[Celiac disease: special features in Africa. Description of 8 cases in Djibouti (horn of Africa)]. / Maladie coeliaque: particularités africaines. A propos de 8 cas à Djibouti.

Celiac disease is poorly documented in intertropical Africa. The purpose of this retrospective report was to describe 8 cases observed at the Groupement Medico-Chirurgical of Bouffard Hospital in Djibouti (Horn of Africa) between January 2003 and January 2006. There were 5 females and 3 males ranging in age from 9 months to 17 years old (mean age: 48 months). Six patients were of Somali ethnic origin and two of Yemenite ethnic origin. Six were classified as middle class and 2 as lower class. All forms were symptomatic associating constant loss of weight with digestive manifestations (diarrhoea and vomiting). Diagnosis of celiac disease was based on the presence of anti-gliadin antibodies IgA and IgG associated with anti-endomysium or anti-transglutaminase antibodies that were measured in six and two cases respectively. Gastroduodenal endoscopy performed in three cases including two with duodenal biopsy demonstrated villous atrophy associated with gross of intra-epithelial lymphocytosis. A gluten-free diet initiated in five patients led to clinical improvement in four cases with a follow-up of 8.25 months. The findings of this study in Djibouti show that celiac disease exists in intertropical Africa. Its presentation is quite similar to elsewhere but diagnosis is more difficult due to poor knowledge about the disease and limited diagnostic facilities. Favourable response to presumptive treatment by a gluten-free diet is an alternative for diagnosis especially in Djibouti where eating habits differ from those in industrialized countries and this type of diet is easier to follow.

Maladie coeliaque : particularites africaines. A propos de 8 cas a Djibouti

La maladie coeliaque est meconnue en Afrique intertropicale. Nous en rapportons 8 observations colligees de facon retrospective entre janvier 2003 et janvier 2006; au Groupement Medico-Chirurgical Bouffard a Djibouti. Elles ont concerne 5 filles et 3 garcons dont l'age varie de 9 mois a 17 ans (age moyen : 48 mois); d'ethnie Somali 6 fois et Yemenite 2 fois; issus des classes moyennes de la population 6 fois et des classes les plus defavorisees 2 fois. Il s'agissait toujours de formes symptomatiques : alteration de l'etat general constante associee a des troubles digestifs (diarrhee ou vomissements). Le diagnostic a ete retenu dans ce contexte sur la presence d'anticorps anti-gliadine de type IgA et IgG; associes a des anticorps anti-endomysium ou anti-transglutaminase respectivement realises dans 75et 25des cas. Une endoscopie gastroduodenale realisee 3 fois avec biopsies duodenales a montre deux fois une atrophie villositaire totale associee a une augmentation de la lymphocytose intra epitheliale. Un regime sans gluten; instaure chez 5 malades; a entraine une reponse clinique favorable dans 4 cas avec un recul de 8;25 mois. La maladie coeliaque existe en Afrique intertropicale dans la region de la Corne de l'Afrique ou elle ne presente pas de particularite; en dehors de difficultes diagnostiques liees a une meconnaissance de l'affection et des moyens diagnostiques souvent insuffisants. La reponse favorable au regime sans gluten d'epreuve peut constituer une alternative diagnostique d'autant que ce regime parait moins astreignant a suivre a Djibouti qu'en occident en raison d'habitudes alimentaires differentes

[Acute renal failure following internal administration of povidone iodine: a case report]. / Insuffisance rénale aiguë secondaire à la povidone iodée: à propos d'un cas clinique.

INTRODUCTION: Using iodine povidone in internal way may be responsible of severe adverse effects, sometimes causing death of the patients. EXEGESE: A 36th years old woman, with a secondary sterility has benefitted of an uterine opacification by iodine povidone before a laparotomy and a salpingotomy. In post surgery she presented an anuric acute renal failure and a severe anaemia which have needed a transfusion. Outcome was favourable with a recuperation of diuresis and a full normalization of the renal function. CONCLUSION: The authors recommend to respect the indications of iodine povidone.

[Septicaemia due to Lactobacillus jensenii: bacteriological diagnostic orientation]. / Septicémie à Lactobacillus jensenii: orientation diagnostique bactériologique.

Septicaemia concerning Lactobacillus jensenii is exceptional. This bacteria commensal of the normal human flora is known for its low pathogenicity. We report here the observation of a woman, 50 years old, who was admitted in our service for an obstructive acute renal failure and who has presented a septicaemia due to L. jensenii, with a favourable issue by use of antibiotics (amoxicillin-clavulanic acid). This observation permits to report the clinical and bacteriological characteristics of L. jensenii. The importance of the immunodepressed status (diabetes mellitus, chronic renal failure) and use of the endoureteral acts is mentioned.

[Means syndrome. 8 cases]. / Syndrome de Means. Huit cas.

BACKGROUND: Euthyroid Graves' disease is defined as an ophthalmopathy without dysthyroidism. We report 8 cases in 6 women and 2 men with an average follow-up of 16 months after diagnosis. CASE REPORTS: Unilateral ex-opythalmos was the first and most frequent sign. Ultrasonographic and immunological thyroid abnormalities were frequent but of questionable significance. Thyroid hormone levels continued to remain normal. The orbital CT-scan, used to screen for infra-clinical exophthalmos, evidenced focal or diffuse hypertrophy of the extraocular muscles and normal tendon insertions. More than 3 years after diagnosis, only one patient developed overt Graves' disease and clinical signs resolved spontaneously in one other. DISCUSSION: In mild to moderate forms of euthyroid Grave's disease, therapeutic abstention is advisable. Corticosteroids and orbital radiotherapy are interesting options in functionally disabling forms. Decompressive surgery is rarely needed.

[De La Chapelle syndrome]. / Le syndrome de De La Chapelle.

OBJECTIVE: The De La Chapelle syndrome (XX male) is a peripheral hypogonadism concerning males with 46,XX karyotype. We conducted a retrospective study of 18 cases and report the main clinical biological and hormonal characteristics. PATIENTS AND METHODS: Clinical features (weight, height, aspect of the external genital organs, body hair, gynecomastia), hormone levels (testosterone, gonadotrophin, baseline and stimulated prolactin estradiol), and results of a Barr test and karyotype were recorded in all patients in addition to search for the SRY gene (in 8 of the 18 patients). Findings were compared with a matched male population and a Klinefelter syndrome population. RESULTS: Microrchidia was found in almost all the patients while the penis had a normal size. Signs of hypoandrogenism were frequent and gynecomastia was present in half the cases. De La Chapelle patients differed from Klinefelter patients by the absence of dysmorphism. DISCUSSION: Patients with De La Chapelle syndrome diagnosed around the age of 20 years do not have borderline disorders associating genitalia anomalies or sexual ambiguity. The majority of the patients bear the testis determining SRY gene on one of the X chromosomes, providing the rational explanation of the male phenotype, but 20% of the XX males doe not have this gene. The role of certain key genes that could be implicated in abnormal sexual differentiation is known, but the complexity and heterogeneous nature of this syndrome leaves many questions unanswered. Therapy is based on androgen replacement therapy given at an early stage.

[Calculation of the absolute cardiovascular risk in practice]. / Le calcul du risque cardiovasculaire absolu en pratique.

Epidemiologic studies underline the responsibility of the cumulative effect of cardiovascular risk factors on mortality and morbidity. These data have let to the elaboration of equations predicting the risk of coronary heart disease. This probability, which defines the absolute cardiovascular risk, can be assessed using Framingham formula, Laurier-Chau's risk table, derived from the Framingham model, Ducimetière and PROCAM study' prediction models. The main interest of these equations is to give the cumulative effect of risk factors. They can be used to evaluate the advantages of primary prevention. Equations do not take into account several factors, such as body mass index, fibrinogene and lipoprotein (a) values, which have an influence on cardiovascular mortality while others are studied in their qualitative aspects (smoking status, diabetes). Furthermore, equations are not applicable to the whole population. The absolute cardiovascular is a useful tool from an epidemiologic and individual standpoint, but the limits of the different models must be known.

Optimized transient insulin infusion in uncontrolled type 2 diabetes: evaluation of a pragmatic attitude.

Glucotoxicity generated by hyperglycemia creates a vicious circle worsening the imbalance of diabetes mellitus. A pump-optimized transient insulin treatment can be used to break this fate and restore some degree of insulin sensitivity in uncontrolled type 2 diabetes. The aim of this retrospective study was to evaluate type 2 diabetics with a secondary failure to a maximal oral antidiabetic therapy, treated with a transient subcutaneous insulin therapy during 3 days. The following criteria were analysed: delay before permanent insulin treatment, prognosis factors of evolution, weight evolution and glucose control in patients maintained under oral treatment. We studied 250 type 2 diabetics, and 515 insulin infusions. The average follow-up was 3.5 years. At the end of the follow-up 63 patients required insulin from the inception of the study (Group 1), 76 secondarily resumed insulin (Group 2), and 111 remained with oral treatment (Group 3). Patients in Group 1 were significantly older, with higher HbA1c and a lower body mass index (BMI). On average, the patients in Group 3 were submitted to less than 2 insulin infusions, their BMI from the beginning to the end of the follow-up remained stable, while HbA1c improved. We conclude that transient optimized insulin treatment during 3 consecutive days is effective. Thus, 45% of the initial global population remain under oral therapy after 3.5 years with a better glucose control and a stable weight.

[Biological monitoring of chronic hemodialysis]. / Suivi biologique de l'hémodialyse chronique.

Patients suffering from acute renal failure must undergo dialysis to substitute for the kidney's excretory function. Dialysis is a means of exchange between two solutions: blood and a liquid known as dialysate, across a semi-permeable membrane. This membrane permits the passage of water and aqueous solutions of low molecular weight but not that of the aqueous solutions of high molecular weight such as proteins. Dialysed patients are biologically monitored to prevent the various complications arising from blood dialysis, to check the efficacy of the treatment as well as to reduce the risk of morbidity and mortality. Because of the multiple and complex nature of renal functions, biological monitoring of a dialysis patients presents many, often inter-related facets. In addition to the complications inherent in dialysis itself, the dialysed patient is exposed to other causes of mortality or morbidity such as nutritional, hematologic, cardiovascular and infectious problems. Clinicians is confronted by other complications, albeit less common and easier to control, in the form of osteoarticular troubles or even aluminium poisoning. Lastly, biological assessment of the efficacy of purifying processing depends principally on calculating the dose of dialysate and the ureic index of subtraction which, in turn, requires the measurement of uremia.

[Familial benign hypercalcemia revealed by renal colic]. / Hypercalcémie familiale bénigne révélée par une colique néphrétique.

BACKGROUND: Diagnosis of familial benign hypercalcemia can be challenged by the association with a urinary lithiasis, which, in the presence of hypercalcemia, will most frequently lead to affirm primary hyperparathyroidism. CASE REPORTS: We report the case of a 23 year-old patient who presented with a left ureteral stone composed of calcium oxalate. His serum total calcium value was 2.92 mmol/l. Serum PTH value was inappropriately in the normal range (32 ng/l, normal values 10 to 58 ng/l). Hypercalcemia persisted despite a subtotal parathyroidectomy and new investigation biochemical revealed familial benign hypercalcemia. CONCLUSION: This diagnosis is usually made fortuitously, since most patients have few, if any, symptoms. A past familial history of hypercalcemia and several biochemical features (such as a reduced fractional excretion of calcium, mild hypermagnesemia, and a normal serum inorganic phosphorus level) are helpful dues to the diagnosis of familial benign hypercalcemia. In some cases, however, searching for an inactivating mutation of the extracellular calcium-sensing receptor gene is necessary to distinguish this autosomal dominant disease from primary hyperparathyroidism.

[IgD myeloma manifesting as acute renal insufficiency]. / Myélome à IgD révélé par une insuffisance rénale aiguë.

INTRODUCTION: IgD myeloma is a rare disease, comprising only 1-2% of all cases of myeloma. EXEGESIS: A 71-year-old woman was admitted with acute renal failure, hypercalcemia and IgD lambda multiple myeloma. Dialysis was necessary. Six monthly cures of chemotherapy of induction according to the protocol VAD (vincristine, doxorubicin and dexamethasone) allowed to achieve moderate chronic renal failure (serum creatinine = 120 mumol/L). Sixteen months later, the patient developed an abdominal mass due to an IgD plasmocytoma in spite of treatment with interferon alpha and dexamethasone. Chemotherapy with melphalan and dexamethasone allowed to the disappearance of plasmocytoma and remission. The death occurred 36 months after the diagnosis. CONCLUSION: This observation allows to display the particularities of IgD myeloma: remarkable preponderance of lambda-type light chains, small or no visible monoclonal spike on serum electrophoresis, frequent extraosseous spread of tumor, renal failure and presence of osteolytic lesions. Over the last years, management and prediction of the survival time of IgD myeloma patients have improved.

[Hemolytic-uremic syndrome caused by mitomycin c: long-term management]. / Syndrome hémolytique et urémique secondaire à la mitomycine C: guérison à long-terme.

We describe the case of a 51-year-old woman who developed a hemolytic uremic syndrome nine weeks after the end of chemotherapy with mitomycin C for cancer of the anus. Episodes of hemolytic uremic syndrome recurred within a period of up to six months. They were treated with plasma infusion and exchange, antiplatelet agents and rigorous control of blood pressure. The patient was followed for eight years. Her blood pressure has been normal without the use of antihypertensive agents and the renal function has remained stable with serum creatinine at 110 mumol/1. The tumor has remained in remission. This case suggests that recovery from the acute phase of hemolytic uremic syndrome leads to good long-term prognosis.