Evaluation of cases with infantile hemangioma requiring treatment.

AIM: Infantile hemangiomas are the most common benign vascular tumours in infants. In this study, we aimed to evaluate the effectiveness of propranolol therapy in patients with infantile hemangioma.

Prenatal diagnosis and treatment of intrahepatic arteriovenous fistulas: case reports and the literature review.

BACKGROUND: Congenital arteriovenous intrahepatic fistulas, which are hepatic hemangiomas and arteriovenous malformations (AVMs) are rare and they confused with each other. Knowledge of prenatal medical treatment of AVMs is insufficient. OBJECTIVES: First is to emphasize the distinction between hepatic hemangioma and AVMs. Second is discussion of the first case of hepatic AVM that responded well to steroid-propranolol treatment in the prenatal period. METHODS: Color Doppler ultrasonography, fetal and postnatal MR were used for diagnosis. RESULTS: The first case is a giant hepatic hemangioma diagnosed and progressively growing in the prenatal period and gradually shrinking in the postnatal period. The second case was hepatic AVM with no signs of heart failure during the prenatal period and postnatal right extended hepatectomy was performed as the anastomosis was enlarged and intraportal collateral vessels were developed. The third case is the first hepatic AVM which reaches a term that was prenatally diagnosed and responded to treatment with marked reduction. CONCLUSION: Color flow and pulse Doppler imaging have a key role in the prenatal diagnosis of arteriovenous fistulas. Intrahepatic AVM are abnormal intrahepatic vascular network formation primarily fed by the hepatic artery or its branches and drained by the hepatic venous system. This vascular region looks like a mass, but it does not contain a solid area, which allows the separation of hepatic AVMs from hepatic hemangiomas. Steroid and propranolol therapy should be considered in management.

A new clinical and dermoscopic monitoring of infantile hemangiomas treated with oral propranolol.

Oral propranolol (OP) demonstrated high efficacy and safety profile for treatment of critical infantile hemangiomas (IHs). Our aim was to assess the morphologic changes of IHs with standard and high-resolution video dermoscopy (HRVD) from baseline to 18 months either in presence or absence of OP therapy; to investigate if extended anamnestic perinatal data and clinical-dermoscopic characteristics of the IHs can correlate with therapeutic outcome. We enrolled 94 patients (112 IHs): 58 were treated with OP, 35 (42 IHs) for 6 months (group 1), and 23 (25 IHs) for 12-months (group 2); 36 (45 IHs) were followed-up. Clinical-dermoscopic examinations were performed every 3 months during therapy and follow-up. Among 67 treated IHs, superficial and deep IHs with homogenous clinical-dermoscopic aspect developed after the 2 weeks of life achieved the better outcome, stable at 9-month follow-up, independently form treatment duration. Under HRVD, glomerular vessels were prevalent at baseline; corckscrew, comma, and linear-irregular vessels were the prevalent pattern at 1, 3, and 6 months of therapy, respectively. At 12-month follow-up, adequate healing was achieved by 96% of IHs in group 2 and by 78% in group 1, showing dotted vessels. Persistent IHs displayed a reticulated aspect and linear irregular vessels, while arborizing vessels characterized relapsed IHs. A 12-month OP therapy can be considered for newborns presenting with nonhomogenous mixed IHs >3 cm on the perineal area/lower extremities. In conclusion, HRVD allows a real time monitoring of vascular changes in IHs treated with OP and can support physicians in identifying relapses before they become clinically evident.

[Infantile haemangioma: clinical and demographic characteristics, experiences in the treatment]. / Infantilis haemangioma: klinikai és demográfiai jellemzok, kezelési-gondozási tapasztalatok.

INTRODUCTION: Infantile haemangiomas are the most common vascular tumours of infancy. The vast majority of the lesions do not require dermatological treatment due to their unique clinical course and the high rate of spontaneous regression. Approximately 10-15% of the tumours result in severe complications and sequale, requiring special management and close follow-up. AIM: The aim of the present study was to assess the data of the patients treated with infantile haemangiomas, and to summarize the results of the therapy during 4.5 years of study period, in the Paediatric Dermatology Outpatient Clinic of the Department of Dermatology and Allergology, at the University of Szeged. METHOD: Demographic data of the infants (gender, gestational age and weight, perinatal history of the infant and medical history of mothers), exact date of the visits at Paediatric Dermatology Outpatient Clinic and tumour characteristics (number, subtype, anatomical localisation and complications) were analysed in details. Treatment modalities and therapy intervals, outcomes and the adverse events of the therapies were also discussed in the survey. RESULTS: During the study period, 96 infants with 163 infantile haemangiomas were observed. 54 patients required regular observations, while 42 infants required local or systemic beta-blocker therapy. All of the tumours treated with local or systemic therapy showed marked clinical regression; adverse effects were observed in only 6 cases. The gestational age and gestational weight of infants requiring beta-blocker therapy was significantly lower as compared to children needed only observation. CONCLUSIONS: Systemic propranolol is currently the first-line treatment modality for complicated infantile haemangiomas. Our results confirm the significant therapeutic efficacy of propranolol. Early introduction of the treatment is relevant; unfortunately a great proportion of patients are referred late to Paediatric Dermatology Centres. Orv Hetil. 2017; 158(39): 1535-1544.

Long-term effect of a chronic low-protein multideficient diet on the heart: Hypertension and heart failure in chronically malnourished young adult rats.

BACKGROUND: We investigated whether a chronic low-protein multideficient diet (BRD) from weaning turns on cardiovascular adaptive responses that could culminate in hypertension and heart failure. METHODS AND RESULTS: Systolic pressure (SP) and heart rate (HR) were determined in CTRL (normal diet) and BRD rats. Plasma albumin, plasma urea and urinary urea excretion decreased in BRD rats. In this group, echocardiography and the Langendorff technique showed: (i) increased HR and hypertension; (ii) decreased LVDP, dP/dtmax, dP/dtmin, cardiac output, ejection fraction, stroke volume and left ventricular diameter. BRD rats were less sensitive to isoproterenol (ISO) in LVDP and dP/dtmax, with unchanged dP/dtmin; Pressure-volume relationships indicated left-oriented shifts in LVDP, SP and DP, and decreased capacitance compared to CTRL. BRD rats had higher cardiac and lung indexes, accompanied by muscle atrophy and recent ventricular-infarcted areas, higher ventricular ß1-AR content, and decreased ß2-AR and α1-AR. Propranolol treatment gave similar ISO responses in both groups, disappearance of the infarcted regions and, except for ß2-AR, recovery of normal receptor expression. BRD rats had intense stimulation of plasma membrane Ca2+-ATPase (PMCA) activity, with increased Ca2+ affinity and inhibition of the sarco(endo)plasmic reticulum Ca2+-ATPase (SERCA). Ventricular phospholamban increased and Na+/Ca2+ exchanger decreased. PMCA activity correlated with an increase in its PKC-mediated phosphorylation, overlying a decrease in PKA-catalyzed phosphorylation. Propranolol normalized PKC and PKA activities with recovery of PMCA but not SERCA. CONCLUSION: BRD triggers sympathetic exacerbation and dysfunction in Ca2+ handling, accompanied by early onset of hypertension and left ventricle congestive heart failure.

Uso del propranolol en hemangioma parotídeo / Use of Propranolol in Parotid Hemangioma

El uso del propranolol es una alternativa terapéutica en los hemangiomas de la infancia. Los resultados que se describen en la literatura con su empleo lo colocan en las primeras líneas de elección en el tratamiento de esta enfermedad. Se reportó el caso de una lactante de cuatro meses de edad con hemangioma de la parótida izquierda, diagnosticada y tratada en el Hospital Pediátrico de Cienfuegos, que no tuvo una respuesta satisfactoria con los esteroides, y que con el propranolol presentó regresión completa de la lesión; se mantuvo el tratamiento por un año, no presentó ninguna complicación con su uso.

The use of the propranolol is a therapeutic alternative in the infantile hemangiomas. The results described in the literature regarding their use place this therapy in one of the first election lines in the treatment of this illness. An infant of four years of age with left parotid hemangioma was reported. The patient was diagnosed and treated in Cienfuegos Pediatric Hospital and did not have a satisfactory response with the steroids, and with the use of propranolol a complete regression of the lesion was observed, keeping the treatment for one year, not presenting any complication with its use.

Infantile hemangiomas: A review.

Infantile hemangiomas (IH) are the most common eyelid and orbital tumors of childhood. Although they are considered benign lesions that have a generally self-limited course, in the periocular region, they have the potential to cause amblyopia, strabismus, and severe disfigurement. The decision for treatment can be a source of anxiety for patients, parents, and physicians alike. There are numerous treatment modalities, including emerging therapies that may make treatment safer and more effective than ever before. This review discusses our current understanding of this disease, its management, and future therapies.