Pancreatic panniculitis is an uncommon disorder with a distinctive histopathology. We report the youngest patient with pancreatic panniculitis caused by hypertriglyceridemia in association with nephrotic syndrome.
There is lack of information and evidence-based studies on the treatment of pediatric pustular psoriasis. Previous reports have emphasized the limitations of the existing data and encouraged the exploration of therapy optimization through more structured research. The objective of the current study was to perform a systematic review of systemic interventions for pediatric pustular psoriasis with an emphasis on clinical response and treatment outcomes. A systematic literature search was conducted using the PubMed and Embase databases from 1982 to 2012. Of 632 references identified, 14 met our inclusion criteria and were included in the analysis. A cohort of eight patients from the Hospital for Sick Children, Toronto, Canada, was also included. Information was limited to systemic treatments in children. Only English- and Spanish-language articles were included. Information was gathered from 24 patients, 22 of whom (92%) presented with generalized pustular psoriasis and 2 (8%) with acral distribution. The mean age at presentation was 6.3 ± 4.9 years. More than one intervention was required in 12 (50%) cases. The most common therapies used for generalized pustular psoriasis were acitretin, cyclosporine, and methotrexate. We identified that there is lack of information regarding long-term response to systemic drugs because the data were focused on treatment initiation. Treatment of pustular psoriasis in pediatrics is challenging. Although acitretin, methotrexate, and cyclosporine seem to control generalized pustular psoriasis within 3 months of therapy initiation, information on long-term follow-up is lacking. Furthermore, physicians may encounter difficulties after discontinuing or tapering medications.
Incontinentia pigmenti is a rare X-linked genodermatosis, often associated with male lethality in utero. Occurrences of this disease in boys have been reported, however, its clinical phenotype has not been well characterized. The purpose of this study was to report on additional instances of incontinentia pigmenti in boys and to review the clinical, laboratory, and molecular characteristics of all published such patients. A retrospective chart review and Medline search using the keywords incontinentia pigmenti, males, and NEMO gene was undertaken. Six new boys with incontinentia pigmenti were found in our database and 36 more were previously reported in the literature. The vesiculo-bullous stage was the most frequent clinical presentation at diagnosis (80%). Fifteen percent of patients had an initial unilateral presentation. Recurrences of this stage were noted in 16%. Stages 2 and 3 of the disease were present in only 72.5% and 75% of patients, respectively. Only 15% of the boys had a documented stage 4. Extracutaneous manifestations were also documented (30% - central nervous system manifestations, 35% - eye involvement, 30% - alopecia, 40% - teeth anomalies). Thirty two percent of boys had peripheral eosinophilia. Only five had evidence of NEMO gene mutation. The male phenotype has clinical features similar to those of the female phenotype. Unilateral presentation is a distinct occurrence in boys, especially in early stages. Anomalies are the most common extracutaneous findings, followed by eye, hair, and central nervous system abnormalities.
To inquire into and report on the ultimate results obtained in the 'treatment of simple fractures with or without operation. The committee is desirous of having the assistanee and cooperation of such surgeons, surgical registrars of hospitals, and other practitioners throughout the country as may be in a position to assist the inquiry by letting the committee have access to records of suitable cases, and by affording assistance in following up the patients to whom the records relate. The investigations of the committee will be limited to simple fractures of the long bones which have occurred within the five years from January, 1906, to December, 1910. We shall be glad if members of the profession able to help the committee in the ways stated will kindly communicate with the Medical Secretary of the Association, 429, Strand, W.C.-We are, Sir, yours faithfully,
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