BackgroundChildren are believed to be the most vulnerable group to leprosy. Childhood leprosy reflects disease transmission in the community as well as the efficiency of ongoing disease control programmes. In Cuba, leprosy is not a national health problem; however, new childhood leprosy cases are diagnosed every year.ObjectiveWe summarise the experience of Cuba on childhood leprosy control over the past two decades.ResultsBetween 2000 and 2017, a total of 103 children in Cuba have been diagnosed with leprosy, showing that active transmission of cases remains in 13 of 15 provinces of Cuba. The majority of cases were multibacillary (66%), and 34% were paucibacillary cases. Clinically 60% of children have more than five lesions all over their body. Voluntary reporting was the principal method of case detection. The presence of familial and extrafamilial contact with leprosy cases may be a cause of concern, as it implies continuing transmission of the disease. Only four children had disabilities (one with grade 2 disabilities and three with grade 1 disabilities). A set of national investigations have been developed to intervene in a timely manner. Intervention strategies that combine clinical surveillance and laboratory test could be an option for early detection of childhood leprosy.ConclusionsEarly detection of cases due to effective health education campaigns, regular and complete treatment with MDT, and contact tracing may be important in reducing the burden of leprosy in the community.

RESUMEN En el presente artículo se identifican rasgos distintivos y esenciales en las transformaciones del sistema de salud cubano que han permitido la erradicación y disminución de las tasas de incidencia de algunas enfermedades transmisibles a niveles inferiores a 0,1 por 100 000 habitantes. Los resultados obtenidos son consecuencia de la importancia otorgada a la prevención y control de estas enfermedades, así como a los riesgos y daños potenciales. La estructura y funcionamiento del subsistema de higiene y epidemiología y sus interrelaciones con el resto del sistema a partir de los diferentes modelos de prestación de servicios han constituido escenarios de integración permanentes para la toma de decisiones. Se eliminaron enfermedades como la poliomielitis, paludismo, difteria, tosferina, rubéola, parotiditis, meningitis posparotiditis, sarampión, fiebre amarilla, cólera, formas graves de la tuberculosis, rabia humana transmitida por caninos, leishmaniasis, enfermedad de Chagas, la transmisión vertical del virus de inmunodeficiencia humana, sífilis congénita y formas clínicas como el tétanos neonatal y el síndrome de rubéola congénita. Se hace énfasis en algunas enfermedades transmisibles y, en especial, en la respuesta social desarrollada contra la tuberculosis, la lepra, el sida y las enfermedades transmitidas por vectores. Se demuestra que el contexto sanitario actual revela aún desafíos para la sostenibilidad de los logros alcanzados en el país. Garantizar el mantenimiento de la cobertura universal con acceso de la población cubana a los servicios de salud será siempre un principio de la salud pública cubana.

Introduction In 1993, Cuba achieved leprosy elimination according to the World Health Organization’s (WHO) indicator of less than one case per 10,000 population. Despite this achievement, detection of new cases occurs every year among all age groups including children. Detection of new cases in children reveals persistent transmission of the infection. Objective To describe the clinical and epidemiological features of leprosy in individuals younger than 15 years (childhood leprosy) reported to the Cuban National Leprosy Control Program (NLCP) between 2012 and 2019. Methods We conducted a retrospective descriptive study between 2012 and 2019 to assess the clinical and epidemiologic features of individuals under the age of 15 years with a confirmed diagnosis of leprosy reported to the NLCP. We reviewed the NLCP database and collected data to better define the total number of cases of leprosy in adults, children (younger than 15 years). We assessed socio-demographic variables (age, gender, and province of residence) as well as variables of clinical interest including operational classification and staging at diagnosis, bacillary index, grade of disability by WHO staging. Additionally, we evaluated epidemiological variables including passive versus active surveillance of cases, contact investigation focusing specifically in household transmission, and the degree of kinship as well as standing of the child within the focus of transmission when there were additional cases. Results We identified fifty children during the study period corresponding to 3% of the overall cases of leprosy comprising all age groups in Cuba. In the age group younger than 15 years, the majorities of cases was from the Granma province and most were between the ages of 10 and 14 years. Clinically, multibacillary/lepromatous forms were the most common type identified with positive bacillary index. The majority of children diagnosed with leprosy during our study period had a history of a relative with a confirmed diagnosis of leprosy. Conclusions Detection of cases of leprosy in individuals younger than 15 years of age in Cuba demonstrates ongoing transmission of M. leprae in specific geographic hotspots. Its frequency in the early adolescence, the predominant clinical forms, and the mode of detection associated with sources of suspected familiar infection demonstrated that there is a need for further efforts by the NLCP to conduct active surveillance activities among affected communities to identify cases of leprosy earlier with the goal of preventing further household and community transmission.

Von Willebrand factor (vWF) binds to platelets and mediates platelet adhesion to subendothelium to support haemostasis. Factor VIII concentrates containing vWF have been recommended for treatment of bleeding episodes in von Willebrand disease (vWD) types 2 and 3. Their clinical efficacy in normalizing FVIII coagulant levels, shortening the bleeding time, stopping or preventing clinical bleeding and safety have been tested. However, the basic mechanisms of their effects on haemostasis have not been fully characterized. We have analysed the ability of vWF present in an intermediate-purity factor VIII concentrate (Haemate-P, Centeon) to bind to platelets (ultrastructural studies) and to support platelet adhesion under flow conditions (perfusion studies). For this purpose, Haemate-P or cryoprecipitate was added to washed platelet suspensions, or to vWF-depleted reconstituted healthy anticoagulated blood. Immunoelectron microscopy (IEM) revealed vWF arrangements on platelet surfaces which have been exposed to mixtures of the vWF-rich concentrate plus ristocetin. vWF levels in perfusates were confirmed by determination of ristocetin co-factor and vWF-antigen. Baumgartner's perfusion method and computer-assisted morphometry were used to evaluate platelet adhesion of the perfusates onto everted rabbit aorta subendothelium under standardized conditions (shear rate, 800 s(-1) , 10 min, 37 °C). vWF-depleted perfusates showed 15.8% (SEM 1.7) total covered surface (CS) with platelets. An increase in CS resulted when 0.40 or 0.80 IU vWF mL(-1) from Haemate-P (30.1%, SEM 3.0, P<0.05; 39.4%, SEM 3.1, P<0.008, respectively) were added. A similar increase was observed when cryoprecipitate was added to perfusates (28.6%, SEM 2.4, P<0.05 for 0.40 IU vWF mL(-1) ; 27.2%, SEM 2.9, P<0.01 for 0.80 IU vWF mL(-1) ). IEM confirmed that vWF from concentrates binds to platelets. Furthermore, perfusion studies revealed that the fractionated vWF supports platelet adhesion, thus providing experimental evidence of the therapeutic benefits exerted by Haemate-P.

Even though it is generally held that cryoprecipitate (cryo) and fraction I-0 correct the prolonged bleeding time (BT) in patients with von Willebrand disease (VWD), perusal of reported data indicates that the correction is usually short lasting and often partial. We decided to do a controlled study of the relationship between the multimeric structure of von Willebrand factor (VWF) in 5 patients with severe VWD after infusion of three plasma concentrates : "wet" cryo, lyophilized (lyo) cryo, and fraction 1-0 given in random order. The dosage of concentrates was tailored to achieve post infusion levels of RiCof above the lower normal limit (50 U/dL) for at least 3 hours. The post-infusion BT values are shown in the table.These findings indicate that the attainment of a normal BT is the exception rather than the rule after infusion of three plasma fractions used for treatment of severe VWD. In all the concentrates the proportions of large VWF multimers, calculated by scanning the electrophoretic gels, were the same as in normal standard plasmas. An intact multimeric structure was recovered in post-infusion plasma of patients treated with wet cryo, whereas there was post infusion loss of large multimers after lyo and fraction I-O. In conclusion, an intact multimeric structure in post infusion plasmas is necessary but not sufficient to sustain a normal BT in VWD patients.