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PubMed1983

Differential diagnosis between amoebic liver abscess and acute cholecystitis.

The Research Committee of the World Organization of Gastroenterology has gather information regarding the etiology of acute abdominal pain. Seven diseases cover 96% of the causes of this syndrome in many countries of the world, but some geographical variations have been observed. One example of these variations is amoebic liver abscess, present in 5 to 10% of Mexico City patients. Right upper quadrant pain is often present in amoebic liver abscess and acute cholecystitis. Thus, differential diagnosis of these two entities is difficult. Using discriminant analysis and "stepwise" procedures in 100 cases with cholecystitis and a similar number of patients with amoebic liver abscess, we found six variables (symptoms and signs with a significant chi square to distinguish between these two diseases. The symptoms and signs chosen were hepatomegaly, Murphy's sign, duration of pain greater than or equal to 48 hours, previous history of abdominal pain, dysentery, and facial pallor. These variables proved to be better than laboratory test results. With five of these variables it was possible to obtain an accuracy of 92%. Using six variables, if cases of tie (three variables present and three absent) were excluded, accuracy rose to 96%.

Abdomen, Acute↗
PubMed1993

Spasm of gastric muscularis mucosae might play a key role in causing focal mucosal ischemia and ulceration. An experimental study in guinea pigs.

While transilluminating guinea pig gastric wall, we noticed foci of irregular compression of submucosal veins and arteries, featuring parallel bands across arteries, with complete cessation of blood flow, mucosal pallor, and shrinkage of the area. Other features demonstrated that this was not due to artifactual pressure. We attribute it to compression of vessels by spasm of the muscularis mucosae, because these vessels are embedded in the muscle. Arteries and veins returned to normal with normal mucosal perfusion, either spontaneously within 5 min, or within 5 sec following mucosal stimulation. However, in 20%, "ischemia" remained for 2-3 hr, resulting in full-thickness necrosis of the underlying mucosa but not in surrounding areas. Since in man mucosal arteries pass through the muscularis mucosae before reaching mucosa, and in view of evidence that they may be end arteries, we suggest that stress-induced spasm of muscularis mucosae may play an important role in human acute and chronic ulceration.

Animals↗
PubMed1993

Thalassemia intermedia: compound heterozygous beta zero/beta(+)-thalassemia and co-inherited heterozygous alpha(+)-thalassemia.

The relative excess of alpha- over beta-globin chains in the erythroid precursors is the chief pathophysiological factor of homozygous beta-thalassemia. The clinical picture is usually characterized by a transfusion-dependent dyserythropoietic anemia (thalassemia major). However, some patients present with moderate anemia that does not require regular blood transfusions (thalassemia intermedia). The molecular heterogeneity of beta-thalassemia mutations and changes of alpha- and gamma-globin gene expression play an important role in modifying the clinical phenotype. We report here on a female Greek patient with homozygous beta-thalassemia but normal growth and development, excellent exercise tolerance, and no need of blood transfusions. She is thus mildly affected clinically, although there is marked pallor, jaundice, and hepatosplenomegaly. These signs correspond to her marked hypochromic, microcytic anemia with erythroid hyperplasia of the bone marrow. beta-Globin genotyping shows here to be compound heterozygous for the codon 39 C-->T beta zero-nonsense mutation and for the T-->C beta(+)-mutation at position 6 of the splice consensus at the exon 1/intron 1 junction (CD39 C-->T/IVS1-6 T-->C). alpha-Globin gene mapping demonstrates the presence of a 3.7-kb alpha (+)-thalassemia deletion on one allele (-alpha 3.7/alpha alpha). Taken together, this study identifies a complex interaction of genetic factors that do not significantly alter the clinical phenotype when present alone but ameliorate the course of homozygous beta-thalassemia when inherited in combination.

Base Sequence↗
PubMed1978

Phenylketonuria in Indian children.

Three untreated phenylketonuric Indian children aged respectively 3 1/2 years, 1 1/2 years and 1 year showed rapid neurological deterioration. Plasma, cerebrospinal fluid and urine phenylalanine concentrations were significantly raised and the phenylalanine-tyrosine ratio was high. Analysis of a biopsy of the right frontal lobe of the brain in one case showed the myeline lipids--cerebroside and sulphatide--to be decreased. The total cerebroside in white matter was low. Light microscopy showed marked pallor of the white matter of the brain and extensive spongy degeneration. Ultrastructurally these spongy vesicles are located between the lamellae of the myelin sheath.

Brain Chemistry↗
PubMed1991

Coombs-positive giant cell hepatitis of infancy: effect of steroids and azathioprine therapy.

An 8-month-old boy and a 7-month-old girl presented with an acute, Coombs-positive auto-immune haemolytic anaemia and severe hepatitis. The clinical manifestations were pallor, jaundice and hepatomegaly. The liver histology revealed diffuse giant cell transformation and extensive necrosis with central-portal bridging. Combined immunosuppressive regimen with steroids and azathioprine led to prolonged clinical and biological remission with a respective 2 years and 7 months follow up. The girl, however, after 7 months developed a progressive encephalopathy of unknown aetiology, while liver and haematological disease were still under control. She died subsequently from severe recurrent seizures. We conclude that acute Coombs-positive giant cell hepatitis of infancy can be improved by sustained immunosuppressive therapy.

Acute Disease↗
PubMed1996

Liver glycogen synthase deficiency: a rarely diagnosed entity.

UNLABELLED: Three children from two German families are described and the observation compared with the previously published three families comprising eight patients. The two index cases presented with morning fatigue, had ketotic hypoglycaemia when fasting which rapidly disappeared after eating, and hepatic glycogen deficiency and absent or very low hepatic glycogen synthase activity. Metabolic profiles comprising glucose, lactate, alanine, and ketones in blood were typical for hepatic glycogen synthase deficiency. Symptoms were rapidly relieved and chemical signs corrected by introducing frequent protein-rich meals and night-time feedings of suspension of uncooked corn (maize) starch. The discovery of oligosymptomatic and asymptomatic siblings suggests that there are more persons with undiagnosed hepatic glycogen synthase deficiency. CONCLUSION: Liver glycogen synthase deficiency is likely to be more common than is believed today. It should be sought in children who, before the first meal of the day, present with drowsiness, lack of attention, pallor, uncoordinated eye movements, disorientation or convulsions and who have hypoglycaemia and acetone in urine.

Age of Onset↗
PubMed1995

Dumping syndrome in a young child.

UNLABELLED: We describe a 17-month-old child with dumping syndrome after plication of the right diaphragm. He presented with periods of abdominal distension and pallor, recurrent convulsions, glucosuria and refusal of feeding. After changing the diet the symptoms disappeared. CONCLUSION: Although dumping syndrome in children is rare, early recognition is important. Serial determination of blood glucose after bolus feeding can lead to the diagnosis. Treatment should consist of dietary changes.

Blood Glucose↗
PubMed1991

Dumping syndrome after combined pyloroplasty and fundoplication.

Dumping syndrome in infancy is a rare complication following gastric surgery. We describe an 11-month-old infant affected by recurrent peptic oesophagitis who underwent a combined Nissen fundoplication and pyloroplasty. Early dumping symptoms such as irritability, pallor, sweating, abdominal distension and watery diarrhoea were observed postoperatively after bolus feeding. Gastric emptying, measured after the administration of 150 ml of regular cow milk mixed with 200 microCi (8 MBq) of technetium-99m sulfur colloid (99mTc-SC), demonstrated an early rapid and massive emptying of the isotopes into the small intestine, followed by duodenogastric reflux and a second wave of emptying and reflux at 9 min. The initial pattern of gastric emptying and duodenogastric reflux was followed by a slow emptying phase with half-emptying time of 81 min. Isotope studies should be used to investigate motility disorders caused by this type of anti-reflux operation.

Dumping Syndrome↗
PubMed1986

Respiratory and circulatory influence on photopapillometry.

Respiratory and circulatory conditions that could have an influence on measurements of pallor of the papilla were evaluated with our photopapillometer in a controlled study on canines. The brightness of the papilla rim and the width of the arterioles emerging from the optic nerve head were measured in six anesthetized dogs under normal, hypertensive, and hypotensive circulatory conditions, as well as under various respiratory manipulations, and the parameters were compared for statistical correlation. Both brightness and vessel width remained constant for a wide range of arterial pressures when the rest of the experimental conditions were kept stable at naturally occurring levels. If respiration and circulation were brought to extreme levels, however, there was a statistical tendency for the brightness of the papilla to be dependent on arterial and pulmonary arterial blood pressure. The importance of these findings is discussed with regard to their influence on objective measurements of fundus brightness.

Animals↗
PubMed1982

Measurement of the optical density of the optic nerve head II: short-time fluctuations of the optical density of the nerve head.

The fluctuation of pallor with increasing IOP could be due to either insufficient reliability of the method or to an unrecognized pulsating phenomenon. A better arrangement should eliminate imperfection of the method. Coupling the release with the ECG should reveal a relationship to the arterial pulse rate. Neither condition could be demonstrated as the reason for the fluctuation. The connection of the fluctuation and its possible relationship to central venous pressure is discussed. The margin of error is shown, which is 9% on average.

Electrocardiography↗
PubMedDate not supplied

Apoptosis occurs in a new model of thermal brain injury.

Apoptosis has been implicated recently as a prominent response of the brain to a variety of insults, such as ischemia and trauma. In this study, we demonstrate that apoptosis is a prominent part of the brain's response to a thermal insult. To examine the brain's response to a thermal insult, a new model of thermal brain injury in the laboratory rat was developed. Water heated to 60 degrees C was passed over an area of thinned calvarium for 1 min. This resulted in an actual brain temperature of 47-48 degrees C. A uniform area of 2,3,5-triphenyl-tetrazolium chloride pallor was demonstrated and pyknotic neurons were seen in the area of injury by hematoxylin-eosin staining. Apoptosis was demonstrated by the characteristic DNA fragmentation seen by agarose gel electrophoresis, ApopTag in situ staining and electron microscopy. The findings of apoptosis were localized to the area of thermal injury and were time dependent, starting 6 h after the insult and peaking approximately 18 h after the insult. This represents one of the first demonstrations that apoptosis occurs in the brain in response to a thermal injury.

Animals↗
PubMed1996

Infant behaviour in response to a change in body position from side to prone during sleep.

UNLABELLED: The consequences of provoking a change in the sleeping position from side to prone during quiet (non-REM) and active (REM) sleep in young infants were studied in terms of ability to turn the face away from the mattress within 3 min, and in terms of ventilatory and heart rate responses in those who remained face down. Twenty-six infants were exposed to repeated tilts from the side to prone at 2.5 months, and 20 at 5 months of age. Eighteen infants were tested on both occasions. A computer-aided multichannel system was used for polysomnographic recordings. Approximately 66% of the infants did not rotate the face away from the mattress on at least one occasion. There were no significant differences in the rate of face down outcome between sleep states or ages, and no consistent pattern of final face position following repeated tilts. The face to side position was commonly accomplished after considerable difficulties involving vigorous body movements, particularly if the arm became positioned between the body and the mattress or alongside the trunk after the tilt. Apnoeas of 3-14 s in immediate response to the tilt were observed in 75% of the infants monitored electronically. In 13% of the infants, all in a face down position, the test was terminated because of increases in heart and respiratory rates, drop in oxygen saturation, or marked pallor. CONCLUSIONS: The observed difficulties of obtaining a face to side position when suddenly exposed to the prone position during sleep, may render some young infants at risk of sudden infant death (SID).

Apnea↗
PubMed2003

Necrotizing fasciitis with chickenpox.

Necrotizing fasciitis can be observed in a wide variety of clinical settings in the pediatric age group. But necrotizing fasciitis as a complication of chickenpox is rare. Five cases of necrotizing fasciitis following chickenpox were treated in the authors' institutions. Clinical signs like fever, tachycardia, pallor, erythema, marked edema of the affected area and patchy areas of discoloration or gangrene of the skin were present and were the important clues to the seriousness of infection. Patients were treated with aggressive surgical debridement, frequent dressings, broad-spectrum antibiotics, nutritional support, and split skin grafting. Early diagnosis and aggressive surgical therapy are critical. Literature search showed necrotizing fasciitis following chickenpox has not been reported in India. We feel early suspicion and aggressive management can decrease the morbidity and mortality in this rare complication.

Chickenpox↗
PubMed2000

Adolescent nutrition in a rural community in Bangladesh.

The objective of this study was to assess the nutritional status of adolescent boys and girls in a rural community in Bangladesh. Between December 1996 and January 1997, a cross-sectional survey was carried out in 803 households, each containing at least one adolescent, sampled consecutively from four purposely-selected villages in Rupganj Thana, Narayanganj district. Initially, the guardians of 1483 healthy and unmarried 10-17 year old adolescents (51% boys and 49% girls) were interviewed about family structure and socio-economic status. Out of these children, 906 (47% boys and 53% girls) from 597 households were weighed, had their height and MUAC measured and were clinically examined. Blood was then collected from 861 adolescents for haemoglobin estimation. The median monthly income per person in these 597 families was approximately Taka 554 (US $12). Twenty seven per cent of the household heads were labourers, 21% were solvent farmers, 14% ran small scale businesses and 6% were unemployed. Sixty seven per cent of adolescents were thin (defined as BMI < 5th centile of WHO recommended reference) with 75% boys and 59% girls being affected. The percentage of thin adolescents fell from 95% at age 10 years to 12% at age 17 years. The prevalence of stunting (height for age < 3rd centile NCHS/WHO) was 48% for both boys and girls and rose from 34% at age 10 to 65% at age 17. On clinical examination angular stomatitis was present in 46%, 27% had glossitis, 38% had pallor, 11% had dental caries, 3.2% had an conspicuously enlarged thyroid and 2.1% had eye changes of vitamin A deficiency. According to INACG (International Nutritional Anaemia Consultative Group, 1985) cut-off values, 94% of the boys and 98% of the girls were anaemic. We conclude that rural Bangladesh adolescents suffer from high rates of malnutrition and almost universal anaemia. Nutritional interventions to target this population are urgently required.

Adolescent↗
PubMedDate not supplied

Osteopetrosis--improvement of hematologic findings with age.

A three months old girl was admitted to the hospital for evaluation of pallor and abdominal distension. There was anemia, thrombocytopenia and extramedullary hematopoiesis. In the long bone roentgenogram medullary areas could not be distinguished and bone densities were increased. The patient was diagnosed for osteopetrosis with these findings and prednisolone treatment was begun and then discontinued since it was not effective. In the follow up, the need for blood transfusions decreased and then disappeared. We present a case which was diagnosed as osteopetrosis and followed up by blood transfusions and of which hematologic findings improved with age.

Age Factors↗
PubMedDate not supplied

Autoimmune hemolytic anemia.

Immune hemolytic anemia can be either isoimmune or autoimmune. Autoimmune hemolytic anemias (AIHA) consist of group of disorders whose common characteristics are the presence of an antibody which in turn causes short red blood cell (RBC) life. The rate and site of hemolysis and hence the clinical manifestations depends on the type of antibody attached and its propensity to fix complement. Antibodies of the IgG class are most commonly responsible for AIHA in children. Rh erythrocyte antigen is involved in more than 70% of cases. Since the antibody has its maximal activity at 37 degrees C, the resultant hemolysis is called warm antibody induced hemolytic anemia. This is a severe life threatening condition, the clinical features are: sudden onset of pallor, jaundice and dark urine. The cornerstone of diagnosis is a positive Coomb's antiglobulin test in the presence of hemolysis. Coomb's test has false negative and false positive rates in about 2-4% and 8% of all cases respectively. The modalities for treatment of warm AIHA include blood transfusion, steroid therapy, intravenous gammaglobulin, plasma-pheresis and splenectomy. The choice depends on the severity of the disease and child's response to therapy.

Anemia, Hemolytic, Autoimmune↗
PubMed2005

Vaginal endodermal sinus tumor.

Malignant germ cell tumors are rare tumors of childhood accounting less than 3% of pediatric malignancies, and endodermal sinus tumor (EST) is the most common histological subtype. The vagina is an extremely rare site for germ cell tumors (GCT). A one-year female was admitted with history of bleeding per vagina. She had pallor and a mass was palpable anteriorly on rectal examination. Magnetic Resonance Imaging (MRI) showed a tumor mass at the junction of cervix and vagina and biopsy was suggestive of a vaginal EST. The serum alpha fetoprotein (AFP) was elevated. She underwent vaginohysterectomy. The tumor was excised in toto and patient received four courses of cisplatin, etoposide and bleomycin. At one year of follow up, patient was symptom free and serum AFP remained at normal level. EST of the vagina is a rare, highly malignant GCT that exclusively involves children less than 3 years of age. It is both locally aggressive and capable of metastasis. Even though more conservative surgery is advised to maintain sexual and reproductive function, at times, radical surgery is mandatory depending on the infiltration of the tumor to the surrounding structures. Simple tumor excision is not sufficient, as residual cells induce recurrence and make chemotherapy ineffective. The serum AFP level is a useful marker for diagnosis and monitoring the recurrence of vaginal EST in infants.

Endodermal Sinus Tumor↗
PubMedDate not supplied

Congenital tuberculosis.

A 29 day old male infant presented with a history of fever, cough, increasing respiratory distress and abdominal distension from the 10th day of life. Examination revealed failure to thrive, marked tachypnea, pallor, hepatosplenomegaly and harsh vesicular breath sounds. Chest skiagram showed extensive broncho-pneumonic changes. As the infant did not respond to antibiotics, he was investigated for tuberculosis. The gastric aspirate smear showed plenty of acid fast bacilli (AFB) and culture showed M. tuberculosis growth. Endometrial biopsy of the mother showed tuberculous granuloma and acid fast bacilli and culture of the aspirate from endometrium grew M. tuberculosis. The need for endometrial biopsy of mothers of infants with congenital tuberculosis is highlighted.

Antitubercular Agents↗