Social and professional consequences of COVID-19 lockdown in patients with multiple sclerosis from two very different populations. / Consecuencias sociolaborales del confinamiento por la COVID-19 en pacientes con esclerosis múltiple en dos poblaciones muy diferentes.

The global lockdown measures implemented due to the COVID-19 pandemic have nearly always had negative consequences for patients with multiple sclerosis (MS). OBJECTIVE: We compared the social and professional effects of confinement on patients with MS in 2 very different populations, from Spain and China. METHODS: Questionnaires were administered to a group of patients with MS who consulted at the MS unit of Vithas hospital (DINAC Foundation) in Seville, and patients with MS attended in several provinces of China in April 2020, with the aim of analysing the differences and similarities between populations in the social and professional effects of confinement. To this end, a database was created and subsequently analysed. RESULTS: The Chinese population includes a higher proportion of younger patients and no differences were identified regarding sex. Most of the variables studied behaved in the same way in both patient populations. Spanish patients presented a lesser impact (30.7%) on their socio-economic situation than Chinese patients (44%) (P < .05). There were no significant differences between populations in the remaining variables. Social networks were widely used in the majority of patients from both populations. CONCLUSIONS: The social and professional consequences of the pandemic were very similar in both groups; the use of social networks and family support was also similar. Spanish patients seem to present greater economic stability, perhaps due to the social support they receive.

Cost of management of invasive growth hormone-secreting macroadenoma.

BACKGROUND: At the time of diagnosis, macroadenomas represent 60-80% of GH secreting adenomas, of which 25-30% are invasive macroadenomas. These aggressive tumors have the worst surgical success rates in terms of cure, and often need several therapeutic approaches in order to control disease status. Acromegalic patients are subject to increased mortality and important health resource consumption related to their associated co-morbidities, in addition to the costs that are related to diagnosis itself and initial treatment of the disease. OBJECTIVE: Assessment of the cost of initial management and outcome of acromegalic patients with invasive pituitary adenomas. STUDY DESIGN: Retrospective and observational study of review of records. SETTING: Two tertiary hospitals. PATIENTS: 11 consecutive patients between 18 and 80 yr old diagnosed with acromegaly due to an invasive pituitary macroadenoma. INTERVENTION: Collection of data of biochemical and radiological tests, specialist visits, hospitalisation, surgery, pharmacological and radiotherapy treatment at diagnosis and over 4 yr of follow-up after initial treatment. Costs were evaluated using the data of the Centre for Health Economics and Social Policy Studies and the Official College of Pharmacists of Spain. MAIN OUTCOME MEASURE: Global and patient/yr follow-up costs of illness. RESULTS: The mean costs for acromegaly for the period of follow-up ranged from 7,072 to 9,874 euro/patient/yr, for biochemically non-controlled (no.=6) and controlled patients (no.=5) respectively. The most important cost in the perioperative period was for admission in the intensive care unit. After surgery, SS analogues were the principal contributors to the economic burden. CONCLUSION: In this paper we have for the first time presented a pharmacoeconomic study of GH secreting invasive macroadenoma. The poor prognosis of our cohort of patients and the higher rate of controlled patients and normal IGF-I levels warrant the employment of multiple therapeutic options. The cost associated with this treatment in this complex disease of low prevalence is not excessive and can be supported by healthcare services.

Consecuencias sociolaborales del confinamiento por la COVID-19 en pacientes con esclerosis múltiple en dos poblaciones muy diferentes / Socio-labor consequences of confinement by COVID-19 in patients with multiple sclerosis in two very different populations

El confinamiento debido a la pandemia de la COVID-19, realizado a nivel mundial, ha tenido consecuencias casi siempre negativas en los pacientes con esclerosis múltiple (EM). OBJETIVO: Hemos comparado el efecto sociolaboral que el confinamiento ha podido tener en pacientes con EM de dos poblaciones tan diferentes como son España y China. MÉTODO: Se elaboraron unos cuestionarios a lo que respondieron un grupo de pacientes de EM que son revisados en la unidad de EM del hospital Vithas (Fundación DINAC) en Sevilla, y pacientes con EM atendidos en varias provincias de China durante el mes de abril de 2020, con el objetivo de analizar las diferencias y similitudes del efecto sociolaboral entre ambas poblaciones. Para llevar a cabo este análisis se creó una base de datos que se analizó posteriormente. RESULTADOS: La población china tiene una mayor proporción de pacientes más jóvenes y no hay diferencia respecto al género. La mayoría de las variables estudiadas se comportaron de igual forma en los pacientes con EM tanto españoles como chinos. Los pacientes españoles presentaron menos impacto (30,7%) en su situación socioeconómica que los chinos (44%), p < 0,05. No hubo diferencias importantes en el resto de las variables entre las dos poblaciones. Las redes sociales fueron muy utilizadas en la mayoría de los enfermos de ambas poblaciones. CONCLUSIONES: Los pacientes con EM padecen de forma muy similar las consecuencias de la pandemia en su situación sociolaboral y utilizan de forma parecida las redes sociales y el apoyo de la familia. Los pacientes españoles disfrutan de más estabilidad económica, probablemente gracias al apoyo social que reciben

The confinement due to the global COVID-19 pandemic has almost had negative consequences in patients with multiple sclerosis (MS). OBJECTIVE: We wanted to compare the socio-labor effect of confinement in two populations as different as Spain and China, in patients with MS. METHOD: Questionnaires were applied to a group of MS patients who have been reviewed in the MS unit of the Vithas hospital (DINAC Foundation) in Seville, and MS patients attended in various provinces of China during the month of April 2020, with the aim of analyzing the differences and similarities of the socio-labor effect between both populations. To carry out this analysis, a database was created and subsequently analyzed. RESULTS: The Chinese population has a higher proportion of younger patients and there is no difference regarding gender. Most of the variables studied behaved the same way in both Spanish and Chinese MS patients. Spanish patients had less impact (30.7%) on their socio-economic situation than Chinese (44%), P < .05. There were no important differences in the rest of the variables between the two populations. Social networks were widely used in the majority of patients in both populations. CONCLUSIONS: MS patients suffer in a very similar way from the consequences of the pandemic on their socio-labor situation and similarly use social networks and family support. Spanish patients seem to have more economic stability, which may be due to social economic support

The role of diabetes duration, pubertal development and metabolic control in growth in children with type 1 diabetes mellitus.

OBJECTIVE: To investigate whether pubertal development, duration of type 1 diabetes mellitus (DM1), or metabolic control play some role in the anomalies in growth observed in diabetic children. PATIENTS: We conducted a prospective evaluation of 83 patients (37 female, 46 male) who were followed from the onset of DM1 at the prepubertal stage until they reached final height. All patients were treated with a conventional regimen of insulin. METHODS: Height SDS, weight SDS, BMI SDS, duration of DM1 in years, and values of HbA1c were the study variables. RESULTS: In prepubertal (P1) girls (data for the initial vs the intermediate evaluations): weight SDS was -0.14 +/- 0.19 vs 0.11 +/- 0.20, p = ns; BMI SDS -0.25 +/- 0.15 vs 0.01 +/- 0.13, p = ns. In postpubertal (P3) girls, weight SDS was 0.49 +/- 0.2 vs 1.2 +/- 0.32, p <0.01; BMI SDS 0.09 +/- 0.16 vs 1.03 +/- 0.24, p <0.01, whereas in P1 boys, height SDS was 0.16 +/- 0.30 vs -0.20 +/- 0.27, p <0.05; and in P3 boys: 0.09 +/- 0.21 vs -0.28 +/- 0.26, p <0.05. Thus pubertal development influenced changes observed in girls with DM1, but did not do so in boys. The anomalies described in children with DM1 were observed from the third year of DM1 duration in both girls and boys. We did not observe any correlation between HbA1c values with height SDS, weight SDS or BMI SDS. CONCLUSIONS: The anomalies in growth observed in girls with DM1 are related to pubertal development, but this is not the case in boys. Alterations in children with DM1 were found from the third year of DM1 duration. Furthermore, the present data also indicate that the degree of metabolic control observed in our patients treated with modern but conventional regimen did not play a major role in the anomalies observed.

Factors associated with mortality of patients with myxoedema coma: prospective study in 11 cases treated in a single institution.

This study was carried out to investigate the clinical and biochemical factors which might be of importance in predicting the outcome of patients with myxoedema coma. Eleven patients (ten female) aged 68.1+/-19.5 years attended our institution over a period of 18 years. Glasgow and APACHE II scores and serum free thyroxine and TSH were measured in all the patients on entry. Patients were selected at random to be treated with two different regimens of l-thyroxine. Four patients died with the mortality rate being 36.4%. The patients in coma at entry had significantly higher mortality rates than those with minor degrees of consciousness (75% vs 14.3% respectively, P=0.04). The surviving patients had significantly higher Glasgow scores than those who died (11.85+/-2.3 vs 5.25+/-2.2 respectively, P<0.001). Comparison of the mean values of APACHE II scores between the surviving group and those who died was significantly different (18.0+/-2.08 vs 31.5+/-2.08 respectively, P<0.0001). The degree of consciousness, the Glasgow score and the severity of the illness measured by APACHE II score on entry were the main factors that determined the post-treatment outcome of patients with myxoedema coma.

Novel point mutation in exon 10 of the RET proto-oncogene in a family with medullary thyroid carcinoma.

Medullary thyroid carcinoma (MTC) may occur sporadically or as part of the autosomal dominant multiple endocrine neoplasia type 2 (MEN 2). Three hereditary forms of MEN 2 have been identified: MEN 2A, MEN 2B, and familial MTC (FMTC). Missense germ-line mutations in the RET proto-oncogene have been identified as cause of these endocrine diseases. Mutations are found in exons 10 and 11 in MEN 2A and FMTC families and in a small number of families in exons 13, 14, and 15. Although a strong correlation between codon mutations and phenotypes has been described, not all the expected cystein codon mutations have been found. Therefore, the more mutations are found, the better it is possible to establish phenotype-genotype correlations. We report on a novel RET mutation at codon 611 in a family with MTC without other clinical manifestations and of rather benign course.

Monitoring of treatment success in patients with acromegaly: the value of serum insulin-like growth factor binding protein-3 and serum leptin measurements in comparison to plasma insulin-like growth factor I determination.

The aim of the present study was to determine the value of serum insulin-like growth factor binding protein-3 (IGFBP-3) and serum leptin measurements in comparison with plasma insulin-like growth factor I (IGF-I) measurements as indicators of treatment success in patients with acromegaly. Thirty-five acromegaly patients, 25 female and 10 male, divided into groups of patients with postadenomectomy "active" acromegaly (n = 20) and patients with postadenomectomy "controlled" acromegaly (n = 15), and 44 healthy volunteers sex- and age-matched with the acromegaly patients were included in the present study. We comparatively analyzed plasma IGF-I, serum IGFBP-3, and serum leptin levels in the aforementioned groups. Because serum leptin has sex dimorphism, the groups were divided into sexes when leptin was evaluated. As expected, the patients with active acromegaly had significantly higher mean values of plasma IGF-I and serum IGFBP-3 and lower mean values of serum leptin (only in women) than the control group. However, individual evaluation showed that 1 of 20, 9 of 20, and many patients with postadenomectomy active acromegaly patients had values that overlapped values of control subjects for plasma standard deviation score (SDS)-IGF-I, serum SDS-IGFBP-3, and sex-adjusted serum leptin, respectively. Application of the receiver operating characteristic (ROC) curves method shows that plasma IGF-I measurement has the best discriminatory power to differentiate patients with postsurgical active acromegaly from healthy people. Its area under the curve (AUC) was 0.95, with a sensitivity and specificity of 86% and 94%, respectively. Its positive and negative likelihood ratios were 14 and 0.15. Serum IGFBP-3 has certain discriminatory power, its AUC being 0.89, with a sensitivity and specificity of 83% and 77%. Its positive and negative likelihood ratios were 3.6 and 0.22. Serum leptin, both in women and in men, has a poor performance with sensitivity and specificity of 53% and 50% for women and 55% and 56% for men and positive and negative likelihood ratios of 1.06 and 0.94 for women and 1.26 and 0.8 for men. Application of the ROC curves method and the determination of positive and negative likelihood ratios in comparative evaluation of serum IGFBP-3 and serum leptin with plasma IGF-I as indicators of treatment success in acromegalic patients showed that neither serum IGFBP-3 nor serum leptin determinations have accuracy better than or similar to that of plasma IGF-I for monitoring treatment success in acromegaly patients. Serum IGFBP-3 is accurate but does not increase accuracy for age-adjusted plasma IGF-I, whereas determination of serum leptin level has no value in monitoring these patients.

Comparative study of insulin-like growth factor-I (IGF-I) and IGF-binding protein-3 (IGFBP-3) level and IGF-I/IGFBP-3 ratio measurements and their relationship with an index of clinical activity in the management of patients with acromegaly.

To evaluate the utility of measuring the serum insulin-like growth factor (IGF)-binding protein-3 (IGFBP-3) level and the IGF-I/IGFBP-3 ratio in the management of acromegalic patients, we comparatively studied the basal concentration of the aforementioned parameters with determination of plasma IGF-I levels and an index of clinical activity of acromegaly in 16 newly diagnosed acromegalic patients (aged 34 to 64 years) before and after hypophysectomy. After adenomectomy, 10 patients remained with "active" disease and six were "cured." Twenty-nine healthy sex- and age-matched volunteers were also studied. Comparison of individual values between untreated acromegalic patients and control subjects showed that none of the patients had overlapping values for IGF-I, whereas five of 16 and three of 16 patients had overlapping values for serum IGFBP-3 and IGF-I/IGFBP-3 ratio, respectively. When we compared the study parameters between the patients who remained with active disease after adenomectomy and the controls, two of 10 had overlapping values for IGF-I, but six of 10 and five of 10 had overlapping values for serum IGFBP-3 and IGF-I/IGFBP-3 ratio, respectively. Moreover, comparison of these parameters between cured and active patients after hypophysectomy showed that none had overlapping values for IGF-I, whereas three of six and one of six had overlapping values for serum IGFBP-3 and IGF-I/IGFBP-3 ratio, respectively. All biochemical variables studied showed significant relationships with an index of clinical activity of disease. In conclusion, our results seem to indicate that among determinations of plasma IGF-I, serum IGFBP-3, and the ratio IGF-I/IGFBP-3 in the evaluation of acromegalic patients, measurement of the plasma IGF-I level has the most discriminative value in the management of these patients.

Primary adrenocortical nodular dysplasia presented as adrenal adenoma by functional scintigraphy.

This is a report of a case of a 12-year-old boy with Cushing's syndrome in which high doses of dexamethasone failed to suppress glucocorticoid secretion. Neither CT nor MRI (morphologic studies) revealed any adrenal abnormality. Functional images with NP-59 (I-131 beta-iodomethyl-norcholesterol) clearly revealed intense unilateral uptake in the right adrenal gland. A right adrenalectomy was performed, and the pathologic diagnosis was primary adrenocortical nodular dysplasia. One year later, the cushingoid signs had virtually disappeared and urinary steroid excretion and serum cortisol levels were within the normal range. Nevertheless, primary adrenocortical nodular dysplasia is essentially a bilateral disease. In this case, a remission in the adrenocortical hyperfunction was observed. Although there was confusion in the etiological diagnosis, functional scintigraphy correctly indicated the status of adrenal function.

[Association between post-initial remission and HLA phenotype in Type I diabetics]. / Asociación entre remisión postinicial y fenotipo HLA en diabéticos tipo I.

Our goal was to estimate the occurrence rate of the post-initial remission and its relation with HLA phenotype in type I diabetics (DMI). We studied 50 type I diabetics, 22 women and 28 men, with an average age at the onset of the disease to 15.8 +/- years (range 3-30 years). All patients were conventionally treated with insulin therapy, diet and regular exercise. Six (12%) of the diabetics presented a complete remission during 57.3 +/- 46 weeks, whereas fifteen (30%) patients presented partial remission during 20.1 +/- weeks. No significant differences were observed with regard to age at the onset of the disease, sex, BMI, initial ketoacidosis and stage of gonadal development between those diabetics presenting remission and those who did not presented so. We observed a higher incidence of the HLA-DR4 antigen among diabetics with remission (complete and partial, 61.9%), compared with patients without remission (20%, p less than 0.5). In conclusion, our results support the findings suggesting the presence of a clinical-immunological heterogenicity in DMI, genetically determined and linked to the HLA system.

Riedel's thyroiditis associated with hypothyroidism and hypoparathyroidism.

Hypoparathyroidism secondary to Riedel's thyroiditis is rare, only 2 previous cases having been reported. We present the case of a 36 year old woman with Riedel's thyroiditis which developed into hypothyroidism and hypoparathyroidism.

MR imaging findings in granular cell tumor of the neurohypophysis: a difficult preoperative diagnosis.

Granular cell tumor is a rare neoplasm arising within the neurohypophysis. We describe the MR imaging findings in two symptomatic patients. In one patient with history of panhypopituitarism, MR images showed a large sellar and suprasellar mass. The other patient presented with acute loss of vision in her left eye, and MR images showed a suprasellar mass with compression of the optic chiasm.

Congenital atresia of the foramen of Monro.

Two cases of congenital atresia of the foramen of Monro are described, one 8 days old and the other 2 months old. Both are associated with a parieto-occipital paraventricular diverticulum and spontaneous ventriculostomy with the passing of the cerebrospinal fluid to the posterior fossa and the formation of a cyst in the supracerebella region which compressed and displaced the structures of the posterior fossa.

Spontaneous ventriculostomy and ventricular diverticulum.

Four cases of ventricular diverticulum are presented, 2 with spontaneous ventriculostomy. In 2 patients, the cause was monoventricular hydrocephalus through congenital stenosis of the foramen of Monro, with paraventricular diverticula and spontaneous ventriculostomy. Both patients had a cystic space in the quadrigeminal and supracerebellar cistern. In one patient during pneumoencephalography, the ventricular diverticulum filled via the ventriculostomy. The other 2 patients had congenital stenosis of the Sylvian aqueduct with the formation of a large supratentoria cyst connecting with the posterior part of the third ventricule.

[Radiological manifestations of Meckel's diverticulum (author's transl)]. / Nuestra experiencia en el diagnóstico radiológico del divertículo de Meckel.

Multiple clinical and radiological manifestations of Meckel's diverticulum are commented, presenting four cases with acute abdominal manifestations and one with chronic symptomatology, which were selected from a series of nineteen cases that were surgically verified. The authors realize a critique of the radiological techniques in the literature of the suspected cases of said entity.

[Cavernous hemangioma of the liver (author's transl)]. / Hemangioma cavernoso hepático.

A three-month old asymptomatic infant was incidentally found to have an abdominal mass. Through standard radiological and vascular procedures it was defined as being of hepatic origin, vascular etiology and of benign prognosis. Differences among cavernous hemangioma, hepatoma, metastasis and hemangio-endothelioma are summarized. The importance of angiography is emphasized as an essential procedure previous to the surgical evaluation and therapy.