[Hemolytic uremic syndrome by Streptococcus pneumoniae]. / Síndrome hemolítico urémico asociado a neumonía neumocócica.

Hemolytic uremic síndrome (HUS) is an acute disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure, usually,but not always, associated with the prodrome of diarrhea. A 2-year-6-month old boy with pneumonía caused by Streptococcus pneumoniae developed the third day after admission oliguric acute renal failure, anemia, and thrombocytopenia. The patient needed veno-venous hemodiafiltration/ hemofiltration during ten days. He received five packed red blood cell, and one platelet transfusions, and was treated with plasma infusions during six days. Renal function was normal at discharge. HUS caused by Streptococcus pneumoniae has more morbility and mortality that classic HUS, although they usually recovery renal function. In those cases of atypical HUS, different from others, blood transfusions that contains IgM should be avoided,because they can increase antigen-antibody reaction and cellular damage. The low incidence of HUS by Streptococcus pneumoniae and the systemic infectious component difficult the early diagnosis. Taking into account the different transfusion approach in these cases, it is necessary to be aware for diagnosing HUS by Streptococcus pneumoniae in all patients with systemic infection and sudden renal function deterioration.

[Hypoacusis after the neonatal period. Situations requiring hearing assessment]. / Hipoacusia de detección posneonatal. Circunstancias que precisan evaluación auditiva.

INTRODUCTION: Because hearing plays a major role in language development, pediatric hypoacusis is especially damaging. The high frequency of hearing impairment in newborns and the need for an early diagnosis have led to the establishment of neonatal screening. Nevertheless, there are other situations which may compromise hearing quality in later stages and it is essential to identify them in order to be able to provide early and effective treatment. OBJECTIVES: To describe the most frequent reasons for referring patients for hearing evaluation to a third level hospital and to identify common situations that require hearing assessment among the pediatric population. PATIENTS AND METHODS: The clinical histories of 197 non-neonates evaluated for hypoacusis were reviewed. Clinical parameters and diagnosis were compared in patients with impaired and normal hearing. RESULTS: One hundred sixty-one patients had no previous known hypoacusis. The main reason for evaluation was suspicion by the family or child minder (53.4 %), followed by language underdevelopment. In the first examinations 78 children had hypoacusis (48.4 %), which was more frequently bilateral than unilateral. In 29.5 % of hypoacusic patients, the disease was related to recurrent otitis or adenoiditis and in 25.6 % it was genetic. The most frequent antecedent was deaf relatives in hypoacusic patients and abnormal phenotypes in children with normal hearing. Three patients with previous bacterial meningitis were studied and two of these had hypoacusis. CONCLUSIONS: Hypoacusic evaluation outside the context of newborn screening is mainly motivated by clinical suspicion of hypoacusis or language underdevelopment. Other situations such as recurrent otitis with effusion, syndromic phenotypic characteristics or bacterial meningitis are related to hearing problems and therefore require detailed evaluation.

Bovine surfactant therapy and retinopathy of prematurity.

We reviewed the results of three randomized clinical trials of prophylactic bovine surfactant therapy on babies under 30 weeks gestational age to assess the effects of this treatment on the frequency and severity of retinopathy of prematurity (ROP). Of the 119 babies who received surfactant treatment, 54 (45.4%) had ROP compared to 33 (47.1%) of the 70 babies in the control group. Stage 2 ROP or greater was noted in 10 (8.4%) babies in the treatment group and in 10 (14.3%) of the untreated group. Prophylactic bovine surfactant replacement therapy does not have a significant effect on the frequency of ROP in preterm babies. The severity of ROP also appears to be unaffected by surfactant.

The ocular manifestations of the Sturge-Weber syndrome.

Sturge-Weber syndrome (SWS) is a rare neuro-oculocutaneous disorder. The ocular component manifests as glaucoma and vascular malformations of the conjunctiva, episclera, choroid, and retina. The authors reviewed the records of and recalled for examination 51 consecutive patients with a diagnosis of SWS seen at The Hospital for Sick Children, Toronto, Ontario, Canada, from 1980 to 1991. Of the 51 patients, 36 (71%) had glaucoma, 26 experienced onset before 24 months of age and the remainder after 66 months of age, 35 (69%) had conjunctival or episcleral hemangiomas, and 28 (55%) had choroidal hemangiomas, which were bilateral in 12 patients. Other ocular manifestations (retinal vascular tortuosity, iris heterochromia, retinal detachment, and strabismus) also occurred. Thirty-four of 51 (67%) glaucomatous eyes had a final visual acuity of 20/40 or better, or central, steady, and maintained fixation in each eye.

Retinal findings after head trauma in infants and young children.

BACKGROUND: Many authorities believe that the finding of retinal hemorrhages in a child younger than 3 years of age with a history of head trauma, in the absence of an obvious cause for the injury, is pathognomonic of child abuse. To date, no studies have examined the prospective retinal examination of children who have had head trauma. The authors undertook such a study because the presence of retinal hemorrhage from any head trauma in children may have medicolegal diagnostic significance in differentiating accidental from nonaccidental trauma. METHODS: Seventy-nine children younger than 3 years of age, each of whom experienced head injury, underwent an ophthalmologic assessment, which included a dilated funduscopic examination. RESULTS: Seventy-five children sustained accidental head injuries and had normal funduscopic examinations. Three children had nonaccidental head injuries and all were found to have varying degrees of retinal hemorrhages. One child, with a normal fundus examination, had injuries that were of indeterminate cause. CONCLUSION: The finding of retinal hemorrhages in a child with a head injury suggests a nonaccidental cause.

Screening for fungal endophthalmitis in children at risk.

To evaluate the efficacy of screening ophthalmologic examinations in high-risk children, we reviewed the medical records for all patients hospitalized from 1985 through 1989 at The Hospital for Sick Children, Toronto, Ontario, who underwent ophthalmological consultation to rule out endogenous fungal endophthalmitis (n = 176). The patients were divided into groups: Group 1 (n = 47), those with deep-tissue fungal infection, and Group 2 (n = 129), those at risk for invasive fungal disease. Group 2 was subdivided further into two subgroups: Group 2a (n = 48), those with evidence of superficial fungal colonization (positive fungal culture) but no deep-tissue involvement, and Group 2b (n = 81), those with no evidence of fungal colonization (negative fungal culture). Of these 176 patients, 7 were diagnosed with endogenous fungal endophthalmitis: 6 from Group 1, 1 from Group 2a, and 0 from Group 2b. We found a significant association between the development of endogenous fungal endophthalmitis and the status of the fungal culture result (P less than .005). The odds ratio indicated the risk of endogenous fungal endophthalmitis in Group 1 patients with deep-tissue infection was at least 19 times that of Group 2 at-risk patients. The risk of endogenous fungal endophthalmitis in Group 1 patients was at least 7 times that of Group 2a colonized patients and 12 times that of Group 2b patients with no positive fungal culture. Our study confirms the necessity of careful dilated ophthalmoscopic examination in patients with invasive fungal disease and suggests screening for those at-risk patients with superficial fungal colonization.(ABSTRACT TRUNCATED AT 250 WORDS)

Corneal diameter, axial length, and intraocular pressure in premature infants.

PURPOSE: Seventy premature infants 25 to 37 weeks' postconceptional age were examined during their first week of life to determine the correlation of corneal diameter, axial length, and intraocular pressure with gestational age and birth weight. METHODS: Corneal diameter measurement was determined with corneal templates, total axial length with standardized A-scan ultrasound, and intraocular pressure with a Tonopen II tonometer. RESULTS: Corneal diameter and total axial length showed parallel linear increases from 6.2 mm to 9.0 mm and 12.6 mm to 16.2 mm, respectively; however, no significant correlation was found between intraocular pressure and gestational age or birth weight. The mean intraocular pressure was 10.3 mmHg (standard deviation, 3.5). CONCLUSION: Normative values are established for corneal diameter and total axial length as they relate to birth weight and gestational age, and a mean and standard deviation for intraocular pressure in the premature newborn. These values will aid the ophthalmologist in assessing ocular dimensions in premature infants.

Management of congenital dacryocystocoele.

Congenital dacryocystocoele occurs when the nasolacrimal drainage apparatus in the newborn has concomitant blocks at the level of the junction of the common canaliculus with the lacrimal sac and at the distal end of the nasolacrimal duct. This results in a typical pink or blue swelling in the region of the medial canthus. Spontaneous resolution is common, although dacryocystitis may supervene. Treatment should be conservative unless dacryocystitis occurs, or intranasal extension coexists. A series of seven consecutive cases is presented and a management plan for the neonate with congenital dacryocystocoele is proposed.

The surgical management of congenital lacrimal fistulae.

Congenital lacrimal fistulae are developmental anomalies of the lacrimal apparatus that are usually symptomatic, frequently causing epiphora and occasionally causing fistulitis or dacryocystitis. They may be associated with other abnormalities of the lacrimal system or with systemic anomalies. Complete excision alone, or in combination with nasolacrimal intubation and/or dacryocystorhinostomy is recommended for treatment.

Iris sector heterochromia as a marker for neural crest disease.

A 6-month-old female infant with biopsy-proved Hirschsprung's disease had associated sector heterochromia of the irides. The association between sector heterochromia and Hirschsprung's disease has been previously reported and both conditions have been ascribed to neural crest defects. Histologic characteristics of the ocular involvement have not previously been reported, to our knowledge. Histopathologic examination of the globes revealed decreased iris stroma, decreased pigmentation in the anterior stroma, and reduced numbers of pigment-producing cells in the affected areas. Both the ocular and gastrointestinal findings reflect abnormalities in tissues of neural crest origin.

Corneal transplantation in infants, children and young adults: experience of the Toronto Hospital for Sick Children, 1979-88.

Between 1979 and 1988, 85 penetrating keratoplasty procedures were performed in 54 patients aged 1 month to 18.2 years at the Hospital for Sick Children, Toronto. The minimum length of follow-up was 3 months. A clear transplant was obtained in 27 eyes: 7 of 16 eyes with Peter's anomaly, 0 of 8 eyes with congenital glaucoma, 2 of 5 eyes with herpes simplex keratitis, 6 of 8 eyes with corneal dystrophy and 12 of 17 eyes with traumatic corneal scars. The most recent visual acuity was best in the trauma and dystrophy groups and worst in the congenital glaucoma group. Visual acuity results were better in older children and were fair in younger children and those with postoperative complications. Although penetrating keratoplasty is more difficult in children than in adults, it has a reasonable chance of success. However, the poor outcome in the congenital glaucoma group indicates that the procedure is not warranted in such patients.

Pars plicata lensectomy/vitrectomy for developmental cataract extraction: surgical results.

Fifty-two cases of developmental cataracts extracted using the pars plicata lensectomy/vitrectomy technique were reviewed. Eyes with additional ocular anomaly other than microphthalmos were not included. No early complications were detected in a postoperative period ranging from 1.5 to 7 years with a median of 4 years, and late complications were limited to secondary membranes occurring in small eyes. Since secondary membranes occurred only in eyes in which the axial length was less than or equal to 17.4 mm and the corneal diameter was less than or equal to 9.5 mm, we suggest that eyes that are small by the absolute dimensions cited here are at greater risk of developing secondary membranes postoperatively. Small eyes by absolute dimensions should be distinguished from microphthalmic eyes, since the relative term microphthalmic is less predictive of risk of complication than are absolute dimensions. Linear Snellen acuity in 15 patients capable of response ranged as follows: 20/20-20/80 with a median of 20/40 in eyes with partial bilateral cataracts; 20/25-20/80 with a median of 20/50 in eyes with complete bilateral cataracts; 20/30-20/400 with a median of 20/200 in eyes with unilateral partial cataracts; and 20/60-CF with a median of 20/400 in eyes with complete unilateral cataracts. Mean patient ages at surgery were 3 months for those with bilateral complete cataracts, 5 months for those with unilateral complete cataracts, 18 months for those with bilateral partial cataracts, and 25 months for those with unilateral partial cataracts. The earliest possible removal of visually significant opacities must be combined with aggressive postoperative visual rehabilitation to obtain the best possible visual outcome.

Glaucoma as an early complication of Hurler's disease.

We report three cases of Hurler's disease in which glaucoma developed in early childhood. We draw attention to the fact that glaucoma may be a commonly unrecognised early complication of this condition.

Horner's syndrome in children.

Ten cases of Horner's syndrome with pharmacologic testing and computed tomography scans are described in patients up to age 8 years. The patients also were assessed for iris color and facial sweating. Classical preganglionic Horner's syndrome associated with brachial plexus birth injury was not identified in any case. Two patients who presented with ptosis had neuroblastoma. Two other children had undergone corrective cardiothoracic surgery. Two patients had major congenital abnormalities. In four patients, no cause of Horner's syndrome was determined.