Childhood molluscum contagiosum.
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Adjuvants, Immunologic↗
Biomedical subjects
Justin Brown
Publications and source records attributed to Justin Brown.
Regression of intracranial rosai-dorfman disease following corticosteroid therapy. Case report.
Rosai-Dorfman disease (RDD) is an idiopathic histioproliferative disorder usually presenting with massive, painless lymphadenopathy. Extranodal involvement has been reported including at least 50 cases affecting the central nervous system (CNS). The treatment of CNS RDD as reported in the literature has primarily involved a surgical technique. The authors report on the case of a 53-year-old man presenting with multiple skull base lesions mimicking meningiomas. The patient suffered visual deterioration and underwent a right orbitopterional craniotomy as well as optic nerve decompression. Histopathological analysis revealed histiocytic cells and emperipolesis consistent with RDD. Following surgery, corticosteroid agents were administered, leading to marked resolution of both the remaining surgically untreated lesions and the balance of the patient's symptoms. This report represents the first case of the resolution of intracranial RDD following corticosteroid therapy. Corticosteroid agents should be considered an effective option in the treatment of CNS RDD.
Brain Diseases↗
Practical management of the intersex infant.
Intersex occurs when the appearance of the internal or external genitalia is at variance with normal development for either sex. The first question asked by, and of new parents in relation to their offspring is often "Is it a boy or a girl"? A rational approach, based on knowledge of normal prenatal sexual development, and based on a careful physical examination to guide further investigation, is required to reach a diagnosis. We briefly review prenatal sexual development to provide a background to the assessment of genital ambiguity in the newborn. Aspects of physical examination are discussed in detail, with reference to published normative data where possible. We provide a classification of genital ambiguity and an approach to differential diagnosis. We highlight some of the many syndromes associated with genital ambiguity, with reference to their genetic basis where possible. In 46,XX individuals, the commonest cause of genital ambiguity is congenital adrenal hyperplasia due to 21-hydroxylase deficiency; however, in 46,XY individuals the differential diagnosis is wide, and may remain unexplained, even after extensive investigation. Two algorithms are presented, one of which provides an initial approach based on the presence of a uterus and palpable gonads alone, and a second illustrating a comprehensive differential diagnosis of the undervirilised 46,XY individual. We discuss our approach to sharing information on the diagnosis and management with the parents and highlight the early involvement of an experienced multidisciplinary team. Finally, we consider current controversial issues relating to gender assignment and management of genital ambiguity.
Chromosome Aberrations↗
Supernumerary nipples and renal malformations: a family study.
We describe a family with supernumerary nipples and kidney and urinary tract malformations. The proband is a 59-year-old man with a supernumerary nipple and recently identified hypoplastic kidney, bladder diverticulum, and hypotonic bladder. His mother also has a supernumerary nipple and a recently identified hypoplastic kidney. Of his three children, all male, one has a supernumerary nipple but has not been evaluated for a kidney or urinary tract malformation. This familial disorder is discussed.
Abnormalities, Multiple↗
Palmoplantar hyperhidrosis: a therapeutic challenge.
Excessive sweating from the palms and soles, known as palmoplantar hyperhidrosis, affects both children and adults. Diagnosis of this potentially embarrassing and socially disabling condition is based on the patient's history and visible signs of sweating. The condition usually is idiopathic. Treatment remains a challenge: options include topical and systemic agents, iontophoresis, and botulinum toxin type A injections, with surgical sympathectomy as a last resort. None of the treatments is without limitations or associated complications. Topical aluminum chloride hexahydrate therapy and iontophoresis are simple, safe, and inexpensive therapies; however, continuous application is required because results are often short-lived, and they may be insufficient. Systemic agents such as anticholinergic drugs are tolerated poorly at the dosages required for efficacy and usually are not an option because of their associated toxicity. While botulinum toxin can be used in treatment-resistant cases, numerous painful injections are required, and effects are limited to a few months. Surgical sympathectomy should be reserved for the most severe cases and should be performed only after all other treatments have failed. Although the safety and reliability of treatments for palmoplantar hyperhidrosis have improved dramatically, side effects and compensatory sweating are still common, potentially severe problems.
Administration, Topical↗
L-carnitine reduces brain injury after hypoxia-ischemia in newborn rats.
Perinatal hypoxia-ischemia remains a significant cause of neonatal mortality and neurodevelopmental disability. Numerous lines of evidence indicate that cerebral ischemic insults disrupt normal respiratory activity in mitochondria. Carnitine (3-hydroxy-4-N-trimethylammonium-butyrate) has an essential role in fatty acid transport in the mitochondrion and in modulating potentially toxic acyl-CoA levels in the mitochondrial matrix. There are no naturally occurring esterases available to reduce the accumulation of acyl-CoA but this process can be overcome by exogenous carnitine. We used a newborn rat model of perinatal hypoxia-ischemia to test the hypothesis that treatment with l-carnitine would reduce the neuropathologic injury resulting from hypoxia-ischemia in the developing brain. We found that treatment with l-carnitine during hypoxia-ischemia reduces neurologic injury in the immature rat after both a 7- and 28-d recovery period. We saw no neuroprotective effect when l-carnitine was administered after hypoxia-ischemia. Treatment with d-carnitine resulted in an increase in mortality during hypoxia-ischemia. Carnitine is easy to administer, has low toxicity, and is routinely used in neonates as well as children with epilepsy, cardiomyopathy, and inborn errors of metabolism. l-Carnitine merits further investigation as a treatment modality for the asphyxiated newborn or as prophylaxis for the at-risk fetus or newborn.
Animals↗
A retrospective chart review of the use of olanzapine for the prevention of delayed emesis in cancer patients.
Chemotherapy-induced delayed emesis (DE) affects approximately 50-70% of patients receiving moderately and highly emetogenic chemotherapy. DE most commonly occurs within the first 24-48 hours of chemotherapy administration and can persist for 2-5 days. Olanzapine, which has been used anecdotally for chronic nausea in advanced cancer patients, might be a useful treatment for the prevention of delayed emesis in chemotherapy patients. We conducted a chart review to explore this hypothesis and to plan potential studies. Using pharmacy records or an electronic medical record, we identified all patients who had received olanzapine in the oncology clinic (n = 98). We reviewed these records and selected all patients (n = 28) who had received olanzapine for the prevention of delayed emesis for structured review. There were 17 women (60.7%) and 11 men (39.3%). Eleven patients (39.3%) had at least one instance of nausea recorded while undergoing olanzapine treatment and seven (25%) had an episode of vomiting recorded. During 95 total cycles of chemotherapy with olanzapine (mean = 3.4 cycles per patient), there were 21 incidents of nausea (22.1%) and 10 instances of vomiting (10.5%). Side effects were rarely noted. These data suggest that olanzapine was well tolerated and may reduce the incidence of delayed emesis in patients receiving moderate to highly emetogenic chemotherapy. A series of prospective trials are underway.
Adult↗
Response to intravenous immunoglobulin predicts splenectomy response in children with immune thrombocytopenic purpura.
OBJECTIVE: Response to intravenous immunoglobulin (IVIG) has been shown to predict response to splenectomy in adults with immune thrombocytopenic purpura (ITP). However, reports in children have been inconsistent. We sought to determine whether response to IVIG is predictive of response to splenectomy in children. METHODS: Thirty-two assessable children were identified by a retrospective chart review. Response was graded according to previously published criteria as follows: "excellent" (platelets >150 000 within 1 week), "good" (platelets between 50 000 and 150 000), and "poor" (platelets <50 000). "Response" refers to both splenectomy and IVIG, and response to splenectomy was counted only when it was durable. RESULTS: Twenty-one of 23 patients who had a good or excellent response to IVIG also had an excellent response to splenectomy. Six of 9 patients who had a poor response to IVIG also had a poor response to splenectomy. Response to IVIG was a sensitive predictor of response to splenectomy in 88% of patients. Response to IVIG had a specificity of 75%, a positive predictive value of 91%, and a negative predictive value of 67%. Response to prednisone and length of time to splenectomy were not correlated with splenectomy response. CONCLUSIONS: These results suggest that response to IVIG is predictive of response to splenectomy in children with chronic ITP. This correlation may be of value in deciding whether a splenectomy should be performed in children with chronic ITP.
Adolescent↗
Supernumerary nipples: an overview.
Supernumerary nipples (SNs) are relatively common, minor congenital anomalies. SNs usually arise within the embryonic milk lines, but they have been known to form elsewhere. The prevalence of SNs varies, depending on the population. SNs are normally benign entities, but they are susceptible to hormonal changes and disease processes and may signify internal disease.
Congenital Abnormalities↗
Neonatal diabetes mellitus: Insulin pump as an alternative management strategy.
Neonatal diabetes mellitus (hyperglycaemia within the first month of life, with an insulin requirement) may be transient or permanent. Management is complex, due to lack of subcutaneous fat and the need for small doses of insulin, and may be complicated by additional medical problems. Three cases are presented, the first of which was treated with conventional insulin therapy. The latter two were successfully treated with subcutaneous insulin pump therapy. We present suggested guidelines for treatment of neonatal diabetes, using this novel approach to management.
Diabetes Mellitus↗