Low bone mass is known to be associated with an increased risk of fractures. Osteoporosis prevention by maximizing bone mass will be crucial and requires a better knowledge of bone mass acquisition during adolescence. Bone mass was assessed in 574 healthy volunteer females aged 10-24 years. Spine bone mineral density (BMD) in anteroposterior (AP L2-4) and lateral (LAT L3) views was measured using dual-energy X-ray absorptiometry (DXA) and AP bone mineral content (BMC) was calculated. At the same time, spine AP-BMD (L2-4) was evaluated in 333 normal menstruating women, aged 27-47 years. Bone values, osteocalcin and IGF-1 serum concentrations were correlated with chronological age, skeletal age, pubertal stages and time after menarche. In this cross-sectional study, AP- and LAT-BMD and BMC increased dramatically between skeletal ages 10 and 14 or until the first year after menarche. Between 14 and 17 skeletal years of age, AP-BMD and BMC increased moderately, whereas LAT-BMD remained unchanged. After skeletal age 17, or the fourth year after menarche, there was no significant increase in BMD or BMC, and their values did not differ from those of menstruating women. A serum osteocalcin peak was observed at skeletal ages 11-12 or at stage P3, whereas IGF-1 peaked at 13-14 skeletal years of age or at P4 and the first year after menarche. Eighty-six per cent of the adult bone mass of the spine is acquired before skeletal age 14 or the second year after menarche; therefore osteoporosis prevention programs will be particularly effective before that age.

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BackgroundLiver adenomatosis (LA) is a rare disease resulting from biallelic inactivation of the hepatocyte nuclear factor‐1 alpha (HNF1A) gene, which induces the proliferation of adenoma cells in liver parenchyma. Liver adenomatosis has only been documented in case reports from patients carrying a HNF1A germline mutation. We have evaluated the frequency of LA among a large cohort of patients with HNF1A‐maturity onset diabetes of the young (MODY), previously termed “MODY3,” and herein describe its clinical, radiological, and pathological characteristics.MethodsIn all, 137 HNF1A‐MODY subjects from 74 families were screened by liver ultrasonography in 13 centers, and 15 additional cases of LA were later included in the series. Liver adenomatosis was confirmed by liver computed tomography, magnetic resonance imaging (MRI), and/or histopathology.ResultsAmong 137 carriers of an HNF1A mutation, 9 patients (6.5%) from seven families were diagnosed with LA. Diabetes mellitus was present in 87.5% of patients with LA. In 25% of patients, LA was diagnosed due to intra‐abdominal or intratumoral bleeding. Liver biochemistry was near normal in all patients. Liver imaging showed adenomas of various sizes and numbers. On MRI, most nodules had the radiological characteristics of steatotic adenomas. Histopathological confirmation of LA was available in 13 cases, and these adenomas were mostly steatotic. Surgery was initially performed in 37.5% of patients, and liver disease progression was observed in 30%. No disease progression was observed in 14 pregnancies.ConclusionsThe frequency of LA in a cohort of screened HNF1A‐MODY patients and the high incidence of LA progression and/or hemorrhage warrants systematic screening for liver adenomatosis in HNF1A‐MODY families.

In this study, we report a complete (clinical, radiological, and virological) sustained (1 year) response after nivolumab salvage therapy in a progressive multifocal leukoencephalopathy patient. Analyses of the cells infiltrate in a pretreatment brain biopsy suggest that parenchymal programmed cell death-L1+ macrophages could be the T-cells partnership in immune exhaustion and virus escape.

During a 5-year period, superior mesenteric vein (SMV) thrombosis was detected with computed tomography (CT) in six patients shortly after an appendectomy. No sign of SMV was present at appendectomy, and a period of more than 2 weeks free of clinical symptoms had elapsed between the appendectomy and the onset of the SMV thrombosis. In four cases, the appendicitis was complicated. These patients had nonspecific signs and symptoms, although two of them had elevation of blood hepatic enzyme levels. In all cases, postcontrast CT demonstrated enlargement of the SMV, with well-defined enhancement of the vascular wall and an intraluminal clot. In one case, CT showed extension of the thrombus to the portal vein with the presence of low-attenuation areas in the liver, consistent with hepatic infarcts. Two patients had predisposing diseases: idiopathic hypersplenism in one case and chronic hepatic disease in the other. SMV thrombosis is a possible complication of appendicitis, and early appendectomy in appendicitis can prevent this complication. Moreover, as in any abdominal surgery, early appendectomy may be complicated by thrombosis of the SMV, thus creating problems of postoperative diagnosis. The complication is more frequent when the initial operation is performed under difficult conditions (peritonitis), or when the patient presents with a coagulopathy. CT is useful in the diagnosis of SMV thrombosis, thus leading to early management with anticoagulant therapy, with a view to avoiding complications such as intestinal ischemia, portal vein thrombosis, and hepatic infarction.