[Failure to draw up medical certificates increase judiciary releases]. / La moindre argumentation des certificats médicaux accroît le risque de mainlevée.

OBJECTIVES: In France, a systematic control of compulsory psychiatric admissions has existed since the enactment of the law of July 5th 2011. The Court of Cassation clarified that the liberty and custody judges (JLD) cannot supersede the medical opinion described in the medical certificates. In 2015, the JLD ordered the release of 8.4 % of all compulsory psychiatric admissions. The goal was to compare the quality of medical certificates derived from judicial release based on medical grounds with non-released witnesses from the cohort of compulsory psychiatric admissions ordered in the Groupe Hospitalier Universitaire Paris Psychiatrie & Neurosciences (GHU-Paris) between November 1, 2017 and October 31, 2018. METHODS: We included as cases all the medical certificates derived from judicial release based exclusively on medical grounds from the release cohort of the GHU-Paris from November 1, 2017 to October 31, 2018, concerning the systematic control 12 days after compulsory psychiatric admissions. A witness whose compulsory care had been maintained was matched according to the same judge, place and date of hearing, mode of compulsory care and site of hospitalization. Each certificate was analyzed according to a reading grid relating to the good decisions in matters of compulsory admission and medical certificates' redaction. An overall score, based on the description of the clinical and symptomatic evolution, the level of discernment, the capacity of consent and the mode of compulsory care was awarded to each certificate. RESULTS: Seventeen release files were included in the comparative study. Globally, the clinical progression, psychiatric symptoms, level of consciousness and ability to consent did not differ in the two groups. The grade of quality of certificate was lower in case of withdrawal (2.92±1.08 VS 3.28±0.88, P=0.026). Psychiatric symptoms in "justifiable notice" (the last medical certificate prior to the judicial hearing) were less specified in case of withdrawal (58.8 % VS 94.1 %, P=0.015). Not describing any symptoms led to a 12.51 risk of withdrawal (95 % CI=[1.16; 135.19], P=0.038). Even with witness certificate, clinical progression was noticed in only 85.3 % of cases, in 89.3 % of psychiatric symptoms, in 68.0 % of level of consciousness and 80.0 % for the ability to consent. CONCLUSIONS: Judiciary releases of compulsory psychiatric admissions exclusively based on medical grounds are not arbitrarily decided by the JLD but are based on a failure to draw up medical certificates. Doctors must comply with a careful drafting of all medical certificates: description of symptoms, clinical course, level of consciousness and ability to consent. It is necessary to be attentive to judiciary releases based on medical grounds to evaluate and improve medical practices concerning the drafting of medical certificates.

[Descriptive study of the releases pronounced by the liberty and detention judge at groupe hospitalier universitaire Paris psychiatrie & neurosciences from November 2017 to October 2018]. / Étude descriptive des mainlevées prononcées par le juge des libertés et de la détention au groupe hospitalier universitaire Paris psychiatrie & neurosciences du 1er novembre 2017 au 31 octobre 2018.

OBJECTIVE: In France, a systematic control of compulsory psychiatric admissions has existed since the enactment of the law of 5 July 2011. In 2015, the judge of freedoms and detention ordered the withdrawal of 8.4 % of the compulsory psychiatric admissions. The aim of the study is to describe the grounds for judiciary withdrawals of compulsory admissions ordered in the groupe hospitalier universitaire paris psychiatrie & neurosciences (GHU-Paris) between November 1, 2017 and October 31, 2018. METHODS: All of the withdrawal decisions adjudged during the mentioned period in the GHU-Paris were analysed following a specific framework. The main analysis deals with the classification of the "administrative", "medical", and "mixed" grounds. The secondary analysis looks at the fundamental facts affecting the judge of freedoms and detention's decision. RESULTS: Of the 127 orders decided by 21 judges of freedoms and detention analysed in this study, the majority were part of the systematic control of compulsory psychiatric admissions (74.8 %) and were made through a referral procedure by the director's hospital (69.3 %). The main reasons for withdrawal decisions were "mixed" (52.9 %), among which were described: failure to respect time limit (20.2 %), failure to inform the patient (11.6 %), third party's proceeding (8.7 %) and lack of documents (8.7 %). "Medical" grounds account for 31.8 % of all grounds. More precisely, failure to respect the required elements for involuntary admissions in psychiatric services was the greatest subcategory (29.5 %). "Other" grounds represented 15.8 %. No "administrative" ground was found. Judges ordered 69.3 % withdrawals within 24 hours to allow community treatment orders to be put into place. In the centre hospitalier Sainte-Anne, 70.8 % of the 24 appeals quashed the first decision by the judge of freedoms and detention. CONCLUSION: Several reasons justify withdrawals of compulsory psychiatric admissions. Scrupulously respecting procedures and drafting psychiatric certificates might decrease the number of withdrawals.

Early-life pulmonary arterial hypertension with subsequent development of diffuse pulmonary arteriovenous malformations in hereditary haemorrhagic telangiectasia type 1.

The case history is presented of a male infant who was thought to have idiopathic pulmonary arterial hypertension (PAH) at 3 months of age. Subsequently the PAH decreased unexpectedly and diffuse pulmonary arteriovenous malformations (PAVMs) were seen at 6.9 years of age for the first time. Hereditary haemorrhagic telangiectasia type 1 (HHT1) related to an endoglin mutation was diagnosed. At 10.3 years of age a lung biopsy showed diffuse PAVMs as well as pulmonary arteriopathy with medial hypertrophy. This is the first case of HHT1 presenting with PAH at such a young age. The subsequent decrease in pulmonary arterial pressure (PAP) was probably caused by the development of PAVMs. In the presence of PAVMs, measurement of the PAP may underestimate the extent of PAH-related vasculopathy.

Importance of environmental transmission in cases of EHEC O157 causing hemolytic uremic syndrome.

A local outbreak of Shiga toxin (Stx)-producing enterohemorrhagic Escherichia coli (EHEC) O157:H7 causing severe hemolytic-uremic syndrome (HUS) was found to be caused by environmental transmission. Automated ribotyping and pulsed-field gel electrophoresis revealed that four stx2-positive EHEC isolates obtained from two unrelated children, one mother and one cow were identical. Results of an epidemiological investigation strongly suggest that both children were infected via a meadow strewn with manure containing EHEC-positive feces from the infected cow a few days prior to the onset of illness. The cow belonged to a cattle farm neighboring the meadow. This report highlights the risk of acquiring EHEC O157 through indirect contact with a farm environment.

Echo-enhanced color Doppler cystosonography of vesicoureteral reflux in children. Improvement by stimulated acoustic emission.

PURPOSE: To prospectively evaluate the potential of echo-enhanced color Doppler sonography (ee-CDS) using the acoustic stimulated emission technique in the diagnosis of vesicoureteral reflux (VUR) in children. MATERIAL AND METHODS: 30 children (age: 0-9 years) with suspicion of VUR were submitted for echo-enhanced cystosonography (ee-CS) and conventional voiding cysto-urethrography (VCU). Conventional US and then color Doppler sonography (CDS) were performed after instillation of Levovist into the catheterized urinary bladder that had been prefilled with saline. Finally, output gain was increased in order to 'burst' the bubbles (= stimulated acoustic emission, burst-CS). Thereafter VCU was performed in the same session. The results of conventional ee-CS and burst-CS were compared to VCU findings. RESULTS: Twenty-four refluxing systems were found in a total of 66 collecting systems by both techniques. Ee-CS and burst-CS detected VUR in four systems not seen by VCU (grades 1-4), but missed low-degree VUR in 2 cases. In low-degree VUR, burst-CS was particularly helpful in 9 patients with equivocal results on conventional ee-CS. In 6 of them, VUR was proven by burst-CS, the other 3 showed VURs neither on VCU nor on burst-CS. CONCLUSION: Levovist ee-CS is feasible in the pediatric bladder. It enables reliable visualization of VUR in the renal collecting system. Burst-CS improves visualization of contrast reflux, particularly in non-dilative or minimally dilative VUR, which is sometimes difficult to depict with conventional ee-CS.

Autosomal dominant familial Mediterranean fever--like syndrome.

UNLABELLED: We report a syndrome characterized by recurrent episodes of fever and serositis in an Austrian family. Three family members over two successive generations were affected. The febrile episodes had their onset at the age of 11-12 years, lasted 1-5 weeks, and occurred in intervals of 6-24 months. While the disorder resembles familial Mediterranean fever (FMF) clinically, ethnic distribution and other features suggest a distinct entity. Clinically, the attacks last longer than the usual FMF attacks, and in the male patients are associated with scrotal inflammation. Genetically, the disorder appears to be inherited as an autosomal dominant syndrome, whereas FMF is autosomal recessive. Molecular analysis made the involvement of a gene in the FMF region of chromosome 16p13.3 highly unlikely. CONCLUSION: An Austrian family with recurrent fever syndrome is reported. Ethnicity, clinical features, and molecular studies point to a distinct clinical entity.

Perinatal manifestations of idiopathic long QT syndrome.

A neonate who had presented with sustained irregular heart rate during labor was found to have QT prolongation and repetitive polymorphic ventricular tachycardia (torsades de pointes) postnatally. Propranolol and propafenone successfully controlled the ventricular arrhythmias. Follow-up electrocardiograms and Holter records show persistent QT prolongation, bizarre T waves, and intermittent episodes of T wave alternans. On propranolol monotherapy the boy is thriving and completely free of ventricular arrhythmias. In the rare case of long QT syndrome in the neonate, early detection and therapy are mandatory to prevent ventricular arrhythmias and sudden death.

[Neonatal hyperthyroidism caused by TSH receptor antibodies in maternal autoimmune hyperthyroidism]. / Neonatale Hyperthyreose durch TSH-Rezeptor-Antikörper bei mütterlicher Autoimmunhyperthyreose.

Between July 1993 and December 1994 five term infants of mothers with Graves' disease were hospitalised at the Frühgeburtenstation of the Univ.-Frauenklinik Graz. Four Mothers had elevated TSH-receptor-antibody (TRAb)--levels during pregnancy, one had normalised TRAb-titers. In one case hyperthyroidism was first diagnosed during pregnancy. Three newborns had elevated TRAb-titers; in one of them thyrotoxicosis was diagnosed clinically and biochemically at the second day of life, one newborn had mild hyperthyroidism with tachycardia at the end of the first week of life and one newborn was asymptomatic by immediately initiated therapy. The two other newborns had normal thyroid hormone and antibody levels and no symptoms or signs of hyperthyroidism. The cases are reported and discussed in detail and our overall approach to diagnosis and treatment of neonatal hyperthyroidism in case of maternal Graves' disease will be given.

Adoptive immunotransfer with viable donor mononuclear cells for recurrent chronic myelogenous leukemia after allogeneic bone marrow transplantation in two children.

Two children with Ph+ chronic myelogenous leukemia (CML) relapsed in the chronic phase after allogeneic bone marrow transplantation (BMT). They were treated with transfusions of peripheral blood mononuclear cells (PBMC) obtained from the former bone marrow donors in combination with interferon alfa-2. In one child, CML was successfully controlled as shown by disappearance of Ph+ metaphases as well as negativity for BCR-ABL fusion gene transcripts demonstrated by polymerase chain reaction (PCR) analysis. The patient has remained in complete remission without evidence of disease for 12 months after donor PBMC transfusions. The other child showed disappearance of BCR-ABL gene transcripts by PCR analysis only in peripheral blood cells, but PCR positivity persisted in bone marrow samples. These results indicate that adoptive immunotherapy may be a further alternative in children with relapse of CML after allogeneic BMT as previously described for adult patients.

Familial haemophagocytic lymphohistiocytosis. A report of three cases with unusual lung involvement.

Three cases of familial haemophagocytic lymphohistiocytosis are presented with lung infiltration by haemophagocytic histiocytes. In all patients the diagnosis was based on hepatosplenomegaly, thrombocytopenia and anaemia, abnormal increase in triglycerides, ferritin and LDH, hypofibrinogenaemia and lymphohistiocytosis with haemophagocytosis in bone marrow. Two patients died of respiratory failure due to interstitial pneumonia. In these two patients the pneumonia was obscured and misinterpreted by the pathologists. A careful re-examination revealed lymphohistiocytosis and haemophagocytic cells within the lung parenchyma. The third patient showed alveolar wall infiltration by haemophagocytic histiocytes and lymphocytes. In early childhood acute or recurrent interstitial pneumonia should prompt a search for haemophagocytic histiocytes, and familial haemophagocytic lymphohistiocytosis should be included in the differential diagnosis.

Amyloid goiter in a child with familial Mediterranean fever.

A 7 year-old Turkish boy presented with a euthyroid goiter, which was noted during evaluation of familial Mediterranean fever. Amyloid deposits in the thyroid were found on fine-needle aspiration biopsy. Slight involution of the goiter within seven months may be attributed either to colchicine therapy or to treatment with levothyroxine and iodide.

Familial hemophagocytic lymphohistiocytosis associated with disseminated T-cell lymphoma: a report of two siblings.

Two siblings with evidence of disseminated T-cell lymphoma at the time of diagnosis of familial hemophagocytic lymphohistiocytosis (FHL) are reported, an association which has not been described previously. The first child with typical clinical and laboratory features of FHL died shortly after admission, before diagnosis could be established. Retrospective analysis of autoptic tissue revealed marked hemophagocytosis as well as morphological and immunohistochemical features suggestive of disseminated T-cell lymphoma. In the second child, FHL was diagnosed in time. Subsequent histologic investigation of bone marrow biopsies displayed a focal infiltration by T-cell lymphoma. DNA hybridization studies provided evidence of a monoclonal T-cell receptor beta chain gene rearrangement. Following conventional chemotherapeutic induction for FHL, the patient received an allogeneic bone marrow transplant (BMT) from a related healthy donor. Currently, 17 months after BMT, the boy is in unmaintained remission from FHL and T-cell lymphoma. The current pathogenetic concepts for FHL and a possible relationship between T-cell lymphoma and FHL are discussed.

SHORT syndrome and insulin resistance.

We describe a further case of SHORT syndrome. This girl shows nearly all the typical manifestations reported in patients with SHORT syndrome. However, at 14 years she presented with non-ketotic hyperglycemia. At 16 1/2 years, the diagnosis of diabetes mellitus secondary to severe insulin resistance was made by intravenous insulin challenge. Insulin resistant diabetes mellitus seems to be a new finding in SHORT syndrome, not previously described in this condition.

Undifferentiated (embryonal) sarcoma of the liver in childhood. Successful combined-modality therapy in four patients.

BACKGROUND: Undifferentiated (embryonal) sarcoma of the liver is a rare, highly malignant, mesenchymal tumor presenting predominantly in late childhood. Four girls, ages 6-13 years, who were treated with combined-modality regimens are reported. METHODS: In the first and second patients, hemihepatectomy resulted in complete removal of the tumor, and multiple-agent chemotherapy was administered postoperatively. In the third patient, only partial resection could be accomplished initially. By synchronous radiation therapy and chemotherapy, the tumor decreased to an extent that it could be resected completely and was totally devitalized on histologic examination. Postoperative chemotherapy was discontinued after 8 weeks. In the fourth patient, the tumor was not resectable at diagnostic biopsy. Polychemotherapy led to a significant reduction of the tumor size, and resection with clear margins could be performed subsequently. Because histologic necrosis amounted to about 95%, postoperative chemotherapy was also discontinued after 6 weeks. RESULTS: All four patients remain well without evidence of tumor recurrence after 79, 41, 36, and 22 months from diagnosis. CONCLUSIONS: The authors suggest that a multimodal therapeutic regimen should be used in patients with undifferentiated hepatic sarcoma.

Single dose of filgrastim (rhG-CSF) increases the number of hematopoietic progenitors in the peripheral blood of adult volunteers.

Hematopoietic progenitor cell levels were monitored in the peripheral blood of ten healthy adults receiving a single dose of recombinant human granulocyte colony-stimulating factor (rhG-CSF). The objective was to determine the time and number of progenitor cells released into the peripheral blood, induced by a single dose of 15 micrograms/kg rhG-CSF administered intravenously. In all cases the absolute number of circulating progenitor cells including granulocyte-macrophage and erythroid lineages increased up to 12-fold (median 9.4-fold) 4 days after treatment. These findings were based on flow cytometric quantification of CD34+ cells and on progenitor assays. The relative distribution of granulocyte/macrophage and erythroid progenitors remained unchanged. rhG-CSF was well tolerated; mild to moderate bone pain was the most common side-effect and was noted in 6 of 10 subjects. Thus a single dose of rhG-CSF is effective in mobilizing progenitor cells into the peripheral blood in healthy adults. If these progenitors are capable of reconstituting bone marrow, peripheral progenitor cell separation following rhG-CSF administration could be a reasonable alternative to conventional bone marrow harvest in healthy adults.

Treatment of aplastic anemia with a monoclonal antibody directed against the interleukin-2 receptor.

Severe aplastic anemia (SAA) has been related in most cases to underlying autoimmune conditions. Various immunosuppressive regimens have been recommended in the absence of an HLA-identical bone marrow donor. Prednisolone, antithymocyte globulin, and cyclosporin A have been shown to be effective. This report describes the successful treatment of a 23-year-old woman suffering from severe aplastic anemia who had become multiresistant against previously administered immunosuppressive agents, using a monoclonal IL-2-receptor blocking antibody. The patient responded within 4 weeks. The time to the next relapse was 8 months; however, another remission with a second course of horse-antithymocyte globulin was achieved and has been maintained for 27 months to date with low doses of cyclosporin A. Although this is an anecdotical report, IL-2-receptor blockade using a monoclonal antibody might be considered as a further alternative in multi-resistant SAA, perhaps increasing the susceptibility to further immunosuppressive trials.