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Biomedical subjects

E Rosenthal

Publications and source records attributed to E Rosenthal.

At least 55 records · Page 3Linked to original sources

Outcome of staged reconstructive surgery for hypoplastic left heart syndrome following antenatal diagnosis.

BACKGROUND AND AIMS: Staged reconstructive surgery has radically altered the prognosis of hypoplastic left heart syndrome (HLHS). Antenatal diagnosis allows for appropriate counselling, and time to consider treatment options. We report outcome from a centre where most cases are antenatally diagnosed and delivered on site. METHODS: Information was collated on 188 consecutive cases of HLHS between 1995 and 2000, including timing of diagnosis, outcome of pregnancy, and age and outcome at each stage of surgery. At Guy's Hospital, 174 cases were diagnosed antenatally, of whom 50 underwent surgery. Fourteen others (five diagnosed antenatally at other centres, and nine diagnosed postnatally) also underwent surgery. RESULTS: Survival after stage I (the Norwood operation) was 52% (33/64). Postoperative survival after stage II (the hemi-Fontan operation, performed in 29), and stage III (the Fontan operation, performed in 10), was 100%. Two late deaths occurred 3 and 10 months after stage II, giving overall survival of 48% (31/64). At follow up, three children have neurological impairment, and one had poor right ventricular function necessitating cardiac transplantation. CONCLUSIONS: Antenatal diagnosis allows informed decisions about treatment options, and facilitates preoperative care. Mortality following stage I is high, irrespective of timing of diagnosis, but medium term outcome for survivors is good.

Cardiac Surgical Procedures↗

Development of a protocol for coronal reconstruction of the maxillofacial region from axial helical CT data.

Using a fresh frozen cadaver head, a series of axial helical CT scans were obtained using varying imaging parameters both before and after traumatizing the head. The appearance of reformatted coronal images was optimized for the lowest radiation dose. A protocol for imaging the maxillofacial region was developed that produced diagnostic coronal reconstructed images from the axial helical CT data.

Cadaver↗

Absence of conduction defects in the electrocardiograms [correction of echocardiograms] of mothers with children with congenital complete heart block.

OBJECTIVE: To investigate the association between complete congenital heart block (CCHB) in the fetus and adult disease. METHODS: We prospectively studied 111 consecutive patients with systemic lupus erythematosus (SLE) or a positive extractable nuclear antigen (Ro, La, or RNP) attending a connective tissue disease (CTD) clinic and 19 patients who had had children with CCHB. Electrocardiographs were recorded on all patients and subsequently analyzed blindly. RESULTS: There was a significantly shorter PR interval in the mothers of children with CCHB compared to patients attending the CTD clinic (p < 0.02). There was no significant difference in PR interval between Ro positive and Ro negative patients in the CTD clinic. There were no significant differences in QRS or QTc duration between mothers with CCHB children and Ro positive or Ro negative patients attending the CTD clinic. The shorter PR interval in the mothers with CCHB children may be explained by their significantly younger age compared to patients in the CTD clinic (p < 0.02, 95% CI) and the lower incidence of CTD. CONCLUSION: We were unable to confirm the association between anti-Ro antibodies and cardiac conduction defects in adults. This supports theories that CCHB is due to vulnerability peculiar to the fetal heart during a particular stage of development between 16 and 30 weeks of gestation.

Adolescent↗

Amplification of immunological functions by subcutaneous injection of intermediate-high dose interleukin-2 for 2 years after autologous stem cell transplantation in children with stage IV neuroblastoma.

BACKGROUND: Immunotherapy given post-autologous stem cell transplantation may eliminate residual tumor cells escaping the conditioning protocol. METHODS: Five children suffering from stage IV neuroblastoma were treated by recombinant interleukin-2 (IL-2) post-autologous peripheral blood stem cell transplantation. The patients' peripheral mononuclear cells were monitored for CD3+ and CD56+ levels, their proliferative response and killing of various cell lines targets. RESULTS: An increase in the level of total lymphocytes, mainly due to expansion of T cells, and enhanced proliferative response to phytohemaglutinin were observed. Elevated cytotoxicity against K562 and neuroblastoma target cells was detected in four patients and against K562 targets in one patient. Toxicity included mild thrombocytopenia, and fever in four patients and mild to moderate encephalopathy which necessitated withdrawing one patient from the protocol. Three of five patients studied are alive today, one of them whose IL-2 was stopped, is in relapse. Two patients have died. CONCLUSIONS: Immunotherapy with s.c. intermediate-high dose IL-2 is feasible and results in expansion of T cells and in stimulation of killing activity against several targets including in some cases, neuroblastoma tumor cells.

Child, Preschool↗

[Disseminated Penicillium marneffei infection suggesting visceral leishmaniasis in an HIV infected patient].

BACKGROUND: Penicilliosis is a fungal infection caused by Penicillium marneffei. In Southeast Asia, this infection is common in HIV-infected patients but few cases have been reported in Europe. CASE REPORT: The patient lived in southern France. He had HIV infection and was immunocompromised. The clinical signs suggested visceral leishmaniasis. Iterative travels to Southeast Asia were reported leading to the diagnosis of P. marneffei infection. DISCUSSION: In southern France, imported penicilliosis marneffei may be misdiagnosed as visceral leishmaniasis.

AIDS-Related Opportunistic Infections↗

Hepatitis C virus infection and lymphoproliferative diseases in France: a national study. The GERMIVIC Group.

The putative role of hepatitis C virus (HCV) infection in the pathophysiology of lymphoproliferative diseases (LPD) is supported by North American and southern European studies reporting high HCV seroprevalence in patients with B-cell-non-Hodgkin lymphoma (NHL). In order to evaluate the situation in France, we conducted a retrospective national study about the association of chronic HCV infection and LPD. 72 Internal Medicine and Infectious Diseases departments were contacted. Response rate was 51.4%. We recorded 43 LPD (19 males, 24 females): 31 B-cell-NHL, 4 Waldenström's macroglobulinemia, 3 chronic lymphocytic leukemia, 2 multiple myeloma, 2 lymphomas of the mucosa-associated lymphoid tissue, and 1 Hodgkin's disease. Mean age at HCV diagnosis was 62 years (range 33-84). In 16 cases, LPD occurred in patients known to be HCV-infected. For 11 patients, LPD diagnosis preceded the diagnosis of HCV infection, whereas diagnosis was done simultaneously in 11 patients. For those with accurate infection date, mean interval between both events was 15.2 years. Fourteen patients had HCV extrahepatic manifestations: 9 mixed cryoglobulinemia, including 7 with NHL, 5 sicca syndrome (5 NHL), and both in one patient. Cohort of HCV-infected patients could be accurately determined for 16 departments, totaling 1,485 patients and 37 cases. Thus, from our data the frequency of LPD among HCV-infected patients approximates 2. 49%. Despite possible bias inherent to this retrospective study, our data support the hypothesis of HCV-associated LPD and particularly B-cell-NHL. In France, this association is much lower than in Italy. Further studies are needed to assess the precise role of HCV in the multistep process leading to monoclonal proliferation.

Adult↗

Severe pulmonary disease in association with Crohn's disease in a 13-year-old girl.

Pulmonary manifestations of Crohn's disease are infrequent in adults and even less common in children. Our literature search found only a few cases of Crohn's disease causing pulmonary manifestations in children. We report on the case of a 13-year-old girl in whom severe pulmonary disease was found four years after the onset of Crohn's disease. Open lung biopsy uncovered bronchiolitis obliterans and granulomatous lung disease. Aggressive treatment has yielded gradual improvement. This case emphasizes the importance of recognizing the association, the differential diagnosis, and treatment implications.

Adolescent↗

Pulmonary arterial thrombosis in a neonate with homozygous deficiency of antithrombin III: successful outcome following pulmonary thrombectomy and infusions of antithrombin III concentrate.

We report a newborn male who presented with severe central cyanosis on the third day of life. Partial thrombotic obstruction of the pulmonary trunk secondary to Antithrombin III (homozygous defect of heparin binding site) deficiency was subsequently diagnosed. Surgical thrombectomy, and infusions of Antithrombin III concentrate, led to a successful outcome. We postulate that intrauterine thrombosis occurred to give this unusual presentation.

Anticoagulants↗

Clinical manifestations of visceral leishmaniasis associated with HIV infection: a retrospective study of 91 French cases.

A retrospective study was conducted in France to determine the clinical features of visceral leishmaniasis (VL) seen, between 1986 and 1997, in 91 patients co-infected with human immunodeficiency virus (HIV). Fever (87% of patients), splenomegaly (74%) and hepatomegaly (49%) were common, 43% of the patients having all of these signs and only 9% having none of them. Amastigotes were reported in atypical locations in 31 (34%) of the patients, and 15 patients had only had their VL diagnosed following accidental discovery of amastigotes in samples from their digestive tract and lungs (one), digestive tracts only (11 patients), lungs only (two), or skin (one). Some of the digestive symptoms observed are probably attributable to the intestinal infections with Leishmania. Overall, VL diagnosis was fortuitous in 27% of the subjects. Even in endemic areas, therefore, VL is not considered routinely by physicians attempting diagnoses.

Adult↗

HIV and Leishmania coinfection: a review of 91 cases with focus on atypical locations of Leishmania.

A retrospective study was conducted in France in 1998 to determine the clinical features of visceral leishmaniasis (VL) in 91 patients infected cocomitantly with human immunodeficiency virus. Our data suggest that the clinical manifestations of VL may be influenced by the immunological status, with atypical locations of Leishmania amastigotes more frequently found in severely immunocompromised patients. In such patients, the involvement of atypical locations may lead to the discovery of VL.

AIDS-Related Opportunistic Infections↗

Efficacy of bismuth-based triple therapy in children with abdominal pain and Helicobacter pylori gastritis.

BACKGROUND: To evaluate the effect of a therapeutic regimen of 7 days versus 14 days on the clinical manifestations of Helicobacter pylori gastritis in children. METHODS: Ninety children (age 2-19 years) who had abdominal pain and/or recurrent vomiting were determined to have H. pylori gastritis by endoscopy, histology, and a Giemsa stain positive for H. pylori. The patients were randomized to receive amoxicillin, metronidazole, and bismuth subcitrate for 7 days (group A; 45 children) or 14 days (group B; 45 children) and were observed clinically for 19 +/- 11.5 months. Resolution of all abdominal and gastrointestinal symptoms was considered a good response. RESULTS: A good response was obtained in 36 (80%) children from group A, and in 37 (82%) from group B. A recurrence of symptoms occurred in four (11%) of the responders from group A, and in six (15.2%) from group B. CONCLUSIONS: A 7-day course of bismuth-based triple therapy for H. pylori gastritis in children appears to be clinically as effective as a 14-day regimen. The feasibility of a shorter therapeutic regimen may enhance patient compliance and provide a better chance of clinical benefit.

Abdominal Pain↗

Extrahepatic manifestations associated with hepatitis C virus infection. A prospective multicenter study of 321 patients. The GERMIVIC. Groupe d'Etude et de Recherche en Medecine Interne et Maladies Infectieuses sur le Virus de l'Hepatite C.

From January 1996 to January 1997, 321 patients with an average age of 46 +/- 16 years and chronically infected with hepatitis C virus (HCV) were prospectively enrolled in a study designed to determine the prevalence of extrahepatic manifestations associated with HCV infection in a large cohort of HCV patients, to identify associations between clinical and biologic manifestations, and to compare the results obtained in human immunodeficiency virus (HIV)-positive versus HIV-negative subsets. In a cross-sectional study, clinical extrahepatic manifestations, viral coinfections with HIV and/or hepatitis B virus, connective tissue diseases, and a wide panel of autoantibodies were assessed. Thirty-eight percent (122/321) of patients presented at least 1 clinical extrahepatic manifestation including arthralgia (60/321, 19%), skin manifestations (55/321, 17%), xerostomia (40/321, 12%), xerophthalmia (32/321, 10%), and sensory neuropathy (28/321, 9%). Main biologic abnormalities were mixed cryoglobulins (110/196, 56%), thrombocytopenia (50/291, 17%), and the presence of the following autoantibodies: antinuclear (123/302, 41%), rheumatoid factor (107/280, 38%), anticardiolipin (79/298, 27%), antithyroglobulin (36/287, 13%) and antismooth muscle cell (27/288, 9%). At least 1 autoantibody was present in 210/302 (70%) of sera. By multivariate logistic regression analysis, 4 parameters were significantly associated with cryoglobulin positivity: systemic vasculitis (p = 0.01, odds ratio OR[ = 17.3), HIV positivity (p = 0.0006, OR = 10.2), rheumatoid factor positivity (p = 0.01, OR = 2.8), and sicca syndrome (p = 0.03, OR = 0.27). A definite connective tissue disease was noted in 44 patients (14%), mainly symptomatic mixed cryoglobulinemia and systemic vasculitis, HIV coinfection (23%) was associated with 3 parameters: anticardiolipin (p = 0.003, OR = 4.18), thrombocytopenia (p = 0.01, OR = 3.56), and arthralgia or myalgia (p = 0.017, OR = 0.23). HIV-positive patients presented more severe histologic lesions (p = 0.0004). Extrahepatic clinical manifestations in HCV patients involve primarily the skin and joints. The most frequent immunologic abnormalities include mixed cryoglobulins, rheumatoid factor, antinuclear, anticardiolipin, and antithyroglobulin antibodies. Cryoglobulin positivity is associated with systemic vasculitis and rheumatoid factor and HIV positivity. HIV coinfection is associated with arthralgia or myalgia, anticardiolipin antibodies, and thrombocytopenia.

Adolescent↗