The Role of Adenovirus in Hepatitis of Unknown Etiology Among Children in Turkey, July 2022 to January 2023.

BACKGROUND: In the first half of 2022, the increase in cases of severe acute hepatitis in children with no epidemiological link and unknown cause has aroused concern worldwide. In this study, we aimed to determine the frequency of adenovirus in children with hepatitis of unknown origin in Turkey. METHODS: In this study, which was conducted with the participation of 16 centers from Turkey, between July 2022 and January 2023, 36 pediatric patients under the age of 18 who met the definition of acute hepatitis were included. RESULTS: Twenty (55,6%) of patients were female and 16 (44,4%) were male, with a mean age of 41.55 ± 39.22 (3-192) months. The most common symptoms were fever (66.7%), weakness (66.7%), loss of appetite (66.7%), fatigue (61.1%), vomiting (61.1%), and diarrhea (47.2%). Six (16 %) children had acute hepatic failure. Eight of the 15 children (46%) had respiratory viral infections (rhinovirus/enterovirus, n = 4; rhinovirus + parainfluenza, n = 1; parainfluenza, n = 1, SARS-CoV-2 n = 2). Adenovirus was detected in 16 (44%) patients. Adenovirus and coinfections were detected in 7 patients. History of diarrhea and fatigue in the last month was significantly higher in the adenovirus group ( P = 0.023 and 0.018). One child who had both adenovirus and rhinovirus infection required liver transplantation; all others recovered with supportive care. CONCLUSION: There were no deaths in our series; however, the number of cases, etiology, and severity of the clinical course may have differed between countries owing to differences in case definitions, laboratory facilities, or regional genetic differences.

Growth, tolerance and safety outcomes with use of an extensively hydrolyzed casein-based formula in infants with cow's milk protein allergy.

Objective: To evaluate growth, tolerance and safety outcomes with use of an extensively hydrolyzed casein-based formula (eHCF) in infants with cow's milk protein allergy (CMPA). Methods: A total of 226 infants (mean ± SD age: 106.5 ± 39.5 days, 52.7% were girls) with CMPA who received eHCF comprising at least half of the daily dietary intake were included. Data on anthropometrics [weight for age (WFA), length for age (LFA) and weight for length (WFL) z-scores] were recorded at baseline (visit 1), while data on infant feeding and stool records, anthropometrics and Infant Feeding and Stool Patterns and Formula Satisfaction Questionnaires were recorded at visit 2 (on Days 15 ± 5) and visit 3 (on Days 30 ± 5). Results: From baseline to visit 2 and visit 3, WFA z-scores (from -0.60 ± 1.13 to -0.54 ± 1.09 at visit 2, and to -0.44 ± 1.05 at visit 3, p < 0.001) and WFL z-scores (from -0.80 ± 1.30 to -0.71 ± 1.22 at visit 2, and to -0.64 ± 1.13 at visit 3, p = 0.002) were significantly increased. At least half of infants never experienced irritability or feeding refusal (55.7%) and spit-up after feeding (50.2%). The majority of mothers were satisfied with the study formula (93.2%), and wished to continue using it (92.2%). Conclusions: In conclusion, eHCF was well-accepted and tolerated by an intended use population of infants ≤ 6 months of age with CMPA and enabled adequate volume consumption and improved growth indices within 30 days of utilization alongside a favorable gastrointestinal tolerance and a high level of parental satisfaction.

Long-Term Follow-Up and Outcome of Pediatric Acute Pancreatitis: A Multicenter Study.

OBJECTIVE: Over the past decades, the incidence of acute pancreatitis is increasing, but the progression of acute recurrent pancreatitis and chronic pancreatitis is still not well documented in children. The aim of this multicenter study is to delineate the changes that occur in a certain time period in the course of childhood pancreatitis. MATERIALS AND METHODS: The data of consecutive patients hospitalized with acute pancreatitis between 2010 and 2017 in 4 different pediatric gastroenterology units were reviewed. The clini- cal characteristics of the disease were defined. RESULTS: A total of 165 patients (55.2% female) were included. Over the years, the rate of acute pancreatitis admissions increased while the duration of hospitalization decreased (P < .05). Nearly two-thirds of the patients with acute pancreatitis resolved spontaneously, 30.9% and 4.3% of the cases developed acute recurrent pancreatitis and chronic pancreatitis, respectively. Furthermore, 27.4% patients with acute recurrent pancreatitis progressed to chronic pancre- atitis, and eventually, 12.7% of cases developed chronic pancreatitis within 3-4 years. Local complications developed in 13.3% of the patients with pancreatitis in this cohort. CONCLUSION: The result of this study confirmed the increased incidence of acute pancreatitis in recent years. Conversely, the length of hospital stay decreased over the years. Patients with pancreaticobiliary abnormalities or genetic risk factors had a higher rate of progression to acute recurrent pancreatitis or chronic pancreatitis. Therefore, genetic testing and radiological imaging should be considered early in the follow-up of patients with acute pancreatitis having risk factors for progression to acute recurrent pancreatitis/chronic pancreatitis.

Nutritional characteristic of children with inflammatory bowel disease in the nationwide inflammatory bowel disease registry from the Mediterranean region.

BACKGROUND/OBJECTIVES: We analyzed the nationwide pediatric inflammatory bowel disease (PIBD) registry (1998-2016), to evaluate the nutritional status at the time of diagnosis. SUBJECTS/METHODS: Nine types of nutritional status by the combination of weight-for-length (<2 years)/body mass index (>2 years) and length/height-for-age with three categories (<-2, -2 to 2, and >2 SD) were described. Malnutrition was defined by WHO criteria. Univariate and multivariate regression analysis was used to identify risk factors for malnutrition. RESULTS: In total, 824 IBD patients (498 Ulcerative colitis (UC); 289 Crohn's Disease (CD); 37 Indeterminate Colitis (IC); 412 male; the median age 12.5 years) were eligible. The prevalence of eutrophy, wasting/thinness, stunting, overweight, tall stature, concurrent wasting/thinness and stunting, tall stature with overweight, tall stature with wasting/thinness, and short stature with overweight were 67.4%, 14.9%, 6.6%, 3.1%, 3.2%, 3.3%, 1.1%, 0.4%, and 0.1%, respectively. The prevalence of malnutrition was 32.7%, indicating a higher prevalence in CD (p < 0.001). Incidence of overweight was less common in the CD than UC and IC (p < 0.001). Multivariate analysis revealed that age of onset (>10 years), prepubertal stage, severe disease activity, perianal involvement, and high C reactive protein level were independently associated with malnutrition in pediatric IBD. CONCLUSION: We showed the frequency of nutritional impairment in PIBD. The percentage of overweight subjects was lower than the other studies. The age of onset, disease activity, CRP level, perianal involvement, and pubertal stage were associated with a higher risk for developing malnutrition. Our results also confirmed that CD patients are particularly vulnerable to nutritional impairment. CLINICAL TRIAL NUMBER: ClinicalTrials.gov Identifier: NCT04457518.

Familial Mediterranean Fever Mutation Analysis in Pediatric Patients With Inflammatory Bowel Disease: A Multicenter Study.

BACKGROUND: The aim of the study was to evaluate familial Mediterranean fever (FMF) mutation analysis in pediatric patients with inflammatory bowel disease (IBD). The relation between MEFV mutations and chronic inflammatory diseases has been reported previously. METHODS: Children with IBD (334 ulcerative colitis (UC), 224 Crohn's disease (CD), 39 indeterminate colitis (IC)) were tested for FMF mutations in this multicenter study. The distribution of mutations according to disease type, histopathological findings, and disease activity indexes was determined. RESULTS: A total of 597 children (mean age: 10.8 ± 4.6 years, M/F: 1.05) with IBD were included in the study. In this study, 41.9% of the patients had FMF mutations. E148Q was the most common mutation in UC and CD, and M694V in IC (30.5%, 34.5%, 47.1%, respectively). There was a significant difference in terms of endoscopic and histopathological findings according to mutation types (homozygous/ heterozygous) in patients with UC (P < .05). There was a statistically significant difference between colonoscopy findings in patients with or without mutations (P = .031, P = .045, respectively). The patients with UC who had mutations had lower Pediatric Ulcerative Colitis Activity Index (PUCAI) scores than the patients without mutations (P = .007). CONCLUSION: Although FMF mutations are unrelated to CD patients, but observed in UC patients with low PUCAI scores, it was established that mutations do not have a high impact on inflammatory response and clinical outcome of the disease.

The frequency of and factors affecting functional gastrointestinal disorders in infants that presented to tertiary care hospitals.

This study aimed to determine the prevalence of infantile functional gastrointestinal disorders (FGIDs) based on Rome IV diagnostic criteria, and to determine the associated patient demographic and nutritional characteristics. A total of 2383 infants aged 1-12 months which were evaluated by 28 general pediatricians and pediatric gastroenterologists on the same day at nine tertiary care hospitals around Istanbul, Turkey, between November 2017 and March 2018, were included in the study. Patients included consulted the pediatric outpatient clinics because of any complaints, but not for vaccines and/or routine well child follow-ups as this is not part of the activities in the tertiary care hospitals. The patients were diagnosed with FGIDs based on Rome IV diagnostic criteria. The patients were divided into a FGID group and non-FGID group, and anthropometric measurements, physical examination findings, nutritional status, risk factors, and symptoms related to FGIDs were evaluated using questionnaires. Among the 2383 infants included, 837 (35.1%) had ≥1 FGIDs, of which 260 (31%) had already presented to hospital with symptoms of FGIDs and 577 (69%) presented to hospital with other symptoms, but were diagnosed with FGIDs by a pediatrician. Infant colic (19.2%), infant regurgitation (13.4%), and infant dyschezia (9.8%) were the most common FGIDs. One FGID was present in 76%, and ≥2 FGIDs were diagnosed in 24%. The frequency of early supplementary feeding was higher in the infants in the FGID group aged ≤6 months than in the non-FGID group (P = 0.039).Conclusion: FGIDs occur quite common in infants. Since early diversification was associated with the presence of FGIDs, nutritional guidance and intervention should be part of the first-line treatment. Only 31% of the infants diagnosed with a FGID were presented because of symptoms indicating a FGID. What is Known: • The functional gastrointestinal disorders (FGIDs) are a very common disorder and affect almost half of all infants. • In infants, the frequency of FGIDs increases with mistakes made in feeding. When FGIDs are diagnosed in infants, nutritional support should be the first-line treatment. What is New: • This study shows that only a third of children presented to hospital because of the symptoms of FGIDs, but pediatricians were able to make the diagnosis in suspected infants after appropriate evaluation. • The early starting of complementary feeding (<6 months) is a risk factor for the development of FGIDs.

Broadly effective metabolic and immune recovery with C5 inhibition in CHAPLE disease.

Complement hyperactivation, angiopathic thrombosis and protein-losing enteropathy (CHAPLE disease) is a lethal disease caused by genetic loss of the complement regulatory protein CD55, leading to overactivation of complement and innate immunity together with immunodeficiency due to immunoglobulin wasting in the intestine. We report in vivo human data accumulated using the complement C5 inhibitor eculizumab for the medical treatment of patients with CHAPLE disease. We observed cessation of gastrointestinal pathology together with restoration of normal immunity and metabolism. We found that patients rapidly renormalized immunoglobulin concentrations and other serum proteins as revealed by aptamer profiling, re-established a healthy gut microbiome, discontinued immunoglobulin replacement and other treatments and exhibited catch-up growth. Thus, we show that blockade of C5 by eculizumab effectively re-establishes regulation of the innate immune complement system to substantially reduce the pathophysiological manifestations of CD55 deficiency in humans.

Assessment of Hearing Function in Children with Inflammatory Bowel Disease.

OBJECTIVES: Inflammatory bowel disease (IBD), a chronic pathology that affects many organ systems, appears after dysregulated immune response in genetically predisposed patients. Inner organ involvement has been shown in various autoimmune diseases because of its immunosensitivity. In this study, we aimed at showing sensorineural hearing loss (SNHL) as a result of possible subclinical inflammation in patients with IBD during the remission period. MATERIALS AND METHODS: We included 32 children with IBD and 31 healthy volunteers with comparable sex and age. Detailed ear-nose-throat examination was conducted for all, and patients were excluded if they had a history of ear infectionor trauma. Thereafter, the results of pure tone audiometry (PTA), high-frequency audiometry, and distortion product otoacoustic emissions testing were compared between the groups. RESULTS: There were no differences in terms of age, sex, and PTA values between controls and children with IBD. No statistical differences were found between responses at 250; 500; 2,000; 4,000; DP1000; DP1400; DP2000; DP2800;and DP4000 Hz as well as the signal-to-noise ratio (SNR) at 1,000 Hz when the controls and children with IBD (p>0.05 for all) were compared. However, the mean responses at 1,000; 8,000; 10,000; 12,500; 16,000; SNR1400; SNR2000; SNR2800; and SNR4000Hz of the children with IBD were significantly higher than those of the controls (p<0.05 for all). CONCLUSION: Initial SNHL appears at high frequencies in pediatric patients with IBD.

Helicobacter pylori infection in children with celiac disease: Multi-center, cross-sectional study.

BACKGROUND: An inverse association has been suggested between celiac disease (CD) and Helicobacter pylori (Hp) infection in children; however, there are inconsistent data. The purpose of this multi-center study is to evaluate the association between Hp and CD in childhood. METHODS: Children who underwent endoscopy between July 2016 and November 2017 in four pediatric gastroenterology centers were included in the study. Patients with a history of previous Hp eradication, antibiotic or acid-suppressive drug therapy in the last 4 weeks, and any underlying chronic disease were excluded. The presence of Hp infection was confirmed by both histopathology and the rapid urease test. The ones who had the diagnosis of CD were compared with the children who underwent endoscopy during the same period and had another diagnosis. Duodenal histopathology of children with CD was categorized according to the modified Marsh classification. RESULTS: Of 3056 endoscopies performed in the study period, 2484 cases were eligible for the study. A total of 482 CD patients (mean age: 9.71 ± 4.63 years, 58.5% girls) and 2060 controls (mean age: 9.92 ± 4.66 years, 54.6% girls) were included in the study. The rate of Hp infection was significantly lower in CD group (26.3% vs 50.1%, P < .01). The difference was prominent even in children younger than 6 years old (P < .01). There was no correlation between Hp infection and the modified Marsh scores in CD (P > .05). CONCLUSION: In this cross-sectional study, where Hp infection is common even in the pediatric population, the frequency of Hp infection was significantly lower in children with CD compared with the controls. Systematic cohort studies are necessary to clarify causal association between Hp infection and the development of celiac disease.

Evaluation of autonomic nervous system functions in frame of heart rate variability in children with inflammatory bowel disease in remission.

Aghdasi-Bornaun H, Kutluk G, Keskindemirci G, Öztarhan K, Dedeoglu R, Yilmaz N, Tosun Ö. Evaluation of autonomic nervous system functions in frame of heart rate variability in children with inflammatory bowel disease in remission. Turk J Pediatr 2018; 60: 407-414. Heart Rate Variability (HRV) is one of the reliable and noninvasive parameters to evaluate autonomic control of the cardiovascular system in patients. The aim of our study was to assess autonomic function in pediatric patients with inflammatory bowel disease (IBD) in remission using Power Spectral Analysis of HRV. Autonomic cardiovascular function was evaluated by time and frequency-domain indexes of spontaneous heart rate in 36 IBD children patients in remission phase and 36 sex and age matched healthy controls. Twenty children with Ulcerative Colitis (UC) and 16 patients with Crohn`s disease (CD) were diagnosed according to their history, physical and laboratory examination, endoscopic, histopathological and radiological findings of upper and lower gastrointestinal system. Significant decrease was observed at HRV parameters in IBD patients when compared with control group. These differences was found in some of time domain parameters (NNmean, SDNNtotal, SDNNday) and frequency domain parameters (TP, LF,VLF) (p < 0.05). Also, there was a significant higher minimum heart rate ratio (p < 0.04) in patients in comparison to the control group. In the IBD group, there was a relative tendency for parasympathetic suppression and sympathetic predominance which reflects an autonomic dysfunction. This imbalance has a circadian rhythm and it is more obvious during the day. These observations may suggest a previously unrecognized role of chronic inflammation for autonomic modulation in IBD.

Prolonged P-Wave and QT Dispersion in Children with Inflammatory Bowel Disease in Remission.

Objectives. Ulcerative colitis (UC) and Crohn's disease (CD) are chronic inflammatory bowel diseases (IBD) with unclear underlying aetiologies. Severe cardiac arrhythmias have been emphasised in a few studies on adult IBD patients. This study aimed to investigate the alteration of the P-wave and QT interval dispersion parameters to assess the risk of atrial conduction and ventricular repolarisation abnormalities in pediatric IBD patients. Patients and Methods. Thirty-six IBD patients in remission (UC: 20, CD: 16) aged 3-18 years and 36 age- and sex-matched control patients were enrolled in the study. Twelve-lead electrocardiograms were used to determine durations of P-wave, QT, and corrected QT (QTc) interval dispersion. Transthoracic echocardiograms and 24-hour rhythm Holter recordings were obtained for both groups. Results. The P-wave dispersion, QT dispersion, and QTc interval dispersion (Pdisp, QTdisp, and QTcdisp) were significantly longer in the patient group. The mean values of Pminimum, Pmaximum, and QTcminimum were significantly different between the two groups. The echocardiography and Holter monitoring results were not significantly different between the groups. Furthermore, no differences in these parameters were detected between the CD and UC groups. Conclusion. Results suggest that paediatric IBD patients may carry potential risks for serious atrial and ventricular arrhythmias over time even during remission.

HAX-1 deficiency: Characteristics of five cases including an asymptomatic patient.

BACKGROUND: Mutations in the HAX-1 gene cause an autosomal recessive form of severe congenital neutropenia (SCN), which particularly manifests with recurrent skin, lung and deep tissue infections from the first few months of life. OBJECTIVE: We retrospectively evaluated the clinical and laboratory findings of the patients diagnosed with SCN carrying HAX1 gene mutations. METHODS: A total of five patients with SCN, carrying a HAX1 gene mutation, were evaluated in terms of clinical and laboratory findings. Mutation analysis of the candidate genes (HAX1, ELANE and CSF3R) was performed. RESULTS: All of the patients lived in Turkey; four of them were of Kurdish origin and one was Turkish. Of the five patients, three were girls and two were boys, and the mean age of the patients was 8.8 years old (range 4-15 years). The mean age of diagnosis was 25.8 months (range 2 months-5 years). The infections diagnosed included recurrent gingivitis, stomatitis, and skin and soft tissue abscesses. Developmental retardation and epilepsy were present in only one patient, whereas speech retardation was present in two. All of our patients had a HAX1 mutation, and are still alive and none of them has shown malignant transformation yet. CONCLUSION: Complete blood count should be performed and absolute neutrophil count should be evaluated in patients with recurrent severe infections. In the event that neutropenia is detected, they should be investigated in terms of SCN and mutation analysis should be performed.

Sequential therapy versus standard triple therapy for Helicobacter pylori eradication in children: any advantage in clarithromycin-resistant strains?

OBJECTIVE: There has been a marked decrease in the eradication rates of Helicobacter pylori infection with standard triple therapy worldwide. Hence, sequential therapy has gained attention as a promising treatment during the last few years. This study was carried out to compare the efficacy of sequential versus standard triple therapy in the context of clarithromycin (CLA) resistance. MATERIALS AND METHODS: In this study, children between 3 and 18 years of age, who had documented H. pylori infection, were randomized to receive either standard triple or sequential therapy. H. pylori eradication was ascertained using the C-urea breath test 4-6 weeks after the completion of the treatment. Real-time PCR was performed on gastric biopsy samples for assessment of CLA resistance. RESULTS: In all, 148 children (median age: 12.18±3.51 years) were recruited randomly into the study. The intention-to-treat eradication rates were 50% (37/74) for the sequential treatment group and 52.7% (39/74) for the standard triple treatment group (P=0.87). A total of 136 children completed the study. The per-protocol eradication rates were 56% (37/66) and 55.7% (39/70) for sequential and standard triple therapy groups, respectively. CLA resistance was assessed and 113 children were included in the final analysis. Of 113 participants, 53 were in the sequential treatment group and 60 were in the standard triple treatment group. The success rates of the respective therapies (29/53=54.7% in sequential, 33/60=55% in standard therapy) were similar (P=0.98). CLA resistance was detected in 29 (25.7%) of the patients. Eradication rates with sequential therapy in CLA susceptible and resistant cases were 60.5% (23/38) and 40% (6/15), respectively (P=0.23). The corresponding figures for the standard triple treatment group were 63% (29/46) and 28.6% (4/14) (P=0.033). Although a higher eradication rate was observed in CLA-resistant cases with sequential therapy, the difference did not reach statistical significance (P=0.69). CONCLUSION: In this study, standard triple treatment failed to eradicate H. pylori infection in the majority of the children, and sequential therapy offered only a small advantage over standard triple therapy in the eradication of CLA-resistant strains.

Current diagnosis and management of simple hepatic cysts detected prenatally and postnatally.

PURPOSE: The optimal management of simple hepatic cysts (SHCs) remains controversial. A retrospective review was carried out to evaluate the clinical presentation and surgical consideration of children with SHCs and outcome of management at our institution. METHODS: We performed a retrospective review of all patients diagnosed with SHCs between January 1993 and May 2013. Patient variables included prenatal findings, age, gender, presenting symptoms, signs and diagnostic modality. Operative management and nonoperative follow-up were abstracted. RESULTS: The 67 patients comprised 42 females and 25 males: 16 had prenatally detected, and 51 had postnatally detected hepatic cysts with a mean follow-up of 3.2 years (10 months-12 years). The average age was 5.9 years (0-17 years). Ten patients (six females and four males) were treated surgically. Fifty-seven asymptomatic patients were observed. Of those 57 patients, 9 (17%) had enlarged cysts, and 1 (2%) had clinical signs. The average enlargement time was 36 months (6-67 months). The cysts occurred in the right lobe in 47 patients, in the left lobe in 17 patients and in both lobes in 3 patients. The median cyst size for the asymptomatic cases was 2.1 cm (1.5-6 cm) and 13.7 cm (8-25 cm) for the surgery group. A statistically significant difference in cyst size was found between these two groups (p < 0.05). Prenatally detected cysts (n = 5) were subjected to surgery postnatally at an average age of 1.5 months (1 week-6 months). The main indication was the compressive effect of the cyst on the surrounding area. There was one case of hepatomegaly found to be causing pressure symptoms, two cases of respiratory distress, one case of hydroureteronephrosis, and one case of jaundice due to compression of the biliary system. Five patients underwent surgery in the postnatally detected group, and the main indication was a complication of the cyst or progressive enlargement. Symptoms included abdominal discomfort and pain in all five patients: one case exhibited a hemorrhage within the cyst, one case displayed a rupture with possible peritonitis, one case had a 25-cm cyst leading to secondary infection, and two cases were admitted with progressive enlargement with clinical symptoms. CONCLUSION: Conservative management of SHCs is practical. Surgical excision is recommended in patients with larger cysts, causing pressure symptoms, or if the cyst size does not seem to involute with intractable abdominal pain or becomes complicated in the follow-up period.

What is the diagnostic utility of endoscopic scoring systems in children?

BACKGROUND/AIMS: The aim of this study was to evaluate the consistency of the Savary-Miller, the Hetzel-Dent and the Los Angeles endoscopic classification systems and to compare them with the esophageal histopathology in children. MATERIAL AND METHODS: Children between the ages of 5-17 years who underwent esophagogastroduodenoscopy were included in the study. The endoscopic reports and the still images of the esophagus were reclassified by the same gastroenterologist according to the Savary-Miller, Hetzel- Dent and Los Angeles scoring systems. The esophageal biopsies were also reevaluated by the same pathologist and the consistency between endoscopic and histopathologic esophagitis was evaluated. RESULTS: A total of 113 out of 192 pediatric patients were included in the study. Seventy-three patients (64.6%) had esophagitis according to the Hetzel-Dent classification, whereas only 20 (17.7%) patients were defined as having esophagitis according to the other two classification systems. The consistency between the Savary-Miller and Los Angeles classifications was excellent (κ: 0.92) but the agreement between the Hetzel-Dent and Savary-Miller and between the Hetzel-Dent and Los Angeles classifications were poor. A total of 82 patients (72.6%) had histopathological esophagitis, and there was a weak consistency between all 3 endoscopic scoring systems and the histopathology. CONCLUSIONS: Since pediatric patients have milder esophagitis than in adults, the use of endoscopic scoring systems developed for adults seems to be inapplicable for children. The inclusion of minimal endoscopic changes in endoscopic scoring systems by using more sensitive and novel endoscopic techniques would increase the sensitivity of these scoring systems in children.

Niemann - Pick disease associated with hemophagocytic syndrome.

Hemophagocytic lymphohistiocytosis (HLH) is a disease characterized by phagocytosis of blood cells by macrophages within the lymphoreticular tissue. It can develop secondary to some diseases or be familial as a result of genetic mutations. Niemann-Pick disease (NPD) is a very rare lipid storage disease. A three-month-old girl presented with high fever (39°C), abdominal distension and paleness. The parents were consanguineous. The liver and spleen were palpable 10 cm and 11 cm below the costal margins, respectively. Bicytopenia (Hb: 5.5 g/dl, platelet: 77000/mm3), hypertriglyceridemia (351 mg/dl), hyperferritinemia (>1500 ng/dl) and hypofibrinogenemia (120 mg/dl) were detected. Bone marrow aspiration demonstrated foam cells and hemophagocytosis by macrophages and Niemann-Pick cells. Lysosomal sphingomyelinase activity was 0.24 nmol/h/mg/protein (normal: 0.86-2.8). Due to the parents' refusal of further evaluation, the nature of HLH as primary or secondary could not be determined. To the best of our knowledge, this is the first case of NPD associated with HLH and the first demonstration of hemophagocytosis by Niemann-Pick cells.

Penicillin sensitivity among hospital nurses without a history of penicillin allergy.

AIM: This paper reports a study to document the prevalence of penicillin sensitivity among nurses who had no previous history of sensitivity and worked in an inner city hospital in Turkey. BACKGROUND: Frequent and prolonged exposure to any drug including penicillin may increase the risk of hypersensitivity to these drugs. Local administration of drugs increases the likelihood of sensitization. Nurses are the main group of healthcare workers who prepare and administer antibiotics to patients, so they may be at highest risk for sensitization. Skin testing with major and minor determinants of penicillin has proved useful for identifying penicillin-specific IgE antibodies in sensitized individuals. METHOD: Skin prick tests were performed with benzyl penicilloyl polylysine (PPL) and a mixture of minor antigenic determinants (MDM) solution with 83 nurses who had been handling beta-lactam antibiotics. The study was conducted in 2004. FINDINGS: Ten nurses (12%) had positive skin reactions to penicillin antigens. Six were sensitive to PPL (7 x 2%) and four were sensitive to MDM (4 x 8%). CONCLUSION: Occult sensitization to beta-lactam antibiotics, without symptoms, may develop in hospital nurses. It is possible that these healthcare workers might be at increased risk of hypersensitivity reactions should they be exposed to beta-lactams, administered for therapeutic purposes. Further research is needed to explore this important issue.

Nutritional vitamin B12 deficiency in hospitalized young children.

The authors sought to determine prevalence, social, economic, and dietary patterns of young children (n = 20) identified as having vitamin B12 deficiency anemia after admission to their hospital in the last 3 years. The diagnosis of vitamin B12 deficiency was based on symptoms and clinical findings, findings on peripheral blood films and bone marrow aspirates, and serum levels of vitamin B12. The children had been exclusively breast-fed without any animal food supplementation. Serum vitamin B12 levels were also measured in the sera of mothers and found to be low. The authors concluded that vitamin B12 deficiency might be an important health problem among children of mothers who do not consume animal foods adequately.

Severe iron-deficiency anemia among hospitalized young children in an urban hospital.

The aim of this study was to investigate the prevalence and some characteristics of young children with severe iron-deficiency anemia hospitalized in an urban hospital. All of the young children (aged 7-24 months) hospitalized in a city hospital during the last 3 years were searched for iron-deficiency anemia and those with severe anemia were retrospectively evaluated. During this period, 3117 young children were hospitalized and 61.6% of them had a hemoglobin value of less than 11 g/dL and 52 children (2.7%) had severe iron-deficiency anemia (hemoglobin < 6 g/dL). It was concluded that iron-deficiency anemia is still an important health problem among young children in our society.