Fatal cryptococcosis involving multiple sites in an immunocompetent child.

Disseminated cryptococcosis is less common in immunocompetent individuals. Herein, we report a fatal case of cryptococcosis in apparently immunocompetent child with multiple site involvement. The yeast isolated from cerebrospinal fl uid, blood, endotracheal, gastric and lymph node aspirate was identifi ed by molecular method as Cryptococcus neoformans var. grubii.

Malrotation of Gut and Hiatus Hernia in a Child with Familial Dyskeratosis Congenita.

Dyskeratosis congenita (DKC) is a rare inherited genodermatosis. We report familial occurrence of the disease. The index patient 12 years old had all classical features of DKC. There are 4 other siblings in the family suffering from similar disease. In additions to the features of DKC, the index patient presented with pain abdomen and vomiting. On investigation he had malrotation of gut and hiatus hernia. To the best of our knowledge this is being documented for the first time in association with DKC.

Celiac Disease Presenting As Pancytopenia.

Celiac disease is a systemic immune-mediated disorder that can have multiple hematological manifestations. We report a ten years male, who presented with recurrent episodes of loose stools, poor weight gain and on investigations was diagnosed to have celiac disease with pancytopenia which responded dramatically to gluten free diet and hematinic supplements. Pancytopenia is not a common presentation of celiac disease.

Embolization of congenital intrahepatic porto-systemic shunt by n-butyl cyanoacrylate.

Congenital intrahepatic portosystemic venous shunt (IHPSVS) is rare vascular anomaly. We present one case of a 14- month male child who presented with global developmental delay. Child had high ammonia levels with low glutamine and high bile salts on the previous investigations and had history of neonatal seizures since day 13 of life. On admission, serum ammonia levels were elevated to 112μmol/L. Other laboratory investigations including liver and renal function test, and electrolytes were normal. He was, diagnosed to have IHPSVS on the basis of Doppler and CT, and treated by embolization with n-butyl cyanoacrylate (glue). A brief review of diagnostic modalities and endovascular management for the IHPSVS is presented including the present case.

Role of colostrum in gastrointestinal infections.

Colostrum is breast milk produced after the birth of the newborn and lasts for 2-4 days. Colostrum is very important part of breast milk and lays down the immune system and confers growth factors and other protective factors for the young ones in mammals. This is the source of passive immunity transferred to the baby from the mother. The biological value of bovine colostrum in present day medical practice is documented in clinical trials and large databases containing case reports and anecdotal findings. The main actions include an antibacterial effect and modulation of immune response with the ability to neutralize lipopolysaccharides arising from gram negative bacterial pathogens. It has been found to be effective in infantile hemorrhagic diarrheas, other diarrheas and reduces the likelihood of disease progressing to hemolytic uremic syndrome. It has also been tested in H. pylori infection and diarrhea in immunodeficiency. Side effects of clinical relevance are limited to possible intolerance due to lactose and sensitivity to milk proteins.

Liver function tests and their interpretation.

Liver function tests (LFT) are a helpful screening tool, which are an effective modality to detect hepatic dysfunction. Since the liver performs a variety of functions so no single test is sufficient to provide complete estimate of function of liver. Often clinicians are faced with reports that do not tally with the clinical condition of the patient and they face difficulty in interpreting the LFT. An attempt is being made to study and understand the LFT and simplify their interpretation with algorithms.

Mitochondrial neuro-gastrointestinal encephalopathy syndrome.

Mitochondrial neurogastrointestinal encephalomyopathy is a rare disorder affecting the pediatric age group with a heterogeneous multisystem involvement. We happen to manage a young child with symptoms of constipation since infancy along with cachexia, seizures and peripheral neuropathy. The child later went into encephalopathy preterminally. This clinical syndrome fitted very well with mitochondrial neurogastrointestinal encephalomyopathy. The child had elevated lactate levels and electron microscopy of the rectal biopsy was suggestive of a mitochondrial disorder To the best of our knowledge there is no case report of this syndrome from India and since this presents with diagnostic difficulties so is being reported.

Pancytopenia in a child associated with hepatitis A infection.

Pancytopenia is a very rare condition associated with hepatitis A infection. We managed a 12 year old boy who had hepatitis A infection with anemia. His hemogram and bone marrow examination were suggestive of pancytopenia. Pancytopenia recovered without any specific therapy. There are case reports of severe aplastic anemia with hepatitis A infection that required immunosuppressive therapy. The present case did not require any aggressive therapy and recovered. In a young child with hepatitis A infection and anemia, bone marrow depression should be suspected. The pancytopenia may be transient as exemplified by the present case.

Therapeutic potentials of bovine colostrums.

Colostrum is the first milk produced by mammals for their young ones. This transfers the passive immunity gained by the mother to the baby. The bovine colostrum (BC) can be obtained in large quantity and has properties similar to human colostrum. It has been used for various disorders of the body. It has properties to stimulate immune system, contains growth factors and many bioactive substances needed for the body to combat with wear and tear. The BC has been used for various gastrointestinal disorders, respiratory tract infection, rheumatoid arthritis, healing injured tissues of body etc. There are not much double blind placebo-controlled trials to prove its efficacy, though a lot of experience about its good effects in various disorders is available in the literature. The dosage and duration of therapy need to be worked up. The BC has potential to treat as well to prevent certain diseases in the body. In future this will prove to be a very useful product to treat and control diseases in a natural way.

Health factors in colostrum.

Colostrum is a breast milk produced after the birth of the newborn and lasts for 2-4 days. Colostrum is very important part of breast milk and lays down the immune system and confers growth factors and other protective factors for the young ones in mammals. This is the source of passive immunity achieved by the mother and is transferred to the baby. This is the major source of secretory IgA and gives protection against gastrointestinal infections. In view of so many health factors through colostrum, the use of colostrum has been extended to so many health problems of mankind. Human and bovine colostrums have many similarities barring that bovine colostrum can be obtained in large quantity, so bovine colostrum has been used in various disorders in human beings. This is the nature's gift that is for the young ones to grow as well as for the treatment of many health problems in older age group.

Celiac crisis.

Celiac crisis is a life-threatening cause of acute diarrhea and multiple metabolic emergencies. It is extremely rare these days. We successfully managed a 5-year old girl with celiac crisis for multiple metabolic problems viz., hyponatremia, hypokalemia, metabolic acidosis, hyperglycemia and polyuria in Pediatric Intensive Care Unit. Steroid therapy was life saving. The case may serve as a reminder of the condition, which presents a difficult therapeutic challenge.

Crohn's disease: disastrous consequences of late diagnosis.

Chronic and recurrent perianal abscess is an uncommon condition in children. Tuberculosis is thought to be the common etiology for such a presentation in India. We report a case of a child with colonic and perianal disease due to Crohn's disease and emphasize the disastrous complication due to delayed diagnosis.

Nutrition support in a surgical patient.

Nutrition has very important role to play during health and disease state in human beings. Neonates and younger children are more prone to develop nutritional deficiencies. They have very critical reserves and are rapidly growing. Any surgical insult leads to multiple nutritional problems. Careful planning of nutritional management in a surgical patient is mandatory. Nutritional support should start from the day when the child develops the surgical condition to withstand stress of disease and surgical procedure. In the postoperative period nutritional support should start as early as possible. Start with small amount and build up gradually till the normal enteral nutrition (EN) is tolerated. When EN is not tolerated parenteral nutrition (PN) should be considered. Parenteral nutrition on short term basis is very important to tide over the crisis due to postoperative complications. Parenteral nutrition on long term basis is required in short bowel syndrome resulting from resection of large part of the gut. During PN, enteral nutrition should be continued in small amounts in order to maintain the integrity of mucosal lining of the gut. Enteral nutrition is more physiological and can provide adequate amount of immunonutrients, minerals and vitamins.

Indigenous rapid diagnostic method for Escherichia coli O157:H7.

Rapid methods for detection of Escherichia coli O157: H7 diarrhoea are preferred to limit differential diagnostic evaluation including invasive procedures. Fifty stool samples from children and adult patients having diarrhoea and ten control samples from volunteers without diarrhoea were collected. Each sample was inoculated into two tubes containing peptone water and one containing MacConkey broth. They were incubated for 5 hours or longer till growth occurred. The first tube of peptone water with growth was tested for production of indole. All samples were tested for enzyme beta-glucuronidase in the pellet of the second peptone water growth. The MacConkey broth growth was tested with latex beads sensitized separately with antisera to E. coli O157 and E. coli H7. All indole positive and beta glucuronidase negative samples with positive agglutination with E. coli O157 and H7 coated latex beads were taken as E. coli O157: H7 positive samples. Three out of 50 diarrhoeic samples were found to be positive for E. coli O157:H7. Confirmation of the results of our rapid assay was done by parallel conventional culture of faecal specimens on sorbitol MacConkey agar. Our rapid assay required only 7-10 hours compared to the conventional technique where the report is available only on the third day. It can therefore be used routinely for initial screening of faecal specimens for E. coli O157: H7.