scholarly journals Novel facial characteristics in congenital rubella syndrome: a study of 115 cases in a cardiac hospital of Bangladesh

2020 ◽  
Vol 4 (1) ◽  
pp. e000860
Author(s):  
Nurun Nahar Fatema Begum

ObjectiveTo establish novel facial characteristics unique to congenital rubella syndrome (CRS) as prediagnostic criteria to supplement disease diagnosis in patients with or without a history of maternal rubella infection.DesignAn analysis of 115 CRS case series (2018–2020) based on the presence of any of the triad features.SettingOutpatient department of a tertiary care referral cardiac hospital in Dhaka, Bangladesh.ParticipantsIn total, 115 participants (53.1% men) were enrolled. Participants underwent echocardiography if they presented with suspected cardiac symptoms along with deafness, cataract or microcephaly.Main outcome measuresAge, sex and socioeconomic status of the participants; history of maternal vaccination and infection; facial characteristics unique to CRS (triangular face, prominent nose, wide forehead and a whorl on either side of the anterior hairline) named ‘rubella facies’ and frequency of systemic involvements in CRS.ResultsThe median patient age was 2 years. The income of 50.4% of the participating families was <US$1500. Further, 32 mothers (27.8%) were infected with rubella during the first trimester of pregnancy, 15 (13.0%) during the second trimester and 3 (2.6%) during the third trimester. The remainder (65.2%) recalled no history of infection during pregnancy. Rubella facies presented as a triangular-shaped face in 95 (82.6%) cases, a broad forehead in 88 (76.5%) and a prominent nose in 75 (65.2%). A rubella whorl was present on the right or left side of the anterior hairline in 80% and 18.2% of cases, respectively. IgG and IgM antibodies were present in 91.3% and 8.6% of children, respectively. Cataract, deafness, microcephaly, and congenital heart disease were detected in 53.0%, 75.6%, 68.6% and 98.2% of cases, respectively.ConclusionsRubella facies, a set of unique facial characteristics, can support early CRS diagnosis and treatment and may supplement the existing CRS triad.

2020 ◽  
Vol 20 (3) ◽  
pp. 284-290
Author(s):  
Jocelyn Chan ◽  
Yue Wu ◽  
James Wood ◽  
Mohammad Muhit ◽  
Mohammed K. Mahmood ◽  
...  

Background and Objectives: Congenital Rubella Syndrome (CRS) is the leading cause of vaccine-preventable congenital anomalies. Comprehensive country-level data on the burden of CRS in low and middle-income countries, such as Bangladesh, are scarce. This information is essential for assessing the impact of rubella vaccination programs. We aim to systematically review the literature on the epidemiology of CRS and estimate the burden of CRS in Bangladesh. Methods: We conducted a systematic review of existing literature and transmission modelling of seroprevalence studies to estimate the pre-vaccine period burden of CRS in Bangladesh. OVID Medline (1948 – 23 November 2016) and OVID EMBASE (1974 – 23 November 2016) were searched using a combination of the database-specific controlled vocabulary and free text terms. We used an age-stratified deterministic model to estimate the pre-vaccination burden of CRS in Bangladesh. Findings: Ten articles were identified, published between 2000 and 2014, including seven crosssectional studies, two case series and one analytical case-control study. Rubella seropositivity ranged from 47.0% to 86.0% among all age population. Rubella sero–positivity increased with age. Rubella seropositivity among women of childbearing age was 81.0% overall. The estimated incidence of CRS was 0·99 per 1,000 live births, which corresponds to approximately 3,292 CRS cases annually in Bangladesh. Conclusion: The estimated burden of CRS in Bangladesh during the pre-vaccination period was high. This will provide important baseline information to assess the impact and cost-effectiveness of routine rubella immunisation, introduced in 2012 in Bangladesh.


2010 ◽  
Vol 124 (9) ◽  
pp. 999-1002 ◽  
Author(s):  
N Guinand ◽  
T Just ◽  
N W Stow ◽  
H Cao Van ◽  
B N Landis

AbstractIntroduction:Chorda tympani injury as a complication of middle-ear surgery has been extensively studied with regard to its effects upon taste. However, the chorda tympani also carries parasympathetic fibres to the salivary glands of the oral cavity. To date, little has been reported about the effect of chorda tympani section upon salivary function.Setting:Tertiary care centre.Material and methods:We report a case series of three patients with bilateral chorda tympani lesions. Chorda tympani function was assessed using ‘taste strips’ and unstimulated sialometry. A careful history of oral symptoms was taken.Results:All patients showed transient or permanent bilateral ageusia of the anterior two-thirds of the tongue, and a decreased resting salivary flow rate. In addition, all patients suffered from transient or persistent, distressing xerostomia.Conclusion:Taste disorders may occur after middle-ear surgery but they are mostly transient, even when the chorda tympani nerves are sectioned bilaterally. In contrast, bilateral chorda tympani lesions may lead to severe, persistent and distressing xerostomia. Based on this neglected aspect of chorda tympani function, we emphasise the importance of preserving the chorda tympani whenever possible.


2012 ◽  
Vol 2012 ◽  
pp. 1-2 ◽  
Author(s):  
Yezenash Ayalew ◽  
Fazlihakim Khattak

A 25-year-old Bangladeshi lady presented to neurology with a three-month history of involuntary movements of her right arm, associated with loss of power. There was progression to the right leg, and she subsequently developed episodes of slurred speech and blurred vision. At the time of presentation, she was 12 weeks pregnant and the symptoms were reported to have started at conception. Past medical history was unremarkable apart from one first trimester miscarriage and there was no significant family history suggestive of a hereditary neurological condition. MRI of the head revealed no abnormalities but serology showed positive antinuclear antibodies (ANAs) at a titre of 1/400. Further investigations revealed strongly positive anticardiolipin antibodies (>120) and positive lupus anticoagulant antibodies. The patient had a second miscarriage at 19 weeks gestation strengthening the possibility that the chorea was related to antiphospholipid antibody syndrome and she was started on a reducing dose of Prednisolone 40 mg daily and aspirin 300 mg daily. Six months later, she had complete resolution of neurological symptoms. There are several reports of chorea as a feature of antiphospholipid syndrome, but no clear consensus on underlying pathophysiology.


Author(s):  
Vadlakonda Sruthi ◽  
Annaladasu Narendra

Background: Tramadol use has been increasing in the adult and pediatric population. Practitioners must be alert because Tramadol misuse can lead to severe intoxication in which respiratory failure and seizures are frequent. Overdoses can lead to death. We report 47 pediatric cases with history of accidental tramadol exposure in children.Methods: An observational, retrospective, single center case -series of children with a history of accidental tramadol exposure in children admitted in pediatric intensive care unit of tertiary care center, Niloufer Hospital (Osmania Medical College) Hyderabad, Telangana India.Results: Of 47 children, 22 (47%) are male and 25 (53%) were female. At presentation 11 (23%) had loss of consciousness, 14 (29%) seizures, 17 (36%) hypotonia was noted. Pupils were miotic in 22 (47%) mydriatic in 2 (4.2%) normal in rest of children. Hemodynamic instability noted in 13 (27.6%). Serotonin syndrome (tachycardia, hyperthermia, hypertension, hyper reflex, clonus) was noted on 5 (10.6%) children. Respiratory depression was seen in 4 (8%) children who needed ventilatory support. Antidote Naloxone was given in 7 children. No adverse reaction was noted with Naloxone. All 47 children were successfully discharged.Conclusions: Overdoses can lead to death and practitioners must be alert because of the increasing use of tramadol in the adult and pediatric population. The handling of the tramadol should be explained to parents and general population and naloxone could be efficient when opioid toxicity signs are present.


2021 ◽  
Author(s):  
Meenakshi Wadhwani

Congenital facial palsy is a very rare entity with an incidence of 2 per 1000 live births. It can be congenital, traumatic associated with birth trauma in the form of forceps delivery or developmental. We present the case of a 1-year-old girl who presented to the eye department of our tertiary care hospital with a deviation of face to the right side since birth along with watering of left eye and difficulty in taking feeds. There was a history of forceps-assisted vaginal delivery; a provisional diagnosis of congenital facial palsy was done with the probable cause of trauma.


PEDIATRICS ◽  
1982 ◽  
Vol 69 (4) ◽  
pp. 495-496
Author(s):  
Theodore W. AvRuskin ◽  
Mario Brakin ◽  
Christina Juan

Thyroid disorders have been reported following congenital rubella: four cases of thyroiditis1-4 and two patients with thyrotoxicosis5,6 are known. We report a patient with childhood myxedema and congenital rubella, and indicate that myxedema may occur as a sequela to rubella. This combination of disorders should be added to thyroiditis,5 hypopituitarism7 and adrenal insufficiency4 in the spectrum of possible late diabetes mellitus complications of the congenital rubella syndrome. Patients with congenital rubella should be tested periodically for these endocrine deficiencies. CASE REPORT An 8[unknown]-year-old white girl was the product of a seven-month gestation complicated by maternal rubella in the first trimester. Birth weight was 1 kg.


2020 ◽  
Vol 48 (8) ◽  
pp. 030006052093606
Author(s):  
Li-Fang Shen ◽  
Ya-Lian Chen ◽  
Shui-Hong Zhou

Tumors of the parapharyngeal space (PPS) are rare, most originate from salivary and neurogenic tissues, and most are benign. However, there are some rarer masses in the PPS, with just a few published reports in the literature worldwide, and we may not consider them in the differential diagnosis of PPS neoplasms. We report three cases of rare masses in the PPS: Warthin’s tumor, branchial cleft cyst, and carcinoma ex pleomorphic adenoma. The three patients were admitted to our department with complaints of painless swelling in the lower side of the right face or a long history of snoring; diagnoses were confirmed histopathologically. An endoscopy-assisted transoral approach was used that allowed wide visibility for safe resection and resulted in a short hospitalization time and good functional and cosmetic outcomes. All patients have been followed to the current time, and there have been no recurrences. The transoral endoscopy-assisted approach appears to be safe, effective, and less invasive for excision of masses in the PPS.


Author(s):  
Anthony R. Mawson ◽  
Ashley M. Croft

Rubella is a systemic virus infection that is usually mild. It can, however, cause severe birth defects known as the congenital rubella syndrome (CRS) when infection occurs early in pregnancy. As many as 8%–13% of children with CRS developed autism during the rubella epidemic of the 1960s compared to the background rate of about 1 new case per 5000 children. Rubella infection and CRS are now rare in the U.S. and in Europe due to widespread vaccination. However, autism rates have risen dramatically in recent decades to about 3% of children today, with many cases appearing after a period of normal development (‘regressive autism’). Evidence is reviewed here suggesting that the signs and symptoms of rubella may be due to alterations in the hepatic metabolism of vitamin A (retinoids), precipitated by the acute phase of the infection. The infection causes mild liver dysfunction and the spillage of stored vitamin A compounds into the circulation, resulting in an endogenous form of hypervitaminosis A. Given that vitamin A is a known teratogen, it is suggested that rubella infection occurring in the early weeks of pregnancy causes CRS through maternal liver dysfunction and exposure of the developing fetus to excessive vitamin A. On this view, the multiple manifestations of CRS and associated autism represent endogenous forms of hypervitaminosis A. It is further proposed that regressive autism results primarily from post-natal influences of a liver-damaging nature and exposure to excess vitamin A, inducing CRS-like features as a function of vitamin A toxicity, but without the associated dysmorphogenesis. A number of environmental factors are discussed that may plausibly be candidates for this role, and suggestions are offered for testing the model. The model also suggests a number of measures that may be effective both in reducing the risk of fetal CRS in women who acquire rubella in their first trimester and in reversing or minimizing regressive autism among children in whom the diagnosis is suspected or confirmed.


2009 ◽  
Vol 140 (5) ◽  
pp. 692-696 ◽  
Author(s):  
Brandon Isaacson ◽  
Timothy Booth ◽  
Joe W. Kutz ◽  
Kenneth H. Lee ◽  
Peter S. Roland

Objective: To determine the accuracy of preoperative MRI in predicting cochlear obstruction in pediatric patients with a history of bacterial meningitis. Methods: A case series with chart review was performed at a tertiary care multidisciplinary cochlear implant program. Forty-five children with hearing loss that resulted from bacterial meningitis were implanted from 1991 to 2006. Twenty-five children had preoperative MRI with high-resolution axial T2-weighted images to assess for cochlear patency. Results: Seventeen of 25 patients (68%) had surgical evidence of cochlear obstruction. Six patients (37.5%) required circummodiolar drill-outs, and one patient (6.25%) underwent placement of a double array cochlear implant. The nine remaining patients (56%) with cochlear obstruction required removal of fibrous tissue or drilling of the inferior basal turn, but did not require manipulation of the ascending basal turn to achieve full electrode insertion. The sensitivity, specificity, and positive and negative predictive value of MRI predicting intraoperative cochlear obstruction with 95 percent confidence intervals was 94.1 percent (71–99), 87.5 percent (47–99), 94.1 percent (71–99) and 87.5 percent (47–99), respectively. Conclusion: Preoperative high-resolution T2 MRI may be useful in predicting cochlear obstruction in patients with a prior history of bacterial meningitis.


2012 ◽  
Vol 45 (01) ◽  
pp. 115-120 ◽  
Author(s):  
Jyoti Dvivedi ◽  
Sanjay Dvivedi

ABSTRACT Objectives: To study the cleft lip and cleft palate in the poverty stricken Sub-Himalayan Garhwal region of India, being a commonly seen congenital abnormality and scarcity of studies about the demography of cleft in this region. Design: A prospective cohort observational case series was performed on 4657 cleft patients at a Tertiary care Hospital in Dehradun, India, over a period of 5 years. Outcome measures: The authors investigated the differences between age and sex with cleft status and family history of clefts, birth order, religion, socioeconomic status, parent literacy, source of information for treatment, haematological investigations showing the status of infection and coagulation in such children and satisfaction after treatment. Results: Seventy-two percent parents of cleft lip patients were illiterate, and only 8% were graduates, the majority of patients were from the low socioeconomic class. The siblings of 1.1% of the cleft patients had similar deformity. Anemia was seen in 83.16% cases which was commonly microcytic hypochromic type and eosinophilia was seen in 25.50% of cases. In the coagulation profile, International Nationalized Ratio was found to be raised in 52.12%. Almost 95% of the families were fully satisfied by the treatment and results. Conclusion: This study will provide baseline information on the status of these less privileged cleft patients in this mountainous region for future reference to health workers.


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