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We retrospectively studied the records of 6 Malaysian children who were diagnosed with Alagille Syndrome (AGS) according to this criteria from January 1999 to January 2001, at the Institute of Paediatrics, Kuala Lumpur Hospital. Four patients (66%) had a positive family history. Thirteen individuals (6 patients and 7 relatives) were diagnosed with AGS in these 5 families. Only 6/13 (46%) of them presented with liver involvement. All 6 patients presented with typical facies and cholestasis (100%). Three (50%) presented with portal hypertension (PHT) with synthetic liver dysfunction (1 died), 1/6 (17%) have PHT and normal synthetic liver function. Two have cleared their jaundice but have biochemical evidence of hepatitis and hepatomegaly, four have congenital heart disease 5/6 posterior embryotoxon, 2/6 butterfly vertebrae, 4/6 hyperlipidaemia and 4/6 failure to thrive. One patient has a Jagged-1 gene disruption at the translocation breakpoint locus 20p12.3 2n = 46,XX,t(12.20) (q22, p12.3). 5/6 (83%) are still alive. Two-thirds of our patients developed chronic liver disease by 3 years of age. Two-thirds of the index patients have a family history. Only 46% of individuals in these families have clinical evidence of liver involvement. Mortality depends on cardiac/renal disease, end-stage liver failure and intercurrent infection.
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PMID:Morbidity in Alagille syndrome in 6 Malaysian children. 1519 Jun 47

The aim of the present study was to draw an outline of the clinical epidemiology of bleeding gastrointestinal angiodysplasias. The study includes a report of a case and of our series of patients with bleeding gastrointestinal angiodysplasias admitted, between 1993 and 2003, to a ward of Internal Medicine where digestive endoscopy is also performed. A review of the literature is also provided. An 80-year-old cirrhotic woman with aortic stenosis, was referred to our Department because of anemia due to melena. In spite of 13 hospitalizations during which numerous diagnostic procedures including endoscopy, X-ray studies, arteriography, labeled red blood cells scanning and laparotomy with intraoperative ileoscopy, the site and nature of the bleeding lesion remained unidentified. Her red blood cell requirement progressively increased from 6 U in 1993 to 24 U in 1994 to 40 U as of September 1995. Enteroscopy disclosed duodeno-jejunal angiodysplasia. The patient subsequently received 35 additional red blood cell units during 7 new admissions. Between 1993 and 2003, 24 patients were identified. They were mainly women and their average age was 77 years. Angiodysplasias were localized in the large bowel in 92% of cases. Comorbidities included: heart disease (79%), chronic liver disease (29%) and chronic renal failure (21%). One fourth of patients were under anticoagulant drugs or had a hemostatic blood disorder. All patients received blood transfusions and endoscopic treatment was performed in approximately half of the cases. The most relevant updates are related to the pathogenic relationship between aortic stenosis, von Willebrand's disease and bleeding gastrointestinal angiodysplasias, the hemostatic alterations associated with liver cirrhosis or with chronic renal failure and the diagnosis and treatment of bleeding gastrointestinal angiodysplasias. A better understanding of the clinical epidemiology of bleeding gastrointestinal angiodysplasias may facilitate their diagnosis and contribute to an effective clinical management.
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PMID:[Bleeding gastrointestinal angiodysplasias: our experience and a review of the literature]. 1531 67

Hypertrophic osteoarthropathy is a syndrome characterized by clubbing of the digits of the hand/foot, periosteal reaction and arthralgia or arthritis which is usually secondary to cyanotic congenital heart disease and chronic pulmonary infections. This syndrome rarely occurs in association with chronic liver disease in childhood. Here, we report on a child with biliary atresia who developed arthralgia and arthritis during follow-up and which was diagnosed as hepatic hypertrophic osteoarthropathy. It is emphasized that hypertrophic osteoarthropathy should be considered in the differential diagnosis of arthralgia and arthritis in children with long-standing chronic liver diseases, especially if finger clubbing is also present.
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PMID:Hypertrophic osteoarthropathy in a child with biliary atresia. 1537 Jun 95

The Alagille syndrome is one of the most common inherited disorders causing chronic liver disease during childhood. During the 1990s, 38 children with Alagille syndrome were evaluated at two pediatric centers in Buenos Aires, Argentina. Characteristic clinical, humoral, and cutaneous features were analyzed. The average age of diagnosis was 29 months old (range of between 2 months and 15 years). Cholestasis was evident in 92% of patients during the neonatal period. Family antecedents related to the syndrome were found in 18.5% of the patients. Peculiar facies developed in 85% of patients. Chronic cholestasis and pruritus were observed in all of the patients and jaundice was evident in 78%. Eighty-four percent of the patients had heart disease (pulmonary stenosis, intraauricular communication, intraventricular communication), 76% of them showed growth retardation, and vertebrae abnormalities were found in 63%. Embryotoxon appeared in 76% of patients, and renal disturbances in 21%. Eleven children (28%) had xanthomas, in the neck, elbows, palms, helixes, inguinal area, gluteus, and knees. The earliest findings appeared in the first months of life, and the latest at 5 years of age. The xanthomas located in the folds had a stony aspect. Cholesterol levels ranging from 220 to 1600 mg percentage (mg%) were demonstrated in all of the children with xanthomas. Liver transplantation was performed in seven of the patients (18.4%). Two of them died after this operation. The disappearance of xanthomas after transplantation was remarkable in all of the patients.
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PMID:Alagille syndrome: cutaneous manifestations in 38 children. 1566 Aug 89

Patients with liver cirrhosis are characterized by decreased synthesis of both pro- and anticoagulant factors, and recently there has been evidence of normal generation of thrombin resulting in a near normal haemostatic balance. Although it is generally recognized that bleeding is the most common clinical manifestation as a result of decreased platelet function and number, diminished clotting factors and excessive fibrinolysis, hypercoagulability may play an under recognized but important role in many aspects of chronic liver disease. In fact, they can encounter thrombotic complications such as portal vein thrombosis, occlusion of small intrahepatic vein branches and deep vein thrombosis (DVT). In particular, patients with cirrhosis appear to have a higher incidence of unprovoked DVT and pulmonary embolism (PE) compared with the general population. In dedicated studies, the incidence of DVT/PE ranges from 0.5% to 1.9%, similar to patients without comorbidities, but lower than patients with other chronic diseases (i.e, renal or heart disease). Surprisingly, standard coagulation laboratory parameters are not associated with a risk of developing DVT/PE; however, with multivariate analysis, serum albumin level was independently associated with the occurrence of thrombosis. Moreover, patients with chronic liver disease share the same risk factors as the general population for DVT/PE, and specifically, liver resection can unbalance the haemostatic equilibrium towards a hypercoagulable state. Current guidelines on antithrombotic prophylaxis do not specifically comment on the cirrhotic population as a result of the perceived risk of bleeding complications but the cirrhotic patient should not be considered as an auto-anticoagulated patient. Therefore, thromboprophylaxis should be recommended in patients with liver cirrhosis at least when exposed to high-risk conditions for thrombotic complications. Low molecular weight heparins (LWMHs) seem to be relatively safe in this group of patients; however, when important risk factors for bleeding are present, graduated compression stockings or intermittent pneumatic compression should be considered.
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PMID:Should we give thromboprophylaxis to patients with liver cirrhosis and coagulopathy? 1981 8

Lifestyle diseases characterize those diseases whose occurrence is primarily based on the daily habits of people and are a result of an inappropriate relationship of people with their environment. The main factors contributing to lifestyle diseases include bad food habits, physical inactivity, wrong body posture, and disturbed biological clock. A report, jointly prepared by the World Health Organization (WHO) and the World Economic Forum, says India will incur an accumulated loss of $236.6 billion by 2015 on account of unhealthy lifestyles and faulty diet. According to the report, 60% of all deaths worldwide in 2005 (35 million) resulted from noncommunicable diseases and accounted for 44% of premature deaths. What's worse, around 80% of these deaths will occur in low and middle-income countries like India which are also crippled by an ever increasing burden of infectious diseases, poor maternal and perinatal conditions and nutritional deficiencies. According to a survey conducted by the Associated Chamber of Commerce and Industry (ASSOC-HAM), 68% of working women in the age bracket of 21-52 years were found to be afflicted with lifestyle ailments such as obesity, depression, chronic backache, diabetes and hypertension. The study 'Preventive Healthcare and Corporate Female Workforce' also said that long hours and working under strict deadlines cause up to 75% of working women to suffer from depression or general anxiety disorder, compared to women with lesser levels of psychological demand at work. The study cited scientific evidence that healthy diet and adequate physical activity - at least 30 minutes of moderate activity at least five days a week - helped prevent NCDs. In India, 10% of adults suffer from hypertension while the country is home to 25-30 million diabetics. Three out of every 1,000 people suffer a stroke. The number of deaths due to heart attack is projected to increase from 1.2 million to 2 million in 2010. The diet [or lifestyle] of different populations might partly determine their rates of cancer, and the basis for this hypothesis was strengthened by results of studies showing that people who migrate from one country to another generally acquire the cancer rates of the new host country, suggesting that environmental [or lifestyle factors] rather than genetic factors are the key determinants of the international variation in cancer rates. Some of the common diseases encountered because of occupational lifestyle are Alzheimer's disease, arteriosclerosis, cancer, chronic liver disease/cirrhosis, chronic obstructive pulmonary disease (COPD), diabetes, hypertension, heart disease, nephritis/CRF, and stroke. Occupational lifestyle diseases include those caused by the factors present in the vicinity like heat, sound, dust, fumes, smoke, cold, and other pollutants. These factors are responsible for allergy, respiratory and hearing problems, and heat or cold shock. So, A healthy lifestyle must be adopted to combat these diseases with a proper balanced diet, physical activity and by giving due respect to biological clock. Kids spending too much time slouched in front of the TV or PCs, should be encourage to find a physical sport or activity they enjoy. Fun exercises should be encouraged into family outings. A pizza-and-video evening should be replaced for a hike and picnic. Kids who do participate in sport, especially at a high competitive level, can find the pressure to succeed very stressful. To decrease the ailments caused by occupational postures, one should avoid long sitting hours and should take frequent breaks for stretching or for other works involving physical movements.
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PMID:Occupational lifestyle diseases: An emerging issue. 2044 27

Although the health status of Americans has improved, particularly decreases in incidence and prevalence rates of diseases, these improvements are not consistent and equally distributed within all population groups - particularly black Americans. To describe the relative differences, an analytical procedure of calculating the differential deficit ratio (DDR) was developed. It was observed that for males the death rate from all causes increased in 1950 from a .426 to a .473 DDR or 47.3 percent in 1982 with black males dying at a much higher rate than white males. For females there was a closing of the gap. In 1950 the rate was .703, higher in black females than white females, but in 1982 the ratio was .431. These trends were also observed for deaths due to heart disease, cerebrovascular, malignant neoplasms, diabetes mellitus, pneumonia and influenza and chronic liver disease and cirrhosis. An epidemic in homicide exists among black males. The rate among black Americans is more than 5 times higher than among white. Infant mortality DDR in 1950 was .638 and in 1982 it was .949, the highest in more than 32 years. Also the infant mortality rate among blacks was higher than in any other ethnic or racial group. Indices show that there is a direct correlation between level of poverty, income level, education and infant mortality. Future directions indicate a need for overall change in the health care system, re-education for the consuming public, rebasing health care in the community and correlating health care with other social problems which impact on health.
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PMID:Socio-economic factors influencing the health status of black americans. 2084 Dec 74

Endomyocardial fibrosis (EMF), an idiopathic cardiac disorder, occurs predominantly in the tropical and subtropical regions of the world. We present herein a report of EMF in a young man from the savannah belt of Nigeria. He was a 19-year-old young man who presented with recent onset of palpitation and dyspnea and was followed up elsewhere for 3 years as a case of chronic liver disease due to progressive abdominal distension. Clinical examination revealed an undernourished young man with atrial fibrillation and features of heart failure. His electrocardiogram showed features of atrial fibrillation, while echocardiogram showed enlarged right atrium, tricuspid incompetence, and signs of fibrosis in the ventricles. Although EMF occurs primarily in the subtropical regions of Africa in its typical manifestation, in this report it was in the savannah belt.
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PMID:Endomyocardial fibrosis presenting outside the tropical rain forest. 2128 10

Pulmonary arterial hypertension (PAH) is a disease that leads to characteristic vascular wall remodeling and hemodynamic alterations. Consequently, this pulmonary vascular disease contributes to substantial morbidity and mortality in afflicted patients. PAH may be idiopathic in nature or associated with connective tissue disease, chronic liver disease, human immunodeficiency virus, congenital heart disease, and a growing list of other conditions. There are currently nine Food and Drug Administration-approved therapies for specific PAH treatment. Therapeutic targets include prostacyclin replacement, endothelin-1 antagonism, and phosphodiesterase-5 inhibition. This article focuses on the prostanoid treprostinil and explores its role in the management of patients with PAH.
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PMID:Clinical utility of treprostinil and its overall place in the treatment of pulmonary arterial hypertension. 2287 90

Growth failure is a common problem in many children with chronic diseases. This article is an overview of the most common causes of growth failure/growth retardation that affect children with a number of chronic diseases. We also briefly review the nutrition considerations and treatment goals. Growth failure is multifactorial in children with chronic conditions, including patients with cystic fibrosis, chronic kidney disease, chronic liver disease, congenital heart disease, human immunodeficiency virus, inflammatory bowel disease, short bowel syndrome, and muscular dystrophies. Important contributory factors to growth failure include increased energy needs, increased energy loss, malabsorption, decreased energy intake, anorexia, pain, vomiting, intestinal obstruction, and inflammatory cytokines. Various metabolic and pathologic abnormalities that are characteristic of chronic diseases further lead to significant malnutrition and growth failure. In addition to treating disease-specific abnormalities, treatment should address the energy and protein deficits, including vitamin and mineral supplements to correct deficiencies, correct metabolic and endocrinologic abnormalities, and include long-term monitoring of weight and growth. Individualized, age-appropriate nutrition intervention will minimize the malnutrition and growth failure seen in children with chronic diseases.
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PMID:Growth failure and nutrition considerations in chronic childhood wasting diseases. 2537 56


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