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LETTER TO EDITOR (VIEWERS CHOICE)

RECURRENT VOMITING IN A CHILD: A RARE CASE OF ISOVALERIC ACIDEMIA

Patil SV, Kalyanshettar SS, Naren SD, Kiran kumar

A 3 1/2 years old girl, born to 2 nd degree dried blood spot specimen. Treatment involves reducing consanguineous marriage was admitted with chief protein intake, particularly the branched-chain amino complaints of recurrent episodes of projectile non- acid leucine. During an acute episode, aggressive bilious vomiting since 6 months of age each lasting for use of glucose and electrolytes is necessary. Glycine 3-4 days and recurs every 4 to 5 months and each time supplementation has proven beneficial because this requires hospital admission. Child used to be normal in amino acid is conjugated to isovalerate, forming the between episodes. She had a NICU admission on 5th less harmful isovalerylglycine. Carnitine treatment is day of life for vomiting. A sibling had died on 19th day similarly effective (4-6). of life with similar complaints. On examination, she was drowsy, had tachycardia, tachypnea and moderate REFERENCES dehydration. A clinical diagnosis of cyclical vomiting 1. Crombez E, Koch R, Cederbaum S. Pitfalls in newborn was made and child was started on antiepileptic for screening. J Pediatr. 2005; 147: 119-120 abdominal migraine. Vomitus used to have a peculiar 2. Holtzman NA. Expanding newborn screening: how good odor and this made us suspect other causes for the is the evidence? JAMA. 2003; 290: 2606-2608 condition of the child. The child was investigated further. 3. Blau N, Scriver CR. New approaches to treat PKU: how far Urine routine was normal except for ketone bodies. are we? Mol Genet Metab. 2004; 81: 1-2 Blood gases showed metabolic acidosis with high anion 4. Berry GT, Yudkoff M, Segal S. Isovaleric acidemia: medical gap. Urine screening (thin layer chromatography) and neurodevelopmental effects of long-term therapy. J revealed mild increase in leucine, isoleucine and Pediatr. 1988; 113: 58-64 valine, alanine and hydroxyproline. Blood tandem 5. Chalmers RA, Roe CR, Stacey TE, Hoppel CL. Urinary mass spectrometry revealed marginally elevated serum excretion of L-carnitine and acylcarnitines in patients homocysteine level and acylcarnitine pattern which was with disorders of organic acid metabolism: Evidence for suggestive of isovaleric acidemia. Patient was started secondary insufficiency of L-carnitine, Pediatr Res. 1984; on dietetic management, carnitine 100mg/kg/day & 18: 1325?1338. megavitamin therapy. The child improved subsequently 6. de Sousa C, Chalmers RA, Stacey TE, Tracey BM, Weaver and was advised to continue carnitine. CM, Bradley D. The response to L-carnitine and glycine Isovaleric acidemia is a rare autosomal recessive therapy in isovaleric acidaemia. Eur J Pediatr. 1986; 144: disorder with an incidence of 1 in 2 lakhs (1). It is due to 451-456 deficiency of Isovaleryl-CoA dehydrogenase required for the metabolism of the amino acid, leucine. It presents From: Department of Pediatrics, BLDE University, Sri within a few days or weeks of life with vomiting and BM Patil Medical college, Bijapur, Karnataka. ketoacidosis progressing to lethargy, coma and death Address for Correspondence: Dr SS Kalyanshettar, in greater than 50% of the patients (2). We report a Department of Pediatrics, BLDE University, Sri BM rare case of isovaleric acidemia presenting as recurrent vomiting. Distinctive odor of “sweaty feet” during an Patil Medical college Bijapur, Karnataka, India. illness due to accumulation of volatile isovaleric acid is Email: [email protected] classical (3). Diagnosis involves screening for isovaleric E-published: 1st August 2011. Art#51 acidemia using tandem mass spectrometry analysis of

LETTER TO EDITOR (VIEWERS CHOICE) THE ROLE OF BREAST MILK IN PREVENTION OF H. PYLORI INFECTION IN EARLY INFANCY

Pishva Narjes, Alian Zahra, Haghighat Mahmood, Moslehi Mohammad Ashkan

Infection with plays a crucial class and overcrowding are important risk factors which role in the pathogenesis of both chronic active gastritis increase the rate of infection by H.pylori (Rosenthal). and in children and adults. An Longer duration of breast feeding has been reported increasing amount of the evidence also supports the to lead to lesser H. pylori infection. (3,4) hypothesis that H. pylori is an important cofactor in the development of gastric cancer (1). H. pylori We undertook a study to compare the incidence of infection is predominantly acquired in early childhood. H.pylori infection between exclusively breast fed and The prevalence of H. pylori infection in the pediatric exclusively formula fed infants in similar conditions. population ranges from less than 10% to more than From March 2005 to September 2006, 85 healthy 80%. The prevalence in Europe and Japan are low, infants between 2 to 6 months old were studied. whereas in India, Bangladesh and Africa is high. Forty three of the infants were formula fed and 42 (2) Even though the mechanism of transmission of were exclusively breast fed. None of the infants were helicobacter pylori is not clearly known, socioeconomic receiving baby food. The fresh stool samples were

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collected from the infants and stored at -700 C. The H. gastric cancer. J Physiol Pharmacol. 2003; 54 Suppl 3: pylori antigen was detected by HPAT kit (Diagnostic 23-41 Bioprobes srI company, Italy). Maternal serum also was 2. Brown LM. Helicobacter pylori: epidemiology and routes studied for H.pylori IgG antibody by ELISA. The rate of of transmission. Epidemiol Rev. 2000; 22: 283-297 H.pylori infection in breast fed infants was 32% and in 3. Okuda M, Miyashiro E, Koike M, Okuda S, Minami K, formula fed infants was 60% (p=0.006). The rate of Yoshikawa N. Breast-feeding prevents Helicobacter pylori H. pylori antibody in mothers of both groups (breast infection in early childhood. Pediatr Int. 2001; 43: 714- milk and formula fed) was similar and 70%. 715 We found that the incidence of H.pylori infection is 4. Fujimura S, Kato S, Nagai K, Kawamura T, Iinuma K. significantly lower in the infants who are exclusively Detection of Helicobacter pylori in the stools of newborn breast fed. infants. Pediatr Infect Dis J. 2004; 23: 1055-1056

ACKNOWLEDGEMENT From: Department of Pediatrics, Gastroenterohepatology The authors would like to thank the office of Research Center, Nemazee Hospital Shiraz University Vice-Chancellor for Research of Shiraz University of of Medical Sciences, Shiraz, Iran. Medical Sciences for financial support and Dr. Davood Mehrabani at Center for Development of Clinical studies Address for Correspondence: Mohammad of Nemazee Hospital for editorial assistance. Ashkan Moslehi, MD, Pediatrician, Department of Pediatrics, Nemazee Hospital, Shiraz, Iran. REFERENCES Email: [email protected] 1. Konturek JW. Discovery by Jaworski of Helicobacter pylori and its pathogenetic role in peptic ulcer, gastritis and E-published: 1st August 2011. Art#52

LETTER TO EDITOR (VIEWERS CHOICE) A RARE CASE OF KINGAE OF LEFT FEMUR IN A NEWBORN

Rahul Sinha

A 3.5kg term female neonate was delivered to 24 in a high risk of concomitant in these yrs old primigravida by emergency cesarean section joints (3). The most commonly affected joints are the (for breech with meconiun stained liquor). Baby was hip joints (31%), knee joints (25%), and ankle joints well till day 4 of life when she developed high grade (18%) (4). Affected neonates usually survive, but with fever, lethargy, poor feeding and swelling over left permanent skeletal deformities (5). Etiologically, the upper third of thigh. She was shifted to neonatal most common organisms affecting the joints in the intensive care unit (NICU) and detailed examination neonatal period are aerobes. The predominant aerobes revealed unhealthy umbilicus with marked swelling, causing osteomyelitis in children are Staphylococcus tenderness and fluctuation of left upper third of thigh aureus, influenzae type-b, Gram-negative with marked restriction of movements at left hip joint. enteric , beta-haemolytic Streptococci, Investigations revealed hemoglobin of 15gm%, white pneumoniae, henselae and cell count of 34,000/cmm with polymorphs 76% and Borrelia burgdorferi. Anaerobes have rarely been platelets of 2,80,000/cmm. Peripheral smear showed reported as a cause of these infections in children. toxic granules, CRP was 24 mg/dl, ESR 40 mm at Many patients with osteomyelitis due to anaerobic end of 1hour. X ray of left femur showed a significant bacteria have evidence of anaerobic infection elsewhere periosteal reaction in the proximal humerus, along in the body, which is the source of the organisms with an irregularity at the proximal metaphysis. The involved in osteomyelitis (6,7). Kingella kingae often umbilical swab and local aspirate grew kingella kingae. colonizes the oropharyngeal and respiratory tracts of She was treated meropenem and vancomycin for 3 children but infrequently causes invasive disease (7). weeks and analgesics along with skin traction for 7 K. kingae is a slow-growing, fastidious, gram-negative days. microorganism that colonizes mucous membranes of Osteoarticular infections, although uncommon, the upper respiratory tract (8). To our knowledge this represent a severe condition in neonates. Infections in is the first reported case of Kingella osteomyelitis in newborns are largely of an acute nature, transmitted early neonatal period from India. by haematogenous means (1). The osteomyelitic focus is usually found in the metaphysis of a long REFERENCES bone, although infection may spread to the contiguous 1. Dessi A, Crisafulli M, Accossu S, Setzu V, Fanos V. Osteo- epiphysis and joint in neonates (1,2). The intracapsular articular infections in newborns: diagnosis and treatment. metaphysis of proximal femur and humerus, results J Chemother 2008; 20: 542-550

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