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These data provide substantial support for renal nutcracker as a common cause of orthostatic proteinuria.

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Scintigraphy with autologous granulocytes labelled by technetium-99m hexamethyl, propylene amine oxime ('Tc-HMPAO) was performed in 103 Crohn's disease patients and 52 healthy controls. In 31 patients endoscopic and histologic activity was compared with scan activity index. In the 98 patients with a positive scan, the extent of Crohn's disease, assessed by scintigraphy, was compared with that evaluated by small bowel x ray or colonoscopy with biopsies. In 48 patients, Crohn's disease activity index, activity index, simple index, erythrocyte sedimentation rate, C-reactive protein were correlated with the scan results. In 16 patients the five parameters and scan were repeated after treatment with methylprednisolone (10 cases), enteral nutrition (3), and 5-acetylsalicylic acid (3). The results showed that 'Tc-HMPAO granulocyte scan had a 95% sensitivity and 100% specificity to detect active inflammation; it correctly showed an abscess or a fistula in all the 24 cases found. The correlation between histological inflammatory activity and scan activity index was highly significant (r=0.85; p<001), less significant (r=0.65; p<0-01) between endoscopy and scan activity index. The evaluation for the extent of Crohn's disease by scan was completely correct in the small bowel (100%) and 93% correct in the large bowel. No correlation was seen between the three clinical activity parameters and scanning; in more than 80% of the cases in remission on the basis of a clinical or laboratory index, scintigraphy remained positive. Medical treatment was effective on the clinical indices but not on the active inflammation in the ileum, whereas it led to a negative scan in 5/11 cases in the large intestine. Scintigraphy with 'Tc-HMPAO granulocyte plays an important part in Crohn's disease for the diagnosis of complications, for activity and assessment of the extent, and for the treatment results evaluation.

Acute moderate to severe gastroenteritis is traditionally associated with hypernatremia but recent observations suggest that hypernatremia is currently less common than hyponatremia. The latter has sometimes been documented also in children with acute community-acquired diseases, such as bronchiolitis and pyelonephritis. We investigated the prevalence of dysnatremia in children with acute moderate severe gastroenteritis, bronchiolitis and pyelonephritis. This prospective observational study included 400 consecutive previously healthy infants ≥4 weeks to ≤24 months of age (232 males and 168 females): 160 with gastroenteritis and relevant dehydration, 160 with moderate-severe bronchiolitis and 80 with pyelonephritis admitted to our emergency department between 2009 and 2017. Circulating sodium was determined by means of direct potentiometry. For analysis, the Kruskal-Wallis test and the Fisher’s exact test were used. Hyponatremia was found in 214 of the 400 patients. It was common in gastroenteritis (43%) and significantly more frequent in bronchiolitis (57%) and pyelonephritis (68%). Patients with hyponatremia were significantly younger than those without hyponatremia (3.9 [1.6–13] versus 7.5 [3.4–14] months). The gender ratio was similar in children with and without hyponatremia. Hyponatremia was associated with further metabolic abnormalities (hypokalemia, hyperkalemia, metabolic acidosis or metabolic alkalosis) in gastroenteritis (71%) and pyelonephritis (54%), and always isolated in bronchiolitis. In conclusion, hyponatremia is common at presentation among previously healthy infants with gastroenteritis, bronchiolitis or pyelonephritis. These data have relevant consequences for the nutrition and rehydration management in these conditions.

Objective To develop and validate new Juvenile Arthritis Disease Activity Score 10 (JADAS10) and clinical JADAS10 (cJADAS10) cutoffs to separate the states of inactive disease (ID), minimal disease activity (MiDA), moderate disease activity (MoDA), and high disease activity (HDA) in children with oligoarthritis and with rheumatoid factor–negative polyarthritis, based on subjective disease assessment by the treating pediatric rheumatologist. Methods The cutoffs definition cohort was composed of 1,936 patients included in the multinational Epidemiology, Treatment and Outcome of Childhood Arthritis (EPOCA) study. Using the subjective physician rating as an external criterion, 4 methods were applied to identify the cutoffs: mapping, Youden index, 90% specificity, and maximum agreement. The validation cohort included 4,014 EPOCA patients, patients from 2 randomized trials, and 88 patients from the PharmaChild registry. Cutoff validation was conducted by assessing discriminative and predictive ability. Results The JADAS10 cutoffs were 1.4, 4, and 13, respectively, for oligoarthritis and 2.7, 6, and 17, respectively, for polyarthritis. The cJADAS10 cutoffs were 1.1, 4, and 12, respectively, for oligoarthritis and 2.5, 5, and 16, respectively, for polyarthritis. The cutoffs discriminated strongly among different levels of pain and morning stiffness, between patients who were and those who were not prescribed a new medication, and between different levels of improvement in clinical trials. Achievement of ID and MiDA according to the new JADAS cutoffs at least twice in the first year of disease predicted better outcome at 2 years. Conclusion The 2021 JADAS and cJADAS cutoffs revealed good metrologic properties in both definition and validation samples, and are therefore suitable for use in clinical trials and routine practice.

A morphometric study was carried out on biopsy specimens taken from 40 smokers (27 with chronic bronchitis and 13 asymptomatic) submitted to bronchoscopy to identify and quantify the possible structural differences between the two groups. The chronic bronchitic group had a mean age of 65.67 years and 57.04 pack-years of smoking, the asymptomatic group had a mean age of 44.69 years and 22.62 pack-years of smoking. 70 biopsy specimens (45 from chronic bronchitics and 25 from asymptomatic smokers), in which large areas of best-preserved and perpendicularly cut epithelium were present, were considered suitable for the study and examined by light and transmission electron microscopy. The mean thickness of surface epithelium (p < 0.001), the number of layers of basal cells (p < 0.001), the intercellular space of the superficial zone of the epithelium (p < 0.05) and the percentage of abnormal bronchial cilia (p < 0.05) were significantly greater in patients with chronic bronchitis than in asymptomatic smokers. No significant difference between the two groups was observed in the thickness of the lamina reticularis of the basement membrane. Goblet cell hyperplasia was more marked in chronic bronchitics than in the asymptomatic smokers (p < 0.001), whereas the frequency of epidermoid metaplasia did not show a significant difference. The morphological study of the bronchial epithelium has allowed the identification of transitional cells, which gives rise to the concept that epidermoid metaplasia may result from conversion of mucous cells. This finding suggests that the different histologic types appearing in lung tumours may originate from one undifferentiated pluripotential stem cell, which is able to differentiate into different histogenetic types.

In cases of unexplained hematuria with or without abdominal pain, clinicians should consider the diagnosis of renal vein congestion, especially in males with varicocele. Ultrasonic Doppler flow scanning is the recommended initial diagnostic modality in these patients. Expectation management is advised in the great majority of cases.