Pulmonary haemosiderosis in a 13-year-old girl with coeliac disease after 3 months on a gluten-free diet: case report and review of the literature.

A 13-year-old girl with controlled coeliac disease who had been on a gluten-free diet for the past 3 months was admitted with respiratory distress and hypoxia for the past week. Chest radiograph and CT scan showed bilateral widespread alveolar shadowing suggestive of a hypersensitive pneumonitis. There was a dramatic radiological and clinical response to oral corticosteroids. Bronchial lavage was suggestive of recurrent aspiration and histology of a transbronchial biopsy showed pulmonary haemosiderosis. We conclude that pulmonary haemosiderosis may occur in children with coeliac disease who are on a gluten-free diet.

Pulmonary alveolar microlithiasis in an 8-month-old infant.

Pulmonary alveolar microlithiasis (PAM) in an 8-month-old male infant is reported. He presented initially at 2 months of age with worsening respiratory distress and cyanosis. Chest imaging by X-ray and by CT scan showed widespread reticulo-nodular densities mostly in the mid and lower zones. The diagnosis of PAM was confirmed by an open lung biopsy which showed diffuse alveolar calcium deposits.

Acute rheumatic fever in Jordanian children.

Rheumatic fever remains a significant health problem in Jordan. We retrospectively reviewed medical charts of 28 boys and 22 girls (mean age at presentation 10.5 +/- 2.6 years) with confirmed diagnosis based on modified Jones criteria at Queen Alia Heart Institute from February 1999 to February 2002. Arthritis was the commonest major manifestation (88%; 68% migratory), carditis was second commonest (48%; 8% silent carditis) and chorea was seen in 6%. None had subcutaneous nodules or erythema marginatum. The mitral valve was most commonly affected (80%); both mitral and aortic valves were affected in 25%. Pericarditis was seen in 12.5% and acute congestive heart failure in 4%. Practitioners should be aware of diverse clinical presentations and emphasize strict adherence to prophylaxis guidelines.

Outcome of asthma in children and adolescents at a specialty-based care program.

BACKGROUND: Asthma remains a major cause of morbidity for children despite national guidelines. OBJECTIVE: To evaluate outcome from a structured specialty-based care program. METHODS: Comparison of previous and subsequent years for children ranging from infancy to adolescence who entered a specialty clinic program at a university hospital serving a widely dispersed patient population. One hundred fifty-seven patients previously receiving primary care for their respiratory symptoms were seen during the study period; 23 were lost to followup, 15 were excluded because of other serious concurrent medical problems, and 119 were available for outcome analysis. Evaluation included historical data base from a structured interview, evaluation of pulmonary physiology, and allergy skin testing. Treatment decisions were evidence-based. Patient and/or family education was targeted at decision-making. Toll-free telephone access to the specialty service was provided around the clock. Frequency of unscheduled medical care, hospitalizations, sleep disturbance, activity interference, attainment of defined criteria for control, and medication use were quantified. RESULTS: Seven hundred thirty-five acute care visits were reduced to 47, and 99 hospitalizations were decreased to 10 (P < 0.001 for both). Nocturnal symptoms and exercise limitation decreased significantly (P < 0.001 for both). All criteria for control of asthma were met in 89% of 75 without tobacco smoke exposure and 50% of 44 with exposure (P < 0.0001 for the difference in outcome). Frequent antibiotic use for respiratory symptoms were eliminated after entering the program. Maintenance medications were not used in 72 with an intermittent pattern of viral respiratory infection-induced asthma. Inhaled corticosteroid use increased from 38 to 68% among 47 subjects with a chronic pattern. CONCLUSIONS: Morbidity from asthma is largely prevented with often less, but better selected, medication than had been occurring in previous primary care. These data have implications for revised guidelines directed at primary care physicians.

Wolfram's (DIDMOAD) Syndrome and Chronic Renal Failure.

Wolfram's syndrome is usually considered as an autosomal recessive condition, with wide phenotypic variation. The syndrome is commonly called DIDMOAD (diabetes insipidus, diabetes mellitus, optic atrophy and deafness), although some patients have additional clinical findings including ataxia, hypogonadism, hydronephrosis and psychiatric illnesses. We report a patient with DIDMOAD syndrome with emphasis on the urological tract and its progressive complications. Unfortunately, he developed end-stage renal failure and needed hemodialysis at the age of 14 years. The presentation, investigations and management are discussed.

Late-onset nephrotic syndrome in galloway-mowat syndrome: a case report.

Galloway-Mowat Syndrome (GMS) has a wide variety of clinical manifestations and histologic findings. All reported cases had developed nephrotic syndrome in the first two years of life. We report a case of 12 years old boy with microcephaly, mental retardation, and typical dysmorphic features of GMS with a late onset of minimal change nephritic syndrome which first manifested at seven years of age.

Causes of chronic renal failure in children in a single hospital in jordan: a 10 years retrospective study.

Case records of patients aged 13 years or below with chronic renal failure (CRF), seen during the last 10 years at the King Hussein Medical Center, Amman, Jordan were reviewed. There were 46 patients with CRF, (25 males and 21 females), aged 1/2 to 13 years. Chronic pyelonephritis was the major cause of CRF seen in 23 cases (50%), followed by glomerulonephritis in eight (17.4%), congenital hypodysplasia in six (13%), hereditary nephritis in five (10.9%) and unknown etiology in four (8.7%). Of the study patients, 17.3% were below the age of five years at first presentation. Twenty one patients progressed to end-stage renal disease and among them chronic glomerulonephritis was the most common primary renal disease. The higher incidence of chronic pyelonephritis causing CRF in our series in comparison to 23.9% incidence reported in the developed countries shows that our infants and young children probably are not investigated early enough nor do they receive adequate treatment at the time of their first urinary tract infection. Prevention of renal failure, availability of replacement therapy, and extension of treatment opportunities to more children are the main problems concerning CRF in children in our country.