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Biomedical subjects

I J Frieden

Publications and source records attributed to I J Frieden.

At least 37 records · Page 2Linked to original sources
PubMed1999

Review of modern techniques in detecting port-wine stain response to laser therapy.

BACKGROUND: Traditional mechanisms of assessing port-wine stain response to laser therapy have rested mainly on subjective determinations by physician and patient. However, the wide variation in treatment response poses a profound need for objective devices to measure treatment outcomes so that maximum effectiveness of therapy can be achieved without unnecessary repeat treatments. OBJECTIVE: The purpose of this paper will be to review noninvasive techniques to measure port-wine stain response to laser therapy. METHODS: This report is based on a review of medical and bioscience literature. RESULTS: Several techniques including laser Doppler, reflectance spectrophotometry, tristimulus colorimetry, and videomicroscopy have been developed to address the need for objective measurement devices. CONCLUSION: While many instruments are available, these techniques are limited by cost, small test size area, and/or inconclusive correlation with clinical response. A number of experimental techniques may circumvent many of the problems inherent in currently-available commercial technologies.

Diagnostic Imaging↗
PubMed1998

Growing up with a facial hemangioma: parent and child coping and adaptation.

OBJECTIVE: To better understand the experiences, challenges, and adaptations of parents with children who have disfiguring facial hemangiomas. DESIGN: Qualitative, descriptive. METHODS: In-depth interviews were performed with the parent(s) of 25 children, aged 5 months to 8 years. Each child was referred to the Pediatric Dermatology Practice, Department of Dermatology, University of California, San Francisco, with a facial hemangioma of 1 cm diameter or greater. Interviews were ethnographic in style, centering on the description by parents of the particular challenges faced, supports received, and adaptive strategies developed in coping with their child's hemangioma. Analysis was by open coding of interview transcripts. Coded statements were organized within common categories and these further gathered into 4 principal themes: 1) parental emotion and adaptation; 2) experiences with public reactions; 3) issues related to parent-child interactions; and 4) expressed satisfaction/dissatisfaction with medical care. RESULTS: Disfiguring facial hemangiomas were found to be associated with parental reactions of disbelief, fear, and mourning, particularly during the growth phase. Reactions of strangers forced parents to confront varied aspects of social stigmatization. A broad array of effects on the parent-child interaction were observed, often connected with variables extrinsic to the hemangioma, including especially the support and acceptance by the extended family. Half of those studied expressed substantial dissatisfaction with aspects of their medical care. CONCLUSIONS: Disfiguring facial hemangiomas in young children are frequently associated with parental reactions of loss and grief, despite the generally benign nature of the lesion and the prognosis for eventual involution. Physicians are faced with specific challenges in providing effective anticipatory guidance and support to parents.

Adaptation, Psychological↗
PubMed1997

Kaposiform hemangioendothelioma. An aggressive, locally invasive vascular tumor that can mimic hemangioma of infancy.

BACKGROUND: Kaposiform hemangioendothelioma is a rare, aggressive vascular proliferation in children that is clinically and histologically distinct from hemangioma of infancy. It often manifests later than infantile hemangioma, and complication by Kasabach-Merritt syndrome is common. OBSERVATIONS: We examined 3 children with kaposiform hemangioendothelioma, all of whom were initially misdiagnosed as having infantile hemangioma. All 3 children developed Kasabach-Merritt syndrome, in association with a rapidly growing cutaneous vascular mass. Treatment with systemic corticosteroids, interferon alfa, vincristine, and radiation therapy appeared to halt progression of the disease. None had evidence of human herpesvirus 8 infection. CONCLUSIONS: Cutaneous kaposiform hemangioendothelioma may appear in early infancy but often appears months to years later. It is frequently complicated by Kasabach-Merritt syndrome, has no known association with Kaposi sarcoma related to human immunodeficiency virus infection, and demonstrates aggressive local behavior with invasion but not distant metastasis. Awareness of this entity is important to prevent a mistaken diagnosis of common hemangioma of infancy.

Child, Preschool↗
PubMed1997

Infants with Kasabach-Merritt syndrome do not have "true" hemangiomas.

OBJECTIVE: In 1940 Kasabach and Merritt described an infant with a vascular anomaly, extensive purpura, and thrombocytopenia; they called his lesion "capillary hemangioma." Hemangioma is a benign tumor that grows in infancy and is characterized by proliferation of endothelial cells and regression during childhood. Although Kasabach-Merritt syndrome (KMS) is frequently mentioned as a possible complication of hemangioma, our experience suggests that the anatomic vascular lesion underlying the thrombocytopenia is not a "true," classic, involuting type of hemangioma of infancy and childhood. STUDY DESIGN: We reviewed the clinical and hemostasis data and the response to treatment in 22 cases of KMS, and we analyzed the biopsy specimens of 15 of them. RESULTS: Clinically none of the 22 patients had classic hemangioma. There was no female preponderance. All patients had severe thrombocytopenia (lowest platelet count = 3000/mm3) and consumption of fibrinogen. Histologically, none had the typical "capillary," involuting type of hemangioma of infancy: they exhibited either a tufted angioma or a kaposiform hemangioendothelioma pattern; all specimens also contained numerous abnormal lymphatic-like vessels; lymphatic malformation was the major component in two patients. The infants exhibited a heterogeneous response to a number of therapeutic regimens, as noted in other reports. Severe morbidity was present; three of our patients died, and one had leg amputation. "Residua" were, in fact, residual vascular neoplasia, variable in duration, and not a stable fibrofatty residuum, as in classic involuted hemangioma; only the hematologic phenomenon was "cured" after a period of years. CONCLUSIONS: KMS is a distinctive disease of infancy, but the underlying vascular lesion is not a "true," classic, involuting type of hemangioma of infancy. This is a different vascular tumor with a resemblance pathologically to either tufted angioma or kaposiform hemangioendothelioma in association with lymphatic-like vessels. Whether the underlying lesion in KMS is a single anatomic entity or heterogeneous cannot be definitely concluded from this study. We need a better understanding of the pathogenesis of KMS to improve our therapeutic management.

Biopsy↗
PubMed1997

Tinea capitis in California children: a population-based study of a growing epidemic.

OBJECTIVE: To describe trends in tinea capitis incidence among California children and to determine subpopulations at increased risk. DESIGN: Retrospective population-based study. SETTING: California, 1984 through 1993. POPULATION: Children < 10 years of age enrolled in Medi-Cal. OUTCOME MEASURES: California Medi-Cal provider data for first-time prescriptions of oral griseofulvin suspension were used to estimate annual incidence of tinea capitis and calculate risk ratios. RESULTS: From 1984 through 1993, the incident rate for prescriptions of oral griseofulvin suspension increased by 84.2% for all children, 140.4% for white children, and 209.7% for African-American children. In 1993, incidence rates (per 10,000 enrolled) were 252.1 claimants for African-American children, 23.1 for white, 17.5 for Hispanic, and 14.3 for Asian/Pacific Islander. The highest rate by location was San Francisco County (172.2). In age groups < 5 years and 5 to 9 years, African-American children were 13.1 and 17.6 times more likely to be prescribed griseofulvin than Hispanic children. Since 1987, incidence rates for children 5 to 9 years of age were higher compared with children ages < 5 years. CONCLUSIONS: Tinea capitis is epidemic among California children with higher rates in the northern counties studied. African-American children are the most affected by this epidemic; however, white children have also experienced increased rates.

Administration, Oral↗
PubMed1997

Methylmalonic acidemia, cobalamin C type, presenting with cutaneous manifestations.

BACKGROUND: Erosive dermatitis resembling the skin lesions of acrodermatitis enteropathica has been described in a number of aminoacidopathies and organic acidemias. In some, the dermatitis is a manifestation of untreated disease, while in others, including methylmalonic acidemia, skin lesions have been ascribed to nutritional deficiency due to therapeutic amino acid restrictions. OBSERVATIONS: We report 2 cases of methylmalonic acidemia presenting with cutaneous manifestations in the perinatal period before restrictive nutritional interventions. The cutaneous involvement consisted of cheilitis and diffuse erythema with erosions and desquamation. Methylmalonic acidemia, cobalamin C type, was subsequently diagnosed in both cases. CONCLUSIONS: An erosive, desquamating dermatitis with histopathologic characteristics resembling acrodermatitis enteropathica may be a presenting sign in cobalamin C methylmalonic acidemia, even in the absence of long-standing nutritional restrictions or deficiency.

Acrodermatitis↗
PubMed1996

PHACE syndrome. The association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities.

BACKGROUND: Large facial hemangiomas can have associated central nervous system malformations, particularly the Dandy-Walker posterior fossa malformations. Abnormal arteries, especially those of the central nervous system, coarctation of the aorta, cardiac defects, and unusual ophthalmologic abnormalities can also occur. OBSERVATIONS: We describe two patients with large facial hemangioma, congenital cataracts, and structural arterial abnormalities, particularly of the central nervous system vasculature. One of these infants also had a Dandy-Walker malformation detected on prenatal ultrasound at 12 weeks' gestation, suggesting that this syndrome had its origin during the first trimester of pregnancy. This infant also had a lingual thyroid and developed symptomatic hypothyroidism, possible induced by interferon alfa therapy of her hemangioma. These cases are discussed, along with 41 previously reported cases with similar findings. CONCLUSIONS: Large facial hemangiomas may have a distinctive group of associated arterial, central nervous system, and ophthalmologic anomalies. We propose the acronym PHACE syndrome to emphasize the characteristic findings of this neurocutaneous syndrome: posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities.

Abnormalities, Multiple↗
PubMed1996

The risk intraocular juvenile xanthogranuloma: survey of current practices and assessment of risk.

BACKGROUND: Juvenile xanthogranuloma (JXG) is an uncommon, usually uncomplicated disease of childhood. Ocular involvement, however, can result in glaucoma and blindness if left untreated. The incidence of ocular complications and how best to screen for their occurrence is unknown. OBJECTIVE: We attempted to ascertain management and referral practices among ophthalmologists and dermatologists and to characterize the risk for ocular complications in children with JXG. METHODS: A total of 431 dermatologists and 438 ophthalmologists were surveyed. In addition, the literature was reviewed. RESULTS: The response rate were 28% (dermatologists) and 44% (ophthalmologists). Most believed screening was important, but referral and surveillance practices varied widely. The survey incidence of ocular complications in patients with cutaneous JXG was approximately 0.3% (7 of 2371). The literature incidence was 0.4% (1 of 260). Children at maximum risk were 2 years of age or younger, had multiple skin lesions, and had newly diagnosed JXG. CONCLUSION: Ophthalmologic screening of patients with JXG should be particularly targeted to patients with risk factors of multiple skin lesions, new diagnosis, and age of 2 years or younger.

Age Factors↗
PubMed1996

Spontaneous atrophic patches in extremely premature infants. Anetoderma of prematurity.

BACKGROUND: Anetoderma, characterized clinically by macular depressions or outpouchings of skin, is associated with loss of dermal elastic tissue as noted on histopathologic findings. We report on 9 extremely premature infants who developed patches of anetoderma during their course in the neonatal intensive care unit. OBSERVATIONS: All 9 patients were born between the ages of 24 and 29 weeks of gestation and had numerous complications associated with prematurity. Eight of the 9 infants were noted to have developed anetoderma on the trunk and proximal extremities while in the neonatal intensive care unit. The locations of the lesions on the skin were not explained by previous trauma, although many areas corresponded with placement of monitoring leads or with adhesive for a monitoring device. Reduction or absence of elastic tissue supported the diagnosis of anetoderma in 4 of 5 biopsy specimens. CONCLUSION: We report a previously unrecognized type of anetoderma associated with extreme prematurity. The exact cause is uncertain, although reactions to cutaneous monitoring leads or adhesives is suspected.

Atrophy↗