A case of neonate effectively treated with everolimus for giant hepatic hemangioma complicated with congenital duodenal atresia and Kasabach-Merritt syndrome.

BACKGROUND: Disseminated intravascular coagulation (DIC) with Kasabach-Merrit syndrome from a large hepatic hemangioma is life-threatening. We report a case of giant hepatic hemangioma of the newborn with KMS. RESULTS: The patient was born at 37 gestational weeks and 2 days via cesarean section; weight at birth was 2952 g. Congenital duodenal atresia was noted during the fetal period. DIC developed after delivery and a giant liver hemangioma was diagnosed via abdominal CT. The cause of DIC was Kasabach-Merritt syndrome owing to a giant hepatic hemangioma. First, combination therapy of 2 mg/kg/day of prednisolone and 0.2 mg/kg/day of propranolol was initiated form enterostomy. However, the size of the hepatic hemangioma did not alter, as observed via image evaluation. Therefore, 0.3 mg/kg/day of everolimus was administered frorm enterostomy. Subsequently, the size of the hepatic hemangioma was assessed via image evaluation. Although it did not alter, blood flow to the hepatic hemangioma decreased and thrombocytopenia was also suppressed. We performed hepatic lateral segmentectomy, radical operation for duodenal atresia. The pathological diagnosis of the removed tumor was infantile hemangioma. CONCLUSION: We report everolimus may be useful when PSL and propranolol are ineffective.

Effects of assisted reproductive technologies in neonates with indications for surgery (2007-2016).

PURPOSE: Recently, the number of births using assisted reproductive technologies (ART) has increased. An associated increase in the incidence of congenital malformations in babies conceived using this technology has also been reported. Therefore, we aimed to investigate the rate of malformations in babies with neonatal surgical diseases, who were conceived using ART. MATERIALS AND METHODS: Between January 2007 and December 2016, 1737 patients were admitted to our hospital. We analyzed the incidence of congenital cardiac diseases, genetic anomalies, and congenital anomalies of the kidney and urinary tract (CAKUT) in neonates conceived by ART. The χ2 test and logistic regression analysis were used to assess the odds ratios (ORs) for congenital malformations. A P-value < 0.05 indicated statistical significance. RESULTS: The OR for CAKUT was 16.94 for the first-birth neonates conceived using ART, [P < 0.05, AUC (area under the curve) = 0.86]. However, for non-surgery neonates, the OR for CAKUT was 5.99 (P = 0.15, AUC = 0.87), compared to 32.27 (P < 0.05, AUC = 0.93) for parallel conditions in surgery-neonates. CONCLUSION: Neonates conceived using ART are prone to develop CAKUT, which will need surgical treatment. Therefore, more management is necessary for associated malformations in these babies, particularly in cases with CAKUT.

Ceramide synthase 4 is highly expressed in involved skin of patients with atopic dermatitis.

BACKGROUND: Ceramide is a crucial lipid in the stratum corneum (SC) which maintains the barrier function and hydration of the skin. In atopic dermatitis (AD) patients who have defective skin barrier function, ceramide levels are altered. We previously reported that although the amount of total ceramide was lower in involved skin compared with uninvolved skin of AD patients and with healthy control skin, the amounts of smaller ceramide species of Cer[NS] (<40 total carbons, which are total carbons of both sphingoid base and amide-linked fatty acid), especially Cer[NS] with 34 total carbons (C34-Cer[NS]), were higher. However, the enzyme(s) that produces the higher levels of smaller ceramide species in involved skin of AD patients was unclear. OBJECTIVE: To identify the enzyme(s) that produces higher levels of smaller ceramide species of Cer[NS] in the involved skin of AD patients. METHODS: Eight female Caucasian subjects who were diagnosed with AD on their arms (age range: 21-45 years) were enroled in this study. We compared ceramide levels in the SC and the expression levels of enzymes involved in ceramide metabolism using real-time PCR and immunohistochemistry between involved and uninvolved skin of AD patients. RESULTS: Level of mRNA encoding ceramide synthase 4 (CERS4), which is one of the enzymes that synthesize ceramide from a sphingoid base and an amide-linked fatty acid, was significantly higher in involved skin than in uninvolved skin (P < 0.01). Additionally, the protein expression level of CERS4 in the epidermis was also higher in involved skin compared with uninvolved skin. The expression level of CERS4 correlated with the amount of C34-Cer[NS] (P < 0.01) and the skin hydration value (P < 0.05). CONCLUSIONS: The elevated expression level of CERS4 contributes to the increase of C34-Cer[NS] and the impaired SC barrier function in involved skin of AD patients.

The roughness of lip skin is related to the ceramide profile in the stratum corneum.

OBJECTIVE: The structure of skin on the lips is different from skin at other areas of the body; the water content of the stratum corneum (SC) and the barrier function there is low. Therefore, the lips can easily become dry and rough. The SC plays an important role in the barrier and water-holding functions of the skin. Above all, ceramides (CERs) are important SC lipids which maintain SC functions. The purpose of this study was to clarify the relationship between the degree of lip roughness and the CER profile. METHODS: Forty-one Japanese female subjects with normal skin (age range 22-52 years; mean 34.1) were enrolled in this study. The degree of lip roughness was scored, the values of capacitance and transepidermal water loss (TEWL) were measured and CER profiles of the SC were analysed using tape-stripping. RESULTS: The degree of lip roughness was related to the capacitance values but not the TEWL values. The roughness level correlated with the levels of CER[NH], CER[NP], CER[AH], CER[EOS] and CER[EOH] and the average carbon numbers of CER[NDS], CER[NH] and CER[NP] but not with the level of total CERs. The capacitance values of the lips were related not only with the levels of total CERs but also with the levels of CER[NH], CER[NP], CER[AH], CER[AP], CER[EOS], CER[EOH] and CER[EOP] and with the average carbon numbers of CER[NDS], CER[NS], CER[NH] and CER[NP]. The TEWL values of the lips were related not only with the levels of total CERs but also with the levels of CER[NH], CER[AH], CER[AP], CER[EOS] and CER[EOH]. CONCLUSION: The relationship between lip roughness and the CER profile was clarified for the first time. The results suggest that not only the level of total CERs but also the specific CER species and their carbon numbers affect the maintenance of SC function.

Eccentric training for shoulder abductors improves pain, function and isokinetic performance in subjects with shoulder impingement syndrome: a case series.

BACKGROUND: Conservative treatments have been proposed for people with shoulder impingement syndrome (SIS), such as strengthening of the rotator cuff and scapular muscles and stretching of the soft tissues of the shoulder. However, there is a lack of studies analyzing the effectiveness of eccentric training in the treatment of SIS. OBJECTIVES: To evaluate the effects of eccentric training for shoulder abductors on pain, function, and isokinetic performance during concentric and eccentric abduction of the shoulder in subjects with SIS. METHODS: Twenty subjects (7 females, 34.2 SD 10.2 years, 1.7 SD 0.1 m, 78.0 SD 16.3 kg) with unilateral SIS completed the study protocol. Bilateral isokinetic eccentric training at 60º/s for shoulder abductors was performed for six consecutive weeks, twice a week, on alternate days. For each training day, three sets of 10 repetitions were performed with a 3-minute rest period between the sets for each side. The range of motion trained was 60° (ranging from 80° to 20°). The Disabilities of the Arm, Shoulder and Hand (DASH) questionnaire was used to evaluate functional status and symptoms of the upper limbs. Peak torque, total work and acceleration time were measured during concentric and eccentric abduction of the arm at 60º/s and 180º/s using an isokinetic dynamometer. RESULTS: DASH scores, peak torque, total work and acceleration time improved (p<0.05) after the period of intervention. CONCLUSIONS: This study suggests that isokinetic eccentric training for shoulder abductors improves physical function of the upper limbs in subjects with SIS.

Eccentric training for shoulder abductors improves pain, function and isokinetic performance in subjects with shoulder impingement syndrome: a case series / Treino excêntrico para abdutores do ombro melhora dor, função e desempenho isocinético em sujeitos com síndrome do impacto: série de casos

BACKGROUND: Conservative treatments have been proposed for people with shoulder impingement syndrome (SIS), such as strengthening of the rotator cuff and scapular muscles and stretching of the soft tissues of the shoulder. However, there is a lack of studies analyzing the effectiveness of eccentric training in the treatment of SIS. OBJECTIVES: To evaluate the effects of eccentric training for shoulder abductors on pain, function, and isokinetic performance during concentric and eccentric abduction of the shoulder in subjects with SIS. METHODS: Twenty subjects (7 females, 34.2 SD 10.2 years, 1.7 SD 0.1 m, 78.0 SD 16.3 kg) with unilateral SIS completed the study protocol. Bilateral isokinetic eccentric training at 60º/s for shoulder abductors was performed for six consecutive weeks, twice a week, on alternate days. For each training day, three sets of 10 repetitions were performed with a 3-minute rest period between the sets for each side. The range of motion trained was 60° (ranging from 80° to 20°). The Disabilities of the Arm, Shoulder and Hand (DASH) questionnaire was used to evaluate functional status and symptoms of the upper limbs. Peak torque, total work and acceleration time were measured during concentric and eccentric abduction of the arm at 60º/s and 180º/s using an isokinetic dynamometer. RESULTS: DASH scores, peak torque, total work and acceleration time improved (p<0.05) after the period of intervention. CONCLUSIONS: This study suggests that isokinetic eccentric training for shoulder abductors improves physical function of the upper limbs in subjects with SIS.

CONTEXTUALIZAÇÃO: Tratamentos conservadores têm sido propostos para pessoas com síndrome do impacto (SI) do ombro, como fortalecimento do manguito rotador e dos músculos escapulares e alongamento dos tecidos moles do ombro. No entanto, são escassos os estudos que analisaram a eficácia do treinamento excêntrico no tratamento da SI. OBJETIVOS: Avaliar os efeitos do treinamento excêntrico para abdutores do ombro na dor, função e desempenho isocinético durante a abdução concêntrica e excêntrica do ombro em indivíduos com SI. MÉTODOS: Vinte indivíduos (sete mulheres, 34,2 DP 10,2 anos, 1,7 DP 0,1 m, 78,0 DP 16,3 kg) com SI unilateral completaram o protocolo do estudo. Realizou-se treinamento isocinético excêntrico bilateral a 60º/s para abdutores do ombro durante seis semanas consecutivas, duas vezes por semana, em dias alternados. Para cada dia de treinamento, foram realizadas três séries de dez repetições, com um período de descanso de 3 minutos entre as séries para cada lado. A amplitude de movimento treinada foi de 60° (de 80° a 20°). O questionário Disabilities of the Arm, Shoulder and Hand (DASH) foi utilizado para avaliar a função e os sintomas dos membros superiores. O pico de torque, o trabalho total e o tempo de aceleração foram avaliados durante a abdução concêntrica e excêntrica do braço a 60º/s e 180º/s por meio de um dinamômetro isocinético. RESULTADOS: As pontuações do DASH, o pico de torque, o trabalho total e o tempo de aceleração melhoraram (p<0,05) após o período de intervenção. CONCLUSÕES: Este estudo sugere que o treinamento isocinético excêntrico para abdutores do ombro melhora a função física dos membros superiores em indivíduos com SI.

Serum levels of IGF-1 are related to human skin characteristics including the conspicuousness of facial pores.

Conspicuous facial pores are one type of serious aesthetic defects for many women. However, the mechanism(s) that underlie the conspicuousness of facial pores remains unclear. We previously characterized the epidermal architecture around facial pores that correlates with the appearance of those pores in various ethnic groups including Japanese. The goal of this study was to evaluate the possible relationships between facial pore size, the severity of impairment of epidermal architecture around facial pores and sebum output levels to investigate the possible role of IGF-1 in the pathogenesis of conspicuous facial pores. The subjects consisted of 38 healthy Japanese women (aged 22-41 years). IGF-1 was measured using immunoradiometric assay. Surface replicas were collected to compare pore sizes of cheek skin and horizontal cross-section images of cheek skin were obtained non-invasively from the same subjects using in vivo confocal laser scanning microscopy and the severity of impairment of epidermal architecture around facial pores was determined. The skin surface lipids of each subject were collected from their cheeks and lipid classes were determined using gas chromatography/flame ionization detection. The serum level of IGF-1 correlated significantly with total pore area (R = 0.36, P < 0.05), with the severity of impairment of epidermal architecture around facial pores (R = 0.43, P < 0.05) and with sebum output levels (R = 0.41, P < 0.01). The sebum output levels correlated with total pore area (R = 0.32, P < 0.05). Our study found that serum levels of IGF-1 are correlated with facial skin characteristics including facial pore size and with the severity of impairment of epidermal architecture around facial pores.

[The role of suture material on healing of vascular anastomosis].

The effect of suture material on healing of vascular anastomosis was examined. Four types of vascular grafts, i.e., autogenous vein, preserved human umbilical cord vein, expanded polytetrafluoroethylene and double velour knitted Dacron, were implanted into the abdominal aorta of 78 adult mongrel dogs using two kinds of absorbable sutures (multifilament polyglycolic acid: PGA and monofilament polydioxanone: PDS) and a nonabsorbable suture (polypropylene: PP). The macroscopic findings and the histologic examinations showed that hyalinoid degeneration and calcification resulting from tissue ischemia due to tight and long lasting suture loops interfered with tissue healing at the PP-anastomotic site. On the other hand, fairly good healing of the anastomoses was observed with absorbable sutures because of the reduction of ischemia. The absorbable suture-anastomoses could tolerate systemic blood pressure within one month after implantation, and there was no anastomotic disruption at 1000 mmHg pressure in the bursting test 12 months after grafting. From Dec., 1984, 55 anastomoses in 34 bypass-operations employing autogenous vein grafts were performed using PGA and PDS in 28 cases clinically. There were no anastomotic complications. In conclusion, the synthetic absorbable suture material, especially in the form of monofilament, seems to be most suitable for suturing or anastomosing autogenous small vessels at present.