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Neonatal hypoglycaemia and nesidioblastosis
772 Neonatal
ABSTRACTS Hypoglycaemia
and Nesldioblastoslso
Leading
GENITOURINARY TRACT
article. Lancet 1:193-194 (January), 1978 The author discusses inappropriate insulin secretion, which occurs in 20%-33% of persistent neonatal hypoglycemia, and the underlying condition of nesidioblastosis. The term nesidioblastosis describes a diffuse proliferation of the islet cells of the pancreas, usually affecting the whole gland. It is characterized histologically by lack of normal islets, by clusters of endocrine cells about exocrine ducts, and by microadenomata. Medical treatment with diazoxide or protamine zinc somatostatin may fail, and surgical resection of the pancreas may be necessary. The author reviews the work of Heitz et al., who have shown that four types of endocrine cells participate in the proliferation, namely B, A, D, and pancreatic polypeptide. Their ratio appears to be constant in the differing morphologic patterns of endocrine proliferation. Endocrine parenchyma is increased fivefold in nesidioblastosis, as compared with controls, and probably represents failure of the normal mechanisms controlling embryogenesis of the endocrine pancreas and its transition to the normal neonatal state. The author suggests that a systematic analysis of all hormones known to regulate pancreatic function may reveal the identity of the controlling factor whose abnormality is responsible for the uncontrolled expansion of the islet cells.--J. G. Harvey
Immune Response After Splenectomy. J. L. Sullivan, H. D.
Ochs, G. Schiffman, 114. R. Hammerschlag. J. Miser, E. Vichinsky, and R. J. Wedgwood. Lancet 1:178-181 (January), 1978
The authors report the humoral immune response to intravenously administered QX174 bacteriophage and to subcutaneous tridecavalent pneumococcal polysaccharide vaccine in 31 patients age 2-49 yr who had undergone previous spleneetomy secondary to trauma or as a consequence of congenital spherocytosis, idiopathic thrombocytopenic purpura, portal hypertension, and Hodgkin's disease. Their results show normal clearance of antigen from the serum, but an abnormal primary response to phage QX174. Secondary responses are quantitatively normal, but they fail to show conversion from IgM to IgG response. The depression of the immune response is particularly marked in patients with Hodgkin's disease, All groups other than Hodgkin's disease showed significant and normal seroconversion to subcutaneous injection of pneumococcal antigen. The results confirm previous studies showing deficient antibody responses to intravenously administered heterologous red blood cells and confirm the previous findings of normal responses to subcutaneously administered polysaccharide antigen. In conclusion, the authors state that the spleen is necessary for normal immune response to blood-borne antigens but that subcutaneous challenge circumvents the need for the splenic response. In view of the frequency of Streptococcus pneumoniae in overwhelming postsplenectomy infection, the authors suggest that all patients with anatomic or functional asplenia should receive prophylactic pneumococcal polysaccharide vaccine.--J. G. Harvey
Congenital Unilateral MulUcystic Kidney. T. Kishikawa, H.
Tsunooka, S. Sasaki, et aL J Jpn Soc Pediatr Surg 14:457-468 (April), 1978
Eight patients with unilateral multicystic kidney are reported (1 male and 7 female). Abdominal masses were found at the age of 10 days in 5 patients, 1 mo in 1 patient, 3 mo in 1 patient, and 5 mo in 1 patient. The affected side was the left in 5 patients and the right in 3 patients. The ureter of the affected kidney was normal in 1 patient but absent or hypoplastic in 7 patients. The contralateral kidney was normal in each of these 8 patients. The authors collected 59 patients with unilateral multicystic kidney from the J a p a n e s e literature. Twenty-seven were male, 25 were female, and sex was not described in 7 patients. The affected side was the left in 28, the right in 23, and not specified in 8 patients. The ureter of the affected kidney was normal in 1 patient but absent or hypoplastic in 42 of 59 patients.--H. Suzuki Spectrum of MuIticysUc Renal Dysplasia. D. Walker, R.
Fennell, E. Garin, and G. Richard. Urology 11:433-436 (May), 1978
The entity of multicystic kidney is expanded to the concept of multicystic renal dysplasia. Presenting symptoms, pathogenesis, diagnostic modalities, and management possibilities are reviewed. Presentation in the newborn is almost always an abdominal transilluminable mass. In older children, incidental discovery (or, occasionally perfusion) is responsible for detection. The authors have reviewed their experience with multicystic dysplasia in 11 patients, correlating their findings with the most up-to-date thoughts in this area. An embryogenic theory is proposed, based both on early in utero ureteral obstruction and on abnormal ureteral budding. Release of an inducer substance normally responsible for metanephros differentiation results in multicystic dysplasia. Late fetal ureteral obstruction results in hydronephrosis. The diagnosis is usually readily established using intravenous urography with total body opacification and sonography. The renal scan is useful for differentiating the poorly functioning hydronephrotic kidney from the nonfunctioning cystic dysplastic kidney. Focal or segmental dysplasia is more difficult to distinguish, and the diagnosis is usually made at the time of surgery. Traditionally, management has been surgical removal of the affected kidney; however, a nonoperative approach may have merit in some forms of segmental or focal dysplasia. Surgical exploration is indicated, however, if there is any question of the diagnosis.--M. David Gibbons Clinical Features
and Treatment
of Urinary Calculi In
Childhood. W. Vahlensieck and H. P. Bastian. Eur Urol
2:129-134, 1976 During the period 1926 to 1975, 94 children were treated for urinary calculi. Eighty percent had upper tract calculi. Forty-four children were idiopathic stone formers. Malformations were responsible for calculi in 36 patients.
ABSTRACTS Hypoglycaemia
and Nesldioblastoslso
Leading
GENITOURINARY TRACT
article. Lancet 1:193-194 (January), 1978 The author discusses inappropriate insulin secretion, which occurs in 20%-33% of persistent neonatal hypoglycemia, and the underlying condition of nesidioblastosis. The term nesidioblastosis describes a diffuse proliferation of the islet cells of the pancreas, usually affecting the whole gland. It is characterized histologically by lack of normal islets, by clusters of endocrine cells about exocrine ducts, and by microadenomata. Medical treatment with diazoxide or protamine zinc somatostatin may fail, and surgical resection of the pancreas may be necessary. The author reviews the work of Heitz et al., who have shown that four types of endocrine cells participate in the proliferation, namely B, A, D, and pancreatic polypeptide. Their ratio appears to be constant in the differing morphologic patterns of endocrine proliferation. Endocrine parenchyma is increased fivefold in nesidioblastosis, as compared with controls, and probably represents failure of the normal mechanisms controlling embryogenesis of the endocrine pancreas and its transition to the normal neonatal state. The author suggests that a systematic analysis of all hormones known to regulate pancreatic function may reveal the identity of the controlling factor whose abnormality is responsible for the uncontrolled expansion of the islet cells.--J. G. Harvey
Immune Response After Splenectomy. J. L. Sullivan, H. D.
Ochs, G. Schiffman, 114. R. Hammerschlag. J. Miser, E. Vichinsky, and R. J. Wedgwood. Lancet 1:178-181 (January), 1978
The authors report the humoral immune response to intravenously administered QX174 bacteriophage and to subcutaneous tridecavalent pneumococcal polysaccharide vaccine in 31 patients age 2-49 yr who had undergone previous spleneetomy secondary to trauma or as a consequence of congenital spherocytosis, idiopathic thrombocytopenic purpura, portal hypertension, and Hodgkin's disease. Their results show normal clearance of antigen from the serum, but an abnormal primary response to phage QX174. Secondary responses are quantitatively normal, but they fail to show conversion from IgM to IgG response. The depression of the immune response is particularly marked in patients with Hodgkin's disease, All groups other than Hodgkin's disease showed significant and normal seroconversion to subcutaneous injection of pneumococcal antigen. The results confirm previous studies showing deficient antibody responses to intravenously administered heterologous red blood cells and confirm the previous findings of normal responses to subcutaneously administered polysaccharide antigen. In conclusion, the authors state that the spleen is necessary for normal immune response to blood-borne antigens but that subcutaneous challenge circumvents the need for the splenic response. In view of the frequency of Streptococcus pneumoniae in overwhelming postsplenectomy infection, the authors suggest that all patients with anatomic or functional asplenia should receive prophylactic pneumococcal polysaccharide vaccine.--J. G. Harvey
Congenital Unilateral MulUcystic Kidney. T. Kishikawa, H.
Tsunooka, S. Sasaki, et aL J Jpn Soc Pediatr Surg 14:457-468 (April), 1978
Eight patients with unilateral multicystic kidney are reported (1 male and 7 female). Abdominal masses were found at the age of 10 days in 5 patients, 1 mo in 1 patient, 3 mo in 1 patient, and 5 mo in 1 patient. The affected side was the left in 5 patients and the right in 3 patients. The ureter of the affected kidney was normal in 1 patient but absent or hypoplastic in 7 patients. The contralateral kidney was normal in each of these 8 patients. The authors collected 59 patients with unilateral multicystic kidney from the J a p a n e s e literature. Twenty-seven were male, 25 were female, and sex was not described in 7 patients. The affected side was the left in 28, the right in 23, and not specified in 8 patients. The ureter of the affected kidney was normal in 1 patient but absent or hypoplastic in 42 of 59 patients.--H. Suzuki Spectrum of MuIticysUc Renal Dysplasia. D. Walker, R.
Fennell, E. Garin, and G. Richard. Urology 11:433-436 (May), 1978
The entity of multicystic kidney is expanded to the concept of multicystic renal dysplasia. Presenting symptoms, pathogenesis, diagnostic modalities, and management possibilities are reviewed. Presentation in the newborn is almost always an abdominal transilluminable mass. In older children, incidental discovery (or, occasionally perfusion) is responsible for detection. The authors have reviewed their experience with multicystic dysplasia in 11 patients, correlating their findings with the most up-to-date thoughts in this area. An embryogenic theory is proposed, based both on early in utero ureteral obstruction and on abnormal ureteral budding. Release of an inducer substance normally responsible for metanephros differentiation results in multicystic dysplasia. Late fetal ureteral obstruction results in hydronephrosis. The diagnosis is usually readily established using intravenous urography with total body opacification and sonography. The renal scan is useful for differentiating the poorly functioning hydronephrotic kidney from the nonfunctioning cystic dysplastic kidney. Focal or segmental dysplasia is more difficult to distinguish, and the diagnosis is usually made at the time of surgery. Traditionally, management has been surgical removal of the affected kidney; however, a nonoperative approach may have merit in some forms of segmental or focal dysplasia. Surgical exploration is indicated, however, if there is any question of the diagnosis.--M. David Gibbons Clinical Features
and Treatment
of Urinary Calculi In
Childhood. W. Vahlensieck and H. P. Bastian. Eur Urol
2:129-134, 1976 During the period 1926 to 1975, 94 children were treated for urinary calculi. Eighty percent had upper tract calculi. Forty-four children were idiopathic stone formers. Malformations were responsible for calculi in 36 patients.