Classical Features (classical + feature)

Distribution by Scientific Domains

Selected Abstracts

Hyperphagia, weight gain and neonatal drug withdrawal

R Shephard
Hyperphagia, a classical feature of neonatal drug withdrawal, has been reported not to lead to excessive weight gain, but this is contrary to our clinical experience. The aim of this study was to determine whether infants with neonatal drug withdrawal suffered excessive weight gain because of hyperphagia and, if so, to determine the risk factors. The study population comprised 48 infants consecutively admitted to the neonatal intensive care unit, 11 of whom gained weight by more than 20 g kg -1 d -1 for at least 10 d (excessive weight gain). All 11 infants were hyperphagic (>200 ml/kg) for at least part of the excessive weight gain period. During the perinatal period, the 11 infants had a greater fluid intake (p > 0.01) but similar weight gain to gestational-age-matched, neonatal drug-withdrawal infants who did not suffer any excessive weight gain. Compared to the rest of the cohort, the infants with excessive weight gain were more likely to require treatment with morphine/chlorpromzaine (p > 0.05) and had a higher maximum withdrawal score (p > 0.01). Conclusion: Hyperphagia can lead to excessive weight gain in infants with neonatal drug withdrawal. Our results suggest that hyperphagia occurs in those who require treatment for severe withdrawal. [source]

Monsoon in the Americas: Opportunities and Challenges

Thomas M. Rickenbach
This article presents a comparative review of the North and South America Monsoon Systems and highlights the challenges and opportunities presented to those regions by the seasonal rains. Monsoon precipitation represents a major component of the water resources available to the southwestern US and to Brazil. Although each system shares classical features of the well-known southwest Indian monsoon, water use, agriculture, public safety, and energy policy in these two countries have been shaped by the unique regional complexities of monsoon rain across each region. A comparison between these two systems may offer perspective for ways by which these societies may adapt to current and future challenges, and take advantage of new opportunities. [source]

A Japanese case of Kindler syndrome

Yasushi Suga MD
A 25-year-old Japanese woman presented with contracture of the fingers and toes, and difficulty in opening her mouth. Her grandparents are first cousins, but none of the other members of the family are affected. Bulla formation started at birth on areas of the skin that received pressure, and in infancy and early childhood the lesions were limited only to the acral areas. She also had bilateral, incomplete syndactylies involving all web spaces ( Fig. 1a). The formation of blisters ceased after the age of 15 years, but a generalized progressive poikiloderma then appeared with accompanying cutaneous atrophy of the skin of the neck, trunk, and extremities ( Fig. 1b). The patient experienced mild photosensitivity of the face and neck. At age 18 years, surgical removal of the webbing of all her fingers was performed. Oral examination showed atrophy of the buccal mucosa, and an inability to fully open the mouth. The patient also suffered from poor dentition and easily bleeding gums, but had no symptoms of esophageal dysfunction. Figure 1. Clinical manifestations of the patient with Kindler syndrome. (a) Dorsal surface of the patient's hands. Note the marked cutaneous atrophy with a severely wrinkled appearance on the dorsal surface of the hands, as well as the proximal fusion of the fingers. (b) Lower left leg of the patient. Atrophic thinning of the skin and poikiloderma with reticular pigmentation are evident Histology of separate biopsy specimens, taken from the poikilodermatous pretibial and trunk skin, showed classical features of poikiloderma, namely epidermal atrophy with flattening of the rete ridges, vacuolization of basal keratinocytes, pigmentary incontinence, and mild dermal perivascularization ( Fig. 2a). Interestingly, dyskeratotic cells ( Fig. 2b) and eosinophilic rounded bodies (colloid bodies) ( Fig. 2c) were frequently found at the basal keratinocyte layer and in the upper dermis, respectively. Pigment was also present in the upper epidermis. Figure 2. Hematoxylin and eosin staining of a biopsy specimen taken from pretibial skin. (a) Epidermal atrophy with flattening of the rete ridges. Note the dyskeratotic cells (arrowheads) and vacuolar degeneration of the basal layer in the epidermis. Bar = 50 ,m. (b) Higher magnification of dyskeratotic cells (arrowheads). Bar = 10 ,m. (c) Higher magnification of colloid bodies (arrowheads) in the superficial dermis. Bar = 10 ,m To rule out the possibility of a congenital epidermolysis bullosa, ultrastructural and immunofluorescence studies were performed. Ultrastructural studies demonstrated the reduplication of the basal lamina with branching structures within the upper dermis and cleavage between the lamina densa and the cell membrane of the keratinocytes ( Fig. 3a). The numbers of associated anchoring fibrils did not seem to be reduced, and colloid bodies and dyskeratotic cells were detected. Immunofluorescence studies with the antibody against type VII collagen (LH 7 : 2) were subsequently carried out. The results showed extensive broad bands with intermittently discontinuous and reticular staining at the dermo-epidermal junction (DEJ) ( Fig. 3b), whereas a linear distribution is typically seen in healthy tissue (data not shown). Interestingly, direct immunofluorescence studies revealed intracellular accumulation of immunoglobulin G (IgG), IgM, IgA, and C3 in colloid bodies under the basement membrane ( Fig. 3c). Figure 3. Ultrastructural and immunohistochemical findings of the patient with Kindler syndrome. (a) Ultrastructural study of the dermo-epidermal junction. The branching structures of the lamina densa (arrowheads) were frequently seen. The asterisks show the cleavage in the lamina lucida. Bar = 1 ,m. (b) Immunohistochemical studies with the antibody to type VII collagen (LH 7 : 2). An extensive broad band with reticular patterns is evident. Bar = 50 ,m. E, epidermis; D, dermis. (c) Direct immunofluorescence study. Intracytoplasmic deposition of IgM in the basal keratinocytes is evident (arrowheads). Bar = 50 ,m. E, epidermis; D, dermis [source]

Wilson's disease with superimposed autoimmune features: Report of two cases and review

Piotr Milkiewicz
Abstract We describe two females, 15 and 23 years old, respectively, who presented with classical features of Wilson's disease (WD) and several features of autoimmune hepatitis (AIH). The first patient was initially diagnosed as AIH and treated with prednisolone which caused clinical improvement, with an increase of serum albumin from 22 to 30 g/L, and a decrease of aspartate aminotransferase from 103 to 47 U/L. Subsequent diagnosis of WD and introduction of penicillamine gave excellent improvement and complete normalization of liver function tests. The second patient, at first also diagnosed as having AIH, was treated with steroids and azathioprine with initial improvement, but subsequent deterioration. The diagnosis of WD was made 2 years after initial diagnosis of AIH, as the patient reached end-stage liver disease and required a transplant. Therefore, d -penicillamine treatment was not attempted. We conclude that, in patients with AIH, a thorough screening for WD is necessary, particularly when the response to steroid therapy is poor. Conversely, in patients suffering from WD with superimposed features of AIH, a combination of steroids and penicillamine may be of benefit. [source]

Cranial magnetic resonance imaging of Wolfram (DIDMOAD) syndrome

E Pakdemirli
Summary Wolfram syndrome is a rare neurodegenerative disorder characterized by diabetes insipidus, diabetes mellitus, optic atrophy and deafness (DIDMOAD). A wide spectrum of abnormalities of the central nervous system, urinary tract and endocrine glands is also observed. We report cranial MRI findings in a 32-year-old female patient with Wolfram syndrome. In addition to the classical features, including absence of the normal high signal of the neurohypophysis, atrophy of visual pathways, the brainstem, cerebellum and cerebral cortex, we observed bilateral hyperintensity on proton density- and T2- weighted images related to the optic radiations in the periventricular white matter of the temporal and parieto-occipital lobes, which may reflect gliosis pathologically. [source]

Nutritional status of children with coeliac disease

B Aurangzeb
Abstract Aims:, The main aim of this study was to assess the nutritional status of children with newly diagnosed Coeliac disease (CD)with comparison to matched controls. A further aim was to assess relationships between presentation patterns and nutrition in childhood CD. Methods:, The nutritional status of newly diagnosed CD was assessed by anthropometry, Bioelectrical Impedance and serum leptin levels, and contrasted to age and gender matched controls. Results:, Twenty-five children with CD (mean age of 8.2 4.5 years) and 25 control children (mean age 8.1 4.4.) were enrolled. Thirteen (52%) children with CD had gastrointestinal symptoms with 14 having a family history of CD. At presentation 8.7% were wasted, 4.2% were stunted and 20.8% overweight, although none were obese. Mean height and weight for age, other nutritional parameters and serum leptin did not differ between the groups. Serum leptin correlated with BMI in both groups. Conclusions:, Children with CD more commonly present with atypical symptoms than with classical features. Variations in nutrition (under to overnutrition) may be seen at diagnosis, without relationship to the presence of symptoms. Leptin levels were not altered specifically in the setting of CD. Nutritional assessment remains important in the assessment and management of CD in children. [source]