Suspected Severe Malaria in a Sudanese Patient Affected by Sickle Cell Disease Who Was Treated with Hydroxyurea.

Sickle cell disease (SCD) is the most common genetic disease in sub-Saharan Africa. The signs and symptoms of SCD usually begin in early childhood. Characteristic features of this disorder include anaemia, repeated infections, and periodic episodes of pain. Malaria is one of the infections that can occur in patients with SCD in endemic countries. Many guidelines recommend antimalarial chemoprophylaxis in these patients, although the debate on which drug should be used is still ongoing. Hydroxyurea (HU), which is considered a safe and effective treatment for both children and adults with SCD, seems to affect the incidence and severity of malaria, although these impacts have yet to be fully demonstrated. We report a case of an eight-and-a-half-year-old Sudanese boy with SCD treated with HU admitted for suspected severe malaria who showed a recrudescence after first-line treatment. Although he had undergone splenectomy and thus belonged to a category of patients at high risk for infectious complications, he was not receiving any malaria chemoprophylaxis. This case emphasises the importance of the routine administration of malaria prophylaxis to children with SCD living in endemic areas, even when they are treated with HU, and especially if they are at high risk for infectious complications because they have undergone splenectomy. There is an urgent need for further research to evaluate the most appropriate regimen and its optimal duration.

Three case reports of post immunization and post viral Bullous Pemphigoid: looking for the right trigger.

BACKGROUND: Bullous pemphigoid (BP) is a blistering skin disorder infrequent in infancy and rarely reported in medical literature. CASE PRESENTATION: Here we describe three cases of BP which were referred to our department in the last 15 years. Two of them developed an eruption of bullous lesions just a few days after vaccination for diphtheria, tetanus, pertussis, poliomyelitis, hepatitis B and Haemophilus influenzae B. The third patient developed the same blistering lesions shortly after herpetic stomatitis. In all three cases, clinical diagnosis was confirmed by histological examination which showed subepidermal bullae with a dermal inflammatory infiltrate, and direct immunofluorescence of perilesional skin showed linear IgG and C3 deposits along the basement membrane zone. Immunoblot assay was positive for BP antigen 180. Treatment with oral prednisone was instituted and the lesions resolved in two out of three patients; the third one was treated with an immunosuppressive agent (tacrolimus) and corticosteroid and subsequently with intravenous immunoglobulin and plasmapheresis, due to an underlying complex autoimmune disease. CONCLUSION: Although the mechanism of induction of BP is still unclear, the close relationship between trigger events (immunization or viral infection) and onset of the disease arises a possible association.

Aplasia cutis congenita: an association with vanishing twin syndrome.

Aplasia cutis congenita (ACC) is an inborn skin defect, variably associated with absence of the underlying subcutaneous fat and bone. Frieden IJ proposed a classification system for ACC consisting of 9 groups according to lesion distribution and associated malformations. We report a child resembling ACC group 5, with extensive truncal and limb skin defects and fetus papyraceous, the so-called vanishing twin syndrome. According to our experience, the risk of developing ACC with fetus payraceous is unpredictable, although twin abortion occurred during the first weeks of gestation. This case emphasizes the importance of a team approach to patients affected by wide skin lesions, and the usefulness of a precise anamnesis in order to clarify the etiology of this rare but often dramatic disorder.

Methemoglobinemia caused by the ingestion of courgette soup given in order to resolve constipation in two formula-fed infants.

Methemoglobinemia is not a rare condition arising from the exposure to hemoglobin-oxidizing agents such as nitrates-nitrites present in well water or vegetables. Infants < 3 months of age are more susceptible than adults because of lower amounts of a key enzyme, NADH-cytochrome b5 reductase, which converts methemoglobin back to hemoglobin. We report 2 infants, aged respectively 2 and 1 months, suffering from methemoglobinemia, fed with a formula that was reconstituted with a high concentration of courgette soup to resolve constipation. They developed a severe cyanosis with methemoglobinemia (respectively 30.4 and 27%) and were hospitalized and treated with methylene blue at 1%. After 12 h the syndrome was completely resolved. Home-prepared infant foods containing vegetables are potential causes of methemoglobinemia. It is important not to feed infants with vegetables having a high nitrate content (e.g., courgette, spinach, beets and green beans) to resolve constipation since, particularly in the first months of life, they may cause severe methemoglobinemia.

Advances in the management of digestive problems during the first months of life.

UNLABELLED: A study was carried out on 168 full-term infants with digestive problems such as regurgitation and/or constipation to evaluate the efficacy of new infant formulas containing partially hydrolysed whey protein, modified vegetable oil with a high beta-palmitic acid content, prebiotic oligosaccharides and starch. Infants receiving the new formulas had an increase in stool frequency of 0.60 between day 1 and day 7 (95% CI 0.19-1.01; p=0.004) and 0.53 (95% CI 0.11-0.90; p=0.015) between day 7 and day 14. A reduction of 1.06 in the number of regurgitation episodes was reported between day 1 and day 7 (95% CI 0.24-1.88; p=0.012) and 1.31 (95% CI 0.42-2.21; p=0.005) between day 7 and day 14. CONCLUSION: A prebiotic mixture of galacto-/fructo-oligosaccharides with a high beta-palmitic acid content may reduce digestive problems and improve intestinal tolerance in infants during the first months of life.