Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Wildervanck syndrome i.e. cervico (Klippel-Feil anomalad) -oculo (Duane-Stilling-Turk phenomenon with bilateral abducens palsy)-acoustic (deafness) is a rare syndrome. We report here 4 cases diagnosed as Wildervanck syndrome and analyse their findings. One patient had an an atrial septal defect. Such association of congenital heart disease with Wildervanck syndrome has not been reported previously.
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PMID:Wildervanck syndrome (cervico-oculo-acoustic syndrome). 130 89

Among the TORCH agents, the occurrence of rubella and human T-lymphotropic virus type 1 (HTLV-1) in Japan were studied. Rubella epidemics occurred throughout Japan from 1964 to 1969 and from 1975 to 1979. Low prevalences of CRS were observed in northeastern Japan, and high prevalences in southwestern Japan, with the highest in Okinawa. These conditions could be explained by the lower rate of rubella H1 antibody in the female population of southwestern Japan. Time of maternal rubella was in the gestational age interval from 26 to 57 days for cataract, from 25 to 62 days for heart disease and from 16 to 131 days for deafness. HTLV-1 is the causative agent of adult T-cell leukemia. Main route of transmission of this virus is mother-to-child transmission, through breast milk. Among the 311 mother-child pairs in Okinawa, 65 mothers (20.9%) and 10 children (3.2%) were seropositive for HTLV-1. Ten (15.4%) of the 65 seropositive mothers had seropositive children. These children had acquired their HTLV-1 antibodies by the age of 3 years. A significant difference existed between the prevalence rate of HTLV-1 antibodies in mothers and children.
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PMID:Perinatal viral infections. 139 25

A retrospective study of pneumococcal meningitis was carried out. This study included 22 cases of this illness that occurred in 17 pediatric patients in Cantabria between 1977 and 1990, inclusive. Three children suffered from recurrent meningitis. The age range of the patients was 0.3-14 years, with a mean age of 4 years. Of these cases, 77.4% occurred in the winter or spring. In 14 cases of meningitis (63.6%), corresponding to 9 patients, underlying pathology was observed: cranial fracture, occipital dermoid cyst, splenectomy, congenital cardiopathy, epilepsy and gastroduodenal "situs inversus". The isolated pneumococci were found to be resistant to penicillin and chloramphenicol in 4 cases. One patient suffered from septic shock. The outcome was complete recovery in 19 cases (86.4%), recovery with sequelae (deafness and epilepsy, respectively) in 2 cases (9.1%) and death in another 2 cases. CSF protein levels elevated above 150 mg/dl and the absence of glucose in the CSF were found to be the most useful analytical indicators of the seriousness of the illness.
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PMID:[Pneumococcal meningitis in the infantile population of Cantabria]. 149 25

The association between catastrophic airway events and developmental delay was examined in patients with CHARGE (coloboma, heart disease, atresia choanae, retarded growth and/or development, genital hypoplasia, and ear anomalies and/or deafness) association. A retrospective chart analysis was performed from The Children's Hospital in Boston, Mass. Sixteen patients were identified with CHARGE association. Nine patients had at least one respiratory arrest, and 7 had no airway difficulties. Some degree of developmental delay was seen in 14 patients, but was most severe in those patients who suffered a respiratory arrest. We conclude that children with CHARGE association have a propensity for airway instability and that cerebral hypoxia contributed to the developmental delay in some of our patients. We recommend early tracheotomy rather than early choanal atresia repair in these patients to protect the central nervous system.
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PMID:Airway complications in CHARGE association. 169 49

We report on a male infant with congenital hypothyroidism owing to athyreosis occurring with the CHARGE association (bilateral papillary coloboma, congenital heart disease, dysmorphic ears, sensorineural deafness, psychomotor retardation, cryptorchidism, facial palsy, and vesicoureteral reflux). The coexistence of these two disorders has not been described previously.
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PMID:The CHARGE association and athyreosis. 205 59

Waardenburg syndrome type I is transmitted as an autosomal dominant trait. The main features are dystopia canthorum, partial pigmentary disorder and perceptive deafness. Osteo-articular and intestinal malformations may be observed. In an affected family, the unusual combination of Waardenburg syndrome and severe congenital heart disease has been observed in a child.
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PMID:[Waardenburg's syndrome and severe cyanotic cardiopathy]. 207 26

One hundred three patients with ischemic heart disease (IHD) were compared with 29 patients with organic heart disease and normal coronary arteries (OHD) and with a control group of 101 patients free of heart disease and matched for age and sex. Twelve patients in the control group, 4 in the OHD group, and 34 patients in the IHD group were found to have hearing loss (HL) of different degrees, cause, and duration (P = 0.0003). Of the HL-IHD group, 19 of the 34 patients had no underlying etiologic factor compared with 4 of the 12 patients in the control group (P = 0.0005); age was not an important factor. Multiple logistic regression analysis suggests that the probability of a patient with HL of unknown etiology to have IHD is eight times greater than in individuals with normal hearing. In the HL groups (12 controls and 34 with IHD), there were no significant differences in sex, hypertension, obesity, or smoking, but there was a lower incidence of diabetes and a higher incidence of family history in the HL-IHD group than in the HL-control group. Two patients in the HL-IHD group had families with many members affected by both deafness and IHD, suggesting a heredofamilial disease. HL always preceded the clinical manifestation of IHD and appears to be an important "early marker" of a vascular or generalized arteriosclerotic process.
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PMID:Hearing loss and ischemic heart disease. 320 30

Coenzyme Q10 (CoQ10) has already been favorably evaluated in the clinical treatment of heart disease. In the otolaryngological field, it has been reported that CoQ10 is effective in promoting recovery from acute sudden deafness. However, the pharmakinetics of CoQ10 in the inner ear is not yet clarified. The present study focuses upon the pharmacokinetics of CoQ10 using guinea pigs with acute sensorineural hearing loss artificially induced by hypoxia conditions. The respiration of the animals was controlled in an artificial respirator while the ABR, ECG and blood pressure were monitored. Repeated hypoxia caused a gradual disappearance of the ABR. After the experiments, the animals were sacrificed and brain and inner ear were examined by histological and histochemical methods as well as by SEM and TEM. The results indicated that CoQ10 is effective in promoting recovery from damage in auditory hairs as well as preventing respiratory metabolic impairment of hair cell due to hypoxia.
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PMID:Pharmacokinetics of coenzyme Q10 in recovery of acute sensorineural hearing loss due to hypoxia. 324 40

Accurate congenital rubella syndrome surveillance in Japan is of great interest because of the hypothesis that the Japanese rubella virus was less virulent than the strains prevalent in Europe and the Americas 20 years ago. A nationwide survey of deaf children with a history of maternal rubella in special schools for the deaf in Japan yielded a total of 365 deaf school children with congenital rubella syndrome born between 1963 and 1982. The overall average prevalence was 0.31 case per 100,000 population, and the incidence was 0.1-6.1 cases per 100,000 annual live births in Japan. If one considers the decrease in the number of deaf children attending special schools for the deaf because of the recent adoption of the policy of integrating deaf children into ordinary schools, the number of cases of congenital rubella syndrome might actually be much greater than that revealed by this survey and much greater than the 88 cases previously reported in Japan. Among the 365 cases who had deafness, 8.2% were found to have cataract (with and without congenital heart disease), and 11.0% were found to be complicated by congenital heart disease (but without cataract).
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PMID:Incidence of congenital rubella syndrome in Japan (1965-1985). A nationwide survey of the number of deaf children with history of maternal rubella attending special schools for the deaf in Japan. 376 13

In 1814, George Maton, first recognized that a mild illness characterized by rash, adenopathy, and little or no fever was a discrete entity. Henry Veale, in 1866, named the disease rubella. The illness attracted little attention until 1942, when Norman Gregg noticed that first-trimester maternal rubella caused serious birth defects. The full spectrum and impact of rubella embryopathy remained unclarified until rubella virus was isolated in tissue culture in 1962 by two independent groups: Parkman, Buescher, and Artenstein; and Neva and Weller. Using the new tools of the virus laboratory, many investigators concentrated on the consequences of a severe rubella epidemic in 1964, which affected approximately 1% of pregnancies. Newly recognized transient manifestations of congenital rubella infection (CRI) include neonatal thrombocytopenic purpura, hepatitis, bone lesions, and meningoencephalitis and late-emerging sequelae such as diabetes mellitus and progressive rubella panencephalitis added to the cataract, heart disease, mental retardation, and deafness previously defined as due to CRI. Sharp contrasts were documented between the patterns of virus excretion and immune response of postnatal vs. congenital rubella. Licensure and widespread distribution of attenuated rubella virus vaccines in 1969 have prevented epidemic rubella. Pockets of illness remain, even in the United States. Continued effort will be required to eliminate the rubella problem.
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PMID:The history and medical consequences of rubella. 389 Jan 5


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