Lipid Profile in Children with Celiac Disease: Effect of Six Months Gluten Free Diet

Introduction: Cardiovascular disease is one of the leadingcauses of death among adult celiacs. Increased risk isattributed to unfavourable cholesterol profile. Studies on lipidprofile among celiacs have shown variable results. Effectof gluten free diet (GFD) on cardiovascular risk profile isfurther controversial. Knowledge among children can help inrisk stratification and timely modification of diet if needed.Current study objective was to record the lipid profile amongchildren with celiac and the impact of six months of glutenfree diet on it.Material and methods: In this single arm pre and postinterventional study, newly diagnosed symptomatic children1-14 years with Tissue Transglutaminase (tTG) IgA antibodypositive and biopsy proven (Marsh 3) celiac were enrolled.Sample size of 31 was calculated as per TC and HDL referencelevels. Fasting Lipid profile was evaluated at baseline and atsix months of GFD. Children with diabetes, hypothyroidism,nephrotic syndrome, congenital hyperlipidaemia and on drugscausing hyperlipidaemia were excluded. The data analysis wasdone using Statistical software R and Stata 15.0. Quantitativevariables were compared using Paired T test/Wilcoxon test.Qualitative variables were correlated using Chi-Square test/Fisher’s exact test.Results: Median age of children was 8 years (5-10 years).Median age at onset of symptoms was 6 (3.15 -8.75) yearsand median duration of illness prior to diagnosis was 12 (7.5-24) months. 83.87% presented with typical GI symptoms.Mean Total cholesterol (TC) was 131.8±39.7 mg/dl, meanHDL cholesterol was 41.2±12.6 mg/dl, mean Triglycerideswas 92.0±35.5mg/dl and mean LDL was 75 ± 32.3 mg/dl atdiagnosis. Average increase in HDL after GFD was 13 (4.9 to21.3) mg/dl. TC/HDL ratio was decreased significantly afterGFD {-0.5 (-0.9 to -0.03)}.Conclusion: Children with celiac have a normal lipid profile.GFD for six months increases HDL-C plasma concentrationand lowers TC/HDL ratios.

Irrational Drug Use And Gastro-Duodenal Ulcerationsin A 3-Month-Old Infant: A Case Report

Upper gastrointestinal bleed (UGIB) is an unusual but potentially life-threatening emergency in children. Irrational use of medicines is one of most common cause. We, herein report a case of a three-month-old male child who presented with massive hematemesis and melena, probably due to the use of multiple antibiotics and analgesic use. On upper gastrointestinal endoscopy, multiple diffuse gastric and duodenal ulcers were detected. He was treated with proton pump inhibitors (PPI) and recovered completely within seven days.

Atypical Presentation Of Caroli’s Syndrome: A Case Report

Caroli’s disease and Caroli’s syndrome are rare congenital disorders. Caroli’s disease is characterized by multiple sequential cystic or saccular dilatations of the large intrahepatic biliary ducts while Caroli’s syndrome has small bile duct involvement and congenital hepatic fibrosis. The incidence of Caroli’s disease is as low as 1/1,000,000 people. The average age of presentation is early adolescence. Magnetic resonance cholangiopancreatography is a most valuable investigation in diagnosis. Here, we report the case of Caroli’s Type II without renal involvement as late as 6 years of age with severe portal hypertension and hypersplenism. The child had no history of jaundice or recurrent abdominal pain in the past.

Early Diagnosis Of Congenital Methemoglobinemia Type 1 In A 4-Year-Old Child

Bluish discoloration of the skin and mucous membrane is known as cyanosis which is a clinical sign that occurs in many diseases. The causes of central cyanosis are cardiac shunts causing mixing of oxygenated and deoxygenated blood, lung diseases with ventilation-perfusion mismatch, polycythemia, and methemoglobinemia. Methemoglobin is the oxidized form of hemoglobin, which does not bind oxygen and increases the affinity of oxygen for the partially oxidized portion of hemoglobin. Methemoglobinemia may be congenital or acquired (usually drug induced). Congenital methemoglobinemia is a very rarely reported disease that is caused by a deficiency of nicotinamide adenine dinucleotide phosphate-cytochrome b5 reductase enzyme deficiency or by an abnormal hemoglobin called hemoglobin H. Acquired methemoglobinemia is caused by drugs, namely the sulfonamide group and local anesthetics such as benzocaine and prilocaine. Here, we present the case of a 4-year-old girl who presented with complaints of bluishness of the fingers and lips without any other associated symptoms and later on diagnosed as congenital methemoglobinemia.

Idiopathic Musculoskeletal Pain in Indian children-Prevalence and Impact on Daily Routine / Prevalência e impacto nas atividades diárias da dor musculoesquelética idiopática em crianças da Índia

ABSTRACT Objectives: To study the prevalence of idiopathic musculoskeletal pain (IMSP) in school going children and its impact on daily life. Methods: One thousand eighteen apparently healthy school children aged 5–16 years were assessed and analysed for IMSP and its associated problems. Standard tests for significance were applied. Results: One hundred and sixty-five (16.2%) children mostly males (55.2%) reported IMSP. Lower limbs (52.1%) were the most common location of pain. More than 1 year of pain history was present in 15%. Thirty-seven percent children complained of discomfort during walking, 30.9%, had pain during physical exercise, 29.2% had difficulty attending lessons and 4.2% had interference in pursuing hobbies. The children were also further sub grouped into preadolescents and adolescents. There was significant difference in pain duration and duration of each pain episode in the two groups (p = 0.01). A significant number of children (21.2%) with IMSP reported school absenteeism (p < 0.001). A significant number of adolescents had history positive for contact sports (p = 0.001). Sleep disturbances were also reported to be higher in children with IMSP (29% vs. 5.7%, p = 0.001). Other associated problems in children with IMSP found were day time tiredness (51.1%), headache (47.3%) and abdominal pain (24.8%). Conclusions: Prevalence of IMSP in school children aged 5–16 yrs was found to be 16.2% and a significant percentage of these children experience interference with daily activities including school absenteeism.

RESUMO Objetivos: Estudar a prevalência de dor musculoesquelética idiopática (DMEI) em crianças em idade escolar e seu impacto nas atividades diárias. Métodos: Foram avaliadas e analisadas 1.018 crianças em idade escolar aparentemente saudáveis entre cinco e 16 anos quanto à presença de DMEI e seus problemas associados. Foram aplicados os testes de significância padrão. Resultados: Relataram DMEI 165 (16,2%) crianças, em sua maior parte do sexo masculino (55,2%). Os membros inferiores (52,1%) foram a localização mais comum da dor. A história de dor presente havia mais de um ano foi encontrada em 15% das crianças; 37% delas queixaram-se de desconforto durante a caminhada, 30,9%, tinham dor durante o exercício físico, 29,2% tinham dificuldade de frequentar as aulas e 4,2% sofriam interferência na participação em passatempos. As crianças foram ainda subagrupadas em pré-adolescentes e adolescentes. Houve diferença estatisticamente significativa na duração da dor e na duração de cada episódio de dor nos dois grupos (p = 0,01). Uma quantidade significativa de crianças com DMEI (21,2%) relatou absentismo escolar (p < 0,001). Uma quantidade significativa de adolescentes tinha história positiva de prática de esportes de contato (p = 0,001). Os distúrbios do sono também foram relatados como maiores em crianças com DMEI (29% vs. 5,7%, p = 0,001). Outros problemas associados encontrados em crianças com DMEI foram o cansaço durante o dia (51,1%), a cefaleia (47,3%) e a dor abdominal (24,8%). Conclusões: A prevalência de DMEI encontrada em crianças entre cinco e 16 anos foi de 16,2%. Uma percentagem significativa dessas crianças relata interferência nas atividades diárias, incluindo absentismo escolar.

Disseminated infection with Cryptococcus neoformans var neoformans in an 8 years immunocompetent girl.

Disseminated cryptococcosis is a rare and often fatal disease in children. The majority of cases usually occur in individuals with defective cell-mediated immunity, most commonly due to HIV infection. The authors here in report an 8-year-old girl from Nepal who presented with fever, cough, headache, lymphadenopathy, hepatosplenomegaly and cutaneous lesions. Lymph node biopsy revealed multiple granulomas composed of histiocytes and epitheliold cells along with numerous yeast forms of cryptococcus. Cultures of CSF, sputum and urine yielded cryptococcus neoformans. Surprisingly,the immune function in terms of T-cell number, CD4 : CD8 ratio, serum immunoglobulins and HIV serology was normal. After the diagnosis of disseminated cryptococcosis was established, the patient was treated with 5-fluorocytosine (100 mg/kg/day) for initial two weeks and amphotericin B (1 mg/kg/day) for 13 weeks. Patient responded well to the treatment with disappearance of presenting symptoms, cutaneous lesions, and lymphadenopathy, though she still had hepatosplenomegaly, which also decreased. Unfortunately, she developed loss of vision in 10th week of therapy. The patient was discharged on oral fluconazole (6 mg/kg/day) and no recurrence was found during the follow-up period of more than 9 months. This is the first case of disseminated cryptococcosis with no detectable immune deficit, from India.

Bjornstad syndrome.

A 9-month-old boy presented with the complaints of loose motion, vomiting and difficulty in breathing. His scalp hairs were thin, brittle, and sparse and were of differing lengths with twisted appearance. Hair shaft microscopy revealed alternate light and dark segments and twisting of the hair shafts by 180 degrees along the axis. Serum copper levels were normal. The audiological testing revealed bilateral sensorineural hearing loss. Child was diagnosed as a case of Bjornstad Syndrome.

Henoch schonlein purpura with rheumatic carditis.

Henoch Schonlein Purpura with acute rheumatic carditis is a rare entity and only few cases have been reported. An 8 year-old-girl presented with abdominal pain, arthralgia and rashes and was diagnosed as a case of Henoch Schonlein Purpura. She was managed conservatively and discharged. She was readmitted after 1 week with abdominal pain, fever and cough. She developed tachycardia with gallop rhythm on the third day of admission and pansystolic murmur of mitral regurgitation. Echocardiography showed features of myopericarditis, mild pericardial effusion and mitral regurgitation. She was diagnosed and managed as a case of acute rheumatic carditis.

Progressive bulbur paralysis (Fazio-Londe disease).

Progressive bulbar paralysis of childhood is characterised by progressive paralysis of muscles innervated by cranial nerves. The authors report a case of progressive bulbar paralysis of childhood in a 12-year-old child. Child was admitted with the complaints of drooping of eyelids, difficulty in swallowing and hoarse voice. She had involvement of III, VII, IX, X, XI and XII cranial nerves and the corticospinal tracts. Electromyography revealed spontaneous activity in the form of fasciculations, giant motor unit potential and discrete recruitment of motor neurons suggestive of denervation pattern. Hearing assessment was normal. Muscle biopsy was also suggestive of neurogenic atrophy.