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Biomedical subjects

L Schachner

Publications and source records attributed to L Schachner.

At least 19 recordsLinked to original sources

Kwashiorkor in the United States: fad diets, perceived and true milk allergy, and nutritional ignorance.

BACKGROUND: Kwashiorkor is the edematous form of protein-energy malnutrition. It is associated with extreme poverty in developing countries and with chronic malabsorptive conditions such as cystic fibrosis in developed countries. Rare cases of kwashiorkor in affluent countries unrelated to chronic illness have been reported. We present 12 cases of kwashiorkor unrelated to chronic illness seen over 9 years by pediatric dermatologists throughout the United States, and discuss common causative themes in this easily preventable condition. OBSERVATIONS: Twelve children were diagnosed as having kwashiorkor in 7 tertiary referral centers throughout the United States. The diagnoses were based on the characteristic rash and the overall clinical presentation. The rash consisted of an erosive, crusting, desquamating dermatitis sometimes with classic "pasted-on" scale-the so-called flaky paint sign. Most cases were due to nutritional ignorance, perceived milk intolerance, or food faddism. Half of the cases were the result of a deliberate deviation to a protein-deficient diet because of a perceived intolerance of formula or milk. Financial and social stresses were a factor in only 2 cases, and in both cases social chaos was more of a factor than an absolute lack of financial resources. Misleading dietary histories and the presence of edema masking growth failure obscured the clinical picture in some cases. CONCLUSIONS: Physicians should consider the diagnosis of kwashiorkor in children with perceived milk allergies resulting in frequent dietary manipulations, in children following fad or unorthodox diets, or in children living in homes with significant social chaos. The presence of edema and "flaky paint" dermatitis should prompt a careful dietary investigation.

Diet Fads↗

Ten days of orotracheal intubation with successful extubation in an infant with junctional epidermolysis bullosa.

OBJECTIVE: The most severe form of generalized junctional epidermolysis bullosa, the Herlitz variant, is associated with a number of extracutaneous manifestations. We report on a 45-day-old infant with laryngotracheobronchial mucosa involvement who underwent successful tracheal extubation after 10 days of orotracheal intubation and mechanical ventilatory support. Issues regarding airway management and mechanical ventilatory support in the pediatric intensive care unit are discussed.

Journal Article↗

Neurilemmomatosis, NF2, and juvenile xanthogranuloma.

The gene locus for neurilemmomatosis has been reported to lie within the neurofibromatosis type 2 gene region, suggesting that these 2 diseases may be identical. We describe an adolescent girl with multiple neurilemmomas and juvenile xanthogranulomas, who was found to have bilateral acoustic neuromas and multiple central nervous tissue tumors as seen in patients with a diagnosis of neurofibromatosis type 2. This case and recent genetic studies suggest that neurilemmomatosis and neurofibromatosis type 2 are the same disease.

Adolescent↗

Scabies. How to find and stop the itch.

Scabies is a pruritic skin infestation caused by the itch mite Sarcoptes scabiei. Physical proximity allows the mite to move from one host to another. The diagnosis is made by identifying the mite or its eggs or excrement microscopically from a sample of skin scrapings. Treatment is achieved with topical application of a scabicide and elimination of the mite from the patient's surroundings.

Diagnosis, Differential↗

Acquired progressive lymphangioma.

Acquired progressive lymphangioma is rare and can histologically mimic well-differentiated angioendothelioma. In a 9-year-old boy with acquired progressive lymphangioma, we demonstrated that the neoplasm consists of at least a vascular component and a smooth muscle component. The presence of type IV collagen around many vascular channels suggests that an intermediate stage of differentiation between blood vessels and lymphatics may be present. Our findings indicate that acquired progressive lymphangioma is most likely a complex hamartoma composed of vascular channels and smooth muscle.

Basement Membrane↗

Screening for psychosocial dysfunction in pediatric dermatology practice.

The Pediatric Symptom Checklist, a brief psychosocial screening questionnaire, was used in a multi-center study of pediatric dermatology clinics (n = 377). Overall rates of positive screening indicated that approximately 13% of patients screened positive, a rate similar to findings in primary care pediatric settings. Examining the sample in greater detail demonstrated that children whose dermatologic disorder is perceived to have a greater impact on their appearance are at higher risk for psychosocial dysfunction.

Child↗

Topical erythromycin and zinc therapy for acne.

A double-blind, 12-week study was undertaken to determine the safety and efficacy of a formulation of 4% erythromycin plus 1.2% zinc acetate compared with its vehicle. The study was continued for 40 weeks after the 12-week double-blind phase by switching vehicle-treated patients to active treatment and continuing to give patients treated with active drug the same treatment. Seventy-three female patients started the study; 39 completed 1 full year of study. In the first 12 weeks statistically significant differences were noted in the efficacy of the erythromycin-zinc compared with vehicle for acne severity grades (global assessment) and for papule, pustule, and comedo counts. After crossover, the vehicle-treated group receiving active therapy duplicated the improvement of the group initially treated with erythromycin-zinc. No clinical problems with superinfection or secondary infection occurred during 1 year of treatment in 39 patients.

Acne Vulgaris↗

A clinical trial comparing the safety and efficacy of a topical erythromycin-zinc formulation with a topical clindamycin formulation.

One hundred three patients with acne vulgaris were randomly designated to receive either a topical formulation of erythromycin plus zinc or a topical solution of 1% clindamycin phosphate (Cleocin-T). The patients treated themselves twice daily and were examined at 3, 6, 9, and 12 weeks after the start of therapy. By week 6 the overall severity grade was consistently lower and the percent reduction of severity, papules, pustules, and total comedones was higher in the erythromycin-zinc-treated group than in the clindamycin-treated group. In the 92 patients who completed this study (48 receiving erythromycin-zinc and 44 receiving clindamycin), no serious topical or systemic side effects were reported. Two patients, one from each treatment group, suffered mild irritation. One patient was withdrawn from the erythromycin-zinc-treated group. Results of patch tests were negative. The superiority of the erythromycin-zinc formulation may be due to the increased (4%) erythromycin concentration and/or the ability of 1.2% zinc acetate to enhance the product's activity.

Acne Vulgaris↗

Ichthyosis follicularis in two girls: an autosomal dominant disorder.

Ichthyosis follicularis (IF) is a rare disorder of keratinization that has been described primarily in males and proposed as a possible X-linked disorder. We report two black girls with nonscarring alopecia; photophobia; follicular hyperkeratoses; hyperkeratosis of the extensor aspects of the hands, knees, and elbows; fixed, erythematous, perineal plaques; and angular cheilitis who seem to fit the clinical criteria for IF. One girl also had gingival hypertrophy and a hearing deficit. One child's father had identical symptoms. We propose that these girls may have a variant of IF that is inherited as an autosomal dominant trait.

Alopecia↗

Eosinophilic pustular folliculitis.

Eosinophilic pustular folliculitis was first described by Ofuji et al in 1970. It is characterized by pruritic circinate plaques that are studded with follicular papules and pustules. Lesions are located chiefly on the face, trunk, and arms. Biopsies of lesions demonstrate an infiltrate of eosinophils and neutrophils within hair follicles, dermis, and epidermis. Peripheral leukocytosis and eosinophilia are common.

Adult↗

Assessing child abuse in childhood condyloma acuminatum.

Adults with condyloma acuminatum are considered to have a sexually transmitted disease. Childhood condyloma acuminatum may often, but not always, be a manifestation of sexual abuse. We provide a checklist of criteria for suspecting child abuse. We recommend that if clinicians find a "yes" response on this checklist when caring for a child with condyloma acuminatum, consultation with local child protective services (CPS) be deemed mandatory.

Child↗

Childhood systemic lupus erythematosus. Association with pancreatitis, subcutaneous fat necrosis, and calcinosis cutis.

Pancreatitis developed in a 12-year-old girl with a one-year history of systemic lupus erythematosus. The pancreatitis was first manifested by panniculitis of the lower extremities. Calcinosis cutis subsequently developed, both in areas of panniculitis and in areas free of panniculitis. The patient's medications at the time of onset of pancreatitis included prednisone, hydrochlorothiazide, and azathioprine, all of which have been reported to be causes of pancreatitis. We wish to alert physicians that pancreatitis associated with cutaneous panniculitis and calcinosis cutis may develop in children with systemic lupus erythematosus.

Adipose Tissue↗