Kwashiorkor e distúrbio de coagulação: apresentação atípica de fibrose cística / Kwashiorkor and coagulation disturbance: atypical presentation of cystic fibrosis

Enfatizar a apresentação clínica precoce da fibrose cística (FC) em lactente com Kwashiorkor e distúrbio de coagulação, decorrente de hipovitaminose K. DESCRIÇÃO DO CASO: Paciente com três meses e meio, sexo feminino, nascida a termo, peso de 2655g, estatura de 46cm, foi encaminhada para investigação de lesões perineais associadas à monilíase de difícil controle, refratária a diversos antifúngicos e corticoides. Quadro geral caracterizado por baixo ganho ponderal, edema e diarreia. Admissão hospitalar para investigação com hipótese diagnóstica de Kwashiorkor de origem primária ou secundária. Paciente mantida em aleitamento materno exclusivo, sendo observadas perda ponderal e persistência da diarreia. Na internação, foi iniciado tratamento de infecção do trato urinário. A paciente evoluiu com hemorragia digestiva alta e sangramento pela flebotomia em safena direita, sendo identificada coagulopatia responsiva à vitamina K e plasma fresco congelado. Na evolução, foi confirmada esteatorreia e hipoalbuminemia; as sorologias para sífilis, toxoplasmose, mononucleose, citomegalovírus, rubéola, HIV e hepatite B, apresentaram resultado negativo e a pesquisa da mutação ∆F508 heterozigoto para FC foi positiva. A paciente apresentou piora do estado geral com sinais de sepse, evoluindo para óbito. O laudo necroscópico evidenciou elementos característicos de choque séptico com infecção pulmonar, sinais acentuados de desnutrição e fibrose cística do pâncreas. COMENTÁRIOS: A FC pode manifestar-se com quadro de Kwashiorkor e distúrbio de coagulação por deficiência de vitamina K. Os profissionais de saúde devem estar atentos à possibilidade de FC no diagnóstico diferencial dessa situação.

To address the clinical presentation of cystic fibrosis (CF) in an infant presenting Kwashiorkor along with coagulation disturbance due to vitamin K deficiency. CASE DESCRIPTION: A female baby aged three and a half months, born at term, with birth weight of 2655g, and height of 46cm, was referred to a university center due to perineal moniliasis refractory to therapy, including antifungal drugs and corticosteroids. She had poor weight gain, edema, and diarrhea. After hospital admission under the diagnostic hypothesis of Kwashiorkor of primary or secondary origin, the child received exclusive breastfeeding, but lost weight and maintained the diarrhea. At admission, a urinary tract infection was detected and treated. The child developed bleeding of upper digestive tract and phlebotomy incision at the right saphenous vein treated with vitamin K and fresh frozen plasma. Laboratory exams showed steatorrhea and hypoalbuminemia. Serology was negative for syphilis, toxoplasmosis, mononucleosis, cytomegalovirus, rubella, HIV and hepatitis B. Heterozygous ∆F508 mutation for CF was positive. The patient died with a septic shock. Necropsy showed that the septic shock had a pulmonary origin and that malnutrition was secondary to cystic fibrosis of pancreas. COMMENTS: CF may have a clinical presentation as Kwashiorkor with coagulation disturbance caused by vitamin K deficiency. Health professionals should be aware of this possibility in the differential diagnosis of infants with severe malnutrition and edema.

Prevalence of HIV infection and AIDS symptomatology in malnourished children--a hospital based study.

One hundred and seventy five malnourished children aged between 1(1/2) and 12 years attending pediatric department of Regional Institute of Medical Sciences Hospital, Imphal from January 2001 to June 2002 were screened for human immunodeficiency virus (HIV) infection along with their biological mothers after pretest counselling and informed consent. The prevalence rate of HIV seropositivity among malnourished children was 21.7%. Children aged between 1(1/2) and 3 years had the highest seroprevalence (47.4%) and male to female ratio was 1.5: 1. Underweight children showed the highest seroprevalence (47.4%) and children with kwashiorkor showed least seroprevalence (10.5%). Mode of HIV transmission was vertical in 94.7%. The causative agent was HIV-I in all the cases. AIDS defining children features were seen more frequently among HIV seropositive malnourished children as compared to the seronegative children. Prolonged fever (p 0.001), oropharyngeal candidiasis (p<0.001), generalised lymphadenopathy (p<0.001) and disseminated maculopapular dermatitis (p<0.001) were significantly related to HIV infection. Among seronegative children 18.2% fulfilled the clinical criteria for AIDS and among seropositive children 94.7% had AIDS. The total mortality encountered among seropositive children was 34.2%. It is suggested to confirm findings based on larger community based data before recommending mandatory HIV testing in all malnourished children. Specific guidelines on the nutritional management of children with HIV/AIDS is needed in Manipur where HIV is spreading rapidly.

Parkinsonian-like tremors in the recovery phase of kwashiorkor

Kwashi shakes is described in a 17-month-old Jamaican male infant. This is the first reported case seen at the Tropical Metabolism Research Unit at the University of the West Indies, Mona, Jamaica an the first documented case in the West Indian literature

Microbiological agents in Napkin dermatitis

Isolation and identification of fungus and bacteria in napkin was studied. Sampling of the skin in the diaper area was carried out in 24 infants with gastroenteritis, 18 cases of malnutrition and 25 well nourished infants used as controls. Direct microscopical examination of the gram stained film, BR for gram negative bacilli and coagulase test for staphylococci was done