Congenital viral infections and the occurrence of septo-optic dysplasia, which is a combination of optic nerve hypoplasia, abnormal formation of structures along the midline of the brain, and pituitary hypofunction, support the biological plausibility of endocrine dysfunction in Zika-related microcephaly. In this case series we ascertained the presence and describe endocrine dysfunction in 30 children with severe Zika-related microcephaly from the MERG Pediatric Cohort, referred for endocrinological evaluation between February and August 2019. Of the 30 children, 97% had severe microcephaly. The average age at the endocrinological consultation was 41 months and 53% were female. The most frequently observed endocrine dysfunctions comprised short stature, hypothyroidism, obesity and variants early puberty. These dysfunctions occurred alone 57% or in combination 43%. We found optic nerve hypoplasia (6/21) and corpus callosum hypoplasia (20/21). Seizure crises were reported in 86% of the children. The most common—and clinically important—endocrine dysfunctions were pubertal dysfunctions, thyroid disease, growth impairment, and obesity. These dysfunctions require careful monitoring and signal the need for endocrinological evaluation in children with Zika-related microcephaly, in order to make early diagnoses and implement appropriate treatment when necessary.
The genitourinary tract was recently identified as a potential site of complications related to the congenital Zika syndrome (CZS). We provide the first report of a series of cryptorchidism cases in 3-year-old children with Zikarelated microcephaly who underwent consultations between October 2018 and April 2019 as part of the follow-up of the children cohort of the Microcephaly Epidemic Research Group, Pernambuco, Brazil. Of the 22 males examined, eight (36.4%) presented with cryptorchidism. Among 14 undescended testis cases, 11 (78.6%) could be palpated in the inguinal region. Seven of the eight children had severe microcephaly. Conventional risk factors for cryptorchidism were relatively infrequent in these children. We hypothesize that cryptorchidism is an additional manifestation of CZS present in children with severe microcephaly. As in our cases, for most of the children, the testes were located in the inguinal region, and the possible mechanisms for cryptorchidism were gubernaculum disturbance or cremasteric abnormality.
Purpose: To evaluate the erectile function in patients who underwent partial penectomy and identify factors associated with penile functional status. Materials and Methods: We identified patients who underwent partial penectomy due to penile cancer between 2009 and 2014. Clinical and pathological characteristics included patient age at the time of diagnosis, obesity, hypertension, dyslipidemia, diabetes, smoking, metabolic syndrome, Eastern Cooperative Oncology Group (ECOG) status, penile shaft length, tumor size, primary tumor stage (pT), clinical nodal status, and local recurrence. Erectile function was assessed prospectively with the International Index of Erectile Function (IIEF-5) at least 3 months after partial penectomy. Results: A total of 81 patients met analysis criteria. At the diagnosis, the median age was 62 years (range from 30 to 88). Median follow-up was 17 months (IQR 7-36). Of total patients, 37 (45%) had T2 or higher disease. Clinically positive nodes were present in 16 (20%) patients and seven (8.6%) developed local recurrence. Fifty patients (62%) had erectile dysfunction (ED) after partial penectomy, 30% had moderate or severe erectile dysfunction scores. Patients with ED versus without ED were similar in baseline characteristics except for age, penile shaft length, and presence of inguinal adenopathy (p <0.05). Multivariate analysis using logistic regression confirmed that older patients, shorter penile shaft length, and clinically positive lymph node were significantly associated with ED. Conclusion: Partial penectomy due to penile cancer provides adequate local control of the disease, however, proper counselling is important especially in relation to ED consequences. Preservation of penile length yields to more optimal erectile recovery.
This cohort profile aims to describe the ongoing follow-up of children in the Microcephaly Epidemic Research Group Paediatric Cohort (MERG–PC). The profile details the context and aims of the study, study population, methodology including assessments, and key results and publications to date. The children that make up MERG–PC were born in Recife or within 120 km of the city, in Pernambuco/Brazil, the epicentre of the microcephaly epidemic. MERG–PC includes children from four groups recruited at different stages of the ZIKV microcephaly epidemic in Pernambuco, i.e., the Outpatient Group (OG/n = 195), the Microcephaly Case–Control Study (MCCS/n = 80), the MERG Pregnant Women Cohort (MERG-PWC/n = 336), and the Control Group (CG/n = 100). We developed a comprehensive array of clinical, laboratory, and imaging assessments that were undertaken by a ‘task force’ of clinical specialists in a single day at 3, 6, 12, 18 months of age, and annually from 24 months. Children from MCCS and CG had their baseline assessment at birth and children from the other groups, at the first evaluation by the task force. The baseline cohort includes 711 children born between February 2015 and February 2019. Children’s characteristics at baseline, excluding CG, were as follows: 32.6% (184/565) had microcephaly, 47% (263/559) had at least one physical abnormality, 29.5% (160/543) had at least one neurological abnormality, and 46.2% (257/556) had at least one ophthalmological abnormality. This ongoing cohort has contributed to the understanding of the congenital Zika syndrome (CZS) spectrum. The cohort has provided descriptions of paediatric neurodevelopment and early epilepsy, including EEG patterns and treatment response, and information on the frequency and characteristics of oropharyngeal dysphagia; cryptorchidism and its surgical findings; endocrine dysfunction; and adenoid hypertrophy in children with Zika-related microcephaly. The study protocols and questionnaires were shared across Brazilian states to enable harmonization across the different studies investigating microcephaly and CZS, providing the opportunity for the Zika Brazilian Cohorts Consortium to be formed, uniting all the ZIKV clinical cohorts in Brazil.
Background Complications in the urinary tract related to congenital Zika syndrome have recently been reported. One complication, cryptorchidism, has been reported by the Microcephaly Epidemic Research Group/MERG, in Pernambuco/Brazil. The present article describes for the first time the surgical findings in a case series of boys with Zika-related microcephaly and cryptorchidism, who underwent surgical testicular exploration as a contribution to better understand the possible mechanisms involved in gonads formation and descent. Methods A total of 7 children (11 testicular units), aged 3 to 4 years, were submitted to inguinal or scrotal orchidopexy for the treatment of palpable cryptorchidism between August 2019 and January 2020. Characteristics of the gonads and its annexes related to appendixes, testis-epididymis dissociation, gubernacular insertion, and associated hydroceles and/or hernias were described. Measures in centimetres were taken for volume calculate. Results We found a low prevalence of testicular and epididymal appendix (66.7%), a high prevalence of testis-epididymis dissociation (55.6%), low mean testicular volume for their ages (lower for older boys) and ectopic gubernacular insertion in all cases. There was no evidence of associated hydroceles and/or hernias in any case. No surgical complication was registered or reported, and all explored gonads were properly placed in the scrotal sac. Conclusions We herein describe the surgical findings of these children's orchidopexies and discuss the possible mechanisms of viral action in embryogenesis and postnatal growth and development of the testes and annexes. These children need to be followed over time due to the higher risk of testicular atrophy and malignancy. Surgical timing seems to be relevant to avoid loss of testicular volume.
RACIONAL: A deiscência aponeurótica do abdômen após intervenções cirúrgicas responde por índices de morbi-mortalidade em torno de 9% a 49% e apresenta incidência aproximada de 0,5% a 3%. OBJETIVO: Identificar os fatores de risco mais prevalentes e elaborar aspecto epidemiológico do perfil dos pacientes susceptíveis à esta complicação. MÉTODOS: Foram estudados no período de março a agosto de 2005 15 pacientes que estiveram internados e que apresentaram deiscência aponeurótica abdominal, provenientes da emergência ou admitidos para procedimentos eletivos. Foram pesquisados o sexo, peso e IMC, albumina sérica, linfócitos, causa do atendimento cirúrgico, co-morbidades existentes, terapias associadas, caráter da operação, incisão e fios utilizados, tempo de pós-operatório, achados cirúrgicos relacionados e total de procedimentos no sítio operatório. RESULTADOS: Identificou-se a desnutrição e infecção do sítio cirúrgico como os principais fatores de risco, e o perfil do paciente com esta complicação foi: sexo masculino, entre 25 e 45 anos, atendido de urgência ou emergência com quadro de abdômen agudo não-traumático, desnutrido. A infecção do sítio cirúrgico, ocorreu principalmente na primeira semana de pós-operatório. CONCLUSÃO: A desnutrição e a presença de infecção cirúrgica foram os fatores de risco que mais influenciaram na ocorrência da deiscência aponeurótica.
Aims: Describing the urodynamic parameters of children aged 3 to 5 years with microcephaly related to congenital Zika syndrome and verifying the association with clinical, imaging and neurological characteristics. Methods: From October 2018 to March 2020, children with Zika-related microcephaly underwent urological, ultrasonographic and urodynamic evaluation. In selected cases, complementary exams such as urethrocystography and scintigraphy were performed. The children also underwent a complete neurological evaluation. To compare frequency between groups, we used Pearson’s chi-squared test or Fisher’s exact test. Results: This study evaluated 40 children, of whom 85% were 4 years old, and all had abnormalities on the urodynamic study, with low bladder capacity (92.5%) and detrusor overactivity (77.5%) as the most frequent findings. Only three children had ultrasound abnormalities, but no child had cystographic or scintigraphic abnormalities, and the postvoid residual volume was normal in 80% of cases. In spite of a frequency of 67.5% of intestinal constipation, there was no record of febrile urinary tract infection after the first year of life. All children presented severe microcephaly and at least one neurological abnormality in addition to microcephaly. The homogeneity of the children in relation to microcephaly severity and neurological abnormalities limited the study of the association with the urodynamic parameters. Conclusions: Urodynamic abnormalities in children aged 3 to 5 years with Zika-related microcephaly do not seem to characterize a neurogenic bladder with immediate risks for the upper urinary tract. The satisfactory bladder emptying suggests that the voiding pattern is reflex.
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