Cases reported "Hyaline Membrane Disease"

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1/12. cytomegalovirus endolabyrinthitis.

    A premature male infant, who died 22 days after birth with hyaline membrane disease, was found to have had cytomegalic inclusion disease at autopsy. Histopathologic examination of the temporal bones showed cytomegalovirus (CMV) infection of the entire endolabyrinth without involvement of the neural and sensory structures. These findings support the thesis that late gestational or perinatal fetal CMV infection results in an endolymphatic labyrinthitis. We hypothesize that blood-borne virus passes from the stria vascularis into the endolymphatic spaces and infects the nonneurosensory epithelium. This pattern of infection differs from the perilabyrinthitis of human varicellazoster and experimentally produced mouse CMV.
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2/12. Neonatal renal failure: a complication of maternal antihypertensive therapy.

    Persistent anuria was diagnosed in a neonate born to a mother whose pregnancy was complicated by severe hypertension and systemic lupus erythematosus. Severe maternal hypertension necessitated the use of a battery of antihypertensive medications that included enalapril, an angiotensin converting enzyme inhibitor. The role of enalapril in neonatal renal failure is discussed.
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3/12. Digestive tract and renal small vessel hyalinosis, idiopathic nonarteriosclerotic intracerebral calcifications, retinal ischemic syndrome, and phenotypic abnormalities. A new familial syndrome.

    A new familial syndrome that affected 3 of 7 siblings is described. All 3 patients were young women with a very peculiar phenotype, poikilodermia and hair greying, and idiopathic nonarteriosclerotic cerebral calcifications. Pathological studies demonstrated a marked and progressive hyalinosis involving capillaries and often arterioles and small veins of the digestive tract, kidneys, and calcified areas of the brain. Using electron microscopy, we found that the hyalin substance in the intestinal capillaries consisted of several concentric layers of basal membrane-like deposits within a finely granular fluffy material. Huge deposits of this material were present in the subepithelial and mesangial spaces of the kidneys. endothelial cells and, in the kidneys, mesangial cells were markedly abnormal, and a true mesangiolysis pattern was present in 2 patients. The clinical and biologic expression of these vascular changes was variable. diarrhea, rectal bleeding, malabsorption, and protein-losing enteropathy were the main and lethal clinical problems in the proband. hypertension appeared in the early stage of a second pregnancy in 1 sister, and mild proteinuria was found in all 3 affected patients. Peripheral retinal ischemic syndrome and chorioretinal scars were found in the ocular fundi of both affected sisters of the proband. A subarachnoid hemorrhage, due to a right sylvian aneurism, also occurred in both sisters and was lethal in 1 sister. None of the known causes of distal vessel hyalinosis could be ascertained.
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4/12. A case report of maternal death associated with betamimetics and betamethasone administration in premature labor.

    A fatal case of pulmonary edema is reported after prolonged treatment with beta-mimetics during pregnancy for threatened premature labor. The mother had received betamethasone in order to enhance fetal lung maturity. Myocardial failure occurred 5 days after discontinuation of betamimetics. The potential toxic effects of beta-adrenergic agents and their association with corticosteroids are discussed. Caution is recommended when high doses of betamimetics are to be delivered to prevent premature labor. No patient should be treated unless her cardiac condition is normal. Cardiovascular evaluation should be regularly performed during the course of treatment. No patient should be discharged after treatment without a normal cardiovascular check-up.
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5/12. Pulmonary venoocclusive disease in pregnancy.

    A 23-year-old woman in her 27th week of gestation presented with clinical findings of progressive pulmonary hypertension. After cardiac catheterization she went into labor and was delivered by cesarean section. She died shortly thereafter from right heart failure. Pulmonary venoocclusive disease was found at autopsy. Hemodynamic changes during pregnancy, labor, delivery, and the postpartum period may have contributed to her deterioration and death. This is the first description of pulmonary venoocclusive disease in pregnancy.
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6/12. Acute polyhydramnios recurrent in successive pregnancies. Management with multiple amniocenteses.

    A patient with acute polyhydramnios in two successive pregnancies is described. In both this case and the only previously reported similar one, management by frequent transabdominal removal of relatively small amniotic fluid volumes was associated with prolongation of pregnancy and a living infant, suggesting that multiple amniocenteses can improve the otherwise hopeless prognosis associated with acute polyhydramnois.
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7/12. Congenital cytomegalovirus infection as a result of nonprimary cytomegalovirus disease in a mother with acquired immunodeficiency syndrome.

    A pregnant woman with acquired immunodeficiency syndrome had nonprimary cytomegalovirus (CMV) viremia and died of complications from pneumocystis carinii pneumonia and CMV sinusitis and pneumonitis. A boy was delivered by cesarean section at 34 weeks of gestation as the mother's health deteriorated and fetal distress developed. The infant died soon after delivery of interstitial pneumonitis and hyaline membrane disease with invasive CMV disease that affected the kidneys, adrenal glands, and placenta; the CMV strains from the mother and neonate were identical.
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8/12. Avoidance of red blood cell transfusion in an extremely preterm infant given recombinant human erythropoietin therapy.

    To avoid red blood cell (RBC) transfusions, recombinant human erythropoietin (rHuEPO) was given to an infant born at a gestation of 26 weeks and a birthweight of 830 g to parents who were jehovah's witnesses. The infant had hyaline membrane disease and required 52 days of assisted ventilation and 19 days of oxygen therapy. He received theophylline therapy for 61 days for recurrent apnoea and bradycardia. He developed bilateral intraventricular haemorrhage (IVH) and left-sided periventricular leucomalacia (PVL). Intravenous rHuEPO was started on day 1 at 200 U/kg per day for 1 month followed by subcutaneous rHuEPO 400 U/kg three times a week for 6 more weeks, supplemented with vitamin e, folic acid and iron. blood sampling was kept to a minimum and non-invasive blood-gas monitoring was used consistently. Consequently, the estimated cumulative volume of blood loss from sampling was only 21 mL during his hospital stay. His haemoglobin (Hb) was 150 g/L at birth and this fell to below 100 g/L from day 25 onwards. His lowest leucocyte count was 3.6 x 10(9)/L. He was discharged on day 83 with a Hb of 95 g/L, Hct of 29%, reticulocyte count of 2.8% and weight of 2400 g. At a postnatal age of 3 months, he had a Hb of 113 g/L. At 6 months, investigations showed: Hb 121 g/L, haematocrit 33%, reticulocyte 1% and a weight of 4.4 kg. He was readmitted to hospital once for an episode of vomiting and follow up to date showed developmental delay.(ABSTRACT TRUNCATED AT 250 WORDS)
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9/12. Surfactant protein B deficiency: antenatal diagnosis and prospective treatment with surfactant replacement.

    An infant with a family history of congenital alveolar proteinosis associated with surfactant protein B (SP-B) deficiency was identified when SP-B was not detected in amniotic fluid obtained at 37, 38, and 40 weeks of gestation. Surfactant replacement with commercially available preparations that contained SP-B was begun soon after delivery. Progressive respiratory failure developed despite continued surfactant replacement, corticosteroid therapy, and extracorporeal membrane oxygenation. The infant died at 54 days of age while awaiting lung transplantation. Surfactant extracted from amniotic fluid, bronchoalveolar lavage fluid, and lung tissue had no phosphatidylglycerol; surface tension was 24 dynes/cm (normal, < 10 dynes/cm) and did not decrease with in vitro addition of exogenous SP-B. Pulmonary vascular permeability measured with positron emission tomography was twice normal. At autopsy the alveolar proteinosis pattern was less prominent than that seen in affected siblings. Immunoreactivity of SP-B was absent in type II cells, but numerous foreign body granulomas with central immunoreactivity for SP-B and surfactant protein c were present. We conclude that exogenous surfactant replacement did not normalize surfactant composition, activity, or pulmonary vascular permeability. These findings suggest that endogenous SP-B synthesis is necessary for mature surfactant metabolism and function.
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10/12. A 354-gram infant: 48 month follow-up.

    Extreme prematurity (birth weight less than 500 g) with intact survival is very uncommon. This paper describes a preterm infant with birth weight of 354 g who is now 4 years old and developmentally normal. Intervention and management decisions in such a low birth weight group produce an ethical enigma. survival of newborns with a birth weight less than 400 g is unusual. Decisions regarding intervention and care for extremely low birth weight infants impact on financial, emotional, and medical responsibilities of family members and health care professionals. It has been increasingly difficult to set guidelines or policies regarding resuscitation of "micropremies" based on weight and gestational age.
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