RESUMEN
Background: General anesthesia induces unconsciousness and loss of reflexes, facilitating complex medical treatments. The induction of anesthesia is crucial for patient comfort and procedural success, with propofol and etomidate being common intravenous induction agents. Propofol offers a rapid onset and short duration, while etomidate is known for cardiovascular stability. Methods: A prospective randomized controlled trial involving 100 controlled hypertensive patients compared propofol and etomidate for induction. Hemodynamic parameters and biochemical responses were monitored at various intervals. Injection site discomfort and myoclonus were assessed, and cortisol and glucose levels were measured. Results: Baseline hemodynamic values were similar. Etomidate resulted in stable hemodynamics as compared to propofol. Blood sugars were comparable. Though serum cortisol levels were reduced after etomidate was given. But it came back to normal range 24 hours after surgery. Injection site pain was reported by 20% of etomidate patients and 10% of propofol patients. No myoclonus occurred. Conclusions: Etomidate is an effective induction agent for controlled hypertensive individuals, causing transient adrenal suppression without affecting blood sugar levels.
RESUMEN
Though visceral leishmaniasis (VL) is the leading parasitic infection causing deatharound the world after malaria, it is a less suspected cause of pyrexia of unknown origin (PUO). We present a case of a middle aged man who was diagnosed with VL only months later owing to the stealthily masquerading disease as also to a generally low index of suspicion for it. A 59-year-old from Uttarakhand presented to us with complaint of fever of a few weeks duration. He was found to have a bicytopenia with elevated liver enzymes. Routine imaging studies were non-contributory. Cultures revealed candidemia while tests for viral and other atypical infections were negative. A bone marrow examination (BME) revealed haemophagocytosis. Positron emission tomography–computed tomography (PET-CT) showed mildly FDG avid hepatosplenomegaly. He was treated as a case of candidiasis with secondary hemophagocytic lymphohistiocytosis (HLH) and was discharged. He was readmitted months later with recurring fever. Repeat investigations revealed pancytopenia with marked hepatosplenomegaly. A repeat BME, however, revealed Leishmania donovani (LD) bodies. Patient was treated with liposomal amphotericin B (LAmB) and discharged. Though the patient’s symptoms improved soon after, he was again admitted a couple of months later and found to have VL persisting in the BM aspirate. This report underscores the need to extensively evaluate cases of PUO rather than summarily dismissing them as routine. VL is one of the less suspected etiologies despite being the second largest parasitic killer.
RESUMEN
Background: Hematological abnormalities are prevalent in systemic lupus erythematosus (SLE), with approximately 72% of patients experiencing anemia, primarily in the form of autoimmune hemolytic anemia. Other manifestations include leukopenia (32%), lymphopenia (54%), and thrombocytopenia (23%). This study aimed to further investigate these hematological manifestations, which may serve as presentations of SLE and might be overlooked if suspicion levels are low. Methods: A descriptive observational study was conducted over 18 months at a Sir Ganga Ram hospital, a tertiary care centre. One hundred thirteen SLE cases, comprising newly diagnosed patients and previously diagnosed patients’ records, were reviewed, with 13 cases excluded based on exclusion criteria. One hundred patients with hematological abnormalities and fulfilling ?4 SLICC criteria for SLE diagnosis were included in the study. Results: One hundred cases of SLE with hematological abnormalities (88 women, 12 men) were analyzed. At presentation, 83% (n=84) of patients displayed hematological manifestations. The most prevalent abnormality was anemia, present in 72% of the study group, with a mean hemoglobin level of 10.073 gm/dl. Additionally, leukopenia, lymphopenia, thrombocytopenia, and pancytopenia were observed in 32%, 54%, 23%, and 14% of cases, respectively. Neutropenia was detected in only 5% of cases. Conclusions: Hematological manifestations are the most common presenting signs of SLE in North India. Anemia, with a multifactorial basis, is the most frequent hematological abnormality throughout the disease course. A high index of suspicion is crucial when evaluating cases of anemia in daily clinical practice.
RESUMEN
Sideroblastic anemia is a rare cause of anemia. Most of it accounts for the genetic cause, while drug induced is still uncommon. Our patient, a 20 year old female, is a known case of right frontal tuberculoma on ATT presented with complaints of generalized weakness and loss of appetite. On evaluation, she was found to have severe anemia and bone marrow studies confirmed it to be sideroblastic anemia. On revisiting the history, it was noted that she was not taking pyridoxine supplements as advised along with antitubercular drugs. Our patient is one among the few documented cases of Isoniazid induced sideroblastic anemia.This case needs attention because it is a preventable cause of anemia and the clinicians need to be aware about the compliance of the patient with the supplementary drugs.
RESUMEN
The current Study was done to compare the efficacy of intravenous lignocaine 2% versus oropharyngeal topical 10% xylocaine spray before induction of anaesthesia in attenuating the pressor response to direct laryngoscopy and endotracheal intubation. A total of 60 patients were divided randomly into two groups of 30 patients each. Group I received intravenous lignocaine 2% @ 1.5 mg/kg. Group II received topical 10% xylocaine spray @ 1.5 mg/kg body weight just before induction of anaesthesia. All hemodynamic parameters were measured immediately after laryngoscopy and intubation and at 1, 3, 5 minutes after laryngoscopy and intubation in both the groups. Mean values of haemodynamic parameters in Group I increased after intubation and then started declining but did not reach the baseline even at 5 minutes. In Group II all mean values of haemodynamic parameters increased after intubation and then started declining to almost baseline at 5 minutes. The differences in mean values of haemodynamic between the two groups immediately after intubation and at 1, 3 and 5 minutes thereafter were statistically highly significant (p<0.001). Oropharyngeal topical xylocaine 10% when applied before induction of general anaesthesia is more effective method for attenuating the pressor response to direct laryngoscopy and endotracheal intubation when compared to intravenous lignocaine 2%
RESUMEN
Metaplastic carcinoma of breast is a rare neoplasm. Although it is a tumor of ductal type, the predominant component may have appearance other than the glandular pattern and usually runs an aggressive course.
Asunto(s)
Adulto , Neoplasias de la Mama/diagnóstico , Neoplasias de la Mama/patología , Carcinoma/diagnóstico , Carcinoma/patología , Carcinoma de Células Escamosas/diagnóstico , Carcinoma de Células Escamosas/patología , Femenino , Humanos , Metaplasia , PronósticoRESUMEN
Primary pleomorhic adenoma (Chondroid syringoma) arising from the external auditory canal is very rare and because of the unremarkable clinical presentation, the diagnosis is often made after microscopic examination. We reviewed the worldwide literature and found that only forty one cases have been previously reported. In this report, we describe one such unusual case. The treatment of choice is wide local excision without esthetic or functional disruption of surrounding structures, followed by careful long-term post-operative observation of the clinical course.
Asunto(s)
Adenoma Pleomórfico/diagnóstico , Adenoma Pleomórfico/patología , Adenoma Pleomórfico/diagnóstico por imagen , Adenoma Pleomórfico/cirugía , Adulto , Conducto Auditivo Externo/patología , Conducto Auditivo Externo/diagnóstico por imagen , Humanos , Masculino , Literatura de Revisión como Asunto , Tomografía Computarizada por Rayos XRESUMEN
Lupus vulgaris (LV) is the commonest morphological variant of cutaneous tuberculosis. Case of LV of external nose extending to internal nose causing septal perforation is documented here. Histopathology of biopsy taken confirmed the diagnosis of LV. Patient responded well to Anti-tubercular therapy (ATT).
RESUMEN
Chronic retropharyngeal abscess secondary to tubercular spondilitis is a rare phenomenon. Anatomical location of this abscess makes it a life threatening condition requiring prompt diagnosis and treatment thus preventing morbidity and mortality. Authors are documenting a case of tubercular spondylitis of cervical region in a 12-year old male child with huge retropharyngeal abscess extending to superior mediastinum. Clinically, patient had respiratory distress but no neurological deficit. USG (Ultrasonography) guided aspirate of abscess sent for microscopy and culture showed acid fast bacilli. Multiple USG guided aspirations under antibiotic and antitubercular cover (Category I) were done. Patient is doing well at three month follow-up.
RESUMEN
Dengue is a worldwide condition spread throughout the tropical and subtropical zones between 30 degrees north and 40 degrees south. It is endemic in South East Asia, the pacific, East and West Africa, the Caribbean and the Americas. Dengue outbreaks are occurring almost every three yearly in Delhi for the last 12 years. The latest outbreak was in the year 2006, which started late in August, peaked in the month of October and lasted till late November. We describe here the clinical, hematological and biochemical data of 70 patients of dengue fever diagnosed as per WHO criteria in Lok Nayak Hospital, New Delhi during this outbreak. Hematological parameters were estimated by automated counter and dengue serology was done by capture ELISA technique. The results displayed widespread effect of dengue fever on hematological and biochemical profile. Some of our patients also had atypical dengue manifestations. These results suggest that dengue fever is a major public health problem which can lead to significant morbidity and can even be fatal at times. All efforts should be made to prevent it.
RESUMEN
Branchio-oto-renal syndrome (Melnick-Fraser syndrome) is a rare autosomal dominant disorder characterized by syndromic association of branchial cysts or fistulae along with external, middle & inner ear malformations and renal anomalies. Authors are reporting a 19 year male patient, who presented with profound deafness & low set "lop-ear" with right sided preauricular pit. USG abdomen revealed agenesis of the left kidney.
Asunto(s)
Anomalías Múltiples/genética , Adulto , Síndrome Branquio Oto Renal/complicaciones , Branquioma/complicaciones , Sordera/complicaciones , Diagnóstico Diferencial , Oído/anomalías , Humanos , Riñón/anomalías , MasculinoAsunto(s)
Adulto , Humanos , Masculino , Enfermedades de la Lengua/diagnóstico , Lengua Vellosa/diagnósticoRESUMEN
Nemaline rod myopathy (NM) is a rare form of congenital myopathy characterized by slowly progressive or nonprogressive muscle weakness and pathognomonic rod-like structures within the muscle fibers. To the best of our knowledge, this is first documentation of the clinicopathological features of this rare entity from India. All cases of NM diagnosed in our laboratory were retrieved. Clinical and pathological features were reviewed. During a period of 1.5 years (Jan 2004 to June 2005), we received 750 muscle biopsies for various reasons. Of which, 15 were diagnosed as congenital myopathies and four as nemaline rod myopathies. Thus, NM comprises 0.53% of all muscle diseases and 22.6% of all congenital myopathies. All of them presented in childhood (first five years of life) with generalized hypotonia, feeding problems, repeated respiratory infections and muscle weakness. Both males and females were equally affected. The CPK levels were normal and EMG was myopathic. Microscopic examination revealed minimal changes but characteristic red-colored material was seen on modified Gomori trichrome staining which was immunopositive to alpha actinin. Ultrastructural examination confirmed this material to be nemaline rods. NM, although a rare form of congenital myopathies, should be suspected in children who present with generalized hypotonia, repeated chest infections and slowly progressive muscle weakness. This report highlights the importance of histochemistry and ultrastructural examination in the diagnosis of this entity, in the absence of the availability of methodology for genetic studies.
Asunto(s)
Niño , Preescolar , Electromiografía/métodos , Femenino , Humanos , Lactante , Masculino , Microscopía Electrónica de Transmisión/métodos , Músculo Esquelético/patología , Miopatías Nemalínicas/patología , Enfermedades Neuromusculares/complicacionesRESUMEN
BACKGROUND: Multi-minicore disease is a rare form of myopathy characterized by slowly progressive or nonprogressive muscle weakness and characteristic multiple cores within the muscle fibers. To the best of our knowledge, this is first documentation of the clinicopathological features of this rare entity from India. MATERIALS AND METHODS: A ll cases of multi-minicore disease diagnosed in our laboratory were retrieved. Clinical and pathological features were reviewed. RESULT: During a period of two years (January 2004 to December 2005), we received 985 muscle biopsies for various reasons. Of which, 15 were diagnosed as myopathies and four of which were of multi-minicore disease. Thus, multi-minicore disease comprises 0.40% of all muscle diseases and 26.6% of all myopathies. All were male and presented in early childhood (first decade of life) with generalized hypotonia and muscle weakness. All of them had dysmorphic facies and three had high arched palate. CPK levels were normal and EMG was myopathic except in one patient. Microscopic examination revealed minimal changes with Type I fibers' predominance but characteristic multiple cores in the myofibers. Ultrastructural examination showed both structured and unstructured cores. CONCLUSIONS: Multi-minicore disease, although a rare form of myopathies, should be suspected in children who present with generalized hypotonia and slowly progressive muscle weakness along with dysmorphic facies.
Asunto(s)
Niño , Preescolar , Electromiografía/métodos , Humanos , Masculino , Microscopía Electrónica de Transmisión/métodos , Fibras Musculares Esqueléticas/patología , Debilidad Muscular/fisiopatología , Anomalías Musculoesqueléticas , Miopatía del Núcleo Central/patología , Estudios RetrospectivosRESUMEN
CONTEXT: Nephrotic syndrome is one of the commonest renal problem encountered in children. It is difficult to predict at onset, the clinical course in terms of steroid responsiveness or resistance. Angiotensin converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism has been studied as a predictor of clinical course in common multi-factorial diseases including focal segmental glomerulosclerosis. There is no study available from our country till date to find out any correlation of the steroid response in idiopathic nephrotic syndrome and ACE gene polymorphism. AIM: To study distribution and correlation of ACE gene I/D polymorphism in idiopathic steroid sensitive nephrotic syndrome. SETTINGS & DESIGN: Case-control retrospective study. SUBJECTS & METHODS: We studied ACE gene polymorphism in 90 consecutive patients (82% males, 18% females) with steroid sensitive idiopathic nephrotic syndrome and 300 normal controls (NC). STATISTICAL ANALYSIS: Chi-square test and Fisher's exact test (for cases with insufficient expected cell frequencies). RESULTS: The mean age of onset was 5.3 +/- 4 years. Steroid sensitive (SS) patients showed II (SS-48%, NC-26%) genotype was more frequent than normal controls (p=0.002). There was no significant difference in genotype frequencies among steroid SS subgroups. CONCLUSIONS: In our study II genotype was more frequent in steroid sensitive nephrotic syndrome children in comparison to normal controls. Further functional studies with large number of children are required to investigate the role of II genotype in steroid sensitive nephrotic syndrome. Comparison of the genotypic frequency with steroid resistant patients may provide information, which might be useful in clinical practice.
Asunto(s)
Preescolar , ADN/genética , Femenino , Estudios de Seguimiento , Frecuencia de los Genes , Genotipo , Glucocorticoides/uso terapéutico , Humanos , India/epidemiología , Masculino , Síndrome Nefrótico/tratamiento farmacológico , Peptidil-Dipeptidasa A/sangre , Reacción en Cadena de la Polimerasa , Polimorfismo Genético , Prevalencia , Estudios RetrospectivosRESUMEN
BACKGROUND: Limb girdle muscular dystrophy (LGMD) is a phenotypic expression of a heterogeneous group of diseases and sarcoglycanopathy is one of the causes of LGMD. There is only one study on sarcoglycanopathies in the Indian literature. No data is available from northern India. MATERIALS AND METHODS: All cases of muscular dystrophies, which were diagnosed in our laboratory in the last six years, were reviewed. Immunohistochemistry for various sarcoglycan proteins was done. Clinical features and pathological findings of the cases that were diagnosed as sarcoglycanopathies were reviewed. RESULTS: In the last 6 (1/2) years (1998-June 2004), we received 1435 muscle biopsies, of which 498 cases were of muscular dystrophies, and 13 cases were of sarcoglycanopathies (8 of gamma, 3 of alpha, 1 of both alpha and gamma, and 1 with absence of all four sarcoglycans). Sarcoglycanopathies comprised 2.6% of all muscular dystrophies, 11.8% of LGMD and 0.90% of all muscle diseases diagnosed in our laboratory. The mean age of onset was 7.2 years and the M:F ratio was 1.1:1. Most of them presented with difficulty in getting up, climbing stairs, calf hypertrophy and markedly raised CPK levels. Histological features were like dystrophinopathies. CONCLUSION: Sarcoglycanopathies are a relatively rare cause of LGMD and should be confirmed by immunohistochemistry as it will facilitate counseling and also prognostification. Although rare, in patients with muscle weakness, calves hypertrophy and raised CPK levels this possibility should be considered and needs to be differentiated from dystrophinopathies.
Asunto(s)
Adolescente , Biopsia , Niño , Preescolar , Femenino , Humanos , Masculino , Músculo Esquelético/patología , Distrofia Muscular de Cinturas/diagnóstico , Estudios Retrospectivos , Sarcoglicanos/deficienciaRESUMEN
Congenital fiber type disproportion is a rare type of congenital myopathy which presents as hypotonia, delayed motor milestones and dysmorphic facies. During the past 2 years we received 449 muscle biopsies, of which 4 cases were diagnosed as congenital fiber type disproportion (CFTD). In addition to CFTD, one case also had centronuclear features. Three of them were females and one was a male child. Although rare, it should be considered in the differential diagnosis of childhood muscle diseases. Histochemical staining is necessary for the diagnosis of this entity.
Asunto(s)
Adolescente , Biopsia , Niño , Preescolar , Diagnóstico Diferencial , Femenino , Histocitoquímica/métodos , Humanos , Masculino , Músculo Esquelético/metabolismo , Miopatías Estructurales Congénitas/metabolismo , Coloración y EtiquetadoAsunto(s)
Preescolar , Humanos , Lipidosis/patología , Masculino , Enfermedades Musculares/patologíaRESUMEN
A case of tubercular osteomyelitis of nasal bones in a 10 year old child is being reported because of its extremely rare occurrence.