ABSTRACT
Lane-Hamilton syndrome is a rare condition that is characterized by idiopathic pulmonary hemosiderosis and celiac disease. We report the case of an 18-month-old girl with Lane-Hamilton syndrome who had unilateral pigmentary retinopathy.
Subject(s)
Celiac Disease/diagnosis , Hemosiderosis/diagnosis , Lung Diseases/diagnosis , Retinitis Pigmentosa/diagnosis , Celiac Disease/diet therapy , Diet, Gluten-Free , Electroretinography , Female , Fluorescein Angiography , Hemosiderosis/diet therapy , Humans , Infant , Lung Diseases/diet therapy , Retinitis Pigmentosa/diet therapy , Tomography, X-Ray Computed , Hemosiderosis, PulmonaryABSTRACT
Idiopathic pulmonary hemosiderosis (IPH) is a rare disease characterized by anemia, hemoptysis and recurrent alveolar hemorrhage. The combination of IPH and celiac disease (CD) is extremely rare. We report a 9-year-old boy with Lane-Hamilton syndrome, co-occurrence of pulmonary hemosiderosis with CD. This presentation is unique presentation because he has also retinal pigmentation.
Subject(s)
Celiac Disease/diagnosis , Hemosiderosis/diagnosis , Lung Diseases/diagnosis , Retinitis Pigmentosa/diagnosis , Celiac Disease/diet therapy , Child , Dietary Supplements , Gliadin/adverse effects , Hemosiderosis/diet therapy , Humans , Iron/therapeutic use , Lung Diseases/diet therapy , Male , Retinitis Pigmentosa/diet therapy , Hemosiderosis, PulmonaryABSTRACT
Lane-Hamilton syndrome refers to the uncommon co-occurrence of idiopathic pulmonary hemosiderosis and celiac disease (CD). Three children aged between 7 and 14 years with IPH were detected to have co-existing non-diarrheal CD. Institution of gluten-free diet in each of the three children resulted in amelioration of the pulmonary symptoms along with improvement of anthropometric parameters and hemoglobin over a short-term follow-up period of 8-17 months. Inhaled/oral steroids and immunosuppressants could be weaned off after dietary exclusion therapy in each of the three children. Gluten free diet should be instituted in all patients diagnosed with Lane-Hamilton syndrome. It ameliorates both the pulmonary as well as the intestinal symptoms although the precise mechanism of the pulmonary response is as yet unclear.
Subject(s)
Celiac Disease/complications , Celiac Disease/diet therapy , Diet, Gluten-Free , Hemosiderosis/complications , Hemosiderosis/diet therapy , Lung Diseases/complications , Lung Diseases/diet therapy , Adolescent , Child , Female , Humans , Male , Hemosiderosis, PulmonarySubject(s)
Anemia, Iron-Deficiency/etiology , Celiac Disease/complications , Hemosiderosis/complications , Lung Diseases/complications , Adult , Bronchoalveolar Lavage Fluid/cytology , Celiac Disease/diagnosis , Celiac Disease/diet therapy , Diagnosis, Differential , Dyspnea/etiology , Glutens/adverse effects , Hemoptysis/etiology , Hemorrhage/diagnosis , Hemosiderin/analysis , Hemosiderosis/diet therapy , Humans , Macrophages/chemistry , Macrophages/pathology , Male , SyndromeABSTRACT
Iron storage disease (ISD) in lemurs has been reported since as early as the 1960s, and in the 1980s was demonstrated to be a consistent finding in postmortem investigations of captive lemurs. Since then this disease has consistently been diagnosed at the point of necropsy. In the current study we describe a preclinical screening procedure, as well as the quantified preventive effects of dietary intervention upon iron absorption. Twenty-three individual lemurs of four species were initially tested with the transferrin saturation test (%TS); 21 of these animals were on conventional zoo diets, and two were fed a specific diabetic diet. Initially, 20 of 21 lemurs on conventional zoo diets were demonstrated to have %TS levels above the normal range for humans; 17 of these lemurs were in the category (for humans) of excessive iron absorption. A dietary change aimed at reducing dietary iron and vitamin C levels and increasing the levels of iron-chelating tannins and/or phytates was instigated. After the animals were retested, a matched-pair comparison of %TS values before and after the diet change revealed significantly (P=0.038, n=7) lower %TS values after the diet change. All species averages were in the human hyperabsorption range on conventional zoo diets (n=21). No species averages were in that range after the dietary change (n=18). The results indicate that further investigations into the use of %TS testing in lemur husbandry, and specific preventive dietary measures, should be conducted.
Subject(s)
Diet, Diabetic/veterinary , Hemosiderosis/diet therapy , Hemosiderosis/veterinary , Iron, Dietary/metabolism , Lemuridae/metabolism , Animals , Hemosiderosis/prevention & control , Iron Chelating Agents/therapeutic use , Phytic Acid/therapeutic use , Transferrin/metabolismSubject(s)
Humans , Child , Male , Hemosiderosis , Lung Diseases , Hemosiderosis/diet therapy , Hemosiderosis/drug therapy , Tomography, Emission-ComputedABSTRACT
Pulmonary hemosiderosis (PH), a rare pathology, usually occurring during infancy and childhood, is characterized by numerous and repeated intra-alveolar bleedings, interstitial iron build-up with consequent progressive fibrosis and severe anemia. PH can be basically divided into 3 different categories: Primitive or idiopathic, involving a primitive deficit of antioxidizing enzymes in the erythrocytes in genetically predisposed subjects; Secondary, subsequent to chronic pneumopathy or cardiopathy; Associated with various pathologies including collagenopathies, glomerulonephritis, myocardiopathies, diabetes, steatorrhea, tireotossicosis. A particular type of Pulmonary Hemosiderosis, associated with allergy to cow's milk, has been described for the first time by Heiner. We present in this paper our personal experience of a young patient suffering from pulmonary hemosiderosis induced by cow's milk protein.
Subject(s)
Hemosiderosis/etiology , Lung Diseases/etiology , Milk Hypersensitivity/complications , Milk Proteins/adverse effects , Child , Female , Hemosiderosis/diagnosis , Hemosiderosis/diet therapy , Humans , Lung Diseases/diagnosis , Lung Diseases/diet therapy , Milk Hypersensitivity/diagnosis , Milk Hypersensitivity/diet therapy , SyndromeABSTRACT
A child with idiopathic pulmonary haemosiderosis for three years required three monthly transfusions. Circulating avian, gliadin, and reticulin antibodies suggested the diagnosis of gluten enteropathy, and jejunal biopsy showed subtotal villous atrophy. During 15 months on a gluten free diet his growth and behaviour improved and he required no transfusions.
Subject(s)
Celiac Disease/complications , Hemosiderosis/etiology , Lung Diseases/etiology , Child, Preschool , Hemosiderosis/diet therapy , Humans , Lung Diseases/diet therapy , MaleABSTRACT
During the 3 decades 1950-1979 onset of idiopathic pulmonary haemosiderosis occurred in 10 Swedish children. Complete records were available from the eight children with onset during 1960-1979, which indicates that the yearly risk of onset is 0.24 case per million children. The first symptoms started at the mean age of 5.8 years (range: 10 months-11 years). From the beginning all children had a severe microcytic, hypochromic, sideropenic anaemia. Pulmonary symptoms were present from the beginning in four children (but haemoptysis in only one) and developed in the remaining children after 1 1/2-2 1/2 years. Various therapeutical regimens were tried. Iron therapy seemed of temporary beneficial effect and most children seemed to benefit from prednisone therapy during disease bouts, although the effect of the therapy in the long run could not be determined. The four children with onsets during the 1960s died of their disease after 2-13 years. The four children with onset during the 1970s are still alive. One of them--a 20-year-old female, has for two years complete clinical remission and has normalized haematological, X-ray and pulmonary function data.
