Central retinal artery occlusion following pediatric blunt ocular trauma.

BACKGROUND: To analyze the clinical profile, presentation, possible pathophysiology, and outcomes of central retinal artery occlusion (CRAO) following blunt trauma in pediatric subjects. METHODOLOGY: The medical charts of subjects aged 18 years or less with a diagnosis of CRAO following blunt ocular trauma were analyzed retrospectively for demography, details of the trauma, ocular findings, additional imaging reports if any, and final outcome. A Medline search was done (key words like central retinal artery occlusion, blunt trauma, children, pediatric subjects, and adolescents) to gather information available in the literature on the subject. RESULTS: A total of 11 patients (11 eyes), mean age of 14.3 ± 3.4 years, and 100% male preponderance, with an average time duration from trauma to presentation to the hospital of 8.1 days were included. Visual acuity ranged from no light perception (four eyes) to finger count at a 1 m distance. Intraocular pressure was raised in three patients, of which two were suffering from sickle cell disease. In two eyes, the CRAO coexisted with optic nerve avulsion and the cilioretinal artery was spared. Disk pallor was seen in six eyes as early as 12 days from the trauma. None of the cases revealed any bony fracture in the CT scan. CONCLUSION: CRAO was observed to be an important primary or contributory cause of visual loss in children following blunt trauma, reflex vasospasm being the most common etiology. Early onset disk pallor could suggest an underlying vascular compromise of both retinal and optic disk circulation in addition to direct disk damage.

[Blastic plasmacytoid dendritic cell neoplasm (BPDCN) : A rare hematologic neoplasm with frequent cutaneous involvement]. / Blastische plasmazytoide dendritische Zellneoplasie (BPDCN) : Eine seltene hämatologische Neoplasie mit häufiger kutaner Beteiligung.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological malignancy that derives from precursors of plasmacytoid dendritic cells and is characterized by disseminated, erythematous or bluish-livid plaques or nodi. Because of the disease's rarity the diagnosis and treatment still pose a significant challenge. We present a case of a patient with BPDCN and show clinical and diagnostic characteristics as well as potential treatment regimes.

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.

Pancytopenia in celiac disease-A case series of 20 children.

Pancytopenia in children with celiac disease (CeD) is postulated to be due to nutritional deficiency such as vitamin B12, folate and copper or an autoimmune process resulting in aplastic anemia with hypoplastic marrow. In the present case series, we report the profile and explore the etiology of pancytopenia among children with CeD. There are only a few case reports of pancytopenia in children with CeD. We enrolled newly diagnosed cases of CeD and pancytopenia presenting in the celiac disease clinic over three years. Detailed evaluation was carried out for the cause of pancytopenia. We followed up on the cases for compliance and response to gluten-free diet at three months, six months and 12 months. Twenty patients were eligible for inclusion. They were divided into two groups: one with aplastic anemia with hypoplastic marrow labeled as Gp CeD-AA and the other with megaloblastic/nutritional anemia labeled as Gp CeD-MA. Patients in Gp CeD-MA presented with classical symptoms of CeD as recurrent diarrhea, abdomen distension, pallor and poor weight gain. They had none or just one transfusion requirement and had an early and complete recovery from pancytopenia. Patients in Gp CeD-AA presented with atypical symptoms such as epistaxis, short stature, fever, pallor and weakness. They had a multiple blood transfusion requirement and had delayed and partial recovery from pancytopenia. Pancytopenia is not a disease in itself but is the presentation of an underlying disease. It can occur due to various coexisting disorders in children with CeD, which can be as simple as nutritional deficiencies to as complex as an autoimmune process or malignancy. CeD should be included in the differential diagnosis of aplastic anemia as CeD and aplastic anemia both have a similar pathological process involving T cell destruction of tissues.

What are the risk factors for death among children with pneumonia in low- and middle-income countries? A systematic review.

Background: Knowledge of the risk factors for and causes of treatment failure and mortality in childhood pneumonia is important for prevention, diagnosis, and treatment at an individual and population level. This review aimed to identify the most important risk factors for mortality among children aged under ten years with pneumonia. Methods: We systematically searched MEDLINE, EMBASE, and PubMed for observational and interventional studies reporting risk factors for mortality in children (aged two months to nine years) in low- and middle-income countries (LMICs). We screened articles according to specified inclusion and exclusion criteria, assessed risk of bias using the EPHPP framework, and extracted data on demographic, clinical, and laboratory risk factors for death. We synthesized data descriptively and using Forest plots and did not attempt meta-analysis due to the heterogeneity in study design, definitions, and populations. Findings: We included 143 studies in this review. Hypoxaemia (low blood oxygen level), decreased conscious state, severe acute malnutrition, and the presence of an underlying chronic condition were the risk factors most strongly and consistently associated with increased mortality in children with pneumonia. Additional important clinical factors that were associated with mortality in the majority of studies included particular clinical signs (cyanosis, pallor, tachypnoea, chest indrawing, convulsions, diarrhoea), chronic comorbidities (anaemia, HIV infection, congenital heart disease, heart failure), as well as other non-severe forms of malnutrition. Important demographic factors associated with mortality in the majority of studies included age <12 months and inadequate immunisation. Important laboratory and investigation findings associated with mortality in the majority of studies included: confirmed Pneumocystis jirovecii pneumonia (PJP), consolidation on chest x-ray, pleural effusion on chest x-ray, and leukopenia. Several other demographic, clinical and laboratory findings were associated with mortality less consistently or in a small numbers of studies. Conclusions: Risk assessment for children with pneumonia should include routine evaluation for hypoxaemia (pulse oximetry), decreased conscious state (e.g. AVPU), malnutrition (severe, moderate, and stunting), and the presence of an underlying chronic condition as these are strongly and consistently associated with increased mortality. Other potentially useful risk factors include the presence of pallor or anaemia, chest indrawing, young age (<12 months), inadequate immunisation, and leukopenia.

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.

Atypical Optic Neuritis: The Potential Red Flags.

Background: Many potential causes of optic nerve inflammation exist, including typical and atypical causes, which require different management strategies. Objective: The objective of this study is to identify red flags that help differentiate typical from atypical optic neuritis (ON). Materials and Methods: This prospective study included 66 patients (100 eyes) with immune-mediated ON from January 2016 to June 2019, carefully excluding the nonimmune causes. The clinico-radiological features, investigations, therapy, and outcome were analyzed. Results: We evaluated 33 cases each of typical and atypical ON. The typical group included 29 idiopathic ON and four associated with multiple sclerosis. Atypical ON included 19 neuromyelitis optica (NMO), seven MOG-associated ON (MOG-ON), and others due to Sjogren's syndrome, granulomatous polyangiitis, sarcoidosis, and IgG4 disease. Atypical ON occurred significantly and more frequently with extremes of ages (<10 or >70 years), bilateral simultaneous or severe vision loss with early disc pallor, multiple attacks, symptoms/neuro-imaging indicating non-MS disease e.g., long segment ON/myelitis, large confluent lesions, the involvement of optic tract, chiasma, area postrema or diencephalon, and (pachy) meningitis. Systemic involvement and poor outcomes despite steroids and second-line immunosuppression were observed more often in the atypical ON. Conclusions: The red flags indicating atypical ON are onset at extremes of age, multiple attacks, bilateral simultaneous or severe to very severe vision loss, early disc pallor, neurological symptoms, or imaging abnormalities suggesting non-MS disease, systemic involvement, and poor steroid responsiveness. The awareness might help the clinician promptly identify and escalate therapy to ensure a better outcome.

True Idiopathic Brachial Artery Aneurysm Treated With a Saphenous Vein Graft.

Purpose: The objective of this study is to report a case of a 65-year-old woman who presented with pallor and pain of her left arm secondary to a true arterial brachial aneurysm, which was successfully treated with saphenous vein bypass and embolization of the aneurysm sac. A review of the literature is also presented. Case report: A 65-year-old woman presented with an acute onset of pallor and pain of her left forearm, and hand. On physical examination, there was a pulsatile mass at the forearm. A doppler ultrasound showed a fusiform aneurysmal dilatation of the brachial artery of 23 mm of diameter. A dynamic contrast-enhanced MRI angiogram confirmed a fusiform dilation of the distal brachial artery. The patient was scheduled for open repair. A fusiform 20 x 60 mm aneurysm of the distal brachial artery extending to the cubital fossa was found and a brachial artery to radial and ulnar arteries bypass with interposed reverse right saphenous vein was created. Embolization of the aneurysm sac was performed using Gelita-spon ® (Gelita Medical, Eberbach, Germany). A final angiogram showed an adequate perfusion through the bypass to the hand, and no contrast in the aneurysmal sac. Postoperative course was uneventful with discharge on the fourth postoperative day. Conclusion: Revascularization with autologous saphenous vein graft and exclusion of the aneurysm with local embolization is a good treatment alternative in a patient with symptomatic brachial aneurysm with distal embolization.

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.

[Harlequin phenomenon associated with neurological abnormalities: A case report]. / Phénomène d'Arlequin associé à des anomalies neurologiques : un cas.

BACKGROUND: Harlequin phenomenon consists of facial flush and erythrosis with unilateral sweating and pallor, associated with contralateral anhidrosis. We present the case of a child in whom the syndrome was associated with Horner's syndrome, epilepsy, mental and psychomotor retardation. PATIENTS AND METHODS: A 9-year-old boy presented with right unilateral hemifacial erythema on effort, with normal colouring and Horner's syndrome on the left side of the face. His medical history revealed generalized myoclonic epilepsy, psychomotor delay and mental retardation. No underlying anomalies were identified. Harlequin phenomenon was diagnosed. DISCUSSION: Despite its stereotypical clinical features, Harlequin phenomenon is a poorly known disease. However, clinicians must be aware of it in order to determine the diagnosis and investigate for causes and any associated abnormalities. The underlying mechanism is an autonomic neuropathy affecting the sympathetic vasodilator neurons. To our knowledge, there have been no previous reports of Harlequin phenomenon in association with Horner syndrome, psychomotor delay and mental retardation.

A case of recurrent facial pain associated with a Pourfour du Petit syndrome: a new entity?

We report the case of a 38-year-old woman with a history of migraine who experienced an association of recurrent unilateral facial pain and Pourfour du Petit syndrome. The episodes occurred for between a few seconds and up to 3 minutes up to 6 times a day mimicking short-lasting unilateral neuralgiform headaches with cranial autonomic symptoms. No lesional cause was found and the use of topiramate led to a nearly complete disappearance of the episodes. This new entity raises the question of a novel autonomic dysfunction in short-lasting unilateral neuralgiform headaches with cranial autonomic symptoms or an unexpected presentation of migraine.

Predictive value of fever and palmar pallor for P. falciparum parasitaemia in children from an endemic area.

INTRODUCTION: Although the incidence of Plasmodium falciparum malaria in some parts of sub-Saharan Africa is reported to decline and other conditions, causing similar symptoms as clinical malaria are gaining in relevance, presumptive anti-malarial treatment is still common. This study traced for age-dependent signs and symptoms predictive for P. falciparum parasitaemia. METHODS: In total, 5447 visits of 3641 patients between 2-60 months of age who attended an outpatient department (OPD) of a rural hospital in the Ashanti Region, Ghana, were analysed. All Children were examined by a paediatrician and a full blood count and thick smear were done. A Classification and Regression Tree (CART) model was used to generate a clinical decision tree to predict malarial parasitaemia a7nd predictive values of all symptoms were calculated. RESULTS: Malarial parasitaemia was detected in children between 2-12 months and between 12-60 months of age with a prevalence of 13.8% and 30.6%, respectively. The CART-model revealed age-dependent differences in the ability of the variables to predict parasitaemia. While palmar pallor was the most important symptom in children between 2-12 months, a report of fever and an elevated body temperature of ≥37.5°C gained in relevance in children between 12-60 months. The variable palmar pallor was significantly (p<0.001) associated with lower haemoglobin levels in children of all ages. Compared to the Integrated Management of Childhood Illness (IMCI) algorithm the CART-model had much lower sensitivities, but higher specificities and positive predictive values for a malarial parasitaemia. CONCLUSIONS: Use of age-derived algorithms increases the specificity of the prediction for P. falciparum parasitaemia. The predictive value of palmar pallor should be underlined in health worker training. Due to a lack of sensitivity neither the best algorithm nor palmar pallor as a single sign are eligible for decision-making and cannot replace presumptive treatment or laboratory diagnosis.

Prevalence of cyclic vomiting syndrome in a sample of Turkish school children in an urban area.

OBJECTIVES: Cyclic vomiting syndrome (CVS) is characterized by recurrent, explosive bouts of vomiting punctuating periods of normal health. Its prevalence is unclear in the world, and is unknown in Turkey. This study was designed to investigate its prevalence in Turkish school children. METHODS: The study was performed on 1263 children, aged 6 to 17 years old, who were selected by systematic sampling method before, for the celiac disease prevalence study. The population was asked the questions relating to CVS. Children whose answers fulfilled CVS criteria were invited for clinical interview and physical examination at the hospital. RESULTS: Of 1263 children, 24 (1.9%) fulfilled the criteria of CVS. Of them, 33.3% were male, 66.6% were female; the age ranged from 7 to 14 years old (mean age 10.5). The mean onset age was 7+/-3.4 years (5 to 12 years). The duration of attack ranged from 3 to 10 days (median: 5 days). Prodromal symptoms were reported in 25% of children with CVS. The episodes were accompanied by pallor, anorexia, feeling unwell, headache, abdominal pain, and photophobia in 100%, 100%, 83.3%, 66.7%, 58.3%, and 16.6%, respectively. Precipitating factors were reported by 20.8% of the children. Six children (25%) had migraine. A positive family history for migraine was noted in 7 (29.2%). Seven children (29.2%) suffered from travel sickness. CONCLUSIONS: In this first CVS prevalence study in Turkey, we found that CVS is highly prevalent in school children. For this reason, we emphasize that clinicians should be aware of this syndrome and consider it in the differential diagnosis of recurrent vomiting.

Cardiac arrest: a rare complication of pallid syncope?

Cardiac arrest is rare in children. Breath-holding, on the other hand, is fairly common. We report a case in which one complicated the other with serious consequences. A review of the literature on the subject was undertaken.

Understanding the dynamic provoked circulatory response of the papilla.

The author explains the difficulties of a topographical quantification of papillary changes. On the one hand this is due to the unprecise definition of the papilla's boundaries, on the other hand these boundaries might themselves fluctuate. Afterwards a description of the dynamic provoked circulatory response follows. This is an examination which tests the change of redness of the rim when the intraocular pressure is artificially risen. Two properties of the vascular system of the papilla are checked with it. When looking at the 'latency time', the time which elapses between beginning of the provocation and beginning of pallor increase, the vascular resistance is controlled. When measuring a decrease of pallor while increasing the intraocular pressure autoregulation of the papillary capillaries are tested. The dynamic provoked circulatory response is the only clinically available test of the papilla's vascular system.

Optic disc pallor and visual field defect in exfoliative and non-exfoliative, untreated ocular hypertension.

In a prospective study of untreated ocular hypertension 117 eyes were non-exfoliative and 20 eyes exfoliative. The percentage of optic disc pallor was significantly larger in the exfoliative than in the non-exfoliative group, whereas no significant differences were found in IOP or in the visual field.

Optic nerve head measurements: the optic nerve head analyzer--its advantages and its limitations.

The Optic Nerve Head Analyzer (ONHA) calculates by means of computer-assisted analysis of stereo images different parameters of the optic disc: disc diameter, disc size, cup/disc ratio (CDR), neuroretinal rim area, and excavation volume for the disc quadrants and for the total disc. To obtain first indications of the clinical value of ONHA measurements for diagnosis and follow-up in glaucoma we examined the reproducibility of measurement results for different diseases. Furthermore, we studied the mean values of the different disc parameters in healthy eyes. It was investigated: 1) whether the reproducibility is different in the different disc parameters; 2) whether the reproducibility in different eye diseases is different as compared to healthy eyes; 3) by which criteria the reproducibility is influenced; 4) whether there is a correlation between disc size and size of rim area. The reproducibility was studied in 178 eyes of 178 patients, who were all examined twice with the ONHA. The mean difference between the results of first and second measurement was calculated for the different disc parameters. Differences were found in the reproducibility of the parameters: e.g., the mean difference between first and second measurement was 1.8 percent for the disc size, and 5.6 percent for the neuroretinal rim area. For the disc quadrants, the reproducibility of values was worse than for the total disc. No marked differences of reproducibility of disc parameters were found for different diseases. Reproducibility depends, for instance, upon correct determination of the disc margin. In healthy eyes, a significant correlation was found between rim area and disc size: larger discs have a larger neuroretinal rim area than smaller discs. Thus, the rim areas of different eyes are only comparable for equally sized discs. Relative values, as for instance, cup/disc ratio, or the quotient of rim area and disc size, are therefore better suited for comparison of different eyes than absolute values. The recent developments in automatic disc analysis equipment and the clinical relevance of the results for diagnosis and follow-up in glaucoma are discussed.

A comparison of methods of extra temporal facial nerve repair.

The development during the past 100 years of methods of repair of the divided facial nerve is discussed, from the early attempts to bring the nerve ends together inside a tube of vein. The success of the repair is enhanced by the care of the blood supply to the nerve and by measures to avoid scar developing between the ends of the nerve. Rest of the suture line is aided by the support of some form of tube, but unless this latter is kept below I cm. in length interference with the radial blood vessels to the nerve occurs. Intubation also encourages parallel growth of the nerve fibres and discourages fibroblast proliferation. Animal experimentation shows that the results when a soft silicone tube of special shape is used are superior to those obtained if the nerve is left free in the tissues, buried in muscle, or placed inside a vein.