Home enteral nutrition does not decrease oral feeding in children during the first year of nutritional support.

BACKGROUND: Enteral nutrition is necessary when nutritional status is poor and oral intake is insufficient or impossible. Although it has been suspected to reduce spontaneous oral feeding, no study has formally assessed the influence of enteral nutrition on pediatric oral intake. The present study aimed to evaluate variation in oral feeding intake after enteral nutrition initiation, and to identify factors influencing oral feeding. METHODS: This retrospective cohort study included 149 pediatric patients from two French tertiary care hospitals, who received home enteral nutrition from 2009 to 2022. The patients were aged 2 months to 17 years (median age 3 years, interquartile range 1.3-9.2). Oral and enteral intakes were assessed when enteral nutrition was initiated (M0), and again at M3 (n = 123), M6 (n = 129), and M12 (n = 134) follow-ups, based on dieticians' and home services' reports. Oral feeding and body mass index z score variations during follow-ups were evaluated using a linear mixed regression model, including "time" as a fixed effect and "patient" as a random effect. Factors associated with oral feeding changes were assessed using a model interaction term. RESULTS: Oral intake did not vary significantly (P = 0.99) over time and accounted for 47.4% ± 27.4%, 46.9% ± 27.4%, 48.4% ± 28.2%, and 46.6% ± 26.9% of the ideal recommended daily allowance (calculated for the ideal weight for height) at M0, M3, M6, and M12, respectively. Delivery method (nasogastric tube versus gastrostomy), prematurity, underlying disease, history of intrauterine growth retardation, and speech therapy intervention did not influence oral intake. Administration (i.e., exclusively continuous nocturnal infusion versus daytime bolus) led to different oral intake development, although oral intake also differed at M0. CONCLUSIONS: Enteral nutrition, although increasing total energy intake, does not alter oral feeding during the first year of administration. Only the mode of administration might influence oral intake.

Phenotyping OSAH patients during wakefulness.

PURPOSE: Although currently there are simplified methods to measure the pathophysiological traits that stimulate the occurrence and maintenance of obstructive sleep apnea-hypopnea (OSAH), they remain difficult to implement in routine practice. This pilot study aimed to find a simpler daytime approach to obtain a meaningful, similar pathophysiological phenotypic profile in patients with OSAH. METHODS: After obtaining diagnostic polygraphy from a group of consecutive patients with OSAH, we performed the dial-down CPAP technique during nocturnal polysomnography and used it as reference method. This allowed assessment of upper airway collapsibility, loop gain (LG), arousal threshold (AT), and upper airway muscle gain (UAG). We compared these results with a daytime protocol based on negative expiratory pressure (NEP) technique for evaluating upper airway collapsibility and UAG, on maximal voluntary apnea for LG, and on clinical predictors for AT. RESULTS: Of 15 patients studied, 13 patients with OSAH accurately completed the two procedures. There were strong (all r2 > 0.75) and significant (all p < 0.001) correlations for each phenotypic trait between the measurements obtained through the reference method and those achieved during wakefulness. CONCLUSION: It is possible to phenotype patients with OSAH from a pathophysiological point of view while they are awake. Using this approach, cutoff values corresponding to those usually adopted using the reference method can be identified to detect abnormal traits, achieving profiles similar to those obtained through the dial-down CPAP technique.

[Cat-scratch disease]. / La griffade de chat.

We present three clinical cases to show the diversity of clinical presentations of Bartonella henselae infection, from classical adenopathy to disseminated form. It is an infection that leads to diagnostic wandering, due to a lack of explicit history and the variety of clinical manifestations. Antibiotic therapy is rarely necessary because it has a spontaneously favourable evolution in the majority of cases.

Nous présentons trois cas permettant de montrer l'hétérogénéité des tableaux cliniques de l'infection à Bartonella henselae, de l'adénopathie classique à la forme disséminée. Il s'agit d'une infection qui peut mener à une errance diagnostique, en raison d'une anamnèse parfois peu parlante et de la diversité des présentations cliniques. L'antibiothérapie est rarement nécessaire puisqu'il s'agit d'une maladie à évolution spontanément favorable dans la majorité des cas.

[How I EXPLORE ... AN APPARENT LIFE THREATENING EVENT OF THE INFANT]. / COMMENT J'EXPLORE ... le malaise du nourrisson.

The ALTE (Apparent Life Threatening Event) of the infant is a frequent presenting complaint. The clinical presentation is varied, ranging from an innocuous event (as a change in skin color) to something as tragic as a sudden infant death. In all circumstances, it is always a very worrying event for the parents and the family circle. Many etiologies can explain the ALTE, and their investigation can be complicated. In this paper, the etiologies and diagnostic tests will be briefly introduced, with a reminder of the sudden infant death syndrome.

Conservative treatment of spontaneous osteonecrosis of the knee in the early stage: pulsed electromagnetic fields therapy.

HYPOTHESIS: pulsed electromagnetic fields treatment might improve symptoms in the early stage of spontaneous osteonecrosis of the knee. METHODS: Twenty-eight patients (19M/9F, age 49.8±16.4 years) suffering from symptomatic (pain) Koshino stage I spontaneous osteonecrosis of the knee, confirmed by magnetic resonance imaging (MRI) were treated with local pulsed electromagnetic fields therapy (6 h daily for 90 days). Clinical evaluation: baseline, 6- and 24-month follow-up by VAS for pain, knee society score (KSS), Tegner and EQ-5D scales. MRI evaluation: baseline and 6-month follow-up, measuring bone marrow lesion's areas and grading these lesions by WORMS score. Failures: patients undergoing knee arthroplasty. RESULTS: Pain significantly reduced at 6 months (from 73.2±20.7 to 29.6±21.3, p<0.0001), which remained almost unchanged at final follow-up (27.0±25.1). KSS significantly increased in first 6 months (from 34.0±13.3 to 76.1±15.9, p<0.0001) and was slightly reduced at final follow-up (72.5±13.5, p=0.0044). Tegner median level increased from baseline to 6-month follow-up (1(1-1) and 3(3-4), respectively, p<0.0001) and remained stable. EQ-5D improved significantly throughout the 24 months (0.32±0.33, baseline; 0.74±0.23, 6-month follow-up (p<0.0001); 0.86±0.15, 24-month follow-up (p=0.0071)). MRI evaluation: significant reduction of total WORMS mean score (p<0.0001) and mean femoral bone marrow lesion's area (p<0.05). This area reduction was present in 85% and was correlated to WORMS grading both for femur, tibia and total joint (p<0.05). Four failures (14.3%) at 24-month follow-up. CONCLUSIONS: Pulsed electromagnetic fields stimulation significantly reduced knee pain and necrosis area in Koshino stage I spontaneous osteonecrosis of the knee already in the first 6 months, preserving 86% of knees from prosthetic surgery at 24-month follow-up. No correlation was found between MRI and clinical scores. LEVEL OF EVIDENCE: Level IV; case series.

[Ischemic cardiopathy in the young: coronarographic and autopsy picture in 100 subjects]. / La cardiopatia ischemica nel giovane: quadro coronarografico ed autoptico in 100 soggetti.

Many aspects of coronary artery disease in young people are not completely understood. Our study concerns a series of 100 patients < 45 years with acute coronary artery disease. Sixty patients survived and 40 died suddenly. All subjects have been divided into four groups: Group I includes 20 deceased patients with anatomic features of acute myocardial infarction. Group II includes 20 subjects dead without features of acute myocardial infarction. Group III includes 30 patients surviving the first acute myocardial infarction. Group IV includes 30 patients surviving unstable angina. The coronary arteries have been studied by anatomic dissection in Group I and II and with coronarography in Groups III and IV. The left main was only involved in Group I and II patients. Multivessel disease was more frequent in Group I and II, but the difference was not significant. These results underline that coronary artery disease with multivessel involvement is not rare in young patients. The rare occurrence of left main disease at coronary angiography could be the consequence of the natural preselection determined by sudden death.

Serum LP(A) levels in randomized healthy men from different European countries.

Serum lipoprotein(a) [Lp(a)], blood lipids, serum insulin and anthropometric parameters were determined in randomized samples of 38-year-old men living in six European cities: Ede (The Netherlands), Deinze (Belgium), Warsaw (Poland), Lumiar (Portugal), Verona and Naples (respectively in northern and in southern Italy). In total, 406 healthy men were studied. Serum Lp(a), blood lipids and serum insulin were measured in one laboratory. All the anthropometric and metabolic variables considered were statistically different among the participating sites, with the exception of Lp(a) serum levels. In spite of the lack of overall significant inter-center differences (Kruskal-Wallis test), the subjects from the two Italian cities had significantly lower Lp(a) serum levels than the subjects from Belgium and Portugal (Mann-Whitney U test, p < 0.01). In all cities the distribution of serum Lp(a) levels were highly skewed; the percentage of subjects with serum Lp(a) levels higher than 30 mg/dl (i.e., the commonly accepted risk level of cardiovascular disease) was 6% in both Verona and Naples (Italy), 12% in The Netherlands, 16% in Poland, 18% in Belgium and 19% in Portugal (for the last two cities, respectively, p < 0.02 and p < 0.01 vs Italian cities, chi-square test). Neither anthropometric (body mass index, waist/hip circumference ratio) nor metabolic (serum lipids and insulin) parameters showed any significant relationship with serum Lp(a) levels in any of the sites (Spearman's rank correlation). These data support the possibility of a difference in serum Lp(a) levels among different European countries.

Use of an anti-idiotypic monoclonal antibody in studying amyloidogenic light chains in cells, urine and fibrils: pathophysiology and clinical implications.

A monoclonal anti-idiotype antibody (IgG1k MoAb 3B11D4) raised against the amyloidogenic DEP lambda chain dimer binds a conformational idiotope also present on the monoclonal DEP IgA immunoglobulin. MoAb 3B11D4 does not recognize the reduced and alkylated lambda chain monomers, nor the 15-17-kDa fibrillar light chain fragments which have the same N-terminal sequence of the urinary light chains. The lack of about 70 amino acid residues of the C terminal of the protein prevents the formation of the self-limiting dimer and may facilitate the deposition of the fragments into amyloid fibrils. MoAb 3B11D4 recognizes the plasma cell clone in bone marrow and 9% of circulating B lymphocytes. Panning experiments demonstrate that this antibody has the capability to selectively eliminate the idiotype positive cells from peripheral blood. Antibodies with these characteristics could become a useful tool for better understanding the pathogenesis of the disease and for new therapeutic options.

Immunocytochemical detection of factor XIII A--subunit in acute leukemia.

Factor XIII (FXIII) is a plasma pro-transglutaminase consisting of A and B subunits in a tetrameric structure. A cellular form of FXIII consisting exclusively of A subunits exists in platelets and monocytes: monocyte FXIII may be involved in connective tissue organization. To evaluate the expression and diagnostic significance of FXIII A subunit (FXIIIA) in acute leukemia, we performed an immunocytochemical study (PAP technique) with rabbit antiserum against FXIIIA on leukemic blasts of 48 cases. FXIIIA was detected only in myelomonocytic (M4), monocytic (M5) and megakaryocytic (M7) cases: in M4 and M5 samples the amount of blast cytoplasmic FXIIIA was closely correlated with the expression of monocyte-specific antigenic and cytochemical markers. Our data show immunocytochemical detection of FXIIIA to be useful for acute leukemia characterization.

Antibodies for denatured human H-ferritin stain only reticuloendothelial cells within the bone marrow.

Human H-ferritin homopolymer was denatured in sodium dodecyl sulphate and injected in mice to obtain antibodies for dissociated H-subunit. The antisera and Moabs obtained were specific for the denatured H-chain with no cross-reactivity with assembled ferritins in immunoblotting experiments. In contrast the Moabs for native recombinant H-ferritin are specific for the assembled ferritin molecules with weak cross-reactivity with the denatured H-subunits. The epitope recognized by one of the anti-denatured H-chain Moabs was mapped on the C-terminal helix of ferritin. The antibodies were used to study H-ferritin conformation in cells. In immunocytochemistry experiments the antibodies for denatured H-ferritin stained HeLa and K562 cells weakly, with a different intensity and pattern to those obtained with anti-native H-ferritin antibody. In human bone marrow smears the anti-denatured ferritin antibodies stained only reticuloendothelial cells, and did not recognize the H-ferritin rich immature erythroblasts. It is concluded that assembled and denatured H-ferritins are immunogenically distinct, and that erythroid and reticuloendothelial cells within the bone marrow contain H-ferritin in different conformations.

The syndrome of abnormal chromatin clumping in leucocytes: clinical and biological study of a case.

The authors report the clinical and biological findings of a case of a rare haematological malignant entity, morphologically characterised by a bizarre nuclear abnormality in granulocytes, consisting of exaggerated chromatin clumping and apparent fragmentation of the nucleus, with a loss of segmentation. They emphasize the coexistence of proliferative and dysplastic characteristics as a distinctive marker of this disorder and suggest it may represent a distinct rare morphological entity among the atypical chronic myeloid leukaemias, Ph1 and ber negative.

5' Nucleotidase in chronic B cell leukemias: a cytochemical and ultrastructural study.

We studied by cytochemical means the distribution of 5' nucleotidase (5' NT), a purine degradative enzyme, in the circulating lymphocytes of 24 healthy donors and 41 cases of chronic lymphoid leukemias, classified according to morphological and immunological criteria. About half the normal circulating lymphocytes were 5'NT positive and exhibited variable degrees of enzyme activity. Among chronic B lymphocytic leukemias we found high percentages of positive cells only in the phenotypically more mature cases. Moreover all cases of hairy cell, follicular cell, lymphoplasmacytic, and plasma cell leukemia showed moderate or weak 5' NT reactivity. Also one case of chronic T lymphocytic leukemia, CD8 positive, was moderately positive, while another, with large granular lymphocyte morphology, was completely negative. Electron microscopy revealed a discontinuous, granular plasma membrane reaction pattern, varying in intensity from case to case. In conclusion, our results confirm the usefulness of the 5' NT cytochemical reaction for identification of lymphoid populations at different stages of maturation in chronic B cell disorders.

Tuberous sclerosis associated with partial gigantism in a child.

The authors report a case of a six-year-old boy with seizures, mental retardation and some classic features of tuberous sclerosis, i.e., cutaneous hypomelanotic macules, poliosis, cyst-like lesions of the right upper limb and cerebral calcifications. From birth he showed partial gigantism of the first two fingers of the right hand associated with an arteriovenous malformation of the ulnar artery and agenesis of the radial artery. The EEG and CT scan studies are also reported. The occurrence of two dysgenetic disorders in one patient as a manifestation of disturbed embryogenesis at a very early stage of development is discussed.