Although an elevated serum IgG4 level is a characteristic feature

Although an elevated serum IgG4 level is a characteristic feature of ISC, some patients with ISC exhibit

normal serum IgG4 levels. Interestingly, positive IgG4 immunostaining of the bile duct specimen occurred despite a normal serum IgG4 level, as shown in all four patients with normal serum IgG4 levels in our study.19 Even after just a couple of weeks of steroids, there was biliary stricture improvement,20 and delaying treatment for 2 weeks likely will not affect the overall outcome. However, follow-up imaging after short-term steroid therapy is needed, selleck compound as the significant infiltration of IgG4-positive cells is also reported in CCC, although this is rare.21 A proposed diagnostic approach for hilar/intrahepatic biliary strictures that are suspicious of ISC is summarized in Figure 6. There are some limitations in this study. Our

enrolled patients were too highly selected. The results drawn from these patients could be biased and might not be representative of ISC. Our study was limited by the small number of patients with ISC. The rare occurrence of ISC makes a large cohort unlikely. In summary, ISC should be considered in the differential diagnosis of hilar or intrahepatic strictures. A past history of AIP, concurrent pancreatic lesions, or extrabiliary involvement of other organs unusual for CCC strongly suggests the possibility of ISC. The significant DAPT purchase infiltration of IgG4-positive cells on endobiliary or liver biopsy specimen

and/or elevated serum IgG4 levels highly supports the diagnosis of ISC and provides the rationale for short-term steroid therapy and reassessment of biliary strictures. This research was supported by the Chung-Ang University Research Grant 2010 (to HC Oh). “
“Acute graft-versus-host disease (GVHD) occurring within 100 days post-transplant is one of critical factors influencing prognosis in transplant recipients. Among cases of GVHD of the gastrointestinal (GI) tract, GVHD rarely affects the upper GI. In this study, we retrospectively examined the frequency of upper GI GVHD and diagnostic accuracy. From among 868 patients who underwent allogeneic hematopoietic stem cell transplantation at our hospital between January 2005 and June 2012, 115 of whom underwent biopsy for upper GI symptoms. The endoscopic 上海皓元 findings and histologic diagnosis from these 115 patients were retrospectively analyzed. GVHD was histologically diagnosed in 85 patients overall (9.8% of all 868 transplant recipients). Although gastric mucosal exfoliation was not commonly observed, this endoscopic finding when used as a diagnostic predictor had both a specificity and positive predictive value (PPV) of 100%. When using redness, luster, and mucosal change as predictors, specificity and PPV were relatively high, suggesting that these gastric endoscopic findings are useful in the diagnosis of upper GI GVHD.

Although an elevated serum IgG4 level is a characteristic feature

Although an elevated serum IgG4 level is a characteristic feature of ISC, some patients with ISC exhibit

normal serum IgG4 levels. Interestingly, positive IgG4 immunostaining of the bile duct specimen occurred despite a normal serum IgG4 level, as shown in all four patients with normal serum IgG4 levels in our study.19 Even after just a couple of weeks of steroids, there was biliary stricture improvement,20 and delaying treatment for 2 weeks likely will not affect the overall outcome. However, follow-up imaging after short-term steroid therapy is needed, Hydroxychloroquine mw as the significant infiltration of IgG4-positive cells is also reported in CCC, although this is rare.21 A proposed diagnostic approach for hilar/intrahepatic biliary strictures that are suspicious of ISC is summarized in Figure 6. There are some limitations in this study. Our

enrolled patients were too highly selected. The results drawn from these patients could be biased and might not be representative of ISC. Our study was limited by the small number of patients with ISC. The rare occurrence of ISC makes a large cohort unlikely. In summary, ISC should be considered in the differential diagnosis of hilar or intrahepatic strictures. A past history of AIP, concurrent pancreatic lesions, or extrabiliary involvement of other organs unusual for CCC strongly suggests the possibility of ISC. The significant selleck chemicals llc infiltration of IgG4-positive cells on endobiliary or liver biopsy specimen

and/or elevated serum IgG4 levels highly supports the diagnosis of ISC and provides the rationale for short-term steroid therapy and reassessment of biliary strictures. This research was supported by the Chung-Ang University Research Grant 2010 (to HC Oh). “
“Acute graft-versus-host disease (GVHD) occurring within 100 days post-transplant is one of critical factors influencing prognosis in transplant recipients. Among cases of GVHD of the gastrointestinal (GI) tract, GVHD rarely affects the upper GI. In this study, we retrospectively examined the frequency of upper GI GVHD and diagnostic accuracy. From among 868 patients who underwent allogeneic hematopoietic stem cell transplantation at our hospital between January 2005 and June 2012, 115 of whom underwent biopsy for upper GI symptoms. The endoscopic MCE公司 findings and histologic diagnosis from these 115 patients were retrospectively analyzed. GVHD was histologically diagnosed in 85 patients overall (9.8% of all 868 transplant recipients). Although gastric mucosal exfoliation was not commonly observed, this endoscopic finding when used as a diagnostic predictor had both a specificity and positive predictive value (PPV) of 100%. When using redness, luster, and mucosal change as predictors, specificity and PPV were relatively high, suggesting that these gastric endoscopic findings are useful in the diagnosis of upper GI GVHD.

Methods: We analyzed data from 5,015 asymptomatic subjects aged b

Methods: We analyzed data from 5,015 asymptomatic subjects aged between 50–70 years who had screening colonoscopy conducted at our bowel cancer screening centre between 2008 and 2012. One binary logistic regression analysis was conducted with the presence of advanced neoplasia or colorectal cancer as the outcome. We controlled for APCS, alcohol drinking, BMI, diabetes, hypertension, CHD and cirrhosis as independent variables in the regression model. Results: The average age was 57.7 years (SD = 4.90) and 47.5%

were male. Advanced neoplasms or cancers were found at colonoscopy in 5.6% of all screening participants. From multivariate regression analysis, APCS ≥ 4 (adjusted odds Dinaciclib manufacturer ratio [AOR] 1.729, 95% C. I. 1.327–2.254, p < 0.001); BMI ≥ 25 (AOR 1.313, 95% C. I. 1.011–1.705, p = 0.041), the presence of hypertension (AOR 1.652, 95% C. I. 1.264–2.159, p < 0.001) and alcohol drinking (AOR 1.505, 95% C. I. 1.066–2.125, p = 0.020) were associated with colonoscopic findings of these lesions. Diabetes, CHD and cirrhosis were not significant factors. Conclusion: Alcohol drinking, hypertension and BMI could be incorporated into the APCS scoring system to enhance its predictive value for prioritizing asymptomatic Asian subjects for colorectal screening. selleck chemical Key Word(s): 1. colorectal cancer; 2. advanced neoplasia;

3. associated factors; 4. screening; Presenting Author: YINGSIU TUNG Additional Authors: PAULAB POLETTI, THIAGOF SECCHI, ARTUR PARADA Corresponding Author: YINGSIU

TUNG Affiliations: Centro Diagnostico E Terapeutico Endoscopico Objective: AIMS: The aim of this study was determine the prevalence, age distribution, the pattern of disease involvement in the colon and clinical findings of the ischemic colitis. Methods: MATERIAL ANDE METHOD: a total of 2228 colonoscopies (videooscope Pentax and Fujinon) were performed from july 2009 to june 2012 at the endoscopic unit of the hospital 9 de Julho/ são Paulo/ brazil. we diagnosed 69 (3,09%) cases of ischemic colitis with histological confirmation. We reviewed the clinicals and endoscopics reports retrospectively. Results: RESULTS: Over a total of 2228 patients, we diagnosed 69 (3,09%) cases of ischemic colitis. The age ranged MCE公司 from 32 to 88 years, with a higher prevalence at the 8 decade with 29 cases (42%). Females had a higher prevalence (56,6%). In the evalution of the location of the disease, we observed 41 (59,45) cases in sigmoid colon, 19 (27,5%) in descending colon and 18 (26%)in the rectum. The most common presenting symtoms were hematochezia in 35 (56,6%), abodominal pain in 23 (33,3%) and diarrhea in 19 (27,5%). The endoscopic findings were: edema with erosions in 33 (47,8%), edema with erosions and ulcers in 16 (23,2%), hyperemics areas in 6 (8,7%) and swollen mucosa in 6 (8,7%) Conclusion: RESULTS: Over a total of 2228 patients, we diagnosed 69 (3,09%) cases of ischemic colitis.


“Severe liver diseases are characterized by expansion of l


“Severe liver diseases are characterized by expansion of liver progenitor cells (LPC), which correlates with Dorsomorphin purchase disease severity. However, the origin and role of LPC

in liver physiology and in hepatic injury remains a contentious topic. We found that ductular reaction cells in human cirrhotic livers express hepatocyte nuclear factor 1 homeobox B (HNF1β). However, HNF1β expression was not present in newly generated epithelial cell adhesion molecule (EpCAM)-positive hepatocytes. In order to investigate the role of HNF1β-expressing cells we used a tamoxifen-inducible Hnf1βCreER/R26RYfp/LacZ EPZ-6438 order mouse to lineage-trace Hnf1β+ biliary duct cells and to assess their contribution to LPC expansion and hepatocyte generation. Lineage tracing demonstrated no contribution of HNF1β+ cells to hepatocytes during liver homeostasis in healthy mice or after loss of liver mass. After acute acetaminophen or carbon tetrachloride injury no contribution of HNF1β+ cells to hepatocyte

was detected. We next assessed the contribution of Hnf1β+-derived cells following two liver injury models with LPC expansion, a diethoxycarbonyl-1,4-dihydro-collidin (DDC)-diet and a choline-deficient ethionine-supplemented (CDE)-diet. The contribution of Hnf1β+

cells to liver regeneration was dependent on the liver injury model. While no contribution was observed after DDC-diet treatment, mice fed with a CDE-diet showed a small population of hepatocytes derived from Hnf1β+ cells that were expanded to 1.86% of total hepatocytes after injury recovery. Genome-wide expression profile of Hnf1β+-derived cells from the DDC and CDE models indicated that no contribution of LPC to hepatocytes was associated with LPC expression of genes related to telomere maintenance, inflammation, and chemokine MCE公司 signaling pathways. Conclusion: HNF1β+ biliary duct cells are the origin of LPC. HNF1β+ cells do not contribute to hepatocyte turnover in the healthy liver, but after certain liver injury, they can differentiate to hepatocytes contributing to liver regeneration. (Hepatology 2014;60:1367–1377) “
“This study aimed to determine the role of morphological patterns seen on imaging in predicting hepatocellular carcinoma recurrence following transarterial chemoembolization therapy. Forty-seven patients from a single center who underwent transarterial chemoembolization to treat unresectable hepatocellular carcinomas between January 2011 and June 2012 were included in this study.

3% vs 32%) [10] Resistance patterns in children have been studi

3% vs 3.2%) [10]. Resistance patterns in children have been studied separately. A pan-European prospective cohort trial suggested alarmingly high levels of clarithromycin resistance (26%) in children. Amoxicillin resistance only affected 0.6% of patients, and metronidazole resistance was observed in 25% [17]. Fluoroquinolones have been less frequently used in children and adolescents and therefore the prevalence of resistance is lower: a study of 174 children in Israel revealed no resistant strains [18]. Compliance with H. pylori eradication therapy is a multifactorial process. Current evidence and published guidelines recommend complex and prolonged eradication selleck chemicals llc regimens,

using a number of antibiotics and involving manipulation of gastric pH as well. This complexity provides challenges for both the physician and the patient. Poor compliance is intricately associated with antibiotic resistance. Most studies of treatment regimes suggest compliance rates of over 95%, which learn more are implausibly high [19]. It is very difficult to accurately assess the level of compliance

as most objective indices are so open to patient manipulation as to make them worthless. It has been suggested in other studies that 10% of patients prescribed H. pylori eradication therapy will fail to take even 60% of medications [20]. It has also been proven that progressively poorer levels of compliance with therapy are associated with significantly lower levels of eradication. This is an important threshold because in an 上海皓元医药股份有限公司 ancient study, eradication levels were far superior for patients who took 60% or more of medications compared to those adhering less than 60% of the time, (96% vs 69%) [21]. We feel that the proof for the importance of compliance may lie in some of the studies, which look at resistance. For example, in one of the earlier quoted studies treatment was observed to fail around 30% of the time even when the infecting strains were found susceptible to the antibiotics used. Compliance with therapy therefore must play the principal role here [8]. Regarding

the newer therapies, compliance appears to be at least equivalent to that of existing ones with studies of levofloxacin, bismuth and sequential therapies arriving at this conclusion [19,22,23]. Enhanced compliance programs are labor intensive but may be worthwhile. In one cohort who had been given greater knowledge of their illness and the importance of compliance stressed, significantly greater levels of both compliance and eradication were achieved [24]. Finally, improvements with respect to compliance are likely to lead to lower rates of resistance. Standard triple therapy has been the accepted standard of care for H. pylori eradication therapy since the mid 1990s. This is reflected in published guidelines in Europe, North American and the Asia-Pacific region [5,25,26].

Because previous studies showed a crucial role for natural killer

Because previous studies showed a crucial role for natural killer

(NK) cells in poly(I:C)-induced liver injury,23 and higher NK cell activating ligand expression has been reported in livers of NASH patients,24 selleck chemical we next investigated the possible role of NK cells. We found increased mRNA expression of the NK-activating ligands, Pan-retinoic acid early inducible protein-1 (Rae), Rae-1α and Mult-1 in MCD-steatohepatitis (Fig. 6E), but poly(I:C) did not induce a further increase in the expression of these ligands (Fig. 6E). Furthermore, we found that recruitment of NK cells after poly(I:C) stimulation occurred in control livers and not in steatohepatitis (Supporting Fig. 1). Hepatocyte apoptosis in NASH has been linked to increased susceptibility of the fatty liver to LPS challenge, whereas hepatocyte necrosis is associated with progressive liver damage.3 There is recent evidence that the mitochondria-associated MAVS can regulate apoptosis in viral infection.25 Apoptosis is triggered by way of intrinsic (involving proapoptotic protein Bim, mitochondria, cytochrome c, and caspase 9) or extrinsic (involving death receptors including TNF-related

apoptosis-inducing ligand [TRAIL]) pathways that connect at the level of caspase 3 to culminate in cell death. We found increased expression of TRAIL Pritelivir clinical trial (extrinsic apoptosis; Supporting Fig. 2A) and Bim (intrinsic apoptosis; Supporting Fig. 2B) in livers of MCD diet–fed mice. Expression of caspase 3 was also induced in mice fed an MCD diet versus mice fed an MCS diet (Fig. 7A). We found that caspase 3 activity was significantly increased by poly(I:C) in normal (MCS) livers (Fig. 7A), but not in steatohepatitis (MCD) (Fig. 7A). There were no differences in the extent of poly(I:C)-induced up-regulation of TRAIL and Bim mRNA expression (Supporting Fig. 2A,B) between MCD and MCS livers, indicating that although steatotic livers exhibit higher apoptosis at

medchemexpress baseline, they fail to progress to tissue death due to apoptosis upon a viral challenge. Notably, the tissue damage was higher in poly(I:C)-challenged steatotic livers compared with control livers (Fig. 6B). Thus, we hypothesized that the increased poly(I:C)-induced liver damage in MCD diet–fed mice was due to necrosis rather than apoptosis. Indeed, we identified increased levels of serum HMGB1 (Fig. 7B), a marker of necrosis, in the poly(I:C)-stimulated MCD group compared with control mice. The balance between apoptosis and necrosis is tightly regulated.26 A recently identified master regulator between apoptosis and necrosis is the protein kinase receptor-interacting protein 3 (RIP3).26 We found increased levels of RIP3 mRNA (Fig. 7C) and protein (Fig. 7D) in livers of mice fed an MCD diet compared with MCS diet–fed control mice. In control mice, poly(I:C) stimulation induced up-regulation of RIP3 protein expression 2 hours after stimulation, which returned to baseline by 6 hours (Fig.

ginkgodens This discovery brings the total number of Mesoplodon

ginkgodens. This discovery brings the total number of Mesoplodon species to 15, making it, by far, the most speciose yet least known genus of cetaceans. “
“We applied temporal symmetry capture–recapture

(TSCR) models to assess the strength of evidence for factors potentially responsible for population decline in bottlenose dolphins (Tursiops truncatus) in Doubtful Sound, New Zealand from 1995 to 2008. Model selection was conducted to estimate recruitment and population growth rates. There were similar levels of support for three different models, each reflecting distinct trends in recruitment. Modeling yielded low overall estimates of recruitment Cobimetinib concentration (0.0249, 95% CI: 0.0174–0.0324) and population growth rate (0.9642, 95% CI: 0.9546–0.9737). The TSCR rate of population decline was consistent with an estimate derived from trends in abundance (lambda = 0.9632, 95% CI: 0.9599–0.9665). The TSCR model selection confirmed the influence of a decline in the survival of calves (<1 yr old) since 2002 for population trends. However, TSCR population growth rates did not exceed 1 in any year between 1995 and 2008, indicating the population was declining prior to 2002. A separate reduction in juvenile survival (1–3 yr old) prior

to 2002 was identified as a likely contributing factor in the population decline. Thus, TSCR modeling indicated the potential cause of the population decline in Doubtful Sound: cumulative impacts on individuals Selleck R788 <3 yr old resulting in a reduced recruitment. "
“Five years of behavioral observations revealed significant effects of high air temperatures and breeding site topography on the mating system of South American sea 上海皓元医药股份有限公司 lions in Peru. Unlike most polygynous mammals that defend females or fixed territories, male sea lions in Peru maintained positions along the shoreline where females passed each day to thermoregulate, and where most copulations occurred. Sex ratios (1 male per 17 females)

and male mating success were extremely skewed (14% of males achieved 50% of the copulations, and 25% of them did not copulate at all). The mass daily movements of females toward the water and cool substrate of the shoreline, along with a highly skewed sex ratio, accentuated the difficulty for males to monopolize and restrict female movements. Females moved freely and chose their mates, unlike in temperate regions of their range where male South American sea lions control groups of females or access to tide pools. Our observations indicate that the South American sea lion in Peru has a lek-like breeding system. This is a rare alternative to the common male strategies of defending females and resources, and is likely an evolutionary product of their highly skewed sex ratio, protracted breeding season, and the extreme subtropical climate where they breed.

ginkgodens This discovery brings the total number of Mesoplodon

ginkgodens. This discovery brings the total number of Mesoplodon species to 15, making it, by far, the most speciose yet least known genus of cetaceans. “
“We applied temporal symmetry capture–recapture

(TSCR) models to assess the strength of evidence for factors potentially responsible for population decline in bottlenose dolphins (Tursiops truncatus) in Doubtful Sound, New Zealand from 1995 to 2008. Model selection was conducted to estimate recruitment and population growth rates. There were similar levels of support for three different models, each reflecting distinct trends in recruitment. Modeling yielded low overall estimates of recruitment PF-02341066 nmr (0.0249, 95% CI: 0.0174–0.0324) and population growth rate (0.9642, 95% CI: 0.9546–0.9737). The TSCR rate of population decline was consistent with an estimate derived from trends in abundance (lambda = 0.9632, 95% CI: 0.9599–0.9665). The TSCR model selection confirmed the influence of a decline in the survival of calves (<1 yr old) since 2002 for population trends. However, TSCR population growth rates did not exceed 1 in any year between 1995 and 2008, indicating the population was declining prior to 2002. A separate reduction in juvenile survival (1–3 yr old) prior

to 2002 was identified as a likely contributing factor in the population decline. Thus, TSCR modeling indicated the potential cause of the population decline in Doubtful Sound: cumulative impacts on individuals ZD1839 ic50 <3 yr old resulting in a reduced recruitment. "
“Five years of behavioral observations revealed significant effects of high air temperatures and breeding site topography on the mating system of South American sea MCE lions in Peru. Unlike most polygynous mammals that defend females or fixed territories, male sea lions in Peru maintained positions along the shoreline where females passed each day to thermoregulate, and where most copulations occurred. Sex ratios (1 male per 17 females)

and male mating success were extremely skewed (14% of males achieved 50% of the copulations, and 25% of them did not copulate at all). The mass daily movements of females toward the water and cool substrate of the shoreline, along with a highly skewed sex ratio, accentuated the difficulty for males to monopolize and restrict female movements. Females moved freely and chose their mates, unlike in temperate regions of their range where male South American sea lions control groups of females or access to tide pools. Our observations indicate that the South American sea lion in Peru has a lek-like breeding system. This is a rare alternative to the common male strategies of defending females and resources, and is likely an evolutionary product of their highly skewed sex ratio, protracted breeding season, and the extreme subtropical climate where they breed.

The diagnostic sonography of the peripheral nervous system is a r

The diagnostic sonography of the peripheral nervous system is a rapidly

evolving and constantly expanding imaging field in the last years, especially in patients presenting with signs of polyneuropathy. We report for the first time a correlation of the sonographic and electrophysiological findings in a patient with MMN. We draw the attention on the usefulness of ultrasonography for detecting and diagnosing segmental lesions of the peripheral nerves in MMN and other immune mediated neuropathies, especially in BTK inhibitor cases where a nervous segment cannot be easily explored in terms of inching technique. “
“We present a case of intraventricular fat deposits discovered on magnetic resonance imaging and computed tomography in the absence of a ruptured dermoid cyst. The patient is a 25-year-old right-handed African-American female who previously had lumboperitoneal (LP) shunt placement for pseudotumor cerebri. She had multiple shunt-related complications that required two revisions as well as an incisional hernia repair. We suggest Pritelivir supplier that the presence of fat in her ventricles is due to retrograde flow from abdominal fat during the time when the shunt tip was located in the anterior abdominal wall as opposed to her peritoneum. Unlike ventriculoperitoneal shunts, LP shunts do not contain valves, making retrograde passage of fat possible when abdominal pressures exceed lumbar subarachnoid

pressures. The presence of these small amounts of fat in the ventricles is unlikely to cause any further symptoms in this patient, but monitoring for any MCE blockage of cerebrospinal fluid flow or neurologic deterioration would be advisable. “
“A 27-year-old male presented with progressive ascending myelopathy leading to tetraparesis. Magnetic resonance imaging of the cervical spine showed dilated perimedullary veins and spinal

cord edema. Catheter angiography demonstrated a direct carotid-cavernous fistula (CCF) with prominent pontomesencephalic and perimedullary venous drainage. Successful coil embolization of the fistula was performed with improvement of the patient’s symptoms. To our knowledge, no case of a direct CCF with perimedullary drainage has been previously reported. “
“In patients with mild cognitive impairment (MCI), poor performances on delayed recall and executive function are risk factors of progression to dementia. The aim of the present study was to clarify neural correlates of these neuropsychological deficits. Thirty patients with amnestic MCI and 15 control subjects underwent neuropsychological tests including three-word delayed recall, visual delayed recall of Rey complex figure (RCF), and two-relational reasoning of Raven’s colored progressive matrices (RCPM) with a 18F-fluorodeoxyglucose (FDG)-position emission tomography (PET) measurement of resting state.

Therefore, we believe that the article published by Connolly and

Therefore, we believe that the article published by Connolly and coworkers addresses an important aspect of the liver microenvironment regarding the role of DCs in fibrosis progression. Additional studies are warranted to clarify the functional and potentially clinically relevant contribution of DCs for the progression or regression of liver fibrosis. “
“Niemann-Pick disease, types A and B, are lipid storage disorders caused by complete or partial deficiency of a lysosomal enzyme, acid sphingomyelinase. The disorders are inherited in an autosomal recessive pattern and result

in the accumulation of sphingomyelin in various organs including the spleen, liver, lungs, bone marrow and brain. The infantile INCB024360 cost variant (type A) usually presents within the first year of life with failure to thrive, hepatomegaly and neurological symptoms, sometimes resulting in liver failure with ascites and jaundice. Most babies die at 2–3 years of age. In Niemann-Pick type B disease, the diagnosis is usually made early in childhood because of failure to thrive and the development find more of hepatosplenomegaly. Patients then develop progressive neurological symptoms and may have deteriorating pulmonary

function because of foam cell infiltrates. Most patients die in childhood. An important clue to the diagnosis of Niemann-Pick disease, types A and B, is the presence of large foamy macrophages in the bone marrow or liver. In most patients, the diagnosis is confirmed by genetic tests for mutations in the SMPD1 gene or by the demonstration of low acid sphingomyelinase 上海皓元医药股份有限公司 activity in peripheral blood white cells or in cultured fibroblasts. At present, there is no specific treatment for Niemann-Pick disease. The patient illustrated below was 15-month-old female who presented with a 4-month history of hepatosplenomegaly and failure to thrive. She was the product

of a full-term normal pregnancy but her parents were consanguineous and a previous baby had died in the neonatal period. The parents described anorexia and recurrent pneumonia but no vomiting or diarrhea. The baby had a weight and height below the 5th percentile for age. Physical examination revealed marked hepatosplenomegaly but no ascites. Liver enzymes were abnormal with elevation of aspartate aminotransferase (311 u/l), alanine aminotransferase (251 u/l), gamma-glutamyltransferase (77 u/l) and alkaline phosphatase (724 u/l). She had a normal complete blood count, coagulation studies and serum albumin. A computed tomography scan of the chest revealed typical reticulonodular infiltrates in the upper lobes of both lungs (Figure 1). A percutaneous liver biopsy showed lipid-laden foam cells (Figure 2). The diagnosis of Niemann-Pick disease was confirmed by the demonstration of low acid sphingomyelinase activity in peripheral blood white cells.