Cases reported "Infant, Newborn, Diseases"

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1/24. "Ground-glass" hepatocytes.

    "Ground-glass" hepatocytes are liver cells which have eosinophilic granular, glassy cytoplasm on light microscopy. This appearance corresponds to a proliferated smooth endoplasmic reticulum ultrastructure. These changes may be drug-induced or associated with hepatitis b antigenaemia, particularly in carriers. In the latter case, hepatitis b antigen may be demonstrated in the liver cell cytoplasm, with a modified orcein stain, by immunofluorescence, electron microscopy and immuno-electron microscopy. Two patients are described in whom "ground-glass" hepatocytes were noted on liver biopsy. One patient had been treated with high doses of phenobarbitone, the other is a hepatitis b antigen carrier as was demonstrated by the modified orcein stain.
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ranking = 1
keywords = hepatitis
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2/24. liver dysfunction and histologic abnormalities in neonatal hypopituitarism.

    Pituitary aplasia or hypoplasia occurs in anencephalic and normocephalic infants. Two infants with hypopituitarism are described; in each the diagnosis was made clinically. The infants had frank clinical and histologic evidence of the neonatal hepatitis syndrome. There may be more than a chance relationship between the clinicopathologic findings of neonatal hypopituitarism and hepatitis.
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keywords = hepatitis
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3/24. cytomegalovirus infection, fetal liver disease, and neonatal hemochromatosis.

    Neonatal hemochromatosis is an uncommon disorder, clinicopathologically defined by severe and generally fatal liver disease of intrauterine onset associated with extrahepatic siderosis that spares reticuloendothelial elements (hemochromatotic siderosis). The agent or agents of liver disease in neonatal hemochromatosis are not known. It also is not known if intrauterine liver disease of defined infective etiology can lead to hemochromatotic siderosis. We present two patients with fetal liver disease and hemochromatotic siderosis whose cases help address these points. In the first patient rare hepatobiliary and numerous renal tubular cytomegalovirus (CMV) inclusions were found; CMV infection was confirmed by the polymerase chain reaction. Studies of the mother of the second patient 1, 5, and 9 weeks post-partum showed recent seroconversion against CMV; seroconversion against other infectious agents (toxoplasma, rubella, herpes, parvovirus B19, hepatitis a/B/C) was not present. Histologic, immunohistochemical, in situ hybridization, or polymerase chain reaction evidence of CMV infection was not present in infant tissues, even though peripartum maternal seroconversion against CMV was observed. We conclude that hemochromatotic siderosis may accompany chronic fetal liver disease of defined infective etiology (patient no. 1) and that recent maternal seroconversion against CMV in the presence of severe fetal liver disease does not necessarily mean that transplacentally acquired CMV infection caused the fetal liver disease (patient no. 2). polymerase chain reaction documentation of infective-agent genomic sequences in fetal or infant tissues permits more accurate interpretation of maternal serologic data.
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ranking = 0.33333333333333
keywords = hepatitis
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4/24. Selective antibody immune deficiency in a patient with smith-lemli-opitz syndrome.

    smith-lemli-opitz syndrome is a rare autosomal recessive disorder characterized by multiple congenital anomalies and various degrees of cognitive deficits. This condition results from a deficiency of 7-dehydrocholesterol reductase, a critical step in cholesterol biosynthesis. Children with smith-lemli-opitz syndrome have frequent infections, particularly of the respiratory tract. Immunodeficiency, however, is not recognized as a part of this metabolic condition. Frequent infections are usually attributed to a decreased patient mobility and reduced respiratory effort secondary to muscular hypotonia and mental retardation, which are often present in affected individuals. We describe a patient with smith-lemli-opitz syndrome and recurrent respiratory infections who was found to have a selective antibody deficiency. The immunological diagnosis was based on an absent immune response to Pneumovax. She also had no immunological response to hepatitis b vaccine and was unable to break down red cells with isoagglutinin B. Therapy with intravenous IgG (IVIG) was initiated. Infections were less severe, although they still occurred in a high frequency after initiation of the IVIG therapy. This finding prompts the need for a higher index of suspicion for an underlying immune deficiency in patients with smith-lemli-opitz syndrome who present with recurrent and chronic infections. Early recognition and appropriate therapeutic interventions may decrease the severity of infections, prevent potentially fatal infections, and eventually improve the quality of life in these patients.
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ranking = 0.33333333333333
keywords = hepatitis
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5/24. Early pregnancy loss and neonatal deaths associated with klebsiella pneumonia infection: a mini review of possible occupational health risk.

    Recurrent pregnancy loss is a disease of grave psychological and economic concern. The etiology in the vast majority of the cases is unknown or at best poorly understood. Although klebsiella pneumonia infections have been reported in humans and animals during pregnancy, there is hardly any information to indicate whether or not these infections may be responsible for early pregnancy loss. We present a review of literature and report for the first time in humans, klebsiella pneumonia infection in placenta of a 38-year-old secondary recurrent aborter (parity 2 3).
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ranking = 0.025757062063531
keywords = animal
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6/24. Probable intrafamilial transmission of coxsackievirus b3 with vertical transmission, severe early-onset neonatal hepatitis, and prolonged viral rna shedding.

    Here we report a familial cluster of 3 cases of coxsackievirus B3 infection: a recent history of illness in a woman's 3-year-old son with a coxsackievirus B3-positive stool culture indicated that he probably infected his mother at home during her last week of pregnancy. Consequently, she delivered an infected neonate who developed severe hepatitis, disseminated intravascular coagulation, and bilateral intracranial hemorrhage. The neonate remained well for the first 2 days of life. On the third day, he developed fever (39 degrees C) and poor peripheral circulation. On the fourth day, he developed petechiae and bruises over his chest wall and extremities, and prolonged bleeding was observed over venipuncture sites. Investigations revealed severe thrombocytopenia (platelets: 41 x 10(9)/L) and a markedly deranged coagulation profile (prothrombin time: 19 seconds [reference: < 10 seconds]; activated partial thromboplastin time: > 120 seconds [reference: 24.2-37.0 seconds], serum D-dimers: 6722 ng/mL [reference: < 500 ng/mL]), suggestive of disseminated intravascular coagulopathy. Clinical examination revealed yellow sclera, hepatomegaly (5 cm), and splenomegaly (2 cm), consistent with hepatitis. Serial chest radiographs showed bilateral pleural effusions, and an ultrasound of the abdomen demonstrated ascites. An echocardiogram showed normal cardiac structure and good contractility of both ventricles. However, a cranial ultrasound revealed bilateral grade 2 intraventricular hemorrhages. serum c-reactive protein increased to 33.9 mg/L. liver-function tests were also markedly deranged at this time, with maximum values for serum alanine transferase, bilirubin, alkaline phosphatase, and ammonia concentration of 1354 IU/L, 258 micromol/L, 189 IU/L, and 147 micromol/L, respectively. serum glucose levels were normal. Over the next 3 days, his fever subsided, and his liver function and clotting profile normalized by day 13 after onset of illness. A stool sample from the older brother, collected 14 days after his onset of illness at home, was positive for coxsackievirus B3 by both virus culture and enterovirus reverse-transcription polymerase chain reaction. He had neutralizing coxsackievirus B3 antibody titers of 1:2560 and 1:1280 on days 14 and 28 after his onset of illness, respectively. No virus was cultured from the mother's stool sample, collected 5 days after her onset of illness, but the enterovirus polymerase chain reaction was positive and maternal sera neutralized the coxsackievirus B3 isolated from the neonate. The maternal sera also showed a more than fourfold rise in antibody titer from 1:80 to 1:640 on days 5 and 16 after her onset of illness, respectively. Neonatal antibody titers also showed a more than fourfold rise from < 1:80 to 1:2560 on days 1 and 21 after his onset of illness, respectively. This demonstrates that both the mother and the neonate had had recent coxsackievirus B3 infections. Serially collected neonatal throat swab and stool samples were culture negative for enterovirus by 4 and 8 days after his onset of illness, respectively. However, enterovirus rna remained detectable by reverse-transcription polymerase chain reaction in these samples for considerably longer, only becoming undetectable by 16, 23, and 41 days after his onset of illness. We show that even mild household infections may have potentially serious consequences for pregnant women and their infants.
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ranking = 2
keywords = hepatitis
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7/24. cytomegalovirus-mononucleosis in a newborn infant.

    A 3-week-old infant with haemophilia A developed fever and mononucleosis and was found to have cytomegalovirus, infection, possibly acquired by blood transfusion. At 6 months, while still excreting cytomgalovirus, he developed transient clinical jaundice with a hepatitis-like picture.
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ranking = 0.33333333333333
keywords = hepatitis
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8/24. Limitations of angiographic differential diagnosis in major hepatic processes.

    In 11 instances (6%) of 183 consecutive hepatic angiograms done for subsequently proven pathologic hepatic processes, either the diagnosis was incorrect (4 patients) or a satisfactory differential diagnosis was not established (7 patients), despite obvious angiographic abnormalities. Incorrect diagnoses were made in patients with suppurative hapatocarcinoma and liver cyst, macronodular regenerative cirrhosis, and multiple intrahepatic abscesses. Satisfactory differential diagnoses could not be established in patients with enlarged intrahepatic ducts, acute viral hepatitis, recurrent cirrhosis and acute liver necrosis. On analysis, means for minimizing diagnostic failure include the use of enhancement techniques such as infusion angiography and pharmacoangiography and an unbiased, detailed analysis of the angiographic findings. Changes secondary to the pathologic process and the coexistence of multiple processes, however, can occasionally prevent an accurate diagnosis.
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ranking = 0.33333333333333
keywords = hepatitis
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9/24. Hepatic disease associated with intrauterine parvovirus B19 infection in a newborn premature infant.

    A hydropic premature infant with intrauterine growth retardation died at 4 days of age and was found at necropsy to have advanced liver disease. Clinical and serologic findings in mother and infant were consistent with recent parvovirus B19 infection. parvovirus can cause fetal liver disease in animals, and some instances of congenital hepatic dysfunction in man may be due to intrauterine parvovirus B19 infection.
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ranking = 0.025757062063531
keywords = animal
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10/24. Neonatal hepatitis and alpha-1-antitrypsin deficiency. The prognosis in five patients.

    Five patients with alpha-1-antitrypsin deficiency (PiZ) are reported. All these patients presented with the neonatal hepatitis syndrome and two fo them had developed cirrhosis at ages 5 and 8 years, respectively. Three patients, ages 1, 9 and 21 years, are asymptomatic. The oldest patient, 21 years of age, has only mild histologic changes in the liver. The prognosis for patients with alpha-1 antitrypsin deficiency (PiZ) presenting with neonatal hepatitis is not necessarily grave, a finding that differs from previous observations.
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ranking = 2
keywords = hepatitis
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