated with dilated smallintestine (with or without dilated large intestine), being activelymanaged in one tertiary referral centre at one time.
Results—Twenty patients (11 men and nine women,median age 43 years, range 22-67) fulfilled the diganostic criteria.Median age at onset of symptoms was 17 years (range two weeks to 59 years). Two patients had an autosomally dominant inherited visceralmyopathy. Major presenting symptoms were pain (80%), vomiting (75%),constipation (40%), and diarrhoea (20%). Eighteen patients requiredabdominal surgery, and a further patient had a full thickness rectalbiopsy. The mean time interval from symptom onset to first operationwas 5.8 years. Histology showed visceral myopathy in 13, visceral neuropathy in three, and was indeterminate in three. In the one otherpatient small bowel motility studies were suggestive of neuropathy. Twopatients died within two years of symptom onset, one from generalisedthrombosis and the other from an inflammatory myopathy. Of theremaining 18 patients, eight were nutritionally independent ofsupplements, two had gastrostomy or jejunostomy feeds, and eight werereceiving home parenteral nutrition. Five patients were opiatedependent, only one patient had benefited from prokinetic drug therapy,and five patients required formal psychological intervention and support.
Conclusions—In a referral setting visceralmyopathy is the most common diagnosis in this heterogeneous syndrome,the course of the illness is usually prolonged, and prokinetic drugtherapies are not usually helpful. Ongoing management problems includepain relief and nutritional support.

Keywords:adult; intestinal; pseudo-obstruction; myopathy; neuropathy

     

Advances in our understanding of the pathology of chronic intestinal pseudo-obstruction

Chronic intestinal pseudo-obstruction (CIP) represents a particularly difficult clinical challenge. It is a rare and highly morbid syndrome characterised by impaired gastrointestinal propulsion together with symptoms and signs of bowel obstruction in the absence of any lesions occluding the gut lumen. CIP can be classified as either "secondary" to a wide array of recognised pathological conditions or "idiopathic" (CIIP). This review will focus on CIIP, and specifically on the underlying pathological abnormalities. Combined clinical and histopathological studies are needed to highlight new perspectives in the understanding and management of chronic intestinal pseudo-obstruction.     

Definition and diagnostic criteria of osteoporosis in Japan

The concept of osteoporosis was introduced in Japan after the World War II and become a major medical problem with rising social and economic consequence according to increasing population of old peoples. Osteoporosis is defined as " a disease characterized by low bone mass and microarchitectural deterioration of bone tissue, leading to enhanced bone fragility and a consequent increase in fracture risk". In Japan a diagnostic criteria was drafted for clinical practice in 1996 by Japanese Society of Bone and Mineral Research.     

Creating a pandemic of prediabetes: the proposed new diagnostic criteria for impaired fasting glycaemia

Aims/hypothesis In November 2003 the American Diabetes Association expert committee on the diagnosis and classification of diabetes mellitus suggested a revision of the diagnostic criteria for IFG, lowering the diagnostic threshold from 6.1 to 5.6 mmol/l. The aim of the present study was to evaluate the consequences of this change with respect to: (i) the prevalence of IFG in five different countries; (ii) the concordance between IFG and IGT (classification of individuals); and (iii) the cardiovascular risk profile of these groups. Finally we discuss the likelihood that intervention for cardiovascular risk and prevention strategies developed for individuals with IGT are applicable to subjects with IFG.Methods The first part of the study is based on the population-based Danish Inter99 study, where 6265 individuals, aged 30 to 60 years and without previously diagnosed diabetes, underwent an oral glucose tolerance test. The second part is based on the DETECT-2 project, in which studies from China, India, France and USA were used to analyse the impact of the proposed revision of the diagnostic criteria in different ethnic groups.Results The proposed change in diagnostic criteria would increase the prevalence of IFG in Denmark from 11.8 to 37.6%. The proposed IFG category would identify 60.0% of all subjects with IGT compared to 29.2% with the old criteria, but among individuals with the new IFG category only 18.5% would also have IGT. Individuals with isolated IFG had lower insulin levels and a lower cardiovascular risk profile with the proposed criteria compared with the current WHO criteria. Data from the DETECT-2 study confirmed the marked increase in the prevalence of IFG, and the estimated number of individuals in the age range 40 to 64 years with IFG in urban India, urban China and the USA would increase by 78%, 135% and 193% respectively.Conclusions/interpretation The proposed revised diagnostic criteria will lead to a dramatic increase in the prevalence of IFG, but the concordance rate between IFG and IGT remains low. This new IFG group will have a more favourable cardiovascular risk profile than the current IFG group as defined by the WHO. This seriously questions whether the existing intervention strategies are applicable to the new category of individuals with IFG.Abbreviations ADA American Diabetes Association - FPG fasting plasma glucose - NHANES III Third National Health and Nutrition Survey - WHO World Health Organization     

An unusual case of intestinal pseudo-obstruction presenting in an adolescent with juvenile-onset systemic lupus erythematosus: A diagnostic challenge

Introduction

Systemic lupus erythematosus (SLE) is an autoimmune disease with multiple systemic manifestations. The disease itself typically causes only 2–30% of SLE-associated gastrointestinal conditions.