Biomedical subjects
Publications and source records attributed to M S Tullu.
We report here a 2.5-year-old male child with community-acquired Pseudomonal sepsis showing the characteristic lesions of ecthyma gangrenosum. The child had development of gangrenous changes of the nose and face - the 'cancrum oris' or 'Noma'. We highlight the possible association of Pseudomonas sepsis and Noma, with malnutrition playing a central role in causing both the diseases.
Infantile tremor syndrome is characterized by coarse tremors, mental and physical retardation, light colored brown hair, skin pigmentation and anemia. Amongst the theories proposed for the etilogy of the disorder, the nutritional theory is most accepted. In this case report, we have presented a fourteen-month-old male child with ITS and documented zinc deficiency. Though most of the previous workers have proposed vitamin-B12 deficiency as the etimology for ITS, our report suggests that zinc deficiency could also have a causative role.
Intrahepatic biliary cysts are rarely seen in the patients with biliary atresia. We describe a ten-month-old child with biliary atresia in whom the abdominal imaging studies (ultrasonography, computed tomographic scan and magnetic resonance cholangiopancreatography) revealed multiple intrahepatic biliary cysts ('bile lakes'). The child also had intrapulmonary shunting of blood due to pulmonary arteriovenous fistulae, which were demonstrated on contrast-enhanced echocardiography. Both these findings, 'bile lakes' and pulmonary arteriovenous fistulae occur rarely in biliary atresia.
Glanzmann's thrombasthenia (GT) is an uncommon cause of bleeding in children. We diagnosed two siblings as having GT on the basis of flow cytometric studies. Both had cutaneous bleedings and epistaxis since early childhood. Hematological investigations revealed prolonged bleeding time and a normal platelet count. Both the patients had absence of aggregation of platelets with the agonist adenosine diphosphate. Absence of the GPIIb/IIIa receptor was confirmed by flow cytometry. A short review of the disorder is presented.
Pulmonary arteriovenous malformations rarely present in childhood. Two cases are presented in this report. Both the cases presented clinically with cyanosis and clubbing without a cardiac murmur. The second case had cerebral abscess in addition. Both the cases underwent a contrast-enhanced echocardiography which suggested the presence of pulmonary arteriovenous malformations. The first case also underwent 99mTc radionuclide scan and pulmonary angiography. The cases are being reported for their characteristic clinical features and for emphasizing the role of non-invasive modalities like contrast-enhanced echocardiography and radionuclide scan in reaching the diagnosis.
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OBJECTIVE: To study clinical features and investigations of children with Krabbe Disease (KD). DESIGN: Retrospective. SETTING: Genetic Clinic of a tertiary care teaching hospital. METHODS: Hospital records of patients with enzymatically confirmed KD were analyzed with respect to their clinical features and investigations including neuroimaging (CT-scan and/or MRI). Galactocerebrosidase (GALC) activity was estimated photometrically in the peripheral blood leukocytes. RESULTS: Nine children (age ranging from 2 1/2 months to 8 years) were studied, of which 5 had the classical infantile disease, 3 had late infantile form and one was diagnosed as juvenile KD. GALC levels in peripheral blood leukocytes were low or absent in all. Most of the children with infantile disease presented with neurodegeneration, seizures or fever. Optic atrophy was uncommon in our series (present only in a single case). Majority of the cases had elevated cerebrospinal fluid (CSF) protein levels and peripheral neuropathy on nerve conduction (NC) studies. Findings typical of KD were noted in 7 patients who underwent magnetic resonance imaging (MRI). The sole patient with juvenile disease presented with developmental delay and progressive spastic quadriparesis. CONCLUSION: Krabbe disease should be considered in the differential diagnosis of early infantile onset of neurodegeneration with seizures. Likewise, older children with progressive ataxia or spastic quadriparesis in whom the etiology remains obscure, must be investigated for Krabbe disease. MRI can be diagnostic in absence of availability of enzyme diagnosis.
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This prospective study was carried out over a period of 6 months in the Paediatric Intensive Care Unit (PICU) of a tertiary care teaching hospital. The aim of the study was to determine the organisms causing catheter related nosocomial infections in the PICU and to study their antimicrobial susceptibility pattern. Patients with endotracheal intubation, indwelling urinary catheters and central venous catheters (CVC)/venous cutdown catheters were included in the study. Colonization of the endotracheal tube, urinary catheter related infections (UCRI) and colonization of the CVC/venous cutdown catheters was studied. E. coli was the commonest organism colonizing the endotracheal tube tip with maximum susceptibility to cefotaxime and amikacin. E. coli was also was the commonest organism causing UCRI with maximum susceptibility to nitrofurantoin and amikacin. Acinetobacter was the commonest organism colonizing the CVC/venous cutdown catheters with maximum susceptibility to ciprofloxacin. All these sites of catheter related infections considered together, E. coli and Klebsiella were the commonest nosocomial organisms. Both had maximum susceptibility to amikacin. Methicillin resistant Staphylococcus aureus (MRSA) was isolated only from one culture. All the organisms had a poor susceptibility to cefazolin and amoxycillin. A knowledge of the resident microbial flora and their antimicrobial susceptibility pattern is necessary for formulating a rational antibiotic policy in an ICU.
The present prospective study was carried out in the Paediatric Intensive Care Unit (PICU) of a tertiary care teaching hospital in Mumbai. The objective was to determine the incidence, risk factors, mortality and organisms responsible for urinary catheter related infections (UCRI). Colonization and/or bacteriuria was labelled as urinary catheter related infection (UCRI). Forty-four patients with 51 urinary catheters were studied. Incidence of UCRI was 47.06%. Age, female sex and immunocompromised status did not increase the risk of UCRI. Duration of catheter in-situ and duration of stay in the PICU were associated with higher risk of UCRI. The mortality was not increased by UCRI. Commonest organism isolated in UCRI was E. coli, which had maximum susceptibility to nitrofurantoin and amikacin.
Prenatal diagnosis of cri-du-chat syndrome is described in 2 pregnancies. In Case 1, the mother was a balanced translocation carrier and had 2 previously affected off springs. Prenatal diagnosis by chorion villus sampling and cordocentesis was successful in diagnosing an affected conceptus and the pregnancy was electively terminated. Case 2 was referred for nonimmune foetal hydrops and cordocentesis revealed deletion 5p. This second case was noteworthy for the fact that deletion 5p has not been reported to cause foetal hydrops.
Penicillin-induced immune haemolytic anaemia is very rare. A ten year-old-female with rheumatic mitral stenosis on benzathine penicillin prophylaxis presented with features of haemolytic anaemia and investigations supported the diagnosis of immune haemolytic anaemia. Patient responded to discontinuation of the drug and therapy with oral prednisolone. This is first such case reported from India.
A two and half year-old-male child, known case of steroid responsive nephrotic syndrome presented with fever and vomiting of acute onset. He was diagnosed to have superior sagittal sinus thrombosis on a contrast computerised tomographic scan of brain. Recovery was complete without anticoagulant therapy. Superior sagittal sinus thrombosis is an extremely rare complication of nephrotic syndrome.
AIMS: To determine the incidence, risk factors, mortality and organisms causing nosocomial pneumonia (NP) in intubated patients in Paediatric Intensive Care Unit (PICU). MATERIALS & METHODS: All patients with endotracheal (ET) tube with or without mechanical ventilation (MV) in a PICU of a tertiary care teaching hospital were included in this prospective study. Clinical parameters and investigations were evaluated in patients who developed nosocomial pneumonia (NP). Colonisation of the ET tube tip was studied by culture and the antibiotic susceptibility pattern of the isolates was determined. RESULTS: Sixty-nine patients had an ET tube inserted and fifty-nine of these underwent MV. ET tube tip colonisation was seen in 70 out of 88 ET tubes inserted. The incidence of NP in patients with ET tube was 27.54% (7.96/100 days of ET intubation). NP developed only in patients undergoing MV. The main risk factors for developing NP were - duration of MV and duration of stay in the PICU. Age, sex, immunocompromised status and altered sensorium did not increase the risk of NP. The mortality in cases with NP was 47. 37%. E. coli and Klebsiella were the commonest organisms isolated from the ET tube tip cultures with maximum susceptibility to amikacin and cefotaxime. CONCLUSIONS: NP developed only in patients undergoing MV. Duration of MV and duration of stay in the PICU increased the risk of developing NP.
Neurofibromatosis (NF), Noonan syndrome (NS), and LEOPARD syndrome are all autosomal dominant conditions, each being a distinct clinical entity by itself. Rarely, one encounters cases with features of NF and NS and is termed as the 'Neurofibromatosis-Noonan syndrome' (NF-NS). The authors report a clinical dilemma with major clinical features of the NF-NS syndrome and LEOPARD syndrome co-existing in the same patient. Also, features of Noonan syndrome and LEOPARD syndrome are compared with the case reported.