1/10. Phakomatosis pigmentovascularis type IIIb associated with moyamoya disease.We diagnosed phakomatosis pigmentovascularis type IIIb in an 11-month-old baby who had a giant nevus spilus, a nevus flammeus, and moyamoya disease. Development of the patient was normal until 6 months of age when he developed a sudden onset of focal seizures and left hemiparesis. This patient represents the sixth case of phakomatosis pigmentovascularis type IIIb, including three cases in the Japanese literature, reported thus far. However, to our knowledge, this is the first case with an association to moyamoya disease.- - - - - - - - - - ranking = 1keywords = baby (Clic here for more details about this article) |
2/10. Koraxitrachitic syndrome: a syndromic form of self-healing collodion baby with residual dappled atrophy of the derma.We report on a child with a generalized skin disorder associated with other minor anomalies. At birth, the child presented as a collodion baby, with patchy erythema, generalized irregular dermal atrophy, alopecia, absent eyelashes and eyebrows, and conjunctival pannus. He also had hypertelorism, prominent nasal root, large mouth, micrognathia, brachydactyly, syndactyly involving all interdigital spaces, and camptodactyly of fingers III-V. The hyperkeratotic membrane thinned progressively, leaving a mottled reticulated skin atrophy, with patchy areas of yellowish hyperpigmentation and papyraceous areas. hair and nails were dystrophic. Mental development was borderline normal. The histological hallmarks of the skin manifestations combined orthokeratotic hyperkeratosis and marked atrophy of the dermis. The dermal extracellular matrix was immature, and factor xiii-a positive dendrocytes were rare and globular rather than dendritic. We frame as a hypothesis that the disease is due to or associated with a defect in maturation of a subset of dermal dendrocytes during fetal life. This entity may be designed as the koraxitrachitic syndrome (kappaomicronrhoalphaxi:grapnel- taurhoalphachiiotatauepsilonsigma: roughness) copyright 1999 Wiley-Liss, Inc.- - - - - - - - - - ranking = 5keywords = baby (Clic here for more details about this article) |
3/10. incontinentia pigmenti in identical twins with separate skin and neurological disorders.incontinentia pigmenti in female identical twins is reported. The first baby showed the typical pigmentation of incontinentia pigmenti, while the second baby had hydrocephalus (colpocephaly) without pigmentation. They were identical, with a rate of 99.9% in 18 blood-type studies. Virus was not detected and cytogenetic studies proved normal. Both showed peripheral eosinophilia. The individual expressions of incontinentia pigmenti in these identical twins were separated into cutaneous lesions and lesion of the central nervous system (intra-uterine hydrocephalus). Cutaneous lesions developed after birth. twins with incontinentia pigmenti are extremely rare and in this family showed different expressions of this disease in space and time.- - - - - - - - - - ranking = 2keywords = baby (Clic here for more details about this article) |
4/10. early diagnosis of the retinopathy of incontinentia pigmenti: successful treatment by cryotherapy.Prospective examination of the ocular fundi was carried out in the newborn female sibling of a girl with incontinentia pigmenti and bilateral retinopathy. At age 3 months the new baby's peripheral retina was found to be avascular in both eyes. At age 2 years progressive vascular proliferation at the watershed area between normal and abnormal retina led to bilateral vitreous haemorrhage. The new vessel tufts promptly regressed when the avascular area in each eye was treated by single-freeze cryotherapy and the blood in the vitreous absorbed. Cicatricial retinopathy was thereby prevented. Prospective retinal examination of newborn female siblings of children with incontinentia pigmenti is advised, with early cryotherapy recommended if progressive neovascularisation is detected.- - - - - - - - - - ranking = 1keywords = baby (Clic here for more details about this article) |
5/10. Green baby: a consequence of intrauterine exposure to Evan's blue dye.Evan's blue dye was accidentally injected into the fetus. This caused a bluish discoloration of the skin that gradually changed to a greenish color. The greenish color disappeared after 3 weeks and there were no other sequelae to this iatrogenic complication.- - - - - - - - - - ranking = 4keywords = baby (Clic here for more details about this article) |
6/10. Hyperbiliverdinemia in the bronze baby syndrome.The bronze baby syndrome is an unusual complication of phototherapy for hyperbilirubinemia in the neonate. The pigment or pigments responsible for the discoloration in this syndrome have not yet been identified. Suspected pigments include photodegradation products of bilirubin and copper-porphyrins. We present here the case of a neonate with bronze baby syndrome whose serum had increased spectral absorbance in the region of maximum absorbance for biliverdin. We suggest that biliverdin pigments may also contribute to the "bronze" color associated with this syndrome.- - - - - - - - - - ranking = 13938.71458363keywords = baby syndrome, baby (Clic here for more details about this article) |
7/10. Bronze baby syndrome. Report of a case.A newborn female infant suddenly developed a generalized gray-brown discoloration of her skin while receiving phototherapy for hyperbilirubinemia. The discoloration waned over the succeeding weeks. Her serum contained an unknown pigment that had a characteristic absorbance on spectrophotometry. The distinctive findings and some possible causes of the bronze baby syndrome are discussed. The findings are compared to other causes of dusky-hued skin in the neonate. The bronze baby syndrome is usually innocuous but can signal life-threatening disease.- - - - - - - - - - ranking = 13938.71458363keywords = baby syndrome, baby (Clic here for more details about this article) |
8/10. incontinentia pigmenti in a Black baby: a case report.This is a case report of a Black baby girl who presented at the age of 7 days with incontinentia pigmenti. While the classic 3 phases of inflammation, hyperkeratosis, and irregular pigmentation are the rule, this patient demonstrates an accepted variation, presenting with irregular pigmentation without having gone through the other phases. Ninety-five per cent of the described cases have been in females. A negative family history, as in this case, is not unusual.- - - - - - - - - - ranking = 5keywords = baby (Clic here for more details about this article) |
9/10. Low reserve albumin for binding of bilirubin in neonates with deficiency of bilirubin excretion and bronze baby syndrome.The plasma reserve albumin concentration for binding of bilirubin was found to be low in four newborn infants with deficiency of bilirubin excretion, of whom two had the bronze baby syndrome. Thus, the risk of bilirubin encephalopathy was increased. Also the ratio of binding fraction of albumin, i.e. unconjugated bilirubin plus reserve albumin, to total albumin was low. Possible causes of the low reserve albumin concentration and the ratio are discussed.- - - - - - - - - - ranking = 11615.595486358keywords = baby syndrome, baby (Clic here for more details about this article) |
10/10. Hypomelanosis of Ito associated with West syndrome: report of a case.Hypomelanosis of Ito (HI) is a rare neurocutaneous disorder characterized by hypopigmented macules along the Blaschko line and multiple abnormalities, including seizures, developmental retardation, ophthalmologic defects, and chromosome mosaicism. We report a four-month-old female baby with typical skin lesions on the right upper limb, chest, and scalp, noted at the age of one week. She also had a seizure disorder which manifested as neck flexion and hand gripping with series formation from the age of three months. Electroencephalogram (EEG) demonstrated modified hypsarrhythmia. The characteristic clinical and electroencephalographic features favored the diagnosis of West syndrome. magnetic resonance imaging revealed an absence of abnormal neuronal migration. The auditory brainstem evoked response, nerve conduction velocity, and ophthalmologic examinations were all within normal limits. The seizure responded to adrenocorticotropic hormone therapy on the 12th day of initial therapy, and the EEG became normal. However, another different pattern of seizures characterized by staring and lateral gaze in a right upward direction occurred two weeks later. carbamazepine and phenobarbital were added to the therapeutic regimen. She was regularly followed up at the outpatient department every two weeks after one-and-a-half months of hospitalization. Infantile spasms with evidence of hypsarrhythmias in the EEG recurred at seven months of age. Oral steroid therapy reduced seizure frequency by 80%. As far as we know, this is the first case report of HI combined with infantile spasms in taiwan.- - - - - - - - - - ranking = 1keywords = baby (Clic here for more details about this article) |
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