Lipid storage disease in 4 sibling superb birds-of-paradise (Lophorina superba).

Pedigree analysis, clinical, gross, microscopic, ultrastructural, and lipidomic findings in 4 female superb bird-of-paradise (SBOP, Lophorina superba) siblings led to the diagnosis of a primary inherited glycerolipid storage disease. These birds were the offspring of a related breeding pair (inbreeding coefficient = 0.1797) and are the only known SBOPs to display this constellation of lesions. The birds ranged from 0.75 to 4.3 years of age at the time of death. Two birds were euthanized and 1 died naturally due to the disease, and 1 died of head trauma with no prior clinical signs. Macroscopic findings included hepatomegaly and pallor (4/4), cardiac and renal pallor (2/4), and coelomic effusion (1/4). Microscopic examination found marked tissue distortion due to cytoplasmic lipid vacuoles in hepatocytes (4/4), cardiomyocytes (4/4), renal tubular epithelial cells (4/4), parathyroid gland principal cells (2/2), exocrine pancreatic cells (3/3), and the glandular cells of the ventriculus and proventriculus (3/3). Ultrastructurally, the lipids were deposited in single to coalescing or fused droplets lined by an inconspicuous or discontinuous monolayer membrane. Lipidomic profiling found that the cytoplasmic lipid deposits were primarily composed of triacylglycerols. Future work, including sequencing of the SBOP genome and genotyping, will be required to definitively determine the underlying genetic mechanism of this disease.

Clinicopathological features of progressive supranuclear palsy with asymmetrical atrophy of the superior cerebellar peduncle.

Progressive supranuclear palsy (PSP) can be diagnosed despite the presence of asymmetrical parkinsonism depending on the clinical diagnostic criteria. Some studies have reported that atrophy of the superior cerebellar peduncle (SCP) is more frequent in PSP than in Parkinson's disease. There have also been reports of PSP cases with an asymmetrically atrophic SCP. Therefore, we analyzed 48 specimens from consecutive autopsy cases that were neuropathologically diagnosed as PSP to investigate the laterality of brain lesions, including the SCP. We measured the width of the SCP and evaluated the laterality of atrophy. We semi-quantitatively evaluated neuronal loss, atrophy/myelin pallor, and tau pathology in three steps. Asymmetrical atrophy of the SCP was present in seven (14.6%) of 48 cases. The atrophic side of the SCP corresponded to the dominant side of the tau pathology in the cerebellar dentate nucleus. It was opposite to the dominant side of the myelin pallor and tau pathology in the red nucleus and of the tau pathology in the central tegmental tract and inferior olivary nucleus, coinciding with the neurologically systematic anatomy of the Guillain-Mollaret triangle. Neurodegeneration of PSP can progress asymmetrically from one side to the initially intact side in PSP with an initial predominance of Richardson's syndrome, progressive gait freezing, ocular motor dysfunction, parkinsonism, or corticobasal syndrome. To our knowledge, no previous study has reported asymmetrical PSP neuropathology; this is the first study to report the presence of PSP cases with asymmetrical SCP atrophy and systematically asymmetrical degeneration of the Guillain-Mollaret triangle.

Button Battery Ingestion in Children (PilBouTox®): A Prospective Study Describing the Clinical Course and Identifying Factors Related to Esophageal Impaction or Severe Cases.

In this study, we aimed to identify the factors related to esophageal impaction following button battery (BB) ingestion in children. PilBouTox, a prospective multicentric observational cohort study, was conducted from French Poison Control Centers between June 1, 2016 and May 31, 2018. Children (0-12 years old) with BB ingestion were included. After ingestion, patients were monitored for 21 days or more if they remained symptomatic (maximum 1 year). Causes of ingestion, clinical manifestations, medical management, and the outcomes were recorded. In total, 415 patients were included; among them, 35 had esophageal impaction and 14 had severe complications or died. Seven symptoms were closely related (relative risk (RR) > 30) to esophageal impaction: anorexia, drooling, dyspnea, fever, hemodynamic instability, pallor, and pain. Furthermore, BBs > 15 mm were related to esophageal impaction (RR = 19, CI95% [4.1; 88]). The absence of initial symptoms was a protective factor for esophageal impaction (RR = 0.013, CI95% [0.002; 0.1]). Nine symptoms were closely related (RR > 30) to major effects and death: dyspnea, cough, dysphagia, drooling, fever, hemodynamic instability, pain, pallor, and vomiting. Seven symptoms were related to esophageal impaction and their rapid recognition could help to ensure that the patient is taken to a health care facility. Nine factors were related to the major effects of BB ingestion. We recommended an X-ray as soon as possible to determine the position of the BB.Trial Registry: Clinical Trial ID: NCT03708250, https://clinicaltrials.gov/ct2/show/NCT03708250.

Thrombocytopenia in Children: A Large Prospective Study on Clinical Manifestations, Seasonal Variation, Etiology, and Outcome.

AIM: To study the clinico-etiological profile of children with thrombocytopeniaMethods: This prospective hospital-based study included all children (<18 years) with thrombocytopenia at the time of hospitalization and/or thrombocytopenia during the course of their hospital stay. A detailed history was recorded and appropriate laboratory investigations were carried out Results: The study group comprised 246 children (mean age, 9.29 years; median age, 10 years) with male to female ratio of 1.5:1. Nearly 45% of children were above 10 years of age. Trends of admissions showed that the majority of children with thrombocytopenia (n = 115) got hospitalized during the rainy season, followed by summer (n = 84). Fever (72.8%), pallor (52.8%), bleeding manifestations (22%), lymphadenopathy (20.3%), and splenomegaly (20.3%) were common clinical features. Petechiae was the most common bleeding manifestation (63%). Septicemia (24%) was the most common etiology, followed by megaloblastic anemia (14.6%), undiagnosed fever (10.2%), local infection (9.3%), hepatitis (6.5%), and scrub typhus (6.1%). About nine children died. All those who died had septicemia and multi-organ dysfunction (MOD). On logistic regression analysis, age >10 years, presence of bleeding, arthralgia, rash, pallor, gastrointestinal (GI) symptoms, hematological disorders, and malignancy were associated with severe thrombocytopeniaConclusion: Thrombocytopenia is a common hematological observation. This study revealed seasonal variation in the occurrence of thrombocytopenia in children, with the maximum number of cases in the rainy season. Septicemia is the commonest etiology. The majority of children with thrombocytopenia have no bleeding manifestations. Age >10 years, presence of bleeding, arthralgia, rash, pallor, GI symptoms, hematological disorders, and malignancy are associated with severe thrombocytopenia.

Postradiation Optic Atrophy Is Associated With Intraocular Pressure and May Manifest With Neuroretinal Rim Thinning.

BACKGROUND: To determine risk factors for postradiation optic atrophy (PROA) after plaque radiotherapy for uveal melanoma. METHODS: A single center, retrospective cohort study of patients diagnosed with uveal melanoma involving choroid and/or ciliary body treated with plaque between January 1, 2008, and December 31, 2016. Outcomes included development of PROA with pallor alone or with concomitant neuroretinal rim thinning (NRT). Cox regression analysis was performed to identify risk factors for PROA. RESULTS: Of 78 plaque-irradiated patients, PROA developed in 41 (53%), with concomitant NRT in 15 (19%). Risk factors for PROA of any type included presentation with worse visual acuity (odds ratio [95% confidence interval] 5.6 [2.3-14.1], P < 0.001), higher baseline intraocular pressure (IOP; 14 vs 16 mm Hg) (1.1 [1.0-1.2], P = 0.03), shorter tumor distance to optic disc (1.3 [1.2-1.5], P < 0.001) and foveola (1.2 [1.1-1.3], P < 0.001), subfoveal subretinal fluid (3.8 [2.0-7.1], P < 0.001), greater radiation prescription depth (1.3 [1.1-1.6], P = 0.002), dose to fovea (point dose) (1.01 [1.01-1.02], P < 0.001), and mean (1.02 [1.02-1.03], P < 0.001) and maximum dose to optic disc per 1 Gy increase (1.02 [1.01-1.03], P < 0.001). On multivariate modeling, dose to disc, baseline IOP, and subfoveal fluid remained significant. Subanalysis revealed risk factors for pallor with NRT of greater mean radiation dose to disc (1.03 [1.01-1.05], P = 0.003), higher maximum IOP (17 vs 20 mm Hg) (1.4 [1.2-1.7], P < 0.001), and subfoveal fluid (12 [2-63], P = 0.004). CONCLUSION: PROA may result in NRT in addition to optic disc pallor. Risk factors for PROA included higher radiation dose to optic disc, higher baseline IOP, and subfoveal fluid. Higher maximum IOP contributed to concomitant NRT.

Lane-Hamilton syndrome - Is it really a needle in a haystack?

Introduction: The association of pulmonary hemosiderosis with celiac disease (Lane-Hamilton syndrome) is extremely rare. Case Details: A five-year-old female child presented with fever, cough, breathlessness, and pallor for 20 days, without any previous history of recurrent lower respiratory tract infections, tuberculosis, or cardiac disease. There was no history of pica, chronic diarrhea, bleeding, or personal or family history of repeated blood transfusions. She had tachycardia, tachypnea, severe pallor, stunting, rickets, and bilateral fine lung crepitations. Peripheral smear and blood indices revealed dimorphic anemia. Anti-tissue transglutaminase IgA antibody levels were high (>200 U/mL) and the upper gastrointestinal endoscopy with duodenal biopsy confirmed the diagnosis of celiac disease. The child was discharged on a gluten-free diet (GFD) and oral hematinic, but her dietary compliance was poor. Interestingly, the child had persistent bilateral pulmonary infiltrates, which was initially attributed to congestive cardiac failure (CCF), which persisted even despite treatment. HRCT chest revealed interstitial thickening and bilateral alveolar shadows and bronchoalveolar lavage showed a few inflammatory cells. The child was readmitted four times with similar complaints and was given packed red cell transfusions. In the fourth admission, a lung biopsy was done, which revealed extensive pulmonary hemosiderosis. The patient was given a course of oral steroids for 6 weeks, with a gluten-free diet, following which both the anemia and the pulmonary infiltrates resolved. Conclusion: Pulmonary hemosiderosis is an important cause of anemia in cases of celiac disease and may be misdiagnosed as CCF due to severe anemia. A strict GFD, with or without corticosteroids, can reverse the clinical and radiological picture.

Study of Clinical, Hematological and Molecular Characteristics of Patients of Thalassemia and Hemoglobinopathies in Tertiary Care Hospital.

To study clinical, hematological and molecular characteristics of patients of thalassemia and hemoglobinopathies and to correlate the molecular characteristics with clinical and hematological presentations. Material: This observational cross sectional study included 100 patients of age >12 years of all genders with chronic haemolytic anemia and history of multiple blood transfusion. Blood and radiological investigations were done. Clinical, hematological and molecular characteristics were studied. Observation and Clinical: Pallor was present in all cases and icterus in 32% cases. Total 48% of the patients had hepatomegaly and 98% had splenomegaly. Among genotypes, 15% cases had α-thalassemia, 62% had ß thalassemia + 뫧 thalassemia, 7% had HbS hemoglobinopathy, and 16% had HbE hemoglobinopathy. Hematological: Hemoglobin showed significant association with molecular genotypes of thalassemia with lowest being present in ß-thalassemia + 뫧 thalassemia and HbE.MCV showed significant association with molecular genotypes, with HbE having the lowest MCV of 65.5 fl. LDH levels showed a significant association with molecular genotype with highest being in HbS hemoglobinopathy. Molecular Characteristics: Common mutations in compound α-thalassemia were 3.7, 4.2 and 20.5 deletion. As for ß-thalassemia and 뫧 thalassemia, 47 cases had heterozygous type and 15 cases had homozygous types. In ß-thalassemia, the homozygous type showed IVS1- 5(G→C),CD 41/42(→CTT) and IVSII-654(G→T) while heterozygous type showed CD16(→G), CD 41/42(→CTT), IVS1-5(G→C), and IVSII-654(G→T) . In 뫧 thalassemia, the heterozygous type showed 뫧 inversion mutation.In HbS hemoglobinopathy, heterozygous type showed Codon 6(A→T) and compound heterozygous type showed IVS1- 5(G→C) and Codon 6(A→T). In HbE hemoglobinopathy,the homozygous type showed CD26(G→A) and compound heterozygous type showed IVS1-5(G→C) and IVSII 654(G→T). Conclusion: The common thalassemia genotypes observed in our study were α-thalassemia (15%), ß thalassemia + 뫧 thalassemia, (62%) HbS hemoglobinopathy (7%), and HbE hemoglobinopathy (16%). The patients presented with pallor, icterus, hepatomegaly, and splenomegaly which were comparable among all molecular genotypes of thalassemia and hemoglobinopathies. α-thalassemia had compound α-thalassemia with common mutations being 3.7, 4.2 and 20.5 deletion. As for ß-thalassemia and 뫧 thalassemia, 47 cases had heterozygous type and 15 cases had homozygous types. In 뫧 thalassemia, the heterozygous type showed 뫧 inversion mutation in 5 cases. MCH, Retic count, ferritin stores, and peripheral blood smear were similar in all molecular genotypes. Hemoglobin, MCV and LDH showed a significant association with molecular genotypes. Microcytic hypochromic anaemia was commonest among all.The findings of the present study show that the genotypes of thalassemia are characterized by diversity as well as significant genetic heterogeneities.

Clinico-epidemiological Profile of Children with Chronic Kidney Disease in Tertiary Care Referral Center in Nepal: Prospective observational study.

Background Chronic kidney disease in children has been increasing over the years and is associated with high degree of morbidity and mortality. The cost of treating children with chronic kidney disease is also substantial. Prevention of this disease is the only long term solution in our context. Till date, there is limited data on chronic kidney disease in Nepalese children. Objective To study the epidemiological, clinical and etiological profile of children with Chronic Kidney Disease from 2 to 16 years of age presenting for the first time in tertiary care hospital. Method This is the prospective observational study carried out at the tertiary care hospital over a period of 3 years. Children between 2-16 years who were first time diagnosed of chronic kidney disease and had estimated glomerular filtration rate < 60 ml/ min/1.73 m2 for last 3 months were enrolled. Result There were total of 124 patients, of whom 78 were male with male to female ratio of 5:3 with mean age of 11.8±2.2 years. Chronic glomerulonephritis (23%), reflux nephropathy (23%) and nephrolithiasis (16%) were the commonest etiological diagnosis. Rapidly progressive glomerulonephritis and systemic lupus erythematous accounted for 85% cases of chronic glomerulonephritis. Vesicoureteric reflux and posterior urethral valve accounted for 85% cases of reflux nephropathy. In 32%, the cause could not be determined. Swelling (45%) and dyspnea (20%) were the chief presenting complaints. Pallor were seen in all cases (100%) followed by hypertension in 68% and short stature in 64%. Majority (60%) of the patients with chronic kidney disease presented in the stage V. Conclusion Chronic glomerulonephritis and reflux nephropathy were the commonest etiologies of chronic kidney disease, majority of them following rapidly progressive glomerulonephritis, systemic lupus erythematosis, vesicoureteric reflux and posterior urethral valve. The commonest clinical features were swelling, dyspnea, pallor and hypertension.

'Diagnosing food protein-induced enterocolitis syndrome'.

Food protein-induced enterocolitis syndrome is still a mysterious disease, pathogenically poorly characterized, although the first FPIES case has been described in 1967. Mainly, food protein-induced enterocolitis syndrome diagnosis is based on clinical history. The oral food challenge remains the gold standard to confirm the diagnosis, especially in particular situations. Although there are no diagnostic laboratory or imaging tests which are specific for diagnosis, they could, however, sometimes be helpful to rule out clinical conditions which are similar to food protein-induced enterocolitis syndrome reactions. The purpose of this review is to define the clinical features of FPIES and to summarize the current available tools for the diagnosis of FPIES. This review is intended to be a practical guide for the clinician facing a patient with food protein-induced enterocolitis syndrome avoiding delayed diagnosis with unnecessary laboratory tests and detrimental treatments. Moreover, it highlights the unmet needs in diagnosis that require urgent attention from the scientific community to improve the management of patients with FPIES.

Dermatological manifestations in children with chronic kidney disease: a study from a North Indian tertiary care institute.

BACKGROUND: Dermatological manifestations due to impaired renal function in children with chronic kidney disease (CKD) are diverse and are an important cause of morbidity. AIM: To determine the prevalence of dermatological manifestations in predialysis children with different stages of CKD and its association with various metabolic parameters. METHODS: This cross-sectional observational study was conducted in 200 predialysis children with CKD stages 1-5 and 200 age- and sex-matched healthy controls (HCs). Detailed dermatological examination was performed to check for any pathological/physiological changes in the skin, mucosa, hair and nails, and changes were recorded in a predesigned proforma. RESULTS: The median age of cases and HCs was 60.50 months (76.5% males) and 62 months (78.5% males) respectively. Skin manifestations were seen in 189 (94.5%) cases and 156 (78%) HCs (P < 0.001). The most common skin manifestation among cases was pallor (75.5%, P < 0.001) followed by xerosis (48.5%, P < 0.001), atopic diathesis (41.5%) and pruritus (13%, P < 0.001), while among HCs, the most common skin manifestation was atopic diathesis (46%), followed by pallor (24%) and xerosis (14%). Xerosis was found to be significantly associated with higher serum creatinine levels (P = 0.04) and hypocalcaemia (P = 0.03). There was no significant association found between pruritus and serum creatinine. CONCLUSION: Xerosis was significantly associated with higher levels of creatinine and hypocalcaemia. Timely recognition of these symptoms and proper management can improve the quality of life in children with CKD.

Phenotypes and natural history of food protein-induced enterocolitis syndrome in the east Mediterranean region.

Background: Food protein-induced enterocolitis syndrome (FPIES) is a rare non-IgE mediated food allergy. Objective: To delineate the differences in the spectrum of culprit foods, remission patterns, and predictors among varying cultures. Methods: We reviewed demographics, culprit foods, outcomes, and predictors in 81 children with a diagnosis of FPIES who were followed up between 2015 and 2020. Results: Eighty-one patients (55.6% boys) were enrolled, including 72 with acute FPIES and 9 with chronic FPIES. Hen's egg was the most common culprit food (36.6%), followed by fish (26.9%), and cow's milk (21.5%). Interestingly, cow's milk was significantly prevalent in chronic FPIES cases (p = 0.006). The most common clinical symptoms were vomiting (100%), pallor (63.4%), and lethargy (55.9%). Emergency department visits were noted in 39 patients (41.9%), of whom 37 (39.8%) were treated with intravenous (IV) fluid. The subjects were followed up for a median (interquartile range) of 19.4 months (12.3-41.2 months), and 26 subjects (32.1%) achieved tolerance. The median (interquartile range) age at tolerance was 2.5 years (2.1-3.2 years). With regard to the culprit foods, hen's egg was observed more frequently in the subjects with resolved FPIES cases (p = 0.008), whereas fish FPIES cases were high in the persistent group (p = 0.001). IgE sensitization of the culprit food was found to be an independent risk factor for the persistence of FPIES (odds ratio 4.855 [95% confidence interval, 1.131-20.844]; p = 0.034). Conclusion: In our cohort, unlike other published series, hen's egg and fish were the two most common culprit foods. Fish differed from other culprit foods, with significantly delayed onset and persistence, and may create a model that allows for the understanding of the disease.

Risk factors associated with clinically significant gastrointestinal bleeding in pediatric ED.

INTRODUCTION: Gastrointestinal bleeding is a common problem in pediatric emergency department (PED). Some of these patients can lose significant amount of blood which may lead to shock. The aim of this study is to determine the risk factors predicting clinically significant gastrointestinal (GIS) bleeding in patients presenting to PED. METHODS: This study was performed prospectively from January 1st 2013 to December 31th 2013 in patients with upper or lower GIS bleeding. Clinically significant GIS bleeding was defined as >2g/dL hemoglobin decrease at any time during observation in PED, need for erythrocyte transfusion or need for rapid endoscopic evaluation. RESULTS: 105 patients were enrolled, 81 of which were eligible for the study. Twenty two patients (26,8%) had clinically significant GIS bleeding. These patients have significantly more commonly have upper GI bleeding and symptoms of melena, pallor and tachycardia. Initial laboratory findings revealed lower hemoglobin, RBC and albumin levels with higher WBC and BUN levels. They need significantly more nasogastric tube placement and PPI and H2 blocker treatment. Final diagnosis included more gastritis and peptic ulcers. These patients have less hematochezia, less lower gastrointestinal bleeding and less commonly diagnosed as acute gastroenteritis or Mallory Weiss tear as a final diagnosis. CONCLUSIONS: Pediatric emergency physicians should be aware of clinical and laboratory parameters of patients with clinically significant GIS bleeding to predict which patients are under risk of life threatening blood loss. Patients who have melena, pallor, tachycardia, anemia and uremia at presentation are more prone to have significant GIS bleeding.

Diagnostic Criteria for Commotio Cordis Caused by Violent Attack: Review of the Literature.

The diagnosis of lethal commotio cordis (CC) is really complex. The forensic pathologist's task is even more relevant when he/she has to explain a CC diagnosis caused by an assault in a trial. The purpose of this study was to analyze the literature on lethal CC as a result of violent attacks and identify relevant parameters that may help in the diagnosis. A review of the relevant articles was performed. Fifty-two cases of CC caused by violent attacks were identified. The collected data allowed to confirm the following literature's criteria for CC diagnosis in case of assaults: witnessed occurrence of a blunt, nonpenetrating blow to the chest preceding cardiovascular collapse; absence of structural damage to the sternum, ribs, or heart itself; and absence of any underlying cardiovascular abnormalities (such as other causes of sudden death). Regarding the assessment of the third criterion, the authors suggest that the pathologist should always specify the scientific autopsy guidelines that he/she used to differentiate CC from the other causes of sudden death. In addition, the authors highlight the importance of a multidisciplinary approach for a correct interpretation of clinical, autopsy, and laboratory findings.

Changing Epidemiology: A New Focus of Kala-azar at High-Altitude Garhwal Region of North India.

Adult cases of visceral leishmaniasis (VL), predominantly males, have been reported in the past decade from natives of high altitude areas of North Indian state of Uttarakhand. We report 14 pediatric cases of VL, who were diagnosed and treated successfully over the past 7 years. All these children were born and brought up in this area and had never visited any of the endemic areas. High prevalence of pallor, splenohepatomegaly, thrombocytopenia and poor association with HIV are cardinal features of VL in this region. Although newer drugs have become available, the protozoan continues to be sensitive to sodium stibogluconate. We conclude that the transmission cycle of VL has been established in this region and VL should be considered in the differential diagnosis of any child presenting with fever and hepatosplenomegaly. However, molecular and epidemiological studies are needed to identify the ancestry, vector and animal reservoir if any in this region.

Clinical Anaemia Detection in Children of Varied Skin Complexion: A Community-based Study in Southeast, Nigeria.

BACKGROUND: Clinicians rely on clinical detection of pallor to diagnose anaemia. This makes it important to evaluate the effect of different skin complexions on the accuracy of the pallor in diagnosing anaemia in children. METHODS: Clinicians conducted blind-independent physical examination, and their reports were compared with HemoCue 301 haemoglobin estimated with. The sensitivity and specificity were calculated. RESULTS: A total of 573 children were reviewed by 27 healthcare workers. The prevalence of anaemia was high. The highest prevalence was among children between the age of 4 and 12 months (urban 63.4% and rural 69.2%). Anaemia was detected better among dark-skinned children. Conjunctivae and palm pallor had the highest sensitivity (78.6% and 69.2%, respectively). DISCUSSION: Clinical pallor is a good screening assessment for anaemia but not diagnostic. Its sensitivity and specificity vary among different sites and skin complexions. Thus combining findings at any of the sites can improve detection of anaemia in children.

Yield of 48-hour Holter monitoring in children with unexplained palpitations and significance of associated symptoms.

OBJECTIVE: To determine the yield of 48-hour Holter monitoring in children with unexplained palpitations and the significance of associated symptoms. METHODS: This descriptive study was conducted at the Children's Hospital and Institute of Child Health, Lahore, Pakistan, from January 1 to December 31, 2015. All children above 5 years of age with history of intermittent palpitations and normal basic cardiovascular workup were enrolled. A 48-hour Holter study was performed using Motara Holter Monitoring System. Frequency of various symptoms and abnormal Holter findings were analysed. SPSS 21 was used for data analysis. RESULTS: Of the 107 patients, 69(64.5%) were males and 38(34.5%) females. The median age was 10 years (interquartile range: 5-18 years). Most common concomitant symptoms with palpitation included syncope/pre-syncope in 35(32.7%) patients, chest pain 22(20.5%), shortness of breath 21(19.6%) and colour change/pallor 11(10.3%). Holter recording was positive in 40(37%) patients. Frequent premature ventricular contractions 12(11.2%) and atrial ectopic beats 9(8.4%) were the most common findings. Holter findings were significantly more common in patients with history of shortness of breath and colour change/pallor during palpitations (p=0.002). CONCLUSIONS: Extended 24-hour Holter monitoring in children with palpitations was an inexpensive, non-invasive investigation with a reasonably high diagnostic yield in detecting arrhythmias.

Visceral Leishmaniasis In Adults.

Visceral Leishmaniasis (also known as Kala Azar) is a parasitic infection caused by Leishmania Donovani in the East and Leishmania Infantum in the west. It is prevalent in many countries including India, Bangladesh, Nepal, Africa and part of America. The disease follows chronic course and is usually lethal if left untreated. It has also been reported from different parts of Pakistan, including Northern areas, Districts Mansehra & Abbottabad and Hilly areas of Khyber Pukhtoonkhawah. Most the cases have been reported in paediatric population. Except one case, reported previously from Abbottabad. The present two cases were also seen in adults.

[Hypotonic-Hyporesponsive Episode after immunization with whole-cell pertussis combination vaccine. Clinical Case Report]. / Episodio Hipotonía-Hiporreactividad posterior a la inmunización con vacuna combinada con pertussis de células enteras. Reporte de un caso.

INTRODUCTION: Hypotonic-Hyporesponsive Episode (HHE) is an adverse event after vaccination, mainly associated with whole-cell pertussis vaccines. It is characterized by a sudden onset of muscle flaccidity, reduced response to stimuli and pallor or cyanosis. Although the HHE is infrequent, it is considered a severe adverse event. OBJECTIVE: To report a case of HHE following the administration of the whole-cell pertussis combination vaccine (DTwP-HB-Hib), which is included in National Im munization Program (PNI) of Chile, and to contributing to the knowledge of this adverse event in the country. CASE REPORT: A 6-month-old infant, 3 hours post-vaccination with the third dose of DTwP-HB-Hib vaccine, presented a decreased level of consciousness that was interpreted as atonic seizure but finally considered as EHH. The infant progressed favorably after 2 hours of clinical observation and was discharged 24 hours later. Parents were suggested to continue the immunization schedule of the infant with acellular pertussis vaccines as a preventive measure. CONCLUSIONS: The lack of knowledge about the EHH may discourage childhood immunization. Therefore, it is important for the medical staff to inform parents of the patients about this benign, self-limited and non-recurrent adverse event. In these cases, it is recommended to continue the immunization schedule of the infant with acellular pertussis vaccines.