Effect of hyperextension of the neck (rose position) on cerebral blood oxygenation in patients who underwent cleft palate reconstructive surgery: prospective cohort study using near-infrared spectroscopy.

OBJECTIVES: To facilitate the best approach during cleft palate surgery, children are positioned with hyperextension of the neck. Extensive head extension may induce intraoperative cerebral ischemia if collateral flow is insufficient. To evaluate and monitor the effect of cerebral blood flow on cerebral tissue oxygenation, near-infrared spectroscopy has proved to be a valuable method. The aim of this study was to evaluate and quantify whether hyperextension affects the cerebral tissue oxygenation in children during cleft palate surgery. MATERIALS AND METHODS: This prospective study included children (ASA 1 and 2) under the age of 3 years old who underwent cleft palate repair at the Wilhelmina Children's Hospital, in The Netherlands. Data were collected for date of birth, cleft type, date of cleft repair, and physiological parameters (MAP, saturation, heart rate, expiratory CO2 and O2, temperature, and cerebral blood oxygenation) during surgery. The cerebral blood oxygenation was measured with NIRS. RESULTS: Thirty-four children were included in this study. The majority of the population was male (61.8%, n = 21). The mixed model analyses showed a significant drop at time of Rose position of - 4.25 (69-74 95% CI; p < 0.001) and - 4.39 (69-74 95% CI; p < 0.001). Postoperatively, none of the children displayed any neurological disturbance. CONCLUSION: This study suggests that hyperextension of the head during cleft palate surgery leads to a significant decrease in cerebral oxygenation. Severe cerebral desaturation events during surgery were uncommon and do not seem to be of clinical relevance in ASA 1 and 2 children. CLINICAL RELEVANCE: There was a significant drop in cerebral oxygenation after positioning however it is not clear whether this drop is truly significant physiologically in ASA 1 and 2 patients.

Arrhythmogenicity of scuba diving: Holter monitoring in a hyperbaric environment.

INTRODUCTION: About 26% of diving-related fatalities are caused by cardiac disease, part of which might be associated with fatal arrhythmias. This raises the question as to whether fatal arrhythmias are being provoked by hyperbaric conditions themselves or if exercise or stress provokes the fatal arrhythmias in cases of underlying (ischemic) cardiac disease. OBJECTIVE: To measure the influence of hyperbaric conditions (50 msw) on cardiac conduction and arrhythmias in professional divers by means of ECG. METHODS: This is a prospective study on military divers in a hyperbaric chamber with continuous ECG monitoring using Holter registrations. Supraventricular and ventricular ectopy was registered during hyperbaric conditions. RR, PR, QRS, QT and QTc intervals were calculated at 50 msw and compared with ECGs at rest. RESULTS: Included were 17 male military divers who made 20 dives. A total of 10 PVCs, 45 PACs, four atrial runs and four atrial pairs were seen. Significant prolongation of the PR interval was seen and a decrease of in QRS duration at 50 msw. There was no significant change in the RR, QT and QTc intervals. CONCLUSION: In these divers, no clinically relevant arrhythmias were observed during wet dives in a recompression chamber at 50 msw. We observed a small prolongation of PR interval that is probably not clinically relevant in divers without any known conduction disorders.

Mediastinal tumor size and response to chemotherapy are the only prognostic factors in supradiaphragmatic Hodgkin's disease treated by ABVD plus radiotherapy: ten-year results of the Paris-Ouest-France 81/12 trial, including 262 patients.

PURPOSE: To identify prognostic factors in 262 patients with supradiaphragmatic Hodgkin's disease (HD), clinical stages (CS) I and II, prospectively treated between 1981 and 1988 according to the Paris-Ouest-France (POF) 81/12 protocol by three 1-month cycles of doxorubicin, bleomycin, vinblastine, and dacarbazine plus methylprednisone (ABVD-MP) followed by subtotal nodal irradiation (RT). PATIENTS AND METHODS: The size of mediastinal tumor (MT) was measured in all patients: 66 did not have MT (NoMT); 105 had a small-size MT (SSMT), ie, mediastinal mass ratio (MMR) less than 0.33; 58 had a medium-size MT (MSMT), ie, MMR > or = 0.33 and less than 0.45; and 33 had a bulky MT (BuMT), ie, MMR > or = 0.45. All patients received three cycles (CS IA, one cycle only) of ABVD-MP; patients in partial remission (PR) or complete remission (CR) after chemotherapy (CT) received supradiaphragmatic RT (involved fields, 40 Gy; adjacent fields, 30 Gy) plus lumboaortic and splenic RT (30 Gy); patients not in CR or PR after CT received salvage CT. RESULTS: Two hundred seventeen patients (82.8%) entered CR after CT and 258 (98.5%) after RT. Ten-year freedom-from-progression (FFP) and survival rateswere 88.6% and 89.4%, respectively. According to univariate analysis, MT size and post-CT status were the only factors to influence both FFP and survival. For patients with NoMT or SSMT, those with MSMT, and those with BuMT, FFP rates were 94.1%, 87.0%, and 63.0% (P < .001), respectively, while corresponding survival rates were 92.6%, 87.2%, and 78.2% (P < .05). FFP rates were significantly different between the patients who achieved CR and those who did not achieve CR after CT: 94.6% versus 65.3% (P < .001); corresponding survival rates were 89.9% and 73.7% (P < .01). Multivariate analysis confirmed that MT size and post-CT status were the only two prognostic factors for FFP; for survival, the same two characteristics, as well as age (< 40 v > or = 40 years), significantly affected prognosis. We were thus able to identify three groups. The 33 patients (12.6%) with a BuMT had 10-year FFP and survival rates of 63.0% and 78.2%, respectively. Of 229 patients without BuMT, the 195 who attained CR after CT had an optimal prognosis (FFP, 96.6%; survival, 93.6%), while those who failed to achieve CR after CT had an intermediate prognosis (FFP, 68.8%; survival, 77.6%). CONCLUSION: These results demonstrate the independent impact on HD prognosis of tumor burden and post-CT status.

Transient acanthosis nigricans following bone marrow transplantation for lymphoid malignancy.

Transient acanthosis nigricans (AN) was observed 3 months after bone marrow transplantation (BMT) for lymphoblastic lymphoma. The patient had no endocrine abnormality and had not received any drug known to induce AN. Forty-eight months post-BMT no malignancy recurred or appeared. Although usual hyperkeratosis, papillomatosis and acanthosis were present on skin biopsy, these were associated with the finding of keratinocyte necrosis and CD8+ lymphocytic infiltrate. It is suggested that graft-versus-host disease may be one of the triggers for AN.

Comparison of autologous bone marrow transplantation and peripheral blood stem cell transplantation after first remission induction treatment in multiple myeloma.

The optimal source of stem cells is a controversial issue in the field of autologous stem cell transplantation. A comparison of autologous bone marrow transplantation (BMT) and peripheral blood stem cell transplantation (PBSCT) after first remission induction treatment in multiple myeloma was made by a retrospective analysis of 132 transplants performed in 18 French Centers from 1984 to 1991 (81 autologous BMT, 51 PBSCT). The two groups differed in the median age (PBSCT 49 years; autologous BMT 55 years, P < 0.001), the duration of chemotherapy prior to transplantation, the interval between stem cell collection and transplantation, and in the conditioning regimen (more total body irradiation and higher doses of irradiation in the PBSCT group). The median time to neutrophil recovery was shorter in the PBSCT group (13 days vs. 20 days, P < 0.001), but the median time to platelet recovery did not differ significantly between PBSCT (26 days) and autologous BMT (22 days). There was no significant difference between the two groups regarding overall response rate (PBSCT 84%, autologous BMT 82%) and complete remission rate (PBSCT 37%, autologous BMT 36%). The actuarial relapse-free survival, time to treatment failure and overall survival were not significantly different. A case controlled study comparing 43 autologous BMT and 43 PBSCT matched for age and status at the time of transplantation did not show any advantage of PBSCT over autologous BMT in terms of immediate outcome, relapse-free survival, overall survival and time to treatment failure. Thus, in this retrospective analysis, the only significant benefit for PBSCT was reduced time to neutrophil recovery.

[Hematopoietic growth factors]. / Les facteurs de croissance hématopoïétiques.

Haematopoietic growth factors play a very important role in the proliferation and maturation of medullary cells. To date, about fifteen of these factors have been characterized and are known to act either on the most immature progenitors, or on the proliferation compartment, or on already mature cells. In fact, myeloid lines depend on complex interactions between these various inductors. All these inductors are made of glycoproteins, and many of them are continuously located on the long arm of chromosome 5, but they do not represent a family of genes. Their receptors are not limited to normal haematopoietic cells, and this lack of selectivity accounts for certain side-effects observed in clinical use and for the (theoretical) risk associated with their use in the treatment of some malignant blood diseases or even solid tumours.

[Non-aggressive diagnostic approach to invasive pulmonary aspergillosis in a hematology unit. Retrospective analysis of a series of 16 cases]. / Approche diagnostique non agressive de l'aspergillose pulmonaire invasive en hématologie. Analyse rétrospective d'une série de 16 cas.

PURPOSE: Among neutropenic patients, diagnosis of invasive pulmonary aspergillosis is difficult. Computed tomographic scan, bronchoalveolar lavage and histology are considered invasive procedures, because they represent an infectious risk for these immunocompromised patients. METHODS: We describe the clinical and noninvasive paraclinical (X-rays, serology) signs of invasive pulmonary aspergillosis, from a retrospective study of 16 cases in a haematology unit. RESULTS: Outside of fever and chills, cough and polypnea are the earliest signs, followed by chest pain, dyspnea, lung auscultation changes, and haemoptysis. The sensitivity of each sign is higher than 56%. Before the onset of lung auscultation changes, the chest X-ray shows mainly unilateral alveolar infiltrates. Sensitivity of serology is weak (25%), but contributed to early diagnosis in 16.6% of cases. CONCLUSION: A better knowledge of the invasive pulmonary aspergillosis clinical, radiological and serological signs could help the practician to prescribe an 'invasive' investigation (computed tomographic scan, bronchoalveolar lavage) to confirm the diagnosis of this fungal infection.

[Occurrence of non-Hodgkin's lymphoma in chronic viral hepatitis C]. / Survenue d'un lymphome non hodgkinien au cours d'une hépatite chronique virale C.

INTRODUCTION: We report the occurrence of non-Hodgkin's lymphoma during the course of chronic hepatitis C treated with alpha-interferon. EXEGESIS: Specific viruses such as Epstein-Barr virus and human T-cell leukemia viruses I and II may be at the origin of various lymphomas in human. The presence of B cell lymphoma in the course of chronic hepatitis C has already been described and could be related to the lymphoid tropism of hepatitis C virus. CONCLUSION: This new report of an association between chronic hepatitis C and B cell lymphoma should lead physicians to search for signs of lymphoma in patients with chronic hepatitis C.

[Histopathologic lesions in erythromelalgia during essential thrombocythemia]. / Lésions histopathologiques de l'érythromélalgie au cours d'une thrombocytémie essentielle.

Erythromelalgia is a vascular disorder of the extremities and is sometimes related to myeloproliferative syndrome with thrombocythemia. We report the cutaneous histopathology in case of erythromelalgia that revealed a thrombocythemia vera. Small arteries were occluded by thrombi of different age and narrowing of the lumen occurred by intimal proliferation of smooth muscle cells. There was no involvement of venules or capillaries. These vascular changes are highly suggestive of erythromelalgia and have not to be confused with necrotizing and/or granulomatous angiitis because of absence of fibrinoid necrosis and sparse inflammatory cells.

[Intra-familial graft. Respecting public health regulation and professional confidentiality may be incompatible]. / Greffes intra-familiales. Respects des règles sanitaires et du secret professionnel parfois incompatibles.

Under specific conditions, French law authorizes organ donation despite the donor's seropositivity for certain infectious conditions. The recipient must however be informed of the potential risk of graft-related infection. Anonymous donation being the rule, the rights of the donor are respected since his/her serological status remains a medical secret. However, an exception to the rule of anonymous donation is allowed for organ donation between family members. In such a situation--often justified by the urgent nature of the transplantation--the donor's serological status would have to be revealed to the recipient, breaking the rule of medical secrecy. The physician who breaks the rule is simply implementing legal regulations (with the subsequent protection against any penal or disciplinary measures) but nevertheless performs an ethically questionable act. The recompense for donation would be an incongruent violation of personal rights. At the present time, there does not appear to be a satisfactory solution to this dilemma. The only solution that could be put forward would be to ask the donor to voluntarily inform the recipient of his/her seropositivity.

[Acquired von Willebrand disease and lymphoproliferative syndromes]. / Maladie de Willebrand acquise et syndromes lymphoprolifératifs.

OBJECTIVE: Acquired von Willebrand disease occurs in patients with or without cutaneous and mucosal bleeding who have no personal or family history of the disease. The clinical course of these patients is poorly known due to the rarity of acquired von Willebrand factor (vWF) deficit. We conducted this study to assess the clinical course of acquired vWF deficit secondary to lymphoproliferative syndromes. PATIENTS AND METHODS: We report the clinical course of acquired von Willebrand disease in 6 patients with monoclonal gammapathy of undetermined significance, multiple myeloma, chronic lymphoid leukemia, Wadenstöm's macroglobulinemia, or lymphoma who were followed for 1 to 11 years. RESULTS: Acquired von Willebrand disease was suspected in non-thrombocytopenic patients with a lymphoproliferative syndrome who developed a hemorrhagic syndrome. The vWF anomaly was symptomatic in 4 of 6 patients at diagnosis. Patients were given symptomatic treatment with vWF replacement therapy as needed and specific treatment for their lymphoproliferative syndrome. Administration of DDAVP was sufficient in 3 out of 4 patients to allow invasive procedures but was unable to control digestive ulcer bleeding that required infusion of factor VIII-vWF concentrate. For 2 patients, chemotherapy was initiated due to threatening massive hemorrhage. The result was spectacular. The 4 other patients have been asymptomatic without treatment for 3, 5, 6 and 11 years during which time their lymphoproliferative syndrome has been quiescent. CONCLUSION: The clinical features and laboratory findings are similar in patients with congenital or acquired von Willebrand disease, but specific and etiologic chemotherapy is indicated for patients with acquired disease.

[Iron deficiency anemia]. / Anémies par carence en fer.

Iron deficiency anaemia is characterized by the conjunction of a microcytic, typically are generative anaemia and a biochemical syndrome in which sideropoenia is associated with transferrin increase. It is usually due to a chronic exsudative gastrointestinal or gynaecological bleeding. Diagnosing the mechanism of anaemia is easy in most cases, but difficulties arise from association with a pathology, such as chronic inflammatory syndrome, disturbing the haematological or biochemical data. The prognosis of iron deficiency anaemia depends on its cause. Treatment is aetiological (the cause of chronic bleeding is suppressed) and symptomatic (the body's iron reserves are quickly restored).

["Vitamin resistant" osteomalacia due to a defect in hepatic hydroxylation of vitamin D; reversible secondary-pseudo-hypoparathyroidism due to calcium administration]. / Ostéomalacie "vitamino-résistante" par défaut d'hydroxylation hépatique de la vitamine D; pseudo-hypoparathyroïdisme secondaire réversible par administration de calcium.

The authors report a case of vitamin resistant osteomalacia in an adult due to an apparently acquired absence of hepatic hydroxylation of vitamin D. This case was also of interest as the osteomalacia caused a state of type II pseudo-hypoparathyroidism which proved reversible on calcium drip. Thus a defect of hepatic hydroxylation of vitamin D may be a cause of vitamin resistant osteomalacia in the adult apart from the forms already described related to defect of renal hydroxylation of vitamin D. Hypocalcemia may be responsible for hypoparathyroidism due to the coexistence of a rise of circulating immuno-reactive PTH and an increase in urinary cyclic AMP. Normalisation of the calcemia permitted in this case, restoral of the efficacy of endogenous PTH.

Four brothers with Waldenstrom's macroglobulinemia.

An exceptional family including four brothers with Waldenstrom's macroglobulinemia was studied. The four patients had different light chain IgM monoclonal components: two of the kappa type and two of the lambda type. Anti-idiotypic rabbit antisera, prepared for each monoclonal component, revealed no cross-reactivity. The four brothers did not share a common HLA A B DR haplotype and a genetic linkage to the HLA complex cannot be ascertained. Five of the 12 relatives had high serum immunoglobulin concentration (four IgG, three IgA, and two IgM) without monoclonal components. Two relatives showed auto-antibodies at low titer. Some of the youngest relatives exhibited immunological abnormalities and they may be high-risk subjects with regard to Waldenstrom's macroglobulinemia.

Ankylosing spondylitis and monoclonal gammopathies.

From 1960 to 1990, 557 patients with ankylosing spondylitis (428 men, 129 women) were diagnosed and indexed in the department of rheumatology. Monoclonal gammopathies were found in seven (five men, two women) patients (1.3%). With one exception, ankylosing spondylitis preceded monoclonal gammopathies by many years. The distribution of the isotypes of the mIg found in these seven patients was striking when compared either with previous reports of an association between ankylosing spondylitis and monoclonal gammopathies or with local data on the epidemiology of monoclonal gammopathies: five patients with IgG, four of them of the lambda (lambda) type, and two IgM, both of the kappa (kappa) type were found; no patients with mIgA were recorded. Two patients were HLA-B27 positive and had slight and transient monoclonal gammopathies, whereas three subjects were HLA-B27 negative and had important spikes, corresponding in two subjects to malignant diseases. This observation raises the question of whether the coexistence of HLA-B27 and ankylosing spondylitis might provide a protective action. Epidemiological studies are required to clarify such points.

Rearrangements of the RARA and PML genes in a cytogenetic variant of acute promyelocytic leukemia.

Acute promyelocytic leukemia (APL) is usually associated with the translocation t(15;17)(q22;q12-21), which disrupts the retinoic acid receptor alpha (RARA) gene on chromosome 17 and the PML gene on chromosome 15. We report a patient with typical APL without the common t(15;17). Cytogenetic studies demonstrated a normal appearance of chromosomes 15, while a small marker seemed to be an i(17q-). Molecular analysis showed RARA and PML rearrangements, suggesting that the chromosome abnormality corresponded to a variant translocation.

Blast crisis supervening on chronic lymphocytic leukaemia. A monoclonal progression of the disease as defined by cell surface markers.

Acute leukaemia is a rare event during the course of chronic lymphocytic leukaemia (CLL), and only a small fraction of such cases have been shown to be true acute lymphoblastic crises. 1 case is described where both small lymphocytes and proliferating lymphoblasts have the same monoclonal pattern as defined by direct immunofluorescence of membrane-bound immunoglobulins. Previous cases are reviewed and do not appear to be mere coincidence: acute blast crisis may represent a part of the natural history of CLL.