Although congenital syphilis can be prevented with prenatal screening, the disease remains problematic. Currently, there are no cases that describe hematuria and pneumonia related to congenital syphilis. We report a case of congenital syphilis that involved nephrotic syndrome and pneumonia alba in a 22-day-old male infant whose mother did not receive adequate prenatal care. The congenital syphilis diagnosis was confirmed with a serologic test and the patient recovered with penicillin treatment. Clinical findings may be subtle in neonates and delayed recognition occurs frequently, thus complete prenatal screening is critical for congenital syphilis prevention. Immediate serologic testing should be performed to obtain a differential diagnosis if an infant is delivered by a mother that has not received appropriate prenatal examinations.
Diagnosis ; Diagnosis, Differential ; Exanthema* ; Hematuria* ; Humans ; Infant ; Infant, Newborn ; Male ; Mothers ; Nephrotic Syndrome ; Penicillins ; Pneumonia* ; Prenatal Care ; Prenatal Diagnosis ; Serologic Tests ; Syphilis, Congenital*

PURPOSE: The aim of this study was to develop an appropriate nursing information guideline according to corrected age, after investigating parents' concerns about the growth, development, and diseases of their premature infants after discharge from the neonatal intensive care unit (NICU). METHODS: The parents of premature infants (birth weight, <2,500 g; gestational age, <37 weeks) who went to a neonatal follow-up clinic after NICU discharge at Seoul St. Mary's Hospital from January 2005 to December 2009, were asked with regard to their concerns about their infants through a questionnaire survey. The results of physical examinations, including body measurements and neurodevelopmental status at 4, 8, 12, and 18 months of corrected age, were retrospectively reviewed in 390 infants. RESULTS: The most common parental concerns were developmental delay, poor growth, and feeding and nutritional problems. Parental concerns about developmental delay, growth failure in improvement in body weight and length, and overweightness were high in specificity but very low in sensitivity. After NICU discharge, 30% of premature infants experienced infectious diseases before 18 months of corrected age, the most common of which was respiratory tract infection. CONCLUSION: For guiding of premature infants in outpatient day clinics after NICU discharge, it is necessary to identify the parents' highest concerns, to educate them about the possibilities of growth and neurodevelopmental disabilities in their infants and to provide them with handouts containing guidelines on the management of infectious diseases, especially respiratory infections.
Body Weight ; Communicable Diseases ; Follow-Up Studies ; Gestational Age ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Intensive Care Units, Neonatal ; Intensive Care, Neonatal ; Outpatients ; Overweight ; Parents ; Physical Examination ; Surveys and Questionnaires ; Respiratory System ; Respiratory Tract Infections ; Retrospective Studies ; Sensitivity and Specificity

Linoleic acid [LA; 18: 2 (n-6)] is the most abundant polyunsaturated fatty acid in human skin. The exclusion of LA from diet induces epidermal hyperproliferation, which is reversible by the inclusion of LA in diet, and hence, LA is heralded as an essential fatty acid (EFA). Since safflower oil (SO) has been widely recognized as the major dietary source of LA and Arctii Fructus (Arctium lappa L.) is recently reported to contain high level of LA, we compared the antiproliferative effects of SO and Arctii Fructus in this study. Epidermal hyperproliferation was induced in guinea pigs by hydrogenated coconut oil (HCO) diet for 8 wk. During following 2 wk, EFA deficient guinea pigs were fed diets of safflower oil (group HS), water extract of Arctii Fructus (group AW) or organic extract of Arctii Fructus (group AO). Normal control group was fed SO containing diet (group SO) and EFA deficient group was fed HCO containing diet (group HCO) for 10 wk. Epidermal hyperproliferation was reversed in groups AO (55.9% of group HCO) and HS(74.1% of group HCO). However, the thymidine incorporation into epidermal DNA of group HS was greater than of normal control group SO. Epidermal hyperproliferation was not reversed in group AW. The accumulations of LA into phospholipids and ceramides, and of 13-hydroxyoctadecadienoic acid (13-HODE), the potent antiproliferative metabolite of LA in the epidermis of group AO were greater than of group HS. In contrast, the de novo synthesis of ceramides, the major lipids maintaining epidermal barrier, did not differ between all of groups. Together, our data demonstrate that organic extract of Arctii Fructus is more prominent than safflower oil in reversing epidermal hyperproliferation by inducing the higher accumulations of LA and 13-HODE in the epidermis of guinea pigs.
Animals ; Ceramides ; Cocos ; Diet ; DNA ; Epidermis ; Guinea Pigs* ; Guinea* ; Humans ; Hydrogen ; Linoleic Acid* ; Phospholipids ; Safflower Oil ; Skin ; Thymidine ; Water

Being born small for gestational age (SGA) has been strongly associated with mortality during the perinatal period and long-term risk of metabolic syndrome, including type 2 diabetes mellitus, hypertension, hyperlipidemia, and coronary heart disease. Insulin resistance is an important factor in the development of metabolic syndrome in SGA, with several proposed hypotheses. Here, we report two cases of non-obese adolescent patients with early onset type 2 diabetes who were born SGA. Of these, one experienced catch-up growth, while the other did not. Both had a high body fat percentage at the time of diagnosis of type 2 diabetes and were diagnosed with fatty liver and hyperlipidemia before adolescence, at the age of 7 years. Early interventions for SGA are needed for healthy catch-up growth to prevent metabolic diseases in the future.

Mycoplasma pneumoniae causes various extra-pulmonary complications. As a rare but fatal hematological complication, hemophagocytic lymphohistiocytosis (HLH) can be observed in children with M. pneumoniae infection. We report a case of a 6-year-old girl with HLH who was initially presumed to have macrolide-refractory M. pneumoniae pneumonia. Despite the combination treatment of antimicrobial and anti-inflammatory agents, she showed persistent fever, hepatosplenomegaly, and thrombocytopenia. Secondary HLH associated with M. pneumoniae should be considered if unexplained clinical deterioration is noted in children with macrolide-refractory M. pneumoniae pneumonia.

Mycoplasma pneumoniae causes various extra-pulmonary complications. As a rare but fatal hematological complication, hemophagocytic lymphohistiocytosis (HLH) can be observed in children with M. pneumoniae infection. We report a case of a 6-year-old girl with HLH who was initially presumed to have macrolide-refractory M. pneumoniae pneumonia. Despite the combination treatment of antimicrobial and anti-inflammatory agents, she showed persistent fever, hepatosplenomegaly, and thrombocytopenia. Secondary HLH associated with M. pneumoniae should be considered if unexplained clinical deterioration is noted in children with macrolide-refractory M. pneumoniae pneumonia.

Being born small for gestational age (SGA) has been strongly associated with mortality during the perinatal period and long-term risk of metabolic syndrome, including type 2 diabetes mellitus, hypertension, hyperlipidemia, and coronary heart disease. Insulin resistance is an important factor in the development of metabolic syndrome in SGA, with several proposed hypotheses. Here, we report two cases of non-obese adolescent patients with early onset type 2 diabetes who were born SGA. Of these, one experienced catch-up growth, while the other did not. Both had a high body fat percentage at the time of diagnosis of type 2 diabetes and were diagnosed with fatty liver and hyperlipidemia before adolescence, at the age of 7 years. Early interventions for SGA are needed for healthy catch-up growth to prevent metabolic diseases in the future.

Purpose: Despite enzyme replacement therapy (ERT) and/or allogeneic hematopoietic stem cell transplantation, individuals with mucopolysaccharidosis (MPS) I or II often experience significant growth deficiencies. This study aimed to assess the safety and efficacy of recombinant human growth hormone (hGH) treatment in children diagnosed with MPS I or II. Materials and Methods: A total of nine pediatric patients—four with MPS I and five with MPS II—underwent treatment with ERT and hGH at Samsung Medical Center. Results: The mean hGH dose administered was 0.26±0.03 mg/kg/week. In the MPS I group, three patients showed an increase in height Z-score from –4.09±0.83 to –3.68±0.43 after 1 year of hGH treatment, and to –3.10±0.72 by the end of the hGH regimen. In the MPS II group, while the height Z-score of four patients decreased according to standard growth charts, it improved from 1.61±1.79 to 2.71±1.68 based on the disease-specific growth chart through hGH treatment. Two patients discontinued hGH treatment due to lack of efficacy after 22 and 6 months each of treatment, respectively. No new-onset neurological symptoms or necessity for prosthetic or orthopedic surgery were reported during hGH treatment. Conclusion This study provides insights into the impact of hGH on MPS patients, demonstrating its potential to reverse growth deceleration in some cases. Further research is needed to explore the long-term effects of hGH on changes in body composition, muscle strength, and bone health in this population.

BACKGROUND: A disruption of the balance between the water content of the stratum corneum (SC) and skin surface lipids may lead to the clinical manifestation of dryness of skin in patients with atopic dermatitis (AD). OBJECTIVE: To determine whether supplementation of gromwell (Lithospermum erythrorhizon), one of herbs used in East Asia in remedies for various abnormal skin conditions, may improve the SC level of hydration and ceramides, major lipid in SC in patients with AD. METHODS: A total of 28 subjects with AD were randomly assigned into two groups: either gromwell group received dextrose contained capsules with 1.5 g of gromwell extracts or placebo group received only dextrose contained capsules for 10 weeks. RESULTS: In contrast to no alteration of SC hydration and ceramides in placebo group, the SC hydration in gromwell group was significantly increased in parallel with an increase of SC ceramides. Furthermore, % increase of SC hydration in gromwell group bore a positive correlation with the clinical severity, which suggests that the increase of SC hydration in gromwell group was more effective as AD was more severe. CONCLUSION: Supplementation of gromwell improves SC hydration in parallel with an increase of ceramides in part.
Capsules ; Ceramides ; Dermatitis, Atopic ; Far East ; Glucose ; Humans ; Lithospermum ; Skin