Prevalence of celiac disease among pediatric patients with cryptogenic cirrhosis and effect of gluten-free-diet.

BACKGROUND: Liver involvement in celiac disease (CD) is classified into autoimmune and cryptogenic. The association between CD and autoimmune liver diseases like autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis is well-established; however, the data on patients with cryptogenic cirrhosis, particularly from India, are scanty. So we did this study to find the prevalence of CD in patients with cryptogenic cirrhosis. METHODS: This was a prospective observational study, involving children of less than 18 years old attending Pediatric and Gastroenterology clinic with a diagnosis of cryptogenic cirrhosis. The patients were evaluated for CD and divided into two groups: chronic liver disease (CLD) with CD, and CLD without CD. Both the groups were followed up for 6 months. CLD with CD group was treated with gluten-free-diet (GFD) and CLD without CD group was followed up without any specific intervention except standard care of CLD. RESULTS: Out of 84 patients, 11 (13.1%) were diagnosed as CLD with CD. There was an improvement in hemoglobin levels, liver function tests, and Child-Pugh score after initiation of GFD in CLD with CD group. CONCLUSION: The prevalence of CD in cryptogenic cirrhosis was 13.1%. Screening for CD is recommended for cryptogenic cirrhosis. Hepatic functions improve with a GFD in CD patients with cirrhosis.

Caudal dysplasia, femoral hypoplasia-unusual facies syndrome and absent radius: a new association in infant of diabetic mother?

The Caudal dysplasia syndrome (CDS) and the femoral hypoplasia-unusual facies syndrome (FHUFS) have been reported to be more frequent among infants of diabetic mothers (IDMs). Infact, uncontrolled maternal diabetes is the most common cause of both the syndromes. Till now, there is no case report to suggest absent radius as a manifestation of IDMs. The authors report a rare case of newborn, who presented with features compatible with both CDS and FHUFS with an additional feature of absent radius, which is not reported in the literature so far. The possibility that all these features represent different manifestations of the same disorder is discussed here.

Clinical profiles of 13 children with Plasmodium vivax cerebral malaria.

BACKGROUND: Bikaner region is endemic for both P. vivax and P. falciparum malaria. Usually, cerebral malaria is caused by P. falciparum but it has been reported recently also in P. vivax mono-infection. Epidemiologic studies and clinical descriptions of P. vivax cerebral malaria in children are rare. AIMS: To describe the clinical features of PCR-confirmed cerebral malaria owing to P. vivax mono-infection and its clinico-laboratory profile in Bikaner, Northwest India. METHODS: This observational prospective study was based on detailed clinical and laboratory investigation of children admitted with cerebral malaria owing to P. vivax between November 2008 and December 2010. Cerebral malaria was categorised according to the WHO (2000) criteria for P. falciparum and the diagnosis of P. vivax mono-infection was established by peripheral blood film and rapid diagnostic tests and confirmed by polymerase chain reaction. The possibility of other diseases/infections causing similar illness were investigated thoroughly. RESULTS: Thirteen children with P. vivax cerebral malaria were studied, eight of whom (61·5%) had multi-organ (two or more organs) dysfunction. Other associated severe manifestations included severe anaemia (7), hepatic dysfunction (2), renal dysfunction (2), bleeding manifestation (2), respiratory distress (2), metabolic acidosis (2) and shock (one). Hypoglycaemia was not observed in any patient. There was no evidence of neurological sequelae. All the children were managed according to WHO guidelines using intravenous artisunate. Thrombocytopenia was detected in five and hyponatraemia in four children. CONCLUSION: P. vivax mono-infection can cause cerebral malaria and multi-organ dysfunction.

Prevalence of rheumatic heart disease in school children in Bikaner: an echocardiographic study.

OBJECTIVE: To determine prevalence of rheumatic heart disease (RHD) using clinical and echocardiographic criteria and to study influence of socioeconomic status (SES) we studied school children in a north-western Indian town. METHODS: 3292 school children, age range 5-14 years, in two private schools, ten middle SES government schools and six low SES government schools were invited to participate in the study. 3002 (1837 boys, 1165 girls) were clinically examined (response 91%) of which 1042 were in private schools, 1002 in middle SES schools and 958 in low SES schools. Prevalence of cardiac murmurs and RHD based on clinical diagnosis was determined in school by a trained team of physicians. Those with a murmur were further evaluated by Doppler-echocardiography in the hospital. Group-specific and age-specific rates (prevalence/thousand) of murmurs and cardiac lesions were determined. RESULTS: A significant cardiac murmur was observed in 55 subjects (18.3/1000) with similar prevalence in boys (20.7) and girls (14.6). The prevalence of murmur was significantly greater in children belonging to low SES schools (29.2/1000) as compared to middle SES (18.9) and higher SES schools (7.6). RHD prevalence based on clinical diagnosis was observed in 50 children (16.7/1000) and was similar in boys (19.1) and girls (12.9). Clinical RHD was more in the low SES school children (28.2/1000) as compared to middle (17.0) and high SES schools (5.8). RHD was demonstrated on echocardiography in 2 children (0.67/1000). Other prevalent cardiac lesions were congenital heart disease in 5 (1.66/1000) and mitral valve prolapse in 37 (12.3/1000). CONCLUSIONS: There is a low prevalence of RHD in school children in this region compared to previous Indian studies. Cardiac murmurs are more prevalent among low SES children.

Endoscopic variceal ligation using multiband ligator.

Esophageal variceal bleed in children is treated with endoscopic sclerotherapy (EST), which is associated with significant complications. Endoscopic variceal ligation (EVL) was found to be more effective and safe in adults. Use of EVL in children has not been studied much. Thirteen consecutive children (mean age 9.4 years) with variceal bleed were subjected to EVL by multi band ligator. Varices were eradicated in 2.8 sessions (range 2-4) and one patient had bleed during procedure. No other complications were noted. EVL could not be performed in 2 children less than 3 years of age.

Serum copper in newborns and their mothers.

Serum copper levels in the cord blood of 100 newborns and the respective maternal serum copper at the time of delivery was estimated by atomic absorption spectrophotometer. The cases were classified into term AGA, term SGA, term LGA, preterm AGA and preterm SGA. The mean maternal serum copper level 152.42 +/- 2.06 micrograms/dl) was significantly higher than the mean cord serum copper level (39.84 +/- 1.19 micrograms/dl). There was positive correlation between the maternal serum copper level and cord serum copper level. The mean serum copper level of term neonates was (44.42 +/- 1.26 micrograms/dl) significantly higher (p < 0.001) than that of preterm neonates (30.30 +/- 1.14 micrograms/dl). There was a positive correlation between cord serum cooper level and gestational age. The mean cord serum copper levels of term AGA, term SGA, preterm AGA and preterm SGA neonates was 45.42 +/- 1.44 ug/dl, 39.22 +/- 2.45 ug/dl, 31.00 +/- 2.11 ud/dl and 29.47 +/- 2.08 ug/dl respectively. There was no statistically significant difference in the mean serum copper level of AGA and SGA group of both term and preterm neonates. The difference amongst mean maternal serum copper level of various neonatal groups was not significant.

Changing scenario of therapeutic apheresis in India in the last 14 years.

There has been a remarkable change in the scenario of therapeutic apheresis in the last 14 years in India. The crude method of manual removal of blood followed by separation of plasma by gravity, keeping it in the bottle for a long time, has now been totally replaced by plasmapheresis, centrifugation, membrane filtration, and immunoadsorption techniques. The indications for use have also changed from a list of limited indications in the beginning to include all immune complex disorders. The clinical beneficiaries have also increased from blood bankers to nephrologists and immunologists in addition to oncologists. Efforts are now underway with the help of the Indian Society for Apheresis (founded in 1985) to popularize the newer methods of cryofiltration, photopheresis and heparin extracorporeal low-density lipoprotein (HELP) and DALI apheresis systems besides the specialized techniques of immunoadsorption using filters, columns, or ligands. This is suggestive of a positive trend for the treatment of immune complex disorders.

Prevalence and pattern of mental health problems.

Three hundred and forty eight children of age group 5-15 years were studied. A reporting questionnaire was administered to the parents and then affected children were examined in detail by history, physical examination and mental status examination. Fifty out of 348 children were having mental health problems. Male and first born children were affected more. Common problems observed were poor scholastic performance, enuresis, hyperkinetic syndrome, speech disorders and sleep disorders. In males, poor scholastic performance, hyperkinetic syndrome and temper tantrums were common while in females enuresis, speech disorders and hysterical symptoms were more frequent. Among the adverse perinatal factors of etiological importance low birth weight, difficult deliveries, birth injuries, delayed cry, neonatal jaundice and convulsions were common. Predominant psychosocial stress factors were quarrels between parents, separated parents, deaths of parents, siblings or relatives and chronic illness in the family.

Oral rehydration therapy.

PIP: The management of diarrhea is mostly dependent on parenteral administration of fluids and electrolytes, yet for the majority of India's population such facilities are practically unavailable. In addition there are several serious limitations to the use of this technique. These include prohibitive cost, the fact that it can only be administered by trained personnel, and that it brings undue distress to both patients and parents. Such procedures should be reserved only for those patients who have severe dehydration, impending shock, electrolyte imbalance, or persistent vomiting. For the others, the majority, oral rehydration therapy should be the treatment of choice. The implementation of oral rehydration therapy in the Infectious Diseases Hospital, Calcutta alone, has resulted in an annual saving of much money. Another benefit is that the family members can participate in the therapy and can continue it at home. The process of water and solute absorption is accelerated in the presence of glucose and sodium, and its effectiveness has been proven and documented. There are various types of oral multi-electrolyte-glucose powders available in India. The mixture recommended by the World Health Organization seems to be the most suitable, and this mixture can be prepared at home by the addition of 3-1/2 grams of common salt, 2-1/2 grams of baking soda, 1-1/2 grams of potassium chloride, and 20 grams of glucose in 1 liter of water. Instructions for the use of oral rehydration therapy are outlined, and other types of rehydration therapy are described.^ieng