Clinical profile of pediatric HIV infection from India.

BACKGROUND: Our aim was to study the clinical profile of pediatric patients admitted with HIV infection. METHODS: The prospective study was conducted from January 2000 to October 2001 at a tertiary care referral teaching hospital in Mumbai, India. Admitted in-patients (aged 1 month to 12 years) detected to be HIV-positive (on triple ELISA test) were enrolled in the study. HIV status of patients < 18 months of age was confirmed by DNA-PCR testing. Demographic data, clinical features, investigations and outcome were recorded in a pre-designed proforma. RESULTS: Fifty HIV-positive children (31 males and 19 females; M:F = 1.6:1) were enrolled. Thirty cases were completely immunized, 9 were partially immunized while 11 were not immunized. Forty-two were perinatally infected, while eight cases were infected via blood transfusion (patients with thalassemia major on chronic transfusion therapy). Clinical features at presentation in 42 symptomatic cases included protein-energy malnutrition (90%), fever > 1 month (50%), weight loss > 1 month (50%), persistent generalized lymphadenopathy (24%) and skin manifestations (79%). The gastrointestinal (62%) and respiratory (52%) were the most commonly involved organ systems. Opportunistic infections noted included tuberculosis (19 cases), candidiasis (6 cases), Pneumocystis carinii pneumonia (4 cases), herpes zoster (3 cases) and giardiasis (1 case). Six patients died (mortality, 14%). CONCLUSIONS: Perinatal transmission is the most common mode of acquiring HIV in the pediatric age group. Most patients have protein-energy malnutrition. Tuberculosis is common in HIV-infected Indian children. Patients with HIV-encephalopathy have a poor outcome.

Rabson-Mendenhall syndrome.

Rabson-Mendenhall syndrome is characterized by growth retardation, dysmorphisms, lack of subcutaneous fat, acanthosis nigricans, enlarged genitalia, hirsutism, premature and dysplastic dentition, coarse facial features, paradoxical fasting hypoglycemia and post-prandial hyperglycemia, extreme hyperinsulinemia and pineal hyperplasia. We describe a six-month-old female child with physical features suggestive of the Rabson-Mendenhall syndrome. The child also had medullary nephrocalcinosis.

A prospective, randomised, double-blind study of comparative efficacy of immediate versus daily cleaning of stethoscope using 66% ethyl alcohol.

OBJECTIVE: Studies have demonstrated frequent contamination of stethoscope and usefulness of different disinfectants. Albeit, studies on the precise mode of cleaning and frequency of cleaning are lacking. This study was carried out to determine efficacy of 66% ethyl alcohol as disinfectant, rate of recontamination without cleaning and benefits of daily versus immediate cleaning. METHODOLOGY: Prospective, randomised, double blind study of 100 stethoscopes. Four cultures were obtained: before cleaning (Group A), immediately after cleaning with 66% ethyl alcohol (Group B), at the end of 4 days without cleaning (Group C) and at the end of 4 days after cleaning once a day (Group D). Samples were analysed using standard microbiological methods and Colony-forming unit (CFU) count and residual microorganism was computed for all the positive cultures. Medical staff was asked about the cleaning practices. Statistical analysis was carried out using 95% confidence interval and Chi-square test. RESULTS: 90% of the stethoscopes were contaminated with one or more microorganisms. Immediate cleaning and daily cleaning were associated with a significant reduction in the rate of contamination to 28% and 25% respectively. CFU count in groups B and D dropped to less than 10 in 75% and 84.7%, while the mean residual rates were 5.2% and 3.65% respectively. Groups B and D showed no statistically significant difference in terms of efficacy of disinfection. CONCLUSIONS: 66% ethyl alcohol is an effective disinfectant. The effects of immediate cleaning and cleaning once a day on residual flora on the diaphragm of stethoscope is comparable.

Mondini dysplasia and pyogenic meningitis.

Mondini dysplasia with cerebrospinal fluid leak is a rare cause of recurrent pyogenic meningitis in children. We describe an eleven-year-old female child who presented with the fifth recurrent episode of pyogenic meningitis and unilateral sensorineural deafness. Mondini dysplasia of the inner ear with CSF-perilymph fistula was proven on an HRCT of the temporal bone and MRI. Successful operative intervention was undertaken to close the defect. Though rare, Mondini dysplasia should be considered as a cause of recurrent meningitis in children, especially if they have sensorineural deafness.

Rapid diagnosis of meningitis using reagent strips.

OBJECTIVES: Identification of causative agent with estimation of cerebrospinal fluid (CSF) glucose, protein, cells is necessary for accurate diagnosis of meningitis. Unfortunately, even these facilities are not available in many areas. Reagent strips that measure glucose and protein in blood and urine can serve this task but have been used with varying results in the past. This study was carried out to evaluate the utility and efficacy of Combur 10 strips in the diagnosis of meningitis. DESIGN, SETTINGS AND METHODS: A prospective clinical single blinded study of 63 children suspected to have meningitis undergoing CSF analysis. Each CSF sample was divided in to two and was utilised for reagent strip analysis in addition to standard laboratory evaluation and a correlation analysis were made. STATISTICAL METHOD USED: Results were analysed using standard statistical tests. Accuracy of the reagent strips as a screening tool was established using Godyn's test. RESULTS: The sensitivity, specificity of the reagent strips for the diagnosis of meningitis was 97.14%, 96.42%. The sensitivity, specificity for tuberculous meningitis and bacterial meningitis were 100%, and 96.55%. That for the aseptic meningitis was 70% and 96.55%. Accuracy for the diagnosis of meningitis as a whole, bacterial meningitis, tuberculous meningitis, and aseptic meningitis were 96.78%, 98.2%, 98.27% and 83.0% respectively. CONCLUSION: Combur10 strips thus can be used for the rapid CSF analysis and screening with good accuracy. In situations where facilities of routine laboratory testing are not available this can be of an immense help.

Rheumatic fever and rheumatic heart disease: clinical profile of 550 cases in India.

BACKGROUND: The aims and objectives of this paper were to analyze the clinical presentation of children with rheumatic fever (RF) and rheumatic heart disease (RHD), to determine the cardiac sequel of RF and valvular affection due to RF, and to study clinical manifestations in recurrences of RF. METHODS: Our study, a retrospective analysis, was conducted at a tertiary-care teaching hospital with specialty follow-up clinic for patients with RF/RHD. This retrospective study was conducted to include a period of 31 years (from January 1971 through December 2001). Pre-completed case protocols of patients with RF/RHD were analyzed to record the following information: demographic data; clinical features on admission/presentation; relevant investigations; recurrences; cardiac valvular affection, and outcome. Modified Jones' criteria were used for diagnosis of RF. RESULTS: The study included 550 patients. Mean age of presentation was 9.62 years and sex ratio was 1.15:1 favoring males. A total of 250 patients presented with initial/first attack of RF. Arthritis and carditis were seen in 169 (67.6%) and 105 cases (42%), respectively. Chorea was seen in 47 cases (18.8%) and erythema marginatum, in four. A total of 224 patients presented with recurrence of RF (with pre-existing RHD). Arthritis and carditis were seen in 109 (48.66%) and 178 cases (79.46%), respectively, in these patients; 76 cases presented with chronic RHD. Mitral regurgitation was the most common cardiac valvular lesion observed (150 cases) followed by a combination of mitral stenosis and mitral regurgitation (98 cases). Congestive cardiac failure was seen in 201 cases (36.54%) and infective endocarditis in 30 (5.45%). 2-D echocardiography and/or color Doppler was performed in 201 patients (36.54%). Average duration of follow-up was 3.19 years; 74.72% of cases were lost to follow-up. Benzathine penicillin prophylaxis was regular in 42.18% cases. Positive family history of RF/RHD was present in 2% of cases. Twenty three patients (4.18%) died. CONCLUSIONS: Arthritis was the most common manifestation in the initial attack of RF, while carditis was the most common manifestation in patients presenting with rheumatic recurrences (with pre-existing RHD) and mitral regurgitation was the most common valvular lesion. Rate of patient dropout from penicillin prophylaxis was high. Clinical manifestations in initial/first attack of RF have not significantly changed in the last 31 years.

Kocher-Debre-Semelaigne syndrome: hypothyroidism with muscle pseudohypertrophy.

Kocher-Debre-Semelaigne syndrome is a rare association of muscular pseudohypertrophy and hypothyroidism in children. We report two cases of this syndrome in this communication. The first case was a seven-years-old female who presented with features of hypothyroidism and muscle pseudohypertrophy. The second child had similar manifestations but was only fifteen months of age at diagnosis. This is one of the youngest patients reported to have the Kocher-Debre-Semelaigne syndrome. A short review of the literature is also presented.

Neurological manifestations of HIV infection.

Case records of HIV infected patients were analyzed for identifying neurological manifestations. Eight patients (7 males) were identified to have probable HIV encephalopathy (in a period of 24 months) as per the CDC revised classification system. Their ages ranged from one year to ten years. The neurological manifestations noted included-developmental delay (2 cases), seizures (6 cases), acute onset alteration of sensorium (4 cases), aphasia (2 cases), loss of vision (2 cases), focal neurological deficits (6 cases), brisk deep tendon reflexes (7 cases), extensor plantar responses (5 cases) and signs of cerebellar dysfunction (2 cases). Other clinical features included growth failure, microcephaly, fever, lymphadenopathy, hepatomegaly, splenomegaly, pneumonia, otorrhea and oral candidiasis. Cerebrospinal fluid studies were normal. The neuroimaging features included cerebral atrophy and ventricular dilatation, cerebral infarction, basal ganglia calcification and cerebellar atrophy. Childhood HIV infection may have a variety of neurological abnormalities. HIV infection should be suspected in children presenting with unexplained neurological manifestations and growth failure.

Neurological complications of rabies vaccines.

The rabies vaccines containing neural elements are used in some countries including India. We report three cases that presented with various neurological complications following the use of these vaccines. The presenting manifestations included those of encephalitis, radiculitis and acute inflammatory demyelinating polyradiculoneuropathy. These neurological complications are highlighted so that scientific evidence compels the community to discontinue the use of the neural tissue rabies vaccines. Newer generation cell culture rabies vaccines should be preferred over the neural tissue rabies vaccines for post-exposure prophylaxis.

Alagille syndrome.

Syndromic paucity of bile ducts or "Alagille syndrome" is characterized by peculiar facies, chronic cholestasis, posterior embryotoxon, butterfly-like vertebral arch defects and peripheral pulmonary artery hypoplasia or stenosis. We present a two-year-old female child with the 'partial' or 'incomplete' Alagille syndrome. The child had three of the five major features of the syndrome. A brief review of literature of the syndrome is presented.