Do children and adolescents with type 1 diabetes suffer from a lack of resources in France? Results from a benchmark study in the New Aquitaine region.

BACKGROUND: A benchmark study was conducted in the southwest of France, in the New Aquitaine region, to investigate metabolic outcomes and availability of resources in pediatric diabetes units. We assessed whether the level of care was in accordance with the International Society for Pediatric and Adolescent Diabetes recommendations. METHODS: Demographic and clinical data were collected, as were all HbA1c tests for the 2017 calendar year. Pediatricians specialized in diabetes care were invited to complete an online survey concerning means allocated to the management of type 1 diabetes in their centers. RESULTS: Sixteen centers provided data for 1277 patients and 3873 clinical visits. A total of 1115 children suffering from diabetes for more than 1 year were studied. Median HbA1c was 8% (7.4-8.6) for the whole region. Only 29.2% of children had good metabolic control in accordance with the <7.5% target. We identified slight but significant variation in glycemic control among centers (P=0.029). The use of an insulin pump varied greatly among centers but did not explain HbA1c differences. We did not identify a correlation between medical or paramedical time dedicated to the follow-up of diabetic patients and the mean HbA1c of each center. For 100 diabetic patients, follow-up was provided by 0.42 physicians (0.23-1.50), 0.15 nurses (0-0.56), 0.12 dietitians (0-0.48), and 0.07 psychologists (0-0.30). CONCLUSION: This study demonstrates a lack of human resources allocated to the management of type 1 diabetes in the region that is far below international recommendations. The proportion of children achieving the international glycemic target is low. There is a clear need to improve glycemic control in children, which will only be possible with improved professional practices, encouraged by benchmark studies, and by increasing the size of our multidisciplinary teams.

[Laparoscopic treatment of abdominal incisional hernias]. / Traitement laparoscopique des éventrations de la paroi abdominale.

Current management of incisional hernias is based on the tension free parietoplasty with prosthesis. This option is considered as the gold standard leading to a recurrence rate lower than 10%. Laparoscopic approach involves the placement of a composite intraperitoneal prosthesis obstructing the wall defect without anatomic reconstruction or peritoneal excision. This article describes the standard technique of the laparoscopic insertion of a prosthesis.

[Osteoarticular brucellosis and signs of autoimmunity]. / Brucellose ostéoarticulaire et signes d'auto-immunité.

UNLABELLED: We report two cases of osteoarticular brucellosis in childhood, which illustrate the diagnostic difficulties in non-endemic areas. CASE REPORT: A 12-year-old boy was admitted for a unilateral sacroiliitis for which the brucellosis origin was established by serological and blood cultures (without fever). Autoimmunity was detected and disappeared following the treatment of the affection. A six-year-old girl was admitted for a monoarthritis of the left ankle (with fever) for which the brucellosis origin was also established by serology and blood cultures. COMMENTS: The above cases underline the importance of knowing the atypical varieties of brucellosis. The brucellosis serologies need to take part in the etiological workup of an infectious osteoarthritis when a classic infectious cause cannot be proved. Blood cultures on a specific medium are essential to carry out, even in the case of apyrexia. Finally, the possibility of autoimmunity signs for the brucellosis has to be known to avoid orientating the diagnosis towards an autoimmune systemic disease.

Classification and surgical treatment of incisional hernia. Results of an experts' meeting.

BACKGROUND: The treatment of incisional hernia (IH) is a current problem in modern surgery. Many important aspects of incisional hernia surgery are yet to be answered, especially the choice of surgical technique and its adaptation to the individual patient. The aim of this experts' meeting was to resolve some current questions in incisional hernia surgery and to organise an international hernia register. METHODS: An international panel of ten experts met under the auspices of the European Hernia Society (GREPA) to investigate the classification and therapeutic alternatives for incisional hernia. Prior to the conference, all experts were asked to submit their arguments in the form of published results. All papers received were weighted according to their scientific quality and relevance. The information from this correspondence was used as a basis for panel discussion. The personal experiences of the participants and other aspects of individualised therapy were also considered. RESULTS: The expert panel suggested a new classification of incisional hernia based on localisation, size, recurrences and symptoms. All experts agreed that the fascia duplication and the fascia adaptation should only be used for small incisional hernias. Fascia duplication is of value only in the horizontal direction. The technical details and the pros and cons of each procedure were discussed for prosthetic implantation using onlay and sublay techniques and the technique of autodermal hernioplasty. CONCLUSIONS: The management of incisional hernia is currently not standardised. In order to answer relevant questions of incisional hernia surgery, an international hernia register should be established.

[Self-healing Hashimoto-Pritzker histiocytosis]. / Histocytose auto-involutive de Hashimoto-Pritzker.

UNLABELLED: Congenital 'self-healing' Langerhans' cell histiocytosis of Hashimoto-Pritzker is a rare disease occurring mainly in the neonatal period. CASE REPORT: We report on the case of a newborn with widespread eruption since birth, consisting of nodules, papulonodules, sometimes with ulcerations and scabs, concerning all the body, with a predilection for the cephalic area and the scalp, without general abnormalities. The clinical examination, histopathological data, immunohistochemistry, and the benign evolution in nine-, 18- and 24-month periods without particular treatment define the diagnosis of congenital self-healing Langerhans' cell histiocytosis of Hashimoto-Pritzker. CONCLUSION: The position of this disease among the Langherans' cell histiocytoses is probably situated at the benign pole. This is a benign self-healing disease restricted to the skin and the prognosis is good (self-involution). It is important to eliminate a malignant form of other Langerhans' cell histiocytosis such as Letterer-Siwe disease by a checkup searching for a visceral disease. The good prognosis should not lead to forget the possibility of error or forms of relapses; it is therefore imperative to have a rigorous, regular and especially long-term follow-up.

Replacement of a tracheal segment by a mixed graft (aorta and prosthesis): an experimental study in rabbits.

OBJECTIVE: To develop a new graft and to verify its technical feasibility, viability, and ability to reduce the risk of infection by interposition of an aortic segment between the airway and the prosthetic component of the graft. DESIGN: Experimental study. SETTING: Multicentre study, France. ANIMALS: 38 New Zealand rabbits. INTRODUCTION: A 1 cm aortic segment was obtained from the thoracic aorta of a rabbit and cryopreserved or stored at +4 degrees C. The aortic segment, surrounded by a ringed expanded polytetrafluoroethylene (ePTFE) prosthesis was used to replace 1 cm of cervical trachea in two groups of rabbits, either with cryopreserved or fresh aorta. MAIN OUTCOME MEASURES: Macroscopic, microbiological, and histological studies four months later. RESULTS: 12/20 animals in the cryopreserved group survived and 13/18 in those had had fresh aorta inserted. Partial or total necrosis of the aortic muscular layer was replaced by connective tissue, which was a guide for epithelialisation from the anastomoses. CONCLUSION: This new graft is worthy of further investigation, as it is technically feasible and easy to insert.

Buprenorphine withdrawal syndrome in a newborn.

A pregnant woman who was addicted to heroin rapidly withdrew from illicit drugs after the onset of a 4 mg/day buprenorphine treatment. In the newborn's blood, urine, and meconium 20 hours after birth, high concentrations of buprenorphine and its metabolite norbuprenorphine were detected, with a higher buprenorphine/norbuprenorphine ratio than in adults, possibly as a consequence of immature hepatic function; no illicit drugs were found. The child had a weak withdrawal syndrome on the second day of life and recovered rapidly. The measured buprenorphine daily dose ingested by the newborn through mother's milk was very low (3.28 micrograms) and probably had little pharmacologic effect because no withdrawal signs could be noted when maternal feeding was later abruptly interrupted. Further investigations are required to determine whether buprenorphine can be considered to be a good alternative to methadone in the treatment of pregnant heroin addicts to prevent marked withdrawal syndromes in newborns.

The abdominal linea alba: an anatomo-radiologic and biomechanical study.

Traditionally, the linea alba represents the principal route of approach in abdominal surgery and in consequence it is the commonest site of incisional hernia. The aim of this study was to review its morphology and to study its mechanical parameters of resistance, deformation and elasticity in order to compare these with the prosthetic materials most often used in the treatment of incisional hernia. Forty fresh cadavers were dissected and tests with a dynamometer and "bursting strength tester" were performed on samples taken from the linea alba at three levels: supra-umbilical, subumbilical and umbilical. Forty abdomino-pelvic scans were analysed. The morphologic results allowed definition of diastasis of the rectus mm. in terms of subject age: below 45 years of age diastasis was considered as a separation of the two rectus mm. exceeding 10 mm above the umbilicus, 27 mm at the umbilical ring and 9 mm below the umbilicus; above 45 years of age the corresponding values were 15 mm, 27 mm and 14 mm respectively. In the biomechanical study the subumbilical region exhibited a coefficient of elasticity greater than that of the supra-umbilical portion, but no significant difference in resistance was found between the different parts studied. The biomechanical results are compared with the corresponding data for prosthetic materials.

Magnetic resonance angiographic analysis of atlanto-axial rotation: anatomic bases of compression of the vertebral arteries.

The aim of this study was to identify the functional anatomic factors involved in the maintenance or disturbance of flow in the vertebral aa. during atlanto-axial rotation. Fourteen healthy volunteers were studied by magnetic resonance angiography (MRA) by a three-dimensional sequence in phase contrast centered on the vertebral aa. at the level of the cranio-cervical junction before and after left rotation of the head. A decrease in the signal intensity of the arterial flow was sought for. The results were compared to the posterolateral development of the loop of the vertebral a. in its atlanto-axial segment in neutral position, and to the measurement of the angular opening between the atlas and axis in dynamic position. Seven subjects also had a three-dimensional CT study (3D CT) of the bony relations of C1 and C2 after rotation. In 4 subjects a disturbance of flow in the right vertebral a. was observed in the transverse foramen of C2. This occurred when two factors were combined: an under-developed atlanto-axial arterial loop and a C1-C2 angle exceeding 35 degrees in maximal rotation. In the other subjects a well-developed arterial loop and/or a C1-C2 angle of less than 35 degrees in maximal rotation were factors preserving the arterial flow. The risk factor associated with the C1-C2 angle seemed correlated in 3D CT with loss of the usual asymmetric character of rotation. A clinical application is reported with a case combining chronic rotational dysfunction of the cranio-cervical junction as shown by 3D CT and complete compression of the vertebral a. in MRA, confirmed by conventional angiography. A knowledge of this physiopathologic mechanism allows clinical detection and evaluation of the risk of any effect of pathology of the cranio-cervical junction on the vertebral a.

[Abdominal wall prostheses. Biomechanic and histological study]. / Les prothèses pariétales abdominales. Etude biomécanique et histologique.

The best prosthetic material is one which provides the best mechanical resistance with the best biological tolerance. In order to assess the mechanical and histological properties of abdominal wall prostheses, we performed experimental tests in animal models comparing four materials: polypropylene, dacron, polyglactine 910 and a dacron-polyglactine 910 composite. One hundred thirty rabbits were used including 10 controls and 120 test animals. A medial laparotomy was closed with an antemuscular aponevrotic prosthesis in the test animals. Animals were sacrificed at one, two and three months after the operation. Abdominal wall and prosthesis samples were tested to determine resistance to pressure and extension, deformability and elasticity. Histology tests were also done to determine resistance quality and biological tolerance. Dacron was tolerated best and was less resistant than polypropylene, though resistance was satisfactory. There was no advantage with polyglactine compared with non-resorbable prostheses; its only indication would be a septic site. The composite material tested had a resistance comparable with that of dacron but was less well tolerated.

[Hyperexplexia]. / L'hyperexplexia.

Hyperexplexia is a disease of neonatal onset characterized by an exaggerated startle reflex. Early diagnosis is important to rule out epilepsy. Clinical findings are mainly hypertony and generalized startle reflex which is exaggerated by tiredness and some exogenous stimuli. Electroencephalogram is normal. The expression of the disease is variable including minor forms that may be unnoticed and major forms with arthrogryposis-like symptoms, orthopedic complications, false passages, apnea and even sudden infant death (SID). The evolution is generally benign and symptoms disappear within 2 or 3 years. A neuromotor retardation is often present, without intellectual deficit. In severe forms, the risk of SID requires a multidisciplinary follow-up including monitoring and treatment with clonazepam. A low GABA level in cerebrospinal fluid has been reported. Present etiological hypotheses include neuromediator and/or receptor dysfunction.

Possible involvement of a gamma-hydroxybutyric acid receptor in startle disease.

Startle disease or hyperreflexia is an autosomal dominant neurological disorder, with a neonatal onset, characterized by muscular hypertonia and myoclonic jerks, exaggerated by the slightest stimulus. Low concentrations of free gamma-aminobutyric acid (GABA) have been found in the cerebrospinal fluid of two affected infants. The involvement of GABA or its receptors has been raised and the use of GABA-agonist drugs has been suggested. We report a newborn with startle disease who also had a low concentration of GABA in the cerebrospinal fluid. No clinical improvement was observed with progabide, a GABA agonist. Furthermore, a high dose (100 mg/kg) of gamma-hydroxybutyrate (GHB) did not improve muscular stiffness and failed to induce general anesthesia. GHB, currently used as an effective general anaesthetic, is a structural analogue of GABA. It is present naturally at low concentrations in the brain and is regarded as an inhibitory neurotransmitter. Two specific GHB receptors, distinct from the GABA receptors, have been identified in rat brain. Failure to induce general anesthesia with a high dose of GHB suggests that one of these receptors could be involved in startle disease.

[Extensive ulceronecrotizing anorectitis caused by abuse of analgesic suppositories]. / Ano-rectite ulcéronécrosante extensive par utilisation abusive de suppositoires contenant des produits antalgiques.

Usually considered as totally unoffensive, antalgic suppositories can, under certain conditions, lead to extensive anorectal injury with anatomic, functional and legal implications which we evaluated on the basis of two cases and a review of the literature. Clinicians, surgeons, pathologists and pharmaceutical firms should be aware of these pathophysiological events. Consumer information is required to reduce the risk of self-medication. There are characteristic psychologic situations and "dose-dependent" toxic effects which, as the saying goes "errare humanum est, perseverare diabolicum". Careful use of scientific knowledge and wise decisions by both experts and judges are required to avoid misinterpreting the pathogenesis of this particular anatomoclinical entity.

The venous drainage of the pancreas.

With the aim of clarifying certain contradictory aspects of the description of the venous drainage of the pancreas, a review of the literature and an anatomic study were carried out. Fifty duodeno-pancreatic blocks were studied by the injection-corrosion technique, of which 45 were available for study of the right pancreas and 37 for that of the left pancreas. The venous drainage of the duodeno-pancreas is effected via two territories: a posterosuperior and an anteroinferior, the former draining toward the portal v. and the second into the superior mesenteric v. The borderline between the two is represented by the inferior posterior pancreatico-duodenal (IPPD) v. Four veins ensure duodeno-pancreatic drainage: the superior anterior, inferior anterior, superior posterior and inferior posterior pancreatico-duodenal vv. (SAPD, IAPD, SPPD and IPPD). The major vein of the ventral aspect is the SAPD; that of the dorsal aspect is the SPPD. Two arches, anterior and posterior, were found in the majority of cases. The left pancreas drains into the splenic v. via several collateral branches. Other veins participate in the venous drainage of the isthmus, body and tail of the pancreas, including the inferior or transverse pancreatic v.

Radioanatomic study of the gastrocolic venous trunk.

The mode of formation, measurements and frequency of occurrence of the gastrocolic venous trunk were studied by the injection-corrosion technique in a series of 54 anatomic specimens and by the analysis of 50 CT studies in patients without hepatic or pancreatic disease. The gastrocolic trunk was found in 51 of the 54 anatomic specimens and in 27 of the 50 CT studies. The great variability in its formation, whether bipodal, tripodal or quadripodal, was noted. With a mean diameter of 4.9 mm, it opened into the anterior, right or antero-left aspects of the superior mesenteric v. at a mean distance of 15 mm below the inferior border of the spleno-mesenteric confluence. The value of preliminary CT assessment before an operation for portal hypertension or a pancreatic tumor or in the diagnosis of a splenic thrombosis is emphasised.