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Introduction: Autoimmune hemolytic anemia (AIHA) is a rare and generally self-limiting disease in children. Material and methods: A descriptive cross-sectional study was performed in children under 18 years diagnosed with AIHA from January 1997 to July 2019. Clinical variables were collected and AIHA was classified according to the direct antiglobulin test (DAT) in warm AIHA (IgG+/-C3d) and cold AIHA (C3d). Response to treatment and evolution were analyzed. Results: 25 patients were included and 72% were males. The median age at diagnosis was 2 years (range 0.4–9). Fever (72%), pallor (68%), jaundice (64%), hepatosplenomegaly and coluria (48%) were the most common presenting symptoms. The median hemoglobin at diagnosis was 5.4 g/dl. DAT was positive in 96%, with detection of IgG antibodies in 76%. A single case presented negative DAT. 20% of the patients associated another cytopenia, one of which was subsequently diagnosed with common variable immunodeficiency. Concomitant viral infection was suspected or documented in 32%. Most of the cases were self-limiting and responded to corticosteroid treatment (72%). Those with partial response (24%), mainly those associated with other cytopenias, required other lines of treatment (rituximab, mycophenolate, immunoglobulins,…). Complications (32%) and relapses (26%) were detected only in warm AIHA. Conclusions: Our case series confirms that AIHA is a very rare disease in childhood. Most cases evolve favorably, although up to a quarter of them require second lines of treatment and, in exceptional cases, they need very aggressive treatments. These latter cases generally correspond to patients who present more than one cytopenia in the course of the disease. Resumen: Introducción: La anemia hemolítica autoinmune (AHAI) es una enfermedad rara en niños, generalmente autolimitada. Material y métodos: Estudio descriptivo transversal en menores de 18 años diagnosticados de AHAI desde enero 1997 a julio 2019. Se recogieron variables clínicas y se clasificaron según el test de Coombs directo (TCD) en AHAI por anticuerpos calientes (IgG+/-C3d) y fríos (C3d). Se analizó la respuesta al tratamiento y su evolución. Resultados: Se incluyeron 25 pacientes, siendo el 72% varones. La mediada de edad al diagnóstico fue 2 años (rango 0,4–9). Los síntomas predominantes fueron fiebre (72%), palidez (68%), ictericia (64%), hepatoesplenomegalia y coluria (48%). La mediana de hemoglobina al diagnóstico fue 5,4 gr/dl. En el 96% el TCD fue positivo, con detección de anticuerpos IgG en el 76%. Un solo caso presentó TCD negativo. Un 20% asociaron otra citopenia, uno de ellos fue diagnosticado posteriormente de inmunodeficiencia variable común. En un 32% se sospechó o documentó una infección viral concomitante. La mayoría de los casos fueron autolimitados y respondieron a tratamiento con corticoides (72%). Aquellos con respuesta parcial (24%), principalmente los que asociaban otras citopenias, precisaron otras líneas de tratamiento (rituximab, micofenolato, inmunoglobulinas,…). Se detectaron complicaciones (32%) y recaídas (26%) únicamente en AHAI por anticuerpos calientes. Conclusiones: Nuestra serie confirma que la AHAI es una enfermedad muy poco frecuente en la infancia. La mayoría de los casos evolucionan favorablemente, aunque hasta una cuarta parte precisan segundas líneas de tratamiento y casos excepcionales tratamientos muy agresivos. Estos últimos suelen corresponder a pacientes con más de una citopenia en la evolución de la enfermedad.

Purpose To report two patients with transient decreased vision and associated paracentral acute middle maculopathy (PAMM) lesions identified with multi-modal imaging, including what we believe to be the first documented patient of PAMM associated with iron deficiency anemia. Observations Case 1 is a 56-year-old man who experienced transient blurred vision one week following cardiac ablation for atrial fibrillation. Symptoms resolved by the time of presentation and visual acuity was 20/20 in each eye. Ocular examination was unremarkable aside from subtle discoloration within the papillomacular bundle in the right eye. Spectral domain optical coherence tomography (SD-OCT) revealed thickening of the middle retinal layers with a corresponding area of hyporeflectivity on the en face infrared image. This area of hyporeflectivity was confirmed on “structural” SD-OCT angiography, although no flow voids were identified. Fluorescein angiography was normal. Case 2 is a 25-year-old man with no past medical history who noted decreased central vision in his right eye upon awakening. Visual acuity was light perception in the right eye and 20/20 in the left eye. Posterior segment examination in the right eye showed tortuous retinal vessels and subtle pallor involving the superior part of the macula. SD-OCT showed thickening of the middle retinal layers of the superior macula with a corresponding area of hyporeflectivity on the en face infrared image. Systemic work-up was completed and identified severe iron deficiency anemia as the most likely inciting factor. In both cases, visual acuity was 20/20 in each eye at follow-up. Conclusions Small arterial occlusions of the retina remain difficult to diagnose and may represent a sequela of systemic disease. Multi-modal imaging is helpful in equivocal cases with subtle clinical findings. Importance The present case report illustrates the utility of multi-modal imaging in diagnosing transient ischemic events of the retina, and reports what we believe to be the first association of PAMM with iron deficiency anemia.

Introduction Hypereosinophilia is defined as an eosinophil count greater than 1.500 cells/mm. It is usually associated with organ dysfunction due to visceral eosinophilic infiltration or tissue damage mediated by eosinophilopoietic cytokines, like IL-5. Determining its etiology represents a diagnostic challenge, as numerous conditions are associated with its occurrence, especially parasitic diseases, allergic disorders, medications and neoplasms. Paraneoplastic eosinophilia usually develops in the context of tumors of the lower gastrointestinal tract, lung, Hodgkin's lymphoma, peripheral T-cell lymphomas (PTCL), and its pathophysiology is associated with interleukin-5 (IL-5) secretion, an eosinophil proliferation-inducing cytokine. Objective To describe a clinical case of paraneoplastic hypereosinophiliadue to lung cancer, a rare laboratory manifestation, in a case with an advanced stage of the disease and catastrophic evolution. Case report A 68-year-old woman, with a history of obesity and SAH, presents to the health service of the HC/FMUSP due to adynamia, dyspnea on exertion and perception of multiple violaceous cutaneous nodules on the scalp, thorax, abdomen and extremities, 45 days long. On physical examination, she had cutaneous-mucosal pallor, poor general condition, hypoxemia, and disseminated cutaneous nodules of up to 5 cm. Blood count revealed: Hb 8.9 g/d, Ht 26%, MCV 89 fl, 75.980 leukocytes/mm3 (31.920 neutrophils with left shift to promyelocytes/ 40.270 eosinophils/ 0 basophils/ 2.280 lymphocytes/ 1.520 monocytes) and 107.000 platelets/mm3; LDH = 592 U/L (NSV up to 214 U/L); IgE = 496 IU/mL (NSV up to 100 IU/mL). Myelogram showed hypercellular bone marrow with massive infiltration by elements of non-hematopoietic origin, as well as prominent medullary eosinophilia. Computed tomography (CT) showed an irregular lung mass, 5 cm in diameter, in the right upper lobe, suggestive of a primary neoplastic site, as well as secondary implants in the liver parenchyma, adrenal glands and subcutaneous cellular tissue/skin. Biopsy of subcutaneous nodules showed undifferentiated malignant neoplasm of epithelioid pattern, with immunohistochemistry: AE1/AE3 positive, 34BE12 positive, MELAN-1 negative, HMB45 negative, S100 negative, p63 negative, characterizing squamous cell carcinoma. During hospitalization, the patient rapidly evolved with tumor lisis syndrome, acute renal failure and fulminant liver failure, and died after a short period of hospitalization. Conclusion We report an atypical case of pulmonary squamous cell carcinoma, marked by paraneoplastic eosinophilia, bone marrow infiltration, cutaneous metastasis, spontaneous tumor lysissyndrome and acute liver failure. Paraneoplastic hyperosinophilia is a marker associated withpoor prognosis in solid tumors, usually reflecting an elevated tumor mass, with multi-metastatic disease, as presented in our case. Although it is a rare condition, paraneoplastic eosinophilia mustfall within the differential scope of causes of sustained elevation of eosinophils. Rapid recognition and prompt treatment of the underlying neoplasm may provide a more favorable evolution.

Background Inmates of child care institutions constitutes a vulnerable group. It is important to monitor their health status. This study aimed at estimating the prevalence of malnutrition and other common comorbidities among them. Methods This is an Analytical Cross-Sectional study done in randomly selected twelve childcare institutions in Puducherry. All eligible 563 inmates, aged 5–18 years, from the selected institutions were included in the study. Pretested structured questionnaire was used to collect data. Nutrition status of participants were assessed using WHO BMI-for-age and Height-for-age growth charts. Inmates were clinically examined for nutrition status, vitamin deficiencies, skin conditions and goiter. Assessment of common comorbidities was done using modified Rashtriya Bal Swasthya Karyakram (RBSK) questionnaire. Inmates were also assessed for Presumptive Tuberculosis. Results The prevalence of undernutrition was 39.4% (95% CI 35.48–43.53). Prevalence of thinness was 27% (95% CI 23.5–30.8) and prevalence of stunting was 20% (95% CI 16.9–23.5). Dental problems were found in 61% of the study population. Other nutritional deficiencies found on examination were palmar pallor (26.8%), Goiter (2%) and Bitot spots (0.7%). Around 4.6% of inmates reported that they had cough for 2 weeks or more and 3.7% had history of contact with a known case of Tuberculosis. Conclusion The common comorbidities among the inmates were dental problems, undernourishment and anemia. There is need for regular health education and screening for these conditions for timely identification and initiation of appropriate interventions. There is also a need for symptomatic screening for early identification and reporting of presumptive Tuberculosis cases.

Background The combination of visceral leishmaniasis (VL) and macrophage activation syndrome (MAS) makes the diagnosis difficult due to their similar clinical presentation, with a poor prognosis especially since the treatment is still poorly codified. We report the case of a 17-month-old female patient from Berkane, presenting for a 3 months history of anarchic fever with anemic syndrome made up of pallor and hemorrhagic syndrome made up of epistaxis. Physical examination revealed a temperature of 39 ° C, lower limbsedema, paleness of skin and mucous membranes, gingival petechiae, bleached hair, and hepatosplenomegaly. Case presentation The complete blood count showed pancytopenia with deep aregenerative normochromic normocytic anemia at 3 g/dL, leukocytes were at 4860/mm 3 with neutropenia at 680/mm 3 and thrombocytopenia at 12.000/mm3, the blood smear was without abnormality. These anomalies were associated with a hypoalbunemia, hypertriglyceridemia, hyperferritinemia, lactate dehydrogenase (LDH) level was at 337 IU/L, low prothrombin time (PT) at 56 % and fibrinogen level at 1 g/L. The direct Coombs test was positive. Examination of the myelogram revealed the presence of leishmania bodies and figures of hemophagocytosis. A diagnosis of visceral leishmaniasis associated with MAS was made. The patient was put on liposomal amphotericin B and corticosteroid therapy with good clinical and biological evolution and good therapeutic tolerance. Conclusion The association of VL and MAS remains rare and should be evoked even in non-endemic areas since late diagnosis worsens the prognosis and may even be responsible for the death of patients despite an aggressive treatment.

Background/Purpose: The clinical presentations of parvovirus B19 in patients with underlying diseases have greater diversity than previously healthy patients. We retrospectively identified patients with polymerase chain reaction (PCR)-confirmed parvovirus B19 infection in attempt to describe its clinical features especially in these populations. Methods: From 2009 to 2018, patients with real-time PCR-confirmed parvovirus B19 infection were collected. Comparisons were done between previously healthy patients and patients with preexisting diseases, as well as patients with high (>5.5 × 105 copies/mL sera) and low viral loads. Results: Parvovirus B19 DNA was detected in 31 patients. Fourteen (45%) patients had underlying diseases, including six (19%) with immunologic diseases, five (16%) with hematologic diseases, and three (10%) with cardiopulmonary diseases. Only seven (23%) patients received an initial impression of erythema infectiosum prior to positive PCR. A higher proportion of patients with underlying diseases presented with fatigue and pallor, and suffered from tachycardia and hepatosplenomegaly compared to previously healthy patients. Among patients with a high viral load, a substantial proportion were of older age, suffered fatigue, and anemia. There was a trend of patients with immunologic comorbidity having a higher viral load. Conclusion: The classical parvovirus B19 manifestations were less frequently observed in patients with a preexisting disease compared with previously healthy patients. Depending on host factors, the symptoms of parvovirus B19 infection can be multifaceted. Keywords: Human parvovirus B19, Erythema infectiosum, Aplastic crisis, Pure red-cell aplasia

We present a case with recurrence of intractable complex partial epilepsy, despite angiographic obliteration of a cerebral arteriovenous malformation in the left medial temporal lobe following gamma knife radiosurgery. Preoperative memory function was normal, with no evidence of hippocampal atrophy by magnetic resonance imaging. Intraoperative electrocorticography demonstrated two independent foci of paroxysmal activity in the lateral and medial temporal lobes. As preoperative magnetic resonance imaging demonstrated subcortical hyperintensity, the lateral temporal lobe was resected. Histologically, there was evidence of subcortical myelin pallor, likely resulting from ischemic damage associated with hemodynamic changes following radiosurgery. As there was a decrease in the frequency of paroxysmal discharges in the hippocampus following arteriovenous malformation nidus removal, the hippocampus was preserved. Histological examination of the nidus revealed that some of the vessels had patent lumina, while ischemia affecting the neighboring hippocampus was considered to show improvement. A good seizure outcome was obtained. In summary, perioperative multimodal examinations, including intraoperative electrocorticography, revealed various epileptogenic mechanisms, probably related to myelin pallor in the lateral temporal lobe and focal ischemia secondary to patent AVM in the medial temporal lobe, of this patient. Keywords: Intraoperative electrocorticography, Myelin pallor, Hippocampus, Patent arteriovenous malformation

A male patient, 40 years of age, arrived at our Institute with diffuse abdominal tenderness, right flank pain, hematuria and early stage of hemorrhagic shock with anemia and initial hypotension. The immediate clinical history revealed no significant previous trauma, only subsequently was reported inconstant pain in the right flank for 4–5 days with pallor and asthenia, signs and symptoms that the patient had not investigated. Abdominal CT scan with angiographic evaluation was performed showing right kidney mass and perirenal fluid collection by blood component. Immediate nephrectomy was performed and histopathological and further immunohistochemical study, revealed the epithelioid variant of angiomyolipoma.

Thoracic outlet syndrome (TOS) is a relatively uncommon condition that involves compression of portions of the brachial plexus (neurogenic or nTOS), arteries (aTOS), or veins (vTOS). The signs and symptoms of this condition can include pain in the neck, shoulder, and arm with paresthesias and pallor in the hand and may mimic other more common problems such as cervical radiculopathy and Raynaud phenomenon. A thorough history, physical examination, and interpretation of appropriate imaging modalities are essential to properly diagnose and treat TOS. The presence of a cervical rib in the setting of arm symptoms can raise suspicion. Treatment options include physical therapy, postural exercises, botulinum toxin injections, and surgical decompression with possible cervical rib resection in the appropriate setting.

Purpose: To report a case of Non-Arteritic Ischemic Optic Neuropathy (NAION) in a middle-aged bodybuilder in excellent physiological condition without any signs or symptoms of vasculopathy and a history of nitric oxide supplement usage. Observations: The patient had visual acuity of 20/25 in the right eye, and 20/30 in the left eye, with a relative afferent pupillary defect and dyschromatopsia in the right eye. Visual field testing with Humphrey perimetry demonstrated an inferior altitudinal field defect OD. Fundus examination showed a small cupless disc OD with mild pallor, and a small cupless disc OS. He denied usage of sildenafil or other phosphodiesterase (PDE) inhibitor medications but frequently ingested megadoses of nitric oxide (NO) as part of his bodybuilding regimen. Conclusions: Nitric oxide supplements act through the same pharmacologic pathway as PDE inhibitors, and this case is suggestive that other vasodilating agents may be similarly associated with NAION. Keywords: Optic nerve, Non-arteritic ischemic optic neuropathy, Nitric oxide, Phosphodiesterase

Anaemia is a leading cause of maternal deaths and adverse pregnancy outcomes in low-income and middle-income countries. Overall, 12% of low birthweight, 19% of preterm births, and 18% of perinatal mortality are attributable to maternal anaemia. 1 The prevention and timely management of anaemia is therefore essential to attain Sustainable Development Goal (SDG) 3 on ensuring healthy lives and promoting wellbeing. Accurate measurement of haemoglobin allows the identifi cation and treatment of individuals with anaemia, controlling the severe consequences of this condition. Unfortunately, in the peripheral health services of low-income countries, where the capacity to identify individuals with anaemia is most needed, haemoglobin is usually assessed only by clinical examination (ie, assessment of conjunctival and palmar pallor), an approach requiring a long training time and vast clinical experience to be fairly accurate. 2

Publisher Summary This chapter focuses on general cerebrospinal motility. The sympathetic or parasympathetic system has come to attention less frequently. Reactivity varies in degree, depending on the individual, from one day to the next. Variations and disturbances of vasomotility are less easily stimulated than horripilation. It is possible to explore arterial pressure and to record local temperatures by means of special thermometers, but the experiment takes longer. In some cases, it is possible to stimulate circulatory variations, and in the course of various pathological processes, one is struck by the sudden changes that come over the face, such as pallor or intense blushing. Certain stimulation, such as the insertion of a thermometer in the anus or brushing of the finger, caused movements of flexion. At moments during the examination, the baby's face pales while he is at rest. Slight stroking of the thorax causes the chin and lips to go pale, the cheeks symmetrically red, and the skin of the lobule of the nose crimson. At any age, the face reacts easily to impressions and emotions, and differently from one person to another.

Publisher Summary Chronic fatigue syndrome (CFS) is a complex disorder characterized by severe, disabling fatigue, associated with several nonspecific systemic symptoms. CFS is a fairly common, incompletely understood disorder, which overlaps clinically with autonomic dysfunction, frequently manifested as orthostatic intolerance. The onset of illness is often acute or subacute and, in many patients, is preceded by an infectious illness. The fatigue in CFS is characterized by persisting or relapsing chronic fatigue lasting at least 6 months in the absence of any definable medical diagnosis. In addition to the case-defining symptoms, many CFS patients experience a constellation of other debilitating symptoms and signs consistent with autonomic dysfunction, particularly those associated with orthostatic intolerance. These autonomic features include orthostatic symptoms and tachycardia, increased sweating, pallor, sluggish pupillary responses, gastrointestinal symptoms, and frequency micturition. Orthostatic intolerance (OI) is defined as the development of characteristic symptoms while standing, which are significantly improved by recumbency.

Propolis is a resinous substance collected from plants by bees. Its composition depends on the vegetation, the season, and the source area. It usually contains many chemical compounds such as polyphenols, steroids and amino acids. The hereditary spherocytosis (HS) is a type of anaemia characterized by microcytic and hyperchromic red cells, spherical in shape and without central pallor. Clinically, subjects present from asymptomatic conditions to severe haemolytic anaemia. In this study it was evaluated the effect of two propolis extracts in the osmotic fragility of HS patient red blood cell (RBC) membrane. It was found that propolis decreases the erythrocytes membrane fragility, being the effect of Bornes propolis more pronounced than Fundao propolis'. This effect was related with the higher phenolic content of the former propolis. The results obtained in vitro suggest that the membrane fragility increases under oxidative stress conditions for the patient RBC's and the protection effect of propolis is due to its antioxidant properties. These results open doors for future investigations in order to elucidate the mechanisms, identify the main compounds involved in this fragility protection of the erythrocyte membrane. This is the first work reporting an evaluation of the propolis effect in a blood disease. (C) 2010 Elsevier Ltd. All rights reserved.

Publisher Summary This chapter discusses the case of a patient suffering from nonconvulsive status epilepticus and frontal lobe seizures due to ring chromosome 20 [r(20)] syndrome. The man developed normally until his first seizure at 3 years of age. Initially, his seizures were characterized by myoclonic jerks of the upper limbs. The seizures increased in frequency until they occurred many times a day during infancy. Over time the seizures changed and began to include impaired consciousness, pallor, abdominal pain, the expression of fear, mydriasis, motor automatisms, and complex visual hallucinations. There were behavioral disturbances with frequent and sudden outbursts of rage. Neurological examination showed only a subtle postural tremor of the hands. Ictal EEG showed an initially diffuse low-voltage fast activity, which was more evident over the frontal leads, followed by a short burst of rhythmic spike-and-wave complexes and then by pseudoperiodic runs of slow waves for many seconds. This case fully confirms that r(20) syndrome represents a new model of a symptomatic partial epilepsy, which certainly deserves to be included in a future classification of epileptic syndromes and diseases.

Publisher Summary This chapter examines the case of JH, an otherwise normal 22-month-old girl, who was diagnosed with left temporal lobe epilepsy. An electroencephalogram (EEG) performed within a few hours demonstrated left temporal theta waves, without epileptiform activity. The diagnosis of complex partial seizures of probable left temporal origin was made, and JH was started on carbamazepine (CBZ). A magnetic resonance imaging scan of the brain demonstrated thickening of the cortex within the left temporal lobe, with a significant reduction in white matter volume in the left temporal lobe compared with the same area on the right. The right hippocampus was very small, with very high signal on T2-weighted imaging. Video-EEG monitoring demonstrated that all interictal spikes and ictal onsets came from the left temporal lobe. A modified left temporal lobectomy with complete resection of the dysplastic cortex was performed, with cessation of seizures and freedom from seizures for more than 3 years of follow-up. The semiology of JH's seizures, like other young children with temporal lobe seizures, was primarily remarkable for a look of fear, cessation of normal play activities and pallor. Physicians who manage children with partial seizures should realize that failure of the seizures to respond to the first drug increases the likelihood that the child will develop intractable seizures. Failure to respond to three drugs used appropriately for partial seizures is predictive of intractability, and early referral to a pediatric epilepsy surgery center can improve the child's long-term outcome.

Establishing vascular access in a child in shock, may be difficult and delay can compromise resuscitation [1]. Intraosseous Infusion (IOI) is an alternate method if peripheral venous access is not established within three attempts or 90 seconds [2]. IOI an alternate route for neonatal resuscitation if unbilical vein catherization (UVC) or other direct venous access is not readily available [3]. Case 1 A term male neonate was received in hospital gasping. He was delivered to a 24 yr old second gravida in a vehicle en route to the hospital. A neighbor, travelling along assisted the delivery and attempted to separate the cord but failed. On examination, the neonate was gasping, heartbeat was 187 bpm, capillary refilling time>3 sec, hypothermic with gross pallor and unrecordable SpO2. There was no cry and neonatal reflexes were absent. Umbilicus was unhealthy with mud applied to it. A working diagnosis of shock due to hemorrhage was made. Immediate attempt for establishing intravenous and umbilical cannulation were unsuccessful. Intraosseous cannulation in the right tibia was done through which resuscitative fluids followed by dextrose and antibiotics were given (Fig. 1). The condition stabilized and peripheral venous access was established after 2 hours and intraosseous cannulation discontinued. The neonate improved with 2 units of blood transfusion that brought up the hematocrit. He was discharged on the 11th day and followed up till the age of 4 months. Fig. 1 Showing a 18 G disposable needle used as an intraosseous needle. A guard of any sternal or bone marrow needle is used. Case 2 A 12 day old female preterm neonate was referred from a civil hospital for recurrent apnea. Born to a primi in hospital at 34 weeks, with a birth weight of 1.7kg, she was managed conservatively and discharged on the 5th day but readmitted on the 9th day for lethargy. She was treated with antibiotics but her condition deteriorated. On admission the neonate was in shock with prolonged periods of apnea and severe bradycardia. Immediate resuscitative measures with bag mask ventilation and chest compression were started. Vascular access could not be obtained due to shock and punctured thrombosed veins due to the prior treatment. Intraosseous infusion of drugs and fluids was given and finally her condition stabilized. The IOI was discontinued after 5 hours when peripheral cannulation was established. Investigations revealed right sided pneumonia with severe septicemia. She was managed accordingly and discharged 3 weeks later and regularly followed up.

PubMedID: 15862197 Congenital muscular dystrophies (CMD) are heterogenous group of muscle disorders with autosomal recessive inheritance. Merosin deficiency has been identified in some patients with CMD all of whom also had white matter abnormalities on MRI. In postmortem studies, the brain showed extensive myelin pallor with a spongy appearance of white matter and moderate astrocytosis or demyelination. Direct assessment of neuropathologic aspects of MN-CMD such as demyelination is possible with MR spectroscopy (MRS). Although previous reports have described several neuro-imaging findings of this disease, MRS findings have not been reported in literature. In this case, we report MRS features of a 4-year old girl with MN-CMD. MRS of brain demonstrated that N-acetylaspartate (NAA)/Creatine (Cr) ratio was normal. Increased Choline (Cho)/Cr and Myo-inositol (MI)/Cr ratios were obtained. These findings were interpreted as demyelination and gliosis of white matter. © 2004 Elsevier B.V. All rights reserved.

Plasma cell myeloma or multiple myeloma is an uncontrolled proliferation of plasma cells in the marrow. The neoplastic cells synthesise abnormal amount of monoclonal immunoglobulins or alpha or lambda light chains [1]. Clinical manifestations of myeloma vary and include bone pains, hypercalcaemia, anaemia, hypogammaglobulinemia, and spinal cord compression etc. [1, 2]. We report here one such case who had an unusual presentation of acute constipation. Case Report A 45-year-old male patient first presented with acute constipation and pain rectal region of 3 days duration. Initially he attributed it to acute exacerbation of fissure-in-ano which he developed 5 years ago. However, clinical examination including per rectal and proctoscopic examination was normal. For next 5 days he was managed with repeated glycerine enema and laxatives but without any relief. At this stage he was subjected to a detailed evaluation. Endoscopic examination of colon, stomach and duodenum failed to detect any abnormality. He was also evaluated for underlying psychiatric disorder but without any positive finding. In view of intractability of symptoms he was referred to us on eleventh day of his illness. A detailed history revealed that at onset he had transient vague pain over the chest at the level of nipple for 2 days. He also gave history of numbness and paraesthesia over lower limbs and hesitance for micturition of 3 days duration. There was no history of pain in the spine or other bones, fever, headache, weight loss, recurrent infections, bleeding diathesis or trauma. There was no history of hypertension, diabetes mellitus or tuberculosis in the past. Clinical examination revealed him to be obese with height 165 cms and weight 80 kgs. There was no pallor, lymphadenopathy or bony tenderness. Neurological examination revealed hypoaesthesia below D6 dermatome (right side being affected more than left side) and weakness in lower limbs. Deep tendon reflexes were sluggish in lower limbs. Abdominal reflexes and plantar reflexes were absent on both sides. There was no papilloedema or signs of meningeal irritation. Tenderness was present over D4 and D5 spine. Examination of other systems was within normal limits. Investigations revealed Hb 12.4 gm per cent, TDLC 6700/cmm with P 59 L38 M2 E1 per cent, ESR 120 mm fall first hour, blood urea 29 mg per cent, serum creatinine 1.0 mg per cent, blood uric acid 8.0 mg per cent and serum calcium 9 mg per cent. Radiograph of chest PA view and 12-lead electrocardiogram were normal. Radiograph of skull, pelvis and long bones in the limbs showed multiple punched out lytic lesions. Scrum electrophoresis showed monoclonal gammopathy, Monoclonal (M) spike in the beta-gamma interzone alongwith hypoalbuminemia and hypogammaglobulinemia. Bone marrow study showed marked infiltration by plasma cells (>30%) with reduction in erythroid and myeloid cells. Magnetic resonance imaging (MRI) of spine demonstrated altered signal intensity of D4-D6 vertebrae. There was an intraspinal soft tissue mass on right side extending from D2 to D5 vertebrae compressing the spinal cord and thecal sac anterioly at that level. There was a paraspinal extension of the mass through intervertebral foramina at D3-4, D4-D6. Radionuclide bone scan showed patchy increased uptake in skull, spine, ribs and major bones of the limbs suggestive of extensive skeletal involvement. Diagnosis of multiple myeloma with myeloradiculopathy was made. He was managed with palliative chemotherapy (vincristine, cyclophosphamide, adriamycin, steroids) and radiotherapy. However, his condition rapidly deteriorated and he died 3 months later.

Publisher Summary This chapter reviews different aspects of mechanisms and treatment of acute and chronic pain. Pain is an unpleasant subjective experience. Most of the time, it signals that something is wrong, for example, some tissue has been injured or has become inflamed, or bodies are being assaulted by extremes of heat, cold, pressure, or other noxious stimuli. When an injured or inflamed part is touched, the patient winces or writhes and withdraws from the stimulus. In addition, autonomic symptoms such as pallor, flushing, or perspiration may be apparent, and the general pattern of activity may be altered. The patient becomes restless or, alternatively, relatively immobile and passive. The chapter presents an introduction to the evaluation of the sensory and psychological determinants of clinical and experimentally induced pain and suffering. It describes the neuroanatomic and neurophysiologic mechanisms of pain and its modulation. It discusses the influence of ethnic and cultural differences and personality on the patient's report of pain and suffering. It also presents a description of pharmacologic, surgical, and behavioral treatments for pain and suffering. The chapter further presents a discussion of the relation between the patient's beliefs and the effectiveness of more controversial treatment approaches, including acupuncture, transcutaneous electrical stimulation, and hypnosis.

This chapter discusses dietary protein intolerance. Clinical food intolerance has many causes and many manifestations, including psychological aversion to the sight, smell, or taste of food as well as psychological intolerance to one or more of the many constituents of food. Dietary protein intolerance is the clinical syndrome resulting from the sensitization of an individual to one or more proteins that have been absorbed via a permeable mucosa in the small intestine. Intolerance to various food proteins, especially to cows' milk, has been recognized in children for many years. Such food intolerance may be the result of a variety of causes—for example, a congenital digestive enzyme defect such as sucrase–isomaltase deficiency or an acquired lactase deficiency secondary to small-intestinal mucosal damage, which in turn can be the result of a food allergy. The incidence of gastrointestinal food allergy diseases is greatest in the first few months and years of an infant's life and decreases with age. The acute syndrome is usually characterized by the sudden onset of vomiting, after cows' milk ingestion, occasionally followed by pallor and a shock-like state; however, acute anaphylaxis is rare. Acute abdominal pain seems to be a particular feature of fish hypersensitivity, while peanuts often produce immediate reactions in the oral mucosa as well as abdominal pain.

This chapter discusses the hematological disorders in brucellosis. The majority of patients with brucellosis have normal hematological pictures. Hematological complications of brucellosis are usually a coincidental laboratory finding. Anemia, when present, is usually very mild and rarely gives rise to any clinical symptoms. Pallor may be noted but is usually very mild. Purpuric rash may rarely be seen and is usually confined to the skin, but may also be noted in the conjunctiva and fundi. Anemia is uncommon in brucellosis in its acute or chronic forms. Chronic infections caused by other organisms such as tuberculosis will cause anemia, which is usually mild. The life span of the red blood cells is slightly shortened by infection and the erythropoietic compensation by the bone marrow is not at its maximum. There is also reduced production of erythropoietin. The serum iron and the total iron binding capacity are low; however, the bone marrow contains an abundant amount of iron. Moreover, hypersplenism has been reported to be the cause of anemia and pancytopenia in brucellosis.

Publisher Summary This chapter discusses the clinical description of the anemia of protein calorie malnutrition (PCM). PCM of various degrees is the most prevalent disease of nutritional deficiency in the world. It affects primarily the young child, although older children and adults of both sexes are not spared. PCM remains one of the major killers in children even when treated in the hospital. Pallor and anemia are among the chief signs of PCM. The present understanding of the anemia of protein calorie malnutrition in children stems from clinical observation, human experimental work, and animal research. The hematological changes observed in children with severe protein calorie malnutrition are primarily an adaptation phenomenon to decrease lean body mass to which various deficiencies and disease states could add their own characteristic changes. The hematological alterations in protein calorie malnutrition must be viewed as a dynamic phenomenon of balance between body composition and the erythron.

Publisher Summary This chapter discusses a few of the common symptoms for psychological conditions of children. A few of these symptoms include sore throat, earache, the running nose, epistaxis, mouth-breathing, tonsils and adenoids, cough, tuberculosis, glands of neck, asthma, vomiting, worms, lassitude, aches and pains, faints, and pallor. The commonest cause of earache is otitis media (middle ear infection). It can also be because of a boil in the ear or an erupting or carious molar tooth. Earache should be reported immediately to the family doctor, as it should be treated straight away if it is because of otitis media. If a child has a yellow thick discharge from the nose except only for a short time at the end of a cold, it means that there is an infection of the antrum (nasal sinus). This should be treated by the family doctor. In the majority of cases, the bleeding is from the front part of the nasal septum and so can be stopped by squeezing the nose between the forefinger and thumb.

Abnormalities of the haemopoietic system may produce anaemia, polycythaemia, lymphadenopathy, hepatosplenomegaly, or purpura alone or in combination. Pallor is the outstanding physical sign of anaemia and is best confirmed by inspection of the conjunctivae and palmar creases. Cardiovascular manifestations of anaemia consist of a high output state with tachycardia and perhaps cardiac failure. If the face and mucous membranes appear plethoric, polycythaemia is suggested and should be confirmed by haemoglobin estimation. A relative polycythaemia may occur in the presence of dehydration. In lymphadenopathy, examination is first carried out from behind, with the patient's neck flexed to relax the sternomastoid muscles. Lymphadenopathy may occur in relationship to the jugular vessels, submandibular and submental regions, and anterior and posterior triangles of the neck. Purpuric spots are spontaneous petechiae or ecchymoses, which represent bleeding into or under the skin. The spots do not blanch on pressure and on fading leave a bluish discolouration as with a bruise. Purpura may be thrombocytopenic or non-thrombocytopenic; it is differentiated by performing a platelet count.