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Key Clinical Message Pancreatic polycystosis is one of rare causes of recurrent abdominal pain of pancreatic origin in children frequently associated with other organ's cysts which are to be searched. Association with pineal cyst is exceptional, and link between the two locations is to be elucidated. MRI is highly valuable to characterize cysts.

International audience; We report a 13-month-old immune-competent male child who was diagnosed with pneumococcal serotype 19A meningitis despite having received three PCV13 injections. Clinicians are reminded that bacterial meningitis can still occur, even in correctly vaccinated children. Investigations should include immune system screening along with abdominal ultrasound to exclude asplenia.

Resume Il faut distinguer les resections intestinales courtes ( a 2 m ; 15 % des cas), qui entrainent un syndrome de grele court (SGC). L’evaluation anatomique du SGC comprend : (a) la longueur et le site, jejunal et/ou ileal, de grele post duodenal restant ; (b) le type d’anastomose intestinale – enterostomie terminale (type I) – anastomose jejuno-colique (type II) ou – anastomose jejuno-ileocolique (type III), et (c) la mesure du pourcentage de colon restant en continuite. Toutes ces donnees conditionnent la clinique du SGC. La resection ileale entraine, si ≥ a 60 cm un deficit en B12 et, si ≥ a 1 m une malabsorption lipidique notable (steatorrhee > a 20 g/J) avec deficit en vitamines liposolubles (D > K > E > A). Leur supplementation – B12 par voie IM et vitamines liposolubles par voie orale – doit etre systematique. Le SGC de type I se traduit par une diarrhee hydroelectrolytique (H20 et Na), profuse en post operatoire, et dont les regles dietetiques hydriques sont strictes. Le SGC entraine un syndrome de malabsorption responsable d’une denutrition proteino-energetique, laquelle doit etre traitee par une hyperalimentation orale libre, hyperlipidique pour le type I, hypergluciqique pour les type II et III. La presence d’un colon en continuite (SGC types II et III)l reduit les pertes hydroelectrolytiques et, par le biais de l’hyperfermentation bacterienne des glucides, augmente la production des acides gras a chaine courte : leur absorption entraine une importante recuperation energetique ; par contre, la lithiase oxalique renale (25 % des cas) doit faire l’objet d’une prevention primaire par regime sans oxalate et calcitherapie orale et une encephalopathie D lactique survient chez 2 % des patients. Le SGC entraine une hypomagnesemie severe quasi constante qui induit une hypocalcemie vitamine D resistante, et une hypokaliemie resistante aux sels de potassium.

Resume Le magnesium a un role majeur dans le metabolisme energetique et proteique et dans l'homeostasie des electrolytes. Un deficit magnesien ou une hypomagnesemie traduit quasi constamment une pathologie sous-jacente d'origine digestive ou renale. Elle est tres frequente en milieu hospitalier notamment en milieu de reanimation. L'efficacite et la tolerance des differents sels de magnesium disponibles sur le marche sont mal connues. Cette mise au point resume les principales causes d'hypomagnesemie ainsi que les differentes methodes de traitement selon les situations cliniques.

To assess the dynamics of taurine metabolism in vivo, two sets of studies were carried out in healthy volunteers. First, pilot studies were carried in a single human subject to determine the time course of plasma and whole blood isotope enrichment over the course of an 8-h, unprimed continuous infusion of [1,2-13C2]taurine. Second, five healthy adult males received two tracer infusions on separate days and in randomized order: 1) a 6-h continuous infusion of [1,2-13C2]taurine (3.1 ± 0.2 μmol·kg−1·h−1) and 2) a bolus injection of [13C2]taurine (3.0 ± 0.1 μmol/kg). Isotope enrichments in plasma and whole blood taurine were determined by gas chromatography-mass spectrometry. The pilot experiments allowed us to establish that steady-state isotope enrichment was reached in plasma and whole blood by the 5th h of tracer infusion. The plateau enrichment reached in whole blood was lower than that obtained in plasma taurine ( P < 0.02). In the second set of studies, the appearance rate (Ra) of plasma taurine, determined from continuous infusion studies was 31.8 ± 3.1 μmol·kg−1·h−1. After a bolus injection of tracer, the enrichment decay over the subsequent 2 h was best fitted by a two-exponential curve. Taurine Ra was ≈85% higher when determined using the bolus injection technique compared with continuous infusion of tracer. We conclude that 1) taurine Ra into plasma is very low in healthy postabsorptive humans, and, due to taurine compartmentation between the extra- and intracellular milieus, may represent only interorgan taurine transfer and merely a small fraction of whole body taurine turnover; and 2) the bolus injection technique may overestimate taurine appearance into plasma. Further studies are warranted to determine whether alterations in bile taurine dynamics affect taurine Ra.

Objective To determine an association between magnesium (Mg) depletion and chondrocalcinosis, which has been reported but not investigated in a cross-sectional study. Methods Prevalence of chondrocalcinosis was investigated in 144 individuals: 72 patients receiving home parenteral nutrition (HPN) compared with 72 age- and sex-matched controls. Presence of chondrocalcinosis was assessed by knee radiographs. Blood serum and globular Mg levels and 24-hour urinary Mg content were compared. Results Mean ± SD age for both patients and controls was 51 ± 17 years, and 51% in both groups were women. Mean duration of HPN was 6.4 years. Prevalence of chondrocalcinosis was markedly higher in patients receiving HPN than controls (16.6% versus 2.7%; P = 0.006, odds ratio [OR] 7.0, 95% confidence interval [95% CI] 1.45–66.1). Mean ± SD serum and globular Mg levels were significantly lower in patients than controls (serum: 0.75 ± 0.09 mmoles/liter versus 0.81 ± 0.08 mmoles/liter, P = 0.0006; globular Mg: 1.8 ± 0.31 mmoles/liter versus 2.0 ± 0.35 mmoles/liter, P = 0.0003). Twenty-four-hour urinary Mg level was lower in patients than controls (mean ± SD 3.85 ± 1.50 mmoles versus 5.37 ± 3.71 mmoles; P = 0.001). Prevalence of chondrocalcinosis was significantly higher in patients with a low serum Mg level (OR 13.5, 95% CI 2.76–127.3, P < 0.0001), with a similarly high but not significant occurrence of chondrocalcinosis in patients with a low globular Mg level (OR 4.09, 95% CI 0.603–20.26, P = 0.08) and in patients with a low 24-hour urinary Mg level (OR 3.9, 95% CI 0.77–16.34, P = 0.05). Conclusion Long-lasting Mg depletion is strongly associated with chondrocalcinosis.

Taurine deficiency in patients on long-term parenteral nutrition may be involved in cholestasis. We aimed to assess plasma taurine and tauro-conjugated bile acids in adults with short-bowel syndrome and their response to intravenous taurine. Thirty-two adult patients, who had been on taurine-free parenteral nutrition for a mean of 59(SE14) months for short-bowel syndrome, were studied retrospectively. In a second study, a subgroup of ten patients with chronic cholestasis received taurine-enriched (6·0(SE0·6)mg/kg per d) parenteral nutrition for 55(SE13) months. Post-absorptive plasma taurine and bile acid concentrations were measured and liver function tests routinely sampled. At baseline, plasma taurine was lower in patients with a jejunal length of less than 35cm (group A,n16) than in those with a jejunal length of 35cm or more (group B,n16): 43(SE3)v. 58(SE4)μmol/l (P=0·01). The groups were no different in terms of chronic cholestasis (1/6v.1/6 patients), total bile acids (26(SE13)v.14(SE5)μmol/l) or the ratio of tauro-conjugated:glyco-conjugated bile acids (5(SE2)v.8(SE 4)%, usual range 30–60%). After supplementation, there was an increase in plasma taurine level (63(SE8)v. 43(SE4),P=0·007) but was no change in either total bile acids or the ratio of tauro-conjugated: glyco-conjugated bile acids. There was a significant decrease in aspartate aminotransferase level. Long-term parenteral nutrition for short-bowel syndrome is associated with an impaired tauro-conjugation of bile acids (enterohepatic pool), irrespective of plasma taurine level (systemic pool) and despite long-term taurine intravenous supplementation.

Chondrocalcinosis is a result of deposition of calcium pyrophosphate dihydrate (CPPD) crystals in cartilage and fibrocartilage. Chondrocalcinosis is usually sporadic but has also been associated with a variety of metabolic diseases including hypomagnesemia. Reported cases of hypomagnesemia associated chondrocalcinosis were mostly due to renal genetic disorders such as Bartter's or Gitelman's syndrome. We describe 3 patients with chronic hypomagnesemia induced by short bowel syndrome who developed symptomatic chondrocalcinosis. CPPD crystals were identified by polarizing light microscopy in one patient. The underlying intestinal pathology was radiation enteritis in 2 patients and mesenteric arterial thrombosis in the third. Our observations strengthen the hypothesis of a role for magnesium in CPPD crystal deposition disease.

The purpose of our study was to systematically evaluate gastric acid output in children with long-lasting gastro-esophageal reflux (GER) in order to assess its mechanism and the need for anti-acid treatment. The investigation was carried out in 20 males and 10 females, aged 7.5 +/- 3.8 years, with prolonged (15 months) clinical manifestations of GER. All underwent routine ambulatory 24-h esophageal pH-monitoring and measurement of gastric acid secretion including gastric basal (BAO) (micromol/kg/h), maximal (MAO) and peak acid outputs (PAO) after pentagastrin (6 microg/kg sec) stimulation. Children with heartburn or abdominal pain underwent upper fiber-endoscopy. In group A (moderate GER, n=12), patients had a normal reflux index (pH4 below 5.2% of total recording time) despite abnormal Euler and Byrne scoring (median 57, 95% confidence interval 53.5-73.4). In group B (severe GER, n=18, among whom 5 were with grade III esophagitis), reflux index was5.2%. When considering all children, esophageal pH (%) was significantly correlated with MAO and PAO, r=0.33, p=0.05 and r=0.37, p=0.04, respectively. Children of group B exhibited significantly higher BAO (75, 53.96-137.81), MAO (468, 394.1-671.3) and PAO (617, 518.8-782.3) than those of group A, BAO (27, 10.8-38.5), MAO (266, 243.2-348.2) and PAO (387, 322.5-452.7), p0.05). The five children of group B with severe esophagitis exhibited significantly higher BAO, MAO and PAO than the other 13 children from the same group and those of group A, p0.05. Children with long-lasting and severe GER hyper-secrete gastric acid. Individual variations in gastric acid secretion probably account for variations in gastric acid inhibitor requirements. Anti-secretory treatment is justified in children with long-lasting GER and high pH-metric reflux index.