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Biomedical subjects

A V Levin

Publications and source records attributed to A V Levin.

At least 19 recordsLinked to original sources
PubMed1996

Natural history of Alström syndrome in early childhood: onset with dilated cardiomyopathy.

Alström syndrome is an autosomal recessive disorder characterized by cone-rod dystrophy, obesity, hearing impairment, and diabetes caused by insulin resistance. By reviewing the charts of eight patients followed for periods of 2 to 22 years, we established the natural history of this syndrome during childhood. Five patients, in four families, were seen between the ages of 3 weeks and 4 months with a dilated cardiomyopathy, a previously unrecognized feature of the syndrome. Photophobia and nystagmus were first documented in the eight patients between the ages of 5 months and 15 months. In all patients, electroretinography initially showed a severe cone impairment with mild (2/8) or no (6/8) rod involvement. Electroretinograms, obtained again at ages 9 to 22 years for four patients, revealed extinguished rod-and-cone responses. Obesity developed during childhood in seven patients, in at least three of them before age 2 years. Hearing impairment (5/8) and diabetes/glucose intolerance (4/8) were diagnosed at the end of the first decade or during the second decade. This constellation of features should facilitate early diagnosis of the syndrome.

Adolescent↗
PubMed1996

Abnormal ocular enhancement in Sturge-Weber syndrome: correlation of ocular MR and CT findings with clinical and intracranial imaging findings.

PURPOSE: To estimate the prevalence of abnormal ocular enhancement in children with Sturge-Weber syndrome as detected with MR imaging and CT and to correlate this with the clinical, fundoscopic, and intracranial imaging findings. METHODS: Fifteen children, 4 years old or younger, with Sturge-Weber syndrome were examined with enhanced CT and MR imaging. Eleven children had unilateral intracranial involvement and 4 had bilateral involvement, for a total of 19 abnormal hemispheres and related orbits. The presence of ocular enhancement was compared with the fundoscopic findings independently. Ocular enhancement was correlated with the extent of leptomeningeal disease, the severity of the cutaneous lesion, and the presence of glaucoma by the calculation of likelihood ratios and 95% confidence limits. RESULTS: Seven of the 15 patients had abnormal ocular enhancement, which was present in 10 (53%) of the eyes associated with the 19 abnormal hemispheres. MR imaging showed choroidal hemangioma in 7 of 8 patients in whom hemangiomas were shown at fundoscopy. The likelihood of ocular enhancement was increased with the presence of bilateral disease, extensive facial nevi, and glaucoma; there was no significant correlation with the extent of hemispheric involvement. CONCLUSION: Both enhanced MR imaging and CT can show diffuse choroidal hemangioma in patients with Sturge-Weber syndrome. However, MR imaging is more sensitive and is recommended to aid in the detection of abnormalities with preventable late complications.

Brain↗
PubMed1995

Absence of the greater sphenoid wing in neurofibromatosis type I: congenital or acquired: case report.

unilateral absence of the greater wing of the sphenoid bone is a distinctive but uncommon manifestation of Type I neurofibromatosis, which has until now been regarded as a developmental anomaly of mesodermal origin. A computed tomographic scan was obtained in a 4-week-old infant with an abnormal left eye. The scan demonstrated an intact ipsilateral sphenoid bone, except for minor expansion of the medial end of the left superior orbital fissure. Another computed tomographic scan was obtained 6 years later, when the child had café-au-lait patches, axillary freckling, Lisch nodules, and left phthisis bulbi. This later scan showed typical sphenoid dysplasia. Much of the greater wing was absent, and the anterior temporal pole was displaced anteriorly. In this article, we discuss the implications of this case in terms of the cause of this condition and the diagnosis of Type I neurofibromatosis.

Eye Abnormalities↗
PubMed1994

Modified Apt test.

Explore the source record for details and available documents.

Emergency Service, Hospital↗
PubMed1992

Retinal findings after head trauma in infants and young children.

BACKGROUND: Many authorities believe that the finding of retinal hemorrhages in a child younger than 3 years of age with a history of head trauma, in the absence of an obvious cause for the injury, is pathognomonic of child abuse. To date, no studies have examined the prospective retinal examination of children who have had head trauma. The authors undertook such a study because the presence of retinal hemorrhage from any head trauma in children may have medicolegal diagnostic significance in differentiating accidental from nonaccidental trauma. METHODS: Seventy-nine children younger than 3 years of age, each of whom experienced head injury, underwent an ophthalmologic assessment, which included a dilated funduscopic examination. RESULTS: Seventy-five children sustained accidental head injuries and had normal funduscopic examinations. Three children had nonaccidental head injuries and all were found to have varying degrees of retinal hemorrhages. One child, with a normal fundus examination, had injuries that were of indeterminate cause. CONCLUSION: The finding of retinal hemorrhages in a child with a head injury suggests a nonaccidental cause.

Battered Child Syndrome↗
PubMed1992

Corneal diameter, axial length, and intraocular pressure in premature infants.

PURPOSE: Seventy premature infants 25 to 37 weeks' postconceptional age were examined during their first week of life to determine the correlation of corneal diameter, axial length, and intraocular pressure with gestational age and birth weight. METHODS: Corneal diameter measurement was determined with corneal templates, total axial length with standardized A-scan ultrasound, and intraocular pressure with a Tonopen II tonometer. RESULTS: Corneal diameter and total axial length showed parallel linear increases from 6.2 mm to 9.0 mm and 12.6 mm to 16.2 mm, respectively; however, no significant correlation was found between intraocular pressure and gestational age or birth weight. The mean intraocular pressure was 10.3 mmHg (standard deviation, 3.5). CONCLUSION: Normative values are established for corneal diameter and total axial length as they relate to birth weight and gestational age, and a mean and standard deviation for intraocular pressure in the premature newborn. These values will aid the ophthalmologist in assessing ocular dimensions in premature infants.

Birth Weight↗
PubMed1992

Screening for fungal endophthalmitis in children at risk.

To evaluate the efficacy of screening ophthalmologic examinations in high-risk children, we reviewed the medical records for all patients hospitalized from 1985 through 1989 at The Hospital for Sick Children, Toronto, Ontario, who underwent ophthalmological consultation to rule out endogenous fungal endophthalmitis (n = 176). The patients were divided into groups: Group 1 (n = 47), those with deep-tissue fungal infection, and Group 2 (n = 129), those at risk for invasive fungal disease. Group 2 was subdivided further into two subgroups: Group 2a (n = 48), those with evidence of superficial fungal colonization (positive fungal culture) but no deep-tissue involvement, and Group 2b (n = 81), those with no evidence of fungal colonization (negative fungal culture). Of these 176 patients, 7 were diagnosed with endogenous fungal endophthalmitis: 6 from Group 1, 1 from Group 2a, and 0 from Group 2b. We found a significant association between the development of endogenous fungal endophthalmitis and the status of the fungal culture result (P less than .005). The odds ratio indicated the risk of endogenous fungal endophthalmitis in Group 1 patients with deep-tissue infection was at least 19 times that of Group 2 at-risk patients. The risk of endogenous fungal endophthalmitis in Group 1 patients was at least 7 times that of Group 2a colonized patients and 12 times that of Group 2b patients with no positive fungal culture. Our study confirms the necessity of careful dilated ophthalmoscopic examination in patients with invasive fungal disease and suggests screening for those at-risk patients with superficial fungal colonization.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1991

Eye emergencies: acute management in the pediatric ambulatory care setting.

Pediatric emergency department visits commonly relate to eye problems. Over 70,000 children under the age of 15 years are treated annually for eye injuries. Ocular examination is also often performed in the ambulatory care setting when the physician is evaluating systemic problems, such as head trauma, to look for abnormalities such as papilledema. For these reasons, it is essential that the acute care physician becomes familiar with the initial evaluation and management of ocular disorders. Unfortunately, this evaluation may be hampered by inadequate instrumentation, an uncooperative patient, or a fear of causing further damage to the eye. However, primary care physicians can perform a wide range of diagnostic and therapeutic interventions with a minimum of technology and a modicum of patience and understanding.

Acute Disease↗
PubMed1989

Shaken baby syndrome diagnosed by magnetic resonance imaging.

We report an infant with Shaken Baby syndrome (SBS) who presented with status epilepticus. The initial evaluation with computerized axial tomography (CAT scan) of the head was normal, and there was no history or physical finding consistent with physical abuse or shaking. This prompted an extensive evaluation to determine the etiology of the seizures. An ophthalmology consultation revealed the presence of severe bilateral retinal hemorrhages, which raised the possibility of SBS. Magnetic resonance imaging (MRI) showed cerebral hemorrhages, hemorrhagic contusions, and bilateral subtemporal subdural hematomas. This is the first reported case of SBS diagnosed by magnetic resonance imaging following a normal initial CAT scan. MRI may be a valuable tool in the diagnosis of brain injury in SBS and may be particularly valuable when the CAT scan of the head is normal, the etiology of neurologic injury is unclear, and the presence of retinal hemorrhages raises the suspicion of SBS.

Brain Injuries↗
PubMed1989

Cytomegalovirus retinitis in an infant with acquired immunodeficiency syndrome.

A case of cytomegalovirus retinitis in an infant with acquired immunodeficiency syndrome (AIDS) is described. Although well recognized as an ocular manifestation of AIDS in adults, only one case of the necrotic retinitis caused by cytomegalovirus has been described in a child with AIDS. Intravenous treatment with ganciclovir resulted in substantial ocular improvement, despite the advanced nature of the disease in one eye in which there was also secondary neovascular glaucoma. Home maintenance treatment was used via Broviac catheter. The patient later died following pulmonary infection with Pneumocystis carinii.

Acquired Immunodeficiency Syndrome↗
PubMed1989

Functional blinking in childhood.

At the Department of Pediatric Ophthalmology, Wills Eye Hospital, 17 children, 18 months to 10 years of age, were seen with a chief complaint of intermittent excessive blinking. Eight (47%) of the patients had been referred for ophthalmologic examination by their pediatrician for evaluation of this symptom. All were in excellent health with no associated symptoms or signs of systemic or ocular disease. None of the children were taking topical ocular or systemic medications. The parents of seven (41%) of these children were able to identify a temporally related stressful event that coincided with the onset of blinking. Durations of the symptom prior to examination ranged from 1 week to 4 months. Spontaneous resolution occurred from one day to 5 months after the first ophthalmologic examination. Recurrence occurred in only one child, but resolution was still completed within 5 months. Excessive eye blinking unassociated with other systemic or ocular findings appears to be a medically benign, self-limited functional disorder.

Blinking↗
PubMed1988

Identification of sequence-specific DNA-binding factors by label transfer: application to the adenovirus-2 major late promoter.

A method of affinity labelling proteins specifically associated with DNA target sequences is proposed. The method utilizes covalent UV-crosslinking of proteins to highly labelled DNA (e.g. in crude cell or nuclear extracts) followed by degradation of the DNA to short oligonucleotides. Proteins selectively labelled by attached residual oligonucleotides are readily amenable to molecular mass determination. Using this approach, we have characterized a HeLa polypeptide specifically bound to a short segment of the adenovirus-2 major late promoter (Ad2 MLP). A molecular mass value (approximately 51 kD) and precise location of the crosslinking site(s) of the protein within the MLP (-55 with respect to the cap site) were determined.

Adenoviruses, Human↗
PubMed1988

Extended-wear contact lenses for the treatment of pediatric aphakia.

The practicality of extended-wear contact lenses in the refractive correction of pediatric aphakia was assessed with 240 eyes in 184 patients. Patient ages at the time of contact lens fitting ranged from 18 days to 9.8 years. One hundred forty-one eyes were fit from 1 day to 55 months postoperatively and then followed for 6 months to 5.7 years (average, 29 months). Only five patients lost more than five lenses. The overall loss rate was less than one lens per year of follow-up. No patient had contact lens-related complications with permanent visual sequelae. Only 14% of patients had contact lens problems or factors related to parental inability to care for the lens which resulted in discontinuation of contact lens therapy. The authors were unable to identify any subset of patients who should be considered for primary surgical optical correction of their aphakia.

Aphakia, Postcataract↗
PubMed1988

Vulvar hemangioma simulating child abuse.

An infant with a vulvar hemangioma that had undergone extensive ulcerative changes was initially diagnosed as having a perineal burn secondary to child abuse. Although genital burns are a well-recognized manifestation of child abuse, knowledge of the natural course of untreated hemangiomas and the uncommon complication of ulceration as well as familiarity with the characteristic patterns of abusive burn injuries may allow the physician to avoid making this misdiagnosis. The confusion in diagnosis led to an extensive social service investigation that, in this case, had a positive outcome for the mother's ability to cope. There exists, however, the possibility of iatrogenic-induced emotional stress if child abuse is misdiagnosed.

Burns↗